Iranian Journal of Pediatrics
Volume:24 Issue: 5, Oct 2014

Clinical Practice guidelines (CPGs) have emerged as a potentially effective intervention in delivering a high quality, consistent, safe and evidence-based health care. CPGs can either be developed by de novo synthesis or by adaptation of existing guidelines formed in another organization. Guideline recommendations are formulated based on strength of the evidence, validity, clinical relevance and patient values. Support of the organization leadership, role modeling of senior staff and involvement of stakeholders is a key to the success of implementation of guidelines. This article aims to enhance a practicing pediatrician's understanding of how guidelines are developed, disseminated, and potentially utilized.

Metformin, an oral anti-diabetic agent in the biguanide class is a widely prescribed drug to treat high blood glucose in patients with type 2 diabetes mellitus. Metformin has three different roles, including blood glucose regulatory effect, protection of kidney tubular cell by acting as an effective antioxidant and finally ameliorative effect on diabetic kidney disease through saving the podocytes. Therefore, diabetic patients may benefit from all of these three distinct ameliorative effects.

Acinetobacter baumannii is an important emerging cause for extensively-drug resistant (XDR) hospital associated infections (HAIs) in pediatric intensive care units (PICU). The study was done to evaluate the risk factors, outcome, antibiotic sensitivity pattern, and predictors of mortality in critically ill children with XDR A. baumannii infection. Retrospective case control study, done in the PICU of a tertiary care pediatric hospital of India from April 2010 to March 2012. Eighty-five children who developed XDR A. baumannii infection matched to 170 controls. Majority (76%) of the organisms were isolated from endotracheal lavage. The mortality rate was 28.2% (24/85). The factors found to be significantly associated with A. baumannii infection were prior use of broad-spectrum antibiotics, major surgeries done, prolonged PICU stay, use of central venous catheters, and mechanical ventilation. The predictors of mortality associated with A. baumannii infection were acute kidney injury, presence of septic shock, and disseminated intravascular coagulopathy. Colistin found to be the single most effective drug against XDR A. baumannii infection. XDR A. baumannii infections are associated with high morbidity and mortality in critically ill children. Early diagnosis and treatment are crucial. Implementation of infection control practices and rational use of antibiotics are required to control such infections.

The incidence of Transient Tachypnea of Newborn (TTN) is higher in infants born by cesarean section than with vaginal delivery. Treatment of transient tachypnea of newborn is supportive. The purpose of this study was to assess the effect of restricted fluid volume intake on the course of respiratory distress in patients with TTN. This is a quasi-experimental clinical trial of 83 neonates diagnosed with TTN admitted to a neonatal intensive care unit in south west Iran. In this study the effect of restriction of maintenance fluid volume in the course of respiratory distress in newborns with transient tachypnea was assessed. In the standard fluid volume intake group 18 (42.8%) cases needed nasal continuous positive airway pressure (NCPAP) and one (2.38%) case mechanical ventilation, and in restricted fluid volume intake group 13 (32.5%) cases needed NCPAP and two (5%) cases mechanical ventilation. 54.82% of cases were supported with oxyhood in the standard fluid volume and 62.5% in the restricted fluid volume intake group. Differences in duration of the needed NCPAP and oxygen hood between the two groups were significant. Fluid restriction had no adverse effect on the urine specific gravity or weight loss of the studied newborns. Limited fluid administered to newborns with transient tachypnea of newborn is safe and resulted in shorter duration of respiratory support.

Motor development is frequently reported to be impaired in very low birth weight (VLBW) infants, but little is known about the moderately low birth weight (MLBW) infants. The aim of this study was to investigate whether MLBW preterm infants present developmental delay. In a historical cohort study, 18±2 month-old infants with a history of low birth weight (LBW) were identified. All infants with complications of LBW with negative effects on development were excluded. Healthy infants with normal birth weight (2500–4000 g) were included as controls. All infants were evaluated by the Peabody Developmental Motor Scale II (PDMS-2) test and final scores compared between the two groups. 88 infants including 58 MLBW and 30 NBW with a mean birth weight of 1900±382.4 g and 3150±473.5 g respectively, were studied. In the MLBW group, gross and fine motor skill scores were below average in 6 (6.8%) and 10 (17%) infants, respectively. There were no significant differences between the two groups according to gross motor quotient (102.5±5.5 in NBW vs 100.1±7.2 in MLBW; P=0.1), but MLBW infants achieved significantly lower scores in fine motor (93.3±5.4 vs 99.6±5.0; P=0.001) and total motor quotient (97.0±5.9 vs 101.53±5.0; P=0.001). The finding of this study show developmental defects in fine motor skills in MLBW infants. Accurate monitoring of the developmental status of this population should be emphasized for an earlier recognition and intervention.

