Refinement of quantitative MRI as an outcome measure in inherited neuropathies

Recent acceleration in discovery of potential drug treatments for inherited neuromuscular diseases (NMD) heralds the urgent need for scientifically sound clinical trials. Given the rarity and slow progression of most of these conditions, simply increasing sample size, or extending trial duration to increase study power, are not viable options. Trials in NMD are in desperate need of highly responsive outcome measures. Lower limb muscle quantitative MRI (qMRI) allows non-invasive assessment of sequelae of nerve and muscle disease. It has recently been shown to be reliable, valid and responsive in a number of NMD, but further refinement is vital in order to ensure the ability to undertake rigorous and meaningful clinical trials in small cohorts of patients with rare and slowly progressive diseases, over short durations. This thesis aims to examine and improve qMRI responsiveness for application in trials for NMD. Two separate inherited neuropathies have been studied. In Charcot-Marie-Tooth disease type 1A (CMT1A), extended follow up has revealed that qMRI has large internal responsiveness over five years, measuring significant fat fraction (FF) change of 0.7 ± 0.6%/year with standardised response mean (SRM) of 1.07 over 5 years. Excellent validity of qMRI-determined FF is confirmed by strong correlation with clinical measures at baseline, and longitudinal validity is demonstrated for the first time in CMT1A with strong correlation with changes in CMT examination score and remaining muscle area. In Hereditary Sensory Neuropathy type 1, qMRI measures significant FF change at all anatomical levels examined, with large responsiveness at calf levels (distal calf FF change 2.2 ± 2.7%, SRM=0.83; proximal calf FF change 2.6 ± 3.0%, SRM=0.84 over 12 months). In both diseases, significant muscle fat gradients are shown to exist with the potential to devastate or enhance longitudinal analysis. FF change is predicted by baseline FF and other MRI measures in both diseases. Improvement in qMRI responsiveness is demonstrated through a host of evidence-based manipulations aimed at maximising and homogenising mean FF change. Quantitative MRI determined FF is shown to be highly responsive as an outcome measure in two different inherited neuropathies, and is ready to be used as a primary outcome measure in drug trials for rare neuromuscular diseases

Correlation of quality of sleep or quality of life and some of blood factors in hemodialysis patients

چکیده: زمینه و هدف: علی رغم پیشرفت‌های زیادی که در درمان بیماری نارسایی مزمن کلیه صورت گرفته کیفیت زندگی به عنوان یک مشکل بزرگ در این گروه از بیماران به مخاطره افتاده است. با توجه به شیوع بالای اختلال خواب و مشکلات وابسته به آن در بیماران دیالیزی، این مطالعه با هدف بررسی ارتباط بین کیفیت خواب، کیفیت زندگی و بعضی فاکتورهای خونی در بیماران تحت درمان با همودیالیز نگهدارنده انجام شد. روش بررسی: این پژوهش یک مطالعه توصیفی- تحلیلی است که در 85 بیمار تحت درمان با همودیالیز نگه دارنده در سال 84- 83 انجام گرفته است. نمونه ها از بخش های همودیالیز سه بیمارستان آموزشی وابسته به دانشگاه علوم پزشکی تهران و ایران و براساس نمونه گیری مبتنی بر هدف انتخاب شدند. کیفیت خواب با استفاده از پرسشنامه کیفیت خواب پیتس برگ (PSQI) و کیفیت زندگی با استفاده از پرسشنامه کوتاه 36 سوالی (SF36) اندازه‌گیری شد. جهت تجزیه و تحلیل داده ها از آزمون های آماری t، من ویتنی، کای دو، تست دقیق فیشر و ضریب همبستگی اسپیرمن استفاده شد. یافته ها: 63 بیمار (71) کم می خوابیدند (نمره کل کیفیت خواب بیشتر از 5 بود). ابعاد روانی (MCS) و ابعاد فیزیکی (PCS)، ‌ارتباط معکوسی با نمره کل کیفیت خواب داشت (01/0

Analysis of Dissipated Power Caused by Lubrication in Ringless Reciprocating Systems

