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The first outbreak of influenza A virus (IAV), which has been circulating for many decades, has resulted in serious morbidity and mortality. The IAV epidemic has caused serious damage to human life and the economy. Following an IAV infection, lymphatic cells immediately enter the lungs to clear the virus and eliminate damaged cells to maintain normal physiological mechanisms. In this study, we used alpha-lipoic acid (ALA), which has versatile antioxidant functions, as a target to treat and evaluate host antiviral effects and immune mechanisms. We observed that pre-treatment with ALA improved the survival rate and pathological state in a mouse model. A reduction in the inflammatory cytokine tumor necrosis factor-alpha was observed following in vivo experiments. It also increased the release of antiviral cytokines into the lungs by T lymphocytes. ALA pre-treatment effectively prevented acute pneumonia by modulating immune composition and contributing to virus clearance.

Background: Surgical intervention is important for acute appendicitis. Perforated appendicitis leads to more complications and is more difficult to manage. Studies differentiating ruptured from nonruptured appendicitis are controversial. Aim: Early identification of perforated appendicitis is important for first-line medical practitioners. Our study aimed to provide more indicators to evaluate the risk of perforation before surgery. Methods: This retrospective study included 116 patients who underwent an appendectomy at the Tri-Service General Hospital between 2014 and 2018 with pathological diagnosis. Based on the surgical records, the patients were classified into two groups: simple (nonperforation) and complicated (perforation). Results: The patients (68 boys, 48 girls) were all under 18 years of age. Simple appendicitis was diagnosed in 87 patients and perforated appendicitis in 29 patients. Analysis revealed significant differences in blood C-reactive protein (CRP) levels between the two groups. The highest Youden's index was at a CRP level of 1.755 mg/dL. Positive findings were obtained for suppurative change as well as chylous ascites. Conclusion: Elevated CRP level was found to be a useful biomarker in predicting perforated appendicitis, excluding other general factors in pediatric patients. Suppurative change and chylous ascites could serve as indicators for the possibility of perforation during surgery.

Objectives: Since April 2022, another wave of the Omicron epidemic has struck Taiwanese society, and children with severe neurological complications have been reported frequently. A few cases even developed acute fulminant encephalitis. To investigate the possible causes of the increased incidence of such complications in Taiwan, we reviewed several cases of pediatric patients with severe neurological symptoms. Methods: We collected the medical records of pediatric patients with COVID-19 infection who presented with severe neurological symptoms. The COVID-19 infection was diagnosed by nasal swab reverse transcriptase-polymerase chain reaction. The remaining samples were sent for whole genome sequencing and spike (S) protein amino acid variation mapping. Results: The increase of several inflammatory markers was observed in all patients included in this study. However, none of the cerebrospinal fluid samples tested positive for SARS-CoV-2. The result of whole genome sequencing showed that all the sequences belonged to the lineage BA.2.3.7. However, the sequences had a K97E mutation in the S protein that differed from other BA.2.3.7 lineage strains, which was located at the S protein N-terminal domain. Conclusion: The new mutation in the S protein, which had not previously been observed but was discovered in this study, potentially explains the sudden increase in incidence of extremely adverse neurological symptoms in pediatric patients.

Abstract Dravet syndrome (DS) is an uncommon epilepsy syndrome that may negatively affect the patients and their caregivers. However, reliable and valid measures of its impact on caregivers and the characteristics of patients with DS in Taiwan are lacking. This study aimed to describe the characteristics of patients with DS and concerns of their caregivers and establish a baseline frequency of disease characteristics using a cross-sectional survey in Taiwan. We assessed the caregivers of patients with DS using an online anonymous questionnaire. The seizure frequency decreased with age, although lacking statistical significance. Vaccines show no influence on the condition of patients with DS. Our findings revealed the highest impact on the domains affecting the caregivers’ daily life, including additional household tasks, symptom observation, further medical plan, and financial issues. Caregivers also expressed concerns regarding the lack of independence/constant care, seizure control, speech/communication, and impacts on siblings because of long-term care of the patients in parents’ absence. Our findings highlight the significant effects of caring for a child with DS on the lives of their caregivers in Taiwan; these findings will help raise awareness regarding the needs of these families. Furthermore, we discussed the possible pathophysiological mechanisms of associated comorbidities.

