Introduction: Sickle cell anemia (SCA) is one of the most common genetic diseases worldwide. Patients with SCA present with varied clinical features and complications that may affect many organs in the human body. There are few treatment options for SCA, and patient responses vary; the only curative therapy is a stem cell transplant or gene therapy. We seek to study the clinical features and treatment options for patients with sickle cell disease treated in King Abdulaziz Medical City, Riyadh. Methods: This study was a retrospective cohort study of all adult SCA patients who were admitted to our institution during the period from 1983 to 2016. Data were entered into Excel spreadsheets and managed with SPSS. Chi-square test was used to compare responses to therapy and in patients with different presentations. Results: A total of 106 patients with sickle cell disease were included in this study. The percentages with respect to hospital admissions per year were as follows: 34.9% (37 patients) were never admitted, 26.4% (28 patients) were admitted fewer than 2 times, 24.5% (26 patients) were admitted 3–5 times, and 13.2% (14 patients) were admitted more than 5 times. The number of complications was used to measure the severity of the disease. The disease severity was higher in males than in females (67.3% vs. 32.7%, respectively; P = 0.018). The disease severity was higher in those who were born in the Western and Southwestern areas than in other areas (84.2% vs. 15.8%, respectively; P = 0.007). Hydroxyurea significantly reduced the severity of SCA with a P = 0.002. Conclusion: Our study showed that vaso-occlusive crisis was the most common complication and indication for hospital admission. Treatment with hydroxyurea led to a significant reduction in the number of hospital admissions.

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Dapsone is a cheap and widely used drug in many diseases. It can rarely cause dose-independent methemoglobinemia. The key to managing such cases is strong clinical suspicion and prompt discontinuation of the drug. Effective antidote is methylene blue which can rapidly reverse effects in seriously ill patients. Here, we present two such cases that we came across who were managed differently and successfully at our center.

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Background: Despite a reduction in mortality rates, hemophilia patients continue to suffer the consequences of repeated bleeding episodes. Assessment of health-related quality of life (HRQoL) attempts to determine how different life aspects are affected and helps guide future decision-making. AIMS: The aim of the study was to assess HRQoL in adult patients with hemophilia managed at King Khalid University Hospital (KKUH), Riyadh and to identify factors affecting HRQoL. Settings and Design: An observational cross-sectional study was conducted at KKUH in Riyadh from September 2014 to April 2015. Subjects and Methods: Sixty-one patients followed up and treated at KKUH from 2000 to 2015 were enrolled in the study. Patients were asked to complete the Arabic version of the Hemophilia-Specific QoL (HAEMO-QoL-A) Questionnaire. Results: Forty-Nine hemophilic patients completed the HAEMO-QoL-A questionnaire. Of these, 81.6% had hemophilia A, with a mean age of 29.84 years. The most common target joint was the knee (89.7%). The majority used on-demand treatment (55.1%). The HAEMO-QoL-A mean total score was 99.89. “Treatment concerns” were the most impaired dimension, whereas the “work/school, family, and social life” dimension was least affected. Patients receiving on-demand treatment had the lowest average bleeding events (8.22, P = 0.007). Conclusion: Frequent bleeding events, prophylactic treatment, and presence of clotting factor inhibitors negatively impact the HRQoL of patients with hemophilia.

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Introduction: Dengue virus infection is an acute febrile illness endemic in several countries including India. Although the management is empirical, early diagnosis is crucial for timely intervention to reduce the morbidity and mortality. The diagnosis of dengue virus infection mainly depends on serological testing and virus isolation which are costly and time-consuming. A simple blood test like complete blood count (CBC) run on five-part differential cell counter with volume conductivity, and scatter (VCS) data can help in predicting dengue infection without additional cost. Methods: In the present study, we analyzed VCS parameters of 337 febrile patients over the period of 1 year (January 2016 to December 2016). Out of these, 255 cases were positive for dengue serology and 82 were febrile controls. Comparative analysis of utility of previously published dengue factor, lymph index, and monocyte factor along with the “New Dengue Factor” introduced in the present study. Results: The “New Dengue Factor” proposed by us gave highest “positive likelihood ratio” for the diagnosis of dengue virus infection. Dengue factor proposed by Sharma et al. was the most “sensitive,” and “lymph index” proposed by Hawaldar et al. was the most specific followed by the “New dengue factor” introduced in the present study. Conclusion: Factors derived from VCS parameters are useful for early prediction of dengue virus infection without incurring additional cost and thus would contribute to better patient care.

