Congenital Hypofibrinogenemia in a Neonate with a Novel Mutation in the FGB Gene

ArticlePediatric reports. 13(1): 113-117(2021)journal articl

Diagnostic imaging in patients with retinitis pigmentosa

Retinitis pigmentosa (RP) is a progressive inherited retinal disease, and patients with RP have reduced visual function caused by a degeneration of the photoreceptors and retinal pigment epithelium (RPE). At the end stage of RP, the degeneration of the photoreceptors in the fovea reduces central vision, and RP is one of the main causes of acquired blindness in developed countries. Therefore, morphological and functional assessments of the photoreceptors in the macula area can be useful in estimating the residual retinal function in RP patients. Optical coherence tomography (OCT) is a wellestablished method of examining the retinal architecture in situ. The photoreceptor inner/outer segment (IS/OS) junction is observed as a distinct, highly reflective line by OCT. The presence of the IS/OS junction in the OCT images is essential for normal visual function. Fundus autofluorescence (FAF) results from the accumulation of lipofuscin in the RPE cells and has been used to investigate RPE and retinal function. More than one-half of RP patients have an abnormally high density parafoveal FAF ring (AF ring). The AF ring represents the border between functional and dysfunctional retina. In this review, we shall summarize recent progress on diagnostic imaging in eyes with RP

Homemade blenderized tube feeding improves gut microbiome communities in children with enteral nutrition

Enteral nutrition for children is supplied through nasogastric or gastrostomy tubes. Diet not only influences nutritional intake but also interacts with the composition and function of the gut microbiota. Homemade blenderized tube feeding has been administered to children receiving enteral nutrition, in addition to ready-made tube feeding. The purpose of this study was to evaluate the oral/gut microbial communities in children receiving enteral nutrition with or without homemade blenderized tube feeding. Among a total of 30 children, 6 receiving mainly ready-made tube feeding (RTF) and 5 receiving mainly homemade blenderized tube feeding (HBTF) were analyzed in this study. Oral and gut microbiota community profiles were evaluated through 16S rRNA sequencing of saliva and fecal samples. The α-diversity representing the number of observed features, Shannon index, and Chao1 in the gut were significantly increased in HBTF only in the gut microbiome but not in the oral microbiome. In addition, the relative abundances of the phylum Proteobacteria, class Gammaproteobacteria, and genus Escherichia-Shigella were significantly low, whereas that of the genus Ruminococcus was significantly high in the gut of children with HBTF, indicating HBTF altered the gut microbial composition and reducing health risks. Metagenome prediction showed enrichment of carbon fixation pathways in prokaryotes at oral and gut microbiomes in children receiving HBTF. In addition, more complex network structures were observed in the oral cavity and gut in the HBTF group than in the RTF group. In conclusion, HBTF not only provides satisfaction and enjoyment during meals with the family but also alters the gut microbial composition to a healthy state

Support for UNRWA's survival

The United Nations Relief and Works Agency for Palestine Refugees in the Near East (UNRWA) provides life-saving humanitarian aid for 5·4 million Palestine refugees now entering their eighth decade of statelessness and conflict. About a third of Palestine refugees still live in 58 recognised camps. UNRWA operates 702 schools and 144 health centres, some of which are affected by the ongoing humanitarian disasters in Syria and the Gaza Strip. It has dramatically reduced the prevalence of infectious diseases, mortality, and illiteracy. Its social services include rebuilding infrastructure and homes that have been destroyed by conflict and providing cash assistance and micro-finance loans for Palestinians whose rights are curtailed and who are denied the right of return to their homeland

