Iranian Journal of Pediatrics
Volume:33 Issue: 2, Apr 2023

 This study aimed to define the normal ranges of echocardiographic parameters that are used to evaluate right ventricular systolic functions.

 A total of 607 children within the age range of 0 - 18 years without any cardiac pathology or chronic disorders were included in the study. The study population was categorized into different age groups and underwent transthoracic echocardiography. In this study, tricuspid annular plane systolic excursion (TAPSE), tricuspid annular peak systolic velocity (TAPSV), and right ventricular myocardial performance index (RVMPI) values were measured.

 There was no statistically significant difference between the mean TAPSE and TAPSV values of male and female subjects. The mean RVMPI was higher in females than in males. The study population was categorized into nine groups according to their age. The TAPSE, TAPSV, and RVMPI values were calculated for each group. Additionally, the 5th, 10th, 25th, 50th, 75th, 90th, and 95th percentiles, and ± 2 standard deviation (SD) and ± 3 SD values of TAPSE measurements were calculated for each age group. The study population was divided into eight groups according to their body surface area (BSA). Moreover, the 5th, 10th, 25th, 50th, 75th, 90th, and 95th percentiles of TAPSE measurements were calculated. There was a strong positive correlation between TAPSE and BSA. The TAPSE was also positively correlated with TAPSV but not with RVMPI.

 This study determined the normal values for TAPSV and RVMPI. It is important to have knowledge of the normal ranges of these parameters to recognize right ventricular dysfunction early in various cardiac disorders.

 Deferring the umbilical cord clamping produces more satisfactory neurological and hematological outcomes. Another alternative for the deferred umbilical cord clamping is the umbilical cord milking.

 This study aimed to evaluate different placental transfusion techniques in terms of hematological parameters for term neonates.

 This observational study included 120 term infants assigned to groups of deferred cord clamping for 60 seconds (DCC), cut cord milking for four times with a speed of 10 cm/second (CCM), intact cord milking for four times (ICM), and a historical control group of immediate cord clamping (ICC). The primary outcome of this study was hematological parameters at birth and 24th hours. Hyperbilirubinemia, polycythemia, or respiratory distress were secondary outcomes.

 The median gestational ages and birth weights of neonates were 39 (37 - 40) weeks and 3270 (2365 - 4850) grams, respectively. Umbilical cord hemoglobin (Hb) and hematocrit (Hct) levels were significantly higher in the ICM group (P < 0.01). Hemoglobin and Hct levels at 24th hours of life were similar in DCC, CCM, and ICM groups and significantly higher than those in the ICC group (P < 0.01). No significant difference was found among the groups in terms of hyperbilirubinemia, polycythemia, and respiratory distress.

 To the best of our knowledge, this study was one of the most comprehensive studies evaluating the effects of different placental transfusion strategies on hematological parameters in term infants and the first study exploring intact cord milking in term infants. Intact cord milking was suggested to be associated with higher hemoglobin levels at birth. All DCC, ICM, and CCM techniques were found to be more effective than ICC in terms of early hematological parameters.

 This study is designed to explore factors associated with death in newborns with persistent pulmonary hypertension (PPHN).

 The clinical data of PPHN cases in the past ten years from a neonatal center in China were retrospectively collected and analyzed, and the death-related factors attributable to PPHN were analyzed by binary stepwise logistic regression analysis.

 A total of 161 neonates with PPHN were included in this study, with a mortality rate of 18.0% (29/161). Multivariate binary logistic regression analysis indicated that cesarean delivery was negatively associated with death in infants with PPHN (adjusted odds ratio [OR] = 0.289, 95% confidence interval [CI] 0.104 – 0.803), while premature rupture of membranes (PROM) (adjusted OR = 4.032, 95% CI 1.32 - 12.32), congenital lung developmental abnormalities/congenital diaphragmatic hernia (CDH) (adjusted OR = 12.65, 95% CI 1.088 – 147.068), respiratory distress syndrome (RDS) (adjusted OR = 4.802, 95% CI 1.512 – 15.251), inhaled nitric oxide (iNO) (adjusted OR = 12.377, 95% CI 3.22 – 47.576) and norepinephrine (adjusted OR = 2.891, 95% CI 1.03 – 8.118) were the independent factors associated with a higher risk of death from PPHN.

