Management of NPC

How to Cite This Article: Jafari N. Management of NPC. Iran J Child Neurol. 2015 Autumn;9:4(Suppl.1): 24. Pls see pdf.

Neurometabolic Disorders, Clinical and Neuroimaging Finding

How to Cite This Article: Jafari N. Neurometabolic Disorders, Clinical and Neuroimaging Finding. Iran J Child Neurol. Autumn 2014;8;4(Suppl.1):23.pls see pdf

Gaucher Disease: New Expanded Classification Emphasizing Neurological Features

 Gaucher disease (GD) is a rare inherited metabolic disorder and themost common lysosomal storage disorder, caused by a deficiency inglucocerebrosidase enzyme activity. It has been classified accordingto the neurological manifestations into three types: type 1, withoutneuropathic findings, type 2 with acute infantile neuropathic signsand type 3 or chronic neuropathic form. However, report of newvariants has led to the expansion of phenotype as a clinical phenotypeof GD considered as a continuum of phenotypes. Therefore, it seemsthat a new classification is needed to cover new forms of the disease. Keywords: Gaucher disease; Neurological manifestations; Phenotypes

Design and Application of a Novel Dehumidifying System for a Fluidized Bed Dryer for the Drying of Canola Seeds

A novel fluidized bed dryer equipped with a dehumidifying system was designed and constructed. The dehumidifying system was based on refrigeration cycle and composed of an evaporator, a compressor, a condenser an expansion valve and a damper. The drying experiments were carried out in three temperature (35, 45 and 55°C) and three relative humidity (30, 50 and 70%) levels. The results showed that the relative humidity of drying air has a significant effect on drying rate. The drying rates decreased as relative humidity increased at a constant temperature, and increased as temperature increased at a constant relative humidity so dehumidifying can accelerate drying and decrease drying times

Homocystinuria: Diagnosis and Neuroimaging Findings - of Iranian Pediatric patients

