The pathogenesis of renal involvement in Wiskott-Aldrich syndrome (WAS) is unclear and renal outcome is generally poor in such situations. Here we present the case of an 8-year-old boy with WAS who developed hematuria, proteinuria, and declining renal function that did not improve with the combined use of immunosuppressive agents and angiotensin-converting-enzyme inhibitor. Renal pathology revealed IgA nephropathy (IgAN). The patient underwent splenectomy for refractory thrombocytopenia. The proteinuria remitted and renal function improved after splenectomy, long-term antibiotic prophylaxis, and tapering of immunosuppressive agents.

CvaB is the central membrane transporter of the colicin V secretion system that belongs to an ATP-binding cassette superfamily. Previous data showed that the N-terminal and C-terminal domains of CvaB are essential for the function of CvaB. N-terminal domain of CvaB possesses Ca(2+)-dependent cysteine proteolytic activity, and two critical residues, Cys32 and His105, have been identified. In this study, we also identify Asp121 as being the third residue of the putative catalytic triad within the active site of the enzyme. The Asp121 mutants lose both their colicin V secretion activity and N-terminal proteolytic activity. The adjacent residue Pro122 also appears to play a critical role in the colicin V secretion. However, the reversal of the two residues D121P - P122D results in loss of activity. Based on molecular modeling and protein sequence alignment, several residues adjacent to the critical residues, Cys32 and His105, were also examined and characterized. Site-directed mutagenesis of Trp101, Asp102, Val108, Leu76, Gly77, and Gln26 indicate that the neighboring residues around the catalytic triad affect colicin V secretion. Several mutated CvaB proteins with defective secretion were also tested, including Asp121 and Pro122, and were found to be structurally stable. These results indicate that the residues surrounding the identified catalytic triad are functionally involved in the secretion of biologically active colicin V.

OBJECTIVES: Differences in disposition between emergency physicians (EPs) have been studied in select patient populations but not in general emergency department (ED) patients. After determining whether a difference existed in admit/discharge decision making of EPs for general ED patients, we focus our study in examining the influence of EP seniority on the decision to discharge ED patients. METHODS: In a 1-year retrospective study, we included a convenience sample of all 18 953 adult nontraumatic ED patients. We reviewed the admit/discharge dispositions at each shift made by 16 EPs. EPs were categorized by seniority to determine whether seniority influenced disposition. Three groups had 5, 4, and 7 EPs each, with >10 years, 5 to 9 years, and <5 years of working experience, respectively. RESULTS: Patient demographics, triage level, and number of patients per shift did not differ statistically between EPs and each group. The number of discharged patients per shift differed statistically between EPs (P <.001) and each group. The most senior EPs had the lowest discharge rates compared with EPs in intermediate and junior groups. They had lower discharge rates for patients at triage levels 1, 2, and 3 as well as for all patients. However, no difference in unscheduled ED revisit rates was found. CONCLUSIONS: EPs vary in their admit/discharge decision making for general ED patients. More importantly, the most senior EPs were found to have the lowest discharge rates compared with their junior colleagues.

Centimeter-sized Te-doped GaSe ingots were grown from the charge compositions of GaSe with nominals 0.05, 0.1, 0.5, 1, and 3 mass% Te, which were identified as ɛ-GaSe:Te (0.01, 0.07, 0.38, 0.67, and 2.07 mass%) single crystals. The evolution of the absorption peaks of the phonon modes E'⁽²⁾(∼0.584 THz) and E"⁽²⁾(1.77 THz) on Te-doping in GaSe:Te crystals was studied by THz time-domain spectroscopy. This study proposes that the evolution of both E'⁽²⁾ and E"⁽²⁾ absorption peaks correlates well with the optical quality of Te-doped GaSe crystals, which was confirmed by experimental results on the efficiency of THz generation by optical rectification. Maximal intensity of the absorption peak of the rigid layer mode E'⁽²⁾ is proposed as a criterion for identification of optimal Te-doping in GaSe crystals.

The growth of porous ZnO nanowires (NWs) via phase transformation of ZnS NWs at 500-850 degrees C in air was studied. The ZnS NWs were first synthesized by thermal evaporation of ZnS powder at 1100 degrees C in Ar. On subsequent annealing at 500 degrees C in air, discrete ZnO epilayers formed on the surface of ZnS NWs. At 600 degrees C, polycrystalline ZnO and the crack along the (0001) interface between the ZnO epilayer and ZnS NW were observed. At 700-750 degrees C ZnS NWs transformed to ZnO NWs, meanwhile nanopores and interfacial cracks were observed in the ZnO NWs. Two factors, the evaporation of SO2 and SO3 and the stress induced by the incompatible structure at the interface of ZnO epilayer and ZnS NW, can be responsible for the formation of porous ZnO NWs from ZnS NW templates on annealing at 700-750 degrees C in air. Rapid growth of ZnO at 850 degrees C could heal the pores and cracks and thus resulted in the well-crystallized ZnO NWs.

