Journal of The Korean Society of Inherited Metabolic disease
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v.13
no.2
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pp.120-125
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2013
Purpose: McCune-Albright syndrome (MAS) is caused by activating mutations in the GNAS gene, resulting in peripheral precocious puberty, caf$\acute{e}$-au-lait spots, and polyostotic fibrous dysplasia (POFD). The aim of the present study was to describe the diverse clinical and endocrine characteristics of patients with MAS. Methods: Seven patients with MAS were included in this study and medical charts were reviewed retrospectively for following parameters: patient's sex and age at diagnosis, POFD, ovarian cysts, and precocious puberty. Results: The mean age at diagnosis was $5.8{\pm}4.2$ years. One patient was male (14%) and the other six patients were female (86%). Peripheral precocious puberty was associated with 6 patients (86%). Five patients manifested premature menarche as early as 2 to 5 years of age. Letrozole was administered to 4 patients, tamoxifen to one patient and GnRH agonist to one patient. Five females developed ovarian cysts. Thyroid function tests were performed in all patients and one patient showed hyperthyroidism (14%) and has been treated with methimazole. One patient presented with pseudohypoparathyroisdism, phosphaturia, calciuria suggesting hypophosphatemic rickets. Six patients (86%) revealed POFD. One patient had symptoms of optic nerve compression and secondary esotropia and 2 patients had bone pain. Conclusion: This study described clinical characteristics and endocrine complications of patients with MAS. Careful physical examinations with history taking and serial endocrine function tests should be needed to detect complications such as endocrinologic hyperfunction and POFD.
Lee, Jong-Ho;Kim, Oh-Yoen;Kim, Ji-Young;Park, Kyoung;Yangsoo Jang
Nutritional Sciences
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v.5
no.1
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pp.13-19
/
2002
A mutation in the promoter region of uncoupling protein 3 (UCF3), specifically the -55C longrightarrow T transition, may influence an individual's energy metabolism and body weight. The objective of this study was to investigate the effect of a weight reduction program on endocrine functions and body fat distribution, related to UCP3 promoter genotype. Ninety overweight pre-menopausal female subjects participated in the weight reduction program at Yonsei University Hospital, and were placed on a calorie-restricted diet (300 kcal less than their daily requirements) for 12 weeks. After 12 weeks, all subjects on the program lost approximately 5% of their initial body weights and had lower Body Mass Index (BMI) values. Among the 90 women, 56 had a normal (without mutation) UCP3 genotype, while 34 women had mutations in the promoter region of UCP3. Despite similar weight reductions in both groups, a significantly higher decrease in abdominal adipose tissue was observed in the normal UCP3 genotype group, compared to the group with mutations. In particular, there was a significant reduction of fat at the lumbar 1 (Ll) level in the without-mutation group. Serum levels of total cholesterol, apolipoprotein Al were significantly decreased in the without-mutation group, by 4.4% and 5.7% respectively. Serum levels of hormones were not significantly changed in both groups artier the intervention. However, in the group without the mutations, the leptin level significantly reduced by 23.4% (p<0.001). Serum free fatty acid (FFA) concentration was significantly increased in the group with mutation following the weight reduction program. On the other hand, FFA responses were shown similar increases in both groups. In conclusion, although no difference was found in the magnitude of weight reduction in both groups, there were significant differences in body fat distribution and in endocrine function between the groups.
Lee, Junguee;Yi, Shinae;Chang, Joon Young;Kim, Jung Tae;Sul, Hae Joung;Park, Ki Cheol;Zhu, Xuguang;Cheng, Sheue-yann;Kero, Jukka;Kim, Joon;Shong, Minho
Molecules and Cells
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v.42
no.2
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pp.113-122
/
2019
Communications at the interface between the apical membrane of follicular cells and the follicular lumen are critical for the homeostasis of thyroid gland. Primary cilia at the apical membrane of thyroid follicular cells may sense follicular luminal environment and regulate follicular homeostasis, although their role in vivo remains to be determined. Here, mice devoid of primary cilia were generated by thyroid follicular epithelial cell-specific deletion of the gene encoding intraflagellar transport protein 88 (Ift88). Thyroid follicular cellspecific Ift88-deficient mice showed normal folliculogenesis and hormonogenesis; however, those older than 7 weeks showed irregularly dilated and destroyed follicles in the thyroid gland. With increasing age, follicular cells with malignant properties showing the characteristic nuclear features of human thyroid carcinomas formed papillary and solid proliferative nodules from degenerated thyroid follicles. Furthermore, malignant tumor cells manifested as tumor emboli in thyroid vessels. These findings suggest that loss-of-function of Ift88/primary cilia results in malignant transformation from degenerated thyroid follicles.
