Diabetic ketoacidosis in children with new-onset type 1 diabetes mellitus: demographics, risk factors and outcome: an 11 year review in Hong Kong.

Objectives: Diabetic ketoacidosis (DKA) is a life-threatening complication of type 1 diabetes (T1D). The aim of this study is to analyze the incidence, clinical characteristics, management and outcome of children presenting with DKA in new-onset T1D from 2008 to 2018 in Hong Kong.

Methods: Data was extracted from the Hong Kong Childhood Diabetes Registry.

Epidemiology and phenotypes of diabetes in children and adolescents in non-European-origin populations in or from Western Pacific region.

Background: Type 1 diabetes (T1D) incidence varies substantially between countries/ territories, with most studies indicating increasing incidence. In Western Pacific region (WPR), reported rates are much lower than European-origin populations. In contrast, there are reports of substantial numbers of young people with type 2 diabetes (T2D).

Transmembrane 29 , a Newly Identified Molecule Showed Downregulation in Hypoxic-Ischemic Brain Damage.

Transmembrane 29 () gene with unknown function is a gene located on the X chromosome of the mouse genome. The gene showed differential expression in the Vannucci neonatal hypoxic-ischemic mouse brain model. We found the gene expresses with different molecular forms, including a group of long non-coding RNA forming a family of transcripts.

A Multicenter Survey of Type I Diabetes Mellitus in Chinese Children.

Purpose: To investigate the features and treatment status of children with type 1 diabetes mellitus (T1DM) in China.

Methods: We recruited patients <14 years of age with T1DM from 33 medical centers in 25 major cities of China between January 2012 and March 2015. All patients completed a questionnaire that was conducted by their pediatric endocrinologists at all centers.

Incidence and clinical characteristics of pediatric-onset type 2 diabetes in Hong Kong: The Hong Kong childhood diabetes registry 2008 to 2017.

Objective: With increasing prevalence of childhood obesity worldwide, the incidence of pediatric-onset type 2 diabetes (T2D) is also increasing in many countries. We aim to analyze the time trend and incidence of T2D in children in Hong Kong from 2008 to 2017, and to characterize clinical characteristics at diagnosis.

Methods: Data were retrieved from the Hong Kong Childhood Diabetes Registry.

Increasing incidence of type 1 diabetes among Hong Kong children and adolescents: The Hong Kong Childhood Diabetes Registry 2008 to 2017.

Objective: The incidence of childhood-onset type 1 diabetes (T1D) has been reported to be rising but there is also evidence that it has been attenuated in recent years. We described the time trends and the incidence of T1D in children in Hong Kong from 2008 to 2017 and compared with the previous local registry in 1997 to 2007.

Methods: Data were extracted from the Hong Kong Childhood Diabetes Registry, which was established in 2016.

Diagnosis, Genetics, and Therapy of Short Stature in Children: A Growth Hormone Research Society International Perspective.

The Growth Hormone Research Society (GRS) convened a Workshop in March 2019 to evaluate the diagnosis and therapy of short stature in children. Forty-six international experts participated at the invitation of GRS including clinicians, basic scientists, and representatives from regulatory agencies and the pharmaceutical industry. Following plenary presentations addressing the current diagnosis and therapy of short stature in children, breakout groups discussed questions produced in advance by the planning committee and reconvened to share the group reports.

Associations between arterial structure and function and serum levels of liver enzymes in obese adolescents.

Aim: To determine the structural and functional alterations of systemic arteries in obese adolescents and their relationships with adiposity, metabolic and lipid profile, and serum liver enzyme levels.

Methods: Carotid intima-media thickness (IMT), carotid stiffness index, and brachial-ankle pulse wave velocity (baPWV) were measured in 56 obese adolescents and 58 lean controls. Obese adolescents had additional liver ultrasound and determination of fasting blood indices of glucose metabolism and lipid profile, and serum levels of liver enzymes.

The Diagnosis and Management of Lipodystrophy Syndromes: A Multi-Society Practice Guideline.

Objective: Lipodystrophy syndromes are extremely rare disorders of deficient body fat associated with potentially serious metabolic complications, including diabetes, hypertriglyceridemia, and steatohepatitis. Due to their rarity, most clinicians are not familiar with their diagnosis and management. This practice guideline summarizes the diagnosis and management of lipodystrophy syndromes not associated with HIV or injectable drugs.

