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Complement-mediated hemolytic uremic syndrome (CM-HUS) following chemotherapy for pediatric acute lymphoid neoplasms has rarely been reported. We report the case of an 8-year-old boy with T-lymphoblastic lymphoma (T-LBL) who developed CM-HUS with complement factor H (CFH) mutations (S1191L, V1197A) during induction therapy. Safe administration of chemotherapy after CM-HUS recovery was challenging. By closely monitoring hemolytic and renal parameters during the 2-year treatment period, we observed four episodes of microangiopathic hemolytic anemia (MAHA) with hypocomplementemia and low haptoglobin but no renal dysfunction or thrombocytopenia. Here, we describe the MAHA and CM-HUS episodes in the hopes of elucidating the complex pathophysiology of disorders associated with CFH mutation.

TRIM28 is a scaffold protein that interacts with DNA-binding proteins and recruits corepressor complexes to cause gene silencing. TRIM28 contributes to physiological functions such as cell growth and differentiation. In the chronic myeloid leukemia cell line K562, we edited TRIM28 using CRISPR/Cas9 technology, and the complete and partial knockout (KO) cell clones were obtained and confirmed using quantitative droplet digital PCR (ddPCR) technology. The amplicon sequencing demonstrated no off-target effects in our gene editing experiments. The TRIM28 KO cells grew slowly and appeared red, seeming to have a tendency towards erythroid differentiation. To understand how TRIM28 controls K562 cell proliferation and differentiation, transcriptome profiling analysis was performed in wild-type and KO cells to identify TRIM28-regulated genes. Some of the RNAs that encode the proteins regulating the cell cycle were increased (such as p21) or decreased (such as cyclin D2) in TRIM28 KO cell clones; a tumor marker, the MAGE (melanoma antigen) family, which is involved in cell proliferation was reduced. Moreover, we found that knockout of TRIM28 can induce miR-874 expression to downregulate MAGEC2 mRNA via post-transcriptional regulation. The embryonic epsilon-globin gene was significantly increased in TRIM28 KO cell clones through the downregulation of transcription repressor SOX6. Taken together, we provide evidence to demonstrate the regulatory network of TRIM28-mediated cell growth and erythroid differentiation in K562 leukemia cells.

In methodology, WJMSCs were treated with a 0.4-T SMF. The cell viability was tested using the MTT assay. For the osteogenic analysis, the alkaline phosphatase activity assay and alizarin red S staining were performed. The osteogenic-related gene expression ofThe cell viability showed no significant difference between the SMF-treated group and the sham-exposed cells. However, the SMF-treated group exhibited significantly more mineralized nodule formation and higher ALP activity than their control counterparts (The results suggest that 0.4-T SMF treatment enhances the osteogenesis of WJMSCs at the early-to-middle stage of osteogenic differentiation by increasing the matrix vesicle secretion and mineralization.

Background: Peritoneal dialysis (PD)-related infection is a serious complication of patients with PD. Refractory peritonitis may lead to failure of PD, shift to hemodialysis (HD) or death. Besides, microbiologic resistance increased worldwide that might impact the treatment choice for such infections. Investigating the causative pathogens and risk factors of PD-related infections in Taiwan was warranted. Methods: This is a retrospective study involving patients with PD from 2007 to 2016 in a southern Taiwan hospital. Patient characteristics, microbiological data, outcomes, and factors associated with refractory peritonitis were analyzed. Results: There were 190 episodes of PD-related peritonitis in 110 patients from this cohort. Gram-positive organisms were the leading cause of PD-related peritonitis, but gram-negative organisms, esp. Pseudomonas aeruginosa, were predominant for exit site infection and tunnel infection. The incidence of peritonitis was 0.25 episode per patient-year (1 episode per 47.69 months). The refractory rate was 14.2% (27/190). Methicillin resistance was noted in 2 (13.3%) of 15 Staphylococcus aureus isolates. Of 114 isolates, 72.8% (83) were susceptible to either cefazolin or gentamicin. Staphylococcus spp. and Escherichia coli infections were significantly associated with refractory peritonitis. Baseline hyponatremia (

Particulate matter exposure has been known as a potential risk for the global burden of disease, such as respiratory and cardiovascular diseases. Accumulating evidence suggests that PM2.5 (particulate matter with a diameter less than 2.5 μm) is associated with increased risk of kidney disease, but the mechanisms underlying the renal injury caused by PM2.5 remain to be elucidated. This study investigated the effects of PM2.5 on human proximal tubular epithelial (HK-2) cells by monolayer and 3D spheroid cultures and explored the potential mechanisms. The typical morphology of HK-2 cells showed epithelial–mesenchymal transition (EMT), resulting in reduced adhesion and enhanced migration after PM2.5 exposure, and was accompanied by decreased E-cadherin expression and increased vimentin and α-SMA expressions. Exposure to PM2.5 in the HK-2 cells could lead to an increase in interleukin-6 (IL-6) levels and cause the activation of signal transducer and activator of transcription 3 (STAT3), which is involved in EMT features of HK-2 cells. Furthermore, blocking IL-6/STAT3 signaling by an IL-6 neutralizing antibody or STAT3 inhibitor was sufficient to reverse PM2.5-induced EMT characteristics of the HK-2 cells. Our study suggests that PM2.5 could induce early renal tubule cell injury, contributing to EMT change, and the induction of IL-6/STAT3 pathway may play an important role in this process.

