Pkhd1 mice have altered renal Pkhd1 mRNA processing and hormonally sensitive liver disease.

Autosomal-recessive polycystic kidney disease (ARPKD; MIM #263200) is a severe, hereditary, hepato-renal fibrocystic disorder that causes early childhood morbidity and mortality. Mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene, which encodes the protein fibrocystin/polyductin complex (FPC), cause all typical forms of ARPKD. Several mouse lines carrying diverse, genetically engineered disruptions in the orthologous Pkhd1 gene have been generated, but none expresses the classic ARPKD renal phenotype.

Nomogram prediction for cervical lymph node metastasis in multifocal papillary thyroid microcarcinoma.

Aim: Accurate preoperative prediction of cervical lymph node metastasis (LNM) in patients with mPTMC provides a basis for surgical decision making and the extent of tumor resection. This study aimed to develop and validate an ultrasound radiomics nomogram for the preoperative assessment of LN status.

Methods: A total of 450 patients pathologically diagnosed with mPTMC were enrolled, including 348 patients in the modeling group and 102 patients in the validation group.

Prevalence and correlates of poor sleep quality among college students: a cross-sectional survey.

Background: Sleep problems are widespread among college students around the globe, especially in China. This study was designed to investigate the prevalence of poor sleep quality and identify associated factors among college students in Jilin Province, China.

Methods: A total of 6284 participants were completely collected by stratified cluster sampling in 2016.

Comparison of Wide Margin and Inadequate Margin for Recurrence in Sacral Chordoma: A Meta-Analysis.

Study Design: .: A meta-analysis.

Objective: .

Accelerated Hyperfractionated Radiotherapy versus Conventional Fractionation Radiotherapy for Head and Neck Cancer: A Meta-Analysis of Randomized Controlled Trials.

Objective: The benefits of accelerated hyperfractionated radiotherapy (HART) and conventional fractionation radiotherapy (CFRT) in the treatment of head and neck cancer (HNC) remain controversial. In this study, we analyzed the therapeutic effects of these two treatment regimens to explore whether HART can improve the overall survival (OS) rate and locoregional control (LRC) rate in patients with HNC.

Methods: The PubMed, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched for eligible studies.

The diagnostic performance of PET/CT scans for the detection of para-aortic metastatic lymph nodes in patients with cervical cancer: A meta-analysis.

Objective: We performed a meta-analysis to evaluate the diagnostic value of positron emission tomography/computed tomography (PET/CT) in the detection of para-aortic lymph node metastasis in cervical cancer.

Methods: We searched the PubMed, Embase, Web of Science, Cochrane Library, Chinese Biological Medicine (CBM), Chinese National Knowledge Infrastructure (CNKI), Wanfang and VIP databases in all languages from their inception to September 2018. Stat15.

Trends in Diabetes Mortality by Gender in Urban and Rural Areas in China From 2003 to 2012: An Age-Period-Cohort Analysis.

We aimed to provide updated estimates for the trends and the effects attributable to age, period, and cohort by gender in urban and rural areas in China. The data were based on the vital registration system in China from 2003 to 2012. The annual percentage change used Joinpoint Regression Analysis.

Functional polymorphisms of the APOA1/C3/A4/A5-ZPR1-BUD13 gene cluster are associated with dyslipidemia in a sex-specific pattern.

Background: Dyslipidemia contributes to the risk of many diseases, including stroke, cardiovascular disease and metabolic-related diseases. Previous studies have indicated that single nucleotide polymorphisms (SNPs) are associated with different levels of serum lipid. Therefore, this study explored the relationship between the gene cluster gene polymorphisms and dyslipidemia in the total sample population and stratified by genders in a northeast Chinese population.

Dosimetric comparison of volumetric-modulated arc therapy and intensity-modulated radiation therapy in patients with cervical cancer: a meta-analysis.

