Monitoring Adherence Rate to Growth Hormone Therapy and Growth Outcomes in Taiwanese Children Using Easypod Connect: Observational Study.

Background: Adherence to growth hormone therapy is difficult to detect reliably. Devices such as easypod have been developed for electronic recording of injections. The easypod connect observational study (ECOS) was an open-label, observational, multinational, phase IV study conducted in 24 countries around the world.

Aortic Root Dilatation in Taiwanese Patients with Mucopolysaccharidoses and the Long-Term Effects of Enzyme Replacement Therapy.

Background: Cardiovascular abnormalities have been observed in patients with mucopolysaccharidosis (MPS) of any type, with the most documented abnormalities being valvular regurgitation and stenosis and cardiac hypertrophy. Only a few studies have focused on aortic root dilatation and the long-term effects of enzyme replacement therapy (ERT) in these patients.

Methods: We reviewed echocardiograms of 125 Taiwanese MPS patients (age range, 0.

A Pilot Study of Two Different Constraint-Induced Movement Therapy Interventions in Children With Hemiplegic Cerebral Palsy After Botulinum Toxin Injection During Preschool Education.

To establish a pilot study on applying two low dose (40 h) constraint-induced movement therapy (CIMT) interventions in children with hemiplegic cerebral palsy (CP) after botulinum toxin (BoNT-A) injection during preschool education. Five children with spastic CP (mean age: 5.31 years; Gross Motor Function Classification System level I and II) undergoing regular BoNT-A injections and rehabilitation programs were included.

Survival and diagnostic age of 175 Taiwanese patients with mucopolysaccharidoses (1985-2019).

Background: Mucopolysaccharidoses (MPSs) are a group of inherited metabolic diseases, which are characterized by the accumulation of glycosaminoglycans, and eventually lead to the progressive damage of various tissues and organs.

Methods: An epidemiological study of MPS in Taiwan was performed using multiple sources. The survival and diagnostic age for different types of MPS between 1985 and 2019 were evaluated.

Perfluorooctanoic acid in indoor particulate matter triggers oxidative stress and inflammation in corneal and retinal cells.

To investigate the particle size distribution of particulate matter and the concentration of specific perfluorinated compounds in indoor dust samples from several locations. Then, we used cell-based assays to investigate the effect of perfluorinated compounds on human corneal epithelial (HCEpiC), endothelial cells (HCEC) and retinal pigment epithelial cells (RPE). Indoor dust samples were collected at five different locations and PM, PM, and PM were fractionized.

Ictal and interictal electroencephalographic findings can contribute to early diagnosis and prompt treatment in KCNQ2-associated epileptic encephalopathy.

Background: KCNQ2-associated epilepsy is most common in neonatal genetic epilepsy. A prompt diagnosis to initialize early treatment is important.

Methods: We studied the electroencephalographic (EEG) changes including automated EEGs and conventional EEGs monitoring of 10 nonconsanguineous cases with KCNQ2 mutations, identified among 162 (6%) childhood epilepsy.

Clustering of Streptococcus gallolyticus subspecies pasteurianus bacteremia and meningitis in neonates.

Background/purpose: Although Streptococcus gallolyticus subspecies pasteurianus (SGSP) is a rare pathogen in children, it can cause invasive infections among neonates and infants. Herein, we report bacteremia/meningitis caused by SGSP in three neonates and review the literature on bacteremia and/or meningitis caused by this organism.

Methods: Three neonates, referred from an obstetrics clinic within a 2-month period, presented with invasive SGSP infections.

Reducing the consumption of personal protective equipment by setting up a multifunctional sampling station in the emergency department to screen for COVID-19 infection in Taiwan.

In Taiwan, high-risk patients have been identified and tested for preventing community spread of COVID-19. Most sample collection was performed in emergency departments (EDs). Traditional sample collection requires substantial personal protective equipment (PPE), healthcare professionals, sanitation workers, and isolation space.

α-Linolenic acid inhibits the migration of human triple-negative breast cancer cells by attenuating Twist1 expression and suppressing Twist1-mediated epithelial-mesenchymal transition.