Pentraxin 3 (PTX3) is a multifunctional molecule that mainly expressed in response to proinflammatory stimuli under physiological and pathological conditions. It is produced in tubule epithelial cells that is involved in the innate immune response and inflammatory reactions in the kidney. However, its role in fine particulate matter (PM2.5)-induced renal injury associated with inflammation remains to be investigated. As a result of PM2.5 exposure, the levels of interleukin (IL)-1β, IL-6 and tumor necrosis factor (TNF)-α levels were increased in HK-2 cells. Notably, the mesenchymal phenotypes with migratory abilities of HK-2 cells were found following PM2.5 exposure. The elevated expressions of PTX3 mRNA and protein in response to PM2.5 were tested by RT-PCR and Western blotting respectively. Further determinate the role of PTX3 by siRNA showed lack of PTX3 could increase IL-6 production and promote epithelial-mesenchymal transition (EMT) process, as evidenced by decreased expressions of E-cadherin, and increased expressions of N-cadherin and α-SMA in HK-2 cells following PM2.5 exposure. Our study indicates that PTX3 mediates early inflammatory response and EMT in PM2.5-exposed HK-2 cells, suggesting a counter-regulatory role of PTX3 in the early course of tubule cell injury induced by PM2.5.

Background An outbreak of SARS-CoV-2 Delta variant infection occurred in Pingtung, Taiwan, in June 2021. In this study, we aimed to elucidate the clinical characteristics of the Delta-variant SARS-CoV-2 infection and the treatment outcome of antiviral agents in patients from Pingtung County in Southern Taiwan. Methods A total of 11 patients with Delta-variant COVID-19 were consecutively admitted to a governmental hospital in June 2021. Baseline characteristics and treatment outcome were evaluated. Results All patients were symptomatic. The most common symptoms were cough (72.7%), followed by fever (54.5%), headache (18.2%) and dysosmia/dysgeusia (18.2%). Two patients developed pneumonia without mechanical ventilation requirement. Compared to patients without pneumonia, those with pneumonia had higher aspartate aminotransferase (AST) (21.0 vs. 126.0 IU/L, P = 0.03) and lactate dehydrogenase (LDH) (143.1 vs. 409.0 IU/mL, P = 0.03), and ferritin (0.2 vs. 2.0 mg/L, P = 0.046) levels. Pneumonia improved after 2-week treatment, and no mortality occurred after 30 days of diagnosis. The median duration of viral shedding duration of viral shedding was 16.5 days (range 11–42 days) (defined by time to repeated negative real-time reverse transcriptase polymerase chain reaction (RT-PCR) or a cycle threshold (CT) value ≥ 30). Conclusion We demonstrated the clinical characteristics of Delta-variant COVID-19 and treatment outcome of antiviral agents. The risk factors attributed to pneumonia were higher serum AST, ferritin, and LDH levels.

With advances in the understanding of characteristics of molecules, specific antigens on the surface of hematological malignant cells were identified and multiple therapies targeting these antigens as neoplasm treatments were developed. Among them, chimeric antigen receptor (CAR) T-cell therapy, which got United States Food and Drug Administration (FDA) approval for relapsed/refractory (r/r) diffuse large B-cell lymphoma (DLBCL) as well as for recurrent acute lymphoblastic leukemia (ALL) within the past five years, and for r/r mantle cell lymphoma (MCL) this year, represents one of the most rapidly evolving immunotherapies. Nevertheless, its applicability to other hematological malignancies, as well as its efficacy and persistence are fraught with clinical challenges. Currently, more than one thousand clinical trials in CAR T-cell therapy are ongoing and its development is changing rapidly. This review introduces the current status of CAR T-cell therapy in terms of the basic molecular aspects of CAR T-cell therapy, its application in hematological malignancies, adverse reactions during clinical use, remaining challenges, and future utilization.

Immunotherapy, including chimeric antigen receptor (CAR) T-cell therapy, immune checkpoint inhibitors, cancer vaccines, and dendritic cell therapy, has been incorporated as a fifth modality of modern cancer care, along with surgery, radiation, chemotherapy, and target therapy. Among them, CAR T-cell therapy emerges as one of the most promising treatments. In 2017, the first two CAR T-cell drugs, tisagenlecleucel and axicabtagene ciloleucel for B-cell acute lymphoblastic leukemia (ALL) and diffuse large B-cell lymphoma (DLBCL), respectively, were approved by the Food and Drug Administration (FDA). In addition to the successful applications to hematological malignancies, CAR T-cell therapy has been investigated to potentially treat solid tumors, including pediatric brain tumor, which serves as the leading cause of cancer-associated death for children and adolescents. However, the employment of CAR T-cell therapy in pediatric brain tumors still faces multiple challenges, such as CAR T-cell transportation and expansion through the blood–brain barrier, and identification of the specific target antigen on the tumor surface and immunosuppressive tumor microenvironment. Nevertheless, encouraging outcomes in both clinical and preclinical trials are coming to light. In this article, we outline the current propitious progress and discuss the obstacles needed to be overcome in order to unveil a new era of treatment in pediatric brain tumors.

