Serum level of lactate dehydrogenase, homocystein, hemoglobin and platelet in preeclampsia

Objectives: Pre-eclampsia affects approximately 5-8% of pregnant women. The aim of this study was to compare the serum level of Lactate dehydrogenase (LDH), Homocystein, Hemoglubin and platelet in pregnant women diagnosed as pre-eclampsia and a normal group in Gorgan city, Northeastern Iran from 2007-2008. Methodology: In this case control study, 50 cases of pre-eclampsia were compared with the control group women hospitalized in Dezyani hospital. Pre-eclampsia criteria were: Blood pressure more than or equal to 140/90 mm hg and Proteinuria greater or equal to 300 mg/ 24 hours urine sample in the third trimester. Hemoglobin, platelet, LDH and hemocystein were measured. Data were analyzed by the mean of SPSS-14 program & Chi-2 or t-student were used. Results: The difference of BMI and family incomes was significant between two groups (P-value0.01). Hemocystein level was more than normal range in five patients with pre-eclampsia (P-value<0.001). Conclusions: In this study, hemocystein level was significantly higher in pre-eclampsia patients but LDH, hemoglobin and platelet level had no significant difference

The Report of Three Rare Cases of the Niemann-pick Disease in Birjand, South Khorasan, Eastern Iran

How to Cite This Article: Noroozi Asl S, Vakili R, Ghaemi N, Eshraghi P. The Report of Three Rare Cases of the Niemann-pick Disease in Birjand, South Khorasan, Eastern Iran. Iran J Child Neurol. Summer 2017; 11(3):53-56. AbstractNiemann–Pick disease type C (NP-C) is a rare neurovisceral and irreversible disease leading to premature death and disabling neurological signs. This autosomal recessive disease with incidence rate of 1:120000 is caused by mutations in either the NPC1 or the NPC2 gene, which leads to accumulation of cholesterol in body tissues especially brain and progressive neurological symptoms. NP-C is characterized by nonspecific visceral, neurological and psychiatric manifestations in infants. The neurological involvement is typically proceeded by systemic signs (cholestatic jaundice in the neonatal period or isolated spleno-or hepatosplenomegaly in infancy or childhood).Early detection of NPC is important so that therapy with miglustat can delay onset of neurological symptoms and prolong survival. We describe here three infants from Birjand, South Khorasan, eastern Iran in 2016 with splenomegaly and different neurological signs that diagnosis was confirmed by genetic study. In all of them, NPC-509 was pathologically increased. They also had an unreported homozygous mutation (c. 1415T&gt;C, p.Leu472Pro) in exon 9 of the NPC1 gene. We found unreported homozygous mutation in NPC gene.Knowing this mutation is significant to our people. Genotype-phenotype correlations for this specific mutation needs to be further studied. References1. Mengel E, Klunemann H, Lourenco C, and et al. Niemann-Pick disease type C symptomatology: an expert-based clinical description. Orphanet J Rare Dis 2013;8:166.2. Vanier MT: Niemann-Pick disease type C. Orphanet J Rare Dis 2010;5:16.3. Di Rocco M1, Dardis A, Madeo A, Barone R, Fiumara A. Early miglustat therapy in infantile Niemann-Pick disease type C. Pediatr Neurol 2012;47(1):40-3.4. Karimzadeh P, Tonekaboni SH, Ashrafi MR, et al. Effects of Miglustat on Stabilization of Neurological Disorder in Niemann–Pick Disease Type C Iranian Pediatric Case Series. J Child Neurol 2013;28(12):1599-606.5. Wijburg F, Sedel F, Pineda M et al. Development of a Suspicion Index to aid diagnosis of Niemann-Pick disease type C. Neurology 2012;78(20):1560-7.6. Wraith JE, Imrie J. New therapies in the management of Niemann-Pick type C disease: clinical utility of miglustat. Ther Clin Risk Manag 2009;5:877-87.7. Patterson M, Hendriksz Ch, Walterfang M, et al. Recommendations for the diagnosis and management of Niemann–Pick disease type C: An update. Mol Genet Metab 2012;106(3):330-44.8. Margaret M, Destinck DJ. Lipidosis(Lysosomal storage disease). Nelson Textbook of Pediatrics. 19th ed.Philadelphia: WB Saunders Company. 2011:488-9.9. Patterson M.C, Mengel E, Wijburg F, et al. Disease and patient characteristics in NP-C patients: findings from an international disease registry. Orphanet J Rare Dis 2013;8:12

