Iranian Journal of Pediatrics
Volume:32 Issue: 3, Jun 2022

The novel coronavirus that swept the world into a pandemic in 2019 has affected many aspects of health care. COVID-19 has infected about 263 million people across the globe and led to the death of 5.2 million people. Its impact on various organs is still vague and requires further research. The increase in hospital visits and administrations has accordingly increased exposure and risk of obtaining the coronavirus. Patients previously hospitalized and being treated with immunosuppressants tend to be very susceptible to serious respiratory infections from the novel virus. Amongst the diseases that require hospitalization are ulcerative colitis and appendicitis. Hospitalization from such diseases inevitably increases the risk and exposure to COVID -19 infection. This study analyzed the management and procedures taken in patients with inflammatory bowel disease and appendicitis during the COVID-19 pandemic. Similarly, the effects of the pandemic on the pediatric ward and admitted children were also discussed and compared.

Prolonged cardiac repolarization may increase cardiovascular susceptibility to ventricular arrhythmias, leading to sudden cardiac death. This condition in obese children is not clearly demonstrated in childhood.

This study aimed to investigate the effect of obesity on cardiac electrical activity in children by comparing Tp-e intervals between obese and healthy children and evaluating the correlation between Tp-e intervals and insulin resistance in obese children.

The study enrolled 50 obese and 50 healthy children. A pediatric cardiologist assessed electrocardiograms and echocardiographs. Electrocardiographic, demographic, and laboratory parameters were analyzed statistically.

There was no statistically significant difference between the two groups in age and gender (P > 0.05). The Tp-e intervals were statistically higher in the obese group than in the control group (P = 0.001). A statistically significant difference was found between the obese group with or without insulin resistance and the control group in Tp-e intervals (P = 0.001 for all). All other ECG parameters were similar in the patient and control groups.

It is essential to recognize the early symptoms of future cardiovascular events in childhood obesity cases. Repolarization differences in obese children, when compared with the normal population, may reflect obesity-related early-stage subclinical findings. Prospective broad-based studies are needed to recognize these early changes.

Because of the high daily mortality and quarantine procedures, coronavirus disease 2019 (COVID-19) can cause several psychological problems, especially in younger people.

Since most studies have mainly focused on physical problems of COVID-19 and they have neglected children in their analysis, this study aimed to evaluate the frequency and severity of stress symptoms and associated factors amongst children and adolescents aged 4 - 18 years during the COVID-19 outbreak.

Using a convenience sampling technique, this descriptive-analytic cross-sectional study was conducted in Shiraz, Iran, from March 2020 to June 2020. To collect data, the link of the online Children's Self-Reported Stress Symptoms Questionnaire was sent to eligible participants via SMS and WhatsApp. The data were analyzed using SPSS software version 21.

According to our results, 270, 77, and 16 cases had low, moderate, and severe stress, respectively. Age group, living with both parents, and birth rank had a significant relationship with the degree of stress. Furthermore, more than 50% of participants reported cognitive-emotional symptoms more than physical ones. There was a significant relationship between the age group and physical symptoms, the duration of quarantine with bad temper and sadness, and between no clear thoughts with mother’s workplace and father’s job.

Generally, almost 25% of participants reported moderate-to-severe stress. The most common symptoms of stress in children and adolescents were worrying, anger, and weakness. Furthermore, older children, those being the first child, and children living with both parents were more exposed to stress. Therefore, these items should be included in the healthcare team's training.

Dried blood spot samples are suitable for diagnosing some congenital errors of metabolism; however, they provide limited benefit in the regular monitoring of amino acids.

The present study aimed to evaluate alanine (Ala), arginine (Arg), citrulline (Cit), glutamic acid (Glu), glycine (Gly), isoleucine (Ileu), leucine (Leu), methionine (Met), ornithine (Orn), phenylalanine (Phe), tyrosine (Tyr), and valine (Val) amino acid concentrations in dried blood and plasma samples obtained simultaneously.

Amino acid concentrations were determined in the plasma, and dried blood spot samples obtained simultaneously from 145 patients (50 females and 95 males). Amino acid concentrations in the plasma and dried blood spot samples were studied by LC-MS/MS using original kits.

