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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.

Background The recommendation from national documents and reports to promote inquiry-related science activities has not been supported by recent studies, which have found the overall frequency of inquiry activities to be negatively associated with student learning outcomes. This study was inspired by such conflicting reports and aimed to clarify the associations of science-specific, inquiry-related activities and epistemological beliefs with students’ mathematical and scientific literacies. Results A secondary analysis of the database from the Programme for International Student Assessment 2015 of Australia (N1 = 14,530) and Taiwan (N2 = 7708) utilizing structural equation modelling revealed that these two countries exhibited similar data patterns. Results suggested that open-inquiry activities (such as debating and planning experiments) had a negative relationship with secondary students’ mathematical and scientific literacies. Structured inquiry learning (such as students explaining their ideas and teacher explaining how an idea can be applied to different phenomena) and epistemological beliefs about science were significant and positive predictors of student mathematical and scientific literacy performance. Conclusions The current study further highlights and provides empirical evidence that the teacher’s role in structured inquiry (especially pertaining to the relevance and applicability of these ideas) appears to be essential to the development of student literacy. Educational implications and recommendations are discussed.

This study investigated a model of the associations between personality traits of male and female college students in Taiwan and their self-worth using structural equation modeling (SEM). College students (1,305 males and 1,494 females) were stratified randomly selected from 12 universities to complete the College Students Questionnaire. SEM results supported the model and network of paths amongst these participating students’ self-worth and the Big-five personality traits. Results indicated significant positive predictive pathways between self-worth and extraversion, conscientiousness, openness, and agreeableness and a significant negative predictive pathway between self-worth and neuroticism. The results of restricting measurement weights indicated that the Big-five traits and self-worth model was adequate for all college students of different ages. In addition, it was found that males had significantly higher academic/general competence and athletic competence/physical appearance scores than females, while females had significantly higher close friendship, conduct behavior, and extraversion, conscientiousness, agreeableness, and neuroticism traits than males. Older college students had significantly higher academic/general competence, close friendship, conduct behavior, job competence, extraversion, agreeableness, conscientiousness, and openness mean scores than their younger counterparts, whereas they had significantly lower neuroticism scores than the younger participants. Finally, the results suggest that self-worth was important in the development of positive personality traits in college students.

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.

Background The tristetraprolin (TTP) family of mRNA-binding proteins contains three major members, Ttp, Zfp36l1, and Zfp36l2. Ttp down-regulates the stability of AU-rich element–containing mRNAs and functions as an anti-inflammation regulator. Methods To examine whether other TTP family proteins also play roles in the inflammatory response, their expression profiles and the possible mRNA targets were determined in the knockdown cells. Results Ttp mRNA and protein were highly induced by lipopolysaccharide (LPS), whereas Zfp36l1 and Zfp36l2 mRNAs were down-regulated and their proteins were phosphorylated during early lipopolysaccharide stimulation. Biochemical and functional analyses exhibited that the decrease of Zfp36l2 mRNA was cross-regulated by Ttp. Knockdown of Zfp36l1 and Zfp36l2 increased the basal level of Mkp-1 mRNAs by prolonging its half-life. Increasing the expression of Mkp-1 inhibited the activation of p38 MAPK under lipopolysaccharide stimulation and down-regulated Tnfα, and Ttp mRNA. In addition, hyper-phosphorylation of Zfp36l1 might stabilize Mkp-1 expression by forming a complex with the adapter protein 14-3-3 and decreasing the interaction with deadenylase Caf1a. Conclusions Our findings imply that the expression and phosphorylation of Zfp36l1 and Zfp36l2 may modulate the basal level of Mkp-1 mRNA to control p38 MAPK activity during lipopolysaccharide stimulation, which would affect the inflammatory mediators production. Zfp36l1 and Zfp36l2 are important regulators of the innate immune response. Electronic supplementary material The online version of this article (doi:10.1186/s12950-015-0088-x) contains supplementary material, which is available to authorized users.