Iranian Journal of Pediatrics
Volume:29 Issue: 3, Jun 2019

In this systematic review and meta-analysis, for the first time, the existing evidence on health-related quality of life (HRQOL) was evaluated in patients with biliary atresia (BA) and compared with that of healthy population. A systematic search was done on PubMed, Scopus, Embase, Cochrane Library and Web of Science from 1990 up to April 2018 in order to identify all articles that assess the HRQOL in patients with BA. Meta-analysis was done using comprehensive meta-analysis and the standard difference of mean (SDM) was calculated. A total of 188 publications were identified from the initial search. After removing duplicates and irrelevant studies, 32 articles remained for full-text evaluation. Out of this number, 22 articles were excluded due to non-relevance to the study scope leaving 10 studies for systematic review and three articles for meta-analysis. HRQOL was assessed using PedsQL4 in three studies, SF-36C in two studies and by assessing different parameters including social status, school, university, and/or sports performance in five studies. HRQOL in BA patients living with their own liver was significantly lower than that of the general population [SDM = -0.55 (95% CI: -0.69, -0.40)]. In the terms of PedsQL4 sub-scores, compared with healthy population, the levels of physical [SDM = -0.40 (95% CI: -0.55, -0.28)], social [SDM = -0.25 (95% CI: -0.40, -0.11)] and school functioning [SDM = -1.19 (95% CI: -2.35, 0.07)] were significantly lower in the group of biliary atresia.

The aim of this study was to report the prevalence, clinical course, and sequela of arthritis in Kawasaki disease (KD), and to establish its relationship to coronary outcomes. We retrospectively reviewed the records of 524 patients between March 2006 and February 2016. The overall prevalence of arthritis was 17.6 % (n = 92/524) and among them, 21 cases (22.8%) had late-onset arthritis and they were older (32.7 ± 12.1 vs. 62.5 ± 23.8 months), had longer fever duration (5.8 ± 1.94 vs. 7.11 ± 2.51 days), more frequent swelling or redness of extremities (35.2 vs. 71.5%), higher neutrophil counts (58.69 ± 13.84 vs. 72.16 ± 16.43%), and higher prohormone brain-type natriuretic peptide levels (ProBNP, 1343.80 ± 1080.44 vs. 3754.42 ± 4272.71 pg/mL) than patients with early onset arthritis. Coronary artery changes were not different and no patients complained of chronic arthritic changes, regardless of arthritis type. Although late-onset arthritis was found in KD patients who were older with longer fever duration, more frequent swelling or redness of extremities, higher neutrophil counts, and higher ProBNP levels than early-onset arthritis patients, there was no statistical significance between arthritis onset type and coronary artery changes, and outcomes were excellent with no chronic arthritis change.

The aim of this study was to evaluate tubular functions of posterior urethral valve patients with preserved glomerular functions. A total of 25 children (ages 1 - 18) who underwent surgery for PUV and had an estimated glomerular filtration rate ≥ 90 mL/min per 1.73 m2 on long term follow-up and age-matched 25 healthy controls were enrolled. Blood and urine samples were collected to assess electrolyte reabsorption, proteinuria, enzymuria, urine acidification and concentration. Even though microalbumin/creatinine ratio was higher in the patient group [1.00 (1.98) vs 0.31 (0.34) mg/gr creatinine, P = 0.005] none of the subjects had enzymuria, overt proteinuria or hyperphosphaturia. Serum bicarbonate levels were lower and urinary pH higher in patients than the controls (23.2 ± 2.1 vs. 24.6 ± 1.9 mmol/L, P = 0.028 and 6.24 ± 0.77 vs. 5.60 ± 0.64, P = 0.004); furthermore, patients had lower urinary osmolality and density (525 ± 214 vs. 743 ± 194 mOsm/L, P = 0.001 and 1011 ± 5.0 vs. 1016 ± 6.8, P = 0.005). When patients were compared among themselves according to presence or absence of renal scarring and when patients without renal scars were compared to healthy controls, similar findings persisted. This study showed that during long-term follow-up of posterior urethral valve patients with normal glomerular filtration rates, even though proximal tubular functions do not seem to be impaired, acidification and concentration defects exist indicating distal tubulopathy.

