This document provides information on various renal diseases. It discusses that kidney disease can be a silent killer, and that childhood nephrotic syndrome is mostly curable while acute post-streptococcal glomerulonephritis mostly recovers and does not recur. It also notes that hematuria in small children is usually harmless, but that aging can lead to developing kidney disease in most people. Acute renal failure can often be prevented in most cases as well.

5. RENAL DISEASESRENAL DISEASES
55

6. WELCOME
ALL 66

7. 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 mostly recoversAPSGN mostly recovers;; does nor recurdoes nor recur
• HematuriaHematuria in small children is usually harmlessin small children is usually harmless
• With ageing most of us develop kidney diseaseWith ageing most of us develop kidney disease
• ARF in most cases can be preventedARF in most cases can be prevented
APSGN: ac. Post-strep. Glomerulonephritis. ARF: ac. Renal failureAPSGN: ac. Post-strep. Glomerulonephritis. ARF: ac. Renal failure
77

8. 88

9. 99

10. Summary:
Renal Function
1010

11. Importance of kidneyImportance of kidney
• MainMain waste excreterwaste excreter
• MaintainsMaintains fluid-, electrolyte- & ABBfluid-, electrolyte- & ABB
• MakesMakes erythropoietin, thrombopoietinerythropoietin, thrombopoietin
• Excretes someExcretes some drugsdrugs
• Biotransforms/activatesBiotransforms/activates VDVD
ABB: acid base balanceABB: acid base balance
1111

12. Peculiarities of Kidney DiseasesPeculiarities of Kidney Diseases
• May be asymptomaticMay be asymptomatic
• Symptoms can beSymptoms can be nonspecificnonspecific
• Few physical signsFew physical signs
• May present withMay present with jaundicejaundice in infantsin infants
• Important c/ofImportant c/of FTTFTT
• Long UT means moreLong UT means more obstructionobstruction
1212

13. 1313

14. 1414

15. 1515

16. 1616

17. Micros. H:Micros. H: in a well child: ~in a well child: ~ no testno test
if not x3/over several mo.;if not x3/over several mo.;
evaluate if HTN, CKD,evaluate if HTN, CKD,
casturia/proteinuria presentcasturia/proteinuria present
Gross H:Gross H: urine is red/tea/colaurine is red/tea/cola
colored. It is also mostlycolored. It is also mostly
benignbenign
Up to 5 RBC/HPF in urine isUp to 5 RBC/HPF in urine is
normal in childrennormal in children
1717

18. Causes:Causes:
GH is more in boys.GH is more in boys.
Assess by CF.Assess by CF.
VCUG is useful inVCUG is useful in
doubtful USG, UTI,doubtful USG, UTI,
or voiding problem.or voiding problem.
Cystoscopy ifCystoscopy if
persistent or withpersistent or with
ambiguous imagingambiguous imaging
GH: gross hematuria. VCUG : voidingGH: gross hematuria. VCUG : voiding
cystourethrographycystourethrography
1818

19. 1919
: ABCDEFGHI: ABCDEFGHI

20. Henoch-Schonlein purpuraHenoch-Schonlein purpura
• Classic triad:Classic triad: purpurapurpura (100%),(100%), arthritis/j. painarthritis/j. pain (80%),(80%), AP (AP (60%)60%)
• 70% affect kidneys (hematuria)70% affect kidneys (hematuria)
• Histologically vasculitis,Histologically vasculitis, IgANIgAN
• May relapseMay relapse
• Severe: steroids, azathioprineSevere: steroids, azathioprine
• Follow until urinalysis normalFollow until urinalysis normal
• 5-20% of children end in ESRD5-20% of children end in ESRD
IgAN: IgA nephropathy. ESRD: end stage renal diseaseIgAN: IgA nephropathy. ESRD: end stage renal disease
2020

