Prognostic utility of ADAMTS13 activity for the atypical hemolytic uremic syndrome (aHUS) and comparison of complement serology between aHUS and thrombotic thrombocytopenic purpura

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) involves dysregulation of the complement system, but whether this also occurs in thrombotic thrombocytopenic purpura (TTP) remains unclear. Although these conditions are difficult to differentiate clinically, TTP can be distinguished by low (<10%) ADAMTS13 activity. The aim was to identify the differences in complement activation products between TTP and aHUS and investigate ADAMTS13 activity as a prognostic factor in aHUS. METHODS: We analyzed patients with thrombotic microangiopathy diagnosed as TTP (N=48) or aHUS (N=50), selected from a Korean registry (N=551). Complement activation products in the plasma samples collected from the patients prior to treatment and in 40 healthy controls were measured by ELISA. RESULTS: The levels of generalized (C3a), alternate (factor Bb), and terminal (C5a and C5b-9) markers were significantly higher (all P<0.01) in the patients than in the healthy controls. Only the factor Bb levels significantly differed (P=0.008) between the two disease groups. In aHUS patients, high normal ADAMTS13 activity (≥77%) was associated with improved treatment response (OR, 6.769; 95% CI, 1.605–28.542; P=0.005), remission (OR, 6.000; 95% CI, 1.693–21.262; P=0.004), exacerbation (OR, 0.242; 95% CI, 0.064–0.916; P=0.031), and disease-associated mortality rates (OR, 0.155; 95% CI, 0.029–0.813; P=0.017). CONCLUSION: These data suggest that complement biomarkers, except factor Bb, are similarly activated in TTP and aHUS patients, and ADAMTS13 activity can predict the treatment response and outcome in aHUS patients.

Frequency of Pro475Ser Polymorphism of ADAMTS13 Gene and Its Association with ADAMTS-13 Activity in the Korean Population

PURPOSE: The in vitro study suggested that proline to serine polymorphism in codon 475 (C1423T) of the A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats-13 (ADAMTS-13) gene is related to reduced activity of ADAMTS- 13. In this study, the frequency of the Pro475Ser polymorphism in Koreans was studied and plasma ADAMTS-13 activity was measured to find out whether this polymorphism contributes to decreased ADAMTS-13 activity in Koreans. PATIENTS AND METHODS: The frequency of the C1423T allele of the ADAMTS13 gene was studied along with measuring plasma ADAMTS-13 activity in 250 healthy Korean individuals. RESULTS: The allele frequency of C1423T polymorphism was 4%, and the median activity of CT type was 107 (69-143)%, which was lower than in controls with the CC genotype [118 (48-197)%, (p=0.021)]. CONCLUSION: Therefore, the Pro475Ser polymorphism seems to be popular in the Korean population, and attenuates ADAMTS-13 plasma activity.

Performance of Interventional Procedures in a Day-hospital System

PURPOSE: We wanted to describe the practice and results of applying the day-hospital system in an interventional radiology clinic. MATERIALS AND METHODS: From Oct. 2004 to Dec. 2005, the day-hospital system was applied to various interventional procedures with using a part of the recovery room of an angiography suite as a facility for hospital admission. The study included 91 cases in 73 patients. The source of the patient referral, the procedures, hospital courses and complications were analyzed and questionnaire surveys were conducted for the available 55 patients. RESULTS: Among the patients, 70% (n=64) were referred from other departments, 5% (n=5) from other hospitals, 5% (n=4) were new patients and 20% (n=18) were re-admissions. The procedures included gastrointestinal, biliary, urinary, hemodialysis related- and implantable port related interventions. 96% (n=87) of the patients were successfully discharged in a day and admission to the general ward was only 4% (n=4). Minor complications occurred after discharges in 3% (n=3). The questionnaire survey revealed that 96% (n=53) of the patients were satisfied with the service and they were not anxious after discharge. CONCLUSION: Most of common interventional procedures were safely done under the day-hospital system with the patients being highly satisfied. The day-hospital system can be a good tool for establishing admitting privileges for an interventional radiology clinic.

Performance of Interventional Procedures in a Day-hospital System

PURPOSE: We wanted to describe the practice and results of applying the day-hospital system in an interventional radiology clinic. MATERIALS AND METHODS: From Oct. 2004 to Dec. 2005, the day-hospital system was applied to various interventional procedures with using a part of the recovery room of an angiography suite as a facility for hospital admission. The study included 91 cases in 73 patients. The source of the patient referral, the procedures, hospital courses and complications were analyzed and questionnaire surveys were conducted for the available 55 patients. RESULTS: Among the patients, 70% (n=64) were referred from other departments, 5% (n=5) from other hospitals, 5% (n=4) were new patients and 20% (n=18) were re-admissions. The procedures included gastrointestinal, biliary, urinary, hemodialysis related- and implantable port related interventions. 96% (n=87) of the patients were successfully discharged in a day and admission to the general ward was only 4% (n=4). Minor complications occurred after discharges in 3% (n=3). The questionnaire survey revealed that 96% (n=53) of the patients were satisfied with the service and they were not anxious after discharge. CONCLUSION: Most of common interventional procedures were safely done under the day-hospital system with the patients being highly satisfied. The day-hospital system can be a good tool for establishing admitting privileges for an interventional radiology clinic.

Short-term follow up of thyroid function after pediatric hematopoietic stem cell transplantation / 소아과

PURPOSE: In this study, we analyzed the short term changes of thyroid function, incidence and risk factors of thyroid dysfunction soon after allogeneic hematopoietic stem cell transplantation (HSCT) in children. METHODS: We enrolled 80 pediatric patients following allogeneic HSCT, at the Catholic HSCT center between January, 2004 and February, 2006. Serum TSH (thyroid stimulating hormone), total serum thyroxine and total serum triiodothyronine levels were systematically measured in 80 patients before the HSCT, and at 1 month, 6 months and 12 months after HSCT. RESULTS: Thyroid function statistically decreased at 1 month after HSCT(P or = II) were risk factors for ETS (P=0.04, 0.01 respectively). In multivariate analysis, we could not detect an independent risk factor for ETS (P=0.19, 0.06 respectively). CONCLUSION: The present study suggests that the incidence of thyroid dysfunction is high after allogeneic HSCT. Therefore, regular monitoring of thyroid hormone levels after HSCT is required.

Usefulness of the IMI Channel of Sysmex SE-9000(TM) Automated Hematology Analyzer Predicting the Optimal Timing of Peripheral Blood Stem Cell Harvest / 임상검사와정도관리

BACKGROUND: An essential prerequisite for successful procurement of sufficient peripheral blood stem cells (PBSC) for engraftment is the optimal timing of collection. The Sysmex SE-9000 automated hematology analyzer provides the immature information (IMI) channel for the identification and counting PBSC. In this study, The optimal timing of PBSC collection was studied using IMI channel. METHODS: 193 peripheral blood stem cell collections were performed from 52 patients with hematologic disorders or solid tumors and 15 donors. Pre-harvest peripheral blood WBC, mononuclear cells (MNC) and IMI were tested and compared with CD34+ cell count and CFU-GM count of harvested products. RESULTS: Peripheral blood WBC and MNC count showed a weak correlation with CD34+ cell yield (r=0.38, P1x10(6)/kg with sensitivity of 88.7%. Positive and negative predictive values of IMI >465/microliter for CD34+ cell >1x10(6)/kg were 65.5% and 87.5%, respectively. CONCLUSIONS: The automated IMI might be used as a simple and efficient indicator of PBSC mobilization and applying variable cutoff values of IMI would be a useful tool to predict the optimal timing of PBSC collection.