Kinetics of Triiodothyronine and Thyroxine in a Case of Thyroxine-Binding Globulin Deficiency with Hypothyroidism

Sato, Toshiro; Yamaguchi, Toru; Yoshida, Katsumi; Demura, Reiko; Yamamoto, Makiko; Aida, Mitsuyasu; Minai, Koji; Saito, Shintaro

doi:10.1507/endocrine1927.49.11_1354

Cited by 3 publications

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“…So the mode of inheritance of decreased TBG capacity in this family may be explained by an X-chromosome linked semi-dominant trait as was pointed out by Nikolai andShishiba. (Nikolai andSeal, 1966: Shishiba et al 1970) In the T4 kinetics of the propositus, the half life of T4 in blood was definitely shortened compared to a normal child, in agreement with previous reports (Ingbar, 1961: Beisel et al, 1962: Nicoloff et al, 1964: Yoshida et al, 1967: Refetoff and Selenkow, 1968: Shishiba et al, 1970: Sakurada et al 1973; While the extrathyroidal T4 iodine and degradation of T4 was decreased from the normal level, in agreement with the thyroid status of this patient.…”

Section: Discussionsupporting

confidence: 89%

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Familial Thyroxine-Binding Globulin Deficiency in a Patient with Congenital Hypothyroidism

Niimi

Sasaki

1975

Endocrinol Japon

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SynopsisA 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 2.1-10.2 pg/ 100 ml) 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 et al., indicating that the inheritance was an X-linked semi-dominant trait. The kinetics of thyroxine (T4) was investigated in a case of partial TBG deficiency with congenital hypothroidism (propositus).The half-life of T4 was shortened, turnover rate increased, extrathyroidal organic iodine and degradation rate of T4 decreased compared to normal values.

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Section: Discussionsupporting

confidence: 89%

“…(Nakagawa et al, 1967;Kashima, 1970: Yuri et al, 197E;Shishiba et al, 1970;. Shiomi et al, 1971;Okano et al, 1971;, Kato et al, 1972;Sakurada et al, 1973) This paper presents an investigation of a kindred with TBG deficiency, the propositusof which has congenital hypothyroidism with ectopic (sublingual) thyroid.…”

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confidence: 99%

Familial Thyroxine-Binding Globulin Deficiency in a Patient with Congenital Hypothyroidism

Niimi

Sasaki

1975

Endocrinol Japon

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“…J Dermatology (Tokyo) 1983;10: 157-60. 6 SUMMARY A boy with congenital hypothyroidism and hereditary raised thyroxine binding globulin is described. This hitherto unreported combination resulted in under treatment of the thyroid deficiency until serum thyroid stimulating hormone measurement became routinely available.…”

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confidence: 99%

Congenital hypothyroidism with hereditary, raised thyroxine binding globulin.

Archer¹,

O'Malley²,

Swift³

1985

Archives of Disease in Childhood

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ism seems unlikely, and an error in handling or assay is more probable. Measurement of thryroid stimulating hormone might have enabled the diagnosis considerably earlier.We suggest that full assessment of thyroid function be carried out in all cases of hypertrichosis or abnormal distribution of body hair. If hypothyroidism is noted and treated the loss of body hair may be dramatic and, to the patient, extremely rewarding.

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Inherited Disorders of Thyroid Metabolism*

1983

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In summary, we have presented a brief survey of the current state of knowledge of inherited disorders of thyroid metabolism. Analysis of cases shows that the biochemical classification covers a wide range of abnormalities and it is likely that further biochemical studies will increase this heterogeneity as well as refining it. Genetic studies are often incomplete, and few in number compared with the classical study by Hutchison and McGirr of Scottish tinker families. Most important, this survey indicates that further research is needed to elucidate the precise molecular mechanisms of the working of the iodide pump, the oxidation and iodination and coupling mechanisms. Study of animal models and DNA sequencing and hybridization work will continue to expand our understanding of abnormalities of thyroglobulin metabolism. We urgently need to find the key to resistance of peripheral and pituitary tissues to thyroid hormone. Subtle dyshormonogenetic abnormalities may await discovery in the field of multinodular goiter and intrathyroidal calcification with goiter. Neonatal screening for hypothyroidism is likely to expand the number of cases for investigation and detailed study. There is an important relationship of dyshormonogenesis to follicular carcinoma. It is hoped that in time we will be able to transform inborn errors into areas of understanding in the realm of the thyroid gland.

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Kinetics of Triiodothyronine and Thyroxine in a Case of Thyroxine-Binding Globulin Deficiency with Hypothyroidism

Cited by 3 publications

References 0 publications

Familial Thyroxine-Binding Globulin Deficiency in a Patient with Congenital Hypothyroidism

Familial Thyroxine-Binding Globulin Deficiency in a Patient with Congenital Hypothyroidism

Congenital hypothyroidism with hereditary, raised thyroxine binding globulin.

Inherited Disorders of Thyroid Metabolism*

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