Part 23. Nephrology
Part 23. Nephrology
Section 1. Structure And Function Of The Kidney
Chapter 462. Anatomy of the glomerulus
1)
*from 6cm, 24gm in fullterm to 12cm
or more, 150gm in adults
2)
cortex ; glomeruli, prox. & distal conv.tubules & collecting ducts
medulla ; straight portions of the
tubules, loops of Henle, vasa recta, terminal collecting ducts
3)
blood supply (COÀÇ 10% Â÷Áö)
; aorta-> renal a. ->
segmental br.-> interlobar a. -> arcuate a. -> interlobular a. ->
aff. arterioles -> glo. capillary network -> eff. arterioles
4)
juxtaglomerular apparatus
;
controls the secretion of renin
;
consists of ¨çspeciallized m. cells in the walls
of aff.a. ¨è lacis cell ¨é
macular densa of distal tubule
5)
Glomerulus
; 1 million nephron in
kidneys
; complete at birth but
functional immature
; ¨ç endothelium
-
fenestrations
¨è
GBM
i)
lamina lara interna ( subendoth.)
ii)
central electron dense lamina densa
iii)
lamina lara externa ( subepith.)
¨é
epithelial cells
-
filtration slit(space between the foot process)
; mesangium
-
¢¾±â´É
¨ç
supporting structure of glomerular capillary
¨è
play a role in regulation of glomerular blood flow, filtration
¨é
play a role in removal of macromolecules(i.e. immune complex) frome glomerulus
by
intracellular phagocytosis
transport
through intercellular channels to JG region
6)
Bowman capsule
462.1. Glomerular filtration
; ultrafiltrate all substance except proteins(like albumin, globulins) exceeding *68,000 MW
;
Forces for ultrafiltration
¨ç
glo.cap.hydrostatic pr. <- systemic art.pr.
¨è
glo.cap.oncotic pr.
¨é
glo.plasma flow rate
¨ê
hydrostatic pr. within Bowman space
¨ë
permeability of glo.cap.wall
;
adult values at *3yr
;
*standardized to surf. area(1.73m2)
of a 70kg adult to comparison
;
¡Úfiltration rate°¡ 70% ÀÌÇÏ·Î
¶³¾îÁ®¾ß serum creatinine »ó½Â
;
*GFRÀÌ
°¨¼ÒÇϸé tubular secretion of
creatinine Áõ°¡ÇϹǷΠfiltration rate°¡
½ÇÁ¦º¸´Ù overestimate µÉ¼ö
ÀÖ´Ù.
;
*serum CrÀÌ 2.0mg/dl(180umol/L) ÀÌ»óÀ̸é GFRÀº little merit
-->
*renal functionÀº secrum creatinineÀ¸·Î F/UÇØ¾ß
ÇÑ´Ù.
¢¾ TRP
= (1- Cpi/Ccr) x 100
= (1- Upi*Scr/Spi*Ucr) x 100
(nl ; 78-93%)
Section 2. Conditions particulary associated with hematuria
¡Ø81ÁÖ
Table XXIII-1 & ǥ17-1(p694)
¡ÚTable
XXIII-2
;
microscopic hematuria
- *> 5 RBC/HPF in the sediment from 10ml of centrifuged freshly
voided urine
- asymptomatic microscopic
hematuria : 0.5-2.0% of school aged children
;
persistent microscopic hematuria
- further outpatient
evaluation
- ¡Ú> 5
RBC/HPF on 3 UA at monthly intervals
Chapter 463. Glomerular Disease
Pathogenesis
#
mechanism of glomerular injury
; immunologic
;
inherited (presumably biochemical)
;
coagulation disorders
#
Immunologic injury
; *¡ãcommon
causes
; Áõ°Å
¨ç
experimental immune mediated GN ¿Í morphologic &
immunopathologic similarity
¨è
immune reactants(Ig & C')À» glo.¿¡¼ ¹ß°ß
¨é
serum C' abn. ¿Í autoAb.(ex.Anti GBM) ÃâÇö
2) 2 major
mechanism of immunologic injury
¨ç
localization of circulating Ag-Ab immune complex
¨è
local Ag in situ¿Í AbÀÇ interaction
ex) non-collagenous domain [NC-1] of type IV
collagen : putative Ag in human antiGBM nephritis
A. immune complex mediated ds.
immune complex -> accumulate in glo. -> injury
½ÇÇè) acute serum sickness in rabbit
iv of bovine albumin
-> acute proliferative GN
cf. glo.localization¿¡ °ü¿©ÀÎÀÚ
+-¨ç complex : conc, charge, size
| ¨è glo.ÀÇ Æ¯Â¡
: mesangial trapping, negatively charged capillary wall
| ¨é hydrodynamic forces
+-¨ê various mediatorsÀÇ
influence : angiotensin II, PG
B.
Anti-GBM antbody dsease : in situ Ag-Ab interaction
: IF»ó linear deposition of Ig. & C'
on GBM
ÀÌ´Â i) Goodpasture disease -+ °ú ºñ½Á
ii) RPGNÀÇ certain type -+
2. Biochemical
mediation
* Complement
system
+-Classic pathway : activated by Ag-Ab immune complex
+-Alternative pathway : activated by polysaccharides & endotoxin
3. Coagulation system
activated +- drectly
by endoth.injury->thrombogenic subendoth.³ëÃâ->coagulation
| cascade
+-- indirectly by C'
activation
Pathology
different ds.
state¿¡¼ similar microscoic change º¸ÀÌ´Â °æ¿ìµµ ÀÖ´Ù.
1. Proliferation of
glomerular cells
¨ç
+-generalized : all glo.
+-focal : only some glo.
¨è
+-diffuse : single glo.ÀÇ all part
+
segmental : ¡¨ some part
¨é ÁÖ·Î +-endoth. &
mesangial cell prolif. ¸¹À½
+-mesangial matrixµµ Áõ°¡
-> glo.size Áõ°¡, glo.cap.lumen °¨¼Ò
-> renal insuff.
2. Crescent formation in Bowman
space
¨ç
proliferation of parietal epith.cells of B space - esp. RPGN
¨è
in response to fibrin deposit in B space
1) new crescent
( fibrin, prolif.epith.cell, BM like material, macrophage )
->
fibroepithelial crescent
->
glomerular cell death (necrosis)
2) generalized
mesangial proliferationÀÌ µ¿¹ÝµÇ°í immune complex &
antiGBM Ab°¡ µ¿¹Ý
3) Glomerular
exudation of blood cells
neutrophils : m.c.
eos, baso, mono
4) thickened
appearance of GBM
¿øÀÎ
+- ¨ç true increase in width
of memb. : membranous glomeruloathy
| ¨è memb.°ú ºñ½ÁÇÏ°Ô ¿°»öµÇ´Â
immune comlexÀÇ massive Ä§Âø
: SLE
+- ¨é
subendothelial space¿¡ mesangial cell°ú
matrixÀÇ interposition (split app.)
:
type I MPGN, others
5) Sclerosis
scar tssue in glomerulus
¶§·Î increase in mesangial matrix ÀǹÌ
Chapter 464. Recurrent Gross Hematuria
¡ÚTable
464-1
Ig A Nephropathy (= Berger Nephropathy)
Pathology & Pathogenesis
#
LM
; focal & segmental
mesangial proliferation
; increased matrix
;
some gerneralized mesangial proliferation
; occasionally crescent
formation
#
IF
; IgA deposition in
mesangirum
; lesser amount of Ig M, Ig
G, C3, properdin deposition
#
recurrence in transplanted kidney
--> suggest systemic
disorders
Clinical & Laboratory Manifestation
; ³²:¿©
- 2:1
; episode of gross hematuria
or microscopic hematuria on routine exam
; renal function
-
relatively normal
-
proteinuria : < 1gm/day (minimal)
; C3 normal
Prognosis & Treatment
¨ç ´ë°³
no kidney damage
¨è
30%¿¡¼ progressive ds.·Î
develop.
no Tx. - activity Á¦ÇÑÀº ÇÊ¿ä ¾ø´Ù.
¨é
number of gross hematuria, persistent microscopic hematuria between
episode´Â Px.¿Í ¹«°ü
#
¡ØPoor
Prognosis Factor
; hypertension
;
diminished renal function
;
proteinuria > 1g/day between episodes of gross hematuria
;
histologic evidence of diffuse GN with crescent, scarring
#
¡ØIg A
deposition diseases
¨ç
Berger ds.
¨è
H-S nephritis
¨é
SLE
¨ê
Liver cirrhosis
¨ë
rarely MCLS
Idiopathic Hematuria (=Benign Familial Hematuria)
; Á¤ÀÇ
: LM & IF »ó normal histologic finding &
recurrent episode of gross
hematuria
EM »ó some case - marked thinning of
GBM
; excellent Px.
; F/up to DDx. Alport
synd.
Alport Syndrome
: m.c. of hereditary
nephritis
marked
variability
X chrosome¿¡ Á¸ÀçÇÏ´Â Á¦
IVÇü collagenÀÇ a5 »ç½½ À¯ÀüÀÚÀÇ µ¹¿¬º¯ÀÌ
1) Pathology
¨ç ³ªÁß¿¡
mesangial proliferation & cap. wall thickening->progressive glo.
sclerosis -> tubular
atrophy, interstitial infl. & fibrosis, foam cell
¨è
EM»ó thickening, thinning, splitting
& layering of GBM & tubule
(not specific to Alport synd.)
¨é
immunopathologic study (-)
2) Clinical sx.
¨ç
renal involvement
m.c.+- Asymptomatic microscopic hematuria
+- recurrent gross hematuria
¨è
sensorineural hearing loss (¼Ò¼ö)
-esp, high frequency range -> Á¡Â÷ ÁøÇàµÇ¾î deafness
¨é
eye abnormality (10%)
i) cataracts
ii) Keratoconus ( anterior lenticonus )
iii) spherophakia ( macular lesion )
3) Genetics
: ¨ç
X-linked dominant => male¿¡¼ severe course
( autosomal dominant µµ ÀÖ´Ù.)
¨è
20%¿¡¼´Â No family Hx. of renal ds.
4) Complication
¨ç
hypertension
¨è UTI ¨é
CRF
5) Px. & Tx.
¨ç
male:10-20´ë¿¡ end stage RF with hearing
loss->dialysis & kidney
transplantation
¨è
female:normal life span & subclinical hearing loss
Idiopathic Hypercalciuria
;
present as ¨ç
RGH
¨è persistent microscopic
hematuria
¨é
dysuria in the absence of stone formation
Etiology
; excessive GI absorption of
normal dietary Ca. intake
; defect in renal tub. Ca.
reabsop.
Diagnosis
; ¡Ú24hr U
ca. excretion > 4mg/kg
; screening test
-
¡Ørandom
Uca/Ucr > 0.2
Differential Diagnosis
; normal infants
; hypercalcemic
hypercalciuria due to hyperparathyroidism or Vit. D intoxication
Complication
; ¡ØNephrolithiasis
Treatment
; Oral thiazide
-
controversal
-
Indication
/
persistent gross hematuria or dysuria
-
*Chlorothiazide 10-20mg/kg/24hr
single morning dose
-->
Uca. excretion < 4mg/kg/24hr.µÇ°í Cl/M. resolve µÉ¶§±îÁö Áõ·®
-->
1³â ÈÄ Áß´Ü
-
S/E : *Hypokalemia
Chapter 465. Gross or Microscopic Hematuria
465.1 Acute Poststreptococcal Glomerulonephritis
#
acute nephritic syndrome
; sudden onset of gross
hematuria, edema, hypertension, renal insuff.
Etiology & Epidemiology
;
nephritogenic groupA ¥â-hemolytic streptococcus
- throat infection : *serotype 12, cold weather
-
skin inf. or pyoderma : *serotype 49,
warm weather
Pathology
; symmetrical enlarged Kidney
;
LM
-
diffuse mesangial cell proliferation with an increase in mesangial matrix
-
PMNL infiltration in glomeruli
-
maybe crescent and interstitial inflammation
; IF
-
*lumpy-bumpy deposit of Ig & C'
on GBM & mesangium
; EM
-
*humps on epithelial side of GBM
(=subepithelial hump)
Pathogenesis
¨ç
immune complexÀÎÇÑ mech.À¸·Î »ý°¢.
-
ÀÌÀ¯+-morphologic study
+-serum C3
depression
but not clear yet
¨è
acute serum sickness in rabbit°ú ºñ½Á
¨é
C'activation˼ alternative rather than classic
immune-comp. med. pathway
Clinical Manifestation
¨ç
child but 3yr ÀÌÀüÀº rare
¨è
streptococcal infectionÈÄ 1-2wk°æ°úÈÄ
latent
period
+- pharyngitis : 9-11ÀÏ
+- pyoderma : 3ÁÖ or ÀÌ»ó
¨é
Sx. :
i)
asymptomatic microscopic hematuria - ARF±îÁö ´Ù¾ç.
ii) edema,
hypertension, oliguria, encephalopathy, CHF
* edema : due to +-water
& salt retention
+-nephrotic syndrome
iii)
nonspecific symptom
malaise, lethargy, abd.
& flank pain, fever
- acute phase´Â ´ë°³
1mo. ³» È£Àü.
urinary abn.´Â
1yr.ÀÌ»ó Áö¼Ó°¡´É.
- acute phase :4-10ÀÏ
Gross hematuria : ´ë°³
1wk.
Microscopic hematuria : 1-2mo.
Diagnosis
¨ç
UA»ó
: i) RBC with ii) RBC
casts iii) proteinuria iv) PMNL ; common
¨è
mild normochromic anemia due to hemodilution and hemolysis
¨é
C3 °¨¼Ò (90%)
complement +-low for 10ds
|
normalize within 4-5wk
+-5-10%´Â S-C3 not reduced
¨ê
evidence of streptococcal inf.
i)throat culture
: Dx. of carrier state or support Dx.
ii) ASO (not
helpful : skin inf.ÈÄ´Â Áõ°¡µÇÁö ¾ÊÀ½.)
: Àεο°ÈÄ 1-2ÁÖÈÄ »ó½Â, 3-5ÁÖ
peak, 2-3 mo. °ÉÃÄ ¼¼È÷ °¨¼Ò.
iii) DNase B
antigen : best single Ab titer
*
alternative ¨Í Streptozyme test ¨Î
Streptolysin ¨Ï DNaseB ¨Ð
hyaluronidase
¨Ñ Streptokinase ¨Ò
NADase
µû¶ó¼
+-acute nephritis
| evidence of recent streptococcal inf.
+-low C3
-> À̷μ clinical Dx. of PSGN °¡´É
-> renal biopsy´Â not Ix.
but, ¨çSLE
-------+ rule out Çϴ°ÍÀÌ Áß¿ä.
¨èacute
exacerbation of chronic GN -
#
¢¾Renal biopsy indication for PSGN
¨ç development of ARF or
nephrotic synd
¨è absence of evidence of
streptococcal inf.
¨é absence of
hypocomplementemia
¨ê persistent of marked
hematuria or proteinuria
¨ë diminished renal
function
¨ì low C3
level > 3 mo. after onset
Complication
; *complication of ARF
-
volume overload, circulatory congestion, hypertension, hyperkalemia,
hyperphosphatemia, hypocalcemia, acidosis, seizures, uremia
Prevention
; early systemic antibiotic
therapy of streptococcal throat and skin infections
-
*not eliminate the risk of GN
; *culture of family member
-
if culture(+), treated
Treatment
; No specific Tx.
; 10-day systemic antibiotics
course
-
Penicillin recommended
; *restricted activity
-
*only acute phase of diseases
Prognosis
;
complete recovery
- *95% ÀÌ»ó
;
no evidence of progression to chronic GN
;
if severe acute phase, glo. hyalinization and then progression to chronic renal
insufficiency
;
*recurrence : extremely rare.
Chapter 466. Membranous Glomerulopathy
;
*¡ãcommon
cause of nephrotic syndrome in adult
Pathology
#
LM
; *diffuse thickening of GBM without significant proliferative change
-
due to memb. like material production by visceral epi.cell in response to
immune comlex -
deposited on the epithelial side of membrane
-
spike on the epithelial side of GBM
#
IF
; granular deposit of IgG
& C3
#
EM
; *EDM deposits on epithelial side of GBM
Pathogenesis
- immune complex mediated
ds.
- experimental Heymenn
nephritis ºñ½Á
Clinical Manifestation
- 2nd decade of life¿¡
m.c.
- Nephrotic syndrome ¼Ò°ß
with
microscopic hematuria
occasionally gross hematuria
- B.P & C3 : normal
Diagnosis
- renal biopsy => confirm
Ix.
¨ç presentation of nephrotic
syndrome over 8yr.old
¨è unexplained hematuria &
proteinuria
#
associated diseases
; SLE, cancer, gold or
penicillamine therapy, syphilis, HB viral inf.
Complication
; ¡Úincreased
risk of renal v. thrombosis
Treatment
¨ç
spontaneous resolution in majority of children
¨è
salt restriction & diuretics for nephrotic state
¨é
immunosuppressive therapy : ÀϺΠȯÀÚ¿¡¼ progressive renal
insuff. ¸¦ ´ÊÃá´Ù.
Chapter 467. Systemic Lupus Erythematosus
¨ç
fever ¨è
wt. loss ¨é
rash ¨ê hematologic abn.
¨ë
arthritis ¨ì
heart, lung, CNS, kidney ħ¹üÇÏ´Â systemic ds.
- kidney involvement : m.c.
manifestation in child
occasionally only manifestation
Pathogenesis & pathology
- immune complex mediated
ds.
