Antimicrobial Resistance Profiles and Molecular Characteristics of Salmonella enterica Serovar Agona Isolated from Food-Producing Animals During 2010-2020 in South Korea.

Multidrug-resistant (MDR) Salmonella enterica serovar Agona infections affect public health globally. This investigation aimed to ascertain the antimicrobial resistance profiles and molecular characteristics of Salmonella Agona isolates obtained from food-producing animals. A total of 209 Salmonella Agona isolates were recovered from mostly chickens (139 isolates), pigs (56 isolates), cattle (11 isolates), and ducks (3 isolates) between 2010 and 2020 in South Korea. In addition, these Salmonella Agona isolates were obtained from 25 slaughterhouses nationwide. Furthermore, this serotype suddenly increased in chickens in 2020. Salmonella Agona from chickens showed high resistance (69-83%) to ampicillin, streptomycin, tetracycline, trimethoprim/sulfamethoxazole, and chloramphenicol. Moreover, chicken/duck isolates (83.1%) showed significantly higher levels of MDR than cattle/pig isolates (1.5%). For molecular analysis by pulsed-field gel electrophoresis, infrared spectroscopy biotyping, and multilocus sequence typing in combination, a total of 23 types were observed. Especially two major types, P1-III-2-13 and P1-IV-2-13, comprised 59.3% of the total isolates spreading in most farms. Moreover, Salmonella Agona sequence type (ST)13 was predominant (96.7%) among three different STs (ST13, ST11, and ST292) widely detected in chickens (94.3%) in most farms located nationwide. Taken together, MDR Salmonella Agona in chickens might pose a potential risk to public health through direct contact or the food chain.

Clinical use of whole exome sequencing in children with developmental delay/intellectual disability.

BACKGROUND: Identifying the underlying etiology of developmental delay/intellectual disability (DD/ID) is challenging but important. The genetic diagnosis of unexplained DD/ID helps in the treatment and prognosis of the disability in patients. In this study, we reported our experience of using whole exome sequencing (WES) of children with unexplained DD/ID. METHODS: We conducted a retrospective analysis of WES results of children under 19 years of age with unexplained DD/ID between January 2020 and December 2021. The demographic data of all patients and variants identified through WES were evaluated. Furthermore, we evaluated the clinical characteristics that influenced the identification of genetic causes. RESULTS: Forty-one patients with DD/ID were included, of whom 21 (51.2 %) were male. The average age at symptom onset was 1.6 ± 1.3 years, and the duration from symptom onset to diagnosis was 3.1 ± 3.7 years. Hypotonia was the most common symptom (17 patients, 41.5 %), and epilepsy was confirmed in 10 patients (24.4 %). Twenty-two pathogenic/likely pathogenic variants were identified in 20 patients, and three variants of uncertain significance were identified in three patients. Family-based trio Sanger sequencing for candidate variants of 12 families was conducted; 10 variants were de novo, one variant paternally inherited, and two variants compound heterozygous. The diagnostic yield of WES for DD/ID was 48.8 % and was significantly high in patients with an early onset of DD/ID and facial dysmorphism. In contrast, patients with autism spectrum disorder (ASD) were more likely to have negative WES results compared with others without ASD. CONCLUSION: The diagnostic yield of WES was 48.8 %. We conclude that patients' characteristics, such as dysmorphic features and the age of symptom onset, can predict the likelihood that WES will identify a causal variant of a phenotype.

Effects of a bowel function improvement program for patients with rectal cancer surgery: A randomized controlled trial.

OBJECTIVES: This study aimed to examine the effectiveness of a bowel function improvement program for male patients with rectal cancer who underwent low anterior resection. METHODS: A prospective, unblinded, and randomized controlled trial was conducted. The enrolled 42 patients were assigned to the experimental or control group at a 1:1 ratio. The bowel function improvement program comprised a 4-week intensive program (face-to-face education and telephone coaching) and an 8-week maintenance program (text messages). Self-efficacy, bowel function, health-related quality of life, and healthcare resource utilization were measured. Collected data were analyzed using independent t-tests, chi-square tests, analysis of covariance, and generalized estimation equations to evaluate the effects of the program based on intention-to-treat. RESULTS: The bowel function improvement program was effective in improving bowel function 3 months after discharge. Additionally, the number of unplanned pharmacy visits was lower in the experimental group than in the control group. Health-related quality of life, self-efficacy, and utilization of other healthcare resources were not statistically or clinically significant. CONCLUSION: These findings indicated that the bowel function improvement program for male patients with rectal cancer was effective in improving bowel function and reducing unplanned healthcare resource utilization. The bowel function improvement program can be delivered as a nurse-led program in clinical practice to promote early recovery after low anterior resection. TRIAL REGISTRATION NUMBER: KCT0003505. https://cris.nih.go.kr/cris/search/detailSearch.do/13708.

