Agn ns

Rural Bangladesh: mature rice field

WELCOME

ALL 66

RENAL RENAL DIASEASESDIASEASES

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dr m nurul huqdr m nurul huqBangladesh Bangladesh Medical CollegeBangladesh Bangladesh Medical College

At the end… you will learnAt the end… you will learn

• Kidney disease can be Kidney disease can be a a 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

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Summary: Renal Function

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Importance of kidneyImportance of kidney

• Main Main waste excreterwaste excreter• Maintains Maintains fluid- and ABBfluid- and ABB• Makes Makes erythropoietinerythropoietin• Excretes some Excretes some drugsdrugs• Biotransforms Biotransforms VDVD

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Peculiarities of Kidney DiseasesPeculiarities of Kidney Diseases

• May be asymptomatic May be asymptomatic • Symptoms can be Symptoms can be nonspecific nonspecific • Few Few physical signsphysical signs• May present with May present with jaundice jaundice in infantsin infants• Important c/of Important c/of FTTFTT• Long UT means more Long UT means more obstructionobstruction

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Micros. H:Micros. H: in a well child: ~ in a well child: ~ no Ix.no Ix. if if not x3/over several mo.; evaluate if not x3/over several mo.; evaluate if HTN, CKD, casturia/ proteinuria HTN, CKD, casturia/ proteinuria presentpresent

Gross H: Gross H: urine is red/tea/cola urine is red/tea/cola colored; also mostly benigncolored; also mostly benign

Up to 5 rbc per HPF in urine Up to 5 rbc per HPF in urine is normal in childrenis normal in children

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GH is more in GH is more in

boys. Assess by boys. Assess by

CF. VCUG is CF. VCUG is

useful in useful in

doubtful USG, doubtful USG,

UTI, or voiding UTI, or voiding

problem. problem.

Cystoscopy if Cystoscopy if

persistent or persistent or

with ambiguous with ambiguous

imagingimagingVCUG : voiding VCUG : voiding

cystourethrographycystourethrography 2020

A Case History: hematuriaA Case History: hematuria

A 12y-boy has hematuria. He has occasional dark A 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; no He 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?

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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 no Glomerular d. causes <10% of hematuria in pts. with no proteinuriaproteinuria

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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

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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; may cells supporting glomeruli: phagocytic; may contain molecules that may aid in Dxcontain molecules that may aid in Dx

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• ARF: ARF: Ac. Renal Failure Ac. Renal Failure (hours-days)(hours-days)

• ESRD: ESRD: Total CRF. Needs dialysis/transplant Total 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 brown Muddy brown casts" casts" (epith. cells) is (epith. cells) is pathognomonicpathognomonic

• ESWLESWL (Extracorpo. Shockwave Lithotripsy):(Extracorpo. Shockwave Lithotripsy): to to break kidney stonesbreak kidney stones

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ATN: muddy brown casts

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Causes of ARFCauses of ARF

• Severe Severe dehydrationdehydration• Shock, ac. Shock, ac. hgehge• BlockageBlockage of renal BV, o of renal BV, obstructionbstruction in UT in UT• Renal Renal injuryinjury• Ac. Ac. GN, aGN, acc. PN. PN

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• HUS:HUS: destroys destroys lininglining of BV of BV and and RBCRBC; often c/by ; often c/by E. coliE. coli; ; may get ARF or coagulopathymay 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; destroy inherited: 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

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• Interstitial Nephritis: Interstitial Nephritis: Inflam. of supporting tissue Inflam. 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: weak kidneys fail to remove acids normally: weak bones, kidney stones and FTTbones, kidney stones and FTT

RTA: renal tubular acidosisRTA: renal tubular acidosis

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CKD (CRF): CKD (CRF): GFR <90ml/min/1.73mGFR <90ml/min/1.73m2 2 >3 mo>3 mo

5 stages5 stages• GFR 90ml/min/1.73m2 GFR 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

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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 Ab3434

Urea frost in CRF

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Goodpasture Syn. Goodpasture Syn. ((anti-GBM d)anti-GBM d)• Autoim. pulmo-renal syn.: anti-collagen ABs in Autoim. pulmo-renal syn.: anti-collagen ABs in

lungs: lungs: vasculitis: vasculitis: hge., kidneys: hge., kidneys: GN (anti-GBM ABs)GN (anti-GBM ABs)• It is It 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 ages Men more. All ages

• No Rx for early/mild IgAN with normal BP and <1g No 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

Paroxysmal Noc. Hb.uria (PNH)Paroxysmal Noc. Hb.uria (PNH)

Rare. Acquired. Some proteins cannot fix to RBCs to Rare. Acquired. Some proteins cannot fix to RBCs to protect from complement: hemolysis: Hb.emia and protect from complement: hemolysis: Hb.emia and Hb.uria; at night/early morningHb.uria; at night/early morning

• Any age. May cause Any 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

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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

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Rx for PNHRx for PNH• Steroids/immunosuppressantsSteroids/immunosuppressants• BT. Iron and B9. Blood thinnersBT. Iron and B9. Blood thinners• Eculizumab Eculizumab 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 most people survive >10 y after Dx. Death occur from thrombosis or bleedingoccur from thrombosis or bleeding

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GlomerulonephritisGlomerulonephritis

• Inflam. and proliferation of Glo. tissue with damage Inflam. 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. Often hematuria, 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

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Ac Nephritic Ac 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 of protein Affection of nephrons with leakage of protein

(usually no(usually no inflam.) inflam.)