マンガに描かれる女性の体形と日本人若年女性のボディイメージ

日本のマンガの持つ内容の広さ、豊かさ、洗練性および画像表現の自由度は、読む者のボディイメージに強い影響を与える可能性がある。マンガにおける若年女性の体形の描かれ方に関して、ジャンル別(男性向け一女性向け、成人向け-子供向け)の体形の特徴、物語上の役割や人物の性格が体形に及ぼす影響、作者の性別と作品のジャンルの関係について検討した。描かれた身体像は、現代日本の若年女性の標準体形と比較して、若年女子短期大学生らの視点から評価した。男性向けのマンガではやや細いウェストと大きなヒップを持った若年女性が描かれているのに対して、女性向けのマンガではウェスト・ヒップ共に細い若年女性が描かれていた。作品中の若年女性の役割や性格と体形の関係では、女性誌において女性性や母性がいずれも細い身体像と結びつき、また依存性が太いウェストと結びついていた。男性向けのマンガを男性が画いている一方、女性向けのマンガは女性が画いていた。これらの結果から、マンガにおいては非現実的な「やせ理想像」が、女性読者を対象として、女性マンガ家により作られていることが示された

Identification of Subtilase Cytotoxin (SubAB) Receptors Whose Signaling, in Association with SubAB-Induced BiP Cleavage, Is Responsible for Apoptosis in HeLa Cells▿

Subtilase cytotoxin (SubAB), which is produced by certain strains of Shiga-toxigenic Escherichia coli (STEC), causes the 78-kDa glucose-regulated protein (GRP78/BiP) cleavage, followed by induction of endoplasmic reticulum (ER) stress, leading to caspase-dependent apoptosis via mitochondrial membrane damage by Bax/Bak activation. The purpose of the present study was to identify SubAB receptors responsible for HeLa cell death. Four proteins, NG2, α2β1 integrin (ITG), L1 cell adhesion molecule (L1CAM), and hepatocyte growth factor receptor (Met), were identified to be SubAB-binding proteins by immunoprecipitation and purification, followed by liquid chromatography-tandem mass spectrometry analysis. SubAB-induced Bax conformational change, Bax/Bak complex formation, caspase activation, and cell death were decreased in β1 ITG, NG2, and L1CAM small interfering RNA-transfected cells, but unexpectedly, BiP cleavage was still observed. Pretreatment of cells with a function-blocking β1 ITG antibody (monoclonal antibody [MAb] P5D2) enhanced SubAB-induced caspase activation; MAb P5D2 alone had no effect on caspase activation. Furthermore, we found that SubAB induced focal adhesion kinase fragmentation, which was mediated by a proteasome-dependent pathway, and caspase activation was suppressed in the presence of proteasome inhibitor. Thus, β1 ITG serves as a SubAB-binding protein and may interact with SubAB-signaling pathways, leading to cell death. Our results raise the possibility that although BiP cleavage is necessary for SubAB-induced apoptotic cell death, signaling pathways associated with functional SubAB receptors may be required for activation of SubAB-dependent apoptotic pathways

Association between Pet Ownership and Obesity: A Systematic Review and Meta-Analysis

Obesity is a major risk factor for lifestyle-related diseases, including cardiovascular disease, type 2 diabetes, and hypertension. Several studies have investigated the association between pet ownership and obesity, but the findings have been inconsistent. This systematic literature review and meta-analysis assessed the association between pet ownership and obesity. Using PubMed and Scopus, we overviewed the literature published until December 2019 and selected pertinent data for meta-analysis. Two independent reviewers extracted the data. Pooled relative risks (RRs) with 95% confidence intervals (CIs) for obesity were calculated using the random-effects model with inverse-variance weighting. The 21 included articles were cross-sectional studies. Five publications (nine analyses) that reported adjusted RRs for BMI ≥ 25 were included in the meta-analysis. No significant association existed between pet ownership and obesity (pooled RR = 1.038; 95% CI, 0.922–1.167; I2 = 51.8%). After stratification by age group (children vs. adults), no significant association was detected (pooled RR = 0.844; 95% CI, 0.604–1.179; I2 = 64.1% vs. pooled RR = 1.099; 95% CI, 0.997–1.212; I2 = 25.2%). Similarly, no significant association was observed between dog ownership and obesity, indicating no association between pet ownership and obesity. However, no infer causation can be reported because all studies included in this meta-analysis were cross-sectional. Therefore, further prospective studies are needed