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Context: The prevalence of iron deficiency anemia (IDA) in adults from the Sultanate of Oman is unknown. Aims: The aim of this study was to assess the prevalence of IDA and latent iron deficiency (ID) in students admitted to a university hospital. Settings and Design: This is a prospective, cross-sectional cohort study. Subjects and Methods: In asymptomatic university students, blood samples were obtained for blood counts and iron status after obtaining consent. Students who were found to have IDA or ID were given oral iron therapy for a minimum of 3 months to study the response to treatment. Statistical Analysis Used: Student's t-test (continuous variables) and Chi-square test (categorical variable) were used for statistical analysis. Results: The mean age + standard deviation was 21 + 1.3 years with a range from 17 to 29 years in 350 students, with 274 students being females (78.8%). Using the World Health Organization criteria, 91 (26%), 133 (38%), and 126 (36%) students, respectively, were classified as IDA, ID, and normal. HPLC showed that 28 students had sickle cell trait (HbS: 26%–35%), and one each had sickle cell disease (HbS 92%), Hb C trait (31%), Hb D trait (30%), Hb E trait (19%), and delta gene variant (HbA2 – 1.6%). Among the students who received treatment (52 IDA and 20 ID), Hb, mean corpuscular volume, mean corpuscular hemoglobin, and serum ferritin showed statistically significant improvement after oral iron therapy (P < 0.001, paired Student's t-test). Conclusions: The prevalence of IDA was 26% and that of ID was 38%, with a preponderance of females.

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Background: The sickling process of sickle cell disease (SCD) has harmful effects on the vascular endothelium manifested as vascular blockade, diminished tissue oxygenation, and consequently reperfusion injury. Statins pleiotropic effects can be demonstrated through improvement of endothelial function. Studies on the role of statins (e.g., atorvastatin) on endothelial dysfunction in SCD are minimal. Objective: The objective is to assess the possible therapeutic use of atorvastatin in patients with SCD. Subjects and Methods: Thirty SCD patients (15 female and 15 male) with a mean age of 27.0 ± 8.9 years and 30 healthy controls (18 female and 12 male) with a mean age of 29.7 ± 9.1 years participated in the study. Endothelial function was assessed with flow mediated dilatation (FMD) and endothelial independent dilatation (EID) of the brachial and common carotid arteries at baseline and after 6 weeks therapy with atorvastatin, 20 mg/day. Results: SCD patients had diminished FMD% and EID% values compared with corresponding values in the controls. The values were improved significantly after atorvastatin treatment (P = 0.002 for FMD%; P = 0.001 for EDI%). Conclusion: SCD patients have endothelial dysfunction. Atorvastatin, 20 mg daily for 6 weeks, improved the markers of endothelial function, FMD%, and EID%, in these patients.

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We report an atypical manifestation of Antiphospholipid syndrome (APS) in a 40-year-old male with nonartertic anterior ischemic optic neuropathy (NAION) presenting with 12-day history of a headache and left eye pain not associated with visual changes. On his initial evaluation, our differential diagnoses included optic neuritis, ischemic optic neuropathy (ION), and intracranial hemorrhage. Onset and course of the disease, lack of vision changes as well as the presence of retinal hemorrhages on fundoscopic examination excluded optic neuritis. The diagnosis was made after exclusion of all other causes including infectious and noninfectious causes of optic neuritis, ION, and intracranial hemorrhage. This case demonstrates the importance of including APS as a differential diagnosis of NAION in middle-aged individuals.

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