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重型β-地中海贫血患者的皮肤表现。

Cutaneous Manifestations in Patients with Beta-Thalassemia Major.

机构信息

Department of Paediatrics, CMH Lahore Medical College & Institute of Dentistry, Lahore, Pakistan.

Department of Dermatology, CMH Lahore Medical College & Institute of Dentistry, Lahore, Pakistan.

出版信息

J Mother Child. 2023 Oct 8;27(1):142-146. doi: 10.34763/jmotherandchild.20232701.d-23-00047. eCollection 2023 Jun 1.

Abstract

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.

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,这表现为高血清铁蛋白血症。我们旨在报告重型β地中海贫血患者中各种皮肤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。连续纳入重型β地中海贫血患者,并由皮肤科医生对其进行检查,记录任何存在的皮肤状况。同时采集血样检查血清铁蛋白水平。数据使用 SPSSv25 进行分析。

结果

共有 113 名患者纳入研究。队列的平均年龄为 9.32 ± 4.54 岁。队列的平均铁蛋白水平为 3334 ± 1676 微克/升。89.4%(n=101)的患者出现皮肤表现,常见的有皮肤干燥(44.2%)、雀斑(39.8%)和瘙痒(44.2%)。我们注意到,有雀斑的患者血清铁蛋白水平显著更高(p=0.00288)。瘙痒的原因似乎不是黄疸(p=0.973)。最后,在输血起始年龄小于 1 岁的患者中,皮肤状况的数量更高(p=0.0011)。

结论

皮肤表现是重型β地中海贫血患者中常见的问题。定期检查这些患者的各种皮肤疾病非常重要,这有助于提高他们的生活质量并降低与皮肤相关的发病率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/501b/10560103/ae8970e7ec83/j_jmotherandchild.20232701.d-23-00047_fig_001.jpg

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