This study aimed to evaluate the prevalence of celiac disease (CD) in the patients with dilated cardiomyopathy (DCM). Simultaneous presentation of these two diseases has been recently reported in some studies; however, few researches have been done on children. The sooner CD is diagnosed, the better the prognosis will be, especially in the patients with a chronic disease like DCM. In this study, 82 cases were screened for CD by measuring the level of anti-body against transglutaminase (anti tTG). These cases included 41 patients with DCM labeled according to clinical evaluation and echocardiography and 41 healthy children who had been referred for routine checkup. All the patients were between 1 and 18 years old. The expired patients and those with previous diagnosis of CD were excluded from the study. Besides, the patients with positive antibody results underwent intestinal biopsy to match the serology findings with histopathology of CD in the intestine. Finally, the data were analyzed by the SPSS statistical software (v. 16) and through t-test and Pearson correlation coefficient. According to the findings, 1/41 (2.5%) DCM cases had positive tTG antibody level and negative intestinal biopsy which is classified as potential CD in the children with DCM. In addition, 7/41 (17%) patients had borderline anti body level. A direct correlation was observed between age and anti tTG level. It is beneficial to assess CD in DCM children with unknown cause.

Bronchiolitis is a common lower respiratory tract infection in the first year of life. In this disease upper respiratory tract infection is associated with nasal congestion, respiratory distress and hypoxia. We studied the effect of phenylephrine drops as a decongestant in treatment of light and moderately severe cases of acute bronchiolitis. This is a double blind randomized trial involving 100 children aged 4 weeks to 12 months. The patients were divided into two groups, the first group received 0.1 ml phenylephrine 0.5% and the second group 0.1 ml sodium chloride (NaCl) 0.9% as placebo in both nostrils. Respiratory rate, heart rate, O2 saturation, dyspnea, retractions and wheezing were assessed before and 30 minutes after medication. After medication, O2 saturation and respiratory muscles retractions in the phenylephrine group were significantly better than those of the placebo group (P=0.004 and P=0.002, respectively). In the phenylephrine group, O2 saturation, retractions and wheezing were also significantly better before than those after medication (P=0.003 and P<0.0001 respectively). In the placebo group no significant difference before and after intervention was observed. Phenylephrine as a topical decongestant is an inexpensive, easily available and suitable means in the treatment of mild to moderately severe bronchiolitis.

The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a nonhereditary idiopathic febrile syndrome belonging to the group of autoinflammatory diseases. No longtime sequel was reported in this disease. Early diagnosis can lead physicians to treatment of this disorder with a short course steroid application and provide satisfaction of the patient’s family. This study is a prospective review of patients diagnosed with PFAPA syndrome who were registered in Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR) through periodic fever clinic in the Children's Medical Center, Pediatric Center of Excellence in Tehran, Iran from January 2013 to March 2014. One hundred thirty patients were registered in our databases. Twenty-one (16.1%) patients including 15 males and 6 females had PFAPA. Normal growth was seen in all patients. The median age at onset was 18 months. The mean duration of fever was 4 days and the mean duration of intervals between fever episodes 21 days. Along with fever, all patients had characteristic symptoms. All patients were asymptomatic between fever episodes. Steroid was used in all patients and causing immediate reduction by 84.61%. Two patients received both steroid and colchicine because of their clinical feature and positive laboratory tests for PFAPA and familial Mediterranean fever. No patient received biological therapy or a tonsillectomy. The long diagnostic delay of PFAPA gives cause to concern indicating a need for greater awareness of the disease so that the diagnosis may be made timely.