Background/purpose This study aims to examine the characteristics of Taiwanese children with chronic kidney disease (CKD) and delineate the factors that lead to disease progression in this population. Methods We reviewed the records of the Taiwan Pediatric Renal Collaborative Study, a multicenter database of Taiwanese children with CKD. Multivariate regression analysis was used to identify the main factors associated with disease progression. Results A total of 382 children aged 1-18 years were included in the study (median age was 10.6 years; interquartile range: 6.4-13.8). There were 197 males (51.6%) and 185 females. CKD Stage 1 was diagnosed in 159 children (41.6%), Stage 2 in 160 (41.9%), Stage 3 in 51 (13.4%), and Stage 4 in 12 (3.1%). Fifty-six children (14.7%) experienced CKD progression. A multivariate analysis for all patients indicated that the risk for disease progression was increased in children with CKD secondary to a structural abnormality, genetic disease, anemia, elevated diastolic blood pressure, or elevated blood urea nitrogen. Compared with children with Stage 1 CKD, those with Stage 2 and Stage 4 CKD had decreased risk for CKD progression in this short-term cohort follow-up. Conclusion CKD etiology affects disease progression. Careful monitoring and treatment of anemia and elevated blood pressure in children with CKD may slow disease progression.

The incidence of acute kidney injury (AKI) in critically ill children varies among countries. Here we used claims data from the Taiwanese National Health Insurance program from 2006 to 2010 to investigate the epidemiological features and identify factors that predispose individuals to developing AKI and mortality in critically ill children with AKI. Of 60,338 children in this nationwide cohort, AKI was identified in 850, yielding an average incidence rate of 1.4%. Significant independent risk factors for AKI were the use of extracorporeal membrane oxygenation, mechanical ventilation or vasopressors, intrinsic renal diseases, sepsis, and age more than 1 year. Overall, of the AKI cases, 46.5% were due to sepsis, 36.1% underwent renal replacement therapy, and the mortality rate was 44.2%. Multivariate analysis showed that the use of vasopressors, mechanical ventilation, and hemato-oncological disorders were independent predictors of mortality in AKI patients. Thirty-two of the 474 patients who survived had progression to chronic kidney disease or end-stage renal disease. Thus, although not common, AKI in critically ill children still has a high mortality rate associated with a variety of factors. Long-term close follow-up to prevent progressive chronic kidney disease in survivors of critical illnesses with AKI is mandatory.

Background Enterobacter species has emerged as an important pathogen of nosocomial bacteremia. The purpose of this study is to review the clinical characteristics of bacteremia in pediatric patients. Materials and methods We reviewed retrospectively the medical records of patients (under the age of 18 years) having Enterobacter bacteremia who were treated at Taipei the Veterans General Hospital from January 2001 to June 2011. Results In total, 853 positive blood cultures were obtained from 620 patients during the study period. Among them, 96 episodes of Enterobacter bacteremia were found in 83 patients, accounting for 11.3% of all bacteremia. Eighty-two cases (98.8%) were nosocomial infections. Most of the cases were neonates (62 cases, 74.7%) and premature infants (51 cases, 61.5%). The common sources of bacteremia were the respiratory tract (53.0%), followed by intravascular catheter (10.8%), multiple sources (10.8%), and the gastrointestinal tract (8.4%). The overall case fatality rate was 18.1%, with the highest rate being reported among premature infants. The factors responsible for the deaths were leukocytosis and a higher median number of underlying diseases. Conclusion Based on the findings of the present study, it can be concluded that Enterobacter species are probably an important pathogen of nosocomial bacteremia in premature neonates. The number of underlying diseases should be considered a major factor influencing the prognosis.

The toxic effect of urethane dimethacrylate (UDMA), a major dental resin monomer, on human dental pulp is not fully clear. In this study, we investigated the influence of UDMA on the cytotoxicity, cell cycle distribution, apoptosis and related gene expression of dental pulp cells. The role of reactive oxygen species, hemeoxygenase-1 (HO-1) and carboxylesterase (CES) in UDMA cytotoxicity, was evaluated. UDMA induced morphological changes of pulp cells and decreased cell viability by 29-49% at concentrations of 0.1-0.35 mM. UDMA induced G0/G1, G2/M cell cycle arrest and apoptosis. The expression of cdc2, cyclinB1 and cdc25C was inhibited by UDMA. Moreover, UDMA stimulated COX-2, HO-1 and CES2 mRNA expression of pulp cells. The cytotoxicity of UDMA was attenuated by N-acetyl-l-cysteine, catalase and esterase, but was enhanced by Zn-protoporphyrin (HO-1 inhibitor), BNPP (CES inhibitor) and loperamide (CES2 inhibitor). Exposure of UDMA may potentially induce the inflammation and toxicity of dental pulp. These findings are important for understanding the clinical response of human pulp to resin monomers after operative restoration and pulp capping, and also provide clues for improvement of dental materials.

