Comparing the efficacy of 7%, 5% hypertonic saline and 0.9% normal saline in acute bronchiolitis: a randomized controlled trial

Background: The study aimed to investigate the effect of nebulized 5% and 7% Hypertonic Saline (HS) versus Normal Saline (NS) in acute viral bronchiolitis.Methods: This is a prospective, randomized controlled trial study that has been done on 90 infants presenting with acute bronchiolitis. All infants divided randomly in three equal size and matched groups (n=30). Subjects received 4ml nebulized 5% and 7% HS along with 0.15mg/kg Adrenaline or 4 ml 0.9%NS every 6 hours from enrolment until hospital discharge. For all patients, clinical symptoms such as sputum, wheezing, retraction rate, heart rate, fever, crackle, irritability, week nutrition and breathing were recorded in baseline and length of wheezing, cough, and crackle, length of stay (LOS), fever and sputum based on days in the end of study. Collected data analyzed by statistical methods in SPSS.19.Results: At baseline, study groups were similar in demographic and clinical characteristics. The mean age of all patients was 5.5±3.6 months, and 59 (65.6%) were male. The length of cough and crackle, and the length of LOS in 5% and 7% HS groups was significantly lower than NS group.Conclusions: Among infants admitted to the hospital with viral bronchiolitis, treatment with nebulized 5% HS and 7% HS had significant effect on decreasing clinical symptoms length and LOS when compared with NS

Autistic Feature as a Presentation of Inborn Errors of Metabolism

Autism spectrum disorder (ASD) is a category of neurodevelopmental disorders characterized by social and communication impairment and restricted or repetitive behaviors. The pathogenesis of ASD is not well understood and it’s proved that genetic is strongly associated with ASD in 5 to 25% of cases. Inborn errors of metabolism(IEMs), defined by a vast array of disorders that are caused by specific enzyme deficiencies or transport protein defects, is as frequent as in 1 in 800 births. IEMs can manifest several psychiatric or behavioral manifestations such as self-injuriesincreased activity and aggression, personality changes, paranoia, depression, catatonia, and psychosis. IEMs underlie autistic symptoms in less than 5% of cases. The literature on the association between ASD and respiratory chain abnormalities is growing, including complex III/IV deficiency and MELAS (mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes) syndrome, as well as glucose-6-phosphate dehydrogenase deficiency. Google Scholar, Pubmed, and SCOPUS databases were searched using a combination of the following keywords: “autism spectrum disorder”, “autism spectrum”, “autistic feature” and “inborn error of metabolism”, “ IEM”, “congenital error of metabolism”. Initially, 655 articles were found and our expert and methodologist altogether selected 187 articles based on the titles, relevance, and text language.After reading full texts, 37 studies were selected for review. We think it’s best to consider IEMs in children with syndromic ASD and/or if there is a strong familial history of autism or parental consanguineous marriage

The relationship between serum vitamin D level and asthma severity in asthmatic children (aged 1-15 years) in Ardabil, 2012-13

Background:Vitamin D plays an important role in many immune and allergic diseases, and its deficiency is more in areas with low solar radiation. Asthma is a common disease in children of Ardabil city. The present study was designed to evaluate vitamin D levels in asthmatic children in Ardabil.Methods:A total of 100 asthmatic children (40% boys and 60% girls) with a mean age of 5.7 ± 3.46 years (1-15 years) and who visited the Bou-Ali pediatric hospital were included. Vitamin D levels, disease course, IgE levels, and eosinophil counts were evaluated. A group of healthy children was also included to compare vitamin D levels between asthmatic and non-asthmatic children. All data were analyzed by statistical methods in SPSS v.16.Results: The vitamin D levels in asthmatic children were lower than those in the healthy group. Furthermore, the increase in the vitamin D level was significantly associated with lower history of hospitalization (P = 0.02), better response to bronchodilator treatment (P = 0.01), lower IgE level (P = 0.02), and decrease in the mean age of children (P = 0.04). However, no significant association between vitamin D levels and sex (P = 0.08), history of anti-inflammatory drug use (P = 0.78), and eosinophil count (P = 0.08) was observed in asthmatic children.Conclusion:Results showed that vitamin D deficiency was more common in asthmatic than in non-asthmatic children and the level of vitamin D was directly associated with the response of the children to treatment.