Kim, Junhyung;Yoon, Seon Jin;Moon, Ju Hyung;Ku, Cheol Ryong;Kim, Se Hoon;Lee, Eun Jig;Kim, Sun Ho;Kim, Eui Hyun
Journal of Korean Neurosurgical Society
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v.62
no.1
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pp.114-122
/
2019
Objective : Silent corticotroph adenomas (SCA) are endocrine-inactive pituitary adenomas with positive immunohistochemistry staining for adrenocorticotropic hormone (ACTH). We investigated whether SCA-associated clinical profiles were more aggressive than hormonally negative adenomas (HNA). Methods : Among 627 patients with pathologically proven endocrine-inactive pituitary adenomas between 2004 and 2013, positive immunohistochemistry revealed 55 SCAs and 411 HNAs. Surgical outcomes and radiological and endocrinological characteristics were compared. Results : Strong female predominance was observed in the SCA group (p<0.001). Cavernous sinus invasion was identified in 22 (40%) SCA patients and 72 (17.6%) HNA patients (p<0.001). There were no differences in ACTH or cortisol levels between the two groups. The incidence of preoperative hypopituitarism and postoperative hormonal outcome did not differ between two groups. Total resection was achieved in 35 patients (63.7%) with SCA and 332 patients (80.8%) with HNA (p=0.007). When tumors were completely removed, recurrence rates were not statistically different between two groups (p=0.60). When complete resection was not achieved, tumors regrew from these remnants in seven patients (35.0%) with SCA and 12 patients (15.2%) with HNA (p=0.05). Conclusion : Total surgical resection for SCA is often challenging as these tumors frequently invade a cavernous sinus. Early remnant tumor intervention is justified, because untreated residual pituitary tumors regrow when patients were followed up for a long time. Prophylactic radiotherapy is not warranted for completely resected SCAs as tumor recurrence is uncommon.
Endocrine-disrupting chemicals found in many commercial products may interfere with the normal functioning of the endocrine system and are unsafe because of their cumulative effect on the human body. However, little is known about the effects of combinations of endocrine-disrupting chemicals in humans. Methoxychlor and bisphenol A are toxic to male reproductive organs. Therefore, we studied the effects of methoxychlor and bisphenol A on male reproductive function. Male mice were divided into four treatment groups: control, 400 mg methoxychlor, 1 mg bisphenol A, and 400 mg methoxychlor + 1 mg bisphenol A/kg/day. Methoxychlor and bisphenol A were dissolved in sesame oil and acetone and administered orally for 4 weeks. After administration, the weight and histological changes in the testicles and epididymis, sperm count and health were observed biochemical tests and whole blood counts were performed. The results showed that the mice in the bisphenol A and methoxychlor + bisphenol A groups gained more weight than those in the control and methoxychlor group. The weights of the testes and epididymis were higher in the experimental groups than in the control. Sperm motility and progression were significantly reduced in the bisphenol A and methoxychlor + bisphenol A groups. Histological observation showed a reduced number of sperm, smaller seminiferous tubules, and destroyed lumen in the methoxychlor + bisphenol A group compared to the other groups. In conclusion, our study showed that methoxychlor and bisphenol A destroy male reproductive tissues and decrease sperm quality.
Purpose: This study attempted to determine the factors related to behaviors of reducing exposure to endocrine disrupting chemicals (BRE to EDCs) in pregnancy, based on the PRECEDE model. Methods: A cross-sectional survey was conducted with participants (N=239) who met the medical records and eligibility criteria from the Women's Hospital and Public Health Center. Data were collected using a specially-designed questionnaire based on the PRECEDE model and included BRE to EDCs predisposing factors, reinforcing factors, and enabling factors. The data were analyzed using the chi-square test, independent t-test, one way ANOVA, Pearson's correlation coefficients and a hierarchical multiple regression analysis. Results: In Model I it was found that participation in prenatal education influenced the BRE to EDCs significantly; and a regression showed that the determinant variables accounted for 3.4%. In Model II, the predisposing factors of perceived barriers of BRE to EDCs and environmental self-efficacy were added. It was shown that they significantly influenced BRE to EDCs in the order named, and a regression revealed that increases in the determinant variables accounted for 22.5%. In Model III, to which enabling factors were added, the information acquisition experience of BRE to EDCs interacted significantly with BRE to EDCs and a regression showed an increase in the determinant variables accounting for 25.3%. Conclusion: The results of this study, the content of endocrine disruptors must be included in the prenatal care education program for pregnant women, and it should be composed of contents that can reduce the perceived obstacle to BRE to EDCs, enhance the environmental self-efficacy, and provide the information in regard to reducing exposure to EDCs.
