22q11.2 deletion syndrome complicated with pulmonary alveolar proteinosis in a child: a case report.

BACKGROUND: To analyze the clinical data and next generation sequencing (NGS) results from a child with 22q11.2 deletion syndrome (22q11DS) complicated with pulmonary alveolar proteinosis (PAP) who was admitted to the Department of Pediatrics of Fuyang People's Hospital and to present a review of the literature. CASE PRESENTATION: A 9-year-old male child, whose face had a small mandible and high-arched palate, but lacked a cleft palate, had repeated respiratory tract infections and bronchiectasis. Clinical examination, computer tomography, and electronic bronchoscopy were performed. Genetic testing via NGS was undertaken. PAP was confirmed by Periodic Acid Schiff staining of milky white alveolar lavage fluid isolated by electronic bronchoscopy. A deletion of approximately 2.46 Mbp on chromosome 22q11.2 was confirmed by NGS. During hospitalization, anti-infection, nebulization, alveolar lavage, and regular application of thymosin were administered to the patient. The condition of the patient stabilized following treatment. CONCLUSIONS: 22q11DS and PAP are both rare diseases, and the manifestation of 22q11DS combined with PAP has not been previously reported. The diagnosis and treatment of this case will be a reference for future clinical work.

CXCL-10/CXCR3 in macrophages regulates tissue repair by controlling the expression of Arg1, VEGFa and TNFα.

Macrophages have been reported to participate in inflammation, tissue homeostasis and tissue repair. The detailed mechanism of macrophage-mediated tissue repair is not clear. CXCL-10, secreted by monocytes, endothelial cells and fibroblasts, mediates immune response and angiogenesis by binding to CXCR3. In this study, the expression of CXCL-10 and CXCR3 in porcine lung injury induced by porcine reproductive and respiratory syndrome virus (PRRSV) infection was firstly examined. The results showed that the expression of both CXCL-10 and CXCR3 increased in the infected pig lungs. In addition, the increased expression of CXCL-10 and CXCR3 in macrophage treated by poly (I:C) was also observed, suggesting the autocrine system existed in macrophages. Furthermore, CXCL-10 treatment induced upregulation of Arg1 and VEGFa, and downregulation of TNFα in macrophage, and CXCR3 antagonist AMG487 treatment presented the contrary effects on the expression of Arg1, VEGFa, and TNFα. CXCL- 10-stimulated effects were dependent on PI3K/Akt signaling pathway. Wound-healing assay showed that CXCL-10 treatment macrophage conditioned medium promoted the healing process of endothelial cells. Our results suggested that CXCL-10/CXCR3 in macrophage may mediate tissue repair by regulating the macrophage expression of Arg1, VEGFa and TNFα. Modulation of CXCL-10/CXCR3 axis in macrophage may be a potential therapeutic strategy for tissue injury and repair.

Hydrodynamic and Sediment Responses of Open Channels to Exposed Pipe Encasements.

The effects of exposed pipe encasements on the local variation of hydrodynamic and sediment conditions in a river channel are examined. Laboratory experiments are performed to assess the response of water level, flow regime and bed deformation to several representative types of concrete encasements. The experimental conditions considered are: three types of exposed pipe encasements exposed on the bed, including trapezoidal shape, circular-arc shape and polygonal shape, and three sets of discharges, including annual discharge, once-in-3-year flood, and once-in-50-year flood. Our experiments show that: (1) the amount of backwater definitely depends on the encasement geometric shape and the background discharge; (2) smaller discharges generally tend to induce local scour of river bed downstream of the encasement, and the order of sensitivity of bed deformation to the encasement geometric shape is trapezoidal > circular-arc > polygonal; (3) comparatively speaking, the polygonal encasement may be considered as a suitable protective structure for pipelines across alluvial rivers, with relatively modest effects on the local hydrodynamic conditions and bed stabilization.

Solexa sequencing of microRNAs on chromium metabolism in broiler chicks.

AIM: The aim of this study was to determine the effect of chromium picolinate (CrPic) on the differential expression of the known microRNAs (miRNAs) in broiler skeletal muscle. METHODS AND RESULTS: A total of 288 1-day-old male Arbor Acres broilers were randomly assigned to one of four dietary treatments supplemented with 0, 0.4, 2.0, or 10.0 mg·kg(-1) CrPic, respectively. Dietary CrPic supplementation at 10.0 mg·kg(-1) increased the average daily feed intake in broilers (p < 0.05). On day 42, the serum total protein level was highest in animals treated with 2.0 mg·kg(-1) (p < 0.05) and 10.0 mg·kg(-1) CrPic (p < 0.05). Dietary supplementation with 10.0 mg·kg(-1) CrPic decreased the levels of serum glucose (p < 0.05) on day 42 and of serum triglyceride (p < 0.05) on days 21 and 42. To further identify miRNAs from broiler skeletal muscles, we sequenced two small RNA libraries using the Solexa sequencing approach, and 57 miRNAs were found to be significantly differentially expressed (p < 0.05). Among them, 6 upregulated and 2 downregulated miRNAs were validated by real-time qPCR (p < 0.05). CONCLUSIONS: The results of the present study provide a valuable clue regarding the role of miRNA target genes in the mechanism of the dietary CrPic effect on protein synthesis in skeletal muscles of broilers.

