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 Association Between Hyponatremia and Reflux-Related Renal Injury in Acute Pyelonephritis

Introduction: The kidney regulates sodium balance and is the principal site of sodium excretion. Sodium is unique among electrolytes because water balance, not sodium balance, usually determines its concentration. Although water balance is usually regulated by osmolality, volume depletion stimulates thirst, renal protection of water and ADH secretion. Volume reduction has priority over osmolality; volume depletion stimulates ADH secretion, even if a patient has hyponatremia. The aim of this study was to consider scar nephropathy in children with UTI and hyponatremia and compare it with children without hyponatremia.Material and Methods: 200 children with pyelonephritis were included in this case–control study as case and control groups, respectively. Subjects were selected from children referred to the pediatric clinic of our hospital in Arak, Iran. Case group included children with hyponatremia and UTI (with VUR) and control group included children with UTI (With VUR) and normal serum sodium. Data was analyzed using SPSS ver.18Results: Among 200 (100%) children in both groups, 5 children (5%) had normal sodium and reflux nephropathy and 23 children had hyponatremia and reflux nephropathy.Conclusions: Hyponatremia in children with reflux nephropathy was significantly more common than children without reflux nephropathy. The observed correlation between reflux-related injury and hyponatremia necessitates evaluation of electrolytes in children with pyelonephritis.Keywords: Pyelonephritis; Child; Hyponatremia; Vesico-Ureteral Reflux

Mean Platelet Volume as a Predictive Marker for Poor Prognosis of Acute Renal Failure in children

Introduction: Acute renal failure (ARF) is a clinical syndrome in which a sudden deterioration in renal function results in the inability of the kidneys to maintain fluid and electrolyte homeostasis. A classification system has been proposed to standardize the definition of acute kidney injury in adults. These criteria of risk, injury, failure, loss and end-stage renal disease were given the acronym of RIFLE. Our goal was to study the mean platelet volume (MPV) as a prognostic predictor of ARF in children. Mean platelet volume (MPV) is a machine-calculated measurement of the average size of platelets in blood and typically included in blood tests as part of CBC (Complete Blood Count). Since the average platelet size is larger when the body is producing increased numbers of platelets, MPV can be used to make inferences about platelet production in bone marrow or platelet destruction problems.Material and Methods: The records of 200 patients with ARF were investigated prospectively. Complete blood count including MPV, erythrocyte sedimentation rate, serum C-reactive protein and electrolytes of patients were measured and compared.Results: MPV values were low in loss (p=0.0012) and failure (p<0.005). The sensitivity and specificity of MPV for the diagnosis of loss and failure were higher than those of the other inflammation markers. MPV<8.2 fL was significantly associated with poor prognosis in renal functions.Conclusions: MPV is a fast and reliable measurement with considerable predictive value for prediction of prognosis in acute renal failure.Keywords: Acute Kidney Injury; Mean Platelet Volume; Blood Platelets; Acute Kidney Injury; Prognosis

Assessment of Blood Pressure in Primary Monosymptomatic Nocturnal Enuresis

Introduction: Enuresis is defined as the repeated voiding of urine into bed at least twice a week for at least 3 consecutive months in a child who is at least 5 years of age. Primary enuresis occurs in children who have never been consistently dry through the night.  Monosymptomatic enuresis has no associated daytime symptoms. Increased nocturnal urine production in primary nocturnal enuretic patients could possibly be associated with autonomic nervous system dysfunction. The aim of this study was to investigate autonomic nervous system function in enuretic children.Materials and Methods: In this study, children with monosymptomatic primary nocturnal enuresis (MPNE) and healthy children without MPNE were enrolled and their blood pressure was measured twice a day (in the morning and afternoon). Urinalysis, urine electrolyte levels, urinary culture, and urinary system ultrasound were performed in all the children. They were also requested to have a diary about daily fluid intake and the volume of daily urine.Results: The MPNE group comprised 100 children (M/F: 58/42) and the control group included 100 healthy children (M/F: 51/49). The mean age of the children was 8.1±2.3 and (8.9±2.53 years in MPNE and control groups, respectively. The mean diastolic blood pressure (DBP) during the nighttime and daytime did not differ between the groups (p-value>0.05); however, the mean systolic blood pressure (SBP) was significantly higher in the nighttime in the MPNE group (p-value<0.05) but did not differ between the groups during the daytime (p-value>0.05).Conclusions: Nighttime SBP was significantly higher in children with MPNE. These subtle abnormalities of the circadian blood pressure regulation may reflect autonomic nervous system dysfunction and contribute to the pathogenesis of MPNE.Keywords: Child; Enuresis; Blood pressure; Autonomic dysfunctio

