摘要:

SUMMARYA congenitally goitrous, mentally retarded, hypothyroid child, whose parents were first cousins, was studied for the cause of a strongly positive (86–5%) perchlorate discharge test. The mother had a recidivant goitre after being thyroidectomized in 1971. Her perchlorate discharge test was negative, but she displayed an exaggerated TSH response to TRH administration. The father also showed a negative perchlorate discharge, but he had a large cold nodule in the left thyroid lobe. The father also presented with a retinitis pigmentosa, detected additionally in two of his brothers and three nephews.Thyroid tissue, obtained from the three patients at surgery, was assayed for thyroid peroxidase (TPO) by three different assays:1iodination of goitre thyroglobulin;2oxidation of guaiacol;3oxidation of iodide.In a standard iodination assay (100 μM I−) and in a standard guaiacol assay 4–7 mM guaiacol) the TPO activity in the son's tissue was extremely low. Preincubation with haematin did not restore activity. The father displayed a normal TPO activity, but that of the mother was somewhat low. In the iodide oxidation assay employing 12 mM I−in the incubation system, and in iodination and guaiacol assays employing elevated concentrations of substrate, the TPO activity in the son's tissue was quite appreciable and was much closer to that of the parents. Presumably, therefore, the defect in the son's thyroid did not involve an absence of TPO. Rather, the results suggest a qualitative defect in the son's TPO, involving an abnormality in the binding of substrate for oxidation.Thyroglobulin was isolated from the thyroid soluble fraction by sucrose density gradient centrifugation and its protein and iodine content determined. Thyroglobulin from the son's thyroid tissue contained only 0–043% iodine, compared to 0–45% and 0–29% for mother and father, respectively. The very low iodine content of the son's thyroglobulin accords with the low thyroid peroxidase activity and with the clinical and laboratory findings.The fact that the parents in the present study are first cousins and that both showed some type of thyroid abnormality suggests that the severely affected son (goitre, mental retardation, and hypothyroidism) is homozygous for a recessi

ISSN:0300-0664    

DOI:10.1111/j.1365-2265.1982.tb02628.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

SUMMARYWe have tested the hypothesis that physiological puerperal hyperprolactinaemia may be secondary to reduced hypothalamic dopaminergic inhibition of prolactin (PRL) release. Nine post‐partum females with physiological hyperprolactinaemia (aged 19–40 years; mean basal PRL±SE, 2099±257 mU/l, range 1002–3762 mU/l) were studied and results compared with fourteen normoprolactinaemic females (basal PRL<400 mU/l; aged 18–36 years). Puerperal hyperprolactinaemic females showed a reduced TSH response to dopamine (DA) receptor blockade with metoclopramide (10 mg i.v.) compared with normal females over a 60‐min period following drug administration (total incremental TSH change, mean ±SE, mU/l; 0.5±0.3 v. 3.1±1.0,P<0.005). This finding of reduced dopaminergic inhibition of TSH release in females with physiological puerperal hyperprolactinaemia supports the view that an overall reduction in hypothalamic dopaminergic tone may contribute towards hyperprolactinaemia in pos

ISSN:0300-0664    

DOI:10.1111/j.1365-2265.1982.tb02629.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

ISSN:0300-0664    

DOI:10.1111/j.1365-2265.1982.tb02630.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

SUMMARYThe pulsatile secretion of gonadotrophins and ovarian steroids was studied in normal women at different stages of the menstrual cycle. The concentration of LH, FSH, Prolactin (PRL), oestradiol and progesterone were measured in samples of plasma collected every 15 min for 6 h and the frequency and amplitude of each episodic pulse of hormone estimated. Although significant fluctuations occurred in the concentration of each hormone, LH showed the most easily identifiable pulses the frequency of which increased significantly from the early follicular to the late follicular phase of the cycle (3.4±0.3 v. 4.4±0.2 pulses 6 hP<0.01). During the luteal phase the basal concentration of LH (5.6±0.9 U/l), pulse amplitude (7.4±1.7 U/l) and frequency (1.6±0.2/6 h) were much lower than at any stage of the follicular phase (P<0.001). The concentration of FSH and PRL showed a similar but less marked change to that of LH throughout the menstrual cycle with a significant decline in both basal concentration and pulse frequency in the luteal phase of the cycle. Although only 47% of all LH pulses were associated with a pulse of FSH, 70% of FSH and prolactin pulses occurred within 15 min of an LH pulse.The basal concentration of oestradiol increased significantly from the early follicular to the late follicular phase of the cycle (P<0.001). There was evidence of episodic secretion of oestradiol with the frequency of pulses declining from a maximum of 4.3±0.6/6 h in the mid‐follicular phase to 1.8±0.5/6 h in the luteal phase (P<0.02). In the follicular phase 74–80% of LH pulses were followed within 100 min by a significant rise in the concentration of oestradiol. It is suggested that:1the secretion of LH, FSH, PRL, oestradiol and progesterone is episodic in nature;2a significant number of secretory episodes of FSH, LH and PRL are coincidental;3the increased secretion of oestradiol from the pre‐ovulatory follicle is associated with a rise in the frequency of episodic

ISSN:0300-0664    

DOI:10.1111/j.1365-2265.1982.tb02631.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