Rapid and ultrasensitive miRNA detection by combining endonuclease reactions in a rolling circle amplification (RCA)-based hairpin DNA fluorescent assay.

MicroRNA (miRNA) sensing strategies employing rolling circle amplification (RCA) coupled with the hairpin DNA (HD) probe-mediated FRET assay have shown promise, but achieving rapid, sensitive, and specific detection of target miRNA remains a challenge in clinical diagnostics. Herein, we incorporate PstI endonuclease cleavage (PEC) into a conventional RCA-based HD probe FRET assay to develop an effective and feasible method. Long single-stranded RCA products are synthesized from miRNA-21 loaded on a circular dumbbell DNA, and the resultant RCA products self-assemble to generate long HD structures with double-stranded stem regions that are specifically recognized and cleaved by PstI endonucleases when incubated with PstI enzymes. This releases large amounts of short single-stranded DNA fragments that hybridize and open to the complementary loop-stem regions of HD probes labeled with FAM at one end and BHQ-1 at the other, resulting in a reduction in FRET efficiency. This assay achieves a 39.7 aM detection limit for target miRNA-21, approximately 37-fold higher than that of the conventional assay (1.5 fM). Moreover, quantitative detection is possible in a wide range from 1 aM to 1 pM within 90 min with high sequence specificity. We demonstrate the assay with the detection of target miRNA-21 in total RNA extracted from MCF-7 cancer cells.

Antimicrobial Resistance in Escherichia coli Isolated from Healthy Dogs and Cats in South Korea, 2020-2022.

The occurrence of antimicrobial-resistant bacteria in companion animals poses public health hazards globally. This study aimed to evaluate the antimicrobial resistance profiles and patterns of commensal E. coli strains obtained from fecal samples of healthy dogs and cats in South Korea between 2020 and 2022. In total, 843 E. coli isolates (dogs, n = 637, and cats, n = 206) were assessed for susceptibility to 20 antimicrobials. The resistance rates of the most tested antimicrobials were significantly higher in dog than in cat isolates. Cefalexin (68.9%) demonstrated the highest resistance rates, followed by ampicillin (38.3%), tetracycline (23.1%), and cefazolin (18.7%). However, no or very low resistance (0-0.6%) to amikacin, imipenem, piperacillin, and colistin was found in both dog and cat isolates. Overall, 42.3% of the isolates exhibited multidrug resistance (MDR). MDR in isolates from dogs (34.9%) was significantly higher than in those from cats (20.9%). The main components of the resistance patterns were cefalexin and ampicillin in both dog and cat isolates. Additionally, MDR patterns in isolates from dogs (29.2%) and cats (16%) were shown to encompass five or more antimicrobials. Multidrug-resistant commensal E. coli could potentially be spread to humans or other animals through clonal or zoonotic transmission. Therefore, the incidence of antimicrobial resistance in companion animals highlights the urgent need to restrict antimicrobial resistance and ensure the prudent use of antimicrobials in Korea.

Coffin-Siris Syndrome: Case Series of Three Patients and a Novel ARID2 Variant.

Coffin-Siris syndrome (CSS) is a rare congenital disorder characterized by coarse facial features, intellectual disability or developmental delay, and aplasia or hypoplasia of the tips of the fifth finger and/or toes. Mutations in genes affecting the switch/sucrose non-fermenting ATP-dependent chromatin remodeling complex are reported to cause CSS. Here, we describe three CSS patients. Two girls aged 3 and 2 years old presented with global developmental delay, poor growth, and a dysmorphic face. Whole-exome sequencing (WES) was performed and they were diagnosed with CSS due to heterozygous frameshift variants (c.3443_3444del, p.Lys1148ArgfsTer9 and c.2869_2890del, p.Pro957CysfsTer20) in ARID1B A 2-year-old girl presented with gross motor delay and dysmorphic face. She was diagnosed with CSS due to a novel heterozygous frameshift variant (c.4942_4943del: p.Gln1648GlyfsTer8) in ARID2.

