Iranian Journal of Pediatrics
Volume:26 Issue: 6, Dec 2016

Context: Pediatric abusive head trauma (AHT) can be defined as an injury to the skull or intracranial contents of a child under the age of 5 due to inflicted blunt impact or violent shaking.
Evidence Acquisition: AHT is the most common cause of traumatic death in children younger than 1 year, and it is the leading cause of death due to child abuse. Clinical presentation observed in children with AHT depends on the type of AHT and accompanying injuries. History and physical examination are important for diagnosing AHT and for distinguishing it from other conditions that can mimic shaken baby syndrome, such as accidental trauma, cancer, metabolic diseases, and others.
Progress in research on the medical diagnosis of AHT has been remarkable, while the development of treatment strategies has been limited. For these reasons, there is an urgent need to develop effective treatment strategies for AHT able to improve the outcomes.
The construction of a nationwide database that supports clinical studies is required in the future.

Context: Functional constipation (FC) and irritable bowel syndrome (IBS) represent very common pediatric functional gastrointestinal disorders (FGIDs). Controversial results have suggested a potential role of food allergy as a trigger of functional bowel symptoms.
Evidence Acquisition: This review summarizes the literature regarding the role of allergic diseases in children with FC and IBS and discusses the hypothesis of the pathogenesis of constipation due to cow’s milk protein allergy (CMPA). We searched systematic reviews, guidelines, or original data in PubMed, MEDLINE, and the Cochrane central register of controlled trials.
The pathogenesis of FGIDs remains elusive and is likely multifactorial. Among these factors, adverse reactions to food may play a pathogenic role. Some features, such as abnormal bowel motility, visceral hypersensitivity, and changes in mucus composition caused by inflammation of the gastrointestinal wall, have been found both in IBS or FC and in food allergy. Since 1978, an increasing number of reports have suggested a relationship between CMPA and FC. Two randomized controlled studies conducted in children showed that CMPA may induce chronic FC; one study indicated that fermentable oligosaccharide, disaccharide, and monosaccharide polyols (FODMAP) foods may play a role in triggering IBS.
Food allergy in children with chronic constipation should be identified using an oral food challenge after being on a diet free of cow’s milk. A diet low in FODMAPs might also be recommended for children with IBS. This approach could be suggested for children with chronic FC and IBS, especially when they do not respond to standard treatment. However, it should also be considered that a minority of patients with FC or IBS could respond to an elimination diet. Further studies are needed to understand the complex pathogenic mechanisms of FGIDs; they also might be helpful to recognize markers for identifying children with IBS and FC caused by foods and to improve their management.

Inborn errors of metabolism (IEM) are disorders caused by genetic mutations of the normal metabolic pathways.
The aim of this study was to investigate clinical findings and laboratory tests for IEMs to determine a proper clinical and laboratorial approach to these disorders.
This descriptive study involved 180 patients with suspected IEM at the children’s medical center (CHM) in Tehran. The patients’ information was recorded in special forms, and three levels of examinations were performed. Data were subjected to frequency, percent, and chi square tests using SPSS version 17.
The study sample included 87 (48.3%) female patients and 93 (51.7%) male patients. The most common (20%) clinical presentation of patients with IEMs was poor feeding or failure to thrive, and the frequency of the condition in children younger than age 1 year was approximately 35%. Eighty percent of patients received a definitive diagnosis by the first and second level of examination. The most common (17%) disorders were proponic acidemia, methylmalonic acidemia (14%), and urea cycle disorder (14%).
Considering the non-specific primary presentation of IEMs and the importance of clinical diagnosis other than specific laboratory tests, it is necessary to develop a general guide for an approach to IEM that provides appropriate management for high-risk families and infants.

To demonstrate the effects of the injection of intravitreal bevacizumab (IVB) on the clinical course of established bronchopulmonary dysplasia (BPD).
This is a multicenter retrospective case-control study performed without any randomization or masking procedure. A total of 70 patients with BPD, including 35 cases (the IVB group) and 35 controls (the control group) were studied. Patients in the IVB group received intravitreal anti-VEGF (bevacizumab) treatment for type 1 prethreshold retinopathy of prematurity (ROP). The control group consisted of infants with BPD whose gestational age, birthweight, and gender were matched with those of the IVB group. None of the infants in the control group needed to be treated for ROP using either anti-VEGF or laser photocoagulation.
There was no statistically significant difference (P = 0.11) between the groups in terms of the total duration of oxygen prior to the IVB injection (65.9 ± 23.5 and 79.1 ± 33.1 days in the IVB group and control group, respectively). However, after the injection of IVB, the total duration of oxygen was significantly lower in the IVB group (mean 7 (1 - 70) days vs. 16 (1 - 98) days, P = 0.01). In 14 cases with mild BPD and their matched controls, the median time (25% - 75%) for the discontinuation of oxygen therapy was 3 (2 - 7) days and 10 (5 - 15) days, respectively (P = 0.36). In 21 cases with moderate and severe BPD and their matched controls, the median time (25 - 75%) for the discontinuation of oxygen therapy was 14 (7 - 21) days and 22 (16 - 43) days, respectively (P = 0.024).
Intravitreal bevacizumab injection treatment for ROP cases with BPD was found to be associated with a shorter duration of oxygen use. The results of the study not only demonstrate a pathogenic correlation between ROP and BPD through an abnormal vasculogenesis, but also raise a question regarding whether or not the systemic side effects of IVB are actually adverse.