21. 2121
PurpurasPurpuras ((necrotizingnecrotizing
vasculitis in dermalvasculitis in dermal smallsmall
BV; usuallyBV; usually extensorextensor
surfaces ofsurfaces of limbs,limbs,
sometimessometimes buttocksbuttocks
AP, V, gut bleedingAP, V, gut bleeding::
vasculitis in GITvasculitis in GIT
Renal:Renal: commonest iscommonest is
hematuria. In adults:hematuria. In adults: it isit is
more severe &more severe & maymay
evolve into aevolve into a rapidlyrapidly
progressive,progressive, crescentic GNcrescentic GN

22. A Case HistoryA Case History
• A 12y-boy has hematuria. He has occasional darkA 12y-boy has hematuria. He has occasional dark
urine after heavy exercise but not GHurine after heavy exercise but not GH
• No h/o medicine, deafness; no FH of renal d.No h/o medicine, deafness; no FH of renal d.
• PE:PE: normal:normal: BP 130/80BP 130/80
• Trace proteinuriaTrace proteinuria
• 10-15 rbc/hpf. No casts10-15 rbc/hpf. No casts
• What is the most probable Dx?What is the most probable Dx?
2222

23. Answer: Exercise Induced HematuriaAnswer: Exercise Induced Hematuria
• Hematuria: asymptomaticHematuria: asymptomatic
• 5-10% in the community5-10% in the community
• No features of NS/GNNo features of NS/GN
2323

24. 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
2424

25. TerminologiesTerminologies
• Black water F:Black water F: (malaria(malaria hemolysishemolysis:: hemoglobinuriahemoglobinuria))
• CKD/CRF:CKD/CRF: progressive RF over 3mo (Dm, HTN, GN)progressive RF over 3mo (Dm, HTN, GN)
• GN:GN: glomeruli & tubules inflamedglomeruli & tubules inflamed
• Mesangium:Mesangium: cells supporting glomeruli:cells supporting glomeruli: phagocyticphagocytic
• ARF:ARF: Ac. Renal FailureAc. Renal Failure
(hours-days)(hours-days)
2525

26. • ESRD:ESRD: Total CRF (GFR <15ml). Rx:Total CRF (GFR <15ml). Rx: dialysis/transplantdialysis/transplant
(renal replacement therapy)(renal replacement therapy)
• ATN:ATN: Ac. kidney Injury: severe ARF (severe infx./Ac. kidney Injury: severe ARF (severe infx./
hypotension). May need dialysishypotension). May need dialysis. “. “Muddy brown casts"Muddy brown casts"
(epith. cells) is(epith. cells) is pathognomonicpathognomonic
• ESWLESWL (Extracorporeal Shockwave Lithotripsy):(Extracorporeal Shockwave Lithotripsy): to breakto break
kidney stoneskidney stones
2626
ESRD: end stage renal disease. ATN: ac. Tubular necrosis

27. ATN: muddy brown casts
2727

28. • HUS:HUS: destroysdestroys lininglining of BV & RBCof BV & RBC; often c/by; often c/by E. coliE. coli;;
may get ARF or coagulopathymay get ARF or coagulopathy
• Alport Syn.:Alport Syn.: inherited. Hematuria, proteinuria. Moreinherited. Hematuria, proteinuria. More
serious in boys; leads to ESRD, hearing & visual lossserious in boys; leads to ESRD, hearing & visual loss
• PKD:PKD: inherited, AD: grape-like cysts in kidneys; destroyinherited, AD: grape-like cysts in kidneys; destroy
kidneys: CKD & ESRDkidneys: CKD & ESRD
HUS: hemolytic uremic syn. PKD: polycystic kidney DHUS: hemolytic uremic syn. PKD: polycystic kidney D
2828

29. • Interstitial Nephritis:Interstitial Nephritis: Inflam. of supporting tissue ofInflam. of supporting tissue of
kidney; can lead to ARF/ESRDkidney; 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: weak bones,kidneys fail to remove acids normally: weak bones,
kidney stones & FTTkidney stones & FTT
RTA: renal tubular acidosisRTA: renal tubular acidosis
2929

