Iranian Journal of Pediatrics
Volume:34 Issue: 1, Feb 2024

 It is crucial to determine the normal Portal Vein Diameter (PVD) in different populations, and ages since changes in the size of the vein are used as an index in the diagnosis of some diseases. Therefore, it is important to determine its normal size. In this regard, the present study aimed to investigate the PVD and Peak Systolic Velocity (PSV) in healthy Iranian children.

 The present descriptive-analytical study examined 250 healthy Iranian children who visited the Imaging Center of the Children Medical Center of Excellence. The PVD and PSV were examined by a radiologist using a Doppler ultrasound. Children were classified intofive age groups: under one month, 1 month to 2 years, 2 to 6 years, 6 to 10 years, and 10 to 18 years, and the above-mentioned indices were measured and compared in them. Statistical analysis was conducted using SPSS 26 employing the Mann-Whitney, Kolmogorov-Smirnov, and Kruskal-Wallis tests.

 In children under one month, one month to 2 years, 2-6 years, 6-10 years, and 10-18 years of age, the mean values of PVD were 4.08, 5.64, 6.14, 7.50, and 8.32 mm, and the mean PSV values were 19.26, 22.20, 21.68, 22.86, and 21.48, respectively. The mean PVD and PSV increased with increasing age; however, no statistically significant difference was found between the mean values of the indices in both boys and girls (P = 0.18).

 The PVD and PSV in healthy Iranian children were relatively similar to their non-Iranian counterparts, and the mean values increased with age. Additionally, no significant difference was found between the mean values of PVD and PSV in boys and girls based on gender.

 Cardiomyopathy (CM) is an important factor affecting inherited metabolic disorders (IMDs). Therefore, documentation of real-world data can increase awareness, and early diagnosis and treatment may improve the outcome.

 The aim of this study was to delineate the underlying etiologies and clinical findings of patients with CM due to IMDs.

 In this single-center retrospective study, we aimed to examine the demographic, clinical, and echocardiographic data and prognosis of patients aged 0 - 18 who were diagnosed with CM associated with IMDs between 2010 and 2020.

 A total of 102 patients were included in the study. Families with consanguineous parents constituted 84.3% of the study population. The mean age of the patients at the time of diagnosis of CM was 26.2 ± 28.2 months. Eighty-six (84.3%) of the patients were diagnosed with hypertrophic CM (HCM), 12 (11.7%) with dilated CM (DCM), and 4 (3.9%) with mixt-type CM. Of the patients, 60 (58.8%) had glycogen storage disease (GSD), 17 (16.6%) had lysosomal storage disorders (LSDs), 11 (10.7%) had fatty acid oxidation disorders, 11 (10.7%) had mitochondrial disorders, and 3 (2.9%) had amino acid and organic acid metabolism disorders. Arrhythmia was detected in 24 patients (23.5%). Fifty-six patients received enzyme replacement therapy (ERT). The echocardiographic parameters of the Pompe patients improved significantly with ERT. The mean follow-up period was 60.9 ± 54.6 months. Thirty-eight (36.1%) of 95 patients with regular follow-up died. Cardiomyopathy type did not affect mortality.

 Increasingly, cardiac complications have been defined as IMDs. Evaluation should be made in terms of IMDs in unexplained CM and rhythm disorders. On the other hand, patients with certain IMDs should be regularly screened for cardiac complications.

 The measurement of various components of infant growth is not only essential for monitoring infant health but is also associated with the well-being of infants and their parents, the use of human resources, frequent repositioning of infants, and a considerable amount of time.

 Therefore, the present study aimed to design and evaluate a device for the “simultaneous measurement of infants’ weight, height, head circumference, temperature, and heart rate”.

 This study follows a quantitative and semi-experimental approach. The “simultaneous measurement of weight, height, head circumference, temperature, and heart rate of a child” device was officially registered as an invention with the number 104232 on May 18, 2021. The sample for this study comprised 100 infants selected from hospitals affiliated with Shahid Beheshti University of Medical Sciences, Tehran, Iran. The data related to all 100 infants were collected and recorded using both routine measurement methods and digital devices. Data collection tools included the Wong-Baker Faces scale to assess infant distress and a demographic questionnaire for recording growth components and the time of measurement. Data analysis was conducted using SPSS software (version 20), and statistical significance was determined using the t-test (P < 0.05).