The purpose of this paper was to evaluate the electromotor input power loss caused by oil viscosity between piston and cylinder in reciprocating systems such as compressors, presses and pumps with crank and slider driver without oil ring or ringless pistons. Using the numerical and analytical approaches respectively for nonlinear and linear oil velocity profiles assumed between piston and cylinder, dissipated power caused by oil viscosity was calculated and results of these two approaches were compared to validate finite difference results. Finally, the effect of vertical or horizontal position of piston and cylinder were compared in the case of nonlinear oil velocity profile for different applications

Diabetes: Changing the fate of diabetics in the dialysis unit

The prevalence of diabetes mellitus (DM) is very high worldwide. According to the World Health Organization in 2000 the worldwide prevalence of DM was 171,000,000. Diabetic nephropathy is a major vascular complication of DM. If DM is not treated early and adequately, many diabetic patients may reach end-stage renal disease (ESRD) secondary to advanced irreversible diabetic nephropathy. In many countries diabetic nephropathy has become the single most frequent cause of prevalent ESRD patients undergoing maintenance hemodialysis (MHD). In the early era of renal replacement therapy (RRT) by means of intermittent hemodialysis the prognosis of diabetic patients undergoing MHD was extremely poor and disappointing. While the prognosis of patients suffering from diabetic ESRD and maintained by chronic intermittent dialysis has greatly improved, the rehabilitation rate and survival of these patients continue to be worse than those of non-diabetic patients. A preexisting severely compromised cardiovascular condition, vascular access problems, diabetic foot disease, interdialytic weight gain, and intradialytic hypotension explain most of the less favorable outcome. Despite improved techniques and more aggressive medical therapy in recent years, a review of the fate of diabetics in dialysis units since 1972 reveals that these patients have had significant morbidity and mortality. We still have a long way to go in order to achieve more ideal outcomes for our patients. Most of the diabetic ESRD patients are still maintained by MHD, but they can choose other modalities of RRT such as chronic ambulatory peritoneal dialysis (CAPD), kidney and kidney plus pancreas transplantation. The results of different studies and national registries on the mortality and morbidity of ESRD patients being maintained on different modalities of dialysis are conflicting. It can be concluded that the two modalities of dialysis (CAPD and MHD) are almost comparable in terms of survival. The recent suggestions for nocturnal daily hemodialysis, short daily hemodialysis, and an integrative care approach for the management of diabetics with ESRD provides better promise for these patients. Copyright © 2007 S. Karger AG

Transplantation activities in Iran.

Iran is a tropical country with a land area of 1,648,000 square kilometers and a population of 68,100,000. Iran has a recorded history that dates back 2553 years. Its earliest medical school was Pasargad. Jondi Chapour University was founded 1753 years ago during the Sassanid dynasty as a center for higher education in medicine, philosophy, and pharmacology. Indeed, the idea of xenotransplantation dates back to days of Achaemenidae (Achaemenian dynasty), as evidenced by engravings of many mythologic chimeras still present in Persepolis. Avicenna (980-1037 AD), the great Iranian physician, performed the first nerve repair. Transplantation progress in Iran follows roughly the same pattern as that of the rest of the world, with some 10-20 years' delay. Modern organ transplantation dates back to 1935, when the first cornea transplant was performed at Farabi Hospital in Tehran, Iran. The first living-related kidney transplantation performed at Shiraz University Hospital dates back to 1968. The first bone marrow transplant was performed at Dr. Shariaati's Hospital in Tehran. The first heart transplant was performed 1993 in Tabriz, Iran. The first liver transplant was performed in 1993 in Shiraz. The first lung transplant was performed in 2001, and the first heart and lung transplants were performed in 2002, both at Tehran. In late 1985, the renal transplantation program was officially started in a major university hospital in Tehran and was poised to carry out 2 to 4 transplantations each week. Soon, another large center initiated a similar program. Both of these centers accepted surgical, medical, and nursing teams from other academic medical centers for training in kidney transplantation. Since 2002, Iran has grown to include 23 active renal, 68 cornea, 2 liver, 4 heart, 2 lung, and 2 bone marrow transplantation centers in different cities. In June 2000, the Organ Transplantation Brain Death Act was approved by the Parliament, followed by the establishment of the Iranian Network for Transplantation Organ Procurement. This act helped to expand heart, lung, and liver transplantation programs. By 2003, Iran had performed 131 liver, 77 heart, 7 lung, 211 bone marrow, 20,581 cornea, and 16,859 liver tranplantations. Sources of these donations were living-unrelated donor, 82; cadaver, 10; and living-related donor, 8. The 3-year renal transplant patient survival rate was 92.9, and the 40-month graft survival rate was 85.9. Another large step in expanding the transplantation program is the construction of the Avi- Cenna (Abou Ali Sina) Transplant Hospital in Shiraz. This hospital hopefully will begin operation in 2 years. It will offer the opportunity for the exchange and sharing of organs and increased cooperation between transplant teams in the Middle East. The hospital offers great promise for transplant medicine in Iran and other Persian Gulf countries