 Caesarean delivery with medical indication is an independent protective factor against death in neonates with PPHN. PROM, congenital lung developmental abnormalities/CDH, RDS, iNO, and norepinephrine were independent death-related factors in neonates with PPHN.

 Enuresis is one of the most common diseases in children. Although there are several factors involved in the occurrence of this diseases, the root cause of it has remained undetermined.

 Identifying various factors responsible for enuresis may enormously contribute to solving this problem. This study, therefore, aimed to determine the level of vitamin B12 and folic acid in children with enuresis in Gorgan in 1400.

 In this case-control study, 43 children with primary enuresis and 99 children without enuresis, as the control group, referring to Taleghani Hospital in 1400 were included. Folic acid and vitamin B12 levels in two groups were measured and analyzed using statistical techniques.

 Out of all participants, 23 (53.5%) in the case group and 53 (53%) in the control group were male. The mean age of children in the case and control groups were 7.60 ± 3.02 and 8.93 ± 3.15, respectively, and the two groups were not significantly different in terms of gender and age. Vitamin B12 and folic acid levels in the case group were significantly lower than those in the control group (P-value = 0.001). There was a significant difference between the case and control groups regarding the mean levels of vitamin B12 and folic acid levels based on sex and gender.

 In sum, it was found that children with enuresis suffered from deficiency of vitamin B12 and folic acid to some extent, which may have been a factor responsible for delaying the maturation of the central nervous system and, consequently, inducing enuresis in children.

 Preliminary studies have shown that electrical source imaging (ESI) has numerous advantages for the pre-surgical evaluation of epileptic patients. However, the role of ESI for children with non-lesional drug resistance in focal epilepsy has been poorly characterized.

 This study aimed to investigate this issue according to interictal epileptiform discharges (IEDs) and constraints in developing countries.

 The present study used long-term video electroencephalography (EEG) monitoring (LTM) data that were recorded using the standard 19 scalp electrodes (10 - 20 system) and 3 tesla T1 image data. Accordingly, first, IEDs were clustered and then assessed by an epileptologist. Afterward, some operations were conducted that included EEG inverse problem solving with three known methods, namely Brain Electrical Source Analysis (BESA) with the individual head model, cortical classical LORETA analysis recursively analysis with the individual head model, and BESA with the age template head model. Seven children were processed in this project.

 In most cases (n = 5, 71%), the seizure onset zone (SOZ) was the same in the LTM report and the present proposed methodology. Moreover, this study succeeded in localizing the region of the predicted SOZ.

 According to limitations in a developing country, for the configuration of multi-modal studies (e.g., 3T magnetic resonance imaging, LTM, and ESI) with a specific and valuable protocol, this investigation defined a pilot study with a 7 data sample for the first step. These findings, based on the small sample size, suggest that ESI based on combining ensemble methods improves information for children with focal drug-resistant epilepsy. It is hoped that future studies with large sample sizes show the role of ESI in developing countries more than before.

 There is anecdotal evidence regarding the simultaneous occurrence of vesicoureteral reflux (VUR) and gastroesophageal reflux disease (GERD), which indicates the probability of pathophysiological commonality.

 In the present study, we evaluated the concurrence of VUR and GERD in children candidates for the voiding cystourethrogram (VCUG) study.

 This cross-sectional study was conducted on 62 children between 1 and 14 years old referred to a tertiary referral teaching hospital for VCUG in 2019 - 2020. All subjects underwent ultrasound to assess GERD and VCUG to rule out VUR.

 According to the ultrasound assessment, 14.5% of subjects were diagnosed with GERD: 8.3% in males and 18.4% in females. VUR was detected in 48.4% of children (50.0% in males and 47.4% in females) using VCUG. Overall, seven (23.3%) had concomitant VUR and GERD: 4.2% in boys and 15.8% in girls, indicating no difference between the two genders (P = 0.125). The prevalence of concurrent GERD and VUR was also independent of age. In the two groups with and without VUR, the prevalence of GERD was 23.3% and 6.2%, respectively, indicating a relative risk of 2 (95% confidence interval [CI]: 1.32 - 3.02, P = 0.001).

