Neurofibromatosis type-1 with seizures and cerebrovascular malformation: a case study

Neurofibromatosis type 1 (NF1) is the most common autosomal dominant neurocutaneous among humans. Epilepsy is more prevalent in NF1 patients than in the general population. NF1 vasculopathy is also a significant but underrecognized complication of the disease, affecting both arterial and venous blood vessels. Herein, we report a 2 year old female child with seizures and multiple cafe-au-lait spots on the body. The patient was diagnosed with NF1 based on clinical findings and family history. MRI Brain revealed middle cerebral artery dysplasia. Here we discuss diagnostic and treatment challenges and briefly reviews the existing literature.

Gabapentin pre-treatment for pressor response to direct laryngoscopy and tracheal intubation: a randomized, double-blind, placebo controlled study.

Background: Laryngoscopy and endotracheal intubation are associated with an increase in blood pressure (BP) and heart rate (HR). The present study was conducted to evaluate the role of gabapentin in attenuation of these hemodynamic changes. Methods: Forty patients undergoing elective laparoscopic cholecystectomy under general anesthesia with standardized premedication and anesthetics were randomized to receive gabapentin or a matching placebo. The patients of Group I received gabapentin 600 mg orally 2 hrs before surgery and patients in Group II received a matching placebo. Patient’s HR, systolic BP (SBP), diastolic BP (DBP), mean BP (MBP), were monitored before and after 1, 2, 5, and 10 mins of endotracheal intubation. Results: Comparison of SBP, DBP, and MBP at 1, 2, 5 and 10 mins after endotracheal intubation showed statistically significant attenuation in the gabapentin group when compared to placebo. Changes in the HR were not significant. Conclusion: Gabapentin 600 mg, given 2 hrs before induction is effective in attenuating the pressor response to laryngoscopy and tracheal intubation.

Gabapentin for post-operative nausea and vomiting: a pilot study.

Background: Gabapentin has been used in perioperative setting for the management of post-operative pain for surgery performed under general anaesthesia. Post-operative nausea and vomiting (PONV) even with the use of newer agents remains a major problem. The primary aim of this study was to see if gabapentin use decreased PONV. Methods: A total of 40 patients undergoing elective laparoscopic cholecystectomy under general anesthesia with standardized premedication and anesthetics were randomized to receive gabapentin or a matching placebo. The patients in Group I received gabapentin 600 mg orally 2 hrs before surgery and 12 hrs after the first dose. The patients in Group II received a matching placebo orally 2 hrs before surgery and 12hrs after the first dose. Patients in both groups received diclofenac sodium 75 mg i.m b.i.d for pain and ondensetron 4 mg i.v for PONV. Additional doses were given on demand and recorded. The treatment was double blinded. Results: The present study did not find significant reduction in PONV score and antiemetic consumption in gabapentin group when compared to a placebo for a period of 24 hrs. Conclusions: Gabapentin in the doses used was found to ineffective in post-operative nausea and vomiting in patients undergoing planned laparoscopic cholecystectomy with standardized pre-anaesthetic and anaesthetic medication.

Study to assess the psychosocial aspects of refractive errors and effectiveness of health education in correcting stigmas related to spectacle use in high-school students of rural India.

Background: Refractive error is the most common cause of blindness which can be corrected easily using simple modality like spectacles but because of ignorance, stigmas and cost related issues it is underutilized. Aims & Objective: Study to assess the psychosocial aspects of refractive error and effectiveness of health education in correcting stigmas related to spectacle use in high-school students of rural India. Material and Methods: This was a cross sectional study in which total of 255 high school students from a school near Bhopal were included. The responses were recorded on a pre-designed and pre-tested questionnaire. The health education was provided to all the participants and they were reassessed after one month using same questionnaire. The researcher used the STATA version 12.1 for data entry and analysis. Results: Amongst total of 255 students, 165 were males and 90 were females. During initial phase most of the respondents believed that common reasons for low vision were nutritional deficiency (68%) and bad eye care (56%). The respondents refused to use spectacles at all if needed as spectacles are cosmetically unacceptable (62%) , fear of rejection from opposite sex (54%) and likely teasing from colleagues (36%).Following health education there were statistically significant changes in the knowledge, attitude and care seeking behaviour of spectacle use. Only two parameters i.e. cosmetic acceptance of spectacles and that traditional methods were more than spectacles did not changed significantly. Conclusion: Prevalent stigmas regarding spectacle use among students of rural India were effectively corrected with health education.

