Part 24. Urologic disorders in infants and childhood

넬슨 정리

Part XXIV. Urologic Disorders In Infants And Children

Chapter 491. Congenital Anomalies & Dysgenesis Of The Kidney

; *생후 1일 내 voiding이 없으면 underlying anatomic abnormality 검사

; 출생시 urine

1) pH 5∼7

2) osmolality : 60∼600mOsm/Kg (mmol/Kg) H2O

3) many epithelial cell

4) RBC : 간혹 존재

5) *WBC : 無

6) culture : sterile

7) glucose & protein : trace amount

Renal agenesis(aplasia)

Bilateral Agenesis

; incidence : 1/,3000

; 결과 : stillborn fetus

# ※96객 Potter phenotype

; oligohydramnios에 의해 발생하는 특징적인 임상증상

- wide separated eyes

- epicanthic folds

- receding chin

- limb anomalies

- hypoplastic lungs

; causes

- renal agenesis, cystic renal dysplasia, obstructive uropathy, infantile polycystic kidney, renal hypoplasia, medullary dysplasia

# *DDx of agnesis & aplasia

; aplasia

- extreme form of dysplasia

- *presence of nonfunctional tissue capping a normal or abnormal ureter

2) hereditary renal dysplasia

: autosomal dominant

50∼90%의 penetrance

다양한 expression

anorectal, cariovascular, skeletal abnormality

4. 진단

1) maternal ultrasound (2nd trimester)

: oligohyramnios

bladder의 nonvisualization

absent kidney

5. 1개월 내 death의 원인

: uremia or pulmonary insufficiency

Unilateral Renal Agenesis

; 다른 검사도증 우연히 발견되는 경우가 대부분

; *single umbilical artery 있을 때 반드시 의심

; absence of ureter, ipsilateral bladder, hemitrigone

contralateral kidney : 출생후 compensatory hypertrophy

associated anomaly

1) genitourinary aystem : 40%

2) skeletal system : 30%

3) cardiovascular system & gastrointestinal system : 각각 15%

4) CNS & respiratory system : 각각 10%

incidence

1) 1/1,000 live births

; *male > female

; *left > right

♥ Mayer-Rokitansky Syndrome

; unilateral renal agenesis & vaginal atresia or agenesis

vas defens의 agenesis, seminal vesicle cyst

Anomalies In Shape And Position

ascent & rotation의 불완전 Fig 491-2

→ renal ectopia or nonrotation 발생

# Horseshoe Kidney

; 신장의 lower pole이 중심선에서 융합

; incidence - 1/500 birth

; *Turner syndrome의 7%

; *Wilm`s tumor의 발생빈도가 2∼8배 더 높다

Hypoplasia

definition : nephron수가 감소한 small kidney

; *not inherited

unilateral involvement

1) segmental hypoplasia or Ask-upmark kidney : 일부 involve

2) entire involvement

3) 10세 미만에서 hypertension유발하는 more common cause

bilateral involvement

1) chronic renal failure의 증상 가짐

2) 10세 미만에서 ESRF의 주원인

3) polyuria & polydypsia : common

4) oligomeganephronia(rare form) : nephron수의 현저한 감소 & 현저한 hypertrophy

Dysplasia

1. definition

: fetal kidney의 structural diffentiation의 상당한 변화로 cyst, abnormal duct,

undefferentiated mesenchyme, or cartilage같은 nonrenal element가 존재

2. 원인

1) urinary tract의 intrauterine obstruction

: prune-belly syndrome, ureterocele, urethral valve, ureteropelvic junction

⇒ 보통 bilateral & ESRF 발생 가능

2) multicystic dysplasia

① unilateral or bilateral

② lower tract의 developmental anomaly or contralateral normal kidney와 관련

③ unilateral form

: nonfunctioning flank mass

⇒ renal tissue가 거의 없거나 전혀 없는 cystic mass

hypertension이 생기면 제거

④ bilateral form

: chronic renal failure와 관련 ⇒ 심한 경우 Potter syndrome를 보임

CHAPTER 492. Urinary Tract Infections

Prevalence And Etiology

# newborn의 symptomatic UTI

; 1.4/1,000

# ★Risk Factors

; variable markedly with age & sex

; *uncircumcised male infant(<1yr)

; *female(>1yr)

; 7-11yr age

- *▲common age group (2.5%)

; sexually active female : cystitis 위험 증가

; sexually active adolescent male & female : urethritis 발생 가능

# Pathogens

; mostly colonic bacteria

; female

- ★E. coli(75∼90%) > Klebsiellla > Proteus

; male over 1yr

- *Proteus=E. coli in some report

- *G (+) cocci in another report

; *Staphylococcus saprophyticus

- both sex

Pathogenesis And Pathology

; *in newborn

- *bloodstream or urethra통해 침범

- *이후 : ascending infection

UTI의 susceptibility에 관여하는 factor

1) host factor : urethra cervical Ab (Ig A)

2) bacterial adherence에 관여하는 factor : P blood grup phenotype

3) immunosuppression, diabetes, urinary tract obstruction,

chronic granulomatous disease 시 증가

infection의 severity에 관여하는 factor

1) bacteria의 virulence

2) anatomic factors

: ·vesicoureteral reflux

+→ vesical overdistention

-+→ bladder wall의 blood flow 감소

+→ infection에 대한 bladder의 natural resistance 감소

·obstruction

·urinary stasis : bacterial multiplication

·prisence of calculi

Acute Bacterial Cystitis

1. 특징

: mucosal congestion, edema와 petechiae, hemorrhage

→ detrusor muscle hyperactivity

→ bladder의 functional capacity 감소 → vesicoureteral reflux

2. cystitis cystica : chronic or recurrent infection 있는 경우 발생

Acute Pyelonephritis

# ★P1 blood group

; VUR이 없어도 ascending recurrent pyelonephritis

; *E. coli - specific binding to P1 antigen of epithelial cell

medulla와 pelvis에 acute inflammatory cell infiltration → enlarged kidney

→ microabscess 형성 → renal scar 형성 : chronic pyelonephritis

obstruction있으면 더 severe

즉각적인 치료 : 완전 healing

Chronic Pyelonephritis

; difficult to DDx

- medullary cystic disease, ischemia, irradiation, analgesic abuse

* characteristic finding ; cortical scar with underlying calyceal deformity

; LM

- pathy with glomerular fibrosis

- interstitial chronic inflammation

- tubule의 fibrosis & atrophy

; *cortex 보다 medulla가 susceptible

- high osmolality

--> leukocyte의 phagocytosis 감소

# reflux nephropathy

; renal scars in VUR children

; no history of UTI

; *flat papillae에서 발생

; *autoimmune response to Tamm-Horsfall protein

--> play a role in development & progression of pyelonephritic scars

; *90% of chronic pyelonephritis

- *VUR

★reflux nephropathy or chronic pyelonephritis

; ▲ cause of arterial hypertension in children

Urea-Spliting Organism : Proteus

; *stone formation

- urea로부터 ammonia생성 → alkaline urine 형성 → calcium phosphate, triple calcium, magnesium, ammonium phosphate의 precipitation가능 → calculi 형성 → foreign body로 작용 → infection