Park, Yun-Yong;Park, Ki Cheol;Shong, Minho;Lee, Soon-Jung;Lee, Young-Ho;Choi, Hueng-Sik
Molecules and Cells
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v.24
no.3
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pp.372-377
/
2007
The orphan nuclear receptor, SF-1, plays a pivotal role in the development and differentiation of the endocrine and reproductive systems, and also regulates the transcription of a host of genes, including those encoding several steroidogenic enzymes and gonadotropins. We found that a previously unidentified repressor, EID-1, is an SF-1-interacting protein that inhibits the transactivation of SF-1. A transient transfection assay revealed that EID-1 inhibits SF-1, but not LRH-1, $ERR{\gamma}$, or mCAR. Using the yeast two hybrid and GST pull-down assays, we determined that EID-1 interacted strongly with SF-1. In addition, it colocalized with SF-1 in mammalian cells and interacted specifically with the AF-2 domain of SF-1, competing with SRC-1 to inhibit SF-1 transactivation. EID-1 is expressed in the mouse testis, and its expression decreases during testis development. The results of the present study suggest that EID-1 can act as a repressor, regulating the function of SF-1.
Recent studies reveal one of the representative endocrine disrupters of nonylphenol affects on the composition of a planktonic community. Since nonylphenol is sometimes discharged into eutrophic waters, we monitored planktonic community composition of a eutrophic pond after receiving nonylphenol when cyanobacterium Microcystis aeruginosa mainly dominated. The experiment was carried out two times using small-scale microcosms in a laboratory. In both two experiments, ciliate abundances significantly decreased when nonylphenol was added. On the seventh day, the ciliate abundances in $10{\mu}g\;L^{-1}$ added treatments decreased by 36.9% in the first experiment and 33.6% in the second, when compared to the control. The response of other planktonic groups was less obvious to nonylphenol addition. In particular, in the first experiment, Chl. b/Chl. $\alpha$ and Chl. c/Chl. $\alpha$ significantly increased with the addition of nonylphenol, while total Chl. $\alpha$ concentration did not change. Indeed, bacillariophyceae and chlorophyceae abundances tended to increase with nonylphenol dosing. From these results, we tentatively hypothesized that nonylphenolloading positively affects on abundances of edible phytoplankton such as Scenedesmus spp. and diatoms by releasing from grazing pressure due to decrease in ciliate abundances. The present study emphasizes that the indirect effect of endocrine disrupters should be paid more attention when freshwater resources are polluted by them.
A benchmark dose (BMD) approach has been evaluated us a replacement for the traditional NOAEL methodology currently being wed to assess the noncancer effects of toxicants. The endocrine disrupt-ing effect of endosulfan which showed decrement of sperm count and testicular testosterone level in animals, was currently reported. The amount of endosulfan used as pesticide in the country has been continuously increased. The aim of this study was to suggest the permissible intake level (PIL), corresponding to Accept-able Daily Intake (ADI), based on endocrine disrupting effect wing BMD. Various animal data were collected by consideration of critical effect showing endocrine disruption and an animal data for reproductive toxicity was selected. The Power model from BMD software for induction of $BMD_10$ having meaning which is the dose at the 95% lower confidence limit on a 10% response was used due to that the form of selected dose-response animal data was continuous data. The $BMD_10$ was estimated to be 0.393 mg/kg/day based on reproductive toxicity showing decrement of sperm count. The permissible intake level (PIL) was calculated by dividing the $BMD_10$ by the uncertainty factors of 100 with consideration of from animal to human and human variability. The PIL as 0.004 mg/kg/day was compared with traditional ADI as 0.006 mg/kg/day based on the incidence of marked progressive glomerulonephrosis and blood vessel aneurysm in males.
Recently public concerns about the endocrine disrupting chemicals (EDC) have resulted in the increased legistrative and regulatory attentions in many countries. Endocrine disrupter is an exogeneous chemical interfering the synthesis, transport binding, action or elimination of natural hormones in the body, which are responsible for the maintenance of homeostasis, reproduction, development and/or behavior. Reported possible harmful effects on human and animal lives, possible developmental anomalities, reproductive malfunctions and behaviors, and the ways of EDC accumulation in animal kingdom are reviewed. The current scientific papers and knowledges on the global contamination of EDCs demonstrates the conclusive data that EDCs have the potentials to cause the calamitous extermination of human and animal species by the global contamination.
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