Morganella morganii is a rare cause of neonatal infections, and early-onset neonatal sepsis caused by M. morganii is serious and even fatal. We describe an extremely low birth weight female infant with early-onset M. morganii sepsis to highlight the importance of awareness of this potentially fatal organism.

Background: Vagus nerve stimulation (VNS) is used as an add-on treatment for epilepsy. This study aimed to use Taiwanese nationwide registry data to analyze the therapeutic effects of VNS in children with refractory epilepsy (RE) and try to explore predictive factors of VNS treatment effectiveness. Methods: This retrospective study collected data from December 2007 to December 2014. Patient variables included gender, age, VNS implantation date, epilepsy duration, seizure frequency, seizure type, etiology, and antiepileptic drug (AED) history. We divided patients into three groups: Group I as seizure frequency >80 times per month, Group II as seizure frequency 24–80 times per month, and Group III as seizure frequency

Introduction: Fluoroquinolone exposure is reportedly associated with a higher risk of tendon disorders, tendonitis, or tendon rupture. However, studies in East Asian populations have not confirmed these risks in patients with comorbidities or concomitant medication use. This cohort study was designed to investigate the associations among fluoroquinolone exposure, comorbidities, medication use, and tendon disorders in Taiwan.Materials and Methods: This population-based, nationwide, observational, cohort study used data from the National Health Insurance Research database in Taiwan, a nationwide claims database that covers more than 99% of the Taiwanese population. The study period was from January 2000 to December 2015, and the median follow-up time was 11.05 ± 10.91 years. Patients who were exposed to fluoroquinolones for more than three consecutive days were enrolled, and patients without fluoroquinolone exposure who were matched by age, sex, and index year were enrolled as controls. The associations of comorbidities and concomitant medication use with tendon disorder occurrence were analyzed using Cox regression models.Results: The incidence of tendon disorders were 6.61 and 3.34 per 105 person-years in patients with and without fluoroquinolone exposure, respectively (adjusted hazard ratio, 1.423; 95% confidence interval [1.02,1.87]; p = 0.021). Sensitivity analyses yielded similar results. Patients under 18 and over 60 years with fluoroquinolone exposure; those with chronic kidney disease, diabetes, rheumatologic disease, cardiac disease, lipid disorder, or obesity; and those who concomitantly used statins, aromatase inhibitors, or glucocorticoids, had a significantly higher risk of tendon disorders.Conclusion: The long-term risk of tendon disorders was higher in patients with fluoroquinolone exposure than in those without fluoroquinolone exposure. Clinicians should assess the benefits and risks of fluoroquinolone use in patients at high risk of tendon disorders who require fluoroquinolone administration.

Chromosome 17p13.3 microduplication syndrome is considered a multisystem disorder that results in a wide variety of clinical manifestations including dysmorphic facial characteristics, brain structural malformations, developmental restriction, growth restriction, and neurocognitive disorders. The two major classes of chromosome 17p13.3 microduplication, which have different clinical presentations, are associated with specific genetic regions. Among the various known phenotypes, scattered cases with congenital heart disease (CHD) have been reported for both classes of chromosome 17p13.3 microduplication syndrome. Unfortunately, there is insufficient understanding of the correlation between chromosome anomaly induced alterations in gene expression and aberrant cardiac development, and thus early diagnosis of CHD among patients with chromosome 17p13.3 microduplication is difficult without routine prenatal cardiac assessment. One such congenital heart anomalies known to affect a substantial number of newborns worldwide is ventricular septal defect (VSD), which has been found in 17p13.3 microduplication carriers, and seems to sometimes undergo spontaneous closure. We report an unprecedented case of moderate sized perimembranous-outlet VSD and congestive heart failure (CHF) in a Chinese Han male infant with a class II chromosome 17p13.3 microduplication. Despite the fact that cytogenic testing and fetal echocardiography confirmed a 249-Kb chromosome duplication within 17p13.3 that encompassed the PAFAH1B1 gene and showed the presence of VSD during prenatal period, this patient still developed a range of symptoms including sustained prolonged feeding, dyspnea, diaphoresis and retarded growth. A physical examination indicated hepatomegaly and a grade III/VI pan-systolic murmur along the left upper sternal border. Laboratory testing showed a high serum pro–B-type natriuretic peptide (pro-BNP). Imaging studies revealed cardiomegaly and a persistent VSD with related pulmonary stenosis. Since the clinical findings were compatible with CHF, we provided mainline treatment with digoxin, captopril, and furosemide, as well as fluid restriction. Despite sustained poor weight gain, the feeding behavior and the respiratory conditions of the patient improved gradually. This case report and literature review suggest that patients carrying chromosome 17p13.3 microduplication who have VSD may have an increased risk of developing CHF as young infants and hence a comprehensive cardiac evaluation is warranted to allow the early diagnosis and management of any severe heart anomalies.