ureteral obstruction시의 infection

: septicemia, pyelonephrois, renal & perirenal abscess formation

Xanthogranulomatous Pyelonephritis

1. 정의 : giant cell과 foamy histiocyte가 있는 granulomatous inflammation

2. renal mass, acute or chronic infection 유발

3. predisposing factor

: renal calculi, obstruction, Proteus or E. coli의 infection

4. treatment : nephrectomy

Clinical Manifestation

; asymptomatic bacteriuria

- mostly

# urinary tract infection의 symptom

1) infancy

; *fever, weight loss, failure to thrive, N/V, diarrhea, jaundice

2) children

; urinary frequency, pain durine micturition, urinary incontinence asso. /c urgency, bedwetting in a previously dry child, abdominal pain, foul smelling urine

# chronic or frequently recurrent cystitis

; daytime incontinence, bladder instability의 증상

# *hematuria

; *E. coli에 의한 hemorrhagic cystitis

# acute pyelonephritis

; *fever, chill, flank or abdominal pain, tenderness, enlarged kidney

# chronic pyelonephritis

; asymptomatic이 多

; renal scar : arterial hypertension

# reflux nephropathy

; 15% of ESRF in childhood

# sepsis

; common in infecion & urinary tract obstruction

# *hyperammonemia wih CNS symptom

; *Proteus UTI and urinary stasis & obstruction

Laboratory Data

# culture

1) toilet trained children ; midstream urine culture

① 의의 : single organism이 10⁵ colonies/mL

→ UTI에 90% 이상의 specificity가짐

② ※87,94 low colony나오는 경우

; overhydration, recent bladder emptying, antibiotic therapy

2) adhesive, sealed, sterile collection bag

3) catheterized specimen

① infant : 5F polyethylene feeding tube

② older children : 8F polyethylene feeding tube

4) suprapubic puncture

① full bladder 만들고 실시

② 25 or 22 gauge needle 사용

③ pubis의 midline에서 1 finger-breadth 상방, 10∼20° 각도

④ ※87,94 false negative culture

; unrecongnized antibiotic therapy, overhydration에 의한 dilution, antiseptic solution의 contamination

# urinalysis

1) pyuria : 농뇨 없이도 infection recur가능

2) microscopic hematuria : acute cystitis

3) urinary casts : renal involvement

4) alkaline pH : Proteus infection

# upper & lower UTI의 감별

; acute renal infection

- leukocytosis, neutorphilia, ESR & CRP 증가

- Ab-coated bacteria의 detection

- single dose antibiotic therapy에 대한 반응

- 다른 immunologic or biochemical test

/ urine을 농축하지 못하는 경우

/ 30%에서 일시적인 serum creatinine 증가

/ sepsis 때문에 febrile infection시 blood culture 필요

; ♥표17-12(p722)

Imaging Studies

# Renal Ultrasonography

; *Indication

① acute febrile infection시 hydronephrosis와 renal, perirenal abscess와 감별

② antibiotic therapy에 대한 반응이 즉각적이 않을 때

③ severely ill & toxic child

④ serum creatinine level 증가

# Technitium labeled 2,3dimercaptosuccinic acid(DMSA) or glucoheptanate renal scanning

; *acute pyelonephritis의 진단이 불확실한 경우 유용

; parenchymal filling defect

- pyelonephritis 진단 가능

- chronic과 acute 감별은 불가능

# CT

; acute pyelonephritis의 *definitive diagnostic method

# voiding cystourethrography

; *acute infection치료후 약 3주째 reflux 평가위해 실시

; ♥Indications

- all males & females under 5yr with initial infection

- older femaleat the time of second infection

; *female - radioisotopic VCUG

; male - radiographic VCUG

# ★VUR이 존재한다면

--> IVP with nephrotomography or radioisotope renal scanning 실시

; IVP

- kidney size 평가

- 가능한 calyceal blunting, ureteral dilatation, renal scarring의 detection

; DMSA or glucoheptanate radioisotopic renal scan

- *renal scar의 detection

- 장점

/ urography보다 더 sensitive

/ IV contrast media의 adverse effect 無

/ abdominal gas로 인한 urography의 해석 방해가 있는 경우

/ infant와 young child에게 특히 유용

Differential Diagnosis

1. external genitalia의 inflammation

: yeast, pinworm에 의한 vulvitis, vaginitis

다른 agent와 cystits의 감별

2. viral, chemical cystitis : history, urine culture

3. hypoplastic, dysplastic kidney, vascular accident에 의한 small kidney와

chronic pyelonephritis의 감별 : X-ray상 유사

+→ 보통 vesicoureteral reflux 존재

*4. acute hemorrhagic cystitis

; E. coli

; *adenovirus type 11 & 21

- male에 多

- self-limited,

- 대개 4일 정도 지속

5. eosinophilic cystitis

1) symptom

: hematuria, ureteral dilatation

eosinophilic mass에 의한 bladder의 filling defect

Treatment

# acute cystitis시 pyelonephritis 안가게 prompt treatment 필요

; *symptom이 severe → culteure와 동시에 치료 실시

1) trimethoprim-sulfamethoxazole

- 7∼10일

- effective aganist E. coli

2) nitrofurantoin

- 5∼7mg/Kg/day, 3∼4 divided dose

- effective against Klebsiella-Enterbacter organism

3) amoxicillin : 50mg/Kg/day

; symptom이 mild하거나 진단이 의심될 때 culture 후 치료 실시

; culture 결과가 불확실한 경우 재실시

→ 치료시작전에 catheterization or suprapubic aspiration이용해 2nd culture 실시

# pyelonephritis로 생각되는 acute febrile infection 시

1) cefataxime(100mg/Kg/day)

2) ampicillin(100mg/Kg/day) with aminoglycosides

; gentamicin(3mg/Kg/day, 3 divided dose)

; ototoxicity & nephrotoxicity of aminoglycosieds

--> serum creatinine measurements(initial and daily)