Benefits of stem cell therapy on remodelling and cardiac function have been described in adults with dilated cardiomyopathy and acute myocardial infarction. We investigated the effect of this treatment modality amongst children with severe dilated cardiomyopathy. Intracoronary injection of autologous bone marrow mononuclear stem cells was performed in our centers for 8 severely ill children during 2015 - 2016. The mean age of the patients was 10.1 years (5 girls, 3 boys). They were followed by longitudinal speckle tracking echocardiography (STE) and conventional echocardiography for 6 months. Heart functional class improved in 62% of patients. M-mode echocardiography showed significant improvement in ejection fraction (mean 24.8 ± 8.3 vs. 37.4 ± 10.5) and in STE, the mean global longitudinal strain improved (GLS: -2.8 ± 1.9 vs. -5.2 ± 3.9). None of the patients had serious complications. Intracoronary injection of autologous mononuclear stem cells might improve the ventricular function and cardiac remodelling in pediatric patients with dilated cardiomyopathy and could be considered in critically ill patients.

Bronchopulmonary dysplasia (BPD) is a chronic lung disease of preterm neonates with a few therapeutic options. Our previous studies suggested that bone marrow-derived mesenchymal stem cell (BMSC) therapy is effective in the treatment of BPD in mouse models. Furthermore, microRNA-206 (miR-206) suggested having a correlation with BPD by regulating the expression of fibronectin 1 (FN1). The aim of this study was to characterize the role of BMSCs with the up-regulation of miR-206 in the treatment of BPD in newborn mice. The BPD mouse model was induced by 60% oxygen. BMSCs transfected with miR-206 or negative control (NC) plasmid were administered on postnatal day 7 (P7) and every following week through an intraperitoneal injection (105 cells/animal) until P35. The mice were weighed and their lungs were weighed and prepared for histology. The expression levels of miR-206, FN1, and pulmonary surfactant-associated protein-C (SP-C) were determined through the quantitative polymerase chain reaction and under the immunofluorescence. The protein expression levels of transforming growth factor-β1 (TGF-β1) and interleukin-6 (IL-6) were determined by ELISA. BMSCs with up-regulation of miR-206 reduced the degrees of pulmonary edema, improved pulmonary architecture, and attenuated inflammation in BPD mice compared to the NC plasmid group. Furthermore, the up-regulation of miR-206 in BMSCs down-regulated the expression levels of TGF-β and IL-6 in plasma samples and of SP-C and FN1 in the lung tissue of BPD mice. BMSCs with up-regulation of miR-206 are effective in the treatment of BPD in newborn mice, and the study introduced a novel idea for the treatment of BPD.

For growth monitoring of brain, head circumference (HdC) reference data are needed. Rapid increase in the HdC is usually seen within first few years of life which marks histological changes in the brain. However, in Pakistan, there is a dearth of research on the potential use of HdC measurements. We aimed to develop the age and gender specific smoothed HdC growth reference charts for the Pakistani children, aged 2 to 5 years. A representative cross-sectional sample of 1474 Pakistani children, aged 2 - 5 years, was studied. Age and gender specific smoothed HdC growth reference values and their charts were obtained using the lambda-mu-sigma (LMS) statistical method. The smoothed HdC percentiles (3rd, 5th, 10th, 25th, 50th, 75th, 90th, 95th and 97th) were calculated and their charts were drawn. The percentile values of HdC increased with age in both sexes. The boys had more increase in HdC percentiles than the girls had, except for the 95th and 97th percentiles. Comparison of 50th percentiles of HdC with those of the other countries demonstrated that the Pakistani children had substantially lower HdC percentiles than their counterparts in the other populations. The results showed that reference values for the HdC were smaller than the corresponding reference values provided by the World Health Organization. Therefore, it is suggested that each country should produce its own HdC growth charts because the use of the charts of other countries as standards may provide misleading information of the children population under study.

Vitamin D deficiency is associated with prematurity, respiratory problems and infections. These are important causes of death in premature neonates. The aim of this study was to compare the vitamin D level in the blood of preterm live neonates discharged with those neonates who did not survive. In a cross-sectional study, 335 premature infants (less than 34 weeks of gestation) were discharged and 48 infants died. We examined their umbilical cord blood for vitamin D. Eighty-eight percent of our premature neonates had vitamin D deficiency. The mean of vitamin D in the survived neonates was 14.87 ± 10.94 and in those who succumbed their prematurity it was 9.4 ± 6.52 ng/mL (P < 0.01). There was a significant difference between the two groups in terms of the type of delivery, Apgar score of first and fifth minutes, duration of oxygen therapy and gestational age (P < 0.05). The results of this study showed that most premature neonates have a severe vitamin D deficiency, and this deficiency was more pronounced in the dead neonates than in those that survived, despite eliminated the role of gestational age. Measurement of cord blood vitamin D level may help predict the prognosis for premature neonates.