How to Cite This Article: Karimzadeh P, Jafari N, Alai MR, Jabbehdari S, Ahmad Abadi F, NejadBiglari H. Homocystinuria: Diagnosis and Neuroimaging Findings - of Iranian Pediatric Patients. Iran J Child Neurol. 2015 Winter;9(1):94-98.AbstractObjectiveHomocystinuria is a neurometabolic diseases characterized by symptoms include Neurodevelopmental delay, lens dislocation, long limbs and thrombosis.Materials & MethodsThe patients who were diagnosed as homocystinuria marfaniod habits, seizure in the Neurology Department of Mofid Children’s Hospital in Tehran, Iran between 2004 and 2014 were included in our study. The disorder was confirmed by clinical andneuroimaging findings along withneurometabolic and genetic assessment fromreference laboratory in Germany. We assessed age, gender, past medical history, developmental status, clinical manifestations, and neuroimagingfindings of 20 patients with homocystinuria.ResultsA total of 75% of patients were offspring from consanguineous marriages. A total of 95% of patients had a history of developmental delay and 40% had developmental regression. A total of 75% had seizures from these 45% showed refractory seizures. Seizures among 13 patients werecontrolled with suitable homocystinuria treatment. The patients with homocystinuriawere followed for approximately 10 years and the follow-ups showed that the patients with an early diagnosis and treatment had more favorable clinical responses for growth index,controlled refractory seizures, neurodevelopmental status, and neuroimaging findings. Neuroimaging findings include brain atrophy and/or white matter involvement.ConclusionAccording to the results of this study, we suggest that early assessment and detectionplayan important role in the prevention of disease progression and clinical signs. Homocystinuria in patients with a positive family history, developmental delays, or regression, refractory, or recurrent seizures should take precedence over other causes.ReferencesBrustolin S1, Giugliani R, Félix TM. Genetics of homocystine metabolism and associated disorders.Braz J Med Biol Res. 2010 Jan; 43(1):1-7.Iacobazzi V, Infantino V, Castegna A, AndriaG. Hyperhomocysteinuria: Related genetic diseases and congenital defects, abnormal DNA methylation and newborn screening issues. Mol Genet Metab. 2014 September - October; 113(1-2):27-33.SelhubJ.Homocystinemetabolism.Annu Rev Nutr. 1999;19:217-46.Ali Z, Troncoso JC, Fowler DR.Recurrent cerebral venous thrombosis associated with heterozygote methylenetetrahydrofolate reductase C677T mutation and sickle cell trait without Homocysteinuria: An autopsy case report and review of literature.ForensicSci Int. 2014 Sep;242:e52-5.RozenR.Genetic modulation of Homocysteinuria. SeminThrombHemost. 2000;26(3):255-61.Palma Reis R, Sales Luis. Homocisteinemia e Doença Vascular – O Nascer de um Novo Factor de Risco. Rev Port Cardiol 1999; 18:507-14McCully KS. Homocystine theory of arteriosclerosis: development and current status. Atherosclerosis Rev 1983;11:157-246.Malinow MR. Homocystinuria: a common and easily reversible risk factor for occlusive atherosclerosis. Circulation 1990;81:2004-6.Boushey CJ, Beresford SAA, Omenn GS, Motulsky AG. A quantitive assessment of plasma homocystine as a factor for vascular disease: probable benefits of increasing folic acid intakes. JAMA 1995;472:1049-57.Carson NAJ, Neill DW. Metabolic abnormalities detectedin a survey of mentally backward individuals in NorthernIreland. Arch Dis Child 1962;37:505-13.Gerritsen T, Waisman HA. Homocystinuria, an error inthe metabolism of methionine. Pediatrics 1964;33:413-20.Mallory T. Case records of the Massachusetts GeneralHospital.Case 19471. Marked cerebral symptoms followinga limp of three months duration. N Engl J Med 1933;209:1063-6.Shih VE, Efron ML. Pyridoxine-unresponsive homocystinuria. Final diagnosis of MGH case 19471. N Engl Med1970;283:1206-8.Kang SS, Wong PWK, Malinow MR. Hyperhomocyst(e) inemia as a risk factor for occlusive vascular disease.Ann Rev Nutr 1992;12:279-98.Mudd SH, Skovby F, Levey HL, et al. The natural history of homocystinuria due to cystathionine B-synthase deficiency. Am J Hum Genet 1985;37:1-31.Fonseca N1, Vidal N, Santos J, Brito AP, Bernardino L, Silvestre I, InêsL.Hyperhomocysteinuria–case report.Rev Port Cardiol. 2003 Feb;22(2):223-30.Mudd SH, Levy HL. Disorders of transsulfuration. InStanbury JB, Wingarden JB, Fredrickson DS, Goldstein JL,Bronw MS (eds.). The Metabolic Basis of Inherited Disease.New York, McGraw-Hill 1983;522-559.Sachdeva V1, Mittal V, Pathengay A, Kekunnaya R, Gupta A, Rao BV.Isolated abducens nerve palsy with hyperhomocysteinuria: Association and outcomes.Indian J Ophthalmol. 2013 Oct;61(10):598-600

Accidental Children Poisoning With Methadone: An Iranian Pediatric Sectional Study

