Apart from hemoglobin-S (HbS), there are other Hb variants (non-S sickling Hb variants) that cause sickle cell disease. However, the profiles of these non-S sickling Hb variants have neither been collated nor harmonized. A literature search revealed 14 non-S sickling Hb variants (HbC-Harlem, HbC-Ziguinchor, HbS-Travis, HbS-Antilles, HbS-Providence, HbS-Oman, HbS-Cameroon, HbS-South End, Hb Jamaica Plain, HbC-Ndjamena, HbS-Clichy, HbS-San Martin, HbS-Wake, and HbS-São Paulo). Generally, the non-S sickling Hb variants are double mutants with the HbS mutation (GAG>GTG: βGlu6Val) and additional β-chain mutations. Consequently, non-S sickling Hb variants give positive solubility and sickling tests, but they differ from HbS with respect to stability, oxygen affinity, and electro-chromatographic characteristics. Similarities and discrepancies between HbS and non-S sickling Hb variants create diagnostic pitfalls that can only be resolved by elaborate electro-chromatographic and/or genetic tests. It is therefore imperative that tropical hematologists should have a thorough understanding of these atypical sickling Hb variants. Collated and harmonized appraisal of the non-S sickling Hb variants have not been previously undertaken. Hence, this paper aims to provide a comprehensive but concise historical, genetic, comparative, diagnostic, and clinical overview of non-S sickling Hb variants. The elaborate techniques often required for precise diagnosis of non-S sickling Hb variants are regrettably not readily available in low resource tropical countries, which paradoxically carry the heaviest burden of sickling disorders. We strongly recommend that tropical countries should upgrade their diagnostic laboratory facilities to avoid misdiagnosis of these atypical Hb mutants.

Keywords: Hemoglobin, Sickle; Anemia, Sickle Cell.

Rheumatoid arthritis (RA) is the most common inflammatory arthritis disease with a worldwide prevalence of 1–3%. RA patients are at higher risk of atherosclerosis than their matched age-sex controls. Cardiovascular diseases (CVDs) account for a 50% risk of increased mortality and morbidity in RA. The pattern of CVD in RA patients differs from that in the general population; RA patients are more likely to have silent ischemic heart disease, sudden death, heart failure, and die early. RA patients tend to have a 5–10 years reduction in their life span than their matched healthy population. Traditional (classical) CV risk factors work separately or synergistically with the underlying inflammation to increase CVD risk in RA. Moreover, inflammation is defined as an independent CVD risk factor. This literature review aims to discuss the traditional CVD risk factors and their association with inflammation in RA.

Keywords: Arthritis, Rheumatoid; Cardiovascular Diseases; Atherosclerosis; Inflammation.

Objectives: Our study sought to assess the maternal and neonatal outcomes of operative vaginal deliveries (OVDs) at Sultan Qaboos University Hospital (SQUH). We assessed the proportion of OVDs along with the proportion of maternal and neonatal outcomes of kiwi OmniCup vacuum, metal cup vacuum, and forceps deliveries. Methods: We conducted a retrospective cohort study in the Obstetrics and Gynecology Department at SQUH from June 2015 to March 2018. The hospital information system was utilized to obtain records of all women who delivered at SQUH by vacuum or forceps during the study period. We collected data on maternal demographics, maternal and neonatal outcomes, and total number of deliveries. Results: During the study period, 3.8% of deliveries were OVDs. The most common instrument used was the Kiwi OmniCup vacuum device. No significant difference was found between the type of tears and instrument used except perineal tears (p = 0.003), which was seen more in the vacuum group, particularly Kiwi OmniCup. Neonatal birth weight (p = 0.046) was significantly higher in the metallic vacuum cup group. Thirty-one neonates (6.6%) were admitted to the neonatal intensive care unit, and most were born using Kiwi OmniCup vacuum (67.7%). Conclusions: OVD is an ideal alternative to cesarean section with fewer maternal and neonatal complications in women who cannot deliver spontaneously if performed by a well-trained obstetrician.

Keywords: Delivery, Obstetric; Retrospective Studies; Vacuum Extraction, Obstetrical.

