• Journal of mucopolysaccharidosis and rare diseases
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• v.1 no.2
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• pp.35-39
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• 2015

Sleep problems occur frequently among patients with Prader-Willi syndrome (PWS). The most common problem is excessive daytime sleepiness (EDS) that are closely related to of sleep-related breathing disorder (SRBD) such as obstructive sleep apnea (OSA) and congenital hypoventilation syndrome. Obesity, craniofacial dysmorphism and muscular hypotonia of patients with PWS may increase the risk of SRBD. Sleep apneas can interrupt the continuity of sleep, and these disruptions result in a decrease in both the quality and quantity of sleep. In addition to SRBD, other sleep disorders have been reported, such as hypersomnia, a primary abnormality of the rapid eye movement (REM) sleep and narcolepsy traits at sleep onset REM sleep. Patients with PWS have intrinsic abnormalities of sleep-wake cycles due to hypothalamic dysfunction. The treatment of EDS and other sleep disorders in PWS are similar to standard treatments. Correction of sleep hygiene such as sufficient amount of sleep, maintenance of regular sleep-wake rhythm, and planned naps are important. After comprehensive evaluation of sleep disturbances, CPAP or surgery should be recommended for treatment of SRBD. Remaining EDS or narcolepsy-like syndrome are controlled by stimulant medication. Bright light therapy might be beneficial for disturbed circadian sleep-wake rhythm caused by hypothalamic dysfunction.

• Journal of Korean Neurosurgical Society
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• v.58 no.5
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• pp.432-441
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• 2015

Objective : To assess the impact of the complete resection of craniopharyngioma (CP) in adults on oncologic and functional outcomes. Methods : We retrospectively analyzed 82 patients with CP who were surgically treated by the same neurosurgeon at our institution between January 1994 and December 2012. Results : Gross total resection (GTR) was achieved in 71 patients (86.6%), near total resection (NTR) in 7 patients (8.5%), and subtotal resection (STR) in 3 patients (3.7%). The disease-specific overall survival rate was 100% with the exclusion of 2 surgery-related mortalities. The overall recurrence rate was 12.2% (10 of 82 patients), however the recurrence rate according to extent of resection (EOR) was 9.9% (7 of 71 patients) after GTR, 14.3% (1 of 7 patients) after NTR, and 66.7% (2 of 3 patients) after STR. The overall recurrence-free survival (RFS) rates at 5 and 10 years were 87.0% and 76.8%, respectively. Postoperatively, most patients (86.3%) needed hormone replacement for at least 1 hypothalamic-pituitary axis. Vision improved in 56.4% of the patients with preoperative abnormal vision, but deteriorated in 27.4% of patients. Hypothalamic dysfunction developed in 32.9% of patients. There were no significant differences in the risks of pituitary dysfunction, visual deterioration, or hypothalamic dysfunction between the groups with complete vs. incomplete removal. The overall rate of postoperative complications was 22.0%, which did not differ between groups (p=0.053). Conclusion : The complete removal of a CP at first surgery can provide a chance for a cure with acceptable morbidity and mortality risks.

• Journal of Ginseng Research
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• v.41 no.4
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• pp.589-594
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• 2017

Ginseng effectively regulates the immune response and the hormonal changes due to stress, thus maintaining homeostasis. In addition to suppressing the occurrence of psychological diseases such as anxiety and depression, ginseng also prevents stress-associated physiological diseases. Recent findings have revealed that ginseng is involved in adjusting the hypothalamic-pituitary-adrenal axis and controlling hormones, thus producing beneficial effects on the heart and brain, and in cases of bone diseases, as well as alleviating erectile dysfunction. Recent studies have highlighted the potential use of ginseng in the prevention and treatment of chronic inflammatory diseases such as diabetes, rheumatoid arthritis, and allergic asthma. However, the mechanism underlying the effects of ginseng on these stress-related diseases has not been completely established. In this review, we focus on the disease pathways caused by stress in order to determine how ginseng acts to improve health. Central to our discussion is how this effective and stable therapeutic agent alleviates the anxiety and depression caused by stress and ameliorates inflammatory diseases.