 The comparison of mean distress scores in both the control and intervention groups revealed a significant reduction in infant distress scores during all measurements except for weight (P < 0.05). It is worth noting that the number of infant repositions required for each measurement was reduced from 6 in the routine method to 2 with the digital device. Additionally, although 2 or 3 staff members were needed for measurements in the control group, only 1 staff member was sufficient for measurements in the intervention group.

 Based on the evaluation results of the digital device, it is anticipated that this device could serve as a suitable alternative to the routine methods of measuring infant growth components. However, further studies are warranted to explore the broader applications of this digital device.

 The quality and congeniality of the hospital educational environment (HEE) is a major determinant for the success of the ‎training of future health professionals. Satisfactory and effective engagement of fellow physicians in the clinical learning program ultimately affects their clinical performance.

 To evaluate and assess the HEE of residents and fellow trainees at a university hospital in Jeddah using a psychometric tool, Post-graduate Hospital Educational Environment Measure (PHEEM).

 The PHEEM questionnaire developed by Roff et al. (2005) was used to survey 71 pediatric post-graduate trainees in the second half of 2021. The HEE perception was correlated with the trainee’s demographic and academic data. Principal component ‎analysis was performed to examine the validity of the PHEEM 3-dimensional construct.

 Most of the participants were from the first (23.9%) and second post-graduate year (33.8%) and ‎had majorly general pediatrics as a specialty (83.1%). The mean PHEEM score was 99.35 ± 22.46 out of 160 with a distribution pattern of poor (2.8%), suboptimal (9.9%), more ‎positive with the need for improvement (73.2%), and optimal (14.1%). The PHEEM score was significantly lower among trainees of < 27 years (94.33 ± 23.48; P = 0.037) compared to the ones aged > 27 years (105.47 ± 19.83). Medical residents and trainees from the first post-graduate year scored remarkably low (87.86 ± 21.21; P = 0.008) compared to the other senior fellow peers. The correlation observed in overall PHEEM scores showed a similar trend in 3 individual components. For the principal component analysis (PCA), 10 components met the ‎initial criteria of eigenvalue > 1 and loading factor > 0.5, encompassing 75.9% of the scale ‎variance. The thematic analysis highlighted several areas for improvement, such as trainee rights and psychological support.

 The HEE of the pediatric department was broadly ‎suitable for post-graduate training programs. There are still several areas for improvement, including organizational and logistical aspects that include adequate learning time. ‎In addition, the psychological safety of trainees should also be considered.

 Children with type 1 diabetes are at risk of developing microvascular and macrovascular complications, with diabetic nephropathy (DN) being a significant microvascular complication. Microalbuminuria is a crucial diagnostic marker for the early detection of DN and can predict the risk of vascular complications.

 This study aimed to investigate the relationship between microalbuminuria and body mass index (BMI), changes in hemoglobin A1c (HbA1c), and the age of disease onset in children and adolescents with type 1 diabetes.

 This cross-sectional study was conducted in Mofid and Imam Hossein hospitals in Tehran, Iran, within 2021 - 2022. This study included 120 children and adolescents aged 5 - 20 years who had been diagnosed with type 1 diabetes for at least 3 years. All patients underwent evaluation for microalbuminuria, and its association with BMI, HbA1c levels, and the age of diabetes onset was assessed.

 Among the 120 patients, 51% were female. Microalbuminuria was present in 15% of patients; however, 13.3% were overweight or obese. Furthermore, 91% of patients had HbA1c levels above 7%. The mean age of diabetes onset was 13 years, and the mean disease duration was 5.5 years. The study revealed a significant association between microalbuminuria and HbA1c levels, BMI, and the age of diabetes onset (P-values < 0.05).

 Microalbuminuria showed a positive correlation with increased BMI and poorer glycemic control. An early age of diabetes onset was identified as a risk factor for microalbuminuria in children and adolescents with diabetes.

