Many patients with Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome(TTP-HUS) satisfactorily respond to plasma exchange. Some patients, however, respond either not at all or only transiently and incompletely. In the refractory case, endothelial cell-derived unusually large von Willebrand factor multimers(ULvWFM) have an important role in the formation of microthrombi. As the ULvWFM may be removed in the cryoprecipitate, we reason the plasma depleted of cryoprecipitate(the plasma cryosupernatant) should be considered for effectiveness in the treatment of refractory TTP- HUS. We experienced a 48 year old woman presented with diarrhea, jaundice and oliguria. She had microangiopathic hemolytic anemia, renal impairment, platelets of 21,000/mm3 and LDH 3,258U/L. She had not improved after plasma exchange with fresh frozen plasma(FFP)(1.5 plasma volumeX7 days). On hospital day 8, her HUS had not responded, platelets of 37,000/mm3 and LDH 1,588U/L. Substitution of cryosupernatant for FFP was associated with prompt increased in the platelet count to normal and complete resolution of HUS. Therefore. the cryosupernatant fraction of plasma should be considered as an alternative to whole FFP for plasma exchange if there is continuing platelet consumption and microvascular thrombosis in spite of intensive conventional plasma therapy.
Anemia, Hemolytic ; Blood Platelets ; Diarrhea ; Female ; Hemolytic-Uremic Syndrome* ; Humans ; Jaundice ; Middle Aged ; Oliguria ; Plasma Exchange* ; Plasma* ; Platelet Count ; Thrombosis ; von Willebrand Factor

STUDY DESIGN: Retrospective study. PURPOSE: To assess the feasibility and limitations of fracture risk assessment tool (FRAX) for osteoporotic vertebral fractures in the Korean population. OVERVIEW OF LITERATURE: The FRAX algorithm is country specific and uses clinical risk factor data to calculate an individual patient's 10-year probability of hip fracture and 10-year probability of major osteoporotic fracture. However, it has not been adequately investigated for Korean. METHODS: One hundred ninety four patients who had all risk factor data for the calculation of FRAX were divided into two groups depending on the existence of vertebral fractures: the fracture group was comprised of 88 patients and the non-facture group comprised of 105 patients. We analyzed prediction of the fracture by applying respectively the Korean, Japanese, USA and UK model, and compared their FRAX results by calculating lumbar bone mineral density (BMD) instead of femoral neck BMD. RESULTS: The prediction of vertebral fracture using FRAX was 10.9 +/- 6.2% in the fracture group, 9.5 +/- 5.5% of the non-fracture group in the Korean model (p = 0.108); 17.9 +/- 10.2% in the fracture group, 14.6 +/- 9.0% in the non-fracture group in the Japanese model (p = 0.017). Only the Japanese model exhibited significant difference in vertebral fracture risk. The prediction of vertebral fracture using lumbar BMD instead of femoral neck BMD was 19.5 +/- 12.1% in the fracture group, 16.0 +/- 10.3% in the non-fracture group in the Korean model (p = 0.029). All models had statistically significant differences for the prediction of osteoporotic vertebral fracture. CONCLUSIONS: The 10-year probability of osteoporotic vertebral fracture had underestimation of the risk considering treatment eligibility based on the National Osteoporosis Foundation guidelines. BMD that accurately reflects the contribution of each result to fracture risk should be preferred for the prediction of fracture using FRAX, when lumbar spine and hip BMD measurements are both performed for clinical purposes in Korean.
Asian Continental Ancestry Group ; Bone Density ; Femur Neck ; Hip ; Humans ; Korea ; Osteoporosis ; Osteoporotic Fractures ; Retrospective Studies ; Risk Assessment ; Risk Factors ; Spine

Syringocystadenocarcinoma papilliferum in situ is extremely rare. A 51-year-old female presented with a solitary yellowish patch on the scalp, accompanied by hair loss and a protruding mass. Histopathological evaluation revealed a large number of sebaceous glands and mild papillomatosis without hair follicles. The tumor contained many cystic spaces filled with amorphous material and a connection to the epidermis. The tumor cells showed papillomatous projections and were multilayered with nuclear atypia but no dermal involvement or lymphovascular invasion. On immunohistochemical analysis, the tumor cells showed immunopositivity for carcinoembryonic antigen, Ki-67, epithelial membrane antigen, and gross cystic disease fluid protein-15. Therefore, the patient was diagnosed with syringocystadenocarcinoma papilliferum in situ originating from a nevus sebaceus. The tumor was removed by wide excision, and no recurrence was observed. Our case report supports the multistep pathogenesis of syringocystadenocarcinoma papilliferum originating from a nevus sebaceus.

Syringocystadenocarcinoma papilliferum in situ is extremely rare. A 51-year-old female presented with a solitary yellowish patch on the scalp, accompanied by hair loss and a protruding mass. Histopathological evaluation revealed a large number of sebaceous glands and mild papillomatosis without hair follicles. The tumor contained many cystic spaces filled with amorphous material and a connection to the epidermis. The tumor cells showed papillomatous projections and were multilayered with nuclear atypia but no dermal involvement or lymphovascular invasion. On immunohistochemical analysis, the tumor cells showed immunopositivity for carcinoembryonic antigen, Ki-67, epithelial membrane antigen, and gross cystic disease fluid protein-15. Therefore, the patient was diagnosed with syringocystadenocarcinoma papilliferum in situ originating from a nevus sebaceus. The tumor was removed by wide excision, and no recurrence was observed. Our case report supports the multistep pathogenesis of syringocystadenocarcinoma papilliferum originating from a nevus sebaceus.

PURPOSE: Human metapneumovirus(hMPV) is a respiratory viral pathogen that causes a wide spectrum of illnesses, ranging from asymptomatic infection to severe bronchiolitis. The virus has been identified world widely, but so far it has not been published in Korea. METHODS: We obtained clinical samples by nasopharyngeal aspiration from 218 children hospitalized due to acute lower respiratory tract infections at Soonchunhyang University Hospital in Cheonan from October, 2004 to April, 2005. We designed specific primers from conserved region of fusion glycoprotein of hMPV. Total RNA was extracted and RT-PCR was performed, and single specific 423 bp product was obtained. The PCR product was confirmed to be fusion glycoprotein RNA by sequencing. RESULTS: We detected hMPV in 15(6.9 percent) of the 218 hospitalized children. The infected children comprised nine boys and six girls; their mean age was 2.8 years(5 mo-12 yrs) and they were diagnosed with pneumonia(60 percent), bronchiolitis(33.3 percent), croup(6.6 percent). The number of cases of detected hMPV in Korea increased dramatically during the period from March to May 2005. CONCLUSION: hMPV is circulating in Korean children and is associated with respiratory tract infection. Additional studies are required to define the epidemiology and the extent of diseases in the general population caused by hMPV.
Asymptomatic Infections ; Bronchiolitis ; Child* ; Child, Hospitalized ; Chungcheongnam-do ; Epidemiology ; Female ; Glycoproteins ; Humans* ; Korea ; Metapneumovirus* ; Polymerase Chain Reaction ; Respiratory Tract Infections ; RNA