Mean Weight Z-scores Research Articles

Social determinants of health and somatic growth in preterm infants and children with chronic lung disease.

Bronchopulmonary dysplasia (BPD), a common complication of prematurity, is associated with impaired lung function and increased morbidity in childhood. These children display heterogeneous somatic growth patterns which may ultimately impact their risk for later respiratory disease. We aim to understand the relationship between socioenvironmental exposures and growth in this population. A retrospective longitudinal cohort study was conducted using participants (n = 616) enrolled in the Johns Hopkins Outpatient BPD Registry. Growth measurements between 0 and 36 months of age were obtained. Somatic growth measurements, both corrected and uncorrected for gestational age, were converted to z-scores using Center for Disease Control and Prevention normative data. Using a participant's residential zip code, we utilized the 2019 Area Deprivation Index (ADI), the Childhood Opportunity Index (COI) 2.0, and neighborhood food insecurity data from the Maryland Food Bank. Linear regression analysis was performed employing individual socioenvironmental measures as independent variables and growth z-scores as dependent variables in univariate analysis. Univariate analysis was repeated adjusting for gestational age, BPD severity, and the presence of a gastrostomy tube (GT). Of the three measures analyzed, national COI (nCOI) showed a significant association with mean weight z-scores, mean ADI showed some association with mean weight z-scores, and the percentage of a community experiencing food insecurity showed no association with mean weight z-scores. After adjusting for gestational age, BPD severity, and presence of a GT, children living in areas of greater opportunity (higher nCOI values), had significantly lower weight z-scores at 12 months corrected and at 24 and 36 months uncorrected CONCLUSIONS: Our findings suggest that the COI 2.0, a multidimensional measure, captures more facets of an individual's social environment, as compared to the singular nature of a measure of food insecurity. There are several potential explanations for the phenomenon seen, and further understanding of this dynamic is crucial for designing effective interventions and policies to better address inequities in outcomes.

Nutritional and gastrointestinal manifestations in Rett syndrome: long-term follow-up.

Rett syndrome is a rare neurodevelopmental disorder associated with methyl CpG binding protein 2 (MECP2) gene mutations. We aimed to characterize the long-term nutritional and gastrointestinal course of Rett syndrome in a large national patient population. We conducted a retrospective cohort study of patients followed during 1991-2021 at a national center for Rett syndrome. The data retrieved included clinical features, laboratory and genetic analyses. Continuous anthropometric measurements were calculated for the closest visit to the median ages: 2.5, 7.5, 12.5 and 17.5 years. Kaplan Meier curves were used to describe the appearance of clinical manifestations during the follow up period. Generalized estimating equation models were used to compare repeated measurements. Included were 141 patients (139 females), the median age at the first visit was 3.2 years (interquartile range [IQR] 2.3-5.7), and the median length of follow-up was 94.5 months (IQR 28.6-153.3). Mean weight, height and BMI Z-scores were -1.09, -1.03 and -0.56, respectively, at median age 2.5 years; and deteriorated to -3.95, -3.01 and -1.19, respectively, at median age 17.5 years (P < 0.001). Gastrointestinal features included constipation (47.5%, 67/141) and chewing/feeding difficulties (20%, 28/141) at presentation; and an additional 47 (33.3%) and 24 (17.0%), respectively, during follow up. Twenty-eight patients (20%) developed aerophagia and 44 (31.2%) gastroesophageal reflux. No relation was found between genetic mutation types and clinical manifestations. GI manifestations were more prevalent in patients with typical form of Rett syndrome. Anthropometric parameters were shown to deteriorate with age, regardless of the specific genetic mutation. Chewing/feeding difficulties, constipation and gastroesophageal reflux are common in Rett patients.

