Journal of Korean medical science
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J. Korean Med. Sci. · Feb 2014
Impact of enzyme replacement therapy on linear growth in Korean patients with mucopolysaccharidosis type II (Hunter syndrome).
Hunter syndrome (or mucopolysaccharidosis type II [MPS II]) arises because of a deficiency in the lysosomal enzyme iduronate-2-sulfatase. Short stature is a prominent and consistent feature in MPS II. Enzyme replacement therapy (ERT) with idursulfase (Elaprase®) or idursulfase beta (Hunterase®) have been developed for these patients. ⋯ ERT may have less effect on the growth of patients with the severe form of Hunter syndrome. The height z-scores in groups 2 and 3 revealed a significant change (the estimated slopes before and after the treatment were -0.047 and -0.007, respectively: difference in the slope, 0.04; P<0.001). Growth in response to ERT could be an important treatment outcome or an endpoint for future studies.
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J. Korean Med. Sci. · Feb 2014
Case ReportsA case of nonisland pedicled foot fillet flap for below-knee amputation stump wound: treatment option for compartment syndrome after fibular free flap surgery.
Despite the frequent use of the fibular free flap, there have been no reports of severe compartment syndrome of the donor leg that necessitated limb amputation. A 66-yr-old man had a fibular osseous free flap transfer from the left leg to the mandible that was complicated by postoperative compartment syndrome. ⋯ Various foot fillet flaps may be used acutely as a free or an island pedicled flap, but dissection of the vascular pedicle may be difficult in a chronically inflamed wound because of inflammation and adhesions to surrounding tissue. The nonisland pedicled foot fillet flap may be considered as a useful option for treatment of a chronically inflamed stump wound after below-knee amputation.
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J. Korean Med. Sci. · Feb 2014
Case ReportsSuccessful pregnancy in a patient with autosomal dominant polycystic kidney disease on long-term hemodialysis.
Recent advances in dialysis and a multidisciplinary approach to pregnant patients with advanced chronic kidney disease provide a better outcome. A 38-yr-old female with autosomal dominant polycystic kidney disease (ADPKD) became pregnant. ⋯ By means of intensive HD and optimal management of anemia, pregnancy was successfully maintained until vaginal delivery at 34.5 weeks of gestation. We discuss the special considerations involved in managing our patient with regard to the underlying ADPKD and its influence on pregnancy.
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J. Korean Med. Sci. · Feb 2014
Decreasing incidence of Trichophyton mentagrophytes in Korea: analysis of 6,250 cases during the last 21-year-period (1992-2012).
Trichophyton mentagrophytes is the second common dermatophyte in Korea. However, few reports have been issued on the epidemiological and mycological characteristics of T. mentagrophytes in Korea based on long-term, large-scale study. The purpose of this study was to elucidate the epidemiological and mycological characteristics of T. mentagrophytes in Korea. ⋯ Mycological analysis showed a powdery colony appearance was the most common, followed by persicolor and granular colonies. Toewebs were most frequently involved. This investigation on T. mentagrophytes provides insights into its incidence and characteristics.
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J. Korean Med. Sci. · Feb 2014
Differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schönlein purpura.
We aimed to investigate differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schönlein purpura (HSP), and to analyze the factors associated with poor prognosis for HSP nephritis. This retrospective 10-yr study enrolled 160 patients with HSP who visited Severance Hospital. Purpura was mostly detected in lower extremities, but purpura in upper extremities was more frequently observed in adults than children (41.7% vs 19.3%). ⋯ Our results showed several differences in the clinical features of HSP between adults and children. Adults with HSP had a higher frequency of renal insufficiency and worse renal outcomes than children. Renal insufficiency at diagnosis might be of predictive value for the progression to chronic renal failure in HSP patients.