Two sisters, aged 15 and 13 years, had previous epithelioid angiomyolipoma of the kidney and suspected thin basement membrane disease, respectively. They presented with 2 years of gross hematuria and new-onset heavy proteinuria. Extensive investigations failed to find an overt cause of their urinary manifestations. The diagnosis of child abuse in a medical setting was confirmed by DNA short tandem repeats analysis, which are the first documented cases in which factitious hematuria was thus diagnosed. Complex forms of child abuse in a medical setting may require forensic tests such as DNA short tandem repeats analysis for diagnosis.

Chronic graft-versus-host disease (cGVHD) is one of the most frequent and serious complications of allogeneic hematopoietic stem cell transplantation (HSCT). Nephrotic syndrome (NS) is an uncommon and underrecognized manifestation of cGVHD. We report a patient who developed NS 18 months after allogeneic bone marrow transplantation. The onset of NS was accompanied by active manifestations of cGVHD, and immunosuppressants had not been tapered recently. Renal biopsy revealed membranous nephropathy. The patient failed to improve with three combined immunosuppressants (prednisolone, cyclosporine, and mycophenolate mofetil), but achieved partial remission after intravenous immunoglobulin (IVIG) infusion. Twenty-four months after the diagnosis of NS, the patient was still in hematological remission, with normal serum creatinine level, urinary protein loss of 0.7–1.9 g/day and mild oral mucositis. Our report suggests that NS can be a cGVHD-related immune disorder in HSCT patients. Monitoring of renal parameters, especially proteinuria, is important in cGVHD patients. Our case indicated that post-transplant NS, occurring without history of tapering or following immunosuppressant withdrawal, presents a more severe activity of cGVHD and a relatively severe clinical course. IVIG may modify and control the refractory GVHD-related NS, and can be one of the choices of treatment.

Background Peritonitis is the most serious complication of peritoneal dialysis (PD). We previously showed that high levels of interleukin (IL)-12 and IL-18 in PD effluents (PDE) during the early phase of peritonitis correlated with a predominant Type 1 immune response and a favorable outcome in PD-related peritonitis. To further clarify the longitudinal changes of peritoneal immunity during PD-related peritonitis, we examined the kinetic production of IL-17 in PDE during peritonitis. The correlation between the IL-17 expression pattern and peritonitis outcome was analyzed. Methods The levels of IL-17 were measured in PDE during various phases of peritonitis in 38 patients undergoing PD. The patients were divided into two groups, according to whether they had a rapid versus a delayed response to antibiotic treatment. Results The kinetic expression of IL-17, as measured by enzyme-linked immunosorbent assay, differed between the two groups. In the rapid response group, high level of IL-17 was detected in PDE initially and progressively decreased during treatment. In the delayed response group, IL-17 levels in PDE were persistently low throughout the whole course of treatment. In the early phase of peritonitis, the IL-17 levels in PDE were significantly higher in the rapid response group ( p Conclusion These data suggested that local IL-17 production is part of a protective early immune response to PD-related peritonitis. High levels of IL-17 in PDE during the early phase of peritonitis correlated with a favorable outcome. Manipulation of IL-17 cytokine expression in patients with peritonitis may modulate peritoneal immune response and affect peritonitis outcome.

Rhabdomyolysis is a life-threatening condition that involves muscle cell destruction. Among its etiologies, severe hyper-natremia is a less common cause. We report a teenage girl with congenital central hypoventilation syndrome and hypothalamus dysfunction syndrome who presented with extreme hypernatremia (sodium, 211 mmol/L) with rhabdomyolysis (creatine kinase, 32,850 U/L) and acute renal failure (creatinine, 6.1 mg/dL) following gastroenteritis with 7-kg weight loss. Rhabdomyolysis subsequently led to acute renal failure and hyperkalemia. Acute hemodialysis was initiated on hospital day 3 for hyperkalemia. This resulted in a 13 mmol/L fall in serum sodium in 3 hours despite using a 156 mmol/L sodium bath, but without the development of cerebral edema or neurological defect. This report highlights an unusual cause of rhabdomyolysis in children and the experience of managing such a difficult clinical situation. [J Chin Med Assoc 2009;72(10):555–558]

