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家族性低磷血症性维生素D抵抗性佝偻病的早期病史。三例自出生起观察病例的报告。

Early history of familial hypophosphataemic vitamin D-resistant rickets. Report of three cases observed since birth.

作者信息

Schimert G, Fanconi A

出版信息

Helv Paediatr Acta. 1983 Oct;38(4):383-98.

PMID:6317614
Abstract

Three patients with familial hypophosphataemic vitamin D-resistant rickets (FHR) born to affected mothers have been clinically, radiologically and biochemically observed since birth. The value of the different early diagnostic signs is evaluated, and the efficiency of early phosphate and vitamin D treatment is discussed. Our observations suggest the following conclusions: Infants of FHR affected mothers can be diagnosed as having the disease by careful physical examinations and by a pathological increase of serum alkaline phosphatase with normal serum calcium, before radiological signs of rickets occur. The serum phosphate concentrations in early infancy are variable and of little or no diagnostic help. Early treatment is useful to cure or even to avoid rickets and to normalize serum alkaline phosphatase, but not hypophosphataemia. In spite of some favorable results, the influence of early started treatment on growth and leg deformities is difficult to assess due to the variations of individual expressivity of the disease.

摘要

对3名母亲患有家族性低磷性维生素D抵抗性佝偻病(FHR)的患儿自出生起就进行了临床、放射学和生物化学观察。评估了不同早期诊断体征的价值,并讨论了早期磷酸盐和维生素D治疗的效果。我们的观察结果得出以下结论:患有FHR的母亲所生婴儿,在出现佝偻病的放射学体征之前,通过仔细的体格检查以及血清碱性磷酸酶病理性升高而血清钙正常,可诊断为患有该病。婴儿早期血清磷酸盐浓度变化不定,对诊断帮助不大或没有帮助。早期治疗有助于治愈甚至避免佝偻病,并使血清碱性磷酸酶恢复正常,但不能纠正低磷血症。尽管有一些良好的结果,但由于该疾病个体表现的差异,很难评估早期开始治疗对生长和腿部畸形的影响。

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