Part 19. The Respiratory System

PART 19. RESPIRATORY SYSTEM

section 1. development and function

# respiratory system의  development.

    1. morphogenesis or formation of all necessary structures.

    2. adaptation to postnatal atmospheric breathing.

    3. dimensional growth.

Chapter 319. Development Of Respiratory System

Prenatal Development : Morphogenesis

1) embryogenic period

    ; begin *4wk of gestation

    ; peribronchial mesenchyme or splanchnopleura가 이 기간에 lung형성에 필수적인 역할.

2) *pseudoglandular period

    ; 6-16wks

    ; *TEF, CDH(Bochdale)의 congental malformation 이 시기에 생김

3) canalicular period

    ; 16-28wks

    ; accessory structure（cart,.gl,m.）완성typeⅠⅡpneumocytes.

4) saccular period

    ; 28-36wks

    ; terminal sac 완성

5) alveolar period

    ; *after 36주

  ;  at birth 50 million alveoli→adult 300 million.

    ; acinus형성에 fetal lung내의 liguid에 의한 stretch와 respiratory muscle의 period distension이 필수적.

    ; lung or chest compress（diaphragmatic hernia or oligohydramnios）or fetal breathing abolished（spinal cord lesion）시 pulmonary hypoplasia.

Adaptation To Air Breathing.

# Pulmonary Surfactant

  ; *hetergenous mixture of phospholipid and protein

    ; synthesis by typeⅡ pneumocyte

           - first recognized *as early as 24wks

           - *not detectable in amniotic fluid until 30wks : chronologic gap

    ; apoprotein：3가지 type （SP-A, SP-B, SP-C）

    ; glucocorticoid

           - apoprotein ＆lipid 합성↑

           - parenteral administration to prevent RDS

  ; thyroid H

           - phospholipid 합성↑

  ; β-adrenergic agonists ＆ other agent

           - apoprotein ＆phosphatidylcholine secretion↑

    ; ★insulin, hyperglycemia, ketosis, androgen

           - *surfactant 합성에 negative effect

    - DM mother의 infant or male infant에서 respiratory distress syndrome high incidence.

# pu.l resistance

    ; high→low resistance

    ; 원인

           ① lung tissue expanding에 의해 pulmonary vascular wall에 mechanical forces.

           ② alveola의 oxygen 농도에 의해 pul arterial smooth muscle의relaxation, vasodilator의 endogenous release.

    ; foramen ovale ＆ ductus arteriosus가 closure되므로 pulmonary와 system circulation 완전히 분리

Postnatal Development

first phase：① 출생후∼18개월

② gas exchange에 관여하는 compartment의 surface ＆ volume↑

③ air space vol보다 capillary vol이 더 많이↑

second phase：① alveolar ＆ capillary surface↑.

Chap 320. ★Regulation Of Respiration

The Respiratory Control System Is Negative Feedback System With A Central Controller

   · Co2↑시 ventilation 증가시켜 Co2 ↓.

   · feedback system

       afferent limb : airway, carotid 같은 receptor ending을 가지는 tissue

       efferent limb : respiratory muscle and their innervation

                      ex) intercostal m, diaphragm, airway m.

The Central Controller Integrates Incoming Afferent Information And Generates And Maintains Respiration

1) respiratory controller는 아마 neuron group이 emergent network를 형성하          든지, endogenous or conditional burster일 것이다.

     2) medullary neuron. two group : nucleus tractus solitarius

                                      nucleus ambiguus

        ⇒ respiration initiation의 potential site

     3) medulla (pre-B  tzinger) : respiration 조정에 중요

Afferent Information In Not Necessary For Initiating Respiration But Plays An Important Role In Modulating Breathing

    1）peripheral nerve sys

     （1）larynx ＆ upper airway의 chemoreceptor ＆ mechanoreceptor

        ：mucosa에 stretch, air temp, chemical change

        →superior laryngeal nerve ＆ 10th cranial nerve.

     （2）Carotid ＆ aortic bodies：o2 or co2 tension

     （3）Skin or mucosal receptor hypothalamic neuron：thermal or  metabolic             change

    2）high brain center

     （1）medulla oblongata의 ventral surface：co2 sense

     （2）limbic system

          ：emotion ＆ changes in mood

Centeral Integration And Processing In The Brain Stem Hierachical

    ·다양한 요구에 의한 neurophysiologic signals이 있을때,

        central  controller는 stimuli에 따라 반응을 향상시키기로 하고 감쇄시키기           도 한다.

      ex）trigeminal afferent impulse는 REM sleep or 깨어 있을때 보다

          quiet sleep시에 cortical influence을 덜 받아 더 respiration에           

          trigeminal stimulation이 저명해 진다.

Respiratory Muscles And Chest Wall Properties(E.G. Efferent Organ) Undergo Postnatal Maturation And Can Fatigue

    ·respiratory muscle innervation의 maturational aspect

     +-adult-one muscle은 one motoneuron애 의해 지배

     |

     +-newborn-polyneuronal innervation

    ·newborn에서 adult에 비해 end plate 내에 acetylcholine content적어

      diaphragm의 neuromuscular transmission susceptibility↓

    ·newborn infant의 chest wall이 아주 complaint해서

        REM sleep에 특히 paradoxic respiration

      →어떤이는 이것이 muscle fatigue와 respiratory failure가 newborn에서

        잘 생기는 중요한 이유라고 생각

The Newborn And Young Respond Differently To Stimuli Compared With Mature Subject

    ·O2 low에 대한 반응이 neonate와 adult에서 차이가 있는데,

      이유：1）hypoxia 동안 metabolic rate차이

            2）lung ＆ airway의 mechanical property의 차이

            3）centeral neuron의 cellular ＆ membrance property의  alteration

Clinical Implications

Apnea

; 각각의 age의 infant or child의 mean breath time에 3 standard deviation 이상 resp. pause가 있을때

    （2）adult보다 O2 consumption이 높고, 비교적 lung vol이 적으며, O2

         store가 적어서very young or premature에서는 short resp pause에도

         serious consequence을 가져올 수 있다.

    （3）sleep 〉waking state      ---+          

                                      | apnea발생빈도↑        

         REM sleep 〉quiet sleep  ----+          

Upper Airway Obstruction

    （1）children

      ①cause：·tonsillar ＆ adenoidal hypertrophy

                 due to repeated upper resp infaction

               ·craniofacial malformation

               ·micrognathia

               ·muscular hypotonia

    （2）adulat： 불분명

    （3）UAO의 usual site ：oropharynx

                             （ post Pharyngeal wall, soft palate, genioglossus                                  사이）

    （4）sleep 동안 long-standing sign ＆ Sx of UAO

        →Rt ventricular failure ＆ cor pulmonale

Chapter 321. Respiratory Function ＆ Approach To Respiratory Disease

Mechanism Of Breathing

   1. Elastic properties of the respiratory system and restrictive disaese

    1）resp.system이 elastic behavior 보이는 이유

     （1）air space 내에 surfactant가 정상적인 양이 들어 있어도 surface tension             은 lung의 elastic recoil의 65％ 정도 기여한다.

        →surfactant없으면 elastic recoil이 markedly increase하며       

          atelectasis 유발：respiratory distress syndrome of the newborn

     （2）lung ＆ chest wall의 fibrous network가 elastic property가짐.

         →pul.fibrosis, lung overdistention

    2）restrictive lung disease cause

     （1）surface tension이 비정상적으로 증가

         →respiratory distress synd of the newborn.

     （2）interstitial edema, pneumonitis, fibrosis 같이 lung의 soild

          structure or composition의 change

     （3）alveolar edema, pneumonia 같이 alveolar space가 liquid or

          inflammatory cell로 차있을때

     （4）restrictive chest wall

          ：abdominal distention, cong malformation, neuromuscular

            disease

  2. Resistive properties of the respiratory system

                                   and obstructive disease.

    （1）motion에 의해 유발된 molecular interaction 결과로 resistive

         생각

  3. Response to respiratory disease：efficiency of the developing

                                    respiratory system.

  1）respiratory work load ↑시 2type responses

   （1）respiratory muscle contraction 증가

        →substrate availability와 muscle의 maximal contraction force

          ability에 의해 제한

   （2）respiratory pattern을 변화시켜 respiratory efficiency↑

Gas Exchange

pulmonary capillary and the neighboring portion of the air space로 구성된 몇 백만의 small unit에서 생긴다.

# PCO2

    ; *propotional to carbon dioxide production and inversly propotional to alveolar ventilation

# aleveolar ventilation

    ; minute ventilation - dead space ventilation

# PO2

# *regional differences in ventilation/perfusion ratios exist in normal lungs

# respiratory disease

    ; *increase alveolar-arteriolar PO2 difference

PaO2  =  PIO2 - PaCo2／0.8

PIO2：partial pressure of inspired oxygen

P2Co2：arterial partial pressure of carbon dioxide

0.8：respiratory quotient（carbon dioxide production／oxygen

     consumption）

321.1 Diagnostic Approach To Respiratory Disease

Physical Examination

   1. breathing pattern의 alteration

     ：breathing rate 증가, chest wall retraction, nasal flaring

   2. restrictive disease：breathing rate↑

                        expiratory grunt

     obstructive disease：slover deeper breaths

                         extrathoracic의 경우 inspiratory stridor

  3. stridor

    rales or crackles

    wheezes

Blood Gas Analysis

  1. cyanosis：skin perfusion and blood hemoglobin concentration

              영향받음

              hypoxia의 unreliable sign.

  2. arterial Po2, PCo2, PH의 direct measurement

  3. skin surface Po2 determination으로 arterial oxygenation 측정시

    skin perfusion에 영향받음.

Respiratory Function Test

1. restrictive disease

  ; typically total lung capacity （TLC）의 감소

    →TLC는 residual vol포함하므로, gas dilution method,

      plethysmography로 indirectly measure.

  ; vital capacity（VC）감소

    →spirometry로 측정

2. obstructive disease

  ; residual vol ↑ functional residual capacity↑

Ventilation／Perfusion Studies

    ：radionuclide tracer에 의해 도움받음.

Exercise Testing

Sleep Studies

1. newborn young infant에서 특히 respiration function에 중요한 영향을 미친다.

2. Polysomnographic studies：

     1）central respiratory control abnormality

     2）muscle disorder

     3）gastroesophageal reflux에 의한 resp. complication시 유용.

Chapter 322. Resp. Failure

Etiology

1) intercurrent acute illness (eg. influenza)

2) 이전의 건강한 아이

  ① pn.

  ② epiglottitis

  ③ status asthmaticus

  ④ aspiration

  ⑤ poisoning

  ⑥ severe heart failure

3) cystic fibrosis or severe scoliosis, bronchopulmonary dysplasia

4) acute CNS ds.

5) severe m. ds & thoracic abnormality

6) CHD with large Rt. to Lt. shunt

Clinical Manifestation and Diagnosis

      ·sit up & lean forward

     1) Hypercapnia → central depression : impaired consciousness

                                          & confusion

     2)+- PaCO2 40mmHg↑→ ARF 가능성

       |  PaCO2 50mmHg↑→ imminent

       +- PaCO2 55mmHg↓ in room air        blood의 O2 content가 vital

           organ의 요구를 충족시키기에 부적당

     3) ① severe headache

            acute hypoxemia & hypercapnia

          → dilat of cerebral blood의 O2 content가 vital organ의 요구를

               충족시키기에 부적당

        ② cold extremites

        ③ restlessness

        ④ dizziness

        ⑤ impaired thought

     4) multisystem Cx. i) GI hemorrhage

                        ii) cardiac arrhythmia

                        iii) renal failure

                        iv) malnutrition

Treatment

     1) Theophylline : improve diaph. strength

                      COPD pt에 유용

     2) mecha. vent. : pneumonia child에서 hypoxia & hypercarbia

                      develop시

     3) 100% O2 by bag & mask/endotrach. intubation

         : apnea나 gasping시

            * PEEP : alv. collpse방지

            * Goal of Tx

               i) 적당한 oxygen saturation 유지

               ii) n'l PaCO2 유지 (using 최소한의 pr. & lowest FiO2)

     4) Bedside measurement of tidal vol. & VC & (-)inspiratory force

        * direct O2 toxicity의 risk는 Fi O2 70∼100%시 매우↑

           (40%↓에서 증명)

Chapter 323. Defence Mechanism Of Lung

# infancy이후 collateral alveolar ventilation이 일어남

  a) pore of Kohn between alveoli

     - lobule 사이 or caillary 사이의 gas passage 되게 함

  b) canal of Lambert beetween bronchiol & alveoli

     → atelectasis 방지

# Defense  a) filtering of particle

           b) warming & humidify of inspired air

           c) noxious fame & gas를 vascular upper airway에서 흡수

           d) temporary cessation of breathing   

              shllow breathing                

              laryngospasm or bronchospasm   

              swallowing & closure of epiglottis 

Clearance Of Particle

      1) mucocillary mech에 의해

      2) phagocytosis by alv. macrophage

Defense Against Microbial Agent

      - phinciple by macrophage

         phagocytosis Ab in resp. secretion - Ig A

                                           - 1Mo에 adult level에 도달

      ·lysozyme, lactoferrin, interferon도 defense role

Impaired Defense Mechanism     

      1) macrophage phagocytic activity & mucociliary clearance↓

        a) ethanol  

        b) smoking  

        c) hypoxemia starvation chilling

        d) corticosteroid nitrogen dioxide ozone narcotics some anesthetics

      2) antibacterial killing capacity of macrophage↓

        a) acidosis    b) azotemia

        c) recent viral inf. - 특히 rubella, influenza

      3) toxic to epi. cell

         - beryllium, asbestos, organic dust from cotton & sufarcan

         - sulfur, N20, ozone, chlorine, ammonia, cigar

      4) mucociliary clearance↓

         a) hypothermia & hyperthermia

         b) morphine, codeine

         c) hypothyroidism

      5) resp. epi. damage

         a) reversible - rhinitis, sinusitis, bronchitis, bronchiolitis

                        resp. inf. with air pollution

                        asthma, bronchospasm, edema, congestion

                        mild surf. ulceration

         b) permanent - ① servere ulcer

                        ② bronchiectasis bronchiolectasis

                        ③ squamous cell metaplasia & fibrosis

         c) other adverse effect : hyperventilation, alveolar hypoxia,

                 pulmonary thromboembolism, alveolar, pulmonary

                 thromboembolism, pul edema, hypersensitivity reaction,

                 salicylate 같은 agent

323.2 Metabolic Function Of Lung

   1) (1)Lung은 40개의 cell type 가짐

        이 중 lung에만 특수한 것  +-① type I,II pneumocyte

                                  | ② alv. macrophage

                                  +-③ clara cell

      (2)lung이 합성하는 lipid & protein

         ① glycoprotein,

         ② secretory

         ③ interferon

         ④ proteolytic & fibrinolytic enzyme & activator

         ⑤ collagen

         ⑥ elastin

      (3) ① thromboplastin   ⇒ lung에 많음

         ② megakaryocyte    

      (4)① serotonin         

         ② bradykinin         ⇒ 대개 pul. circulation을 통해

         ③ ATP                  완전 제거 or inactivation

         ④ PGE1 E2 E3     

      (5)① EPi

         ② PGA1 A2          ⇒ minimally affected

         ③ Angiotensin 2

         ④ vasopressin

Chapter 324. Diagnostic Procedures

Radiologic Technique

Chest X-Ray

CT & MRI

Upper Airway Film

 xerography soft tissue

Sinus, Nasal Films

Fluorscopy

   * useful (1) stridor시

            (2) abn'l move. of diaph. or mediat

            (3) needle asp. or biopsy of peri. lesion

Contrast Study

Barium swallow

    ; Ix  

           ① recurrent pn.