Ethnicity and age play important roles in the epidemiology of idiopathic nephrotic syndrome (INS) in children. The purposes of this study were to compare the clinical features, renal histopathology, steroid response, and long-term prognosis in Chinese children between different age groups. This is a retrospective cohort study of children aged between 2 and 18 years old with INS. Patients were divided into two groups according to age. Group I consisted of children between 2 and 8 years old (n = 49). Group II consisted of the remaining patients (n = 50). The clinical biochemical parameters, response to steroid treatment, renal histology, and long-term outcomes were analyzed. The biochemical parameters at the onset were similar in the two groups. Group II had a significantly higher frequency of microscopic hematuria (P = 0.011). Of the 67 children biopsied, minimal change disease was the most common histopathology for both groups. There was a higher frequency with focal and segmental glomerulosclerosis in group II (24% vs. 6.1%), but the difference between the two groups was not significant. During follow-up, the frequency of hypertension was significantly higher in group II (P = 0.006). Two cases in group I developed chronic kidney disease (CKD) vs. eight cases in group II. The frequency of progression to CKD is significantly higher (P = 0.042) in Group II. In conclusion, children beyond 8 years of age with INS have a higher incidence of microscopic hematuria, higher risk of hypertension and progression to CKD in long-term follow-up.

Little information is available regarding the clinical characteristics and prevalence of complications in children with chronic kidney disease (CKD), especially in early disease stages. The objective of this study was to determine the clinical characteristics and prevalence of complications in children with predialytic CKD. This multicenter, cross-sectional study enrolled children at all stages of predialytic CKD. Children who were between the ages of 1 year and 18 years and who fulfilled the clinical criteria of CKD were included in the study. Baseline demographic data, previous history, clinical characteristics, and laboratory data were collected. A total of 757 children were included in the study. The median age at the time of enrollment was 10.6 years; 397 patients (52.4 %) were males. A total of 39.0 % of the patients were in CKD stage 1, 37.6 % were in stage 2, 14.8 % were in stage 3, 3.0 % were in stage 4, and 5.5 % were in stage 5. Nonglomerular renal diseases were the primary cause of CKD, comprising 51.9 % of the patients with CKD. The age at disease onset, gender, CKD stage distribution, and proportion of co-morbidities varied between the glomerular and nonglomerular CKD cases. Anemia, hyperlipidemia, hypocalcemia, and hyperphosphatemia were more prevalent in patients with glomerular CKD. The overall prevalence of complications was as follows: uncontrolled blood pressure, 44.1 %; anemia, 34.2 %; hyperlipidemia, 44.9 %; short stature, 10.3 %; and failure to thrive, 8.2 %. Uncontrolled blood pressure (BP), anemia, and hyperlipidemia were common, even in the early CKD stages. The prevalence of CKD complications generally increased with the worsening stage of CKD. This study reveals differences in CKD etiology and prevalence of specific complications according to the stage of CKD. Early recognition and awareness of complications are mandatory for clinicians during the follow-up visits of children with CKD.

In this study, we investigated the effects of functional group counseling on inspiring low achievers' self-worth and self-efficacy in Taiwan. Forty-three 10th grade low-achieving students volunteered as the Experimental Group to join a 24-week intervention, which integrated and utilized functional group counseling; another 51 10th grade low-achieving students volunteered to be Comparison Group I. In addition, 43 10th grade moderate or high academic achieving students volunteered to be Comparison Group II. All participants completed the Vocational School Student Questionnaire at the beginning and end of this study to measure their self-worth and self-efficacy. In addition, six target students (two boys and four girls) with the lowest total scores on self-worth or self-efficacy in the pretest were selected from the Experimental Group to be interviewed at the end of the intervention and observed weekly. Analyses of variance, analyses of covariance, and paired t-tests assessed the similarity and differences among groups. The initial findings were as follows: Experimental group students had significantly higher scores on self-efficacy and self-worth than both Comparison Group I and Group II students and functional group counseling was shown to significantly affect the low-achieving students. Qualitative results from interviews and observations were used for triangulation and consolidation of quantitative results. Implications of the study included the recommended use of functional group counseling with low-achieving students.

Cardiovascular event and infection are leading causes of death from peritoneal dialysis (PD). This study examined in vitro cellular mechanism for cardiotoxicity induced by PD-related peritonitis. Cultured human cardiomyocytes were treated with PD effluent (PDE) during peritonitis (PPDE), and effects of PPDE on cultured cardiomyocytes in terms of apoptosis, with expression its related genes assessed. Results showed PPDE treatment of cardiomyocyte leading to onset of apoptosis, as confirmed by phosphatidylserine exposure plus DNA fragmentation and damage. This apoptosis is mediated by reduced Bcl-2/Bax and Bcl-xL/Bax ratios, as well as reduced expression of GATA-4, an important cardiomyocyte survival factor, at the level of transcription. These changes activated pro-apoptotic pathways. PPDE treatment also inhibited ERK signals, contributing to cardiotoxicity. Our findings revealed that PPDE contains potent pro-apoptotic factors that regulate expression of GATA-4 and Bcl-2 families, inducing cultured cardiomyocyte apoptosis. This pinpoints a key role of apoptosis in PD-associated cardiovascular events, along with a potential therapeutic target. J. Cell. Physiol. 226: 94–102, 2010. © 2010 Wiley-Liss, Inc.