Myocardial Integrated Backscatter in Obese Adolescents: Associations with Measures of Adiposity and Left Ventricular Deformation.

Background: Myocardial fibrosis has been proposed to play an important pathogenetic role in left ventricular (LV) dysfunction in obesity. This study tested the hypothesis that calibrated integrated backscatter (cIB) as a marker of myocardial fibrosis is altered in obese adolescents and explored its associations with adiposity, LV myocardial deformation, and metabolic parameters.

Methods/principal Findings: Fifty-two obese adolescents and 38 non-obese controls were studied with conventional and speckle tracking echocardiography.

The clinical significance of aldosterone synthase deficiency: report of a novel mutation in the CYP11B2 gene.

Background: Aldosterone synthase (CYP11B2) deficiency is a rare autosomal recessive disorder, usually presenting with severe salt-wasting in infancy or stress-induced hyperkalaemia and postural hypotension in adulthood. Neonatal screening for congenital adrenal hyperplasia, another cause of salt wasting, using 17-hydroxyprogesterone measurement would fail to detect aldosterone synthase deficiency, a diagnosis which may be missed until the patient presents with salt-wasting crisis. Due to this potential life-threatening risk, comprehensive hormonal investigation followed by genetic confirmation for suspected patients would facilitate clinical management of the patient and assessment of the genetic implication in their offspring.

Diagnosis of 5α-reductase 2 deficiency: is measurement of dihydrotestosterone essential?

Background: 5α-Reductase 2 deficiency (5ARD) is a known cause of 46,XY disorders of sex development (DSD). Traditionally, the diagnosis relies on dihydrotestosterone (DHT) measurement, but the results are often equivocal, potentially leading to misdiagnosis. We reviewed alternative approaches for diagnosis of 5ARD.

Management of massive osteolysis of the mandible: a case report.

The management of a previously reported case of massive osteolysis of the mandible with intravenous bisphosphonate, vascularized free bone graft, and dental implants is reported. Restoration of adequate cosmetics and masticatory function was achieved. Despite the use of bisphosphonates, there were no complications with osseointegration.

Late measures of microstructural alterations in severe neonatal hypoxic-ischemic encephalopathy by MR diffusion tensor imaging.

Neonatal hypoxic-ischemic encephalopathy is a major cause of brain damage in infants, and is associated with periventricular white matter injury and chronic neurological dysfunctions. However, the mechanisms of the chronic white matter injury and reorganization are still unclear. In this study, in vivo diffusion tensor imaging (DTI) was employed to evaluate the late changes of white matter microstructural integrity in the rat brains at 10 weeks after severe neonatal hypoxic-ischemic insults at postnatal day 7.

Characterization of white matter injury in a hypoxic-ischemic neonatal rat model by diffusion tensor MRI.

Background And Purpose: We evaluate white matter (WM) injury after hypoxic-ischemic (HI) insult in a neonatal rat model using diffusion tensor imaging (DTI) to determine whether lambda(parallel) and lambda(perpendicular) are able to characterize type and severity of brain damage.

Methods: Eighteen 7-day-old Sprague-Dawley rats underwent unilateral ligation of left common carotid artery followed by 50 minutes (n=9) or 90 minutes (n=9) of hypoxia at 37 degrees C. Rats were divided into 2 groups, according to absence (group A, n=11) or presence (group B, n=7) of cystic lesions on D7 post-HI T2-weighted imaging.

Proteomic analysis of neonatal mouse brain: evidence for hypoxia- and ischemia-induced dephosphorylation of collapsin response mediator proteins.

Perinatal hypoxia and ischemia (HI) are a significant cause of mortality and morbidity. To understand the molecular mechanisms for HI-induced brain damage, here we used a proteomic approach to analyze the alteration and modification of proteins in neonatal mouse brain 24 h after HI treatment. Significant changes of collapsin response mediator proteins (CRMPs) were observed in HI brain.

Manganese-enhanced MRI detection of neurodegeneration in neonatal hypoxic-ischemic cerebral injury.