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 & 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&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&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

Methylmalonic Acidemia: Diagnosis and Neuroimaging Findings of This Neurometabolic Disorder (An Iranian Pediatric Case Series)

How To Cite This Article: Karimzadeh P, Jafari N, Jabbehdari S, Taghdiri MM, Nemati H, Saket S, Alaee MR, Ghofrani M, Tonakebni SH. Methylmalonic Acidemia: Diagnosis and Neuroimaging Findings of This Neurometabolic Disorder (An Iranian Pediatric Case Series). Iran J Child Neurol. 2013 Summer; 7(3): 63-66. ObjectiveMethylmalonic acidemia is one of the inborn errors of metabolism resulting in the accumulation of acylcarnitine in blood and increased urinary methylmalonic acid excretion. This disorder can have symptoms, such as neurological and gastrointestinal manifestations, lethargy, and anorexia.Materials & MethodsThe patients who were diagnosed as methylmalonic acidemia in the Neurology Department of Mofid Children’s Hospital in Tehran, Iran, between 2002 and 2012 were included in our study. The disorder was confirmed by clinical findings, neuroimaging findings, and neurometabolic and geneticassessment in reference laboratory in Germany. We assessed the age, gender, past medical history, developmental status, clinical manifestations, and neuroimaging findings of 20 patients with methylmalonic acidemia.ResultsEighty percent of the patients were offspring of consanguineous marriages. Half of the patients had Failure to thrive (FTT) due to anorexia; 85% had history of developmental delay or regression, and 20% had refractory seizure, which all of them were controlled. The patients with methylmalonic acidemia were followed for approximately 5 years and the follow-up showedthat the patients with early diagnosis had a more favorable clinical response in growth index, refractory seizure, anorexia, and neurodevelopmental delay. Neuroimaging findings included brain atrophy, basal ganglia involvement (often in putamen), and periventricular leukomalacia.ConclusionAccording to the results of this study, we suggest that early assessment and diagnosis have an important role in the prevention of disease progression and clinical signs.References:1. Trinh BC, Melhem ER, Barker PB. Multi-slice proton MR spectroscopy and diffusion-weighted imaging in methylmalonic acidemia: report of two cases and review of the literature. AJNR Am J Neuroradiol 2001;22(5):831-3.2. Mahoney MJ, Bick D. Recent advances in the inherited methylmalonic acidemias. Acta Paediatr Scand1987;76(5):689-96.3. Radmanesh A, Zaman T, Ghanaati H, Molaei S, Robertson RL, ZamaniAA. Methylmalonic acidemia: brain imaging findings in 52 children and a review of the literature.Pediatr Radiol 2008 Oct;38(10):1054-61.4. Cantani A. [Methylmalonic acidemia: classification, diagnosis and therapy]. KlinPadiatr 1983;195(6):388-93.5. Avery ME, First LR, Pediatric Medicine, 2nd ed. Williams & Wilkins, Waverly Company; 1994. p.1075.6. Matsui SM, Mahoney MJ, Resenberg LE. The natural history of the inherited methylmalonic acidemias. N Engl J Med 1983;308(15):857-61.7. Holliday MA, Barrat M, Arner ED. Pediatric Nephrology, 3rd ed. William and Wilkins 1994; p. 890.8. Soda H, Yoshida I, Aramaki S, Kuriya N, Aoki K, Inokuchi T, et al. Renal handling of methylmalonic acid in a uraemic patient with vitamin B12 unresponsive methylmalonica cidaemia. J Inherit Metab Dis 1996;19(1):90-1.9. Imen M, Hanene B, Ichraf K, Aida R, Ilhem T, Naziha K, et al. Methylmalonic acidemia and hyperglycemia: an unusual association. Brain Dev 2012;34(2):113-4.10. Ma X, Zhang Y, Yang Y, Liu X, Yang Z, Bao X, et al. Epilepsy in children with methylmalonic acidemia: electroclinical features and prognosis. Brain Dev 2011;33(9):790-5.11. Brismar J, Ozand PT. CT and MR of the brain in disorders of the propionate and methylmalonatemetabolism. AJNR Am J Neuroradiol 1994;15(8):1459-73.12. Nicolaides P, Leonard J, Surtees R. Neurological outcome of methylmalonic acidaemia. Arch Dis Child 1998;78:508-12.

