Endocrinology and Metabolism

Joong Yeol Park (Park JY)

17 Articles

A Case of Familial Multiple Endocrine Neoplasia Type 1 with a Novel Mutation in the MEN1 Gene.: Min Jung Kim, Eun Hee Kim, Mi Seon Shin, Joo Hui Kim, Hee Kyung Na, Seong Joon Park, Sang Ah Lee, Eun Hee Koh, Woo Je Lee, Ki Ho Song, Joong Yeol Park, Ki Up Lee, Gu Hwan Kim, Han Wook Yoo, Min Seon Kim; Endocrinol Metab. 2011;26(2):171-176. Published online June 1, 2011; DOI: https://doi.org/10.3803/EnM.2011.26.2.171

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Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by the occurrence of multiple tumors in the parathyroid gland, pancreatic islet, and pituitary gland. This condition is caused by mutations of MEN1, a tumor suppressor gene. Thus far, 565 different germline and somatic mutations of the MEN1 gene have been reported. Herein, we describe the case of a 23-year-old woman who suffered from a repetitive loss of consciousness. After workup, the patient was diagnosed with MEN1 with insulinoma, hyperparathyrodism due to parathyroid adenoma, and non-functioning pituitary microadenoma. She underwent a partial parathyroidectomy and distal pancreatectomy. Familial screening of MEN1 revealed that her brother had prolactinoma, hyperparathyroidism, pancreatic gastrinoma and non-functioning adrenal adenoma. Her father had hyperparathyroidism, pancreatic tumor, and adrenal adenoma. Upon genetic analysis of the MEN1 gene, a novel mutation in the MEN1 gene (exon 1, c.251del; p.Ser84LuefsX35) was detected in the patient, as well as her father and brother.

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Parathyroid disorder and concomitant thyroid cancer in patients with multiple endocrine neoplasia type 1
Ying Wang, Sheng Cai, He Liu, Rui-Na Zhao, Xing-Jian Lai, Ke Lv, Yu-Xin Jiang, Jian-Chu Li
Medicine.2021; 100(36): e27098. CrossRef
Genetic and Epigenetic Analysis in Korean Patients with Multiple Endocrine Neoplasia Type 1
Yoon Jung Chung, Sena Hwang, Jong Ju Jeong, Sun Yong Song, Se Hoon Kim, Yumie Rhee
Endocrinology and Metabolism.2014; 29(3): 270. CrossRef

Diabetic Ketoacidosis in a Patient with Acromegaly.: Eun Hee Koh, Min Kyung Kim, Jin Tae Park, Il Seong Nam-Goong, Joong Yeol Park, Ki Up Lee, Min Seon Kim; J Korean Endocr Soc. 2004;19(4):393-398. Published online August 1, 2004

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Abstract PDF: Acromegaly is a chronic, debilitating condition caused by excessive secretion of growth hormone (GH). Impaired glucose tolerance is present in about 20-40% of acromegaly, with diabetes mellitus developing in about 10~15% of patients, but diabetic ketoacidosis is a rare association. Herein is reported a case of diabetic ketoacidosis in a 33 year-old female, with a 4 year history of typical acromegaly features. She presented with severe hyperglycemia and ketoacidosis, but with no other cause for this metabolic derangement. She had elevated plasma GH (50 ng/mL) and IGF-1 (1533 ng/mL) levels, and a pituitary macroadenoma. About 200 units of insulin per day were required for her glycemic control. However, the serum IGF-1 level and daily insulin requirement were significantly tapered after a transsphenoidal adenomectomy and long acting somatostatin analogue treatment. There was a good correlation with the daily insulin requirement and plasma IGF-1 level. This case demonstrates that severe GH excess can cause diabetic ketoacidosis, and that its successful treatment improves glucose metabolism.

Improvement of Metabolic Syndrom by Alpha-lipoic Acid.: Eun Hee Koh, Woo Je Lee, Min Seon Kim, Joong Yeol Park, Ki Up Lee; J Korean Endocr Soc. 2004;19(3):267-273. Published online June 1, 2004

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Abstract PDF: No abstract available.

