发展中世界的原发性免疫缺陷疾病：来自印度一家医院登记处的数据。
Primary immunodeficiency disorders in the developing world: data from a hospital-based registry in India.
作者信息
Chinnabhandar Vasant, Yadav Satya Prakash, Kaul Dinesh, Verma I C, Sachdeva Anupam
机构信息
1Division of Pediatric Hematology-Oncology and Bone Marrow Transplantation.
出版信息
Pediatr Hematol Oncol. 2014 Apr;31(3):207-11. doi: 10.3109/08880018.2013.805346. Epub 2013 Jun 12.
Primary immunodeficiency disorders (PID) are under-reported from the developing world. We present data regarding diagnosis and outcome from a hospital-based registry in India. Forty-seven patients fulfilled diagnostic criteria. Majority were males. Subgroups were disorders of immune dysregulation-29%, B&T-cell abnormalities-28%, predominant antibody deficiencies-23%, other well-defined immunodeficiencies-15%, and phagocyte disorders-4%. Molecular diagnosis was attempted in 12 and was positive in seven. Overall 24 children died. Only three out of 28 children needing stem cell transplant (SCT) underwent the same. Registry data highlights that molecular diagnosis and SCT are a rarity for children with PIDs in the developing world and mortality is high.
原发性免疫缺陷病（PID）在发展中世界的报告不足。我们展示了来自印度一家医院登记处的关于诊断和结果的数据。47名患者符合诊断标准。大多数为男性。亚组包括免疫失调疾病——29%，B和T细胞异常——28%，主要抗体缺陷——23%，其他明确的免疫缺陷——15%，以及吞噬细胞疾病——4%。对12名患者尝试进行分子诊断，其中7名呈阳性。总体上有24名儿童死亡。28名需要干细胞移植（SCT）的儿童中只有3名接受了移植。登记处数据凸显出，在发展中世界，PID儿童进行分子诊断和SCT的情况很罕见，且死亡率很高。
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