• 대한핵의학회지
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• 제10권1호
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• pp.15-20
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• 1976

8 cases of sheehan's syndrome, diagnosed clinically and with laboratony tests, ware tested for the TSH secretion after TRH ($200{\mu}g$) stimulation. 2 cases out of the 8 showed normal response in its TSH secretion, and was interpreted as an evidence for clinical complexity in pituitary insufficiency of the sheehan's syndrome.

• 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.

• 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.

• Radiation Oncology Journal
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• 제9권2호
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• pp.185-195
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• 1991

가톨릭의대 강남성모병원 방사선치료실에서는 1983년 3월부터 1990년 4월 사이 7년여 동안에 뇌하수체선종으로 확진되었던 74명의 환자($7\~65$세, 평균 37세, 남:여=39:35)에 대하여 뇌하수체부위에 외부방사선치료를 하였다. 방사선치료는 6 MV 선형 가속기를 사용하여 선원중심축거리 80 cm, $4{\times}4{\times}4\~8{\times}8{\times}8cm^3$ 조사야로 3문조사하였다(180cGy/일, 5회/주, $20\~65$Gy). 5예에서는 정위다방향고선량단일조사($15\~25$ Gy/l회)를 실시하였다. 이 환자들의 수술전후 및 방사선치료 후에 복합뇌하수체자극검사를 실시하여 종양의 호르몬분비에 따른 분류와 방사선치료가 호르몬분비에 미치는 영향을 추적조사하였다. 호르몬분비에 따른 뇌하수체선종의 분류는 프로랙틴종 23예($29\%$), 성장호르몬분비종 20예($26\%$), 부신피질자극호르몬분비종 6예 ($8\%$) (쿠싱병 4예와 넬슨증후군 2예)였으며 4예 ($5\%$)에서는 프로랙틴과 성장호르몬을 동시에 분비하였다. 한편, 호르몬분비기능없는 종양이 29예($37\%$)였다. 23예의 프로랙틴종 중 15예 ($65\%$)와 20예의 성장호르몬분비종 중 3예 ($15\%$)에서는 방사선치료$2\~3$년 후 정상 호르몬 수치로 전환되었다. 부신피질자극호르몬분비종은 6예 중 1예를 제외한 전예에서 치료 후 모두 정상이되었다. 방사선치료에 따른 부작용으로서 범하수체기능저하증 및 갑상선기능저하증이 각각 7예($9\%$)씩 그리고 성선기능저하증 2예 ($3\%$) 등이 관찰되었다. 전예 중 15예 ($20\%$)에서는 정기적인 호르몬 추적검사를 실시하지 못하였다.

• 대한핵의학회지
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• 제13권1_2호
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• pp.37-43
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• 1979

This is an analysis of 39 patients studied at the Yonsei Medical Center from January, 1976 to March 1979. Of these35 patient were suspected of having hypothalamic insufficiency and subjected to the L-Dopa stimulation test to observe growth hormone sceretory function while four acromegaly patient received the glucose loading test and L-Dopa stimulation test. The results are as follows: 1. The basal level of GH in the various disease was as follows: a. The basal level was lower than the control level but was not statistically significant b. In diabetes the mean value tended to be higher than the control level but was not significant statistically c. In all four acromegaly patients the GH level was significantly higher than the control level 2. Of 13 patients with diabetes, nine had diabetic retinopathy, and of those nine, sir showed increased L-Dopa response. However, of the four non retinopathic DM patients, only one showed increased response to L-Dopa. 3. Two patients out of ten with Sheehan's syndrome responded to L-Dopa stimulation. 4. One Patient of eight with pituitary chromophobe adenoma responded to L-Dopa stimulation. 5. Four acromegaly patients revealed 3 acidophilic adenoma and one chromophobe adenoma histologically. Of patients receiving the L-Dopa stimulation test. Two showed a paradoxical response. Two patients who received the glucose loading test showed supressed response. 6. Of two craniopharyngioma patients, one showed increased GH response after L-Dopa stimulation. Increased response of GH after L-Dopa stimulation was seen in one of two craniopharyngioma patients and also in one of two patients with

• Clinical and Experimental Pediatrics
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• 제49권3호
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• pp.292-297
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• 2006

목 적 : 소아 수모세포종은 수술과 중추신경계 방사선조사 그리고 항암화학요법의 병합치료로 장기 생존율의 향상을 가져왔으나 성장호르몬결핍을 포함한 여러 내분비적 장애가 잘 발생하기에, 본 저자들은 수모세포종 치료 후 내분비적 장애와 성장변화를 관찰하고 해당 호르몬 치료의 성과를 분석하였다. 방 법 : 1986-2004년까지 서울대학교병원에서 수모세포종 치료를 마친 후 소아내분비 외래를 방문한 37명(남아 24명, 여아 13명)을 대상으로 내분비 장애와 성장변화에 대하여 분석하였다. 결 과 : 성장속도가 4 cm/yr 미만인 환자 중 16명에서 성장호르몬자극검사가 시행되었고 14명(전체환자 중 37.8%)이 성장호르몬결핍증으로 진단받았으며(완전 5명, 부분 9명), 2명의 환자는 신경분비장애로 의심되었다. 성장호르몬결핍증 환자군과 성장호르몬결핍이 없는 군 모두에서 방사선치료 전후의 키 표준편차 점수는 모두 유의한 감소를 보였으며(P<0.001), 성장호르몬결핍이 있는 12명과 신경분비장애가 있는 1명의 환자에게서 성장호르몬 치료를 시작하였다. 치료 전 골연령은 $9.3{\pm}4.7$세로 역연령에 비해 2.1세 저하되어 있었다. 치료 전 성장속도는 $3.4{\pm}1.2cm/yr$에서 1년째 $5.4{\pm}2.9cm/yr$로 유의하게 증가하였으나 키표준편차점수는 치료 전과 치료 후 유의한 변화가 없었다. 전체 37명 중 12명(32.4%)이 일차성 갑상선기능저하증으로 갑상선호르몬 치료를 받았다. 또한 다른 6명(16.2%)에서는 보상성 갑상선기능저하증이 의심되었다. 1명의 환자에서 방사선 치료종료 7년 후에 갑상선암이 발생하여 갑상선 전절제술을 시행 받은 후 갑상선호르몬을 복용 중이다. 성선자극호르몬결핍 1명(2%), 성선부전증이 2명(4%) 진단되었다. 결 론 : 수모세포종 치료 후 대부분 성장장애가 관찰되며 주로 성장호르몬결핍증과 척수방사선조사 등에 기인한다. 성장호르몬결핍증과 갑상선기능저하증 및 다른 내분비적 장애가 발생할 수 있으므로 지속적인 정기 추적 관찰 및 적절한 치료가 필요하다.