; *Pseudomonas에 특히 effective

; sodium bicarbonate에 의한 *urine alkalinization으로 효과 증가

3) bactrim

; Pseudomonas 제외한 G(-)에 effective

4) *ciprofloxacin

; *18 세이상에서 resistant microorganism의 alernative agent

※87 UTI 치료 후 planning

; 1주일후 urine culture 필요

- urine sterility 확인

; asymptomatic이라도 1∼2년간 3개월 간격으로 follow-up culture 실시

- UTI는 predisposing factor없이도 recur 多

# anti-biotic prophylaxis

; agent

- bactrim, nitrofurantoin

- *1/3 dose once a day

; ※89,93,95 Indication

① UTI의 frequent recurrence

② persistent vesicoureteral reflux

③ persistent cystitis - incontinence, frequency, urgency

④ neurogenic bladder

⑤ urinary tract stasis & obstruction

⑥ calculi

⑦ reflux

# excellent long-term prognosis

★ main consequence of chronic renal damage by pyelonephritis

; arterial hypertension , renal insufficiency

8. infrequent voiding, constipation에 대한 education

9. antibiotic therapy & surgical or percutaneous drainage

: renal & perirenal abscess or obstructed urinary tract의 infection가진 경우

CHAPTER 493. Vesicoureteral Reflux

1. 원인 : ureterovesical junction의 valvular mechanism의 incompetence

2. why reflux harmful to kidney

1) renal pelvis를 voiding 시 higher vesical pressure에 노출

cf) normal renal pelvis pressure : 10 mmHg

2) bacteria가 bladder로부터 kidney로 passage가 용이

① intrarenal reflux도 scar에 관여

: intrarenal collecting system에서

collecting tubule로의 urine reflux & bladder dilatation

→ renal scar 생성에 impotant role

② megaureter-megacystic syndrome (fig 493-1)

→ dilated ureter로 massive reflux

→ incomplete bladder emptying

→ bladder로 rapid return

→ progressive bladder emptying

3.※89 reflux nephropathy

1) children과 young adult의 ESRF의 *15∼20%

2) children의 hypertension의 주요원인

Classification

Table 493-1

# Primary VUR

*1) 원인 : UVJ의 congenital anomaly

① short intramural ureteral tunnel

--> valvular mechanism의 efficiency 감소

② urteral orifice - lateral & cephalad direction

③ trigone - underdeveloped

2) Fig 493-2

# Cystic & Bladder Instability

; reflux를 precipitation or perpetuation

# Ureteral Duplication & Ureterocele

1) ureterocele

; *upper collecting system - obstruction

; lower collecting system - reflux or contralateral side reflux

2) ※96 Duplicated system

① *reflux ; more common in lower ureter

② lower ureter

; *enter bladder higher & more laterally

; less competent vlave

③ureter가 divertivulum으로 들어가는 경우

; reflux more common

# Congenital Neurogenic Bladder

1) 원인 : myelomeningocele, sacral agenesis

2) reflux : 출생시 ⅓에서 발생 → ½에서 posterior valve에 발생

→ 결국 ½이상에서 발생

# Intravesical Pressure가 증가한 Reflux

1) bladder outlet obstruction & vesical dysfunction 발생

2) infection 없이도 severe renal damage 가능

#★ VUR Grade

★Fig. 493-5

1) base

① severity

② ureteral dilatation

& calyceal deformity의 정도

2) 의의 : prognosis & therapy

Natural History

1. reflux garde에 증가에 따라 renal scarring, reflux nephropathy 확률 증가

2. intrarenal reflux시 scarring의 risk 증가

3. *grade I & II reflux

1) no ureteral dilatation

2) vesicoureteral region의 anatomy 거의 정상

3) child가 성장하면서 *80%에서 spontaneous하게 소실

4. grade III & IV reflux

1) *spontaneous resolution ; 15%

5. familial tendency 有

∴ 5세 이하의 sibling도 검사

Clinical Manifestation

1. 발견

1) UTI evaluation 시

2) voiding dysfunction, renal insufficiency, hypertension or 다른 UT의 의심되는 pathology시 실시하는 VCUG 시

Differential Diagnosis

1. history & radiograph

: primary or secondary reflux 감별

1) US : renal size

2) isotopic parenchymal renal scan : scar의 rule out

2. *continued voiding dysfunction in spite of antibacterial prophylaxis

; anticholinergics로 치료 or lower urinary tract의 urodynamic study

3. reflux, infection & voiding dysfunction가진 환자

: voiding dysfunction이 infection에 의해 생긴것인지 infection을

predisposing하는 reflux의 원인인지 결정하기가 어렵다.

Evaluation

: reflux의 diagnosis & grading후 실시

1. IVP & tomography : renal size, scar 여부 확인

2. blood pressure

3. baseline creatinine clearance

4. cystoscopy

Treatment

Primary reflux & reflex ass. /c complete duplication

1. ㉿Grade I & II reflux

1) spontaneous resolution이 多

; infection 방지 & antibiotics prophylaxis(bactrim, nitrofurantoin

; long-term treatment

2) 치료시작시 *매달 urine culture 실시

; *prophylaxis가 effective하면 3개월 간격으로 culture

3) asymptomatic bacteriuria & reflux

; harmful

; *symptom이 없어도 urine culture 중요

4) radionulide VCUG ; *1년 마다 실시

5) renal US ; *1년마다 실시 → renal growth 평가

6) *radionuclide study상 spontaneous cessation

; *another study in 3∼6mo before discontinued AB therapy

--> confirm

7) ♥Indication of surgery

; failure of antibiotic prophylaxis

8) *DMSA renal scan ; 치료의 마지막에 실시

→ residual scar가진 경우 blood pressure의 long term follow up 필요

2. ※89 Grade III reflux

1) *new scar formation 의심시 periodic parenchymal scan

2) *more than 50% of children ; surgery 실시

3. Grade IV & V(ass. /c significant ureteral dilatation & upper urinary tract change)

1) *early surgery after a brief prophylaxis & reconfirmation

; esp. infant와 young child

∵ 5세이하에서 scar 빈도 증가

; *warrant

- reflux에 의한 prenatal hydronephrosis가진 newborn

--> 1세경 spontaneous improvement

Secondary reflux

1. *duplication 있는 경우 ; A와 동일

2. *periureteral divertivulum 있는 경우 ; early surgery

3. severe cystitis 있는 경우 ; primary cause의 remove

4. iatrogenic ; surgery

Results Of Surgery

1. *Cohen operation ; 97%의 성공률

2. antireflux surgery의 complication

1) reflux의 persistence

2) distal ureter의 obstruction

3. surgery 후 반대측 reflux는 대개 자연 소실

다른 치료 방법

1. *endoscopic injection of poly tetrafluoroethylene(polytef) under ureteral orfice

