Endocrine profile of β-thalassemia major patients followed from childhood to advanced adulthood in a tertiary care center.

作者: Vincenzo De Sanctis , Heba Elsedfy , AshrafT Soliman , IhabZaki Elhakim , NadaA Soliman

DOI: 10.4103/2230-8210.183456

关键词:

摘要: Aim: Chronic iron overload resulting from frequent transfusions, poor compliance to efficient chelation therapy and chronic liver disease is basically responsible for the most severe complications of thalassemia major (TM). Before conventional treatment, TM was entirely childhood with a very short survival. Today, survival improved 40–50 years becomes prevalent adulthood in near future it will be one senility. Furthermore, clinical phenotype changing age appearance heart endocrine glands that require special health care well-informed specialists. Objectives: The aims our study were to: (1) Imprint profile long-lived patients; (2) evaluate retrospectively cumulative incidence diseases; (3) identify potential risk factors; (4) orient physicians modified relative patients' needs. Design: A retrospective cross-sectional followed by same physician tertiary clinic. Participants: Forty-three patients (mean age: 50.3 ± 10.8 years; range: 45.8–59.5 23 females) studied. Patients Methods: An extensive medical history, detailed laboratory data, complications, current treatments, obtained. Results: data indicate 88.4% adult suffered at least complication. majority developed second decade life when serum ferritin level high (12/23 female 8/20 male patients, levels diagnosis above 5.000 ng/ml). Conclusions: These underline bone are highly necessitate close monitoring, follow-up. Physicians' strategies optimize include identifying who developing organ damage, plans, promoting compliance, educating patients. Several aspects remain elucidated such as growth impairment glucose tolerance relation hepatitis C virus infection. affordable worldwide-established long-term treatment protocols hypogonadism osteoporosis needed.

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