Background: Intensity-modulated radiation therapy (IMRT) and volumetric-modulated arc therapy (VMAT) are two of the main treatment techniques for cervical cancer. Whether either technique significantly reduces irradiated volumes of organs at risk (OARs) remains controversial. The aim of this study was to explore which of these treatment paradigms is the superior technique in cervical treatment, taking clinical outcomes and treatment efficiency from published findings into consideration.

Association Between Schizophrenia and DNA Demethylase Activity in Human Peripheral Blood Mononuclear Cells.

Background: DNA demethylase is a crucial enzyme in the epigenetic modification and regulation mechanisms of gene transcription. Based on previous assertions that the pathophysiology of schizophrenia is associated with epigenetics, we aimed to explore whether DNA demethylase activity might be related to schizophrenia in northeast China.

Methods: We recruited 25 patients with first-episode schizophrenia and 29 normal controls from a northeast Chinese Han population.

DICER1-related Sertoli-Leydig cell tumor and gynandroblastoma: Clinical and genetic findings from the International Ovarian and Testicular Stromal Tumor Registry.

Background: Ovarian sex cord-stromal tumors (OSCST) include juvenile granulosa cell tumors (JGCT), Sertoli-Leydig cell tumor (SLCT) and gynandroblastoma (GAB) among others. These ovarian sex cord-stromal tumors as well as other tumors including pleuropulmonary blastoma (PPB) may be associated with DICER1 mutations. We sought to describe the clinical and genetic findings from the first 107 individuals enrolled in the International Ovarian and Testicular Stromal Tumor Registry.

Placental Pathology of Zika Virus: Viral Infection of the Placenta Induces Villous Stromal Macrophage (Hofbauer Cell) Proliferation and Hyperplasia.

Context: -The placenta is an important component in understanding the fetal response to intrauterine Zika virus infection, but the pathologic changes in this organ remain largely unknown. Hofbauer cells are fetal-derived macrophages normally present in the chorionic villous stroma. They have been implicated in a variety of physiological and pathologic processes, in particular involving infectious agents.

Temporal order of RNase IIIb and loss-of-function mutations during development determines phenotype in pleuropulmonary blastoma / syndrome: a unique variant of the two-hit tumor suppression model.

Pleuropulmonary blastoma (PPB) is the most frequent pediatric lung tumor and often the first indication of a pleiotropic cancer predisposition,   syndrome, comprising a range of other individually rare, benign and malignant tumors of childhood and early adulthood. The genetics of  -associated tumorigenesis are unusual in that tumors typically bear neomorphic missense mutations at one of five specific "hotspot" codons within the RNase IIIb domain of  , combined with complete loss of function (LOF) in the other allele. We analyzed a cohort of 124 PPB children for predisposing   mutations and sought correlations with clinical phenotypes.

Patterning of ventral telencephalon requires positive function of Gli transcription factors.

The ability of neuroepithelial cells to generate a diverse array of neurons is influenced by locally secreted signals. In the spinal cord, Sonic Hedgehog (Shh) is known to induce distinct cell fates in a concentration-dependent manner by regulating the activities of the three Gli transcription factors in neural precursors. However, whether Gli-mediated Shh signaling is also required to induce different cell types in the ventral telencephalon has been controversial.

Wnt signaling determines ventral spinal cord cell fates in a time-dependent manner.

The identity of distinct cell types in the ventral neural tube is generally believed to be specified by sonic hedgehog (Shh) in a concentration-dependent manner. However, recent studies have questioned whether Shh is the sole signaling molecule determining ventral neuronal cell fates. Here we provide evidence that canonical Wnt signaling is involved in the generation of different cell types in the ventral spinal cord.

Segregation of Nogo66 receptors into lipid rafts in rat brain and inhibition of Nogo66 signaling by cholesterol depletion.

NogoA, a myelin-associated component, inhibits neurite outgrowth. Nogo66, a portion of NogoA, binds to Nogo66 receptor (NgR) and induces the inhibitory signaling. LINGO-1 and p75 neurotrophin receptor (p75), the low-affinity nerve growth factor receptor, are also required for NogoA signaling.