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논문분류 춘계학술대회 초록집
제목 Clinicopathological Characteristics of Korean Focal Segmental Glomerulosclerosis Patients
저자 Beom Jin Lim
출판정보 2016; 2016(1):
키워드
초록 Focal segmental glomerulosclerosis (FSGS) is one of the most common glomerular diseases presenting with nephrotic range proteinuria. Primary FSGS is related with podocyte injury and secondary FSGS develops due to previous medical conditions. FSGS is classified as collapsing, tip, cellular, perihilar and not otherwise specified (NOS) variants according to the character of sclerotic lesion. The frequency of these variants and their clinical significance in Korean FSGS patients have not been well studied due to the lack of nation-wide data. In a study on Korean adult FSGS patients, the frequency of NOS, tip, perihilar, cellular and collapsing variants was 63.1%, 18.0%, 15.3%, 2.7% and 0.9%, respectively. The low frequency of cellular and collapsing variants in adult patients is in accordance with other Asian studies, and may partly be associated with the low incidence of HIV-associated nephropathy in Asian countries. Clinical feature of these variants was similar to that of Western countries, showing more frequent complete remission after therapy in tip variant. For elementary school students, mandatory urine mass screening test has been performed since 1998. 4.4% of urinary abnormality detected by this test was related with FSGS. The frequency of histologic variant in childhood FSGS was 72.7% (NOS), 6.1% (tip), 9.1% (perihilar), 1.5% (cellular), and 10.6% (collapsinig). Interestingly, no significant difference in prognosis was observed in these variants. Studies on genetic alteration associated with congenital and infantile FSGS in Korean patients showed that WT1 and NPHS1 mutations were the most frequent alterations while NPHS2 mutations were rare in contrast to the results of Western studies.
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