    ② 원인 불명의 persistent cough

    ③ stridor

    ④ persistant wheezing

eg. H-TEF시 simple barium swallow는 불충분

     cath.를 통해 eso.의 몇 부위에 barium inj.

if eso. atreesia 의심시 0.5ml↑은 X.

Bronchogram

    ; ※Indication

           - suspected bronchiectasis

           - airway anomalies

Pul. Arteriogram

    ; Dx

           - cong.anomaly

                   ① lobar agenesis

                ② unilat. hyperlucent lung

                ③ vascular ring

           - solid or cystic lesion

Aortogram

      : vas. ring or pul. sequestration

Pneumoperitoneum, Pneumothorax

      : diaph. eventration시 Diaph.의 outline 결정

Radionuclide Lung Scan

     ·iv inj. of macroaggregated human serum alb.

     ·useful   i) pul. embolism

               ii) cong. cardiov. & pul. defect

     ·method : iv inj. of 133Xe

               +- inj.후 조기에 Xe이 나타남 : perfusion 측정

               +- breathing동안의 washout rate : ventilation 측정

324.1 Endoscopy

Laryngoscopy

    ; *evalution of stridor & local upper airway abnormality

    ; infant & small children → direct laryn

  ; older CH & adult → indirect laryn

Bronchoscopy

# ★Indication Of Diagnositic Bronchoscopy

    1) recurrent/persistent pneumonia or atelectasis

  2) suspected foreign body

  3) unexplained & persistent wheeze & infiltrates

  4) hemoptysis

  5) *pneumonia in the immunocompromised host

  6) suspected cong. anomalies

    7) mass lesions

  8) unexplained interstitial disease

# ★Indication Of Therapeutic Bronchoscopy

    1) removal of FB & mucus plug

    2) bronchial toilet

  3) bronchopul. lavage

# *extration of FB, massive hemoptysis, removal of tissues mass에서는 open rigid bronchoscope을 사용해야 한다.

# small flexible fiberoptic broncho.의 잇점

 1) easy insertion

 2)↑ peripheral range

 3)↓ Cx

 4) general anesth. 불필요

# postbronchoscopy croup의 Tx

 1) O2

 2) mist

 3) vasoconstrictor aerosol (racemic epi.)

 4) corticosteroid

# ★Bronchoscopy Complication

  1) transient hypoxia

  2) cardiac arrhythmias

  3) laryngospasm

  4) bronchospasm

  5) iatrogenic inf

  6) bleeding

  7) pneunothorax

    8) pneumomediastinum

Bronchoalveolar Lavage

; obtain a representative specimen of fluid and secretion from lower respiratory tract

; *useful for cytologic and micorbiologic diagnosis of lung diseases

Throacoscopy

324.2 Thoracentesis

1. Method    1) sitting position

             2) 1.5 inch, No 22 gauge needle

             3) rib margin 상부

2. Cx        1) inf.

             2) pneumothorax

             3) bleeding

             4) puncture or laceration of capsule of liver (Rt)

               & capsule of spleen (Lt)

3. transudate : pleural fluid의 formation이나 reabsorption에 영향을

              주는 mechanical factor에 의함

             1) total protein 3g/dl↓

             2) pleural protein/serum pr. 0.5↓

             3) leukocyte count 2,000↓: mononu. 우세

4. exudate : pleural surf.나 lung의 infl.이나 ds.

             1) protein↑

             2) PMNI 우세        except Tb, malignancy

        ex. Tb - glucose↓ & cholesterol↑

324.3. Percutaneous Lung Tap

; *▲direct method of obtaining bacteriologic specimens from the pulmonary parenchyma

; major Ix

    - *X-ray infiltrates of undetermined etiology, esp. unresponsive to therapy in immunosuupressed patients

; pneumothorax Cx이 thoracentesis 보다↑

324.4. Lung Biopsy

; *▲appropriate when there is diffuse lung disease such as Pneumocystis pneumonia

; infant & small children : open sung. biopsy

; older : transbronchial. bx.

324.5. Transillumination Of Chest Wall

    1) 6Mo↓시 fiberoptic light probe로 transill하여 pneumothorax Dx

    2) unreliable in  (1)older pt.

                    (2) subcut. emphysema

324.6. Microbiology

    1. method   1) nasopharyngeal or throat swab

                2) sputum (expectoraton X→ cough후 deep throat swab

                3) nasotracheal asp.        heavy contamination

                  transtracheal asp.        어린이에는 위험

                4) percut. lung tap or open biopsy

    2. exam. of secretion

         1) alveolar macrophage : hallmark of tracheobr. sec.

         2) early morning gastric aspirate → AFB smear & culture

         3) sputum의 Wright stained smear시 PMNL(-)

            → lower RT의 bact. inf.

         4) eosino. : allergic ds

         5) iron stain상 macrophage내 hemosiderin granule

             : pul. hemosiderosis

         6) viral pn. : wright stain에서 intranu나 cytoplasmic inclusion body

324.7. Blood Gas Analysis

# arterial bl. gas

    ; *▲most useful test of pul. function

# arterialized cap. bl.

  1) may be used if tissue perfusion is good

  2) vasodil. in finger, heel, ear lobe by warming or nitroglycerin

     or nicotinic acid cream

# venos PCO2

    ; 6∼8mmHg↑ than arterial PCO2

  ; pH는 약간↓

324.8. Pulmonary Funcion Testing

Measurement Of Ventilatory Function

# Flow Rate By Spirometer          

    ; FEV1

           - 첫 1초간 expired한 vol

    ; MMEF (max. midexp. flow rate)

    ; MEFV (max. exp. flow vol. curve)

       - 75% VC↓의 lung vol.에서의 flow rate는 effort와 무관

        - 50% VC↓의 flow rate는 small airway에 영향받음

       - 25% VC

                   / index of small airway function

# Airway resistance Raw by plethysmograph

  - cm H2O/L/sec

    * SRaw (specific airway R)

      = Raw X lung volume

        : n'l 7sec/cmH2O↓

        : 6세 이상은 일정

Measurement Of Gas Exchange

# DLCO

    ; Diffusing capacity for carbon monoxide

  ; method 

           - rebreathing from container having a known intial conc. of CO

           - single breath technique

    ; ↓경우  

           - effective alv. cap. surf. area↓

       - alv-cap. memb.을 통한 gas의 diffusability↓

# regional gas exchange

 : P/V Xe scan

# arterial blood gases

 : alv. gas exchange의 effectiveness

Measurement Of Perfusion

        ·pul. blood flow 측정 by   i) cardiac catb.

                                   ii) NO의 uptake

           → pul. arteriogram이나 radioisotope으로 distribution 측정

Clinical Use Of PFT

; 6세 이후는 어려움 없이 test 가능

   1) defining the type of process (eg. obst. rest)

   2) defining degree of functional impairment

   3) estimating the prog.

   4) preop. evaluation

   5) confirmation of functional impairment in pt. with subjective complaints. but n'l P/E

section 2. upper respiratory tracts

Chapter. 325. Congenital Disorders Of Nose

# Down synd.

  ; nasal passage의 narrowing

         → ① URI시 airflow의 severe obst.

                 ② chr. or recurrent hypoventilation

Choanal Atresia

; *▲common anomaly of nose

; nose와 pharynx 사이에 unilateral or bilateral bony or membranous septum으로 구성.

; *50% with other cong. anomaly

    - ★CHARGE syndorme

           / coloboma

           / heart disease

           / atresia choanae

           / retarded growth & development and/or CNS anomalies

           / genital anomalies and/or hypogonadism

           / ear anomalies and/or deafness

; unilat.시 resp. inf.전까지 오래동안 aSx일 수 있다.

; Diagnosis

    - *inability to pass firm catheter through each nostril 3-4cm

  - fiberoptic rhinoscopy로 직접 봄

  - CT scan

; Treatment

    - oral airway 유지 or open mouth

  - feeding

           / nipple : large hole

    / assited airway없이 breathing과 eating이 될 때까지 (대개 2∼3주) gavage feeding

  - elective op. correction

           / 수주나 수개월후

    / bilat.C.A.시 immediate op.는 드뭄.

    / unilat.시 수년 늦을 수도 있다.

Cong. Defect Of Nasal Septum

          - rare

        1) perforation  (1) develpmental

                       (2)  2°to syphilis, Tbc

        2) septal deviation : op. collection은 midface growth disturb.를

                           피하기 위해 대개 연기

        3) cong. midline nasal mass : dermoids, gliomas, encephaloceles

Chapter 326. Acquired Disorder Of Nose

326.1 Foreign Body

        1) initial Sx : local obstr. sneezing. mild discomfort. rarely pain

        2) unilat. nasal discharge & obst. : FB시사

326.2 Epistaxis

        ·rare in infancy

        ·common in childhood

        ·↓after puberity

※91 Etiology

; *trauma

    - *▲common

    - picking nose ＆ FB

; *adenoidal hypertrophy, allergic rhinitis, sinistis, polyp, acute infection

; disease with paroxysmal ＆ forceful cough

    - cystic fibrosis

; congenital vascular abnormalities

    - telangiectasia, varicosities

; thrombocytopenia, def. of clotting factor, hypertension, renal failure, venous congestion

; adolescent girls with menarche

Clinical Manifestation

 ·source (1）vascular plexus of ant septum（kiesselbach

              plexus）

          (2) mucosa of ant.portion of turbinates.

※주 Treatment

    ; compression on nares ＆ quiet

           - erect position with head forward

    ; *0.25-1% neosynephrine with or without topical thrombin

    ; ant nasal pack

           - bleeding persist시

         - bleeding이 post, rare에 있을시combined ant ＆ post packing

    ; *cautery with silver nitrate

           - bleeding site를 알때

    ; bl.transfusion

           - severe or repeated epistaxis시

    ; otolaryngologic evaluation

           - bilat, bleeding, no arising from Kiesselbach plexus

    ; clotting factor replacement

# Juvenile nasopharyngeal angiofibroma

  ·peak：adol. ＆ preadol.boys

  ·특징：profuse epistaxis with nasal mass

  ·Dx：CT scan with contrast

  ·Tx：arteriography, embolization, extensive surgery

Chap. 327 Infection Of Upper Respiratory Tract

Etiology

    ; virus ＆ mycoplasma

           - *▲common

           - 예외 ：epiglottitis

    ; *Streptococci ＆ Diphtheria

           - major bacteria of primary pharyngeal ds

1) RSV：⅓ of bronchiolitis

2)parainfl. ⅴ：croup

3) influenza ⅴ

4) adenovirus：10％↓of resp.ds

               pharyngitis ＆ PCF가 TMC in children

5)rhinov. ＆ coronavirus：“common cold”synd

6) Coxackie ⅴ. A,B：nasopharynx의 Ds

7) Mycoplasma：upper ＆ lower resp. ds

                 bronchiolitis, ph, bronchitis, pharyn-

                 gotonsillitis, myringitis, OM

327.1 Acute Nasopharyngitis（=URI, Common Cold）

; *▲common infectious condition of children

; *more extensive than adult

; *significance depend on frequency in which complication occur

Etiology

; *rhinovirus

    - *principal agent

; coronav

    - 10％

; period of infectivity

; bacteria

  - *Group A streptococci : principle bacterias

    - Coryn. diphtheria

  - Mycop. pn.

    - N. meningitidis

  - n. gonorrhea

Epidemiology

; children

    - *average of five to eight infection a year

3) Nursery, school ＆ day care center：epidemic

Pathology

1) edema ＆ vasodilation in submucosa

2) cilia의 structural ＆ functional change

 → mucus clearance에 지장 초래

Clinical Manifestation

3 Mo-3Yr：inf.초기에 fever

purulent Cx은 age 어릴수록↑

# 3 Mo ↑

 (1) initial Sx：fever, irritability, restlessness, sneezing

    →수 hr내 nasal discharge ＆ obst

    - 2-3일：ear drum congestion ＆ fluid behind drum

         (2）febrile phase：몇 hr-3 days

# older children

         (1) initial Sx：dryness ＆ irritation of nose

           → 수 hr：sneezing, chill, m.ache, thin nasal discharge

                때로 coughing, headache, malaise, anorexia, low grade

                fever

           - 1일 내 secretion thicker, sore throat

             acute phase 2-4 days지속

Differential Diagnosis

# initial Sx of measles, pertussis

# poliomyelitis, hepatitis, mumps

# ※Bloody Persistent Nasal Discharge

  ; foreign body

    ; diphtheria

    ; choanal atresia

    ; congenital syphilis

# allergic rhinitis

  ; fever (-)

    ; not become purulent

    ; persistent sneezing ＆ itching of eyes ＆ nose

    ; nasal mucous memb：pale

    ; nasal smear：eosino 多

    ; antithistamine → Sx사라짐

Complication

 1) cervical lymphadenitis

 2) mastoiditis

 3) peritonsillar cellulitis or periorbital cellulitis

 4) OM - 25％ of small infant

        - TMC Cx

        - fever recur시 suspect

 5) LTB, bronchiolitis or pneumonia

Prevention

         ：γ-globulin, vit.C …not recommend

Treatment

; no specific Tx

; AB

    - *not affect course & reduce incidence of bacterial complication

; bed rest

; acetaminophen：첫 1-2일 irritability, aching, malaise↓

; aspirin

    - not recommend

; nasal instillation

    - sterile saline

           / infant

    - phenylephrine (0.125-0.25%)

    - *AB, C-S, antithistamine addition은 효과가 없다.

  - 15-20min before feeding and at bed time              

         / supine with neck extesion

            → 1-2 drop을 각각nostril에

         → 5-10분후 다시 1-2drop

    - cotton application not recommend

    - *sterile saline이외에는 4-5일이상 사용하면 안된다.

