假性甲状旁腺机能减退综合征5例报道及文献复习  

Radiological Manifestations of Pseudohypoparathyroidism: A Report of Five Cases with A Literature Review

作　　者：陈闪 罗建光[2] 白末了[2] 刘军[2] 李德泰[2] 

机构地区：[1]长沙县第一人民医院放射科 [2]中南大学湘雅第二医院放射科,长沙410011

出　　处：《临床放射学杂志》2002年第3期222-225,共4页Journal of Clinical Radiology

摘　　要：目的　探讨假性甲状旁腺机能减退综合征的X线表现和诊断方法。资料与方法　回顾分析 5例经临床和实验室证实的典型病例的手、足、颅骨X线表现并文献复习。结果　掌 (跖 )指 (趾 )骨对称性不成比例短缩畸形5例 ,颅内基底节和脑实质钙化 3例 ,骨质疏松 ,颅板增厚、板障变窄 ,下颌角变浅各 2例。结论　掌 (跖 )指 (趾 )骨对称性不成比例短缩畸形 ,尤其是第 4、5掌骨和拇指末节指骨变短是该病特征性X线表现 ,结合临床表现和实验室检查发现低血钙、高血磷 ,甲状旁腺激素 (PTH)含量正常或升高 。Objective To study the radiological manifestations of pseudohypoparathyroidism (PHP), or Seabright Bantam syndrome, and its diagnostic technique.Materials and Methods The X ray findings of hands, feet and head in 5 cases with clinically and laboratorially proved PHP were retrospectively analyzed. The relevant medical literature was reviewed.Results Symmetrical and disproportional shortening of the metacarpals, metatarsals and phalanges were found in all cases. Shortened 4 th and 5 th metacarpals were seen in all cases, while shortened distal phalanges of the thumbs in 4. Basal ganglia and brain parenchyma calcification were observed in 3 cases, while osteoporosis, calvarium thickening, narrowed diplo? and flattened mandibular angle in 2 cases each.Conclusion Symmetrical and disproportional shortening of the metacarpals, metatarsals, phalanges, and abnormal calcification are the common radiological findings in PHP. Shortening of the 4th, 5th metacarpals and distal phalanx of the thumb is the characteristic sign. Based on the radiological signs, clinical history and laboratory findings, such as hypocalcemia, hyperphosphatemia, normal or increased parathyroidism (PTH) level and no response to PTH therapy, etc., the disease can be correctly diagnosed.

关 键 词：骨疾病 假性甲状旁腺功能减退综合征 放射摄影术 X线诊断 病因 诊断 治疗 

分 类 号：R582.2[医药卫生—内分泌]