Midazolam Efficacy and Side Effects in Generalized and Partial Refractory Status Epilepticus in Children

ObjectiveMidazolam is a significant and effective drug for control of a life-threatening condition, generalized and partial refractory convulsive status epilepticus. The goal of this study was evaluation of midazolam efficacy for management of this serious disease and its two side effects, hypotension and respiratory failure.Materials & MethodsOur study was done using a quasi experimental method; 22 children with generalized refractory convulsive status epilepticus and 13 with partial refractory convulsive status epilepticus were enrolled for the study. All patients received 0.2mg/kg/dose as a bolus intravenous midazolam followed by 1-6 mcg/kg/min continuous intravenous midazolam. Following this, termination of seizures as well as hypotension and respiratory failure were evaluated.ResultsMidazolam ceased stop convulsions in 81.81% (18) patients with generalized seizures, and in 76.92% (10) patients with partial seizures, showing no significant difference between these two types of seizures (p=0.52) Hypotension was induced in 18.18% (4) patients with generalized seizures and in 30.70% (4) patients with partial seizures, again difference not significant (p=0.14). There was respiratory failure in 21.73% (5) patients with generalized seizure and in 7.69% (1) patients with partial seizure, difference not significant.(p=0.09)ConclusionThere was no significant difference in efficacy and creation of hypotension and respiratory failure after continuous intravenous infusion of midazolam between generalized and partial refractory convulsive status epilepticus.

Spinal Muscular Atrophy from Northern Iran: A Clinical and Genetic Spectrum of Ten Patients

AbstractObjectiveAutosomal recessive spinal muscular atrophy (SMA) is, after cystic fibrosis, the second most common fatal monogenic disorder and the second most common hereditary neuromuscular disease after duchenne dystrophy. The disease is characterized by degeneration of anterior horn cells leading to progressive paralysis with muscular atrophy. Depending on the clinical type (Werdnig- Hoffmann = type I, intermediate form = type II, Kugelberg-Welander = type III), some workers also have delineated an adult form of SMA (SMA type 4).SMA causes early death or increasing disability in childhood. The aim of this investigation was to describe the clinical findings of patients with spinal muscular atrophy (SMA) with survival motor neuron (SMN) gene deletion.Materials & methodsThis is a descriptive study conducted on 10 patients of SMA, confirmed by deletion of the SMN gene. All 10 patients had symmetrical muscle weakness, which was diffuse in those with onset of symptoms up to 1 months of age, and either proximal or predominant in lower limbs. Frequency determination of positive clinical and laboratory data was done according to revised diagnostic criteria.ResultsIt was found that all patients with SMA had homozygous deletions of exons 7 and 8 of the survival motor neuron 1 (SMN1) gene, which is one of the candidate genes identified within 5q13. Fasciculations, atrophy and decreased DTR were frequent findings. Laboratory metabolic tests and all brain CT scans were normal. EMG and NCV findings, all showed normal motor and Sensory NCV and denervation of muscles of upper and lower extremities were compatible with a diagnosis of spinal muscular atrophy.ConclusionOur results confirm that SMN1 copy number analysis is an important parameter for identification of couples at risk of having a child affected with SMA and reduces unwarranted prenatal diagnosis for SMA

The quality of life in boys with Duchenne muscular dystrophy

We conducted a study to evaluate the quality of life in boys with Duchenne muscular dystrophy aged 8–18 years, compared with that in matched healthy controls. A total of 85 boys with Duchenne muscular dystrophy aged 8–18 years and 136 age, sex and living place matched healthy controls were included in this study. Patients and one of their parents separately completed the 27-item Persian version of KIDSCREEN questionnaire (child and adolescent version and parent version). From the children's perspective, the quality of life in patients was found to be lower in two subclasses: “physical activities and health” (p < 0.001) and “friends” (p = 0.005). Parental estimation of their sick child's quality of life was significantly lower than children's own assessment in two subclasses: “physical activities and health” (p < 0.001) and “general mood and feelings” (p < 0.001). Our results indicate that boys with Duchenne muscular dystrophy have quite a satisfactory quality of life. A happier and more hopeful life can be promoted through increasing social support and improving the parental knowledge regarding their child's more positive life perspective. © 2016 Elsevier B.V

