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Year : 2012  |  Volume : 2  |  Issue : 1  |  Page : 22-24

Nephrotic syndrome in the first year of life

1 Department of Nephrology, Osmania Medical College/ Osmania General Hospital, Hyderabad, Andhra Pradesh, India
2 Department of Histopathology, Apollo Hospital, Hyderabad, Andhra Pradesh, India
3 Department of Nephrology, Osmania Medical College/ Osmania General Hospital; Consultant, Medwin Hospital, Hyderabad, Andhra Pradesh, India
4 Department of Nephrology, Osmania Medical College/ Osmania General Hospital; Consultant, Mediciti Hospital, Hyderabad, Andhra Pradesh, India

Correspondence Address:
Manisha Sahay
Department of Nephrology, Osmania General Hospital, Afzalgunj, Hyderabad, Andhra Pradesh- 500 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2249-4855.104011

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Objective: To study the clinical profile, laboratory data, histopathology, and outcome of infantile nephrotic syndrome (INS). Materials and Methods: Over a 5 year period, 30 infants with nephrotic syndrome were seen at Osmania General Hospital. They were studied with regard to family history, antenatal details, birth history, clinical features including anthropometry and developmental milestones, laboratory investigations and histopathology of renal tissue (light microscopy and immunofluorescence), and treatment outcome. Results: There were 17 males and 13 females (1:0.8). The mean age at presentation was 8.6 months (2.5-12 months). History of consanguinity was obtained in 12 infants (40%) and birth asphyxia was present in 8 infants (26%). Eighteen (60%) were growth retarded and 10 (33.3%) had developmental delay. Hypertension was recorded in 8 (26%) and edema in 24 (80%). At presentation, renal failure was evident in 18 (60%) infants. Nephrotic range proteinuria and hypoalbuminemia were found in all and microscopic hematuria in 4 (13%) infants. Histology of renal tissue revealed congenital Finnish type (CNF) in 8 (26%), diffuse mesangial sclerosis (DMS) in 6 (20%), minimal change disease (MCD) in 6 (20%), mesangial proliferation with IgM deposits in 4 (13%), and focal segmental glomerulosclerosis (FSGS) in 4 (13%). Mesangial hypercellularity with cytomegalovirus infection was found in 2 (6.6%) infants. CNF and DMS on histopathology carried a very poor prognosis with none recovering, while the others showed variable response to treatment with steroids and cytotoxic therapy. Conclusion: INS is a rare disorder. The predominant histopathological lesions are CNF and DMS which carry a very poor prognosis.

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