Background: To investigate whether serial morphometric measurements of the brainstem using high resolution trans-foramen-magnum ultrasound (US) in premature neonates correlate with neurological outcomes.Methods: Serial brain ultrasound scans were performed in 36 consecutive preterm infants born at

Influenza is an infectious disease caused by an RNA virus that affects birds and mammals. It is a leading cause of morbidity, mortality and economic loss worldwide. Under influenza A virus infection, an uncontrolled inflammatory response in the lungs frequently leads to severe pneumonia. The anti-inflammatory and immune modulatory effects of melatonin may provide a beneficial effect for this disease. In this study, we found that melatonin treatment significantly decreases the expression of TNF-α, IL-6 and IFN-γ, and increases the production of IL-10 and TGF-β. The induction of IL-10 by melatonin occurs via the upregulation of IL-27 expression in dendritic cells. Melatonin also significantly inhibits the production of TNF-α in CD8 T cells. Co-treatment of melatonin and ribavirin significantly increases the survival of virus-infected mice compared to ribavirin alone. Our study suggests that melatonin possesses a therapeutic potential in influenza-induced pneumonia as an adjuvant treatment with anti-viral drugs.

While oxidative stress has been linked to multiple sclerosis (MS), the role of superoxide-producing phagocyte NADPH oxidase (Nox2) in central nervous system (CNS) pathogenesis remains unclear. This study investigates the impact of Nox2 gene ablation on pro- and anti-inflammatory cytokine and chemokine production in a mouse experimental autoimmune encephalomyelitis (EAE) model. Nox2 deficiency attenuates EAE-induced neural damage and reduces disease severity, pathogenic immune cells infiltration, demyelination, and oxidative stress in the CNS. The number of autoreactive T cells, myeloid cells, and activated microglia, as well as the production of cytokines and chemokines, including GM-CSF, IFNγ, TNFα, IL-6, IL-10, IL-17A, CCL2, CCL5, and CXCL10, were much lower in the Nox2−/− CNS tissues but remained unaltered in the peripheral lymphoid organs. RNA-seq profiling of microglial transcriptome identified a panel of Nox2 dependent proinflammatory genes: Pf4, Tnfrsf9, Tnfsf12, Tnfsf13, Ccl7, Cxcl3, and Cxcl9. Furthermore, gene ontology and pathway enrichment analyses revealed that microglial Nox2 plays a regulatory role in multiple pathways known to be important for MS/EAE pathogenesis, including STAT3, glutathione, leukotriene biosynthesis, IL-8, HMGB1, NRF2, systemic lupus erythematosus in B cells, and T cell exhaustion signaling. Taken together, our results provide new insights into the critical functions performed by microglial Nox2 during the EAE pathogenesis, suggesting that Nox2 inhibition may represent an important therapeutic target for MS.