How to Cite This Article: Jabbehdari S, Farnaghi F, Shariatmadari SF, Jafari J, Mehregan FF, Karimzadeh P. Accidental Children Poisoning With Methadone: An Iranian Pediatric Sectional Study. Iran J Child Neurol. 2013 Autumn;7(7): 32-34.ObjectiveToxic poisoning with methadone is common in children in Iran. Our study was carried out due to the changing pattern of methadone poisoning in recent years and increasing methadone toxicity. Materials & MethodsIn this descriptive-sectional study, all of the methadone poisoned children younger than 12 years who were admitted to the Loghman Hakim Hospital in 2012, were assessed. Clinical symptoms and signs, para-clinical findings, and treatment were evaluated. ResultsIn this study, 16 boys and 15 girls who had been poisoned by methadone were enrolled. The mean age of patients was 55 months. All patients had been poisoned randomly or due to parent’s mistakes. The mean time of symptoms onset after methadone consumption was 1 hour and 30 Min, indicating a relatively long time after onset of symptoms.Clinical findings were drowsiness (75%), miotic pupil (68 %), vomiting (61%), rapid shallow breathing (57%) and apnea (40%). In paraclinical tests, respiratory acidosis (69%) and leukocytosis (55.2%) were seen. The most important finding was increase in distance of QT in ECG (23.8%). The mean time of treatment with naloxone infusion was 51 hours. Three percent of patients had a return of symptoms after discontinuation of methadone. In patients with apnea, a longer course of treatment was required, and this difference was significant. Also, 17% of patients with apnea had aspiration pneumonia, which was statistically significant. ConclusionWe suggest long time treatment with naloxone and considering the probability of return of symptoms after discontinuation of methadone.ReferencesGoldfrank L, Flomenbaum N, Lewin N. Goldfrank’s Toxicologic Emergencies. 7th ed. McGraw–Hill 2002; p. 590-607.Schelble DT. Phosgene and phosphine. In: Haddad LM, Shannon MW, Winchester J, eds. Clinical Management of Poisoning and Drug Overdose. 3rd ed. Philadelphia: WB Saunders; 2007. p. 640-7.Jennifer C, Gibson A. Accidental methadone poisoning in children: A call for Canadian research action. Child Abuse Negl;2010;34(8):553-4.Binchy JM, Molyneux E, Manning J. Accidental ingestion of methadone by children in Merseyside. BMJ 1994;308(6940:1335-6.Zamani N, Sanaei-Zadeh H, Mostafazadeh B. Hallmarks of opium poisoning in infants and toddlers. Trop Doct 2010;40(4):220-2.LoVecchio F, Pizon A, Riley B, Sami A, D’Incognito C. Onset of symptoms after methadone overdose. Am J Emerg Med 2007;25(1):57-9.Thanavaro KL, Thanavaro JL. Methadone-induced torsades de pointes: a twist of fate. Heart Lung 2011;40(5):448-53.Gaalen FA, Compier EA, Fogteloo AJ. Sudden hearing loss after a methadone overdose. Eur Arch Otorhinolaryngol 2009;266(5):773-4.Lynch RE, Hack RA. Methadone-induced rigid-chest syndrome after substantial overdose.Pediatrics. 2010; 126(1):232-4.Sidlo J, Valuch J, Ocko P, Bauerová J. Fatal methadone intoxication in a 11-month-old male infant. Soud Lek 2009;54(2):23-5.

The clinical features and diagnosis of Metachromatic leukodystrophy: A case series of Iranian Pediatric patients

How to Cite This Article: Jabbehdari S, Rahimian E, Jafari N, Sanii S, Khayatzadeh Kakhki S, Nejad Biglari H. The Clinical Features and Diagnosis of Metachromatic Leukodystrophy: A Case Series of Iranian Pediatric Patients. Iran J Child Neurol. Summer 2015;9(3):57-61.AbstractObjectiveMetachromatic leukodystrophy disorder (MLD) is one of the rare neurometabolicdiseases caused due to lack of saposin B and arylsulfatase A enzyme deficiency.Materials & MethodsEighteen patients diagnosed as metachromatic leukodystrophy in the NeurologyDepartment of Mofid Children’s Hospital in Tehran, Iran between 2010 and2014 were included in our study. The disorder was confirmed by clinical,EMG-NCV, arylsulfatase A enzyme checking and neuroimaging findings alongwith neurometabolic and genetic assessment from reference laboratory in Iran.We assessed age, gender, past medical history, developmental status, clinicalmanifestations, and neuroimaging findings of 18 patients with metachromaticleukodystrophy.ResultsFrom 18 patients, 80% were offspring from consanguineous marriages. A familyhistory of metachromatic leukodystrophy disease was positive for four patients.Twelve patients had late infantile form of this disorder and six patients had juvenile form. A history of tonic type seizure was positive in 20% of the patients and tonic spasm was confirmed with clinical information. Electromyographgraphy (EMG) in 96% of patients was abnormal with demyelinating sensorimotor neuropathy pattern. MRI in all patients showed the leukodystrophic pattern as arcuate fibers sparing and subcortical rim in white matter and periventricular involvement. Our diagnosis was confirmed by EMG-NCV findings with sensorimotor neuropathy pattern and the assessment of arylsulfatase A enzyme function. ConclusionMLD is an inheritance metabolic disorder, which was confirmed by theassessment of arylsulfatase A enzyme function, peripheral blood leukocyte thatassessed in a referral laboratory in Iran

Utility of Seizure Pattern and Related Clinical Features in the Diagnosis of Neurometabolic Disorders

