Endocrinologia Japonica

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ISSN / EISSN : 0013-7219 / 2185-6370
Published by: Japan Endocrine Society (10.1507)
Total articles ≅ 2,929
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Yukihiro Hasegawa, Tomonobu Hasegawa, Tetsuo Yokoyama, Shinobu Kotoh, Yutaka Tsuchiya
Published: 1 January 1992
Endocrinologia Japonica, Volume 39, pp 165-167; https://doi.org/10.1507/endocrj1954.39.165

Abstract:
We present here a 13-year-old male with hypopituitarism which accompanied an insidious and gradual progress of ACTH deficiency. ACTH deficiency finally led to an overt crisis of adrenal insufficiency at the age of 12 years and 7 months. This patient is unique because the insidious and gradual progress has been proved by not only the laboratory results but also the clinical course for over 13 years. The cause of panhypopituitarism including ACTH deficiency is thought to have existed before or at the delivery because of the stalk transection seen on the magnetic resonance image (MRI). At the crisis, his laboratory results suggested that he had secondary adrenal insufficiency, whereas he showed normal adrenal function proved by the insulin tolerance test (ITT) at the age of 4 years. Abrupt crisis of secondary adrenal insufficiency developed at the age of 12 years, although he had been well until the crisis.
Reiko Demura, Mitsuhide Naruse, Masahiro Isawa, Noritaka Onoda, Kiyoko Naruse, Minoru Yamakado, Hiroshi Demura
Published: 1 January 1992
Endocrinologia Japonica, Volume 39, pp 169-176; https://doi.org/10.1507/endocrj1954.39.169

Abstract:
A patient with a rare combination of prolactinoma and aldosterone producing adrenal adenoma (APA) was reported in relation to studies concerning dopaminergic regulation of PRL and aldosterone secretion. The patient is a 38-year-old female with plasma PRL and aldosterone concentrations (PAC) of 563 ng/ml and 54 ng/dl, respectively. A bolus of 10 mg of metoclopramide significantly increased plasma PRL in 6 normal subjects and in 4 patients with APA, whereas the responses were blunted in 7 patients with prolactinoma and in our patient. The response of aldosterone to metoclopramide was less than that of PRL, but similar in all studied subjects, indicating that the dopaminergic inhibition of aldosterone secretion is less than that of PRL in normal subjects and did not change in patients with APA or prolactinoma. Oral administration of 2.5 mg of bromocriptine suppressed plasma PRL significantly in all the subjects studied, but did not produce any consistent changes in PAC. Discrepancies in the response of PRL and aldosterone to metoclopramide and to bromocriptine suggest a difference in the dopaminergic regulation of PRL and aldosterone secretion in both normal subjects and patients with prolactinoma and APA. It is unlikely that reduced dopaminergic inhibition is the basis for hypersecretion of PRL and aldosterone in our patient.
Haruhiko Kouhara, Toyoshi Tatekawa, Masafumi Koga, Shoju Hiraga, Norio Arita, Hiroshi Mori, Bunzo Sato
Published: 1 January 1992
Endocrinologia Japonica, Volume 39, pp 177-184; https://doi.org/10.1507/endocrj1954.39.177

Abstract:
A case of a 29-year-old man with an ACTH-producing pituitary tumor disseminated into the subarachnoid space is described. After total adrenalectomy for Cushing's disease at the age of 15, Nelson's syndrome developed. Transsphenoidal adenomectomy at 17 and 21 years of age, pituitary irradiation and medical therapies with sodium valproate, baclofen and bromocriptine failed to lower his plasma ACTH level. Multiple intracranial and intraspinal tumors associated with the symptoms of left hemiparesis developed. The removal of a tumor grown at the level of C1-3 was performed with successful palliation of his symptoms. Histologically, the tumor cells showed sinusoidal, papillary and diffuse patterns with a preponderance of the former over the latter two, although the papillary pattern predominated in the primary pituitary tumor. Immunohistochemical analysis demonstrated most cells to be positive for ACTH in the metastatic tumor as well as the primary adenoma. The clinical significance of his course is discussed with a review of 11 reported cases with metastatic ACTH-producing pituitary tumors.
Hiroyuki Hashimoto, Noboru Igarashi, Tamotsu Sato, Takuma Hashimoto
Published: 1 January 1992
Endocrinologia Japonica, Volume 39, pp 525-531; https://doi.org/10.1507/endocrj1954.39.525

