Abnormal Neurologic Findings in Patients With Sickle Cell Disease Without a History of Major Neurologic Events.

Background and Objectives: Patients with sickle cell disease (SCD) are prone to symptomatic neurologic complications. Previous studies reported accrual of neural injury starting at early age, even without having symptomatic neurologic events. The aim of this study was to assess the prevalence and risk factors of abnormal neurologic findings in patients with SCD with no history of major symptomatic neurologic events. Methods: Our study extracted patients diagnosed with SCD from the Cooperative Study of Sickle Cell Disease. Patients who underwent a neurologic evaluation were included in our analysis. Patients with previous documented major symptomatic neurologic events were excluded. We compared patients with SCD with abnormal neurologic findings with those without in terms of clinical and laboratory parameters using multivariate binary logistic regression. Results: A total of 3,573 patients with SCD were included (median age = 11 [IQR = 19] years, male = 1719 [48.1%]). 519 (14.5%) patients had at least one abnormal neurologic finding. The most common findings in descending order were abnormal reflexes, gait abnormalities, cerebellar dysfunction, language deficits, nystagmus, abnormal muscle tone and strength, Romberg sign, Horner syndrome, and intellectual impairment. History of eye disease (odds ratio [OR] = 2.76, 95% confidence interval [CI] = 1.63-4.68) and history of osteomyelitis (OR = 2.55, 95% CI 1.34-4.84) were the strongest predictors of abnormal neurologic findings, followed by smoking (OR = 1.59, 95% CI 1.08-2.33), aseptic necrosis (OR = 1.57, 95% CI 1.06-2.33), hand-foot syndrome (OR = 1.48, 95% CI 1.04-2.12), and male sex (OR = 1.42, 95% CI 1.01-2.02). Discussion: Neurologic deficits are relatively common in patients with SCD, even without documented major neurologic insults. They range from peripheral and ophthalmic deficits to central and cognitive disabilities. Patients with SCD should have early regular neurologic evaluations and risk factor modification, particularly actively promoting smoking cessation.

Patient's Perceptions and Attitudes Towards Medical Student's Involvement in Their Healthcare at a Teaching Hospital in Jordan: A Cross Sectional Study.

Background: Medical students' involvement in patients' care varies according to patient's willingness, as some might consider it a privacy invasion. Thus, exploring patients' perceptions and attitudes towards this interaction should be thoroughly investigated, to better understand it and to provide evidence-based data for improving the training of future doctors. Methods: A cross-sectional study was conducted at Jordan University Hospital. A questionnaire was answered by 420 patients from the out-patients' clinics. Results: Of the patients interviewed, 94% were aware that they were in a teaching hospital, 92% approved the presence of medical students during their consultation and 80% accepted to be observed and examined by medical students in the presence of a senior doctor. However, this decreased to 30.5% if there was no senior doctor. Almost 83% of the patients believed that their consent should be obtained first. However, only 58% of them indicated that the student asked for consent prior to interacting with them. Patients who were more likely to approve the existence of medical students during consultation and allow students to perform procedures on them, respectively, were those who were asked for consent by students (P = 0.001 and P = 0.021), considered seeing medical students' enjoyable (P < 0.001 and P < 0.001), thought that their privacy was not violated (P < 0.001 and P < 0.001), and were provided with useful information about their health problems (P < 0.001 and P < 0.001). Conclusion: Patients showed an overall positive stance towards the involvement of medical students in their healthcare. The majority were delighted to see medical students while some expressed concerns over their privacy due to the large number of students and because several students do not ask for their consent. It is essential to apply medical ethics practices together with patient-centered approach.

Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease.