The Impaired Glucose Metabolism in Patients with Cushing's Syndrome.: Hyung Joo Park, Joong Yeol Park; J Korean Endocr Soc. 2003;18(4):356-359. Published online August 1, 2003

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Abstract PDF: No abstract available.

Oxidative Stress and Atherosclerosis.: Ha Young Kim, Joong Yeol Park; J Korean Endocr Soc. 2000;15(1):1-14. Published online January 1, 2001

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Abstract PDF: No abstract available.

A Case of Somatostatinoma which Manifested as Insulinoma when Metastasized to the Liver.: Joong Yeol Park, Byung Doo Lee, Kyung Soo Ko, Kyung Yub Gong, Ki Soo Kim, Sung Jo Bang, Jae Hwan Lee, Yoon Ey Chung, Sang Wook Kim, Hye Je Cho, Ki Ub Lee; J Korean Endocr Soc. 1998;13(4):670-676. Published online January 1, 2001

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Abstract PDF: We report a case of somatostatinoma, which manifested as insulinoma after liver metastasis. A 74-year-old man suffered from diabetes mellitus and jaundice. The abdominal CT scan of this patient showed a mass in the pancreas head, which obstructed biliary duct. He underwent Whipples procedure. Immunohistochemical staining of postoperative specimen disclosed that this tumor was strongly positive for somatostatin. After 4 months, follow up CT scan showed multiple metastatic lesions in the liver. We performed transarterial chemoinfusion two times, but the response was disappointing. After 2 months, he suffered from altered mentality, which was relieved by intake of sugar. Biochemical laboratory findings and immunohistochemical staining of liver biopsy disclosed that the metastatic lesion in this patient was insulinoma. We performed embolization of hepatic artery with gelform. The biochemical response was dramatic, but he died of septic shock, which was caused by gas forming liver abscess. In summary, we report a case of somatostatinoma which manifested as insulinoma after metastasis to the liver.

A Case of Polyglandular Autoimmune Syndrome.: Chul Hee Kim, Hong Kyu Kim, Joong Yeol Park, Young Ki Song, Ki Soo Kim, Kyo Sang Yoo; J Korean Endocr Soc. 1997;12(4):672-676. Published online January 1, 2001

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Abstract PDF: The polyglandular autoimmune syndrome is constellation of multiple endocrine insufficiencies often associated with diseases of nonendocrine organs occurring in individual patients and their families. In 1980, Neufeld classified this syndrome into three major types. Type II is characterized by adrenocortical insufficiency, autoimmune thyroiditis, and insulin-dependent diabetes mellitus. We experienced a case characterized by adrenocortical insufficiency, autoimmune thyroiditis, and ovarian failure and report with the review of the literature. A 38-year-old woman visited our clinic because of progressing brown colored pigmentation of skin and mucosa which is developed a year ago. Nine years ago prior to visit, amenorrhea was developed after right oophrectomy. Three years ago, she revealed feature of hyperthyroidism such as palpitation, loss of body weight (8kg/1-2years), heat intolerance, and sweating, so received antithyroid therapy for 14 months. Brown colored pigmentation of skin and mucosa, especially scar and gingiva, has been progressively aggravated during last year. She had no past or family history of other endocrine disease. Diffuse pigmentation of skin, loss of axillary and pubic hair, and diffuse enlargement of both thyroid glands were shown on physical examination. Blood cell count, serum chemistry and blood sugar test were all within normal range. Basal hormone levels were T3-uptake 29.7% (30~40), T3 153 ng/dL (85~185), T4 7.5ug/dL (5.5~11.5), TSH 2.4 IU (0.34~3.5), anti-TG antibody <100 U/mL (0~100), anti-microsome antibody <50 U/mL (0~100), TBII (thyrotropin binding inhibiting immunoglobulin) 2.2% ( (-15)~15), ACTH 989 pg/mL (0~37), cortisol 0.1 ug/dL (5~25), renin 7.1ng/mL/hr (1~2.5), aldosterone 81.0pg/mL (50~194), LH 115.2 mIU/mL (0.6~16.8), FSH 122 mIU/mL (1.6~19.0), and estradiol <10.0pg/mL (30~120). In ACTH stimulation test, levels of basal cortisol, 30 minutes, and 60 minutes were <0.1, <0.1, and <0.1 g/dL respectively. And, in glucagon stimulation test, levels of basal C-peptide, 5 minutes, 10 minutes, and 15 minutes were 0.9, 5,1, 6.3, and 5.5 ng/dL respectively. Thyroid scan showed diffuse enlargement of bilateral thyroid glands and pelvic ultrasonogram showed atrophy of left ovary. We administered corticosteroid, estrogen, and progesterone which were deficient to the patient, and has followed up the clinical course of the patient.

