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A 1-year-3-month-old boy

presented with generalized edema

for 1 month

20 December 2007

History A 1-year-3month-old boyChief complaint :

Generalized edema for 1 month

Present history: He was no underlying disease.1 month PTA, his mother noticed that her

child had enlarged abdomen and edema of both feet and ankles. The patient’s body weight increased 4 kg in 2mo.

History- Normal urination and defecation, no gross

hematuria- No previous history of fever, cough, sore

throat and skin infection- No rash, no arthralgia - No dyspnea, no orthopnea- No chronic diarrhea

History Past history

Caesarean section due to previous C/Sbirth weight 2616 gm, Apgar score 9,10

Family historyNo history of kidney disease in family

Nutritional statusSolid food 3 feedsPowdered milk 8 oz 3 feeds per day

Drugs and immunizationNo history of drug allergyNo routine drug use

ImmunizationUp to date

Developmental historySay some words and walk

History

Physical Examination Vital signs : T 36.9 C, PR140/min,

BP 99/72 mmHg, RR 32/min GA : 1 3/12-year-Thai boy, uncooperative, not

pale, no jaundice, no dyspnea, no cyanosis, puffy eye lids, generalized pitting edema 1+

Ht 80 cm (P50-75), BW 11 kg (P50-75), HC 46 cm (P25-50), Abdominal circumference 49 cm

HEENT : no dental caries

no injected pharynx and tonsils Lungs : clear CVS : normal S1 and S2, no murmur Abdomen : soft, not tender, liver and spleen not

palpable, no palpable mass, mild ascites, bowel sound active

KUB: CVA-not tender, bimanual palpation-negative Genitalia: scrotal edema Others: unremarkable

Physical Examination

PROBLEM LIST

Generalized pitting edema for 1 month

Weight gain 4 kg in 2 months

Generalized edema Nephrotic syndromeAcute or chronic glomerulonephritisCongestive heart failureProtein losing enteropathyProtein malnutrition

DIFFERENTIAL DIAGNOSIS

Nephrotic syndrome

PROVISIONAL DIAGNOSIS

Characteristic features of nephrotic syndrome

1.Generalized pitting edema 2.Hypoalbuminemia (≤2.5g/dl)3.Hypercholesterolemia (≥250g/dl)4.Heavy proteinuria (≥40 mg/m²/hr)

Question 1

“What is the initial investigation

in this patient?”

INVESTIGATIONS AT INITIAL PRESENTATION

Complete blood countRenal profiles, urinalysis and quantification

for urinary protein excretionSerum albuminLipid profiles

INVESTIGATIONS CBC(11/12/50)

Hb 13.3 g/dl , Hct 39.9% WBC 14070 /ul (N 32.2%, L 58.9%, M 4.8%, E 3.7%, B

0.4%)

Plt 428,000 /ul

Urinalysis(11/12/50) pH 7.0, spgr.1.010 protein 4+, sugar neg, wbc 0-1/HP, rbc 1-2/HP

Blood chemistry(11/12/50) BUN 9 mg/dl, Cr 0.1 mg/dl Cholesterol 366 mg/dl Albumin 1.5 g/dl Na 131 mmol/L K 3.8 mmol/L Cl 104 mmol/L HCO₃ 24 mmol/L

INVESTIGATIONS

Urine 24 hrsVolume 440 ml (1.67ml/kg/hr)urine creatinine 31.3 mg/24hrurine protein 607 mg/24hr

(50.6mg/m²/hr)

INVESTIGATIONS

Diagnosis

Nephrotic syndrome

Types of nephrotic syndrome

Primary nephrotic syndrome(90%)

Secondary nephrotic syndrome(10%)

Nephrotic syndrome

Primary nephrotic syndrome(90%)Most common at age 2-6 yr

(6 mo. patient has been reported)Male : female = 2 : 1

Distribution of primary nephrotic syndrome

Histology No. of Patient (%)

Minimal change disease

Focal segmental sclerosis

Membranoproliferative glomerulonephritis (MPGN)