Antioxidant Levels in Cord Blood of Term Neonates and Its Association with Birth Weight

How to Cite This Article: Mirzarahimi M, Ahadi A, Bohlooli SH, Namakikhalajan E, Barak M. Antioxidant Levels in Cord Blood of Term Neonates and Its Association with Birth Weight. Iran J Child Neurol. Winter 2016; 10(1):31-34. AbstractObjectiveDue to excessive production of free radicals and antioxidants evolved mechanisms against oxidative stress, infants are very vulnerable. As there was a significant relation between antioxidant levels and birth weight, we aimed verify this relationship.Materials & MethodsIn this descriptive analytical study we evaluated the antioxidant status of 40 healthy term newborns (gestation age 38-42 wk) with weight >2500 g (AGA) and 40 healthy term newborns (gestation age 38-42 wk) with LBW babies (weight < 2500 g) (SGA) in Ardabil Buali Hospital, Ardabil, northwest Iran in 2014. About 15 Ml of cord blood was collected after the second stage of labor.The levels of vitamin A, E, and C, catalase, glutathione peroxidase (GPX), bilirubin and serum uric acid were measured by standard methods. Informed consent was obtained from newborn mothers and study protocol was approved by university Ethics Committee. Data were analyzed using SPSS.19.ResultsThe mean levels of bilirubin, vitamin C, E, catalase and GPX in AGA group were significantly higher than SGA group but the mean of serum uric acid in SGA group was more than AGA. In addition, the mean of vitamin A was similar in two groups.There was a significant relation between antioxidant levels and birth weight in term newborns.ConclusionIn line with other studies the amounts of antioxidant levels except serum uric acid in AGA group was significantly more than SGA group

Considering lactate dehydrogenase (LDH) concentration in nasal-wash (NW) as a marker in evaluating the outcome of patients with bronchiolitis

Background: Estimation of bronchiolitis severity in infants is still an important issue and there are no standard methods to help physicians for better evaluation and management of clinical status of these patients. The aim of this study was to investigate the role LDH concentration in NW as a biomarker in evaluation the outcome of patients suffering bronchiolitis in Bu Ali Hospital, Ardabil.Methods: 100 children with bronchiolitis aged below 2 years entered the study. Nasal wash sample was extracted from all patients using 2 ml of normal saline. Samples were sent to laboratory to measure LDH level. Data were analyzed by statistical methods in SPSS.16.Results: The mean age of patients was 6.9±3.7 months and 57% of them were male. 42% of patients had mild bronchiolitis and 58% of them suffered from severe bronchiolitis. The LDH level of nasal wash fluid was neither related with gender nor with age. But it was significantly lower in patients who required oxygen therapy and had fever compared with those who did not require oxygen therapy and without fever. Moreover, LDH level showed a significant negative association with hospital stay (r= -0.570, p<0.001) and bronchiolitis severity (r= -0.440, p<0.001) in a way that its concentration was significantly lower in patients with hospital stay longer than 24 hours compared with hospital stay shorter than 24 hours, and in patients with severe bronchiolitis compared with mild bronchiolitis.Conclusions: According to results of this study, LDH measurement in nasal wash fluid can be used as a biochemical marker to evaluate clinical outcomes of bronchiolitis in children younger than 24 months

Study on MRI Changes in Phenylketonuria in Patients Referred to Mofid Hospital/Iran

