Analysis of disease characteristics of a large patient cohort with congenital generalized lipodystrophy from the Middle East and North Africa.

BACKGROUND: Congenital generalized lipodystrophy (CGL) is a rare inherited disease characterized by a near-total absence of adipose tissue and is associated with organ system abnormalities and severe metabolic complications. Here, we have analyzed the disease characteristics of the largest CGL cohort from the Middle East and North Africa (MENA) who have not received lipodystrophy-specific treatment. METHODS: CGL was diagnosed clinically by treating physicians through physical assessment and supported by genetic analysis, fat loss patterns, family history, and the presence of parental consanguinity. Data were obtained at the time of patient diagnosis and during leptin-replacement naïve follow-up visits as permitted by available medical records. RESULTS: Data from 43 patients with CGL (37 females, 86%) were collected from centers located in eight countries. The mean (median, range) age at diagnosis was 5.1 (1.0, at birth-37) years. Genetic analysis of the overall cohort showed that CGL1 (n = 14, 33%) and CGL2 (n = 18, 42%) were the predominant CGL subtypes followed by CGL4 (n = 10, 23%); a genetic diagnosis was unavailable for one patient (2%). There was a high prevalence of parental consanguinity (93%) and family history (67%) of lipodystrophy, with 64% (n = 25/39) and 51% (n = 20/39) of patients presenting with acromegaloid features and acanthosis nigricans, respectively. Eighty-one percent (n = 35/43) of patients had at least one organ abnormality; the most frequently affected organs were the liver (70%, n = 30/43), the cardiovascular system (37%, n = 16/43) and the spleen (33%, n = 14/43). Thirteen out of 28 (46%) patients had HbA1c > 5.7% and 20/33 (61%) had triglyceride levels > 2.26 mmol/L (200 mg/dl). Generally, patients diagnosed in adolescence or later had a greater severity of metabolic disease versus those diagnosed during childhood; however, metabolic and organ system abnormalities were observed in a subset of patients diagnosed before or at 1 year of age. CONCLUSIONS: This analysis suggests that in addition to the early onset of fat loss, family history and high consanguinity enable the identification of young patients with CGL in the MENA region. In patients with CGL who have not received lipodystrophy-specific treatment, severe metabolic disease and organ abnormalities can develop by late childhood and worsen with age.

Antimicrobial resistance patterns, virulence genes, and biofilm formation in enterococci strains collected from different sources.

BACKGROUND: Currently, antibiotic-resistant strains of Enterococcus are considered to be one of the critical health challenges globally. This study aimed to investigate the antibiotic susceptibility pattern, biofilm formation capacity, and virulence genes of enterococci isolated from different sources. METHODS: In this cross-sectional study, environmental and fecal samples were collected from the hospital environment, volunteers, and hospital staff from October 2018 to August 2019. The isolates were identified by morphological and biochemical tests (gram staining, catalase, bile resistance, esculin hydrolysis, carbohydrate fermentation, growth in 6.5% NaCl, Pyrrolidonyl arylamidase, arginine dehydrolase), and PCR for ddl gene. An antimicrobial susceptibility test was performed by the standard disk agar diffusion method according to the Clinical and Laboratory Standards Institute (CLSI) guidelines. Quantitative microplate assays were used to assess biofilm production. The bacterial DNAs were extracted by alkaline lysis method and polymerase chain reaction technique was used detect the esp, ace, and efaA virulence genes. RESULTS: Out of 145 isolates, 84 (57.9%) were identified as E. faecalis and 61 (42.1%) as E. faecium. Resistance to kanamycin and quinupristin-dalfopristin was 82.1% (69/84) and 85.7% (72/84), respectively, in E. faecalis isolates. Out of 61 E. faecalis isolates, 38 (62.4%) were resistant to kanamycin. Among the E. faecalis isolates, esp was the most dominant virulence gene (73.80%), followed by efaA, and ace, which were detected in 60.71%, and 30.95% isolates, respectively. In total, 68.27% of the strains were biofilm producers. Further, esp and efaA genes were more frequently found among E. faecalis strains with moderate and strong biofilm biomass. CONCLUSIONS: According to the findings of our study, enterococci strains isolated from different samples possess distinctive patterns of virulence genes. The esp, ace, and efaA genes were more prevalent among E. faecalis than E. faecium. Besides, the high level antibiotic resistance of normal flora and environmental enterococci strains is alarming the researchers.