This article is the first to provide characterization data regarding naive C57BL/6 transgenic mice with overexpression of B lymphocyte-induced maturation protein 1 (Blimp-1) under a T cell-specific pLCK promoter. The data presented are related to phenotype, Blimp-1 overexpression levels, T cell development and T cell proliferation for Blimp-1 transgenic mice. For further Blimp-1 overexpressed T cell findings regarding skin allotransplantation, please refer to the research article “Blimp-1 prolongs allograft survival without regimen via influencing T cell development in favor of regulatory T cells while suppressing Th1” (Wang et al., 2018) [1]. Keywords: Blimp-1, Transgenic mice, T cell

Objectives: The purpose of this study was to evaluate parenting stress in parents of children with refractory epilepsy before and after their children received vagus nerve stimulation (VNS) implantation. Methods: Parents of children with refractory epilepsy completed the Parenting Stress Index (PSI) under a psychologist's assessment before and at least 12 months after their children received VNS implantation. The PSI questionnaire measures parenting stress in two domains; a parent domain with seven subscales, and a child domain with six. Age, gender, epilepsy comorbidity, VNS implantation date, seizure frequency, and anticonvulsant history before and after VNS implantation were obtained from reviews of medical charts. Results: In total, 30 parents completed the first and follow-up PSI questionnaires. Seventeen of their children (56.7%) were boys. The children aged from 1 to 12 years (7.43 ± 3.59 years, mean ± SD). After VNS implantation, the mean total parenting stress scores decreased from 282.1 ± 38.0 to 272.4 ± 42.9. A significant decrease was found on the spouse subscale of the parent domain. For the parents of boys, the mean total parenting stress scores decreased significantly. The mean total parenting stress scores also decreased significantly for parents of epileptic children without autism and who did not taper off the number of different anticonvulsants used after VNS. Conclusions: VNS is an advisable choice to treat refractory epilepsy. Our study showed that 12 months or more after VNS implantation, seizure frequency and parenting stress typically decreased. However, in some special cases the parenting stress may increase, and external help may be required to support these patients and their parents. Key Words: children, refractory epilepsy, parenting stress, vagus nerve stimulation

Purpose. Interleukin-1α (IL-1α) is a potent cytokine that plays a role in inflammatory arthritis and bone loss. Decoy receptor 3 (DCR3) is an immune modulator of monocytes and macrophages. The aim of this study was to investigate the mechanism of DCR3 in IL-1α-induced osteoclastogenesis. Methods. We treated murine macrophages with DCR3 during receptor activator of nuclear factor kappa Β ligand- (RANKL-) plus IL-1α-induced osteoclastogenesis to monitor osteoclast formation by tartrate-resistant acid phosphatase (TRAP) staining. Osteoclast activity was assessed using a pit formation assay. The mechanisms of inhibition were studied by biochemical analyses, including RT-PCR, immunofluorescent staining, flow cytometry, an apoptosis assay, immunoblotting, and ELISA. Results. DCR3 suppresses IL-1α-induced osteoclastogenesis in both primary murine bone marrow-derived macrophages (BMM) and RAW264.7 cells as it inhibits bone resorption. DCR3 induces RANKL-treated osteoclast precursor cells to express IL-1α, secretory IL-1ra (sIL-1ra), intracellular IL-1ra (icIL-1ra), reactive oxygen species (ROS), and Fas ligand and to activate IL-1α-induced interleukin-1 receptor-associated kinase 4 (IRAK4). The suppression of DCR3 during RANKL- or IL-1α-induced osteoclastogenesis may be due to the abundant secretion of IL-1ra, accumulation of ROS, and expression of Fas ligand in apoptotic osteoclast precursor cells. Conclusions. We concluded that there is an inhibitory effect of DCR3 on osteoclastogenesis via ROS accumulation and ROS-induced Fas ligand, IL-1α, and IL-1ra expression. Our results suggested that the upregulation of DCR3 in preosteoclasts might be a therapeutic target in inflammatory IL-1α-induced bone resorption.

The existence of red, inflammatory, and chronic itchy condition in the skin is commonly speculated as the presence of Atopic Dermatitis (AD) in patients. The use of silk clothing as a non-pharmacological approach in the management of AD has been noticed as an effective alternative therapy; however, the evidence based on its usage is poorly served. Hence, we aim to evaluate the effectiveness of using pure silk clothing in the therapy of AD patients. The clinical trial was performed by recruiting 30 patients with AD for up to 8 weeks of observation. They were instructed to wear pure silk clothing for the whole day without any additional medication and were investigated using the AD-related questionnaires. The findings revealed a significant decrease of AD occurrence along with a great improvement of patient’s quality of life at each time point. Our investigation demonstrated that this treatment promotes good skin appearance, comfort, and remarkable improvement in the quality of life. This promising preliminary outcome warrants a further study; hence, it can be a potential non-pharmacological treatment choice for controlling the severity of AD.