BACKGROUND: Sickle cell retinopathy (SCR) is one of the most important ocular manifestations of sickle cell disease (SCD). This study aims to assess the prevalence of SCR in SCD, identify risk factors for its development and progression to proliferative sickle cell retinopathy (PSCR), and evaluate the potential implications of these results on clinical practice. METHODS: This research is a secondary analysis of patients diagnosed with SCD from the epidemiological, multicenter Cooperative Study of Sickle Cell Disease (CSSCD). We included all patients who completed a full ophthalmic evaluation. We identified clinical and laboratory SCD characteristics associated with SCR using multivariate logistic regression models. Proliferative sickle cell retinopathy (PSCR) was diagnosed according to the Goldberg classification system. RESULTS: Of the 1904 study participants with SCD who met the inclusion criteria, 953 (50.1%) had retinopathy; of which 642 (67.3%) had bilateral disease. SCR was associated with older age (p < 0.001), history of smoking (p = 0.001), hematuria (p = 0.050), and a lower hemoglobin F (HbF) level (p < 0.001). PSCR risk increased with smoking (p = 0.005), older age (p < 0.001) higher hemoglobin level (p < 0.001) and higher white blood cell count (p = 0.011). Previous blood transfusion (p = 0.050), higher reticulocyte count (p = 0.019) and higher HbF level (p < 0.001) were protective factors against the development of PSCR. Ocular symptoms were associated with progression to PSCR in patients with SCR (p = 0.021). CONCLUSION: In this cohort of individuals with SCD, half of the participants had signs of SCR. Smoking and blood hemoglobin level were the two modifiable risk factors associated with increased retinopathy progression. Screening to identify the different stages of retinopathy, actively promoting smoking cessation, and optimizing the hematological profile of patients with SCD should guide treatment protocols designed to prevent the vision-threatening complications of the disease.

Paroxysmal and Non-Paroxysmal Atrial Fibrillation in Middle Eastern Patients: Clinical Features and the Use of Medications. Analysis of the Jordan Atrial Fibrillation (JoFib) Study.

(1) Background: Atrial fibrillation (AF) is the most common arrhythmia causing an increased risk of mortality and morbidity. It is classified into paroxysmal and non-paroxysmal AF depending on the duration and frequency of the episodes. (2) Aims: Our goal was to investigate and compare the clinical profiles, risk of co-morbidities, the use of oral anticoagulation, and outcomes of patients with paroxysmal and non-paroxysmal AF in inpatient and outpatient settings. (3) Methods: Data were extracted from 28 different hospitals and centers in Jordan with a total of 2160 patients enrolled in the study using an observational non-interventional study model. The clinical features and the use of oral anticoagulants were compared in patients with paroxysmal and non-paroxysmal AF. (4) Results: Paroxysmal AF was documented in 35.6% (769) of the patients and non-paroxysmal types in 63.9% (1380); in addition, the type of AF was unknown in 11 (0.5%) patients. Our results showed that non-paroxysmal AF patients tend to be older with more co-morbidities and higher CHA2DS2-VASC and HAS-BLED scores. They also have higher rates of hypertension and diabetes. Anticoagulant, antiarrhythmic, and diuretic agents, overall, were used more in non-paroxysmal AF than paroxysmal AF. Hospital admissions were also more frequent in non-paroxysmal AF due to various factors, some of which are heart failure, bleeding risk, and COPD. (5) Conclusions: Non-paroxysmal AF is more common among Jordanian AF patients. The prevalence of comorbidities and the use of different types of therapies, especially anticoagulants, were higher in these patients.

Cutaneous manifestation of polycystic ovary syndrome.

The aim of this cross-sectional study, that included 146 polycystic ovary syndrome (PCOS) patients, was to evaluate the prevalence, severity and pattern of cutaneous manifestation in Jordanian women with PCOS, as well as their correlation with hormonal abnormalities. A thorough complete cutaneous examination, hormonal assays (Luteinizing hormone [LH], follicle stimulating hormone [FSH], prolactin, total testosterone, free testosterone, dehydroepiandrosterone sulfate) and pelvic ultrasonography were done. The most common cutaneous features of PCOS were acne vulgaris (75.3%) followed by hirsutism (59.6%) then seborrhea (43.2%) and androgenetic alopecia (42.5%). Patients who had acne vulgaris presented at a younger age than patients who did not. Patients who had androgenetic alopecia and stria were older than patients with no such features. Moreover, all cutaneous manifestations of PCOS, except for acne, were associated with higher body mass index (BMI). Elevated LH:FSH ratio of more than 2:1 was the most common hormonal abnormality, followed by increased LH and total testosterone. Acne, hirsutism, androgenetic alopecia, seborrhea, acanthosis nigricans and skin tags are common cutaneous manifestations among Jordanian patients with PCOS. The existence of one or more of these features, especially in overweight and obese patients, should alert the physician towards the possibility of having PCOS.