# In infant

    ; suction with soft bulb syringe

  ; prone position

  ; highly humidified environment

# Oral decongestant

  ; pseudoepherine

  ; antihistamine +  adrenergic agonist

327.2 Acute Pharyngitis

; tonsillitis 와 pharyngotonsillitis 를 포함한 pharynx의 모든 acute infection

  - principal involvement : throat

  - uncommon under 1 Yr

  - peak : * 4-7 세

# ★Prominent Pharyngeal Involvement

    ; *diphtheria, herpangina, adenovirus infection, infectious mononucleosis

Etiology

    1) virus : 대부분

    2) Group A  B-hemolytic streptococcus : only common bacterial agent

      ( 15%이하)      

Clinical Manifestations.

Viral Pharyngitis

  ① gradual onset

  ② early sign : fever, malaise, anorexia, throat pain.

  ③ sore throat : present intially, peak 2-3rd day

  ④ hoarseness, cough, rhinitis.

  ⑤ pharyngeal inflammation : slight.  occasionally severe - small ulcers on

     soft palate, post pharyngeal wall.

  ⑥ exudate : on lymphoid follicles of palate and tonsil.

  ⑦ cervical LN : enlarged and firm.

* ⑧ laryngeal involvement : common.

  ⑨ WBC : 6000- 30000, predominantly polymorphonuclear cells in early phase.

  ⑩ entire illness : 보통 5일 이상 계속되지 않음.

Streptococcal Pharyngitis

    ; 2세이상.

  ; headache, abdominal pain, vomiting 으로 시작.

  ; 수시간내 sorethroat.

  ; 1/3 에서 tonsillar enlargement , exudation, pharyngeal erythema.

  ; 2/3 에서 mild erythema, no enlargement of tonsil without exudate.

  ; ant. cervical lymphadenopathy.

  ; fever : as high as 40 ^o C . 1-4일 지속.

     very severe case- as long as 2 weeks.

    ; ♥ Physical Finding Asso. With Streptococcal Disease

     - diffuse redness of tonsil and tonsillar pillars.

     - petechial mottling of soft palate.

    ; ★conjunctivitis, rhinitis, cough, hoarseness

           - rarely symptom

           - *2개 또는 그이상의 증상이 나타나면 viral pharyngitis시사

Streptococcosis

      ①   B-hemolytic streptococcus 의 early inf. 과 관계된 acute . inf. 이 있을시

          systemic variation.

      ②  infant: 1 주 이하로 지속되는 mild episode

                 특징: variable fever, mucoserous nasal discharge, pharyngeal                       infection.

      ③  6 Mo- 3 Yr : most severely ill,

         - 초기: coryza with postnasal discharge, reddened pharynx, fever,

                 vomiting,  loss of appetite.

         - fever : 38-39.5 ^o C ,* 4- 8 주간

         - cervical node : enlarge and tender, adenopathy 는 fever와 같이존재.

Diagnosis and Differential Diagnosis

    1) Dx : by rapid detection method for streptococcal antigen.

            by culture after pharyngeal swabbing

    2) purulent nasal discharge, pharyngitis, fever - pneumococci or H. influenza.

    3) mambranous exudate in tonsil - diphtheria, infectious mononucleosis

    4) vesiculoulcerative lesions on ant. pillars, fauces, soft palate- herpangina.

    5) Agranulocytosis

        ① first manifestation : acute pharyngitis 증상.

        ② tonsil and post. pharyngeal wall: covered by yellow or dirty white  

           exudate.

        ③ mucous membranes under exudate-->necrotic and ulceration extends to

           mouth and tongue : very painful.

        ④ dysphagia : severe.

        ⑤   enlargement of cervical lymph node.

        ⑥  mucosal hemorrhages.

    6) Smoke tobacco or marijuana : pharyngeal inflammation and sorethroat.

    7) allergic rhinitis : cause sore throat.

    8) gonococcal pharyngeal infeciton : asymptomatic.

Complication

    1) purulent bacterial OM : viral

    2) chr. ulcer in pharynx : viral and strep.

    3) peritonsillar abscess, sinusitis, OM, meningitis : strep.

    4) glomerulonephritis , rheumatic fever : strep.

    5) Mesentric adenitis : viral or bacterial

Treatment

    1) viral origin : no specific Tx.

    2) strep. pharyngitis 시

       ① oral  PC ( 125-250 mg pc v tid for 10 days )

            - 24시간내 defervescense, course of illness 를 1.5일 정도 shorten.

            - pc Tx 시작후 수시간내 noninfectious.

       ② EM : 만일 환자가 pc 에  allergic 한경우.

   3) acetaminopen or ibuprofen : pain이 심한경우.

   4) gargling with warm saline solution : old children 시.

   5) inhalation of steam : younger children.

   6) cool bland, liquids : acceptable than solids or hot foods.

   7) rheumatic fever 의 과거력이 있는경우 : AB prophylaxis 필요.

327.3  Acute Uvulitis.

  - fever, pain with swallowing, drooling

  - most case: Gr A strep. or H. influenza type b.

      strep. uvulitis : 5 세이상.

      H. influenza  : 5 세 이하.

327.4 Chronic Rhinitis And Nasopharyngitis

Chronic Rhinitis

# ★Underlying Disturbance

  ; nasal polyps , chronic sinusitis , chronically infected adenoids, cystic fibrosis, dysmotile cilia syndrome, allergy, foreign bodies, deviated septum, various congenital malformation, nasal diphtheria, syphilis

    ; chronic debilitating infection, nutritional immunologic or metabolic deficiency

Clinical Manifestation

    ① chronic nasal discharge 가 주증상.

  * ② bloody discharge : i) syphilis,                                           

                         ii) diphtheria

                         iii) foreign bodies

                         iv) persistent nose picking

    ③ disturbance of taste and smell

    ④ fever : 감염이 병발하지 않으면 열은 별로 없다.

    ⑤ persistent allergic rhinitis : common and seasonal.

    ⑥ rhinitis medicamentosa : 장기간 topical nasal decongestants를 사용한경우.

    ⑦ atrophic rhinitis : i) uncommon

                        ii) associated with general debilitating condition or long-

                           continued nasal infection.

                        iii) sense of smell: impaired

                        iv) crusting and sense of dryness

                        v) foul nasal discharge ( ozena )

Treatment

    ① lanoline, silicone, petrolatum-base ointment : protects against skin

         excoriation.

    ② humidified air

    ③ mucosa - shrinking solutions ( eg phenylephrine ): symptomatic relief.

                                                      also causes further damage

    ④ local AB : avoid

         systemic AB : Ix in selected cases.

Chronic Pharyngitis

   - rare

    - secondary to chronic infections of sunuses, adenoids, tonsils.

    - 편도 절제후에도 발생.

Clinical Manifestations

   ① throat discomfort : dryness and raspy irritation.

   ② throat clearing (잦은 헛기침) and irritative cough.

   ③ mucous membrane : inflamed, pale, blood vessel 이 prominent.

   ④ pharyngeal wall : mucopurulent secretion

                       lymphoid tissue : hypertrophied --> pebbled appearance.

Treatment

   ① directed toward any disturbance.

   ② general nutriton and hygiene.

327.5 Retropharyngeal Abscess

# ★4세이하에 잘 오는 이유

    ; 4세이전에 post pharyngeal wall과 prevertebaral fascia 사이의 space에 small lymph nodes 가 존재

           --> lymphatic channel communicating with portion of nasopharynx as well as post. nasal passages

           --> nasopharynx infection이 spread

    ; *3-4세이후에 사라진다.

Etiology

    ; *bacterial pharyngitis

  ; vertebral osteomyelitis

    ; wound infeciton.

    ; ※96 pathogens

           ① Group A hemolytic streptococci                

    ② oral anaerobes

    ③ S. aureus

 Clinical Manifestations

  ; abrupt onset of high fever with difficulty in swallowing

  ; refusal of feeding.

  ; severe distress with throat pain.

  ; hyperextension of head.

  ; noisy, gurgling respirations.

  ; labored respiration.

  ; drooling.  

    ; *bulge in posterior pharyngeal wall : digital exam. with Trendelenburg position.

  ; lateral roentegnogram of nasopharynx or neck

           - reveal retropharyngeal mass.

       - air may be seen.

    - loss of normal cervical lordosis.

    ; ※96 Death

           - aspiration

           - airway obstruction

           - erosion into major blood vessels

           - mediastinitis

★Differential Diagnosis

  1) croup

           ; larynx 에 압박시 stridor and high fever

    2) meningismus : limited neck motion

    3) nonfluctuant lymphadenitis

    4) Tbc of cervical spine

Treatment

  1) prefluctuant stage

    ① semisynthetic penicillin.

    ② single agent with clindamycin or ampicillin - sulbactam

    ③ analgesic drugs : for pain

    ④ narcotics : use only with great care.

  2) fluctuance.

     : incision and AB.

327.6 Lateral Pharyngeal Abscess.

  1) occurs in space lateral to pharynx --> extends from hyoid bone to base of                                                 skull.

  2) carotid vessel and jugular vein associated

  3) Sx : ①high fever, trismus, ill app. , severe pain, difficulty swallowing.

          ②bulge in lat. pharyngeal wall

          ③cervical adenitis

          ④torticollis toward side of abscess due to muscular spasm.

  4) Tx: lat. neck drainage.

327.7 Peritonsillar Abscess

; occurs *space between sup. constrictor muscle and tonsil

Etiology

    ; *Group A β-hemolytic streptococcus or oral anaerobes.

Clinical Manifestations

    ; *preceded by attack of acute pharyngotonsillitis

    ; afebrile interval of several days or not subside fever

    ; severe throat pain, trismus (due to spasm of pterygoid muscles)

    ; refuse to swallow or speak

    ; torticollis  

    ; fever

           - septic and reach 40.5℃　　　

    ; *affected swollen and inflamed tonsillar area

    ; uvula

           - dsiplaced to opposite side.

    ; untreated Pt

           - abscess becomes fluctuant --> spontaneous rupture.

Treatment

   1) AB (usually penicillin ) and I & D or aspiration.

   2) Hx of chronic tonsillitis (-) --> no tonsillectomy.

   3) Hx of prior tonsillitis or previous abscess (+) --> immediate tonsillectomy.

327.8 Sinusitis

# full-size 시기

    ; *maxillary antra and ant. post. ethmoid cell - infancy

    ; *frontal sinus - 6-10세

    ; sphenoid sinus - 3-5세  

; incidence of acute and chronic sinus infection : childhood 후반기에 증가.

# sinus infection 을 증가시키는 factor

    ①  allergic factor

    ②  poor sinus drainage with septal deviation or adenoid hypertrophy.

    ③  hereditary condition.

    ④  immunosuppression.

    ⑤  environmental factor.

Acute Purulent Sinusitis

  - Sx or sign 은 acute rhinitis 후 3-5일에 나타남.

Clinical Manifestation

; suggestion

    - "cold" (감기) seems more severe than usual ( fever > 39 C, periorbital edema, facial pain )

    - " cold" lingers for more than 10days

; nightime cough : viral upper resp. inf.

  - *daytime cough : suggestive of sinusitis.

; *headache, facial pain, tenderness, edema : uncommon.

# topical decongestant 후 exam.상

    ; pus in middle meatus

           - involvement of maxillary, frontal , ant ethmoid sinus

    ; pus in sup. meatus

           - involvement of sphenoid or post. ethmoid cell

; ★postnasal discharge

           --> *sore throat, persistent cough at night

# acute ethmoiditis in infants and small children

    ; periorbital cellulitis with edema or soft tissues

  ; redness of skin

# ★Complication

    ; epidural or subdural abscess   

  ; meningitis.

  ; cavernous sinus thrombosis

  ; optic neuritis

    ; periorbital or orbital cellulitis and abscess

  ; osteomyelitis

Diagnosis

# X-ray

    ; often used but misinterpreted

    ; *▲common findings

           - air-fluid level.

    - complete opacification

    - *mucosa width of 4 mm or more : bacteria in sinus.

# CT scan

  ; *▲sensitive indicators

    ; Indication

           - before surgery

    - Cx of sinusitis

# antral puncture

    ; *유일한 믿을만한 method for bacterial culture

    ; ★Ix for sinus aspiration

        ① Tx에 무반응.

        ② immunocompromised hosts

        ③ life threatening Cx

  ; ※92 Organism

        ① S. pneumoniae.

        ② M. catarrhalis

        ③ nontypable H. influenza.

# nasal swab culture : sinus aspirate culture 와 일치하지 않는다.

Treatment

; *Amoxicillin

    - *initial choice

; H. influenza , M catarrhalis producing B-lactamase or treatment failure

    --> i) bactrim

     ii) amoxicillin with potassium clavuanate,

     iii) EM + sulfonamide.

     iv) 2nd and 3rd generation cephalosporin.

; *치료기간 - 14-21 days

; *decongestant and antihistamine - not heipful

; ★Sinus Drainage And Irrigation Ix

    - fail usual Tx

  - intraorbital, intracranial or other Cx

  - intense pain

Chronic Sinusitis.

  - underlying disease : ①  nasal deformity.

                        ②  polyp

                        ③  infected and hypertrophied adenoids

                        ④  infected teeths

                        ⑤  sinus polyp or mucocle.

                        ⑥  allergy, cystic fibrosis, dyskinetic cilia.

* - common in absence of secretory Ab ( IgA ) and immunodeficiency state.

  1) Clinical manifestation.

    ①low grade fever.

    ②malaise, easy fatigability , anorexia.

    ③nasal discharge.

    ④swelling of middle turbinates--> causes nasal obstruction.

    ⑤postnasal discharges in abscence of infected adenoids or aucte URI                                            --> diagnostistic .

    ⑥watery nasal discharge or sneezing --> allergic rhinitis.

**  ⑦sinobronchitis

        -relationship between sinus and lower resp. tract. Sx

        -underlying ds.

          i).  reactive airway.

          ii). cystic fibrosis.

          iii). immunodeficiency.

          iv). dyskinetic cilia.

     ⑧ sinusitis 가 asthma 를 aggravate 함

 

  2) Eti.

    ①acute sinusitis 균종.

    ②a-hemolytic strep.

    ③S. aureus.

    ④anaerobes

  3)Tx

    ①antimicrobials for * 6 weeks.

    ②antihistamine and decongestant.

    ③OP.

Chapter 328. Nasal Polyps

Etiology

; benign pedunculate tumors formed from edematous, chronically inflammed nasal mucosa

; originate from

    - ethmoid sinus, middle meatus, maxillary antrum

; extend to nasopharynx (antrochonal polyp)

; associated disease

  1) *cystic fibrosis(25 %) : *▲common

  2) chr. sinusitis

  3) chr. allergic rhinitis

  4) asthma

Clinical Manifestation

; *obstructon of nasal passage

; hyponasal phonation

; mouth breathing

; mucoid or mucopurulent rhinorrhea

; glistening, gray, grape-like mass

    - distinguished from well-vascularized turbinate tissue : pink or red

Treatment

; local or systemic decongestants

    - not effective.