• Clinical and Experimental Reproductive Medicine
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• v.25 no.3
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• pp.293-297
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• 1998

Hypogonadotropic hypogonadism, or the lack of function of the testis or ovary secondary to the lack of pituitary and or hypothalamic trophic hormones, is also sometimes generally termed Kallmann's syndrome. Whether such deficiencies arise from an inborn error of hypothalamic organization and pituitary connection or damage to the hypothalamic pituitary system in prepubertal life, the manifestations of a eunuchoid or apubertal individual with potentially competent pituitary and gonadal function will result. Beyond the achievement of puberty, a similar situation can be recreated by the administration of a long-acting GnRH analog or by conditions of secondary hypothalamic dysfunction such as anorexia nervosa where shutdown of GnRH and its resultant effects cause cessation of gonadal function and even a regression of secondary sexual characteristics. Technically, these conditions are not Kallmann's syndrome but one must recognize the similarities. We have experienced a case of isolated gonadotropin deficiency which showed a negative KALIG-1 gene in infertile patient with primary amenorrhea. So we report this case with a brief review of literatures.

• Journal of Environmental Health Sciences
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• v.41 no.3
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• pp.147-162
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• 2015

Objectives: The objective of this review was to summarize the primary role of three representative endocrine axes in aquatic vertebrates and discuss the effects on endocrine systems and their interactions in teleost fish after exposure to environmental contaminants. Methods: We summarized individual traits and mechanisms for hormonal and transcriptional interactions between the hypothalamic-pituitary-gonad (HPG), hypothalamic-pituitary-thyroid (HPT), and hypothalamic-pituitary-adrenal (HPA) axes in fish. We also provided a brief discussion on the effects of nonylphenol-induced toxicity on endocrine systems and their interactions in fish as a demonstration of holistic explanation. Results: Currently-available data showed that thyroid dysfunction is associated with reproductive toxicity due to changes in steroidogenic gene expressions and sex hormone levels as well as gonad glands in fish. As an example, we demonstrated that exposure to nonylphenol could induce estrogenicity in male fish by decreasing thyroid hormones, which contributes to increased aromatase expression. Although the mechanisms are complicated and involved in multiple ways, a number of studies have shown that sex steroids influence the HPT axis or the HPA axis in fish, indicating bi-directional crosstalk. Critically missing is information on the primary target or toxicity mechanisms of environmental contaminants among the three endocrine axes, so further studies are needed to explore those possibilities. Conclusions: This review highlights the interactions between the HPG, HPT, and HPA axes in fish in order to better understand how these endocrine systems could interact with each other in situations of exposure to endocrine disrupting chemicals.

• Journal of Korean Neurosurgical Society
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• v.30 no.9
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• pp.1115-1119
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• 2001

Objective : Management strategies for pediatric chiasmatic-hypothalamic gliomas(CHG) include surgery, irradiation, chemotherapy and a combination of these modalities. This study was performed in order to compare the efficacy of various methods of treatment and to describe its optimal management. Material and Method : We have reviewed the results of management of 6 children with a diagnosis of CHG, who were observed closely during the last 8 years. The patients were aged 7 months to 15 years. Our patients presented with diencephalic syndrome, endocrine dysfunction, and progressive visual loss. None of these had evidence of neurofibromatosis-1. Treatment consisted of surgery alone(2), surgry and irradiation(2), surgery, irradiation and chemotherapy(1), and surgery and chemotherapy(1). Results : Four children had large exophytic suprasellar tumors and two showed diffuse midline lesions. Obstructive hydrocephalus was present in all patients. Pathologic examination revealed anaplastic astrocytoma in 1 and lowgrade astrocytoma in 5. Two patients, recently treated with radiation therapy following radical subtotal resection, showed significant tumor reductions and good clinical status. Four patients had partial tumor resection. Of these patients, two developed disease stabilization during follow-up period of 7 and 8 years, respectively. Life-threatening complications were noted in remaining two patients. Conclusion : CHG may follow an unpredicatable course and show a various reponse to each treatment modality. Further studies are indicated to define the optimal method of treatment of CHG in childhood.

