• Journal of Korean Neurosurgical Society
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• 제49권5호
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• pp.262-266
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• 2011

Objective : The endocrine dysfunction after the operation for suprasellar arachnoid cysts is not rare. The careful operation to prevent structures can prevent this complication, but it is not enough and effective to prevent it. Authors present technical surgical considerations to prevent this complication with a review of our suprasellar arachnoid cyst patients who had postoperative endocrine dysfunction. Methods : From January 2002 to December 2009, eight patients who had suprasellar arachnoid cysts with visual impairment underwent surgery. The mean age was 57.1 years (range, 33-77). Preoperatively, their endocrine function was clinically normal, and laboratory hormonal levels were within normal ranges. Cyst fenestration was performed by craniotomy (n=6) or by a neuro-endoscopic procedure (n=2), and, simultaneously, along with a cyst wall biopsy. Results : The surgery was uneventful in all eight patients, and there were no neurological morbidities. However, in four patients, endocrine dysfunction occurred postoperatively. We compared these four patients (group A) to the other 4 patients without endocrine dysfunction (group B) with intraoperative findings and with the histopathological findings of the cyst wall biopsy. The group A patients had more abundant vasculature on the cystic wall than the group B patients according to both the intraoperative findings and the histopathological findings. Conclusion : When performing a surgical cyst wall fenestration, surgeons should try to minimize the destruction of the cystic wall vasculature and not to make the fenestration at a site that contains many vascular striae.

• 대한유전성대사질환학회지
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• 제22권2호
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• pp.46-52
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• 2022

Inborn errors of metabolism (IEM) are very rare and genetically transmitted diseases and have man y different symptoms related with multisystemic involvement. More rarely, endocrinopathies can be an early and first symptom of IEM, but presents with signs of later complications in adolescent or adulthood. The mechanisms of endocrine dysfunction in IEM are poorly understood. Hypogonadotropic hypogonadism is common in hemochromatosis, adrenoleukodystrophy, galactosemia, and glycogen storage disease. Many girls with classic galactosemia are at high risk for premature ovarian insufficiency (POI), despite an early diagnosis and good control. Mitochondrial diseases are multisystem disorders and are characterized by hypo- and hypergonadotrophic hypogonadism, thyroid dysfunction and insulin dysregulation. Glycogen storage disorders (GSDs), especially type Ia, Ib, III, V are assocciated with frequent hypoglycemic events. IEM is a growing field and is not yet well recognized despite its consequences for growth, bone metabolism and fertility. For this reason, clinicians should be aware of these diagnoses and potential endocrine dysfunction.

• The Korean Journal of Physiology and Pharmacology
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• 제18권2호
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• pp.177-182
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• 2014

This study was to determine the correlation between endothelial function and neuro-endocrine-immune (NEI) network through observing the changes of NEI network under the different endothelial dysfunction models. Three endothelial dysfunction models were established in male Wistar rats after exposure to homocysteine (Hcy), high fat diet (HFD) and Hcy+HFD. The results showed that there was endothelial dysfunction in all three models with varying degrees. However, the expression of NEI network was totally different. Interestingly, treatment with simvastatin was able to improve vascular endothelial function and restored the imbalance of the NEI network, observed in the Hcy+HFD group. The results indicated that NEI network may have a strong association with endothelial function, and this relationship can be used to distinguish different risk factors and evaluate drug effects.

• 한국환경독성학회:학술대회논문집
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• 한국환경독성학회 2003년도 춘계학술대회
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• pp.190-191
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• 2003

Endometriosis is classically defined as the growth of endometrial glands and stroma at extrauterine sites. Although it is a common gynecological problem accompanied by chronic pelvic pain, infertility, and adhesion formation, the etiology of this disease is unknown. Endometriosis pathogenesis may involve endocrine and immune dysfunction since uterine endometrial growth is regulated by sex hormones in concert with bioactive mediators produced by uterine immune and endocrine cells. Thus, exposure to environmental toxicants disrupting endocrine and immune responses potentially affect the development and progression of endometriosis.

• Clinical and Experimental Pediatrics
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• 제53권3호
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• pp.420-427
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• 2010

목 적 : 소아와 청소년기에 골수이식을 받은 환자들은 여러 가지 조기 또는 후기 합병증이 발생할 수 있다. 본 연구에서는 이식 후 발생할 수 있는 내분비 기능 부전에 대해 분석하여 이식 후 추적 관리에 도움이 되고자 하였다. 방 법 : 골수이식을 받은 100명(남자 61명, 여자 39명)의 환자들을 대상으로 진단명, 이식 당시 연령, 전처치 방법, 만성 이식편대 숙주병 유무, 성장 패턴, 갑상샘 기능, 사춘기 발달 상태 등을 후향적으로 조사하여 내분비 기능 부전과 관련이 있는 위험 인자가 있는지 분석하였다. 결 과 : 이식 당시, 이식 1년후, 최종 내원시의 신장 표준편차 점수는 각각 $0.08{\pm}1.04$, $-0.09{\pm}1.02$, $-0.27{\pm}1.18$로 의미있게 감소하였다(P =0.001). 전처치로 TBI를 받은 경우 TBI를 받지 않은 군에 비하여 이식 전보다 신장 표준편차 점수가 더 많이 감소하였다(P =0.017). TBI를 시행한 환자 중 1명에서 성장 호르몬 결핍을 보였다. 갑상샘 기능 검사를 시행한 94명 중 30명(31.9%)이 보상성 갑상샘저하증이었고 만성 이식편대 숙주병이 있었던 환자에서 보상성 갑상샘저하증의 빈도가 높았다(odds ratio=2.82, P =0.025). 최종 내원 시 만 14세 이상 남자 17명, 만 13세 이상 여자 15명 중에서 비정상적인 LH 또는 FSH의 상승을 보인 경우는 남자 3명, 여자 13명으로 여아가 의미 있게 많았다(odds ratio=30.3, P =0.001). 결 론 : 소아나 청소년기에 골수이식을 받은 환자에서 가장 흔한 내분비 기능 이상은 난소 기능 부전이며 그 외 높은 빈도의 내분비 기능 이상을 보이므로 정기적인 내분비 기능 검사가 필요하다.