 Intoxications with cardiovascular system drugs constitute a small percentage of all poisoning cases. Calcium Channel Blockers (CCBs) are the most common drug poisoning in this group.

 We aimed to evaluate the effectiveness of treatments for CCB poisoning and add to the current body of literature by outlining the clinical treatments we employ for bradyarrhythmia, hypotension, and resistant vasodilation resulting from CCB poisoning, as well as sharing our clinical insights in this field.

 Twelve patients, ranging in age from 1 month to 18 years, were admitted to the Tertiary Paediatric Intensive Care Unit (PICU) for treatment of medication poisoning related to the CCB group. Patients who ingested several drugs that caused CCB were not allowed to participate in the trial.

 Twelve patients were followed up in the PICU due to poisoning with CCB group drugs. Of the patients, 7 were male and 5 were female. Five of the patients had taken CCB medication with the purpose of committing suicide, and 7 of them accidentally. All of the patients who received CCB to commit suicide had taken verapamil. Five patients whose hypotension and bradycardia continued were administered inotropes. In addition to PI, calcium gluconate, intravenous lipid, glucagon, insulin, bicarbonate, and methylene blue were given as therapy to our symptomatic patients. Plasmapheresis was applied to a patient who was hospitalized in the PICU due to a sudden loss of consciousness.

 In the management of patients with CCB poisoning, the use of hyperinsulinemia euglycemia, intravenous lipid emulsion treatment, glucagon treatments, and treatments including methylene blue and extracorporeal life support should be considered in cases of resistant hypotension, bradycardia, and coma in the early period.

 Elizabethkingia meningoseptica (EM) is a gram-negative aerobic organism that can cause severe infections. Although the incidence is higher in developing countries, human infections associated with this organism are seldom described in Serbia. This bacterium is usually resistant to most antibiotics, making the treatment challenging.We present a single twin born as a late-preterm newborn who developed sepsis and meningitis caused by EM.

 This case report aims to show that this rare bacterium is present in our country and the clinical presentation of the resulting disease and bacterial resistance to antibiotics. In addition, we assessed if a more thorough diagnosis, including the immunological status of the newborn, is needed.

 Considering the high mortality and morbidity rates of EM and its multiresistance, early identification of the infection cause and determining the patient’s immunological status are vital for treatment and improved patient outcomes.

 In some regions, the types and sizes of tunneled central venous catheters (CVCs) are limited.

 The objective of this study was to review our experience with 10F Quinton Permcath catheters used for long-term vascular access in children of various ages and weights with chronic renal failure.

 Children with renal failure on maintenance hemodialysis who underwent placement of the 10F Quinton Permcath between June 2017 and June 2023 at the First Affiliated Hospital of Jinan University were included. We collected clinical data from children with the Permcath, including patients' gender, age, weight, and height. Additionally, data on catheter-related complications were gathered. Imaging data of the patients were obtained to compare the catheter route on imaging and the position of the catheter tip in each case. This enabled an analysis of the characteristics of vascular access establishment and its correlation with catheter-related complications in 11 children implanted with 10F Quinton Permcath.

 Among the 11 children on maintenance hemodialysis with catheters, five were male, and six were female, with a median age of 9 years (range 8 - 10). The median body weight of the included patients was 23.00 kg (range: 19.95 - 24.30 kg). Eight cases involved placement in the right internal jugular vein (IJV), with the catheter tip located at the level of T8 in two cases, T5 in one case, and T7 in the remaining cases, with no early catheter dysfunction. Three patients underwent catheter placement in the left IJV, with the catheter tip located at the level of T5, T4, and the T7/T8 intervertebral space, respectively, and two cases of early catheter dysfunction occurred in two patients. There were no acute complications in the 11 cases following catheter insertion.

 Whenever feasible, the right IJV should be the preferred choice for establishing vascular access for the 10F Quinton Permcath in children with chronic kidney disease, regardless of age and weight. The tunnel route and catheter length within the tunnel can be adjusted to ensure the appropriate placement of double-lumen tunnelled cuffed catheters (TCC).
Keywords

 Celiac disease (CD) is an autoimmune disorder that harms the epithelium and lamina propria of the small intestine in genetically predisposed individuals. CD negatively affects oral and dental health.