Growth development of children and adolescents with inflammatory bowel disease in the period 2000–2014 based on data of the Saxon pediatric IBD registry: a population-based study

BackgroundThe incidence of inflammatory bowel disease (IBD) in children is on the increase worldwide. Growth disorders are common in pediatric patients with inflammatory bowel disease. The aim of this paper is to investigate anthropometric indicators, including height and weight in children with inflammatory bowel disease in Saxony, one of the German federal states, and to evaluate growth trends in patients by comparing their height and weight with that of healthy children in Germany.MethodsIn Saxony, all children and adolescents with IBD were registered in the Saxon Pediatric IBD Registry from 2000 to 2014. The data used are therefore based on a total area-wide survey over 15 years. For this study, 421 datasets of children and adolescents aged 0–14 years with Crohn’s disease (CD) (n = 291) or ulcerative colitis (UC) (n = 130) were analyzed.Z-score and percentile calculations were used to compare differences between IBD patients and the general population.ResultsThe children with CD or UC (both sexes) had a significant lower weight at diagnosis (the mean weight z-score had negative values) versus the general population. The weight values lay mostly below P50 (the 50th percentile, median), more precisely, mostly between P10 and P50 of the body weight child growth curve for corresponding sexes (KiGGS 2003–2006).The height values of both sexes at diagnosis lay also mostly below P50 (the 50th percentile, median) of the child body growth curve for corresponding sexes (KiGGS 2003–2006), i.e. the mean height z-score was negative. But only the children with CD had a significant lower height, more precisely, mostly between P25 and P50 versus the general population (KIGGS). For children with UC the difference was not significant.ConclusionIn pediatric patients with IBD the possibility of growth disturbance, mainly in the form of weight retardation, is very probable.

Growth and neurodevelopmental outcome in newborns with congenital non-cardiac surgical anomalies: A single-centre retrospective study

BackgroundSurvival of infants with congenital anomalies has increased dramatically, shifting the focus of clinical care and research toward optimising growth and neurodevelopment. ObjectiveTo evaluate growth and determine the impact of growth on neurodevelopmental outcomes of neonates born at term gestation with congenital non-cardiac surgical anomalies (NCSA), who required a laparotomy. MethodsThis was a single-centre retrospective cohort study. Demographic details, measurements of growth, and mean scores of Bayley Scales of Infant and Toddler Assessment, third edition, were collected. Means z-scores of growth measurements at birth, discharge and one-year-assessment were compared. Regression analysis was performed to explore predictive ability of growth measurements on mean scores of developmental assessment. ResultsA total of 105 infants were included. Mean z-scores for weight (-0.85) and head circumference (-0.34) at birth, though below population mean, were age-appropriate. Statistically significant decrease in z-score for weight was noted at discharge. Mean scores across the domains developmental assessment were normal. Z-scores for weight and height at one-year assessment predicted scores of gross motor (p-value 0.005) and cognitive development (p value 0.03) respectively. ConclusionsNeonates born at term gestation with NCSA had normal growth at birth. Postnatal growth restriction was noted at discharge. This loss in growth was not fully recouped at one-year assessment. As growth failure at one-year assessment could predict scores of developmental assessment, this group of infants needs careful nutritional and developmental monitoring.

1117-P: Variability in Glycemia Measured by CGM in Children and Young Adults with Acute Recurrent or Chronic Pancreatitis

Post pancreatitis diabetes mellitus is an underrecognized disorder. Children with pancreatitis are at elevated risk for development of diabetes (DM) in the near term (approximately 9%), and lifetime risk of DM approaches 50%. Unfortunately, there is limited data regarding pathophysiology and disease course. This pilot study was undertaken to investigate glycemic physiology in the free-living state in children with acute recurrent (ARP) or chronic pancreatitis (CP). Methods: Children (&amp;lt;21 years of age) with an established diagnosis of CP or ARP, and participants of INSPPIRE-2 (INternational Study Group of Pediatric Pancreatitis: In search for a cuRE) were invited to participate. Pancreatitis type, demographics and growth parameters were extracted from the INSPPIRE-2 database. HbA1c was collected. A continuous glucose monitor was placed (CGM, Dexcom Pro). Glycemic variability metrics were calculated at the participant level using EasyGV software. The relationship between HbA1c and CGM variables was evaluated via multiple linear regression. Results: 12 children were enrolled, age 10-20.8 years (mean 17.2; 66% female). One subject had known DM prior to enrollment. Participants had normal mean height (0.19+/- 0.72 SD), weight (1.1 +/- 0.98SD) and BMI (0.98 +/- 0.81 SD) z-scores. Mean A1c was 5.73% (range 5.0-8.9) with the known diabetic and 5.42% (5.0-6.1) without. Mean CGM glucose was 121.8 mg/dl +/- 21.4. Of the 11 subjects without DM, 2 had CGM glucose &amp;gt;180 mg/dl &amp;gt; 10% time and 1 had CGM glucose &amp;lt;55 mg/dl &amp;gt; 1% time. HbA1c correlated with mean CGM glucose in univariate analysis and when controlled for age and BMI (p = 0.02, r2 = 0.47). Conclusions: In this small study, A1c correlated well with average CGM glucose in children with ARP and CP, and some had abnormal glycemic excursions on CGM. More research is needed on the use of CGM in children with CP and ARP. Disclosure M.G.Parra villasmil: None. M.Bellin: Consultant; Insulet Corporation, Vertex Pharmaceuticals Incorporated, Research Support; Dexcom, Inc., ViaCyte, Inc. C.Pinnaro: None. K.L.Ode: None.

