GM1 and GM2-Gangliosidosis: Clinical Features, Neuroimaging Findings and Electroencephalography.

Abstract: Gangliosidosis is one of the hereditary metabolic diseases caused by the accumulation of Gangliosid in the central nervous system, leading to severe and progressive neurological deficits. Regarding phenotype, GM1 and GM2-Gangliosidosis are divided into Infantile, Juvenile, and Adult. Materials & Methods: In this study, thirty-seven patients with GM1 and GM2-Gangliosidosis were referred to the neurology department of Mofid Children's Hospital in Tehran, Iran, whose disease was confirmed from September 2019 to December 2021. This study assessed age, sex, and developmental status before the onset of the disease, clinical manifestations, brain imaging, and electroencephalography. Results: 97.20% of patients were the result of family marriage. Approximately 80% of juvenile patients were developmentally normal before the onset of the disease. Developmental delay was more common among infantile GM1-Gangliosidosis than infantile GM2-Gangliosidosis, but in total, more than 50% of GM1&GM2-Gangliosidosis patients had reached their developmental milestone before the onset of the disease. With the onset of disease symptoms, 100% of patients regressed in terms of movement, 97.20% of them mentally, and 75% of them had seizures during the disease. The most common clinical findings were cherry-red spot, Mongolian spot, macrocephaly, organomegaly, hyperacusis, and scoliosis. The most common brain imaging findings included bilateral thalamus involvement, brain atrophy, PVL, and delayed myelination. The most common finding in electroencephalography was background low voltage with abnormal sharp waves. Conclusion: This study concluded that most of the patients are the result of family marriage, and most of the juvenile patients are developmentally normal before the onset of the disease. In addition, more than 50% of infantile patients reach their developmental milestones before the onset of the disease. The most common clinical findings of these patients are seizures, cherry-red spot, macrocephaly, hyperacusis, Mongolian spot, and bilateral involvement of the thalamus.

Assessment of Bacterial Pathogens and their Antibiotic Resistance in the Air of Different Wards of Selected Teaching Hospitals in Tehran.

CONTEXT: Exposure to bio-aerosols in a variety of environments has been of great interest due to the health effects on humans. Hospitals can be the reservoir of these biological agents because of the presence of infectious patients; which can lead to hospital infections and various occupational hazards. In this way, we assessed bacterial contamination in two teaching hospitals in Tehran. AIMS: Our purpose in this study assessment of bacterial pathogens and their antibiotic resistance in the air of different wards of selected teaching hospitals in Tehran. SETTINGS AND DESIGN: In this study, sampling was done according to NIOSH 0800. METHODS AND MATERIAL: This descriptive study was carried out in the different sections of two hospitals in Tehran. A total of 180 air samples were evaluated according to NIOSH 0800. In each section sampling was performed on the culture media in three stations including primary room, end room, and nursing position then the number of colonies counted. The zone of inhibition was measured in antibiotic disks to determine antibiotic resistance of samples. STATISTICAL ANALYSIS USED: Data analysis was performed using SPSS version 21. Initially, the data were normalized using the Kolmogorov-Smirnov test. The difference between the two hospitals was achieved with Mann-Whitney U test for un-normal distribution data. RESULTS: Bacterial contamination in hospital 2 was significantly higher than the hospital 1(P < 0.001). The median number of colonies in hospital 1 was 129.87 (87.46-268.97) CFU/m3 and 297.97 (217.66-431.85) CFU/m3 for hospital 2. Bacterial contamination in the all of stations in hospital 2 and 87% of samples in hospital 1 was higher than the acceptable range of ACGIH (75 CFU/m3). CONCLUSIONS: High bacterial contamination may be related to a lack of hygiene management and poor ventilation system. It seems effective infection control processes, appropriate ventilation systems and supervision systems should be improved.

The national rate of intensive care units-acquired infections, one-year retrospective study in Iran.

BACKGROUND: Hospital-acquired infections (HAIs) in intensive care units (ICUs) are among the avoidable morbidity and mortality causes. This study aimed at investigating the rate of ICU-acquired infections (ICU-AIs) in Iran. METHODS: For the purpose of this multi-center study, the rate of ICU-AIs calculated based on the data collected through Iranian nosocomial infections surveillance system and hospital information system. The data expanded based on 12 months of the year (13,632 records in terms of "hospital-ward-month"), and then, the last observation carried forward method was used to replace the missing data. RESULTS: The mean (standard deviation) age of 52,276 patients with HAIs in the ICUs was 47.37 (30.78) years. The overall rate of ICU-AIs was 96.61 per 1000 patients and 16.82 per 1000 patient-days in Iran's hospitals. The three main HAIs in the general ICUs were ventilator-associated events (VAE), urinary tract infection (UTI), and pneumonia events & lower respiratory tract infection (PNEU & LRI) infections. The three main HAIs in the internal and surgical ICUs were VAE, UTI, and bloodstream infections/surgical site infections (BSI/SSI). The most prevalent HAIs were BSI, PNEU & LRI and eye, ear, nose, throat, or mouth (EENT) infections in the neonatal ICU and PNEU & LRI, VAE, and BSI in the PICU. Device, catheter, and ventilator-associated infections accounted for 60.96, 18.56, and 39.83% of ICU-AIs, respectively. The ventilator-associated infection rate was 26.29 per 1000 ventilator-days. Based on the Pabon Lasso model, the lowest rates of ICU-AIs (66.95 per 1000 patients and 15.19 patient-days) observed in zone III, the efficient area. CONCLUSIONS: HAIs are common in the internal ICU wards. In fact, VAE and ventilator-related infections are more prevalent in Iran. HAIs in the ICUs leads to an increased risk of ICU-related mortality. Therefore, to reduce ICU-AIs, the specific and trained personnel must be responsible for the use of the devices (catheter use and ventilators), avoid over use of catheterization when possible, and remove catheters earlier.