α-Linolenic acid (ALA), an essential fatty acid, has anticancer activity in breast cancer, but the mechanism of its effects in triple-negative breast cancer (TNBC) remains unclear. We investigated the effect of ALA on Twist1, which is required to initiate epithelial-mesenchymal transition (EMT) and promotes tumor metastasis, and Twist1-mediated migration in MDA-MB231, MDA-MB468 and Hs578T cells. Twist1 protein was constitutively expressed in these TNBC cells, particularly MDA-MB-231 cells.

Implementation of nutrition practice improves growth velocity and weight gain in premature infants ≤ 1250 grams.

Background: The concept of parental nutritional care for premature infants has been applied and advanced over the past decade. This study compared the clinical outcomes before and after nutrition practice (NP) implementation and evaluated the effects of implementation on growth velocity and weight gain in premature infants.

Methods: Descriptive data of premature infants (gestational age < 30 weeks; body weight ≤ 1250 g) born 4 years before and after NP implementation were retrospectively reviewed in a neonatal intensive care unit at a hospital in Taiwan.

The Risk of Developing Constipation After Neonatal Necrotizing Enterocolitis.

Neonatal necrotizing enterocolitis (NEC) is a complex and lethal inflammatory bowel necrosis that primarily affects premature infants. Gut dysbiosis has been implicated in the pathogenesis of NEC. We aim to assess the association between NEC and two other diseases in children, including allergic diseases and constipation, considered to be associated with the alterations in gut microbiota composition.

Pomegranate extract inhibits migration and invasion of oral cancer cells by downregulating matrix metalloproteinase-2/9 and epithelial-mesenchymal transition.

Discovering drug candidates for the modulation of metastasis is of great importance in inhibiting oral cancer malignancy. Although most pomegranate extract applications aim at the antiproliferation of cancer cells, its antimetastatic effects remain unclear, especially for oral cancer cells. The aim of this study is to evaluate the change of two main metastasis characters, migration and invasion of oral cancer cells.

Systemic hypertension followed by insidious stroke in a 12-year-old boy with childhood neurofibromatosis type 1 presenting with renal and cerebral artery vasculopathy.

Lee ML, Chang TM, Yang RC, Yang AD, Chen M. Systemic hypertension followed by insidious stroke in a 12-year-old boy with childhood neurofibromatosis type 1 presenting with renal and cerebral artery vasculopathy. Turk J Pediatr 2019; 61: 629-634.

Effects of fish oil-containing lipid emulsions on retinopathy of prematurity in very low birth weight infants.

Background: The aim of the study was to assess the impact of different types of parenteral emulsions on retinopathy of prematurity (ROP) in very low birth weight (VLBW, birth body weight < 1500 g) infants by comparing fish oil-containing and soy-based parenteral emulsions.

Methods: Data of preterm infants with body weights below 1500 gm at birth and receiving total parenteral nutrition (TPN) for a minimum of 7 days during the period between January 2009 and November 2017 were analyzed in this retrospective study. We compared clinical outcomes in two epochs using different lipid emulsions: epoch 1 (soybean-based lipid emulsions, January 2009-February 2014) versus epoch 2 (fish oil-containing lipid emulsions, January 2015-November 2017).

Minimally invasive surfactant therapy versus intubation for surfactant administration in very low birth weight infants with respiratory distress syndrome.

Background: Minimally invasive surfactant therapy (MIST) is a new mode of surfactant administration without intubation to spontaneously breathing preterm infants with respiratory distress syndrome (RDS). The aims of this study were to assess the feasibility, efficacy and safety of using MIST to give surfactant for very low birth weight (VLBW) infants with RDS.

Methods: In total, 53 VLBW infants who were born before 32 gestational weeks with spontaneous breathing, respiratory distress, and requiring surfactant therapy were divided into two groups.

Intravenous immunoglobulin therapy enhances suppressive regulatory T cells and decreases innate lymphoid cells in children with immune thrombocytopenia.

