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      • KCI등재

        제1형 당뇨병환자에서 동반된 만성 염증성 탈수초성 다발신경병증 1예?

        장이선,이종민,김혜수 대한당뇨병학회 2006 Diabetes and Metabolism Journal Vol.30 No.2

        -Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated disorder characterized by the symmetrical weakness in both proximal and distal muscles for at least 2 months, hyporeflexia or areflexia, nerve conduction abnormalities, and high CSF protein level. Diabetes mellitus, monoclonal gammopathy, hepatitis C infection, HIV infection, SLE, Sjogren syndrome and lymphoma have been associated with CIDP. The incidence of CIDP in diabetes is not known exactly, but occur more common among diabetic than nondiabetic patients. There is sometimes a difficulty in distinguishing between diabetic polyneuropathy and CIDP, but differential diagnosis is important because CIDP is treatable with immune-modulating therapy. We report a case of CIDP in 22-year-old girl with type 1 DM who presented with generalized motor weakness and walking disturbance which were treated with iv immunoglobulin (J Kor Diabetes Assoc 30:130~135, 2006). 저자들은 근육 위축 및 보행장애를 주소로 내원한 22세 여자 환자에서 제1형 당뇨병을 발견하고 임상증세, 전기생리학적 검사 및 뇌척수액 검사를 종합하여 만성 염증성 탈수초성 다발신경병증을 진단하여 면역글로불린 치료를 시행한 1례를 경험하였기에 보고하는 바이다.

      • KCI등재

        A novel PRKAR1A mutation resulting in a splicing variant in a case of Carney complex

        장이선,문성대,김주희,이인석,이종민,김혜수 대한내과학회 2015 The Korean Journal of Internal Medicine Vol.30 No.5

        Almost all PRKAR1A mutations in CNC are located in exons and the most frequent is a deletion in exon 5 [3], but we present a novel mutation of the PRKAR1A gene in intron 4. The test for an inactivating mutation of PRKAR1A is only a supplemental criterion for diagnosing CNC [1], but it can be useful to provide genetic counseling and monitor tumor growth in an asymptomatic CNC patient.

      • KCI등재

        고혈압 환자에서 당뇨병 유무에 따른 원발성 알도스테론증의 유병률 비교

        장이선,김군순,김혜수 대한내분비학회 2009 Endocrinology and metabolism Vol.24 No.4

        Background: Primary aldosteronism is the most common cause of secondary hypertension in humans. Its prevalence is estimated to be 10-15% among hypertensive patients. It is also associated with insulin resistance and diabetes mellitus. The aim of our study was to compare the prevalence of primary aldosteronism in hypertensive patients with presence of diabetes mellitus. Methods: We reviewed retrospectively the clinical records of 104 hypertensive patients for whom we also measured plasma renin activity (PRA) and plasma aldosterone concentrations (PAC). Results: Among 104 hypertensive patients, 44 had diabetes and 60 did not. There were no significant differences in clinical characteristics between non-diabetic and diabetic patients except for age and the number of antihypertensive agents. Patients with target organ damage were more common among diabetic patients. There was no correlation between PAC and the number of target organs damaged. In addition, Four patients from the non-diabetic and two from the diabetic group had a ratio over 30 for PRA/PAC and a PAC of over 15 ng/dL. Two non-diabetic patients and one diabetic patient were found, on abdomen CT, to have an adrenal adenoma. The rest of the patients refused further tests. Conclusion: The prevalence of primary aldosteronism in diabetic patients does not differ significantly from that in non-diabetic patients. Therefore, the present routine screening test for primary aldosteronism in hypertensive diabetic patients is not recommended. Background: Primary aldosteronism is the most common cause of secondary hypertension in humans. Its prevalence is estimated to be 10-15% among hypertensive patients. It is also associated with insulin resistance and diabetes mellitus. The aim of our study was to compare the prevalence of primary aldosteronism in hypertensive patients with presence of diabetes mellitus. Methods: We reviewed retrospectively the clinical records of 104 hypertensive patients for whom we also measured plasma renin activity (PRA) and plasma aldosterone concentrations (PAC). Results: Among 104 hypertensive patients, 44 had diabetes and 60 did not. There were no significant differences in clinical characteristics between non-diabetic and diabetic patients except for age and the number of antihypertensive agents. Patients with target organ damage were more common among diabetic patients. There was no correlation between PAC and the number of target organs damaged. In addition, Four patients from the non-diabetic and two from the diabetic group had a ratio over 30 for PRA/PAC and a PAC of over 15 ng/dL. Two non-diabetic patients and one diabetic patient were found, on abdomen CT, to have an adrenal adenoma. The rest of the patients refused further tests. Conclusion: The prevalence of primary aldosteronism in diabetic patients does not differ significantly from that in non-diabetic patients. Therefore, the present routine screening test for primary aldosteronism in hypertensive diabetic patients is not recommended.

