Congenital nephrotic syndrome of Finnish type. Study of 75 patients.

Authors:
N P Huttunen

Arch Dis Child 1976 May;51(5):344-8

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.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1545982PMC
http://dx.doi.org/10.1136/adc.51.5.344DOI Listing
May 1976

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References

(Supplied by CrossRef)

Helsinki et al.
Statistical Yearbook of Finland 1976
Nephrotic Syndrome in newborn and young infants
Hallman, N.; Hjelt, L.; Ahvenainen, E.K. et al.
Annales Paediatriae Fenniae 1956
Main features By a few months of age the oedema often disapof the congenital nephrotic syndrome
Hallman, N.; Norio, R.; Kouvalainen, K. et al.
Acta Paediatrica Scandinavica 1967
though the abdominal distension usually persists
1970
Length of survival of CNF patients has remained practically unchanged since
al et al.
1965
Das kongenitale nephrotische Syndrom. Ergebnisse der inneren Medizin undKinderheilkunde
Congenital nephrotic syndrome
Hallman, N.; Norio, R.; Rapola, J. et al.
Nephron 1973
Immunological features in the congenital nephrotic syndrome. A clinical and experimental study
Kouvalainen, K. et al.
Annales Paediatriae Fenniae 1963
Placenta in congenital nephrotic syndrome
Kouvalainen, K.; Hjelt, L.; Hallman, N. et al.
Annales Paediatriae Fenniae; Activation of Hageman factor in the nephrotic syndrome 1962
also been observed in other types of nephrotic syndrome
British MedicalJournal; Thrombosis of pulmonary arteries and the nephrotic syndrome 1967
The hypothesis that steroid therapy might be an aetiological factor in thrombosis
Lewy; Wellington et al.
et al; receives no support here, because none of the CNF patients with thrombotic complications had been given steroids. Hypovolaemia and an increased incidence of sepsis (Lieberman et al., 1968) together with disturbances in coagulation (Lange 1974

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