Prediction of survival and mortality rates in costly environments such as the intensive care unit (ICU) is of great importance for the assessment of new treatments, resource consumption control, and improvement of quality control. This study aimed to determine the ability to predict mortality and discharge rate of patients using the FOUR score in the pediatric ICU (PICU) of Ali Ibn Abitalib Hospital, Zahedan and compare the results with those of Glasgow Coma Scale (GCS). This prospective study was conducted on 200 patients admitted to the PICU. Convenience purposive sampling was used. Research data was collected using the Full Outline of Unresponsiveness (FOUR) score and GCS using questionnaires. Obtained data was analyzed with SPSS 16 using descriptive statistics and correlation analyses. Of the 200 children admitted to the PICU, 71.5% and 28.5% were discharged and died, respectively. The inter-rater reliability for the FOUR score was good to excellent (weighted κ: eye, 0.72; respiration, 0.82; brainstem, 0.74; motor, 0.78), In terms of mortality and discharge prediction, logistic regression analyses (FOUR score = OR: 0.129; 95% CI: 0.06–0.29; P<0.001; GCS=OR: 2.49; 95% CI: 1.44–4.32; P<0.001) showed that the FOUR score is a good predictor for in-hospital mortality. Results indicated that the FOUR score is more capable than GCS in predicting the mortality and discharge of patients admitted to the PICU.

It is believed that fingerprints and palm patterns may represent genetically determined congenital abnormalities in Cystic Fibrosis (CF). The main idea of this paper was to determine differences of fingerprints and palm patterns in CF and normal children. Forty-six CF children (27 males, 19 females) and 341 (113 males, 228 females) healthy individuals were recruited for this study. Fingerprint patterns, Total ridge count (TRC) of each finger, a-b ridge count, and atd angles of all participants were recorded. Asymmetry of the right and left hand for each value was determined and dissimilarity in fingerprint patterns between homologous fingers was compared using Chi-square analysis, Mann-Whitney U test and Fisher's exact test. There were significant differences in the mean TRC of the right digit IV (P=0.009), left digit III (P=0.02), left digit IV (P=0.03), and left digit V (P=0.03). Furthermore, we found significant differences in right atd angel (P=0.001), left atd angel (P=0.002), right a-b ridge (P=0.007) and left a-b ridge (P=0.001). In contrast, we found no significant differences in atd angle asymmetry, a-b ridge count asymmetry and pattern dissimilarity score between both groups (P>0.05). Dermatoglyphic characteristics could be used as a supplementary diagnostic method in CF children.

Mean platelet volume (MPV) is a determinant of inflammation. The aim of the present study was to investigate the MPV levels in children with rotavirus gastroenteritis and to evaluate the possible relationship between MPV and severity of gastroenteritis. Children diagnosed with acute rotavirus gastroenteritis and healthy controls were enrolled in this study. Patients were classified into three disease severity groups based on their Vesikari score (<7 mild, 7-10 moderate and >11 severe). Rotavirus was determined in fresh stool samples using ELISA test. Leukocyte and platelet counts, MPV and C-reactive protein (CRP) levels were assessed for all children. A total of 151 patients with rotavirus gastroenteritis (mean age 2.41± 0.14 years) and 80 healthy controls (mean age 2.63±0.22 years, P=0.129) were enrolled. MPV levels of children with rotavirus gastroenteritis were significantly lower than those of healthy peers (7.48±0.04 vs 7.79±0.07 fl, P=0.000). MPV levels were not significantly different among three gastroenteritis groups. Gastroenteritis score was positively correlated with leukocyte (r=0.670, P<0.01) and platelet count (r=0.159, P<0.05) and CRP level (r=0.256, P<0.01) in patients group. MPV was inversely correlated with platelet count. There was no significant correlation between MPV and gastroenteritis score. MPV levels were significantly lower in children with rotavirus gastroenteritis compared to controls. MPV can be used as a negative acute phase reactant in children with rotavirus gastroenteritis.