; corticosteroid nose spray

    - *not helpful , although warranted in recurrent case.

; surgical removal

    - Indication

           ① complete obstruction.

    ② uncontrolled rhinorrhea.

    ③ deformity of nose appears.

; *antihistamine : helpful

Chapter 329. Tonsil And Adenoids

 

- adenoid : nasopharyngeal tonsil.

- Waldeyer ring: 1) consists of  ① lingual tonsil

                                ② two faucial tonsil

                                ③ adenoid

                                ④ lymphoid tissue on post. pharyngeal wall.

                  2) defense against infections.

- seperate tonsillectomy and adenoidectomy : 4-5세전

- infancy 에는 tonsillar disturbance가 드뭄.

- neoplasm of tonsil -- Waldeyer ring; non-Hodgkin lymphoma.

                        nasopharynx: rhabdomyosarcoma.

Chronic Tonsillits (Chronically Hypertrophic And Infected Tonsils)

Clinical Manifestation

   ①recurrent or persistent sore throat.

   ②obstruction to swallowing or breathing.

   ③sense of dryness and irritation in throat.

   ④breath : offensive.

   ⑤ obstructing upper airway ( rarely ) --> resp. distress, chronic hypoxemia,

                                              pulmonary hypertension.

Indication For Tonsillectomy

# Decision for removal of tonsils

  ; based on Sx and sign of hypertrophy, obstruction, chronic infection

# ★chr. infection 의 reliable guide

  ; persistent hyperemia of ant. pillars

           - *more reliable sign

    ; enlargement of cervical LN

  ; persistent enlargement of node just below and in front of angle of jaw

# ♥Only Absolute Ix For Tonsillectomy

    ; rule out tumor

  ; severe aerodigestive tract obstruction

# age relation

    ; *postpone after 2-3yr

# active infection relation

    ; *postpone until 2-3wks after subsidence of infection

       except  ① acute resp. obstruction with pul. artery HT.

               ② cor pulmonale

Complication of tonsillectomy

   (1) postop. sorethroat ( for 5 days )

   (2) referred ear pain and halitosis.

   (3) minor hemorrhage.

   (4) postop throat inf. or anesthetic Cx.

   (5) severe hemorrhage or life threatening Cx.

   (6) pul. edema.

Adenoidal hypertrophy

(=hypertrophy of pharyngeal tonsil: " adenoid" )

Clinical Manifestation

* ① mouth breathing and persistent rhinitis : 가장 특징적 Sx.

  ② chronic nasopharyngitis.

  ③ voice : nasal, muffled.

  ④ breath: offensive.

  ⑤ taste and smell : impaired.

  ⑥ harassing cough : at night.

  ⑦ impaired hearing : common.

  ⑧ chronic OM

  ⑨ snort and snore loudly : sign of resp. distress.

  ⑩ respiratory insufficiency ( hypoxemia, hypercapnia, acidosis ) : during sleep.

     --> pul. a. HT --> cor pulmonale.

    # upper airway obstruction in sleep --> mimicking adenoidal hypertrophy syn.

      ① very obses children ( e.g. Prder-Willi-Robin syn. )

      ② large or post. placed tongue ( e.g. Pierre-Robin syn. )

      ③ cow's milk hypersensitivity.

      ④ Down syn : macroglossia, tonsillar enlargement, skull base anomaly.

Diagnosis

    ① digitial palpation

    ② indirect visualization with pharyngeal mirror.

    ③ fiberoptic bronchoscopie

    ④ lat. pharyngeal X-ray.

Treatment

# ♥Ix of adenoidectomy

  ① persistent mouth breathing

  ②nasal speech

  ③adenoid facies

  ④repeated attacks of OM (esp. when accompanied by a conductive hearing loss)

  ⑤persistent or recurrent nasopharyngitis related to infected hypertrophic adenoid tissue

Chapter 330 Obstructive Sleep Apnea And Hypoventilation In Children

  - combination of prolonged partial upper airway obstruction and intermittent           complete obstruction  ( obstructive apnea ) --> disrupts normal ventilation         and sleep patterns.

# habitual snoring

    ; *▲common Sx

  ; 8-10 % of all young school children

  - severe OSA/H : 1 % of snoring children.

  - peak age : 2-5 Yr.

  - incidence   in male and female.

     ① prepubertal children: similar.

     ② adults: male predominance.

Pathogenesis.

 1) normally during sleep.

      dsepite pharyngeal airway collapse and upper airway muscle tone decrease ,        especially during REM sleep.--> upper airway patency : maintained and            ventilatory output and oxygenation not impaired.

      --> Pa 02 decrease and Pa 02 increase : only slightly.

 2) certain anatomic factor ( adenotonsillar hypertrophy, nasal obstruction, obesity,

     craniofacial anomaly ) or neurologic condition ( hypotonia in trisomy 21,            cranial nerve weakness ) --> occur OSA/ H

3) cascade of events.

  ① upper airway muscle activity decreases, upper airway narrows and resistance

      increase in anatomic or neurologic factor.

  ② partial or complete airway obstruction --> impaired gas exchange with            hypoxemia and hypercapnia.

  ③ potent stimuli for increased ventilatory effort and upper airway muscle              activity.

  ④ increased effort and airway muscle tone lead to resumption of airway potency.

  ⑤ arousal from sleep occur --> helps restoring blood gas.

  ⑥ airflow restored, oxygen and carbon dioxide level return to normal.

  ⑦ sleep re-established , upper airway muscle activity decrease -->

     cycle starts again.

 Figure 330-1   Pathophysiology of OSA/H in children.)

 

4) OSA/H 의 serious cardiorespiratory and neurobehavioral consequence.

  (1) chronic hypoxemia lead to

                              ① polycythemia

                              ② growth failure

                              ③ increased pulmonary artery pressure.

                              ④ pulmonary hypertension.

                              ⑤ Rt. heart failure.

                              ⑥ arrythmia.

                              ⑦ death.

  (2) recurrent arousal lead to   

                              ① sleep fragmentation.

                              ② loss of normal sleep pattern.

                              ③ excessive daytime sleepiness.

  (3) hypersomnolence

                              ① behavoral problems.

                              ② impaired school performance.

                              ③ accidents.

  (4) sleep fragmentation.

                              ① suppress arousal response.

                              ② further impair ability to reestablish upper airway

                                 patency and restore gas exchange.

      

5) Anatomic factor lead to obstruction in childrem.

  ① adenotonsillar hypertrophy : most common.

  ② micrognathia, retrognathia, macroglossia.

  ③ fat deposition from obesity.

  ④ congenitally small airway narrow nasopharynx

Clinical manifestation

 1) daytime hypersomnolence

    - most common Sx in adults.

    - rare in children.

 2) chronic mouth breathing , snoring , restlessness during sleep with or without

    awakening.

 3) unusual sleep position

    - neck hyperextended or bottom up in air.

 4)breath normally while awake in most children with OSA/H,                           but, more severe presentation 시 : nosisy, labored awake breathing

                                        --> worsen with sleep.

 5) snoring very loud. followed by silience. snort, arousal, resumption of snoring.

 6) triad of Sx --> highly suggestive of OSA/H

                    ① snoring

                    ② nocturnal breathing difficulty.

                    ③ witnessed respratory pauses.

 7) behavioral problems and poor school performance.

 8) secondary enuresis

 9) underweight or present with failure to thrive in some children.

    -- contribute factor ① dysphagia from large tonsil.

                        ② chronic hypoxemia.

                       ③ higher metabolic expenditure from increased work of                                 breathing.

                       ④ insufficiency growth hormone release in absence of deep

                           NREM sleep.

 10) unexplained Rt. heart failure.

 11) more severe case ; respiratory failure.

Diagnosis and Assessment

# ♥Delayed Dx due to Several Reasons

   ① absence of awake symptoms

   ② failure to obtain sleep history

   ③ symptoms of snoring or restless sleep are considered inconsequential

   ④ parents may be unaware of problem because child's most severe symptoms appear during REM sleep in the last 3rd of night

   ⑤ young children may not generate loud, disruptive snoring noises

# P/Ex

    (1) features associated with OSA/H

      ① unusual facies.

      ② month breathing

      ③ hyponasal speech.

      ④ macroglossia.

      ⑤ cleft palate.

      ⑥ enlarged tonsil.

      ⑦ not consistent feature of obesity

    (2) stridor or hoarse voice : indicate cranial nerve dysfunction.

3) Lab. finding

      ① polycythemia or metabolic alkalosis : absence in majority of pediatric pt.

4) ECG : Rt. ventricular hypertrophy.

5) Lat. soft tissue radiograph of neck: identify adenoidal tissue.

6) CT or MRI of nasopharynx.

7) fluroscopy or endosccopy

# ★polysomnography (PSG)

  ; *" gold standard " for Dx of OSA/H

  ; overnight recording of multiple physiologic sensors during sleep.

    ; provide powerful , quantitative, noninvasive assessment of

       i) gas exchange impairment.

       ii) respiratory pattern.

      iii) thoraco-abdominal movement.

      iv) sleep disruption.

    ; useful i) confirming Dx

              ii) determing severity of OSA/H

             iii) decumenting efficacy of Tx.

# DDx

    ① nocturnal asthma.

    ② upper airway obstruction from gastroesophageal reflux.

    ③ esophageal foreign body.

    ④ laryngomalacia, vascular ring, intraluminal mass

    ⑤ vocal cord dysfunction.

    ⑥ night terrors or nocturnal seizures.

    ⑦ narcolepsy and restless leg syn.

Treatment

    ① adenotonsillectomy  

        : risk for incomplete resolution of OSA/H after adeno-tonsilletomy

            i) trisomy 21

            ii) craniofacial disorders.

           iii) extreme obesity

           iv)  neuromuscular disorders such as cerebral palsy or Chiari                           malformation .

            v) present before 2 Yr of age.

    ② nasal continous airway pressure ( CPAP )

           : option in children who fail adenotonsillectomy.

    ③ supplemental oxygen.

    ④ snoring : treated with nasal decongestants and topical steroids.

    ⑤ medroxyprogesterone acetate.

            i) Tx of daytime hypoventilaton associated with obesity-hypoventilation                  syndrom

            ii) adverse effects: growth and pubertal developments.

    ⑥ protriptyline : nonsedating antidepressant with REM suppressant activity in

                      adult.-- not recommended

    ⑦ tracheostomy.

            : serious upper airway obstruction in both wakefulness and sleep.

SECTION 3. Lower Respiratory Tract

Chapter 331. Congenital Anomalies

331.1 Laryngeal Anomalies

1. complete  atresia of larynx

   : incompatible with life.

2. laryngeal webs.

    (1)  uncommon , occasionally familial

    (2)  resulting from incomplete separation of fetal mesenchyme between two               side of  larynx .

    (3)  most webs : between vocal cords.

    (4)  Sx : ① respiratory distress  with severe stridor.

             ② cry: weak and abnormal.

             ③ obstruction 이 incomplete 한경우 : mild stridor and dyspnea.

    (5)  Dx : direct laryngoscopy.

    (6)  Tx : ① lysis with carbon dioxide laser.

              ② OP.

3. Laryngotracheoesophageal cleft.

    (1)  rare congenital lesion.

    (2)  long connection between airway and esophgus.

    (3)  caused by failure of dorsal fusion of cricoid - normally complete by 8th                                                             wk  gestation.

    (4)  type

        ① type 1 : above sup. portion of post. cricoid plate.

        ② type 2 : extend to inf. aspect of post. cricoid palate.

        ③ type 3 : involve " cervical trachea"

        ④ type 4 : extend to thoracic trachea and below.

     (5)  Sx

        ① chronic aspiration.

        ② gagging during feeding

        ③ pneumonia ( H-type tracheoesophageal fistula )

     (6)  Dx

         ① roentgenographic study of swallowing.

         ② endoscopy.

331.2 Congenital Laryngeal Stridor (=Laryngomalacia And Tracheomalacia)

; congenital deformities or flabbiness of epiglottis and supraglottis aperture.

        : weakness of airway wall.

          => inspiration 시 collapse and airway obstruction 야기.

; *▲common congenital laryngeal abnormality

Clinical Manifestation

; *noisy , crowing resp. sound in inspiration

    - common in neonate and first 1yr

; stridor at birth

    - may not appear utill 2 months

; male : female = 2.5:1

; worse lies on back

; noisy breathing, laryngeal " crow " hoarseness or aphonia.

; dyspnea, inspiratory retracton

; severe dyspnea 시 undernutrition and poor wt. gain.

; substantial stridor

    - *persist for several months to 1 Yr

  - *worse in first few months and then gradually disappearing with airway growth and development

Diagnosis

# direct laryngoscopy

# ★Differential Diagnosis

    ; congenital laryngeal disturbance from neonatal tetany

    ; laryngeal edema secondary to trauma or aspiration

    ; others

           ① malformation of laryngeal cartilages or vocal cords

    ② intraluminal webs

    ③ generalized severe chondromalacia of larynx and trachea

    ④ Tm of larynx

    ⑤ mucus retention cyst

    ⑥ bronchial cleft cyst

    ⑦ thyroglossal duct remnant

    ⑧ hypoplasia of mandible

    ⑨ macroglossia

    ⑩ hemangioma, lymphangioma, Pierre Robin syn., congenital goiters, vascular anomalies

# *4mo이후에 증상을 나타나내는 경우에는 other respiratory anomalies를 동반할 수 있다.

Treatment

    ; *no specific Tx

           - resolves spontaneously

  ; small nipple dropper 드물게 gavage.

  ; *more comfortable or less noisy in prone position

    (4) severe Sx -> nasotracheal intubation or thacheostomy.

Prognosis

; *18 mo에 clinically resolve

    - some degree of inspiratory obstruction persist a little longer

; pulmonary function test 상 teen age 까지 minor abnormality.

       --> not clinically problem.

       --> not require Tx.

Other anomalies

    1) bifid epiglottis

      : resulting from cleavage of two thirds or more of epiglottis.

    2) total absence of epiglottis : extremly rare.

    3) laryngeal cyst and laryngoceles

      : tx with endoscopic " unroofing"

331.3 Agenesis or Hypoplasia Of Lung

# bilat. pul. agenesis or hypoplasia.: incompatable with life

# associated disorders of bilat. pul. hypoplasia

   ①  anecephaly.

   ②  diaphragmatic hernia

   ③  urinary tract anomaly.

   ④  thumb anomaly.

   ⑤  deformity of thoracic spine and rib cage ( thoracic dystrophy)

   ⑥  renal anomaliles.

   ⑦  Rt. sided heart malformation.

   ⑧  congenital pleural effusion.

# unilat. agenesis or hypoplasia : few Sx

  - Lt. sided lesions : more common.

  - No specific Tx  : older pt. 의경우 - AB for pulmonary infection.