- aberration in both B &
T cell function
#
¢ÞClassification
by WHO by EM, IF, LM
WHO
class I nephritis
;
no histologic abnormality
WHO
Class II (= mesangial lupus nephritis)
;
*mesangial deposits containing
immunoglobulin and complements in some glomeruli
class
IIA
-
normal LM
class
IIB
-
focal & segmental mesangial hypercellularity & increased matrix
WHO
Class III (= focal proliferative LN)
;
*mesangial deposit in almost all
glomerulus
;
subendothelial deposit in some
;
*focal & segmental mesangial
proliferation
;
occasionally capillary wall necrosis & crescent formation
WHO
Class IV (= diffuse proliferative LN)
;
*¡ãcommon
& severe form
;
*massive mesangial &
subendothelial deposit in all glomeruli
;
mesangial proliferation in all glomeruli
;
¡Úwire loop lesion
-
thickened cap.wall (= subendoth. deposit)
;
commonly necrosis, crescent formation, scarring
WHO
Class V (= membranous LN)
;
¡ãleast common
#
common transformation
Clinical manifestation
´ë°³
adolescent girl
¨ç
milder form (all class II, some class III) : hematuria, normal renal function,
proteinuria ( < 1gm/day )
¨è
some class III & all class IV : hematuria & proteinuria
reduced
renal function
nephrotic synd. or ARF
¨é
some prolif.LN½Ã normal UA º¸Àϼöµµ ÀÖ´Ù.
¨ê
Class V : nephrotic synd.
Diagnosis
¨ç ÀǽÉ
: crculating ANA
¨è È®Áø
: anti DNA Ab ( native double stranded )
¨é
C3 & C4 °¨¼Ò
in active ds.
¨ê
renal biopsy´Â ¹Ýµå½Ã ½ÃÇà.
Treatment
#
immunosuppressive therapy
; aims
-
clinical & serologic remission
-
*normalization of anti DNA Ab & C3, C4
; initiation in all patients
- Pds 60mg/m2/day div. 3 or 4 doses
; some class III, all class
IV Áï more severe case
- azathioprine
2-3mg/kg once daily Ãß°¡
¨é 1-2mo.ÈÄ
serologic remission µÇ¸é Pds 60mg/m2/day - ADT, single dose
-> 5mg¾¿ taperingÇØ
30mg/m2±îÁö.
¨ê azathioprineµµ °¨·®ÇÏ¿©
1yr.ÈÄ discontinue.
- À̶§
serologic study, renal monitoring ÇÊ¿ä.
Prognosis
: dramatically improved
ds. is controlled, not
cured
: relapse & side effect ÁÖÀÇ
Chapter 468. Membranoproliferative Glomerulonephritis (=Mesangiocapillary GN )
;
*¡ãcommon
cause of chronic GN in older child & young adults
Pathology & Pathogenesis
;
*hypocomplementemia
- DDx from other form of
chronic GN
;
some C3 nephritic factor
#
¡ÚThree Histologic Types
1) Type I MPGN
;
*¡ãcommon
form
; LM.
- accentuation of
lobular pattern by generalized increased mesangial cell & matrix
-
glomerular capilary wall thickening & duplicated or split due to
interposition of mesangial cytoplasm and matrix
/
¡Údouble tract or tram-tract app. of GBM
- *crescent : high relation to poor Px.
;
IF
- C3
& lesser amount of Ig in mesangium & along the pph. capillary wall
; EM
- immune
complex deposit in *mesangium &
subendothelium
2) Type II MPGN
; LM
- *less prominent mesangial change
- capilary wall
/
*ribbon-like thickening due to dense
deposit at GBM in EM
/
*rare splitting of membrane
- *crescent
: common
;
IF
-
*Ig in Bowman capsule, mesangium,
tubular BM
-
*C3 with
minimal Ig along the margin of the dense deposits on GBM
;
EM
-
*immune complex deposit in region of
but distinct from lamina densa with GBM thickening
3) Type III MPGN
;
LM, IF
-
resemble in type I
;
EM
-
*contiguous subepith. &
subendoth. deposits
-
*disruption & layering of lamina
densa of BM
Clinical Manifestation
; *¡ãcommon in 2nd decade
; mostly nephrotic syndrome
; some gross hematuria,
asymptomatic mcroscopic hematuria, proteinuria
; *HT - common
; *decreased C3
Diagnosis & Differential Diagnosis
#
¡Úonset of nephrotic syndrome in child more than 8yr
--> *renal biopsys ÇØ¾ß ÇÑ´Ù.
#
D/Dx with APGN
; °øÅëÁ¡
¨ç
gross hematuria
¨è
C3 °¨¼Ò
¨é
ASO Áõ°¡ (coincidental)
; ´Ù¸¥Á¡
-
APGN : 2mo. ³» dramatically improve
-
MPGN
/
persistent clin. Sx. -> Renal biopsyÇÊ¿ä
/ 6ÁÖ ÀÌ»ó C3
°¨¼Ò
Prognosis & Treatment
; all type is poor prognosis
-
*esp. type II
; I,II´Â
kidney transplantationÈÄ¿¡µµ Àç¹ß
¨ë
No difinite therapy -+
long term
alternate Pds-+·Î clinical courseÀÇ
stabilization.
Chapter 469. GN Of Chronic Infection
¡ÚEtiology
¨ç subacute bacterial
endocarditis ( Str. viridans or other organisms )
¨è
infected ventriculoatrial shunt for hydrocephalus ( staphylococcus epidermidis
)
¨é
syphilis
¨ê
hepatitis B, hepatitis C
¨ë
Candidiasis
¨ì
malaria
Pathogenesis
infecting organismÀÌ
foreign AgÀ¸·Î ÀÛ¿ë.
-> high
level of circulating AgÁ¸Àç¿¡ ´ëÇÑ host Ab response·Î
immune complexÇü¼º.
->
Kidney¿¡ deposition
-> GN ÃÊ·¡
3) histopathologic findings may
resembles
+- PSGN
| Membranous GN
+- MPGN
Sx : acute
nephritic or nephotic syndrome
C3
°¨¼Ò : common
4) Tx.
¨ç
eradication of inf. -> usually resolution
¨è
progression to end-stage RF °¡´É.
Chapter470. Rapidly Progressive (Crescentric) GN
;
*presence of crescents in majority of
glomeruli
;
*rapidly progressive clinical course
Classification
#
idiopathic
#
¡Ø87
2ndary group RPGN (with underlying ds.)
; PSGN
; Lupus
; MPGN
; GN of Goodpasture ds.
; GN of anaphylactoid purpura
Pathology & Pathogenesis
#
Crescents
;
inside of Bowman capsule
; *consists of proliferating epithelial cells of capsule, fibrin,
BM-like material, macrophages
; mechanism of crescents
formation
-
necrosis or disruption of glomerular capillary wall
-->
*deposition of fibrin in bowman
capsule
-->
crescents formations
#
pathogenesis
; *no evidence for immunologic mechanism
; some Ab against GBM
; others immune complexs on
capillary wall
; ¡Únormal
C3 level
Clinical Manifestation
¨ç
ARF : ´ë°³
¨è
end-stage RF within wks to mos.(after onset )
Diagnosis & Differential Diagnosis
¨ç
ANA, C3, anti DNase B titer ÃøÁ¤À¸·Î ŸÁúȯ
R/O
¨è
confirm : Kidney biopsy
Prognosis & Treatment
; PSGN associated RPGN
-
spontaneously recovery
; Lupus or Anaphylactoid
purpura associated RPGN
-
PRS + Azathioprine
-->
success
; ¡ÚRPGN
associated with remaining types
-
*poor prognosis
-
¡Ø94
some effective reports
/
*pulse methylprednisolone + oral
cyclophosphamide
/
*plasmapheresis
Chapter 471. Goodpasture Disease
;
pulmoonary hemorrhage, GN associated Ab against lung & GBM
#
Goodpasture syndrome
; clinical picture of
pulmonary hemorrhage with GN in several ds
¨ç
SLE
¨è
Ana. purpura
¨é
polyarteritis nodosa
¨ê
Wegener granulomatosis
Pathology
;
*resemble RPGN in LM
;
IF
- *continuous linear pattern of IgG along the GBM (antiGBM Ab)
Clinical Manifestation
extremely rare
in child
¨ç
hemoptysis : usually presenting complaints, pulmonary hemorrhage
¼öÀÏ ¶Ç´Â ¼öÁÖÈÄ
¨è
hematuria
¨é
proteinuria develop
¨ê Progressive RF ¹ß»ý
; *C3 : normal
Prognosis
»ýÁ¸Çϸé
commonly end stage RF ÁøÇà
No definite therapy
+-
pulse met0hylprednisone
| oral cyclophosphamide
+-
plasmpheresis
cause of death :
pulmonary hemrrhage
Chapter 472. Hemolytic Uremic Syndrome
;
*¡ãcommon
cause of ARF in young children
Etiology
; bacterial
-
¢ÞE.
coli (O157:H7) : ¡ãcommon
-
shigella, salmonella, campylobacter, s.pneumonia
- Bartonella
; viral
-
*coxsackie, ECHO, influenza,
variecella, EBV
; oral contraceptive
;
pyran copolymer(inducer of interferon)
; *SLE
; malignant hypertension
; pre-eclampsia
;
postpartum RF
; radiation nephritis
Pathology
¨ç Ãʱâ
glo. change
i)
cap.wall thickening ii)
cap.lumen narrowing iii)
mesangial widening
¨è
fibrin thrombi -> cortical necrosis
¨é
severely -> partial or total sclerosis
¡Ø92 Pathogenesis
; endothelial cell injury :
primary event
-->
localized clotting
-->
microangiopathic anemia due to altered vasculature
-->
thrombocytopenia due to intrarenal plt. adhesion or damage
; *no evidence of DIC
Clinical Manifestation
; *¡ãcommon under 4yr
¨ç ¼±ÇàÁõ»ó
´ë°³ gastroenteritis ( fever,
vomiting, diarrhea, abd. pain )
URI, less commonly
¨è
5-10ÀÏÈÄ
sudden onset of pallor,
irritability, weakness, lethargy, oliguria
¨é
P/Ex»ó
dehydration, edema, petechiae, hepatosplenomegaly, marked irritability
Diagnosis & Differential Diagnosis
;
syndrome
- *microangiopathic hemolytic anemia, thrombocytopenia, ARF
;
Laboratory Finding
-
Hb 5-9 g/dl
-
PBS
/
helmet cells, burr cells, fragmented RBCs
-
diminished Haptoglobin
-
moderate increased reticulocyte
- Coombs (-)
- *leukocytosis > 30,000/mm3
- thromocytopenia
20,000-100,000/mm3
- normali PT, PTT
- UA
/
surprisingly mild low grade microscopic hematuria, proteinuria
;
vary from mild renal insufficiency to ARF
;
barium enema
- colonic spasma and
transient early filling defects
-->
subsequential intestinal stenosis
#
Differential Diagnosis
;
Lupus, malignant hypertension
; ¡Úbilateral
renal v. thrombosis
-
*¡ãdifficult
-
°øÅëÁ¡
/
preceding AGE
/ dehydration, pallor
/
microangiopathic hemolytic anemia, thrombocytopenia, ARF
- Â÷ÀÌÁ¡
/
*marked enlarged kidney in renal vein
thrombosis
#
¢¾Renal Bipsy Ix.
;
prolonged renal failure > 2wks
; thrombocytopenia(-)
Complication
; anemia, acidosis,
hyperkalemia, fluid overload, CHF, hypertension, Uremia
; ¢¾Extrarenal Complication
-
CNS manifestation : irrtability, seizure, coma
-
colitis : melena, perforation
- *diabetes mellitus
- *rhabdomyolysis
Prognosis & Treatment
; more than 90% normal renal
function recovery
; corticosteroid
-
*no value
; anticoagulants
-
heparin
/
no beneficial effects
; *fibrinolytic therapy
-
*theoretical
-
risks to outweigh the pontential gains
; plasmapheresis,
; FFP
; ¡Úearly
& frequent peritoneal dialysis
-
*¡ãgood
treatment
-
*control of uremic state and promote
recovery by removing an inhibitor of fibrinolysis
Chapter 473. Infection As A Cause Of Hematuria
; Gross or microscopic
hematuria
- UT ÀÇ bacterial,
micobacterial, viral infectin °ú
°ü·ÃÀÖ´Ù.
1) cystitis: bacterial,
micobacterial, viral
2) urethritis:
. gross or
microscopic hematuria, urgency, urethral discomfort
. UA ß¾
RBC, pyuria
. urine culture:
usually negative
bacterial, ureaplasma, chlamydia ʦ
. spontaneous
relieve ʦ
. Tx. : 10 day
tetracycline
+ urinary analgesics(phenazopyridine hydrochloride)
. conservative
Tx. ·Î ¾ÈµÇ¸é cystoscopy ½ÃÇà
--> underlying Abn. detect
Chapter 474. Hematologic Disease Causing Hematuria
474.1 Coagulopathies & Thrombocytopenia
inherited acquired disorders of
coagulation
hemophilia
DIC
thrombocytpenia
but
usually hematuria is not a presenting sign. usually after other
manifestations
474.2 Sickle Cell Nephropathy
gross or microscopic hematuria
; sickling in hypoxic acidic, hypertonic medulla --> vascular
stasis-->
bl. flow
--> ischemia --> papillary necrosis --> interstitial fibrosis
clinical manifestation
; urinary concentrating defect, RTA
nephrotic syndrome (rarely) - focal sclerosis or MPGN °ú ºñ½Á
´ëºÎºÐÀÇ hematuria °¡
spontaneousely recover µÈ´Ù.
474.3 Renal Vein Thrombosis
Epidemiology
#
¡Ú2 Distinct Patterns
; new born & infant
-
asso. with *asphyxia, dehydration,
shock, sepsis, infant of DM mother
; after infancy
-
asso. with *NS(¡ãfrequently membranous nephropathy), cyanotic heart ds, use of
angiographic contrast agent
Pathogenesis
;
intrarenal venous radicle ¿¡¼ ½ÃÀÛÇÏ¿© antegrade &
retrograde spread
--> renal v. involve ´Â ¾ÊµÊ
;
thrombus formation
endoth. cell
injury
by i) hypoxia, ii) endotoxin, iii) contrast media
; conjunction /c hypercoagulable state (nephrotic syndrome)
diminished vs.
blood flow
hypovolemia: shock, dehydraton, NS
intravascular sludging of blood(polycythemia)
Clinical manifestaions
in
infant
i)
sudden onset of gross hematuria
ii)
unilat or bilat. flank mass
in
old children
i)
gross & microscopic hematuria
ii) flank pain
unilat. > bilat.
bilat. ½Ã
ARF
Diagnosis
Hx.
: predisposing clinical factor ÀÖ´Â pt. ¿¡¼
hematuria & flank
mass êó
Lab/F
: i) microangiopathic hemolytic anemia
ii) thrombocytopenia
U/S
ß¾: marked enlargement
radionuclide
study : little or no renal function in involved kidney
venacarvogram
& doppler flow study to
confirmed the diagnosis
in occult case --> µÉ¼öÀÖÀ¸¸é
ÇÇÇÑ´Ù.
Differential Diagnosis
hematuria
: esp. hemorrlytic uremic syndrome
renal
enlargement
i)
hydronephrosis, ii) cystic ds, iii) Wilms tumor, iv) abscess
v)
hematoma
Treatment
unilat. renal v. thrombosis
supportive tx.
i) fluid & electrolyte correction
ii) Tx. of infection
iii) prophylactic anticoagulation to
prevent thrombosis
- ȯÀÚ°¡
DIC ¿¡ ºüÁø°æ¿ì¸¦ Á¦¿ÜÇϰí´Â ÀÇ¹Ì ¾ø´Ù.
bilat. renal v. thrombosis
: CRF ·Î °¡´Â °æ¿ì°¡ ¸¹´Ù.
i) thrombectomy
ii) systemic fibrolytic agents
Prognosis
in infant
progressive atrophy --> small scared
kidney
nephrectomy ; if, hypertension or chr.
infection ( + )
-> acute phase ¿¡¼´Â ½Ç½ÃÇÏÁö¾ÊÀ½
in old children
involved kidney may recover
( ƯÈ÷ nephrotic SD À̳ª
CHD¿Í ¿¬°üµÈ thrombosisÀÇ °æ¿ì)
Chapter 475 Anatomic Abnormalities associated with Hematuria
Congenital Abnormalities
;
gross or microscopic hematuria ´Â UT. malformation ÀÇ ´ëºÎºÐÀÇ ÇüÅ¿¡¼ ³ªÅ¸³¯¼ö ÀÖ´Ù.
;
flank ¿¡ minor trauma ÈÄ
sudden onset of usually painless grosshematuria½Ã ÀǽÉ
-->
ureteropelvic junctional obstruction
cystic
kidney °¡ ÀÚÁÖ µ¿¹ÝµÊ
Trauma
;
gross or microscopic hematuria, flank pain, abdominal regidity
--
may occur
475.1 Autosomal Recessive PCK( Polycystic Kidney)
(=infantile
polycystic diseases)
;
may not detected until after infancy
;
*hepatic cysts with significant liver
disease
Patholgy
; markedly both enlarged
kidney
; grossly innumerable cysts
throughout cortex & medulla
; LM
-
*¡°cyst¡± is collecting duct dilatation
-
normal interstitium & remainder of the tubules
-->
progressing to interstitial fibrosis & tubular atropy
-->
renal failure
; hepatic cyst
-
cirrhosis, portal hypertension, eso. varix rupture
-
*when severity of hepatic
manifestation exceeds renal involvements
-->
called ¡Ú¡° congenital hepatic fibrosis¡±
Clinical Manifestation
bilat. flank mass at birth
oligo hydroamnios µ¿¹Ý Òý
--> Potter synd. ÃÊ·¡
: flat nose, recessed chin, epicanthal fold,
low set ear, limb abn. ( due to compression of fetus)
pul. hypoplasia --> neonatal resp. destress -->spont. pneumothorax
cf) spont. pneumothorax ½Ã kidney U/S é©
(in neonate)
gross & microscopic hematuria , hypertension ÀÌ ÈçÇÏ´Ù.
renal function Àº Á¤»ó ȤÀº °¨¼Ò ( renal malformation ÀÇ
severity¿¡ ÀÇÁ¸)
Diagnosis
; Hx. & P/Ex.
