Endocrinology and Metabolism

Sei Hyun Kim (Kim SH)

6 Articles

Two Case of Primary Aldosteronism Induced by Aldosterone Producing Adrenal Adenoma in a Family.: Young Rock Jang, Sei Hyun Kim, Young Sil Eom, Ki Young Lee; Endocrinol Metab. 2012;27(4):329-333. Published online December 20, 2012; DOI: https://doi.org/10.3803/EnM.2012.27.4.329

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Abstract PDF: Primary aldosteronism, is defined as a group of disorders characterized by the excess of aldosteron, with suppressed rennin activity, resulting in hypertension and hypokalemia. In most cases, primary aldosteronism is sporadic due to a unilateral adrenal adenoma or bilateral adrenal hyperplasia. Familial hyperaldosteronism is a rare cause of primary aldosteronism and its prevalence has not been established well. We describe two cases of primary aldosteronism in a family involving a sister and brother due to an aldosterone producing adenoma in the left adrenal gland. Their hypokalemia and hypertension were cured by complete resection of the adrenal adenoma. Genetic analyses could not be done because of patients' rejection.

A Case of the Type B Insulin Resistance Syndrome with Chronic Hepatitis B.: Hyun Seok Choi, Byoung Ho Choi, Seok Hoo Jeong, Shung Han Choi, Dong Su Shin, Sei hyun Kim, Young Sil Eom, Sihoon Lee, Yeun Sun Kim, Ie Byung Park, Ki Young Lee; Endocrinol Metab. 2011;26(4):360-363. Published online December 1, 2011; DOI: https://doi.org/10.3803/EnM.2011.26.4.360

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Abstract PDF: Type B insulin resistance syndrome is rare autoimmune disease that is characterized by various abnormalities of glycemic homeostasis, from hyperglycemia caused by extreme insulin resistance to fasting hypoglycemia. It can combine with other autoimmune diseases, most commonly systemic lupus erythematosus. It usually occurs in women and accompanies acanthosis nigricans, hyperandrogenism, and, in many cases, ovary dysfunction. The diagnosis of type B insulin resistance syndrome is based largely on the presence of insulin receptor autoantibodies and hyperglycemia, or hypoglycemia and hyperinsulinemia. In some cases, patients with the type B insulin resistance have been successfully treated with immunosuppressive therapy and plasmapheresis. We experienced type B insulin resistance syndrome in a patient with chronic hepatitis B and used only plasmapheresis for treatment. The immunosuppressive therapy was omitted due to the state of activation of chronic hepatitis B. We present this case with a review of relevant literature.

A Case of Ascites and Extensive Abdominal Distension Caused by Reversible Pulmonary Arterial Hypertension Associated with Graves' Disease.: Byoungho Choi, Young Sil Eom, Sei Hyun Kim, Hyun Seok Choi, Wook Jin Chung, Sihoon Lee; Endocrinol Metab. 2011;26(3):248-252. Published online September 1, 2011; DOI: https://doi.org/10.3803/EnM.2011.26.3.248

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Abstract PDF

Patients with hyperthyroidism can develop left ventricular dysfunction and heart failure, but severe pulmonary hypertension association with hyperthyroidism is rarely seen. Herein, we describe the case of a 27-year-old female who presented with abdominal distension accompanied by pulmonary arterial hypertension and Graves' disease. Her pulmonary arterial hypertension was improved by treating the hyperthyroidism and pulmonary artery hypertension. Additionally, the patient's symptoms of right-side heart failure improved after pulmonary arterial pressure was reduced. Hyperthyroidism should be regarded as a reversible cause of associated pulmonary arterial hypertension.

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A Case of Pulmonary Hypertension Recurred by Graves’ Disease
Jun Seop Lee, Young Sik Choi, Jae Woo Lee, Jin Seok Yoo, Youn Jung Choi, Dong Hyun Park
Kosin Medical Journal.2013; 28(2): 171. CrossRef

Mutational Analysis of the NF1 Gene in Two Families with Neurofibromatosis 1 Accompanied by Pheochromocytoma.: Hyon Seung Yi, Sei Hyun Kim, Jihoon Kim, Eun Jin Bae, Suntaek Hong, Ie Byung Park, Yu Jin Kim, Sihoon Lee; Endocrinol Metab. 2011;26(2):177-184. Published online June 1, 2011; DOI: https://doi.org/10.3803/EnM.2011.26.2.177

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Abstract PDF

Neurofibromatosis type 1 (NF1) is one of the most common autosomal dominant inherited disorders affecting the nervous system. NF1 is associated with mutations in the NF1 gene, which is located on chromosome sub-band 17q11.2 and contains 57 exons spanning approximately 300 kb of genomic DNA. NF1 is caused by a loss of function mutation of the NF1 gene, a tumor suppressor gene, which encodes for neurofibromin, a GTPase-activating protein (GAP) involved in the negative regulation of Ras activity. The GAP-related domain, which is encoded for by exons 20-27a, is one of the most important functional domains in neurofibromin. The cysteine-serine-rich domain has been recognized as an important functional domain in NF1-related pheochromocytomas. As the result of many genetic analyses of NF1-related pheochromocytomas, pheochromocytoma has generally been recognized as a true component of NF1. We recently experienced two families with NF1 accompanied by pheochromocytoma. The proband of family 1 is a 31-year-old female diagnosed with NF1 and pheochromocytoma. Gene analysis of the proband and her sister showed that the mutation of the NF1 gene (c.7907+1G>A) led to the skipping of exon 53 during NF1 mRNA splicing. The proband of family 2 is a 48-year-old male who was diagnosed with the same condition. Gene analysis demonstrated the mutation of the NF1 gene (c.5206-8C>G) with missplicing of exon 37. These novel germline mutations did not fall into the GAP-related nor the cysteine-serine-rich domains, but into the C-terminal area of the NF1 gene. This suggests that the correlation between the genotype and phenotype of NF1-related pheochromocytoma is somewhat difficult to characterize. Further studies will be necessary to confirm the function of the C-terminal area of the NF1 gene and its contribution to the development of NF1 and pheochromocytoma.

