Iranian Journal of Pediatrics
Year:2016 | Volume:26 | Issue:5|Papers Count:16

Background: Tyrosinemia is an inherited metabolic disorder characterized by elevated levels of tyrosine and its metabolites in plasma. Without treatment, the disease will progress to hepatic and renal failure, so that without liver transplantation will cause death in less than 10 years of age. So, early diagnosis and treatment can be life saving and crucial. It means that with early treatment starting in the neonatal period, the patient can have normal life with very few restrictions in diets containing tyrosine and phenylalanine.Objectives: In this study we wanted to evaluate an easy to perform, rapid and sensitive qualitative test with low cost, as a part of neonatal screening tests to help early diagnosis and treatment of hereditary tyrosinemia.Patients and Methods: In this cross sectional study, during the study period (2013 - 2014), 100 patients were selected. Fifty three (53) of these patients had proven tyrosinemia and the other 47 cases biliary atresia, paucity of intrahepatic bile ducts, cytomegalovirus (CMV) hepatitis, galactosemia and storage diseases.Results: There were 2 false negative and 14 false positive cases of hereditary tyrosinemia (HT-1) in the test. Six cases of biliary atresia, 7 cases of paucity of intrahepatic bile ducts and one patient with cytomegalovirus (CMV) hepatitis were falsely positive with the test.Sensitivity of the test was 96.23%, specificity 71.43%, positive predictive value (PPV) 78.46%, and negative predictive value (NPV) 94.59%.Conclusions: This rapid qualitative test on dried blood sample is an easy, cheap, and feasible method for the screening of hereditary tyrosinemia in neonatal period.

Dear Editor, Environmental tobacco smoke (ETS) exposure, or passive smoking, is a preventable cause of disease and disability, and a very important public health problem (1). ETS is an inclusive term describing any tobacco smoke exposure outside of active smoking, comprising secondhand smoke (SHS) and thirdhand smoke (THS) (2).

Context: Proper accountability to needs of preterm infants’ parents requires recognition of these needs and how they change in different conditions. The aim of this study was to assess the needs of parents of preterm infants in Iran, as compared to those in other regions in the world.Evidence Acquisition: A search of Iranian databases (IranMedex, Magiran, and SID) and international resources (PubMed, Scopus, and Google Scholar) was conducted, with no time limitations, to 5 October 2014. Using standard tools, all quantitative studies that considering the parental needs of preterm infants and parental support were extracted. The STROBE checklist was used for the evaluation of the studies. Thirty-one studies were extracted in the qualitative evaluation, of which 17 were included in the metaanalysis.The variance between the studies was analyzed using tau-squared (Tau2) and review manager 5 software.Results: The results obtained using the nurse-parent support tool (NPST) showed that mothers considered that all the fields of support were of great importance. The parental needs in Iran were similar to those of parents in other regions worldwide. However, the mean score for Iranian parents’ assessment of the support they received was 2.20 ± 0.06, whereas it was 3.84 ± 0.72 for other countries. The mean scores for parents’ assessment of the provision of emotional, informational appraisal, and instrumental support in Iran were 1.73±0.06, 2.1±0.06, 1.54±0.6, and 3.44±0.04, respectively, compared to 3.18±1.34, 4.11±0.5, 4.26±0.18, and 4.51±0.14, respectively, in other countries.Conclusions: Parents always prefer the priorities of their babies to their individual needs. Given the lower scores for the parental assessment of received support in Iran, it is important to focus on these specific items in providing interventions to meet the needs of Iranian parents.