;
U/S finding
-
marked enlarged & uniformly hyper echogenic kidney
;
IVP
-
opacification of dilated collecting duct
-
*radial stresks similar to spokes of
wheel
; open surgical bx. of the
Liver & Rt. kidney toward the end of the 1st year
-
confirm of Diagnosis
Differential Diagnosis
.
( bilat. renal enlargement)
multicystic dysplasia
hydronephrosis
wilm`s tumor
renal v. thrombosis
Treatment
supportive ( includiing careful management of HT.)
Prognosis
severe renal involve ½Ã neonatal period ¿¡
pul. & renal insuff. ·Î ÞÝ
»ýÁ¸ÀÚ´Â renal insuff. ¹ß»ýÀü ¼ö³âµ¿¾È »ì¼öµµ ÀÖ´Ù.
À̶§ Kid. size ´Â ÁÙ°í
HT. Àº less severeÇÏ´Ù.
renal failure ¹ß»ý½Ã
-- dialysis , kid. transplantation
hepatic fibrosis, or cirrhosis ½Ã portal hypertensionÃÊ·¡µÇ¸ç
poor Px.
475.2 Autosomal Dominant
PCK
;
also known as Adult polycystic kidney
A.D.
enlarged kid. /c cortical &
medullary cysts ( dilated tubule)
sx. : 4th or 5th decade ¿¡
i) gross or
microscopic hematuria
ii) flank pain &
mass bilat.
iii) Hypertension
µ¿¹ÝÁúȯ
i) hepatic cyst of no
clinical importance
ii) aneurysm of
cerebral circulaton -- ICH ÃÊ·¡
Supportive Tx.
Px.
end stage renal failure at 6th or
7th decade
475.3 Vascular Abnormalities
hematoma -----+
of kid. & lower UT. -- extremly rare cause of hematuria
AV
malformation -+
usually
present /c gross hematuria & the passage of blood clots
renal
colic -- upper UT. involve ½Ã ¹ß»ý °¡´É
Dx. --
confirmed by angiography
Chapter 476. Miscellaneous Etiologies Of Hematuria
476.1 Exercise Hematuria
gross or microscopic hematuria °¡ vigorous exercise ÈÄ¿¡ ¿Ã¼ö ÀÖ´Ù.
benign,
rare in female, /ass. c dysuria
color of urine -- red to black
urine ¿¡¼ blood clot ÀÌ º¸Àϼö ÀÖ´Ù.
RBC
cast ¾ø°í renal ds. evidence ¾ø´Ù.
¿îµ¿¸ØÃß°í
48½Ã°£³» ¼Ò½Ç
lower UT. origine À¸·Î »ý°¢
DDx.
Rhabdomyolysis, march hemoglobinuria
476.2 Drugs
#
alteration in coagulation system
; heparin , waparin, aspirin
#
tubular damage
; PC, sulfonamide
#
hemorrhagic cystitis
; cyclophosphamide
Chapter 477. Evaluation Of The Child With Hematuria
Hx. & P/Ex.
; ÃÖ±Ù
URI, skin & GI infection --> AGN or hemolytic uremic synd.
;
frequency, dysuria, unexplained fever --> UTI
; ¡ØFlank Mass
-
hydronephrosis, cystic ds., renal v. thrombosis or tumor
; gross hematuria ÀÇ ÃÖ±Ù
Hx. --> IgA nephropathy, idiopathic hematuria
Alport
SD. hypercalciuria
rash &
joint pain µ¿¹Ý½Ã --> HSP, Lupus
±×¿Ü
trauma Hx. bleeding difficulties, drug usage, °¡Á·ÀÇ Kid. Ds. Hx. HT Hx.
Laboratory Finding
anemia
- dilutional ; ARF ¶§ fluid over load ¶§¹®
- blood loss ; pul. hemorrhage in
Goos pasture ds.
melena in HSP, HUS
- hemolytic ; HUS, SLE
---- hemorrlytic
state ÀÇ confirm - reti. count Áõ°¡
plasma Hb
level Áõ°¡
plasma haptoglobin °¨¼Ò
PBS (
microangiopathic process in HUS, renal v. thrombosis, vasculitis, SLE
autoantibody °¡ ( Coomb`s test (+), ANA,
leukpenia, multisystem. ds. )
sickle Hb
for black child --> anemia ¾øÀ»¶§µµ
urine
culture ; UTI °¡´É¼º Æò°¡
CCr,
protein & Ca excretion -- À̰ÍÀÌ ºÒ°¡´É Çϸé S-Cr., urine protein
by
dipstick , ca. to cr. ratio
in random urine
urinary
RBC morph. °¡ lower tr. À̸é
normal, glomerular À̸é dysmorphic.
-- ¹Ýµå½Ã
hematuria ÀÇ À§Ä¡¿Í ÀÏÄ¡ÇÏÁö´Â ¾ÊÀ½
#
decreased C3
; PSGN
; MPGN
; Lupus GN
; chr. infection GN
#
thrombocytopenia
: plt. »ý»ê ¡é (mal.)
: plt. ÆÄ±« ¡è ( ITP, HUS, RVT)
Imaging Studies
3) IVP, U/S --> str. Abn ¸¦
R/o ÇÔ
cytogram --> infection À» °¡ÁøÈ¯ÀÚ, lower tr. ÀÇ
lesionÀÌ ÀǽɵǴ ȯÀÚ
Renal Biopsy
persistent microscopic hematuria /ass, c
i) decreased renal function
ii) proteinuria
iii) hypertension
one or more episode of unexplained gross hematuria
persistent high grade microscopic hematuria
5) cystoscopy ; hematuria ¸¦ °¡ÁøÈ¯¾Æ¿¡¼ routine evaluationÀº ¾Æ´Ï´Ù.
more helpful - i) bright red hematuria
ii)
dysuria
iii) sterile urine culture
iv) ³²¾Æ¿¡¼ ÁÖ·Î trauma¿¡ ÀÇÇÑ
urethral lesionÀ» È®ÀÎÇÔ
v) lower UT. ÀÇ neoplasm È®ÀÎ
* hematuria °¡
6 mo ÀÌÀüµ¿¾È ÀÖÀ»½Ã
- streptococcal inf. ÀÇ serologic evidence ¸¦ È®ÀÎ ÇØ¾ß ÇÑ´Ù.
; throat. & skin inf. culture
; ANA for Lupus
** »ó±â study ¿¡¼ ¸ðµÎ Á¤»óÀÌ ³ª¿À¸é ´õÀÌ»óÀÇ
study´Â ÇÊ¿ä¾ø´Ù.
ÇÏÁö¸¸
IgA nephropathy or Alport SD. Àº ÀÖÀ»¼ö ÀÖ´Ù.
-->
long term f/u ÀÌ ÇÊ¿äÇÔ.
Section 3. Condition Particularly Associated with Proteinuria
;
upper limit of normal protein excretion in healthy person
- 150mg/24hr
- half
:
plasma origin - mostly Albumin ( 30mg/24hr)
- the others
:
Tamm-Horsfall protein (mucoprotein produced in distal tubule)
#
ÃøÁ¤ ¹æ¹ý
1) dipstick test
;
-, ¡¾, +(30mg/dl), ++(100mg/dl),
+++(300mg/dl), ++++( 2000mg/dl ¡è)
;
primarily detection of Albumin
;
*less sensitive other form protein
-
*Bence Jones protein, gamma globulin
2) Sulfosalicylic method
#
¢¾False Positive
1)
dipstick
;
highly concentrated urine
; gross hematuria
; contamination with chlorhexidine or benzalkonium
;
pH over 8.0
; phenazopyridine therapy
2) Sulfosalicylic acid method
; radiographic contrast media
; PC or cephalosporin therapy
; tolbutamide
; sulfonamide
#
semiquantitative fashion
; U-protein/U-Cr in a random
specimen (ÇÑ±ÛÆÇ:¾ÆÄ§ ù¼Òº¯)
-
2¼¼ÀÌÇÏ¿¡¼ 0.5 ¡é
-
older child ¿¡¼ 0.2 ¡é
- ¡Úmore than 3
-->
*suggest nephrotic range proteinuria
Chapter 478 Non-Pathologic Proteinuria
¡ÚTable
478-1
#
proteinuria ÀÇ Á¤ÀÇ (ÇÑ±ÛÆÇ)
; qualitative
-
1ÁÖÀÌ»óÀÇ °£°ÝÀ¸·Î 3ȸÀÌ»ó °Ë»ç½Ã
¿äºñÁß ¡Â
1.015 À϶§ : 1+ ¡è
¿äºñÁß ¡Ã
1.015 À϶§ : 2+ ¡è
; semiquantitative
¾ÆÄ§ ù ¼Òº¯¿¡¼ ´Ü¹é/Cr.(mg.dl)
ºñ°¡ 0.2 ¡è
; quantitative
i) Á¤»ó ¡´
4mg/m2/hr
ii) ´Ü¹é´¢
: 4 - 40mg/m2/hr
iii) N S ¡µ40mg/m2/hr
#
non-pathologic proteinuria
; *less than 1000mg/24hr and never
associated with edema
478.1 Postural(Orthstatic) Proteinuria
Clinical Manifestation
; may increase 10-fold time
or more
; unknown etiology
;
normal laboratory finding & renal bx.
Diagnosis
#
urine collection
; supine collection
-
¹ã¿¡ 30ºÐ°£ ´©¾îÀÖÀºÈÄ ÀÌ»óÅ¿¡¼ ¹è´¢ÇÏ¿© ÀÌ uriine Àº
discard ( time check)
--> large glass liquid ¸ÔÀÎÈÄ Àç¿ò --> ¾ÆÄ§¿¡ ´©¿îä
collection
; upright collection
-
normal daily activity ÇÏ¸é¼ 2¹øcollection
#
*supine collection˼
Á¤»óÀ̰í, upright collectionÀÌ proteinuriaÀ» º¸¿©¾ß
ÇÑ´Ù.
Treatment
; *ÃßÀû°üÂûÀÌ ¿äÇÑ´Ù.
478.2 Febrile Proteinuria
38.3¡É ¡è ½Ã õó(
101¢µ)
±âÀü
: unknown
2+ ´Â ³ÑÁö¾Ê´Â´Ù.
fever »ç¶óÁú¶§
resolve µÇ¸é benign À¸·Î »ý°¢
478.3 Exercise Proteinuria
48 hr rest
ÈÄ normal À̸é
benign
2+ ÀÌ»ó ³ÑÁö¾Ê´Â´Ù.
; marked prteinuria
glomerular
ds.
CHF
constrictive pericarditis
Chapter 479. Pathlogic Proteinuria
479.1 Tubular Proteinuria
;
loss of low molecular weight protein than albumin
- *lysozyme, light chains of Ig, ¥â2
-microglobulin, insulin, growth hormone
;
*rarely exceeds 1g/24hr
;
*not asso. with edema
;
*asso. with other defects of tubular
function
- glucosuria, phosphaturia,
bicarbonate wasting, aminoaciduria
;
Dx. by electroporesis of urine
479.2 Glomerular Proteinuria
;
*¡ãcommon
cause of proteinuria
;
variable range from less than 1g to more than 30g/24hr
;
index of protein selectivity
- C
IgG/ C transferrine or albumin
£¼ 0.1 highly selective
£¾ 0.2 poorly selective
- selective
/
*loss of plasma protein up to albumin
including albumin
/
*MCGN
- nonselective
/
alb. & largest MW protein loss ( IgG)
/
mostly GN
Chapter 480. Persistent Asymptomatic Proteinuria
#
¢¾Definition
; apparently healthy child
; *persist hematuria for 3mo without hematuria
; amount of proteinuria -
less than 2g/24hr
; *never associated with edema
#
prevalence
; 6% in school age children
#
¡Ú¿øÀÎ
;
postural proteinuria, membranous, MPGN, pyelonephritis, hereditary nephritis,
develpmental anormaly, benign proteinuria
#
¡ØEvaluation
Of Persistent Asymptomatic Proteinuria
; urine culture, Ccr, 24hr
protein excretion, s-alb., C3, renal U/S
; ¡ØAnnual Evaluation
-
UA, Ccr, BP, P/Ex, 24hr protein excretion
#
renal bx. Ix.
;
persistent asymptomatic proteinuria ¡µ1g/24hr
;
hematuria, HT., or diminished renal function µ¿¹Ý½Ã
Chapter 481. Nephrotic Syndrome ( Nephrosis )
Etiology
#
clinical
; 90% idiopathic NS
; 10% glomerulonephritis
-
membranous GN, MPGN
#
pathologic
; 85% minimal change
; 5% mesangeal proliferation
; *10% focal sclerosis
Pathphysiology
ºÎºÐÀûÀ¸·Î Cap. wall³»ÀÇ
negative charged glycoprotein ÀÇ loss·Î
capillary permeability Áõ°¡
--> proteinuria
*
nephrosis ¶§
. proteinuria > 2g/day ÀÌ»ó ÁÖ·Î
alb.
. alb. 2.5g/dl in serum ÀÌÇϽÃ
edema ¹ß»ý
#
¡ÚMechanism Of Edema Formation In Nephrosis
; urinary protein loss
-->
hypoalbuminemia
-->
decreased plasma oncotic pr.
-->
transudatoin of fluid into interstitial space
-->
decreased intravascular volume
-->
decreased renal perfusion pr.
-->
renin-angiotensin- aldosterone system activation & release of ADH
--> stimulation distal tubular reabsorption of sodium &
collecting duct reabsorption of water
-->
reabsorped Na & water shift to interstitial space due to decreased plasma
oncotic pr.
Na.
& water excretion ¿¡ÀÖ¾î intrarenal defect
Cap.
wall permeability ¸¦ Áõ°¡½ÃŰ´Â circulating agent
# ¢¾Hyperlipidemia Mechanism
; hypoproteinemia
-
stimulation of generalized protein synthesis in the liver including the
lipoprotein
; diminished lipid catabolism
-
due to decreased level of lipoprotein lipase
481.1 Idiopathic Nephrotic Syndrome
;
90%
;
has been transformed into another type
Etiology
;
unknown
;
immunologic mechanism ¿¡ ÀÇÇØ mediate
;
IgE mediation in some
;
̧̿: abn. in thymus-derive T-cell
lymphocyte function
--> vascular permeability Áõ°¡ÀÎÀÚ °ü¿©
Pathology
#
¡Ú3 Morphologic Pattern
1)
MCNS (85%)
;
normal or minimal increased in mesangial cell & matrix
;
*EM - retraction of epith. foot
process
;
IF - typically negative
;
*95%À̻󿡼
corticosteroid ¿¡ responce
2) Mesangial proliferative
group(5%)
;
diffuse increase in mesangial cell & matrix
;
IgM, C3 µîÀÌ ÁÖ·Î Ä§ÂøµÊ
;
*50 - 60% ¿¡¼
corticosteroid¿¡ ¹ÝÀÀ
3) *Focal Sclerosis (10%)
;
mostly glomerulus - normal or mesangeal proliferation
;
*juxtamedullary glomerulus -
segmental scarring in one or more lobules
;
*progressive course
-->
ultimately involvement of all glomeruli
-->
*end stage RF
;
*20% response to prednisolone or
cytotoxic therapy
;
transplanted kid. ¿¡µµ Àç¹ßʦ
Clinical Manifestation
; *male:female = 2 : 1
-
MCNS or FS : 66% male
-
MPGN : 65% female
; *¡ãcommon between 2-6yr
.
URI °¡ ¼±ÇàµÇ¾î ³ªÅ¸³ª´Â °æ¿ì°¡ Òý
pitting edema ( periorbital, lower ext.)
--> generalized edema, wt. loss, ascites, pleural effusion, UO ¡é.
anorexia, abd. pain, diarrhea; common
hypertension ; uncommon
Diagnosis
UA: proteinuria 3+ or 4+
Microscopic hematuria (+). Gross
hematuria : rare
Renal func. : normal or diminished
lower Ccr. ; vol. ¡é ÀÎÇØ
, renal perfusion ¡é
--> intravascular vol. dl restore µÇ¸é
normal ·Î µ¹¾Æ¿Â´Ù.
protein excretion ; 2g/24hr ¡è
(40mg/m2/day¡è)
s- Alb. ¡´ 2g/dl
s- Ca. ¡é ( owing to alb.
bounding fraction ¡é)
C3 ; normal
8¼¼ÀÌÀü (1-8¼¼)
onset; renal bx. ¾øÀÌ corticosteroid tx.
8¼¼ÀÌÈÄ MCNS °¡ ¸¹Áö¸¸
membrain or MPGN ÀÌ increasingly common
--> renal bx. ¸ÕÀú½ÃÇàÇϰí
Ä¡·á
Complication
#
Intection
; major Cx
; ¢ÞWhy Susceptable ?