; 짧은 기간 effective

2. *subureteric injection ; collagen, silicone particle

3. success rate ; 65-70%

※89 Cx of VUR

; UTI

; renal scar & atrophy

; hypertension(20%)

; CRF(reflux nephropathy)

Chapter 494 Obstruction of the urinary tract

; 원인 - congenital(children에서는 M/C), trauma, neoplasma, calculi, inflamatory process, surgical procedure

Etiology

Table 494-1

# High grade ureteral obstruction of early onset in fetal life

; result in renal dysplasia

- from multicystic kidney to cortical dysplasia

# Chronic ureteral obstraction

; result in hypertrophy and later dilatation of ureter and upper collecting system

Clinical manifestation

① urethral obstruction in fetus

; result in patent urachus, urethral dilatation, ureteral dilatation, VUR, urinary extravasation (urinoma, urinary ascites)

② Bilateral ureteral obstruction or urethral obstruction

; cause *oligohydroamnion and pulmonary hypoplasia

; severe obstructive uropathy일 경우 신생아에서 즉각적인 반응은 renal damage정도보다 pulmonary insufficiency에 더 관계된다.

③ 그밖에 obstruction이 relief된 후에도 polyuria, dilute urine, chronic acidosis(serum creatinine level은 정상), hypertrophy & dilatation in the bladder and collecting system postobstructive diuresis가 생길 수 있다.

Diagnosis

; often silent

; in newborn

- palpable abdominal mass due to hydronephrotic kidney

- *patent urachus

- *ascites(due to intraperitoneal urinary extravasation)

; prenatally diagnosed by US

- oligohydroamnios & pulmonary hypoplasia 고려

; *Infection and sepsis

- maybe first indication of obstructive lesion of urinary tract

- renal US for all children with acute stage of febrile UTI

; obstructive renal insufficiency

- *failure to thrive, vomitting diarrhea, other nonspecific symptom

; *overflow urinary incontinence, or poor urinary stream

- *in older children

- infravesical obstruction

; flank or abdominal pain with N/V in acute obstruction

; silent or vague abdominal or typical flack pain with increased fluid intake in chronic obstruction

--> *abdominal pain이 있는 all children에게서 intial study로 abdominl US

Imaging Studies

1) US

① screening method ② evaluate renal size, parenchymal thickness, ③ determine ureteral dilatation, ④ bladder evaluation 그러나 acute 또는 intermittent obst. 때는 minimal dilatation되므로 misleading할 수 있다.

2) DTPA

; gross estimate of different renal fuction dilatation

; renal pelvis에 isotope있을 때

→ Furosemide adm unobst 있을 때 : prompty excretes isotope, obst 있을 : no or slowly excrete

isotopic renography with iodine labeled Hippuran : more accurate but exposed kidney에 radiation↑

3) IVP

; preliminary radiography of the abdomen → calculi, spinal abnormalities,

abdominal & intestinal gas pattern을 본다.

; postvoiding film → residual bladder urine을 본다.

; obstruction side에 radiopaque medium이 남아있다.

; *intermitten obstruction

- *pain등이 유발되는 acute phase에 IVP하는게 가장 유용하다.

Pressure Flow Studies (Whitaker test)

; percutaneous로 renal pelvis에 needle 넣은 후 radiopaque dye perfusion 후 renal pelvis와 bladder사이 압력차 측정

- *20cm of water 이상 pr. grad시 obst.

Voiding cystourethrography

모든 ureteral dilatation시

① VUR 보기위해 시행

② urethral obst, esp. post urethral valve

③ infravesical obstruction palpable bladder

ureth, catheterization이 잘 되지 않으면 ureth stricture의심 obstructive ureth, lesion other than valve.

Specific Type of Urinary tract Obstruction

Hydrocalycosis

① infundibular obst.인한 localized calyceal dilatation

② 원인 ┏developmental

┗2°to infl. process : exp. *Tbc

③ cong. obst. due to stenosis or extrinsic vas. compr.시 증상 : pain(obst의 surgical correction시 사라짐)

④ Dx. : IVP

Obstruction of the UPJ

; ※93 ▲ obstructive lesion in children

- *▲often by cong, stenosis of UPJ

- others causes

/ ureteral kinks, fibrous band, aberrant vv.

; ♣UPJ obstruction의 가장 흔한 증상들

- fetal hydronephrosis in maternal ultrasonography

- palpable renal mass in newborn

- abd, flank or back pain

- febrile UTI

- hematuria after minimal trauma

; *bilateral (20%)

# Unilateral hydronephrosis in the fetus (normal contralat. kidney, normal AF)

; not indication for prenatal finding

; *US - 출생 3일이후 실시(oliguria가 dilatation을 masking할 수 있기때문)

- ★정상이라도 3 or 6 mo때 repeat

; mild to moderate hydronephrosis

- observation & F/U

; marked dilatation, renal parenchymal thin

- isotope renogram

- normal renal fuction이면 F/U

/ no improvement - *diuresis renogram을 6-12mo

; ♥surgical repair Ix

- involved kidney function decreased

- bilateral involvement or solitary kidney

- diminished overall renal function

- palpable mass

⑥ Sx가진 old children

→ IVP, 만약 IVP에서 잘 안보인다면 US.

⑦ D/Dx

i) megacalycosis

pelvis, ureteric dilatation없이 congenital non-obstructive calyceal dilatation

ii) VUR with marked dilated & kinking of the ureter

iii) midureteral, distal ureteral obst.

# newborn /c renal mass D/Dx UPJ obst

i) multicystic renal dysplasia

ii) solid renal tumor

iii) renal vein thrombosis

⑧ Tx. surgical pyeloplasty

⑨ Follow up 시 diuresis renography

Midureteral Obstruction

① Cause

ⅰ) cong. ureteral stenosis or ureteral valve in midureter

ⅱ) partially obstructed retrocaval ureter

ⅲ) circumcaval ureter

; Rt. anomalous development of vena cava with persistent ventral infrarenal subcardinal veins

; IVP 上 Rt.ureter가 3rd lumbar vert. level에서 medially deviated.

② 이외의원인 ┏ retroperitoneal tumor

┠ fibrosis by surgery. infl. process (CGP)

┗ radiation

Ureteral ectopia

① mesonephric duct migration path 中 어느곳에나 발생가능

② drain single collecting system

; commonly belongs to upper moiety of duplicated collecting system

③ ureteral orifice of upper collecting syst.

