Emine Tugba ALATAS, Volkan KARAKUS, Suzan Demir PEKTAS, Gursoy DOGAN, Gokhan PEKTAS, Fatif MEHMET AZİK

Analysis of Skin, Hair and Nail Diseases in the Adults with Beta Thalassemia Major

Amaç: ?-talasemi majör (BTM), çoklu organlarda fonksiyonel ve fizyolojik bozukluklar ile karakterize, sık kan transfüzyonu gerektiren genetik bir hastalıktır. Yetişkin BTM hastalarında ve sağlıklı bireylerde kontrollerde cilt, saç ve tırnak hastalıklarının yaygınlıklarını ve bunların klinik ve laboratuvar özellikleriyle olan ilişkilerini araştırmayı amaçladık. Materyal ve Yöntem: İleriye dönük olarak, buna bağlı deri, saç ve tırnak hastalıklarının yaygınlığını analiz ettik. Katılımcıların demografik özellikleri, laboratuar bulguları, saç, tırnak ve saç bulgularını kaydettik. Bulgular: Araştırmaya toplam 71 BTM hastası (36 erkek ve 35 kadın) ve 50 yaş ve cinsiyet ile uygun sağlıklı bireyler (36 kadın ve 36 erkek) dahil edildi. Hastaların 32'sinde (%45.1) ve kontrol gruplarının 3'ünde (%4.2) saç bulguları, hastaların 26'sında (%36.6) ve kontrol gruplarının 6'sında (%8.3) tırnak bulguları tespit edildi. En yaygın deri bulguları kserozis 66 (% 93), saç bulguları, telogen effluvium 16 (% 22.5), tırnak bulguları, distrofik tırnak 9 (% 12.7) hastada tespit edildi. BTM'li hastalarda, kontrol grubuna göre anlamlı derecede yüksek sıklıkta kserozis, yara, pigmentasyon bozukluğu, nevüs, pruritus, enfeksiyon, tırnak ve saç hastalıkları vardı. Pruritus ve nevüs hastalarının ortalama yaş önemli derecede yüksekti. Erkek hastalarda postenflamatuvar hiperpigmentasyon ve melanositik nevüs, kadın hastalardan daha yüksekti. Kadın hastalarda telogen effluvium, erkek hastalardan daha yüksekti. Efelid, diffuse bronz pigmentasyon (DBP) ve telojen effluim bulunan hastaların ferritin düzeyleri anlamlı olarak yüksekti. Sonuç: BTM'li hastalarda deri hastalıklarının gelişiminde yaş, cinsiyet, ilaç öyküsü ve ferritin düzeyi önemli faktörlerdir

Erişkin Beta Talasemi Major Hastalarında Eşlik Eden Deri, Saç ve Tırnak Bulgularının Araştırılması

Objective: β-thalassemia major (BTM) is a genetic disorder necessitating frequent blood transfusions, which is characterized by functional and physiological disorders in multiple organs. We aimed to research the prevalence of associated skin, hair, and nail disorders, and their relationship with clinical and laboratory features of the disease in adult BTM patients and healthy control groups. Materials and Methods: We prospectively analyzed the prevalence of associated skin, hair, and nail disorders. We recorded demographic features, laboratory findings skin, hair, and nail disorders in the participants. Results: Atotal of 71 BTM patients (36 males and 35 females) and 50 age and sex matched healthy individuals (36 females and 36 males) were included in the study. 32 (45.1%) of the patients and 3 (4.2%) of the control groups had hair, 26 (36.6%) of the patients and 6 (8.3%) of control groups had nail diseases. The most common skin findings xerosis were determined in 66 (93%), hair findings, telogen effluvium were determined in 16 (22.5%), and nail findings, dystrophic nail were determined 9 (12.7%) in patients. BTM patients had significantly higher fruquent xerosis, scar, pigmentation disorders, nevus, pruritus, infections, nail and hair disorders than control groups. Average age of patients with pruritus and nevus were significantly enhanced. Postinflammatory hyperpigmentation and melanocytic nevus in male patients had more higher than female patients. Telogen effluvium in female patients was more higher than male patients. Ferritin levels of the patients with ephelide, diffuse bronze pigmentation (DBP) and telogen effluim had significantly enhanced. Conclusion: Age, gender, medication history and ferritin level are important factors for the development of dermatological disorders among BTM patients

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