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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

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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 Age Any 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

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PathophysiologyPathophysiology Exact mechanism is unclear. Autoim. D: both CMI Exact mechanism is unclear. Autoim. D: both CMI and humoral. and humoral. Immune complex Immune complex in glomeruli: activates in 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

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CF of APSGNCF of APSGN

Abrupt: Abrupt: H/o earlier URTI, skin infx. a few weeks H/o earlier URTI, skin infx. a few weeks before (latent period)before (latent period)

Puffy face, scanty HC/Puffy face, scanty HC/coca colored coca 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)

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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 urea Elevated urea ±± creatinine creatinine• Low complement C3Low complement C3

Anti-Dnase B: AB made by GAS. Raised levels indicate: Rh. F, PSGN, Strep. throat or Strep. skin infection

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Renal BiopsyRenal Biopsy

• Declining RFDeclining RF• Atypical presentationAtypical presentation• F/history of renal DF/history of renal D• Persistent HTN or gross hematuria and -Persistent HTN or gross hematuria and -

hypocomplementemiahypocomplementemia• Hallmark in PSGN Hallmark in PSGN is subepithelial ‘humps’ is subepithelial ‘humps’

representing immune complex depositionrepresenting immune complex deposition

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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 normal C3 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.

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Henoch-Schonlein purpuraHenoch-Schonlein purpura

• 70% affect kidneys: MC hematuria +/- proteinuria70% affect kidneys: MC hematuria +/- proteinuria• Histologically Histologically 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

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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

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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

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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

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S/o Glomerular Diseases S/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 glomerular GN and G. sclerosis include many forms of glomerular damage. Some RF can be slowed down, but scarred damage. Some RF can be slowed down, but scarred glomeruli cannot be repairedglomeruli cannot be repaired

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Glomerular Glomerular vsvs Non-G Hematuria? Non-G Hematuria?

• Chemical trauma to RBC as they pass through Chemical trauma to RBC as they pass through nephrons causes peculiar changes: they lose nephrons 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

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Mickey mouse cells

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ProteinuriaProteinuria

Normal valuesNormal values

– Premature: Premature: 140 mg/m 140 mg/m22/d/d– FTFT 70 mg/m 70 mg/m22/d/d– Children <10yr Children <10yr 150 mg/d 150 mg/d– Children 10-18 yrChildren 10-18 yr 300 mg/d 300 mg/d– Adults Adults 150 mg/d 150 mg/d

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Case history: Case history: A 3y old child has heavy proteinuria with A 3y old child has heavy proteinuria with anasarca. No familial KD. He is not on any drugs. Wt 17 kg anasarca. 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 lipid Urine: 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?

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Glomerular vs. Tubular ProteinuriaGlomerular vs. Tubular Proteinuria

Traits Traits 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 peak6060

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

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PeculiaritiesPeculiarities of Childhood NS of 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%

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Spot urineSpot urine

Urinary protein/creatinine Urinary protein/creatinine >200>200Significant proteinuria: Significant proteinuria: >4mg/m>4mg/m22/h/hHeavy proteinuria: Heavy proteinuria: >40mg ,,>40mg ,,Remission:Remission: <4mg/m2/h<4mg/m2/h

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NS of childhood: classificationNS of childhood: classification

MCD: 85%FSGS: 10%Others: 5%(membranoproliferativeGN, mesangiocapillary GN, diffuse proliferative GN), congenital

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• 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

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PathophysiologyPathophysiology• Non-immuneNon-immune factors in MCD and FSGS factors in MCD and FSGS• Immune factors in MPGN, PSGN and SLEImmune factors in MPGN, PSGN and SLE• Mutations in podocyte or slit diaphragm in Mutations 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

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Filtration barrier. A. The endothelium with fenestraB. GBM: 1. lamina interna 2. L. densa 3. L. externaC. Podocytes: 1. enzymatic and structural protein 2. filtration slit 3. diaphragma 6868

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

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Focal Segmental Glomerulosclerosis Focal Segmental Glomerulosclerosis (FSGS) (FSGS) and and

Membranoproliferative GN Membranoproliferative GN (MPGN)(MPGN)

• In about 15% of childhood NS, a kidney biopsy In 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 protect ACEI can decrease HTN and proteinuria and protect

kidneyskidneys

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C/FC/F

• M: F =2:1M: F =2:1• Gross edema, scanty Gross edema, scanty

urine, SoBurine, SoB• There may be an There 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 hypotensionBedside urine: heavy proteinuriaBedside urine: heavy proteinuria

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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

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

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ComplicationsComplications

• Infection: Infection: loss of Ig, complement: UTI, SBP loss of Ig, complement: UTI, SBP (commonest) and pneumonia (commonest) and pneumonia (pneumococcus)(pneumococcus)

• Thrombosis: Thrombosis: loss of anti thrombin iii, antiplasmin loss of anti thrombin iii, antiplasmin and proteins S&C in urine, more coagulants by and 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 from steroid, nephrotoxcity from

cyclosporin A or tacrolimuscyclosporin A or tacrolimus

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Management: generalManagement: general

• Check Check 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. Usually Salt and fluid restriction. Usually no diureticno diuretic• Hypovolemia and hypoalbuminemia: FFP 20ml/kg Hypovolemia 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

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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/d Prednisolone 60mg/m2/d x 4 wx 4 w, then , then 40mg/m2/d 40mg/m2/d

EAD for 4w EAD for 4w then STOPthen STOP

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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 Dose Cyclophosphamide 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

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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

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DDDDSSNSSSNS

• 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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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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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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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. measles8484

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 8585

Prognosis:

in idiopathic NS of childhood is excellentMortality 1-2%

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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 of Hyperkalemia is a recognized complication of

APSGNAPSGN

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ThankThank

You You8989

Agn ns

Health & Medicine

Transcript of Agn ns