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말단비대증 환자에서 소마토스타틴 유사체 사용에 대한 권고안
진상욱 ( Sang Ouk Chin ),구철룡 ( Cheol Ryong Ku ),김병준 ( Byung Joon Kim ),김성운 ( Sung-woon Kim ),박경혜 ( Kyeong Hye Park ),송기호 ( Kee Ho Song ),오승준 ( Seungjoon Oh ),윤현구 ( Hyun Koo Yoon ),이은직 ( Eun Jig Lee ),이정민 대한내과학회 2019 대한내과학회지 Vol.94 No.6
Acromegaly is a chronic disorder caused by excessive growth hormone (GH) secretion. In most cases, the excess GH originates from GH-producing pituitary adenomas. Surgery is the preferred first-line treatment for patients with acromegaly, but medical man-agement is considered when the disease persists after surgery or in cases where patients refuse surgery or are poor candidates for surgery. Somatostatin analogues are commonly used to treat acromegaly. The Korean Endocrine Society and the Korean Neuroendocrine Study Group have developed a position statement for the use of somatostatin analogues in the medical treatment of acromegaly. This position statement is based on evidence from the current literature and expert opinions. In the case of discrep-ancies among expert opinions, the experts voted to determine the recommended approach. (Korean J Med 2019;94:485-494)
Btk 유전자 인트론 2 돌연변이에 의한 X연관 무감마글로불린혈증
정문재 ( Moon Jae Chung ),정지예 ( Ji Ye Jung ),손지영 ( Ji Young Son ),구철룡 ( Cheol Ryong Ku ),박병훈 ( Byung Hoon Park ),변민광 ( Min Kwang Byun ),문지애 ( Ji Ae Moon ),김영삼 ( Young Sam Kim ),김세규 ( Se Kyu Kim ),장준 ( Jo 대한결핵 및 호흡기학회 2008 Tuberculosis and Respiratory Diseases Vol.65 No.3
X-linked agammaglobulinemia is the most common type of primary immunodeficiency disorder. Mutation ofthe cytoplasmic tyrosine kinase gene, Btk (Bruton`s tyrosine kinase), is known to be the etiology of X-linked agammaglobulinemia. The patients with this disease manifest a B-cell deficiency and low levels of serum immunoglobulin; due to the deficient antibodies, they suffers from recurrent upper and lower respiratory infections. We report here a 24-year-old male with an initial clinical impression of recurrent pneumonia and bronchiectasis. The patient presented with marked pan-hypogammaglobulinemia and the absence of circulating B-lymphocytes on the immunologic study, and he carried a splicing mutation of intron 2 in the Btk gene (IVS2 -3C>G). (Tuberc Respir Dis 2008;65:207-211)
만성신부전증에서 진단된 일측 신동맥 협착 및 반대측 신장의 국소분절사구체경화증
홍성진 ( Sung Jin Hong ),김동기 ( Dong Ki Kim ),구철룡 ( Cheol Ryong Ku ),이한성 ( Han Sung Lee ),최규헌 ( Kyu Hun Choi ),이호영 ( Ho Yung Lee ),한대석 ( Dae Suk Han ),정현주 ( Hyeon Joo Jeong ),김범석 ( Beom Seok Kim ) 대한신장학회 2007 Kidney Research and Clinical Practice Vol.26 No.2
We report a case of focal segmental glomerulosclerosis (FSGS) with chronic renal failure in a patient with unilateral renal artery stenosis. A 38-year-old woman presented with malignant hypertension, massive proteinuria and renal insufficiency. MR angiography showed proximal stenosis of the right renal artery and biopsy of the left kidney revealed focal segmental glomerulosclerosis. The patient responded favorably to the angiotensin Ⅱ receptor antagonist, as the blood pressure and urinary protein decreased. This case showed that FSGS is a pathophysiologic mechanism of renal injury in uncontrolled renovascular hypertension.