30. Acute Kidney InjuryAcute Kidney Injury ((AKIAKI) () (ARFARF))
abrupt loss of RF within 7d. Renal damage is due to low BFabrupt loss of RF within 7d. Renal damage is due to low BF
(low BP), renotoxins, inflam., or obs. of UT(low BP), renotoxins, inflam., or obs. of UT
• Dx.:Dx.: typical lab.: raised BUN & creatinine, or low UOPtypical lab.: raised BUN & creatinine, or low UOP
• Complications:Complications: m. acidosis, high K+, uremia, FE imbalance,m. acidosis, high K+, uremia, FE imbalance,
& effects on other systems, death. More risk of CKD& effects on other systems, death. More risk of CKD
• CausesCauses are numerous. Commonare numerous. Common
– SevereSevere dehydration, sdehydration, shock, ac.hock, ac. hgehge
– BlockageBlockage of renal BV,of renal BV, obstructionobstruction in UTin UT
– RenalRenal injuryinjury
– Ac.Ac. GN, aGN, acc. PN. PN
• Rx.: underlying cause & supportive like RRTRx.: underlying cause & supportive like RRT
3030

31. 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
3131

32. Causes of CKDCauses of CKD
• Primary: Primary: FSGS, MGN, MPGNFSGS, MGN, MPGN, crescentic GN,, crescentic GN,
Goodpasture syn.Goodpasture syn.
• Systemic: Systemic: DM, HTN, SLE, HSP, IgAN, APSGN, HIV, HBV, HCVDM, HTN, SLE, HSP, IgAN, APSGN, HIV, HBV, HCV
• Vascular: Vascular: Nephrosclerosis, ANCA, HUSNephrosclerosis, ANCA, HUS
• Hereditary: Hereditary: Amyloidosis, PKD, Alport syn.Amyloidosis, PKD, Alport syn.
• Tubulointerstitial: Tubulointerstitial: drugs/toxins, VUR, obs. uropathydrugs/toxins, VUR, obs. uropathy
ANCAs: Anti-neutrophil cytoplasmic AbANCAs: Anti-neutrophil cytoplasmic Ab:: mainly IgG, against neutrophil & monocytemainly IgG, against neutrophil & monocyte
cytoplasm. Seen in some AID. Particularly associated with systemic vasculitis (ANCAcytoplasm. Seen in some AID. Particularly associated with systemic vasculitis (ANCA
vasculitides)vasculitides)
3232

33. Urea frost in CRF
3333

34. Goodpasture Syn.Goodpasture Syn. ((anti-GBM d.)anti-GBM d.)
• AID: pulmo-renal syn.: anti-collagen Ab in lungs:AID: pulmo-renal syn.: anti-collagen Ab in 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: silentIgA deposits after URTI: silent
hematuria; may go for yrshematuria; may go for yrs
• Men more. All agesMen more. All ages
• No Rx if early/mild with normal BP & <1g 24TUP: if more,No Rx if early/mild with normal BP & <1g 24TUP: if more,
Rx. with ACEI or ARBsRx. with ACEI or ARBs
AID: autoim. d. GBM: glomerular basement membrane. RF: renal failure. TUP: total urinaryAID: autoim. d. GBM: glomerular basement membrane. RF: renal failure. TUP: total urinary
proteinprotein

35. Paroxysmal Noc. Hb.uria (PNH)Paroxysmal Noc. Hb.uria (PNH)
Rare.Rare. Acquired. Life-threatening d. characterizedAcquired. Life-threatening d. characterized
byby complement-induced IV hemolysiscomplement-induced IV hemolysis
• Some proteins cannot fix to RBCs CW to protect them fromSome proteins cannot fix to RBCs CW to protect them from
complements: hemolysis: Hb.emia & Hb.uria; atcomplements: hemolysis: Hb.emia & Hb.uria; at
night/early morningnight/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; easyRAP, backache, HA, SoB, clotting; dark urine; easy
bruisingbruising
3535
MDS:MDS: myelodysplastic syn. CW: cell wall. SoB: short of breathingmyelodysplastic syn. CW: cell wall. SoB: short of breathing