Abstract:
To clarify whether serum free fatty acid (FFA) is an inhibitor of extrathyroidal conversion (IEC) of thyroxine (T4) to thyronine (T3), we measured the concentration of FFA, IEC activity and thyroid hormones in normal subjects, acute ketotic children and children with low T3 syndrome due to nonthyroidal illness (NTI). Iodothyronine (I) 5'-deiodinase activity was assayed with reverse triiodothyronine (rT3) as substrate and liberated 125I-was measured. The IEC was determined by the inhibition of I 5'-deiodination by ether extract of sera or standard oleate solution. IEC values were represented as mM oleate. The serum concentration of FFA was 0.470 +/- 0.117 (SD) mM in 11 normal subjects, and it was significantly higher (1.242 +/- 0.248 mM; P < 0.01) in 10 acute ketotic children and in 7 samples from 6 NTI children (0.904 +/- 0.530 mM; P < 0.05). In contrast, there was no difference in IEC among three groups (normal subject, 0.451 +/- 0.069 mM; acute ketosis, 0.437 +/- 0.040 mM; NTI, 0.465 +/- 0.224 mM). No correlations were found between IEC activity and the serum FFA concentration or thyroid hormones in 28 samples from three groups. The sequential changes in serum thyroid hormones, FFA and IEC in 3 of 6 NTI children revealed no consistent relationship. Furthermore, one NTI child had significantly high IEC (> 1.000 mM) but its serum FFA (1.182 mM) was below the mean value for the acute ketotic group. These results indicate that 1) many NTI patients may bear no relation to IEC and 2) IEC may not be caused by serum FFA only but includes several factors.
Toshiko Iwai, Shunzo Taii, Hiroshi Ozasa, Takahide Mori
Published: 1 January 1992
Endocrinologia Japonica, Volume 39, pp 539-548; https://doi.org/10.1507/endocrj1954.39.539

Abstract:
To examine whether or not dehydroepiandrosterone sulfate (DHAS) is a substrate for steroidogenesis in the corpus luteum, we studied 17 women in the luteal phase, the follicular phase, and after castration. Following suppression of adrenal function with dexamethasone, DHAS was administered intravenously and the serum levels of DHAS, dehydroepiandrosterone (DHA), androstenedione (ADS), testosterone (T), 17 beta-estradiol (E2) and progesterone (P) were measured serially for 24 h. An obvious increase in the serum levels of all steroids except for E2 and P was observed in each subject for at least 8 h after DHAS administration. To evaluate the effect of DHAS on the serum levels of the steroid hormones, the integrated response area (IRA) was calculated for each hormone in all the subjects. The IRA values for ADS, T and E2 (at 2 and 4 h) in the luteal phase group were significantly higher than in the other DHAS treated groups, and the IRA values for DHA and P tended to be higher than in the other groups. These results suggest that the corpus luteum utilizes serum DHAS as a substrate for steroidogenesis.
GunaM Valli, Muthiah Gladson, Narasimhan Srinivasan, Pera Govindarajulu
Published: 1 January 1992
Endocrinologia Japonica, Volume 39, pp 549-554; https://doi.org/10.1507/endocrj1954.39.549

Abstract:
Increasing evidence of the interaction of glucocorticoids and ovarian steroids prompted the current study. Effects of exogenously administered corticosterone acetate (3.5 mg/100 g b.w/day for one week) were examined on splenic nucleic acids, protein, lactate, and on lactate dehydrogenase (LDH) specific activity and its isozymes in ovariectomized and ovary-intact Wistar rats (65-75 days old). Ovariectomy resulted in no significant change in the parameters studied except DNA which increased significantly. The administration of corticosterone to these rats did not produce any remarkable change in the ovariectomy caused increase in splenic DNA content. Nevertheless, it decreased the ratio of heart type subunits (H)/muscle type subunits (M) [H/M] of LDH isozymes. In the case of ovary-intact rats, corticosterone produced an increase in the concentration of splenic lactate but a decrease in the H/M ratio. Exogenously administered corticosterone exerts selective synergistic interaction with ovarian hormones on splenic lactate. The specific activity of LDH and the concentrations of RNA and protein remained unchanged during the interaction between ovarian hormones and corticosterone.
Zhong Hua Sun, Kaoru Nomura, Shohzoh Toraya, Makoto Ujihara, Nobuo Horiba, Toshihiro Suda, Toshio Tsushima, Hiroshi Demura, Atsushi Kono
Published: 1 January 1992
Endocrinologia Japonica, Volume 39, pp 563-569; https://doi.org/10.1507/endocrj1954.39.563

Abstract:
Adrenal computed tomographic (CT) scanning was conducted in twelve patients with Addison's disease during the clinical course. In tuberculous Addison's disease (n = 8), three of four patients examined during the first two years after disease onset had bilaterally enlarged adrenals, while one of four had a unilaterally enlarged one. At least one adrenal gland was enlarged after onset in all six patients examined during the first four years. Thereafter, the adrenal glands may atrophy bilaterally, in contrast to adrenal glands in idiopathic Addison's disease, which atrophy bilaterally from disease onset (n = 2). Adrenal calcification was a less sensitive clue in tracing pathogenesis, i.e., adrenal calcification was observed in five of eight patients with tuberculous Addison's disease, but not in idiopathic patients. Thus, adrenal CT scanning could show the etiology of Addison's disease (infection or autoimmunity) and the phase of Addison's disease secondary to tuberculosis, which may be clinically important for initiating antituberculous treatment.
Tetsuya Shirotani, Hideki Kishikawa, Nakayasu Wake, Nobuhiro Miyamura, Yasuyoshi Hashimoto, Sadatoshi Motoyoshi, Kohei Yamaguchi, Motoaki Shichiri
Published: 1 January 1992
Endocrinologia Japonica, Volume 39, pp 577-584; https://doi.org/10.1507/endocrj1954.39.577

