MLASA Syndrome: A Case Report

Mitochondrial myopathy, lactic acidosis, and sideroblastic anemia (MLASA) syndrome is a rare autosomal recessive disorder of oxidative phosphorylation and iron metabolism. The association between myopathy and sideroblastic anemia was initially reported in 1974. Here we report an 8.5 year old boy with normal cognitive function, suffering from chronic progressive weakness in his lower extremities, inability to walk and palor. Microcytic sideroblastic anemia, mild lactic acidosis and inflammatory myopathy (myositis) in muscle biopsy was detected and treated; the response to corticosteroid therapy and rehabilitation was excellent and the patient was ambulatory after four months

IV LIDOCAINE: A SECOND LINE DRUG THERAPY FOR PEDIATRIC CONVULSIVE STATUS EPILEPTICUS?

ObjectiveRefractory convulsive Status Epilepticus (SE) which does not respond to first line drugs (benzodiazepines, phenytoin and phenobarbital) heralds an emergency condition in pediatric neurology which can cause irreparable brain damage. There is no consensus on the choice of drug treatment for refractory generalized  convulsive status epilepticus in children. Lidocaine is a valuable forgotten antiepileptic agent with favorable properties which include prompt responses, less alteration of consciousness, and fewer adverse effects such as respiratory depression.Materials & MethodsIn a retrospective study conducted to investigate clinical efficacy and safety of intravenous lidocaine in treating refractory generalized convulsive SE of children, the medical records of 13 patients admitted to the Shaheed Sadoughi Hospital of Yazd between 2003 and 2005 and treated with intravenous lidocaine, were reviewed.Results13 patients, 7 girls and 6 boys (average age 3 years, SD=2.7years) were treated with lidocaine. Neurodevelopmental delay was seen in 38.5 %, and in 46.2% of them EEG and neuroimaging abnormalities were observed.Seizures ceased in eight patients (61/5 %), without any undesirable side effects. Two patients had to be intubated because of non-responsiveness to lidocaine, and other treatments were begun .Mean duration of ICU stay was 4.77 days, SD=3days.ConclusionIt is recommended that lidocaine be used as a second-line, anticonvulsive drug in the treatment of status epilepticus, especially when faced with unavailability of appropriate respiratory care and intubation equipment.Key words:Status Epilepticus, Refractory Status Epilepticus, Generalized Convulsive Status Epilepticus, Intravenous Lidocaine

Efficacy and Safety of Lamotrigine in Lennox - Gastaut Syndrome

ObjectiveThe Lennox-Gastaut syndrome (LGS), one of the most difficult epilepsy syndromes to treat, is characterized by a triad of intractable seizures of various types, a slow (< 2.5-hertz) spike-wave pattern in EEG and mental retardation. The aim of this study was to evaluate the efficacy and safety of lamotrigine as add-on therapy in intractable epilepsy of children with LGS.Materials & MethodsIn a quasi- experimental study, 40 children with LGS referred to the pediatric neurology clinic of Shaheed Sadoughi Hospital in Yazd, between August 2007 and to November 2008, were evaluated.ResultsTwenty-two boys and 18 girls with a mean age of 4.12 ±1.8 years were evaluated. At the end of three months of treatment with lamotrigine, 12 % were seizure free, 52% had> 50% reduction in seizure frequency and 12% had increase in seizures. Means of seizure frequency/per week, before and after treatment were 70 (range 1-180) and 18.6 (range 0-60) respectively, indicating effectiveness of the drug in seizure reduction (P value = 0.003). The drug was effective in 72 % of mixed type seizures, 40 % of generalized tonic-clonic and 33% of drop attack and tonic seizures. Transient side effects were seen in 12.5 % (drowsiness in 3 and ataxia in 2 children). No serious side effects were seen.ConclusionLamotrigine should be considered as an add-on therapy in management of intractable epilepsy in LGS

Introducing a simple and economical method to purify Giardia lamblia cysts

Direct microscopic examination of stool to diagnosis giardiasis (wet mount) has low diagnostic value, but immunologic methods (like IFA and especially ELISA) that are based on the determination of parasite antigens in fecal samples (antigen detection) have relatively high sensitivity and specialty. To prepare anti-Giardia lamblia antibodies needed to design diagnostic kits as well as parasite culture and other molecular studies, we require purification of the parasite cysts. In this study, we designed a rapid, simple and inexpensive method to purify parasite cysts from fecal samples of the patients suffering from giardiasis. Initially, fecal samples that the presence of G. lamblia in them was affirmed by direct microscopic observation of cysts were subjected to various purification methods like one- and twophase sucrose gradient isolation, percoll-sucrose gradient isolation, and a modified two-phase method run by 0.85 and 1.5 M sucrose. The first procedure contained some contents of bacteria and small particles of feces. In the second and third procedure, bacteria were almost removed and the cysts were intact but the suspension contained some extras and cellulose particles. The recovery rate for modified two-phase method was 1.5 × 104 cysts for each two grams of fecal sample. In this study, by using and comparing with the results of some other studies, we introduce and run a modified method that in fact is a mélange of them with some changes. So this method could be recommended as a fast, advantageous and simple method in purification of G. lamblia cysts.Key words: Giardia lamblia, cyst, purification

