Children's Hospital, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland.
Haemophilia. 2013 Mar;19(2):200-5. doi: 10.1111/hae.12021. Epub 2012 Sep 19.
Adults with haemophilia have a higher incidence of chronic kidney disease than general male population. We recently showed that children with haemophilia have higher urinary calcium excretion and lower whole body bone mineral density than controls in spite of prophylaxis with the deficient coagulation factor concentrate, serum vitamin D concentrations comparable to those of healthy children and physically active lifestyle. Persistent hypercalciuria may result in nephrocalcinosis and impact renal function. This study sought to assess persistence of urinary calcium excretion and kidney function in children with haemophilia. We investigated retrospectively urinary calcium excretion in 30 children with haemophilia (mean age 12.5 years) from consecutive urine samples over a 2-year period. Renal evaluation included blood and urine specimen, blood pressure, and renal ultrasound. High number of children with haemophilia had intermittent hypercalciuria. Hypercalciuria was not associated with age, severity of haemophilia or previous hypercalciuria. Kidney function and renal ultrasound were normal with the exception of suspected kidney stone in one patient with haemophilia and transient hypercalciuria. Vitamin D concentrations improved after the families had received information and recommendations concerning vitamin D substitution. Our findings indicate that haemophilia per se predisposes to hypercalciuria which may in turn affect bone mineral content and kidney function. Whether childhood-onset intermittent hypercalciuria contributes to hypertension and renal complications in adulthood remains to be elucidated in future studies.
成人血友病患者的慢性肾脏病发病率高于普通男性人群。我们最近发现,尽管使用缺乏的凝血因子浓缩物进行预防,儿童血友病患者的尿钙排泄量较高,全身骨矿物质密度较低,而其血清维生素 D 浓度与健康儿童相当,且生活方式积极。持续的高钙尿症可能导致肾钙沉着症并影响肾功能。本研究旨在评估儿童血友病患者尿钙排泄和肾功能的持续性。我们回顾性地研究了 30 名儿童血友病患者(平均年龄 12.5 岁)在 2 年内连续尿液样本中的尿钙排泄情况。肾脏评估包括血液和尿液样本、血压和肾脏超声检查。大量儿童血友病患者间歇性高钙尿症。高钙尿症与年龄、血友病严重程度或既往高钙尿症无关。除了一名血友病患者疑似肾结石和短暂性高钙尿症外,肾功能和肾脏超声检查均正常。在向患者家属提供有关维生素 D 替代的信息和建议后,维生素 D 浓度有所改善。我们的研究结果表明,血友病本身易导致高钙尿症,进而可能影响骨矿物质含量和肾功能。儿童期间歇性高钙尿症是否会导致成年期高血压和肾脏并发症,仍需在未来的研究中阐明。