Growth and risk of adverse neuro-developmental outcome in newborns with congenital heart disease: A single-centre retrospective study

BackgroundMortality in infants with CHD is decreasing and focus has shifted to improving long-term outcomes. Growth and neurodevelopmental outcomes are important long-term endpoints for both clinicians and parents. ObjectiveTo evaluate growth and determine the impact of growth on neurodevelopmental outcomes at one year of age in infants who had an operative or therapeutic catheter procedure for CHD in neonatal period. MethodsThis was a single-centre retrospective cohort study of infants born at term gestation with CHD. Demographic details, measurements of growth, and scores of Bayley Scales of Infant and Toddler Assessment (third edition), were collected. Enrolled study participants were categorised in subgroups based on the procedures required prior to one-year assessment. Regression analysis was performed to explore predictive ability of anthropometric measurements on mean scores of developmental assessment. ResultsA total of 184 infants were included in the study. Mean z-scores for weight and head circumference at birth were age-appropriate. Mean scores for various developmental domains ranged from borderline to normal except for infants with single ventricular physiology who had gross motor delay concomitant with growth failure. In this group, z-score of weight at one-year-assessment predicted mean score of cognition (p-value 0.02), fine motor (p-value 0.03) and gross motor nearly (p-value 0.06). ConclusionsInfants born at term gestation, with CHD, without genetic diagnosis had normal fetal growth. Postnatal growth restriction and developmental delay was most marked in infants with single ventricle physiology, suggesting careful nutritional and developmental monitoring.

Analysis of Growth Velocity in Children with Attention-Deficit/Hyperactivity Disorder Treated for up to 12 Months with Serdexmethylphenidate/Dexmethylphenidate.

Objective: Serdexmethylphenidate/dexmethylphenidate (SDX/d-MPH) is approved for the treatment of patients aged ≥6 years with attention-deficit/hyperactivity disorder (ADHD). A 12-month, open-label safety study with SDX/d-MPH in children with ADHD showed that SDX/d-MPH was well tolerated and comparable with other methylphenidate products. In this post hoc analysis of the 12-month study, the objective was to characterize the effect of SDX/d-MPH on growth in children with ADHD over 12 months. Methods: This was a post hoc analysis of a dose-optimized, open-label, phase 3 safety study of SDX/d-MPH in children aged 6-12 years with ADHD (NCT03460652). Weight and height Z-score analyses were conducted. Z-score change from baseline was calculated based on the baseline values for the subjects remaining in the study at the observation time point. Results: Subjects (N = 238) from the treatment-phase safety population included all enrolled subjects who received ≥1 dose of study drug and had ≥1 postdose safety assessment. During treatment, the mean weight and height Z-scores decreased over time from their respective baselines. At the 12-month time point, mean (standard deviation [SD]) Z-score changes from baseline for weight and height for the subjects remaining in the study were -0.20 (0.50) and -0.21 (0.39), respectively; however, these mean changes in Z-scores were not clinically significant (change <0.5 SD). Long-term treatment with SDX/d-MPH was associated with modest reductions in expected weight and lower-than-expected increases in height: effects that plateaued or diminished later in treatment. Conclusion: The overall effects of SDX/d-MPH on growth velocity (the change in weight and height from one time point to the next) were minimal, and the range of changes was not considered clinically significant. ClinicalTrials.gov identifier: NCT03460652.