Background: There is limited data on sexual maturation among girls with intellectual, sensory, or physical disabilities.Objectives: The present cross-sectional study wasconducted to assess the sexual maturation of girls with these disabilities in special schools.Methods: In this cross-sectional study, we evaluated the onset and progression of sexual maturation in 642 six to 18-year-old girls with intellectual, sensory, or physical disabilities from special schools in Tehran. The participants were selected by multi-stage random sampling. Pubertal stages were assessed by visual inspection and palpation based on the rating scales of Tanner. Stage two (breast budding and pubic hair growth) and stage five were considered the onset and end of puberty, respectively.Results: The mean ages of onset of puberty indicated by breast budding (B2 stage) and by pubic hair growth (P2 stage) were 10.8± 1.48 and 10.79 ± 1.64 years, respectively. The process of puberty based on breast budding and pubic hair growth was completed at 15.58 ± 1.85 and 15.59 ± 1.8 years, respectively. The average height at the onset of puberty (stage B2) among participants was 128 ± 28.79 cm and the average weight was 8.31±36.47 kg.Conclusions: Among our patients, the mean ages of onset of puberty indicated by breast budding (B2 stage) and by pubic hair growth (P2 stage) were 10.8 ± 1.48 and 10.79 ± 1.64 years, respectively. Compared to the data from healthy Iranian girls, our findings indicate that the mean age of pubertal onset among schoolgirls with disabilities is slightly higher than that of their healthy counterparts.

Background: It is stated in the literature that a mother’s breast milk is sufficient for more than one baby.Objectives: This descriptive study aimed to determine whether twin babies are breastfed during their first six months of life.Materials and Methods: We studied the twin birth cases in a state hospital in Sakarya, Turkey between October 2011 and March 2013.The population of the study consisted of entire mothers who had delivered twins in the maternity ward of the hospital. The sample comprised 30 twins’ mothers who agreed to participate in the study via telephone for six months. During these phone calls, they were asked how they preferred to feed their babies. The data were expressed as the mean and percentage.Results: The average age of the mothers participating in the study was 30.17±5.16 years (min.19; max.38). The number of mothers who stated that they had no previous experience of breastfeeding twin babies and had received training to breastfeed multiple babies was 17 (56.7%). Twenty-seven (90%) of the mothers had had caesarean sections, and half of the babies were preterm. Only a few of the babies were fed breast milk for five months. During the following months, the breastfeeding regimen was as follows: 5 babies were breastfed for a month, 5 babies for 2 months, 4 babies for 3 months, 4 babies for 4 months, 2 babies for 5 months, and no babies in the sixth month.Conclusions: It was found that the number of twin babies who were only breastfed in the first six months of life was low. According to the literature, a mother’s breast milk is sufficient for multiple babies. Mothers expecting twin babies should be informed about the benefits of breastfeeding and be encouraged to breastfeed. They should also receive training on this subject.

Background: Although several different types of natural surfactants are available, including Alveofact, Curosurf, and Survanta, the preferred type and the magnitude of their effects are unknown.Objectives: This study was designed to compare the effects of these three surfactants on the gas exchange and clinical outcomes of neonates with respiratory distress syndrome (RDS).Methods: This triple-blind randomized clinical trial studied all preterm neonates ≤37 weeks with RDS who were admitted to the neonatal intensive care unit (NICU) of Taleghani hospital (Tabriz, Iran) between 2012 and 2013. The patients were divided into three groups, each of which received one of these surfactants. The incidences of ventilator dependency, patent ductus arteriosus (PDA), broncho-pulmonary dysplasia (BPD), retinopathy of prematurity (ROP), intraventricular hemorrhage (IVH), hospital-stay length, and continuous positive airway pressure (CPAP) failure, as well as blood gas levels, were recorded as endpoint measurements and compared.Results: In total, 165 neonates of gestational age≤37 weeks with RDS were examined. Neonates were allocated to three different groups randomly, including a Survanta group (n=49), a Curosurf group (n=62), and an Alveofact group (n=54). The mean gestational age of the neonates was 31.6± 3.7 weeks, and their mean weight was 1, 840±790 grams. The male/female ratio was 2: 1 (67% male, 33% female); 104 (63%) neonates were>32 weeks gestational age, and 61 (37%) were>32 weeks. There were no significant differences for gender or demographic characteristics among the neonates in relation to the type of applied surfactant. According to the clinical parameters (BPD, IVH, ROP, hospital-stay length, and mechanical ventilation requirement), no significant differences were observed between the groups before and after surfactant administration, but the differences between the Survanta and Alveofact groups for the incidence rates of pneumothorax (P=0.03) and pulmonary hemorrhage (P=0.03) were statistically significant.Conclusions: Nosignificant differences were observed inmostof the clinical variables between the three types of natural surfactant, but in neonates ≤32 weeks, the incidence of pneumothorax was significantly higher in the Alveofact group; in neonates>32 weeks, the incidences of PDA, mean hospital-stay length, and mean mechanical ventilation time were also significantly higher in the same group. It thus appears that Curosurf and Survanta replacement therapies among premature neonates with RDS perform better than Alveofact replacement therapy.

