奥门氏病
Omenn's disease.
作者信息
Dyke M P, Marlow N, Berry P J
机构信息
Department of Child Health, University of Bristol.
出版信息
Arch Dis Child. 1991 Oct;66(10):1247-8. doi: 10.1136/adc.66.10.1247.
The importance of accurate pathological diagnosis is emphasised in the case of a newborn infant who presented with alopecia, a generalised erythrodermatous skin eruption, and hepatosplenomegaly. She subsequently developed generalised lymphadenopathy and recurrent septicaemia and died aged 2 months. The histological findings of widespread lymphocytic, histiocytic, and eosinophilic tissue infiltration, associated with thymic hypoplasia, were consistent with autosomal recessive Omenn's disease.
对于一名出现脱发、全身性红皮病皮疹和肝脾肿大的新生儿，准确病理诊断的重要性得到了强调。她随后出现全身性淋巴结病和复发性败血症，并在2个月大时死亡。广泛的淋巴细胞、组织细胞和嗜酸性组织浸润以及胸腺发育不全的组织学发现与常染色体隐性奥门氏病相符。
Zotero 插件