Renal hamartomas and nephroblastomatosis with fetal gigantism: a familial syndrome
| Autor: | G.M. Goldberg, G. Danovitch, Jacob Bar-Ziv, M. Perlman |
|---|---|
| Rok vydání: | 1973 |
| Předmět: |
Male
Pathology medicine.medical_specialty Hamartoma Autopsy Consanguinity Wilms Tumor Gigantism Muscle hypertrophy medicine Humans Abnormalities Multiple Perlman syndrome Nephroblastomatosis Fetus business.industry Infant Newborn Syndrome medicine.disease Kidney Neoplasms Pediatrics Perinatology and Child Health business Consanguineous Marriage |
| Zdroj: | The Journal of pediatrics. 83(3) |
| ISSN: | 0022-3476 |
| Popis: | A familial syndrome is described, presumably for the first time, the main features of which are bilateral renal hamartomas with or without nephroblastomatosis, somatic gigantism at birth, hypertrophy of the islets of Langerhans, and unusual facies. The condition was lethal in the perinatal period in 5 siblings, born of a consanguineous marriage. There are similarities between this syndrome and that described by Beckwith, as well as cross correlations with other malformations associated with Wilms' tumor. Attention is drawn to important relationships between disordered fetal growth, congenital malformations, and embryonal renal tumors. |
| Databáze: | OpenAIRE |
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