36. Investigations for PNHInvestigations for PNH
• Pancytopenia,Pancytopenia, Hb.emia & hb.uriaHb.emia & hb.uria
• Coombs' test; haptoglobin levelCoombs' test; haptoglobin level
• Flow cytometry to measure certain proteinsFlow cytometry to measure certain proteins
• Ham (acid hemolysin) testHam (acid hemolysin) test
• Sucrose hemolysis testSucrose hemolysis test
• Urine hemosiderinUrine hemosiderin
3636

37. Rx for PNHRx for PNH
• Steroids/immunosuppressantsSteroids/immunosuppressants
• BT. Iron & B9. Blood thinnersBT. Iron & 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. Death occurmost people survive >10 y after Dx. Death occur
from thrombosis or bleedingfrom thrombosis or bleeding
3737

38. Ac NephriticAc Nephritic
(Glomerulonephritic)(Glomerulonephritic) Syn.Syn.
• Ac. inflam. of the glomeruli & nephronsAc. inflam. of the glomeruli & nephrons
Nephrotic SyndromeNephrotic Syndrome
• Affection of nephrons with leakage ofAffection of nephrons with leakage of
protein (usually noprotein (usually no inflam.)inflam.)
3838

39. GlomerulonephritisGlomerulonephritis
• Inflam. & proliferation of Glomerular tissue with damage toInflam. & proliferation of Glomerular tissue with damage to
BM, mesangium/capillary endotheliumBM, mesangium/capillary endothelium
• Acute:Acute: hematuria, proteinuria & RBC casts. Often with HTN,hematuria, proteinuria & RBC casts. Often with HTN,
edema & impaired RFedema & impaired RF
• ChronicChronic: above with scarring of nephrons & progressive RF: above with scarring of nephrons & progressive RF
3939

40. Ac Nephritis: causesAc Nephritis: causes
– Group A Streptococcus Group A Streptococcus 80%80%
– OthersOthers 20%20%
• Systemic:Systemic: HSP,HSP, SLE, IgAN,SLE, IgAN, Goodpasture, gold,Goodpasture, gold,
penicillaminepenicillamine
• Infx.:Infx.: staph, pneumococci, Gram-ve, malaria, HBV,staph, pneumococci, Gram-ve, malaria, HBV,
HCV, MMR, HIVHCV, MMR, HIV
• Infective endocarditisInfective endocarditis
• Renal d:Renal d: MGN, MPGN, FSGS, etc.MGN, MPGN, FSGS, etc.
4040

41. A.P.S.G.N.A.P.S.G.N.
• 15% of all GAS infx.; mostly RTI (skin 10%)15% of all GAS infx.; mostly RTI (skin 10%)
• Lag period: 2-3wLag period: 2-3w
• 2% clinically overt2% clinically overt
• No recurrenceNo recurrence
• Any AgeAny Age (2-15y; 2% <2y; 10% >40y)(2-15y; 2% <2y; 10% >40y)
• Boys moreBoys more
• Excellent prognosisExcellent prognosis:: <2% MR. 2% Chr. GN<2% MR. 2% Chr. GN
• Cerebral vasculitis may occurCerebral vasculitis may occur
GAS: group A streptococciGAS: group A streptococci
4141

42. PathophysiologyPathophysiology
 Exact mechanism is unclearExact mechanism is unclear
 Autoim. d:Autoim. d: both CMI & humoral.both CMI & humoral. Immune complexImmune complex deposits indeposits in
glomeruli, activates complement: inflam.glomeruli, activates complement: inflam.
 Strep. itself Strep. itself does not attack the kidneydoes not attack the kidney
 Kidneys may enlarge ~50%Kidneys may enlarge ~50%
 Histology:Histology: swelling of glomeruli, polymorphs infiltrateswelling of glomeruli, polymorphs infiltrate
IF:IF: deposition of Ig & complementdeposition of Ig & complement
4242

43. 4343

44. CFCF of APSGNof APSGN
 Acute:Acute: H/o earlier URTI, skin infx. a few weeks beforeH/o earlier URTI, skin infx. a few weeks before
 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)
HC: high coloredHC: high colored
4444