                                      - annual influensa vaccine

  - Px 는 associated anomaly 에 의존

  - right lung involve 시 mortality 증가

  - death : ①  overwhelming pul. inf.

            ②  CHD 와 연관된 pul. HT 의 Cx.

331.4 Pulmonary Sequestraton

# sequestration

    ; nonfunctioning embryonic and cystic pul. tissue mass

    ; entire blood supply from systemic circulation

# associated anomalies

    ; diaphragmatic hernia, esophageal cysts

Intralobar Sequestraton

    ; lower lobe

    ; *ass. with infection

    ; *hemoptysis in older pt

    ; mass lesion and air- fluid level in X-ray

    ; Treatment

           - removal operation

Extralobar Sequestration

    ; *more common in male & Lt. lobe

    ; *ass. with diaphragmatic hernia

    ; *mostly asymptomatic

           - others : respiratory Sx or heart failure.

    ; Tx : op. removal.

Diagnosis

  1) phy/finding :

           ① dullness to percussion and breath sound 감소.

           ② infection 시 rales

           ③ continuous or purely systolic murmur heard over back.

  2) chest X-ray

  3) bronchography

  4) ultrasound : rule out diaphragmatic hernia.

  5) aortography :

           ① confirm diagnosis.

           ② lesions 의 blood supply를 규명.

  6) Doppler ultrasonography and MRI.

331.5 Bronchoenic Cyst

* cilliated epi. 으로 lined - inf. 시 소실되어 pathologic Dx 곤란.

* midline structure 에서 가깝게 발생( eg. trachea, eso., carina )

* Sx ① fever

      ② chest pain

      ③ productive cough

 

* X-ray : cyst - contain air fluid level.

* Tx for Sx cyst : 적당한 AB후 surgical excision.

* ASx 시 no Tx.

331.6 Bronchobiliary Fistula

1) Def. : Rt. middle lobe bronchus와 Lt. hepatic ductal system사이의 fistula.

2) Sx: recurrent severe bronchopul inf. starting in early infancy.

3) Dx: endoscopy and bronchography or exploratory surgery.

4) Tx: surgical excision of entire intrathoracic portion of fistula.

331.7 Congenital Pulmonary Lymphangiectasis.

1) Def. : dilated lymphatic duct throughout lung (신생아기에 dyspnea and cyanosis)

2) 2 form of disease

  ① lung 에 국한된 abnormality.

  ② 2⁰ to pulmonary venous obstructon

   : 3rd form - pulmonary lymphangiectasis with other organ disease.

3) Dx : lung biopsy.

4) no specific Tx.

331.8 Cystic Adenomatoid Malformation.

; *2nd ▲common congenital lung disease

    - lobar emphysema가 most common 

# ★Sx

    ; neonatal respiratory distress.

  ; recurrent respiratory infeciton.

  ; pneumothorax

2) Most pt: symptomatic and die in newborn period.

3) Dx

  ①P/Ex : diminished breath sound with mediastinal shift away.

  ②X-ray ; cystic mass with mediastinal shift

            air-fluid level -> lung abscess 시사.

4) DDx : diaphragmatic hernia.

5) Tx: surgical exicision.

       after surgery -> increased risk for primary pulmonary neoplasm.

Chapter 332. Acute Inflammatory Upper Airway Obstruction

General Consideration

  * infant and small children 에서는 airway 가 작으므로 older child와 같은  정도의

    inflammation에도 더 narrow 해진다.

  * larynx : composed of four cartilage ( i. e. thyroid, circoid, arytenoid, epiglottic)

  * cricoid cartilage : encircle just below vocal cords

                     narrowest portion of pediatric upper airway.

  * inflammation involving vocal cords and structures inf. to cords : called              laryngitis, laryngotracheitis, laryngotracheobronchitis.

  * inflammation of structures sup. to cords ( i. e. arytenoids, aryepiglottic fold, 

    epiglottis )

# croup 의 특징.

    ① brassy or " croupy" cough with or without inspiratory stridor.

    ② hoarseness.

    ③ sign of respiratory distress.

332.1 Infectious Upper Airway Obstruction

Etiology and epidemiology

# ※92 Virus

    ; *▲common agents

    ; *parainfluenza virus : 75 % of case

    ; adenovirus

  ; respiratory syncyital

  ; influenza

  ; measles viruses

# Mycoplasma pneumoniae : 3.6 %

# ★Cause Of Acute Epiglottis

  ; *H. influenza type b : usual cause

    ; strep. pyogens

  ; strep. pneumoniae

  ; staph. aureus

  ; virus : rare

# Age

  ; *viral croup : 3 mo- 5 yr

  ; *H. influenza & corynebacterium diphtheria croup : 3-7 yr

# incidence

  ; *higher in male

  ; common cold season

  ; *15 % croup family Hx

  ; laryngitis : recur 잘함. 

Clinical Manifestation

Croup (=Laryngotracheobronchitis)

    ; *▲common form of acute upper airway obstruction

    ; URI before cough

Clinical Manifestation

    ; at first, mild brassy cough with intermittent inspiratory stridor

    ; as increasing obstruction

           - worsening cough, nasal flaring & chest retraction

           - respiratory difficulty

           - labored & prolonged expiratory phase of respiration

    ; *slight elevated fever

    ; *worsening at night & often recur with decreasing intensity for several days

    ; older children : not seriously ill.

    ; *다른 가족중 mild respiratory illness를 가짐.

    ; duration

           - *from several days to rarely several weeks

    ; recurrence

           - freguent from 3-6yr

    ; agitation & crying : aggrevate Sx.

    ; prefer to sit up or upright.

    ; air hunger & restlessness.

    ; severe hypoxemia, hypercapnia, weakness.

    ; eventual death from hypoventilation.

    ; hypoxemic child 에 tongue depressor를 사용하는 pharynx의manupulation 은sudden cardiorespiratory arrest 야기할수 있다.

    ; X-ray of nasopharynx & upper airway

           - *typical subglottic narrowing “steeple sign”

※91 Acute Epiglottitis ( Supraglottitis )

    ; dramatic , lethal condition

    ; usually occurs in 2-7yr, peak at 3.5yr

    ; 특징

       - *fulminating course of high fever, sorethroat, dyspnea, rapidly progressive respiratory obstruction, prostration

    - *respiratory distress : first manifestation

    ; 수시간에서 적절한 치료를 하지 않으면 death.

    ; 적절한 치료시 2-3일간 경과는 드뭄.

    ; younger child

           - well at bedtime

           --> *awaken later in the evening, high fever, aphonia, drooling, respiratory distress with stirdor

    ; *no URI family history

    ; older children

           - *initial : sorethroat, dysphagia

    --> severe resp. distress within minutes or hours with inspiratory stridor, hoarseness, brassy cough, irritability, restlessness

    ; neck : hyperextended.

    ; prefer sitting position, leaning forward, mouth open, tongue protuding

    ; progress rapidly to shocklike state : pallor , cyanosis, impaired consciousness.

Physical Exam

moderate to severe resp. distress with insp. & exp. stridor.

inflamed pharynx with abundance of mucus, saliva

 -> rhonchi

 -> 진행시 stridor & breath sound 감소 ( 환자가 tiring )

 -> air hunger with restlessness, agitation

 -> cyanosis, coma, death.

 때로 mild hoarseness & large , shiny, cherry red epiglottis.

Diagnosis

    ; *Direct exam or laryngoscopy

           - *large, swollen cherry-red epiglottis

           - some patients

                   / reflex laryngospasm, acute complete obstruction, aspiration of secretions, cardiorespiratory arrest during or after exam.

                   --> *full preparation for endotracheal intubation한 뒤에 검사를 해야 한다.

    ; ABGA

           - *after definite diagnosis and establishment of artificial airway

    ; *lat. X-ray of nasopharynx & upper airway

           - *before P/Ex of pharynx

Treatment

    ; nasotracheal intubator or tracheostomy 로 airway 확보.

    ; acute epiglotitis 는 2-3 일간 intubated.

    ; parentral AB

           - cefotaxime, ceftriaxone, apmicillin with sulbactam ( unasyn )

    ; concomitant infection.

        - meningitis, pneumonia, cervical adeonpathy, OM,-rarely occur.

Acute Infectious Laryngitis

common illness

대부분 virus에 의함(except for diphtheria)

Clinical Manifestation

     ① sorethroat, cough, hoarseness.

     ② generally mild.

     ③ respiratory distress: unusual except young infant.

     ④ hoarseness and loss of voice

     ⑤ severe case : severely inspiratory stridor, retraction, dynpnea,

                     restlessness.-> 진행시 air hunger & fatigue,

                     agitation 과 exhaustion 반복.

P/Ex:

     ① pharyngeal inflammation

     ② resp. distress

     ③ evidence of high respiratory obstruction.

Laryngoscopy : inflammatory edema of vocal cord & subglottic tissue.

Principal site of obstruction : subglottic area.

Acute Spasmodic Laryngitis (Spasmodic Croup)

    ; often 1-3yr

    ; *similar to croup except findings of absence of infection of pt & family

    ; ★Suggestive Causes

           - viral origin

           - allergic & psychologic factor

           - gasroesophageal reflux

                   / important role in spasmodic croup

    ; pathology

     ① intact epithelium. ( unlike acute infectious laryngotracheobronchitis )

     ② pale , watery edema.

    ; familial predisposition (+)

    ; Sx       

           - evening 이나 night 때 발생 ( sudden onset ): coryza 와 hoarseness

           - barking, metallic cough, noisy insp. , resp. distress. anxious, frightened

         - 대개  afebrile , cool & moist skin.

           - severity of Sx diminish within several hrs.-> 다음날은 appear well   

         - 종종 recur.

★Differential Diagnosis

    1) *bacterial tracheitis : ▲importnat DDx

    2) diphtheritic croup

    ① preceded by URI

    ② slow developed Sx

    ③ serous or serosanguineous nasal discharge

    ④ P/Ex 상 gray white memb. on pharynx

    3) measles croup

    4) aspiration of FB

    5) others

           - retropharyngeal or peritonsillar abscess, ext. compression of airway, intraluminal obst. from mass, endotracheal intubation 후의 edema, angioedema of subglottic area as anaphylaxis, hypocalcemic tetany, IM, trauma, Tm, malformation of larynx.

Complication

1) 15 % of viral croup.

    ① inf. of middle ear, terminal bronchioles, pul. parenchyma : most common.

    ② bacterial tracheitis

    ③ interstitial pneumonia

    ④ bronchopneumonia.

    ⑤ secondary bacterial pneumonia.

    ⑥ supprative tracheobronchitis.

2) epiglottitis ( during the course )

    ① pneumonia

    ② cervical lymphadenitis

    ③ otitis, meningitis, septic arthritis.

3) tracheotomy 후의 TMC Cx.

    ① mediastinal emphysema.

    ② pneumothorax

Prognosis

# death from

    ; laryngeal obstruction.

    ; Cx of tracheostomy.

# unTx epiglottitis

    ; mortality 6 %

# acute LTB, laryngitis, spasmodic croup

    ; excellent prognosis

Treatment

; maintaining or providing for adequate resp. exchange

;AB for Bacterial form

Treatment Of Croup

; shower or vaporizer or cold steam

    --> terminates acute laryngeal spasm & respiratory distress within minutes

    --> same effect observed by child out into cold night air

; induction of vomiting by coughing or by syrup of ipecac

    --> decreased laryngeal spasm

; after laryngeal spasm diminished

    - use of warm or cool humidification

    - near child's bed for 2-3 Days

; ㉿Hospitalization indication

  - actual or suspected epiglottis

  - progressive stridor

  - *severe stridor at rest

  - respiratory distress

    - hypoxemia

    - restlessness

    - cyanosis

    - pallor

    - *depressed sensorium

    - *high fever in toxic- appearing child

; watched carefully for Sx of resp. obstruction

; continous monitering of resp. rate.

  - increasing tachypnea -> first sign of hypoxemia & tatal

; parentral fluid.

; sedative

    - contraindication

; opiate

    - contraindication

; oxygen

; expectorants, bronchodilatory agent, antihistamine

    - *not helpful

Treatment For Laryngotracheobronchitis & Spasmodic Croup.

    ; not response to AB

  ; racemic epinephrine by aerosol.-> transient relief of Sx.

  ; nasotrhcheal intubation or tracheotomy : rarely

★Corticosteroid

    ; *indicated for hospitalized child with croup

    ; *reduce inflammatory edema

  ; prevent destruction of ciliated epithelium

Treatment For Epiglottitis

    ; medical emergency

  ; immediately artificial airway

  ; oxygen

  ; *ineffective racemic epinephrine & corticosteroid

         - artificial airway 의 필요성을 감소시키지 않음.

       - delay definitive Tx.

    ; ceftriaxone or cefotaxime or ampicillin with sulbactam: contunued for 7-10 days

Treatment For Acute Laryngeal Swelling On Allergic Basis    

    ; epinephrine

           - 1: 1000 dilution in dose of 0.01ml/kg to maxium of 0.3ml/dose

    - subcutaneously

    ; isoproterenol by aerosol

  ; corticosteroid ( 1-2 mg/kg/24hr of prednisone every 6h )

    ; endotracheal intubation Ix ( under general anesthesia )

      * reactive mucosal swelling.

      * severe stridor

      * resp. distress unresponsive mist Tx.

Tracheostomy & Endotracheal Intubation

    # epiglottitis

        ; routine introduction

    # ♥Ix of impending resp. failure

       ; > pulse rate 150/min

    ; elevated Pco2 , especially in tiring child.

    # useful to faciliate extubation .

    ; hydrocortisone ( 50-100 mg/ 24hr )

       ; dexamethasone ( 0.25-0.5mg/kg/dose every 6 hr )

       ; racemic epinephrine

332.2 Bacterial Trachetis

# 원인균

    ; *S. aureus - ▲common

  ; parainfluenza virus type I , Moraxella catarrhalis

  ; H. influenza

; 대부분 3세 이하.

; viral ds 의 bacterial Cx ( rather than 1⁰ bacterial illness )

Clinical Manifestation

    ; brassy cough, high fever, toxicity with respiratory distress.

  ; *croup 의 usual Tx 는 ineffective

  ; usually tracheotomy or intubation

    ; major pathology

           - mucosal swelling at cricoid cart. level

           - copicous, thick, purulent secretion.

Diagnosis

 1) bacterial upper airway dis. evidence.

              ① mod. leukocytosis with band form

              ② high fever

              ③ purulent airway secrection.

 2) absence of classic finding of epiglottitis.

Treatment

  ① antimicrobial Tx includes antistaphylococcal agent.

  ② artificial airway.

  ③ supplemental oxygen.

Complication

    ; X-ray 상 patchy infiltrate & focal density.

  ; subglottic narrowing

    ; *rough-ragged tracheal air column

    ; cardiorespiratory arrest

    ; Toxic-shock synd.