Effects of Bathing Skills Training on Independence and Satisfaction of Older Adults Living in a Nursing Home: A Randomized Controlled Trial.

Background: Dependence in bathing is the most common activities of daily living (ADLs) dependency among older adults. The aim of this study was to evaluate the effect of bathing skills training on the independence and satisfaction of older adults living in nursing homes. Methods: In this randomized controlled trial, 80 participants were assigned randomly to the intervention (n = 40) and control groups (n = 40). The intervention group received 10 weekly bathing skills training sessions, with each session lasting about 60 minutes, while the control group received no direct training. The evaluation was conducted using the Modified Barthel Index (MBI) and the Canadian Occupational Performance Measure (COPM). Analysis of variance for repeated measurements was used to test the effect of intervention at the baseline, post-intervention, and follow-up. Results: The mean improvement in the MBI was greater for the intervention group (P < 0.001; partial η2 = 0.34), which remained significant at the follow-up (P < 0.001; partial η2 = 0.41). The greater mean change of the COPM-Performance was significant in the intervention group (P < 0.001; partial η2 = 0.17), which remained significant at the follow-up (P < 0.001; partial η2 = 0.19). The greater mean improvement of the COPM-Satisfaction was observed for the intervention group (P < 0.001; partial η2 = 0.36), which remained at the follow-up (P = 0.001; partial η2 = 0.42). Conclusion: Bathing skills training is effective in improving the ADLs independence and satisfaction in older adults living in nursing homes; thus, it is recommended to be included in the schedules of nursing homes.

The experiences of families raising autistic children: A phenomenological study.

BACKGROUND: Conditions and needs of autistic children have impacts on both the children and the family members who have crucial roles in raising the child. The prevalence of autism is increasing, and this fact makes it necessary to focus more on experiences of parents who have children with autism spectrum disorder (ASD). Hence, this study aimed to reflect the experiences of parents who have autistic children. MATERIALS AND METHODS: The present study was a qualitative research with phenomenology approach which was conducted using content analysis approach. Participants were 14 parents with ASD children in Ahvaz, and they were included based on purposive sampling method. To gather the required data, semi-structured interviews were formed. Data analysis was performed by Colaizzi's seven-step method. RESULTS: Fifty-four conceptual codes were extracted from interviews of ASD children parents. Parents of these children had two main experiences: first family related and second education and treatment problems. Family problems included three categories (financial, psychological, and family relationships). Education and treatment was categorized into three (schooling, transporting, and quality in facilities). CONCLUSIONS: Problems mentioned by parents of autistic children highlight the need for providing training and counseling services as well as emotional supports from both society and government. Planning and implementing supportive plans empower parents to strategically face problems and eventually improve their life quality and mutual understanding.

Diagnosis and management of X-linked hypophosphatemia in children and adolescent in the Gulf Cooperation Council countries.

INTRODUCTION: X-linked hypophosphatemia (XLH) is a rare inherited cause of hypophosphatemic rickets and osteomalacia. It is caused by mutations in the phosphate-regulating endopeptidase homolog, X-linked (PHEX). This results in increased plasma fibroblast growth factor-23 (FGF23), which leads to loss of renal sodium-phosphate co-transporter expression leading to chronic renal phosphate excretion. It also leads to low serum 1,25-dihydroxyvitamin D (1,25(OH)2D), resulting in impaired intestinal phosphate absorption. Chronic hypophosphatemia in XLH leads to impaired endochondral mineralization of the growth plates of long bones with bony deformities. XLH in children and adolescents also causes impaired growth, myopathy, bone pain, and dental abscesses. XLH is the most frequent inherited cause of phosphopenic rickets/osteomalacia. Hypophosphatemia is also found in calcipenic rickets/osteomalacia as a result of secondary hyperparathyroidism. Thus, chronic hypophosphatemia is a common etiologic factor in all types of rickets. RESULTS: There is considerable overlap between symptoms and signs of phosphopenic and calcipenic rickets/osteomalacia. Wrong diagnosis leads to inappropriate treatment of rickets/osteomalacia. Nutritional rickets and osteomalacia are common in the Gulf Cooperation Council countries which include Saudi Arabia, United Arab Emirates, Kuwait, Qatar, Bahrain, and Oman. Due to high levels of consanguinity in the region, genetic causes of phosphopenic and calcipenic rickets/osteomalacia are also common. CONCLUSION: This guideline was developed to provide an approach to the diagnosis of XLH, especially where there is no family history of the disease, and that other related conditions are not mistaken for XLH. We also guide the medical management of XLH with conventional treatment and with burosumab, a recombinant human IgG1 monoclonal antibody to FGF23.

