Familial Inherited TSH Deficiency

2019 ◽  
pp. 7-22
Author(s):  
Geraldo Medeiros-Neto
Keyword(s):  
Tsh Deficiency

Novel Autoimmune Polyendocrine Syndrome: Adult Combined GH, Prolactin and TSH Deficiency Associated with Circulating PIT-1 Antibody

2011 ◽  
pp. OR16-1-OR16-1
Author(s):  
Yutaka Takahashi ◽  
Masaaki Yamamoto ◽  
Genzo Iguchi ◽  
Ryoko Takaeno ◽  
Yasuhiko Okimura ◽  
...  
Keyword(s):  
Autoimmune Polyendocrine Syndrome ◽  
Tsh Deficiency

scholarly journals Outcome in surgically treated Rathke's cleft cysts: long-term monitoring needed

2011 ◽  
Vol 165 (1) ◽  
pp. 33-37 ◽  
Author(s):  
R Trifanescu ◽  
V Stavrinides ◽  
P Plaha ◽  
S Cudlip ◽  
J V Byrne ◽  
...  
Keyword(s):  
Visual Field ◽  
Visual Field Defects ◽  
Rathke's Cleft Cysts ◽  
Long Term Monitoring ◽  
Rathke's Cleft ◽  
Term Monitoring ◽  
Field Defects ◽  
Tsh Deficiency

ObjectiveTo clarify the outcome of all cases of Rathke's cleft cysts (RCC) treated surgically and followed up in Oxford during a long-term period.Subjects and methodsThe records of all patients with RCC seen in the Department of Endocrinology between January 1978 and June 2009 were reviewed.ResultsA total of 33 patients (20 females, median age 43 years) were identified. At presentation, major visual field defects were detected in 58% of patients and gonadotrophin, ACTH and TSH deficiency in 60, 36 and 36% of patients respectively. Desmopressin treatment was required in 18% of patients. Treatment consisted of cyst evacuation combined with or without biopsy/removal of the wall. Post-operatively, visual fields improved in 83% of patients with impairment, whereas there was no reversal of ACTH or TSH deficiency or of diabetes insipidus. All but one subject had imaging follow-up during a mean period of 48 months (range 2–267). Cyst relapse was detected in 22% of patients at a mean interval of 29 months (range 3–48 months); in 57% of them, the recurrence was symptomatic. Relapse-free rates were 88% at 24-months and 52% at 48-months follow-up. At last assessment, at least quadrantanopia was reported in 19% of patients, gonadotrophin, ACTH and TSH deficiency in 50, 42 and 47% of patients respectively. Desmopressin treatment was required in 39% of patients.ConclusionsIn this study of patients with RCC and long-term follow-up, we showed a considerable relapse rate necessitating long-term monitoring. Surgical intervention is of major importance for the restoration of visual field defects, but it does not improve endocrine morbidity, which in the long-term affects a substantial number of patients.

ABNORMAL THYROXINE METABOLISM IN HYPOSOMATOTROPHIC DWARFISM AND INHIBITION OF RESPONSIVENESS TO TRH DURING GH THERAPY

PEDIATRICS ◽  
1973 ◽  
Vol 51 (4) ◽  
pp. 668-674
Author(s):  
Basil A. Porter ◽  
Samuel Refetoff ◽  
Robert L. Rosenfield ◽  
Leslie J. De Groot ◽  
Victor S. Fang ◽  
...  
Keyword(s):  
Growth Rate ◽  
Human Growth ◽  
Free Thyroxine ◽  
Total Serum ◽  
Gh Therapy ◽  
Gh Treatment ◽  
Degradation Rates ◽  
Secondary Hypothyroidism ◽  
Tsh Deficiency

Thyrotropin (TSH) release and thyroxine metabolism have been studied in order to determine whether human growth hormone (GH) administration is causally related to the occasional development of secondary hypothyroidism in GH-deficient dwarfs. Five hyposomatotrophic dwarfs and one normal child were studied. The TSH response to synthetic thyrotropin-releasing hormone (TRH) was normal in all, regardless of thyroid functional status. Before GH administration, two of the three clinically euthyroid dwarfs were found to have daily thyroxine degradation rates in the hypothyroid range although serum total and free thyroxine levels were normal. Following six to ten days of GH treatment, TSH levels fell slightly and the TSH response to TRH was blunted. During long-term GH treatment of four dwarfs no significant change in thyroid function was observed. In addition, TSH responsiveness to TRH recovered in three of these four chronically treated patients, including one who was frankly hypothyroid. The growth rate of the three clinically euthyroid dwarfs improved during long-term GH therapy; nevertheless, the addition of 50µg triiodothyronine to the treatment regimen of the dwarf in whom the thyroxine degradation rate was lowest resulted in a markedly improved growth rate. These studies indicate that a state of hypothyroidism that is undetectable by measurement of total serum thyroxine and free thyroxine exists in some GH-deficient patients. GH may be necessary for normal thryoxine metabolism and action. Such a GH effect might explain the observed influence of GH on TSH release. On the other hand, the demonstrated abnormality in thyroxine metabolism may result from mild, preexisting TSH deficiency.

