Zunaira Zulfiqar, Ayesha Kanwal, Manahil Chaudhry, Muhammad Aadil, Sehrish Qaiser, Ayesha Malik, Mohammad Abdullah
{"title":"严重β地中海贫血患者的皮肤表现。","authors":"Zunaira Zulfiqar, Ayesha Kanwal, Manahil Chaudhry, Muhammad Aadil, Sehrish Qaiser, Ayesha Malik, Mohammad Abdullah","doi":"10.34763/jmotherandchild.20232701.d-23-00047","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Material and methods: </strong>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.</p><p><strong>Results: </strong>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).</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":73842,"journal":{"name":"Journal of mother and child","volume":"27 1","pages":"142-146"},"PeriodicalIF":0.0000,"publicationDate":"2023-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10560103/pdf/","citationCount":"0","resultStr":"{\"title\":\"Cutaneous Manifestations in Patients with Beta-Thalassemia Major.\",\"authors\":\"Zunaira Zulfiqar, Ayesha Kanwal, Manahil Chaudhry, Muhammad Aadil, Sehrish Qaiser, Ayesha Malik, Mohammad Abdullah\",\"doi\":\"10.34763/jmotherandchild.20232701.d-23-00047\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>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.</p><p><strong>Material and methods: </strong>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.</p><p><strong>Results: </strong>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).</p><p><strong>Conclusion: </strong>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.</p>\",\"PeriodicalId\":73842,\"journal\":{\"name\":\"Journal of mother and child\",\"volume\":\"27 1\",\"pages\":\"142-146\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2023-10-08\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10560103/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of mother and child\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.34763/jmotherandchild.20232701.d-23-00047\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2023/6/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of mother and child","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.34763/jmotherandchild.20232701.d-23-00047","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2023/6/1 0:00:00","PubModel":"eCollection","JCR":"","JCRName":"","Score":null,"Total":0}
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.