In this study, we investigated the Mn-enhanced MRI (MEMRI) for detecting neurodegenerative processes in neonatal hypoxic-ischemic (H-I) cerebral injury. Seven-day-old rats were induced with H-I injury, and scanned for T1-weighted image (T1WI) and T2-weighted image (T2WI) with and without systemic MnCl2 administration. Serial histological analysis was performed for Mn-superoxide dismutase (Mn-SOD) and glutamine synthetase (GS), which are Mn-binding enzymes against the oxidative stress and glutamate excitotoxicity in neurodegeneration.

A 3D collagen microsphere culture system for GDNF-secreting HEK293 cells with enhanced protein productivity.

Mammalian cell culture technology has been used for decades in mass production of therapeutic proteins. However, unrestricted cell proliferation usually results in low-protein productivity. Controlled proliferation technologies such as metabolism intervention and genetic manipulation are therefore applied to enhance the productivity.

Comparing diffusion-weighted and T2-weighted MR imaging for the quantification of infarct size in a neonatal rat hypoxic-ischemic model at 24h post-injury.

Purpose: In a neonatal rat model of hypoxic-ischemic (HI) brain injury, using T2-weighted imaging (T2WI) and diffusion-weighted imaging (DWI), we aim to determine the best MRI method of lesion quantification that reflects infarct size.

Materials And Methods: Twenty 7-day-old rats underwent MRI 24h after HI brain injury was induced. Lesion size relative to whole brain was measured using T2WI and apparent diffusion coefficient (ADC) maps, applying thresholds of 60%, 70% and 80% contralateral control hemisphere mean ADC, and at day 10 post-HI on pathology with TTC staining.

Therapeutic expression of an anti-death receptor 5 single-chain fixed-variable region prevents tumor growth in mice.

The clinical use of the single-chain fixed-variable (scFv) fragments of recombinant monoclonal antibodies as credible alternatives for classic therapeutic antibodies has two limitations: rapid blood clearance and inefficient local expression of functional molecules. In attempt to address these issues, we have developed a novel gene therapy protocol in which the anti-death receptor 5 (DR5) scFv fragments were either in vitro expressed in several tumor cell lines, or in vivo expressed in mice, using recombinant adeno-associated virus (rAAV)-mediated gene transfer. Viral transduction using the rAAV-S3C construct, which encodes a scFv molecule (S3C scFv) specific to DR5, led to stable expression in tumor cell lines and showed apoptosis-inducing activity in vitro, which could be inhibited by recombinant DR5 but not by DR4.

Cytoglobin overexpression protects against damage-induced fibrosis.

Cytoglobin (Cygb), a member of the hexacoordinate globin superfamily (hxHb), is expressed in fibroblasts from a broad range of tissues. The physiological functions of hxHb are still unclear, but biochemical studies reveal that they can scavenge toxic species, such as nitric oxide, peroxynitrite, and hydrogen peroxide. We demonstrate that the overexpression of Cygb in rat hepatic stellate cells, both in vitro and in vivo, protects against oxidative stress, inhibiting their differentiation into a myofibroblast-like phenotype.

[Expression of NMDA receptor-1 induced by NMDA in the brain of neonatal SD rats].

Objective: To determine the regulation of the expression of NMDA receptor-1 induced by NMDA in the brain of neonatal SD rats.

Methods: Neonatal SD rats (n=90) were randomly divided into normal control (n=6) and NMDA injected group (subdivided into 10 nmol-0 min, 15 min, 30 min, 1 h, 2 h, 4 h groups, and 10, 20, 50 nmol groups, each n=6). NMDA fluorescent inmmunohistological staining and TTC (2,3,5-triphenyltetrazoliun chloride) staining techniques were used.

Severe hyperbilirubinaemia in a Chinese girl with type I Crigler-Najjar syndrome: first case ever reported in Mainland China.

Jaundice is common in ethnic Chinese infants, but to our knowledge Crigler-Najjar syndrome (CN syndrome) type I has never been reported in China. A Chinese girl with severe jaundice was recently diagnosed to have CN syndrome type I by analyzing the bilirubin-uridinediphospho (UDP)-glucuronosyltransferase gene (UGT1A1). The patient was homozygous for a nonsense mutation that replaced glutamine (CAG, amino acid 239) with stop codon (TAG) at nucleotide number 715 (715C-->T) in exon 1.