BACKGROUND: Therapeutic plasma exchange (TPE) for desensitization in ABO incompatible kidney transplantation (KT) has raised concerns regarding efficiency and safety. The purpose of this study was to determine the number of TPE prior to KT required to reach target titer for KT according to ABO blood groups. METHODS: The distribution of ABO antibody (Ab) titer of 117 patients was investigated. The relationship between initial ABO Ab and number of TPEs required to reach target titer to ≤1:8 prior to KT was evaluated retrospectively according to blood groups and ABO Ab classes. RESULTS: The initial IgG ABO Ab titers were the highest in blood O group recipients, and the average number±standard deviations (range) of TPEs performed prior to ABO incompatible KT was 3.0±1.1 (0~5) in blood group A, 3.7±1.5 (0~8) in blood group B, and 5.3±1.9 (2~13) in blood group O, respectively. The best correlation was observed in the linear relationship between initial ABO Ab titer and number of TPEs required (y=0.6829x+0.0523, R2=0.946, x=log2 initial ABO Ab titer, y=number of TPE required), regardless of the specific ABO blood group. CONCLUSION: The number of TPEs can be highly deduced from initial ABO Ab titer and our developed equation in desensitization programs would help increase the efficiency of TPE and patient safety.
Blood Group Antigens ; Blood Group Incompatibility ; Humans ; Immunoglobulin G ; Kidney Transplantation* ; Kidney* ; Patient Safety ; Plasma Exchange* ; Plasma* ; Retrospective Studies

BACKGROUND: Grafts survive despite blood group antigens on the transplant being continuously exposed to antibodies in the blood of recipients in ABO-incompatible kidney transplantation (ABOi KT), owing to the mechanism of accommodation. We analyzed the immunodynamics of soluble ABH antigens in allografts from secretor donors and the influence of such immunodynamics on accommodation and subsequent graft survival in ABOi KT. METHODS: The genotype of a known human β-galactoside α-1,2-fucosyltransferase gene (FUT2), which determines soluble ABH antigen secretor status, was established in 32 donors for ABOi KT at the Severance Hospital, from June 2010 to July 2015. Clinical outcomes of recipients, such as anti-A/B antibody titer change, renal function, and graft survival, were evaluated. RESULTS: Twenty-five donors were secretors (78.1%), and seven were nonsecretors (21.9%). The frequency of anti-A/B IgG or IgM antibody titer elevation or reduction post-transplantation was not significantly related to donor secretor status. However, IgM titer was rapidly reduced in recipients transplanted from nonsecretor donors (P=0.01), which could be explained by the lack of absorption effect of soluble antigens, enhancing the binding of antibodies to antigens in the allografts. Interestingly, soluble ABH antigens did not affect rejection-free graft survival, which may be due to the nature of β-galactoside α-1,2-fucosyltransferase. CONCLUSIONS: Soluble ABH antigens produced by transplanted kidneys from secretor donors played a role in inducing accommodation within three months of KT through neutralization; however, major graft outcomes were not affected.
Absorption ; Allografts ; Antibodies ; Blood Group Antigens ; Blood Group Incompatibility ; Genotype ; Graft Survival ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Kidney Transplantation ; Kidney* ; Tissue Donors* ; Transplants

PURPOSE: The aim of the present study was to investigate communication function using classification systems and its association with other functional profiles, including gross motor function, manual ability, intellectual functioning, and brain magnetic resonance imaging (MRI) characteristics in children with cerebral palsy (CP). MATERIALS AND METHODS: This study recruited 117 individuals with CP aged from 4 to 16 years. The Communication Function Classification System (CFCS), Viking Speech Scale (VSS), Speech Language Profile Groups (SLPG), Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS), and intellectual functioning were assessed in the children along with brain MRI categorization. RESULTS: Very strong relationships were noted among the VSS, CFCS, and SLPG, although these three communication systems provide complementary information, especially for children with mid-range communication impairment. These three communication classification systems were strongly related with the MACS, but moderately related with the GMFCS. Multiple logistic regression analysis indicated that manual ability and intellectual functioning were significantly related with VSS and CFCS function, whereas only intellectual functioning was significantly related with SLPG functioning in children with CP. Communication function in children with a periventricular white matter lesion (PVWL) varied widely. In the cases with a PVWL, poor functioning was more common on the SLPG, compared to the VSS and CFCS. CONCLUSION: Very strong relationships were noted among three communication classification systems that are closely related with intellectual ability. Compared to gross motor function, manual ability seemed more closely related with communication function in these children.
Brain ; Cerebral Palsy* ; Child* ; Classification ; Humans ; Logistic Models ; Magnetic Resonance Imaging ; White Matter