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Mutation Spectrum of NF1 and Clinical Characteristics in 78 Korean Patients With Neurofibromatosis Type 1
Jung Min Ko, Young Bae Sohn, Seon Yong Jeong, Hyon-Ju Kim, Ludwine M. Messiaen
Pediatric Neurology.2013; 48(6): 447. CrossRef
Oncologic manifestations of neurofibromatosis type 1 in Korea
Eui Tae Kim, Hwan Namgung, Hyun Deok Shin, Soon Il Lee, Jee Eun Kwon, Myung Chul Chang, Dong Guk Park
Journal of the Korean Surgical Society.2012; 82(4): 205. CrossRef

Acromegaly with Diabetes Insipidus after Pituitary Tumor Removal: Successful Pregnancy and Delivery.: Sei Hyun Kim, Joo Il Kim, Yae Min Park, In Sik Won, Kwen Chul Shin, Yunjeong Jo, Sihoon Lee, Yeun Sun Kim, Ki Young Lee, Ie Byung Park; J Korean Endocr Soc. 2010;25(1):56-60. Published online March 1, 2010; DOI: https://doi.org/10.3803/jkes.2010.25.1.56

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Abstract PDF: A 33-year-old woman visited our hospital because of oligomenorrhea. Acromegaly was diagnosed based on elevated insulin like growth factor-I (IGF-I) and paradoxical growth hormone (GH) rise in oral glucose tolerance test. Pituitary macroadenoma was detected on magnetic resonance imaging (MRI). The pituitary tumor was removed. Still, diabetes insipidus developed. We prescribed desmopressin and bromocriptine. Two months post-surgery, IGF-I was decreased and a combined pituitary function test was normal, except for the follicle stimulating hormone response. Residual tumor was detected on MRI. The bromocriptine dose was increased and treatment with the long-acting somatostatin analogue octreotide long acting release (LAR) was begun. After the fifth round of octreotide LAR, IGF-I was normalized. After the seventh round of octreotide LAR, the patient became pregnant. Bromocriptine and octreotide LAR were stopped, and desmopressin was continued. Successful delivery occurred at week 38 of pregnancy. The patient was discharged without any complications. Acromegaly is a disease caused by chronic GH hypersecretion, generally related to a somatotroph adenoma. Amenorrhea and menstrual irregularities are common in acromegaly. Pregnancy rarely occurs because chronic anovulation usually exists. When gonadotroph axis was preserved, the possibility of pregnancy in a woman of child-bearing age with acromegaly should be considered.

A Case of Hashimoto's Thyroiditis Accompanied by Autoimmune Hepatitis Diagnosed with Liver Biopsy.: Young Jun Lee, Ji Yoon Sung, Sei Hyun Kim, Hyon Seung Yi, Yun Soo Kim, Sihoon Lee, Ie Byung Park; J Korean Endocr Soc. 2009;24(4):287-292. Published online December 1, 2009; DOI: https://doi.org/10.3803/jkes.2009.24.4.287

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Abstract PDF

Autoimmune thyroid diseases, including Hashimoto's thyroiditis (HT), are common organ-specific autoimmune disorders that often occur in conjunction with other autoimmune diseases. Autoimmune hepatitis (AIH) is a relatively rare disease of unknown etiology. In this condition, progressive destruction of the liver parenchyma occurs. Without proper treatment with immunosuppressive agents, such as prednisone and azathioprine, this condition leads to cirrhosis and liver failure. Timely detection and appropriate treatment of the AIH is prerequisite for the long-term survival of affected patients. We report here a case of HT accompanied by AIH confirmed by liver biopsy. On the basis of this case report, we suggest that, a sustained elevation of aminotransferases refractory to thyroid dysfunction correction should result in a liver biopsy to differentiate AIH from other forms of liver dysfunction or secondary to thyroid disorders. Treatment should commence promptly.

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Autoimmune Hashimoto thyroiditis with concomitant autoimmune hepatitis
Nevena Manevska, Natasa Stojkovska, Ljubica Tasheva, Marija Jovanovski-Srceva, Tanja Makazlieva, Sinisha Stojanoski
Archives of Public Health.2022;[Epub] CrossRef
A Case of Demyelinating Peripheral Neuropathy Associated with Hashimoto`s Thyroiditis
Jung Hwan Park M.D., Sang Mo Hong M.D., Chang Bum Lee M.D., Yong Soo Park M.D., Dong Sun Kim M.D., Woong Hwan Choi M.D., You Hern Ahn M.D.
Journal of the Korean Geriatrics Society.2011; 15(4): 234. CrossRef

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