Background: This study aimed to investigate the relationship between CD4 regulatory T cells (Tregs) and innate lymphoid cells (ILCs) in children with primary immune thrombocytopenia (ITP) undergoing high-dose intravenous immunoglobulin (IVIG) therapy.

Methods: We enrolled a cohort of 30 children with newly diagnosed ITP and 30 healthy controls and collected blood samples for levels of Tregs, ILCs, relevant cytokines, and Treg suppression assay at the diagnosis, two days, four weeks, and one year (only platelet count) after high-dose IVIG treatment. IVIG partial responders was defined by a platelet count less than 100 × 10 /L at 12 months after IVIG treatment.

Effect of high-frequency oscillatory ventilation combined with volume guarantee on preterm infants with hypoxic respiratory failure.

Background: The aim of this study was to assess the effect of volume guarantee (VG) on high-frequency oscillatory ventilation (HFOV) compared with HFOV alone in preterm infants with hypoxic respiratory failure (HRF).

Methods: Fifty-two preterm infants with HRF refractory to conventional mechanical ventilation (CMV) were enrolled in this study. Between June 2012 and February 2016, HFOV alone was used as rescue therapy when CMV failed for 34 infants, whereas HFOV combined with VG was used as rescue therapy for the other 18 infants between March 2016 and December 2017.

Relationships among Height, Weight, Body Mass Index, and Age in Taiwanese Children with Different Types of Mucopolysaccharidoses.

Background: Children with mucopolysaccharidosis (MPS) generally appear unaffected at birth but may develop multiple clinical manifestations including profound growth impairment as they grow older. Each type of MPS has a variable age at onset and variable rate of progression, however, information regarding growth in Asian children is limited.

Methods: This retrospective analysis included 129 Taiwanese patients with MPS (age range, 0.

An At-Risk Population Screening Program for Mucopolysaccharidoses by Measuring Urinary Glycosaminoglycans in Taiwan.

Background: The mucopolysaccharidoses (MPSs) are a group of rare lysosomal storage disorders characterized by the accumulation of glycosaminoglycans (GAGs) and which eventually cause progressive damage to various tissues and organs. We developed a feasible MPS screening algorithm and established a cross-specialty collaboration platform between medical geneticists and other medical specialists based on at-risk criteria to allow for an earlier confirmative diagnosis of MPS.

Methods: Children (<19 years of age) with clinical signs and symptoms compatible with MPS were prospectively enrolled from pediatric clinics between July 2013 and June 2018.

Birth defects in Taiwan: A 10-year nationwide population-based, cohort study.

Background/purpose: Birth defects (BDs) are main causes of mortality and disability in infants and children. The aims of this study were to analyze the prevalence, types and risk factors of BDs in Taiwan.

Methods: Data of all births (including live and stillbirths), types, characteristics, and associated risk factors of BDs were obtained from the National Birth Registry and National Health Insurance Research Data base in Taiwan between 2005 and 2014.

PM2.5 and NOx exposure promote myopia: clinical evidence and experimental proof.

Myopia is caused by complex genetic and environmental factors. However, information regarding the effect of long-term exposure to air pollutants on the risk of development of myopia is lacking. We collected data from two linked databases: the Taiwan National Health Insurance Research Database (NHIRD) and the Taiwan Air Quality-Monitoring Database (TAQMD).

A Pilot Survey of Potentially Hazardous Trace Elements in the Aquatic Environment Near a Coastal Coal-Fired Power Plant in Taiwan.

Background: A limited number of potentially hazardous trace elements were quantified in the aquatic environment near the world's second largest coal-fired power plant (CFPP) and the coal combustion residual (CCR) disposition sites in Central Taiwan. We postulated that contamination from specific trace elements would be present in the abovementioned aquatic environments.

Methods: Cross-sectional sampling of trace elements was first performed between September 24, 2017 and October 3, 2017 outside the CFPP, in the effluent sampled from Changhua, a county south of metropolitan Taichung, and at the historical CCR disposal sites, using the intertidal zone surface seawater and the seawater in an oyster farm as controls.