The Role of Low-dose ACTH Stimulation Test in the Diagnosis of Adrenal Insufficiency.: Chul Hee Kim, Ghi Su Kim, Hong Kyu Kim, Joong Yeol Park, Young Kee Shong, Ki Up Lee, Il Min Ahn, Sung Kwan Hong; J Korean Endocr Soc. 1997;12(2):222-229. Published online January 1, 2001

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Abstract PDF: BACKGROUND
Rapid adrenocorticotropin (ACTH) stimulation test using 250ug of ACTH (1-24) has been used as a standard test in the initial assessment of adrenal function. However, it has recently been suggested that a rnaximal cortisol response can be achieved with a much lower ACTH dose, and reducing the dose might further enhance the sensitivity of the test in the detection of mild adrenal insufficiency. This study was performed to evaluate the role of low-dose (lug) ACTH stimulation test in the assessment of adrenal function and the diagnosis of subtle adrenal insufficiency. METHODS: Twenty-two subjects with suspected adrenal insufficiency due to long-term corticosteroid use were included in this study. The correlations between clinical features and the serum cortisol responses to low dose (lug) and high dose (250 ug) ACTH stimulation were evaluated. RESULTS: In high dose test, 10 (67%) out of 15 subjects with clinical features of adrenal insufficiency showed decreased serum cortisol response (peak cortisol level <18 ug/dL), but 5 (33%) subjects showed normal response (peak cortisol level > 18ug/dL). On the other hand, 14 (93%) subjects with clinical features of adrenal insufficiency showed decreased serum cortisol response in low dose test, while only one showed normal response. In 7 subjects without clinical features of adrenal insufficiency, 5 subject (71%) showed normal response, and 2 subjects (29%) showed decreased response in both low and high dose tests. CONCLUSION: These results suggest that the 1-ug low dose ACTH stimulation test might be more sensitive than conventional 250-ug test in the detection of mild adrenal insufficiency. Further studies are needed to determine the optimal dose of ACTH and the criteria for normal response to ACTH stimulation.

Piruitray Thyrotropin-Secreting Tumors in Korean.: Chul Hee Kim, Ghi Su Kim, Hong Kyu Kim, Joong Yeol Park, Young Kee Shong, Sang Bum Hong, Jung Min Ko, Chang Jin Kim; J Korean Endocr Soc. 1997;12(2):165-175. Published online January 1, 2001