Background: Healthcare-associated bloodstream infections (HCA-BSI) are a major cause of morbidity and mortality in neonatal intensive care units (NICUs).Objectives: We aimed to determine the causative organisms and risk factors of HCA-BSIs in NICUs.Methods: This study was performed between January 2011 and December 2014 in the neonatal intensive care unit of Dicle university, Turkey. The study consisted of 126 patients (infected group) with positive blood culture and 126 randomly selected patients (uninfected control group) with negative blood culture after four days of hospitalization.Results: We found that the most common causative agents isolated from nosocomial infections (NIs) were 20.7% Staphylococcus epidermidis, 26.7% Klebsiella spp., and 13.3% Acinetobacter spp. Incidences of low gestational age, low birth weight, vaginal birth type, and long length of hospitalization were higher in the infected neonates than in the uninfected neonates. In the univariate analysis, surgical operation, ventriculoperitoneal shunt, use of umbilical catheter, nasogastric or orogastric tube, urinary catheter, mechanical ventilation, surfactant treatment, erythrocyte transfusion, plasma transfusion, thrombocyte transfusion, total parenteral nutrition infusion, intracranial hemorrhage, length of hospital stay, fifth-minute Apgar score, and total parenteral nutrition time were significantly associated with NIs. In the multiple logistic regression analysis, fifth-minute Apgar, use of erythrocyte transfusion and surgical operation were found as the independent risk factors for HCA-BSI.Conclusions: This study determined the causative organisms and risk factors of HCA-BSIs in NICUs.

Background: Several research trials have analyzed the impact of prematurity on the prevalence of hypertension (HT). However, prospective long-term studies are lacking.Objectives: The aim of this study was to evaluate the prevalence of HT at the age of 7 and 11 years in a regional cohort of preterm infants with a birth weight of ≤1000 g.Patients and Methods: This study included 67 children with a birth weight of ≤1000 g who were born in Malopolska between September 2002 and August 2004. The control group consisted of 38 children born at term, matched for age. Each child underwent 24-h ambulatory blood pressure measurement (ABPM) twice, once at the age of 7 and again at 11 years. The presence of HT was estimated according to the mean arterial pressure (MAP) and a number of individual measurements.Results: At aged 7 years, preterm infants had a significantly higher incidence of HT, defined on the basis of MAP (15% vs.0%; P< 0.02) and on the percent of individual measurements (56% vs.33%, P<0.036). After taking into account the group of patients who received anti-HT treatment after the first part of the study, the incidence of HT at the age of 11 years based on MAP was 19% vs.10%.Based on the individual measurements, it was 36.5% in the preterm infants vs.24% in the control group. The differences were not statistically significant. At both time points, the preterm group had a higher mean heart rate (HR) than the control group.Conclusions: Children born prematurely are predisposed to HT in later life, in addition to the persistence of an increased HR.

Introduction: Lamellar ichthyosis (collodion baby) is a cornification disorder classified under the category of autosomal recessive congenital ichthyosis and characterized by hyperkeratosis. Early-stage retinoid treatment has been shown to improve survival in these patients. In this article, a lamellar ichthyosis case is presented of an infant who had the symptoms at birth and was treated successfully with acitretin.Case Presentation: A term newborn infant presented after delivery. Physical examination showed that the skin on her outer mouth, neck, axillae, and inguinal fold areas had collodion membranes and peelings. On the third day of life, the skin all over her body became dry and seemed similar to parchment paper, with peeling in some areas, as well as ectropion and eclabium development. After her daily bath, liquid Vaseline was applied all over her body, but it did not provide enough benefits. The infant was started on acitretin treatment. On the 14th day of treatment, the skin appeared nearly normal. On the 28th day of life, the infant was discharged.Conclusions: Early oral retinoid treatment facilitates increased quality of life improves survival rates for ichthyosis patients.