Mesengial proliferation

Proliferative glomerulonephritis

Membranous nephropathy

Chronic glomerulonephritis

unclassified

398 (76.5)

44 (8.5)

39 (7.5)

12 (2.3)

12 (2.3)

8 (1.5)

3 (0.6)

4 (0.8)

* Adapted from a report of the International Study of Kidney Disease in Children

Nephrotic syndrome

Secondary nephrotic syndrome(10%) Suspected in

Age>8 yrsHypertension HematuriaRenal dysfunctionExtrarenal symptoms (rash, arthralgia, fever)

Secondary nephrotic syndromeCauses

InfectionsDrugs Immunologic or allergic disordersMalignant diseaseGlomerular hyperfiltration

In this patientPrimary nephrotic syndrome

More common (90%)Lower age groupMaleNo clinical suspection of secondary

nephrotic syndrome

ManagementGeneral

Low salt, normal protein for age dietSalt poor albumin/diuretic if indicatedClear infectionEducation Immunization

ManagementSpecific

PrednisoloneCyclophosphamide Cyclosporin Levamisole

Indications for renal biopsy

Pretreatment Macroscopic hematuria Persistent hypertension and microscopic

hematuriaRenal failure not attributable to hypovolemiaLow plasma C3Onset age < 6 months

Before start steroid therapy

Complete physical examinationCXR, PPD skin testStool conc. for parasiteDental exam

In this patientComplete physical examinationCXR: no infiltrationPPD skin test: negativeStool conc. for parasite: not found 3 daysDental exam: no dental caries

CORTICOSTEROIDS 1

. Nephrotic Syndrome Initial Diagnosis

Prednisolone 60 mg/m2/day (max 80/day) for 4 weeks

Response No ResponsePrednisolone 40 mg/m2/48 hours for 4 weeks

Renal

Biopsy*Discontinue *Steroid taper at 25% monthly

over 4 months

2. Relapse

Progression

Admit 11-14/12/50V/S stableBW 11 – 11.1 kgBalanced I/O

Discharge14/12/50Home med

Prednisolone[5] 4 tab oral OD pcFollow up

OPD 2 weeks with urinalysis

Follow up 1

. Nephrotic Syndrome Prednisolone 60 mg/m2/day (max 80/day) for 4 weeks

Response No ResponsePrednisolone 40 mg/m2/48 hours for 4 weeks

Renal

Biopsy*Discontinue *Steroid taper at 25% monthly

over 4 months

INDICATIONS FOR RENAL BIOPSY

Steroid resistantSteroid dependenceFrequent relapse

Definitions REMISSION:

Urinary protein excretion < 4 mg/m2/hour or urine dipstix nil/trace for 3 consecutive days.

RELAPSE: Urinary protein excretion > 40 mg/m2/hour or urine dipstix ++ or more for 3 consecutive days.

FREQUENT RELAPSES: Two or more relapses within 6 months of initial response or four or more relapses within any 12 month period.

Definitions STEROID DEPENDENCE:

Two consecutive relapses occurring during the period of steroid taper or within 14 days of its cessation.

STEROID RESISTANCE: Failure to achieve remission in spite of 4 weeks of

standard prednisolone therapy

Question 2

‘What will you advice the

patient’s caregiver ?”

PrognosisThe prognosis depends on the cause of

nephrotic syndrome. It is usually good in children.Minimal change disease responds very well

to steroids and does not cause chronic renal failure.

Focal segmental glomerulosclerosis frequently lead to end stage renal disease.

Prognosis Children who present with hematuria and

hypertension are more likely to be steroid resistant

Poor patient response to steroid therapy seems to be the finding most predictive of a poor outcome

Frequent relapses are more common with

young age of onset and in boys.

Patient Education

Nephrotic syndrome is a chronic illness characterized by relapses and remissions

Ensure normal activity and school attendance.

Infections are an important cause of morbidity and mortality

Patient Education

Live vaccines can be administered 6 weeks after cessation of corticosteroid therapy.

Peer support and psychological counseling are important.