PURPOSE: The purpose of this study is to investigate differentially expressed genes using DNA microarray between advanced gastric cancer (AGC) with aggressive lymph node (LN) metastasis and that with a more advanced tumor stage but without LN metastasis. MATERIALS AND METHODS: Five sample pairs of gastric cancer tissue and normal gastric mucosa were taken from three patients with T3N3 stage (highN) and two with T4N0 stage (lowN). Data from triplicate DNA microarray experiments were analyzed, and candidate genes were identified using a volcano plot that showed > or = 2-fold differential expression and were significant by Welch's t test (p < 0.05) between highN and lowN. Those selected genes were validated independently by reverse-transcriptase-polymerase chain reaction (RT-PCR) using five AGC patients, and tissue-microarray (TMA) comprising 47 AGC patients. RESULTS: CFTR, LAMC2, SERPINE2, F2R, MMP7, FN1, TIMP1, plasminogen activator inhibitor-1 (PAI-1), ITGB8, SDS, and TMPRSS4 were commonly up-regulated over 2-fold in highN. REG3A, CD24, ITLN1, and WBP5 were commonly down-regulated over 2-fold in lowN. Among these genes, overexpression of PAI-1 was validated by RT-PCR, and TMA showed 16.7% (7/42) PAI-1 expression in T3N3, but none (0/5) in T4N0 (p=0.393). CONCLUSION: DNA microarray analysis and validation by RT-PCR and TMA showed that overexpression of PAI-1 is related to aggressive LN metastasis in AGC.
Gastric Mucosa ; Humans ; Lymph Nodes* ; Neoplasm Metastasis* ; Oligonucleotide Array Sequence Analysis ; Plasminogen Activator Inhibitor 1 ; Plasminogen Activators* ; Plasminogen* ; Stomach Neoplasms*

PURPOSE: The purpose of this study is to investigate differentially expressed genes using DNA microarray between advanced gastric cancer (AGC) with aggressive lymph node (LN) metastasis and that with a more advanced tumor stage but without LN metastasis. MATERIALS AND METHODS: Five sample pairs of gastric cancer tissue and normal gastric mucosa were taken from three patients with T3N3 stage (highN) and two with T4N0 stage (lowN). Data from triplicate DNA microarray experiments were analyzed, and candidate genes were identified using a volcano plot that showed > or = 2-fold differential expression and were significant by Welch's t test (p < 0.05) between highN and lowN. Those selected genes were validated independently by reverse-transcriptase-polymerase chain reaction (RT-PCR) using five AGC patients, and tissue-microarray (TMA) comprising 47 AGC patients. RESULTS: CFTR, LAMC2, SERPINE2, F2R, MMP7, FN1, TIMP1, plasminogen activator inhibitor-1 (PAI-1), ITGB8, SDS, and TMPRSS4 were commonly up-regulated over 2-fold in highN. REG3A, CD24, ITLN1, and WBP5 were commonly down-regulated over 2-fold in lowN. Among these genes, overexpression of PAI-1 was validated by RT-PCR, and TMA showed 16.7% (7/42) PAI-1 expression in T3N3, but none (0/5) in T4N0 (p=0.393). CONCLUSION: DNA microarray analysis and validation by RT-PCR and TMA showed that overexpression of PAI-1 is related to aggressive LN metastasis in AGC.
Gastric Mucosa ; Humans ; Lymph Nodes* ; Neoplasm Metastasis* ; Oligonucleotide Array Sequence Analysis ; Plasminogen Activator Inhibitor 1 ; Plasminogen Activators* ; Plasminogen* ; Stomach Neoplasms*