There were significant differences between dried blood spots and plasma in all amino acid concentrations, except for Met and Val. Bland-Altman analysis revealed the highest mean differences in Glu (-148.1), Gly (-70.1), and Ala (-58.1). Deming regression analysis showed that plasma and dried blood spot samples were consistent concerning Cit, Met, Phe, and Tyr concentrations.

Differences in methodology and sample can influence amino acid concentrations. Dried blood spot samples might cause errors in amino acid screening programs.

The chronic inflammatory state that occurs in obesity causes metabolic complications such as dyslipidemia, hypertension, insulin resistance, hepatosteatosis, and cardiovascular disorder. Dyslipidemia secondary to obesity, which is an important cause of morbidity, is a worldwide concern. Dyslipidemia has an essential role in the pathogenesis of cardiovascular disease (CVD).

The aim of this study was to determine the prevalence and risk factors associated with dyslipidemia in obese children and adolescents.

This single-center, retrospective, cross-sectional study included 1,136 obese patients. Fasting lipid, liver transaminases, glucose, and insulin levels were measured, and ultrasound scans (US) were performed. Other clinical assessments included waist-to-hip ratio, blood pressure, and the presence of acanthosis nigricans and striae.

Dyslipidemia was present in 644 (56.7%) cases. Puberty, high body mass index (BMI), increased waist-hip ratio, increased homeostasis model of assessment for insulin resistance (HOMA-IR), high uric acid, and acanthosis nigricans were found to be risk factors for dyslipidemia.

The prevalence of dyslipidemia in obese children was high and increased with puberty. Therefore, obese children and adolescents should be closely monitored for dyslipidemia that predisposes them to adolescent CVD.

Celiac disease (CD) is a common autoimmune disorder that presents intestinal and extra-intestinal symptoms. It is also associated with cardiovascular diseases and malignancies, and mortality risk. The only way to control the disease is to follow a strict gluten-free diet (GFD) for the rest of life.

This survey aimed to investigate GFD non-adherence and causes in the pediatric setting.

In this study, 187 children aged between 2.5 to 14 years old with a confirmed diagnosis of CD at least for a year were studied using a questionnaire-based interview in a census study between 2018 to 2019 in a referral center in southern Iran.

About 40% of children adhered to a GFD poorly. This group significantly complained of more symptoms than the group with high adherence. Improper access was the most important cause of non-adherence to a GFD. The mean current weight and at the time of diagnosis as well as the mean current BMI and at the time of diagnosis in the non-adherent group were significantly lower than the adherent group. However, IgA anti-transglutaminase antibodies and histopathologic examination did not change remarkably. Furthermore, no significant relationship was found between following a GFD and age, age at the time of diagnosis, gender, and parental educational status.

According to our results, inaccessibility, high costs, and lack of food labeling were the primary reasons for non-adherence to GDF. Therefore, to increase compliance, easy access to GFD with proper food labeling and suitable price should be implemented.

Incidence of ulcerative colitis (UC) in children has increased worldwide.

In the current study, we summarized our clinical experience using infliximab (IFX) in the treatment of children with steroid-refractory UC.

The clinical data of 9 steroid-refractory UC patients with average age of 8 years who were treated with IFX in our hospital were analyzed.

At the end of the induction period, 6 achieved a clinically significant response. Of the 6 children, 4 had mucosal healing and 2 had endoscopic remission. At week 30, among the 6 children who achieved a clinically significant response, 3 had persistent clinical remission and mucosal healing, 1 achieved mucosal healing from endoscopic remission, 1 had mild disease, and the other child had not reached 30 weeks of treatment as of this writing. At week 54, 6 of 9 children achieved clinical remission and 5 had mucosal healing. The hemoglobin concentration in the children who achieved a clinically significant response was higher than pre-treatment and the inflammation markers were lower than pre-treatment. During IFX treatment, five children had a loss of response, three had a primary non-response, and two had a secondary non-response. The latter children achieved clinical remission with optimized treatment.

IFX is a salvage treatment option for children with moderate-to-severe steroid-refractory UC. The course of treatment and the timing of drug withdrawal warrants further study.