AbstractObjectives: The current study aimed at identifying the role of seizure types and related features in differentiation between neurometabolic disorders and other causes of seizure.Materials & Methods: The current cross-sectional study was conducted at two referral children hospitals in Tehran, Iran from 2011 to 2018. The study population included 120 patients presenting with seizure due to neurometabolic disorders and 120 cases due to other causes. The types of seizure and related clinical findings were assessed in both groups.Result: There was a significant difference in the frequency of seizure types in two groups. Tonic and myoclonic seizures as well as infantile spasm were observed more commonly in the patients with neurometabolic disorders, while atonic, partial and generalized tonic-clonic seizures , and lack of seizure were more common in the control group. In addition, frequency of refractory seizure, age at onset of seizure, and pattern of involvement in brain imaging were helpful for seizure differentiation.Conclusion: The pattern of seizure and related findings vary in patients with metabolic disorders and are helpful for seizure differentiation. Thus, these factors can contribute to early diagnosis and treatment.

Neurometabolic Diagnosis in Children who referred as Neurodevelopmental Delay (A Practical Criteria, in Iranian Pediatric Patients)

How to Cite This Article: Karimzadeh P, Jafari N, Nejad Biglari H, Jabbehdari J, Khayat Zadeh S, Ahmad Abadi F, Lotfi A. Neurometabolic Diagnosis in Children who referred as Neurodevelopmental Delay (A Practical Criteria, in Iranian Pediatric Patients). Iran J Child Neurol. Summer 2016; 10(3):73-81. ObjectiveWe aimed to investigate the clinical and para clinical manifestations of neuro metabolic disorders, in patients who presented by neuro developmental delay in their neuro developmental milestones.Materials & MethodsThe patients diagnosed as neuro developmental delay and regression with or without seizure at the Neurology Department of Mofid Children Hospital in Tehran, Iran between 2004 and 2014 were included in our study. These patients diagnosed as neuro developmental delay by pediatric neurologists in view of diagnostic /screening neuro developmental assessment tests. The patients who completed our inclusion criteria as neuro metabolic disorders were evaluated in terms of metabolic and genetic study in referral lab.ResultsOverall, 213 patients with neurometabolic disorders were diagnosed. 54.3% of patients were male. The average age of patients was 41 +-46.1 months. 71.4% of parent’s patients had consanguinity of marriages. Eighty seven percent of patients had developmental delay (or/and) regression. 55.5% of them had different type of seizures. Overall, 213 patients with 34 different neurometabolic disorders were diagnosed and classified in the 7 sub classes, consisting of:1- organic acidemia and aminoacidopathy (122 patients), 2-storage disease (37 patients) 3- eukodystrophy (27 patients), other classes consisted: lipid oxidation disorders, urea cycle disorders, progressive myoclonic epilepsy; and peroxizomal disorders (27 patients).ConclusionIn patients with developmental delay or regression, with or without seizure, abnormal neurologic exam along with positive family history of similar disorder or relative parents, abnormal