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      • KCI등재

        췌장 신경내분비 종양으로 나타난 von Hippel-Lindau (VHL) 질환 1예

        온정헌,김정희,이현정,서원우,황보율,홍은실,박진주,김성연 대한내분비학회 2011 Endocrinology and metabolism Vol.26 No.1

        Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder that results from a germline mutation of the VHL gene. The affected individuals might develop several benign or malignant tumors such as central nervous system or retinal haemangioblastomas,endolymphatic sac tumors, renal cell carcinomas, pheochromocytomas or pancreatic cysts and neuroendocrine tumors. We report here on a case of a 21 year old female with von Hippel-Lindau disease and she presented with only pancreatic neuroendocrine tumor and no evidence of haemangioblastomas or other visceral complications. Further, direct sequencing of the VHL gene reveals a novel germline frameshift mutation of codon 198 from the deletion of nucleotide 592 (cytosine), leading to truncation of the VHL protein.

      • KCI등재

        Transformation of Nonfunctioning Pancreatic Neuroendocrine Carcinoma Cells into Insulin Producing Cells after Treatment with Sunitinib

        온정헌,김영기,이세훈,정혜승 대한내분비학회 2013 Endocrinology and metabolism Vol.28 No.2

        We report a rare case of severe hypoglycemia after sunitinib treatment for pancreatic neuroendocrine carcinoma. We describe the initial clinical presentation, laboratory results, pathologic findings, and managment in a patient with a nonfunctioning pancreatic neuroendocrine carcinoma with liver metastases who developed life threatening hypoglycemia after 2 months of sunitinib therapy. A 46-year-old woman presented to the emergency department with loss of consciousness from hypoglycemia. Serum C-peptide and insulin levels at fasting state revealed that the hypoglycemia resulted from endogenous hyperinsulinemia. She had been diagnosed with nonfunctioning pancreatic neuroendocrine carcinoma based on a biopsy of metastatic cervical lymph node and was being treated with sunitinib, a small molecule tyrosine kinase inhibitor. Immunohistochemical stain of the metastatic liver mass demonstrated that the initially nonfunctioning neuroendocrine carcinoma cells had changed into insulin-producing cells after sunitinib therapy. Transarterial chemoembolization of the liver masses and systemic chemotherapy with streptozotocin/adriamycin relieved the hypoglycemia. A nonfunctioning pancreatic neuroendocrine carcinoma was transformed into an insulin-producing tumor after treatment with sunitinib, causing endogenous hyperinsulinemia and severe hypoglycemia.

      • KCI등재

        Expression of Thyroid Stimulating Hormone Receptor mRNA in Mouse C2C12 Skeletal Muscle Cells

        온정헌,한선경,박도준,박경수,박영주 대한내분비학회 2013 Endocrinology and metabolism Vol.28 No.2

        Background: We analyzed whether thyroid stimulating hormone receptor (TSH-R) is expressed in a skeletal muscle cell line and if TSH has influence on the differentiation of muscle cells or on the determination of muscle fiber types. Methods: TSH-R gene expression was detected with nested real-time polymerase chain reaction (RT-PCR) in C2C12, a mouse skeletal muscle cell line. The effect of TSH on myotube differentiation was assessed by microscopic examination of myotube formation and through the measurement of expression of muscle differentiation markers, i.e., myogenin and myoD, and muscle type-specific genes, i.e., MyHC1, MyHC2a, and MyHC2b, with quantitative RT-PCR before and after incubation of C2C12 myotube with TSH. Results: TSH-R was expressed in the mouse skeletal muscle cell line. However, treatment with TSH had little effect on the differentiation of muscle cells, although the expression of the muscle differention marker myogenin was significantly increased after TSH treatment. Treatment of TSH did not affect the expression of muscle type-specific genes. Conclusion: TSH-R is expressed in a mouse skeletal muscle cell line, but the role of TSH receptor signaling in skeletal muscle needs further investigation.

      • KCI등재

        An Acute Medical Unit in a Korean Tertiary Care Hospital Reduces the Length of Stay and Waiting Time in the Emergency Department

        온정헌,김낙현,김은선,백선하,임예지,허재형,이윤종,김의석,장학철 대한의학회 2017 Journal of Korean medical science Vol.32 No.12

        A hospitalist-run acute medical unit (AMU) opened at a tertiary care hospital on August 2015 for the first time in Korea. Patients visiting the emergency department (ED) with acute medical problems are admitted to the AMU. They stay in that unit for less than 72 hours and are discharged or transferred to specialty wards if longer treatment is necessary. We reviewed 19,450 medical admissions through the ED from January 2014 to September 2016. The median length of stay (LOS) significantly decreased from 10.0 days (interquartile range [IQR], 5.5–16.7) to 9.1 days (IQR, 5.1–15.0) (P < 0.001) after the establishment of the AMU. The median waiting time in the ED significantly shortened by 40% (P < 0.001). Future studies on the impact of AMU on in-patient morbidity, mortality, re-admission rate, and patient or staff satisfaction are necessary.