Self-assembled DNA dendrons as signal amplifiers in a DNA probe-based chemiluminescence assay for enhanced colorimetric detection of short target cDNA.

A simple, reliable, and cost-effective method for nucleic acid detection is of increasing interest in clinical diagnostics of infectious and genetic diseases. Currently, enzyme-mediated methods such as polymerase chain reactions and loop-mediated isothermal amplification are the most widely used methods in the qualitative and quantitative diagnosis of long nucleic acid sequences with high sensitivity. However, a high detection sensitivity for short-length sequences remains a significant challenge because it is difficult for the primers to bind to their sequences. Our previous study demonstrated a simple, enzyme-free, and sequence-specific colorimetric detection of 24-nucleotide short target DNA at room temperature using a developed assay that combines catalytic hairpin assembly (CHA) and enzyme-linked immunosorbent assay (ELISA)-mimicking methods. In this follow-up study, we aim to design and develop DNA-based signal amplifiers, or DNA dendrons, to improve the colorimetric detection of short target cDNA in the CHA-mediated ELISA-mimicking assay. DNA dendrons are highly branched conformations synthesized by the molecular self-assembly of three DNA oligomers. The assay using DNA dendrons demonstrates an enhanced detection sensitivity with the detection of approximately 50 pM of 24-nucleotide short target cDNA, which is a 16.4-fold higher detection limit compared to that obtained without DNA dendrons under the same conditions. Thus, applications of the developed DNA dendrons as an effective signal amplifier in DNA probe-based chemiluminescence assays have the potential to improve the colorimetric detection of short target cDNA with high sensitivity for the diagnosis of different diseases.

Chromosomal Microarray in Children With Developmental Delay: The Experience of a Tertiary Center in Korea.

Background and Objectives: Chromosomal microarray (CMA) is a first-tier genetic test for children with developmental delay (DD), intellectual disability (ID), autism spectrum disorders (ASDs), and multiple congenital anomalies (MCA). In this study, we report our experiences with the use of CMA in Korean children with unexplained DD/ID. Methods: We performed CMA in a cohort of 308 children with DD/ID between January 2010 and September 2020. We also retrospectively reviewed their medical records. The Affymetrix CytoScan 750 K array with an average resolution of 100 kb was used to perform CMA. Results: Comorbid neurodevelopmental disorders were ASD (37 patients; 12.0%), epilepsy (34 patients; 11.0%), and attention deficit hyperactivity disorders (12 patients; 3.9%). The diagnostic yield was 18.5%. Among the 221 copy number variants (CNVs) identified, 70 CNVs (57 patients; 18.5%) were pathogenic. Deletion CNVs were more common among pathogenic CNVs (PCNVs) than in non-PCNVs (P < 0.001). The size difference between PCNVs and non-PCNVs was not significant (P = 0.023). The number of included genes within CNV intervals was significantly higher in PCNVs (average 8.6; 0-347) than in non-PCNVs (average 47.5; 1-386) (P < 0.001). Short stature and hearing difficulty were also more common in the PCNV group than in the non-PCNV group (P = 0.010 and 0.070, respectively). Conclusion: This study provides additional evidence for the usefulness of CMA in genetic testing of children with DD/ID in Korea. The pathogenicity of CNVs correlated with the number of included genes within the CNV interval and deletion type of the CNVs, but not with CNV size.

Effect of Quinoa (Chenopodium quinoa Willd.) Starch and Seeds on the Physicochemical and Textural and Sensory Properties of Chicken Meatballs during Frozen Storage.

