Congenital nephrotic syndrome
| Autor: | Niilo Hallman, Lars Hjelt |
|---|---|
| Rok vydání: | 1959 |
| Předmět: |
medicine.medical_specialty
Proteinuria Nephrotic Syndrome business.industry Physiology Infant Urine medicine.disease Blood proteins Blood pressure Endocrinology Internal medicine Edema Pediatrics Perinatology and Child Health medicine Humans medicine.symptom business Child Congenital nephrotic syndrome Blood urea nitrogen Nephrotic syndrome |
| Zdroj: | The Journal of pediatrics. 55(2) |
| ISSN: | 0022-3476 |
| Popis: | Summary The clinical course and postmortem findings of 18 infants with the congenital nephrotic syndrome are presented. The infants came from 12 families with a total of 49 children, 37 per cent of whom were affected with CNSY. Eleven other infants in these sibships were born prematurely and died soon after birth. With three exceptions, the childrenwith congenital nephrosis were born 3 to 5 weeks prematurely. In the cases for which information is available, the placenta was large. Edema frequently occurred immediatelyafter birth and was present in all of the infants by two months of age, by which time proteinuria was pronounced. Changes in the urine and plasma proteins were typical of the nephrotic syndrome. In the initial phase of the disease no erythrocytes were found in the urine, although some appeared later. The blood urea nitrogen was low, as was the blood pressure. The plasma cholesterol level was often under 200 mg. per cent, but it rose consistently as the disease progressed. The serum fat curve following intravenous injection of a fat emulsion was also typical of the nephrotic syndrome. With the exception of one child whois now 8 months of age and still suffering from the disease, all of the infants have died, the oldest at the age of 19 months. Postmortem examinations showed the kidneys to have changes typical of the nephrotic syndrome. In the patients who died at the earlier ages the changes in the basement membranes of the glomerular capillaries were mild; more numerous proliferative changes were found in the patients dying later. Fatty degeneration was always present in the cells of the tubules. The absence of typical foot processesand of splitting of the basement membranes was established by electron microscopy in 3 cases. The tubules had a thickened basement membrane which was lamellar in structure. |
| Databáze: | OpenAIRE |
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