Background: Advances in perinatal and neonatal care have substantially improved the survival of at-risk infants over the past two decades.Objectives: The purpose of this study was to assess the reliability and validity of the Bayley Scales of infant and toddler developmental Screening test in Persian-speaking children.Methods: This was a cross-sectional prospective study of 403 children aged 1 - 42-months. The Bayley scales screening instrument, which consists of five domains (cognitive, receptive, and expressive communication and fine and gross motor items), was used to measure infants’ and toddlers’ development. The psychometric properties examined included the face and content validity of the scale, in addition to cultural and linguistic modifications to the scale and its test-retest and inter-rater reliability.Results: An expert team changed some of the test items relating to cultural and linguistic issues. In almost all the age groups, cultural or linguistic changes were made to items in the communication domains. According to Cronbach’s alpha for internal consistency, the reliability of the cognitive scale was r=0.79, and the reliability of the receptive scale was r=0.76. The reliability for expressive communication, fine motor, and gross motor scales was r=0.81, r=0.80, and r=0.81, respectively. The construct validity of the tests was confirmed using a factor analysis and comparison of the mean scores of the age groups. The intra- and inter-rater reliabilities of the Bayley Scales were good-to-excellent.Conclusions: The results indicated that the Bayley Scales had a high level of reliability in the present study. Thus, the scale can be used in a Persian population.

Niemann-Pick is a lipid storage disease that results from a lysosomal enzyme deficiency (sphingomyelinase). It has different presentations, and it may affect various organs such as the central nervous system, kidney, liver, and spleen. Due to the complexity of the disease, careful perianesthetic management is necessary in order to reduce the risks and sequels. As there is little evidence available in the literature regarding the anesthetic implications of such patients, in this case report we describe the anesthetic management of a two-year-old female with Niemann-Pick disease.

Background: Pyeloplasty for ureteropelvic junction obstruction correction is a common procedure, but the optimal method for protective diversion after pyeloplasty is still a matter of debate.Objectives: Here, we present our clinical trial experience using a single percutaneous externalized nephroureteral (NU) 5-Fr catheter (infant feeding tube) with multiple side holes as the sole instrument of drainage to provide a protective mechanism.Materials and Methods: In this prospective study, we analyzed the charts of 142 patients who underwent pyeloplasty from August 2001 through October 2008. We used a single externalized NU 5-Fr catheter with multiple side holes for postoperative upper tract diversion. The catheter was removed in the office after 10 - 14 days. Complications from the use of this catheter, including poor catheter function, premature dislodgement, urinary tract infection, leakage, urinoma, and anastomotic stenosis, were evaluated.The operations were performed by two surgeons at two separate centers.Results: In all, 148 pyeloplasty procedures were performed on 142 patients. Themeanhospital stay length was 2 (1 - 3) days. A contrast study through a catheter demonstrated excellent drainage with no leakage in all patients. Immediately after catheter removal, febrile urinary tract infection and transient obstructive symptoms and signs occurred in 15 patients.Conclusions: Using a percutaneous externalized NU 5-Fr catheter was sufficient as a protective measure after open pyeloplasty. It costs less than other diverting systems, such as DJ, and can be removed in the office. Therefore, it can be a safe and cost effective procedure, especially in developing countries where cystoscopic set ups are not readily available. There were only a few notable complications.

Background: Although poor health conditions and decreased developmental levels have been investigated in migrant children, no study in China has focused on these children’s individual motor development.Objectives: This study aims to explore the prevalence of motor impairment in Chinese migrant children and to determine the contributory factors.Patients and Methods: In this cross-sectional study, a structured questionnaire was administered to primary caregivers of preschool children aged 3 - 6 (n=2, 976) in ten kindergartens from two districts of Suzhou, China, to assess the children’s home socioeconomic status and motor environment, that is, the presence of affordances for motor development. Motor ability was assessed using the Movement Assessment battery for children-second edition (MABC-2). Multiple logistic regression analysis was used to determine the risk factors for motor impairment in migrant children.Results: Migrant children showed correlations with impairment in manual dexterity, aiming and catching, and motor development (odds ratios [ORs] =1.320, 1.255, 1.260, respectively; P<0.05). Outdoor movement affordances and toys for fine motor development were significantly associated with motor impairment in migrant children (ORs=0.834 [movement affordances, 0.843 [toys], P<0.05).Conclusions: Chinese migrant children are at a high risk of motor impairment, which is associated with a lack of outdoor movement affordances and toys for fine motor development. Future prevention and intervention should focus on the motor environment of the home.