In order to enable children with type 1 diabetes to live in harmony with their disease, it is necessary to become better acquainted with the attitudes developed by such children toward their condition.
This study was conducted to determine the attitudes of children with type 1 diabetes toward their disease, as well as to determine the factors that affect those attitudes.
Patients and This descriptive study was conducted in the pediatric endocrinology polyclinics of two hospitals in eastern Turkey between September 2013 and June 2014. The study population consisted of 150 children with diabetes who were registered at the pediatric endocrinology polyclinics. Without using a sampling method, the study was conducted with 104 diabetic children. The children’s introductory form and the child attitude toward illness scale were used to collect data for the study. To evaluate the data and determine the relevant percentages and means, an independent samples t-test, the Mann-Whitney U test, the Kruskal-Wallis test, and Cronbach’s alpha coefficient were used.
It was determined that 58.7% of the children were aged 13 - 18 years, 56.7% were female, and 45.2% had received education to the ninth to twelfth grade level. The conditions that affected the children’s attitudes toward their disease were whether or not they had social security, their mothers’ education level, their mothers’ working conditions, their fathers’ education level, and whether or not members of their immediate family also had diabetes (P This study revealed that children with type 1 diabetes had neutral attitudes (i.e., neither a positive nor a negative attitude) toward their disease, which were affected by certain variables.

Because some children are already being selected for some sports disciplines by the age of eight, it is important to know whether quantitative measurement tests could serve as a good selection tool in this context.
The aim of this study was to determine the possible influence of motor-functional abilities and morphological characteristics on fundamental movement skills in eight-year-old children.
A total of 90 children (45 boys and 45 girls) participated in this study and were subjected to two tests for morphological status assessment (body height and body weight), three tests of motor-functional abilities (forward bow, standing long jump, and 1/4 mile run), and one test of fundamental motor skills (FMS-POLYGON). Multiple regression analysis was used to determine the influence of morphological characteristics and motor-functional abilities on fundamental movement skills.
The results of this study show that explosive strength and endurance are significant predictors of FMS-POLYGON performance in both genders, whereas body height is a significant predictor only in boys.
We conclude that girls with high endurance and strength have better results in the FMS-POLYGON, whereas boys must be taller and have higher degrees of endurance and strength to excel. Future studies should determine the trends and laws of influence concerning the analyzed factors in children of different ages.

Establishing venous access is a challenging job for pediatric nurses, especially in the emergency department. Measures to ensure higher success rates on the first attempt are important to provide quality nursing care.
To explore the effect of grade management on the success of establishing peripheral venous access in the child population of China and to analyze the factors influencing failed IV access on the first attempt.
This is an observational study on children aged 0 - 16 years old undergoing peripheral venous catheterization in a children’s hospital. Patient information was collected before attempting each puncture. Logistic regression was used to identify independent factors for success prediction.
A total of 1,016 subjects enrolled. The success rate of intravenous puncture on the first attempt was 86.02%. This can be influenced by several factors, such as patient age, department, venous condition, and nurse experience. The success rate within two attempts was 96.85%; the number of catheters used per IV attempt was 1.21.
To date, only a few studies have explored the success rate of peripheral IV catheterization in the pediatric population of China. Grade management of peripheral veins and pediatric nurses facilitated higher first-attempt success than in previously published reports. Failures of catheterization were multifactorial. The success rate of peripheral intravenous insertion in children can be improved through applying assistance devices or enhancing the venipuncture skills of pediatric nurses.

Celiac disease is an autoimmune disorder characterized by damage to the small intestinal mucosa following the intake of gluten-containing foods in genetically predisposed individuals. Studies suggest that celiac disease is associated with asthma and allergic rhinitis.
The aim of this study was to examine the prevalence of asthma and allergic rhinitis in children with celiac disease compared to healthy controls.
This study was conducted in 53 celiac children and 80 children with nonspecific abdominal pain as a control group; all participants were between 6 - 19 years. The children completed the ISAAC questionnaire, and spirometry was performed in each participant.
The prevalence rates of asthma symptoms and physician-diagnosed asthma were similar in both groups (30% and 19%, respectively, in the celiac group and 17.5% and 22.5%, respectively, in the control group) (P > 0.05). Six (11%) patients with celiac disease and seven (9%) children in the control group showed obstructive changes on pulmonary function tests. The prevalence rates of allergic rhinitis symptoms and physician-diagnosed allergic rhinitis were also similar in both groups (36% and 9%, respectively, in the celiac group, and 34% and 9%, respectively, in the control group) (P > 0.05).
The prevalence rates of asthma and allergic rhinitis in patients with celiac disease were not significantly higher when compared to the healthy controls.