45. Lab InvestigationsLab Investigations
• UrineUrine:: RBCs, RBC casts, WBC, +/++ proteinRBCs, RBC casts, WBC, +/++ protein
• FBC:FBC: dilutional anemia, leucocytosisdilutional anemia, leucocytosis
• Evidence of recent strep. infEvidence of recent strep. inf.: ASO titer,.: ASO titer, Anti-Dnase B,Anti-Dnase B,
throat/wound CSthroat/wound CS
• ?Elevated urea?Elevated urea ±± creatininecreatinine
• Low complement C3Low complement C3
Anti-Dnase B: Ab made against GAS. Raised levels indicate: Rh. F,
PSGN, Strep. throat or Strep. skin infection
4545

46. Renal BiopsyRenal Biopsy
• Declining Renal FunctionDeclining Renal Function
• Atypical presentationAtypical presentation
• F/history of renal DF/history of renal D
• Persistent HTN or gross hematuriaPersistent HTN or gross hematuria
• HypocomplementemiaHypocomplementemia
Hallmark in PSGNHallmark in PSGN is subepithelial ‘humps’is subepithelial ‘humps’
representing immune complex depositionrepresenting immune complex deposition
4646

47. 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 inf.HUS, other inf.
4747

48. Rx Of APSGNRx Of APSGN
Mainly supportive.Mainly supportive. Bed restBed rest
• Fluid & salt restriction.Fluid & salt restriction. FEBFEB
• Rx of hyperkalemia.Rx of hyperkalemia. No fruits!No fruits!
• Penicillin x 10d: why?Penicillin x 10d: why?
• BP control. ACEI can cause hyperkalemiaBP control. ACEI can cause hyperkalemia
• Rx of complicationsRx of complications
• Admit if renal failureAdmit if renal failure
4848

49. Complications of AGNComplications of AGN
• ARF (uremia/azotemia)ARF (uremia/azotemia)
• Volume over-load: HTN: LVFVolume over-load: HTN: LVF
• HTN: encephalopathy, convulsionHTN: encephalopathy, convulsion
• HyperkalemiaHyperkalemia
• AcidosisAcidosis
• Microhematuria may persist for yearsMicrohematuria may persist for years
• NSNS
• CGN: 2%CGN: 2%
4949

50. APSGN: PrognosisAPSGN: Prognosis
• Excellent. Most recover completelyExcellent. Most recover completely
• 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
5050

51. SS of Glomerular DiseasesSS of Glomerular Diseases
• May be silent for many yearsMay be silent for many years
• Hematuria, proteinuria, azotemiaHematuria, proteinuria, azotemia
• HTN, edema, hyperlipidemiaHTN, edema, hyperlipidemia
Some CRF can be slowed down, but scarredSome CRF can be slowed down, but scarred
glomeruli cannot be repairedglomeruli cannot be repaired
5151

52. GlomerularGlomerular vsvs Non-G Hematuria?Non-G Hematuria?
• Chemical trauma to RBCs as they pass through nephronsChemical trauma to RBCs as they pass through nephrons
causes peculiar changes: they lose biconcavity & havecauses peculiar changes: they lose biconcavity & have
blebs:blebs: “Mickey Mouse Cells”“Mickey Mouse Cells”
• RBC casts & proteinuria supports a GDRBC casts & proteinuria supports a GD
5252

53. Mickey mouse cells
5353

54. 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
5454

55. A 3y old child has heavy proteinuria with anasarca. NoA 3y old child has heavy proteinuria with anasarca. No
familial KD. No drugs. Wt 17 kg, BP 90/50; 4+ edemafamilial KD. No drugs. Wt 17 kg, 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?
5555

56. Ans.: Nephrotic SyndromeAns.: Nephrotic Syndrome
• Massive proteinuria >3.5g/d (>40mg/mMassive proteinuria >3.5g/d (>40mg/m22
/hr)/hr)
• Hypoalbuminemia: <30g/dlHypoalbuminemia: <30g/dl
• AnasarcaAnasarca
• HyperlipidemiaHyperlipidemia
• LipiduriaLipiduria
Age: 1½ - 5yAge: 1½ - 5y
Boys moreBoys more
5656