; always caudal to ureteral orifice of lower collecting system

④ male

i) usually *single ectopic ureter

ii) bladder neck, urethra above external sphinncter seminal vesicle, vas deferens등으로 유입

iii) high grade obstruction, UTI or epididymitis Sx.

iv) 반대측이 normal시 nephroureterectomy

v) bilateral single ectopic ureter unilateral case with functioning involved kid. reimplantation

⑤ female

i) *ass. /c duplication

ii) ureter of upper collecting system

--> bladder neck, urethra at or above the level of sphincter

; upper pole nephroureterectomy

iii) ureter --> vestibule, vagina, uterus

; urinary incontinence, vaginal discharge

iv) Dx

; IVP, US, endoscopy with high degree of suspicion

v) bilateral simple ectopic ureter

; *bladder hypoplasia 동반

Ureterocele

; congenital cystic dilatation of the distal ureter → bladder내로 protrusion되어 pin point

; 여 〉남

; simple ureterocele

ⅰ) non-duplicated collecting system orifice는 제위치(bladder내에 有)

ⅱ) IVP 上

; varying degree of ureteral & calyceal dilatation, round filling defect in bladder delayed film에 ureter가 cleanred visible하거나 & filling of contrast media일 수도 있다.

ⅲ) Tx.

transurethral incision of uretercele (때로 VUR 초래하여 ureteral reimplant 후에 可)

open excision of ureterocele & reimplartation

upper tract dilatation 없는 small ureterocele시 No Tx.→ pr. flow study & diuresis

; ureteral duplication 동반

- 주로 *upper renal moiety에서 drain하는 ureter가 ectopic ureterocele 多

- upper renal moiety : poor fuction, cong.obst.으로 dysplastic

- lower renal moiety : frequently reflux

- ectopic ureterocle may extend submucosally into post. urethra

/ 대부분 unilateral

; UTI

; bladder neck obst.

; urinary retention, reflux 등의 양상을 보임

; IVP

ⓐ large filling defect in bladder

ⓑ characteristic finding of duplication

┏ upper collectin syst. - poor or absent function.

┗ lower collecting syst. - caudal displacement

→ "drooping lily app"

; Tx

ⓐ excision of upper collecting system : partial nephrectomy & ureterectomy

ⓑ small ectopic ureterocele with ipsilateral duplicated ureter에 low or no reflux시 : No Tx.

ⓓ obstructing, ureterocele을 가진 acutely ill, septic infant시 involved collecting system의 drainage by transureterally or by percut. nephrostomy가 필요함

Megaureter

① dilated ureter

② careful Hx. PE. voiding cystourography 등이 2°megaureter와의 DDx위해 중요

③ 보통 distal ureter가 more dilated되어 있고 bladder와의 junction에서 갑자기

tappering된다.

④ obst. megaureter와 nonobst. megaureter DDx위해 dieuresis renogram press

flow study 등 해서 obst이면 op해준다.

Table 494-2

※91슬 Prune-Belly Syndrome

(=abdominal muscle deffciency syndrome =Eagle -Barrett syndrome)

; 1/40,000

; *97% male

㉿ Characteristics

- deficient abdominal muscle

- undescended testes

- urinary tr. abnormality result from urethral obstruction in fetal life

/ ureter & upper part의 mass dilatation, large bladder with patent urachus, VUR, urachal diverticulum, dilated prostatic urethra, hypoplastic prostate, dysplastic kidney, megalourethra

; *oligohydramnios, pul. hypoplasia - frequent Cx

; bowel malrotation c universal mesentery

; cardiac anomaly (10%)

; musculoskeletal anomaly (more than 50%)

; 1/4 - at birth urethral obst.(＋)

; 치료

- obstruction이 없으면 UTI예방이 치료의 목표다.

- obst.. (＋) / temporary drainage & later reconstructive surgery

- orchiopexy : 1세이내

; 예후

- ★pulmonary & renal dysplasia정도에 달렸음.

- 1/3은 사산되거나 출생후 수개월 내에 pulmonary Cx으로 죽음

- 장기 생존자의 경우 1/2가 CRF로 가며 이 경우 KTP후의 경과는 양호하다.

Bladder Neck Obstruction

① 원인

2°to ectopic ureterocele.

bladder calculi, prostatic tumor(rhabdomyosarcoma)

post. ureth. valve ; functional disturbance는 rare neurogenic bladder dysfuction

② Sx. : difficulty voiding, urinary retention, UTI bladder distention with overflow incontinence

③ 1°bladder neck obst is exceptional in males

※94 Posterior Urethral Valves

; urethral valve 中 *▲common type

; sail-shaped membrances with eccentric opening

- *verumontanum에서 시작하여 distally extend

--> attach to anterolateral wall of urethra

; dilated prostatic urethra, detrusor m. & bladder neck hypertrophy

--> VUR

; oligohydroamnios, pul. hypoplasia

# prenatally diagnosed case

; severe obstruction

--> prenatal bladder decompression by percutaneous vesicoamniotic shunt or open fetal surgery

; *worse prognosis than detected after birth

# severe case

; *failure to thrive due to uremia

; *sepsis due to UTI

# lesser severe case

; *difficulty in maintaining urinary continence

Diagnosis

; VCUG

- prostatic urethral dilatation with transverse linear filling defect

- VUR : 2/3

Treatment

# serum Cr - normal or return to normal

; primary ablation of valve through transurethral approach

; by temporary vesicostomy if insufficient urethral caliber

# serum Cr - high or ↑ despite bladder drainage

; suspect 2ndary ureteral obstruction, irreversible renal damage, renal dysplasia

; upper tract drainage

- cutaneous pyelostomy, high ureterostomy

# no improvement of renal function

; reconstructive surgery at early age

# septic & uremic Pt

; electrolite imbalance

; Abt 후 ⓐ percut. nephrostomy

ⓑ renal analysis stable한 후 evalutation & Tx

# incontinence가 주소인 경우

; primary valve ablation

# *VUR

; expectant Tx. with AB therapy

--> *relux가 1 yr ↑ & kidney function ↓

--> surgical correction

# *Tx.후에도 urinary incontinence 50%

ⅰ) surgical damage of sphincter

ⅱ) dilatation of prostatic urethra

ⅲ) poor bladder compliance

ⅳ) polyuria from renal damage

--> *나이들면 (exp. after puberty)호전

; *Px in newborn

- depend on pul.hypoplasia & renal function recovery potential

- neonate이후에 1/3에서 어느 정도의 Renal insuff.있어 renal transplantation 요함

Urethral Stricture

① male

ⅰ) congenital : rare

ⅱ) urethral trauma

; iatrogenic (cath, endoscopic, ureth, reconstr.)

