• The Korean Journal of Nuclear Medicine
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• v.10 no.1
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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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• v.55 no.7
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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.

• Korean Journal of Clinical Laboratory Science
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• v.49 no.4
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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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• v.9 no.2
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• pp.185-195
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• 1991

Seventy four patients with pituitary adenoma received radiation therapy (RT) on the pituitary area using 6 MV linear accelerator during the past 7 years at the Division of Radiation Therapy, Kangnam St. Mary's Hospital, Catholic University Medical College. Thirty nine were men and 35 were women. The age ranged from 7 to 65 years with the mean being 37 years. Sixty five ($88\%$) patients were treated postoperatively and 9 ($12\%$) primary RT, To evaluate the effects of RT, we analyzed the series of endocrinologic studies with prolactin (PRL), growth hormone (GH), adrenocorticotrophic hormone (ACTH), leuteinizing hormone (LH), follicular stimulating hormone (FSH) and thyroid stimulating hormone (TSH) etc after RT. All but one with Nelson's syndrome showed abnormal neuroradiologic changes in the sella turcica with invasive tumor mass around supra- and/or parasella area. The patients were classified as 23 ($29\%$) prolactinomas and 20 ($26\%$) growth hormone (GH) secreting tumors, and 6 ($8\%$ ACTH secreting ones consisting of 4 Cushing's disease and 2 Nelson's syndrome. Twentynine ($37\%$) had nonfunctioning tumor and four ($5\%$) of those secreting pituitary tumors were mixed PRL-GH secreting tumors. The hormonal level in 15 ($65\%$) of 23 PRL and 3 ($15\%$) of 20 GH secreting tumors returned to normal by 2 to 3 years after RT, but five PRL and five GH secreting tumors showed high hormonal level requiring bromocriptine medication. Endocrinologic insufficiency developed by 3 years after RT in 5 of 7 panhypopituitarisms, 4 of seven hypothyroidisms and one of two hypogonadisms, respectively. Fifteen ($20\%$) patients were lost to follow up after RT.

• The Korean Journal of Nuclear Medicine
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• v.13 no.1_2
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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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• v.49 no.3
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• pp.292-297
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• 2006

Purpose : In medulloblastoma, craniospinal radiation therapy combined with chemotherapy improves the prognosis of tumors but results in significant endocrine morbidities. We studied the endocrine morbidity, especially growth pattern changes. Methods : The medical records of 37 patients with medulloblastoma were reviewed retrospectively for evaluation of endocrine function and growth. We performed the growth hormone stimulation test in 16 patients whose growth velocity was lower than 4 cm/yr. Results : The height loss was progressive in most patients. The height standard deviation score (SDS) decreased from $-0.1{\pm}1.3$ initially to $-0.6{\pm}1.0$ after 1 year(P<0.01). Growth hormone deficiency(GHD) developed in 14 patients. During the 2 years of growth hormone(GH) treatment, the improvements of height gain or progressions of height loss were not observed. Twelve patients(32.4 percent) revealed primary hypothyroidism. One of six patients diagnosed with compensated hypothyroidism progressed to primary hypothyroidism. Primary and hypergonadotropic hypogonadism were observed in two and one patients respectively. There was no proven case of central adrenal insufficiency. Conclusion : Growth impairment developed frequently, irrespective of the presence of GHD in childhood survivors of medulloblastoma. GH treatment may prevent further loss of height. The impairment of the hypothalamic-pituitary-gonadal and hypothalamic-pituitary-thyroidal axis is less common, while central adrenal insufficiency was not observed.