Datta U, Kaur K J, Kumar L, Walia B N, Mehta S, Mehta S K, Sehgal S
Department of Immunopathology, Postgraduate Institute of Medical Education and Research, Chandigarh.
Indian Pediatr. 1993 Apr;30(4):461-7.
Twenty three patients with primary immunoglobulin(Ig) deficiency were seen during the last ten years. Nine had hypogamma globulinemia (hypo-Ig) and the rest, selective Ig deficiency. Most were in pediatric age group. There was preponderance of males with only one female. Clinical symptoms pertaining to gastrointestinal and sinupulmonary infections were most common. Complete absence of B cells was not observed in any patient with hypogammaglobulinemia. They could be typed as physiological in one patient, X-linked immunodeficiency in 2 patients and common variable immunodeficiency in the remaining six. Three patients with selective IgA deficiency were above 20 years of age. Two had only secretory IgA deficiency, confirmed by jejunal fluid examination and the rest had both secretory and serum IgA deficiency. Low IgM was seen in one patient. We see a spectrum of immunoglobulin deficiencies varying from subtle defects like absence of secretory IgA only, to severe depletion of all immunoglobulins. Therapy is still not ideal due to economic reasons.
在过去十年中，共诊治了23例原发性免疫球蛋白（Ig）缺乏症患者。其中9例为低丙种球蛋白血症（低Ig），其余为选择性Ig缺乏症。大多数患者处于儿童年龄组。男性占优势，仅1例为女性。与胃肠道和鼻窦肺部感染相关的临床症状最为常见。在任何低丙种球蛋白血症患者中均未观察到B细胞完全缺失。其中1例患者的低丙种球蛋白血症可归为生理性，2例为X连锁免疫缺陷，其余6例为常见可变免疫缺陷。3例选择性IgA缺乏症患者年龄超过20岁。2例经空肠液检查证实仅存在分泌型IgA缺乏，其余患者同时存在分泌型和血清IgA缺乏。1例患者出现低IgM。我们发现了一系列免疫球蛋白缺乏症，从仅缺乏分泌型IgA这样的细微缺陷到所有免疫球蛋白的严重耗竭不等。由于经济原因，治疗仍然不理想。
