• Clinical and Experimental Pediatrics
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• 제53권7호
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• pp.759-765
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• 2010

Purpose: This study evaluated the clinical manifestations of and risk factors for pituitary insufficiency in children and adolescents with Rathke's cleft cysts. Methods: Forty-four patients with Rathke's cleft cysts younger than 19 years who visited Seoul National University Children's Hospital between January 1995 and September 2009 were enrolled. Rathke's cleft cysts were confirmed histologically through an operation in 15 patients and by brain magnetic resonance imaging (MRI) in 29 patients. The clinical, hormonal, and imaging features were reviewed retrospectively. Results: The clinical presentation of symptomatic patients was as follows: headache (65%), endocrinopathy (61%), and visual disturbance (19%). Endocrinopathy included central precocious puberty (18%), diabetes insipidus (14%), general weakness (11%), and decreased growth velocity (7%). After surgery, hyperprolactinemia resolved in all patients, but growth hormone insufficiency, hypothyroidism, and diabetes insipidus did not improve. Pituitary insufficiency except gonadotropin abnormality correlated significantly with severe headache, visual disturbance, general weakness, and cystic size. Suprasellar extension of cysts and high signals in the T2-weighted image on brain MRI were related to hypothyroidism, hypocortisolism, and diabetes insipidus. Multivariable linear regression analysis showed that only general weakness was a risk factor for pituitary insufficiency ($R^2$=0.549). Conclusion: General weakness is a risk factor for pituitary insufficiency in patients with Rathke's cleft cysts. When a patient with a Rathke's cleft cyst complains of general weakness, the clinician should evaluate pituitary function and consider surgical treatment.

• Journal of Korean Neurosurgical Society
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• 제54권2호
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• pp.132-135
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• 2013

The small pituitary mass was incidentally found in 40-years-old women with renal cell carcinoma. The endocrinological and ophthalmological evaluation revealed no deficit and the short-term follow-up was recommended. In 6 months later, the visual disturbance was reported and the size of mass was increased. The tumor was removed totally via the trans-sphenoid approach. The post-operative endocrinological insufficiency was not noticed. During one year of follow-up period, there was no evidence of recurrence without adjuvant radiotherapy. The clinical features of pituitary metastasis from renal cell carcinoma were similar to those of pituitary adenoma. The possibility of pituitary metastasis should be kept in mind in patients with sellar mass and renal cell carcinoma.

• Clinical and Experimental Pediatrics
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• 제50권3호
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• pp.236-240
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• 2007

Majority of sick full term newborns have adequate adrenal cortical function in response to stress. Acutely ill neonates with a basal cortisol level less than $15{\mu}g/dL$ (414 nmol/L) suggest adrenal insufficiency and require function testing of adrenal function. In premature infant, immaturity of hypothalamic-pituitary adrenal axis (HPA axis), may limit the ability to increase cortisol production in response to stress. The response to low dose ACTH and CRH appears to be useful as an additional test of adrenal function. CRH stimulation has been used increasingly in neonates. The ACTH and CRH stimulated cortisol response of more than $17{\mu}g/dL$ (469 nmol/L) indicates a normal response.

• 대한한방내과학회지
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• 제38권5호
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• pp.583-591
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• 2017

Adrenal insufficiency is caused by adrenal failure or impairment of the hypothalamic-pituitary-adrenal axis. The main symptoms of adrenal insufficiency are chronic fatigue, nausea, vomiting, anorexia, and weight loss. We report a case of adrenal insufficiency in a 38-year-old female. The patient complained of headache, dizziness, anorexia, and general weakness. We treated her with Iggisaengjin-tang and acupuncture. After treatment, the patient's symptoms were improved and serum cortisol levels rose to a normal range without the aid of steroid supplementation therapy. This case suggests that Korean medicine can be effective in the treatment of adrenal insufficiency, but more clinical reports are needed.

• Tuberculosis and Respiratory Diseases
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• 제67권1호
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• pp.47-51
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• 2009

식욕 저하는 진행성 암환자에게 삶의 질을 저하시키는 중요한 요인으로서 이에 대한 보조 치료제로 megestrol acetate는 임상적인 호전을 유도할 수 있다. 이 약제는 부신기능저하의 부작용이 발생할 수 있으며, 암환자에게서의 부신기능저하와 관련된 증상은 기저 질환과 암 치료로 인한 부작용 등으로 간과될 수 있다. Megestrol acetate를 복용하거나 중단한 환자에서 부신기능저하와 관련된 증상과 검사소견이 있을 때 부신기능저하는 생명에 위협을 줄 수 있기 때문에 이에 대하여 항상 인지를 하고 적절한 검사와 치료가 필요함을 염두에 둬야 한다. 저자들은 폐암 환자에서 megestrol acetate 복용 후 발생한 이차성 부신기능저하증 1예를 경험하여 문헌 고찰과 함께 보고하는 바이다.

