Congenital nephrotic syndrome of Finnish type. Study of 75 patients.
Seventy-five patients with congenital nephrotic syndrome of Finnish type were identified in Finland in the period 1965-1973, giving an incidence of 12-2/10(5). A large placenta and proteinuria from birth are the hallmarks of the disease. About one-quarter of the patients had oedema and/or abdominal distension at birth and in all cases the full nephrotic syndrome was documented before 2 months. More than half of the patients died before 6 months and none lived longer than 2 years 3 months. A slight rise in blood urea nitrogen or serum creatinine levels occurred in 14 cases, but in none of these did a frank uraemia develop before death. Infection appeared to be the immediate cause of death in 31% of the cases; in 43% no cause of death other than congenital nephrotic syndrome could be shown. Thrombi in large vessels were found in 11 out of 58 necropsies.
D001724:Birth Weight; D001835:Body Weight; D004487:Edema / Q000150:complications; D005387:Finland; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D009404:Nephrotic Syndrome / Q000151:congenital* / Q000453:epidemiology / Q000401:mortality; D010920:Placenta / Q000033:anatomy & histology; D011507:Proteinuria / Q000150:complications