; accidental (straddling injury, pelvic fracture)

ⅲ) Sx.

; ⓐ bladder instability ⓑ hematuria ⓒ dysuria

ⅳ) Dx

; voiding film of IVP RGCU, endoscope

ⅴ) Tx

; short - endoscopic dilatation & internal urethrostomy

; long - urethroplasty

② female

; true is exceptional

; urethral ring → urethral obst. & UTI 초래

Anterior Urethral Valve & Urethral Diverticula In Male

① 두 개 동반 可

② Dx : VCUG

③ diverticula는 dilatation of Cowper gland & duct로 생각

Male Urethral Meatal Stenosis

; 4세 이하 - 8F ↓, 10세이상 - 10F ↓

→ true meatal stenosis

; neonatal circumcision 후 glans의 ammoniacal dermatitis 인해 발생

; hypospadia 시도 可

; Tx. : meatoplasty

Chapter. 495. Anomalies of the Bladder

Bladder Extrophy

; 빈도

- 1/40,000 birth.

- boys 〉girls

Clinical Manifestations

① mesoderm이 cloacal membrane으로 caphaled extension 안되어 出

② classic 경우

bladder protrusion from the abd. wall

exposure of bladder mucosa

downward displacement of umbilicus

wide separated public rami

separated recti m.

ant. displaced anus with rectal prolapse

: complete epispadia with wide & shallow scrotum undescended testes inguinal hernia

female : epispadia, clitoral duplication wide separation of labia

broad based gait : wide separated public rami때문

③ untreated 경우

ⅰ) total urinary incontinence

ⅱ) bladder Cancer ↑, esp. adenoCa.

Treatment

; 출생 직후 bladder를 silastic shield나 다른 적당한 plastic dressing을 해서 desication of bladder를 막는다.

; *48hr이내 closure of the exstrophied bladder

- before permanant changes in the bladder walls

- flexibility of pelvic joint 있을때 시행

; initial op. 목적

ⅰ) precise closure of the bladder & prostatic urethra in male

ⅱ) elongation of the urethral plate & penis

ⅲ) closure of the abd. wall

; 대부분 후에 VUR동반

; epispadia operation

- 1-2yr

; Tx of incontinence by bladder neck reconstruction

- *after rectal control

Prognosis

① 치료후에 upper urinary tract에서 15%이하의 deterioration. 70% 이상의 continence를 획득한다.

② 이밖에 artificial sphincter를 만들거나 cytoplasty, abdominal stoma, ureterosigmy 등을 필요에 따라 해주기도 하나 이런 op는 ch. pyelonephritis, urinary tract damage 전해질 불균형, 장기간 경과 후 carcinoma 이행 등의 위험률이 있다.

Other Exstrophy Anomalies

① cloacal exstrophy

severe abn. of colon, rectrum, short bowel

② epispadia

distal epispadia : reconstruction of urethra & penis

Bladder Diverticula

; *대개 UVJ에 호발 → VUR

① 원인

┏ congenital

┗ commonly asoc. distal ureth. obst. neurogenic bladder dysf.

② Tx.

; small : none

; large → ineff. voiding, residual urine stasis. UTI시 excision

[urachal anomaly] : male 〉fe

Chaper 496. Neurogenic Bladder

Etiology

; congenital

; meningomyelocele, lipomeningocele, sacral agenesis, other spinal abnormalities

; acquired & traumatic

- less frequent

- cerebral palsy, CNS tumor & their treatment pelvic op. (imperforated anus, sacrococygeal, teratoma)

; ★two ▲important consequences

- *upper tract deterecration, urinary incontinence

Renal Damage

; dys-synergia

--> functional obstruction of bladder outlet

--> high intravesical pressures

--> bladder muscles hypertrophy, trabeculation

--> VUR

--> deterioration of upper tracts

; VUR

- 30% of neonate with myelomeningoceles, later 20%

- *more severe than primary reflux

; infection

- compounds problems

; neurologic anomaly 가진 Pt의 모두가 neurogenic bladder 있는 것은 아니므로 urodynamic study(cytometrography sphincter electromyography)하여 R/O시켜야 함.

Urinary incontinence

; 원인

ⓐ total or partial denervation of sph.

ⓑ bladder hyperreflexia or poor bladder compliance

3) Tx

① Neurogenic bladder 가진 환자 치료의 목적

protection of upper urinary tract

eventually providing continence

② newborn with low intravesical pr. & No VUR

expectant therapy

US & radioisotopic cystogram f-up

recurrent UTI시 prolonged antibiotic therapy

urodynamic study at 6 mo.에 repeat

③ VUR(+), elevated intravesical pr. (grade Ⅰ&Ⅱ)

┏ Antibiotics prophylaxis intermittent catheterization

┣ anticholinergic drug(oxybutynin 0.4mg/kg/day Sig 1/2)p.o시 40%에서 호전

┗ severe reflux 있을 경우 corrective surgery

incontinence Tx

ⅰ) sphincter tone 충분하다면 → 4hr 간격으로 intermittent catheterization

ⅱ) 7∼8 yr. old who have adequate manual dexterity → intermittent self catheterization

ⅲ) intermittent catheterization 동안 antibacterial prophylaxis 쓸 수 있다.

⑤ bladder capacity와 compliance는 충분. but urethral resistance가 낮을 때

implanatation of artificial sphincter

⑥ Enterocytoplasty

ⅰ) Tx : neurogenic bladder bladder exstrophy, post urethral valve 등 bladder

capacity를 줄이는 질환

ⅱ) Rt. or Lt. colon 이나 ileum을 끼어 넣음

ⅲ) 이 경우 intermittent cath할 때 colon의 G(-) bacteria 나오므로 Sx 있을 때만

UTI Tx

ⅳ) hypermetabolic acidosis 올 수 있으므로 Tx (enteric mucosal surface 中 urine과 접촉하는 부분에서 ammonium, choloride. H+이온 흡수, K+ loss하므로)

ⅴ) 이런 Cx 막기 위해 ch. renal insufficiency 환자에게는 colon 대신 gastric segment를 사용하기도 함.

ⅵ) Cx으로 perforation, peritonitis, potential for malignancy 등이 있음.