BACKGROUND: Vaccination is an important preventive measure for preparing against the influenza pandemics. This study investigated the attitudes and perceptions of influenza vaccination among doctors and medical students in Hong Kong. METHODS: A cross-sectional survey was conducted among 204 doctors and 242 medical students in a teaching hospital in 2009. Participants' demographic and job characteristics, and influenza experience and vaccination in the previous year were assessed in the questionnaire. Logistic regression models were used to examine the associations between uptake of influenza vaccination and the perceived benefits. RESULTS: Medical students were more likely to have receive an influenza vaccination in the previous year (66.9 vs. 39.7%) and acknowledged the related benefits than doctors. Moreover, uptake of influenza vaccine was associated with perceived benefits of vaccination in both doctors and medical students. CONCLUSIONS: The perceived benefits of influenza vaccination are an important factor in vaccine uptake for both doctors and medical students in Hong Kong, and should be reinforced in the professional training.

The ataxia telangiectasia mutated (ATM) gene plays a major role in repairing the double-strand breaks and maintaining the genome stability. In this case-control study, associations of seven ATM single-nucleotide polymorphisms (rs600931, rs189037, rs652311, rs624366, rs228589, rs227092 and rs227060) with risks in childhood leukemia in a Taiwanese population were investigated. Two hundred and sixty-six patients with childhood leukemia and 266 age-matched healthy controls recruited were genotyped and analyzed. The P-values of the distributions of the genotypic frequencies in the seven ATM polymorphisms were 0.8925, 0.2835, 0.5772, 0.8731, 0.3641, 0.9181 and 0.5071, respectively. The Pvalues of the distributions of the allelic frequencies in the seven ATM polymorphisms were 0.6158, 0.1179, 0.6971, 0.7944, 0.1887, 0.6605 and 0.2747, respectively. Although the results did not indicate that ATM polymorphism is directly associated with childhood leukemia, the gene-gene and gene-environment interactions of ATM with other factors is worthy of further investigation in the future.

OBJECTIVE Vulvar cancer is rare in Eastern females, especially in pre- and peripubertal girls. The prognosis of vulvar cancer is poor and treatment is variable. CASE REPORT A 14-year-old girl suffered from a left vulvar tenderness mass and underwent excision of the mass. The diagnosis of the pathology was vulvar yolk tumor with an embryonal carcinoma. After the vulvectomy, inguinal area lymph node dissection, chemotherapy (bleomycin, etoposide and cisplatin) treatment and radiotherapy, metastasis to lung was also noted after eight months. Resection of lung tumor was performed. She received chemotherapy with a combination of paclitaxel, ifosphamide and cisplatin (TIP) and received peripheral blood stem cell transplantation (PBSCT) twice and chemotherapy treatment of gemitabine and oxaliplatin (GEMOX). Up until now, the patient has been free of disease. CONCLUSION High-dose TIP and GEMOX chemotherapy plus PBSCT for bone marrow rescues could be considered to treat patients with metastatic malignant vulvar germ cell tumor after failed first-line chemotherapy and radiation.

OBJECTIVE: The aim of the work is to determine whether the change between primary and repeated serum inflammatory markers measured 8-12 h later may improve diagnostic accuracy in pediatric appendicitis. METHODS: The study group comprised 258 pediatric patients with clinically suspected appendicitis admitted to the pediatric emergency department from 2005 to 2007. The significant changes in serum parameters between primary and repeated examinations were selected as the discriminating variables. The receiver operating characteristic curves were used to determine the cut-off values of the changes between two examinations in predicting appendicitis. RESULTS: Receiver operating characteristic analysis showed that the cut-off values for the change in total neutrophils (3.0%) on the first day after the onset of symptoms (day 1), the changes in C-reactive protein concentration (4.5 mg/l) and in bands (1%) on day 2, and the change in C-reactive protein concentration (15.0 mg/l) on day 3 were significant serum parameters in predicting pediatric appendicitis. CONCLUSION: Repeated serum laboratory tests at different time points during the progression of acute appendicitis may be helpful in predicting pediatric appendicitis in the pediatric emergency department.

BACKGROUND: The absence of biallelic TCRγ deletion (ABD) is a characteristic of early thymocyte precursors before V(D)J recombination. The ABD was reported to predict early treatment failure in T-cell acute lymphoblastic leukemia (ALL). This study aimed to investigate its prognostic value in Taiwanese patients with T-cell ALL. PROCEDURE: Forty-five children with T-cell ALL were enrolled from six medical centers in Taiwan. Quantitative DNA polymerase chain reaction (Q-PCR) was performed to check the status of TCRγ deletion. The threshold for homozygous deletions by Q-PCR was defined as a fold-change <0.35. RESULTS: ABD was found in 20 patients [20:45] who had higher incidences of induction failure than those without ABD (P = 0.03; hazard ratio [HR] = 8.13; 95% confidence interval [95% CI] = 1.23-53.77) after multivariate regression analysis. Patents with ABD also had inferior EFS and OS (P = 0.071 and 0.0196, respectively). Multivariate Cox analysis indicated that the association between ABD and overall survival was independent of age and leukocyte count on presentation (P = 0.036; HR = 4.25; 95% CI = 1.10-16.42). CONCLUSIONS: The absence of TCRγ deletion is a predictor of a poor response to induction chemotherapy for pediatric patients with T-cell ALL in Taiwan. Providing patients with T-cell ALL and ABD with alternative regimens may be worthwhile to test in future clinical trials. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc.