[抗中性粒细胞胞浆抗体相关性血管炎患儿的临床特征]。

Q3 Medicine

中国当代儿科杂志 Pub Date : 2024-08-15 DOI:10.7499/j.issn.1008-8830.2401074

Jing-Qi Liu, Yong-Zhen Li, Lan-Jun Shuai, Yan Cao, Xiao-Yan Li, Ying Wang, Tian Shen, Shuang-Hong Mo, Xiao-Jie He, Xiao-Chuan Wu

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引用次数: 0

摘要

目的研究抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）患儿的临床特征：方法：对2010年1月至2022年6月中南大学湘雅二医院确诊的25例AAV患儿的临床资料进行回顾性分析：AAV患儿中，男5例，女20例，发病年龄中位数为11.0岁。泌尿系统受累18例（72%）；呼吸系统受累10例（40%）；皮肤受累6例（24%）；眼、耳、鼻受累5例（20%）；关节受累4例（16%）；消化系统受累2例（8%）。11例患者接受了肾活检，其中5例（46%）为局灶型，2例（18%）为新月型，2例（18%）为混合型，2例（18%）为硬化型。5例（45%）出现免疫复合物沉积。7 例患者的慢性肾脏病（CKD）达到了 V 期，其中 2 例导致死亡。两例患者接受了肾移植手术。在随访期结束时，2 例处于慢性肾脏病 II 期，1 例处于慢性肾脏病 III 期。在 16 例显微镜下多血管炎（MPA）患者中，13 例（81%）涉及泌尿系统。在 9 例肉芽肿伴多血管炎（GPA）病例中，有 6 例（66%）患有鼻窦炎。MPA 组的血清肌酐和尿酸水平高于 GPA 组（PPConclusions：AAV在学龄女童中更为常见，MPA是最常见的临床亚型。儿童 AAV 发病的主要特征是肾脏受累，其次是呼吸系统受累。肾脏病变通常表现为局灶型，可能有免疫复合物沉积。患有 MPA 的儿童常伴有肾脏受累，而患有 GPA 的儿童常伴有鼻窦炎。AAV患儿的预后较差，通常伴有肾功能不全。

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[Clinical characteristics of children with anti-neutrophil cytoplasmic antibody-associated vasculitis].

Objectives: To study the clinical characteristics of children with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

Methods: A retrospective analysis was conducted on the clinical data of 25 children diagnosed with AAV at the Second Xiangya Hospital of Central South University from January 2010 to June 2022.

Results: Among the AAV children, there were 5 males and 20 females, with a median age of onset of 11.0 years. Involvement of the urinary system was seen in 18 cases (72%); respiratory system involvement in 10 cases (40%); skin involvement in 6 cases (24%); eye, ear, and nose involvement in 5 cases (20%); joint involvement in 4 cases (16%); digestive system involvement in 2 cases (8%). Eleven cases underwent kidney biopsy, with 5 cases (46%) showing focal type, 2 cases (18%) showing crescentic type, 2 cases (18%) showing mixed type, and 2 cases (18%) showing sclerotic type. Immune complex deposits were present in 5 cases (45%). Seven cases reached chronic kidney disease (CKD) stage V, with 2 cases resulting in death. Two cases underwent kidney transplantation. At the end of the follow-up period, 2 cases were at CKD stage II, and 1 case was at CKD stage III. Of the 16 cases of microscopic polyangiitis (MPA) group, 13 (81%) involved the urinary system. Of the 9 cases of granulomatosis with polyangiitis (GPA), 6 cases (66%) had sinusitis. Serum creatinine and uric acid levels were higher in the MPA group than in the GPA group (P<0.05), while red blood cell count and glomerular filtration rate were lower in the MPA group (P<0.05).

Conclusions: AAV is more common in school-age female children, with MPA being the most common clinical subtype. The onset of AAV in children is mainly characterized by renal involvement, followed by respiratory system involvement. The renal pathology often presents as focal type with possible immune complex deposits. Children with MPA often have renal involvement, while those with GPA commonly have sinusitis. The prognosis of children with AAV is poor, often accompanied by renal insufficiency.

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来源期刊

中国当代儿科杂志 Medicine-Pediatrics, Perinatology and Child Health

CiteScore

1.50

自引率

0.00%

发文量

5006

期刊介绍： The Chinese Journal of Contemporary Pediatrics (CJCP) is a peer-reviewed open access periodical in the field of pediatrics that is sponsored by the Central South University/Xiangya Hospital of Central South University and under the auspices of the Ministry of Education of China. It is cited as a source in the scientific and technological papers of Chinese journals, the Chinese Science Citation Database (CSCD), and is one of the core Chinese periodicals in the Peking University Library. CJCP has been indexed by MEDLINE/PubMed/PMC of the American National Library, American Chemical Abstracts (CA), Holland Medical Abstracts (EM), Western Pacific Region Index Medicus (WPRIM), Scopus and EBSCO. It is a monthly periodical published on the 15th of every month, and is distributed both at home and overseas. The Chinese series publication number is CN 43-1301/R；ISSN 1008-8830. The tenet of CJCP is to “reflect the latest advances and be open to the world”. The periodical reports the most recent advances in the contemporary pediatric field. The majority of the readership is pediatric doctors and researchers.