Eosinophilic peritonitis is an uncommon clinical entity with diagnostic considerations separate from those of tuberculous peritonitis. We report a patient on continuous ambulatory peritoneal dialysis (CAPD) with eosinophilic peritonitis resulting from tuberculous peritonitis. Acid-fast stain and mycobacterial culture of peritoneal dialysis effluent were both negative result. In the peritoneal dialysis effluent and blood samples, Mycobacterium tuberculosis was detected by polymerase chain reaction analyses. The initiation of antituberculous therapy resulted in resolution of the eosionphilia in the dialysis effluent. After 14 days of antituberculous therapy, the polymerase chain reaction analyses of tuberculosis were negative for both the blood and peritoneal dialysis effluents. Evaluation of tuberculosis infection is necessary if the CAPD-related peritonitis presents with an unusual and unexplained clinical course. Polymerase chain reaction can play an important role in the diagnosis of tuberculous peritonitis in patients undergoing CAPD.

MafA is a pancreatic transcriptional factor that controls β-cell-specific transcription of the insulin gene. However, the role of MafA in the regulation of pancreatic transdifferentiation and reprogramming in human stem cells is still unclear. In this study, we investigate the role of MafA in placenta-derived multipotent stem cells (PDMSCs) that constitutively expressed Oct-4 and Nanog. PDMSCs were isolated and transfected with MafA using a lentivector. Our results showed that overexpression of MafA in PDMSCs significantly up-regulated the expression of pancreatic development-related genes (Sox17, Foxa2, Pdx1 and Ngn3). Microarray analysis suggested that the gene expression profile of MafA-overexpressing PDMSCs was similar to that of pancreas and islet tissues. MafA increased the expression levels of the mRNAs of NKx2.2, Glut2, insulin, glucagons and somatostatin, and further facilitated the differentiation of PDMSCs into insulin+ cells. The glucose-stimulated responses to insulin and c-peptide production in MafA-overexpressing PDMSCs were significantly higher than in PDMSCs with vector control. Our results indicated that MafA-overexpressing PDMSCs were more resistant to oxidative damage and oxidative damage-induced apoptosis than PDMSCs carrying the vector control were. Importantly, the expression of MafA in PDMSCs xenotransplanted into immunocompromised mice improved the restoration of blood insulin levels to control values and greatly prolonged the survival of graft cells in immunocompromised mice with STZ-induced diabetes. In summary, these data suggest that MafA plays a novel role in the reprogramming of stem cells into pancreatic β-progenitors, promotes the islet-like characteristics of PDMSCs, as well as functionally enhances insulin production to restore the regulation of blood glucose levels in transplanted grafts.

Background: Acute renal failure (ARF) is an important cause of morbidity and mortality in children. Here, we investigate etiology, comorbidities, outcome and risk factors associated with mortality in these children with ARF. Methods: We retrospectively reviewed the characteristics of 58 children with ARF diagnosed between January 1997 and December 2006 at a single institute. Factors including age, sex, clinical features and laboratory parameters were compared between survivors and non-survivors. Results: ARF was secondary to extrarenal causes in 79.3% of cases. Sepsis (18.9%), hematooncologic disease (18.9%) and cardiovascular disease (18.9%), were the main causes of ARF. Primary renal disease due to acute glomerulonephrits, nephrotic syndrome, hemolytic uremic syndrome and obstructive uropathy accounted for 20.7% of the cases. The overall mortality rate was 51.7%. There were no significant differences between survivors and non-survivors in gender and changes in the peak levels of calcium, phosphorous and uric acid levels. The mortality rate was significantly higher when ARF occurred in younger children (p = 0.019), secondary to systemic disease (p = 0.038, odds ratio 4.3; 95% confidence interval (CI) 1.0, 17.9), sepsis (p < 0.001, odds ratio 19.7; 95% CI 5.1, 76.4), use of ventilator (p < 0.001, odds ratio 35; 95% CI 6.7, 182.7), multiple organ failure (p < 0.001) and non-use of renal replacement therapy (p = 0.018, odds ratio, 3.6; 95% CI interval 1.2, 10.6) on univariate analysis. Multiple logistic regression analysis revealed that sepsis (p = 0.011, odds ratio, 11.3; 95% CI 1.7, 73.0) and numbers of organ failures (p - 0.001, odds ratio 8.14; 95% CI 2.5, 26.7) were independently associated with mortality. Conclusion: This study found that sepsis and number of organ failures were independent predictors of mortality in children with ARF.