-
decreased Ig level
-
edema fluid acting as a culture media
-
protein deficency
-
decreased bactericidal activity of leukocyte
-
immunosuppressive therapy
-
decreased perfusion of the spleen due to hypovolemia
-
loss of complement factor (properdin factor B) in urine
; type
-
peritonitis
/
*¡ãcommon
-
sepsis, pneumonia, cellulitis, UTI
; organism
-
¡ÚStreptococcus pneumoniae
/
*¡ãcommon
for peritonitis
-
G(-) bacteriae
; prevention
-
*all patients ´Â remission ½Ã polyvalent
pneumococcal vaccination
#
Tendency To Arterial & Venous Thrombosis Tendency
; ¢Þwhy ?
-
*elevated plasma levels of
coagulation factor in plasma (1, 5, 7, 8, 10)
-
elevated levels of inhibitors of fibrinolysis
-
decreased levels of anti-thrombin III
-
increased platelet aggragation
; site
-
renal v. & IVC
- thrombosis, pul. embolism, cerebrocortical thrombosis
#
deficiency of coagulation factors (9, 11, 12)
#
reduced serum vitamin D
Treatment
#
salt restriction: edema ¾ø¾îÁö¸é ÇØÁ¦
fluid
intake: edema ½ÉÇÏÁö¾ÊÀ¸¸é not restrict
tolerable
physical activity
#
Tx of generalized edema
; diuretics
i) chlorothiazide 10-40mg/kg/day #2
ii) spironolactone 3-5mg/kg/day qid :
hypokalemia Àִ°æ¿ì »ç¿ë
oral
kcl supplement
hospitalization
severe edema -->respiratory
destress(pleural effusion)
--> scrotal swelling, ascites
; ¡ÚSevere
Edema
-
°ú°Å) iv albumin and then iv
furosemide
-
ÇöÀç) ¢Àoral
furosemide 1-2mg/kg every 4hr + metolazone 0.2-0.4mg/kg/day #2
/ should be monitoring of electrolyte
& renal function
-
maybe 25% albumin 1g/kg24hr
/
transient, vol. overload with hypertension & heart failure
#
Corticosteroids
; ¡ÚPrednisone
60mg/m2/day (max. 60mg/day) #3-4
; usually reponse *within 2wks
-
*if not response 1mo after continuous
steroid therapy
-->
*steroid resistant ·Î
»ý°¢Çϰí renal biopsy¸¦
½Ç½ÃÇÑ´Ù.
; 5days after urine becomes
free( -, ¡¾, + on dipstick)
-->
PRS every other day as a single dose with breakfast
-->
continued for 3-6mo
/
¢¾why?
:
maintain the remission using nontoxic dose of PRS
:
avoiding frequent relapse
:
avoiding cumulative toxicity of frequent courses of daily administration of PRS
-
*abruptly discontinued
/
*Ä¡·áÁß´ÜÈÄ 1³â±îÁö´Â severe illnes, surgery°¡ ÀÖÀ»¶§ corticosteroid supplementationÀÌ
ÇÊ¿äÇÔ
#
¡Ú¡°relapse¡±
-
*recurrence of edema not simply of
proteinuria
#
steroid dependent
;
relapses shortly after switching to or after terminating alternative-day
therapy
#
Tx of relapse
; treated in similar manner
; *if frequently relapse or severe toxicity of steroids, considered
cyclophosphamide
-
prolongation of duration of remission
-
3mg/kg/24hr as a single dose for 12wks
-
renal bx. recommanded before trial
-
S/E
/
leukopenia, disseminated varicellar infection, hemorrhagic cystitis, alopecia,
sterility
-
WBC count weekly
/
if WBC < 5000/mm3, discontinued
#
Tx of steroid resistent
; 3-6mo cyclophosphamide +
methylprednisolone
; cyclosporine
#
renal transplantation
; end stage renal failure due
to steroid resistent focal & segmental glomerulosclerosis
#
recurrent nephrotic syndrome
; 15 - 55% of pt.
Prognosis
repeated
relapse µÇ¸é steroid responsive nephrotic
syndrome ÀÇ °æ¿ì
2nd decade ¸»¿¡ spontaneous resolution µÊ
+-residual renal dysfunctionÀ̾ø°í
| generally not hereditary
+-fertile ( unless cyclophosphamide or chorambucil tx. )
--> reassurance é©
remission ½Ã´Â normal & unrestricted diet
& activity
--> ´õÀÌ»ó urine protein check ÇÊ¿ä¾øÀ½
481.2 Glomerulonephritis
:
2ndary nephrotic syndrome /ass,c Glomerulonephritis
--
nephrotic syndrome may develop during the course of any type of GN.
/mc in ass. membranous
MPGN
PSGN
Lupus
Chr. infection( malaria, Schistosomiasis)
H S P nephritis
; secere Glo. ds. ÀǹÌÇϳª nephritis ÁÁ¾ÆÁö¸é¼
resolve ʦ
481.3 Tumors
#
solid tumor such as carcinoma
; membranous glomerulopathy
immune complex ds.( tumor Ag. °ú tumor specific Ab. ·Î ±¸¼ºµÈ
immune complex ¿¡ ÀÇÇØ mediate µÊ
)
#
Lymphoma ( esp. Hodgkin ds.)
; ¡Øminimal change ds.
-
*¡ãcommon
; proliferative change
; lymphokine by tumor --> Glo. cap. permeability
481.4 Drugs
#
membranous glomerulonephropathy
; penicillamine, gold,
mercury compounds, captopril
#
minimal change ds
;
probenecid, ethosuximide, methimazole, lithium
#
proliferative GN
;
procainamide, chlorpropamide, phenytoin, trimetadione, paramethadione
481.5 Congenital
Nephrotic Syndrome
#
¢¾Causes Of Nephrotic Syndrome In 1st 6mo Of Life
; congenital nephrotic
syndrome
; congenital infection
-
syphilis, toxoplasmosis, CMV
;
diffuse mesangial sclerosis of unknown origin
-
Drash synd. : nephropathy, Wilms tumor, genital abnormalities
#
causes of nephrotic syndrome between 6-12mo
; idiopathic nephrotic
syndrome
; drugs
Congenital Nephrotic Syndrome (= Infantile
Microcystic Ds., Finnish Type)
; A.R.
; common in scandinavian descent
Pathology
; proximal convoluted tubule ÀÇ
dilatation ( microcystic ds.)
; glomeruli -- mesangial prolif.
& sclerosis
Pathgenesis
; unknown
Clinical Manifestation
; proteinuria ( birth or ´ë°³
3mo À̳»)
; ass. sx.
i) prematurity
ii) enlarged placenta
iii) resp. destress
iv) separation of cranial suture
Prognosis
course: persistent edema
recurrent infection
death by 5yr of age (¡ñ
infection or renal failure ·Î »ç¸ÁÇÑ´Ù.)
Treatment
; supportive
; kidney transplantation--> ±Ã±ØÀûÀ¸·Î
corticosteroid & immunesuppressive
agent : no value
AFP: prerenal Dx. °¡´É
(20ÁÖÀÌÀü¿¡)
Section 4. Tubular Disorders
Chapter 482. Tubular Function
Sodium
1) reabsorption
+-65% of filtered Na+ : proximal
tubule
|
glucose & aminoacid °¡ ÇÔ²² reaborption
|
--> isotonically absorbed
+-25% : ascending liimb of loop of Henle
|
active transport of Cl ¿Í ÇÔ²²
+-10% : distal tubule & collecting duct
mediated by aldosterone
ÀÌ¿Í °°Àº reabsorptionÀ¸·Î »ýÈÄ
1¼¼ÀÌÈĺÎÅÍ
urinary Na concentration ÀÌ 1mEq/L À¯Áö
2) excretion
ECF vol.°ü¿© ¡ç ECF vol. regulating factor¿¡ ¿µÇâ ¹ÞÀ½
Potasium
1)
absorption
all of the filtered potasium, reaborbed in proximal tubule
2)
excretion
distal tubular & collecting duct secretion
modified by ECF
pH
aldosterone
urine
flow rate & Na + conc.
Calcium
98% Èí¼ö at tubule
65% °¡ prox. tubule ¿¡¼
Na Èí¼ö¿Í °ü·Ãêó
. Èí¼öÀÇ Áõ°¡ PTH
reduction of ECF
thiazide diuretics
.
Excretion Áõ°¡ saline infusion
furosemide
Phosphate
:
´ëºÎºÐ prox. tubule
reabsoption inhibited by PTH
Magnesium
:
prox. tubule ¿¡¼ 25%Èí¼ö( 1/4)
´ë°³ Èí¼ö ¹× moderation of Mg
excretion ÀÇ ÁÖ¿äÀå¼Ò:
thick ascending
limb of Henle
Maturation Of Tubular Function
; at birth
& ±×ÈÄ ¸î°³¿ù±îÁö´Â tubular functional
capabilities °¡ ¼ºÀκ¸´Ù ³·´Ù.
healthy newborn ÀÇ maximal urinary conc.
capacity °¡ ³·´Ù.
( 600 - 700mOsm/kgH2O )
# ³·ÀºÀÌÀ¯
reduced GFR
tubular cell immaturity
reduced nephron length
reduced medullary solute gradient
due to +-increased medullary blood flow
+-low urea production
ADH
¿¡ diminished tubular responsiveness
newborn ÀÇ diluting capacity´Â
adult ¿Í À¯»ç
water load excretion °¨¼Ò in NB ( low GFR ¶§¹®)
newborn ˼ Na+ , K+ , H+ , phosphate
excretion °¨¼Ò
--> due to +-low GFR
+-immaturity
of tubular function
# Tubular Ds.
1) functonal
defect in the function of tubular cell
relatively rare
inherited or acquired
initially normal renal function
ÀϺδ progressive renal damage
2)
anatomic disorder
structure of tubule ÀÇ ÀÌ»ó
ex) cystic ds.
Chapter 483. Renal Tubular Acidosis
: impaired urinary acidification ¿¡ÀÇÇÑ systemic
hyperchloremic acidosis ÀÇ
clinical state.
.
Type
type I : distal RTA
type II : proximal RTA
type IV : mineralocorticosteroid deficiency
cf) type III : variant type I
Normal Urinay Acidification
filtered HCO3 ÀÇ
85% ´Â prox. tubule ¿¡¼
reabsorption µÈ´Ù.
³ª¸ÓÁö
15%´Â distal tubule ¿¡¼
reabsorption. --> premature ¿Í neonate ¿¡´Â
ÀϽÃÀûÀ¸·Î ¾à°£ °¨¼ÒµÊ
acidification mediated by
i) distal tubular secretion of H+
(ÀϺδ mineralocortocoid - dependent)
ii) distal tubular secretion of ammonia
( acidic urine ¿¡¼ ammonium ion Çü¼º
)
¡¤
normal kid. ´Â filter µÈ ¸ðµç
bicarbonate¸¦ Èí¼öÇÑ´Ù.
¡¤
bicarbonate ˂ prox. tubular reabsorption˼
Na+ ¿Í ±³È¯ÇÏ¿© tubular umen
³»·Î
H+ ÀÇ ºÐºñ¸¦ À¯¹ßÇÑ´Ù.
¡¤
H+ Àº carbonic acid ¸¦ Çü¼ºÇϱâ À§ÇÏ¿©
carbonic anhydrase ¿¡ ÀÇÇØfiltered
bicarbonate ¿Í °áÇÕÇÑ´Ù. --> ÀÌ´Â
water ¿Í carbon dioxide ·Î ÇØ¸®µÈ´Ù.
¡¤
carbon dioxide ´Â proximal tubular cell ·Î
diffusion µÈ´Ù.
¡¤ ¿©±â¼
carbonic anhydrase ÀÇ ¿µÇâÀ¸·Î ´Ù½Ã carbonic acid ·Î
change µÈ´Ù.
¡¤
carbonic acid ´Â H+¸¦ »ý¼ºÇϱâ À§Çؼ ÇØ¸®µÇ°í ÀÌ´Â ´Ù½Ã bicarbonate ¸¦
Èí¼öÇϱâÀ§ÇØ ºÐºñµÈ´Ù. ±×¸®°í À̶§
bicarbonate ´Â ´Ù½Ã peritubular cap. ·Î
µé¾î°£´Ù.
¢ÞTable
483-1
Proximal RTA ( Type II)
Pathgenesis
1) reduced prox. tubular
reabsorption of bicarbonate
presumably
due to deficient carbonic anhydrase production --> prox. tubule
¿¡¼ 60%¸¸ reabsorption ( distal tubuleÀº
15% ¸¸ Èí¼ö )
25% ±îÁö
urinary loss
; prox. RTA more
severe than distal RTA
2) Lab/f
s-bicarbonate ¡é: 15 - 18mEq/L (bicarbonate
threshold )
urine Àº acidified( pH¡´
5.5) ¡ñ distal tubule ÀÇ
acidification mechaniam ˼
intact Çϱ⠶§¹®¿¡ filtered bicarbonate °¡ °¨¼Ò
hypokalemia
distal tubule ¿¡¼ NaHCO3 ´Ù·®
--> Na reabsorption in exchange
for k+
Hyperchloremia
ECF vol. °¨¼Ò·Î ÀÎÇØ +- chloride reabsorption ÀÚ±Ø
+- aldosterone secretion ¡è --> k+ loss¡è
3) classification of prox. RTA
a) isolated prox. RTA
transient or persistent
sporadic or inherited ( ´ë°³ A.D. )
b) Fanconi synd.
;
generalized defect in prox. tubular transport
Ư¡
: glucosuria
phosphaturia
aminoaciduria
prox. RTA
* primary
Fanconi synd. : A.D. or recessive
* secondary Fanconi synd. : inherited form ,
acquired form
Inherited
Form
Cystinosis
:
AR
i) nephropathic form: 3¼¼ÀÌÇÏ õó
Sx.: polyuria, polydipsia(conc. defect), Fever (dehydration),
growth
retardation
rickets, hotophobia, blond hair & fair skin(diminished pigment)
Dx.: cystine crystal in cornea by slit lamp
elevated cystine contents of leukocytes
course : progressive renal damage ¡ç intracellular cystine
accum.
end stage RF at end of 1st decade
Tx. : none, cysteamine À¸·Î ÀÓ»ó½Ãµµ
ii) Juvenile form: later in life, less severe
Lowe Syndrome
i) X-linked
ii) mental retardation
iii) Hypotonia
iv) cataract, glaucoma
v) generalized prox. tubular dysfunc.
unknown metabolic defect
Galactosemia
prox. tubule ¿¡¼ prolonged ÇÑgalactose
acc. À¸·Î ÀÎÇØ¼ renal manifestationÀÌ
³ªÅ¸³²
Hereditary fructose intolerance
AR
fructose 1-phosphate aldolase def.
tyrosinemia
Wilson ds.
medullary cystic ds.
AD
cf) ºñ½ÁÇÑ ÁúȯÀÎ
nephronophthisis ´Â AR
children Àº recessive °¡ Òý
adult ´Â dominant °¡ Òý
.Pathology
cyst
in the medulla: dilatation of the distal tubule & collecting duct
progressive interstitial inflammation & fibrosis ·Î ÀÎÇØ
-->
glomerular sclerosis, cortical atropy, renal insufficiency
cause of acquired Fanconi synd.
heavy metals( lead, mercury, cadmium, uranium)
out-dated tetracycline
proteinuric states ( myeloma, nephrotic syndrome )
interstitial nephritis
hyperparathyroidism -+
| carbonic anhydrase inhibition ¿¡ÀÇÇØ prox. RTA
| ÀÇ ¿øÀÎ
vit-D def. reckets ---+
Distal RTA (Type I)
distal tubule °ú
cdllecting duct ¿¡¼ H+ secertion deficiency
urinary HCO3 ÀÇ loss´Â ´ë°³
(5 - -15%)
severe systemic acidosis Áö¸¸ urine pH ´Â
5.8 ÀÌÇÏ´Â µÇÁö¾ÊÀ½
hyperchloremia, hypokalemia
nephrocalcinosis ʦ
+-isolated
| sporadic
|
inherited: AD or AR
+-2ndary i) interstitial nephritis
ii) toxin, amphotericin B, lithium, toluene
Medullary Sponge Kidmey
non-inherited ds.
º´¸® : cystic dilatation of the
terminal portion of the collecting duct
( renal pyramid µé¾î°¡´ÂºÎºÐ)
renal func. & life span : normal
µ¿¹Ý
pyelonephritis, hypercalciuria, nephrocalcinosis
nephrolithiasis, distal RTA , impaired concentrating capacity
Mineralocorticoid Deficiency ( Type IV)
1) ±âÀü : aldosterone ÀÇ
inadequate production
aldosterone ¿¡ distal tubule ÀÇ
responsiveness °¨¼Ò
--> tubular cell mb. ¿¡ electrochemical gradient Çü¼º ¾ÊµÊ
tubular lumen ÀÌ negative µÇ¾î
H+ ion secretion °¨¼Ò ÀߵǰÔÇÏ´Â
gradientÇü¼º¾ÊµÊ
--> Aldosterone mediated Na+ resorption °¨¼Ò
--> hyperkalemic , hyperchlooremic acidosis (-> renal ammonia
production °¨¼Ò->
ammonium ion excretion °¨¼Ò -> urine pH 5.5 ÀÌÇÏ
)
2) ¿øÀÎ
adrenal gl. ds. ( aldosterone ¡é, renin ¡è)
i) addison ds.
ii) cong. adrenal hyperplasia
iii) primary hyper aldosteronism
:
ald. production °¨¼Ò
normal renal fx.
urinary Na+ wasting, plasma renin level ¡è
Hyoreninemic hypoaldosteronism ( renin & aldo. ¡é)
i) kid. ds. /c interstitial damage &
juxtaglomerular
apparatus destruction
ii) vol. expansion --+°úµµ °ü¿©
PG inhibition ---+
: renal function °¨¼Ò
pseudohypoaldosteronism ( renin & ald. ¡è)
i) distal tubular responsiveness to ald.