According to the literature, urine analysis to diagnose urinary tract infection (UTI) in neonates is not the basis. It even is not recommended as a part of the evaluation, but some abnormalities in a simple urine test in neonates with UTI may be associated with urinary tract anomaly. Therefore, a simple urine test in neonates with UTI can be helpful for early diagnosis and timely treatment of urinary tract anomalies.

This study was performed as a cross-sectional study. First, 100 neonates hospitalized for various reasons and diagnosed with urinary tract infections were chosen. Before treatment, urine samples were taken from all neonates by catheterization or suprapubic sterility for culture and urine bag for urinalysis. Finally, neonates with positive culture were treated with antibiotics and evaluated; kidney and urinary tract ultrasound and voiding cystourethrography (VCUG) is used to diagnose renal anomalies. The obtained information from urine analysis of neonates without urinary tract anomalies was compared with urinary tract anomalies neonates by SPSS 21 software. To compare the qualitative data, the chi-square and Fisher’s exact tests were used, whereas to compare the quantitative data, we used Wilcoxon test. P < 0.05 was considered statistically significant.

Out of 100 patients who were evaluated, only 30 patients had renal anomalies. No statistically significant correlation was found between gender, cause of hospitalization, positive culture, and type of organism with the renal anomaly. However, pyuria (P-value 0.003), bacteriuria (P-value 0.016), fever (P-value 0.002), nitrite positive (P-value 0.001), and leukocyte esterase (P-value 0.001) showed a statistically significant correlation with the renal anomaly.

Certain indicators such as pyuria, bacteriuria, nitrite, and leukocyte esterase can be seen in a simple urine analysis as criteria for suspicious urinary tract anomalies in neonates with UTIs.

This study aimed to comprehensively analyze the biochemical blood indices of children with intussusception.

A total of 8235 children with intussusception were included in this study, consisting of 5743 children with successfully reduced intussusception by hydraulic enema (the HE group) and 2492 children with surgical management after unsuccessful enema treatment (the SM group). The SM group consisted of the managed surgically with necrosis group (the SN+ group, n = 398) and the managed surgically without necrosis group (the SN- group, n = 2094). This study conducted a comprehensive analysis of 74 clinical and biochemical parameters in these children with intussusception.

Temperature, plateletcrit (PCT), mononuclear cell ratio (MONO%), monocyte count (MONO#), γ-glutamyltranspeptidase (GGT), direct bilirubin (DBIL), and C-reactive protein (CRP) increased gradually in HE, SN-, and SN+ groups and might be risk factors for further development of HE into SN- or SN+. The neutro cell count (NEUT#), neutro cell ratio (NEUT%), and white blood cell count (WBC) showed significant differences only in HE/SN+ and SN-/SN+ groups, which might be risk factors for intussusception complicated with intestinal necrosis in children. The prediction model composed of seven variables (i.e., NEUT#, platelet (PLT), albumin (ALB), β-2MG macroglobulin (β-2MG), glucose (Glu), uric acid (UA), and chlorine (Cl) was suitable for HE/SM. The average prediction accuracy of this model was 81.82%, and the area under the receiver operating characteristic curve (AUROC) was 0.87 (95% CI: 0.85 - 0.90). The prediction model composed of six variables (i.e., NEUT#, average hemoglobin concentration (MCHC), PLT, the total protein (TP), CRP, and urea (BUN) was suitable for SN-/SN+. The average prediction accuracy of this model was 87.93%, and the AUROC was 0.95 (95% CI: 0.92 - 0.97).

This study screened out the risk factors of intussusception patients needing surgical treatment and established the appropriate prediction models.

Children admitted to the intensive care unit are at risk of malnutrition, mainly due to chronic diseases they are suffering from. These patients require a different nutritional diet regimen from those in a normal or stable disease state due to change in metabolism under the stress of diseases.

According to the SIGN guideline based on evidence, first, articles matching our criteria were extracted from the literature, and then the strength of evidence was evaluated. Finally, a summary of statements consisting of details regarding the strength of evidence and recommendation level was reviewed by 12 experts, and two-round surveys were accomplished according to the Delphi method to reach a consensus.

Twenty-seven statements in 5 categories with strength of evidence, grade of recommendations, and expert opinions are summarized.

