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Nephrotic syndrome in children. for under graduates

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Page 1: Nephrotic syndrome in children. for under graduates
Page 2: Nephrotic syndrome in children. for under graduates

Dr. Muhammad Sajjad SabirMBBS, DCH, MCPS, FCPS

Assistant Professor of Paediatrics

Page 3: Nephrotic syndrome in children. for under graduates

Manifestation of glomerular disease, characterized by nephrotic range proteinuria and a triad of clinical findings associated with large urinary losses of protein : hypoalbuminaemia , edema and hyperlipidemia

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EdemaHeavy proteinuria > 40mg/m2/hrHypoalbuminemia <2.5g/dlHyperlipidema >250mg/dl

Page 5: Nephrotic syndrome in children. for under graduates

15 times more common in children than adults

2 – 7 cases per 100,000 children per year (Global)

Incidence South Asia 16/100,000 children

Most common= 1.5-6 year

boys : girls--- 2:1 ratio

Page 6: Nephrotic syndrome in children. for under graduates

Defined as protein excretion of > 40 mg/m2/hr First morning protein : creatinine ratio of > 2-3 : 1

- Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1801

Page 7: Nephrotic syndrome in children. for under graduates

Idiopathic or Primary GeneticSecondary

Page 8: Nephrotic syndrome in children. for under graduates

Minimal Change disease ( >80 % ) Mesangial proliferation Focal segmental Glomerulosclerosis Membranous Nephropathy Membranoproliferative glomerulonephritis

- Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1804

Page 9: Nephrotic syndrome in children. for under graduates

Finnish type Congenital Nephrotic SyndromeFocal Segmental GlomerulosclerosisDiffuse Mesangial SclerosisDenys-Drash Syndrome

- Nelson Textbook of Paediatrics, Vol 2, 19th edition, page 1802, table 521-1

Page 10: Nephrotic syndrome in children. for under graduates

Congenital Oligomeganephronia

Infectious Hepatitis (B,C) , HIV-1, Malaria, Syphilis, Toxoplasmosis

Inflammatory Glomerulonephritis

Immunological Castleman Disease, Kimura Disease, Bee sting, Food allergens

Neoplastic Lymphoma, Leukemia

Traumatic ( Drug induced ) Penicillamine, Gold, NSAIDS, Pamidronate, Mercury, Lithium

Page 11: Nephrotic syndrome in children. for under graduates

Permeability of glom.cap.memb. Proteinuria

Intravascular vol

ADH Renal perfusionpressure

WaterReabsorptnInCollectingducts

Actv. reininAng. ald. sys

Tubular reabsorp.Of Na

Hypoalbuminemia

Hepatic protein synthesis Plasma oncoticpressure

Hyperlipidemia Transudation of fluidfrom intravascularcomp. To interstialspace

Edema

Page 12: Nephrotic syndrome in children. for under graduates

Preceding flu-like illnessGeneral health (anorexia, weight gain ,lethargy)Edema Urinary symptoms(hematuria, oliguria) Infection, diarrhea, abd. painDrug intakePast history

Page 13: Nephrotic syndrome in children. for under graduates

Edema Mild to start with – peri orbital puffiness, lower

extremities Progression to generalized edema, ascites, pleural

effusion, genital edemaDecreased urine output Anorexia, Irritability, Abdominal pain and diarrhoea Absence of Hypertension Gross hematuria Vital & BP Height & weight for age Anemia

- Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1802

Clinical Features-Examination

Page 14: Nephrotic syndrome in children. for under graduates

CLINICAL FEATURES Minimal Change Nephrotic Syndrome

Focal Segmental Glomerulosclerosis

Membranous Nephropathy

Age ( yr ) 2 - 6 2 - 10 40 - 50

Sex ( M : F ) 2 : 1 1.3 : 1 2 : 1

Nephrotic Syndrome

100 % 90 % 80 %

Asymptomatic proteinuria

0 10 % 20 %

Hematuria 10 – 20 % 60 – 80 % 60 %

Hypertension 10 % 20 % early infrequent

Rate of progression to renal failure

Non progressive

10 yrs 50 % in 10 – 20 yrs

Associated Conditions

Usually none None Renal vein thrombosis, SLE, Hepatitis B

Page 15: Nephrotic syndrome in children. for under graduates

Periorbital puffiness

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Protein losing enteropathy Hepatic failure Heart failure Acute/Chronic Glomerulonephritis Protein Malnutrition• < 1 year old• Family history of nephrotic Syndrome• Hypertension• Pulmonary edema• Gross hematuria• Extrarenal findings

• < 1 year old• Family history of nephrotic Syndrome• Hypertension• Pulmonary edema• Gross hematuria• Extrarenal findings

Page 17: Nephrotic syndrome in children. for under graduates

URINE ANALYSIS PROTEINURIA: 3+ Or 4+ MICROSCOPIC HEMATURIA: 20% PUS CELLS: underlying UTI CELLULAR CASTS: not in minimal

change disease 24HRS URINARY PROTEIN

EXCRETION: Children : >40mg/m2/hr

URINARY spot PROTEIN : CREATININE > 2.0 (Spot UPC ratio > 2.0)

trace /nil (10-20mg/dl) + (30mg/dl) ++ (100mg/dl)+++(300mg/dl)++++(1000-2000mg/dl)

Page 18: Nephrotic syndrome in children. for under graduates

SERUM:S. CREATININE: NormalS. CHOLESTROL: ElevatedS. ALBUMIN: <2.5g/dlC3 & C4: NormalTOTAL CALCIUM: Decreased

Page 19: Nephrotic syndrome in children. for under graduates

VITRAL SEROLOGY: HBV associated with membranous nephritis

BLOOD COUNTS: Hb, TLC & DLC Normal ESR raised

X-RAY CHEST: R/O pulmonary TB R/O pleural effusion

Page 20: Nephrotic syndrome in children. for under graduates

MANTOUX TEST: R/O TB before starting steroids

RENAL BIOPSY ANA: R/O SLE

• Age below 12 months• Gross or persistent microscopic hematuria• Low blood C3• Hypertension• Impaired renal Function• Failure of steroid therapy

Indications for Renal biopsyIndications for Renal biopsy

Page 21: Nephrotic syndrome in children. for under graduates

Other forms of glomerulonephritis including post streptococcal glomerulonephritis

Pyelonephritis Obstructive Uropathies Hemolytic Uremic Syndrome Fever, Exercise, Orthostatic protein urea Renal Failure Congestive cardiac failure Liver failure

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Management

Page 23: Nephrotic syndrome in children. for under graduates

DIETARY ADVICE: A balanced diet adequate in proteins and

calories is recommended foods high in sodium avoided High protein diet Edema no added salt

Treatment of infections Parent Education Can attend school Can participate in physical activities as tolerated

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If significant edema Diuretics + Aldosterone antagonist ( Fursemide, spironolactone ) Salt restriction DIURETICS INDICATIONS: Severe symptomatic edema Steroid toxicity or steroid contraindicated

Q.Best diuretic in Nephrotic Syndrome?

Page 25: Nephrotic syndrome in children. for under graduates

ROLE OF INTRAVENOUS ALBUMIN

INDICATIONS: Signs of hypovolemia Sever oedema

DOSAGE & ADMINISTRATION: I/V salt poor 25% albumin infusion 0.5-1 gm/kg/doze over 6-12 hrs followed

by Frusemide 1-2 mg/kg/dose (I/V)

Page 26: Nephrotic syndrome in children. for under graduates

CORTICOSTEROID THERAPY: DOSAGE & ADMINISTRATION:(after a -ve PPD test)

Prednisolone 60mg/m2 /day (max 80mg) single daily dose {or 2-3 dd} for 6 wks consecutively

After the initial 6-wk course, prednisone dose tapered to 40 mg/m2/day given every other day as a single daily dose for at least 4 wk.