Prognosis

  ① excellent.

  ② 적절한 antimicrobial Tx 후 afebrile within 2-3 days

  ③ hospitalization 의 meanduration: 12일.

Chapter 333. Foreign Bodies In The Larynx, Trachea, And Bronchi

Tracheal FB

1) 특징

  ① wheeze

  ② audible slap

  ③ palpable thud at subglottic level.

2) definite Dx : broncoscopy.

Bronchial FB

Clinical manifestations

    ; cough, wheeze, blood wtreaked sputum, metallic taste with metallic FB

    ; slight obstruction ( e. g. bypass valve )

       - passing of air in both direction

       - only slight interference

           - wheeze

    ; obstruction allows air entry but not exit ( i. e. check valve or ball valve )

       - obstructive overinflation

    ; complete obstruction  allows neither air entry not exit

        - obstruction atelectasis

  ; Rt. and left mainstem bronchial foreign body aspiration

       - *equal frequency

  ; episode of chocking, gaging, paroxysmal coughing

    ; if acute episode not occur, latent period of minutes to months with only occasional cough or slight wheezing

       --> recurrent lobar pneumonia , intractable asthma & episode of status asthmaticus.   

    ; Homoptysis

           - months or years after aspiration

# physical exam

  ; tracheal shift.

  ; breath sound : decreased on side of obstruction.

# ♥Vegetal Or Arachidic Bronchitis

  ; *severe condition due to vegetable foreign body(e.g. peanuts)

    ; cough, septic type of fever, dypnea

Diagnosis

# history

# ※94 Physical Signs Of Bronchial Obstruction From Foreign Body

  ; limited chest expansion.

  ; *decreased vocal fremitus

  ; impaired ( i. e. atelectasis ) or hyperresonant ( overinflation ) percussion.

  ; diminished breath sound distal to FB

  ; crakles on uninvolved side ( than involved one )

# X-ray

    ; check valve obstruction

           - obstructive overinflation

        - localize bronchial FB by fluoroscopy.

       - obstructed lung : expanded during expiration.

        - heart & mediastinum : shift to opposite side.

        - diaphragm : low, flattened, fixed on obstructed side.

         - X-ray 상 두 lung 의 현저한 차이 ( 특히 expiration 시 )

    ; stop valve obstruction (complete obstruction)

           - obstructive atelectasis

        - heart, mediastinum : drawn toward obstructed side ( both phase of respiration

        - diaphragm: - obstructed side : high

                - unobstructed side : more normally.

         - X-ray 상 only slight difference at end of inspiration & expiration.

# Direct visualization by bornchoscopy

    ; definitive diagnosis

  ; *rigid or open- tube bronchoscopy

           - diagnostic instrument of choice

Prevention

 ① 제거하지 않으면 serious problem 야기.

 ② 대부분 safely removal

 ③ 만일 진단이 24시간 이상 지연된경우 Cx 의 incidence 증가.

    Cx: i) aspiration pneumonia

        ii) airway trauma 

       iii) need for tracheostomy.

Treatment

; ★endoscopy & removal of FB under direct vision

    - *rigid open- tube bronchoscope as soon as possible

; *pulmonary physiotherapy & bronchodilator

    - *not recommanded

  - due to

           / risk of dislodging distal FB -> obstruct larger airway.

         / endoscopy delay 시켜 morbidity 증가.

# emergency procedure for aphonic & not breathing children

    ; for infants ( < 1Yr )

  ① 4 back blow & 4 chest thrust 반복.

  ② abdominal thrust : not used

  ③ 4 back blow : head 를 낮추고 scapula사이를 heel of hand로 4 blow

    --> loosen FB

  ④ 4 chest thrust : back blow 이후 돌려서 hand position 을 closed cardiac

     compression 과 같이 하여 4 chest thrust.

    --> increase intrathoracic pressure

    --> expulsion of FB.

  ⑤ blind finger sweep of month : not used in infant & young children.

  ⑥ 4 chest thrust 이후 mouth opened & vusualized FB

     --> grasped & removed.

  ⑦ after each sequence, resque breathing for unconscious Pt.

  ⑧ if unsuccessful, repeated.

    ; young child ( > 1 Yr )

  ① placed on back

  ② rescuer kneels next to pt.

  ③ heels of one hand 를 사용하여, umbilicus 와 rib cage 사이중간에서, 6-10

     abdominal thrust by pushing upward & inward from midabdomen.

  ④ 실패시 mouth opened by using tongue - jaw lift --> visualized FB removed.

Chapter 334  Subglottic Stenosis

334.1 Acute Subglottic Stenosis

1. result from  ① acute infection producing edema of subglottic, epiglottic,   

                  arytenoid region

               ② inflammation secondary to inspriration of vegetal FB

               ③ edema of allergic reaction.

               ④ FB lodged in larynx

2. TX

               ① intubation or tracheotomy

                ② medical Tx

334. 2 Chronic Subglottic Stenosis

Etiology

            ①  sequele of high tracheotomy

                (damage of first tracheal ring or cricoid cartilage)

         ② larygeal diphtheria

            ③ syphilis

            ④ TB

            ⑤  radiation burns

            ⑥ external trauma

            ⑦ neonatal intubation : most common cause

            ⑧ congenital laryngeal stenosis : autosomal dorminent trait

            ⑨ "silent" gastroesophageal reflux

Clinical manifestation

    ① dyspnea with stridor, suprasternal, supraclavicular, intercostal retraction

    ② limited to inability to decannulate patience's tracheotomy

            or remove endotracheal tube

    ③ scarring and stenosis in subglottic region

    ④ necrosis of cartilage : occassionally                                      

Diagnosis

       ① direct laryngoscopy

    ② X-ray

Treatment

       (1) milder case : not need Tx

       (2) mild case of difficulty in decanulating pt's tracheostomy

        ① replacing tracheostomy cannula with smaller one and closure of this tube

                --> reeducate pt to mouth breathing

               --> removal of cannula

        ② dilation through direct laryngoscope

    (3) external surgery

Prognosis

 : good, but treatment require months or years        

Chapter 335. Trauma To The Larynx

Birth Trauma

Postnatal Trauma

Chapter 336. Neoplasms Of The Larynx

# Papilloma

    ; *▲common laryngeal tumor

Chapter 337. Bronchitis

337.1 Acute Bronchitis

Clinical Manifestation

Treatment

    ; no specific therapy

    ; *pulmonary drainage by frequent shifting in position

    ; high humidity

           - not shortening the duration of illness

    ; cough suppressants (including codein)

           - symptomatic relief

    ; *antihistamines, expectorants

           - ★not helpful

    ; AB

           - *not shortening the duration of viral illness or decrease the incidence of bacterial complication

    ; ※Repeated Attacks 시 고려 질환

           - respiratory tract anomalies, foreign bodies, bronchiectasis, immune deficiency, tuberculosis, allergy, sinusitis, tonsillitis, adenoiditis, cystic fibrosis

337.2 Chronic Bronchitis

Chapter 338. Bronchiolitis

; during 1st 2yr of life

    - *peak incidence : 6mo

Etiology And Epidermiology

# ★RSV

    ; *more than 50%

# parainfluenza 3 virus, mycoplasma, some adenoviruses

Pathophysiology

# bronchial obstruction

    ; *due to edema and accumulation of mucus and cellular debris

           *and by invasion of the smaller bronchial radicles by virus

# ★ball valve obstruction

    ; early air trapping and overinflation

Clinical Manifestation

    ; Hx of exposure to older children or adults with minor respiratory diseases within a week

    ; initially mild URI symptoms

           - serous nasal discharge and sneezing

    ; *diminished appetite and fever 38.5-39℃

    ; *gradual development of respiratory distress

           - *paroxysmal wheezy cough, dyspnea, irritability

# ※94 X-ray findings

    ; hyperinflation of the lungs

    ; increased AP diameter on lateral view

    ; scattered areas of consolidation - 30%

    ; atelectasis

Differential Diagnosis

    ; bronchial asthma

           - *▲common confused disorder

           - ㉿DDx points

                   / FHx

                   / *repeated episodes : ▲important

                   / sudden onset without preceding infection

                   / markedly prolonged expiration

                   / eosinophilia

                   / immediate favorable reponse to single dose of aeroxolized albuterol

    ; congestive heart failure

    ; FB in the trachea

    ; pertussis

    ; organophosphate poisoning

    ; cystic fibrosis

    ; bacterial bronchopneumonias

Course And Prognosis

# *▲critical phase

    ; *during 1st 48-72hr

# recovery

    ; *complete in a few days

# case fatality rate

    ; *1%

# ※Death

    ; prolonged apneic spells

    ; severe uncompensated respiratory acidosis

    ; profound dehydration secondary to loss of water vapor from tachypnea, inability to drink fluids

※90,91,92 Treatment

# if respiratory distress, hospitalization

# supportive treatments

    ; only

# ribavirin (virazole)

# bronchodilator

# AB

    ; *no benefits

# corticosteroids

    ; *no benefits

Chapter 339. Bronchiolitis Obliterans

Pathogenesis

    ; brochioles, small airway injury

           --> granulation tissue & fibrosis during repair

           --> airway obstruction

# ★Associated Conditions

    ; inhalation of oxides of nitrogen

    ; connective tissue diseases

    ; drug (e.g. penicillamine)

    ; pul. infection

           - meales, influenza, adenovirus, mycoplasma, pertussis

  ; lung transplantation complication

Clinical Manifestation

 ① 초기 ; cough, respiratory distress, cyanosis

 ② 진행시 ; dyspnea가 증가, productive cough, wheezing

Diagnosis

    ; X-ray

           - range from normal to miliary Tbc pattern

           - ★cf) Swyer-James syndrome

                   / 10%

                   / *unilateral hyperlucency and decrease in pulmonary vascular marking

    ; Bronchography

           - bronchioles의 폐쇄와 함께 lung의 periphery에 no contrast.

    ; CT ; Bronchiectasis

  ; ★lung biopsy

           - *confirmed diagnosis

Treatment

 ; no specific Tx

 cf> corticosteroid Tx -  성인에서는 효과가 있으나 소아에서는 입증된 data가 없음.

Prognosis

 ; 대부분 생존하나 소수는 빠르게 진행되어 초기증상 발현 수주내에 사망.

339.1 Follicullar Bronchitis

1. Etiology ; unknown

2. 2주내에 tachypnea와 cough를 보임

3. 청진상 diffuse crackles

4. X-ray ; diffuse interstitial pattern.

5. CT ; subtle interstitial nodules

6. 진단은 lung biopsy로

7. Px ; 대부분 점차적으로 호전되나 소수는 respiratory failure로 life threatening.

8. Tx ; corticosteroid에 반응하는 예도 있음.

Chapter 340. Aspiration Pneumonia & GER Related Respiratory Disease

    * GI content의 respiratory tract내로의 흡입을 막는 여러 가지 기전

      (Table 340-1)에도 불구하고 dysfunctional swallowing 및 GER의 많은

      예에서 respiratory ds를 야기하거나 악화시킨다.

    * reflux와 관련된 호흡기 질환의 기전은 aspiration 및 mechanical &

      chemical effect 그리고

      airway 및 esophagus로부터의 신경학적인 영향 등이다. ( Fig 340-1)

340.1 Aspiration Pneumonia

Aspiration Of Food & Vomitus

# ★Predisposing Factors

    ; with obstruction

           - esophageal atresia, duodenal obstruction

    ; without no obstruction

           - hypotonic, weak & debilitated infants & children

  ; familial dysautonomia

    ; impaired consciousness

Clinical Manifestation

    ; ★brief latent period

           - *hydrochloric acid is important determinants

       - 90%이상 1시간내에 Sx 출현 

    - *거의모두 2시간내에 Sx 출현

    ; *fever, tachypnea, cough

    ; Apnea, Shock

  ; P/E   ① diffuse crackles, wheezing

           ② cyanotic

  ; X-ray ; alveolar & reticular infiltration

Treatment

    ① 기도내의 즉각적인 suction

  ② Oxygen

  ③ Endotracheal intubation with suction

    & mechanical ventilation - severe case시

    ; AB

           - ★previously healthy nonhospitalized patients

                   / *Clindamycin or PC

           - chronically ill hospitalized patients

                   / *added to Aminoglycoside

Prognosis

    ; *대개 2 주내에 infiltrates가 소실

  ; mortality rate ; 약 25%

Aspiration Of Baby Powder

    ; Zinc stearate

        - *드물지만 가장 dangerous

  ② Tx ; 고습도하에서의 oxygen therapy

  ③ 그외 magnesium silicate, calcium undecylemate

  ④ Talc ; i) asbestos와 관련이 있으며 malignancy 유발 가능성

           ii) severe dyspnea시 systemic corticosteroid Tx가 유용.

Pneumonitis From Other Chemicals

  ①  +-high concentration ; 염증반응, 세포 침윤, acute respiratory distress 야기.

      +- low concentration ; 육아종 형성의 chr. interstitial pneumonitis

  ② 예 ; Shellac, polyvinylpyrrolidone, gum arubic, beryllium, mercury vapors,

          chlorine

  ③ corticosteroids가 치료에 효과.

340.2  Hydrocarbon Pneumonia

Etiology

    ; furniture polish (광택제), kerosene, charcoal lighter fluid, gasoline

Pathogenesis

    ; interact with pul. surfactant

           --> alveolar collapse

    ; alveolar macrophage injury

Clinical Manifestation

    ; *cough, vomiting follow ingestion immediately

    ; *fever 38-40℃ within hours

  ② pul. Sx ;  dyspnea,

               ↓resonance on percussion

               ↓B/S, rale 

  ③ pul. involve는 P/E보다 chest X-ray에서 더 자주 disclosed

  ④ 대부분 2-5일이면 recover

  ⑤ systemic Sx : sommlence, convulsion, coma

Complication 

① pneumothorax

       ② subcutaneous emphysema

       ③ pleural effusion, emphysema

       ④ pneumatocele - 1주후에

       ⑤ 2⁰ inf. with bacteria or virus

Treatment

; *never sent home in less than 6hr although asymptomatic

; ♥Admission Ix

           - symptomatic on first exam

           - symptomatic during 1st 6hr obseration

           - ingestion of toxic agent (eg. furniture polish)

; *no pulmonary therapy before symptoms develops

; induction of vomiting or gastiric lavage

    - contraindication

    - *large volume hydrocarbon in stomach 일때는 실시

           / CNS toxicity 막기위해

; AB

    - not recommended as routine use

; corticosteroid

    - *no benefit

Prognosis

      Cx없이 대개 survive하지만 일부는 resp. failure와 death로 진행

      Px factor  ①  volume of ingestion or aspiration

                 ②  specific agent

                 ③  medical care의 adequacy

340.3  Lipoid Pneumonia

; lipoid material의 aspiration으로 인한 chr. interstial, prolif. inflammation

; deblilitated Pt.에 ↑

Pathogenesis 

# aspiration 관련 factor

    ① intranasal instillation of oil

    ② cleft palate, debilitation, feeding시 horizontal position

    ③ forced feeding

     : 특히 우는 아이에 cod liver oil, castor oil, mineral oil을 먹일 시.