Seizures risk factors in sickle cell disease. The cooperative study of sickle cell disease.

INTRODUCTION: Although evidence suggests that neurological complications, including seizures and epilepsy, are more common among sickle cell disease (SCD) patients, few studies have assessed the risk factors of developing seizures among SCD patients METHODS: We used a nested case-control study design to compare pediatric and adult SCD patients who experienced seizures with patients who did not experience any seizure regarding clinical and laboratory parameters. We conducted a secondary analysis using the data from the Cooperative Study of Sickle Cell Disease in this study RESULTS: There were 153 out of 2804 (5.5%) pediatric patients who had seizures with a median age of 8.5 (Interquartile range [IQR] = 10.1) years at first seizure and 115 out of 1281 (9.0%) adult patients who had seizures with a median age of 28.0 (IQR = 10.6) years at first seizure. Cerebrovascular accident ([CVA], OR = 5.7, 95% CI = 2.9-11.0), meningitis (OR = 3.6, 95% CI = 1.8-7.2), and eye disease (OR = 3.4, 95% CI = 1.5-8.0) were associated with increased risk of developing seizures among pediatric patients. While CVA (OR = 7.5, 95% CI = 3.5-16.0), meningitis (OR = 5.6, 95% CI = 1.5-20.0), nephrotic syndrome (OR = 3.0, 95% CI = 1.2-7.9), spleen sequestration (OR = 2.7, 95% CI = 1.1-6.3), and pneumonia (OR = 2.1, 95% CI = 1.0-4.4) were associated with increased risk of developing seizures among adult patients CONCLUSION: These findings suggest the need for treatment optimization and regular neurological follow up for SCD patients with these identified risk factors to prevent the development of seizures.

Neurological manifestations and complications of coronavirus disease 2019 (COVID-19): a systematic review and meta-analysis.

BACKGROUND: The spectrum of neurological involvement in COVID-19 is not thoroughly understood. To the best of our knowledge, no systematic review with meta-analysis and a sub-group comparison between severe and non-severe cases has been published. The aim of this study is to assess the frequency of neurological manifestations and complications, identify the neurodiagnostic findings, and compare these aspects between severe and non-severe COVID-19 cases. METHODS: A systematic search of PubMed, Scopus, EBSCO, Web of Science, and Google Scholar databases was conducted for studies published between the 1st of January 2020 and 22nd of April 2020. In addition, we scanned the bibliography of included studies to identify other potentially eligible studies. The criteria for eligibility included studies published in English language (or translated to English), those involving patients with COVID-19 of all age groups, and reporting neurological findings. Data were extracted from eligible studies. Meta-analyses were conducted using comprehensive meta-analysis software. Random-effects model was used to calculate the pooled percentages and means with their 95% confidence intervals (CIs). Sensitivity analysis was performed to assess the effect of individual studies on the summary estimate. A subgroup analysis was conducted according to severity. The main outcomes of the study were to identify the frequency and nature of neurological manifestations and complications, and the neuro-diagnostic findings in COVID-19 patients. RESULTS: 44 articles were included with a pooled sample size of 13,480 patients. The mean age was 50.3 years and 53% were males. The most common neurological manifestations were: Myalgia (22.2, 95% CI, 17.2 to 28.1%), taste impairment (19.6, 95% CI, 3.8 to 60.1%), smell impairment (18.3, 95% CI, 15.4 to 76.2%), headache (12.1, 95% CI, 9.1 to 15.8%), dizziness (11.3, 95% CI, 8.5 to 15.0%), and encephalopathy (9.4, 95% CI, 2.8 to 26.6%). Nearly 2.5% (95% CI, 1 to 6.1%) of patients had acute cerebrovascular diseases (CVD). Myalgia, elevated CK and LDH, and acute CVD were significantly more common in severe cases. Moreover, 20 case reports were assessed qualitatively, and their data presented separately. CONCLUSIONS: Neurological involvement is common in COVID-19 patients. Early recognition and vigilance of such involvement might impact their overall outcomes.