This report describes two children who presented with status epilepticus. Both patients had hyponatremia, hypochloremia, low serum osmolality, and low urine osmolality caused by voluntary drinking of excessive quantities of water or diluted formula. Seizures were controlled by the administration of benzodiazepines, antiepileptic drugs, and hypertonic saline. Educating parents regarding hazards of excessive ingestion of fluid could reduce the incidence of this preventable and life-threatening condition.

Background: Intracranial arteriovenous malformation (ICAVM) is a rare and life-threatening disease. Clinical presentations of pediatric ICAVMs are variable and unpredictable. The Spetzler-Martin grading scale (SMGS) is a commonly used system for predicting prognoses and guiding therapeutic protocols. However, the application of this system to children is controversial. Objective: The purposes of this study were to retrospectively analyze clinical presentations of children with ICAVM and to investigate the relationship between the SMGS and the prognoses of children with ICAVM. Materials and Methods: Based on the International Classification of Diseases, 9 th revision, Clinical Modification (ICD-9-CM) code 747.81, we retrospectively reviewed eight children with ICAVMs admitted to a medical center from 1991 to 2012. The primary selection criterion was the diagnosis of the ICAVM, which was not irrelevant to traumatic injury or known coagulopathy. Patients′ outcomes were determined based on evaluation records of admission, discharge, and clinic visit 6-month after the ICAVM episode. For purposes of data analysis, outcomes were divided into "1 = death," "2 = persistent vegetative state," "3 = severe disability," "4 = moderate disability," and "5 = low disability." The relationship between the Glasgow outcome scale (GOS) and SMGS was analyzed using a scatter plot and Spearman′s correlation coefficient. Results: A total of eight patients with at least follow-up 6-month consisted of four males (50.0%) and four females (50.0%). Their ages ranged from 7 to 15 years, and the mean age was 12.63 years. Common sites of ICAVM nidus included basal ganglia (n = 3, 37.5%), and frontal-temporal region (25.0%). 62.5% of patients experienced bleeding on the right side of the brain. About 62.5% of patients complained of headache. 62.5% of patients showed loss of consciousness. About 37.5% of patients showed generalized seizures. The SMGS were Grade I in one patient (12.5%), Grade II in two patients (25.0%), Grade III in two patients (25.0%), and Grade IV in three patients (37.5%). The clinical outcomes for the overall series were 87.5% excellent or good (GOS score equal or above 4) and only one case dead. The correlation between SMGS and GOS, analyzed by Spearman′s correlation coefficient, was insignificant. Treatment, either by stereotactic radiosurgery (SRS) or surgery, was irrelevant to the prognosis of patient with ICAVM. Conclusion: Intracranial arteriovenous malformation is a life-threatening disease in children. Headache is a warning. Clinical presentations, including altered level of consciousness and generalized seizures in patient with ICAVM may suggest bleeding. The use of this system in children with ICAVM should be considered patients′ age for their better potential neuroplasticity and good healing capacities in higher grade of patients. Treatment, either by surgery or SRS, may be irrelevant to patients′ prognoses in this study.

Head trauma is one of the most challenging fields of traumatology and demands immediate attention and intervention by first-line clinicians. Symptoms can vary from victim to victim and according to the victim's age, leading to difficulties in making timely and accurate decisions at the point of care. In children, falls, accidents while playing, sports injuries, and abuse are the major causes of head trauma. Traffic accidents are the main cause of disability and death in adolescents and adults. Injury sites include facial bones, muscles, ligaments, vessels, joints, nerves, and focal or whole-brain injuries. Of particular importance are cranial and intracranial injuries. A closed injury occurs when the head suddenly and violently hits an object but the object does not break through the skull. A penetrating injury occurs when an object pierces the skull and affects the brain tissue. Early diagnosis and proper management are crucial to treat patients with potentially life-threatening head and neck trauma. In this review, we discuss the different cases of traumatic brain injury and summarize the current therapies and neuroprotective strategies as well as the related outcomes for children with traumatic brain injury.