: normal renal function
salt wasting
ii) renal insufficiency & medllary ds. °¡ÀÖ´Â
adult ¿¡¼ º¼¼öÀÖÀ½
Clinical Management Of RTA
Clinical Manifestation
#
isolated form of prox. & distal RTA
; *growth failure toward the end of 1yr of life
; GI symptoms
#
secondary
;
similar to isolated form
; underlying ds features
#
Mineralocorticoid deficiency
;
underlying ds feature
#
distal RTA
; hypercalciuria
-
nephrocalcinosis
nephrolithiasis
renalparenchymal
destruction
(2)
¿øÀÎ :unknown
°¡´É calcium carbonate release À§ÇØ
bone destruction
( acidosis ±³Á¤À§ÇØ carbonate -->
bicarbonate )
urinary citrate °¨¼Ò --> calcium chelation
Diagnosis
1)
systemic acidosis ÀÏÀ¸Å°´Â ÁúȯÀ» ¸ÕÀú R/o
diarrhea, lactic acidosis, DM,
renal failure
2)
Lab.
prox. & distal RTA : s-
bicarbonate ¡é, s- k+¡é,
hyperchloremia
Mineralocorticoid
deficiency, RTA, systemic acidosis, hyperkalemia
3) 1st morning urine pH ¿Í µ¿½Ã ¿¡
s- electrolyte ÃøÁ¤ ; prox. & distal RTA
ÀǽɽÃ
s- HCO3 < 16mEq/L
pH < 5.5: proximal RTA
pH > 5.5: distal RTA
s- HCO3 ( 17 - 20mEq/L)
i) ammonium chloride loading ÈÄ °¨º°Ê¦
ii) fractional excretion of bicarbonate
4)
prox. RTA ½Ã glucosuria, phosphaturia, aminoaciduria
ã±â
5)
RTA confirm ½Ã underlying cause ã±â
Treatment
Goal +-- correction of acidosis
+-- maintenance of normal HCO3, k+
Shohl solution
: normal alkalinizing solution for
oral use containing
1,mEq/L of sodium as sodium
citrate
potassium citrate ÷°¡
( 1mEq/L of Na, K & 2mEq/L HCO3)
NaHCO3 tablet (325 - 650mg) in order pt.
diuretics & kayexalate for
Mineralocorticoid deficiency
Prognosis
1)
isolated prox. RTA
óÀ½¿£ distal º¸´Ù ½ÉÇϳª
resolution ʦ
2) isolated
distal RTA
life long ds. ( renal failure ´Â ¹ß»ýÇÒ¼ö ÀÖÁö¸¸)
early detection & treatment before nephrocalcinosis ½Ã
excellent Px.
continuing alkali therapy & life long monitoring of clinical status
3) Mineralocorticoid
RTA
´ë°³
obstructive uropathyÈÄ õó ÇϹǷΠobstruction correction ÈÄ
12mo ³» ¼Ò½Ç
483.1 Rickets Associated with RTA
rickets may be present
type II RTA or proximal RTA
hypophosphatemia &
phosphauria
hyperchloremia,
metabolic acidosis, bicarbonuria, hyperkaliuria
bone demineralization
- Type I & distal RTA
type I - ÀûÀýÇÑ
acid urine Çü¼ººÒ°¡
type II -
lowered renal threthold for bicarbonate
impaired urinary acidification
metabolic bone ds. -
occured in both type
¢¹ Ư¡ : bone pain, growth
retardation, osteopenia, pathologic fracture
RTA pt. ¿¡¼
1,25(OH)2D ´Â normal
RTA pt. ¿¡¼
azotemia ³ª renal mass ÀÇ
loss °¡ ÀÖ´Â °æ¿ì
-- serum
1,25(OH)2D ´Â Á¾Á¾ °¨¼ÒÇÑ´Ù.
distal RTA ¿¡¼
bone dimineralization
- dessolution of bone
- because -- CaHCO3 °¡ metabolic acidosis ¿¡´ëÇÑ
buffer·Î ÁÖ¾îÁö±â¶§¹®
acidosis ¸¦ ¿ªÀü½Ã۱â
À§ÇÑ bicarbonate ÀÇ Åõ¿©´Â
bone dissolution & hypercalciuria ¸¦ ¸ØÃß°Ô ÇÔ.
¢¹Treatment.
proximal RTA - bone ds. ÀÇ Ä¡·á¸¦ À§ÇØ
bicarbonate ¿Í oral phosphate¸¦ Åõ¿©
vit-D : secondary
hyperparathyroidism¿¹¹æ
483.2 Fanconi Syndrome
(rickets
associated with multiple defect of the proximal renal tubule)
1) °³¿ä
1. Ư¡
: generalized aminoaciduria, renal glycosuria. & phosphaturia resulting in
hypophosphatemia
2. ass. but inconstant
renal tubular abn.
a. excessive bicarbonaturia leading to RTA
b. hyperkaliuria leading to hypokalemia
c. rmdhl sodium wasting, uricosuria, proteinuria & hyposthenuria
3. clinical hallmarks
a. linear growth failure
b. rickets resistant to dose of vit.-D that are ordinarily adequate for
tx. of
nutritional deficiency
2) Etiology
1. genetically
transmitted inborn error of metabolism
; cystinosis,
fructose intolerance, galactosemia, glycogenosis, Lowe synd.
tyrosinemia, & wilson ds.)
2. some acquired ds.
:
including exposure to environmental toxins
a. heavy
metals; Cd, Pb, Hg.
b. drugs;
tetracycline, gentamicine, azathiopurine
3. Idiopathic : /mc
sporadic or inherited as a mendelian dominant or recessive
3) Pathogenesis
1. abn. in some final
common pathway for normal mb. trnsport in the prox.
renal tubule
:
deficient energy production, abnormalities in mb. str. or both°¡
impaired
tubular uptake or back-leak of solutes À» ÃÊ·¡ÇÔ
2. renal potassium
wasting ˼ bicarbonate & glucose ˂
excessive urinary loss
¿¡ÀÇÇØ ÃÊ·¡µÊ
3. urinary sodium loss´Â
large excretin of urinary anion ¿¡ ÀÇÇÔ
4. normal to low serum
calcium, varable urinary calcium
5. vasopressin -
resistant urinary concentrating defect °¡ Á¾Á¾ ³ªÅ¸³².
6. rickets´Â combined effects of metabolic acidosis
& hypophosphatemia
or hypophosphatemia alone ¿¡ ÀÇÇØ ÃÊ·¡µÊ
7. vitamin D
resistance ´Â metabolic acidosis Á¸Àç½Ã ³ªÅ¸³ª´Â
abn. prox. tubular
cells ¿¡ ÀÇÇØ¼ conversion of vitamin
D to 1.25(OH)2 D2 ÀÇ Àå¾Ö¿¡ ÀÇÇÔ
8. nonspecific
microscopic findings
a.
dilatated renal tubules, with variation in size & shape, swelling of
epithelial
cells, & atropy
b.
common foci of interstitial fibrosis
c.
enlarged mitochondria on EM
d. Ư¡ÀûÀ¸·Î
glomerular architecture´Â ¸»±â±îÁö º¸Á¸µÊ
4) Clinical manifestations
1. primary Fancony
synd. Àº Ư¡ÀûÀ¸·Î first 6mo of life ȤÀº
3rd or 4th decade
¿¡ ³ªÅ¸³².
2. ¿µ¾Æ±â¿¡´Â
vomiting , polydipsia, polyuria, contipationÀÌ ¹ß»ýÇÔ
3. episode og
weakness, fever /c dehydration, & metabolic acidosis
4. failure to thrive,
especially in linear growth
5. adquate vit-D
intake & the absence of glomerular insufficiency ¿¡µµ ºÒ±¸Çϰí
¹æ»ç¼±ÇÐÀûÀ¸·Î rickets , steopenia ³ªÅ¸³². À̼ҰßÀº
renal tubular cause ¸¦
³ªÅ¸³½´Ù.
5) Lab.
1.
hyperchloremic metabolic acidosis /c normal "anion gap", hypokalemia,
hypophosphatemia, & hypouricemia
2. elevated
fractional excretion of phosphate
3. elevated ALP
activity if rickets is present
4. glycosuria at
normal serum glucose concentrations
5. generalized
nonspecific aminoaciduria
6.
inappropriatly elevated urinary pH , /c low levels of urinary ammonia &
titratable acid
7. ¸»±â¿¡
GFR ÀÌ °¨¼ÒÇϸé s-electrolyte ¿Í
aminaciduria, glycosuria.&
phosphaturia ÀÇ "paradoxic" improvement
¸¦ º¸ÀÓ
6) Dx.
1. no definite
diagnosis test for idiopathic Fanconi syndrome
2.
aminoaciduria, diminished tubular reabsorption of phosphate, & elevated ALP
activity
3. child /c
stunted growth. rickets refractory to ordinary doses of vit-D , renal
glycosuria ´Â multiple tubular dysfunctionÀ» ÀÏÀ¸Å´
4. metabolic
acidosis, hypokalemia : È®ÁõÀû
5. fluid
deprivative to test urinary concentrating ability
: risky in the face of obligatory hyposthenuria
6. glucose loading
: profound symptomatic hypokalemia by shifting potassium
into cells À» ¾ß±â
7) Treatment
1. 2ndary Fanconi syndrome ȯÀÚ´Â
underlying cause ¸¦ ã¾Æ¾ß ÇÑ´Ù.
2. primary Fanconi syndrome ˼
mineral & electrolyte balance µîÀÇ
symptomatic therapy ¸¦ ½ÃÇàÇϸé, »ý¸íÀ» ¿¬ÀåÇϰí Á¾Á¾
normal life ¸¦ ¿µÀ§ÇÑ´Ù.
3. ricketsÀÇ Ä¡·á,
skeletal deformities ¿¹¹æ
4. rickets or osteopenia ÀÇ Ä¡·á
a. i) large dose
of vit.-D : usual starting dose 5000u/24hr
maximal dose 2000 - 4000 u/kg/day
ii)
dihydrotachisterol : starting dose 0.05 - 0.1 mg/24hr
( 1mg is equivalent to 120000 u of vit-D)
iii)
1,25(OH)2D : greater potency, shorter half-life, hypercalcemia ÃÊ·¡°¡ ÀåÁ¡
b. vit-D over
dose ¿¡ÀÇÇÑ hypercalcemia ¸¦ ¹æÁöÇϱâ À§Çؼ
s-Ca. level À»
ÁÖ±âÀûÀ¸·Î ( weekly at first, then monthly) À¸·Î ÃøÁ¤
c.
hypophosphatemia ÀÇ Ä¡·á
i) oral
supplementation /c 1 - 3g for neutral phosphate/24hr given in
4 - 5 equally spaced dose
ii)
abdominal pain, diarrhea °¡ Ä¡·áÁß ³ªÅ¸³ª¸é ÀϽÃÀûÀ¸·Î Áß´ÜÈÄ lower dose
·Î ´Ù½Ã ½ÃÀÛ
iii)
phosphate ´Â hypocalcemia, 2ndary
hyperparathyroidism À» ¹æÁöÇϱâ À§ÇØ
¼ ¹Ýµå½Ã vit.-D ¿Í µ¿½Ã¿¡ Åõ¿©
5. metabolic acidosis due to
excessive bicarbonaturia ±³Á¤Àº ¸¹Àº ¾çÀÇ alkali
( 2 - 15 mEq/ kg/24hr of alkali ) °¡ ÇÊ¿äÇÔ
a. i) sodium
bicarbonate : 1 mEq of base = 1ml
ii)
shohl solution : 1 mEq of
base = 1ml
( 140 g of citric acid, 90 g of sodium citrate qs to 1L with water)
iii)
polycitra : 2 mEq of base = 1ml
( 5ml = 550mg of potasium citrate, 500mg of sodium citrate, 334mg of
citric acid )
b. serum
bicarbonate level À» °ÅÀÇ normal level
(18-20mEq/L)±îÁö
±³Á¤ÇÏ¿©¾ßÇÔ
c. alkali´Â
1-1¨ö hr after meals in 3-4 divided
dose/day ¿¡ º¹¿ë
d. extra salt
& water should be provided to counter excessive losses
483.3 Cystinosis ( Lignac Synd., Fanconi Syndrome With Cystinosis )
1) Ư¡
1. the clinical &
lab. features of Fanconi syndrome
2. the additional
distinctive finding of abn. accumulation of cystine in
various tissues
2) Pathgenesis
1. unknown cause
2. increased uptake of
cystine ´Â lysosomes ¿¡ ÃàÀûÀ» ÃÊ·¡ÇÔ
ÀÌ´Â ÀÌ
amino acide ÀÇ lysosomal release ÀÇ
failure ¿¡ ÀÇÇÔ
specific enzyme defect ´Â ¹ß°ßµÇÁö ¾Ê¾ÒÀ½
3. cystine deposition ÀÇ ºÎÀ§
i) RES
system : esp. in spleen, liver, lymph node & BM
ii) renal
tubular cells, cornea & conjunctiva
iii)
periperal blood leukocyte & fibroblasts
iv) but
not in muscle & brain
3) Á¶Á÷ ¼Ò°ß
1. early renal change ´Â
primary Fanconi syndrome °ú À¯»ç
Ư¡ÀûÀÎ
"swan neck" lesion : atropy & shortening of the proxiomal tubule
just
beneath the glomerulus
2. birefringent cystine
cristals in interstitial tissue, but rare in tubular cells
3. renal failure °¡ ÁøÇàÇÔ¿¡ µû¶ó
kid. ´Â shrunken & contracted ,
with
glomerular sclerosis & interstitial fibrosis
4) ÀÓ»ó¼Ò°ß
1. AR rait
2. three clinical
patterns
a.
infantile or nephropathic form
i) 3 -12 mo°æ Fanconi syndrome À¸·Î ³ªÅ¸³²
ii) generalized aminoaciduria /s predominance of cystine
iii) GFR ÀÌ Á¡Â÷ °¨¼ÒÇÏ¿© CRF °¡
1st decade À̳»¿¡ ¹ß»ý
iv) severe growth failure, hypothyroidism
v) distinctive clinical features
;brond hair & fair complexion, owing to defect inb melanin synthesis
photophobia 2ndary to deposite
of cystine crystals on the cunjunctiva
b.
adolescent or intermediate form
i) mild renal involvement with onset in the 2nd decade & slow
progression
ii) growth failure is not a feature of this form
c.
adult type
i) benign, no renal ds.
ii) cystine crystals in the cornea, BM, & leukocytes
5) °Ë»ç¼Ò°ß
1. deposition of
cystine crystals ¿Ü¿¡´Â Fanconi syndrome °ú À¯»ç.
2. tubular proteinuria
°¡ nephropathic cystinosis ÀÇ
early phase Ư¡À̳ª
renal failure °¡ ÁøÇàÇÔ¿¡ µû¶ó glomerular proteinuria
°¡ ÀÕµû¶ó ³ªÅ¸³´Ù.
6) Áø´Ü
1. in the asymptomatic
NB infant from an affected family
:
cystine content of leukocytes or fibroblasts (Á¤»óÀÇ 80 - 100¹è)
2. later, granular
& circinate irreguralities in the peripheral pigmentation of the
retina
3. cystine crystals in
the BM, lymph nodes, conjunctivae, and rectal mucosa
4. slit lamp
examination»ó cornea¿¡ crystal
5. prenatal diagnosis´Â
amniotic fluid cells¿¡ increased cystine concentration
6. cystine depositionµµ
Fanconi syndromeµµ ³ªÅ¸³ªÁö ¾Ê´Â cystinuria¿Í È¥µ¿ÇÏ¿©
¼ ´Â ¾ÈµÇ¸ç, ÀÌ´Â
an inborn error of specific amino acid transportÀÌ´Ù.
7) Ä¡·á
1. Ãʱ⿡
tubular dysfunction¿¡ ´ëÇÑ symptomatic therapy´Â
primary Fanconi
syndrome³ö À¯»çÇÏ´Ù.
2. cysteamine(sulfhydryl
binder) : in vivo¿¡¼ intracellular cystineÀ» °¨¼Ò½Ã۸ç,
¸î¸î ¼Ò¾Æ¿¡¼´Â renal failure ÁøÇà¼Óµµ¸¦
¿ÏȽÃŲ´Ù.