brain imaging with specific patterns, neurometabolic disorders should be considered as one of the important treatable diseases. ReferencesBrismar J. CT and MRI of the brain in inherited neurometabolic disorders. J Child Neurol 1992 Apr;7 Suppl:S112-31.Barkovich AJ. An approach to MRI of metabolic disorders in children. J Neuroradiol 2007; 34(2):75-88.Barkovich AJ. A magnetic resonance approach to metabolic disorders in childhood. Rev Neurol 2006 10;43 Suppl 1:S5-16.Zimmerman RA. Neuroimaging of inherited metabolic disorders producing seizures. Brain Dev 2011;33(9):734-44.Cakir B, Teksam M, Kosehan D, Akin K, Koktener A.Inborn errors of metabolism presenting in childhood. J Neuroimaging 2011;21(2):e117-33.Burton BK. Inborn errors of metabolism in infancy: a guide to diagnosis. Pediatrics 1998;102(6):E69.Iman G. Mahmoud M, Marwa M, Miral R, Marian G, Nevin W, Ameera E. Clinical, Neuroimaging, and Genetic Characteristics of Megalencephalic Leukoencephalopathy With Subcortical Cysts in Egyptian Patients. Pediatr Neurol 2014;140e-148.Sarar M, Ebtessam M, Melegy B, Iman T, Amany H, Khaled K. Neurometabolic Disorders-Related Early Childhood Epilepsy: A Single-Center Experience in Saudi Arabia. Pediatr Neurol 2015; xx, 1e9.Talebian A, Jahangiri M, Rabiee M, Masoudi N, Akbari H, Sadat Z. The Etiology and Clinical Evaluations of Neonatal Seizures in Kashan, IRAN. Iran J Child Neurol 2015;9(2):29-35.Vigevano F, Bartuli A. Infantile epileptic syndromes and metabolic etiologies. J Child Neurol 2002;17:3S9e13.Karimzadeh P. Approach to Neurometabolic Diseases from a Pediatric Neurological Point of View. Iran J Child Neurol 2015;9(1): 1-16.Hee Eun S, Houn Hahn S. Metabolic evaluation of children with global developmental delay. Korean J Pediatr 2015;58(4):117-122.Kroll R, Pagel M, Roman S, Barkovich A, D’Agostino A, Neuwelt E.White matter changes associated with feline GM2 gangliosidosis (Sandhoff disease): correlation of MR findings with pathologic and ultrastructural abnormalities. Am J Neuroradiol 1995;16(6):1219-26.Karimzadeh P, Jafari N, Nejad Biglari H, Jabbeh Dari S, Ahmad Abadi F, Alaee M,. GM2-Gangliosidosis (Sandhoff and Tay Sachs disease): Diagnosis and Neuroimaging Findings (An Iranian Pediatric Case Series). Iran J Child Neurol 2014;8(3):55-60.Wang Q, Ding Y, Liu Y, Li X, Wu T, Song J, et al.(Clinical and laboratory studies on 28 patients with glutaric aciduria type 1). Zhonghua Er Ke Za Zhi 2014 Jun;52(6):415-9.Karimzadeh P, Pirzadeh Z, Ahmadabadi F, Jafari N, Jabbehdari S, Nemati H,. Glutaric aciduria type 1: diagnosis and neuroimaging findings of this neurometabolic disorder in an Iranian pediatric case series. Int J Develop Dis 2014; 60(3): 1–6Weller S, Rosewich H, Gärtner J. Cerebral MRI as a valuable diagnostic tool in Zellweger spectrum patients. J Inherit Metab Dis 2008;31(2):270-80.Groeschel S, Kehrer C, Engel C, I Dali C, Bley A, Steinfeld R, et al. Metachromatic leukodystrophy; natural course of cerebral MRI changes in relation to clinical course. J Inherit Metab Dis 2011;34(5):1095-102.Klee D, Thimm E, Wittsack HJ, Schubert D, Primke R, Pentang G,et al. Structural white matter changes in adolescents and young adults with maple syrup urine disease. J Inherit Metab Dis 2013;36(6):945-53.Karimzadeh P. Approach to neurometabolic diseases from a pediatric neurological point of view. Iran J Child Neurol 2015;9(1):1-16.Leuzzi V1, Tosetti M, Montanaro D, Carducci C, Artiola C, Carducci C,et al. The pathogenesis of the white matter abnormalities in phenylketonuria. A multimodal 3.0 tesla MRI and magnetic resonance spectroscopy (1H MRS) study. J Inherit Metab Dis 2007;30(2):209-16. Epub 2007 Jan 23.