57. 5757

58. PeculiaritiesPeculiarities of Childhood NSof Childhood NS
• Most cases: no inflammation/RFMost cases: no inflammation/RF
• Most respond to steroidMost respond to steroid
• Well for 3y: no more relapseWell for 3y: no more relapse
• No relapse after 15yoaNo relapse after 15yoa
• Auto-remission 5%Auto-remission 5%
5858

59. Spot urineSpot urine
Urinary albumin:creatinineUrinary albumin: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
5959

60. ClassificationClassification
CongenitalCongenital
Acquired:Acquired:
Primary:Primary: MCNS/MCD (MCNS/MCD (85% of NS in children)85% of NS in children)
Secondary:Secondary:
Infection: HBV, HCV, malariaInfection: HBV, HCV, malaria
SLE, HSP, SCD, PAN, HTN, DMSLE, HSP, SCD, PAN, HTN, DM
amyloidosis, malignancyamyloidosis, malignancy
gold, penicillamine, Hg, Heavy metalgold, penicillamine, Hg, Heavy metal

61. NS of childhoodNS of childhood
MCD: 85%
FSGS: 10%
Others: 5%
membranoproliferative
GN, mesangiocapillary
GN, diffuse proliferative GN,
congenital
6161

62. EpidemiologyEpidemiology
• Incidence: 2-7/10,000/yIncidence: 2-7/10,000/y
• x15 common in childrenx15 common in children
• Non-immuneNon-immune factors in MCD & FSGSfactors in MCD & FSGS
• Immune factors in MPGN, PSGN & SLEImmune factors in MPGN, PSGN & SLE
• Age of onset varies with type of diseaseAge of onset varies with type of disease
6262

63. Filtration barrier.Filtration barrier. A.A. The endothelium with fenestraThe endothelium with fenestra
B.B. GBM: 1. lamina interna 2. L. densa 3. L. externaGBM: 1. lamina interna 2. L. densa 3. L. externa
C.C. Podocytes: 1. enzymatic & structural protein 2. filtration slit 3.Podocytes: 1. enzymatic & structural protein 2. filtration slit 3.
diaphragmdiaphragm 6363

64. • Steroid sensitiveSteroid sensitive (90%)(90%)
• Steroid resistant:Steroid resistant: no response in 4w (10%)no response in 4w (10%)
• SteroidSteroid dependent:dependent: relapse on 2 consecutiverelapse on 2 consecutive
occasions as steroid is being tapered or within 2w ofoccasions as steroid is being tapered or within 2w of
being discontinuedbeing discontinued
• Remission:Remission: nil protein in morning urine x3dnil protein in morning urine x3d
• Relapse:Relapse: U. Protein: >40/m2/h or Albustix ≥++ x 3dU. Protein: >40/m2/h or Albustix ≥++ x 3d
morning urine.morning urine. Frequent RFrequent R: ≥2 in 6mo of Dx.: ≥2 in 6mo of Dx. oror ≥≥4/y.4/y.
Infrequent RInfrequent R: after 3 mo of remission: after 3 mo of remission
6464

65. MCDMCD
• Commonest 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
6565

66. Focal Segmental GlomerulosclerosisFocal Segmental Glomerulosclerosis (FSGS) &(FSGS) &
Membranoproliferative GNMembranoproliferative GN (MPGN)(MPGN)
• In 15% of childhood NS, a kidney biopsy showsIn 15% of childhood NS, a kidney biopsy shows
scarring or deposits in glomeruliscarring or deposits in glomeruli
• Steroid is less effective in these; need cytotoxicsSteroid is less effective in these; need cytotoxics
• ACEI can decrease HTN & proteinuria & protectACEI can decrease HTN & proteinuria & protect
kidneyskidneys
6666