How to Cite This Article: Karimzadeh P, Ahmadabadi F, Jafari N, Shariatmadari F, Nemati H, Ahadi A, Karimi Dardashti S, Mirzarahimi M, Dastborhan Z, Zare Noghabi J. Study on MRI Changes in Phenylketonuria in Patients Referred to Mofid Hospital. Iran J Child Neurol. 2014 Spring 8(2):53-56.ObjectivePhenylketonuria is one of the most common metabolic disorders and the first known cause of mental retardation in pediatrics. As Screening for phenylketonuria (PKU) is not a routine neurometabolic screening test for neonates in Iran, many PKU cases may be diagnosed after developing the clinical symptoms. One of the findings of PKU is myelination disorders, which is seen as hypersignal regions in T2-weighted (T2W) and FLAIR sequences of brain MRI. The aim of our study was to assess MRI changes in PKU patients referred to Mofid Children’s Hospital, 2010-2011.Materials &amp; MethodsWe studied all PKU cases referred to our clinic as a referral neurometabolic center in Iran for brain MRI and assessed the phenylalanine level at the time of Imaging. The mean phenylalanine level (in one year), clinical manifestations,and MRI pattern based on Thompson scoring, were evaluated.ResultsThe mean age of our study group was 155±99 months and the mean diagnosis age was 37±27.85 months. There were 15 patients with positive and 15 with negative family history. The mean phenylalanine level at the time of imaging was 9.75±6.28 and the mean 1 year phenylalanine level was 10.28±4.82. Seventy percent of our patients had MRI involvement, in whom 20% showed atrophic changes, in addition to white matter involvement. Based on modified Thompson scoring, the score for our study group was 4.84.The maximum involvement in MRI was in occipital region, followed by parietal, frontal, and temporal zones. There was not any correlation between MRI score and patients’ age. But we found significant relationship between MRI score andthe age of regimen cessation. No correlation was seen between phenylalanine level (at the time of Imaging) and MRI score. But there was a relationship between mean 1 year phenylalanine level and MRI score.ConclusionAccording to the results of this study, brain MRI and white matter involvement can be used for evaluation of long-term control of phenylalanine level in PKU cases. References1. Blau Nenad. Phenylketonuria and BH4 Deficiencies. London: UNI-MED; 2010.2. Buck PS. The child who never grew. Woodbine House; 1992.3. Rezvani I, Melvin JJ. Defects in metabolism of amino acids. In: Kliegman RM, Stanton BF, St. Geme J, Behrman RE, editors. Nelson Textbook of Pediatrics. 19th ed. USA: Elsevier; 2011. p. 418-22.4. Menkes J, Wilcox WR. Inherited Metabolic Diseases of nervous system. In: Menkes JH, editor Child neurology. 7th ed. Philadelphia: Lippincott Williams&amp;Wilkins; 2006. p. 34-36.5. Aicardi J. Diseases of the nervous system in childhood. London: Mac Keith press; 2009.6. Enns GM, Cowan TM, Klein O, Packman S. Aminoacidemias and organic acidemias. In: Swaiman KF. Swaimans Pediatric Neurology principle and practice. 5th ed. China: Saunders; 2012. p. 330-7.7. Barkovich J. Toxic and Metabolic Brain disorders. In: Barkovich J, editor Pediatric Neuroimaging. 4th ed. USA: Lippincott William&amp;Wilkins;2005. p. 88-92.8. Van der knaap MS. Phenylketonuria. In: Van der knaap MS, Valk J, editor. Magnetic resonance of Myelination and Myelin Disorders. 3rd ed. Germany: Springer; 2005. p. 285-90.9. Manara R, Burlina AP, Citton V, Ermani M, Vespignani F, Carollo C, et al. Brain MRI diffusion-Weighted imaging in patients with classical phenylketonuria: Neuroradiology (2009)51:803-12.10. Möller HE, Weglage J, Bick U, Wiedermann D, Feldmann R, Ullrich K. Brain imaging and proton Magnetic Resonance Spectroscopy in Patients with Phenylketonuria Pediatrics 2003;112(6 Pt 2):1580-3.11. Phillips MD, McGraw P, Lowe MJ, Mathews VP, Hainline BE. Diffusion-Weighted Imaging of White Matter Abnormalities in Patients with Phenylketonuria. AJNR Am J Neuroradiol 2001 Sep;22(8):1583-6.12. Cleary MA, Walter JH, Wraith JE, Jenkins JP, Alani SM, Tyler K, et al. Magnetic resonance Imaging of the Brain in Phenyl ketonuria. Lancet 1994;344(8915):87-90

Comparing the efficacy of 7%, 5% hypertonic saline and 0.9% normal saline in acute bronchiolitis: a randomized controlled trial

Background: The study aimed to investigate the effect of nebulized 5% and 7% Hypertonic Saline (HS) versus Normal Saline (NS) in acute viral bronchiolitis.Methods: This is a prospective, randomized controlled trial study that has been done on 90 infants presenting with acute bronchiolitis. All infants divided randomly in three equal size and matched groups (n=30). Subjects received 4ml nebulized 5% and 7% HS along with 0.15mg/kg Adrenaline or 4 ml 0.9%NS every 6 hours from enrolment until hospital discharge. For all patients, clinical symptoms such as sputum, wheezing, retraction rate, heart rate, fever, crackle, irritability, week nutrition and breathing were recorded in baseline and length of wheezing, cough, and crackle, length of stay (LOS), fever and sputum based on days in the end of study. Collected data analyzed by statistical methods in SPSS.19.Results: At baseline, study groups were similar in demographic and clinical characteristics. The mean age of all patients was 5.5±3.6 months, and 59 (65.6%) were male. The length of cough and crackle, and the length of LOS in 5% and 7% HS groups was significantly lower than NS group.Conclusions: Among infants admitted to the hospital with viral bronchiolitis, treatment with nebulized 5% HS and 7% HS had significant effect on decreasing clinical symptoms length and LOS when compared with NS