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. Iran J Child Neurol 2014 ;8(2):53-6.Abdelhalim AN, Alberico RA, Barczykowski AL, Duffner PK. Patterns of magnetic resonance imaging abnormalities in symptomatic patients with Krabbe disease correspond to phenotype. Pediatr Neurol 2014;50(2):127-34.Santosh Rai PV, Suresh BV, Bhat IG, Sekhar M, Chakraborti S.Childhood adrenoleukodystrophy - Classic and variant - Review of clinical manifestations and magnetic resonance imaging. J Pediatr Neurosci 2013;8(3):192-7.George U, Varte N, Rathore S, Jain V, Goyal S. “Split thalamus”: Internal medullary involvement in Wilson’s disease. Neurol India 2010;58:680Oder W, Prayer L, Grimm G, Spatt J, Ferenci P, Kollegger H, et al. Wilson’s disease: evidence of subgroups derived from clinical findings and brain lesions. Neurology 1993;43:120-4.Bickel H.(Brain atrophy and disorders of the amino acid metabolism). Monatsschr Kinderheilkd 1967;115(4):254-8.Karimzadeh P, Jafari N, Alai M, Jabbehdari S, Nejad Biglari H. Homocystinuria: Diagnosis and Neuroimaging Findings of Iranian Pediatric patients. Iran J Child Neurol 2015;9(1):94-8.Sreenivasan P, Purushothaman KK.Radiological clue to diagnosis of Canavan disease. Indian J Pediatr 2013;80(1):75-7.Karimzadeh P, Jafari N, Nejad Biglari H, Rahimian E, Ahmadabadi F, Nemati H, Nasehi MM, Ghofrani M, Mollamohammadi M. The Clinical Features and Diagnosis of Canavan’s Disease: A Case Series of Iranian Patients. Iran J Child Neurol 2014 ;8(4):66-71.Nguyen HV, Ishak GE. Canavan disease - unusual imaging features in a child with mild clinical presentation. Pediatr Radiol 2014 Aug 9.Rogers T, al-Rayess M, O’Shea P, Ambler MW.Dysplasia of the corpus callosum in identical twins with nonketotic hyperglycinemia. Pediatr Pathol 1991;11(6):897-902.Johnson JA, Le KL, Palacios E.Propionic acidemia: case report and review of neurologic sequelae. Pediatr Neurol 2009;40(4):317-20.Karimzadeh P, Jafari N, Ahmad Abadi F, Jabbedari S, Taghdiri MM, Alaee MR, Ghofrani M, Tonekaboni SH, Nejad Biglari H. Propionic acidemia: diagnosis and neuroimaging findings of this neurometabolic disorder. Iran J Child Neurol 2014;8(1):58-61.35.Desai S, Ganesan K, Hegde A.Biotinidase deficiency: a reversible metabolic encephalopathy. Neuroimaging and MR spectroscopic findings in a series of four patients. Pediatr Radiol 2008;38(8):848-56.Karimzadeh P, Ahmadabadi F, Jafari N, Jabbehdari S, Alaee MR, Ghofrani M, Taghdiri MM, Tonekaboni SH. Biotinidase deficiency: a reversible neurometabolic disorder (an Iranian pediatric case series). Iran J Child Neurol 2013;7(4):47-52.Brismar J, Ozand PT.CT and MR of the brain in disorders of the propionate and methylmalonate metabolism. Am J Neuroradiol 1994;15(8):1459-73.Karimzadeh P, Jafari N, Ahmad Abadi F, Jabbedari S, Taghdiri MM, Nemati H, Saket S, Shariatmadari SF, Alaee MR, Ghofrani M, Tonekaboni SH. Methylmalonic acidemia: diagnosis and neuroimaging findings of this neurometabolic disorder (an Iranian pediatric case series). Iran J Child Neurol 2013;7(3):63-6.