3. hemodialysis and renal
transplantation for end-stage renal failure
4. renal transplantation ÈÄ¿¡´Â ȯ¾Æ´Â Àß Áö³»³ª
long-term survivors´Â
a. progressive
photophobia or retinopathy¸¦ °æÇèÇÑ´Ù. ÀÌ °æ¿ì
cystiamine eye
dropsÀÌ È¿°úÀÖÀ½
b. associated
with long-term extrarenal manifestations
: swallowing dysfunction, myopathy,
pancreatic endocrine and exocrine
insufficiency, and various CNS problems(Áï seizure, cerebral
atrophy)
483.4 Oculocerebrorenal Dystrophy (=Lowe Syndrome)
1)°³¿ä
1. rare disorder, X-linked
recessive trait
2. in addition to Fanconi
syndrome, organic aciduria, decreased production of
urinary ammonia, an occasionally heavy proteinuria
3. distinctive clinical
features
: congenital
cataract, glaucoma, and buphthalmos
4. ±×¿Ü Ư¡À¸·Î
a. severe hypotonia and
hyporeflexia in the lst year
b. severe and
often progressive mental retardation
c. rickets,
marked osteopenia, and pathologic fracture as a result of metabolic
acidosis and phosphate depletion
2)PATHOGENESIS
1. unknown pathogenesis, ±×·¯³ª ÃÖ±Ù
in vitro studies¿¡¼ collagen metabolismÀÇ
ÀÌ»óÀ» ÀǽÉÇÔ
2. pathologic studies´Â
splitting of the glomerular basement membrane, with
marked variation
in their thickness¸¦ º¸ÀÓ
3)ÀÓ»ó¼Ò°ß
1. early life : eye findings
and mental retardation
later : Fanconi
syndromeÀÌ ÀÓ»óÀûÀ¸·Î ºÐ¸íÇØÁü
2. childhood½Ã±â¸¦ »ì¾Æ³²À¸¸é
Fanconi syndromeÀº spontaneouslyȸº¹µÇ³ª,
CRF°¡
µÉ ¼ö ÀÖÀ½
4)Ä¡·á
1. no specific therapy
2. supportive, as in primary
Fanconi syndrome
483.5 Renal Osteodystrophy
1)°³¿ä
1. renal osteodystophyÀÇ Àǹ̴Â
chronic renal disease¸¦ °¡Áø ȯ¾Æ¿¡¼ mineral°ú
bone metabolismÀÇ ÀÌ»óÀ¸·Î ³ªÅ¸³ª´Â skeletal growth¿Í
remodelingÀÇ º¯ÈÀÌ´Ù.
2. these abnormalities include
a. malabsorption of
calcicum, phosphaste retention, hyperfuncion of the
parathyroid glands
b. cutaneous, vascular, and visceral
calcifications
c. impairment in the renal production of
biologiclally active vitamin D
3. GFRÀÌ Á¤»óÀÎ
tubular dysfunction½Ã¿¡µµ ³ªÅ¸³¯ ¼ö ÀÖÀ¸³ª, glomerular
insufficiency³ª
uremia¸¦ °¡Áø ȯ¾Æº¸´Ù´Â ÈçÇÏÁö ¾Ê´Ù.
4. ÀÌÀü¿¡´Â renal (uremic) rickets
ȤÀº renal dwarfismÀ̶ó ºÒ·È´Âµ¥,
ÀÌ´Â severe
linear growth failure°¡ rickets¿Í °°Àº
X-ray change¸¦ º¸¿´±â ¶§¹®
a. ÀÌ ¼Ò°ßÀº ÀÏÂ÷ÀûÀ¸·Î
vitamin D deficiency¿¡ ´ëÇÑ mineralization defect¿¡ ±âÀÎ ÇÑ´Ù°í ¸ÕÀú »ý°¢µÇ¾úÁö¸¸,
b. ÀÌÂ÷ÀûÀÎ
hyperparathyroidismµµ ÀÓ»óÀû, ¹æ»ç¼±ÇÐÀûÀ¸·Î
¶È°°ÀÌ Áß¿äÇÑ ¿ªÇÒÇÔ
5. pediatric dialysis, kidney
transplantationÀ» ½ÃÇàÇÔ¿¡ µû¶ó renal osteodystrophy°¡ CRFÀÇ ÁÖ¿äÇÕº´Áõ(acidosis,
anemia, calororic deficiency µî°ú ÇÔ²²)À¸·Î ¶°¿Ã¶ú´Ù.
2)PATHOGENESIS
1. normal dietary intake of
phosphorus¸¦ °¡Áø early in the course of chronic
renal
insufficiency(GFR 25 50§¢/min/1.73m2)¿¡´Â
a. renal tubular
synthesis of 1,25(OH)2D3°¡
phosphate-mediated suppressionµÇ¾î
a) malabsorption of calcium and
phsphorus
b) unknown mechanismÀÇ
defective mineralization of osteoid(osteomalacia)ÃÊ·¡
b.Áõ°¡µÈ
PTHÀÇ °á°ú, increased bone resorption°ú
increased renal phophate
excretionÀ» ÃÊ·¡Çϸç, serum calcuimÀº À¯ÁöµÇ¸ç
serum phophorus´Â ½ÇÁ¦ÀûÀ¸·Î
normal¿¡¼ lowÀÌ´Ù.
c. dietary phophate restriction˼
serum 1,25(OH)2D3
levelÀÇ Áõ°¡¿Í,
2ndary
hyperparathyroidismÀÌ improveµÊ
2. critical renal threshold(¿¹¸¦ µé¾î
GFRÀÌ Á¤»óÀÇ ¾à 25 30%)ÀÏ ¶§´Â
a. Áõ°¡µÈ
PTH¿¡ ´ëÇÑ phophaturic renal responseÀÇ ¼Ò½Ç
b.
compensatory hyperparathyroidismÀÌ serum calciumÀ» Á¤»óÀ¸·Î ȸº¹½Ã۱â
À§Çؼ ÀÕ´Þ¾Æ ÀϾÙ.
c.
roentgenographic and histologic evidende of exaggerated osteoclast
mediated
resorption of bone(osteitis fibrosa)
d.
endosteal fibrosis, increased bone turnover and replacement of regularly
textured
lamellar bone with disorganized and structurally deficient woven bone
3. chronic metabolic acidosisµµ ¾Æ¸¶µµ
bone¿¡¼ calcium resorption°ú
renal
excretionÀ» Áõ°¡½ÃÅ´À¸·Î½á bony change¸¦ ¾ß±â½ÃÅ´
3)Á¶Á÷¼Ò°ß
1. variable
2. trabecular bone˼
predominant osteomalacia, predominant osteitis fibrosa ȤÀº
most commonly, a mixed patternÀ» ³ªÅ¸³¿
3. ÀϺΠȯÀÚ¿¡¼´Â
fracturing osteomalacia¿Í low bone turnover´Â
mineralization
front¿¡ aluminumÀÇ ÃàÀûÀ» ÃÊ·¡Çϴµ¥,
ÀÌ´Â orally administered aluminum
binders ȤÀº aluminum-containing dialysis
solutions¿¡ ³ëÃâµÇ¾ú±â ¶§¹®ÀÌ´Ù.
4)¹æ»ç¼±ÇÐÀû ¼Ò°ß
1. abnormalities at
the epiphyseal growth plate
: °¡²û
nutritional ricketsÀ» ´àÀ¸³ª Á¾Á¾ ÇöÀúÈ÷ ±¸º°µÊ
2. growth plateÀÇ
longitudinal width°¡ ½ÇÁ¦·Î´Â Áõ°¡µÇ¾î ÀÖÁö ¾ÊÁö¸¸, ±×·¸°Ô º¸ÀÌ ´Â °ÍÀº
dysplastic trabeculasÀ» °¡Áø metaphyseal fibrosisÀÇ
bar Çü¼º ¶§¹®ÀÌ´Ù.
3. concomitant defect in
mineralizationÀº ´ÙÀ½À» ÃÊ·¡ÇÔ
a. a failure in
modeling, with persistence of cartilage
b. an expanded epiphyseal diameter
c. frequent overriding of the lateral
border of the metaphysis
5)ÀÓ»ó ¼Ò°ß
1. CRFÀÇ ¹ßº´½Ã±â°¡
younger child, ±×¸®°í renal failureÀÇ
longer duration˼
osteodystrophyÀÇ incidence¿Í
severityÀ» Å©°Ô ÇÑ´Ù.
2. congenital diseases of the
kidney¸¦ °¡Áø ¼Ò¾Æ(5¼¼ ÀÌÇÏ¿¡¼ ¿ì¼¼)´Â ¼Ò¾Æ ÈĹݱ⠿¡ ³ªÅ¸³ª´Â
glomerulonephritides º¸´Ù disease onset°ú
end-stage renal failure
»çÀÌÀÇ intervalÀÌ ´õ ±æ´Ù.
3 ±×·¯³ª,
congenital nephropathies ȯÀÚ´Â bone disease°¡ °¡¼ÓµÇ´Âµ¥
ÀÌ´Â maximal growth, bone modeling and
remodeling ½Ã±âÀ̱⠶§¹®ÀÌ´Ù.
4. the earliest sign of renal
osteodystrophy
: usually growth failure
±×¿Ü CRF¿¡ µ¿¹ÝµÈ
anemia, metabolic acidosis, protein-calorie malnutrition, hormonal disorders, and trace
mineral deficiencies
5. advancing diseaseÀÇ
additional clinical manifestations
: muscle weakness, bone pain, bone
deformities, slipped epiphyses, metaphyseal fractures, metastatic
calcification, and pruritus
6. genu varum, frontal
bossing ±×¸®°í dentgal abnormalities´Â
young children¿¡¼
ÇöÀúÇÔ
7. hypocalcemia¿¡µµ ºÒ±¸Çϰí
tetany´Â µå¹®µ¥, ÀÌ´Â
metabolic acidosis¿Í hyperparathyroidismÀÇ
combined protective effects ¶§¹®ÀÌ´Ù.
6)°Ë»ç½Ç ¼Ò°ß
1. mild hypocalcemia, but usually
elevated Ca P product by increased
levels of serum phosphorus
2. Áõ°¡µÈ ALP activity´Â Áõ°¡µÈ
bone turnover¸¦ ³ªÅ¸³»³ª, ¼Ò¾Æ¿¡¼´Â
¼ºÀθ¸Å reliasble signÀº ¾Æ´Ï´Ù.
3. sensitive early indicator of
osteitis fibrosa : hand¿Í wristÀÇ
X-ray¿¡¼ subperiosteal
erosions of the middle and distal phalanges, ±×¸®°í erosionsÀº
distal clavicle, inner aspects of the distal femur and proximal tibia¿¡µµ ³ªÅ¸³².
4. the earliest indication of bone
disease : elevated serum levels of PTH
GFRÀÌ
50 75§¢/min/1.73m2 À϶§µµ ³ªÅ¸³².
5. PTHÀÇ
degree of elevationÀº osteitis fibrosaÀÇ ¹æ»ç¼±ÇÐÀû,
Á¶Á÷ÇÐÀû ¼Ò°ß°ú ÀÏÄ¡ Çϳª,
histologic osteomalaciaÀÇ degree´Â
serum³» chemical abnormalities
ȤÀº coarsening of trabeculasÀÇ ¹æ»ç¼±ÇÐÀû
¼Ò°ß°ú ÀÏÄ¡ÇÏÁö ¾Ê´Â´Ù.
7)Ä¡·á
1. renal osteodystrophy can
usually be successfully managed by
a. controlling
hyperphosphatemia
b. supplying adquate oral calcium intake
c. providing extra vitamin D
2. hyperphosphatemiaÀÇ Ä¡·á
a. dietary phophate restriction : ½ÃÀÛÇÏ´Â
indication˼ raised PTH level
b. aluminum-containing phosphate bindersÀÇ
oral administration
a) risks of aluminum intoxication ¶§¹®¿¡ °¡´ÉÇϸé ÇÇÇØ¾ß Çϸç,
dietary
restrictionÀÌ ÃæºÐÇÏÁö ¾ÊÀ¸¸é »ç¿ëÇÔ
b) aluminum hydoxide or
aluminum carbonate gel
: a
starting dose of 20 30mg/kg/24hr in
divided doses with meals
ÀÌÈÄ ¿ë·®À» Á¶ÀýÇÏ¿© serum phosphorus¸¦
4 5mg/dl·Î À¯Áö½ÃÅ´
c) long-term treatment with
high-dose aluminum-containing bindersÀÇ ÇÕº´Áõ
:
osteomalacic osteodystrophy and a progressive, irreversible
encephalopathy
3. calcium supplementation
a. calcium carbonate ÇüÅ·ΠÇÏ·ç¿¡
1 1.5g of elemental calciumÀ»
diet¿¡ ÷°¡
b. calcium carbonateÀÇ ÀåÁ¡
a) the highest percentage of
elemental calcium
b) some degree of phosphate
binding, particularly when given with meals
4. acidosis control˼
sodium bicarbonate¸¦ »ç¿ë
: usually 1 2mEq/kg/24hr, divided into thirds and
given 1hr after meals
5. providing extra vitamin D
a. 1,25(OH)2D3
a) preferred form
b) indication˼
symptomatic bone disease with hypocalcemia
secondary hypoparathyroidism
evidence of osteitis fibrosa on
X-ray or bone biopsy
c) starting dose´Â
15 40ng/kg/24hrÀ̸ç,
À̸¦ hypercalcemia risk¸¦ ¹æÁöÇϱâ
À§Çؼ 8 12½Ã°£ °£°ÝÀ¸·Î Åõ¿©Çϰí, ¿ë·®ÀÇ
stepwise adjustment¿Í
biochemical monitoringÀÌ ÇÊ¿äÇÔ
b. dihydrotachysterol(DHT)
a) ÀåÁ¡
: better ratio of therapeutic to toxic effects
shorter
half-life´Â hypercalcemiaÀÇ ÇÕº´ÁõÀ» °¨¼Ò½ÃÅ´
b) starting dose´Â
0.1 0.2mg/24hrÀ̸ç,
serum calciumÀ» Á¤»óȽÃ۰í, X-ray
»ó bone abnormalities¸¦
healing½Ã۵µ·Ï ¿ë·®À» weekly or biweekly·Î Á¶Àý
ÇÑ´Ù.
c. frequent measurements of serum
calcium and phosphorus
d. CRF¿¡¼´Â serum calciumÀÇ
"set point"°¡ Áõ°¡ÇϹǷÎ, vitamin D therapyÀÇ
therapeutic
goalÀº serum calciumÀ»
10.5 11.0mg/dl±îÁö ¿Ã¸®´Â °ÍÀÌ´Ù.
6. hemodialysis or chronic
peritoneal dialysis
7. autotransplantation of the
parathyroid gland
a. medical therapy¿¡ ¹ÝÀÀÀÌ ¾ø´Â
severe secondary hyperparathyroidism ȯÀÚ¿¡
¼ ½ÃÇà
b. indication : severe bone pain,
mental aberrations, severe pruritus, fractures, chronic hypercalcemia, and metastatic
calcification
Chapter 484.Nephrogenic Diabetes Inspidus
Etiology
#
primary NDI
; *rare, inherited ds, X-linked recessive
ds
; ³²
: complete unresponsive to ADH
; ¿©
: partial
#
2ndary NDI
; medullary concentrating
gradient lossÃÊ·¡ Áúȯ
a)acute or
chronic renal failure
b)obstructive
& p ostobstructive uropathy
c)vesicoureteral reflux
d)cystec
ds. : interstitial nephritis
e)osmotic
diuresis, nephrocalcinosis
;
tubule¿¡ ADH effect °¨¼ÒµÈ °æ¿ì
a)hypokalemia b)hypercalcemia c) lithium d) democlocycline therapy
Pathogenesis
cf.concentration of urine
(1)hypertonic renal
medulla<countercurrent mech.
(2)permeability of distal tubule
& collecting duct to water <ADH
Clinical Manifestation
#
male with primary NDI
; polyuria, polydipsia in
infancy
; hypernatremic dehydration
episode
#
female in primary NDI
; milder
; not detected until later
life
Diagnosis
#
primary NDI
;
clinical Hx.
;
male with family Hx
#
Laboratory Finding
;
hypernatremia, diluted urine
; ¡Ús-osmolality
> 295mosm/kg H2O & concurrent urine osmolality < s-osmolality
-
diagnosis
-
*dehydration test ºÒÇÊ¿ä
#
confirm diagnosis
; aqueous vasopressin
0.1-0.2u/kg im ÈÄ 4h °£°ÝÀ¸·Î serum & urine
osmolarity ÃøÁ¤.
-> urine / plasma
osmolality
ratio < 1.0 ; nephrogenic D.I
ratio > 1.0 ; central D.I
psychogenic polydipsia
#
s-osmol < 295 mOsm / Kg ÀÌÇÏÀ̸é fast½ÃÄÑ
295ÀÌ»óµÇ¸é dehydration test
cf.