Chaper 497. Voiding dysfunction

Nocturnal Enuresis

① 5세 child의 20%

; 그 이후 해마다 15% 감소하여 adult에서는 1%이하

② 원인

; *delayed maturation of the cortical mechanism

; alteration of the circadian rhythm of ADH secretion

③ primary ; night-time continence period (-)

secondary ; formely dry child에서 emotionally disruptive event 후에 발생

④ male(3배) 〉female

⑤ FHx있다.

⑥ neurologic & spinal abnormality, fluid intack, urinary output

⑦ urinalysis

; Bacteriuria

; specific gravity & osmolarity after overnight fast

⑧ daytime sx. (-). P/E, UA, urine culture normal시 → No further evaluation

needed

⑨ Tx

ⅰ) reassurance. self - limited, eliminate punitive measure

ⅱ) fluid restriction, midnight awakening systems of reward & punishment :

not successful

ⅲ) imipramine drug 끊으면 대개 recur

ⅳ) desamino - D - arginine vasopressin : 50%

ⅴ) Tx. of conditioning with alarm.

Unstable Bladder

① voiding dysfunction not related to neurologic abNl or dys Fx

② Sx : frequency, urgency, blader pain없이 diurnal urinary incontine

③ Study

Hx P/E UA, urine culture, uroflowmeter, abdo U/S, male - VCUG

④ Fx

UT pathology없을 때 oxybutynic & other anticholinergic

Non-Neurogenic Neurogenic Bladder

① non neurogenic destrusor/sphincter dys과 같은 말

② neurologic abnormal 없이 voiding 시 ext sphincter가 relax(-)

③ Hx상 UTI Constipation Hx 있다.

④ P/E상

VUR trabeculated bladder, decreased urine flow rate intermittent pain

⑤ toilet training과 관련

⑥ Tx

ⅰ) treatment of reflux & antibiotics prophylaxis

ⅱ) behavioral modefication & encouragement of relaxation during voiding

ⅲ) Buifeedback

ⅳ) intermittent catheterization & anticholinergic drug

ⅴ) diazerpam

⑦ Px : poor

Infrequent Voiding

① UTI와 동반된 micturition 때 common disorder

② girls에 多, FHx가 있다.

③ 원인은 behavioral

④ Tx : antiotics prophylaxis.

encourage to frequently void

complete emptying by double voiding

Other Cause Of Incontinence In Females

table 497-1

1) Ureteral ectopia

① 대개 female에서 duplicated collection system과 동반

② Nl voiding pattern 보이면서 constant dribbing of urine

③ Dx : IVP, US, CT of kid, Exam under anesthesia for ectopic ureteral orifice in vestibula or vagina

④ Tx : partial nephroureterectomy

Chapter 498. Anomalies of the penis and urethra

Hypospadiasis

① 빈도 : 1/500 newborns.

② mildest한 경우 uretheral meatus가 glans의 vent로 open하는 것에서부터 severe할 수록 penis가 ventral로 curved되면서 penile urethra가 짧아진다. (chordee동반되어서)

③ meatus의 opening : ventral aspect of glans, penoscrotal juction, perineum

④ 10%에서 undescended testes 동반

⑤ 치료의 목적 mild case : cosmatic

severe case : 서서 voiding할 수 있게

future Nl sexual function

avoid psychogenic consequence

⑥ ideal age for repair는 controversial하지만 현재 18mo.이전에 시행 → toilet training(1세전)에 실시 newborn period에 시작 (routine circum 피할 것)

Agenesis and Micropenis

A. agenesis : rare하여 anorectal and rectal anomalies와 동반 많이 함.

B. Micropenis

normal NB penic : 3.5±0.7cm

primary

secondary testicular failure after complete morphogenesis

anencephaly

pituitary agenesis

Kallmann, Noonan, Prader - Willi synd

rudimentary testes

dwarfism

materna hormone administration

Tx. : ① hormone stimulation

② rearing as female with later genital reconstruction

Phimosis & Paraphimosis

phimosis : prepuce가 retraction될 나이(3세)가 지나도 retraction되지 않는 것으로 inflamation의 sequal로 되는 것과 congenital로 되는 것이 있다.

Paraphimosis : phimotic preuce가 coronal sulcus 뒤로 retract되어 reduce될 수 없을 때, 이 때 edema severe pain 호소하며 초기 발견하면 heavy sedation이나 전신마취 시킨 후 lubrication해서 foreskin을 reduce시킨다.

Chapter 499. Disorders and Anomalies of the Scrotal Contents

Undescended Testes

Andescended & Ectopic testes

1) Cryptorchidism

① true undescended testes : normal path of descent 따라 존재

processus vaginalis : patent

ectopec testes : inguinal canal 통해 complete descent 했으나 subcut location에

end up

M/C : lat to the ext. inguinal ring below subcut. fascia

# incidence

; 0.7% of children after 1yr and adults

; 3.4% in newborn

; prematurity(2000∼2500g) - 17%

; 900gm↓ - 100%

# 7th mo. of gestation시 testicular descent 出

# *spontaneous testicular descent dose not occur after 1yr

# Complication

ⅰ) infertility in adulthood

ⅱ) tumor development : 20∼44% ↑ risk

ⅲ) associated hernia

ⅳ) torsion of the cryptorchid testis

ⅴ) psychologic effects of an empty scrotum

# bilateral cryptorchidism : 30%

⑤ undescended testis는 원래 조직학적으로는 정상이나 failure of developement and atrophy가 일어나 3rd or 4th decade of life에서는 maligrant testcular가 될 확률이 20∼44% 증가된다. 이 중에서 M/C tumor는 Seminoma(60%)이다. (정상 testes에 서는 30%)

⑥ true undescended testes에서 indirect inguinal hernia가 반드시 나타나며 이밖에

torsion infarction이 동반될 수 있다.

Treatment

# unilataterl cryptorchid testes

; *orchiopexy during early in the 2nd yr. of life

# bilateral

; palpable testis

- unilateral 과 동일하게 치료

; not palpable testes

- *hCG stimulation test

/ testosterone↑시 abd. exploration & orchoipexy

/ negative response일 수도 있다

ⅲ) HCG or LHRH

surgical Tx. replacement 못함

1,000u im.3 times weekly for 3 wk 후 testes하강 (-), orchiopexy

Retractile Testes

exaggerated cremasteric reflex로 inguinal canal내로 retract

puberty 때 scrotum으로 내려옴

No Cx

Abscent testes

non-palpable testes의 200%

┏congenital : quite rare

┗acquired : vascular accident

Torsion Of The Testis Or Appendices

① 6살 이하에서 acute scrotal pain swelling을 호소하는 환아의 40%에서 testicular torsion 이다.

