Prindull G, Stubbe P, Kratzer W
Z Kinderheilkd. 1975 Jul 1;120(1):37-49.
This is a study of the largest family with Fanconi's anemia known in the world literature, namely of 6 affected siblings. All patients showed the typical features of the disease, including pancytopenia, skeletal and kidney deformities, hyperpigmentation of the skin, and physical as well as mental retardation. Four of the patients have died of their disease, the 2 patients who are still living at present have a deteriorating clinical course. Case histories, clinical and laboratory findings are reported here. In the second and third part of this study [48, 52] endocrinologic and genetic findings will be reported.
这是一项对世界文献中已知的患范科尼贫血的最大家族(即6名患病兄弟姐妹)的研究。所有患者均表现出该疾病的典型特征,包括全血细胞减少、骨骼和肾脏畸形、皮肤色素沉着以及身体和智力发育迟缓。其中4名患者已死于该疾病,目前仍在世的2名患者临床病程呈恶化趋势。本文报告了病例史、临床和实验室检查结果。本研究的第二和第三部分[48, 52]将报告内分泌学和遗传学检查结果。