Ethnic-Based Assessment of Vitamin D and Magnesium Status in the Kingdom of Bahrain.

BACKGROUND: Vitamin D deficiency is a major global health problem. Most previous studies focused attention on the significant role of sunlight exposure in the homeostasis of vitamin D and calcium blood levels. Magnesium is pivotal in the proper functioning of vitamin D, and the physiologic functions of different organs require a balanced vitamin D and magnesium status. The relationship between sunlight exposure and blood levels of vitamin D and magnesium has often been overlooked. The aim of this study was to evaluate vitamin D and magnesium status based on sunlight exposure and ethnicity in Bahraini and expatriate workers. METHODS: A cross-sectional study was conducted between October 2018 and September 2019. One hundred and seventy-four subjects participated in this study were subdivided based on their ethnicity and work environment-dependent exposure to sunlight into four groups: (1) Bahraini exposed (n=94), (2) Bahraini non-exposed (n=25), (3) expatriate exposed (n=31), and (4) expatriate non-exposed (n=24). Blood levels of vitamin D and magnesium were evaluated for all the participants. RESULTS:  Independent of ethnicity, vitamin D levels were insignificantly different among the studied groups and were all below the normal reference range. Yet, there was still a sunlight-dependent increase in vitamin D level that could be seen only in Bahraini workers. Magnesium levels were significantly higher in expatriates when compared to Bahraini workers. Sunlight-exposed expatriates had significantly higher magnesium levels than their Bahraini counterparts, while there was no significant difference between both ethnicities in the non-exposed groups. CONCLUSION: Country- and ethnic-specific definitions for vitamin D status and sunlight exposure are recommended. The assessment of magnesium status is pivotal in the overall assessment of vitamin D status.

A Rare Concurrence of Myelodysplastic Neoplasia and Tetrasomy 8 in a 3-Year-Old Bahraini Male.

Myelodysplastic neoplasia (MDS) is a group of stem cell disorders involving ineffective hematopoiesis. It can be associated with an increased risk of progression toward acute myeloid leukemia (AML). In Bahrain, MDS is the fifth most common primary hematologic malignancy. MDS has an annual incidence of up to 4 million cases. Some of the presenting signs and symptoms of MDS are often nonspecific, such as fatigue, pallor, malaise, fevers, bleeding, bruising, weight loss, and anorexia. Approximately 40% of patients with MDS progress to AML. This paper outlines a case of a 3-year-old Bahraini male (known to have sickle cell trait) who presented to the emergency department of Salmaniya Medical Complex with a five-day history of fever, congested throat, left ear pain, and abdominal pain. He had one episode of vomiting gastric content the previous day. He had previously gone to a private clinic with similar symptoms. Physical examination revealed a short neck and short stature, which was found to be below the 5th percentile. He had generalized pallor and hepatosplenomegaly. A blood smear showed leukopenia and normochromic normocytic anemia. There were excessive blasts found which consisted of 17% of nucleated cells and few granulopoietic cells. Erythropoiesis was active with a few showing mild megaloblastic changes. There were rare megakaryocytes noted. Moreover, the bone marrow aspirate showed two populations on dim CD45. The first population consisted of 3.15% on dim CD45 comprising of hematogones which brightly expressed CD19, HLA-DR, CD79a, and dim CD10. The second population consisted of 14.85% on dim CD45 which expressed CD34, CD13, CD117, HLA-DR, and dim CD7. Based on the peripheral blood smear and bone marrow immunophenotyping findings, a diagnosis of myelodysplastic syndrome with excessive blasts was made, which soon transformed into a diagnosis of AML. Furthermore, increased levels of dysplastic changes and percentage of blasts in the peripheral blood smear and bone marrow lead to a higher possibility of transformation into AML. As per the WHO classification, a diagnosis of MDS needs evaluation of the morphology of blood and bone marrow.

Molecular insights into hereditary elliptocytosis and pyropoikilocytosis: NGS uncovers multiple potential candidate genes.

Hereditary elliptocytosis (HE) and pyropoikilocytosis (HPP) are considered a group of hemolytic anemias (HE/HPP) due to inherited abnormalities of erythrocyte membrane proteins with a worldwide distribution. Most cases are associated with molecular abnormalities linked to spectrin, band 4.1, and ankyrin. The present study aimed to identify significant molecular signatures on a target panel of 8 genes using whole exome sequencing (WES) in 9 Bahraini patients with elliptocytosis. Case selection was based on presence of anemia not associated with iron deficiency or hemoglobinopathy and demonstrating > 50% elliptocytes in blood smears. The c.779 T > C mutation of SPTA1 (Spectrin alpha), which is a known deleterious missense mutation that inhibits normal association of spectrin molecules to form tetramers, was seen in 4 patients in homozygous (n = 1) and heterozygous (n = 3) states. The αLELY abnormality in association with compound heterozygous mutations in SPTA1 was present in 5 patients (2 associated with the SPTA1 c.779 T > C variant; 3 with c.3487 T > G and various other SPTA1 mutations of uncertain/unknown significance). Seven patients had SPTB (Spectrin beta) mutations, predicted as likely benign by in silico analysis. A novel EPB41 (Erythrocyte Membrane Protein Band 4.1) mutation with potential deleterious impact was also seen. Finally, 2 cases showed an InDel (insertion-deletion mutations) abnormality in the gene that codes for the mechanosensitive ion-channel PIEZO (Piezo Type Mechanosensitive Ion Channel Component 1). PIEZO mutations are reported to cause red cell dehydration but have not been previously described in HE/HPP. Results of this study confirm the involvement of previously reported abnormalities in SPTA1 and suggest possible involvement of other candidate genes in a disorder involving polygenic interactions.