Extrahepatic biliary atresia (EHBA) is one of the main causes for neonatal cholestasis. It’s early diagnosis could help infants with early surgery and increase the survival. In this study we evaluated the diagnostic accuracy of procalcitonin and apolipoprotein E (Apo-E) levels in infants with and without EHBA. In this prospective study, 18 infants with EHBA and 15 infants with other causes of cholestasis were included. Blood samples were taken from each patient and different markers including procalcitonin and Apo-E levels were measured. ROC analysis was used to define sensitivity, specificity, positive and negative predictive value (PPV and NPV) for procalcitonin and Apo-E. Gamma-glutamyl transpeptidase (GGT) and alkaline phosphatase were significantly higher in EHBA than other causes of neonatal cholestasis. There was significantly positive correlation between Apo-E and SGOT (r=0.37, p=0.03), SGPT (r=0.38, p=0.02) and GGT (r=0.38, p=0.02), and inverse correlation between procalcitonin and GGT (r=-0.45, p=0.01). Area under curve (AUC) for procalcitonin was 0.69 (p=0.053) with cut-point of 0.735 ng/ml. The sensitivity, specificity, PPV and NPV was 67%, 61%, 69% and 59%, respectively. AUC for Apo-E was 0.68 (p=0.06) for cut-point of 61.25 ng/ml with sensitivity, specificity, PPV and NPV of 67%, 67%, 71% and 67%, respectively. Both PCT and Apo-E have relatively good accuracy in diagnosing EHBA cases; we could not rely on these markers for diagnosis of EHBA, however, combinations of these biomarkers with other markers and imaging tests could improve their accuracy and may help for rapid and accurate diagnosis of EHBA.

To evaluate the efficacy and safety of metoclopramide in the treatment of feeding intolerance in preterm neonates less than 36 weeks of gestational age. A randomized, controlled, masked cross-over study. A block of 4 randomizations was used. The “drug group” received intravenous metoclopramide before feeding and placebo group received placebo at the same time. The time to full enteral feeding and suspected adverse effects of metoclopramide, length of hospital stay or incidence of NEC or septicemia were the main outcome measures. Mean (standard deviation) of weight and Apgar score among metoclopramide and placebo groups were 1638.3±321 gr, 8.9±1.4 and 1593.3±318.8 gr, 8.8±1.3 respectively. Times to full feeding were significantly shorter in the metoclopramide group than in the control group (12.9±5.6 vs 17.0±6.3; P<0.0001) and also the numbers of withheld feedings were significantly lower (P<0.0001). According to the regression analysis, lower weight and placebo group were significantly related to increasing of lavage frequency, number of vomits, start time of feeding, number of feeding cessations and decreased feeding completion time (P<0.0001).No adverse effects of this treatment modality were observed in the two groups. Intravenous metoclopramide may be considered as an attempt in facilitating and treatment of feeding intolerance in preterm neonates.

To determine various perinatal factors leading to birth asphyxia among term newborns in a tertiary care hospital. In a cross sectional study in, a total of 196 asphyxiated cases were selected through consecutive non-probability sampling technique from neonatal intensive care unit (NICU) of a tertiary care Military Hospital in Pakistan from 1st December 2012 to 1st December 2013. Data obtained was analyzed using SPSS version 15.0. Descriptive statistics were used to calculate means, standard deviations and frequencies. Stratification with respect to maternal age, gestational age, newborns weight, parity and gravidity was done and post stratification chi-square test was applied to find statistical significance. Out of 196 cases, 125 (64%) were males and 71 females (36%). Mean maternal age was 27.04+4.97 years and gestational age of babies was 39.86+1.24 weeks. Majority (57.14 %) of 112 mothers were 1-3 para and ≥4 parity was recorded in 84 (42.86%) cases. Majority (64.80%) of the 127 mothers were 1-3 gravida while 69 (35.20%) had ≥4 gravidity, mean of 3.45+0.87. Mode of delivery as a factor leading to birth asphyxia was found in 32.14% (n=63) cesarean section, 44.39% (n=87) spontaneous vertex delivery, and instrumental delivery in 23.47% (n= 46). Prolonged second stage of labor reported in 72% (n=141), 29.08% (n=57) had prolonged rupture of membranes, 7.65% (n=15) had meconium staining, 5.61% (n=11) had multiple births, 21.94% (n=43) had maternal fever, and 58.84% (n=113) had anemia at delivery. Birth asphyxia is a preventable problem and long term neurological sequelae almost untreatable. Timely identification of the perinatal risk factors and their prompt solution can prevent and reduce the neonatal morbidity and mortality from birth asphyxia. Early identification of high-risk cases with improved antenatal and perinatal care can further decrease such high mortality.