67. 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: depressiondepression, lethargy, anorexia, skin striae,, lethargy, anorexia, skin striae,
diarrhea, AP, orthostatic hypotensiondiarrhea, AP, orthostatic hypotension
Bedside urine: heavy proteinuriaBedside urine: heavy proteinuria
6767

68. 6868

69. 6969

70. 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 7070

71. Renal BiopsyRenal Biopsy
RarelyRarely done in Paediatric cases.done in Paediatric cases. Consider in:Consider in:
• Cong. NSCong. NS
• >8y at onset>8y at onset
• Steroid resistanceSteroid resistance
• Frequent relapsesFrequent relapses
• Significant nephritic featuresSignificant nephritic features
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72. ComplicationsComplications
• Infection:Infection: loss of Ig, complement: UTI, SBP (commonest)loss of Ig, complement: UTI, SBP (commonest)
& pneumonia& pneumonia (pneumococcus)(pneumococcus)
• Thrombosis:Thrombosis: loss of antithrombin iii, antiplasmin &loss of antithrombin iii, antiplasmin &
proteins S & C in urine, more coagulants by liver,proteins S & C in urine, more coagulants by liver,
raised hct., relative immobility, steroidraised hct., relative immobility, steroid
• Hypovolemia:Hypovolemia: postural hypotensionpostural hypotension
• From Drug toxicity:From Drug toxicity: steroid, nephrotoxcity fromsteroid, nephrotoxcity from
cyclosporin A or tacrolimuscyclosporin A or tacrolimus
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73. Management: GeneralManagement: General
• CheckCheck BP, wt, abdo. girth, I.O. chart, proteinuriaBP, wt, abdo. girth, I.O. chart, proteinuria
• Bed rest in gross edemaBed rest in gross edema
• Diet: lean proteinDiet: lean protein (no role of excess protein),(no role of excess protein), low fat;low fat;
low-saltlow-salt
• Salt & fluid restriction. UsuallySalt & fluid restriction. Usually no diureticno diuretic
• Hypovolemia & hypoalbuminemia: FFP 20ml/kg or saltHypovolemia & hypoalbuminemia: FFP 20ml/kg or salt
poor albumin 20%poor albumin 20%
• Anticoagulants can help decrease clottingAnticoagulants can help decrease clotting
• Statins can help lower cholesterolStatins can help lower cholesterol
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74. Management: SpecificManagement: Specific
Objective:Objective: Rx underlying causeRx underlying cause
• MCD: up to 8y age: no renal biopsyMCD: up to 8y age: no renal biopsy
• Prednisolone 60mg/m2/d (not >80mg/d)Prednisolone 60mg/m2/d (not >80mg/d) x 6wx 6w, then, then
40mg/m2/d EAD for 6w40mg/m2/d EAD for 6w then STOPthen STOP
• Exclude TB, HBV, HCV or other infectionExclude TB, HBV, HCV or other infectionss
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75. RelapseRelapse
• First 2:First 2: “treat same way”“treat same way”
• Frequent R:Frequent R: keep steroid 0.5mg/kg EAD for 3-6 mo. Ifkeep steroid 0.5mg/kg EAD for 3-6 mo. If
relapse: Levamisole EAD for 4-12 morelapse: Levamisole EAD for 4-12 mo
• If still R:If still R: Cyclophosphamide x 8w plus Low DoseCyclophosphamide x 8w plus Low Dose
PrednisolonePrednisolone
• Still rStill relapse:elapse: Cyclosporin A for 1y plus LD Pred.Cyclosporin A for 1y plus LD Pred.
Other drugs: Tacrolimus, MycophenolateOther drugs: Tacrolimus, Mycophenolate
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76. SRNSSRNS
• Refer to specialized unitRefer to specialized unit
• Full remission not achievedFull remission not achieved
• Aim: lower proteinuria to non-nephrotic rangeAim: lower proteinuria to non-nephrotic range
• Risk of HTN & renal failureRisk of HTN & renal failure
• In FSGS: 20-40% risk of relapse post transplantIn FSGS: 20-40% risk of relapse post transplant