The Effectiveness of Early Prevention Policies Among Adolescents in the Benefits of Drug Abuse

In the rational choice model, the individual decides to use drugs after calculating their cost-benefit. However, since using drugs involves economic and health costs, public policies are being implemented by adolescents to prevent the experience and consumption of these drugs. As at present, these policies are primarily based on tax increases, consumption restrictions or warning advertisements about the dangers associated by using these substances. In other words, the purpose of these policies is to increase the costs imposed on the consumer, and the pleasure of consumption. However, today these policies are trying to reverse the rising trend in using drugs among adolescents. In this article, we emphasize the importance of pleasure from using drugs, and in particular the expected pleasure and real pleasure at first use. To this end, we use a theoretical framework for adolescent drug experience to define new ways to adopt public policies related to the primary prevention of using drugs. In particular, we identify, evaluate, and promote alternative activities for drug use: sports and creative activities, entertainment, and more

Comparative Evaluation of Spectral Transmittance of Some Welding Glasses with ANSI Z87.1

Background: Welding emits harmful rays to the eyes. We evaluate and compare the UV, blue light and IR transmittance characteristics of some currently available welding safety protectors with ANSI Z87.1 criteria.Materials and Methods: Three type of welding safety protectors (three of each type) have been evaluated for spectral transmittance. One-sample T-test was performed to establish is there a statistically significant difference between the standard criteria and UV, blue and IR regions for the welding protectors (α=0.05).Results: In ultraviolet (UV) (far and near) region, two types of the tested protectors (P1, P2) transmitted lower than the maximum allowable value specified in the standard (P<0.001). In infrared (IR) spectrum only one type (P3) transmitted lower than the maximum allowable value specified in the standard (P<0.001). In blue light region, all of the tested protectors transmitted lower than the maximum allowable value specified in the standard (P<0.001).Conclusion: Although all of the tested glasses could meet some parts of the criteria, but none of them could meet the complete spectral transmittance requirements of ANSI Z87.1

Viable Extreme Preterm Birth and Some Neonatal Outcomes in Double Cerclage versus Traditional Cerclage: A Randomized Clinical Trial

The pregnant women at higher risk of preterm labor, referred to the perinatal clinic of Kosar University Hospital in Urmia district of Iran, were enrolled into a parallel randomized clinical trial. In the investigational arm of the clinical trial, a double cervical cerclage procedure was performed addition to McDonald cerclage. In the control group however, only McDonald cerclage was performed. Extreme preterm labor (GA < 33 weeks) was the primary endpoint of this clinical trial. Age, gestational age at cerclage time, and gravidity were not found to be statistically different between the groups. Means of gestational age were 37.4 and 36.2 weeks, respectively, for the investigational and control groups. The gestational age was 1.2 weeks longer for double cerclage group but the difference was not found to be statistically significant. Preterm birth before 33 weeks of gestation was not experienced by any of the patients who received double cerclage, but five women in control group developed such an extreme preterm labor (P < 0.05). The absolute risk reduction in using double cerclage over traditional method was 18 percent (95% confidence interval, 4%–32%). Double cerclage appeared to have higher efficacy than traditional cerclage in preventing preterm labor <33 weeks of gestation