Improvement of Diet after an Early Nutritional Intervention in Pediatric Oncology

Pediatric cancer survivors may experience cardiometabolic sequelae over the course of their lives as a result of the treatments they have received. While nutrition consists of an actionable target for cardiometabolic health, few nutritional interventions have been documented in this population. This study assessed the changes in diet during a one-year nutritional intervention for children and adolescents undergoing cancer treatments and the participants' anthropometric and cardiometabolic profiles. A total of 36 children and adolescents (mean age: 7.9 years, 52.8% male) newly diagnosed with cancer (50% leukemia) and their parents underwent a one-year individualized nutrition intervention. The mean number of follow-up visits with the dietitian during the intervention was 4.72 ± 1.06. Between the initial and one-year assessments, there was an improvement in diet quality reflected by the Diet Quality Index (5.22 ± 9.95, p = 0.003). Similarly, the proportion of participants with moderate and good adherence (vs. low adherence) to the Healthy Diet Index score almost tripled after one year of intervention (14% vs. 39%, p = 0.012). In parallel, there was an increase in the mean z-scores for weight (0.29 ± 0.70, p = 0.019) and BMI (0.50 ± 0.88, p = 0.002), and in the mean levels of HDL-C (0.27 ± 0.37 mmol/L, p = 0.002) and 25-hydroxy vitamin D (14.5 ± 28.1 mmol/L, p = 0.03). Overall, this study supports that a one-year nutritional intervention deployed early after a pediatric cancer diagnosis is associated with an improvement in the diets of children and adolescents.

Growth trajectories in children with cleft lip and/or palate.

Introduction: the nutritional status and growth of children with cleft lip and/or palate (CL/P) can be affected due to feeding difficulties caused by their anatomy and the surgical interventions. Objective: this retrospective longitudinal study aims to analyse the growth trajectories of a cohort of children with CL/P and compare them with a healthy representative cohort of children from Aragon (Spain). Methods: type of cleft, surgical technique and sequelae, and weight, length/height and body mass index (BMI) (weight/height2) at different ages (0-6 years) were recorded. Normalized age- and sex-specific anthropometric Z-scores values were calculated by World Health Organization (WHO) charts. Results: forty-one patients (21 male, 20 female) were finally included: 9.75 % cleft lip (n = 4/41), 41.46 % cleft palate (n = 17/41) and 48.78 % cleft lip and palate (n = 20/41). The worst nutritional status Z-scores were achieved at the age of three months (44.44 % and 50 % had a weight and a BMI lower than -1 Z-score, respectively). Mean weight and BMI Z-scores were both significantly lower than controls at one, three and six months of age, recovering from that moment until the age of one year. Conclusions: the highest nutritional risk in CL/P patients takes place at 3-6 months of age, but nutritional status and growth trajectories get recovered from one year of age compared to their counterparts. Nevertheless, the rate of thin subjects among CL/P patients is higher during childhood.

Feeding Practice, Nutrition, and Growth in Infants with Abdominal Wall Defects and Esophageal Atresia: A Retrospective Case-Control Study.

Infants born with abdominal wall defects and esophageal atresia (EA) are at risk of impaired growth. Little is known about the optimal nutritional strategy and its impact on growth for these infants. This study aims to explore nutrition, focusing on breastfeeding, and the presumed impact on infant growth during the first year of life. We performed a registry study. The participants comprised infants born with gastroschisis, omphalocele, or EA from 2009 to 2020. Breastfed healthy infants from the Odense Child Cohort served as the control group. Descriptive statistics were applied when presenting data on nutrition. Growth data were converted to weight z-scores at birth and at discharge, and estimated weight z-scores at 6 and 12 months were calculated. Univariate regression analysis was applied. The study included 168 infants in the study group and 403 infants in the control group. Exclusive breastfeeding rates at discharge were as follows: 55.7% (gastroschisis), 58.3% (omphalocele), 50.9% (EA), and 7.7% (long-gap EA). For the study group our data demonstrate no difference in growth at 1 year of age when comparing mother's milk to formula feeding. During the first year of life, infants in the study group showed slower growth compared with the control group. At 12 months of age, all infants had a mean weight z-score above -2. Breastfeeding in infants with abdominal wall defects and EA can be established without compromising growth. Mother's milk can be recommended for infants with abdominal wall defects and EA.