PURPOSE: Churg-Strauss syndrome (CSS) is a rare disease characterized by pulmonary and systemic small vessel necrotizing vasculitis and peripheral blood eosinophilia occurring in asthmatics. Cases of CSS with hepatic involvement have been rarely reported. Here, we reported a case of CSS involving liver, in which liver biopsy revealed eosinophilic vasculitis. METHODS: A 75-year-old man complained of dyspnea and hemoptysis. He had severe blood eosinophilia (white blood cell 28,320/microL, eosinophils 79%). Computed tomography of chest and abdomen showed infiltrations in lungs and multifocal infiltrations in both hepatic lobes. Methacholine PC20 was 2.89 mg/mL, which was in asthmatic range. RESULTS: Ultrasonography-guided liver biopsy was performed, showing eosinophilic vasculitis and portal granulomas. CSS can be diagnosed based on evidence of asthma, blood eosinophilia, pulmonary infiltration and vasculitis on biopsy. CONCLUSION: Taken together, in a suspected case of CSS presenting as hepatic involvement, liver biopsy may be useful to demonstrate the presence of vasculitis.
Abdomen ; Asthma ; Biopsy ; Blood Cells ; Churg-Strauss Syndrome ; Dyspnea ; Eosinophilia ; Eosinophils ; Glycosaminoglycans ; Granuloma ; Hemoptysis ; Liver ; Lung ; Methacholine Chloride ; Pulmonary Eosinophilia ; Rare Diseases ; Thorax ; Vasculitis

PURPOSE: The modified Glasgow Prognostic Score (mGPS) consisting of serum C-reactive protein and albumin levels, shows significant prognostic value in several types of tumors. We evaluated the prognostic significance of mGPS in 285 patients with diffuse large B cell lymphoma (DLBCL), retrospectively. MATERIALS AND METHODS: According to mGPS classification, 204 patients (71.5%) had an mGPS of 0, 57 (20%) had an mGPS of 1, and 24 (8.5%) had an mGPS of 2. RESULTS: Our study found that high mGPS were associated with poor prognostic factors including older age, extranodal involvement, advanced disease stage, unfavorable International Prognostic Index scores, and the presence of B symptoms. The complete response (CR) rate after 3 cycles of R-CHOP chemotherapy was higher in patients with mGPS of 0 (53.8%) compared to those with mGPS of 1 (33.3%) or 2 (25.0%) (p=0.001). Patients with mGPS of 0 had significantly better overall survival (OS) than those with mGPS=1 and those with mGPS=2 (p=0.036). Multivariate analyses revealed that the GPS score was a prognostic factor for the CR rate of 3 cycle R-CHOP therapy (p=0.044) as well as OS (p=0.037). CONCLUSION: mGPS can be considered a potential prognostic factor that may predict early responses to R-CHOP therapy in DLBCL patients.
Adult ; Aged ; Antibodies, Monoclonal, Murine-Derived/therapeutic use ; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use ; C-Reactive Protein/*metabolism ; Cyclophosphamide/therapeutic use ; Doxorubicin/therapeutic use ; Female ; Glasgow Outcome Scale ; Humans ; Lymphoma, Large B-Cell, Diffuse/blood/*diagnosis/*drug therapy/mortality ; Male ; Middle Aged ; Multivariate Analysis ; Prednisone/therapeutic use ; Prognosis ; Remission Induction ; Retrospective Studies ; Serum Albumin/*metabolism ; Survival Rate ; Treatment Outcome ; Vincristine/therapeutic use

The sibling relationship and its potential impact on neurodevelopment and mental health are important areas of neuroscientific research. Validation of the tools assessing the quality of the sibling relationship would be the first essential step for conducting neurobiological and psychosocial studies related to the sibling relationship. However, to the best of our knowledge, no sibling relationship assessment tools have been empirically validated in Korean. We aimed to evaluate the psychometric properties of the Korean version of the Lifespan Sibling Relationship Scale (LSRS), which is one of the most commonly used self-report questionnaires to assess the quality of the sibling relationship. A total of 109 adults completed a series of self-report questionnaires including the LSRS, the mental health subscale of the Medical Outcomes Study-Short Form 36 version 2 (SF36v2), the Satisfaction with Life Scale (SLS), and the Marlowe-Crowne Social Desirability Scale (MC-SDS). The internal consistency, subscale intercorrelations, one-week test-retest reliability, convergent validity, divergent validity, and the construct validity were assessed. All six subscale scores and the total score of the LSRS demonstrated good internal consistency (Cronbach's alpha=0.85-0.94) and good test-retest reliability (intraclass correlation coefficient=0.77-0.92). Correlations of the LSRS with the SF36v2 mental health score (r=0.32, p=0.01) and with the SLS (r=0.27, p=0.04) supported the good convergent validity. The divergent validity was shown by the non-significant correlation of the LSRS with the MC-SDS (r=0.15, p=0.26). Two factors were extracted through factor analysis, which explained 78.63% of the total variance. The three Adult subscales loaded on the first factor and the three Child subscales loaded on the second factor. Results suggest that the Korean version of the LSRS is a reliable and valid tool for examining the sibling relationship.
Adult ; Child ; Humans ; Mental Health ; Psychometrics ; Reproducibility of Results* ; Siblings* ; Social Desirability ; Surveys and Questionnaires