Evaluating of psychiatric behavior in obese children and adolescents

Abstract IntroductionObesity is a medical condition  that it may have a harmful effect on health, leading to increased illness and reduced life expectancy. This study is aimed to evaluate the relationship of psychiatry disorders in overweight and obese children and adolescents.MethodsIn this was case-control study, one hundred and sixty child and Adolescent were recruited. The sampling method of this study was non-probability and biased. Study instruments were SDQ, CDI, STAI, Peds QL. All questionnaires were self-administrating that was completed by subjects or their parents. Differences between groups were examined using t-test and chi-square tests as appropriate. ResultsThe results our study showed no significant different in scores of anxiety between two groups. But showed significant different in scores of depression, quality of life, and strength and difficult between two groups.  Also there was no significant difference in gender effect on anxiety and Depression. However, in Quality of life test showed that emotional symptoms were more in girl than boys. In contrast, the conduct problems were more in boys than girls. Anxiety and Depression was more in adolescents than childrenConcussion Our study showed obesity has a negative effect on the anxiety, depression, and self-esteem of children and adolescents. It can be suggested that obesity might be a more important risk factor for depression, anxiety, and other psychiatry disorders. This study also emphasizes the importance of prevention of obesity

Epidemiology of Shigella-Associated diarrhea in Gorgan, north of Iran

Objective : Shigella is an important etiological agent for diarrhea and especially dysentery. Shigellosis is an intestinal infection that is a major public health problem in many developing countries. The aim of this study was to evaluate the prevalence of Shigella and its various species in diarrheal samples in Gorgan located in the north of Iran. Materials and Methods: Between January-December 2005, the epidemiology of Shigella- associated diarrhea was studied among 634 patients in Gorgan. The diarrheal samples accompanied with a questionnaire, which contained the demographic and main symptoms of the patients, were transported to the laboratory and inoculated in different culture media. Colonies suspected to be of Shigella were detected using differential biochemical tests and subsequently, the serotype of Shigella was defined using antisera. Results : Shigella was isolated from 56/634 diarrheal samples) (8.8%) of which S. sonnei was the predominant species (55%). Occurrence of Schigella was highest in the 2-5 years′ age group (70.9%) and highest in summer (73.2%) with the most frequent clinical manifestation being abdominal pain (67.8%). The prevalence of Shigella in males and females was 8 and 9.8% respectively, but this difference was not statistically significant. Conclusion: It has been shown that Shigella sonnei is the most common Shigella serogroup among 2-5 year-old children in Gorgan. It is therefore suggested that hygienic training be given to childcare attendants and the children themselves

Hypoparathyroidism as the first manifestation of Kearns-sayre syndrome. A case report