Comparison of two Specialized Cleft Palate Feeders.

To evaluate feeding efficiency and weight gain in infants with cleft palate fed using 1 of the 2 specialty feeders. Retrospective cohort study. Cleft palate clinic in a tertiary pediatric hospital. Infants with cleft palate (with or without cleft lip) fed using the Medela SpecialNeeds® Feeder (n = 39) or the Dr. Brown's® Specialty Feeding System (n = 16) and who had documented feeding and growth data available from the time of initial assessment in the first month of life and at subsequent follow-up between 2 and 4 months. Feeding velocity (mL/min). Calorie velocity (kcal/min), weight gain, and complications associated with poor feeding. No statistically significant differences in feeding or calorie velocities were identified between infants with cleft palate fed with the Medela SpecialNeeds® feeder and those fed with the Dr. Brown's® feeder. Mean weight z-scores by month did not differ significantly between the 2 bottle groups at the time of initial assessment (P = .84) and follow-up (P = .20). Mean weight z-scores by month for the infants included in this study fell below the 50th percentile. The proportion of infants who developed otitis media, reflux requiring treatment, or who required hospital admission for nasogastric (NG) feeds did not differ significantly between the 2 groups. Despite being adequately powered for the primary outcome, no significant differences were identified between infants fed with the Medela or the Dr. Brown's feeders in terms of feeding velocity, calorie velocity, weight gain, or complications.

Current epidemiology and factors contributing to postnatal growth restriction in very preterm infants in China

BackgroundPostnatal growth restriction (PGR) is common in very preterm infants (VPIs) and is associated with adverse short and long-term developmental outcomes. Postnatal growth status for VPIs in middle- or low-income countries remains unclear. AimsTo evaluate PGR in VPIs and identify maternal and neonatal factors, clinical practice, and major neonatal morbidities associated with PGR in China. Study designProspective cohort study. SubjectsWe included 6085 infants born at <32 weeks gestation who were admitted at 57 hospitals in the Chinese Neonatal Network in 2019. Outcome measuresBirth and discharge weights were converted to age-specific Z-scores. PGR was defined as a decrease in weight z-score from birth to discharge >2. ResultsThe overall incidence of PGR was 19.9 %. The mean (standard deviation [SD]) weight Z-score was 0.12 (0.78) at birth and decreased to −1.36 (0.98) at discharge. About 4.0 % of VPIs were small for gestational age (SGA) at birth and 25.5 % of SGA infants had PGR. The incidence of PGR increased with decreasing gestational age except in the SGA subgroup. Each 1-unit increase in birthweight Z-score was associated with a 1.49-fold increased risk for PGR. Late initiation of enteral feeds and late achievement of full enteral feeds were positively associated with PGR. The common morbidities that influenced PGR were necrotizing enterocolitis ≥ stage II, patent ductus arteriosus requiring medical or surgical treatment, sepsis, bronchopulmonary dysplasia, and respiratory distress syndrome requiring surfactants. ConclusionNearly one fifth of VPIs were PGR, and one fourth of SGA had PGR, which warranted further study to investigate underlying causes by which to improve postnatal growth in very preterm infants in future.

Extrauterine growth restriction in preterm infants: Postnatal growth pattern and physical development outcomes at age 3-6 years.

ObjectivesTo investigate the postnatal growth trajectories of preterm infants and evaluate the association between extrauterine growth restriction (EUGR) at discharge and adverse physical growth outcomes at age 3–6 years.MethodsPremature infants admitted to Shanghai Children’s Medical Center within 24 h after birth from 1 January 2016 to 31 December 2018 were enrolled. Neonatal complications, nutrition support, and anthropometric data were collected and analyzed to diagnose EUGR on different definitions at discharge. The weight and the height of each subject were collected by telephone investigation from 1 September 2021 to 31 November 2021 to access the incidences of overweight/obesity, short stature, and thinness at age 3–6 years.ResultsA total of 527 preterm infants were included in the final sample. The overall mean weight and height Z-scores were –0.37 ± 0.97 SD and –0.29 ± 1.18 SD at birth, and increased to –0.03 ± 1.11 SD and 0.13 ± 1.2 SD at follow-up, respectively. The logistic regression analysis indicated longitudinal EUGR on head circumference as the risk factor of overweight or obesity, cross-sectional EUGR on height as the risk factor of short stature, and delayed EN as the risk factor of thinness.ConclusionThe growth trajectories of the preterm newborns tended toward the normal direction. Longitudinal EUGR on the head circumference and cross-sectional EUGR on height at discharge were associated with adverse physical growth outcomes at age 3–6 years.