Objectives: Our study reports the immunomodulatory potency of Quercus infectoria gall extract in vitro. The aqueous extract was prepared and examined for its effects on cell proliferation, phagocytic activity, nitric oxide (NO) production, and cytokine synthesis by murine macrophages. Methods: Proliferative, phagocytic activity, and NO production of extract-treated and control cells were studied using proliferative assay, flow cytometry, and Griess reaction, respectively. An enzyme-linked immunosorbent assay was performed to determine the levels of pro- and anti-inflammatory cytokines in the macrophage culture. Results: Treated macrophages had a higher proliferative rate and phagocytic activity compared to untreated macrophages. The cell treatment with an extract concentration of 64 μg/mL demonstrated a significant decrease in NO production (p < 0.001). An increase in cytokine levels (IL-2, IL-5, IL-10, IL-17A, IL-23, TGF-β1) was observed; however, this increase was not statistically significant. Conclusions: Our study suggests that gall extract possesses the potential for augmenting immunomodulatory activity by cellular mediated mechanism and could play a role in regulating the innate immune response.

Keywords:

Nitric Oxide; Cytokines; Quercus infectoria; Immunomodulation; Cell Proliferation; Phagocytosis; Macrophages.

Objectives: We sought to investigate the incidence of preoperative anemia in cardiac surgery and its association with outcomes. Methods: A retrospective review of clinical, laboratory, and transfusion data for all patients who underwent cardiac surgery at Sultan Qaboos University Hospital between 2008 and 2014 was performed. Patients were divided into two groups, anemic and non-anemic, with anemia defined as hemoglobin levels < 13 g/dL (males) and < 12 g/dL (females). Clinical variables were compared using chi-square and independent t-test. Factors influencing preoperative mortality were analyzed using multivariate binary logistics regression. Results: A total of 599 patients (69.9% males and 30.1% females) were included in the study; 69.3% underwent coronary artery bypass surgery. Preoperative anemia was found in 76.1% of females and 26.7% of male patients. Rates of intraoperative red blood cell transfusions were higher among anemic patients (75.9% vs. 52.3%, p < 0.001). Anemic patients had a worse risk profile with higher incidence of diabetes mellitus (53.8% vs. 38.9%, p < 0.001), congestive heart failure (51.4% vs. 28.3%, p < 0.001), arrhythmia (16.5% vs. 8.6%, p = 0.004), and cerebrovascular disease (10.0% vs. 4.9%, p = 0.015). In addition, they had a higher risk of overall mortality (6.4% vs. 2.6%, p = 0.023). Preoperative anemia remained a risk factor for intraoperative mortality after logistic regression (odds ratio = 4.08, 95% confidence interval: 1.43–11.66; p = 0.009). Conclusions: Preoperative anemia in cardiac surgery is independently associated with increased intraoperative mortality and early readmission rates post-surgery.

Keywords: Retrospective Studies; Blood Transfusion; Anemia; Cardiac Surgical Procedures.

Objectives: We sought to investigate the nutritional and hematological status of Sudanese women of childbearing age with sickle cell anemia (SCA). Anthropometry and hematology were used to assess nutritional status and health and disease conditions, respectively. Methods: Women with steady-state (HbSS, n = 39; age = 19.0±2.7) and without (HbAA, n = 36; age, 19.8±2.7) SCA were recruited during a routine visit to the Hematology Clinic, Ibn-Auf Teaching Hospital, Khartoum, Sudan. Results: The two groups of women lived in similar environmental conditions and ate similar diets three times a day. However, despite taking regular meals, the women with sickle anemia were thinner and lighter (p < 0.001) and shorter (p = 0.002) compared with those who do not have the disease. Also, they had higher levels of mean corpuscular hemoglobin (Hb) concentration and white cell count (p < 0.001), mean corpuscular volume (p = 0.003), and platelet (p = 0.002) and lower packed cell volume and Hb (p < 0.001). There was no difference in levels of anthropometric and hematological variables between the hydroxyurea treated and untreated SCA patients (p > 0.050). Conclusions: The low anthropometric (height, weight, and body mass index) and abnormal hematological values in the women with SCA in steady-state reflect sustained nutritional insults inflected by the disease and poverty. Tailored nutritional counseling/advice must be an integral part of managing patients with SCA. Such advice is particularly vital for women of childbearing age because of the adverse effects of prepregnancy nutritional deficiency on birth outcomes.