Primary Hyperhidrosis: Prevalence, Severity, and Impact on Quality of Life among Jordanian Patients.

BACKGROUND: Primary hyperhidrosis (PHH) is characterized by idiopathic, focal, bilateral, and symmetrical excessive and exaggerated sweating with a major impact on the quality of life (QoL). To date, there are no studies about the prevalence of PHH in Jordan and in the Arab region. AIM: To assess the prevalence, severity, and characteristics of PHH in the Jordanian population as well as its impact on QoL. MATERIAL AND METHODS: This study was conducted in five hospitals in Jordan and included 4,500 attendants of outpatient clinics who were evaluated for the presence of PHH. To assess the severity of hyperhidrosis (HH), we used the Hyperhidrosis Disease Severity Scale (HDSS). To evaluate the impact of HH on QoL, the Dermatology Life Quality Index (DLQI) questionnaire was answered by our patients, either digitally or on paper. RESULTS: The overall prevalence of PHH in the Jordanian population was 3.2% (n = 144). The most common site was the axillae (63%), either isolated or in association with other sites. Both sexes were affected equally. The overall mean age of onset was 14.7 years. Positive family history was found in 35% of the patients. Nearly two-thirds of the patients presented with HDSS of 3 or 4. The impact on QoL was substantial with a mean DLQI of 12.8. CONCLUSION: PHH prevalence in the Jordanian population is 3.2%, which has a major impact on QoL. This raises the need for addressing this disease to reduce its burden on patient lives.

The Association Between Personality Traits and Specialty Preference Among Medical Students in Jordan.

AIM: To assess the association between personality traits by the five-factor model and specialty choice preference among medical students and immediate medical graduates in Jordan and to identify if there were any significant differences in personality profiles between those planning to pursue different career pathways. METHODS: This is a cross-sectional study including fifth- and sixth-year medical students and post-graduate internship doctors at five universities in Jordan. An online survey was used to collect data from participants, which included their general characteristics, their personality profile using the Big Five Inventory-2, and questions about their most preferred specialty choice. RESULTS: One thousand and twelve participants were enrolled in this study. Only 4.9% were interested in pursuing a specialty in basic medical sciences, and about 12% wanted to be non-practicing medical doctors. Almost half of all participants wanted to pursue a career in surgery-oriented specialties. Those were more extraverted, more conscientious, and had less negative emotions than students who chose medicine-oriented specialties. Students who decided to pursue clinical specialties and students who wanted to be practicing doctors were more extraverted and more conscientious. CONCLUSION: Medical students and fresh medical graduates from Jordan who exhibited higher extraversion and conscientiousness and lower negative emotions preferred to be practicing clinicians. They were more inclined to pursue a career in surgery-oriented specialties. These findings might be helpful in understanding the preferences of young doctors and in counseling them about their career paths. Medical educators may wish to incorporate personality trait evaluation in planning post-graduate programs.

Characteristics, fates and complications of long-term silicone oil tamponade after pars plana vitrectomy.

