[Treatment of ornithine transcarbamylase deficiency in a child with glyceryl phenylbutyrate].

Glyceryl phenylbutyrate (GPB) serves as a long-term management medication for Ornithine transcarbamylase deficiency (OTCD), effectively controlling hyperammonemia, but there is a lack of experience in using this medicine in China. This article retrospectively analyzes the case of a child diagnosed with OTCD at Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, including a review of related literature. After diagnosis, the patient was treated with GPB, followed by efficacy follow-up and pharmacological monitoring.

A randomized double-blind clinical trial: Comparison of oclacitinib with a traditional Chinese herbal medicine product (Dihuang Guiqin capsule) in the treatment of canine atopic dermatitis.

Canine atopic dermatitis (cAD) is a common chronic inflammatory skin disease, which seriously affects the quality of life for both dogs and their owners. Currently, the common therapeutic drugs in the clinic have disadvantages such as obvious adverse effects and high prices. Traditional Chinese herbal medicine (TCHM) has great potential for the treatment of cAD.

Ji-Chuan decoction ameliorates slow transit constipation regulation of intestinal glial cell apoptosis.

Background: Slow transit constipation (STC) is a common intestinal disease with increasing incidence. STC results from various factors, such as the enteric nervous system and metabolic changes. As a classical formula of traditional Chinese medicine, Ji-Chuan decoction (JCD) has been extensively and effectively used in STC treatment, yet its pharmacological mechanism remains unclear.

Kabuki syndrome: review of the clinical features, diagnosis and epigenetic mechanisms.

Background: Kabuki syndrome (KS), is a infrequent inherited malformation syndrome caused by mutations in a H3 lysine 4 methylase (KMT2D) or an X-linked histone H3 lysine 27 demethylase (UTX/KDM6A). The characteristics in patients with KS have not yet been well recognized.

Data Sources: We used databases including PubMed and Google Scholar to search for publications about the clinical features and the etiology of Kabuki syndrome.

Two New Cytotoxic Compounds from a Deep-Sea XT20-134.

XT20-134 (MCCC 3A00956) is a fungus with cytotoxic activity, derived from deep-sea sediment. Five new compounds, adeninylpyrenocine (), 2-hydroxyl-3-pyrenocine-thio propanoic acid (), ozazino--(2,3-dihydroxyl-trp-tyr) (), 5,5-dichloro-1-(3,5-dimethoxyphenyl)-1,4-dihydroxypentan-2-one (), and 2,3,4-trihydroxybutyl cinnamate (), together with 19 known compounds (-), were isolated from an ethyl acetate (EtOAc) extract of its fermentation. The structures of the new compounds were comprehensively characterized by high-resolution electrospray ionization-mass spectrometry (HR-ESI-MS), 1D and 2D nuclear magnetic resonance (NMR).

Development of an on-line mixed-mode gel liquid chromatography×reversed phase liquid chromatography method for separation of water extract from Flos Carthami.

A novel on-line comprehensive two-dimensional liquid chromatography (2D-LC) method by coupling mixed-mode gel liquid chromatography (MMG-LC) with reversed phase liquid chromatography (RPLC) was developed. A mixture of 17 reference compounds was used to study the separation mechanism. A crude water extract of Flos Carthami was applied to evaluate the performance of the novel 2D-LC system.

[Endocrine and metabolic features of female children with Prader-Willi syndrome: an analysis of 4 cases].

This article reports the clinical features and endocrine and metabolic features of 4 children with Prader-Willi syndrome (PWS). All the patients were female and aged 6-12 years at diagnosis. All of them had clinical manifestations of obesity, unusual facies, developmental retardation, and intellectual disability.

A novel nomogram based on LODDS to predict the prognosis of epithelial ovarian cancer.

Background: To develop and validate a nomogram based on log of odds between the number of positive lymph node and the number of negative lymph node (LODDS) in predicting the overall survival (OS) and cancer specific survival (CSS) for epithelial ovarian cancer (EOC) patients.

Materials And Methods: A total of 10,692 post-operative EOC patients diagnosed between 2004 and 2013 were obtained from the Surveillance, Epidemiology, and End Results (SEER) database and randomly divided into training (n = 7,021) and validation (n = 3,671) cohorts. Multiple clinical pathological parameters were assessed and compared with outcomes.

A high-fat high-energy diet influences hepatic CYP3A expression and activity in low-birth-weight developing female rats.

Background: The objective of this study was to investigate the effects of a high-fat, high-energy (HFHE) diet on the hepatic expression of CYP3A in low-birthweight developing female rats.

Methods: Pregnant rats were divided into nourished and undernourished groups. The offspring of the nourished rats were defined as the normal-birth-weight (NBW) group, and those of undernourished rats were defined as the low-birth-weight (LBW) group.

Cord blood C-peptide, insulin, HbA1c, and lipids levels in small- and large-for-gestational-age newborns.

Background: Small- and large-for-gestational-age (SGA, LGA) newborns are associated with metabolic syndrome in their later life. Cord blood C-peptide, insulin, glycosylated hemoglobin (HbA1c), and lipids levels may be altered in SGA and LGA newborns; however, the results are conflicting. Therefore, this study aimed to determine the effect of cord blood markers on SGA and LGA newborns.

Effect of maternal lipid profile, C-peptide, insulin, and HBA1c levels during late pregnancy on large-for-gestational age newborns.

Background: Large-for-gestational age (LGA) newborns can increase the risk of metabolic syndrome. Previous studies have shown that the levels of maternal blood lipids, connecting peptide (C-peptide), insulin and glycosylated hemoglobin (HbA1c) were significantly different between LGA and appropriate-for-gestational age (AGA) newborns. This study aimed to determine the effect of the levels of maternal lipids, C-peptide, insulin, and HbA1c during late pregnancy on LGA newborns.