SUMMARYA 24‐year‐old negress with the triad of acanthosis nigricans, hirsutism associated with polycystic ovaries and insulin resistance is reported. Metabolic studies were done 3 years after a bilateral ovarian wedge resection. Partial remission of the hirsutism and return of menstrual cycles occurred after surgery. Extreme resistance to endogenous and exogenous insulin was observed. Three studies of insulin receptors on circulating red blood cells (RBC) showed abnormal inhibition‐competition curves, characterized by increased percentage insulin binding at higher unlabelled insulin levels. Scatchard plots suggested an apparent increase in the number of low affinity receptors. Despite the changes in receptor‐insulin interaction, the defect does not seem to explain the insulin resistance since binding of insulin to a target tissue (RBC) appeared to be quantitatively normal at physiological insulin levels, suggesting a simultaneous post receptor

ISSN:0300-0664    

DOI:10.1111/j.1365-2265.1982.tb02632.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

SUMMARYThe serum concentrations of 25‐hydroxycholecalciferol (25 OH D3), 24,25‐dihydroxycholecalciferol [24,25(OH)2D3] and 1,25‐dihydroxycholecalciferol [1,25(OH)2D3] were measured in twenty‐one patients with untreated hyperthyroidism. Compared with control subjects, 25 OH D3concentrations were not altered, 24,25(OH)2D3concentrations were increased, although not significantly and 1,25(OH)2D3concentrations were decreased (P= 0.01). Following oral carbimazole therapy, 24,25(OH)2D3concentrations fell (P<0.01), 1,25(OH)2D3concentrations increased (P<0.01) and 25 OH D3concentrations were unchanged. The altered 1,25(OH)2D3and 24,25(OH)2D3concentrations found in hyperthyroidism are probably due to the effects of thyroid hormone on bone and mineral metabolism. Increased serum calcium and phosphate concentrations with secondary hypoparathyroidism result in stimulation of the renal 24‐hydroxylase and suppression of the 1‐hydroxylase enzymes. In addition, serum 24,25(OH)2D3concentrations were significantly correlated with serum triiodothyronine levels (T3) (r= 0.66,P<0.002) before treatment. This may indicate a direct stimulatory effect of T3 on 24‐hydroxylase activity. No relationship was found between serum 1,25(OH)2D3concentrations before therapy

ISSN:0300-0664    

DOI:10.1111/j.1365-2265.1982.tb02633.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

SUMMARYSpecific binding of [125I]‐insulin was measured in erythrocytes and fat obtained at the time of mastectomy in twenty‐three subjects. There was a significant negative correlation between the level of specific binding to fat and fasting insulin concentration (r= 0.55,P<0.05,n= 23) suggesting down‐regulation of fat insulin receptors by insulin. Insulin binding to erythrocytes did not correlate with insulin level nor was there any relationship between insulin binding to erythrocytes and fat from the same subject. Thus while the erythrocyte insulin receptor may be of interest in its own right, it may not be subject to the normal regulatory influences seen in classical insulin target tissues. Insulin binding to erythrocytes may therefore be invalid as a model to study down‐regulation of insulin re

ISSN:0300-0664    

DOI:10.1111/j.1365-2265.1982.tb02634.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

SUMMARYThe excretion of 6‐sulphatoxy melatonin in urine has been shown to provide a reliable index of melatonin production in man. Daily measurements made in two healthy ovulating women showed that both the total amount excreted/day and the proportion excreted during the sleep period remained relatively constant for each subject throughout the menstrual cycle. These data do not support the view that there are changes in melatonin production which correlate with cyclical reproductive endocrine function in the huma

ISSN:0300-0664    

DOI:10.1111/j.1365-2265.1982.tb02635.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

SUMMARYIn ten patients with Graves' disease before, during and after treatment and three patients in remission, we have compared the TSH‐binding inhibition caused by serum, by 1.6 M ammonium sulphate precipitates from serum and by the fractions (19S, 7S and 4S) obtained after G‐200 gel chromatography of serum and ammonium sulphate precipitates.Five out of ten patients had a positive thyrotrophin‐binding inhibitor immunoglobulin (TBII) index before treatment, three of whom relapsed. The binding inhibition caused by 7S fractions from ammonium sulphate precipitates of the ten untreated patients could be correlated with their TBII index (r= 0.9,P<0.001).By contrast, the 7S fractions prepared directly from the sera of the same ten patients all showed marked TSH‐binding inhibition (P0.05).It is concluded that ammonium sulphate precipitates, which are used to calculate the TBII index, do not reflect the presence of anti‐TSH receptor antibodies in serum accurately in a

ISSN:0300-0664    

DOI:10.1111/j.1365-2265.1982.tb02636.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

SUMMARYTwo adult patients are described in whom131I therapy for thyroid carcinoma was followed by the development of non‐Hodgkin's lymphoma in salivary glands. Histologically one was lymphocytic and the other histiocytic. The total doses of131I given were 27 GBq (675 mCi) and 14 GBq (350 mCi), lymphoma presenting 10 and 3 years respectively after the initial therapeutic dose of131I. We suggest that the relationship is a causal one, because of the rarity of salivary gland lymphoma and the high dose of radiation to the salivary glands with this mode of therap

ISSN:0300-0664    

DOI:10.1111/j.1365-2265.1982.tb02637.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY