childhood kidney diseases in short

3. Rural Bangladesh: mature rice field

6. WELCOME
ALL 66

7. RENALRENAL
DIASEASESDIASEASES
77
dr m nurul huqdr m nurul huq
Bangladesh Bangladesh Medical CollegeBangladesh Bangladesh Medical College

8. At the end… you will learnAt the end… you will learn
• Kidney disease can beKidney disease can be aa silent killersilent killer
• Childhood NS is mostly curableChildhood NS is mostly curable
• APSGN does nor recur; mostly recoversAPSGN does nor recur; mostly recovers
• Hematuria in small children is usually harmlessHematuria in small children is usually harmless
• With ageing most of us develop renal diseaseWith ageing most of us develop renal disease
• ARF in most cases can be preventedARF in most cases can be prevented
88

9. 99

10. 1010

11. 1111

12. Summary:
Renal Function
1212

13. Importance of kidneyImportance of kidney
• MainMain waste excreterwaste excreter
• MaintainsMaintains fluid- and ABBfluid- and ABB
• MakesMakes erythropoietinerythropoietin
• Excretes someExcretes some drugsdrugs
• BiotransformsBiotransforms VDVD
1313

14. Peculiarities of Kidney DiseasesPeculiarities of Kidney Diseases
• May be asymptomaticMay be asymptomatic
• Symptoms can beSymptoms can be nonspecificnonspecific
• FewFew physical signsphysical signs
• May present withMay present with jaundicejaundice in infantsin infants
• Important c/ofImportant c/of FTTFTT
• Long UT means moreLong UT means more obstructionobstruction
1414

15. 1515

16. 1616

17. 1717

18. 1818

19. Micros. H:Micros. H: in a well child: ~in a well child: ~ no Ix.no Ix. ifif
not x3/over several mo.; evaluate ifnot x3/over several mo.; evaluate if
HTN, CKD, casturia/ proteinuriaHTN, CKD, casturia/ proteinuria
presentpresent
Gross H:Gross H: urine is red/tea/colaurine is red/tea/cola
colored; also mostly benigncolored; also mostly benign
Up to 5 rbc per HPF in urineUp to 5 rbc per HPF in urine
is normal in childrenis normal in children
1919

20. GH is more inGH is more in
boys. Assess byboys. Assess by
CF. VCUG isCF. VCUG is
useful inuseful in
doubtful USG,doubtful USG,
UTI, or voidingUTI, or voiding
problem.problem.
Cystoscopy ifCystoscopy if
persistent orpersistent or
with ambiguouswith ambiguous
imagingimaging
VCUG : voidingVCUG : voiding
cystourethrographycystourethrography 2020

21. 2121

22. 2222

23. A Case History: hematuriaA Case History: hematuria
A 12y-boy has hematuria. He has occasional darkA 12y-boy has hematuria. He has occasional dark
urine after heavy exercise but no gross hematuriaurine after heavy exercise but no gross hematuria
He does not use medicine. No h/of deafness; noHe does not use medicine. No h/of deafness; no
family h/of renal diseasefamily h/of renal disease
Physically normal:Physically normal: BP 130/80BP 130/80
Trace proteinuria and 10-15 rbc/hpf. No castsTrace proteinuria and 10-15 rbc/hpf. No casts
What is the most probable Dx?What is the most probable Dx?
2323

24. Isolated HematuriaIsolated Hematuria
• AsymptomaticAsymptomatic
• No features of NS/GNNo features of NS/GN
• Prevalence: 5-10% in the communityPrevalence: 5-10% in the community
Glomerular d. causes <10% of hematuria in pts. with noGlomerular d. causes <10% of hematuria in pts. with no
proteinuriaproteinuria
2424

25. Renal Function in NewbornRenal Function in Newborn
GFRGFR
– 5ml/ min in first week of life5ml/ min in first week of life
– 10ml/ min 1-2 mo10ml/ min 1-2 mo
– Preterm has lower GFRPreterm has lower GFR
2525

26. TerminologiesTerminologies
• Black water F:Black water F: (malaria hemolysis(malaria hemolysis:: hemoglobinuriahemoglobinuria))
• CKD/CRF:CKD/CRF: progressive RF over 3mo (Dm, HTN, GN)progressive RF over 3mo (Dm, HTN, GN)
• GN:GN: glomeruli and tubules inflamedglomeruli and tubules inflamed
• Mesangium:Mesangium: cells supporting glomeruli: phagocytic; maycells supporting glomeruli: phagocytic; may
contain molecules that may aid in Dxcontain molecules that may aid in Dx
2626

27. 2727

28. • ARF:ARF: Ac. Renal FailureAc. Renal Failure (hours-days)(hours-days)
• ESRD:ESRD: Total CRF. Needs dialysis/transplantTotal CRF. Needs dialysis/transplant (renal(renal
replacement therapy)replacement therapy)
• ATN:ATN: Ac. kidney Injury: severe ARF (severe infx./Ac. kidney Injury: severe ARF (severe infx./
hypotension). May need dialysis.hypotension). May need dialysis. ““Muddy brownMuddy brown
casts"casts" (epith. cells) is(epith. cells) is pathognomonicpathognomonic
• ESWLESWL (Extracorpo. Shockwave Lithotripsy):(Extracorpo. Shockwave Lithotripsy): toto
break kidney stonesbreak kidney stones
2828

29. ATN: muddy brown casts
2929

30. Causes of ARFCauses of ARF
• SevereSevere dehydrationdehydration
• Shock, ac.Shock, ac. hgehge
• BlockageBlockage of renal BV, oof renal BV, obstructionbstruction in UTin UT
• RenalRenal injuryinjury
• Ac.Ac. GN, aGN, acc. PN. PN
3030

31. • HUS:HUS: destroysdestroys lininglining of BVof BV andand RBCRBC; often c/by; often c/by E.E.
colicoli; may get ARF or coagulopathy; may get ARF or coagulopathy
• Alport Syn.:Alport Syn.: inherited: x-linked. Hematuria,inherited: x-linked. Hematuria,
proteinuria; more serious in boys; leads to ESRD,proteinuria; more serious in boys; leads to ESRD,
hearing and visual losshearing and visual loss
• PKD:PKD: inherited: grape-like cysts in kidneys; destroyinherited: grape-like cysts in kidneys; destroy
kidneys: CKD and ESRDkidneys: CKD and ESRD
HUS: hemolytic uremic syn. PKD: polycystic kidney DHUS: hemolytic uremic syn. PKD: polycystic kidney D
3131

32. • Interstitial Nephritis:Interstitial Nephritis: Inflam. of supporting tissueInflam. of supporting tissue
of kidney; can lead to ARF/ESRDof kidney; can lead to ARF/ESRD
• Renal osteodystrophy:Renal osteodystrophy: RF causing weak bones;RF causing weak bones;
more in dialysis pts.: high PO4/low VDmore in dialysis pts.: high PO4/low VD
• RTA:RTA: kidneys fail to remove acids normally: weakkidneys fail to remove acids normally: weak
bones, kidney stones and FTTbones, kidney stones and FTT
RTA: renal tubular acidosisRTA: renal tubular acidosis
3232

33. CKD (CRF):CKD (CRF): GFR <90ml/min/1.73mGFR <90ml/min/1.73m22
>3 mo>3 mo
5 stages5 stages
• GFR 90ml/min/1.73m2GFR 90ml/min/1.73m2 NormalNormal
• 60-89 ….60-89 …. MildMild
• 30-59 ….30-59 …. ModerateModerate
• 15-29 ….15-29 …. SevereSevere
• <15/dialysis<15/dialysis ESRDESRD
3333

34. Aetiology of CKD
• Primary: MCD, FSGS, MGN, MPGN, Crescentic GN,
Goodpasture syn.
• Systemic: Dm, HTN, SLE, HSP, IgAN, APSGN, HIV,
HBV, HCV, malaria
• Vascular: Nephrosclerosis, ANCA, HUS
• Hereditary: Amyloidosis, PKD, Alport syn.
• Tubulointerstitial: drugs/toxins, VUR, obs. urop.
ANCAs: Anti-neutrophil cytoplasmic Ab
3434

