Familial Thyroxine-Binding Globulin Deficiency in a Patient with Congenital Hypothyroidism
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概要
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A kindred with deficiency of thyroxine binding globulin (TBG) was presented. The propositus, a 1-year old female also had congenital hypothyroidism with ectopic (sublingual) thyroid. Decrease in TBG-binding capacity (TBG values ranging from 2.1-10.2μg/ 100 m<I>l</I>) was detected in the seven relatives on the maternal side. Of these subject 2 were male and 5 female; the males had the lowest binding capacities. But six members of paternal relatives were entirely normal in this respect. The mode of inheritance of the abnormality in this family was compatible with the presumption of Nikolai et al. and Shishiba <I>et al</I>., indicating that the inheritance was an X-linked semi-dominant trait. The kinetics of thyroxine (T<SUB>4</SUB>) was investigated in a case of partial TBG deficiency with congenital hypothroidism (propositus). The half-life of T<SUB>4</SUB> was shortened, turnover rate increased, extrathyroidal organic iodine and degradation rate of T<SUB>4</SUB> decreased compared to normal values.
- 社団法人 日本内分泌学会の論文
著者
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Niimi Hiroo
Department Of Pediatrics Chiba University School Of Medicine
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Sasaki Nozomu
Department Of Pediatrics Saitama Medical College
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Sasaki Nozomu
Department of Pediatric Cardiology and Cardiovascular Surgery, Saitama Heart Institute, Saitama Medical School Hospital
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SASAKI NOZOMU
Department of Pediatrics, Chiba University School of Medicine
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