Keywords: Female; Anemia, Sickle Cell; Nutritional Status; Sudan.

Objectives: The association of obesity and family history of type 2 diabetes mellitus (T2DM) provides an opportunity for risk stratification and prevention, as these two conditions are the most well-known risk factors for T2DM. We aimed to test the feasibility and effects of a diabetes mellitus prevention education program designed for overweight and obese Emirati people with at least one parent with T2DM. Methods: We conducted a pilot study using a pre-post design without a control arm at the Diabetes Center at Tawam Hospital in Al Ain, UAE. Overweight and obese subjects with at least one parent with T2DM were invited to participate. Three study assessments were conducted at baseline, three months, and six months including a questionnaire, anthropometry, and laboratory assessments. Interventions included three individualized or family-engaged counseling sessions based on the DiAlert protocol. The study outcomes included awareness of risks and prevention opportunities to T2DM, behavior changes in nutrition and exercise, decreased waist-circumference, and clinical/metabolic/inflammatory markers. Pre-post changes were analyzed using repeated-measures analysis of variance. Results: One hundred twenty-two overweight or obese individuals were approached. Forty-four individuals met the eligibility criteria, and 32 individuals (35.0±9.0 years; 75.0% female) completed the study. At six months, there were significant improvements in the glycated hemoglobin levels (p = 0.007), high-density lipoprotein (p < 0.049), serum creatinine (p < 0.025), estimated glomerular filtration rate (p = 0.009), and adiponectin levels (p < 0.024). Sixteen of 32 participants had ≥ 2 cm reduction in waist circumference. They demonstrated notable physical and laboratory improvements in moderate-vigorous activity, average activity counts per day, tumor necrosis factor-alpha, and interleukin-6 total cholesterol, triglyceride, and low-density lipoprotein. Conclusions: Offering family-oriented diabetes education to people at risk for T2DM is well received and has favorable effects on relevant risk factors. Better testing with large-scale randomized controlled studies is needed, and implementing similar educational programs for the Emirati population seems warranted.

Keywords: Diabetes Mellitus, Type 2; Health Education; Overweight; Obesity; United Arab Emirates.

Objectives: Modified Early Warning Score (MEWS) is a reliable, safe, instant, and inexpensive score for prognosticating patients with acute pancreatitis (AP) due to its ability to reflect ongoing changes of the systemic inflammatory response syndrome associated with AP. Our study sought to determine an optimal MEWS value in predicting severity in AP and determine its accuracy in doing so. Methods: Patients diagnosed with AP and admitted to a single institution were analyzed to determine the value of MEWS in identifying severe AP (SAP). The highest MEWS (hMEWS) score for the day and the mean of all the scores of a given day (mMEWS) were determined for each day. Sensitivity, specificity, negative predictive value (NPV), and positive predictive values (PPV) were calculated for the optimal MEWS values obtained. Results: Two hundred patients were included in the study. The data suggested that an hMEWS value > 2 on day one is most accurate in predicting SAP, with a specificity of 90.8% and PPV of 83.3%. An mMEWS of > 1.2 on day two was the most accurate in predicting SAP, with a sensitivity of 81.2%, specificity of 76.6%, PPV of 69.8%, and NPV of 85.9%. These were found to be more accurate than previous studies. Conclusions: MEWS provides a novel, easy, instant, repeatable, and reliable prognostic score that is comparable, if not superior, to existing scoring systems. However, its true value may lie in its use in resource-limited settings such as primary health care centers.

Keywords: Early Warning Score; Pancreatitis; Inflammation;  Systemic Inflammatory Response Syndrome.