Prevalence of Enterobacteriaceae spp. and its multidrug-resistant rates in clinical isolates: A two-center cross-sectional study.

Enterobacteriaceae spp., owing to their high durability and antibiotic-resistant mechanisms, are described as an eminent part of health treatments in hospital-acquired infections. This study aimed to estimate the prevalence of clinical isolated Enterobacteriaceae spp., and their multidrug-resistant rate in the north of Iran. In this cross-sectional study, over two years (2017-2019), clinical isolates were collected and Enterobacteriaceae spp. were identified using the standard media culture and Analytical Profile Index (API 20E) kit from two centers in the north of Iran. Isolates were confirmed by targeting the rpoB gene. Moreover, the susceptibility patterns of isolates were assessed using disc diffusion methods according to the Clinical Laboratory Standard Institute (CLSI) guidelines. Out of 2645 clinical specimens, 297 (11.2%) were confirmed as Enterobacteriaceae spp. containing Eshershia. coli 93 (31%), Citrobacter freundii 65 (21.9%), Klebsiella pneumoniae 48 (16.2%), Enterobacter spp. 43 (14.5%), and Proteus spp. 23 (7.7%). As much as 8.7% of other spp. Ampicillin (81.1%) and cephalexin (80.9%) have been shown to have the greatest resistant, and nalidixic acid (65%) and amikacin (59.2%) were the most sensitive drugs. Multidrug-resistance (MDR) strains are more isolated in the Burn and Burn intensive care unit (BICU) than other wards. The MDR frequency in Bouali and Zareh hospitals were 65 (49.61%) and 130 (78.31%), respectively. Considering the high isolation rates of MDR Enterobacteriaceae spp., preventive measures need to be taken to remove the mentioned bacteria from hospital wards.

The Relationship between Nurses' Moral Sensitivity and Patients' Satisfaction with the Care Received in the Medical Wards.

Introduction: The quality of care affects patients' satisfaction. To provide high quality care, nurses face ethical challenges in daily practice. Moral sensitivity is the first phase in moral implementation. This study aimed to determine the relationship between nurses' moral sensitivity and patients' satisfaction in medical wards. Methods: In descriptive correlational study 198 nurses and 198 patients in 17 medical wards filled out the Moral Sensitivity Questionnaire (MSQ) and Patient Satisfaction with Nursing Care Quality Questionnaire (PSNCQQ), respectively. Nurses were sampled by the census method. For each nurse, a patient was selected randomly from the same ward. Data were analyzed using SPSS version 13. Results: The highest scores were in the dimensions of "relational orientation" and "following the rules", and the lowest scores were in the dimensions of "autonomy" and "experiencing moral conflicts". The highest level of patients' satisfaction was with "nurses' professional performance" 3.98 (1.09), and the lowest level was with "nurses' routine work" 2.69 (1.22). There was no significant relationship between the mean of patient satisfaction and moral sensitivity of nurses. Conclusion: Considering that nurses had a higher score in dimension of "following the rules" and a lower score in dimension of "autonomy", it seems ethical performance in the real situation is not merely due to the nurses' moral sensitivity and it seems the complexity of the organization causes nurses face difficulties in making decisions related to clinical practice; therefore, policy makers in the health system should be able to identify barriers.

Evaluation of the Synergistic Antioxidant Effect of Resveratrol and Zataria multiflora Boiss Essential Oil in Sodium Alginate Bioactive Films.