Evaluation of One Hundred Pediatric Muscle Biopsies During A 2-Year Period in Mofid Children And Toos Hospitals

 How to Cite This Article:Nilipor Y, Shariatmadari F, Abdollah gorji F, Rouzrokh M, Ghofrani M, Karimzadeh P, Taghdiri MM,  Delavarkasmaei H, Ahmadabadi F, Bakhshandeh bali MK, Nemati H, Saket S, Jafari N, Yaghini O, Tonekaboni SH.  Evaluation of One Hundred Pediatric Muscle Biopsies During A 2-Year Period in Mofid Children And Toos Hospitals. Iran J Child Neurol. 2013 Spring;7(2):17-21. ObjectiveMuscle biopsy is a very important diagnostic test in the investigation of a child with suspected neuromuscular disorder. The goal of this study was to review and evaluate pediatric muscle biopsies during a 2-year period with focus on histopathology diagnosis and correlations with other paraclinicstudies.Materials & MethodsWe investigated 100 muscle biopsies belonging to patients with clinical impression of neuromuscular disorder. These patients have been visited consecutively by pediatric neurologists during 2010 to 2012. Samples were investigated by standard enzyme histochemical and immunohistochemical techniques.ResultSixty-nine (69%) males and 39 (39%) females with a mean age of 5.7 years were evaluated. Major pathologic diagnoses were Muscular dystrophy (48 cases), Neurogenic atrophy (18 cases), nonspecific myopathic atrophy (12cases), congenital myopathy (6 cases), storage myopathies (4 cases) and in 6 cases there was no specific histochemical pathologic finding. EMG was abnormal in 79 cases. Degree of correlation between EMG and biopsy result was significant in children ≥ 2 years of age.ConclusionThis study confirms the high diagnostic yields of muscle biopsyespecially only if standard and new techniques such as enzyme study and immunohistochemistry are implemented. Also, we report 11 cases of Merosin negative congenital muscular dystrophy. This is the largest documented case series of Merosin deficient congenital muscular dystrophy reported from Iran. References1. Harvey B. Sarnat. Evaluation and Investigation. In:Kliegman. Stanton.Schor. Behrman.Nelson Text Book of Pediatrics.19th edition.Philadelphia: Elsevier,2011. P.2109-2112. 2. Harvey B. Sarnat and John H Menkes. Disease of TheMotore Unit. In: John H Menkes, Harvey B Sarnat, Bernard L Maria. Child Neurology. 7th edition.california: lippincott,2006.p.969-972.3. Marius Kuras Skram, Sasha Gulati, Erik Larrson. Muscle Biopsies In Children-An Evaluation Of Histopathology And Clinical Valueduring A 5-Year Period. Upsala J Med Sci 2009 March:114 (1);41-45.4. Owji M, Modaressi F. Diagnosis of Myopathies Using Histology. Histochemistry, Immunohistochemistry and Electron Microscopy 2010,12 (4):434 -440.5. Dua T, Das M, Kabra M. Spectrum of Floppy Children in Indian Scenario. Indian Pediatric J 2001, 38:1236-1243.6. Rabie M, Jossiphov J, Nevo Y. Electromyography accuracy compared to muscle biopsy in childhood. J Child Neurol 2007 jul; 22(7):803.8.