HLA-B*1502 in Iranian Children with Anticonvulsant Drugs-Induced Skin Reactions

How to Cite This Article: Tonekaboni SH, Jafari N, Mansouri M, Jabbehdari S, Eftekhari R, Chavoshzadeh Z, Abdollah Gorji F, Mesdaghi M. HLA-B*1502 in Iranian Children with Anticonvulsant Drugs-Induced Skin Reactions. Iran J Child Neurol. Spring 2017; 11(2):26-30. AbstractObjectiveAnticonvulsant drugs can cause various forms of skin drug reactions, ranging from exanthema to severe blistering reactions. An association between HLA-B*1502 allele and severe skin reactions have been reported.Materials & Methods Fifteen patients with severe skin reactions following treatment with anticonvulsant drugs (Carbamazepine, lamotrigine, phenobarbital, primidone) and 15 controls (age-matched epileptic patients taking similar anticonvulsants without drug eruption) were included. They were referred to Mofid Children’s Hospital in Tehran, Iran, between Jan 2012 to Jan 2014. Genomic DNA was extracted from peripheral blood of all patients and HLA- B*1502 genotype was detected by real-time PCR.Results None of the patients was positive for HLA- B*1502, but two patients in control group had positive HLA- B*1502.Conclusion The HLA- B*1502 is not correlated with severe anticonvulsant drugs -induced skin reactions in Iranian children. References 1.Roujeau JC. Clinical heterogeneity of drug hypersensitivity. Toxicology 2005; 209: 123 –9.2.McCormack M, Alfirevic A, Bourgeois S, Farrell JJ, Kasperavičiūtė D, Carrington M, et al. HLA-A*3101 and carbamazepine-induced hypersensitivity reactions in Europeans. N Engl J Med 2011; 364(12):1134-43.3.Daly AK, Donaldson PT, Bhatnagar P, Shen Y, Pe’er I, Floratos A, et al. HLA-B*5701 genotype is a major determinant of drug-induced liver injury due to flucloxacillin. Nat Genetic 2009; 41:816–9.4.Amstutz U, Ross CJ, Castro-Pastrana LI, Rieder MJ, Shear NH, Hayden MR, Carleton BC. CPNDS Consortium; HLA-A 31:01 and HLA-B 15:02 as genetic markers for carbamazepine hypersensitivity in children. Clin Pharmacol Ther 2013; 94(1):142-9.5.Man CB, Kwan P, Baum L, Yu E, Lau KM, Cheng AS, Ng MH. Association between HLA-B*1502 allele and antiepileptic drug-induced cutaneous reactions in Han Chinese. Epilepsia 2007; 48(5):1015-8.6.Zeng T, Long YS, Min FL, Liao WP, Shi YW. Association of HLA-B*1502 allele with lamotrigine-induced Stevens– Johnson syndrome and toxic epidermal necrolysis in Han Chinese subjects: a meta-analysis. Int J Dermatol 2015; 54(4):488-93.7.Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol 1993; 129(1):92-6.8. Hung SI, Chung WH, Jee SH, Chen WC, Chang YT, Lee WR, et al. Genetic susceptibility to carbamazepine-induced cutaneous adverse drug reactions. Pharmacogenet Genomics 2006; 16(4):297-306.9. Wang Q, Zhou JQ, Zhou LM, Chen ZY, Fang ZY, Chen SD, et al. Association between HLA-B*1502 allele and carbamazepine-induced severe cutaneous adverse reactions in Han people of southern China mainlan. Seizure 2011; 20 (6):446-8.10. Li LJ, Hu FY, Wu XT, An DM, Yan B, Zhou D. Predictive markers for carbamazepine and lamotrigine-induced maculopapular exanthema in Han Chinese. Epilepsy Res 2013; 106 (1-2):296-300.11. Kim SH, Lee KW, Song WJ, Kim SH, Jee YK, Lee SM, et al; Adverse Drug Reaction Research Group in Korea. Carbamazepine-induced severe cutaneous adverse reactions and HLA genotypes in Koreans. Epilepsy Res 2011; 97 (1-2):190-7.12. Criado PR, Criado RFJ, Avancini JM, Santi CG. Drug reaction with eosinophilia and systemic symptoms (DRESS)/drug-induced hypersensitivity syndrome (DIHS): a review of current concepts. An Bras Dermatol 2012; 87(3):435–49.13. Chung WH, Hung SI, Hong HS, Hsih MS, Yang LC, Ho HC, et al. Medical genetics: a marker for Stevens– Johnson syndrome. Nature 2004; 428: 486.14. Man CB, Kwan P, Baum L, Yu E, Lau KM, Cheng AS, Ng MH. Association between HLA-B*1502 allele and antiepileptic drug induced cutaneous reactions in Han Chinese. Epilepsia 2007; 48: 1015–8.15. Locharernkul C, Loplumlert J, Limotai C, Korkij W, Desudchit T, Tongkobpetch S, et al. Carbamazepine and phenytoin induced Stevens–Johnson syndrome is associated with HLA-B*1502 allele in Thai population. Epilepsia 2008; 49:2087–91.16. Kaniwa N, Saito Y, Aihara M, Matsunaga K, Tohkin M, Kurose K, et al. HLA-B locus in Japanese patients with anti-epileptics and allopurinol-related Stevens– Johnson syndrome and toxic epidermal necrolysis. Pharmacogenomics 2008; 9: 1617–22.17. Alfirevic A, Jorgensen AL, Williamson PR, Chadwick DW, Park BK, Pirmohamed M. HLA-B locus in Caucasian patients with carbamazepine hypersensitivity. Pharmacogenomics 2006; 7: 813–8.18. Tangamornsuksan W, Chaiyakunapruk N, Somkrua R, Lohitnavy M, Tassaneeyakul W. Relationship between the HLA-B*1502 allele and carbamazepine-induced Stevens- Johnson syndrome and toxic epidermal necrolysis: a systematic review and meta-analysis. JAMA Dermatol 2013; 149(9):1025-32