Objectives: Non-alcoholic fatty liver disease (NAFLD) or non-alcoholic steatohepatitis (NASH) is a common problem associated with obesity and type 2 diabetes mellitus (T2DM). There have been anecdotal reports of the efficacy of sodium-glucose cotransporter 2 inhibitors (SGLT2Is) in improving liver function parameters in those with concomitant T2DM and NAFLD/NASH. We sought to systematically evaluate the evidence of SGLT2Is in improving liver function parameters in T2DM patients with NAFLD, considering the risks of random error based on trial sequential analysis (TSA). We also performed a meta-analysis based on a random-effects model. Methods: A systematic literature search was performed using the Medline, Cochrane, and Embase databases from inception to 20 October 2018. Primary outcome for meta-analyses was the changes in hepatic enzyme levels (alanine transaminase, aspartate transaminase, and gamma-glutamyl transpeptidase). We also performed a meta-analysis on changes in insulin resistance, glycemic, and lipid parameters using SGLT2Is as a secondary objective. Results: Eight eligible randomized controlled studies were eligible for analysis. Meta-analysis showed the efficacy of two SLT2Is, dapagliflozin, and canagliflozin in reducing these enzymes level. TSA showed that canagliflozin significantly reduced the gamma-glutamyl transpeptidase level by weighted mean difference (-5.474, 95% confidence interval (CI): -6.289–-4.659) compared to others comparators, and the evidence is conclusive. Dapagliflozin also had a statistically significant reduction in glycated hemoglobin, which is a parameter of glycemic control and homeostatic model assessment for insulin sensitivity (HOMA-IR), which is a parameter of insulin sensitivity by a weight mean difference, -0.732 (95% CI: -1.087–-0.378) and -0.804 (95% CI: -1.336–0.272), respectively. Conclusions: This study indicated that canagliflozin effectively improves liver function parameters among patients with diabetes, while dapagliflozin is more effective in improving glycemic indices and insulin sensitivity.

Keywords: Non-alcoholic Fatty Liver Disease; Sodium-Glucose Transporter 2 Inhibitors; Canagliflozin.

Objectives: Qatar has witnessed significant reforms in its health care system, including the care of cancer patients. In 2011, the National Cancer Strategy was released with the aim to deliver a high standard of care to cancer patients across the country. We sought to investigate the featuring trends in the epidemiological and clinical characteristics of lung cancer in Qatar following the publication of the National Cancer Strategy. Methods: We conducted a retrospective cohort study documenting the epidemiological and clinical characteristics of primary lung cancer cases in Qatar diagnosed from 1 January 2011 to 31 December 2018. Results: The overall age-standardized incidence rate was 8.7 per 100 000 persons (11.6 per 100 000 and 5.4 per 100 000 persons for males and females, respectively). The one, three, and five-year overall survival rates were 67.0%, 48.0%, and 28.0%, respectively. The three-year overall survival rates for stages I, II, III, and IV were 97.0%, 78.0%, 52.0%, and 31.0%, respectively. The three-year survival rates for males and females were 43.0% and 64.0%, respectively (p = 0.029), for Qatari and non-Qatari nationals were 42.0% and 49.0%, respectively (p = 0.252), and for smokers and non-smokers were 39.0% and 69.0%, respectively (p ≤ 0.001). The overall age-standardized mortality rate was 5.5 per 100 000 persons. Adenocarcinoma was the most common histologic type. Conclusions: Despite the low overall lung cancer incidence rate in Qatar, there is a rise in the incidence among females when compared to previous studies. Qatar has favorable five-year lung cancer survival rates compared to many developed and neighboring countries. Policymakers in the country should consider the changing patterns in lung cancer incidence when planning future preventive strategies.

Keywords: Lung Neoplasms; Incidence; Survival Rate; Retrospective Studies; Delivery of Health Care; Qatar.