BACKGROUND: Silicone oil tamponade has become a mainstay in treatment of advanced retinal detachment due to multiple etiologies. The aim of this study is to assess the characteristics, fates and complications of long-term silicone oil tamponade after par plana vitrectomy (PPV), and to compare the outcomes of different silicone oil viscosities used in a cohort of consecutive patients. METHODS: This is a retrospective comparative case series of eyes undergoing vitrectomy with silicone oil tamponade for retinal detachment by a single surgeon using different oil viscosities that were followed for one year with the silicone oil in situ. Visual acuity (VA), intraocular pressure (IOP) and complications associated with the follow up period were analyzed and compared. RESULTS: Eighty-five eyes of 85 patients were included in this study. Forty three patients had 1000 centistoke (cs) oil injected and 42 patients had 5000cs oil utilized. Demographic, cause of retinal detachment and preoperative ocular characteristics were similar in both groups. Long term complications in both groups included ocular hypertension (67.4% vs 66.7%), keratopathy due to silicone oil emulsification and migration to the anterior chamber (7.0% vs 11.9%), recurrent retinal detachment (4.7% vs 19%) and epiretinal membrane formation (7% vs 19%). In the 1000cs oil group, there was no significant difference between baseline IOP and any subsequent visit. There was a significant difference between baseline IOP and visits at day 1 (with IOP difference of 2.61 mmHg (±6.5)) (p = 0.028), 1 month (with IOP difference of 3.52 mmHg (±8.1)) (p = 0.026), 4 months (with IOP difference of 6.38 mmHg (±9.3)) (p = 0.005), and one year (with IOP difference of 4.24 mmHg (±11.1)) (p = 0.048), all higher in the post-operative period in the 5000cs oil group. Excluding the first post-operative day, no significant difference was found for VA between baseline visits and subsequent visits for either silicone oil groups. CONCLUSION: In this cohort of patients with long-term silicone oil tamponade after PPV to treat retinal detachment, IOP increased significantly in patients who received 5000cs silicone oil. There was no significant difference between other complication rates in patients receiving either oil viscosities. Long term silicone oil tamponade remains a viable option in certain cases, and a vigilant follow up for complications is necessary to limit any adverse effects and improve visual and surgical outcomes.

Factors Affecting Compliance to Anti-Vascular Endothelial Growth Factor Treatment of Diabetic Macular Edema in a Cohort of Jordanian Patients.

PURPOSE: To determine compliance rates and characteristics and to investigate factors affecting patients' adherence to treatment with anti-vascular endothelial growth factors (anti-VEGFs) for diabetic macular edema (DME) in a cohort of Jordanian patients. METHODS: A retrospective case series wherein the files of DME patients treated with anti-VEGFs were reviewed and analyzed for factors affecting treatment compliance was undertaken. Demographic, clinical and ocular characteristics were recorded. All patients were also interviewed by phone using a structured questionnaire. Univariate and multivariate analyses were performed to determine factors associated with compliance. RESULTS: A total of 117 patients (65 males 52 females) were included in this study with a mean age of 62.93 years (±9.75). Approximately, 85% of patients were compliant to their treatment and follow-up plan during the first year of management. Subjective perception of visual improvement after receiving three loading doses was the only independent variable with a unique statistically significant contribution to compliance. All other studied factors in this group of patients were not significantly associated with patient compliance. CONCLUSION: VEGF suppression via the intravitreal route to treat DME is a long-term process that requires caregiver dedication but also proper patient compliance. Addressing real-life barriers in those patients may help guide future strategies to improve the treatment experience, lower the financial burden and contribute to better outcomes. Patients' perceptions of possible treatment outcomes at the short term may influence their long-term commitment to therapy.

Ocular Manifestations of Sickle Cell Disease: Signs, Symptoms and Complications.

BACKGROUND: Sickle cell disease is an inherited hematological disorder that can affect any organ in the body including the eyes (1-6). Previous studies on ocular manifestations of sickle cell disease generally included samples of less than 100 patients. In this study, we aim to assess the frequency of different ocular signs, symptoms and complications among sickle cell disease patients. METHODS: This study was conducted using data from the Cooperative Study of Sickle Cell Disease (CSSCD). Patients with major sickle cell hemoglobinopathies (SS, SC, S ß-thal) were eligible for enrollment. Patients from all age groups were included. Patients underwent detailed ophthalmological examination under standardized conditions. RESULTS: A total of 1904 patients were included in this study, with a mean age of 27.67 (±11.72) years. 1,802 (96.4%) patients had BCVA of more than 20/40 in the better-seeing eye. On slit lamp examination, the presence of vascular loops and segment, representing a positive conjunctival sign, was the most common reported abnormal finding (54.1%). The most common complication was peripheral retinal artery occlusion detected in225 patients (20.3%) bilaterally and 77 patients (6.9%) unilaterally. CONCLUSION: In this study that included one of the largest samples ever studied to assess ocular complications of sickle cell disease, we identified the frequency and percentages of different ocular signs, symptoms and complications in different age groups.