35. Urea frost in CRF
3535

36. Goodpasture Syn.Goodpasture Syn. ((anti-GBM d)anti-GBM d)
• Autoim. pulmo-renal syn.: anti-collagen ABs inAutoim. pulmo-renal syn.: anti-collagen ABs in
lungs:lungs: vasculitis:vasculitis: hge., kidneys:hge., kidneys: GN (anti-GBM ABs)GN (anti-GBM ABs)
• It isIt is fatalfatal unless quickly Rxunless quickly Rx..
IgA NephropathyIgA Nephropathy ((Berger DBerger D):): RF is rareRF is rare
• Commonest GN in WestCommonest GN in West. IgA deposits after URTI:. IgA deposits after URTI:
silent hematuria; may go for yrssilent hematuria; may go for yrs
• Men more. All agesMen more. All ages
• No Rx for early/mild IgAN with normal BP and <1gNo Rx for early/mild IgAN with normal BP and <1g
24TUP: if more, Rx. with ACEI or ARBs24TUP: if more, Rx. with ACEI or ARBs
GBM: glomerular basement membrane. RF: renal failureGBM: glomerular basement membrane. RF: renal failure 3636

37. Paroxysmal Noc. Hb.uria (PNH)Paroxysmal Noc. Hb.uria (PNH)
Rare. Acquired. Some proteins cannot fix to RBCs toRare. Acquired. Some proteins cannot fix to RBCs to
protect from complement: hemolysis: Hb.emia andprotect from complement: hemolysis: Hb.emia and
Hb.uria; at night/early morningHb.uria; at night/early morning
• Any age. May causeAny age. May cause aplastic a., AML, MDSaplastic a., AML, MDS
• SS:SS: RAP, backache, HA, SoB, clotting; dark urine;RAP, backache, HA, SoB, clotting; dark urine;
easy bruisingeasy bruising
3737

38. Investigations for PNHInvestigations for PNH
• Pancytopenia,Pancytopenia, Hb.emia and hb.uriaHb.emia and hb.uria
• Coombs' test; haptoglobin levelCoombs' test; haptoglobin level
• Flow cytometry to measure certain proteinsFlow cytometry to measure certain proteins
• Ham's (acid hemolysin) testHam's (acid hemolysin) test
• Sucrose hemolysis testSucrose hemolysis test
• Urine hemosiderinUrine hemosiderin
3838

39. Rx for PNHRx for PNH
• Steroids/immunosuppressantsSteroids/immunosuppressants
• BT. Iron and B9. Blood thinnersBT. Iron and B9. Blood thinners
• EculizumabEculizumab can block hemolysiscan block hemolysis
• BMT can cureBMT can cure
• Vaccinations against certain types of bacteriaVaccinations against certain types of bacteria
Outlook:Outlook: most people survive >10 y after Dx. Deathmost people survive >10 y after Dx. Death
occur from thrombosis or bleedingoccur from thrombosis or bleeding
3939

40. GlomerulonephritisGlomerulonephritis
• Inflam. and proliferation of Glo. tissue with damageInflam. and proliferation of Glo. tissue with damage
to BM, mesangium or capillary endo.to BM, mesangium or capillary endo.
• Acute:Acute: hematuria, proteinuria and RBC casts. Oftenhematuria, proteinuria and RBC casts. Often
with HTN, edema and impaired RFwith HTN, edema and impaired RF
• ChronicChronic: scarring of nephrons with progressive RF: scarring of nephrons with progressive RF
4040

41. Ac NephriticAc Nephritic
(Glomerulonephritic)(Glomerulonephritic) Syn.Syn.
• Ac. inflam. of the glomeruli and nephronsAc. inflam. of the glomeruli and nephrons
Nephrotic Syn.Nephrotic Syn.
• Affection of nephrons with leakage ofAffection of nephrons with leakage of
protein (usually noprotein (usually no inflam.)inflam.)
4141