② 원인 → abnl. fixation of the testis to the scrotal envelope

③ Sx → acute scrotal pain, tenderness, swelling absent cremasteric reflex

④ DDx

ⅰ) incarcerated hernia → 이 때는 inguinal area에 swelling 있다.

ⅱ) torsion of one of the 0testicular or epididymal appendicies → 이 때는 pain

swelling 적고 testis위에 blue dot 보이고 local tenderness

ⅲ) epididymitis : 13이후에는 반드시 R/O

18세 이상시 M/C cause of scrotal pain & swelling

이 때 UA가 abnormal

⑤ 진단법 : ultrasonogram, color doppler, ultrasound, isotope scan

⑥ Tx

prompt surgical exploration 6h전에 retorsion시 90%↑에서 survival of gonad contrlateral testes는 scrotum에 fixation

Varicocele

; pampaiform venous dilatation due to valvular incompetence of spermatic vein

; *predominantly Lt side

- bilateral 10%

; rare before 10yr, adult 15%

③ subfertility, decreased size of testis 可

④ large시 painful, fixed varicocele : retreoperitoneal tumor의심

⑤ surgery : pain relieve, normal testis development 목적

⑥ varicocel의 early correction과 fetility에 대한 관계는 아직 밝혀지지 않았음.

Hydrocele

① tunica vaginalis에 fluid accumulation transillumination (+)

② small hydrocele은 1yr내 소실

③ communicating hydrocele (vary in amount)시는 indirect inguinal hernia처럼 Tx.

Epididymitis

acute scrotal pain & swelling

cong. anomaly of wolffian duct (ectopic ureter entering the vas.)

UA : pyurea gonococcus Chlamydia

Tx. : bed rest antibiotics

Chapter 500. Trauma to the Genitourinary Tract

① Trauma로 인한 accidental injury일 경우 skeletal이나 CNS 등 다른 곳은 trauma와 동반될 경우가 많고 만약 kid만 Trauma 받았다면 선행하는 kid anomaly가 있을 확률 이 많다. (horseshoe kid, renal ectopia, hydronephrosis)

② Evaluation of Pt.

ⅰ) bladder cath. : except bleeding from urethra

urethral injury시 retrograde urogram

Tx. : suprapublic cystostomy drainage until resorption of hematoma → urethroplasty

Cx. : erectile impotence urethral stricture urinary incontinence

ⅱ) cystography

ⅲ) IVP

ⅳ) renal angiogram, CT

③ Renal injury 분류

minor - parenchymal 손상, 대부분 이것 op. 필요없다.

major - collecting system, renal pedicle 손상

④ infant & child에서 Kidney에 injury 호발이유

proportionally greater size

less sturdy fatty & fascial envelope

greater flexibility of overlying lower ribs

Chapter 501. Urinary Lithiasis

① 기후, 음식, 사회 경제적 요소와 연관되므로 geographic variation이 있다.

② Sx. 순서

ⅰ) gross or microscopic hematuria

ⅱ) abd. pain

ⅲ) flank or back pain

ⅳ) UTI sx

③ plain abd. 上

ⅰ) faintly seen ⓐ cystine stone ⓑ infectious stone(Struvite)

ⅱ) radiolucent stone

ⅲ) complete functional & radiographic study 1/4에서 VUR(+)

ⅳ) chemical & crystallographic analysis

④ laboratory tests suggested to evaluate urolithiasis

table 501-1

⑤ classification of urolithiasis

table 501-2

Calcium Stones

; calcium oxalate

- *▲common urinary caculi

① 원인

ⅰ) idiopathic urolithiasis

ⅱ) associated with hyperclacemia

ⓐ hyperparathyroidism

ⓑ sarcoidosis

ⓓ hypervitaminosis D

ⓔ idiopathic, isolated form

ⅲ) normocalcemic hypercalciuria

furosemide adm.

uncontrolled distal RTA

TPN, alkalosis

idiopathic

renal tubular calcium leak→ 2ndary hyperparathyroidism.

→ hyperabsorption of calcium

(primary intestinal hyperabsorption of calcium)

② urinary calcium measurement

upper limit of normal : 4mg/kg/24h

urinary Ca/creatinine ration 〉0.25mg/mg

③ calcium oxalate stone

small bowel ds. & malabsorption

→ intestinal hyperoxaluria 1°hyperoxaluria (type 1 or 2)

Cystinuria

dibasic amino acids(cystine, ornithine arginine, lysine,)의 transport의 inborn error시

→ excessive urinary excretion of products

→ formation of calculi

Struvite Stones

urea splitting organism

(① Proteus, occasionally ② Klebsiella ③ E. coli ④ Pseudomonas) → urine alkalinazation, excessive → magnesium ammonium phosphate (struvite), calcium phosphate

aluminum phosphate 침착

Uric Acid Stones

① 대개 hyperuricosuria with or without hyperuricemia

radioluscent stone

persistent acid urine & urate crystalluri시 의심

② 원인

inborn error of purine metabolism

┌ Lesch - Nyhan. syndrome

│ G-6PD-deficiency

│ short-bowel synd. (esp. ileostomy)

│ chronic dehydration & acidosis

└ tumor & myeloprolif. ds.

cf. 2.8. dihydroxyadenine lithiasis

adenine phosphoribosyltransferase def.

Treatment

# 2 prospectives

; underlying metabolic disorder Tx.

- infection, predisposing anatomic factor

; Tx of complication associated with stone itselfs

- infection & obstruction

recurrence 방지

① adequate state of hydration & diuresis

② alteration of pH

ⅰ) cystine, uric acid pH 7.5이상으로 alkalinazation시 solubility ↑

ⅱ) struvite시 acidic urine

③ therapy for underlying 0metalbolic disorder

ⅰ) thiazide : primary renal hypercalciurla

ⅱ) RTA Tx.

ⅲ) allopurinol(uric acid stone. 2.8 - dihydroxvadenosine)

ⅳ) D-penicallamine (cystine, hemicystine)

ⅴ) N-acetyl cystine - cystine stone

④ ⅰ) cellulose phosphate → primary absorptive hypercalciuria

ⅱ) pyridoxine → hyperoxaluria

ⅲ) salts of phosphate, citrate, magnessium → calcium oxalate solubility 증가

# surgical Tx

; *최근에는 거의 하지 않음

; obstruction of collecting system, pain, bleeding, perpetuating infection이 있을때 실시

# endoscopical or percutaneous access to kid

# extracorporeal shock wave lithotripsy

; *renal & ureteral stone 모두 가능

; *succes rate - more than 75%