Familial Thyroxine-Binding Globulin Deficiency

Shishiba, Yoshimasa; Shimizu, Taeko; Yoshimura, S; Shizume, Kazuo

doi:10.1507/endocrine1927.46.1_28

Cited by 3 publications

(4 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…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%

See 1 more Smart Citation

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

Niimi

Sasaki

1975

Endocrinol Japon

View full text Add to dashboard Cite

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.

show abstract

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.…”

mentioning

confidence: 99%

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

Niimi

Sasaki

1975

Endocrinol Japon

View full text Add to dashboard Cite

show abstract

“…There are many studies on the T4 and T3 kinetics in familial T B G deficiency. As for T4 kinetics, it has been reported that the half-time was shortened, the fractional turnover rate and distribution space were in-creased, but the extrathyroidal pool was reduced, the degradation rate being normal (Ingbar, 1961 ;Nikolai and Seal, 1967;Bayley et al, 1969;Shishiba et al, 1970;Suzuki et al, 1973;Sakurada et al, 1974). As to T3 kinetics, on the other hand, the longer half-time and decreased degradation rate were reported (Cavalieri et al, 1971;McConnon et al, 1972;Sakurada el al., 1974).…”

Section: Resultsmentioning

confidence: 99%

Serum thyrotropin response to thyrotropin-releasing hormone and free thyroid hormone indices in patients with familial thyroxine-binding globulin deficiency.

Konno

1976

Endocrinol Japon

View full text Add to dashboard Cite

The response in serum thyrotropin (TSH) to synthetic thyrotropin-releasing hormone (TRH) as well as serum free thyroxine index (FT4I) and free triiodothyronine index (FT3I) was investigated in six patients with familial thyroxine-binding-globulin (TBG) deficiency. The total serum thyroxine (T4) and triiodothyronine (T3) concentrations were significantly decreased, compared with those of normal subjects (3,4 +/- 0.9 microng/dl, mean +/- S.D. vs. 9.0 +/- 1.5 microng/dl, p less than 0.01 and 87 +/- ng/dl vs. 153 +/- 37 ng/dl, p less than 0.01, respectively). FT4I was lower than the normal range in all but one (5.3 +/- 1.5 vs. 8.9 +/- 1.6, p less than 0.01), whereas FT3I was all in the normal range and of no significant difference from the normal control (132 +/- 22 vs 148 +/- 25). Serum TSH concentrations in TBG deficiency were all in the normal range (1.0-4.2 micronM/ml) and the maximum TSH increments following TRH 500 microng iv were 8.9 +/- 2.0 micronU/ml and of no significant difference from the normal control (10.2 +/- 4.5 micronU/ml). These results indicate that the euthyroid state in familial TBG deficiency is more clearly defined by TRH-test and the normal response to TRH in familial TBG deficiency is presumably under the control of the serum free T3 level rather than the serum free T4 level.

show abstract

“…Such an approach was expected to provide the more meaningful comparison of TBG-affinity, based on the theoretical consideration described in our previous paper (Shimizu et al, 1972). (Sterling et al, 1966, andShishiba et al, 1970a). the same as that of original sera when diluted by the same barbital buffer to the same extent.…”

mentioning

confidence: 99%

A Renewal Attempt to Compare the Affinity of Thyroxine-Binding-Globulin To Thyroxine in Normal And Hyperthyroid Sera

et al. 1973

Self Cite

View full text Add to dashboard Cite

Familial Thyroxine-Binding Globulin Deficiency

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

Serum thyrotropin response to thyrotropin-releasing hormone and free thyroid hormone indices in patients with familial thyroxine-binding globulin deficiency.

A Renewal Attempt to Compare the Affinity of Thyroxine-Binding-Globulin To Thyroxine in Normal And Hyperthyroid Sera

Contact Info

Product

Resources

About