Prevalence of Seizure In PKU: An Analytic Historical Study

ObjectivePhenylketonuria (PKU) is an autosomal recessive metabolic genetic disorder characterized by a deficiency in the hepatic enzyme phenylalanine hydroxylase (PAH) which can cause problems with brain development, leading to progressive mental retardation, brain damage, and seizures. In this study we evaluated the frequency of seizure, EEG abnormality and behavioral disorders.Materials & Methods In this case study, 94 PKU children aged between 1 month and 23 years who were referred to Mofid children Hospital between 2009 and 2010 were enrolled.Patients were age and sex matched. Statistical tests were used for comparing patients' data.Results The mean age of patients was 8.4 years. Parents were relatives in 80.9% of the cases (76 patients). Of all, 43% (45 patients) had seizure but EEG was abnormal only in 81% of them (35 patients out of 43 patients). Totally, EEG was abnormal in 67% of the cases (63 patients) of whom 44.4% (28 patients of 63 patients) did not have seizure. Therefore, there was a significant relationship between seizure and EEG abnormality. The phenylalanine level ranged from 8mg/dL to 50mg/dL (mean: 18.88 mg/dL) at the time of diagnosis and from 0.4mg/dL to 18mg/dL (mean: 7.37mg/dL) at the time of evaluation. On the other hand,we observed abnormal behaviors in all EEG abnormalities and there was a significant relationship between EEG abnormality and behavioral disorders.ConclusionIn our study, the prevalence of seizure was less than EEG abnormality and there was a significant relationship between EEG abnormalities and behavioral disorders in patients with Phenylketonuria regardless of seizure.The authors believe that treatment of EEG abnormalities may lead to the correction of behavioral disorders in these patients.

Just-in-time preemptive single machine problem with costs of earliness/tardiness, interruption and work-in-process

This paper considers preemption and idle time are allowed in a single machine scheduling problem with just-in-time (JIT) approach. It incorporates Earliness/Tardiness (E/T) penalties, interruption penalties and holding cost of jobs which are waiting to be processed as work-in-process (WIP). Generally in non-preemptive problems, E/T penalties are a function of the completion time of the jobs. Then, we introduce a non-linear preemptive scheduling model where the earliness penalty depends on the starting time of a job. The model is liberalized by an elaborately–designed procedure to reach the optimum solution. To validate and verify the performance of proposed model, computational results are presented by solving a number of numerical examples

Association of EEG Abnormality and Developmental Delay in Phenylketonuria (PKU): an Analytic Historical Case on Phenylketonuria (PKU): An Analytic Historical Case-Control

ObjectiveElectroencephalogram (EEG) is an easy and non invasive evaluation method for diagnosis and early prognosis in children. Our aim was to assess the association between EEG and the patients' Developmental Quotient (DQ) level in phenylketonuria.Materials & MethodsIn this study, 94 PKU patients (45 boys, 49 girls; mean age: 8.5 6.2 years) who were diagnosed through newborn screening tests or later were followed. PKU was confirmed with a serum phenylalanine concentration above 6 mg/ dl in untreated newborns. The patients were matched in gender, age and phenylalanine level. The ASQ (Age and Stage Questionnaire) was used for evaluation of the developmental stage of the case (abnormal EEG) and control (normal EEG) groups and the Wechsler Intelligence Scale was used to assess cognitive and intellectual abilities. Finally, one way ANOVA and chi square tests were used for analysis and P< 0.05 was considered significant.ResultsThe case group consisted of 63 patients (67%) with abnormal EEGs and the control group consisted of 31 patients (33%) with normal EEGs. In patients with abnormal EEGs, 34 (53%) had mild, nine (14%) had moderate and 20 (33%) had severe EEG changes. Distribution of high and low DQ levels in these three groups show significant difference (p=0.001). Distribution of DQ level in the abnormal and normal EEG patients showed a significant difference (p=0.001).ConclusionThere was no significant difference between phenylalanine level in case and control groups; therefore, EEG findings may affect patients' developmental scores despite a normal phenylalanine level in PKU patients.

The Survey of Presenting New Health Services to Middle-aged Population in Health Transformation Plan: a Qualitative Study

Introduction: Providing health services to middle-aged people is of special importance due to the large population, productivity, and the impact of health behaviors of this age group on various aspects of family health. The purpose of this study is to evaluate the provision of middle-aged health services. Methods: This was a qualitative   study performed in 2017 in   comprehensive health centers of Yazd city. Central part of Iran. Sixty two individuals (specialists, general practitioners and experts) providing health services in comprehensive health centers were selected through targeted sampling . Interviews were done with a semi-structured individualized method. Questions were asked in the following domains: weaknesses and strengths and recommendations about the method of providing services and its content. Results: According to the results, 15 weak points and 5 strong points in the method of providing services, and 8 strong points and 7 weak points about the content of health service packages and 17 recommended mechanisms about the improvement of the providing services and 11 recommended mechanisms about improvement of the content of packages were extracted. The main weak points in providing services were such as not observing referring system, lack of insurance coverage for some services, problems in Integrated Health Record System. The weak points of the packages of health services for middle-aged people were as lack of predicting the service packages for levels 2 and 3, repetition of the questions in evaluation, and low attention to social health and traditional medicine. Conclusion: Concentrating on the improvement of the service providing and the contents of the packages of new health services by utilizing presented recommendations according to professional viewpoints of health service providers helps improve health level in this age group and increase the efficiency in the middle-aged people

