• 한국동물번식학회:학술대회논문집
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• 한국동물번식학회 2001년도 춘계학술발표대회
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• pp.47-47
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• 2001

The neuropeptide vasopressin (VP) is a nine- amino acid hormone synthesized as preprohormone in the cell bodies of hypothalamic magnocellular neurons. The tumor in magnocellular neurons of the hypothalamus is associated with disfunctions of the cell bodies, leading to the diabetes insipidus. In order to study with the diabetes insipidus caused by a defect in VP synthesis and its secretion, we have produced the transgenic mice regulated by vasopressin promoter inserted to SV40 T antigen coding sequence (pVPSV.IGR2.1). One transgenic line expressing high levels of SV40 T antigen was propagated. The founder and all transgene positive adult animals have appeared with shorten mortality or apparent phenotypic abnormalities, including immune complex disease, and eventually die between 4 and 8 months of age. The mRNA and protein of SV40T antigen transgene were detected in brain of fetus as well as in brain, spleen, lung and lymph node in moribund at the age of 20 weeks. Histological analysis of transgenic mice showed that tumor developed in brain similar to primitive neuroectodermal tumors (PNET) in man. We also detected lymphomas in spleen and lymph node, and consequent tumor formation in various tissues of the transgenic mice. In pVPSV.IGR2.1, 21% mice showed brain tumor (PNET) at 5 weeks and 100% mice showed brain tumor after 15 weeks. In addition, Expression of apoptosis related genes (Bcl-28 & Bax) was increased over their age in mice with PNET as compared to control mice. Apoptosis related gene expression might be deregulated in mice with brain tumor. However, transgenic mice were not developed with the diabetes insipidus. These mice represent the first disease model to exhibit primitive neuroectodermal tumor in brain, as well as a unique model system for exploring the cellular pathogenesis of lymphomas.

• 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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• 제29권6호
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• pp.822-825
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• 2000

Lymphocytic infundibuloneurohypophysitis is a rare inflammatory disorder, which may be caused by autoimmune response, affecting the infundibulum and the posterior lobe of the pituitary gland. The authors present a 15-year-old man with central diabetes insipidus, whose sellar magnetic resonance imaging (MRI) showed a focal thickening of the infundibulum and loss of high signal of the normal neurohypophysis on T1-weighted images. The histopathological diagnosis was lymphocytic infundibuloneurohypophysitis. When a patient presents with central diabetes insipidus and infundibular thickening on brain MRI, this entity should be included in the differential diagnosis though it is very rare. The significance of this entity in treatment is that it is basically not a surgical one, and surgery is needed only for tissue diagnosis. Symptomatic treatment and observation make it regress spontaneously.

• 대한한의학회지
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• 제21권1호
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• pp.99-102
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• 2000

Central diabetes insipidus(CDI) results from deficient vasopressin(antidiuretic hormone) secretion and causes polydipsia and polyuria. Its etiologic diagnosis is confirmed with an increase of urine osmolality by administering desmopressin(DDAVP) after water restriction. Because cm is caused by deficiency of vasopressin, up to now, desmopressin, a synthetic analog of vasopressin, has been the drug of choice in the treatment of CDI. However, under such treatment, CDI patients suffer from the continual administration of DDAVP throughout one's life and high cost of the treatment We administrated oriental herb medicine on a cm patient in a state of discontinuance of DDAVP. Prior to the study, brain sella MRI was scanned to exclude germinoma. In addition, urine analysis, serum and urinary osmolality, daily urinary volume, serum electrolyte levels were measured. Chungsimyunjatang was administered for 15 days, and urine analysis, urine osmolality, daily urinary volume, serum Na were measured several times again during the therapy, As a result, urinary frequency increased, serum Na slightly elevated, but specific gravity of urine, urinary osmolality severely decreased and daily urinary volume substantially increased. However, the frequency of DDAVP treatment was reduced from four times per day to once or twice a day with the continual administration of the Chungsimyunja-tang for two months after the discharge.

• 대한한방내과학회지
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• 제44권5호
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• pp.979-986
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• 2023

Objective: The purpose of this study is to report the case of intracranial germ cell tumor-induced central diabetes insipidus (CDI) in a child treated with Korean herbal medicine. Methods: A nine-year-old female patient diagnosed with intracranial germ cell tumor-induced CDI suffering from polyuria, polydipsia, and headache was hospitalized. Nocturia frequency and 24-hour urine volume were assessed. Results: The patient was treated with Korean medicine, including Nokyong-hwan. As a result of the 6-day inpatient treatment, nocturia frequency was decreased 3-4 times to 0-1 time, and 24-hour urine volume was decreased. Conclusion: Korean medicine, including Nokyong-hwan, may be considered an optional treatment for releasing the symptoms of intracranial germ cell tumor-induced CDI. Further studies are needed to confirm this finding.