 This research explored the DMFT index and enamel defects (ED), as well as their association with oral health-related quality of life in children with CD.

 In this descriptive-analytical cross-sectional study, 50 children with CD (case group) and 50 healthy children (control group) were examined. The World Health Organization criteria were used to determine the DMFT index. Aine's classification was employed to diagnose ED, and the standard CHILD-Oral Impacts on Daily Performances scale was used to assess child oral health-related quality of life (COHRQoL). The Chi-Square test and Mann-Whitney test were utilized for data comparison. P-values < 0.05 were considered statistically significant.

 The average DMFT was 2.58 ± 2.39 in the case group and 2.08 ± 1.61 in the control group. According to the Mann-Whitney U test, this difference was not statistically significant (P = .223). The frequency of ED in the case group was significantly higher at 52%, compared to 16% in the control group (P < .001). Additionally, there was a significant difference in the frequency of ED between boys and girls in the two groups (P < .001). The COHRQoL score in the case group, with an average of 24.44 ± 19.91, was significantly higher than that in the control group, which had an average of 13.35 ± 13.65 (P = .002). In both the case and control groups, a significant positive relationship was observed between the COHRQoL score in children and the DMFT index (P < .001). There was also a significant relationship between the COHRQoL score in children with ED in both the case and control groups (P = 0.004).

 According to the results, there was no difference in the DMFT index between the 2 groups. However, as the DMFT index increased in both groups, the COHRQoL score also increased. The number of ED cases was higher in celiac patients, and this had a significant effect on the COHRQoL score. Additionally, the COHRQoL score was higher in celiac patients compared to the control group.

 Autism is a neurological disorder that has seen an increase in prevalence in recent years. It is hypothesized that diet may influence mental health and improve the behavioral symptoms of autism.

 The purpose of this study is to examine the effects of gluten-free and casein-free diets on children with autism.
Data Sources: International databases such as Google Scholar, PubMed, Cochrane, and Science Direct were searched using keywords including Autism, Gluten-free, Casein, Diet, and Autistic Disorder, without any time restriction

Study Selection: 

Ultimately, 8 articles were selected for the final analysis.

Data Extraction: 

The study revealed that gluten-free/casein-free (GF/CF) diets had a significantly positive effect on the behavior index of autistic children. In terms of the behavior index, the effect size was a standard mean difference (SMD) of -0.27 (P = 0.001) with a 95% confidence interval (CI) of -0.424 to -0.116. A meta-analysis comparing intervention and control groups indicated that the GF/CF diet had an effect size of SMD = -0.27 (P = 0.001) with a 95% CI of -0.429 to -0.112 on the behavior index.

 The results of these studies were synthesized using the fixed effects model of meta-analysis with STATA version 14.

 Given the positive impact of the GF/CF diet on improving the behavior of autistic children, this dietary approach can be considered as a means to enhance behavioral symptoms in these children.

 Progressive familial intrahepatic cholestasis (PFIC) is a rare progressive liver disease associated with portal hypertension leading to liver failure. Most PFIC patients ultimately need liver transplantation. Therefore, identifying a precise, non-invasive biomarker for PFIC is very important.

 This study aimed to investigate the microRNA-34a (miR-34a) expression levels in PFIC patients.

 A total of 18 PFIC patients were randomly selected from the Shiraz Pediatric Liver Cirrhosis Cohort Study (SPLCCS), and 18 healthy subjects were enrolled in this study. Blood samples were obtained, and the gene expression of serum miR-34a was analyzed.

 The results of this study showed that the serum level of miR-34a was significantly increased in PFIC patients, compared to the healthy group (P = 0.0327). However, no association was observed between miR-34a expression and clinical and laboratory findings of PFIC cases. Additionally, the results showed no correlation between miR-34a and serum levels of liver enzymes, albumin, gamma-glutamyl transpeptidase (GGT), and international normalized ratio (INR) among PFIC patients.

 miR-34a might be a beneficial, non-invasive target for PFIC diagnosis; however, further investigations with a larger population and longer duration are necessary to determine the potential of miR-34a as a diagnostic and therapeutic biomarker for PFIC.