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土耳其某单一转诊中心 420 例儿童过敏性紫癜的临床表现和转归:三年经验。

Clinical manifestations and outcomes of 420 children with Henoch Schönlein Purpura from a single referral center from Turkey: A three-year experience.

机构信息

Faculty of Medicine, Department of Pediatric Rheumatology, Cukurova University, Adana, Turkey.

Faculty of Medicine, Department of Pediatric Nephrology, Cukurova University, Adana, Turkey.

出版信息

Mod Rheumatol. 2020 Nov;30(6):1039-1046. doi: 10.1080/14397595.2019.1687074. Epub 2019 Nov 14.

DOI:10.1080/14397595.2019.1687074
PMID:31662011
Abstract

Henoch Schönlein Purpura (HSP) is the most common systemic vasculitis in childhood. We aimed to evaluate the clinical features, seasonal variation, treatment outcomes and the possible predicting factors related to outcome among a large cohort of pediatric HSP patients. We conducted a medical record review study between July 2016 and January 2019 and evaluated the clinical manifestations and potential risk factors for severe gastrointestinal (GI) involvement, biopsy-proven nephritis and relapses. The study included 420 HSP patients, of which the mean age at diagnosis was 7.68 ± 3.15 years. Clinical manifestations were arthralgia and/or arthritis ( = 244, 58.1%), abdominal pain ( = 235, 56%), subcutaneous edema ( = 163, 38.8%), and renal involvement ( = 125, 29.8%). Disease recurred for at least once, in 69 (16.4%) patients and colchicine treatment yielded a favorable response in 11 of 12 relapsing patients, who did not respond to ibuprofen or steroids. Frequencies of renal involvement and biopsy-proven nephritis were higher in patients with severe GI involvement. Besides, patients with biopsy-proven nephritis had higher rates of abdominal pain, intussusception, severe GI involvement, and systemic steroid administration. We speculate that renal involvement, biopsy-proven nephritis and severe GI involvement can be related to each other. Colchicine may be effective in patients with relapsing disease.

摘要

过敏性紫癜(HSP)是儿童最常见的系统性血管炎。我们旨在评估大量儿科 HSP 患者的临床特征、季节性变化、治疗结果以及与结局相关的可能预测因素。我们进行了一项病历回顾研究,时间为 2016 年 7 月至 2019 年 1 月,评估了严重胃肠道(GI)受累、活检证实的肾炎和复发的临床表现和潜在危险因素。该研究纳入了 420 例 HSP 患者,其中诊断时的平均年龄为 7.68 ± 3.15 岁。临床表现为关节痛和/或关节炎( = 244,58.1%)、腹痛( = 235,56%)、皮下水肿( = 163,38.8%)和肾脏受累( = 125,29.8%)。至少复发一次的患者有 69 例(16.4%),12 例复发患者中有 11 例对秋水仙碱治疗有良好反应,而对布洛芬或类固醇无反应。严重 GI 受累患者的肾脏受累和活检证实的肾炎发生率更高。此外,活检证实肾炎的患者腹痛、肠套叠、严重 GI 受累和全身类固醇治疗的发生率更高。我们推测肾脏受累、活检证实的肾炎和严重 GI 受累可能相互关联。秋水仙碱可能对复发性疾病患者有效。

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Clinical manifestations and outcomes of 420 children with Henoch Schönlein Purpura from a single referral center from Turkey: A three-year experience.土耳其某单一转诊中心 420 例儿童过敏性紫癜的临床表现和转归:三年经验。
Mod Rheumatol. 2020 Nov;30(6):1039-1046. doi: 10.1080/14397595.2019.1687074. Epub 2019 Nov 14.
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