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77. SSNSSSNS
• Toddler, pre-school
• No HTN
• Mild, intermittent
hematuria
• Normal renal function
• Excellent prognosis,
even if frequently
relapsing
• No biopsy
SRNSSRNS
• <1 year, > 8y
• HTN common
• Persistent haematuria
• Often abnormal RF
• Long term HTN & RF
• Biopsy needed: usual
histology FSGS
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78. Congenital NSCongenital NS
• First 3 mo of life. Large placenta: ~ 40% of BWFirst 3 mo of life. Large placenta: ~ 40% of BW
• Drug resistant. High morbidity: PEM & sepsisDrug resistant. High morbidity: PEM & sepsis
• Types:Types: Finnish type:Finnish type: most severe, AR.most severe, AR. Diffuse mesangialDiffuse mesangial
sclerosis:sclerosis: less severe, AR.less severe, AR. Denys-Drash syn.:Denys-Drash syn.:
pseudohermaphroditism & Wilms T.pseudohermaphroditism & Wilms T. FSGS.FSGS. Secondary CNS:Secondary CNS:
cong. syphiliscong. syphilis
• Rx.:Rx.: Intensive care: 20% albumin, nutrition, early unilateralIntensive care: 20% albumin, nutrition, early unilateral
nephrectomy, RRTnephrectomy, RRT
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79. RxRx of Hypertension in NSof Hypertension in NS
• ACEI reduce BP & proteinuriaACEI reduce BP & proteinuria
• NifedipineNifedipine 0.25mg/kg/dose s.l.; max 8 doses/d (not0.25mg/kg/dose s.l.; max 8 doses/d (not
>2mg/kg/d or>2mg/kg/d or
• HydralazineHydralazine 0.5-2mg/kg/d)0.5-2mg/kg/d)
• Others:Others: Atenolol, MethyldopaAtenolol, Methyldopa
• DiureticDiuretic isis ccontroversial. Use with caution. May beontroversial. Use with caution. May be
dangerous indangerous in hypovolemiahypovolemia
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80. Immunization in NSImmunization in NS
Immunocompromized:Immunocompromized: steroid 2mg/kg/d or 20mg/kg/d xsteroid 2mg/kg/d or 20mg/kg/d x
14d14d
• No live vax.No live vax.
• Killed vax./toxoids are safeKilled vax./toxoids are safe
• Live vax. after 4w of stopping steroidLive vax. after 4w of stopping steroid
• VZIG in case of exposureVZIG in case of exposure
• Ig in case of measles expo. or cl. measlesIg in case of measles expo. or cl. measles
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81. NSNS APSGNAPSGN
H/oH/o NilNil Preceding Strep.Preceding Strep.
Inf.Inf.
AgeAge 2-6y2-6y 5-15y5-15y
EdemaEdema MassiveMassive Mild-moderateMild-moderate
Urine colorUrine color ClearClear Coca-cola coloredCoca-cola colored
SedimentSediment NilNil Red coloredRed colored
ProteinProtein 4+4+ 1-2+1-2+
MicroscopyMicroscopy ClearClear Plenty RBCs, PCPlenty RBCs, PC
CastsCasts HyalineHyaline RBC castsRBC casts
Serum albuminSerum albumin Below 25g/dlBelow 25g/dl NormalNormal 8181

82. PrognosisPrognosis::
in iin idiopathic NS of childhood is excellentdiopathic NS of childhood is excellent
MortalityMortality 1-2%1-2%
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83. 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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84. MCQMCQ
• MCD is common after 8yoaMCD is common after 8yoa
• NS Dx always needs renal biopsyNS Dx always needs renal biopsy
• Hematuria is common in childhood NSHematuria is common in childhood NS
• Levamisole is effective in relapse NSLevamisole is effective in relapse NS
• Usually there is renal failure in NSUsually there is renal failure in NS
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87. NextNext:: ACCIDENTS INACCIDENTS IN
CHILDRENCHILDREN

88. Taiwan Blue Magpie (Urocissa caerulea)

89. ThankThank
YouYou
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