Postural deformities in congenital nephrotic syndrome.

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Postural deformities in congenital nephrotic syndrome.

Six successive cases of congenital nephrotic syndrome are described. Each one showed flexion deformities of the knees and hips, widely open anterior and posterior fontanelles, and wide separation of the skull sutures. These abnormalities were present not only in cases in which the renal histology was of the microcystic Finnish type of congenital nephrotic syndrome, but also in those in which th...

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Congenital Nephrotic Syndrome: A Cases Report

Congenital nephrotic syndrome (CNS) can be caused by neonatal infections and renal diseases that usually occur in early infancy. The most common CNS is the Finnish type, which is an autosomal recessively inherited disease characterized by intrauterine onset of massive proteinuria. In this study, we presented a preterm neonate confirmed as the first case of CNS in Iran by genetic study, who was ...

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Congenital nephrotic syndrome

CNS (Congenital nephrotic syndrome) is a disorder characterized by the presence of a nephrotic syndrome in the first three months of life. Different pathologies can cause this syndrome. In general, we can distinguish primary forms (sporadic and hereditary) and secondary forms (acquired and associated with other syndromes). The most common form is the Finnish CNS (CNF, congenital nephrotic syndr...

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[Congenital and infantile nephrotic syndrome].

Congenital nephrotic syndrome is present at birth or appears during the first three months of life and infantile nephrotic syndrome during the first year. Finnish type congenital nephrotic syndrome is an autosomal recessive disease. Nephrotic syndrome is present at birth, severe and does not respond to therapy. Infectious and nutritional complications are frequent. Renal function deteriorates n...

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Congenital nephrotic syndrome, Finnish type

Keywords Disease name and synonyms Definition Incidence Clinical description Histology Treatment Etiology Antenatal diagnosis References Abstract The congenital nephrotic syndrome of the Finnish type is a hereditary disease with autosomal recessive inheritance. The gene frequency is approximately 1/200 in Finland. The disease is caused by mutations in the gene for nephrin, which is a key compon...

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ژورنال

عنوان ژورنال: Archives of Disease in Childhood

سال: 1981

ISSN: 0003-9888,1468-2044

DOI: 10.1136/adc.56.12.959