The bacterial diversity and microbial communities in the feces of children with cow milk protein allergy (CMPA) may be altered; these changes have not been clearly elucidated in 5 - 8-year-old children with CMPA.
The aim of this study was to compare the bacterial diversity and microbial communities of feces in 5 - 8-year-old children with CMPA and those of healthy children of the same age.
Stool samples from 12 children with CMPA and 12 healthy individuals were collected. Total bacterial DNA of all samples were extracted and analyzed by polymerase chain reaction-denaturing gradient gel electrophoresis (PCR-DGGE) using bacterial v3 universal primers and the species-specific primers of Bifidobacterium, Lactobacillus, and Clostridium coccoides. The preponderant bacterial bands were identified by gene sequencing.
The results revealed that the diversity of fecal dominant microbiota and C. coccoides group in children with CMPA were significantly higher (P There were some notable changes in the fecal microbiota of 5 - 8-year-old children with CMPA.

Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous cholestatic disease (PFIC types 1, 2, and 3). Each type of the disease has different presentation, laboratory findings and prognosis. Up to now, there has been no report on the different types of this disease from Iran.
In this study, we have tried to uncover the frequencies and clinicopathologic presentations of three types of PFIC over four years (2010 - 2014) in Namazi hospital, which is affiliated with the Shiraz University of Medical Sciences and is the main hepatobiliary center in Southern Iran.
During the above-mentioned four-year period, we studied 68 liver tissues taken from patients with a proven PFIC diagnosis, either in the form of a liver needle biopsy or explanted liver. Immunostaining was performed using three antibodies (ATP8B1, ABCB11, and ABCB4) on liver sections. Hematoxylin and eosin-stained sections were also reviewed. All of the demographics, clinical and paraclinical data were retrieved from the patients’ clinical charts.
Among these 68 PFIC cases, the most common type of PFIC in our center was found to be type 2 (50%), followed by type 1 (42.6%), with type 3 (7.4%) representing the least common. Pruritis and jaundice have been identified as the most common symptoms in all three groups; however, patients with PFIC type 3 showed older age with higher GGT levels.
The two most common subtypes of PFIC in Iran are types 2 and 1, similar to some areas in the West. Immunostaining can accurately subclassify patients with PFIC.

Recurrent respiratory papillomatosis (RRP) is usually a relatively benign disease but can have an aggressive clinical course. The primary treatment for laryngeal papillomatosis is endoscopic resection. However, there is no effective treatment for pulmonary spread of laryngeal papillomatosis.
We describe a rare case of a 19-year-old man with pulmonary spread of laryngeal papillomatosis. The treatment regimen was surgical resection under general anesthesia using suspension laryngoscopy and endoscopy. The patient had been diagnosed with laryngeal papillomatosis and had undergone a tracheotomy when he was 18 months old. He underwent 17 endoscopic resections for laryngeal papillomatosis at intervals of 3 months to 1 year. At follow-up, the patient continued to have tracheal intubation and was not extubated. CT scans revealed multiple nodular and cavitary lesions in both lungs. Laryngoscopy performed when the patient was 18 years showed diffuse papillomata in the glottis and left ventricular fold.
This article presents a rare case of juvenile laryngeal papillomatosis associated with the human papilloma virus (HPV) HPV-11 and pulmonary spread. Intravenous cidofovir has oncogenic potential, but it has been used successfully to treat five patients with pulmonary spread of RRP. Intravenous cidofovir should be considered as salvage therapy.

We report on a novel mutation in a two-year-old child with autosomal recessive cutis laxa with severe generalized laxity of the skin, prematurely aged appearance, conjunctival chalasia, episodes of severe rectal prolapse, isolated right ventricular non-compaction, (RVNC), significant pulmonary hypertension at the systemic arterial pressure level, severe tricuspid regurgitation, corpulmonale secondary to recurrent pulmonary infections, and mixed pulmonary fibrosis and emphysema. Next generation sequencing of cutis laxa genes identified a novel homozygous mutation in the FBLN5 gene (homozygous sequence alteration of c.907C > T [p. Gin303*] FBLN5 [ENST00000342058]). Despite severe and generalized disorder in the patient’s connective tissues, she had no primary valvar or vascular abnormalities in the heart. Complete speckle-tracking strain imaging (SI) by two-dimensional echocardiography showed decreased systolic longitudinal and transverse strain in the involved segment of the right ventricle (RV).

Dieulafoy lesion is an unusual cause of gastrointestinal bleeding in which significant, and often recurrent, hemorrhage occurs from a pinpoint non-ulcerated arterial lesion. It is usually located in gastric fundus, and is extremely rare in children.
In this study, we present a male adolescent with jejunal Dieulafoy lesion. The patient was admitted to the hospital due to hematochezia and hypotension. No active bleeders were identified on endoscopy, colonoscopy, and double-balloon enteroscopy. Computed tomography angiography revealed active bleeders in jejunum. An intraoperative enteroscopy, using flexible endoscopy, revealed a jejunal Dieulafoy lesion with spurting bleeding. The patient was successfully treated by bowel resection.
Intraoperative endoscopy is a relatively efficient and safe modality for evaluating the bleeding Dieulafoy lesion in the small intestine, and is essential for directing surgery.