Iron Status in Febrile Seizure: A Case-Control Study

ObjectiveData on the relationship between iron deficiency anemia and febrile convulsions are controversial. The aim of this study was to determine the association between iron deficiency anemia and febrile convulsions among children.Materials &amp; MethodsThis case-control study was conducted during 2006-2007, on 90 children with febrile seizures (case) and 90 febrile children without seizures (control) referred to the Amirkola children hospital (a referral hospital in the north of Iran). Two groups were matched for age and sex. In all children hemoglobin (Hb) level, hematocrit (Hct), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH) and plasma ferritin (PF) were determined and the data collected were analyzed statistically using the t-test.ResultsThe mean PF and TIBC levels were not significantly different in the febrile seizure compared to the reference group; neither were differences in Hb levels statistically significant between two groups. However MCV and MCH were significantly higher in the febrile seizure group (pConclusionPlasma ferritin levels were not significantly lower in children with febrile seizures in comparison with the children in control group. It seems that iron insufficiency does not play a role in pediatric febrile seizures.

Impacts of Forest-Based Activities on Woodland Characteristics in a Forested Watershed of Southern Zagros, Iran

The purpose of this study was to investigate the impacts of forest-based activities on the conditions of the Ganaveh woodland in the southern Zagros, Iran, and to suggest strategies for improving the implementation of forest resource management plans. Woodland inventory data was gathered in 2003, accompanied with data from interviews in 2008, were used in this study. The results show that there is forest degradation in terms of a lack of forest regeneration and a relatively high incidence of bad quality trees. These defects in the woodland attributes reflect the effects of the traditional management on vegetation cover, and are the causes of concern regarding the sustainability and conservation of the woodland. Overgrazing, seed gathering, and drought in some years are probably the main reasons for the poor natural regeneration in the area. Forest activities over the last decades could be the main causes of the relatively high rate of bad quality oak trees and the high rate of oaks in coppice form. Some efforts to gain acceptance from the woodland users for protecting the preserved areas from animal grazing and seed gathering for a period could be a better alternative for woodland rehabilitation than seeding

Genetic analysis of cardiac SCN5A Gene in Iranian patients with hereditary cardiac arrhythmias.

SCN5A encodes alpha subunit of the major sodium channel (Nav1.5) in human cardiac tissue. Malfunction of this cardiac sodium channel is associated with a variety of cardiac arrhythmias and myocardial inherited diseases. Fifty-three members from three families each diagnosed with long-QT syndrome type 3 (LQTS3), Brugada syndrome (BrS), or sick sinus syndrome (SSS) were included in this observational, cross-sectional study. In this study, we analyzed the sequences of coding region of the SCN5A gene. Eleven members of the LQTS family (39%) showed p.Gln1507-Lys1508-Pro1509del mutation, 8 of BrS family (50%) showed p.Arg222Ter nonsense mutation, and 5 of 9 SSS family members (55%) showed a novel p.Met1498Arg mutation in the SCN5A gene. p.Gln1507-Lys1508-Pro1509del mutation, p.Arg222Ter nonsense mutation, and p.Met1498Arg in LQTS, BrS, and SSS, respectively, are reported for the first time in the Iranian population. Information regarding underlying genetic defects would be necessary for verifying certain clinically diagnosed arrhythmia types, carrier screening in affected families, and more precise therapy of the patients are required

Incidence, prevalence, and mortality rate of gastrointestinal cancer in Isfahan, Iran: Application of the MIAMOD method

