Holmes S N, Condliffe A, Griffiths W, Baxendale H, Kumararatne D S
Department of Clinical Immunology, Addenbrooke's Hospital, Hills Road, Box 109, Cambridge, CB2 0QQ, UK,
J Clin Immunol. 2015 Apr;35(3):302-4. doi: 10.1007/s10875-015-0142-8. Epub 2015 Feb 24.
Common Variable Immunodeficiency (CVID) comprises a heterogeneous group of primary antibody deficiencies which lead to a range of complications, including infectious, neoplastic and inflammatory disorders. This report describes monozygotic twin brothers with CVID who developed cryptogenic liver disease and subsequently hepatopulmonary syndrome (HPS). This is the second report of the association of HPS and CVID. Its occurrence in two identical twins implicates a genetic basis.
普通可变免疫缺陷(CVID)包括一组异质性的原发性抗体缺陷,可导致一系列并发症,包括感染性、肿瘤性和炎症性疾病。本报告描述了一对患有CVID的同卵双胞胎兄弟,他们患了隐源性肝病,随后发展为肝肺综合征(HPS)。这是关于HPS与CVID关联的第二篇报告。它在一对同卵双胞胎中的发生暗示了其遗传基础。