• Korean Journal of Radiology
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• 제2권4호
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• pp.222-230
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• 2001

Central diabetes insipidus (DI) can be the outcome of a number of diseases that affect the hypothalamic-neurohypophyseal axis. The causes of the condition can be classified as traumatic, inflammatory, or neoplastic. Traumatic causes include postoperative sella or transection of the pituitary stalk, while infectious or inflammatory causes include meningitis, lymphocytic hypophysitis, and granulomatous inflammations such as sarcoidosis and Wegener's granulomatosis. Various neoplastic conditions such as germinoma, Langerhans cell histiocytosis, metastasis, leukemic infiltration, lymphoma, teratoma, pituitary adenoma, craniopharyngioma, Rathke cleft cyst, hypothalamic glioma, and meningioma are also causes of central DI. In affected patients, careful analysis of these MR imaging features and correlation with the clinical manifestations can allow a more specific diagnosis, which is essential for treatment.

• Clinical and Experimental Pediatrics
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• 제50권5호
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• pp.430-435
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• 2007

Even though we drink and excrete water without recognition, the amount and the composition of body fluid remain constant everyday. Maintenance of a normal osmolality is under the control of water balance which is regulated by vasopressin despite sodium concentration is the dominant determinant of plasma osmolality. The increased plasma osmolality (hypernatremia) can be normalized by the concentration of urine, which is the other way of gaining free water than drinking water, while the low plasma osmolality (hyponatremia) can be normalized by the dilution of urine which is the only regulated way of free water excretion. On the other hand, volume status depends on the control of sodium balance which is regulated mainly by renin-angiotensin-aldosterone system, through which volume depletion can be restored by enhancing sodium retention and concomitant water reabsorption. This review focuses on the urine concentration and dilution mechanism mediated by vasopressin and the associated disorders; diabetes insipidus and syndrome of inappropriate antidiuretic hormone secretion.

• Clinical and Experimental Pediatrics
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• 제45권7호
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• pp.902-905
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• 2002

선천성 신성 요붕증은 일반적으로 성염색체 열성 유전 양식을 취하여 보인자인 어머니로부터 아들에게 유전되고, 발생 빈도는 드문 것으로 알려져 있으며, 항이뇨호르몬의 혈장 농도가 정상이거나 상승되어 있으면서도 신세뇨관이 항이뇨호르몬에 대한 저항성 때문에 요농축능 장애가 발생하여 다음과 다뇨를 특징으로 하는 질환으로 이뇨제와 프로스타글란딘 합성억제제의 사용이 치료의 근간을 이루어 왔다. 본 증례에서 발견된 AVPR2 수용체 유전자의 돌연변이 부위는 선천성 신성 요붕증의 표현형을 보이는 것으로 외국에서는 보고된 바 있으나 국내에서는 최초로 확인된 것이며, 고용량의 항이뇨 호르몬과 이뇨제 치료에 반응을 보였다.

• Tuberculosis and Respiratory Diseases
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• 제57권3호
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• pp.284-288
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• 2004

폐암에서 중추성 요붕증이 병합되는 경우는 드물며, 그 원인은 대개 뇌하수체로의 전이에 의한 것으로, 이런 경우 수술적 치료나 방사선 치료가 도움이 된다. 그러나 부종양성 증후군의 일부로 발현될 수도 있으며, 이 경우에는 데스모프레신으로 효과적으로 치료할 수 있다. 저자들은 갈증, 다음, 다뇨를 주소로 내원한 비소세포폐암 환자에서 수분제한검사로 중추성 요붕증을 확진한 환자를 경험하였기에 이를 보고하는 바이다.

• Childhood Kidney Diseases
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• 제24권1호
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• pp.42-46
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• 2020

Disturbances in water and salt balances are relatively common in children after brain tumor surgery. However, the coexistence of different diseases of water and sodium homeostasis is challenging to diagnose and treat. The coexistence of combined central diabetes insipidus (CDI) and cerebral salt wasting syndrome (CSWS) is rare and may impede accurate diagnosis. Herein, we report the case of an 18-year-old girl who underwent surgery for a germinoma and who presented prolonged coexistence of CDI and CSWS. The patient was diagnosed with panhypopituitarism with CDI at presentation and was treated with hydrocortisone, levothyroxine, and desmopressin. Postoperatively, she developed polyuria of more than 3L/day, with a maximum daily urine output of 7.2 L/day. Her serum sodium level decreased from 148 to 131 mEq/L. Polyuria was treated with desmopressin at incremental doses, and hyponatremia was managed with fluid replacement. At 2 months after surgery, she presented with hyponatremia-induced seizure. Polyuria and hyponatremia combined with natriuresis indicated CSWS. Treatment with fludrocortisone were initiated; then, her electrolyte level gradually normalized. CSWS is self-limiting and generally resolves within 2 weeks. However, the patient in this study still required treatment with vasopressin and fludrocortisone at 16-months after surgery. Hyponatremia in a patient with CDI may be erroneously interpreted as inadequate CDI control or syndrome of inappropriate antidiuretic hormone secretion, leading to inappropriate treatment. The identification of the potential combination of CDI and CSWS is important for early diagnosis and treatment.