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家族性地中海热患儿的营养状况

Nutritional status in children with familial Mediterranean fever.

作者信息

Bahçeci Onur, Aydın Fatma, Kuloğlu Zarife, Karakaş Hatice Dilara, Taş Özen, Öksüz Aydın Betül, Sarısoy Doğacan, Işık Salih, Özçakar Zeynep Birsin

机构信息

Department of Pediatrics, Division of Pediatric Rheumatology, Ankara University School of Medicine, Ankara, Turkey.

Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Ankara University School of Medicine, Ankara, Turkey.

出版信息

Postgrad Med. 2025 Aug 27:1-8. doi: 10.1080/00325481.2025.2552100.

DOI:10.1080/00325481.2025.2552100
PMID:40843473
Abstract

OBJECTIVES

To determine the frequency of malnutrition, including undernutrition and overnutrition, in pediatric patients with familial Mediterranean fever (FMF), and to assess the impact of colchicine treatment on nutritional outcomes and factors associated with malnutrition.

METHODS

The medical records of patients with FMF who were followed up in our pediatric rheumatology clinic between 2011 and 2024 were reviewed. Patients were divided into 3 groups according to their nutritional status: normal, undernutrition and overnutrition. Demographic and clinical characteristics and disease severity were compared before and after treatment. The international severity scoring system for FMF (ISSF) was used to assess disease severity.

RESULTS

This retrospective study included 532 (50.6% female) pediatric patients with a median (IQR) age at diagnosis of 6.5 (6) years. Malnutrition was detected in 32.7% of patients at the time of diagnosis, after colchicine treatment this rate declined to 20.7%, significant reductions in undernutrition (from 23.5% to 12.8%) and marginal improvements in overnutrition (from 9.2% to 7.9%) ( < 0.001) groups were observed. The ISSF was significantly higher in both the undernutrition and overnutrition groups compared to patients with normal nutritional status before and after colchicine treatment ( < 0.001). A severe disease phenotype and malnutrition at the time of diagnosis were identified as significant risk factors for persistent malnutrition ( < 0.001).

CONCLUSION

Malnutrition, including undernutrition and overnutrition, is a common condition in pediatric FMF patients that can be improved with colchicine. The presence of malnutrition at diagnosis and a more severe disease phenotype seemed to be the most important determinants of persistent malnutrition.

摘要

目的

确定家族性地中海热(FMF)儿科患者中营养不良(包括营养不足和营养过剩)的发生率,并评估秋水仙碱治疗对营养结局以及与营养不良相关因素的影响。

方法

回顾了2011年至2024年在我们儿科风湿病诊所接受随访的FMF患者的病历。根据营养状况将患者分为3组:正常、营养不足和营养过剩。比较治疗前后的人口统计学和临床特征以及疾病严重程度。使用FMF国际严重程度评分系统(ISSF)评估疾病严重程度。

结果

这项回顾性研究纳入了532例儿科患者(50.6%为女性),诊断时的中位(IQR)年龄为6.5(6)岁。诊断时32.7%的患者检测到营养不良,秋水仙碱治疗后这一比例降至20.7%,营养不足显著降低(从23.5%降至12.8%),营养过剩有轻微改善(从9.2%降至7.9%)(<0.001)。在秋水仙碱治疗前后,营养不足和营养过剩组的ISSF均显著高于营养状况正常的患者(<0.001)。诊断时的严重疾病表型和营养不良被确定为持续性营养不良的显著危险因素(<0.001)。

结论

营养不良,包括营养不足和营养过剩,在儿科FMF患者中很常见,秋水仙碱治疗可改善这种情况。诊断时存在营养不良和更严重的疾病表型似乎是持续性营养不良的最重要决定因素。

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