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Congenital nephrotic syndrome of Finnish type. Study of 75 patients.
Open accessAbstract
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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Cited by
Kestilä, M., Lenkkeri, U., Männikkö, M., Lamerdin, J., McCready, P., Putaala, H., Ruotsalainen, V., Morita, T., Nissinen, M., Herva, R., et al. (1998) · Molecular Cell · doi:10.1016/s1097-2765(00)80057-x · Cited by 1,866
Patrakka, J., Kestilä, M., Wartiovaara, J., Ruotsalainen, V., Tissari, P., Lenkkeri, U., Männikkö, M., Visapää, I., Holmberg, C., Rapola, J., et al. (2000) · Kidney International · doi:10.1046/j.1523-1755.2000.00254.x · Cited by 310
Metadata
- Authors
- Huttunen, N. P.
- Venue
- Archives of Disease in Childhood
- Publisher
- BMJ
- Published
- 1976-05-01
- Volume
- 51
- Issue
- 5
- Pages
- 344-348
- ISSN
- 0003-9888 (ISSN-L)
- 1468-2044
- OpenAlex
- W2035497158
- Cited by
- 203
- References
- 0
- FWCI
- 5.81
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Huttunen, N. P. (1976). Congenital nephrotic syndrome of Finnish type. Study of 75 patients. Archives of Disease in Childhood. https://doi.org/10.1136/adc.51.5.344
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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