42. Ac. Nephritis: causesAc. Nephritis: causes
– Group A Streptococcus Group A Streptococcus 80%80%
– OthersOthers 20%20%
• Systemic: HSP,Systemic: HSP, SLE, IgAN,SLE, IgAN, Goodpasture, gold,Goodpasture, gold,
penicillaminepenicillamine
• Infx.: staph, pneumococci, Gram-ve, malaria,Infx.: staph, pneumococci, Gram-ve, malaria,
HBV, HCV, MMR, HIVHBV, HCV, MMR, HIV
• Infective endocarditisInfective endocarditis
• Renal d: MGN, MPGN, FSGS, etcRenal d: MGN, MPGN, FSGS, etc
4242

43. APSGNAPSGN
• 15% of all GAS infx.; mostly RTI (skin 10%)15% of all GAS infx.; mostly RTI (skin 10%)
• 2% clinically overt.2% clinically overt. Lag period: 2-3w.Lag period: 2-3w.
• No recurrenceNo recurrence
• Any AgeAny Age (2-15y; 2% <2y; 10% >40y). Boys >(2-15y; 2% <2y; 10% >40y). Boys >
• Excellent prognosisExcellent prognosis:: <2% MR. 2% CGN<2% MR. 2% CGN
• Cerebral vasculitis may occurCerebral vasculitis may occur
GAS: group A streptococci
4343

44. PathophysiologyPathophysiology
 Exact mechanism is unclear. Autoim. D: both CMIExact mechanism is unclear. Autoim. D: both CMI
and humoral.and humoral. Immune complexImmune complex in glomeruli: activatesin glomeruli: activates
complement: inflam.complement: inflam.
 Strep.Strep. does not attack the kidneydoes not attack the kidney
Kidneys may enlarge ~50%Kidneys may enlarge ~50%
 Histology:Histology: swelling of glomeruli, polymorphs,swelling of glomeruli, polymorphs,
infiltration.infiltration. IF:IF: deposition of Ig and complementdeposition of Ig and complement
4444

45. 4545

46. CF of APSGNCF of APSGN
 Abrupt:Abrupt: H/o earlier URTI, skin infx. a few weeksH/o earlier URTI, skin infx. a few weeks
before (latent period)before (latent period)
 Puffy face, scanty HC/Puffy face, scanty HC/coca coloredcoca colored urineurine
 Flank pain (stretching of renal capsule)Flank pain (stretching of renal capsule)
 Weakness, -/+ AP, anorexia, FWeakness, -/+ AP, anorexia, F
 Anasarca, SoB/Anasarca, SoB/exertional dyspnoea,exertional dyspnoea, coughcough
 HTN, HA, LVF, convulsionHTN, HA, LVF, convulsion
 Hematuria (universal)Hematuria (universal)
4646

47. 4747

48. Lab:Lab:
• Urine:Urine: RBCs, RBC casts, WBC, +/++proteinuriaRBCs, RBC casts, WBC, +/++proteinuria
• FBC: dilutional anemia, leucocytosisFBC: dilutional anemia, leucocytosis
• S/of recent strep. infx.: ASOT,S/of recent strep. infx.: ASOT, Anti Dnase B,Anti Dnase B, throat/throat/
wound CSwound CS
• Elevated ureaElevated urea ±± creatininecreatinine
• Low complement C3Low complement C3
Anti-Dnase B: AB made by GAS. Raised levels indicate: Rh. F,
PSGN, Strep. throat or Strep. skin infection
4848

49. Renal BiopsyRenal Biopsy
• Declining RFDeclining RF
• Atypical presentationAtypical presentation
• F/history of renal DF/history of renal D
• Persistent HTN or gross hematuria andPersistent HTN or gross hematuria and
-hypocomplementemia-hypocomplementemia
• Hallmark in PSGNHallmark in PSGN is subepithelial ‘humps’is subepithelial ‘humps’
representing immune complex depositionrepresenting immune complex deposition
4949