 Regarding the relationship between GERD and VUR, despite the deletion of physiologic GER cases, the pathophysiological overlap between the two phenomena could be considered.

 Neonates of infertile couples are golden babies, and taking care of them is of paramount importance. However, it is not yet clear if the prognosis is different between these neonates and those from fertile couples.

 The present study aimed to assess neonates conceived by assisted reproductive technology (ART).

 In this cohort study, 165 newborns conceived by ART and 165 naturally conceived newborns were included. The prospective study was conducted from April 2020 to October 2021. A neonatologist examined all newborns after birth, and outcomes were followed up over one year and compared with neonates of fertile couples using appropriate statistical tests.

 The preterm neonates (60% vs. 38%) were higher in ART cases (P < 0.001) than in the other cases. Also, the mean gravid and parity were lower, but the nulliparity was higher in the ART group (P < 0.001) than in the other cases. Moreover, multiple pregnancies (45% vs. 10%; P < 0. 001) and the C/S rate (91% vs. 67%) were higher in ART cases (P < 0.001) than in the other cases. Similarly, the preeclampsia rate (16 % vs. 6%; P = 0.004) and the number of females were higher in the ART group (P = 0.035) than in the other groups. However, birth weight (P = 0.002) and the Apgar were significantly lower (P = 0.002; P = 0.012) in the ART group than in the other groups. IUGR was significantly higher (17% vs. 7%) (P = 0.006), while NEC and RDS were more common in the ART group (P < 0.001) than in the other groups. In addition, more extended hospital stay (P < 0.001) and more common re-admission and weight < the 5th percentile after one year were observed in the ART group (P = 0.021) than in the other groups.

 According to the findings, pregnancy after ART has more side effects before and after birth and in infancy, not only because of multistation but also due to manipulations in ART.

 In pediatrics, many drugs are used without marketing authorization. Recommendations are often based on clinical experience. Therefore, the risk of inappropriate prescription (IP) is high. It is necessary to have a tool for pediatric IP detection.

 This study was performed to develop and validate a Pediatrics: Omission of Prescriptions and Inappropriate Prescriptions (POPI) screening tool to facilitate its use in pediatric practice in Iran.

 Using forward- and backward-translation procedures, an efficient and effective tool was provided in the current study and clinical settings. The two-round Delphi technique established content validity. The criteria were then piloted in a cross-sectional study in the pediatric patients of Khorasan Razavi and East Azerbaijan in Iran.

 A total of 104 explicit criteria (79 IPs and 25 omissions) were obtained and submitted to an 18-member expert panel (including 8 pharmacists, 2 clinical pharmacists, and 8 pediatricians working in a hospital or the community). Then, 98 out of the 104 criteria submitted to the experts were selected after two Delphi rounds (75 IPs and 23 omissions). The content validity and reliability of the tool were obtained by expert assessment (Cronbach’s alpha for the entire criteria: 0.60). At least, the rate of one inappropriate prescribed medication was 69% in Mashhad, almost twice that of Tabriz (35%).

 The modified POPI criteria comprise the first screening tool to assess rational prescriptions for pediatric patients in hospital and outpatient settings. Clinical validation and reliability studies are needed and planned by the authors to evaluate the usability and reliability of this tool.

 Patent ductus arteriosus (PDA) is a disease presenting on the first days of birth, associated with unpleasant complications such as exaggerated respiratory distress and increased mortality, requiring prompt treatment.

 This study evaluated the relationship between perfusion index and the recovery of patients diagnosed with PDA.

 In this cross-sectional study, 22 neonates with PDA (as the case group) and 22 healthy neonates (as the control group) were selected. The difference in perfusion index (PI) before and after arterial duct closing was determined, and also the relationship between pre-ductal and post-ductal PI in the case and control groups was evaluated. Data were analyzed by SPSS version 21.

 The results showed a significant difference in the mean scores of pre-ductal and post-ductal PI before and after arterial duct closure (P = 0.004). Also, pre-ductal and post-ductal PI scores were significantly lower in the case group than in the control group (P < 0.001)

 According to our findings, PI in infants with PDA is low at presentation and increases after treatment. Therefore, PI can be used as an indicator to evaluate response to treatment during follow-up, particularly when repeated echocardiography is not accessible.

