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首页> 外文期刊>Joint, bone, spine : >Osteomalacia in a patient with primary intestinal lymphangiectasis (Waldmann's disease).
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Osteomalacia in a patient with primary intestinal lymphangiectasis (Waldmann's disease).

机译：原发性肠淋巴管扩张症（沃德曼氏病）患者的骨软化症。

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Primary intestinal lymphangiectasis (PIL), also known as Waldmann's disease, is a rare protein-losing enteropathy characterized by abnormal enlargement of the lymphatic ducts in the bowel wall. The symptoms usually start in early infancy. We report a case of osteomalacia in a 63-year-old patient with delayed-onset of PIL, for which she was on dietary treatment. She presented with a 3-year history of mechanical pain in the back and pelvis. Mild ascites and edema with functional impairment of the lower limbs were noted. The neurological evaluation was normal. Blood tests showed hypocalcemia, hypophosphatemia, alkaline phosphatase elevation, and evidence of intestinal malabsorption. Radiographs of the pelvis disclosed a fracture, Looser's zones in the iliopubic rami and left femoral neck, and a washed-out appearance of the vertebras. Dual-energy X-ray absorptiometry showed bone loss with T-score values of -1.2SD at the lumbar spine and -2.5SD at the femoral necks. A diagnosis of osteomalacia related to vitamin D deficiency was given. Serum 25-OH-vitamin D was 18.2ng/ml (normal, 20-40ng/ml) and serum parathyroid hormone was 620pg/ml (normal, 15-65pg/ml), suggesting secondary hyperparathyroidism. Intramuscular vitamin D was given, together with oral calcium and an adequate diet. At follow-up 8 months later, small improvements were noted in the symptoms and absorptiometry findings.
机译：原发性肠淋巴管扩张症（PIL），也称为Waldmann病，是一种罕见的蛋白质丢失性肠病，其特征是肠壁淋巴管异常增大。症状通常始于婴儿早期。我们报告了一名63岁的PIL延迟发作患者的骨软化症，她正在接受饮食治疗。她具有3年的背部和骨盆机械疼痛病史。注意到轻度腹水和水肿，下肢功能受损。神经学评估正常。血液检查显示血钙过低，血磷过低，碱性磷酸酶升高以及肠吸收不良的迹象。骨盆的X线照片显示骨折，pub骨耻骨区和左股骨颈的Looser区域以及椎骨的外露。双能X线骨密度仪显示骨质流失，腰椎的T评分为-1.2SD，股骨颈的T评分为-2.5SD。诊断为维生素D缺乏相关的骨软化症。血清25-OH-维生素D为18.2ng / ml（正常20-40ng / ml），血清甲状旁腺激素为620pg / ml（正常15-65pg / ml），提示继发性甲状旁腺功能亢进。给予肌内维生素D以及口服钙和适当饮食。在8个月后的随访中，症状和吸光度法检查发现有轻微改善。

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