• Journal of Korean Neurosurgical Society
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• 제64권4호
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• pp.608-618
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• 2021

Objective : The endoscopic endonasal transsphenoidal approach is a widely-used method for the surgical treatment of pituitary adenomas. We aimed to evaluate the results of endoscopic surgery by comparing preoperative classification methods and investigating their relationship with postoperative resection and remission rates and complications. Methods : We retrospectively reviewed the medical records of 236 patients (118 males) who underwent surgery for pituitary adenomas. Preoperative Knosp classification, tumor size (TS), suprasellar extension (SSE), postoperative resection and remission rates, and complications were evaluated. Results : The follow-up period was 3 months to 6 years. The patients' ages ranged between 16 and 84 years. Endocrinologically, 114 patients (48.3%) had functional adenoma (FA), and 122 patients (51.7%) had non-functional adenoma (NFA). Among the FA group, 92 (80.7%) showed remission. A statistically significant difference was found between patients with and without remission in terms of the Knosp, TS, and SSE classifications (p<0.01). Knosp, TS, and SSE classification grades were found to be correlated with the resection rates (p<0.01). Meningitis was seen in seven patients (3.0%), diabetes insipidus in 16 (6.9%; permanently in two [0.9%]), and rhinorrhea in 19 (8.1%). Thirty-six patients (15.3%) developed pituitary insufficiency and received hormone replacement therapy. Conclusion : The resection categories and remission rates of FAs were directly proportional to the adenoma sizes and Knosp grades, while the degree of suprasellar growth further complicated resection and remission rates. Adenoma sizes less than 2 cm and SSEs less than 1 cm are associated with favorable remission and resection rates.

• Journal of Genetic Medicine
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• 제14권1호
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• pp.27-30
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• 2017

Adrenal hypoplasia congenita (AHC) is a rare cause of adrenal insufficiency during neonatal period. Mutations in the gene coding for DAX1 cause X-linked adrenal hypoplasia. Most affected patients are shown to have salt wasting and hyperpigmentation on the skin during the neonatal period and require intensive medical care. In addition, it is usually associated with hypogonadotropic hypogonadism in adolescence. The DAX1 gene is expressed in the adrenal cortex, pituitary gland, hypothalamus, testis, and ovary. We report on a patient with genetically confirmed AHC whose initial clinical presentations were consistent with congenital adrenal hyperplasia. A point mutation in the DAX1 gene identified in this report resulted in a truncated DAX1 protein. Our patient was diagnosed with AHC.

• Journal of Korean Neurosurgical Society
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• 제66권6호
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• pp.618-631
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• 2023

The brain houses vital hormonal regulatory structures such as the hypothalamus and pituitary gland, which may confer unique susceptibilities to critical illness-related corticosteroid insufficiency (CIRCI) in patients with neurological disorders. In addition, the frequent use of steroids for therapeutic purposes in various neurological conditions may lead to the development of steroid insufficiency. This abstract aims to highlight the significance of understanding these relationships in the context of patient care and management for physicians. Neurological disorders may predispose patients to CIRCI due to the role of the brain in hormonal regulation. Early recognition of CIRCI in the context of neurological diseases is essential to ensure prompt and appropriate intervention. Moreover, the frequent use of steroids for treating neurological conditions can contribute to the development of steroid insufficiency, further complicating the clinical picture. Physicians must be aware of these unique interactions and be prepared to evaluate and manage patients with CIRCI and steroid insufficiency in the context of neurological disorders. This includes timely diagnosis, appropriate steroid administration, and careful monitoring for potential adverse effects. A comprehensive understanding of the interplay between neurological disease, CIRCI, and steroid insufficiency is critical for optimizing patient care and outcomes in this complex patient population.

• 정신신체의학
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• 제19권2호
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• pp.109-114
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• 2011

연구목적: 시상하부-뇌하수체-부신 축의 이상은 다양한 정신과적 증상과 연관이 있으며, 우울 증상도 나타날 수 있다. 저자들은 뇌하수체 기능저하 및 이차성 부신기능저하와 연관된 두통, 무의욕, 무기력, 정신운동 저하, 식욕 저하, 불면 및 걱정 등의 우울 증상을 주소로 정신과에 입원한 71세 남자 환자에 대해 증례 보고하고자 한다. 환자는 정신과 입원 후 두통, 불면, 불안 및 소화기 증상은 호전되었으나, 무기력감은 지속되었다. 퇴원 후 고열 및 의식 혼탁으로 감염내과에 재입원을 하였으며, 부신기능 저하소견을 동반한 범뇌하수체기능저하증이 진단되었고, 코티졸 투여로 전반적인 증상이 호전되었다. 뇌하수체 기능저하에 따른 갑상선 기능저하, 부신기능저하, 성장호르몬 저하 등은 무기력, 피곤, 불면, 체중 감소, 식욕 저하 등의 여러 가지 비특이적 증상들을 나타내고, 임상에서 이러한 비특이적 우울 증상을 가진 환자의 경우, 내분비 질환에 대한 감별이 필요하다.

• Clinical and Experimental Pediatrics
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• 제57권10호
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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.