Hypoparathyroidism as the First Manifestation of Kearns-Sayre Syndrome: A Case Report How to Cite This Article: Ashrafzadeh F, Ghaemi N, Akhondian J, Beiraghi Toosi M, Elmi S. Hypoparathyroidism as the First Manifestation of Kearns-Sayre Syndrome: A Case Report. Iran J Child Neurol. 2013 Autumn;7(4):53-57.  ObjectiveKearns-Sayre syndrome is a mitochondrial myopathy, which was first described by Tomas Kearn in 1958. Diagnostic symptoms include retinitis pigmentosa, chronic and progressive external ophthalmoplegia plus one or more of following factors: heart conduction system disorders, cerebellar ataxia, or cerebrospinal fluid (CSF) protein content above 100 mg/dL. The nature of this uncommon disease is yet to be clarified. In this paper, we report a case of Kearns-Sayre syndrome. According to the previous records, the first manifestation of Kearns-Sayre syndrome as hypoparathyroidism is uncommon and in this article, we report a case with this problem.ReferencesAshizawa T, Subramony SH. What is Kearns-Sayer syndrome after all? Arch Neurol 2001;58(7):1053-4.Barragan-Campos HM, Vallee JN, Lo D, Barrera-Ramirez CF, Argote-Greene M, Sanchez-Guerrero J, et al. Brain magnetic resonance imaging findings in patients with mitochondrial cytopathies. Arch Neurol 2005;62(5):737-42.Amemiya S, Hamamoto M, Goto Y, Komaki H, Nishino I, Nonaka I, et al. Psychosis and progressive dementia: presenting features of a mitochondriopathy. Neurology 2000;55(4):600-1.Katsanos KH, Pappas CJ, Patsouras D, Michalis LK, Kitsios G, Elisaf M, et al. Alarming atrioventricular block and mitral valve prolapse in the Kearns-Sayer syndrome. Int J Cardiol 2002;83(2):179-81.Tiranti V, Viscomi C, Hildebrandt T, Di Meo I, Mineri R, Tiveron C, et al. Loss of ETHE1, a mitochondrial dioxygenase, causes fatal sulfide toxicity in ethylmalonic encephalopathy. Nat Med 2009;15(2):200–5.Chinnery PF, DiMauro S, Shanske S, Schon EA, Zeviani M, Mariotti C, et al. Risk of developing a mitochondrial DNA deletion disorder. Lancet 2004;364(9434):592–6.Bosbach S, Kornblum C, Schröder R, Wagner M. Executive and visuospatial deficits in patients with chronic progressive external ophthalmoplegia and Kearns-Sayer syndrome. Brain 2003;126(Pt 5):1231-40.Berenberg RA, Pellock JM, DiMauro S, Schotland DL, Bonilla E, Eastwood A, et al. Lumping or splitting? “Ophthalmoplegia-plus” or Kearns-Sayer syndrome? Ann Neurol 1977;1(1):37-54.Welzing L, von Kleist-Retzow JC, Kribs A, Eifinger F, Huenseler C, Sreeram N. Rapid development of life threatening complete atrioventricular block in Kearns-Sayer syndrome. Eur J Pediatr 2009;168(6):757-9.Berio A, Piazzi A. Kearns-Sayer syndrome with GH deficiency. Pediatr Med Chir 2000;22:43-6.Schmiedel J, Jackson S, Schäfer J, Reichmann H. Mitochondrial cytopathies. J Neurol 2003;250(3):267-77.Chu BC, Terae S, Takahashi C, Kikuchi Y, Miyasaka K, Abe S, et al. MRI of the brain in the Kearns-Sayer syndrome: report of four cases and a review. Neuroradiology 1999;41(10):759-64.Altunbaşak S, Bingöl G, Ozbarlas N, Akçören Z, Hergüner O. Kearns-Sayer syndrome. A case report. Turk J Pediatr 1998;40(2):255-9.Chawla S, Coku J, Forbes T, Kannan S. Kearns-Sayer syndrome presenting as complete heart block. Pediatr Cardiol 2008;29(3):659-62.Gregoratos G, Abrams J, Epstein AE, Freedman RA, Hayes DL, Hlatky MA, et al. ACC/AHA/NASPE 2002 guideline update for implantation of cardiac pacemakers and antiarrhythmia devices: summary article: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/ AHA/NASPE Committee to Update the 1998 Pacemaker Guidelines). Circulation 2002;106(16):2145-61.Basu AP, Posner E, McFarland R, Turnbull DM. Kearnsayre syndrome. Medscape reference. Feb 4, 2010.http://emedicine.medscape.com/article/950897

Maternal serum copper concentration in premature rupture of membrane: A case-control study

Copper is an integrated parts of metal-protein required far varieties of oxide-reductive metabolic pathways in human. Copper deficiency is considered as risk factors in some pregnancies. Premature rupture of membrane is a pregnancy complication with major adverse effects and is believed maternal Copper deficiency can also be considered as interventional factors. This study was done to evaluate if there is a correlation between maternal serum Copper concentration and premature rupture of membrane in pregnancy. In this case-control study 60 pregnant women with Premature Rupture of Membrane (PROM) were selected as case group including term and pre term the control group consist of 60 pregnant women with normal delivery of term and pre term states. Both group were matched for maternal and pregnancy age. In case and control group the pregnancy at term and pre-term were grouped independently as well. In general the maternal mean serum Copper concentration were 192.4±78.2 and 201.08±82.06 in case and control groups, respectively but this differences statistically was not significant. Data in this study revealed that the absolute value of maternal serum Copper concentration of term or pre term in case groups was slightly lower than related controls. Drop in maternal Copper concentration in some disturbed pregnancies such as premature rupture of membrane is previously demonstrated and based on our data the absolute Copper serum concentration of women with premature rupture of membrane was also slightly lower compared to healthy pregnancy but it was not statistically significant. © Asian Network for Scientific Information

Peripheral Neuropathy in Children and Adolescents with Insulin-dependent Diabetes Mellitus

