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Clinical Features of Polyarteritis Nodosa in Korea
Bae, Young Deok,Choi, Hyo Jin,Lee, Jung Chan,Park, Jeong Jin,Lee, Yun Jong,Lee, Eun Bong,Song, Yeong Wook The Korean Academy of Medical Sciences 2006 JOURNAL OF KOREAN MEDICAL SCIENCE Vol.21 No.4
<P>Polyarteritis nodosa (PAN) is a systemic vasculitis characterized by multi-organ involvement with protean manifestations. We evaluated the clinical features of PAN in Korea. Twenty-seven patients were diagnosed as PAN at Seoul National University Hospital between January 1990 and July 2003. The male-to-female ratio was 1.7:1 and mean age at onset (±SD) was 47.4±20 yr. Their presenting features at diagnosis were similar to those reported previously, i.e., myalgia, muscle weakness or leg tenderness (70%), fever (52%), weight loss >4 kg (44%), skin rash (44%), peripheral edema (33%), abdominal pain (33%), and arthralgia/arthritis (30%). However, the prevalence of testicular pain or tenderness was higher (24%) than reported previously and only three (11.5%) had HBsAg positivity without liver enzyme elevation. Nine patients (33%) had a five-factor score (FFS) of 2. Fourteen patients (52%) responded to treatment, 2 patients relapsed and 4 died within 1 yr of diagnosis. During a median follow-up of 55.5 months, three of the four PAN-related deaths had an initial FFS of 2. The clinical features of PAN were not significantly different from those reported previously. However, testicular pain or tenderness was more frequent and patients with a high FFS tended to have a poorer prognosis.</P>
최효진 ( Hyo Jin Choi ),신기철 ( Ki Chul Shin ),배영덕 ( Young Deok Bae ),이정찬 ( Jung Chan Lee ),김진현 ( Jin Hyun Kim ),강은하 ( Eun Ha Kang ),임철현 ( Churl Hyun Im ),이은봉 ( Eun Bong Lee ),송영욱 ( Yeong Wook Song ) 대한류마티스학회 2004 대한류마티스학회지 Vol.11 No.1
1. Protein-losing enteropathy is a rare complication of autoimmune diseases. We report a case of primary Sjogren`s syndrome with protein-losing enteropathy in 50-year-old female who complained of generalized edema. Protein-losing enteropathy of the small intestine was demonstrated by Tc-99m labeled albumin abdominal scintigraphy. Duodenal biopsies showed chronic inflammatory cell infiltration without lymphangiectasis or vasculitis. The patient received oral prednisolone (60 mg/day) for 4 weeks with subsequent clinical improvement. Primary Sjogren`s syndrome or other autoimmune diseases should be considered in cases of protein-losing enteropathy.