Distribution of sorbed phenanthrene and pyrene in different humic fractions of soils and importance of humin.

Contributions of fulvic-humic acids (FA/HA) and humin (HM) to sorption of phenanthrene (PHE) and pyrene (PYR) in a soil were differentiated using a humic separation procedure after multi-concentration sorption experiments. It was found that the amount of solutes in FA/HA did not change significantly after 48 h, while that in HM increased continuously and slowly up to the end of the experimental period (720 h), indicating that HM was the main region for slow sorption. Based on the fitting results using Freundlich equation, it was found that nonlinearity of both solutes was greater in HM than in FA/HA, consistent with the sorption characteristics of individually extracted HA and HM in a separate experiment. The observed nonlinearity of the solute distribution was confirmed by using three other soil samples with organic carbon contents ranging from 0.7 to 7.9%. Distribution dynamics of PHE and PYR among various fractions were also discussed.

Electrochemical studies of the effect of lanthanide ions on the activity of glutamate dehydrogenase.

Using the electrochemical method based on the "diffusion" layer concept of the convective system, the effect of lanthanide ions La3+ and Eu3+ on the activity of glutamate dehydrogenase (GLDH) has been determined. In suitable concentrations, the lanthanide ions La3+ and Eu3+ can activate the GLDH in the reductive amination of alpha-Ketoglutarate (alpha-KG). The activation mechanism would be that the lanthanide ions could interrupt the binding of NAD+ to GLDH by combining preferentially to NAD+. This mechanism was proposed by the voltammetric studies.

Electrochemical study of the effect of ADP and AMP on the kinetics of glutamate dehydrogenase.

A chronoamperometric method based on the 'diffusion' layer concept of the convective system was used to assay the glutamate dehydrogenase (GLDH) activity. Once the reaction was initiated by adding the enzyme GLDH into a well-stirred nicotinamide adenine dinucleotide (NADH, coenzyme) solution, the steady-state oxidation limiting current of NADH would decrease linearly in a short time. The major advantage of this method is that it directly indicates the continuous in-situ change of the coenzyme concentration, thus, the real initial reaction rate of enzyme-catalyzed reaction, V0, can be determined. Using this method, the effect of adenosine-5'-monophosphate (AMP) and adenosine-5'-diphosphate (ADP) on the GLDH activity has been monitored. The results showed that ADP and AMP could increase the activity of GLDH. This activation mechanism was proposed by the voltammetric study.

Surgical resection for recurrent hepatocellular carcinoma: prognosis and analysis of risk factors.

BACKGROUND: Hepatocellular carcinoma (HCC) is common in Asian countries, and tumor recurrence is the most common cause of treatment failure after curative resection. Repeated hepatectomy is performed only for selected patients because most patients with HCC also have liver cirrhosis and poor liver function reserve. The purposes of this study were to clarify the outcome of the patients after second hepatectomy for recurrent HCC and to evaluate the prognostic factors after second hepatectomy. METHODS: We used retrospective cohort study to examine the disease-free survival, cumulative survival, and possible prognostic factors for recurrence and death in 59 patients who underwent surgical resection for recurrent HCC at the National Taiwan University Hospital from August 1986 to December 1993. Another 64 patients with unresectable recurrent HCC were used as a historical control group. The survival curves between those patients with resectable HCC and those with unresectable HCC were compared. RESULTS: After resection for recurrent HCC, gender and multiplicity (n > 3) of tumor affect recurrence rate (p = 0.046 and 0.021, respectively), whereas gender, age, and tumor invasiveness affect survival rate significantly (p = 0.024, 0.021, and 0.046, respectively). The survival rate of patients with resectable HCC was significantly better than that of those with unresectable HCC. CONCLUSIONS: For recurrent HCC surgical resection is an effective mode of treatment in selected patients. Whether surgery is better than other modes of treatment in the treatment of resectable recurrent HCC demands further investigation.

A high sensitivity LiF thermoluminescent dosimeter--LiF(Mg, Cu, P).

This paper describes the dosimetric properties of a new, high-sensitivity thermoluminescence dosimeter material prepared in our laboratory. The sensitivity of the LiF(Mg, Cu, P) phosphor is about 23 times higher than that of LiF (TLD-100). Its TL response is linear from 0.3 mR to 1000 R. Its energy dependence is similar to that of LiF (TLD-100). It is stable and reusable without significant loss of sensitivity. Its application to environmental radiation monitoring is described.