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Abstract PDF: BACKGROUND
Thyrotropin-secreting pituitary adenoma is an uncommon disease and about 150 cases has been reported in the world literature. In Korea, only seven cases were reported as yet. The authors recently experienced four cases of TSH secreting pituitary tumor and analyzed the clinical characteristics and treatment outcomes of TSH-secreting tumors in Korean. METHODS: We analyzed clinical records of the four cases who had been recently treated at Asan Medical Center and the Korean literature which deals with the previously reported seven cases of TSH-secreting pituitary tumor. RESULTS: The average age at diagnosis was 37 years (ranging from 11 to 55 years). Four were men and seven were women. After the detection of hyperthyroidism, TSH-secreting pituitary adenoma was diagnosed 3.6 years later on the average. Ten patients presented with hyperthyroidism, but one had primary hypothyroidism. Typical features of acromegaly were observed in two patients. Visual disturbance was present in three cases, and galactorrhea was present in one case. Serum TSH concentrations ranged from 1.5 to 42.5uIU/mL showing mildly elevated or unsup-pressed TSH levels despite of elevated serum thyroid hormone concentrations. Among six cases in whom a-subunit level was measured, five showed elevated a-subunit level and a-subunit/TSH molar ratio. Two of 11 cases had microadenoma and the remainder had macroadeno#ma. Immunohisto-cheical studies were done in eight cases and revealed that three were positive for TSH only and five patients were positive for multiple hormones. Eight patients underwent transsphenoidal pituitary surgery and seven (88%) of them were cured. External irradiation or octreotide was used as adjunctive treatment in three cases. After treatment, TSH levels decreased in all six patients studied, hyperthyroidism was eliminated in all eight patients studied and visual disturbance was improved in two patients. CONCLUSION: Clinical characteristics of TSH-secreting pituitary adenoma in Koreans were similar with world literature, but were more common in women, had less visual disturbance and better surgical results. Diagnosis was commonly delayed for several years. TSH-secreting pituitary adenoma may be diagnosed more frequently and earlier with widespread use of sensitive TSH assay and early and proper diagnosis would lead proper treatments with improved outcome.

Clinical Features of Multiple Endocrine Neoplasia Type I in Koreans.: Chul Hee Kim, Ghi Su Kim, Hong Kyu Kim, Joong Yeol Park, Young Kee Shong, Ki Up Lee; J Korean Endocr Soc. 1996;11(2):163-174. Published online November 7, 2019

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Abstract PDF: Background
Multiple endocrine neoplasia type I(MEN I) is a rare, eomplex, and potentially lethal disease. In Korean, only five anecdotal cases were reported as yet. The authors rescently experienced four cases of MEN I, and analysed the clinieal characteristics of MEN I in Koreans. Methods: The authors evaluated nine cases of MEN I, retrospectively. Four cases were analysed by clinical records in patients admitted to Asan Medical Center and five cases were reviewed by previously reported Korean literature from 1986 to 1995. Results: The average age was 39 years(ranged from 33 to 59 years). Eight of the nine patients had hyperparathyroidism documented by elevated serum calcium and PTH level with or without evidence of parathyroid mass. Initial presenting manifestations were symptomatic urinary stone, hypoglycernia due to insulinoma, hypogonadism, acromegaly, or peptic ulcer. Eight of nine patients had pancreatic islet cell tumors, and three of them were be malignant by radiologic and/or pathologic findings. The pancreatic tumors produced various hormones, such as gastrin, insulin, glucagon, or combination of them. Six of the nine cases had pituitary lesion. The most cornmon pituitary tumor was prolactinoma and the remaining was GH or GH and TSH producing tumor. In addition to the major components of MEN I, four had adrenocortical hyperplasia or adenoma and two had carcinoid tumor. There was only one familial case. Conclusion: The clinical charateristics of MEN I in Korean are mostly not different from the previous reports except older age at diagnosis, more comrnon adrenal involvement(44%) and gastrointestinal carcinoid tumor(22%). Although only one case was familial, more cases could be found if careful screening were done for the family members of the MBN I patients. In addition, screening and close follow up for endocrine pancreatic tumors are required for MEN I patients without detectable pancreatic lesion becau~se the malignant potential of pancreatic tumors has beeome an increasing concem for the prognosis of MEN I.