Recently, smoothies with a high vegetable content have become very popular, especially among young adults. This in vitro study was conducted to determine the erosive potential of selected green vegetables and green smoothies on human dental enamel. A total of ten green smoothies and eight freshly mixed vegetables were chosen, and pH, calcium and oxalic acid contents were measured. The enamel specimens were obtained from wisdom teeth and incubated with 3 selected foods (spinach, parsley, or green smoothie) for 12 and 24 hours (N = 6). Control samples were incubated with a physiological salt solution. An electron probe microanalyzer (Jeol JXA 8900RL) was utilized to analyze the concentrations of calcium, phosphorus, magnesium and fluorine at various depths ranging from 5 to 50 µm within the enamel samples. A visual examination of the specimen was carried out with a digital microscope. The green smoothies showed pH values ranging from 3.6 to 4.2, and the values of the mixed vegetables ranged from 5.6 to 6.3. Only in the green smoothie group could a time-dependent release of different minerals be observed. After 12 and 24 hours of incubation, a pronounced loss of calcium and phosphorus was found to a depth of 15 µm. Digital microscopy of the enamel surfaces confirmed these findings. In our in vitro study, incubation with the spinach and parsley showed no damage to the enamel specimens; in the case of the investigated smoothies, a mild loss of minerals to a depth of 25 µm was observed. It must be considered that numerous factors influence the enamel surface in vivo, translating these results to in vitro conditions must be done with great caution.

Parents of preterm neonates are exposed to stress leading to post traumatic stress disorders (PTSD). In the present study we compared the prevalence of PTSD in parents of hospitalized preterm and term neonates. A descriptive-comparative study was undertaken in 2 Iranian Hospitals during 2016 and 2017. Parents of NICU hospitalized preterm neonates as well as those of hospitalized term newborns entered the study. Parents were invited for interview at day 3 - 5 after neonate’s birth. Questionnaires for acute stress disorder (ASD) were filled out and scored for both mother and father. After a month, parents were asked for a second interview. Prenatal post traumatic stress questionnaires (PPQ) for all mothers and posttraumatic stress disorder checklist (PCL) for all fathers were completed. All recorded data were analyzed to compare the prevalence of PTSD among parents of term and preterm infants. P values less than 0.05 were considered statistically significant. Parents of 80 NICU hospitalized preterm neonates and parents of 80 term hospitalized neonates entered the study. Both the mean ASD and PPQ scores among mothers of preterm infants were significantly higher than those of mothers of term infants (53.77 ± 13.58 vs. 49.27 ± 12.55; P = 0.032 and 31.67 ± 11.85 vs. 28.08 ± 8.80; P = 0.032). The mean PCL score among fathers of preterm infants was significantly higher than their counterparts in other group (31.5 ± 14.9 vs. 21.37 ± 5.4; P < 0.0001). A significant correlation was observed between mother’s PPQ and father’s PCL scores (P < 0.0001). Mothers with higher ASD scores also showed higher PPQ scores (P < 0.001). Although hospitalization of a neonate is a stressful event for parent, neonate’s condition related age at birth was the main subject for parents’ psychological complication and PTSD. Moreover, we found that, of parents, mothers were more vulnerable for PTSD but fathers indicated delayed onset of PTSD symptoms.

To summarize the experience of intraoperative nursing cooperation in endoscopy-assisted surgery of pyriform sinus fistula (PSF) in children. The charts of 196 children diagnosed with PSF undergone endoscopy-assisted surgery during August 2010 to August 2018 were retrospectively analyzed and in particular, the nursing cooperation reviewed. All 196 cases were treated with endoscopy-assisted surgery following induction of general anesthesia with endotracheal intubation. Fistula tract was successfully found and completely resected. Good outcome was achieved in all but five patients during a follow-up of 2 months to 8 years. One patient had temporary hoarseness after surgery and recovered after 3 months. One patient developed left-side Horner’s syndrome. PSF recurred in 3 cases. Endoscopy-assisted surgery for PSF consists of two parts: endoscopy assistance and open-neck operation resection. As a combined surgery, there are integral parts for operating room nurses to make full preparation, understand the surgical method, master the surgical procedures and special requirements, strengthen the cooperation and collaboration closely with surgical personnel to ensure successful operation.