The effects of starch (corn and quinoa) and quinoa seeds on chicken meatballs' physicochemical, textural, and sensory properties were investigated during frozen storage. The chicken meatballs were prepared with corn starch (CS), quinoa starch (QS), quinoa seeds (Q), and combinations of corn starch and quinoa seeds (CS-Q), and quinoa starch and quinoa seeds (QS-Q), which were subjected to five freeze-thaw (F-T) cycles of temperature fluctuation conditions during frozen storage. Regardless of the type used (CS or QS), adding starch resulted in fewer cooking, drip, and reheating losses in chicken meatballs during frozen storage. The values of the hardness, gumminess, and chewiness of chicken meatballs with CS or QS were half those of chicken meatballs without starch, indicating that the addition of starch inhibited the change in the meatballs' texture. The total volatile basic nitrogen (TVB-N) and thiobarbituric acid reactive substance (TBARS) values were progressive but did not dynamically increase during five F-T cycles. Chicken meatballs containing CS-Q or QS-Q showed significantly lower TBARS values than those with CS, QS, or Q after five F-T cycles. Adding quinoa seeds significantly increased the antioxidant activity and the chewiness of meatballs (p < 0.05) compared with starch only. The addition of the combination of QS-Q to chicken meatballs increased the values of taste, texture, and overall acceptability, indicating that quinoa starch and seeds may be introduced as premium ingredients to frozen meat products.

The effectiveness of preoperative stoma site marking on patient outcomes: A systematic review and meta-analysis.

AIMS: This study systematically reviews the literature regarding preoperative stoma site marking and discusses the effectiveness of the procedure on complication rates, self-care deficits and health-related quality of life (HRQOL). DESIGN: Systematic review and meta-analysis. DATA SOURCE: Our review was conducted following the PRISMA guidelines. PubMed, EMBASE, Cochrane and CINAHL databases were searched to obtain articles published in English. Articles were also retrieved from Korean databases as well. Our last search was conducted on 2 June 2019. REVIEW METHODS: Two reviewers independently selected relevant studies, evaluated their methodological quality and extracted data. Experimental and observational studies were included. Our main focus was on complication rates, self-care deficits and HRQOL. We conducted meta-analysis using the statistical software spss 25.0 and Stata 13.0. RESULTS: Of the 1,039 articles reviewed, 20 were included for review, and 19 were used for quantitative synthesis. Preoperative stoma site marking reduced complication rates (odds ratio [OR]: 0.47; 95% confidence interval [CI]: 0.36-0.62; I2 : 70.6%), lowered self-care deficits (OR: 0.34; 95% CI: 0.18-0.64; I2 : 0%), and increased HRQOL (standardized mean difference, 1.05; 95% CI: 0.70-1.40; I2 : 0%). Quality appraisal results for both the individual studies and the studies overall were excellent. The possibility of publication bias was low. CONCLUSIONS: Our findings indicate that preoperative stoma site marking improves patient outcomes: stoma-related complication rates and self-care deficits decrease and HRQOL rises. For this reason, preoperative stoma site marking should be a mandatory procedure in clinical settings. The practice should also be supported by policymakers and healthcare expert associations. IMPACT: Preoperative stoma site marking reduces overall complication rates by 53% and skin problems by 59%. Preoperative stoma site marking also improves self-care and health-related quality of life. We recommend that preoperative stoma site marking should be a mandatory procedure in clinical settings.

Radiological manifestation of familial acute necrotizing encephalopathy with RANBP2 mutation in a Far-East Asian family: Case report.

RATIONALE: Acute necrotizing encephalopathy (ANE) is a specific type of encephalopathy usually followed by febrile infection. It has an aggressive clinical course; however, it usually does not recur after recovery in cases of spontaneous ANE. Nevertheless, there are several studies reporting recurrences in familial ANE with RAN-binding protein 2 (RANBP2) mutation. There are few cases of familial ANE with RANBP2 mutation in Asian populations. PATIENTS CONCERNS: A 21-month-old Korean boy who was previously healthy, presented with seizure following parainfluenza - a virus and bocavirus infection, followed by 2 recurrent seizure episodes and encephalitis after febrile respiratory illnesses. Meanwhile, his 3-year-old sister had focal brain lesions on neuroimaging studies when evaluated for head trauma. The siblings also had an older brother who presented status epilepticus after febrile respiratory illness at the age of 10 months old. DIAGNOSIS: Brain magnetic resonance imaging was performed to evaluate the seizure and neurologic symptoms. Imaging findings showed variable spectrum - from non-specific diffuse white matter injury pattern to typical "tricolor pattern" of the ANE on diffusion-weighted images. The other 2 siblings showed focal lesions in both external capsules and severe diffuse brain edema. Genetic tests identified a heterozygous missense mutation in the RANBP2 [c.1754C>T (p.Thr585Met)] in 2 siblings and their mother. INTERVENTIONS: Patients were treated conservatively with anticonvulsive agents, intravascular immunoglobulin, and steroids. OUTCOMES: Among the 3 siblings, 2 male siblings died from familial ANE, whereas the female sibling was asymptomatic. LESSONS: These cases highlight the radiological aspects of familial ANE with incomplete penetrance of the RANBP2 gene in 3 family members, showing variable involvements of the brain and natural history on magnetic resonance images. Radiologists should be aware of the typical and atypical imaging findings of familial ANE for prompt management of affected patients.