# 흡입된 oil의 종류에 따라 pul. reaction의 정도가 차이남.

  ; vegetable oil  +- olive, cotton seed, sesame oils

                      +- ↓ irritation, minimal inflammation

                      cf) chaulmoogra(인도산 나무) - extensive damage

  ; animal oil

           - cod liver oil, milk

    - *▲damage

    ; liquid petrolatum, lip gross

# lung reaction

     ① initial : interstitial proliferative inflammation, exudative pneumonia가능

     ② 2nd stage : diffuse, chr. proliferative fibrosis

                   때로 acute infectious bronchopneumonia

     ③ 3rd stage : multiple localized nodules

                  tumor-like paraffinoma

Clinical Manifestation

   1) cough : TMC

   2) severe case 시 dyspnea

   3) 2^o bronchopneumonia가 common

# X-ray findings

    ; mild involvements

           - *increase in density & extent of hilar shadows

    ; severe involvements

           - *greater density of perihilar shadow widen all direction

    ; maybe limited Rt. lung

           - recumbent시 (RUL)

Prevention

   1) oily vehicle의 intranasal medication을 피함.

   2) 가능한 minimal oil, cod liver oil, castor oil의 복용을 피할 것.

   3) 자주 regurgitation 및 vomiting을 보이는 infant는 prone position으로 둘 것.

Treatment

   1) no specific Tx

   2) position change → ↓hydrostic pneumonia

340.4  ★Respiratory Disorders Caused Or Worsened By GER Or It's Treatment

Aspiration pneumonia

     특히 의식이 감소되어 있는 환자에서 흔함.

     chemical pneumonitis, asthma-like Sx 야기

Asthma

     천식환아는 정상아에 비해 reflex정도가 abnormal

     reflex는 vagal pathway에 의해 천식을 야기하거나 악화시킴

Bronchopulmonary dysplasia (BPD)

     reflex는 BPD의 course를 지연시키며 이것의 치료시 pul. function이 호전됨

Cystic fibrosis

     이 질환자의 많은 수에서 GER를 가짐.

Tracheoesophageal Fistula

     모든 환자들은 esophageal dysmotility를 가지며 대부분 reflex를 보임

Obstructive Apnea

Central Apnea & Apparent Life-Threatening Events

Stridor

     laryngomalacia와 같은 mild airway compromise의 경우, reflex시 stridor를

     보이며 antireflex therapy시 stridor가 감소되어짐. 

Hoarseness

     성인에서 후두의 염증과 부종이 reflex와 관련이 있으며,

     acid suppression therapy에 후두증상이 호전됨.

     소아의 경우에도 관련성이 가능

Cough

     reflex의 증상으로 가능하며, antireflex 치료로 호전됨.

Hiccup

Respiratory Side Effects Of Antireflex Tx

     Bethanechol의 경우 bronchospasm을 야기

Chapter 341.  Silo filler Ds

1. 정의 : N2O의 inhalation에 의한 acute or subacute interstitial Pn 

2. Dx : ① exposure Hx (4주이내)

        ② Sx : dyspnea, wheezing, cough, nausea, choking or fatique

           P/E : 청진상 rales (약 1/3에서)

        ③ X-ray : infiltration or edema

        ④ biopsy or autopsy

3. Tx : corticosteroid

Chapter 342.  Paraquat Lung

  1. 제초제로 사용되는 dipyridylium compoud로 lung에 선택적으로 축적되며,

      high toxic

  2. 수일-수주내 resp. failure로 사망

  3. pul. lesion : ① 2o to systemic absorption by GI, skin or lung

                    (오염된 마리화나 흡연) 

                 ② prolif. bronchiolitis, alveolitis,

                    intraalveolar hyaline membrane & fibrosis를 보임

Treatment

    ; no treatment except supportive Tx

    ; *O2 --> increase pul. toxicit

Chapter 343.  Hypersensitivity To Inhaled Materials

organic dust의 반복된 inhalation → chr. pneumonitis

Etiology

   ① moldy hay ( farmer's lung)

   ② maple bark (maple bark stripper's ds)

   ③ sugar cane fiver ( bagassosis )

   ④ red-wood tree bark

   ⑤ pigeon dropping & feather ( pigeon breeder's ds )

   ⑥ cheese

   ⑦ desiccated pituitary powder

   ⑧ dusty output from air conditioner

   ⑨ fungus or mod

Clinical Manifestation

   : 노출 수시간내에  cough, dyspnea, chest pain, fever

    - Ag 에 계속  contact시 severe dyspnea와  cyanosis로 진행

     ( 이때  X-ray : diffuse, fine, intestitial or nodular density &

                     peripheral alveolar infiltrates )

    - 때로 pulmonary function의 irreversible loss

    ** suspect

       : cough, fever 때로 dyspnea같은 mild Sx을 가진 어린이에서

        bronchopneumonia가  AB로 적절히  치료함에도 지속될 때

Pathology

  1)  조직학적으로 plasma cells, lymphocytes, epitheliod cell 그리고 giant cell의

      축적과 함께 subacute granulomatous inflammation으로 구성

  2)  계속 노출시 fibrosis로 replaced

Diagnosis

  1) moderate to marked leukocytosis ( acute attack시 )

  2) serum Ig * ( G,M,A )

  3) PFT상 restrictive pattern

  4) hypoxemia without hypercapnia

  5) skin test상 delayed hypersensitivity response

  6) 피부생검에 의해 Arthus reaction이 증명

  7) 주어진  Ab 에 대한 serum precipitin  증명

  8) lung biopsy ; diffuse fibrotic or granulomatous respose

  9) inhalation challange test

Chapter 344. Pulmonary Aspergillosis

# Allergic bronchopumonary aspergillosis (ABPA)

  ; *allergy to organism(aspergillus fumigntus)

    ; *ABPA without infection or tissue invasion

           - *▲common aspergillus-related disease in children

    ; mostly in patients with chronic pulmonary disease

Clinical Manifestation

; *immunosupressed or chr. illed child가 acute onset of cough, wheezing, low grade fever

; productive cough

    - *occasionally brown plug containing hypae

; immediate skin reaction : strong (+)

    - Arthus type III가 skin test후 증명

; Aspergillus의 strong suspect 경우

     ① aspergillus Ag에 대한 Ab의 precipitation

     ② (+) skin test

     ③ serum IgE ↑

# ★Definite Diagnosis

    ; substantial eosinphilia

    ; aspergillus specific IgE or IgG in serum

Treatment

# Aerosolized amphotericin or tracheal direct insfillation of amphotericin

    ; not estabilished

# systemic amphotericin B (0.5-1 mg/kg/24hr iv) or 5-fluorocytosine (50-150 mg/kg/24hr)

# *systemic corticosteroids

    ; *predenisone 0.5mg/Kg/day for 2wks and then same dose on alternate day for 3mo

    ; *Tx of choice

# Intraconazole + systemic corticosteroid

# aspergilloma

    ; surgical resection with local instillation of amphotericin B

 Px factor : underlying chr. illness

invasive aspergillosis시  antifungal Tx는 효과 없을 수도 있음

; amphotericin B + 5-FC   * for 2-3wks

Chapter 345. Loeffler Syndrome (= Eosinophilic Pneumonia )

# ★Characteristics

    ; widespread transitory pulmonary infiltrates

           - variable size but *resemble miliary Tbc

    ; eosinophilia (70%까지)

# clinical course

   ① 심하지 않으면서 몇일에서 몇달까지 다양.

   ② paroxysmal attack of cough, dyspnea, pleurisy, little or no fever

   ③ hepatomegaly ( 특히 infant & young children에서 )

   ④ hyperglobullinemia - i) hepatic dysfunction

                         ii) parasitic invasion에 대한 response로

Etiology

    ; helminthic infection

           - *▲common

     i) toxocara canis (TMC) & cati

     ii) roundworm

      ; Ascaris lumbricoides, Strongyloides stercoralis

        hookworm

     iii) paragonimiasis

    ; Drug reaction

      : aspirin, PC, sulfonamide, imipramine

# hypereosinophilic synd.

   ① 소아에서는 rare

   ② eosinophilia가 6개월이상 지속

   ③ acute lymphoblastic leukemia의 초기증상으로서 가능

# variant of eosinophilic pn.

   : 최근에는 더 acute course of fever와 rapid progression to

     severe hypoxia, eosinophilia, diffuse pulmonary infiltration

    → oral C-S에 빨리 response하며 relapse도 없다.

Differential Diagnosis

Chapter 346. Pulmonary Involvement In Collagen Ds

# rheumatic pneumonia

   1) fatal rare, Cx of acute RF, rheumatoid arthritis, or other CT ds.

   2) 특징   ① extensive pulmonary consolidation &

                rapid progressive functional deterioration을 보이며

             ② pathology    i) alveolar exudate

                            ii) interstitial infiltrate

                            iii) necrotizing arteritis

   3) X-ray ; pul. edema와 유사한 infiltration이 일시적으로

   4) Tx ; no spesific , but immunosuppressive drug가 효과있다.

Chapter 347. Desquamative Interstitial Pneumonitis

# pathologically Characteristics

    ; massive proliferation & desquamation of TypeII alveolar cell

    ; thickening of alveolar wall

  ; many macrophages filling alveolar spaces

# lonstanding DIP 3→ chr. interstitial fibrosis로 진행

# 대개 URI 선행

# congenital rubella와 관련된 2 case있음

# circulating immune complex와 alveolar deposit of Ig G & complement

       → 이 질환의  immune basis를 시사         

Clinical Manifestation

  ; usually slowly developed

    ; tachypnea & dyspnea

  ; non productive cough, anorexia, wt. loss

  ; cyanosis

Laboratory Finding

   1) X-ray

     ① diffuse, hazy, ground glass appearance at lung bases

     ② poorly defined hilar density

   2) Hypoxemia ; ventilation-perfusion abnormality에 의해서

                  → diffuse defect → severe exercise intolerance

# Definite Diagnosis

    ; open lung biopsy

Treatment

   1) often recover spontaneously (but 1세 이전에 진단시 주목 ! )

   2) pulmonary status악화나 X-ray상 빠른 deterioration시

     → open lung biopsy로 definit Dx후

     → C-S  ①  Sx의 빠른 resolution

              ② X-ray의 gradual improvement

   3) corticosteroid-resistant Pt.의 경우에 chloroquime phosphate가 효과

     ( 10mg/Kg/24hr)

   4) Tx가 빨리 stop될 경우 relapse 가능

Chapter 348. Hypostatic Pneumnia

1. 발병   ① marasmic state에서 혹은 수술 후 prolonged passive pulmonary 

            congestion후 생김

          ② one position으로 오래 누워있을 때

2. P/E ; dullness on percussion, 약한 호흡음, 수포음

3. specific Tx는 없고 prophylaxis가 가장 중요 : frequent position change

Chapter 349. Pulmonary Hemosiderosis

; abnormal accumulation of hemosiderin in the lungs

    - resulting from diffuse alveoli hemorrhage

# ♥4 Type of Primary Pul. Hemosiderosis

  ① idiopathic form

  ② form asso. with cow's milk hypersensitivity (Heiner synd.)

  ③ form asso. with myocarditis

  ④ form asso. with progressive GN (Goodpature synd.)

# ♥3 Type of Secondary Pul. Hemosiderosis

  ① asso. with mitral stenosis & chr. LVF

  ② asso. with collagen ds.

  ③ asso. with hemorrhagic ds.

Idiopathic Primary Pul. Hemosiderosis

   1) onset ;  대개  childhood

   2) Sx ; ① recurrent or chr. ds.로서 cough, hemoptysis, dyspnea, wheezing

              때로는 cyanosis 보임

           ② acute attack시는 대개 2-4일 지속되며, fever 를 보이기도 함.

           ③ 소수에서 초기에 chr. iron deficiency anemia와 관련된 Sx를 보임

           ④ microcytic, hypochromic anemia

Diagnosis

    ; ★Open Lung Biopsy

           - intra-alveolar hemorrhage

           - *large numbers of hemosiderin-laden macrophages

           - alveolar epithelial hyperplasia

           - interstitial fibrosis

           - sclerosis of small vessels

           - absence of Ig or complement deposition on the alveolar basement membrane

    ; closed-needle biopsy

    ; BAL

           - hemosiderin-laden macrophage

② X-ray ; minimal infiltration에서부터 2차적인 atelectasis, emphysema

                그리고 hilar lymphadenopathy를 동반한 

                massive pul. involvement까지 다양

    ③ open lung biopsy

      - 조직학적으로 intra-alveolar hemorrhage,

         hemosiderin을 함유한 macrophage,

        alveolar epithelial hyperplasia, intestitial fibrosis 그리고  small vessels의

        sclerosis를 볼 수 있음

    ④  Bronchoalveolar lavage - hemosiderin을 함유한  macrophage를 발견

Prognosis

     - Pt.의 약 1/2에서 급성 폐출혈 및 진행적인 폐부전으로 1-5년내에 사망

Treatment

     - corticosteroid  ( prednisone, 1mg/Kg/24hr )

Primary Pul. Hemosidrosis With Hypersensitivity To Cow's Milk (Heiner Synd.)

    ; typical pictures of idiopathic hemosiderosis

    ; *high serum titers of precipitiins to cow's milk

  ; *positive results on intradermal skin test to cow's milk protein

    ; chr. rhinitis, recurrent OM, GI symptoms, growth retardation

    ; Treatment

           - 식이요법 ; cow's milk를 제거

           ii) corticosteroid

           iii) cyclophosphamide

  ; 다른 form에 비하여 Px는 좋음

Primary Pul. Hemosiderosis With Myocarditis

   - heart enlargement와 함께 idionpathic hemosiderosis

Primary Pul. Hemosiderosis With Gn (Goodpasture Synd.)

