X-LINKED HYPOPHOSPHATEMIA IN ADULTS - PREVALENCE OF SKELETAL RADIOGRAPHIC AND SCINTIGRAPHIC FEATURES
X-LINKED HYPOPHOSPHATEMIA IN ADULTS - PREVALENCE OF SKELETAL RADIOGRAPHIC AND SCINTIGRAPHIC FEATURES
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DOI：
10.1148/radiology.171.2.2539609
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发表时间：
1989-05-01
期刊：
影响因子：
19.7
通讯作者：
WHYTE, MP
中科院分区：
文献类型：
--
作者：
HARDY, DC;MURPHY, WA;WHYTE, MP
本文回顾了38例X连锁低磷血症（XLH）成人患者的放射学研究，以确定其放射学特征的患病率，比较男性和女性的结果，并通过比较年轻、中年和老年患者的结果来阐明疾病的自然史。骨强化线是常见的，但没有建立特征性的矿物质改变。较松的区域在老年受试者中更普遍。骨关节炎是常见的，发生在脚踝，膝盖，脚，骶髂关节和手腕。末端病在年轻组中并不常见，但在中老年组的每个成员中都存在，并且经常伴有额外的听小骨。下肢长骨的弯曲在所有年龄组中都很常见。XLH中发现三种新的骨骼改变是常见的：髂翼张开、股骨远端梯形髁和距骨形态改变，包括距骨颈缩短和距骨圆顶变平。17例受试者的~（99）Tc亚甲基二膦酸盐X线图常出现异常，表现为关节弓状畸形和骨干皮质及关节周围和关节外区域的局灶性示踪剂蓄积。平均代谢指数中度升高（4.0）。男性的放射学和免疫学检查结果更严重，与半合子一致。在两种性别中，未经治疗的XLH的自然史的特征是在成年期间发生各种年龄相关的骨骼异常。
The radiologic studies of 38 essentially untreated adults with X-linked hypophosphatemia (XLH) were reviewed to determine the prevalence of radiologic features, to compare the findings in men and in women, and to elucidate the natural history of the disease by comparing the findings in young, intermediate-age, and older patients. Bone-reinforcement lines were common, but no characteristic mineral mass alteration was established. Looser zones were more prevalent in older subjects. Osteoarthritis was common, occurring in the ankles, knees, feet, sacroiliac joints, and wrists. Enthesopathy was infrequent in the younger group but was present in every member of the intermediate and older groups and was often accompanied by extra ossicles. Curvatures of the lower-extremity long bones were common in all age groups. Three new skeletal alterations in XLH were found to be common: flaring of the iliac wings, trapezoidal distal femoral condyles, and alterations in talar morphology, including shortening of the talar neck and flattening of the talar dome. Technetium-99m methylene diphosphonate scintigrams of 17 subjects were often abnormal, depicting bowingde formity and focal tracer accumulation in diaphyseal cortices and in periarticular and extraarticular regions. The mean metabolic index was moderately elevated (4.0). Both radiographic and scintigraphic findings were more severe in men, consistent with hemizygosity. The natural history of untreated XLH in both sexes is characterized by the development of a variety of age-related skeletal abnormalities during adulthood.