• Animal cells and systems
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• v.15 no.3
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• pp.203-210
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• 2011

In the present study, we examined the effects of caffeine on food intake and body weight, and pro-opiomelanocortin (POMC) and agouti-related protein (AgRP) expression in the hypothalamus. Rats were administered intraperitoneally with 100 mg/kg caffeine (a high, non-toxic dose) or saline during the light phase. Intraperitoneal administration of caffeine induced a significant reduction in food intake and body weight 12 hr after treatment. In addition, POMC expression was significantly increased and AgRP expression was decreased in the arcuate nucleus (Arc) after caffeine treatment. These results demonstrate that administration of caffeine up-regulates POMC expression and down-regulates AgRP expression in the Arc, suggesting that the activation of the hypothalamic POMC neurons and inhibition of the AgRP neurons might play a role in the regulation of food intake and body weight by caffeine.

• Clinical and Experimental Pediatrics
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• v.50 no.8
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• pp.794-798
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• 2007

Hepatopulmonary syndrome is a triad that includes: hepatic dysfunction, intrapulmonary vascular dilatation and abnormal arterial oxygenation. The incidence of intrapulmonary vascular dilatations, in adults with end-stage liver disease, has been reported to be 13% to 47%, however the incidence in children is unclear and the cases in Korean children have never been reported. The hepatopulmonary syndrome may occur as a result of chronic liver disease following nonalcoholic steatohepatitis in children with hypothalamic or pituitary dysfunction. We report a case of hepatopulmonary syndrome in a 13-year-old child who had rapidly progressive liver dysfunction secondary to panhypopituitarism after craniopharyngioma resection. Careful monitoring and treatment of endocrine abnormalities and metabolic status, as well as liver function, are required in all children undergoing pituitary tumor resection.

• Clinical and Experimental Pediatrics
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• v.57 no.10
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• pp.425-433
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• 2014

Adrenal and thyroid hormones are essential for the regulation of intrauterine homeostasis, and for the timely differentiation and maturation of fetal organs. These hormones play complex roles during fetal life, and are believed to underlie the cellular communication that coordinates maternal-fetal interactions. They serve to modulate the functional adaptation for extrauterine life during the perinatal period. The pathophysiology of systemic vasopressor-resistant hypotension is associated with low levels of circulating cortisol, a result of immaturity of hypothalamic-pituitary-adrenal axis in preterm infants under stress. Over the past few decades, studies in preterm infants have shown abnormal clinical findings that suggest adrenal or thyroid dysfunction, yet the criteria used to diagnose adrenal insufficiency in preterm infants continue to be arbitrary. In addition, although hypothyroidism is frequently observed in extremely low gestational age infants, the benefits of thyroid hormone replacement therapy remain controversial. Screening methods for congenital hypothyroidism or congenital adrenal hyperplasia in the preterm neonate are inconclusive. Thus, further understanding of fetal and perinatal adrenal and thyroid function will provide an insight into the management of adrenal and thyroid function in the preterm infant.

• Annals of Clinical Neurophysiology
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• v.4 no.2
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• pp.140-145
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• 2002

Neurologic sequelae of tuberculous meningitis include hemiparesis, paraparesis, quadriparesis, aphasia, developmental delay, dementia, blindness, visual field defect, deafness, cranial nerve palsies, epilepsy, and hypothalamic and pituitary dysfunction. But cervical epidural abscess and cervical spondylitis are rare. A 64-year-old woman who was diagnosed as tuberculous meningitis presented a severe neck pain and stiffness after 3 weeks of anti-tuberculous medication. Electromyography and cervical X-ray showed a cervical spondylosis with polyradiculopathy. But cervical MRI showed an acute cervical epidural abscess and mild cervical spondylitis. After continuous anti-tuberculous medication with supportive care, she showed a slow clinical improvement. But about 1 month of anti-tuberculous therapy, she presented a more aggravation of neck pain, neck stiffness, radicular pain, and neck motion limitation. Follow-up cervical MRI showed an more advanced cervical spondylitis. Afterthen she has recovered slowly by cervical laminectomy with posterior stabilization and continuous anti-tuberculous medication.