BACKGROUND: Biodegradable films are appropriate alternatives to synthetic polymers due to their potential to enhance food safety and their function as a carrier for wide a range of food additives especially natural antioxidants. OBJECTIVE: The aim of this study was to compare the potential antioxidant activities of sodium alginate films containing resveratrol (RES) alone and in combination with Zataria multiflora essential oil (ZEO) using an in vitro model. METHODS: At first, the major chemical compositions of ZEO were evaluated by GC/MS. Then, the amount of phenolic compounds of sodium alginate films were evaluated by total phenolic contents assay and antioxidant activities of films were evaluated by 2,2-Diphenyl-1-picrylhydrazyl (DPPH) and 2,2'-azino-bis (3-ethylbenzothiazoline-6-sulfonic acid) (ABTS) assays. Finally, the concentrations of ZEO and RES which showed better antioxidant activities in sodium alginate films were incorporated to films in combination forms to evaluate their synergistic effects. RESULTS: According to gas chromatography-mass spectrometry (GC/MS) analysis, 35 compounds of ZEO were identified and Thymol (37.94%), p-Cymene (9.50%) and 3-Carene (4.98%) were the most important compounds of essential oil, respectively. The results of the radical scavenging evaluations, showed better antioxidant activities of the films containing RES than the films containing ZEO and Butylated hydroxytoluene (BHT). Also, the combined uses of ZEO and RES into alginate films had the synergistic effects (P<0.05). CONCLUSION: Therefore, it can be concluded that alginate films incorporated with the combination of ZEO and RES is the appropriate option to postpone the oxidative reactions and can be used as natural antioxidant in the food packaging industry.

Combined antioxidant and sensory effects of corn starch films with nanoemulsion of Zataria multiflora essential oil fortified with cinnamaldehyde on fresh ground beef patties.

The aim of this study was to compare the effects of corn starch films containing Zataria multiflora essential oil (ZEO) and cinnamaldehyde (CIN) in conventional, nanoemulsion (NZEO) and fortified nanoemulsion (NZEOC) forms, on pH value, oxidative stability and sensory properties of ground beef patties during 20 days of storage at 4 ±â€¯1 °C. Results of mechanical evaluation indicated that all treated films exhibited lower tensile strength (P ≤ .05) and higher elongation at break (P ≥ .05) than control samples. Also, film containing NZEO showed the best results at the end of storage with the following scores: Peroxide value (3.70 meq/kg of lipid), thiobarbituric acid reactive substance (1.03 mg MDA/kg sample), carbonyl content (0.83 nmol/mg protein) and sensory analysis (overall acceptibility: 5.85). Furthermore, NZEOC had no positive effect on the antioxidant activity compared with NZEO+CIN. The results of the study can be applied to food packaging industry for particularly meat and meat products.

Relationship between nurses' moral sensitivity and the quality of care.

BACKGROUND: To provide care with high quality, nurses face a number of moral issues requiring them to have moral abilities in professional performance. Moral sensitivity is the first step in moral performance. However, its relation to the quality of care patients receive is controversial. RESEARCH OBJECTIVE: This study aims to determine the relationship between the moral sensitivity of nurses and the quality of care received by patients in the medical wards. RESEARCH DESIGN: A descriptive correlational study using validated tools, including Moral Sensitivity Questionnaire and the Quality Patient Quality Scale. PARTICIPANTS AND RESEARCH CONTEXT: In total, 198 nurses and 198 patients in 17 medical wards of hospitals affiliated with Tabriz University of Medical Sciences, Iran. ETHICAL CONSIDERATIONS: The study was reviewed and approved by the Ethics Committee of Tabriz University of Medical Sciences. FINDINGS: The mean values of nurses' moral sensitivity and nurses' quality care were 136.47 ± 13.30 and 196.36 ± 44.10, respectively. There was no significant relationship between the patient care quality and nurses' moral sensitivity (r = -.14, p = .5). However, there was a significant inverse relationship between the dimension of "Experiencing moral conflicts" and the overall score of quality care (r = -.50, p = .04), the dimensions of "psychosocial (r = -.50, p = .04)" and "physical (r = -.50, p = .03)." CONCLUSION: Considering the significant inverse relationship between the score of patient quality care and the dimension of moral conflict experience, it seems when nurses make moral decisions, they experience a conflict between personal and professional values in their careers and thus experience moral tension. If this tension is not resolved properly, it can provide a way for them to distance themselves from patients, thereby making nurses indifferent to moral care.