Comparison of intrathecal sufentanil and morphine in addition to bupivacaine for caesarean section under spinal anesthesia

Co-administration of small dose of opioids with bupivacaine for spinal analgesia is advocated because of synergistic action between local anaesthetics and opioids, leading to reduction in doses, intraoperative discomfort and postoperative analgesic requirement. We compared the effects of intrathecal sufentanil with intrathecal morphine, when added to bupivacaine for caesarean sections, Sixty ASA I and II parturients, undergoing caesarean section under spinal anaesthesia, were randomly allocated into three groups of 20 each to receive either injection [inj.] bupivacaine 12 mg [Group I], which was labelled as the control group; inj. bupavacaine 12mg + inj sufentanil lOmcg [Group II] or inj. bupavacaine 12 mg + morphine 0.2mg [Group III] in a double blind clinical trial. The parameters studied were the time of onset, sensory level of the block achieved, total duration of analgesia, any need of rescue analgesics, maternal side effects and foetal outcome. Mean duration of analgesia [hrs] was higher in group III as compared to group I and group II [15.9 +/- 0.96 VS. 1.95 +/- 0.55 and 5.83 +/- 0.39 respectively]; total duration of analgesia was significantly longer with the use of sufentanil and morphine as compared to control [5.83 +/- 0.39 and 15.91 +/- 0.96 vs. 1.95 +/- 0.55]. Onset of block was significantly faster with use of sufentanil in Group II [1.92+0.27] vs. Group I and II [4.64 +/- 0.28 and 4.50 +/- 0.22 respectively]. Analgesia was significantly better with the use of opioids compared to control as no additional analgesic were required in both groups. Side effects with insignificant difference noted were hypotension, nausea, and shivering. However, vomiting had a higher incidence in Group I [8[40%] vs. 1[5%] and 6[30%]]; and the incidence of pruritis and somnolence was higher [6[30%]] in Group II as compared to Group III [2[10%] and 1[5%] respectively]. No adverse effects on foetus were seen with use of opioids and comparable Apgar scores were noted. Addition of small doses of sufentanil or morphine to intrathecal bupivacaine is suitable for use in caesarean section, providing rapid onset and prolonged analgesia but with some side effects like pruritis and somnolence

El sistema formador de quinina y su relación con los trastornos cardiovasculares / The kinin-forming system and cardiovascular disorders

Cardiovascular diseases are the most common cause of death worldwide. In Kuwait, death related to cardio vascular diseases may account for about 40%. Thus, it will be the greatest health care burden of the twenty-first century. Hypertension and diabetes are the two major risk factors in the development of cardiac hypertrophy, ischemic heart disease, cardiac failure, cardiac arrhythmias and myocardial infarction. The kallikrein-kinin components (plasma and urinary kininogens, kallikreins, kininases and kinins) are able to regulate BP and blood glucose levels via promoting; vasodilator, natriuretic effects and glucose metabolism. These components are located in the cardiac tissue, kidney and vascular smooth muscle. The kinin system is found to be abnormally depressed in various experimental animal models of hypertension and diabetes which might be responsible for inducing cardiac complications. It has been pointed out that the development of a compound having renal kallikrein-like activity may serve the purpose of excreting excessive sodium from the kidney in the treatment of hypertension. Also it has been demonstrated that transgenic mice over-expressing renal tissue kallikrein were hypotensive and that administration of aprotinin, a tissue kallikrein inhibitor, restored the BP of the transgenic mice. These findings highlight a role of tissue kallikrein in the regulation of BP. It has been proposed that tissue kallikrein gene delivery into various hypertensive models exhibits protection, such as reduction in high BP, attenuation of cardiac hypertrophy, inhibition of renal damage and stenosis. This may indicate the prospect of this kallikrein gene therapy for cardiovascular pathology. Several reports indicate that urinary kallikrein excretion is decreased in essential hypertension in humans and in models of experimental hypertension. Thus, reduced urinary kallikrein may reflect a deficiency in the endogenous kallikrein/kinin vasodilatory system that contributes to the pathogenesis of hypertension. Previous studies conducted in white and black population in the USA demonstrated that urinary kallikrein excretion is diminished in family members at risk for hereditary (essential) hypertension and that urinary kallikrein is one of the major genetic markers associated with family history of hypertension. Also evidence for genetic linkage between the kallikrein locus and blood pressure has been reported in the rat. Previous studies have suggested that diminished urinary kallikrein excretion is associated with salt sensitivity of blood pressure. Kallikrein excretion is also diminished in African-Americans and deficiency of the kallikreinkinin renal vasodilatory system may explain many of the unique features of essential hypertension and heart diseases in some black subjects.