Cauda equina syndrome (CES) in children is very rare and can permanently disable. A remitted intracranial germinoma depositing on the spinal cord, leading to CES, has never been reported. We discuss the case of a 10-year-old girl who presented with sudden ataxia, low back pain, sensory deficits of the left lower extremity, and difficulty urinating and defecating 7 months after totally remitted intracranial germinoma postintracranial surgery and cranial irradiation. Magnetic resonance imaging (MRI) of the brain and spine showed multiple intradural extramedullary homogeneous masses from the cervical to lumbar levels, compressing the conus medullaris and cauda equina. After emergent craniospinal irradiation, the patient's neurologic symptoms dramatically subsided. A remitted intracranial germinoma depositing on her spinal cord could be the cause of CES. Early identification and a proper craniospinal irradiation may halt the progression of symptoms.

Vagus nerve stimulation (VNS), an alternative method to manage patients with medically intractable epilepsy, has shown favorable results in reducing seizure relapse and improvements in quality of life. In 1997, the U.S. Food and Drug Administration approved the use of this device as an adjunctive therapy for intractable seizure in adults and adolescents older than 12 years of age. Methods: We present a preliminary study of pediatric patients, who suffered from medically intractable seizure and underwent VNS implantation after observation of the baseline seizure frequency. Classification of epileptic syndrome, seizure patterns, age of onset, seizure frequency reduction and adverse effects were recorded. Results: Patients who underwent VNS implantation included four adolescents and four children. The follow-up duration ranged from 9–33 months. All the patients were responders after the beginning of the stimulation. Five of the eight patients responded to VNS with a seizure frequency reduction rate > 50%, and four of the eight patients experienced a ≥ 90% seizure reduction. No significant adverse effects were noted in all patients during the observation period. Conclusion: The effective management of medically intractable seizure remains challenging to most clinical physicians. In addition to ketogenic diet and epilepsy surgery, VNS provides an alternative way to manage this issue. Our results suggest that VNS is well tolerated in pediatric patients, and is a favorable and safe method of treating intractable seizure in common clinical practice.

Dandy–Walker syndrome (DWS) is a congenital brain malformation involving the cerebellum and fourth ventricle. We report a 6-month-old girl with DWS presenting an initially normal ventricular system and mild cyst-like lesion over the posterior fossa as assessed by postnatal brain sonography. However, symptoms and signs of increased intracranial cerebral pressure in terms of frequent vomiting and tense anterior fontanel developed, and these were associated with mild hypotonia and poor neck support, and upward-gaze palsy at the age of 6 months. Magnetic resonance imaging revealed a huge cystic lesion of the fourth ventricle, which filled the posterior fossa and ventricular dilatation. The tentorium was progressively displaced upward by the cyst. A nearly complete agenesis of the cerebellar vermis was also confirmed. After a successful endoscopic third ventriculostomy, a series of brain magnetic resonance imaging scans, taken during a follow-up survey, showed normal lateral and third ventricles. Consequently, symptoms of intracranial cerebral pressure resolved, and a developmental milestone was achieved. In conclusion, DWS can be confirmed postpartum, and endoscopic third ventriculostomy was found to be a preferential operative procedure for DWS with hydrocephalus. It may be effective for patients younger than 1 year.

Inhaled β2-adrenergic receptor (β2-AR) agonists are the mainstay of treatment of acute asthma. Polymorphisms of the β2-AR, especially codons 16, 27, and 164, may affect the functions of the receptor. This study was conducted to investigate whether different polymorphisms of the β2-AR are related to the treatment responses of an inhaled β2-AR agonist in children with nocturnal and nonnocturnal asthma in Taiwan. Methods: The nocturnal asthma group consisted of 27 children (mean age of 10.3±2.4 years), and the nonnocturnal asthma group consisted of 24 patients (mean age of 9.9±3.0 years). Allele-specific polymerase chain reaction was performed to determine 16, 27, and 164 loci alleles of β2-AR genetic polymorphisms, and peak expiratory flow (PEF) was measured before and 1 hour after inhalation of 0.2 mg/kg/dose of terbutaline to determine the treatment response in these patients. Results: The polymorphisms of β2-AR 27 but not 16 or 164 were significantly associated with the response to terbutaline nebulizer (p