Spectrum of lymphoma in Bahrain. A retrospective analysis according to the World Health Organization classification.

OBJECTIVES: To determine the spectrum of various types of lymphoma in Bahrain according to the latest World Health Organization classification criteria.  Methods: A retrospective review was conducted for all new lymphoma cases diagnosed at Salmaniya Medical Complex, Manama, Bahrain during the period from January 2010 to December 2015.  Results: Two hundred and twenty-one new cases of lymphoma in Bahraini patients were diagnosed in the study period. Eighty patients had Hodgkin lymphoma, 140 had non-Hodgkin lymphoma, and one patient had composite lymphoma. In the Hodgkin lymphoma group, nodular sclerosis type was the most frequent type (48.75%), followed by mixed-cellularity type (27.5%), and nodular-lymphocyte predominant type (16.25%). In the non-Hodgkin lymphoma group, 124 (88.6%) cases were B-cell lymphomas, while the remaining were T-cell lymphomas. Diffuse large B-cell lymphoma was the most frequent type of non-Hodgkin B-cell type lymphoma (55.7%), followed by follicular lymphoma (10%).  Conclusion: The distribution of lymphoma in Bahrain is similar to neighboring Middle East countries with a predominance of Hodgkin lymphoma and diffuse large B-cell lymphoma.

The Effect of Hydroxyurea Therapy in Bahraini Sickle Cell Disease Patients.

Hydroxyurea (HU) is used as a disease-modifying agent in sickle cell disease (SCD). Its beneficial effects have been ascribed to inhibition of the sickling process through increase of fetal hemoglobin (HbF) levels and influence on multiple factors affecting adhesion of erythrocytes to vascular endothelium. The present study investigates the effect of HU in SCD patients who were grouped on the basis of association with α- and ß-thalassemia using routine laboratory methods. A retrospective cross-sectional chart-review was done of 51 adult Bahraini SCD patients attending Salmaniya Medical Complex, Bahrain. Four sub-groups of cases were identified: (i) homozygous sickle cell anemia, 24 cases; (ii) SCD with microcytosis, 16 cases; (iii) sickle α-thalassemia, seven cases; and (iv) sickle ß thalassemia, four cases. Documented laboratory and clinical data included hemoglobin level (Hb), hematocrit (Hct), red cell indices, hemoglobin fractions, hospital admissions (frequency), number of inpatient-days, pain episodes (frequency) and red cell transfusion requirement (number of units). Pre- and post-treatment data were compared. Hydroxyurea treatment led to highly significant reduction of HbS % and pain crisis episodes in all patient groups. Other changes such as increases of total hemoglobin, Hct and HbF and reduction of hospital admissions, inpatient days and red cell units transfused also occurred but with less consistent levels of significance within patient sub-groups. Treatment with HU is beneficial for all subgroups of Bahraini SCD patients, without or with α- and ß-thalassemia interactions.

Effect of GC METALPRIMER II on bond strength of heat-cured acrylic resin to titanium alloy (Ti-6Al-4V) with two different surface treatments.

This study investigated the bond strength of heat-polymerized acrylic resin to titanium alloy using a proprietary bonding agent (GC Metalprimer II). Two surface treatments (sandblasted or roughened with a tungsten carbide bur) were compared for their effect on bond strength with or without thermal cycling. Eighty specimens of heat-polymerized acrylic resin bonded to titanium alloy (Ti-6Al-4V) were prepared: 20 specimens (control) and 60 used a bonding agent. Four-point bend testing was used to record the load at failure. A chemical bond between heat-polymerized resin and alloy was achieved using GC Metalprimer II with both surface treatments. Reduced failure loads were recorded after thermal cycling. The predominant mode of failure was cohesive. GC Metalprimer II was effective in achieving a chemical bond with either the sandblasted or roughened surfaces.

Primitive neuroectodermal tumor or small cell carcinoma of the kidney, a rare neoplasm: case report.

Small cell carcinoma is a malignancy primarily recognized in the broncho-pulmonary region. Extrapulmonary locations are extremely uncommon. We report here a case of renal tumor encountered in a 34-year-old female, with extensive metastases in liver, lung and bone. Histological examination was most compatible with primitive neuro-ectodermal tumor (PNET) small cell carcinoma. There were negative immunohistochemcal markers for cytokeratin, any hormonal peptides and epithelial membrane antigens, which is consistent with the designation of the neoplasm as PNET. Previously reported cases have all been in the elderly and, to the best of our knowledge, this is the first case of proven PNET of the kidney described in a young female.