Reports of acute hepatitis with unknown origin in children have been published worldwide since April 2022. Due to the unfamiliar nature of the disease and the alarming trend of increasing reports worldwide, health workers must become aware of its diagnosis and treatment. Accordingly, the committee for developing guidelines for the Department of Pediatrics, Tehran University of Medical Sciences, prepared a clinical guideline for more efficient management of these patients. Due to the limited information about this disease, it has been tried to categorize and specify the required diagnostic and treatment measures based on the latest published data. Herein we express this algorithmic approach and diagnostic and therapeutic guidelines for acute hepatitis with unknown origin in children.

 Ichthyosis is an epidermal disruption that increases insensible water loss. Hypernatremic dehydration is a consequence of skin disruption. This study reviewed the treatment of hypernatremic dehydration in patients with ichthyosis comparing to patients with intact skin.

 We studied five neonates with hypernatremia, including three ichthyosis cases and two normal-skin neonates. This case-series study showed that the sodium correction rate is slower in infants with ichthyosis than in infants with normal skin. The first and second neonates needed less sodium than fluid intake than normal skin infants, although fluid requirement was lower in the third ichthyosis infant than in others due to less skin disruption in this infant.

 Fluid therapy in hypernatremic dehydration in ichthyosis patients is different from neonates with intact skin because of excessive insensible water loss in these patients. It may be needed to give more fluid and less sodium depending on the degree of skin disruption, which may not be determined by physical examination.

 Renovascular hypertension is among the significant causes of severe hypertension in pediatrics. Primary therapeutic approaches, including drug therapy or angioplasty, are not always feasible, and their effectiveness is variable. Meanwhile, several conditions leading to renal function impairment, including severe renal artery stenosis, may represent the respective kidney as an apparently non-functioning organ in various imaging modalities. We aim to demonstrate that the only organ in the body of a child with non-treated renovascular hypertension and severe renal artery stenosis is the ipsilateral non-functioning kidney. This kidney is alive and protected from hypertension by renal artery stenosis. Renal autotransplantation (RAT) can be superior to nephrectomy in such cases to protect from severe hypertension. This phenomenon can be confirmed by controlled hypertension and hypertrophy of transplanted kidney accompanied by shrinkage of contralateral compensatory hypertrophy.

 We introduce a 14-year-old child with a history of diagnosed hypertension from seven years ago, presenting with resistance to four-drug therapy. Renal scintigraphy and intravenous pyelography found no activity pertaining to the relative kidney, and angiography exhibited severe stenosis of the renal artery with preserved blood circulation. Renal autotransplantation successfully restored the function of the respective kidney based on pre-and post-surgery intravenous pyelography and decreased the blood pressure to normal limits.

 Our experience shows we cannot mark a kidney as a "non-functioning" organ only on the basis of the preliminary findings from conventional imaging modalities, including intravenous pyelography, scintigraphy, and duplex ultrasound. Moreover, in cases that angioplasty is failed or is not feasible, renal auto-transplantation may be a safe alternative with promising outcomes in patients with renovascular hypertension, being able to restore the function of the kidney.

 Sodium leak channel, non-selective (NALCN) is an ion channel with an important function in neuronal excitability. The activity of NALCN is essential in the balance of rhythmic behaviors. Infantile hypotonia with psychomotor retardation and characteristic facies 1 (IHPRF1), which occurs due to biallelic NALCN variants, leads to dysmorphic characteristics and global developmental delay. We hereby report 2 non-related patients with IHPRF1 syndrome.

 The first case was a 1 - year-old girl referred to our center because of constipation and poor weight gain, and the other was a 2 - year-old girl presenting with hypotonia, constipation, and poor weight gain. Whole exome sequencing led to the detection of homozygote NALCN variants. Interestingly, in both cases, a novel variant of c.1434 + 1G>A in NACLN was identified, which, to the best of our knowledge, has not been reported as a pathogenic variant so far.

 NALCN dysfunctions lead to rare yet clinically significant disorders. Due to the potential mortality, further studies are essential for a more comprehensive understanding of these rare disorders.