Retirement homes: Social participation and quality of life.

INTRODUCTION: Social participation is considered as a means of improving one's quality of life, thus the study aimed at assessing the association between the quality of life and social participation in the elderly. METHODS: This cross-sectional study was conducted in 2016. The study population was selected from among the residents of a retirement home (an elderly residential home) in one of the Northwestern provinces of Iran, and the required data was collected via three questionnaires related to the demographic data, the specific quality of life for the elderly (LEIPAD), and the extent of social participation. Data was analyzed using mean, standard deviation, Spearman correlation coefficient, Mann-Whitney, Chi-square and Kruskal-Wallis test in SPSS software. RESULTS: The social participation level of the population under study was estimated as being appropriate (mean score: 3.81±0.32); whereas, the respondents' quality of life was estimated as being relatively inappropriate (mean score: 2.78±0.41). The "art, sport and leisure activities" subscale showed a significant relationship with the subscales related to the total quality of life and those of "Physical Performance" (r=0.128, p=0.46), "depression and anxiety"(r=0.114, p=0.051), "sexual performance"(r=0.213, p=0.21), and "life satisfaction"(r=0.272, p=0.11); furthermore, there was a significant relationship between total social participation and "life satisfaction" and the subscales related to "depression and anxiety"(r=0.263, p=0.12). CONCLUSIONS: It seems that activities that involve the art, sport and leisure subscale of social participation can result in quality of life improvement for the elderly.

The prevalence, risk factors, and screening measure for prediabetes and diabetes among Emirati overweight/obese children and adolescents.

BACKGROUND: The aim of the study was to estimate the prevalence of prediabetes and type 2 diabetes (T2D) among overweight/obese children and adolescents using different diagnostic/screening methods in comparison. METHODS: We recruited overweight/obese Emirati students; grade 6-12 (age 11-17 years) from 16 government schools in Sharjah (UAE). Anthropometric, demographic, and clinical history data was measured by standard methods. Body mass index (BMI) was categorized according to BMI percentile charts for age and sex - CDC. Capillary fasting blood glucose (FBG) and glycated hemoglobin (HbA1c) were measured by finger prick test, followed by confirmatory oral glucose tolerance tests (OGTT) and venous HbA1c for students with abnormal capillary FBG and/or HbA1c. RESULTS: Of a total of 1034 participants (45 % females) median age 14.7 years, 443 (43 %) students had abnormal screening results. The prevalence of prediabetes and T2D was 5.4 % and 0.87 %, respectively, based on OGTT (gold standard). HbA1c showed a considerable discrepancy regarding the prevalence of prediabetes (21.9 %), but not diabetes. There was a statistically significant difference in the BMI Z-scores between the three different groups of students showing normal glycemic testing, prediabetes and T2D (p = 0.041). Univariate logistic regression analysis showed that glycemic status was significantly associated with family history of T2D first-degree relatives [OR 1.87: 95 % CI: 1.04-3.36; P = 0.036], parents employment [OR 1.79: 95 % CI: 1.06-3.02; P = 0.029] and levels of triglycerides [OR 2.28: 95 % CI: 1.11-4.68; P = 0.024]. CONCLUSIONS: The prevalence of prediabetes and diabetes was high among overweight/obese Emirati children and adolescents. The numbers for prediabetes were considerably higher when using HbA1c as compared to OGTT. Overall adiposity, family history of T2D, employment and high levels of triglycerides were risk factors associated with abnormal glycemic testing.

Fracture Resistance of Simulated Immature Teeth Obturated with Gutta-Percha or Resilon and Reinforced by Composite or Post.