B.W 3%ÀÌ»ó loss ->fluid Åõ¿©
#
biochemical & radiographic study½Ç½ÃÇÏ¿© ÀÌÂ÷Àû ¿øÀÎ ¹èÁ¦
Complication
(1) mental retardation by repeated
hypertonic dehydration
(2) growth retardation
male¿¡ ³ªÅ¸³²
; intrinsic
(3) collecting system
dilatation->¼ö³â¸¶´Ù renal scan À¸·Î
hydronephrosis°üÂû
Treatment
#
¸ñÇ¥
; provision of adequate fluid
& caloric intake
; reduction of urinary solute
load
#
diuretics therapy
; sodium depletion
-->
enhancement of Na & water reabsorption in proximal tubules
-->
paradoxical response
; *chlorthiazide(20-40mg/kg/day in divided dose) with moderate salt
restriction
-
significant reduce need for fluid intake & frequency of voiding
-
*hypokalemia monitoring
; *no effect on 2ndary NDI
#
inhibitors of PG synthesis
; indomethacin
; *diuretic therapy ½ÇÆÐ½Ã ½Ãµµ
Prognosis
pri.NDI: life long.
good Px. if hypernatremic dehy. is avoided genetic counselling
2nd. : underlying ds. nature¿¡ µû¸§
Chapter 485. Bartter Syndrome
Ư¡: hypokalemia
normal BP
vascular insensitivity to pressure agents
elevated plasma renin & aldosterone
AR·Î inherited°æ¿ìµµ ÀÖÀ½
Pathology
IG apparatus hyperplasia
< renin »ý»ê Àå¼Ò
renal parenchyme
´ë´Ù¼ö´Â Á¤»ó
¼Ò¼ö¿¡¼
nonspecific glo. ds or interstitial ds. or both
Pathogenesis
;
*primary defect in chloride
reabsorption in ascending limb of loop of Henle
--> reduced medullary hypertonicity
(concentrating defect)
--> extra NaCl in distal
tubule
--> reabsorption of sodium
exchange for potassium
--> urinary potassium
wasting
--> hypokalemia
--> stimulating
prostaglandins systhesis
--> vascular insensitivity
to pressure agents
--> activation of
Renin-Angiotensin-Aldosterone
--> aggregation of renal
potassium wasting
Clinical Manifestation
;
young children
- *growth failure, muscle weakness, constipation, polyuria,
dehydration
;
older children
- *muscle weakness or cramps, carpopedal spasm
Diagnosis
a)hypokalemia(<2.5mEq/L)
b)normal blood pressure
c)defective platelet aggregation
d)hypochloremia
e)metabolic alkalosis
f)plasma renin, aldosterone, PGE2 Áõ°¡
g)urinary K+, Cl Áõ°¡
¶§·Î
hypercalciuria, hyperuricemia, hypomagnesemia,urinary sodium wasting
confirm by histologic
demonstration of hyperplasia of JG cell
#
DDx
; licorice abuse
; laxative or diuretic use
;
persistent vomiting or diarrhea
; Pyelonephritis
; D.I
#
DDx point
; laxative use,vomiting
diarrhea, D.I½Ã hypovolemiaµ¿¹Ý
-->
low urinary Cl level (cf. Bartter SD½Ã Áõ°¡)
Treatment
5)The goal of Tx; to maintain s-potasium
level>3.5meq/l
and adequate nutriton
a)potassium chloride(K-Lyte/
cl;25-50mEq of KCl);S-K35mEq/lÀÌ»ó
250mg/24/hr½Ã±îÁö Áõ·®
b)sodium chloride
supplementation
c)-fail-->triamterene
5-10mg/kg/day in div.
d)-fail-->indomethacine
3-5mg/kg/day Sig1/3
Prognosis
;
uncertain
;
*¸¹Àº ȯÀÚ´Â remain well
;
ÀϺο¡¼ renal insuff.
;
growh failure À־ normal stature
Chapter 486. Interstitial Nephritis
´ë°³ tubular damage µ¿¹Ý
glome.change´Â
minimal
- Common cause of interstitial
nephritis
1.Acute interstitial nephritis
1)pathology
interstitial infiltrate
a)lymphocytes
b)plasma cells c)eosinophils ¶§·Î d)neutrophils
edema: tubular degeneration & necrosis
2)pathogenesis
unknown
a)hypersensitivity reaction; drug
b)tubular
BM¿¡ immune complexÀÇ
deposition; Lupus.MPGN
c)anti BM Ab ; Goodpasture,
membranous
d)cell
mediated mechanism ; sarcoidosis, transplant rejection
3)C/M
a)drug°¡
hospitalized pt.½Ã m/c cause
1ÁÖÀÌ»ó
drug»ç¿ë½Ã i)fever & rash¹ß°ß
ii)eosinophil Áõ°¡ in blood urine or both
b)others; ARF or
acute GN¿Í À¯»çÇÑ clinical picture
c)onset˼
ant. uveitis¿¡ ÀÇÇØ ¼±ÇàµÉ¼ö ÀÖ´Ù.
4)Dx.
renal biopsy·Î
confirm
¼Òº¯³» È£»ê±¸ Áõ°¡
5)Tx. & Px
a)ARF Tx.
---+
b)inciting agent
withhold
| ·Î
complete resolve
c)precipitating inf.
Tx.
---+
d)severe histologic
injury & renal failure½Ã
--->high dose corticosteroid·Î dramatic improve.
2.Chronic interstitial nephritis
1)patholoty
a)infiltrates;
lymphocytes plasma cells
b)tnterstitial
fibrosis
c)tubular dilatation
& atrophy
d)partial or total
glom. sclerosis
2)clinical manifestation
´ë°³
occult structural abnormality of Kidney & Urinary tract µ¿¹Ý
(cystic
ds. obstruction, reflux)
ÃÊÁõ»ó;a)
CRF
i)N &V ii)pallor iii)headache iv)fatigue v)HT vi)growth failure
b)underlying ds ÀÇ Áõ»ó: (UTI, flank mass)
3)Dx.; renal biopsy not indicated
´ë°³
underlying ds¿Í ¿¬°üµÈ chronic renal
insufficiencyÀÇ ¾ç»óÀ¸·Î Áø´Ü
4)Cx. & Px
progression to end-stage RF
avoidance of phenacetin(analgesics)
& lithium -> adult¿¡¼
renal Fc improvement ÀÖÀ½
section 5. toxic nephropathy - renal failure
Chapter 487. Toxic Nephropathy
. medication
diagnostic agents(iodinated radiographic
contrast media)
chemicals
. directly damage
renal bl.flow °¨¼Ò
, ATN , intratubular obs.
indirectly
allergic or hypersensitivity
in vessels & interstitium
** Preventive measures may reduce the
risk of nephrotoxicity
a)pre-existing renal
ds½Ã c.m´ë½Å US or scan
b)non-nephrotoxic
agent·Î ġȯ
c)lowest effective
dose of agent with bl. level monitoring
d)renal insuff. ½Ã
dose reduction
e)µ¿½Ã¿¡
several nephrotoxic agentÇÔ²² »ç¿ë±ÝÁö
table 487-1
Chapter 488.Cortical Necrosis
a) renal cortical(frequently
medullary)necrosis´Â several typeÀÇ
renal injuryÀÇ
final common result
b) ´ë°³ both kidney involve
c) pathy or entire cortex involve
1)
Etiology
NB¿¡¼ÀÇ ¿øÀÎ:
a)dehydration
b)asphyxia
c)shock
d)DIC
e)renal vein thrombosis
NBÈÄ: Hemolytic uremic
syndromeÀÌ m/c cause
2)
pathology
cortical infarction,
congestion of glo.
thrombosis of arterioles,
tubular necrosis
3)
pathogenesis
toxin -> endoth. cell injury(endotoxin)
intrarenal coagulation ->thrombosis, cortical necrosis
diminished renal cortical
blood flow --> endothelial cell injury
4)
clinical manifestation
a) ARF, b) enlarged Kid., c) U/O °¨¼Ò, d) gross hematuria
5)
Dx.
US»ó enlarged but non -
obstructed kidney
scan»ó °¨¼Ò or no renal bl. flow
& no function
6)
Tx. & Px
supportive care
--> correction of
dehydration, asphyxia shock sepsis Tx.
3.UTI
in newborn
chapter. 101 & 492
Chapter 489. Renal Failure
489.1 Acute Renal Failure
#
oliguria
; daily U.O <400mL/m2
; < 0.5ml/kg/hr in infancy
Etiology
¡ÚTable
489-1
¡ÚTable
489-2
Pathogenesis
#
prerenal cause
; decreased renal
perfusion->GFR°¨¼Ò
; ÀÏÁ¤½Ã°£³»
hypoperfusion ±³Á¤½Ã¿¡´Â reversible
#
renal causes
;
hemolytic uremic syndrome
-
¡ãcommon cause in toddlers
; ATN
-
no arterial or glomerular lesion
-
¡ÚProposed Mechanisms
/
alterations in intrarenal hemodyanmics
/
tubular obstruction
/
passive backflow of glomerular filtrate across injured tubular cells into
peritubular capillary
; GN
#
postrenal causes
; upper collecting systemÀÇ
dilatationÀº acute ureteral obstruction¼öÀÏ ÈÄ¿¡¾ß ÃÊ·¡
Clinical Manifestation
.precipitating ds.µû¶ó
variable
.Áõ»ó & sign related to
RF
a) pallor(anemia)
b) U.O °¨¼Ò
c) edema(salt &
water overload)
d) hypertension
,vomiting, lethargy(uremic encephalopathy)
Diagnosis
#
careful Hx.
cf. flank mass½Ã »ý°¢
a)renal vein
thrombosis, b) tumor, c) cystic ds., d) obstruction
#
Laboratory Finding
a) anemia
+-dilutional
+-hemolytic ; lupus,
renal v. thrombosis, HUS
b) leukopenia ; lupus
c) thrombocytopenia ; lupus, renal
v. thrombosis, HUS
d) hypoNa+ ; dilutional
e) hyperK+
f) acidosis
g) BUN,Cr,uric acid, phosphate Áõ°¡;
diminished RF
h) hypocalcemia(hyperphosphatemia)
i) C3 °¨¼Ò
: i) PSGN ii) lupus iii) MPGN
j) Ab; antistreptococcal ; PSGN
nuclear ; lupus
neutrophilic cytoplasmic antigens(ANCA;Wegener
granulomatosis,microscopic
polyarteritis)
BM antigen ; Goodpasture ds.
k) chest X-ray ; cardiomegaly, pul
congestion -> fluid overload
l) ¸ðµç ARF pt. ½Ã
obstruction°¡´É¼º ¹ß°ßÀ§ÇØ
i) plain abd. X-ray
ii) U/S ; CRF , obst. uropathy
DDx
iii) renal scan é©
iv) RGP ; ¶§·Î
<-- obst½Ã percut. nephrostomy½ÃÇàÀ¸·Î
reverse ʦ
Treatment
1) children /c hypovolemia
; volemic replacement
|
Uosm |
UNa |
Fr.Na excret. |
hypovolemia impending ATN |
>500mOsm/kgH2O <350 |
<20mEq/L
>40mEq/L |
<1%
>1% |
UNa/PNa
Fractional Na excretion = --------- * 100
Ucr/Pcr
.Ä¡·á : isotonic saline iv
20mL/kg over 30 min
´ë°³
2hr. ³» voiding
bladder
cath. CVP ÇÊ¿ä ʦ
hydration ÈÄ diureticsʦ
2) Impending RF
diuretics on prevention of anuria ;
controversy
established anuria¿¡´Â
no value
furosemide -- urine production ¡è by altering tub. func
mannitol --
±×·¯³ª urine ¡è ÀÌ renal func. È£ÀüÀ̳ª natural ds. È£Àü
Àǹ̾øÀ¸³ª, a) hyperkalemia, b) fluid overload ±³Á¤¿¡´Â È¿°ú.
< furosemide >
: hypovolemia¾øÀ» °æ¿ì
a) initial single dose
of 2mg/kg(rate 4mg/min to avoid ototoxicity)
b) 10mg/kg of
furosemide, if no response
b)¿¡µµ
response¾øÀ¸¸é CIx.
c) single iv of
0.5g/kg of mannitol over 30min in addition or in place
of
furosimide
;È¿°ú ÀÖµç ¾øµç ´õÀÌ»ó »ç¿ë¸øÇÔ
3) Fluid restriction
volume expansionÀ̳ª
diuretics¿¡ È¿°ú¾ø´Â °æ¿ì´Â essential
a) 400mL/m2/24h(insensible loss) +
urine ¾ç ; Á¤»ó intravascular volume½Ã
b) 10-30% glucose containing
solution without electrolytes
c) blood. GI loss´Â ±³Á¤
4) hyperkalemia
serum level 6mEq/L½Ã
cardiac arrythmia, cardiac arrest ʦ
a) adequate
renal f. µÇ±âÀü¿¡´Â K+ restriction
b) EKG changes
i)
tall, peaked T waves ; earliest
ii)ST seg. depression
iii)
P-R prolongation
iv)
QRS widening
v)
cardiac arrest
c) s-K+ ÀÌ
5.5mEq/L·Î ¿À¸£¸é Tx½ÃÀÛ
i) ¸ðµç
solution¿¡ high conc. of glucoseÆ÷ÇÔ
ii)
Kayexalate(sodium polystyrene sulfonate resin) exchange Na for K
1g/kg p.o or retention enema
20% 10ml/kg sorbitol ; enema½Ã
orally, suspended in 2mL/kg of 70% of sorbitol½Ã
best results
(¡ñ sorbitolÀº
osmotic diarrheaÀÏÀ¸Å´) every 2hr. À¸·Î
repeat
d) s-K+ÀÌ
7mEq/LÀÌ»ó ¿À¸£¸é
-->sequentially following emergency step½ÃÀÛ
i) 10% calcium
gluconate ; 0.5 mL/kg iv over 10min
H.R
monitoringÇϸç 20beat/minÀÌ»ó
H.R¡èµÇ¸é ½¬¾ú´Ù°¡ °è¼Ó
ii) 7.5 %
NaHCO3 3mEq/kg iv
cx. ; vol. expansion, hypertension, tetany
iii) 50% glucose
1mL/kg with R.I 1u/5g of glucose iv over 1hr.
hypoglycemia monitor é©
iv)kayexalate
¡¤i)Àº
K+¡é½ÃŰÁö´Â ¾ÊÀ¸³ª (K+ induced myocardial
irritability¿¡ counteract
effect´Â ÀÖÀ½)
¡¤ii)Àº
K+ shifting into intracellular compartment from ECF ÀÌ
measurementÀÇ duration of actionÀº ´ÜÁö ¼ö½Ã°£ Áö¼ÓµÇ¹Ç·Î
persistent hyperkalemia½Ã dialysis é©
5) acidosis
a)inadequate excretion of H+ &
ammonia´ë°³ Ä¡·á é©
b)Ä¡·á ÇÊ¿äÇÑ °æ¿ì
severe acidosis -- arterial pH < 7.15
s-HCO3 < 8mEq/L
--> ÀÌ ¶§´Â
resp. drive ¡é , myocardial irritability ¡è
c) partial correction by iv route
¸ñÇ¥
pH=7.20, HCO3=12mEq/L
¡ÅmEq NaHCO3 required = 0.3 ¡¿
BW ¡¿ (12-sHCO3)
³ª¸ÓÁö´Â
s-Ca. phosphatemia /c reciprocal ,
hypocalcemia
acidosis¶§
total calcium ionized fraction ¡è·Î tetany¹ß»ýÀÌ ¹æÁöµÇ¾ú´Ù°¡
acidosis rapid
correctionÀ¸·Î ionized Ca ¡é
->tetany
Hypocalcemia
; Tx by lowering serum
phosphorus
; *no calcium iv if not tetany
-
*calcium(mg/dl)xphosphorus(mg/dl)ÀÌ 70ÀÌ»óÀ̸é calcium salt
deposition in tissue
; ¡Úphosphate-binding
calcium carbonate antacids
-
Titralac Liquid (3M Company, St. Paul, MN)
/
starting dose 5-15ml with meal and before bed
-
Os-Cal 500 tablets (Marion Laboratories, Kansas City, MO)
/
1-3 tablets with meals and before bed
-
regular strength TUMS (SmithKline Beecham, Pittsburgh, PA)
/
1-3 tablets with meals and before bed
-
Á¤»óÀÌ µÉ¶§±îÁö Á¡Â÷ Áõ·®ÇÑ´Ù.
Hyponatremia
;due to excessive amount of
hypotonic sol.
a) Tx. by fluid
restriction
b) s-Na ÀÌ
120ÀÌÇÏÀ̸é cbr. edema & Hemorrhage ʦ
mEq
Nacl required = 0.6 ¡¿ B.W ¡¿
(125 - sNa)
c) risk of
hypertonic sodium
i)vol. expansion
ii)HT
iii)CHF -->¹ß»ýÇϸé
peritoneal dialysis ½Ç½Ã
8) Hypertension
1¡£
ds. process
ECF vol expansion µî¿¡ ÀÇÇØ »ý±è
a) salt & water
restriction ; critical
b) severe acute
symptomatic HT
i)
diazoxide ; DOC
1-3mg/kg(max 150mg) rapidly iv (10ÃÊ À̳») --> 10-20ºÐ³»
BP ¡é
¸¸ÀÏ È¿°ú ºÒÃæºÐ½Ã 30ºÐÈÄ
2nd inject or nifedipine 0.25-0.5mg/kg p.o
HT crisis½Ã sodium nitroprusside, labetalol continuous iv infusion
ii)
furosemide concomitantly
c) less severe hypertension
i) ECF vol.
expansion ¹æÁö
+->
salt & water restriction
+-> furosemide
ii) beta
blocker(propranolol) ; 1-3 mg/kg/12hr p.o
iii)
vasodilator(apresoline) ; 0.5-1.5 mg/kg/6hr iv
9)Seizure
a)¿øÀÎ ; i)primary ds process
; SLE
ii)hyponaremia ; water intoxication
iii)hypocalcemia ; tetany
iv)HT
v)uremic state itself
vi)hypoglycemia
b)Tx.
i)underlying
cause
ii)diazepam; most
effective but metabolite °¡ renal insuff. ½Ã ÃàÀû
10)Anemia
a)´ë°³
hemodilution; Hb 9-10 g/dl
--> no transfusion
b) blood loss by active
bleedingÀº º¸Ãæ é©
c) hemolytic anemia(SLE,HUS
)
--
prolong RF ½Ã
Hb <
7g/dl½Ã transfusion
vol.
overload, CHF , pul, edema , HT À§Çè ÀÖÀ¸¹Ç·Î 4-6hr µ¿¾È
transfusion
/c fresh packed
RBCÀÇ slow transfusion 10ml/kg
11)
diet ; 400cal/m2/day ¡è
a) healthy &
well-nourished À̰í ARF°¡ ªÀ» °æ¿ì´Â
diet´Â
restricted to fat & carbohydrate¸¸ ÁØ´Ù(gum drops or jelly
beans)
b) Na,K ,water restriction
c) 3ÀÏ ÀÌ»ó Áö¼Ó½Ã
expanded oral diet or TPN /c a.a
12) dialysis
Ix ; a) acidosis
b)
electrolyte abn.
c) CNS
disturbance
d) HT
e) fluid
overload
f) CHF
14) continuous hemofiltraton
is useful - a)acute renal failure
(ƯÈ÷, i. wnstable cardiopulmonary
dynamics
ii.severe coagulopathies
iii.unavailablity of the peritoneal cavity due to
surgery or trauma
b)fluid overload
c)severe electrolyte or acid-base disturbances
¡Ø
performed by two basic configurations
a) continuous arteriovenous hemofiltration (CAVH)
b) continuous venovenous hemofiltration (CVVH)
15) life threatening Cx. of
uremia
a)hermorrhage b)pericarditis c)CNS dysfuction
risk
of Cx.Àº Cr.º¸´Ù BUNÄ¡¿Í ´õ °ü·Ã
Prognosis
underlying causeµû¶ó
1)normalized ʦ
a) prerenal causes
b) hemolytic uremic syndrome
c) acute tubular necrosis
d) acute interstitial nephritis
e)uric acid nephropathy
2) recovery of renal
function ÀÌ unusualÇÑ °æ¿ì
a)RPGN, b)bilat. renal v. thrombosis, c) bilat. cortical necrosis
¡Ø
ARF & CRF ÀÇ dialysis Ix.
a) BUN >100 mg/dl
b) uncontrollable hyperkalemia
c)
intractable severe met. acidosis or acidemia which cannot safely
corrected by NaHCO3
d) fluid overload /c circulatory congestion & pul. edema
489.2 Chronic Renal Failure
¡ÚEtiology
#
under the age of 5yr
; *anatomic abnormalities
-
hypoplaia, dysplasia, obstruction, malformation
#
after 5yr of age
;
acquired glomerular ds.