      • KCI등재후보

        갑상선기능항진증이 동반된 비전형적인 McCune-Albright 증후군 1예

        장이선,강석휘,정웅룡,김우태,김혜수,이종민,문성대,차봉연,이광우,손호영,강성구 대한내분비학회 2006 Endocrinology and metabolism Vol.21 No.2

        McCune-Albright syndrome (MAS) is a sporadic disease that's characterized by polyostotic fibrous dysplasia, cafe-au-lait pigmentation of the skin, and multiple endocrinopathies, including sexual precocity, hyperthyroidism, acromegaly, and hypercortisolism. Recent evidence has shown that the clinical manifestations are caused by a postzygotic activating missense mutation in the gene coding for the α-subunit of Gs protein that stimulates c-AMP formation in the affected tissues. Substitution of the Arg201 residue in Gsα with cysteine or histidine have been identified in many MAS patients and Arg201 to Gly or Leu mutations have also been recently identified. We identified the Arg201 to His mutation in the gene encoding Gsα in the thyroid tissue from a 36-year-old man who was suffering with polyostotic fibrous dysplasia and hyperthyroidism. (J Kor Soc Endocrinol 21:158~164, 2006) McCune-Albright 증후군은 다발성 섬유성 골이형성증, 피부의 색소 침착, 내분비계 질환의 3대 증상을 특징으로 하는 드문 질환으로 G단백질의 활성화 변이가 병인으로 알려져있다. 저자들은 다발성 섬유성 골이형성증과 다발성 독성 결절성 갑상선 선종에 의한 갑상선기능항진증을 주증상으로 하는 비전형적인 McCune-Albright 증후군을 가진 36세 남자 환자에서 갑상선 조직을 이용한 유전자 분석으로 G 단백질의 201번째 염기서열이 CGT에서 CAT로 치환되어 arginine이 histidine으로 치환된 돌연변이를 증명하였기에 문헌고찰과 함께 보고하는 바이다.

      • KCI등재

        쉰소리로 발현한 흉부 대동맥류 1예:Ortner씨 증후군

        장이선,진승원,박희철,박진아,윤희정,이종민,김재형 대한내과학회 2003 대한내과학회지 Vol.65 No.1

        Ortner's syndrome is a clinical entity with hoarseness due to a left recurrent laryngeal nerve palsy caused by cardiac disease. It is a recognized complication of a number of conditions including, atrial septal defect, patent ductus arteriosus, primary pulmonary hypertension, Eisenmenger's syndrome and aortic aneurysm. A 71-year-old woman with a history of hypertension and diabetes complained hoarseness and hemoptysis for 3 days. Chest X-ray showed a soft tissue mass shadow in the left upper lung field and chest CT scan revealed a thoracic aortic aneurysm. We report a case of left recurrent laryngeal nerve palsy due to a huge thoracic aortic aneurysm in an old woman.(Korean J Med 64:105-108, 2003) Ortner씨 증후군은 다양한 심장 질환에 이차적으로 생긴 반회 후두 신경 마비에 의한 쉰소리를 특징으로 하 는 임상 질환이다. 저자들은 흉통 없이 발생한 쇤소리를 주소로 내원한 환자에서 좌측 반회 후두 신경의 손상에 따른 성대 마비의 1차적인 원인으로 대동맥궁 부위에서 의 대동맥류를 진단하였기에 문헌 고찰과 더불어 보고 하는 바이다.

      • KCI등재

        쉰소리로 발현한 흉부 대동맥류 1예 : Ortner씨 증후군 orther`s syndrome

        장이선,진승원,박희철,박진아,윤희정,이종민,김재형 대한내과학회 2003 대한내과학회지 Vol.64 No.1

        Ortner씨 증후군은 다양한 심장 질환에 이차적으로 생긴 반회 후두 신경 마비에 의한 쉰소리를 특징으로 하는 임상 질환이다. 저자들은 흉통없이 발생한 쇤소리를 주소로 내원한 환자에서 좌측 반회 후두 신경의 손상에 따른 성대 마비의 1차원적인 원인으로 대동맥궁 부위에서의 대동맥류를 진단하였기에 문헌 고찰과 더불어 보고하는 바이다. Ortner's syndrome is a clinical entity with hoarseness due to a left recurrent laryngeal nerve palsy caused by cardiac disease. It is a recognized complication of a number of conditions including, atrial septal defect, patent ductus arteriosus, primary pulmonary hypertension, Eisenmenger's syndrome and aortic aneurysm. A 71-year-old woman with a history of hypertension and diabetes complained hoarseness and hemoptysis for 3 days. Chest X-ray showed a soft tissue mass shadow in the left upper lung field and chest CT scan revealed a thoracic aortic aneurysm. We report a case of left recurrent laryngeal nerve palsy due to a huge thoracic aortic aneurysm in an old woman. (Korean J Med 64:105-108, 2003)