OBJECTIVES: The purpose of this ex- vivo study was to evaluate the fracture resistance of simulated immature teeth, obturated with Gutta-percha or Resilon and reinforced by either composite resin or fiber post. MATERIALS AND METHODS: Eighty-four human maxillary incisors were divided into seven groups (n=12). Teeth in all groups were prepared until Peeso #5 (1.5mm) could be passed through the apex. Root ends received 4mm of MTA plug as an apical barrier. Groups received the followings: 1.Gutta-percha, 2.Resilon, 3.Gutta-percha + composite resin, 4.Resilon + composite resin, 5.Gutta-percha + fiber post, 6.Resilon + fiber post and 7.No obturation (control group). Access openings were filled with composite resin. Specimens were then subjected to oblique load using Instron Testing Machine. The mean peak load at fracture was recorded and analyzed using ANOVA. RESULTS: Experimental groups had a significantly more fracture resistance than the control group (P< 0.05). No significant difference was seen between experimental groups. Teeth reinforced by fiber post showed favorable fracture resistance. CONCLUSION: Treatment plans used in this study increase the fracture resistance of immature teeth. Use of fiber posts in immature teeth, may be the most favorable clinically applicable technique.

Popular contraceptive methods in women aged 35 years and older attending health centers of 4 cities in khuzestan province, iran.

BACKGROUND: The prevalence of unintended pregnancy and associated risks are higher in late reproductive years. Limited studies have focused on contraceptive choices in these women. The aim of the study was to identify contraceptive choices and their related factors in women 35 years or older attending health centers of Khuzestan province. OBJECTIVES: Additionally, several line of evidence indicated relationship between increasing maternal age and poor pregnancy outcomes (1, 2). Pregnancies above the age of 35 are accompanied with more risks for complication related to pregnancy as compared to younger women (3-5). Risk of spontaneous abortion is 74.4% in mothers aged 45 years or more. PATIENTS AND METHODS: In a cross-sectional study 1584 women aged 35 years and older attending public health centers of four cities of Khuzestan were studied. We used an interviewer-administered questionnaire for data collection. Women investigators were recruited for interview and filling the questionnaire. Participants were assured of the confidentiality of their responses. RESULTS: The mean age of women was 39.8 ± 4.2 years. The most popular contraceptive methods used in this age group were oral contraceptive pills (31.4%), condom (28.1%), and tubal ligation (14.8%). Less effective contraceptive methods were used in 41.5% of women. Significant associations were found between the use of effective methods and literacy of husband (OR = 0.80, 95% CI: 0.75, 0.91), city of residence (OR = 0, 92, 95%CI: 0.87-0.97), women age (OR = 0.97, 95% CI; 0.94-0.99), and women education (OR = 0.87, 95%CI: 0.76-0.99) (P < 0.01). CONCLUSIONS: In spite of risk of pregnancy and unintended pregnancy in this age group, about a half of them used less effective contraceptive methods, hence family planning education, and counseling to older women should be a priority in health centers.

Pre-pregnancy body size dissatisfaction and excessive gestational weight gain.

Body size dissatisfaction has been documented as a risk factor for obesity, but little is known about the effect of body size dissatisfaction on excessive gestational weight gain. The objective of the study was to determine the association of pre-pregnancy body size dissatisfaction with excessive gestational weight gain in Iranian pregnant women. This case-control study compared pre-gravid body satisfaction status in 182 women with excessive gestational weight gain and 180 women who gained weight within the guidelines of the Institute of Medicine. All the participants of the study were 35-41 weeks gestational age and received prenatal care in Shahid Akbarabadi Hospital. The women were asked to think back to their pre-pregnant state and report their body size satisfaction status measured by the Body Image Assessment for Obesity (BIA-O). According to this measurement, the women were divided into three categories: dissatisfied women with a thinner body size preference, dissatisfied women with a heavier body size preference and satisfied women. Among women with excessive gestational weight gain, 56.6% preferred a thinner body size, while 53.9% of those with adequate gestational weight gain were satisfied with their pre-gravid body sizes. After adjusting for cofounders, those with a thinner body size preference were more likely to gain weight excessively during pregnancy when compared to satisfied women (OR: 2.17, 95% CI: 1.17-4.02). Our result showed that thinner body size preference was associated with excessive gestational weight gain. Further studies are needed to investigate whether changes in women's feelings about their body sizes will decrease the proportion of women with excessive gestational weight gain.