Long-Term Growth in Children and Young People with Autoimmune Liver Disease Treated with Daily Steroids.

We aimed to evaluate long-term growth in children and young people with autoimmune liver disease (AILD) treated with daily steroids. This is a retrospective observational cohort study of patients diagnosed between 1992 and 2004 before the age of 16 years. Growth measurements (height, weight and body mass index (BMI)) converted to z-scores were recorded, at diagnosis, 1 and 5 years after commencing treatment and at age 18 years and analyzed together with demographics, disease and treatment related characteristics. Seventy-four patients (35 female) were started on treatment at median age of 12.85 (Inter quartile range (IQR) 9.44, 14.14) years for median duration of 12.07 (IQR 8.68, 13.97) years. At all time-points, the mean z-scores for weight, height and BMI were within the normal range, indicating normal nutritional status. There was no difference in change in z-score for weight, height and BMI from diagnosis until age 18 years when comparing gender (male vs female), ethnicity (Caucasian vs non-Caucasian), diagnosis (AIH vs ASC) and presence of IBD (n = 23). Change in z-score was lower for height and weight for the < 12 years group compared to the ≥12 years age group ( P < 0.05 and P < 0.05, respectively). In addition, change in height z-score correlated positively with age at start of steroid treatment (r = 0.321, P < 0.05) and negatively with duration of steroid treatment (r = -0.321, P < 0.05). Growth of patients with AILD on a daily maintenance dose of steroids remains stable and within normal range during long-term follow up. Small, daily doses are effective in maintaining disease control and minimize the need for high-dose steroid pulses during relapses.

Goat Milk Based Infant Formula in Newborns: A Double-Blind Randomized Controlled Trial on Growth and Safety.

Objectives:We aimed to determine the growth and safety parameters in newborns fed a goat milk based infant formula (GMF) using a randomized double-blind trial, in which a cow milk formula (CMF) served as a control and a breast fed (BF) group as a reference.Methods:Healthy term infants (n = 218) aged up to 14 days were recruited from 25 European study centers and randomized to GMF or CMF. Weight, length, head circumference were measured at baseline, and at 14, 28, 56, 84, and 112 days at the study clinics. Adverse events were recorded and stool characteristics, reflux, fussiness, colic, and flatulence were self-reported by parents in 3-day diaries. Anthropometric measurements were transformed to WHO standardized age- and sex-adjusted z-scores. Analyses of covariance and linear mixed modeling were used to statistically analyze growth, while adjusting for potential confounders when studying the breast-fed group (n = 86).Results:Comparing the GMF to the CMF group, weight gain [mean difference 227.8 g (95% CI −16.6 to −439.0)] and z-scores for anthropometric measurements were similar after 112 days intervention. Infant formula groups showed greater mean (SD) weight z-scores than the BF group from 84 days onwards (GMF: 0.28 (0.84), CMF: 0.12 (0.88), BF −0.19 (1.02), P < 0.05), whereas length and head circumference z-scores were similar. Incidences of serious adverse events and reflux, fussiness, colic, and flatulence were similar among the three groups.Conclusion:Our data demonstrate that GMF provides adequate growth, has a good tolerability, and is safe to use in infants.

Effects of Classic Ketogenic Diet in Children with Refractory Epilepsy: A Retrospective Cohort Study in Kingdom of Bahrain.