Abstract:
Thyroxine-binding globulin (TBG) is the major thyroid hormone transport protein. Several inherited TBG variants resulting in partial or complete TBG deficiencies have been shown to be caused by either one or two nucleotide substitutions, or one nucleotide deletion in the coding regions of the TBG gene. In this report, a Japanese female patient (proband) with hyperthyroid state, whose lower TBG levels did not return to normal under the euthyroid state after treatment was examined. Genomic DNA samples from the proband with thyroxine-binding globulin deficiency (termed TBG-Kumamoto) and her family were subjected to the polymerase chain reaction, and the generated DNA fragments were sequenced. A single nucleotide substitution in the codon for the amino acid 363 of native TBG molecule (CCT to CTT) was found, resulting in the replacement of proline by leucine. It was revealed that the proband was a heterozygote and her father was a hemizygote. The mutation was confirmed by the allele-specific amplification of genomic DNAs from the proband and her father using oligonucleotide primers of normal or mutant residues at the 3' position in the polymerase chain reaction. These results indicate that the abnormality of TBG-Kumamoto is the consequence of this mutation. Genetically, this point mutation observed in TBG-Kumamoto might be classified as a new type of TBG deficiency.
Takahiro Mizutani, Masahiro Sakata, Akira Miyake, Osamu Tanizawa, Nobuyuki Terada, Keishi Matsumoto, Naoki Terakawa
Published: 1 January 1992
Endocrinologia Japonica, Volume 39, pp 615-621; https://doi.org/10.1507/endocrj1954.39.615

Abstract:
The in vivo effects of gestrinone (R2323) and medroxyprogesterone acetate (MPA) on the estrogen production by rat ovaries were investigated. Hypophysectomized immature female rats treated with 2.5 or 5 IU of pregnant mare serum gonadotropin (PMS) were daily given vehicle only, gestrinone (0.5 mg/kg body weight) or MPA (10 mg/kg body weight), and the activities of 3 beta-hydroxysteroid dehydrogenase, 17 alpha-hydroxylase, 17, 20-lyase, 17 beta-hydroxysteroid dehydrogenase and aromatase in ovaries of these rats were measured. Gestrinone suppressed the 3 beta-hydroxysteroid dehydrogenase activity and increased activities of 17 alpha-hydroxylase, 17, 20-lyase and aromatase in ovaries stimulated by 5 IU of PMS, while MPA suppressed activities of 17 alpha-hydroxylase and aromatase in these ovaries. On the other hand, the aromatase activity in ovaries stimulated by 2.5 IU of PMS was suppressed by gestrinone and increased by MPA, and neither gestrinone nor MPA affected the production of aromatizable androgens from progesterone by these ovaries. Thus, gestrinone and MPA administrated in vivo showed divergent influences on steroidogenic enzyme activities in ovaries, but they did not affect the serum concentration of estradiol-17 beta. The present results suggest that neither gestrinone nor MPA reduced estrogen production by the rat ovary under the gonadotropin stimulation although they influenced some process of its steroidogenesis.
Kenwa Ohshiro, Shigeki Sakata, Masafumi Matsuda, Takashi Komaki, Masayuki Saito, Eiichi Goshima, Masahisa Kitada
Published: 1 January 1992
Endocrinologia Japonica, Volume 39, pp 245-250; https://doi.org/10.1507/endocrj1954.39.245

Abstract:
We have examined a hypothyroid patient with stimulating type anti-thyrotropin (TSH) receptor antibodies and without blocking type anti-TSH receptor antibodies. Although she had high serum TSH (240 microU/ml) and low free triiodothyronine (FT3, 0.49 pg/ml) concentrations, which agree with physical findings of hypothyroidism, she had an unusually high free thyroxine (FT4) concentration (3.56 ng/dl). Incubation of her serum with 125I-T4, followed by precipitation with 12.5% polyethylene glycol (PEG) disclosed a higher binding of 125I-T4 (34.4%) than in normal controls, being 5-7%. In addition, binding of 125I-T4 to her serum gamma-globulin was completely displaced by the addition of unlabelled T4. From these results it was concluded that her serum contained anti-T4 autoantibodies. Treatment with synthetic T4 was begun and her thyroid function was monitored by sensitive TSH radioimmunoassay (RIA) and RIA of FT4 after PEG treatment. Since both sensitive TSH RIA and FT4 RIA results after PEG treatment give results concordant with the physical findings, it was concluded that both of the RIA results are useful for the evaluation of thyroid function in patients with thyroid hormone autoantibodies.
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