      • KCI등재

        시상하부-뇌하수체를 침범한 랑게르한스 세포 조직구증에 대한 임상적 고찰

        홍은실,온정헌,김정희,황보율,김진주,권정희,이정원,최세연,이은경,조선욱,신찬수,박경수,장학철,조보연,이홍규,신충호,양세원,김성연 대한내분비학회 2011 Endocrinology and metabolism Vol.26 No.1

        Background: Langerhans cell histiocytosis (LCH) is a rare disease that involves a clonal proliferation of Langerhans cells. LCH has a predilection for hypothalamo-pituitary axis (HPA) dysfunction, and this leads to diabetes insipidus (DI) and/or anterior pituitary dysfunction. Here, we describe the endocrine dysfunction and clinical characteristics of adult patients with LCH and we analyzed the differences between an adult-onset type and a childhood-onset type. Methods: The data was obtained from a retrospective chart review of the patients with LCH that involved the HPA and who attended Seoul National University Hospital. The patients were classified into the adult-onset type (age at the time of diagnosis ≥ 16) and the childhood-onset type (age at the time of diagnosis ≤ 15). Results: Ten patients (9 males and 1 female) were diagnosed with LCH involving the HPA. Five patients were classified as an adultonset type and the other five patients were classified as a childhood-onset type. The median follow-up duration was 6 (3-12) years for the adult-onset type and 16 (15-22) years for the childhood-onset type. All the patients presented with DI as the initial manifestation of HPA involvement. Four adult-onset patients and three childhood-onset patients had a multi-system disease. Panhypopituitarism developed in three adult-onset patients and in one childhood-onset patient. The pituitary lesion of the three adult-onset patients had spread to the brain during the follow-up duration. In contrast, the pituitary lesion of the other two adult-onset patients without panhypopituitarism and all the childhood-onset patients had not changed. Conclusion: DI was the initial presentation symptom of HPA involvement. Anterior pituitary hormone deficiency followed in some patients. Compared with the childhood-onset patients, the adult-onset patients were more likely to have panhypopituitarism and a poor prognosis.

      • KCI등재후보

        입원전담전문의가 바라보는 환자 안전과 의료의 질 향상

        정지수,온정헌 대한내과학회 2022 대한내과학회지 Vol.97 No.4

        Hospitalists are specialists in inpatient care who aim to improve patient safety and quality of care. Accordingly, hospitalist research focuses on patient safety and quality improvement (QI). Major hospital medicine journals publish studies on patient safety and QI. This review introduces the latest research related to patient safety and QI research in the field of hospital medicine.

      • 중증 고칼슘혈증과 고인산혈증을 동반한 갑상선기능항진증 1예

        이계연,전원선,온정헌,홍은경 순천향대학교 순천향의학연구소 2015 Journal of Soonchunhyang Medical Science Vol.21 No.1

        Changes of serum electrolytes in patients with hyperthyroidism are often disregarded. Hypercalcemia has been reported in 17% to 50% of patients with hyperthyroidism. However, severe and symptomatic hypercalcemia is rare among patients with hyperthyroidism. We report a rare case of symptomatic hypercalcemia and hyperphosphatemia in a 31-year-old male patient who was diagnosed with hyperthyroidism. He visited our hospital with nausea, vomiting, and tremor. Thyroid function test showed severe thyrotoxicosis and serum calcium and phosphorus were elevated but parathyroid hormone was low, excluding primary hyperparathyroidism as the cause of hypercalcemia. Saline hydration with diuretics to lower serum calcium and antithyroid medication with lugol solution were administered for six days. But symptoms persisted and he was treated with intravenous pamidronate. Symptoms were relieved after resolution of hypercalcemia and hyperphosphatemia. The case suggests that severe and symptomatic hypercalcemia and hyperphosphatemia can complicate hyperthyroidism and early correction of hypercalcemia can relieve symptoms.

      • KCI등재

        Adult Multisystem Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus Successfully Treated with Chemotherapy

        최정은,이해리,온정헌,문민경,박주리,이성진,최문기,유형준,김정한,홍은경 대한내분비학회 2014 Endocrinology and metabolism Vol.29 No.3

        We report the rare case of an adult who was diagnosed with recurrent multisystem Langerhans cell histiocytosis (LCH) involving the pituitary stalk and lung who present with central diabetes insipidus and was successfully treated with systemic steroids and chemotherapy. A 49-year-old man visited our hospital due to symptoms of polydipsia and polyuria that started 1 month prior. Two years prior to presentation, he underwent excision of right 6th and 7th rib lesions for the osteolytic lesion and chest pain, which were later confirmed to be LCH on pathology. After admission, the water deprivation test was done and the result indicated that he had central diabetes insipidus. Sella magnetic resonance imaging showed a mass on the pituitary stalk with loss of normal bright spot at the posterior lobe of the pituitary. Multiple patchy infiltrations were detected in both lung fields by computed tomography (CT). He was diagnosed with recurrent LCH and was subsequently treated with inhaled desmopressin, systemic steroids, vinblastine, and mercaptopurine. The pituitary mass disappeared after two months and both lungs were clear on chest CT after 11 months. Although clinical remission in multisystem LCH in adults is reportedly rare, our case of adult-onset multisystem LCH was treated successfully with systemic chemotherapy using prednisolone, vinblastine, and 6-mercaptopurine, which was well tolerated.

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