Background: In recent decades, family-centered care (FCC) has come to be known, accepted, and reported as the best care strategy for admitted children and their families. However, in spite of the increasing application of this approach, the experiences of the caregivers have not yet been studied.Objectives: The present study aimed at the description and interpretation of the FCC experience in two neonatal intensive care units (NICU) at Shiraz University of Medical Sciences.Methods: This study was conducted through the hermeneutic phenomenological approach. Semi-structured interviews were conducted with 17 professional and familial caregivers, and their interactions were observed in three work shifts. The interviews were audiotaped and transcribed verbatim. After observations, field notes were also written. Finally, the data were analyzed through van Manen’s methodology.Results: One of the essential themes that emerged in this study was the “evocation of being at home” among familial and even professional caregivers. This theme had three subthemes: i.e., “meta-family interaction, ” “comprehensive support, ” and “reconstruction of a normal family.” Accordingly, FCC eliminated borders between professional and non-professional caregivers and built close relationships among them in the NICU. It also provided for the needs of neonates, their families, and even professional caregivers through perceived and received support.Conclusions: Parents of the neonates admitted to the NICU experience hard moments. They not only play the role of primary caregivers, but they also receive the care. Focusing on the different meanings of this care from the caregivers’ points of view and having managers provide certain requirements can guarantee the establishment of comprehensive care for clients and proper support for the staff in this unit.

Background: Excessive iron accumulation in the visceral organs creates problems for patients with beta-thalassemia major. Despite chelation therapy, mortality rate from the complications of this disease is still quite high.Objectives: This study aimed to investigate the effectiveness of nutrition, exercise, and a praying program at reducing iron overload in patients with beta-thalassemia major.Patients and Methods: This randomized clinical trial assessed the effect of the designed care program on iron overload. The study was conducted in 38 patients with beta-thalassemia major who ranged in age from 15 - 35 years and had been referred to the largest center for thalassemic patients in Shiraz. The patients were randomly assigned to an intervention (n=18) and a control (n=20) group. Blood samples were collected from the participants before and two months after the intervention. Then, the data were statistically analyzed using chi-square, Fisher’s exact test, Mann-Whitney U-test, Wilcoxon, independent samples t-test, and paired samples t-test.Results: The results showed that the mean level of serum ferritin significantly decreased in the intervention group two months after beginning the intervention. Also, the mean level of serum iron decreased in the intervention group, but the difference was not statistically significant.Conclusions: The planned educational program could be used to reduce iron overload and ultimately improve the patients’ health status.

Background: Information from pulse oximeter waves confirms the presence of a pulse and helps obtain waves from tissue when the supplying artery is not readily accessible.Objectives: This study determined the predictive value of pulse oximeters for detecting improved arterial pulses after angiography.Patients and Methods: This cross-sectional, multi-center study included 467 4-day-old to 12-year-old patients and was conducted from January 2012 to January 2016. Angiographies were performed on 12-year-old or younger children for various medical reasons using venous, arterial, or both types of paths. The posterior malleolar or dorsalis pedis were palpated in punctured lower extremities.In the absence of a pulse, pulse oximetry was performed to identify pulse curves at 1 hour, 6 hours, and 12 hours after each angiography.Results: Pulse oximetry displayed the pulses of 319 patients immediately following each angiography. Of these, 262 patients had palpable pulses at 6 hours after angiography (P<0.0001), while 57 patients had no palpable pulse. Of these 57 patients, 15 had no palpable pulse at 12 hours after angiography (P<0.0001). The odds of pulse improvement in children 6 hours after catheter angiography were 76% for the arterial path, 90% for the venous path, and83.2% for both paths. At 12 hours after catheter angiography, these values increased to 91.6% for the arterial path, 100% for the venous path, and 95.9% for both paths.Conclusions: The pulse oximeter can display the pulse curve immediately (1 hour) after angiography and indicate pulse improvement at 12 hours maximally following an angiography. In this case, heparin alone may be used instead of thrombolytic agents.