Las enfermedades cardiovasculares son la causa más frecuente de muerte en todo el mundo. En Kuwait, la muerte relacionada a enfermedades cardiovasculares puede llegar al 40%. De este modo, será la mayor carga para el sistema de salud del siglo XXI. La hipertensión y la diabetes son los dos factores de riesgo mayores en la producción de hipertrofia cardíaca, cardiopatía isquémica, insuficiencia cardíaca, arritmias cardíacas e infarto de miocardio. Los componentes del sistema calicreína-quinina (quininógenos plasmáticos y urinarios, calicreínas, quininasas y quininas) regulan la PA y los niveles de glucosa sanguínea mediante estimulación de la vasodilatación, efectos natriuréticos y metabolismo de la glucosa. Esos componentes se localizan en el tejido cardíaco, riñón y células musculares lisas. El sistema quinina se encuentra deprimido anormalmente en varios modelos animales experimentales de hipertensión y diabetes que podrían ser responsables de la aparición de complicaciones cardíacas. Se ha señalado que el hallar un compuesto con actividad renal similar a la calicreína puede ser útil al propósito de excretar el exceso de sodio por el riñón en el tratamiento de la hipertensión. También se demostró que los ratones transgénicos que sobreexpresan calicreína por el tejido renal eran hipotensos y que la administración de aprotinina, un inhibidor de la calicreína tisular, restaura la PA de los ratones transgénicos. Esos hallazgos realzaron el papel de la calicreína tisular en la regulación de la PA. Se ha propuesto que el gen de la calicreína tisular entregado en varios modelos de hipertensión ejerce protección, como reducción de la PA aumentada, atenuación de la hipertrofia cardíaca, inhibición de la estenosis y del daño renal. Esto puede indicar la posibilidad de este tratamiento con gen de calicreína para trastronos cardiovasculares. Varios informes indican que la excreción urinaria de calicreína está disminuida en la hipertensión esencial en humanos y en modelos de hipertensión experimental. De este modo, la reducción de la calicreína urinaria puede reflejar una deficiencia en el sistema vasodilatador endógeno calicreína/quinina que contribuye a la patogénesis de la hipertensión. Algunos estudios previos realizados en la población blanca y negra en los EE.UU. demostraron que la excreción urinaria de calicreína está disminuida en miembros familiares con riesgo de hipertensión hereditaria (esencial) y que la calicreína urinaria es uno de los mayores marcadores genéticos asociado con historia familiar de hipertensión. También se informó la existencia de unión genética entre el locus de la calicreína y la presión sanguínea, en ratas. Algunos estudios sugirieron que la excreción urinaria disminuida de calicreína se asocia con sensibilidad de la presión sanguínea a la sal. La excreción de calicreína también está disminuida en afroamericanos y la deficiencia del sistema renal vasodilatador de calicreína-quinina puede explicar muchas de las características singulares de la hipertensión y de las enfermedades cardíacas en algunos sujetos de raza negra.