Short and Long-Term Survival Rates Following Myocardial Infarction and Its Predictive Factors: A Study Using National Registry Data.

Background: Coronary artery disease is the most common cause of death worldwide as well as in Iran. The present study was designed to predict short and long-term survival rates after the first episode of myocardial infarction (MI). Methods: The current research is a retrospective cohort study. The data were collected from the Myocardial Infarction Registry of Iran in a 12-month period leading to March 20, 2014. The variables analyzed included smoking status, past medical history of chronic heart disease, hypertension, diabetes, hyperlipidemia, signs and symptoms during an attack, post-MI complications during hospitalization, the occurrence of arrhythmias, the location of MI, and the place of residence. Survival rates and predictive factors were estimated by the Kaplan-Meier method, the log-rank test, and the Cox model. Results: Totally, 21 181 patients with the first MI were studied. There were 15 328 men (72.4%), and the mean age of the study population was 62.10±13.42 years. During a 1-year period following MI, 2479 patients (11.7%) died. Overall, the survival rates at 28 days, 6 months, and 1 year were estimated to be 0.95 (95% CI: 0.95 to 0.96), 0.90 (95% CI: 0.90 to 0.91), and 0.88 (95% CI: 0.88 to 0.89). After the confounding factors were controlled, history of chronic heart disease (p<0.001), hypertension (p<0.001), and diabetes (p<0.001) had a significant relationship with an increased risk of death and history of hyperlipidemia (p<0.001) and inferior wall MI (p<0.001) had a significant relationship with a decreased risk of death. Conclusion: The results of this study provide evidence for health policy-makers and physicians on the link between MI and its predictive factors.

Cerebral palsy information system with an approach to information architecture: a systematic review.

BACKGROUND: Long-term complications and high costs of cerebral palsy (CP) as well as inconsistency in data related to this disease reveal the need for extensive planning to obtain accurate and complete data for the effective management of patients. OBJECTIVE: The present study reviews the information architecture of CP information system. METHOD: The relevant articles published from early 1988 to 31 July 2018 were extracted through searching PubMed, Scopus, Cochran, Web of Science and Embase databases conducted independently by two researchers. RESULTS: A total of 39 articles on CP information system were reviewed. Hospitals, rehabilitation centres and outpatient clinics were found to be the main organisations in charge of generating CP data. Each CP database used several data sources, with hospitals serving as the most important sources of information and the main generators of data. The main CP datasets were categorised into four groups of demographic data, diagnosis, motor function and visual impairment. The majority of data standards were related to the use of the International Classification of Functioning, Disability and Health and the Gross Motor Function Classification System. Finally, accuracy, completeness and consistency were the criteria employed in data quality control. CONCLUSION: Developing a robust CP information system requires deploying the principles of information architecture when developing the system, as these can improve data structure and content of CP system, as well as data quality and data sharing.

Identifying high-risk clusters of gastric cancer incidence in Iran, 2004 - 2009.

BACKGROUND: Gastric cancer is considered as the second most prevalent cancer in Iran. The present research sought to identify high risk clusters of gastric cancer with mapping using space-time scan statistics. MATERIALS AND METHODS: The present research is of descriptive type. The required data were gathered from the registered cancer reports of Cancer Control Office in the Center for Non Communicable Disease of the Ministry of Health (MOH). The data were extracted at province level in the time span of 2004-9. Sat-Scan software was used to analyse the data and to identify high risk clusters. ArcGIS10 was utilized to map the distribution of gastric cancer and to demonstrate high risk clusters. RESULTS: The most likely clusters were found in Ardabil, Gilan, Zanjan, East-Azerbaijan, Qazvin, West-Azerbaijan, Kurdistan, Hamadan, Tehran and Mazandaran between 2007 and 2009. It was statistically significant at the p-value below 0.05. CONCLUSIONS: High risk regions included Northern, West-North and central provinces, particularly Ardabil, Kurdistan, Mazandaran and Gilan. More screening tests are suggested to be conducted in high risk regions along with more frequent epidemiological studies to enact gastric cancer prevention programs.