PURPOSE: Prader-Willi syndrome (PWS) is a well-known genetic disorder, and microdeletion on chromosome 15 is the most common causal mechanism. Several previous studies have suggested that various environmental factors might be related to the pathogenesis of microdeletion in PWS. In this study, we investigated birth seasonality in Korean PWS. METHODS: A total of 211 PWS patients born from 1980 to 2014 were diagnosed by methylation polymerase chain reaction at Samsung Medical Center. Of the 211 patients, 138 were born from 2000-2013. Among them, the 74 patients of a deletion group and the 22 patients of a maternal uniparental disomy (UPD) group were compared with general populations born from 2000 using the Walter and Elwood method and cosinor analysis. RESULTS: There was no statistical significance in seasonal variation in births of the total 211 patients with PWS (chi2=7.2522, P=0.2982). However, a significant difference was found in the monthly variation between PWS with the deletion group and the at-risk general population (P<0.05). In the cosinor model, the peak month of birth for PWS patients in the deletion group was January, while the nadir occurred in July, with statistical significance (amplitude=0.23, phase=1.2, low point=7.2). The UPD group showed the peak birth month in spring; however, this result was not statistically significant (chi2=3.39, P=0.1836). CONCLUSION: Correlation with birth seasonality was identified in a deletion group of Korean PWS patients. Further studies are required to identify the mechanism related to seasonal effects of environmental factors on microdeletion on chromosome 15.
Chromosomes, Human, Pair 15* ; Humans ; Methylation ; Parturition* ; Polymerase Chain Reaction ; Prader-Willi Syndrome* ; Seasons* ; Uniparental Disomy

The sibling relationship and its potential impact on neurodevelopment and mental health are important areas of neuroscientific research. Validation of the tools assessing the quality of the sibling relationship would be the first essential step for conducting neurobiological and psychosocial studies related to the sibling relationship. However, to the best of our knowledge, no sibling relationship assessment tools have been empirically validated in Korean. We aimed to evaluate the psychometric properties of the Korean version of the Lifespan Sibling Relationship Scale (LSRS), which is one of the most commonly used self-report questionnaires to assess the quality of the sibling relationship. A total of 109 adults completed a series of self-report questionnaires including the LSRS, the mental health subscale of the Medical Outcomes Study-Short Form 36 version 2 (SF36v2), the Satisfaction with Life Scale (SLS), and the Marlowe-Crowne Social Desirability Scale (MC-SDS). The internal consistency, subscale intercorrelations, one-week test-retest reliability, convergent validity, divergent validity, and the construct validity were assessed. All six subscale scores and the total score of the LSRS demonstrated good internal consistency (Cronbach's alpha=0.85-0.94) and good test-retest reliability (intraclass correlation coefficient=0.77-0.92). Correlations of the LSRS with the SF36v2 mental health score (r=0.32, p=0.01) and with the SLS (r=0.27, p=0.04) supported the good convergent validity. The divergent validity was shown by the non-significant correlation of the LSRS with the MC-SDS (r=0.15, p=0.26). Two factors were extracted through factor analysis, which explained 78.63% of the total variance. The three Adult subscales loaded on the first factor and the three Child subscales loaded on the second factor. Results suggest that the Korean version of the LSRS is a reliable and valid tool for examining the sibling relationship.
Adult ; Child ; Humans ; Mental Health ; Psychometrics ; Reproducibility of Results* ; Siblings* ; Social Desirability ; Surveys and Questionnaires