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目的比较甲状旁腺激素不敏感综合征(pseudohypoparathyroidism,PHP)与原发性甲状旁腺功能减退症(hypoparathyroidism,HPP)临床特点的异同。方法收集1993年1月-2015年12月在本院住院确诊的36例PHP和62例HPP患者的临床资料,总结两组的临床特点差异及疗效。结果 PHP组就诊年龄、身高、维生素D3以及甲状腺素(TT_4)、游离甲状腺素(FT_4)、促黄体生成素(luteinizing hormone,LH)、促卵泡激素(follicle-stimulating hormone,FSH)水平均低于HPP组(P<0.05);PHP组的碱性磷酸酶[(194.1±125.9)U/L vs(83.6±50.5)U/L]、总Ⅰ型前胶原氨基端延长肽[(409.7±433.4)μg/L vs(51.5±109)μg/L]、骨吸收标记物β-胶原降解产物[(1.8±1.1)ng/ml vs(0.4±0.4)ng/ml]、尿钙[(1.1±1.1)mmol/24 h vs(2.0±1.8)mmol/24 h)]以及骨转换标记物骨钙素[(68.4±56.2)ng/ml vs(13.7±15.3)ng/ml]水平均高于HPP组(P<0.05)。PHP组手足搐搦(80.6%vs 54.8%)、骨骼异常(55.6%vs 8.1%)、异位钙化(69.4%vs 30.6%)等方面的发生率较HPP组高。两组应用碳酸钙D3 600 mg 1~2次/d、骨化三醇0.25μg 1~2次/d治疗后,手足搐搦发作频率明显降低。结论 PHP和HPP组临床表现均为程度不同的甲状旁腺功能减退症。对于年轻、有骨骼体态异常及有家族史患者,应注意甲状腺功能、性腺激素、骨代谢生化标记物等的检查,以提高PHP的早期诊断率。
Objective To compare the clinical characteristics of pseudohypoparathyroidism (PHP) and primary hypoparathyroidism (HPP). Methods The clinical data of 36 cases of PHP and 62 cases of HPP diagnosed in our hospital from January 1993 to December 2015 were collected, and the differences of clinical characteristics and therapeutic effects between the two groups were summarized. Results The age, height, vitamin D3, thyroid hormone (TT_4), free thyroxine (FT_4), luteinizing hormone (LH) and follicle-stimulating hormone (FSH) (P <0.05); alkaline phosphatase in the PHP group [(194.1 ± 125.9) U / L vs (83.6 ± 50.5) U / L], total type Ⅰ procollagen amino terminal prolonging peptide [(409.7 ± 433.4) μg / L vs 51.5 ± 109 μg / L], bone resorption markers β-collagen degradation product [(1.8 ± 1.1) ng / ml vs ) (mmol / 24 h vs (2.0 ± 1.8) mmol / 24 h)], and osteocalcin (68.4 ± 56.2 ng / ml vs 13.7 ± 15.3 ng / ml) (P <0.05). The incidence of tetany (80.6% vs 54.8%), skeletal abnormalities (55.6% vs 8.1%) and ectopic calcifications (69.4% vs 30.6%) were higher in the PHP group than in the HPP group. The frequency of episodes of tetany was significantly lower in both groups when calcium carbonate D3 600 mg 1 to 2 times / d and calcitriol 0.25 μg 1 to 2 times / day were used. Conclusion The clinical manifestations in both PHP and HPP groups are different degrees of hypoparathyroidism. For young, skeletal abnormalities and family history of patients, should pay attention to thyroid function, gonadal hormones, biochemical markers of bone metabolism and other tests to improve the PHP early diagnosis rate.