Rapid nutritional assessment, judging patients with malnutrition or at risk of malnutrition, fast intervention with early enteral nutrition, reaching the protein and energy goals under the supervision of an expert registered dietitian, and persistent monitoring with minimizing the time of fasting are some of the key components of proper nutrition management based on evidence found in the literature.

Perimembranous ventricular septal defect (VSD) is the most common congenital heart defect. There is a trend for percutaneous VSD closure. However, little evidence is available for the effect of this method on ventricular remodeling.

This study aimed to investigate the effect of percutaneous closure of perimembranous VSD on cardiac function and ventricular recovery.

A total of 46 pediatric patients (32 males vs. 14 females) who underwent transcatheter closure of perimembranous VSD from 2010 to 2020 were randomly included in the study. Data regarding the demographic profile, angiographic records, and follow-up echocardiography were extracted from their files and recorded in questionnaire templates. The echocardiographic parameters were recorded and compared with published Z-scores for the corresponding age groups.

The mean duration of follow-up was 15.76 ± 12.20 months. In M-mode echocardiography, 84.6% had interventricular septum diastolic diameter Z-score ≥ 2; 23.8% had interventricular septum systolic diameter Z-score ≥ 2; 38.5% had left ventricular internal diameter in diastole Z-score ≥ 2; 34.6% had left ventricular internal diameter in systole Z-score ≥ 2; and 65.4% had left ventricular posterior wall in diastole Z-score ≥ 2. In the evaluation of Doppler and tissue Doppler, 36.4% of the patients had a Z-score ≥ 2 for E/Ea of tricuspid. Also, VSD size had a positive correlation with interventricular septal diameter in systole Z-score (P = 0.015, r = 0.537).

In the midterm follow-up after percutaneous perimembranous VSD closure, left ventricular dilation and hypertrophy persisted in a significant number of patients. However, early closure of the VSD, especially in patients with lower weight could affect ventricular hemodynamics and remodeling.

According to the World Health Organization, the incidence of severe acute hepatitis of unknown origin in children is increasing worldwide. However, there are no known causes and treatments to deal with this disease. This study presents three children with severe acute hepatitis of unknown origin in a hospital medical center in Iran.

Two 12-year-old children and a six-year-old child were admitted to the hospital with jaundice. Laboratory tests showed markedly elevated liver enzymes and total and direct bilirubin in these patients. However, hepatitis B surface antigen (HBsAg), hepatitis C virus (HCV) antibodies (Ab), hepatitis A virus (HAV) IgM, HEV IgM, Epstein-Barr virus (EBV) IgM, anti-microsomal Ab, anti-liver kidney (LKM Ab), anti-smooth muscle antibodies (ASMA), anti-nuclear antibodies (ANA), and total IgG were all negative. After excluding the probable infectious causes of acute hepatitis and per the progressive course of fulminant hepatic failure, intravenous methylprednisolone was started for two of three cases during hospitalization. These children were discharged from the hospital in good general condition, and the liver function tests gradually decreased and approximated to near normal during the follow-up visits.

General practitioners and pediatricians are advised to examine the underlying causes and consider severe acute hepatitis of unknown origin when visiting children under 16 years with severe jaundice and very high transaminases.

Mitochondrial diseases are caused by disturbances in the oxidative phosphorylation (OXPHOS) system. Leigh syndrome encompasses a spectrum of mitochondrial diseases characterized by necrotizing encephalopathy. Thus far, two cases carrying a variant in NDUFA9 with a diagnosis of Leigh syndrome have been reported. NDUFA9 is a subunit involved in the assembly and stability of the mitochondrial respiratory complex I. We present a lethal phenotype of Leigh syndrome in a four-month-old boy born to a consanguineous (first cousins) Iranian couple. The patient’s clinical course was notable for episodes of cyanosis, seizures, lactic acidosis, nystagmus, spastic paraplegia, apnea, and respiratory arrest. Due to high branched-chain amino acids, an initial diagnosis of maple syrup urine disease was considered; however, the patient did not respond to treatment. Via exome sequencing, we identified a novel homozygous missense variation in NDUFA9 (c.1069C>G, p.Arg357Gly), and a posthumous diagnosis of Leigh syndrome was made. This report highlights the potential differential diagnosis of Leigh syndrome and further describes the phenotypic spectrum of NDUFA9 defects.