Background: The classic ketogenic diet (cKD) has been used worldwide as an effective therapy for children with drug-resistant epilepsy. However, there have been no studies performed in Middle Eastern countries in order to assess the efficacy, side effects, predictors of cKD response and factors mostly associated with diet adherence. This study aims to assess the efficacy of cKD ratios of 4:1 and 3:1 and their influence on growth and biochemical parameters, particularly lipid profile and liver function tests (LFTs), and the factors most associated with diet adherence in a cohort of children with drug-resistant epilepsy in Bahrain. Methods: Baseline and follow-up data related to patients’ demographic and biochemical variables, epilepsy episodes, diet history and anthropometric measurements were retrieved for a total of 24 children treated with cKD in Bahrain. Results: After 6 months cKD initiation, 58.3% were positive responders with >50% seizure rate reduction, and 33.3% became seizure-free at 12 months. After 6 months of intervention with cKD, the level of triglycerides and albumin had a significant (p < 0.05) average increase over time of +1.47 mmol/L and 4.3 g/L, respectively. Although the median values of total cholesterol and alanine transaminase increased, respectively, following cKD initiation, the difference over time was not statistically significant. The mean z-scores for weight, height, and body mass index (or weight-for-length) did not change significantly at 12 months follow-up. cKD duration was the highest correlated variable with cKD efficacy (r = 0.76), which was followed by age at cKD initiation (r = 0.47). The cKD was discontinued by 14 patients (58.3%) during the first follow-up period (6 months), which was mainly due to inefficacy (n = 8), poor compliance (n = 3), food refusal (n = 1), achieved required efficacy (n = 1) and death (n = 1). Conclusions: cKD is an effective treatment for patients with drug-resistant epilepsy, and positive response to cKD was the main factor that increased adherence to the diet. Although long-term cKD could increase the risk of dyslipidemia and hepatic problems, it appears safe for children. Consequently, close monitoring and emphasis on healthy fats is of high priority.

Effect of Sodium Valproate on Weight, Body Mass Index, Uric Acid, Vitamin D3, Blood Insulin, and Serum Lipid Profile in Children

Introduction: Due to the high prevalence of epilepsy and the use of sodium valproate as an antiepileptic drug by these patients, accurate recognition of its side effects and its effects on serum lipids profile, liver enzymes, uric acid level, and thyroid function tests, especially in cases that need long-term treatment seems essential. This study aimed to evaluate the effects of sodium valproate on weight, body mass index (BMI), vitamin D3, blood insulin, uric acid level, and serum lipids profile in children with newly diagnosed epilepsy. Materials and Methods: This prospective study was performed on 30 children between 3 and 8 years of age who suffered from newly diagnosed epilepsy and received sodium valproate as monotherapy. Data including demographic information (age, sex, height, weight, and waist and hip circumference of children), as well as clinical characteristics, such as liver enzymes (ALT, AST, ALK-P), serum lipids level (TG, TC, HDL-C, LDL-C), thyroid tests (TSH, T4), fasting blood sugar (FBS), uric acid level, 25 OH Vitamin D3 (Vit-D3), and blood insulin level of children before and six months after the consumption of sodium valproate, were examined. Results: The mean weight of children before and six months after the start of sodium valproate treatment was 18.54±2.99 and 21.13±3.93 (kg), respectively. This difference was statistically significant (P=0.005). Also, the mean weight Z-score of children before and after taking sodium valproate was -2.497 and -2.293, respectively, which was statistically significant too. In addition to weight gain, there was also a significant increase in the abdominal and hip circumference of children after taking valproate, whereas the increase in mean BMI before and after valproate administration was not statistically significant (P=0.114). However, mean weight gain, as well as the increase in the waist and hip circumference, had no relationship with gender (P&gt; 0.05). Also, sodium valproate significantly increased the ALT level (P=0.046). Moreover, sodium valproate did not affect other liver function markers (AST), thyroid hormones (TSH, T4), fasting blood sugar (FBS), uric acid level, 25 OH Vit-D3, and the children's blood insulin levels (P&gt; 0.05). Conclusion: According to the findings of this study, it can be concluded that sodium valproate is a good drug for children between 3 and 8 years of age, but it should be noted that taking this drug increases the chance of obesity in children. The main side effect of this drug is weight gain. Due to the significant increase in ALT enzyme, as observed in this study, it is recommended to check liver enzymes before, one, and six months after starting treatment as it can prevent the irreversible permanent side effects of this drug.

Differences in longitudinal growth patterns of children and adolescents with transfusion-dependent hemoglobin E/β-thalassemia and those achieving successful hematopoietic stem-cell transplantation.