The diagnosis of child maltreatment leading to head injury is challenging. Here, we present the case of a 3-month-old female infant who presented with focal seizures that lasted for several minutes. After admission, she began to show intermittent clusters of head nods, irritable crying, arching, writhing, stiffening, and jerking of both arms. These results and electroencephalography findings were attributed as the diagnosis of infantile spasms (IS). Brain computed tomography and magnetic resonance imaging (MRI) revealed the presence of chronic subdural hematoma mixed with acute ischemic injuries. Examination of the eye fundus confirmed the presence of retinal hemorrhage. Therefore, all evidence pointed to a diagnosis of shaken baby syndrome (SBS). Based on this case, we suggest that physicians should consider a diagnosis of SBS for children with new-onset IS and that should be evaluated, diagnosed, and treated as promptly as possible.

The polyglutamine (polyQ) diseases are a group of neurodegenerative disorders caused by expanded cytosine– adenine–guanine (CAG) repeats encoding a long polyQ tract in the respective proteins. To date, a total of nine polyQ disorders have been described: six spinocerebellar ataxias (SCA) types 1, 2, 6, 7, 17; Machado–Joseph disease (MJD/SCA3); Huntington's disease (HD); dentatorubral pallidoluysian atrophy (DRPLA); and spinal and bulbar muscular atrophy, X-linked 1 (SMAX1/SBMA). PolyQ diseases are characterized by the pathological expansion of CAG trinucleotide repeat in the translated region of unrelated genes. The translated polyQ is aggregated in the degenerated neurons leading to the dysfunction and degeneration of specific neuronal subpopulations. Although animal models of polyQ disease for understanding human pathology and accessing disease-modifying therapies in neurodegenerative diseases are available, there is neither a cure nor prevention for these diseases, and only symptomatic treatments for polyQ diseases currently exist. Long-term pharmacological treatment is so far disappointing, probably due to unwanted complications and decreasing drug efficacy. Cellular transplantation of stem cells may provide promising therapeutic avenues for restoration of the functions of degenerative and/or damaged neurons in polyQ diseases.

Interferon-beta (IFN-β) treatment may not be effective in neuromyelitis optica (NMO). Whether the poor response to IFN-β is related to long spinal cord lesions (LSCL) or the NMO disease entity itself is unclear. We evaluated the spinal cord involvement of patients with multiple sclerosis (MS) and NMO, as well as the response after receiving IFN-β. Forty-nine MS and 21 NMO patients treated with IFN-β for at least 2 years from 2002-2008 were enrolled in this study and the treatment response was analyzed 2 years post-treatment. In the study, spinal cord lesions were present in 57.1% (28/49) of the MS patients, of which 16.3% (8/49) presented spinal cord lesions longer than 3 vertebral segments (LSCL). Responses to IFN-β treatment were seen in 69.3% (34/49) of all the MS cases, of which the appropriate response rates were 76.1% (16/21) in MS patients without spinal cord lesions and 37.5% (3/8) in patients with LSCL. Only 14.2% (3/21) of NMO patients responded to IFN-β treatment. In conclusion, spinal cord lesion is common in MS patients in Taiwan. Both NMO and MS patients with LSCL had a poor response to IFN-β treatment. NMO patients had a worse response to IFN-β treatment than MS patients with LSCL, which shows that the crucial structural defect is something other than LSCL such as the elevated serum IL17 level in NMO compared to MS.

Parkinson's disease (PD) is a common neurodegenerative disease and typically presents with tremor, rigidity, bradykinesia, and postural instability. The hallmark pathological features of PD are loss of dopaminergic neurons in the substantia nigra (SN) and the presence of neuronal intracellular Lewy body (LB) inclusions. In general, PD is sporadic; however, familial PD, while uncommon, can be inherited in an autosomal dominant (AD) or autosomal recessive (AR) manner. The molecular investigations of proteins encoded by PD-linked genes have clarified that ADPD is associated with α-synuclein and LRRK2, while ARPD is linked to Parkin, PINK1, DJ1, and ATP13A2. Understanding these genes can bring insights into this disease and create possible genetic tests for early diagnosis. Long-term pharmacological treatment is so far disappointing, probably due to unwanted complications and decreasing drug efficacy. Several strategies have been proposed and tested as alternatives for PD. Cellular transplantation of dopamine-secreting stem cells opens the door to new therapeutic avenues for restoration of the functions of degenerative and/or damaged neurons in PD.

