1977
Multiple Hepatic Tumors and Peliosis Hepatis in Fanconi's Anemia Treated With Androgens
Abstract: \s=b\We report the case of a 13-year-old boy who was known to have Fanconi's anemia for five years. For treatment of this condition he was given androgens and corticosteroids. Two months before his death, severe varicella developed complicated by pneumonia, jaundice, and prolonged fever; all of which resolved during a five-week hospitalization. Three weeks later he died of Clostridium septicum sepsis caused by necrotizing enterocolitis. At autopsy he was found to have multiple hepatocellular neoplasms. A strik…
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Cited by 58 publications
(23 citation statements)
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“…Ths 15-year-old boy, the product of a non-consanguinous marriage, was first evaluated when he was 5 years old, at which time he was noted to have mild thrombocytopenia, an elevated fetal Hb, but no anaemia. His older brother had been previously diagnosed as Fanconi's anaemia and is the subject of another report (Shapiro et al, 1977). The only gross anomalies noted in this patient, in addition to his short stature, were hyperpigmentation of the skin a t the base of the neck and an inability to flex his right thumb without also flexing the adjacent index finger.…”
Section: Case Historymentioning
confidence: 73%
“…Ths 15-year-old boy, the product of a non-consanguinous marriage, was first evaluated when he was 5 years old, at which time he was noted to have mild thrombocytopenia, an elevated fetal Hb, but no anaemia. His older brother had been previously diagnosed as Fanconi's anaemia and is the subject of another report (Shapiro et al, 1977). The only gross anomalies noted in this patient, in addition to his short stature, were hyperpigmentation of the skin a t the base of the neck and an inability to flex his right thumb without also flexing the adjacent index finger.…”
Section: Case Historymentioning
confidence: 73%
“…A pathogenic relationship can be assumed to exist between the anabolically induced cholestasis, the occurrence of peliosis hepatis, and beginning cirrhosis. However, the development of primary liver tumors caused by peliosis hepatis as suggested by some authors (11,12,19) seems improbable. Neoplasms in the portal area seldom occurred in our material and are more similar to an adenoma of the bile duct than to primary hepatoma.…”
Section: Resultsmentioning
confidence: 99%
“…As drogas injetáveis, como a testosterona cipionato e as preparações em enantato, parecem não afetar a função hepática. No entanto, são reportados casos de adenoma hepático com o uso dessas substâncias (19) . Urhausen et al estudaram a reversibilidade dos efeitos do uso dos esteroides anabolizantes em inúmeras variáveis, entre elas, algumas enzimas associadas à função hepática.…”
Section: Discussionunclassified