Hepatic CYP3A expression and activity in low birth weight developing female rats.

Background: We aimed to investigate the effects of low birth weight (LBW) on the hepatic expression of cytochrome P-450 3A (CYP3A) in developing female rats.

Methods: Pregnant rats were divided into two groups, a nourished group and an under-nourished group. The offspring of the nourished rats were defined as a normal weight, normal diet group (NN group).

Early life programming and metabolic syndrome.

Metabolic syndrome (MS) has reached epidemic proportions worldwide among children. Early life "programming" is now thought to be important in the etiology of obesity, type 2 diabetes, cardiovascular disease and MS. Nutritional imbalance and exposures to endocrine disruptor chemicals during development can increase risk for MS later in life.

Scalp acupuncture for acute ischemic stroke: a meta-analysis of randomized controlled trials.

Scalp acupuncture (SA) is a commonly used therapeutic approach for stroke throughout China and elsewhere in the world. The objective of this study was to assess clinical efficacy and safety of SA for acute ischemic stroke. A systematical literature search of 6 databases was conducted to identify randomized controlled trials (RCTs) of SA for acute ischemic stroke compared with western conventional medicines (WCMs).

Reversible cataract as the presenting sign of diabetes mellitus: report of two cases and literature review.

Background: Type 1 diabetes mellitus (T1DM) is the most common form of diabetes in young children. Serious optic complications, e.g.

[Detection of thyroid antibodies in children with type 1 diabetes mellitus].

Objective: To investigate the prevalence of positive thyroid antibodies in children with type 1 diabetes mellitus (T1DM) and its influencing factors.

Methods: The clinical data of T1DM children who were treated in the Children's Hospital of Zhejiang University from May 2005 to April 2011 were retrospectively studied. The relationships of thyroid globulin antibody (TGAb) and thyroid peroxidase antibody (TPOAb) with cytokines IL-2, IL-4, IL-6, IL-10, TNF and IFN-γ were evaluated, and the percentages of CD3+, CD4+ and CD8+ T-lymphocytes in peripheral blood were examined.

Metabolism of dl-praeruptorin A in rat liver microsomes using HPLC-electrospray ionization tandem mass spectrometry.

dl-Praeruptorin A (Pd-Ia) is the major active constituent of the traditional Chinese medicine Peucedanum praeruptorum Dunn. Recently it has been identified as a novel agent in the treatment and prevention of cardiovascular diseases. Accordingly, we investigated the metabolism of Pd-Ia in rat liver microsomes.

[The prevalence of type 2 diabetes mellitus and prediabetes in children].

Objective: To investigate the incident and prevalence of type 2 diabetes mellitus (T2DM) and prediabetes in obese children in the last ten years.

Methods: The clinical data of hospitalized children with newly diagnosed diabetes mellitus (DM) or obesity between October 2000 and September 2011 were retrospectively studied.

Results: A total of 503 newly onset cases were diagnosed as DM in the past ten years, of which 31 were diagnosed as T2DM.

Ginseng total saponins enhance neurogenesis after focal cerebral ischemia.

Ethnopharmacological Relevance: Ginseng, the root of Panax ginseng C.A. Meyer, is one of the most commonly used healing herbs for stroke and chronic debilitating conditions in China.

The levels of Pdx1/insulin, Cacna1c and Cacna1d, and β-cell mass in a rat model of intrauterine undernutrition.

Objective: To elucidate the underlying mechanisms responsible for β-cell mass and function in response to intrauterine growth restriction (IUGR).

Methods: Offspring from undernourished mother rats with birth weight < -2 SD (IUGR group) and from standard-nourished rats with birth weight between mean +/- 1SD normal birth weight (NBW group) were examined. Levels of fasting glucose, serum insulin, and insulin-like growth factor-1 (IGF-1) were analyzed.

[Genetic mutation analysis of a kindred with a patient suffering from 17 alpha-hydroxylase/17, 20-lyase deficiency].

Objective: To detect CYP17A1 gene mutation in a patient with 17 alpha-hydroxylase/17, 20-lyase deficiency and her family members.

Method: Genomic DNA was extracted from the blood of the patient, her parents and twin sister. The 8 exons of CYP17A1 gene were amplified with polymerase chain reaction (PCR) and screened for mutations by sequencing.

Tau as a potential novel therapeutic target in ischemic stroke.

Stroke is associated with high mortality and major disability burdens worldwide, but there are few effective and widely available therapies. Tau plays an important role in promoting microtubule assembly and stabilizing microtubule networks with phosphorylation regulating these functions. Based on the "ischemia-reperfusion theory" of Alzheimer's disease, some previous studies have focused on the relationship of tau and Alzheimer lesions in experimental brain ischemia.

Long-time course of protease-activated receptor-1 expression after intracerebral hemorrhage in rats.

Thrombin plays an important role in brain injuries associated with intracerebral hemorrhage (ICH). The protease-activated receptor (PAR)-1 is responsible for the vast majority of the thrombin's cellular activation functions. We tested the hypothesis that thrombin-induced brain damage after ICH, at least in part, is mediated by PAR-1.

Neurogenic hypothesis of cardiac ischemic pain.

Coronary heart disease is the major cause of death worldwide and is affecting millions of people in both developed and developing countries. Patients with myocardial ischemia typically experience chest pain (angina pectoris). Traditional viewpoint of ischemic cardiac pain might be related to "mechanical hypothesis" in early time and "chemical hypothesis" in modern time.