      • KCI등재후보

        악성장폐색 환자에서 Octreotide의 치료 효과

        박지찬,장이선,전은경,김동규,이욱현,이국진,류시영,최현호,박석영,Park, Ji-Chan,Jang, Yi-Sun,Jeon, Eun-Kyoung,Kim, Dong-Kyu,Lee, Wook-Hyun,Lee, Guk-Jin,You, Si-Young,Choi, Hyun-Ho,Park, Suk-Young 한국호스피스완화의료학회 2009 한국호스피스.완화의료학회지 Vol.12 No.4

        목적: 진행성 암 환자에서 악성장폐색은 구역, 구토, 통증을 비롯한 소화기계 증상을 유발하여 삶의 질을 저하시킨다. 악성장폐색의 증상 조절을 위해 octreotide를 투여한 후의 효능에 대한 보고들이 발표되었고, 저자들은 악성장폐색이 있는 환자에게 보존적 치료에 octreotide를 추가하여 투여시의 효과와 안전성을 알아보고자 하였다. 방법: 의무기록을 통하여 일반적인 치료로 호전이 없어 octreotide를 추가한 악성장폐색의 환자 29명에서 octretide를 0.1 mg을 시작으로 증상 조절될 때까지 증량하여, 약 투여 전후의 통증의 변화, 구토 횟수의 변화, 비위관 삽입환자의 경우 배액량의 변화를 조사하였다. 결과: Octreotide 투여량의 중간 값은 0.2 mg이고 0.1~0.6 mg의 범위이며, 약제 투여시점으로부터 사망까지 2일에서 103일의 범위로 중간 값은 20일이었다. 약제 투여 전의 VAS는 평균 5.6$\pm$1.2이고, 약 투여 후 VAS의 평균은 2.7$\pm$1.0이었으며 통계적으로 유의한 감소를 보였다(P<0.05). 약제 투여 전의 구토 횟수는 평균 3.6회/일$\pm$2.5이었고 약 투여 후에는 0.4회/일$\pm$0.8로 감소되었고, 통계적으로 유의한 감소를 보였다(P<0.05). 약제 투여전에 비위관 삽입 환자는 평균 975$\pm$1,083 cc/일의 배액이 확인되었고, 약 투여 후에는 평균 115$\pm$196 cc/일로 유의하게 감소하였다(P<0.05). 결론: 일반적인 약물치료에 반응이 없는 악성장폐색환자에서 octretide의 추가 투여는 효과적이며 안전하였다. 악성장폐색 환자의 증상조절을 위해서 octreotide의 추가 투여를 적극적으로 고려해야 하겠다. Department of Internal Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea Purpose: Malignant bowel obstruction causes gastrointestinal symptoms and leads to diminished quality of life in patients with advanced cancer. Several studies have shown the efficacy of octreotide for the relief of malignant bowel obstruction-related symptoms. The aim of this study is to assess the efficacy and safety of octreotide in patients with malignant bowel obstruction. Methods: We retrospectively reviewed medical records of twenty nine patients who had suffered from malignant bowel obstruction without clinical improvement of conservative care and subsequently, received octreotide treatment. Initial dosage of octreotide was 0.1 mg/day, and dose was escalated depending on the clinical effect. For each patient, we assessed visual analogue scale (VAS) of pain, number of vomiting episode, and amount of nasogastric tube drainage. Results: Median dosage of octreotide was 0.2 mg/day (range 0.1~0.6), and median duration from initial medication to death was 20 days (range 2~103). VAS before and after octreotide treatment were 5.6$\pm$1.24, and 2.7$\pm$0.96, respectively. The numbers of vomiting episode before and after octreotide treatment were 3.6/day$\pm$2.5, and 0.4/day$\pm$0.8, respectively. The mean amounts of nasogastric tube drainage before and after octreotide treatment were 975$\pm$1,083 cc/day and 115$\pm$196 cc/day, respectively. Statistically significant reduction in VAS, the number of vomiting episode and the amount of nasogastric tube drainage were observed after octreotide treatment (P<0.05). Conclusion: Administration of octreotide in patients with malignant bowel obstruction, which is uncontrolled by other medication, was effective and safe. In such clinical situations, physicians should consider to add of octreotide for symptomatic control.

      • KCI등재후보

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