严重β地中海贫血患者的皮肤表现。

Journal of mother and child Pub Date : 2023-10-08 eCollection Date: 2023-06-01 DOI:10.34763/jmotherandchild.20232701.d-23-00047
Zunaira Zulfiqar, Ayesha Kanwal, Manahil Chaudhry, Muhammad Aadil, Sehrish Qaiser, Ayesha Malik, Mohammad Abdullah
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摘要

背景:重型β地中海贫血是一种依赖输血的地中海贫血。持续的疾病相关炎症过程和慢性输血都会导致铁过载,表现为高铁蛋白血症。我们旨在报告β地中海贫血主要患者的各种皮肤病表现的患病率及其与血清铁蛋白水平的关系。材料和方法:这是一项为期六个月的横断面研究。β地中海贫血的主要患者被连续纳入,并由皮肤科医生进行检查,该医生绘制了任何皮肤状况(如果存在)。同时还采集了血样以检查血清铁蛋白水平。使用SPSSv25对数据进行分析。结果:共有113名患者被纳入研究。队列的平均年龄为9.32±4.54岁。该队列的平均铁蛋白水平为3334±1676微克/升。89.4%(n=101)的患者有皮肤表现,常见的有干燥(44.2%)、雀斑(39.8%)和瘙痒(44.2%,年龄在一岁以下开始输血的患者皮肤状况的数量更高(p=0.0011)。结论:皮肤病表现是β地中海贫血主要患者经常遇到的问题。定期检查这些患者的各种皮肤疾病是很重要的,因为这有助于提高他们的生活质量,降低皮肤病相关的发病率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Cutaneous Manifestations in Patients with Beta-Thalassemia Major.

Background: Beta-thalassemia major is a transfusion-dependent thalassemia. Both ongoing disease-related inflammatory processes and chronic transfusions lead to iron overload, which is depicted by hyperferritinemia. We aimed to report the prevalence of various dermatological manifestations in beta-thalassemia major patients and their relationship with serum ferritin levels.

Material and methods: This was a cross-sectional study conducted over a period of six months. Beta-thalassemia major patients were consecutively enrolled and examined by a dermatologist who charted any skin conditions, if present. A blood sample was also taken at the same time to check for the serum ferritin levels. Data was analysed using SPSSv25.

Results: A total of 113 patients were included in the study. The mean age of the cohort was 9.32 ± 4.54 years. The mean ferritin level for the cohort was 3334 ± 1676 micrograms per litre. Cutaneous manifestations were seen in 89.4% (n = 101) patients with the common ones namely xerosis (44.2%), freckles (39.8%) and pruritus (44.2%). We noted that serum ferritin levels were significantly higher in those with freckles (p = 0.00288). The cause of pruritus does not appear to be jaundice (p = 0.973). Lastly, number of skin conditions were higher in those with onset of blood transfusions at age less than one year (p = 0.0011).

Conclusion: Dermatological manifestations are a frequently encountered problem in beta-thalassemia major patients. It is important to examine these patients for various skin disorders periodically as this can help improve their quality of life and reduce dermatological-associated morbidity.

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