Autistic disorder (AD) is one of the most serious psychiatric disorders in children and adolescents. Identification of relevant risk factors is a main step in disease management. The aim of this study was to determine probable risk factors for AD. This case-control study was carried out in the Tabriz University of Medical Sciences. By convenience sampling method, 115 children with autism were selected from Rehabilitation Center of Autism Society in Tabriz, Iran. Moreover, 112 children were selected as control. AD was diagnosed based on DSM-IV-TR criteria by a child and adolescent psychiatrist. Asthma, epilepsy, microcephaly, hearing / vision impairments, allergy to milk/wheat, side effects of rubella vaccines, and language disorders in the immediate family members including siblings, parents’ low education and lack of breastfeeding were more prevalent in AD children. Based on the logistic regression results, mothers’ low education [Exp (B) = 4.59, CI = 2.13 - 9.87, P < 0.001] and lack of breastfeeding until the age of two [Exp (B) = 2.91, CI = 1.54 - 5.50, P < 0.01] were the predictors of AD. Parents’ education and lack of breastfeeding until the age of two could predict AD in children. Improving educational system and increasing families’ awareness about benefits of breastfeeding will be valuable.

To determine the relationship between glucose metabolism and atherosclerosis assessed according to the common carotid artery intima-media thickness (CIMT) in non-diabetic obese children. We performed anthropometric measurements and laboratory tests, including the oral glucose tolerance test (OGTT). The CIMTs on both sides were measured in obesity (body mass index [BMI] ≥ 95 percentile) children by one researcher. There were 44 boys and 22 girls, and the mean age was 10.73 ± 2.48 years (range, 8 - 14 years). The mean CIMT on both sides, age, BMI, blood pressure, lipid profile, OGTT results, and HbA1c level were not significantly different between the boys and girls. Simple correlation analyses showed that the fasting plasma glucose (PG) level (γ = 0.266, P = 0.034), PG at 90 min (γ = 0.318, P = 0.014), PG at 120 min (γ = 0.336, P = 0.009), HbA1c level (γ = 0.306, P = 0.015), PG increase at 90 min (PGΔ90) (γ = 0.310, P = 0.017), and PGΔ120 (γ = 0.338, P = 0.009) were significantly correlated with the left CIMT. In multiple regression analysis, the HbA1c level, PGΔ90, and PGΔ120 were significantly associated with the left CIMT (P < 0.01). Ultrasound-measured CIMT, especially the left CIMT, is a reasonable glucose metabolic indicator of atherosclerotic vascular complications in obese children.

Cleft sternum is a rare congenital malformation of the chest cavity. The failure of embryologic fusion of mescenchymal cells in the midline causes the amomaly, leaving the mediastinal viscera exposed to injuries. The defect is usually divided into partial or complete forms and is often accompanied by other anomalies. The etiology and incidence are not yet known. We present a case of bifid sternum with hemangioma of the face.

We report a novel mutation of the CD40L gene in X-linked hyper-IgM syndrome (XHIGM) in two sibling Chinese patients, about which we made a literature review. The two sibling boys were born to un-consanguineous parents and the proband was presented at six months of age with pneumonia, infectious diarrhea, abdominal pain, perianal abscess, anemia, and thrush alternately despite regularly intravenous immunoglobulin and anti-infection treatment. The proband died at age two due to pneumonia and his brother died at one-year-old due to perianal abscess. The proband had a less detectable expression of CD40L on the surface of peripheral blood leucocyte and granulocytes, whereas his mother had a drop of CD40L expression compared to a control group made up of healthy test subjects. Mutation analysis revealed the two sibling patients had a missense mutation within Exon 5 of CD40L gene at nucleotide position 448 (488T>G), which made a valine code (GTT) changing to a glycine code (GGT) at position 168 (V163G) of CD40L protein expression. The same heterozygous form (V163G, T>G) was also found in their mother, but the mother was not sick. There was no similar mutation former found in the father. In summary, it is the first time to report the two sibling Chinese boys of a novel missense mutation (V163G) in exon 5 in the CD40L gene.