García Rodríguez M C, Fontán Casariego G, Escobar Castro H, Alba Losada J, Lorente Toledano F, Ojeda Casas J A
An Esp Pediatr. 1977 Jan;10(1):19-26.
A child with recurrent infections is presented. The frequent diarrhea leads to a severe malnutrition. The immunological work-up disclosed: absent IgA and low IgG serum levels, deficient cellular immunity and abnormal neutrophil chemotaxis. The cellular immunity and the neutrophil chemotaxis were improved with the nutritional status. At present the patient shows a partial combined immunodeficiency. The diagnosis of the case as a Nezelof's syndrome is discussed, as well as the addition of a secondary immunodeficiency caused by the recurrent infections and the malnutrition.
现报告一名反复感染的儿童。频繁腹泻导致严重营养不良。免疫检查发现:血清IgA缺乏、IgG水平低、细胞免疫缺陷和中性粒细胞趋化性异常。细胞免疫和中性粒细胞趋化性随营养状况改善。目前该患者表现为部分联合免疫缺陷。讨论了该病例诊断为内泽洛夫综合征,以及由反复感染和营养不良引起的继发性免疫缺陷。