Gastrointestinal cancers remain the most prevalent cancers in many developing countries such as Iran. The aim of this study was to estimate incidence, prevalence and mortality, as well as time trends for gastrointestinal cancers in Isfahan province of Iran for the period 2001 to 2010 and to project these estimates to the year 2020. Estimates were driven by applying the MIAMOD method (a backward calculation approach using mortality and relative survival rates). Mortality data were obtained from the Ministry of Health and the relative survival rate for all gastrointestinal cancers combined was derived from the Eurocare 3 study. Results indicated that there were clear upward trends in age adjusted incidence (males 22.9 to 74.2 and females 14.9 to 44.2), prevalence (males 52.6 to 177.7 and females 38.3 to 111.03), and mortality (males 14.6 to 47.2 and females 9.6 to 28.2) rates per 100,000 for the period of 2001 to 2010 and this upward state would persist for the projected period. For the entire period, the male to female ratio increased slightly for all parameters (incidence rate increased from 1.5 to 1.7, prevalence from 1.4 to 1.6, and mortality from 1.5 to 1.7). In males, totals of 2,179 incident cases, 5,097 prevalent cases and 1,398 mortality cases were predicated to occur during the study period. For females the predicted figures were 1,379, 3,190 and 891, respectively. It was concluded that the upward trend of incidence alongside increase in survival rates would induce a high burden on the health care infrastructure in the province of Isfahan in the future. © 2016, Asian Pacific Journal of Cancer Prevention

Dynamics of the self-interacting chameleon cosmology

In this article we study the properties of the flat FRW chameleon cosmology in which the cosmic expansion of the Universe is affected by the chameleon field and dark energy. In particular, we perform a detailed examination of the model in the light of numerical analysis. The results illustrate that the interacting chameleon filed plays an important role in late time universe acceleration and phantom crossing.Comment: 13 pages, 8 figures, to appear in Astrophysics and Space Sc

The therapeutic potential of human adipose-derived mesenchymal stem cells producing CXCL10 in a mouse melanoma lung metastasis model

Abstract Interferon γ-induced protein 10 kDa (IP-10) is a potent chemoattractant and has been suggested to enhance antitumor activity and mediate tumor regression through multiple mechanisms of action. Multiple lines of evidence have indicated that genetically-modified adult stem cells represent a potential source for cell-based cancer therapy. In the current study, we assessed therapeutic potential of human adipose derived mesenchymal stem cells (hADSC) genetically-modified to express IP-10 for the treatment of lung metastasis in an immunocompetent mouse model of metastatic melanoma. A Piggybac vector encoding IP-10 was employed to transfect hADSC ex vivo. Expression and bioactivity of the transgenic protein from hADSCs expressing IP-10 were confirmed prior to in vivo studies. Our results indicated that hADSCs expressing IP-10 could inhibit the growth of B16F10 melanoma cells and significantly prolonged survival. Immunohistochemistry analysis, TUNEL assay and western blot analysis indicated that hADSCs expressing IP-10 inhibited tumor cell growth, hindered tumor infiltration of Tregs, restricted angiogenesis and significantly prolonged survival. In conclusion, our results demonstrated that targeting metastatic tumor sites by hADSC expressing IP-10 could reduce melanoma tumor growth and lung metastasis. Keywords: Melanoma Metastasis Human adipose derived mesenchymal stem cells CXCL1

The decrease in NKG2D+ Natural Killer cells in peripheral blood of patients with metastatic colorectal cancer

Background: Natural killer (NK) cells play important roles in the immune defense against tumors such as colorectal cancer. In humans, NKG2D is an activating immune receptor constitutively expressed in most cytotoxic lymphocytes including NK and CD8+ T cells. In this study, the expression of NKG2D molecule was investigated in peripheral blood NK cells from colorectal cancer patients and compared with healthy subjects. Methods: We studied 21 non-metastatic (low-grade), 17 non-metastatic (high-grade), 16 metastatic colorectal cancer patients, and 24 healthy controls. Peripheral blood samples were obtained to isolate peripheral blood mononuclear cells (PBMCs) and the percentage of peripheral blood NKG2D+CD3-CD56+ NK cells was analyzed by flow cytometry. The expression of NKG2D at mRNA level was also measured by real-time PCR in both, patients and control subjects. Results: The results showed a significant reduction in the percentage of NKG2D+NK cells as well as NKG2D mRNA expression in peripheral blood of metastatic colon cancer patients. Conclusion: This result suggests that decreased expression of activating NKG2D receptor in metastatic colorectal cancer might compromise NK cell function and allow tumor to evade immunity (Tab. 3, Fig. 4, Ref. 33). Text in PDF www.elis.sk