Objectives: Chondrosarcomas are rare tumors with a variable biological characteristic. Their treatment clinically and surgically is controversial. Analysis of the clinical statistics and prognostic factors of pelvic chondrosarcoma provides a reference for clinical diagnosis and treatment. Methods: A total of 73 cases of chondrosarcoma were collected, including 24 pelvic samples, from 2008 to 2017 from the hospital database and divided into two groups: pelvic chondrosarcoma and non-pelvic chondrosarcoma. The clinical characteristics and prognostic factors of pelvic chondrosarcoma were analyzed using different statistical methods. Results: Among the 24 pelvic chondrosarcoma patients, the ratio of male to female was 1.4:1, and the median age was 43.5 years. According to the classification proposed by Enneking, there were five grade I, 14 grade II, and five grade III. Histological grading of chondrosarcoma was grade I in one case, II in 15 cases, and III in eight cases. The histological type was 17 conventional, three dedifferentiated, two secondary, one myxoid, and one mesenchymal. The overall survival rates for 24 cases at three, five, and 10 years were 82.2±8.1%, 77.3±8.9%, and 52.4±12.1%, respectively. The local recurrence rate of pelvic chondrosarcoma after surgical resection (83.3%) was significantly higher than that of other sites (34.7%), and the difference was statistically significant (p < 0.001). The final proportion of amputation rate (50.0%) was also higher than other parts (20.4%), with a statistically significant difference (p = 0.010). The total survival of the two groups was not significantly different (p = 0.216). Conclusions: Chondrosarcoma of bone generally has an excellent prognosis when optimally diagnosed and treated by an experienced team of specialists. Pelvic chondrosarcoma has a higher local recurrence rate than the other sites and tends to result in amputation. Early local recurrence after surgery indicates a poor prognosis.

Keywords: Bone Neoplasms; Chondrosarcoma; Neoplasm Recurrence, Local; Prognosis.

Foreign body ingestion is one of the most common presenting complaints in the emergency room. Psychiatric patients that present with an unusual ingested object are frequently associated with the intention of suicide, especially if they have a history of recurrent foreign body ingestion. Radiographic films help in locating the site and predicting the complications. The best method of object retrieval is dependent on many factors and decisions made for the best of patient safety to minimize expected complications. This is a case report of a patient who presented with unusual foreign body ingestion.

Keywords: Foreign Bodies; Emergency Service, Hospital; X-Ray Film; Patient Safety.

Primary hyperaldosteronism (PA) is a common disease with a prevalence of 5–10% in unselected patients with hypertension. Medullary nephrocalcinosis is a radiological diagnosis and refers to diffuse calcification in the renal parenchyma. The three commonest causes of nephrocalcinosis are hyperparathyroidism, distal renal tubular acidosis, and medullary sponge kidney. PA is not a recognized cause of nephrocalcinosis. There are a few case reports linking PA with nephrocalcinosis published till date. In this case series, we report three cases where PA was possibly associated with medullary nephrocalcinosis. In all three cases, the common causes of nephrocalcinosis were excluded by careful clinical history, biochemical evaluation, and radiological findings. We conclude and emphasize that a diagnosis of PA as an etiology of medullary nephrocalcinosis should be sought after common causes have been excluded, at least in those with hypertension that is difficult to control.

Keywords: Hyperaldosteronism; Nephrocalcinosis; Hypokalemia; Kidney Diseases; Hypertension.

Deep vein thrombosis (DVT) is a common medical condition, but the predisposing anatomical factors, which may be amenable to definitive treatment, are usually overlooked. Therefore, a high index of clinical suspicion is the key to early diagnosis. We report here one such case of May-Thurner syndrome (MTS) to raise awareness. MTS (also known as iliac vein compression syndrome) should be suspected in cases of extensive DVT of the leg, particularly involving the iliac vein on the left side. The prognosis is improved with thrombolysis followed by angioplasty and stent to address the venous stenosis.

Keywords: May-Thurner Syndrome; Iliac Vein; Constriction, Pathologic; Venous Thrombosis.

We present a case that was initially diagnosed as non-ST segment elevation myocardial infarction following a blunt chest trauma from left lateral contusion. Coronary angiography and later cardiac computed tomography were performed that showed narrowing and dissection of the distal circumflex and first obtuse marginal branch artery, which was treated medically without any coronary intervention.

Keywords: Thoracic Injuries; Wounds, Nonpenetrating; Coronary Artery Disease; Tomography, X-Ray Computed; Dissection.