50. DiagnosisDiagnosis
• CF, swab CS, positive ASO and/or anti DNase BCF, swab CS, positive ASO and/or anti DNase B
• C3 is typically low (normalizes 6- 12w). But normalC3 is typically low (normalizes 6- 12w). But normal
C3 does not exclude itC3 does not exclude it
DDDD
• IgANIgAN
• HSP, SLEHSP, SLE
• HUS, other infx.HUS, other infx.
5050

51. Henoch-Schonlein purpuraHenoch-Schonlein purpura
• 70% affect kidneys: MC hematuria +/- proteinuria70% affect kidneys: MC hematuria +/- proteinuria
• HistologicallyHistologically IgANIgAN
• May relapseMay relapse
• Severe: steroids, azathioprineSevere: steroids, azathioprine
• Follow until urinalysis normalFollow until urinalysis normal
• 5-20% of children end in ESRF5-20% of children end in ESRF
5151

52. Rx Of PSGNRx Of PSGN
• Supportive. ~Supportive. ~ Bed restBed rest
• Fluid and salt restriction.Fluid and salt restriction. FEBFEB
• Rx of hyperkalemia.Rx of hyperkalemia. No fruits!No fruits!
• Penicillin x 10d:Penicillin x 10d: why?why?
• BP control. ACE inhibitors can cause hyperkalemiaBP control. ACE inhibitors can cause hyperkalemia
• Rx of complicationsRx of complications
• Admission if renal failureAdmission if renal failure
5252

53. Complications of AGNComplications of AGN
• ARF (uremia/azotemia)ARF (uremia/azotemia)
• CGN: 2%CGN: 2%
• Volume over-load: HTN: LVFVolume over-load: HTN: LVF
– HTN: encephalopathy, convulsionHTN: encephalopathy, convulsion
• Hyperkalemia, acidosisHyperkalemia, acidosis
• Microhematuria may persist for yearsMicrohematuria may persist for years
• NSNS
5353

54. APSGN: Prognosis:APSGN: Prognosis: excellentexcellent
• Mortality 2%.Mortality 2%. CKD: 2% in children.CKD: 2% in children. 30% in adults30% in adults
• ESRD 1-2%ESRD 1-2%
• One attack confers lifelong immunityOne attack confers lifelong immunity
• Almost all APSGN recover completelyAlmost all APSGN recover completely
5454

55. S/o Glomerular DiseasesS/o Glomerular Diseases
• May be silent for many yearsMay be silent for many years
• Hematuria, proteinuria, azotemiaHematuria, proteinuria, azotemia
• HTN, edema, hyperlipidemiaHTN, edema, hyperlipidemia
GN and G. sclerosis include many forms of glomerularGN and G. sclerosis include many forms of glomerular
damage. Some RF can be slowed down, but scarreddamage. Some RF can be slowed down, but scarred
glomeruli cannot be repairedglomeruli cannot be repaired
5555

56. GlomerularGlomerular vsvs Non-G Hematuria?Non-G Hematuria?
• Chemical trauma to RBC as they pass throughChemical trauma to RBC as they pass through
nephrons causes peculiar changes: they losenephrons causes peculiar changes: they lose
biconcavity and have blebs:biconcavity and have blebs: “Mickey Mouse Cells”“Mickey Mouse Cells”
• RBC casts and proteinuria supports a GDRBC casts and proteinuria supports a GD
5656

57. Mickey mouse cells
5757

58. ProteinuriaProteinuria
Normal valuesNormal values
– Premature:Premature: ≤≤ 140 mg/m140 mg/m22
/d/d
– FTFT ≤≤ 70 mg/m70 mg/m22
/d/d
– Children <10yrChildren <10yr ≤≤ 150 mg/d150 mg/d
– Children 10-18 yrChildren 10-18 yr ≤≤ 300 mg/d300 mg/d
– AdultsAdults ≤≤ 150 mg/d150 mg/d
5858

