Zahra Shahraki Ghadimi, Simin Sadeghi Bojd, Nima Parvane, Mehdi Atabaki, Ebrahim Alijani
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引用次数: 0
摘要
肾病综合征的特点是血液中的蛋白质渗漏到尿液中,并伴有蛋白尿、白蛋白尿和外周水肿三联症。蛋白质的流失会导致免疫球蛋白和补体的流失。X 连锁丙种球蛋白血症(XLA)或布鲁顿病是一种原发性免疫缺陷病,由骨髓中 B 细胞发育缺陷和血清中免疫球蛋白水平低下引起。本病例涉及一名患有肾病综合征、骨髓炎和反复感染的 12 岁男孩。我们发现他患有 XLA。这份报告强调了在肾病综合征等蛋白质丢失的病例中考虑先天性免疫错误的重要性。
Nephrotic syndrome is characterized by the leakage of protein from the blood into the urine along with the triad of proteinuria, albuminuria, and peripheral edema. Loss of protein leads to the loss of immunoglobulin and complements. X-linked agammaglobulinemia (XLA), or Bruton disease, is a primary immunodeficiency disease caused by a defect in the development of B cells in the bone marrow and a low serum level of immunoglobulins. The present case involves a 12-year-old boy with nephrotic syndrome, osteomyelitis, and recurrent infections. We discovered that he had XLA. This report underscores the importance of considering inborn errors of immunity in cases of protein loss, such as nephrotic syndrome.
期刊介绍:
The Iranian Journal of Allergy, Asthma and Immunology (IJAAI), an international peer-reviewed scientific and research journal, seeks to publish original papers, selected review articles, case-based reviews, and other articles of special interest related to the fields of asthma, allergy and immunology. The journal is an official publication of the Iranian Society of Asthma and Allergy (ISAA), which is supported by the Immunology, Asthma and Allergy Research Institute (IAARI) and published by Tehran University of Medical Sciences (TUMS). The journal seeks to provide its readers with the highest quality materials published through a process of careful peer reviews and editorial comments. All papers are published in English.