[Endocrine disorders in patients with transfusion-dependent hereditary anemias].

Q4 Medicine Problemy endokrinologii Pub Date : 2022-07-27 DOI:10.14341/probl13149
A V Vitebskaya, Ekaterina S Bugakova, E A Pisareva, Yu V Tikhonovich
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Abstract

Often transfusions red blood cells in patients with hereditary anemias lead to iron overload, that can cause endocrine complications, such as growth retardation, hypothyroidism, hypogonadism, and disorders of carbohydrate metabolism.Clinical case 1. A boy with transfusion-dependent (TD) Diamond-Blackfan anemia at 16.3 years presented with impaired fasting glucose, growth hormone (GH) deficiency, hypogonadotropic hypogonadism; GH therapy was initiated. At the age of 16.8 years old secondary hypothyroidism, secondary hypocorticism and diabetes mellitus were diagnosed. At 17.2 years continuous glucose monitoring (CGM) detected glucose elevations up to 11.7 mmol/l. Therapy with GH and testosterone ethers was continued; levothyroxine and cortef were stopped by patient. At 17.9 years height was 163 cm; no data supporting hypothyroidism nor hypocorticism; glycaemia within goal range.Clinical case 2. A girl with TD beta-thalassemia major at the age of 11.5 years presented with GH deficiency; GH therapy has been conducted from 12.8 to 15.3 years of age. At 13.8 years retardation of pubertal development was diagnosed. At 15.0 hyperglycemia 7.2 mmol/l was detected; normal results of oral glucose tolerance test (OGTT) were observed; glycemia elevations were up to 9.5 mmol/l according to CGM data. At 16.0 height was 152 cm; because of pubertal development arrest hormone replacement therapy was prescribed.CONCLUSION. Growth, pubertal and carbohydrate metabolism disorders were diagnosed in patients with TD hereditary anemias, that confirms the necessity of regularly endocrine investigation. To detect impairment of carbohydrate metabolism investigation of fasting blood glucose, OGTT, and CGM is recommended; glycated hemoglobin measurement is not considered reasonable.

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[输血依赖性遗传性贫血患者的内分泌紊乱]。
遗传性贫血患者经常输红细胞导致铁超载,从而引起内分泌并发症,如生长迟缓、甲状腺功能减退、性腺功能减退和碳水化合物代谢紊乱。临床病例1。1例16.3岁输血依赖性(TD) Diamond-Blackfan贫血的男孩,表现为空腹血糖受损、生长激素(GH)缺乏、促性腺功能低下;开始生长激素治疗。16.8岁时诊断为继发性甲状腺功能减退、继发性皮质功能减退、糖尿病。在17.2岁时,连续血糖监测(CGM)检测到血糖升高高达11.7 mmol/l。继续使用生长激素和睾酮醚治疗;患者停用左甲状腺素和cortef。17.9岁时身高163厘米;没有数据支持甲状腺功能减退或皮质减退;血糖在目标范围内。临床病例2。1例患有TD -地中海贫血的女孩,11.5岁时表现为生长激素缺乏症;生长激素治疗从12.8岁到15.3岁进行。13.8岁时被诊断为青春期发育迟缓。15.0时高血糖7.2 mmol/l;口服糖耐量试验(OGTT)正常;根据CGM数据,血糖升高高达9.5 mmol/l。16.0时身高152厘米;由于青春期发育停滞,给予激素替代治疗。TD遗传性贫血患者的生长发育、青春期发育及碳水化合物代谢紊乱均有诊断,证实了定期进行内分泌调查的必要性。为了检测碳水化合物代谢的损害,建议调查空腹血糖、OGTT和CGM;糖化血红蛋白测量被认为是不合理的。
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来源期刊
Problemy endokrinologii
Problemy endokrinologii Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
1.40
自引率
0.00%
发文量
59
期刊介绍: Since 1955 the “Problems of Endocrinology” (or “Problemy Endocrinologii”) Journal publishes timely articles, balancing both clinical and experimental research, case reports, reviews and lectures on pressing problems of endocrinology. The Journal is aimed to the most topical issues of endocrinology: to chemical structure, biosynthesis and metabolism of hormones, the mechanism of their action at cellular and molecular level; pathogenesis and to clinic of the endocrine diseases, new methods of their diagnostics and treatment. The Journal: features original national and foreign research articles, reflecting world endocrinology development; issues thematic editions on specific areas; publishes chronicle of major international congress sessions and workshops on endocrinology, as well as state-of-the-art guidelines; is intended for scientists, endocrinologists diabetologists and specialists of allied trade, general practitioners, family physicians and pediatrics.
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