59. Case history:Case history: A 3y old child has heavy proteinuria withA 3y old child has heavy proteinuria with
anasarca. No familial KD. He is not on any drugs. Wt 17 kganasarca. No familial KD. He is not on any drugs. Wt 17 kg
(14Kg), BP 90/50; 4+edema(14Kg), BP 90/50; 4+edema
Urine: 0-4 RBC/hpf. Numerous hyaline casts. Some lipidUrine: 0-4 RBC/hpf. Numerous hyaline casts. Some lipid
inclusions appearing Maltese cross under polarized lightinclusions appearing Maltese cross under polarized light
What is the Dx?What is the Dx?
5959

60. Glomerular vs. Tubular ProteinuriaGlomerular vs. Tubular Proteinuria
TraitsTraits TubularTubular GlomerularGlomerular
Sp. Gr. Of urineSp. Gr. Of urine IsosthenuricIsosthenuric High (conc.)High (conc.)
ProteinProtein 1+1+ ≥≥ 3+3+
HematuriaHematuria Usually nilUsually nil OftenOften
CastsCasts Tubular cells/Tubular cells/
nonenone
Variable (RBC,Variable (RBC,
granulargranular
UP ElectrophoresisUP Electrophoresis Broad band –Broad band –
multiple globulinmultiple globulin
Albumin peakAlbumin peak
6060

61. Nephrotic SyndromeNephrotic Syndrome
• Massive proteinuria >3.5g/d (>40mg/mMassive proteinuria >3.5g/d (>40mg/m22
/h)/h)
• Hypoalbuminemia: <30g/dlHypoalbuminemia: <30g/dl
• Anasarca: water pooling. Ac. wt. gainAnasarca: water pooling. Ac. wt. gain
• HyperlipidemiaHyperlipidemia
• LipiduriaLipiduria
Age: 1½ - 5y. Boys moreAge: 1½ - 5y. Boys more
6161

62. 6262

63. PeculiaritiesPeculiarities of Childhood NSof Childhood NS
• Mostly no inflammation/RFMostly no inflammation/RF
• Most respond to steroidMost respond to steroid
• Well for 3y: no more relapseWell for 3y: no more relapse
• No relapse after 15y ageNo relapse after 15y age
• Auto-remission 5%Auto-remission 5%
6363

64. Spot urineSpot urine
Urinary protein/creatinineUrinary protein/creatinine >200>200
Significant proteinuria:Significant proteinuria: >4mg/m>4mg/m22
/h/h
Heavy proteinuria:Heavy proteinuria: >40mg ,,>40mg ,,
Remission:Remission: <4mg/m2/h<4mg/m2/h
6464

65. NS of childhood: classificationNS of childhood: classification
MCD: 85%
FSGS: 10%
Others: 5%
(membranoproliferative
GN, mesangiocapillary
GN, diffuse proliferative GN),
congenital
6565

66. • Steroid sensitive (90%)
• Steroid resistant: no response in 4w (10%)
• Steroid dependent: relapse on 2 consecutive
occasions as steroid is being tapered or within 2w
of withdrawal
• Remission: nil protein morning urine x 3d
• Relapse: UP: >40/m2/h or Albustix ≥++ x 3d
morning urine. Frequent R: ≥2 Rs in 6mo of Dx. or
≥4 R/y. Infrequent R: after 3 mo of remission
6666

67. PathophysiologyPathophysiology
• Non-immuneNon-immune factors in MCD and FSGSfactors in MCD and FSGS
• Immune factors in MPGN, PSGN and SLEImmune factors in MPGN, PSGN and SLE
• Mutations in podocyte or slit diaphragm inMutations in podocyte or slit diaphragm in
inherited, infantile or steroid resistant NSinherited, infantile or steroid resistant NS
• Incidence: 2-7/100,00/yIncidence: 2-7/100,00/y
• x15 common in childrenx15 common in children
• Age of onset varies with type of diseaseAge of onset varies with type of disease
6767

68. Filtration barrier. A. The endothelium with fenestra
B. GBM: 1. lamina interna 2. L. densa 3. L. externa
C. Podocytes: 1. enzymatic and structural protein 2. filtration slit 3.
diaphragma 6868

