A Hagag Adel, S El-Farargy Mohamed, M Abo El-Enein Amany
Pediatricsand Clinical Pathology.
Departments, Faculty of Medicine, Tanta University, Egypt.
Int J Hematol Oncol Stem Cell Res. 2015 Apr 1;9(2):60-6.
Sickle-cell anemia is characterized by defective hemoglobin synthesis with production of sickle hemoglobin. Sickle red blood cells become deformed and rigid with difficulty to pass through narrow capillaries and frequent clotting and thrombosis leading to repetitive vascular occlusions and progressive organ damage. We conducted this work to study some adrenal functions using ACTH stimulation test in Egyptian children with sickle cell anemia in correlation with iron overload.
This study was conducted on 60 children with sickle cell anemia who were attendants of Hematology unit, Pediatric department, Tanta university hospital in the period from April 2012 to May 2014 including 34 males and 26 females with their age ranging from 5-17 years and main age value of 13±2.9 in comparison with 30 healthy children matched for age and sex as a control group. For all patients the following were done: Complete blood count, Hb electrophoresis, serum ferritin, iron, iron binding capacity, sodium and potassium, random blood glucose, thyroid functions , morning basal cortisol levels and adrenocorticotrophic hormone stimulation test. Results : There was significantly lower basal morning cortisol in patients than controls (mean value in patients were 8.78±3.53 ug/dl compared with 11.79±2.32 ug/dl in control group with p value of 0.021). No significant differences in adrenocorticotrophic hormone stimulation test between patients and controls were detected. (Mean value in patients were 23.078±3.709 ug/dl compared with 24.492±5.006 ug/dl in control group with p value of 0.389). Significant negative correlation was found between serum cortisol and ferritin in patients group (r= 0.625 and p value =0.003) CONCLUSION: There was significantly lower basal morning cortisol in sickle-cell anemia patients with significant negative correlation with iron overload so regular follow up to adrenal functions to detect any adrenal impairment, as SCD patients are vulnerable to adrenal hypo function, is recommended.
镰状细胞贫血的特征是血红蛋白合成缺陷并产生镰状血红蛋白。镰状红细胞会变形且僵硬，难以通过狭窄的毛细血管，并且频繁凝血和形成血栓，导致反复的血管阻塞和进行性器官损伤。我们开展这项工作，通过促肾上腺皮质激素（ACTH）刺激试验研究埃及镰状细胞贫血儿童的一些肾上腺功能，并与铁过载相关联。
本研究于2012年4月至2014年5月期间，对坦塔大学医院儿科血液科的60名镰状细胞贫血儿童进行，其中包括34名男性和26名女性，年龄在5至17岁之间，平均年龄为13±2.9岁，同时选取30名年龄和性别匹配的健康儿童作为对照组。对所有患者进行了以下检查：全血细胞计数、血红蛋白电泳、血清铁蛋白、铁、铁结合能力、钠和钾、随机血糖、甲状腺功能、早晨基础皮质醇水平以及促肾上腺皮质激素刺激试验。结果：患者的早晨基础皮质醇显著低于对照组（患者的平均值为8.78±3.53微克/分升，而对照组为11.79±2.32微克/分升，p值为0.021）。未检测到患者和对照组在促肾上腺皮质激素刺激试验方面有显著差异。（患者的平均值为23.078±3.709微克/分升，对照组为24.492±5.006微克/分升，p值为0.389）。在患者组中，血清皮质醇与铁蛋白之间存在显著负相关（r = 0.625，p值 = 0.003）。结论：镰状细胞贫血患者的早晨基础皮质醇显著降低，且与铁过载呈显著负相关，因此建议对肾上腺功能进行定期随访，以检测任何肾上腺功能损害，因为镰状细胞病患者易患肾上腺功能减退。
