Aleem A, Al-Momen A K, Al-Harakati M S, Hassan A, Al-Fawaz I
Department of Medicine and Pediatrics, King Khalid University Hospital, Riyadh, Saudi Arabia.
Ann Saudi Med. 2000 Sep-Nov;20(5-6):364-6. doi: 10.5144/0256-4947.2000.364.
This is a retrospective analysis of case records of AA(2)-thalassemia major patients who developed hypoparathyroidism (HPT). The objective of this study was to assess the prevalence of hypocalcemia and hypoparathyroidism in AA(2)-thalassemia major patients being followed at King Khalid University Hospital (KKUH), Riyadh, Saudi Arabia.
Diagnosis was based on low serum calcium (S/Ca), high serum phosphate (Po4), normal serum magnesium and alkaline phosphatase, and low serum parathyroid hormone levels. Other parameters analyzed included age, sex, serum ferritin levels, age of onset of HPT, any symptoms of hypocalcemia, and presence of other complications in these patients.
Out of 40 patients, eight (20%) were diagnosed to have HPT. The mean age at diagnosis was 13.6 years (range 11-16 years), mean serum calcium was 1.88 mmol/L (range 1.58-2.04), mean serum ferritin was 7490 AA(1/4)g/L (range 2000-23,064) and mean serum phosphate was 1.88 mmol/L (range 1.50-2.73). Serum parathyroid hormone (PTH) levels were low in most of the patients. Only two patients (25%) had mild symptoms of hypocalcemia. Growth retardation was present in all patients, while four patients had liver dysfunction, two had diabetes mellitus and two had cardiac dysfunction.
HPT due to iron overload may develop in a significant number of thalassemia major patients, especially when chelation therapy is not optimal, therefore, all thalassemics should be carefully watched for this complication from early in their second decade.
这是一项对发生甲状旁腺功能减退症(HPT)的重型α地中海贫血(AA(2)-地中海贫血)患者病例记录的回顾性分析。本研究的目的是评估在沙特阿拉伯利雅得的哈立德国王大学医院(KKUH)接受随访的重型AA(2)-地中海贫血患者中低钙血症和甲状旁腺功能减退症的患病率。
诊断依据为血清钙(S/Ca)降低、血清磷酸盐(Po4)升高、血清镁和碱性磷酸酶正常以及血清甲状旁腺激素水平降低。分析的其他参数包括年龄、性别、血清铁蛋白水平、HPT发病年龄、低钙血症的任何症状以及这些患者中其他并发症的存在情况。
40例患者中,8例(20%)被诊断为患有HPT。诊断时的平均年龄为13.6岁(范围11 - 16岁),平均血清钙为1.88 mmol/L(范围1.58 - 2.04),平均血清铁蛋白为7490 μg/L(范围2000 - 23,064),平均血清磷酸盐为1.88 mmol/L(范围1.50 - 2.73)。大多数患者的血清甲状旁腺激素(PTH)水平较低。只有2例患者(25%)有轻度低钙血症症状。所有患者均有生长发育迟缓,4例患者有肝功能障碍,2例有糖尿病,2例有心脏功能障碍。
大量重型地中海贫血患者可能因铁过载而发生HPT,尤其是当螯合治疗不理想时,因此,所有地中海贫血患者从第二个十年早期就应密切观察这一并发症。