Discovery and Functional Study of a Novel Genomic Locus Homologous to Bα-Mating-Type Sublocus of Lentinula edodes.

The interaction of mating pheromone and pheromone receptor from the B mating-type locus is the first step in the activation of the mushroom mating signal transduction pathway. The B mating-type locus of Lentinula edodes is composed of Bα and Bß subloci, each of which contains genes for mating pheromone and pheromone receptor. Allelic variations in both subloci generate multiple B mating-types through which L. edodes maintains genetic diversity. In addition to the B mating-type locus, our genomic sequence analysis revealed the presence of a novel chromosomal locus 43.3 kb away from the B mating-type locus, containing genes for a pair of mating pheromones (PHBN1 and PHBN2) and a pheromone receptor (RCBN). The new locus (Bα-N) was homologous to the Bα sublocus, but unlike the multiallelic Bα sublocus, it was highly conserved across the wild and cultivated strains. The interactions of RcbN with various mating pheromones from the B and Bα-N mating-type loci were investigated using yeast model that replaced endogenous yeast mating pheromone receptor STE2 with RCBN. The yeast mating signal transduction pathway was only activated in the presence of PHBN1 or PHBN2 in the RcbN producing yeast, indicating that RcbN interacts with self-pheromones (PHBN1 and PHBN2), not with pheromones from the B mating-type locus. The biological function of the Bα-N locus was suggested to control the expression of A mating-type genes, as evidenced by the increased expression of two A-genes HD1 and HD2 upon the treatment of synthetic PHBN1 and PHBN2 peptides to the monokaryotic strain of L. edodes.

Clinical utility of the FilmArray meningitis/encephalitis panel in children at a tertiary center in South Korea.

BACKGROUND: We retrospectively evaluated the pathogens in the cerebrospinal fluid (CSF) of pediatric meningitis/encephalitis (M/E) by FilmArray meningitis/encephalitis panel (FA-MEP), and the characteristics of children showing positive and negative FA-MEP results. METHOD: FA-MEP along with conventional tests (bacterial/viral cultures, and polymerase chain reaction tests) was performed in children who presented symptoms of M/E. Clinical and laboratory data were reviewed to evaluate the characteristics of children with pathogens detected by FA-MEP. RESULTS: The CSF specimens from 110 pediatric M/E patients were enrolled. Mean age of the patients was 5.9 ± 5.2 years. Overall positive rate of FA-MEP was 46.4% (51/110). The pathogens detected in the patients were enterovirus (23/51, 45.1%), parechovirus (10/51, 19.6%), S. pneumoniae (7/51, 13.7%), human herpesvirus type 6 (6/51, 11.8%), S. agalactiae (3/51, 5.9%), herpes simplex virus type 2 (1/51, 2.0%), and E. coli (1/51, 2.0%). Aseptic meningitis (OR, 3.24, 95% CI, 1.18-12.73) and a duration of <2 days from onset of symptoms to CSF test (OR, 3.56, 95% CI, 0.1-0.91) significantly contributed to detection of pathogens by the FA-MEP. Among the 14 children who were administered empiric antibiotics before the CSF test, the detection rate was significantly higher in the FA-MEP than in the conventional test (28.6 vs. 0.0%, p = 0.031). CONCLUSIONS: FA-MEP had a higher detection rate in children with M/E compared with conventional tests, particularly aseptic meningitis, and in case of shorter duration of time-to-test. This test was more effective than the conventional test in pediatric M/E patients that had been administered empiric antibiotics.

