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Scholars Journal of Medical Case Reports | Volume-11 | Issue-05
Congenital Nephrotic Syndrome: About A Case
J. Fahri, A. Lalaoui, F. Bennaoui, N. Slitine, F. Maoulainaine
Published: May 13, 2023 | 138 153
DOI: 10.36347/sjmcr.2023.v11i05.041
Pages: 916-921
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Abstract
Summary: Congenital nephrotic syndrome is a nephrotic syndrome present from birth or in the first three months of life, while infantile nephrotic syndrome is defined by a later onset but in the first year of life. Finnish-type congenital nephrotic syndrome and diffuse mesangial sclerosis represent the two main etiologies. Accurate diagnosis is based on clinical, histological and molecular biology criteria, and antenatal diagnosis is possible in some cases. We report the case of a female newborn, premature 35 WA + 9J, without particular pathological ATCDS admitted on D1 of life for a positive infectious assessment objectifying a bicytopenia made of thrombocytopenia and a neutropenia requested following an infectious anamnesis. positive in the mother, put on ATB, the evolution was marked by the appearance of edema of the two lower limbs on D6 of hospitalization taking the bucket in front generalized with puffiness of the face on D13 of hospitalization, a biological assessment was made and confirmed the nephrotic syndrome: hypoproteinemia at 20.3 g/l + hypoalbuminemia at 10.3 g/l + proteinuria for 24 hours at 3.3 g/24 hours proteinuria/creatinuria ratio 55. Associated symptomatic treatment nephroprotective treatment based on converting enzyme inhibitors was started with regular monitoring of renal function. In conclusion the congenital and infantile nephrotic syndromes are rare and generally have a poor prognosis whose evolution towards terminal renal insufficiency requires a program of dialysis and renal transplantationto the in the intensive Care Department A1 of the Hassan II University Hospital of Fez for the management of CT.