;
hereditary disorders - Alport synd. cystic ds
Pathogenesis
¾î¶² Á¾·ùÀÇ
Kid. damageµç renal functional deteriorationÀÇ
critical levelµµ´Þ½Ã
end-stage RF ·Î progressive renal function
deterioration °ü·Ã factors
a)ongoing
immunologic injury
b)hemodynamically mediated hyperfiltration in surviving glomeruli
c)dietary
protein & phosphorus intake
d)persistent
proteinuria -- Á÷Á¢
glomerular cap.wall damage
e)systemic
hypertension -->glomerular sclerosis -->HE filtrate injury
#
*20% of GFRµÇ¾î¾ß uremia ³ªÅ¸³´Ù.
¡ÚTable
489-4
Clinical Manifestation
anatomic abnnormality½Ã
non specific presenting complaints
; headache , fatigue ,lethargy, anorexia, vomiting
polydipsia, ployuria, growth failure
P/E
¡¤unrewarding
¡¤pale
& weak. BP ¡è
¡¤anatomic
abn. ·Î ÀÎÇØ ¼ö³â¿¡ °Éó renal failure °¡
slowly develop ÇÒ¼öÀÖÀ¸¸ç
À̶§
growth retardation & rickets °¡ µ¿¹ÝµÉ¼ö ÀÖ´Ù.
Treatment
management of pt. with CRF
a) clinical status
P/E,
blood pr. close monitoring
b) Lab. status
i) routine
exam. /q 1mo
.Hb(anemia)
.electrolyte -- hyponatremia
hyperkalemia
acidosis
.BUN,Cr -- nitrogen
accumulation
level of renal function
.calcium¡é, phosphorus¡è
.ALP ¡è --hypocalcemia
hypophosphatemia
osteodystrophy
ii)
periodic exam /q 6mo.
.PTH
--
osteodystrophy early evidence
.bone X-ray -- detect ʦ
iii) ÀÌ¿Ü
.chest X-ray -- assess cardiac function
.echocardiogram --
iv) nutritional
status
a)s-albumin, b) zinc, c) trasfusion, d) folic acid, e) iron level
Diet In CRF
a) GFR <50% ½Ã
growth rate ¡é
b) ÁÖ ¿øÀÎÀÌ
inadequate caloric intakeÀ̹ǷΠ-->unrestricted
amount of
+-> Carbohydrate (sugar, jam, honey, glucose polymer)
+-> fat(medium chain TG)
--> intermittent or overnight N-G or gastrostomy tube feeding
recumbiniant human GH therapy /c optimal days
--> linear growth Á¶Àý
c) BUN = 80mg/dl¡è½Ã
dietary protein restriction
; 1.5 g/kg/24h·Î proteinÀÌ
high biologic value(egg>milk>meat>fish>fowl)
d) cow's milk¿¡
phosphate ¡è¹Ç·Î
i)
moderate restriction
ii) use of
a formular containing a reduced amount of
phosphate
( Similac PM 60/40, Loss laboratories, columbus, OH)
¶§¶§·Î oral phosphate binder ¿Í
conjunction µÈ´Ù.
e) water soluble Vit.
deficiency (owing to inadequate intake or dialysis loss)
; Nephrocap(Fleming, fenton, MO) --> routinely supplyÇØ¾ßÇÔ.
f) Zinc & iron
--> deficient È®ÀÎÈÄ °ø±Þ
g) fat soluble vit. A,
E, K supplementation ; not required
Water & Electrolyte Management
a) water;
dialysisÇÊ¿äÇÑ Á¤µµ ¾Æ´Ï¸é no restriction
b) Na+; edema,
hypertension, CHF µîÀÌ ÀÖÀ¸¸é salt restriciton /c
furosemide
(1-4mg/kg/day)
; salt wasting form; Na supplementation
c) K+
Acidosis In CRF
s-HCO3°¡
20ÀÌÇÏÀ̸é Ä¡·á; ¼ºÀå¿¡ µµ¿ò.
Bicitra
NaHCO3 tablet
Renal Osteodystrophy
; develops in asso. with
hyperphosphatemia, hypocalcemia, s-ALP ¡è, PTH ¡è
b) hyperphosphatemia
i) GFRÀÌ
moramalÀÇ 30%¡é ½Ã phosphorus level ¡è
ii) reciprocal
solubility relationshipÀ¸·Î hypocalcemia ->2ndary
hyperparathyroidism(+)
iii) Tx.
¡¤aluminum
hydroxide
cf. aluminum poisoning
; dementia osteomalacia->rarely
¡¤ calcium carbonate suspension
phosphate binder
low phosphate formula(Similac PM 60/40)
enhancing fecal excretion by orl calcium carbonate
c)hypocalcemia
i)±âÀü
. reciprocally by hyperphosphatemia
. inadequate dietary intake
. decreased intestinal Ca absorption caused by a deficiency
in the active form of Vit. D
ii)Tx.
. hyperphospatemia±³Á¤
. ¾ÈµÇ¸é oral calcium supplement(Neo
Calgulucon Syrup, Os-cal
tablets);500-2000mg/day
d) Vit D.
severe kidney dysfuncton½Ã 1-hydroxylation¾ÈµÇ¾î
Vit.D def.
i) Ix. for Vit. D therapy
. s-phosphorus < 6.0mg/dl ÀÌÇÏ·Î ±³Á¤Çϰí calcium
supplementationÇÏ
¿©µµ persistent hypocalcemia
. elevated s-ALP¿Í X-ray ß¾
ricketÀǽɵǴ osteodystrophy pt.¿¡
ii)
Tx.
½ÃÀÛ . 1 capsule(0.25ug)/d of
active dihydroxy Vit D
. 0.05-0.20mg/day of dihydrotachysterol solution
¡¤normal
s-ca. ALP, X-rayß¾ healing½Ã±îÁö
dose ¡èÈÄ initial level
·Î Á¡Â÷ °¨¼Ò.
¡¤subperiosteal
erosion of edge distal phalanges of index & middle finger
; hyperparathyroidism
Anemia In CRF
; ´ë°³ Hb 6-9g/dl
¡¤transfusion;
not indicated
¡¤suppression
of erythropoietin production
¡¤
Hb < 6 ½Ã 10mL/kg of packed RBC ,
¡¤
recombinant erythropoietin therapy
Hypertension In CRF
a) Diazoxide 1-3mg/kg iv or
sublingual nifedipine ; emergency½Ã 0.025-0.5 mg/kg
b) furosemide 2-4mg/kg iv
(rate; 4mg/min);circ. overload µ¿¹ÝµÈ severe HT½Ã
c) sodium nitroprusside´Â
renal insuff.½Ã ÁÖÀÇ toxic thiocyanateÃàÀû
d) Tx. of sustainde
hypertension½Ã
i)
salt restriction(2-3g/day)
ii)
furosemide (1-4mg/kg/day)
iii)
propranolol(1-4mg/kg/day)
iv)
hydralazine(1-5mg/kg/day)
v)
combination
e) d)·Î inadequately control½Ã
minoxidil, captopril»ç¿ë
Drug Dosage In CRF
RF½Ã
dosage modificationÇÊ¿ä¾ø´Â drug
antibiotics; a)
CM, b) clindamycin, c) cloxacillin, d) EM, e) rifampin
489.3 End-Stage Renal Failure
1) successful kidney transplant
; ultimate goal
-->living-ralated donor½Ã creatinineÀÌ
5-6mg/dl½Ã
HLA typingºÎÅÍ ½ÃÀÛ
2) Dialysis
creatinineÀÌ
10mg/dl¿¡ µµ´Þ½Ã
pt's clinical status
Lab/F
availability of kidney donor
µû¶ó ½ÃÇà
a) hemodialysis
b) CAPD(continuous
ambulatory peritoneal D)
; standard
technique for majority of children requiring chr. dialysis
not as effficient as hemodialysis
table 489-6
Chapter 490. Renal Transplantation
#
*Optimal Tx For Children With ESRD
; living related donor renal
transplantation (LRD-RT)
¢¾Table
490-1 Criteria For Performing Living Related Donor Or Cadaveric Renal
Transplantaion In Pediatric Patients
#
ESRDÀÇ ¿øÀÎ
; ³ªÀÌ¿¡ µû¶ó ´Ù¸£´Ù.
-
*13-17¼¼ : glomerulopathy
- 5¼¼¹Ì¸¸ : congenital & obstructive
process
1) *congenital renal disease(53%)
2)
glomerulonephritides(20%)
3)
focal segmental glomerular sclerosis(12%)
4)
metabolic disease(10%)
5)
mescellaneous(5%)
¡ÚTable
490-2 Most Frequent Hereditary Metabolic Disease Of Childhood That Lead To
End-Stage Renal Disease
Table 490-3
Treatment
1. RT procedure
1) 15¡20Kg ¹Ì¸¸
: midline incisionÈÄ transperitoneal graft
2) 20Kg ÀÌ»ó : right iliac fossa¿¡
retroperitoneal location
¡æ renal vessel : external iliac
vessel°ú anastomosis
ureter¸¦ bladder¿¡
reimplantation(ureteroneocystostomy)
lower pole : intestine»çÀÌ¿¡ freeÇÏ°Ô µÒ
2. TABLE 490-4
3. survival rate
|
LRD-RT |
CAD-RT |
1³â |
¾à 90% |
72% |
3³â |
80% |
65% |
4. TABEL490-5
Histocompatibility
1. major histocompatibility complex(MHC)
gene
1) locus : chromosome 6 ÀÇ
short arm
2) ±â´É : human leukocyte
antigen(HLA)¸¦ encode
3) À¯Àü : Mendelian fashion
inheritance
Figure 490-3
4)
±¸¼º
¨ç class I protein(tissue
transplantation antigen) : HLA-A,-B,-C
cell-mediated
immunotoxicity
¨è class II protein :
immune responce À¯µµ
HLA-DP,-DQ,-DR
¨é class III protein :
tumor necrosis factor(TNF)
complement component (C2 & C4)
5) HLA-A,-B &-DR : clinical
transplantation¿¡¼ °¡Àå Áß¿ä
: good HLA match¡æ
graft loss °¨¼Ò
6) class I & II antigenÀÇ
cellular expression
¨ç B & T lymphocyte
& macrophage¿¡ ±¹ÇÑ
¨è inflammation¿¡¼
T cell¿¡ ÀÇÇØ ºÐºñµÇ´Â lymphokine(IL-2,4
& 5)¿¡
ÀÇÇØ multiplication
7) CD4(T-helper) & CD8(T-suppressor)
cell surface marker
: MHC (HLA) I
(histocompatibility) Ag°ú high affinity°¡Áü
¡æ
class I Ag°¡Áø cell¿¡
attach
¡æ
cytokine»ý¼º signal Àü´Þ
or cell lysis
Rejection Reaction
#
Clinical Manifestation
; *swelling & tenderness of grafts, fever, oliguria, hypertension,
progressive elevation of serum creatinine
#
Classification
; acute & chronic
rejection reaction
-
*cellular & humoral mechanism¿¡
ÀÇÇÑ graft damage
; accelerated(hyperacute)
rejection reaction
-
#
Diagnosis
;
renal ultrasound - enlarged graft & echogenic cortex
;
renal scan : blood flow °¨¼Ò
; ¡Úgraft biopsy
-
*definite diagnosis
; glomerular
immunofluorescence (-)
#
Differential Diagnosis
¨ç acute tubular necrosis
¨è cyclosporine-A
toxicity
¨é graftÀÇ
original renal diseaseÀÇ de novo occurrence
#
¹ß»ýºóµµ
¨ç LRD-RT : 50%
¨è CRD-RT : 65%
#
histocompatibility testÀÇ ÀÇÀÇ
: LRD & CAD RTÀÇ
survival ¿¬Àå¿¡ °¡Àå Áß¿ä
1) best survival result
: MLC nonstimulatory
sibling donor¿Í °°Àº HLA-A, -B, -C & -D/DR °¡Áø °æ¿ì
2) second best : haplotye matchÀÖ´Â
sibling or parent donor
Principles Of Immunosuppression And Therapy Of
Rejection Reations
1. RRÀÇ ±âÀü (Fig 490-3)
: foreign bodyÀÎ
renal graft
¡æ
immune systemÀÇ stimulation
¡æ
cell-mediated & humoral-mediated immune inflammationÀÇ
activation
¡æ
cell destruction & RR
¡ÚTable
490-6 Immunosuppressive Agent
#
°¡Àå ÈçÈ÷ »ç¿ëµÇ´Â protocol
; *azathioprine, cyclosporine, & low
dose corticosteroid sequential immunosuppression
2) antithymocyte globulin(ATGAM) »ç¿ë
: prophylaxis·Î »ç¿ë
or postoperative period¿¡ »ç¿ë
¡æ
50%¿¡¼ short-term graft outcome Çâ»ó
¡æ À̽Ä
6°³¿ù ÈÄ mean serum creatinine level °¨¼Ò
¡æ
transplant ÈÄ first RR »çÀÌ
interval Áõ°¡
¡æ
1 year graft survival Áõ°¡
Grwoth And Renal Transplantation
1.
chronic renal failure & ESRD-dialysisÀÇ complication
1) stunted linear growth
overt malnutrition :
less often
2) Ä¡·á
¨ç supportive care :
stamina Çâ»ó °¡´É
well-being sense
¨è successful transplant¸¸ÀÌ À̵é
abnormalityÀÇ ÀûÀýÇÑ ±³Á¤ °¡´É
2. growth retardation
1) RT ½Ç½Ã ÀÌÀü : 5¼¼ ÀÌÀü¿¡
-2.8 SD(standard deviation)
1¹øÀÌ»ó
RT ½Ç½Ã : -3.2 SD
2) transplant-related accelerated growth
¨ç 6¡12°³¿ù Áö¼Ó
¨è younger children¿¡¼ °üÂû
¨é adolescent¿¡¼± Ùí
¨ê weight gain : 2¡3³â µ¿¾È
normal adolescent¿Í À¯»çÇÑ Æò±ÕÄ¡ º¸ÀÓ
3) daily corticosteroid
¨ç growth retardationÀ¯¹ß °¡´É
¨è »ç¿ë ¹æ¹ý
: daily steroid¸¦
6°³¿ù°£ ¿ë·® °¨¼Ò½ÃŰ¸ç »ç¿ë
¡æ
every-other-day steroid »ç¿ëÇϸç 0.5¡0.2mg/KgÀ¸·Î
tapering
¡æ
single-dose every-other day
4) recombinant human growth
hormone(Nuprin)
: ÀÌ½Ä ÀüÈÄ¿¡ »ç¿ë
¡æ
linear growth Çâ»ó
graft functionÀº °¨¼Ò °¡´É
Complication
¡ÚTable
490-7
#
Infection
; *¡ãcommon cause of death
during 1yr after transplantation
; CMV infection
-
*¡ãcommon
-
°ø¿©ÀÚ or À̽ÄÀÚÀÇ CMV Ab titer ¹Ýµå½Ã ÃøÁ¤
-
¡ÚTable 490-8
-
reactivation state
/
*apparent 1-3mo
/
90% : asymptomatic & self-limited
/ 5¡10%
: death
/
Treatment
:
low-dose immunosuppressive agents with antiviral agent
:
*gancyclovir & anti-CMV
immunoglobulin IV
/
kidney biopsy
:
rejectionÀÇ monitoring
-
*direct tissue damage or triggering
of rejection reaction
-
systemic CMV infection(lung, brain, liver ħ¹ü)
/ immunosuppressionÁß´Ü
-
unresponsive rejection : kidney Á¦°Å
-
concomitant viral infection
/ varicella-zoster,
Epstein-Barr, herpes simplex, hepatitis
;
Pneumocystis carinii
-
trimethoprim-sulfamethoxazoleÀÇ prophylactic use½Ã¿¡¸¸ ¹æÁö
(bacterial urinary
tract infectionµµ ¹æÁö)
#
mortality
;
4-4.5%
¡æ ÀÌÁß
40¡45%°¡ infection ¶§¹®
#
tumor
; ÁÖ·Î
lymphoma, sarcoma & carcinoma ¹ß»ý
; high death rate