69. MCDMCD
• Major c/of NS in children
• Light ME: Normal
• EM: fusion of foot processes of visceral epith cells
• IF: no immune complex deposit
• Cause/mechanism unknown
• Drammatic response to steroid
• Excellent prognosis
6969

70. Focal Segmental GlomerulosclerosisFocal Segmental Glomerulosclerosis (FSGS)(FSGS) andand
Membranoproliferative GNMembranoproliferative GN (MPGN)(MPGN)
• In about 15% of childhood NS, a kidney biopsyIn about 15% of childhood NS, a kidney biopsy
shows scarring or deposits in glomerulishows scarring or deposits in glomeruli
• Steroid is less effective in these; need cytotoxicSteroid is less effective in these; need cytotoxic
• ACEI can decrease HTN and proteinuria and protectACEI can decrease HTN and proteinuria and protect
kidneyskidneys
7070

71. C/FC/F
• M: F =2:1M: F =2:1
• Gross edema, scantyGross edema, scanty
urine, SoBurine, SoB
• There may be anThere may be an
antecedal URTI (specially in relapse)antecedal URTI (specially in relapse)
Others:Others: depression, lethargy, anorexia, skin striae,depression, lethargy, anorexia, skin striae,
diarrhea, AP, orthostatic hypotensiondiarrhea, AP, orthostatic hypotension
Bedside urine: heavy proteinuriaBedside urine: heavy proteinuria
7171

72. 7272

73. 7373

74. InvestigationsInvestigations
• Urine RE, CSUrine RE, CS
• UTP/spot urine ACR: >200UTP/spot urine ACR: >200
• CBC, electrolytes, BUN, S. Cr., STP, AG ratio,CBC, electrolytes, BUN, S. Cr., STP, AG ratio,
cholesterol (specifically LDL)cholesterol (specifically LDL)
• ANA; Anti-dsDNA, C3, HBsAg, HCVANA; Anti-dsDNA, C3, HBsAg, HCV
• Renal USGRenal USG
• CXR, MT, worms, before steroid RxCXR, MT, worms, before steroid Rx
DD:DD: CHF, cirrhosis, protein losing statesCHF, cirrhosis, protein losing states 7474

75. Renal BiopsyRenal Biopsy
RarelyRarely done in Paediatric cases. Consider:done in Paediatric cases. Consider:
• Cong. NSCong. NS
• >8 y at onset>8 y at onset
• Steroid resistanceSteroid resistance
• Frequent relapsesFrequent relapses
• Significant nephritic featuresSignificant nephritic features
7575

76. ComplicationsComplications
• Infection:Infection: loss of Ig, complement: UTI, SBPloss of Ig, complement: UTI, SBP
(commonest) and pneumonia(commonest) and pneumonia (pneumococcus)(pneumococcus)
• Thrombosis:Thrombosis: loss of anti thrombin iii, antiplasminloss of anti thrombin iii, antiplasmin
and proteins S&C in urine, more coagulants byand proteins S&C in urine, more coagulants by
liver, raised hct., relative immobility, steroidliver, raised hct., relative immobility, steroid
• Hypovolemia:Hypovolemia: postural hypotensionpostural hypotension
• Drug toxicity:Drug toxicity: steroid, nephrotoxcity fromsteroid, nephrotoxcity from
cyclosporin A or tacrolimuscyclosporin A or tacrolimus
7676

77. Management: generalManagement: general
• CheckCheck BP, wt, abdo. girth, IO chart, proteinuriaBP, wt, abdo. girth, IO chart, proteinuria
• Bed rest in gross edemaBed rest in gross edema
• Diet: lean protein, low fat; low-saltDiet: lean protein, low fat; low-salt
• Salt and fluid restriction. UsuallySalt and fluid restriction. Usually no diureticno diuretic
• Hypovolemia and hypoalbuminemia: FFP 20ml/kgHypovolemia and hypoalbuminemia: FFP 20ml/kg
or salt poor albumin 20%or salt poor albumin 20%
• Anticoagulants can help decrease clottingAnticoagulants can help decrease clotting
• Statins can help lower cholesterolStatins can help lower cholesterol
7777

