Congenital Goiter with Hypothyroidism and Iodo-Serum Albumin Replacing Thyroglobulin

Lissitzky, Serge; Codaccioni, J L; Bismuth, J; Depieds, R

doi:10.1210/jcem-27-2-185

Cited by 41 publications

(22 citation statements)

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“…7). It was reported that serum albumin was iodinated in a patient with congenital goiter whose thyroid lacked TG [18]. In the present patient T4 and T3 showed a peak at the same fraction as TG, but not albumin.…”

Section: Defective Intracellular Processing Of the Mutant Tgcontrasting

confidence: 40%

“…Her physical and mental development was normal. Laboratory examination showed mild hypothyroidism (total T4 38 ng/ml [normal 50-130], total T310 ng/ml [normal [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23], TSH 6.1 mU/l [normal <9]). 123I uptake was 68.8% 3 h after administration and the iodide discharge test by KSCN was negative.…”

Section: Methodsmentioning

confidence: 99%

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Missense Mutation (C1263R) in the Thyroglobulin Gene Causes Congenital Goiter with Mild Hypothyroidism by Impaired Intracellular Transport.

et al. 1998

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Section: Defective Intracellular Processing Of the Mutant Tgcontrasting

confidence: 40%

Section: Methodsmentioning

confidence: 99%

Missense Mutation (C1263R) in the Thyroglobulin Gene Causes Congenital Goiter with Mild Hypothyroidism by Impaired Intracellular Transport.

et al. 1998

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“…This iodoalbumin has ialso been identified from the serum of the patients (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15). From the poorly iodinated iodoalbumin, low peptide-linked levels of hormone were already reported (8,11). However the detection of iodoalbumin is not specific for this type of goiter.…”

Section: Discussionmentioning

confidence: 99%

“…They were found after hydrolysis of the serum (1) or the thyroidal iodoproteins (7) as "resistant to digestion" material, remaining at the origin of the chromatograms (2) or insoluble in acid butanol (9). They were detected in the urine of patients (8,11) and presumed to be shortchain polypeptides (2), suggesting a iodoprotein breakdown defect. In the urine of the patients studied, a large part of these BII compounds was identified as iodohistidines.…”

Section: Discussionmentioning

confidence: 99%

“…An iodoalbumin has been identified in similar goiters (6), and thyroglobulin has been reported as reduced (3)(4)(5)7) or apparently absent (2,(8)(9)(10)(11). From similar patients, other butanol-insoluble compounds were detected either in thyroid (2,4,7) or serum (1,4,8) hydrolysates, and in urines (2,3,10,11). These compounds, constituting the butanol-insoluble iodine (BII)' moiety of the urine, were not identified.…”

Section: Introductionmentioning

confidence: 99%

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Studies on Mono- and Diiodohistidine. II. CONGENITAL GOITROUS HYPOTHYROIDISM WITH THYROGLOBULIN DEFECT AND IODOHISTIDINE-RICH IODOALBUMIN PRODUCTION

Savoie¹,

Massin²,

Savoie³

1973

J. Clin. Invest.

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A B S T R A C T Butanol-insoluble iodinated compounds in the urine of patients with congenital goiters have been generally regarded as iodopeptides. Monoiodohistidine (MIH) and diiodohistidine (DIH) were identified from the urine of four patients with congenital goitrous hypothyroidism. From radioiodine studies, 40-70% of the urinary radioactivity was in the iodide-free fraction from which about 40% was identified as MIH and DIH by crystallizations to a constant specific activity.Iodotyrosines were simultaneously identified in the urine. However the presence of an iodotyrosine-deiodinase activity was demonstrated in the two removed goiters with a normal Km for MIT. In vivo iodotyrosine deiodination was normal for hypothyroid subjects.No thyroglobulin was identified in the thyroids from these patients. The major iodoprotein was iodoalbumin which, after in vivo labeling, contained 84-89% of the total soluble protein radioactivity. The thyroxine content of the goiter iodoalbumins and other iodoproteins was extremely low.Iodohistidines were identified in comparable proportions in the iodoalbumin and in the other iodoproteins isolated from each goiter. The average iodohistidine content of these proteins as crystallizable MIH and DIH was in the individual cases 15 and 4% of the in vivo incorporated radioiodine. DIH was identified in all iodoprotein fractions. The mean DIH/MIH ratios from the individual cases were 1.16 and 0.35. The corresponding DIT/MIT ratios were 3.19 and 1.45, respectively.The major consequence of this thyroglobulin defect is the iodination of inappropriate proteins (mainly albumin) resulting in low yields of thyroxine and high Received for publication 6 March 1972 and in revised form 19 September 1972. yields of iodohistidines. Iodohistidines from the goiter iodoproteins were not deiodinated and, at least for MIH, were quantitatively excreted in the urine of these patients. From the MIH iodoalbumin content and the MIH urinary excretion, goiter iodoalbumin turnover estimates were made and, although elevated, could not maintain a normal thyroxine secretion.The urinary excretion of iodohistidines easily demonstrated by column chromatography is offered as a test for detecting this variety of congenital goiter.

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The pathology of dyshormonogenetic goitre

Kennedy

1969

The Journal of Pathology

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Congenital Goiter with Hypothyroidism and Iodo-Serum Albumin Replacing Thyroglobulin

Cited by 41 publications

References 0 publications

Missense Mutation (C1263R) in the Thyroglobulin Gene Causes Congenital Goiter with Mild Hypothyroidism by Impaired Intracellular Transport.

Missense Mutation (C1263R) in the Thyroglobulin Gene Causes Congenital Goiter with Mild Hypothyroidism by Impaired Intracellular Transport.

Studies on Mono- and Diiodohistidine. II. CONGENITAL GOITROUS HYPOTHYROIDISM WITH THYROGLOBULIN DEFECT AND IODOHISTIDINE-RICH IODOALBUMIN PRODUCTION

The pathology of dyshormonogenetic goitre

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