Management of Chronic Kidney Disease

The name is derived from the fact that amongst the Finnish, this condition has a much higher incidence than in other populations, at 1 per 8000 births. It is an autosomal recessive condition and mutations in the gene NPHS1, which encodes nephrin, a key component of the podocyte slit diaphragm, are responsible.

Clinical findings:

• Enlarged placenta
• Oedema
• Severe nephrotic syndrome soon develops with associated failure to thrive, susceptibility to infection and thrombotic complications.
• End stage renal failure occurs by 3-8 years of age.

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