Myelin oligodendrocyte glycoprotein antibody-associated disorders: clinical spectrum, diagnostic evaluation, and treatment options.

Inflammatory or immune-mediated demyelinating central nervous system (CNS) syndromes include a broad spectrum of clinical phenotype and different overlapping diseases. Antibodies against myelin oligodendrocyte glycoprotein (MOG-Ab) have been found in some cases of these demyelinating diseases, particularly in children. MOG-Ab is associated with a wider clinical phenotype not limited to neuromyelitis optica spectrum disorder, with most patients presenting with optic neuritis, acute disseminated encephalomyelitis (ADEM) or ADEM-like encephalitis with brain demyelinating lesions, and/or myelitis. Using specific cell-based assays, MOG-Ab is becoming a potential biomarker of inflammatory demyelinating disorders of the CNS. A humoral immune reaction against MOG was recently found in monophasic diseases and recurrent/multiphasic clinical progression, particularly in pediatric patients. This review summarizes the data regarding MOG-Ab as an impending biological marker for discriminating between these diverse demyelinating CNS diseases and discusses recent developments, clinical applications, and findings regarding the immunopathogenesis of MOG-Ab-associated disorders.

Autonomy is not but competence and relatedness are associated with physical activity among colorectal cancer survivors.

PURPOSE: The main objective of this study was to use the framework of the self-determination theory, incorporating both internal and external sources of motivation, to identify factors influencing physical activity among colorectal cancer survivors (CRC-S) in Korea. METHOD: In total, 242 patients at a university-affiliated hospital in Seoul, Korea, responded to a descriptive survey, which comprised questionnaire sets including the Global Physical Activity Questionnaire and the Patient Health Questionnaire. Motivation was then assessed on three scales: the Treatment Self-Regulation (autonomy), Perceived Competence (competence), and the multidimensional Scale of Perceived Social Support (relatedness). Logistic regression analysis was then used to identify factors associated with physical activity. RESULT: The mean physical activity score was 16.07 metabolic equivalent hours per week, and only 23.3% of patients had an appropriate level of exercise. In the logistic regression analysis, physical activity was associated with competence (odds ratio (OR) = 1.36, 95% confidence interval (CI): 1.06-1.74), relatedness (OR = 1.11, 95% CI: 1.04-1.18), depression (OR = 0.84, 95% CI: 0.75-0.94), and stage I or II disease (OR = 3.33, 95% CI: 1.28-1.86). This study indicated that competence, relatedness, depression, and the disease stage contributed to physical activity among these subjects while autonomy did not. CONCLUSION: Future interventions to achieve the recommended levels of physical activity among CRC-S could benefit from taking into account the disease stage as well as psychosocial factors including motivation and depression.

EEG Characteristics and Diagnostic Implications in Childhood Headache: A Multi-Center Study.

Introduction: Epilepsy and migraines are frequently observed as comorbidities, with the occurrence of one disorder increasing the probability of the other. The aim of our study was to evaluate the EEG characteristics by the type of headache and the implications of EEGs in headache patients, comparing the clinical characteristics and treatments between the headache patients with normal and abnormal EEGs. Methods: We conducted a retrospective analysis reviewing the medical records of 259 patients with headaches who visited the pediatrics departments of five university hospitals and underwent EEGs over a period of 3 years. Based on the data entered, analyses of the following items were conducted: (1) comparison of the EEG abnormalities by the type of headache and the characteristics of the EEG findings and (2) comparison of the clinical characteristics between patients with normal and abnormal EEGs. Results: Of the 259 patients, 31 showed abnormal EEGs, while 228 had normal EEGs. Of the 31 patients with abnormal EEGs, 17 showed epileptiform discharges, and 11 showed rhythmic slowing. The frequency of EEG abnormalities was significantly high in patients with migraines with auras than other types of headache. The Pediatric Migraine Disability Assessment (PedMIDAS) score was significantly higher in the abnormal EEG group compared with the normal EEG group (p = 0.001). Conclusion: The results of this study suggest that the abnormal EEG group had more significant disruptions in their daily lives due to headaches than the normal EEG group and that patients with migraines with aura may need EEGs and they might also have overlapping pathophysiologic mechanisms with epilepsy.

Longitudinal changes in insulin resistance in children with epilepsy on ketogenic diet: Prevalence and risk factors.

