原发性免疫缺陷障碍(PID)或先天性免疫缺陷:初步工作西非Côte科特迪瓦

L. Siransy, A. Adou, Jokebed Kira, N. Moussa, Jocelyne Sery, Doris Oura
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摘要

免疫系统是一个特别复杂的保护系统。作为我们健康的真正守护者,免疫系统保护我们的身体免受外部或内部的攻击。有时,免疫系统无法抵抗,因为它缺乏或不能正常运作,导致免疫反应无效或缺失。虽然存在400多种疾病,但我国没有相关数据。我们通过2022年2月至4月期间在阿比让医院大学中心儿科开展的一项回顾性研究,对涉及93名PID疑似儿童的流行病学、临床、临床旁和治疗方面进行分析。患者平均年龄2岁,极端22天至15岁,男性为主,性别比为1.45。主要地理来源为阿比让(85.32%)。有血缘关系者占12.9%,兄弟姐妹死亡者占3.49%。最常见的临床表现为反复呼吸道感染(60.71%)、发热(16%)、严重感染一年至少2次(13.04%)、持续真菌感染(12.90%)、发育迟缓(48.7%)。异常如白细胞增多症(42.4%)、中性粒细胞减少症(29.23%)、淋巴细胞减少症(16.44%)、血小板减少症(38.36%)和大多数贫血(62.36%)。吞噬缺陷合并免疫缺陷和体液缺陷分别占95.16%、89.78%和79.03%。这项工作引起了人们对PID的重视,因为PID经常被误诊,缺乏或延迟治疗,预后差。建立一个具有确认数据的队列对于更好地支持我国PID患儿具有重要意义。不支持
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Primary immunodeficiency disorders (PID) or inborn errors of immunity : preliminary work West Africa Côte d’Ivoire
The immune system is a particularly sophisticated protection system. As a true guardian of our health, the immune system defends our body against external or internal aggressions. Sometimes, the immune system is not able to fight because it lacks or does not function properly causing an ineffective or absent immune response. Although more than 400 diseases exist, there is no data available in our country. We analyze through a retrospective study carried out in the pediatric department at Hospital University Center, in Abidjan, between February and April 2022, involving 93 PID suspect children, the epidemiological, clinical, paraclinical and therapeutic aspects. The average age of our patients was 2 years with extremes of 22 days to 15 years, with a male predominance: sex ratio of 1.45. The predominant geographical origin was Abidjan (85.32%). Consanguinity was found in 12.9%, death in siblings was found in 3.49% of patients. The most common clinical manifestations were repeated respiratory infections (60.71%), fever (16%), severe infections at least twice a year (13.04%), persistent fungal infections (12.90), thrive retardation in 48.7%. Abnormalities such as hyperleukocytosis (42.4%), neutropenia (29.23%), lymphopenia (16.44%), thrombocytopenia (38.36%) and mostly anemia (62.36%) were noted. Phagocytosis deficiency combined immune deficiencies and humoral deficiencies were respectively found at 95.16%, 89.78%.79.03%. This work draws attention to the importance of PID which are often underdiagnosed, with absent or delayed treatment and poor prognosis. It is important to build a cohort with confirmed data to better support children with PID in our country. no support
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