The nutrition of children in kindergartens has a great significance for proper growth and development of children. In order to save time and money, the control of macronutrients content is performed by calculations using food composition tables instead of performing a chemical analysis. We examined the macronutrients content of 240 whole day meals using food composition tables and performed chemical analysis of meals to determine adequacy and validity of food composition tables in calculation of macronutrient contents in kindergarten meals. We established no correlation (P>0.05) between the value of proteins, fats and carbohydrates. Significant difference was established between the average content of proteins (t=2.51; P<0.01), and carbohydrates (t=3.19; P<0.01), but not with the content of fats in the meals (t=1.25; P>0.05) (food composition tables vs chemical analysis). Until we establish new food composition tables, chemical analysis remains the only valid method for assessment of macronutrients content and energy value of a meal in kindergarten.

Extrapulmonary complications of Mycoplasma pneumoniae (M. pneumoniae) infection include encephalitis, optic neuritis, acute psychosis, stroke, cranial nerve palsies, aseptic meningitis and also it may be implicated in immune mediated neurological diseases such as acute demyelinating encephalomyelitis, Guillain-Barre syndrome and transverse myelitis. We present five cases with acute neurological diseases after M. pneumoniae infection. The clinical presentations were characterized by encephalitis in 2 patients, Gullain-Barre syndrome in 2 patients, transverse myelitis in 1 patient. M. pneumoniae infection was detected in serum by serological method. Only two patients had respiratory symptoms preceding M. pneumoniae infection. Brain MRI revealed hyperintensities on corpus striatum and mesencephalon in one patient with encephalitis, the other had front parietal coalescent periventricular white matter lesions on T2 images. The patient with transverse myelitis had cervical, dorsal and lumbar scattered hyperintense lesions on T2 images. Two patients were treated with high dose steroid, the other two patients received treatment with intravenous immune globuline. M. pneumoniae may reveal different neurologic complications with different radiologic findings.

Inherited deficiency of phosophomannomutase (PMM2) causes a human glycosylation disorder known as Congenital Disorder of Glycosylation Ia. Herein, we describe a case of congenital disorder of glycosylation Ia, presented with recurrent pericardial effusion and unusual findings of inverted nipples, fat pads, reduced deep-tendon reflexes and multisystem involvement. Congenital Disorder of Glycosylation Ia should be considered in children with developmental delay, those with multi-system disease involving neurologic, gastrointestinal, ophthalmologic, cardiac or endocrine systems. On the other hand, severe cardiac involvement may also be a feature of Congenital Disorder of Glycosylation Ia and diagnosed patients should also be evaluated in this respect.

The term SAPHO (synovitis, acne,pustulosis, hyperostosis, and osteitis) syndrome was proposed in anattempt to unify the group of clinical conditions previouslydescribed as separate entities with hyperostosis of anterior chestwall, synovitis, and multifocal aseptic osteomyelitis, observed inassociation with dermatologic conditions such as palmoplantarpustulosis, severe acne and hidradenitis suppurativa. Controversyremains regarding whether chronic recurrent multifocal osteomyelitis(CRMO) and SAPHO occur on a spectrum or are separate entitiesconsidering that they affect the patients with diferent age groupsand they involve different bones. Normally CRMO is expected to beseen during childhood whereas SAPHO in adults. However, there havealso been recent reports of CRMO occurring in adults and SAPHOsyndrome occurring in children. Herein we report a 12-year-old girldiagnosed with SAPHO syndrome who was treated successfully during herfollow up at our clinic.

Fournier’s gangrene (FG) is a rare, necrotising fasciitis of the genitals and perineum caused by mixture of aerobic and anaerobic microorganisms. The complications of this synergistic infection are multiple organ failure and death.Cases Presentation: We report seven cases of FG in pediatric age group. They have been treated with urgent aggressive surgical debridement and intensive care support, although antibiotic therapy, correct postoperative wound management, and complete excision of all necrotic tissue was performed successfully, Three patients died during treatment. Colostomy was performed in 5 patients, and one case underwent Urinary diversion. Although FG isnt common in children and it may be fetal but early diagnosis is very important. The decision to early wide surgical debridement and early fecal diversion is most part of treatment and preserving of life.