Short stature is a very common endocrinopathy among children with transfusion-dependent (TD) thalassemia. Hematopoietic stem-cell transplantation (HSCT) is the only effective curative treatment for TD thalassemia. This study aimed to identify and compare the longitudinal growth patterns of children with TD hemoglobin E (Hb E)/β-thalassemia against those of children successfully undergoing HSCT. We reviewed the medical records of 39 patients with TD Hb E/β-thalassemia receiving regular blood transfusions, and 39 post-HSCT patients. Longitudinal weight and height Z-scores at each year of age were recorded for TD patients, and longitudinal weight and height Z-scores at each year before and after HSCT were obtained for post-HSCT patients. The mean weight and height Z-scores of TD patients decreased gradually and were lowest at age 13. However, post-HSCT subjects saw significant improvement in their mean weight and height Z-scores 6 and 3years after HSCT, respectively, relative to pre-HSCT baseline values. Longitudinal growth patterns differed between patients successfully undergoing HSCT and children and adolescents with TD Hb E/β-thalassemia. HSCT significantly improved height outcomes of children and adolescents with TD Hb E/β-thalassemia.

Evaluating the use of laparoscopic-assisted gastrostomy tube feeding in children with epidermolysis bullosa: A single-center retrospective study

BackgroundNutritional management of children with epidermolysis bullosa (EB) presents multiple challenges including reduced oral intake compounded by mucosal fragility. Gastrostomy tube feeding is effective in improving nutritional status however there is limited data on the safety and tolerance of this technique in EB children. We aim to review the effectiveness and morbidity of our minimally invasive two-port laparoscopic-assisted gastrostomy (LAG) approach using Seldinger techniques with serial dilatations in children with EB. MethodsA retrospective, observational cohort study was conducted on all consecutive EB patients who underwent LAG tube insertion between 2009 and 2019. Patient demographics, admission details and 12-month clinical outcomes were reported. Results32 EB patients underwent LAG placement. Median age at insertion was 7.3 (IQR ± 6.3) years, with 8 (25.0%) and 3 (9.4%) of patients also undergoing oesophageal dilatation and fundoplication, respectively. Minor complications arose in 58.1% of patients including: peri-stomal overgranulation (25.8%), gastrostomy infection (22.6%), pain (22.6%), mild gastrostomy leakage (16.1%), blockage (9.7%) and device failure (3.2%). 2 patients (6.5%) developed major complications with extensive gastrostomy site leakage. Improvements in growth were reflected in mean height Z-scores (-1.99 to -1.71). Mean weight Z-scores improved in patients aged 0–10 years (-2.30 to -1.61) and mean BMI Z-scores increased in patients more than 10 years (-2.71 to -1.46). No cases of gastrostomy-related mortality were reported. ConclusionLAG is well-tolerated in EB patients with improvements in growth and minimal morbidity 12-months post-gastrostomy insertion. An extended follow-up period is required to ascertain the long-term implications of gastrostomy feeding.

Growth failure of very low birth weight infants during the first 3 years: A Korean neonatal network.

We aimed to evaluate risk factors for growth failure in very low birth weight (VLBW) infants at 18–24 months of corrected age (follow-up1, FU1) and at 36 months of age (follow-up2, FU2). In this prospective cohort study, a total of 2,943 VLBW infants from the Korean Neonatal Network between 2013 and 2015 finished follow-up at FU1. Growth failure was defined as a z-score below -1.28. Multiple logistic regression was used to analyze risk factors for growth failure after dividing the infants into small for gestational age (SGA) and appropriate for gestational age (AGA) groups. Overall, 18.7% of infants were SGA at birth. Growth failure was present in 60.0% at discharge, 20.3% at FU1, and 35.2% at FU2. Among AGA infants, male sex, growth failure at discharge, periventricular leukomalacia, treatment of retinopathy of prematurity, ventriculoperitoneal shunt status and treatment of rehabilitation after discharge were independent risk factors for growth failure at FU1. Among SGA infants, lower birth weight, pregnancy-induced hypertension, and treatment of rehabilitation after discharge were independent risk factors for growth failure at FU1. Mean weight z-score graphs from birth to 36 month of age revealed significant differences between SGA and non-SGA and between VLBW infants and extremely low birth weight infants. Growth failure remains an issue, and VLBW infants with risk factors should be closely checked for growth and nutrition.