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        당뇨병성 경화부종 - 임상 - 병리조직학적 연구 및 문헌고찰 -

        황호근(Ho Geun Hwang),이승하(Seoung Ha Lee),형근영(Keun Young Hyoung),신봉주(Bong Joo Shin),김경년(Kyoung Nyeon Kim),조정구(Chung Gu Cho),김주섭(Ju Seob Kim),차승훈(Seung Hoon Cha),박석돈(Seok Don Park) 대한내과학회 1996 대한내과학회지 Vol.51 No.4

        N/A Objectives: We studied clinical feature, underlying disease and histo-pathologic finding of scleredema diabeticorum that was observed as non-pitting indurated edematous skin of the nape, occiput in non-insulin dependent diabetes mellitus. Methods: We performed clinico-histopathologic study in 5 cases of scleredema diabeticorum which were diagnosed at our hospital and reviewed 6 cases in Korean literatures. Results: 1) The male to female ratio was 4:1, The overweight or obese middle-aged (mean:54.6) males were prone to develop these cutaneous changes. There was no relationship between the development of diabetic scleredema and the duration, the severity of diabetes. Hypertension and hyperlipidemia were frequent associated finding among the patients. 2) The increased level of fasting C-peptide implicated that the ability of insulin secretion was preserved. 3) Non-pitting hardening of the skin on the nape, head and surrounding parts of the body was common skin change in all patients. No evidence of systemic involvements was found. 4) Typical histopathologic findings, consisted of the thickening of dermis, the degeneration of collagen bundles in mid- to deep dermis, and the deposition of acid mucopoleysaccharides, hyaluronic acid, were noted in 4 cases of our study. 5) The therapeutic responses of cutaneous changes were generally poor. Conclusion: These results implies that scleredema diabeticorum is not a rare cutaneous manifestation among the diabetic dermadromes, and special histopathologic stainings with skin tissue sections are necessary for diagnosis. Although the exact mechanisms of this disease are not known, and no effective therapy is available, it needs to be studied exactly that the facts of hyperlipidemia, overweight, hypertension and well-preservation of fasting C-peptide observed in our cases, may be related to syndrome-X.

      • 건 황색종의 가족력과 관상동맥질환을 갖는 이형집합 가족성 고 콜리스테롤혈증 1례

        백승훈,형근영,김경년,조정구,이경근 圓光大學校 醫科學硏究所 1997 圓光醫科學 Vol.13 No.1-2

        Familial hypercholesterolemia is a common autosomal dominant disorder with serious health consequences such as the coronary heart disease, in western area affecting approximately 1 in 500 persons in the heterozygous form, is caused by a mutation in the gene for the LDL(low density lipoprotein) receptor. A-45-years old male came to the hospital because of tendon xanthomas on both elbows, knees, and ankles. He also has retrosternal chest pain intermittently since 5 years ago. 4 kindreds of the patient have tendon xanthomas according to autosomal dominant inheritance. Serum levels of total cholesterol and triglyceride were 359 ㎎/dL, 103 ㎎/dL respectively. Lipoprotein electrophoresis showed type Ⅱa pattern. Treadmill exercise test revealed angina pectoris. The case of familial hypercholesterolemia who has familial inheritance of tendon xanthoma is rarely reported in Korea. We report this case with a review of the related literature

      • 부신피질호르몬과 성장호르몬의 증가를 동반한 제2형 다발성 내분비선종(MEN Ⅱa) 1례

        구기선,이재홍,유경훈,백승훈,형근영,조정구,이경근 圓光大學校 醫科學硏究所 1997 圓光醫科學 Vol.13 No.1-2

        Multiple endocrine neoplasia type 2a(MEN 2a) is autosomal dominant disorders arising from mutations on chromosome 10q11.2 in the region of the RET proto-oncogenes which encodes a receptor tyrosine kinase. The features of MEN 2a include pheochromocytoma (in about 40% of gene carriers), medullary thyroid carcinoma, primary hyperparathyroidism (adenoma or hyperplasia). Carriers of MEN 2a genes can best be identified by serial measurement of stimulated blood calcitonin levels. Recent advances in mapping the MEN 2a gene now allows direct genetic testing, replacing calcitonin testing as a screening tool. We experienced a case of MEN 2a in a 35-year-old female patient. She underwent total thyroidectomy nine years ago due to medullary thyroid carcinoma. The pheochromocytoma, which was detected by biochemical tests, CT scan and ^131I-MIBG scan was successfully removed by bilateral adrenalectomy with preoperative alpha and beta adrenergic blockades. Her blood pressure and blood sugar level became normal after operation.

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