Introduction: Type 1 diabetes mellitus(T1DM) is a chronic immune-mediated disease. Diabetic peripheral neuropathy (DPN) is an important micro vascular complication of T1DM. One of the most important risk factors for the development of DPN is poor glycemic control. The aim of this study was to evaluate the prevalence of DPN among T1DM patients and to determine the association between DPN and glycated hemoglobin(HbA1c) level.Methods: The subjects were recruited prospectively upon initial evaluation at a tertiary hospital. Patients with T1DM were selected based on the inclusion criteria (i.e., age of 6≤years and absence of other co-morbidities). DPN was assessed through electro diagnostic studies and neurological examinations, while diabetes control was evaluated by measuring the HbA1c level.Results: In total, 50 patients with T1DM were enrolled in this study. The mean diabetes duration of patients was 8.38±3.79 years (mean age16.68±6.68 years). The mean HbA1c level was 8.6±2.1% in patients without DPN and 10.5±3 in those with DPN (P=0.016). Overall, 24% of the subjects were presented with DPN according to nerve conduction velocity(NCV) findings. A positive correlation was found between NCV and clinical symptoms with signs (P&lt;0.001, r=0.45 and P&lt;0.001, r=0.644, respectively). Sensitivity and specificity of neurological examination for DPN diagnosis were 91.7% and 63.2%, respectively. Our findings revealed that, poor diabetes control is associated with DPN. Also, HbA1c level was used as an index for glycemic control over the past six months.Conclusion: It seems that rigid blood glucose control and periodic neurological examinations were the best strategies to prevent DPN. Introduction: Type 1 diabetes mellitus(T1DM) is a chronic immune-mediated disease. Diabetic peripheral neuropathy (DPN) is an important micro vascular complication of T1DM. One of the most important risk factors for the development of DPN is poor glycemic control. The aim of this study was to evaluate the prevalence of DPN among T1DM patients and to determine the association between DPN and glycated hemoglobin(HbA1c) level.Methods: The subjects were recruited prospectively upon initial evaluation at a tertiary hospital. Patients with T1DM were selected based on the inclusion criteria (i.e., age of 6≤years and absence of other co-morbidities). DPN was assessed through electro diagnostic studies and neurological examinations, while diabetes control was evaluated by measuring the HbA1c level.Results: In total, 50 patients with T1DM were enrolled in this study. The mean diabetes duration of patients was 8.38±3.79 years (mean age16.68±6.68 years). The mean HbA1c level was 8.6±2.1% in patients without DPN and 10.5±3 in those with DPN (P=0.016). Overall, 24% of the subjects were presented with DPN according to nerve conduction velocity(NCV) findings. A positive correlation was found between NCV and clinical symptoms with signs (P&lt;0.001, r=0.45 and P&lt;0.001, r=0.644, respectively). Sensitivity and specificity of neurological examination for DPN diagnosis were 91.7% and 63.2%, respectively. Our findings revealed that, poor diabetes control is associated with DPN. Also, HbA1c level was used as an index for glycemic control over the past six months.Conclusion: It seems that rigid blood glucose control and periodic neurological examinations were the best strategies to prevent DPN.

Chemical composition and antibacterial activity from essential oil of Artemisia sieberi Besser subsp. Sieberi in North of Iran

The chemical composition and antibacterial effect of Artemisia siberi essential oil were studied in this research. The composition of essential oil from aerial parts was analyzed by GC/MS and its antibacterial effect were determined by disc diffusion method. Artemisia ketone (48.5%), 1, 8-cineole (19.7%), selin-11-en-4-a-ol (4.6%) and lavandulon (2.8%) were the major constituents of this herbal medicine. Inhibitory zone against Pseudomonas aeroginosa, Staphylococcus aureus and Escherichia coli around discs contained 100 mg mL-1 of Artemisia siberi essential oil were 18, 13 and 12 mm, respectively. Further studies for the determination of and Pseudomonas infection in animal model are suggested. © 2007 Asian Network for Scientific Information

Aphthous ulcer and the effective factors on it's incidence among the students of Golestan Medical Sciences University in the north of Iran

The aim of this study was to determine the prevalence and probable predisposing factors of Aphthous ulcer among the medical students. This project was a cross-sectional and descriptive study, which was carried out on 485 students of Golestan University of medical sciences in the north of Iran. Data was collected by filling the questionnaire. The gathered informations entered into SPSS software and were analyzed by Chi square method. The mean age of the students was 21.8 years. 36.9% (179 persons) of subjects had positive history and 8.9% (43 persons) of them had present Aphthous ulcer at the time of interview. 70.6% of students with positive personal history of Aphthous ulcer had positive family history of Aphthous ulcer too. Internal labial mucosa was the commonest site of the lesion among the subjects with present Aphthous ulcer. We found that gender, positive family history, the field of study, the form of diet and stress had a major correlation with the incidence of Aphthous ulcer. © 2007 Asian Network for Scientific Information

Precocious Puberty: An Unusual Presentation of Hypothyroidism

Hypothyroidism is usually associated with delayed pubertal development but in rare occasions precocious puberty may ensue which is seen in cases of prolonged and untreated hypothyroidism. This is also called the Van Wyk Grumbach syndrome. Here we present 4 cases of precocious puberty due to hypothyroidism