• 한국환경보건학회지
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• 제41권3호
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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.

• 대한임상검사과학회지
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• 제49권4호
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• pp.495-497
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• 2017

Thalassemia major is a genetic disorder with a defective synthesis of either the alpha or the beta chain of hemoglobin A. Blood transfusion is crucial for the survival in these patients. Unfortunately, endocrine dysfunction is a very common complication in these patients and is principally due to excessive iron overload as a result of frequent blood transfusions. Although regular blood transfusion may increase life expectancy, disturbances in growth and pubertal development, abnormal gonadal functions, impaired thyroid, parathyroid and adrenal functions, diabetes, and disorderly bone growth are common side effects. We hereby present a case of a 23-year-old, unmarried woman with beta thalassemia major presenting with primary amenorrhea, poor development of secondary sexual character, and short stature. Thorough history, clinical examination, and laboratory investigation, including dynamic function test (insulin tolerance test) were conducted. These tests confirmed that she had multiple endocrinopathies, including hypogonadotropic hypogonadism, growth hormone deficiency, and subclinical adrenal insufficiency, which were caused by iron overload. She required hormone replacement therapy. Early recognition of possible deficiencies in hypothalamo-pituitary-end organ hormones caused by iron overload in thalassemia patients that undergo frequent blood transfusion procedures is essential. Appropriate treatments, including transfusion regimen and chelation therapy, as well as specific treatment of each complication are the crucial for the successful management and improvement of quality of life these patients.

• 한국임상수의학회지
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• 제38권1호
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• pp.41-44
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• 2021

Pituitary pars intermedia dysfunction (PPID), often referred to as equine Cushing's disease, is a common endocrine disorder often diagnosed in older horses. A 13-year-old 460 kg Warmblood gelding showed clinical signs suggestive of PPID, including hypertrichosis, fat redistribution, polyuria and polydipsia (PU/PD), and weight loss. Physical examination, complete blood cell count, and serum chemistry results were normal. However, dexamethasone suppression and plasma adrenocorticotropic hormone (ACTH) level tests confirmed PPID. Three months after the confirmed diagnosis, the horse was referred again with symptoms of laminitis. Radiography and venography were performed to evaluate the laminitis severity level. However, the foot condition continued to worsen, and the horse was eventually euthanized. The purpose of this case report is to describe clinical signs and diagnosis of PPID with laminitis.

• Clinical and Experimental Pediatrics
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• 제55권7호
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• pp.224-231
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• 2012

Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder characterized by hypothalamic-pituitary dysfunction. The main clinical features include neonatal hypotonia, distinctive facial features, overall developmental delay, and poor growth in infancy, followed by overeating with severe obesity, short stature, and hypogonadism later in development. This paper reviews recent updates regarding the genetic aspects of this disorder. Three mechanisms (paternal deletion, maternal disomy, and deficient imprinting) are recognized. Maternal disomy can arise because of 4 possible mechanisms: trisomy rescue (TR), gamete complementation (GC), monosomy rescue (MR), and postfertilization mitotic nondisjunction (Mit). Recently, TR/GC caused by nondisjunction at maternal meiosis 1 has been identified increasingly, as a result of advanced maternal childbearing age in Korea. We verified that the d3 allele increases the responsiveness of the growth hormone (GH) receptor to endogenous GH. This paper also provides an overview of endocrine dysfunctions in children with PWS, including GH deficiency, obesity, sexual development, hypothyroidism, and adrenal insufficiency, as well as the effects of GH treatment. GH treatment coupled with a strictly controlled diet during early childhood may help to reduce obesity, improve neurodevelopment, and increase muscle mass. A more active approach to correct these hormone deficiencies would benefit patients with PWS.

• 대한유전성대사질환학회지
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• 제14권2호
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• pp.191-194
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• 2014

사립체 근병증은 사립체 호흡 사슬의 장애로 인한 것으로 내분비계 관련 증상이 흔히 동반되고 그 중 당뇨병이 상대적으로 높은 빈도를 보인다고 알려져 있다. 사립체 근병증에서의 당뇨병은 사립체 기능 장애로 인한 인슐린 분비의 결함으로 발생하고, 대개 인슐린 의존성이나 당뇨병성 케톤산증으로 발현하거나 자가 항체가 검출되는 경우는 드물다. 저자들은 사립체 근병증 환자에서 당뇨병성 케톤산증으로 발현하고 Anti-GAD antibody와 Anti-insulin auto-antibody가 모두 양성으로 확인된 인슐린 의존성 당뇨병을 진단하였기에 이를 기존의 문헌과 비교하여 보고하는 바이다.