78. SpecificSpecific
• Objective:Objective: Rx underlying causeRx underlying cause
• MCD: up to 8 year age: no renal biopsyMCD: up to 8 year age: no renal biopsy
• Prednisolone 60mg/m2/dPrednisolone 60mg/m2/d x 4 wx 4 w, then, then 40mg/m2/d40mg/m2/d
EAD for 4wEAD for 4w then STOPthen STOP
7878

79. RelapseRelapse
• First 2: “treat same way”First 2: “treat same way”
• Frequent R: keep steroid 0.5mg/kg EAD for 3-6 mo.Frequent R: keep steroid 0.5mg/kg EAD for 3-6 mo.
If relapse: Levamisole EAD for 4-12 moIf relapse: Levamisole EAD for 4-12 mo
• If still R:If still R: Cyclophosphamide x 8w plus Low DoseCyclophosphamide x 8w plus Low Dose
PredPred
• Still rStill relapse: Cyclosporin A for 1y plus LD Predelapse: Cyclosporin A for 1y plus LD Pred
• Other drugs: Tacrolimus, Mycophenolate mofetilOther drugs: Tacrolimus, Mycophenolate mofetil
7979

80. SRNSSRNS
• Refer to specialized unit
• Full remission not achieved
• Aim: lower proteinuria to not-in-nephrotic range
• Risk of HTN and renal failure
• In FSGS: 20-40% risk of relapse post transplant
8080

81. DDDD
SSNSSSNS
• Toddler, pre-school
• No HTN
• Mild, intermittent
haematuria
• Normal renal function
• Excellent prognosis,
even if frequently
relapsing
• No biopsy
SRNSSRNS
• <1 year, > 8 y
• HTN common
• Persistent haematuria
• Often abnormal RF
• Long term HTN and RF
• Biopsy needed: usual
histology FSGS
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82. Congenital NSCongenital NS
• First 3 mo of life. Large placenta ~ 40% of BW
• Drug resistant. High morbidity: PEM & sepsis
• Types:Types: Finnish type: most severe, AR. Diffuse
mesangial sclerosis: less severe, AR. Denys-Drash
syn.: pseudohermaphroditism and Wilm’s T. FSGS.
Secondary CNS: cong. syphilis
• Rx.: Intensive care: 20% albumin, nutrition, early
unilateral nephrectomy, RRT
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83. Rx of Hypertension in NS
• ACEI reduce BP and proteinuria
• Nifedipine 0.25mg/kg/dose s.l.; max 8 doses/d
(not >2mg/kg/d or
• Hydralazine 0.5-2mg/kg/d)
• Others: Atenolol, Methyldopa
• Diuretic is controversial. Use with caution. May be
dangerous in hypovolemia
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84. Immunization in NS
Prednisolone 2mg/kg/d or 20mg/kg/d x 14d:
immunocompromized
• No live vax.
• Killed vax./toxoids are safe
• Live vax. after 4w of stopping steroid
• VZIG in case of exposure
• Ig in case of measles expo. or cl. measles
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85. Trait NS APSGN
H/o Nil Preceding Strep. Inf.
Age 2-6y 5-15y
Edema Massive Mild-moderate
Urine color Clear Coca-cola colored
Sediment Nil Red colored
Protein 4+ 1-2+
Microscopy Clear Plenty RBCs, PC
Casts Hyaline RBC casts
Serum albumin Below 25g/dl Normal
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86. Prognosis:
in idiopathic NS of childhood is excellent
Mortality 1-2%
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87. MCQMCQ
• In APSGN ABT is essential for the pt.In APSGN ABT is essential for the pt.
• APSGN is an autoimmune DAPSGN is an autoimmune D
• Strep. skin infx. can cause RhFStrep. skin infx. can cause RhF
• Fruits are beneficial in APSGNFruits are beneficial in APSGN
• In APSGN LVF can occur from myocarditisIn APSGN LVF can occur from myocarditis
• Hyperkalemia is a recognized complication ofHyperkalemia is a recognized complication of
APSGNAPSGN
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89. ThankThank
YouYou
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