• 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.

• Tuberculosis and Respiratory Diseases
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• 제46권1호
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• pp.110-115
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• 1999

저자들은 호흡곤란과 다음, 다뇨로 내원한 23세 남자에서 임상소견, 흉부 X-선 검사, 고해상도 흉부단층 촬영, 수분제한검사, 개흉 폐생검으로 확진된 흔치 않은 중추성 요붕증을 통반한 원발성 폐 조직구증 X를 경험하였기에 문헌고찰과 함께 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.

• Childhood Kidney Diseases
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• 제16권2호
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• pp.126-131
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• 2012

쇠그렌 증후군은 주로 침샘과 눈물샘을 침범하는 자가 면역 질환으로, 폐나 간, 콩팥, 췌장, 피부, 신경계 등의 다른 장기 역시 침범하여 임상 증상을 나타내기도 한다. 성인의 경우 이런 다른 장기와 관련된 증상은 드물지 않으나 소아에서는 매우 드물다고 알려져 있다. 특히 콩팥을 침범한 쇠그렌 증후군은 성인의 경우 비교적 흔하지만 소아는 매우 드문데, 이에 본 저자들은 신성 요붕증과 신세뇨관 산증을 주 증상으로 진단한 소아의 일차성 쇠그렌 증후군 1례를 경험하였기에 보고한다.

• Clinical and Experimental Pediatrics
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• 제50권11호
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• pp.1110-1115
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• 2007

목 적 : 특별한 원인 없이 발생하는 특발성 중추성 요붕증 중에서 일부는 후에 뇌종양이 발견된다. 이에 저자들은 요붕증 진단 시 특발성 중추성 요붕증으로 진단 받은 20명의 환자들에서 임상적, 방사선학적, 그리고 내분비학적 경과를 추적 관찰하여 기질성 원인으로의 진행 유무를 예측할 수 있는 지표가 있는지 연구하였다. 방 법 : 1984년 1월부터 2006년 12월까지 서울대학교병원에서 중추성 요붕증으로 진단 받았던 환자들 중 뇌 자기공명영상에서 뚜렷한 기질적 원인의 증거가 없었던 20명의 환자들에서 후향적으로 임상소견과 검사소견을 검토하였다. 중추성 요붕증 진단 시 뇌종양, 선천성 중추신경계 기형이 있는 경우는 제외시켰다. 결 과 : 남아 15명, 여아 5명이었고, 진단 시 평균 연령은 $7.8{\pm}3.6$(2.1-14.7)세로, 야뇨증이 11명, 두통이 4명, 구토가 2명에서 있었다. 평균 추적관찰 기간은 $8.6{\pm}5.1$(1.5-18)년 이었다. 추적관찰 도중 뇌종양으로 진단 받은 환자는 6명(30%)이었다(생식세포종 5명, 랑게르한스 조직구증 1명; 추적기간 $2.6{\pm}2.1$년). 성장호르몬결핍증은 10명(50%)에서 관찰되었다. 복합 뇌하수체전엽호르몬결핍은 뇌종양 환자군에서 비뇌종양 환자군에서보다 더 높은 빈도로 관찰되었다(60% vs. 7%, P=0.037). 진단 시 뇌하수체 줄기 비후는 9명(47%), 뇌하수체 후엽 신호 소실은 9명(47%)에서 관찰되었으며, 추적 중 새롭게 뇌하수체 줄기 비후가 생긴 3명의 환자 모두에서 뇌종양이 발생하였다. 결 론 : 특발성 중추성 요붕증 환자의 일부는 추적관찰 중 뇌종양을 발견할 수 있는데, 특히 복합 뇌하수체전엽호르몬결핍증이 동반되거나, 뇌하수체 줄기 비후가 새롭게 발생하는 경우에는 더욱 세심한 뇌종양에 대한 추적관찰이 필요하다.

• Clinical and Experimental Pediatrics
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• 제59권4호
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• pp.202-204
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• 2016

A 15-year-old boy, who was diagnosed with Alport syndrome and end-stage renal disease, received a renal transplant from a living-related donor. On postoperative day 1, his daily urine output was 10,000 mL despite normal graft function. His laboratory findings including urine, serum osmolality, and antidiuretic hormone levels showed signs similar to central diabetes insipidus, so he was administered desmopressin acetate nasal spray. After administering the desmopressin, urine specific gravity and osmolality increased abruptly, and daily urine output declined to the normal range. The desmopressin acetate was tapered gradually and discontinued 3 months later. Graft function was good, and urine output was maintained within the normal range without desmopressin 20 months after the transplantation. We present a case of a massive polyuria due to transient deficiency of antidiuretic hormone with the necessity of desmopressin therapy immediately after kidney transplantation in a pediatric patient.

• Journal of Korean Neurosurgical Society
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• 제29권10호
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• pp.1316-1321
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• 2000

Objectives : The authors analyzed the surgical series of Cushing's disease to evaluate the proper treatment policy and to verify the possible prognostic factors. Material and Methods : Of 50 patients diagnosed as Cushing's disease and operated at Department of Neurosurgery of our institute between 1988 and 1999, 48 patients with available medical records were analyzed retrospectively. Mean follow-up period was 48 months(3 to 109 months). Preoperative diagnosis was made after evaluating the patients with multiple-stage endocrinological studies and 31 selective patients were evaluated with inferior petrosal sinus sampling(IPSS). Magnetic resonance imaging(MRI) and/or high resolution computerized tomography(CT) was done in all patients. A total of 51 transsphenoidal adenomectomy(TSA) were performed including 3 revision for initial surgical failure cases. Remission was decided on the basis of both endocrinological criteria and clinical status. Radiation and/or ketoconazole therapy were applied to failed cases. For the verification of prognostic factors, the authors evaluated the statistical significance of multiple variables over remission rate by chi-square test. Result : Sensitivity of IPSS for central localization was 93.5% which was better than that of MRI(87.5%). But for lateralization, it was 72.4% for IPSS versus MRI 90.5%. Success rate of TSA was 82%(42/51) and recurrence rate was 9%(4/48). When including adjuvant treatments for surgically failed cases, overall success rate was 89.6% and all of 3 reoperated cases(TSA) due to recurrence were successful. Significant complication occurred in 7.8%(4/51) after TSA including hypopituitarism, diabetes insipidus, and visual loss. Non-existence of tumor in MRI and prolonged symptom duration(>3 years) were significant prognostic factors. Conclusion : TSA can be considered as initial treatment for Cushing's disease. In surgically failed cases, multiple treatment modality may improve the overall outcome and repeated TSA for recurrent cases seem to provide similar success.