A telemonitoring system to support CPAP therapy in patients with obstructive sleep apnea: a participatory approach in analysis, design, and evaluation

Background: Continues positive airway pressure (CPAP) therapy is a gold standard treatment for moderate to severe cases of OSA (obstructive sleep apnea). The present research aimed to describe the analysis, design, and evaluation of a telemonitoring system to improve CPAP adherence in patients afflicted with OSA. Methods: The telemonitoring system was developed in five phases. In the exploratory phase, the body of related literature was reviewed. Then a need analysis was conducted through a focus group discussion with sleep medicine specialists and sales company representatives and an interview with patients. The third phase involved data integration. Then the content and system development were done based on the previous phases. Finally, usability and functionality tests were used to evaluate the system. Results: The exploratory phase and the needs analysis were conducted by four sleep medicine specialists, two medical informatics specialists, six key figures of the sales companies, two system developers, and 46 patients in different phases. Based on the results obtained from the data integration phase, the telemonitoring system involved three main parts: a patient’s application, a doctor’s portal, a selling companies’ portal (operator’s portal) along with facilitating software for patients to send the CPAP data. Usability and functionality tests were given to 7 and 10 patients, respectively. The total number of usability issues reported by users in the evaluation process was 18, with an average of 2.5 issues per user. The installation problems, disrupted links and improper playing of videos were the main functionalities problems that were solved. Conclusion: The telemonitoring system, as a means of communication between patients, doctors, and selling companies, can be used to support patients clinically and technically. It has the potential to improve CPAP adherence in patients with OSA

A survey of perceptions, attitudes and practices regarding the antibiotic prescription among Iranian physicians

Background: Over-prescription and overuse of antibiotics are a major cause of concern in terms of healthcare and economic implications, as well as the emergence of microbial resistance worldwide. So far, efforts have been administered by many countries to regulate the consumption of antibiotics and overcome the rapid emergence of resistant bacteria. Assessment of the perspectives and behavior of physicians can play a major role in achieving this objective. Objectives: The objective of the present study was to assess the perspectives on the antibiotic prescription practice of physicians and evaluate their tendency to receive feedback on their performance in outpatient settings. Materials and methods: Attitudes and practices of 330 physicians on antibiotics prescription were evaluated using a validated questionnaire in 2014 in Mashhad, Iran. Results: 94% of physicians asserted that the prescription rate of antibiotics in Iran is higher than the global average. Physicians noted that “patients’ tendency to prescribe antibiotics“ is the primary reason of excessive prescription of antibiotics. Approximately, 84% of physicians were keen on receiving performance feedback. More than 71% of participants specified Short Message Service (SMS) notification as their preferred medium for receiving feedback. Conclusions: The dominant viewpoint of physicians was that the rate of antibiotics prescription in Iran is larger than the global average, which can lead to adverse economic and bacterial resistance consequences. Interventions such as ‘performance feedback’ have the potential to reduce antibiotics over-prescription

Effectiveness of intervention due to feedback on errors arising from inappropriate transportation and storage of blood bags in hospitals: a quasi-experimental study

Background: The limited supply of red blood cells (RBCs) for transfusion has clinical implications, and can lead to the introduction of different approaches in decreasing blood wastage and the safe and effective delivery of blood products. Objective: To identify the influences of feedback-based intervention on inappropriate temperatures during transportation and storage of blood bags. Methods: This was a quasi-experimental pretest-posttest design with a non-equivalent control group that was carried out on 200 RBC. The study was conducted in a teaching hospital in Mashhad, Iran, and in two periods during the same season: winter of 2015 (pre-intervention period) and winter of 2016 (post-intervention period). Staff of the blood bank department, as the intervention group, received the intervention including feedback regarding the inappropriate temperature in the blood bank during the pre-intervention period. The control group included personnel and nurses from the OR, CSICU, and transportation department, who did not receive any feedback. The effect of the intervention on the RBCs' temperature was evaluated by comparing the percentage of inappropriate temperature of RBC bags before and after the implementation of the intervention. Results: Inappropriate temperature in the blood bank decreased from 30% to 12% after the intervention was implemented (relative reduction of 60%). Meanwhile, there was even an increase in inappropriate temperature in the control group including OR (from 6.5% to 20.5%), CSICU (from 1% to 2.5%) and transportation (from 0.6% to 16.6%). Conclusion: The implementation of a feedback-based intervention to increase the awareness of the staff of critical conditions can improve conditions of blood storage and transport. The use of various interventions along with this type of intervention seems necessary to increase effectiveness. Funding: This research was part of the first author's MSc thesis which was supported by a grant from Mashhad University of Medical Science Research Council (Ref. no.: 950116; Date: September 7, 2016)