We previously showed that a local immune response largely composed of type 1 T cells correlated with a favorable outcome of the peritonitis associated with peritoneal dialysis. To clarify how these subsets are recruited to the peritoneal cavity during inflammation, we measured integrin-mediated interactions between the T cells and human peritoneal mesothelial cells. Direct microscopy showed that lipopolysaccharide or peritoneal dialysis effluent stimulated the adherence of T cells to mesothelial cells, a process mediated by the integrins alpha6beta1 and alpha4beta1. Further, the migration of Th1 cell across human mesothelial cell monolayers grown on transwell surfaces was reduced by anti-alpha6beta1 integrin antibody while that of Th2 cell was inhibited by an anti-alpha4 integrin antibody. Pretreatment with either lipopolysaccharide or rapid response peritoneal dialysis effluent stimulated T cell migration and this was significantly decreased by the alpha6beta1 compared to the alpha4 antibody. These results suggest that integrins may play an important role in mediating selective T cell subset adhesion and migration across human peritoneal mesothelial cell monolayers and differential integrin expression and selective T cell subset recruitment during peritonitis may affect outcome.

Acute urinary retention is unusual in children and is usually a candidate for visiting the emergency department upon initial discovery. We report a 12-year-old girl who complained of acute urinary retention. Ultrasonography demonstrated a large echogenic mass over the vagina and mild dilation of the uterus. Imperforate hymen associated with hematocolpos and hematometrium was diagnosed. Cruciate hymenotomy was performed. The symptoms resolved after treatment. Adolescent girls who complain of urinary symptoms with no previous menstruation should have their external genitalia examined in order to rule out the possibility of imperforate hymen as the cause of acute urinary retention.

Background: Focal segmental glomerulosclerosis (FSGS) is a primary glomerular disease that usually progresses to renal failure. Although high-dose pulse methylprednisolone therapy (PMT) has been shown to be effective in the treatment of steroid-resistant FSGS, adverse effects have caused parents to hesitate in approving the treatment. The aim of this study is to investigate whether low-dose PMT based protocol for treatment of young children with steroid resistant FSGS would effectively induce remission of proteinuria and prevent the progression of renal insufficiency. Methods: This is a retrospective study. The authors treated eight children with steroid-resistant FSGS with intravenous methylprednisolone pulse 10 mg/kg per day for three consecutive days weekly for 8 weeks. Partial responders were treated with the addition of chlorambucil or cyclosporine (CsA) and four fortnightly and eight monthly pulses of high-dose PMT (30 mg/kg per day). Results: Of the eight patients, six attained complete remission initially. The median urinary protein excretion in 24 h decreased from 4.25 to 0.39 g following 8 weeks of low dose (P = 0.012). Marked decrease in urinary protein-creatinine ratio was noted soon after treatment (P = 0.012). There was a significant increase in serum albumin level after treatment compared to the pretreatment condition (median, 3.35 vs 4.1 mg/dL, P = 0.018). Five of the eight patients remained in complete remission, and one of the eight patients relapsed during follow up. Relapse responded to repeated treatments of PMT and cyclosporine. The two patients with partial remission initially progressed to renal insufficiency in one patient and end-stage renal disease in the other patient. Conclusions: Low-dose PMT caused a significant decrease in the proteinuria of Chinese children with steroid-resistant FSGS with a low frequency of intolerance.

Rifabutin is a semi-synthetic antimycobacterial agent mainly used in the prophylaxis and treatment of Mycobacterium avium complex (MAC) in acquired immunodeficiency syndrome patients. Uveitis as a side effect of rifabutin has been recognized and established since 1994, but there was no case previously described in Taiwan so far. We report 2 cases of rifabutin-induced hypopyon uveitis in patients with human immunodeficiency virus and MAC infection. Both patients received the regimen of clarithromycin and rifabutin to treat MAC infection. Uveitis resolved after discontinuing of rifabutin and treatment with topical corticosteroid and mydriatics. Early recognition of this entity can prevent invasive ocular procedures and treatments. Doctors who prescribe rifabutin should be aware of this ocular complication of uveitis and drug-drug interactions. Ophthalmologists should put this on the list of differential diagnoses for uveitis.

We previously showed that a positive impact of peritoneal defense response on the outcome of peritoneal dialysis (PD)-related peritonitis is characterized by an increased pattern of peritoneal CD4/CD8 T-cell ratio with a predominant CD4(+)-T helper subtype 1 phenotype. To further explore longitudinal changes in peritoneal immunity during PD-related peritonitis, we examined the production of interleukin 12 (IL-12), IL-18, and interferon gamma (IFN-gamma) in peritoneal dialysate effluent (PDE) and kinetic expression of the transcription factors T box expressed in T cells (T-bet) and guanine adenine thymine adenine (GATA) binding protein 3 (GATA-3) in peritoneal T cells during peritonitis. Correlations between these observations and responses to antibiotics were analyzed.IL-12, IL-18, and IFN-gamma protein and IFN-gamma, T-bet, and GATA-3 messenger RNA (mRNA) were measured in PDE during various phases of peritonitis in 40 patients undergoing PD. Patients were divided into 2 groups according to whether they had a rapid versus delayed response to antibiotic treatment.In the early phase of peritonitis, IL-12, IL-18, and IFN-gamma levels in PDE were significantly greater in the rapid-response group (P0.05). Changes in peritoneal IL-12 and IL-18 levels preceded changes in IFN-gamma levels. The kinetics of IFN-gamma, T-bet, and GATA-3 mRNA expression in peritoneal T cells, measured by means of real-time polymerase chain reaction, differed between the 2 groups. In the rapid-response group, IFN-gamma and T-bet mRNA expression increased, whereas that of GATA-3 decreased over time. Results were opposite in the delayed-response group, with IFN-gamma and T-bet levels decreasing and GATA-3 levels increasing over time.These data suggest that local IL-12 and IL-18 production is part of a protective early immune response to PD-related peritonitis. High IL-12 and IL-18 levels in PDE during the early phase of peritonitis correlated with a predominant type 1 immune response and favorable outcome.