INTRODUCTION: The aim of the study was to evaluate the incidence of insulin resistance (IR) and the associated risk factors in children with epilepsy on a ketogenic diet (KD). METHODS: This longitudinal cohort study analyzed data of children with epilepsy on KD. Insulin resistance was assessed using the homeostasis model assessment of insulin resistance (HOMA-IR). The HOMA-IR value, fasting serum insulin levels, fasting glucose (FG) levels, and lipid profiles were measured before the initiation of the KD and at 6- to 12-month intervals. RESULTS: A total of 28 children were enrolled. The median age at the initiation of KD was 2.7 ±â€¯2.4 years, and the median follow-up duration was 2.1 ±â€¯1.4 years. The median HOMA-IR (HOMA-IR-1) value before the initiation of KD was 1.2 ±â€¯0.2, which significantly increased to 1.8 ±â€¯0.3 at the last follow-up (HOMA-IR-2; ∆HOMA-IR = 0.6 ±â€¯0.3, p < 0.001). The following factors were associated with patients with higher HOMA-IR-2 values (≥1.9): younger age at seizure onset (0.3 ±â€¯0.2 years, p < 0.001), at the initiation of antiepileptic drugs (AEDs; 0.3 ±â€¯0.3 years, p < 0.001), and at the initiation of KD (1.3 ±â€¯0.5 years, p < 0.001) and higher serum alanine transaminase (ALT; 84.0 ±â€¯17.8 U/L, p = 0.022), total cholesterol (TC; 245.0 ±â€¯20.1 mg/dL, p = 0.001), low-density lipoprotein cholesterol (LDL-C, 103.0 ±â€¯6.7 mg/dL, p = 0.003), and triglyceride (387.0 ±â€¯28.8 mg/dL, p < 0.001) levels. Multivariate regression analysis revealed that the age at seizure onset (p = 0.002), at initiation of AEDs (p = 0.021), and at initiation of KD (p = 0.022) and serum levels of LDL-C (p = 0.012) and triglycerides (p = 0.026) were associated with a significantly high HOMA-IR-2 value. CONCLUSION: Close monitoring of serum lipids levels, especially at younger age, may aid in detecting exacerbation of IR.

Predictive value of an early amplitude-integrated electroencephalogram for short-term neurologic outcomes in preterm infants.

BACKGROUND AND OBJECTIVES: The aim of this study was to compare serial scores of amplitude-integrated electroencephalography (aEEG) in preterm infants with favorable neurologic outcome compared with those with unfavorable neurologic outcome and to evaluate whether aEEG in the early days of life has predictive value for short-term neurologic outcome in preterm infants. METHODS: This prospective observational study included infants born at ≤32 weeks of gestational age and ≤1,500 g of birth weight. On the basis of brain ultrasonography findings, the infants were divided into two groups (favorable and unfavorable outcome group) at 36 weeks of corrected age or at discharge. aEEG was performed at 12-14 h (day-1), 46-48 h (day-2), 70-72 h (day-3), and 1 week (day-7) of life. The aEEG recordings were analyzed using the criteria described by Burdjalov et al.23 and the serial scores of aEEG were compared between the two groups. RESULTS: Thirty five infants were enrolled and 18 infants and 17 infants were identified into both groups, respectively. Infants in the favorable outcome group showed high scores in almost all parameters and the score of all parameters increased over time. However, the scores of all components decreased in day-2 compared with those of day 1 in the unfavorable outcome group. The total score less than 3 of day-2 has predictive value of 70.6% of sensitivity and 72.2% of specificity for unfavorable outcome. CONCLUSION: We found that aEEG is a useful predictor for short-term neurologic outcome in preterm infants.

Bone health in pediatric patients with neurological disorders.

Patients with neurological disorders are at high risk of developing osteoporosis, as they possess multiple risk factors leading to low bone mineral density. Such factors include inactivity, decreased exposure to sunlight, poor nutrition, and the use of medication or treatment that can cause lower bone mineral density such as antiepileptic drugs, ketogenic diet, and glucocorticoids. In this article, mechanisms involved in altered bone health in children with neurological disorders and management for patients with epilepsy, cerebral palsy, and Duchenne muscular dystrophy regarding bone health are reviewed.