A Korean Pedigree of Paget Bone Disease.: Young Kee Shong, Joong Yeol Park, Ki Up Lee, Ghi Su Kim, Suhn Hee Kim, Jae Kun Cho, You Sook Cho, Hong Ja Kim, Myung Jin Shin; J Korean Endocr Soc. 1995;10(4):451-455. Published online November 7, 2019

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Abstract PDF: Paget bone disease(PBD) is usually focal, but can be wide spread disorder of the skeletal remodeling characterized by greatly increased osteoclast size and activity. It has extremely variable prevalence worldwide, being common in England and northern European countries and areas populated by their descendants, but strikingly uncommon in Asia, the middle east, Africa and Scandinavia. It's occurrence also shows familial clustering, some postulates autosomal dominant inheritance. Many studies have shown that paramyxoviruses may play a critical role in the etiology of this disorder. However, the precise etiology of PBD remains unknown.We describe a kindred with PBD in 3 successive generations. The propositus, a 55-year-old man, has panostotic PBD and giant cell reparative granuloma of pagets disease involving his head, mandible, abdomen and ileum, rare tumorous complication of Paget's disease. Bowed limbs were first noticed at age 25 years, and progressed for 20 years. Giant cell reparative granuloma began manifesting at age 45 years, and responded dramatically to high-dose dexamethasone therapy. His pretreatment biochemical finding were remarkable for elevated serum ALP, 765(normal 66-220 u/L) and osteocalcin, 154(normal 6.3-30.7 mg/ml), but normal serum calcium, phosphorous, 250HD and PTH. A nondecalcified iliac crest specimen demonstrated classic histopathologic 25OHD and PTH. A nondecalcified iliac crest specimen demonstrated classic histopathologic changes of PBD on light microscopy. His decreased father had a similar degree of bony deformities beginning at age 20 years, but had not been examined. His two asymptomatic daughters, 20 and 24-year-old, were both found to be affected with widespread PBD by bone scan, radiographic study, and their serum ALP levels, 939 and 435U/L, respectively. This is the first report of familial occurance of PBD and a case of giant cell reparative granuloma of Paget's disease in Korea, where PBD is very rare.

A Case of Nesidioblastosis in Adult with Hyperinsulinemic Hypoglycemia.: Young Kee Shong, Hong Kyu Kim, Young Joo Min, Joong Yeol Park, Sung Kwan Hong, Ki Up Lee, Duck Jong Han, Ho Jeong Lee, Ghi Su Kim, Gyung Yub Gong; J Korean Endocr Soc. 1994;10(3):273-277. Published online November 6, 2019

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Abstract PDF: Nesidioblastosis is a primarily childhood disease which is often associated with hyperinsulinemic hypoglycemia. It is very rarely found in adults. Only a few well documented cases are found in the world interature. The authors have recently experienced a case of nesidioblastosis in an adult. A 55-year-old man was admitted due to repeated episodes of reversible loss of consciousness. Hyperinsulinemic hypoglycemia was documented. Under the presumptive diagnosis of insulinoma, localization procedures were done but no definite tumor was found. Only suspicious gradient in insulin concentration was found around the head of pancreas by percutaneous transhepatic portal venous sampling. Exploratory laparotomy was performed and Whipple's operation was done. Seventy percent of proximal pancreas was removed. Histomorphometric study of the resected specimen revealed uneven graded hyperplasia of the islet cells with the most profuse hyperplasia in the head region and progressive decrease in the degree of hyperplasia to the body and tail. The patient remains euglycemia until 6 months after operation since immediate postoperative period and can tolerate 24 hour fasting without any medication.

Nesidioblastosis in Adult with Hyperinsulinemic Hypoglycemia.: Young Kee Shong, Hong Kyu Kim, Young Joo Min, Joong Yeol Park, Sung Kwan Hong, Ki Up Lee, Duck Jong Han, Ho Jeong Lee, Gyung Yub Gong; J Korean Endocr Soc. 1994;10(2):168-169. Published online November 6, 2019

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Abstract PDF: No abstract available.

A Case of Primary Thyroid Lymphoma Involving Bone Marrow (Stage IVE) Assoeiated with Hashimoto's Thyroiditis.: Young Kee Shong, Joong Yeol Park, Ghi Su Kim, Jae Kun Cho, Yun Ho Chu, Wan Sik Eom, Sang Hee Kim, Hyun Sook Chi, Gyung Yub Gong, Ki Up Lee; J Korean Endocr Soc. 1994;9(4):390-394. Published online November 6, 2019

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Abstract PDF: Primary lymphoma of the thyroid is a relatively rare malignant tumor of the thyroid. It is known to be frequently associated with Hashimoto's thyroiditis. In Korea, a few cases of primary lymphoma of the thyroid have been reported and most of these cases were in the stage I E or II E. Recently, we experienced a case of the primary thyroid lymphoma, stage IV E associated with Hashimoto's thyroiditis in a 70-year-old woman who presented with dysphagia and dyspnea due to rapidly enlarging neck mass.She was treated with combination chemotherapy(cyclophosphamide, adriamycin, vincristine and methylprednisolone) and local radiotherapy and achieved partial response with resolution of dysphagia and dyspnea.

Cushing's Disease Due to ACTH Producing Pituitary Carcinoma.: Young Kee Shong, Joong Yeol Park, Ghi Su Kim, Won Kyoung Cho, Jung Kyo Lee, Ghee Young Choe, Mun Ho Lee, Ki Up Lee; J Korean Endocr Soc. 1994;9(2):136-140. Published online November 6, 2019

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Abstract PDF: Primary carcinoma of the pituitary is only rarely reported. About half of the reported cases are nonfunctional. It is generally agreed that the presence of distant metastasis is required to clearly establish the diagnosis of pituitary carcinoma. We have experienced a case of ACTH-producing pituitary carcinoma causing Cushing's syndrome which could be diagnosed by histologic features only without evidence of distant metastasis. A 35-year-old female with Cushingoid appearance was diagnosed as Cushing's disease after biochemical and neuroradiological evaluation. Near total removal of the pituitary tumor was performed. By histopathologic examination, the tumor revealed evidences of histological malignancy such as prominent nuclear pleomorphism, frequent mitoses and extensive tumor necrosis. Pseudosarcomatous components were also noted. By immunohistochemical studies, the tumor cells expressed ACTH. However, there was no evidence of distant metastasis at the initial operation. She was diagnosed as having ACTH producing pituitary carcinoma by histologic feature only. One month later, she complained progressive nausea and vomiting, and follow-up brain MRI revealed regrowing pituitary mass. She was reoperated and near total tumor bulk was removed again followed by radiotherapy with 5580 rads. Four month after the second operation, she developed generalized tonic clonic seizure. Brain CT showed multiple enhancing nodules on left temporal and frontal lobes, and around falx cerebri. She refused further treatment and was managed only with anticonvalsants. About month after discharge she died at home.

Sporadic Nonfamilial Hypophosphatemic Osteomalacia.: Young Kee Shong, Joong Yeol Park, Ghi Su Kim, You Sook Cho, Goo Yeong Cho, Sang Wook Kim, Jung Sik Park, Ki Up Lee; J Korean Endocr Soc. 1994;9(1):25-31. Published online November 6, 2019

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Abstract PDF: Chronic hypophosphatemia caused by decreased intestinal absorption or increased renal clearance, may lead to rickets or osteomalacia independently of other predisposing abnormalities. The conditions commonly associated with increased renal clearance of phosphate are X-linked hypophosphatemic rickets, tumor associated rickets/osteomalacia, RTA and Fanconi syndrome. Recently we experienced 3 men with adult-onset, histologically proven osteomalacia associated with increased renal clearance of phosphate. None of them had a family history of bone disease, tumors or other tubular defects. All of these had remarkable biochemical and clinical improvement with medical treatment such as 1, 25-dihydroxyvitamin D and phosphate supplementation. Although we did not find tumors yet, we could not rule out the possibility of tumor-associated osteomalcia since it often takes several years to make a diagnosis because of small size, benign nature and unusual location of tumors. Thus, careful long-term follow up for tumor occurrence will be maintained in these patients with sporadic nonfamilial hypophosphatemic osteomalacia.

A case of osteoporosis associated with pernicious anemia.: Sang Wook Kim, Seung Won Choi, Jung Shin Lee, Joong Yeol Park, Ki Up Lee, Ghi Su Kim; J Korean Endocr Soc. 1993;8(3):351-355. Published online January 1, 2001

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Abstract PDF: No abstract available.

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