Isolated TSH deficiency presenting as myxedema heart disease

JAMA ◽  
1971 ◽  
Vol 217 (2) ◽  
pp. 205-207 ◽  
Author(s):  
M. P. Gupta
Keyword(s):  
Heart Disease ◽  
Tsh Deficiency

RESPONSE OF NORMAL, HYPOTHYROID AND HYPOTHALAMO-PITUITARY INSUFFICIENT CHILDREN TO SYNTHETIC THYROTROPHIN-RELEASING HORMONE

1971 ◽  
Vol 51 (3) ◽  
pp. 483-488 ◽  
Author(s):  
G. MILHAUD ◽  
P. RIVAILLE ◽  
M. S. MOUKHTAR ◽  
E. BINET ◽  
J. C. JOB
Keyword(s):  
Time Course ◽  
Solid Phase ◽  
Thyroid Stimulating Hormone ◽  
Normal Children ◽  
Thyrotrophin Releasing Hormone ◽  
Releasing Hormone ◽  
Tsh Secretion ◽  
Course Changes ◽  
Serum Tsh ◽  
Tsh Deficiency

SUMMARY Thyrotrophin-releasing hormone (TRH) was synthesized by the solid phase technique, administered to 13 children, and the time-course changes in the serum level of thyroid-stimulating hormone (TSH) assessed. In eight normal children, peak levels of TSH occurred 20 min after the injection, and circulating TSH remained significantly raised for 60 min. In three hypothyroid children, the increase in serum TSH was much greater than in normal children, suggesting the existence of large pituitary TSH stores. In two hypopituitary children with TSH deficiency, TSH reserves seemed normal. One of these patients had a craniopharyngioma; after operation, the increase in serum TSH was reduced. These results show that assay of serum TSH after administration of synthetic TRH provides a test which distinguishes pituitary from hypothalamic defects affecting TSH secretion.

Results of external pituitary irradiation after unsuccessful transsphenoidal surgery in Cushing's disease

1991 ◽  
Vol 125 (4) ◽  
pp. 470-474 ◽  
Author(s):  
A. Vicente ◽  
J. Estrada ◽  
C. de la Cuerda ◽  
B. Astigarraga ◽  
M. Marazuela ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
High Efficiency ◽  
Cushing's Disease ◽  
Anterior Pituitary Function ◽  
Low Incidence ◽  
Pituitary Irradiation ◽  
Age Range ◽  
Pituitary Adrenal Axis ◽  
Tsh Deficiency

Abstract. Fourteen adult patients (10 females and 4 males; age range 20-60 years) with persistent Cushing's disease after transsphenoidal microsurgery were treated with pituitary irradiation. Supervoltage multiportal administration was employed at a total dose of 50 Gy (±0.65 sd). The interval between microsurgery and pituitary irradiation was less than 6 months in 6 patients and more than 6 months in 8. The pituitary-adrenal axis was evaluated postsurgically, before irradiation and every 6 months thereafter. The remaining anterior pituitary function was simultaneously tested. Remission rates at 12 months and 24 months after radiotherapy were 61 and 70%, respectively. Two patients developed TSH deficiency and another gonadotropin deficiency during the follow-up after radiation. We conclude that pituitary irradiation is the treatment of choice for persistent Cushing's disease after unsuccessful surgery because of its high efficiency and low incidence of adverse reactions when compared with other forms of treatment.

scholarly journals Megavoltage pituitary irradiation lowers but seldom leads to safe GH levels in acromegaly: a long-term follow-up study

1998 ◽  
pp. 160-163 ◽  
Author(s):  
NC Thalassinos ◽  
S Tsagarakis ◽  
G Ioannides ◽  
I Tzavara ◽  
C Papavasiliou
Keyword(s):  
Additional Data ◽  
External Beam ◽  
Follow Up Study ◽  
Long Term Follow Up ◽  
Biochemical Cure ◽  
Definition Of ◽  
Pituitary Irradiation ◽  
Tsh Deficiency

Radiotherapy (RT) has long been used in the treatment of acromegaly, but confusion regarding the definition of biochemical cure has hampered interpretation of previous reports on the outcome of this treatment. In the present study we present additional data using the currently accepted criteria of biochemical cure in a large group of patients followed up by our department. Forty-six acromegalic patients were treated with external beam megavoltage RT and followed up for a mean of 7.6 years (range 2-22 years). Only four patients had had previous surgical treatment by either transsphenoidal or transfrontal routes. Following RT, mean basal GH levels decreased from 30.9 ng/ml (5-96 ng/ml) to 11.5 ng/ml (1-36 ng/ml) at 10 years of follow up with a further fall to 6.1 ng/ml (1-29 ng/ml) in those patients followed up for more than 10 years. As a result, although mean GH levels of less than 5 ng/ml were achieved in 9/28 (30.1%) at 5 years, 6/19 (31.6%) at 10 years, and in 6/11 (54.5%) of those patients followed up for more than 10 years post-RT, only 0/28 (0%), 7/28 (25%), 4/19 (21%) and 1/11 (1%) achieved GH levels of <2.5 ng/ml at 2, 5. 10 and >10 years following RT. Thus, in the whole series only 10/48 (20.8%) patients showed a decrease of GH level to less than 2.5 ng/ml at their latest follow up. Hypopituitarism as a result of RT was only infrequently observed in this series; gonadal deficiency developed in 12 (26.6%) patients, thyrotrophin (TSH) deficiency in 3 (6.6%) and adrenocorticotrophin deficiency in 2 (4.4%). In conclusion, megavoltage RT is an effective treatment for the control of GH hypersecretion in acromegaly, with a continuing lowering effect for several years following RT but seldom leads to safe GH levels.

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