Background The peritoneal immune compartment is a microenvironment with a particular T-cell repertoire and susceptible to local inflammation. To clarify the role of T lymphocytes in peritoneal immunity, the changes in T-cell subpopulations in peritoneal dialysis effluents (PDEs), and their influence on the response to the treatment of peritonitis and on its prognosis were studied in patients undergoing long-term, continuous ambulatory peritoneal dialysis (CAPD). Methods A cohort of 36 patients treated with CAPD and who had histories of peritonitis were divided into a group with rapid and a group with delayed response to antibiotics, and were followed for 3 years. CD4/CD8 T-cell ratios, T-cell cytokine mRNA expression patterns and transforming growth factor-beta1 (TGF-beta1) concentrations were examined in PDE during bouts of peritonitis. The change in 4 h D/P creatinine during the peritoneal equilibration test (PET) between year 0 and year 3 was expressed as deltaD/P creatinine. Results The serial changes in T-cell subsets in PDE during peritonitis showed two patterns: (i) pattern 1, manifest as a progressive increase in the CD4/CD8 ratio, and associated with a rapid response to treatment; and (ii) pattern 2, manifest as a progressive decrease in the CD4/CD8 ratio, and associated with a delayed response to treatment. The major T-cell phenotypes in PDE during peritonitis were Th1-CD4(+) and Tc2-CD8(+), determined by cloning techniques, RT-PCR and double immunofluorescence staining. TGF-beta1 in the effluent was undetectable in pattern 1 after 7-8 days, but remained detectable at 2 weeks in pattern 2. Pattern 2 patients had a significantly greater decrease (deltaD/P creatinine: -0.198+/-0.086) in solute transport than pattern 1 patients (deltaD/P creatinine: -0.036+/-0.077, P Conclusions These results suggest that a progressive decrease of the CD4/CD8 ratio in PDE correlates with a persistent expression of TGF-beta1, and plays a pathogenetic role in the evolution of peritonitis, PET deterioration and peritoneal fibrosis. Therefore, patterns of CD4/CD8 T-cell ratio in PDE may predict clinical outcomes of peritonitis in CAPD patients.

To date, the underlying diseases and follow-up of Taiwanese children screened by urinalysis have not been reported. The grading of urine abnormalities varied from grade A (microscopic hematuria only), grade B (light proteinuria only), grade C (light proteinuria and microscopic hematuria) to grade D (heavy proteinuria). From January 1991 to August 1998, 630 students, aged 6-15 years and with positive urinary screening, were admitted to our hospital for further evaluation. Of these, 573 students had confirmed abnormal findings, 298 were boys, 275 were girls, and 294 students received a renal biopsy and have had regular follow-up visits. This study was designed to retrospectively elucidate: (1) the relationship between grading of urine abnormality and underlying disease; (2) the relationships among hypertension, grading of urine abnormality, and underlying disease; (3) the underlying disease of low serum C3 level; and (4) to determine whether urinary screening progressively decreased the number of students with end-stage renal disease (ESRD) annually. The results show that glomerular nephritis (GN) is still one of the major causes of urinary abnormalities. The most-important secondary GN was systemic lupus erythematosus (SLE) with lupus nephritis. One-quarter of the patients fulfilled at least four of the revised American Rheumatology Association (ARA) criteria for SLE at first administration, while the others who fulfilled only two to three of the revised ARA criteria had gradually developing signs and symptoms of SLE at follow-up. The percentage of SLE patients amongst anti-nuclear antibody (ANA) positive children was 72%. Membranoproliferative GN is very rare. The distribution of hypertension was 8.2% in grade A, 10.7% in grade B, 9.7% in grade C, and 28.9% in grade D urinary abnormality. There were statistical differences between grade D and either grade A or B or C (P

Hemorrhagic cystitis is a potentially life-threatening complication in systemic lupus erythematosus (SLE). No safe, effective and conservative treatment exists for patients who fail to respond to standard therapy. We report a 17-year-old girl with SLE who suffered from severe hemorrhagic cystitis. Initially, she received frequent red blood cell and platelet transfusions, continuous bladder irrigation, and blood clots were evacuated. Numerous kinds of treatment were tried, including electrocoagulation of bleeding foci, prostaglandin E1 bladder instillation, and hyperbaric oxygen. However, she remained severely anemic and thrombocytopenic necessitating daily transfusions of blood products. After intravesical formalin instillation was performed twice, the hematuria ceased completely.