Adjunctive use of essential oils following scaling and root planing: a randomized clinical trial

Background: Hitherto no study has been published on the effect of the adjunctive administration of essential oils following scaling and root planing (SRP). This study describes the effect of a mouthrinse consisting of essential oils (Cymbopogon flexuosus, Thymus zygis and Rosmarinus officinalis) following SRP by clinical and microbiological variables in patients with generalized moderate chronic periodontitis. Methods: Forty-six patients (aged 40–65 years) with moderate chronic periodontitis were randomized in a double-blind study and rinsed their oral cavity following SRP with an essential oil mouthrinse (n = 23) or placebo (n = 23) for 14 days. Probing depth (PD), attachment level (AL), bleeding on probing (BOP) and modified sulcus bleeding index (SBI) were recorded at baseline and after 3 and 6 months. Subgingival plaque was taken for assessment of major bacteria associated with periodontitis. Results: AL, PD, BOP and SBI were significantly improved in both groups after three (p < 0.001) and 6 months (p ≤ 0.015). AL improved significantly better in the test than in the control group after 3 and 6 months (p < 0.001), so did PD after three months in the tendency (p = 0.1). BOP improved better in the test group after 3 months (p = 0.065). Numbers of Treponema denticola (p = 0.044) and Fusobacterium nucleatum (p = 0.029) decreased more in the test than in the control group after 3 months, those of Tannerella forsythia after 6 months (p = 0.039). Prevotella micra (p < 0.001,p = 0.035) and Campylobacter rectus (p = 0.002,p = 0.012) decreased significantly in both groups after 3 months. Conclusions: The adjunctive use of a mouthrinse containing essential oils following SRP has a positive effect on clinical variables and on bacterial levels in the subgingival biofilm

Designing of enzyme linked immunosorbent assay (ELISA) kit for diagnosis copro-antigens of Giardia lamblia

The sensitivity of microscopic examination of fecal samples to recognize Giardia parasites is low. In the methods based on antigen scanning of parasites such as enzyme linked immunosorbent assay (ELISA), copro-antigens of parasite will be traced and diagnosed even if the live parasite is absent in the fecal samples. To design this method, a pure antibody against parasite as well as an antibody conjugated to a proper enzyme is needed. In this study, an anti-Giardia IgG extracted from serum of contaminated rabbit was purified by ion-exchange chromatography and conjugated to the enzyme horse radish peroxidase (HRP). This antibody was used to design direct and indirect ELISA kits to measure conjugation titer. In both direct and indirect ELISA methods, optical densities (ODs) were 1 by using dilution of 1/4000 of conjugation. According to the results of both tests and the success in produced conjugate, it could be proceeded to prepare ELISA kits to diagnose giardiasis infections in various samples.Key words: Enzyme linked immunosorbent assay (ELISA), antibody, copro-antigen, Giardia lamblia

Genome-wide analysis of alternative splicing events in Hordeum vulgare: highlighting retention of intron-based splicing and its possible function through network analysis

In this study, using homology mapping of assembled expressed sequence tags against the genomic data, we identified alternative splicing events in barley. Results demonstrated that intron retention is frequently associated with specific abiotic stresses. Network analysis resulted in discovery of some specific sub-networks between miRNAs and transcription factors in genes with high number of alternative splicing, such as cross talk between SPL2, SPL10 and SPL11 regulated by miR156 and miR157 families. To confirm the alternative splicing events, elongation factor protein (MLOC_3412) was selected followed by experimental verification of the predicted splice variants by Semi quantitative Reverse Transcription PCR (qRT-PCR). Our novel integrative approach opens a new avenue for functional annotation of alternative splicing through regulatory-based network discovery.Bahman Panahi, Seyed Abolghasem Mohammadi, Reyhaneh Ebrahimi Khaksefidi, Jalil Fallah Mehrabadi, Esmaeil Ebrahimi

Attentional selection of superimposed surfaces cannot be explained by modulation of the gain of color channels

AbstractWhen two differently colored, superimposed patterns of dots rotate in opposite directions, this yields the percept of two superimposed transparent surfaces. If observers are cued to attend to one set of dots, they are impaired in making judgments about the other set. Since the two sets of dots are overlapping, the cueing effect cannot be explained by spatial attention. This has led to the interpretation that the impairment reflects surface-based attentional selection. However, recent single-unit recording studies in monkeys have found that attention can modulate the gain of neurons tuned for features such as color. Thus, rather than reflecting the selection of a surface, the behavioral effects might simply reflect a reduction in the gain of color channels selective for the color of the uncued set of dots (feature-based attention), as if viewing the surfaces through a colored filter. If so, then the impairment should be eliminated when the two surfaces are made the same color. Instead, we find that the impairment persists with no reduction in strength. Our findings thus rule out the color gain explanation