Type 1 diabetes (T1D) is an autoimmune disease mediated by T cells that selectively destroy the insulin-producing β cells. Previous reports based on epidemiological and animal studies have demonstrated that both genetic factors and environmental parameters can either promote or attenuate the progression of autoimmunity. In recent decades, several inbred rodent strains that spontaneously develop diabetes have been applied to the investigation of the pathogenesis of T1D. Because the genetic manipulation of mice is well developed (transgenic, knockout, and conditional knockout/transgenic), most studies are performed using the nonobese diabetic (NOD) mouse model. This paper will focus on the use of genetically manipulated NOD mice to explore the pathogenesis of T1D and to develop potential therapeutic approaches.

Multiple sclerosis (MS) is an autoimmune disease of the central nervous system, and CD4+ T cells form the core immunopathogenic cascade leading to chronic inflammation. Traditionally, Th1 cells (interferon-γ-producing CD4+ T cells) driven by interleukin 12 (IL12) were considered to be the encephalitogenic T cells in MS and experimental autoimmune encephalomyelitis (EAE), an animal model of MS. Currently, Th17 cells (Il17-producing CD4+ T cells) are considered to play a fundamental role in the immunopathogenesis of EAE. This paper highlights the growing evidence that Th17 cells play the core role in the complex adaptive immunity of EAE/MS and discusses the roles of the associated immune cells and cytokines. These constitute the modern immunological basis for the development of novel clinical and preclinical immunomodulatory therapies for MS discussed in this paper.

The immunologic interaction between the fetus and the mother is a paradoxical communication that is regulated by fetal antigen presentation and/or by recognition of and reaction to these antigens by the maternal immune system. There have been significant advances in understanding of abnormalities in the maternal-fetal immunologic relationship in the placental bed that can lead to pregnancy disorders. Moreover, immunologic recognition of pregnancy is vital for the maintenance of gestation, and inadequate recognition of fetal antigens may cause abortion. In this paper, we illustrate the complex immunologic aspects of human reproduction in terms of the role of human leukocyte antigen (HLA), immune cells, cytokines and chemokines, and the balance of immunity in pregnancy. In addition, we review the immunologic processes of human reproduction and the current immunologic therapeutic strategies for pathological disorders of pregnancy.

Stroke is a sudden onset neurological deficit due to a cerebrovascular event. In children, the recognition of stroke is often delayed due to the low incidence of stroke and the lack of specific assessment measures to this entity. The causes of pediatric stroke are significantly different from that of adult stroke. The lack of safety and efficiency data in the treatment is the challenge while facing children with stroke. Nearly half of survivors of pediatric stroke may have neurologic deficits affecting functional status and quality of life. They may cause a substantial burden on health care resources. Hence, an accurate history, including onset and duration of symptoms, risk factors, and a complete investigation, including hematologic, neuroimaging, and metabolic studies is the key to make a corrective diagnosis. A prompt and optimal treatment without delay may minimize the damage to the brain.

BACKGROUND: Staphylococcus aureus is an important cause of infection, particularly in persons colonized with this organism. This study compared the annual prevalence and microbiological characteristics of methicillin-resistant S. aureus (MRSA) nasal colonization in Taiwanese children from 2004 through 2009. Risk factors for MRSA were determined for the overall study period. METHODS: Children from birth to ≤14 years of age presenting for health maintenance visits or attending 1 of 57 kindergartens were recruited. Nasal swabs were obtained, and a questionnaire was administered. The prevalence and microbiological characteristics of MRSA colonization were also calculated for two 3-year periods: 2004-2006 and 2007-2009. RESULTS: Cultures of the anterior nares were positive for S. aureus in 824 (25.8%) of the 3,200 children, and MRSA colonization was found in 371 (11.6%) children. The prevalence of S. aureus colonization decreased from 28.1% in 2004-2006 to 23.3% in 2007-2009 (p