Alternate-day dose then slowly tapered→discontinued over next 1-2 mo

Page 27: Nephrotic syndrome in children. for under graduates

REPONSE TO STEROID:80-90% of children respond within 3 wk

10% respond by first week 70% by second week 85% by third week 92% by forth week

Response means clinical remission, diuresis, and urine trace or negative for protein for 3 consecutive days

Who respond to prednisone therapy do so within the first 5 wk of treatment.

Page 28: Nephrotic syndrome in children. for under graduates

STEROID DEPENDENT: Patients who relapse while on alternate-day steroid therapy or within 28 days of completing a successful course of prednisone therapy

FREQUENT RELAPSERS: Patients who respond well to prednisone therapy but relapse ≥4 times in a 12-mo period

INFREQUENT RELAPSERS :3 or less relapses per yr

STEROID RESISTANT: Fail to respond to corticosteroid therapy within 8 wks

Children who continue to have proteinuria (2+ or greater) Diagnostic renal biopsy should be performed

Page 29: Nephrotic syndrome in children. for under graduates

Relapses should be treated with 60 mg/m2/day (80 mg daily max) in a single am dose until the child enters remission (urine trace or negative for protein for 3 consecutive days)

The prednisone dose is then changed to alternate-day dosing as noted with initial therapy, and gradually tapered over 4-8 wk.

Ghai Essential Paediatrics,8th edition, page 479

Page 30: Nephrotic syndrome in children. for under graduates

ALTERNATIVE THERAPY: INDICATIONS: steroid dependent frequent relapsers steroid responsive unwanted effects of steroids

Page 31: Nephrotic syndrome in children. for under graduates

Alternate Day prednisoloneSteroid sparing agents

Levamisole ( 2 – 2.5 mg/kg ) Cyclophosphamide ( 2 – 2.5 mg/kg/day) Mycophenolate Mofetil ( 20 – 25 mg/kg/day ) Cyclosporin ( 4 – 5 mg/kg/day ) Tacrolimus (0.1 – 0.2 mg/kg/day ) Rituximab ( 375mg/m2 IV once a week )

Ghai Essential Paediatrics,8th edition, page 479, 480

Page 32: Nephrotic syndrome in children. for under graduates

INFECTIONS: SBP Pneumonia Cellulitis UTI disseminated varicella

THROMBOEMBOLISM: Renal vein thrombosis pulmonary embolism saggital sinus thrombosis

OTHERS: Acute renal failure Hypertension Malnutrition Flare up of tuberculosis Steroid & drug related

toxicity

Page 33: Nephrotic syndrome in children. for under graduates

Blood CPUrine REGrowth parametersGeneral examination Blood PressureEye examination RFTsSerum electrolytesBSR

Serum calcium X-Ray wristX-Ray spineChest X-Ray PT/APTT

Page 34: Nephrotic syndrome in children. for under graduates

Steroid Responsive NS : Good prognosis ( MCNS )

Steroid Resistant NS : Poor prognosis ( FSGS )

Mortality rate 1-2 %

- Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1806

Page 35: Nephrotic syndrome in children. for under graduates

Infants who develop nephrotic syndrome within first 3 months of life

ETIOLOGY: Finish type congenital nephrotic syndrome Congenital infections HIV/HBV Diffused mesengial sclerosis Drash syndrome Minimal change disease Focal segmental glomerulosclerosis

Page 36: Nephrotic syndrome in children. for under graduates

TREATMENT: ACE inhibitors + Indomethacin + unilateral neprectomy B/L nephrectomy → chronic dialysis & kidney transplant no role of steroid or immunosuppressive agents

PROGNOSIS: Poor Progressive renal failure Death by 5 yrs age if untreated

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