; *young adult male

; initial Sx

    - similar to idiopathic type

; *usually proliferative or membranous GN at initial attack

; renal biopsy

    - IgG deposit along alveolar & glomerular basement membrane

; *anti-GBM Ab in serum

; *mostly progressive renal disease with hypertension & eventual renal failure and death

Chapter 350. Pulmonary Alveolar Proteinosis

; rapidly progress to respiratory failure

; *alveolar space filled with periodic acid-Schiff(PAS)-positive proteinaceous material, rich in lipid

# 2 clinical form  

    ① sporadic type

  ② congenital form

Congenital Alveolar Proteinosis

    ; *full-term newborn develops rapidly progressive respiratory distress

    ; similar to RDS

    ; Etiology

           - *SP-B (surfactant apoprotein B)의 genetic homozygous deficiency

    ; pathology

           - alveolar proteinosis

           - desquamation & hyperplasia of alveolar septum

    - interstitial fibrosis

           - impared alveolarization

  ; Clinical Manifestation

           - rapid progressive respiratory distress

    ; X-ray

           - *fine, diffuse infiltrate from hilum to periphery(“butterfly distribution”)

           - later nodular or lobar density, infiltration

    ; PET (Positron emission tomography)

        - vascular permeability와  protein flux가 증가

  ; ★DDx

           - *persistent fetal circulation, meconium aspiration, hyaline membrane ds., alveolar capillary dysplasia, CHD ( esp. TAPVR )

Diagnosis

    ; conform by lung biopsy

  ; RFLP analysis of polymerase chain reaction-amplified gemomic DNA

  ; molecular assays of chorionic villus biopsy

         A/F에서   surfactant protein의 측정

        → 가족력이 있을때 antenatal diagnosis로 가능

Treatment

    ; *replacement therapy - not effective

    ; current therapy

           - ventilatory support & ECMO

                   --> *lung transplantation

Aduldt Form Of Alveolar Proteinosis

   1) 소아에서는 rare

   2) male에서  female보다 3배 더 많다 

   3)

    

2 form 의 차이점	distribution of pathologic  process	amount of alveolar  SP-A
1o form	diffuse	abundant
2o form	patchy	scant

 

 

   4) Sx : dyspnea, fatigue, cough, wt. loss, chest pain, hemoptysis

           →  말기에는 cyanosis와  digital clubbing을 보임

   5) Lab.   1 PFT ; restrictive pattern

             2 ABGA ; marked hypoxemia with respiratory alkalosis

   6) Dx - lung biopsy

   7) Tx - whole lung bronchoalveolar lavage

Chapter 351. Idiopathec Diffuse Intestitial Fibrosis Of Lung

(=Hamman Rich Syndrome)

1. 기전 : uncontrolled imflammatory process

         → chr. imflammation

         → progressive fibrosis

2. Sx

   ① incidious onset of dyspnea (처음에는 운동시 나타나나

      진행시 휴식상태에서도

   ② dry cough가 주 , 때로 productive of blood

   ③ 대개 aferile

   ④ 진행시 anorexia, Wt. loss, fatigability

     → finally cyanosis, clubbing, cor pulmanale, Rt. heart failure

   ⑤ 청진시 lung은 대개 clear하나 종종 rale도 detect

   ⑥ death : intercurrent pulmonary infection으로 인한 resp. failure로

3. X-ray : progressive widespread granular or reticular mottling

           or small nodular densities

4. ⁶⁷Ga scan 상 (+)

5. Tx

  ① C-S는 Symptomatic relief는 가능하나 ds.의 progress를 바꾸거나

     pul. function 호전은 없다.

  ② immunosuppressant drug : some adult에서 benefit

Chapter 352. Pulmonary Alveolar Microlithiasis

   ① rare ds. of unknown eti.

   ② familial incidence ( 약 50% )

   ③ no secific metabolic abnormality

      S-Ca &P ; n'l

   ④ Dx ; lung biopsy

   ⑤ Tx ; 유용한 방법은 없다.

   ⑥ i) Pt. 발견시 가족구성원의 screening

      ii) respiratory infection에 대한 즉각적인 치료

      iii) 금연 및 매연 노출주의

      iv)  철저한 immunization (measles, pertussis, influenza )

Chapter 353. Atelectasis

Acquired Atelectasis

★Etiology

; 3group

External Pressure

    ; direct interference with expansion of lungs

           - *pleural effusion, pneumthorax, intrathoracic tm., diaphragmatic hernia

    ; compression of bronchus or bronchiole

           - enlarged LN, Tm, cardiac enlargement

Intrabronchial Or Intrabronchiolar Obstruction

    ; intrabronchial

           ① foreign body

           ② neoplasm

           ③ granulomatous tissue ; Tbc

           ④ secretion (mucus plug)

                   : cystic fibrosis, bronchiectasis, pul. abscess, asthma, chr. bronchitis, acute laryngotracheobronchitis

    ; intrabronchiolar

           - *bronchiolitis, interstitial pneumonitis, asthma

Reduced Amplitude Of Respiratory Excursion Or Respiratory Paralysis

    ; neuromuscular abnormalities

           - cerebral palsy, poliomyelitis, spinal muscular atrophy, MG

    ; osseous deformities

           - rickets, scoliosis, kyphosis, scleroderma, overly restrictive casts, surgical dressings

Pathology

    Atelectatic area - firm & deep red

Clinical Manifestation

    1) small area - aSx

    2) large area of previously n'l lung에 발병시          

        ; dyspnea with rapid shallow respiration,

          tachycardia, cyanosis

    3) area of severe pre-existing ds.에 발병시

       ; transient pain, 기존질환의 이학적 소견을 보임.

Diagnosis

  ① X-ray

         ② Brinchoscopic exam.

Prognosis

: ① atelectatic area - susceptible to infection

              ∵ mucociliary clearance가 결여, cough가 ineffective

          ② 지속시  Cx ; pul. abscess

Treatment

    1) ♥Bronchoscopic Exam Indication

        ; *FB, any other bronchial obstruction

                   --> immediately

           ; *persist for several wks : 8wks

    2) position change & deep breathing

    3) O2 : dyspnea시 (95%O2 = 5%CO2 혼합한 것)

    4) morphine과 atropine은 가능한 피함

    5) postural drainage

    6) AB도 때로

    7) asthma시 : bronchodilator & C-S

    8) lobectomy

       ; ★Indication

                   - chr. infection threating to remainder lung

         - bronchiectasis

         - systemic Sx(e.g. anorexia, fatique)

Massive Pul. Atelectasis

Etiology

    ; postop. Cx

           - *▲common

    ; trauma / asthma / pneumonia / tension pneumothorax / FB asporation

  ; paralysis in diphtheria or poliomyelitis

Clinical Manifestation

    1) op. 후 24시간내에 onset : dyspnea, cyanosis, tachycardia

    2) 매우 anxious, chest pain 호소, prostration, fever(39.5-40^oC)

  3. P/E

    1) affected chest : flat

    2) ↓respiratory excursion

    3) dullness to percussion

    4) (-) or feeble breath sound

  4. X-ray

    1) lower lobe가 흔히 involve

    2) heart & mediastinum이 affected side로 displace

    3) collapsed

    4) elevation of diaphragm

    5) narrowing of intercostal space

Prevention

    1) op중 - 마취중 충분한 ventilation

    2) op후  ① position change 자주

             ② oropharynx의 secretion 을 aspirate

             ③ 의식회복 후 deep breathing

Treatment

    1) bilat. atelectasis시  즉시 bronchoscopic aspiration 시행

    2) unilat. atelectasis시

       ① 건강한 lung쪽으로 위치하여 forced coughing or crying시킴

       ② bronchoscopic aspiration ← ①에 실패시

    * relapse 많다.

Chapter 354. Emphysema & Overinflation

# emphysema

    ; distension with irreversible rupture of alveoli

# overinflation

    ; reversible disttension without alveolar rupture

Localized Obstructive Overinflation

Etiology

    ① FB & inflammatory reaction

    ② intrabronchial Tbc or Tbc of tracheobronchial LN

    ③ intrabronchial or mediastinal Tm

    ④ abnormally thick mucus ; asthma, cystic fibrosis

Unilateral Hyperlucent Lung

★Etiology

    ; pneumonia

           - more than 1/2

    ; adenovirus infection

    ; *anomalous location of Lt. pul. a. (=pulmonary slings)

           - overinflation of all 3 lobes of Rt. lung

    ; HMD

    ; absent pul. valvetype of TOF

    ; 2nd aneurysmal dilatation of pul. a.

Clinical Manifestation

    ② 대개 Pn의 Sx및 Sg을 보이지만 소수에서는 hemoptysis

    ③ P/F : hyperresonance & decreased BS

    ④ Tx : no specific Tx

Congenital Lobar Emphysema

    ; *usually apparent in neonatal periods

           - 5% appear 5-6mo

    ; severe respiratory distress

    ; ★Lt. upper lobe

           - *▲common

(4) Tx

      ① medical management with (selective) intubation or

        high frequently ventilation 

      ② immediate op. & excision of lobe

         ←  cyanosis와 심한 respiratory distress가 나타나면

354.1 Generalized Obstructive Overinflation

; reversible

; infant에 많다.

Etiology

    ① asthma

  ② CF

  ③ acute bronchiolitis

  ④ interstitial pneumonitis

  ⑤ atypical form of acute LTB

  ⑥ aspiration of zinc stearate powder

  ⑦ miliary Tbc

Clinical Manifestation

    ① expiratory type of dyspnea

  ② respiratory rate ↑& ↓ respiratory excursion

  ③ air hunger, chest wall retraction

  ④ severe한 경우 cyanosis

  ⑤ percussion시  hyper-resonant

  ⑥ Fine or medium crackles

# X-ray

  1) both diaph. low & flattened

  2) wide intercostal space

  3) lung : hyperlucent

  4) fluoroscopic exam.

    ① restricted diaph. movement

    ② exp.동안 n'l downing of diaph ↓

    ③ flattened diaph.

    ④ exp. 동안 lung의 air retention ↑

354.2 Bullous Emphysema (= Pneumatocele)

Etiology

    ; overdistension & rupture of alveoli during birth of 직후

          ② sequele of pneumonia or other inf.

          ③ Tbc

2. DDx : pul. abscess

3. 대부분 수개월 내 자연 소실

  cf> severe respiratory & cardiac embarrassment의 경우 Aspiration or surgery

354.3 Subcutaneous Emphysema

Etiology

    ① fx of orbit (nasal sinus로부터 air leakage)

  ② tracheotomy

  ③ deep ulceration in the pharyngeal region

  ④ esphageal wound

  ⑤ perforating lesion of larynx or trachea

  ⑥ Cx of thoracentesis

  ⑦ asthma의  Cx   

  ⑧ abdominal op의 Cx

  ⑨ gas producing bacteria

2. self-limited & no-specific Tx

  :  드물게 trachea 주위의 soft tissue의 air로 인해 tracheal compression                → op. 요함.       

354.4 α1-Antitrypsin Deficiency & Emphysema

; α1-antitrypsin homozygous deficiency로

    --> early onset of severe pancinar emphysema

; 소아에서는 pul. ds.로 rare한 cause

# α1-antitrypsin

  - lung의 dead bacteria나 leukocyte로부터 유리되는 proteolytic enzyme을inactivation시킴

    → deficiency시 pul. tissue의 proteolytic destruction과 emphysema야기       

3. Type : genotype, Pi type

    +- n'l : Pi type MM

    +- abn'l : null / null ZZ, SZ → early onset emphysema

4. Tx

  1) Danazol ( testosterone analog )

     ; hepatic α1-antitrypsin synthesis ↑

  2) α1-antitrypsin

Chapter 355. Pulmonary Edema

1. Eti. : pul. capillary로부터 alveolar space와 bronchiole로의

        fluid transudation에 의해서

  1) LVF : ↑ pul. venous pr.

  2) hypervolemia

  3) acute or chr. nephritis

  4) pneumonic & other inf. with toxicity (rare)

  5) poisoning by barbiturate, morphine, epinephrine, alcohol

  6) inhalation of toxic gas : illuminating gas, ammonia, NO2

  7) ingestion & aspiration of highly volatile hydrocarbon

      ( e. lighter fluid )

2. Cl/m

  1) 대부분 rapid onset

  2) Sx ; ① breathing difficulty, chest discomfortness or chest pain

          ② cough with frothy, pink-tinged sputum

          ③ tachypnea, rapid & week pulse, pale, 때로  cyanotic

  3) P/E상  +- dullness to percussioin

            +- moist, bubbly rale on lower chest, 초기에 wheezing가능

  4) X-ray ; ① diffuse perihilar infiltrate - butterfly distribution

             ② peripheral & horizontal line - Kerley B

3. Tx

  1) ↑O2 by IPPB

  2) morphine sulfate (0.1mg/Kg)

    ; O2와 함께 dyspnea relief

  3) 만일 2^o to excessive fluid/blood IV administration or cardiac failure

    → ① diuretics (furosemide 1mg/Kg)

       ② digitalis

       ③ bronchodilator

       ④ tourniquet to extremities

       ⑤ withdrawal of blood

  ** High altitude pulmonary edema

    1. children & adolescent at altitude above 2700m (8860ft)

    2.  ① cough, chortness of breath, vomiting, chest pain : TMC Sx

         → exposure 수시간내에

       ②  X-ray ; bilat. patchy pul. infiltration

       ③ recovery : 48시간내

    3. Tx ; O2, Bed rest, diuretics, antibiotics & corticosteroid

Chapter 356. Pul. Embolism & Infarction

# *uncommon in infant, children

Etiology

# thrombi

    ; often from femoral & pelvic vein

    ; *children, adolescent에서는 abd. & head vein에서도 올 수 있다.

# ★Asso. Condition

    ; scoliosis surgery

    ; spinal cord injury

    ; severe burn

  ; prolonged inactivity

    ; complication of IV infusion

  ; ★Sick Neonate

        - *medical device (e.g. IV line), AV fistula, implanted device

                   / *▲often

           - CHD & infant of DM mother

    - infarcted placenta

           - thrombus in umbilical vein

       - asphyxia

           - sub sequent resp. distress

    ; adolescence

    ① recent abortion

    ② drug abuse

    ③ hypercoagulation disorders

    ④ oral contraceptives

    ⑤ indwelling central venous catheter

    ⑥ urokinase - rarely

  6) sickle cell anemia

  7) fat embolism from fractured bone

  8) multiple pulmonary infrct from small emboli

    ① severe dehydration in diarrheal ds.

    ② CHD

    ③ bacterial endocarditis

    ④ ventriculo-atrial shunt in hydrocephalus

Clinical Manifestation

    ; *dyspnea

           - often transient

    ; chest pain & collapse

    ; large infarction

           - impaired resonance, pleural friction rub

           - decreased breath sound, moist rale

           - classic triad

                   / *pleuritic chest pain, hemoptysis, infiltrate

    ; large emboli

           - acute Rt-side heart failure

    ; abscess formation due to secondary infection

Diagnosis

    ; *normal chest X-ray

    ; pulmonary perfusion study , ventilation scintiphotography, pul. angiogram

Treatment

  ; medical emergency  

  1) initial objective  ① cardiovascular support

                     ② prevention of circulatory collapse

                        & pulmonary insuffiency by cardiotonic drug

                        & mechanical ventilation

  2) thrombolytic Tx (heparinization)

   → fail시 emboli source가 분명해지면 op

# Heparinization

  ; after stabilization, prevent further embolization

    ; IV heparin loading 50-70U/kg

           --> *maintenance 25U/kg/hr for 7-10day