Denosumab versus zoledronic acid for patients with beta-thalassemia major-induced osteoporosis.

ABSTRACT: The main aim of this study is to compare the 2 medications denosumab and zoledronic acid for patients with beta-thalassemia major induced osteoporosis. Patients with B-thalassemia major induced osteoporosis will undergo baseline assessment of the bone densitometry by bone density(DEXA) scan as a standard of care by the radiology department, then a blood test for bone-specific alkaline phosphatase and type-1 collagen telopeptide will be measured by the chemistry laboratory.Patients with B-thalassemia major induced osteoporosis, who are 18 years of age or more and willing to participate in the study will be enrolled after consenting by the primary investigator in hematology outpatient clinics. Patients with osteoporosis will receive 1 of the 2 medications; at the end of the year, DEXA scan will be done to compare the response of the 2 medications. The potential risks include drug-related side effects.The outcome will be measured biochemically by measuring bone-specific alkaline phosphatase and type 1 collagen carboxy telopeptide and radiologically by DEXA scan at baseline and 1 year using Z score.

A Case of Acquired von Willebrand Disease Secondary to Myeloproliferative Neoplasm.

Acquired von Willebrand Disease (AVWD) is a rare disorder in which qualitative or quantitative defects in von Willebrand factor (VWF) occur secondary to other conditions. AVWD occurs in patients with myeloproliferative disorders due to formation of autoantibodies against VWF and development of excessive shear stress causing disruption of VWF multimers. AVWD is different from congenital VWD in its acute onset and absence of family history. We report a 42-year-old gentleman with essential thrombocythemia, who was on cytoreductive therapy with hydroxyurea, and presented with an acute history of gum bleeding with hemoptysis, without any antecedent trauma or infections. His platelet count was very high, and prothrombin time and activated partial thromboplastin time were prolonged. The VWF ristocetin cofactor assay (VWF: RCo) was low, but VWF antigen level (VWF: Ag) was normal. Their ratio (VWF: RCo/VWF: Ag) was much lower than the acceptable lower limit. Treatment in AVWD is focused on addressing the underlying disorder. Early recognition of AVWD and its primary cause is mandatory in providing adequate therapy and achieving a cure.

Graves' Disease-Related Pancytopenia Improved after Radioactive Iodine Ablation.

Graves' disease is an autoimmune disease that affects the thyroid glands which often results in enlarged thyroid glands, and it is the most common cause of clinical hyperthyroidism especially in young patients. Radioiodine ablation is a radiation therapy in which radioactive iodine is administered to destroy or ablate thyroid cells. It is commonly used for the treatment of Graves' disease. We report on a 39-year-old male, who presented with Graves' disease, found to have pancytopenia and hypocellular bone marrow. Pancytopenia is a rare complication of thyrotoxicosis that is usually not severe and does not require supportive blood product transfusions. Our patient was treated with antithyroid medications followed by radioactive iodine ablation followed by a spontaneous recovery of pancytopenia.

Reference Jitter Values for Concentric Needle Electrode of Orbicularis Oculi and Frontalis Muscles Using Voluntary Activation Method in Sudanese Population.

Single fibre electromyography is the most sensitive neurophysiological test for the diagnosis of neuromuscular junction disorders, particularly myasthenia gravis. The study aimed at establishing concentric needle (CN) normal jitter values for voluntarily activated orbicularis-oculi (V-OOc) & Frontalis (V-FRO) muscles in Sudanese population. 57 healthy volunteers (20 males & 37 females) were included in the study (mean Age 43.6 ± 14.2 years, range 18-70 years). V-OOc and V-FRO were tested in the same individual using CN. Jitter values were expressed as the mean consecutive difference (MCD) of 30 potential pairs in microseconds. The mean jitter, mean individual fibre pairs jitter & mean outliers jitter values with (upper 95% Confidence Limit-CL) for [OOc] were [26.9 ± 3.3 (31.97), 26.1 ± 8.9 (41.8) & 38.5 ± 5.7 (49.0) µs] & for [FRO] were [27.1 ± 3.0 (31.32), 26.4 ± 9.4 (42.9) & 39.9 ± 5 (49.2) µs] respectively. The suggested practical upper limits for mean jitter & for outliers were (32, 49 µs) for OOc & (31, 49 µs) for FRO. Our CN-jitter values were within the range of the few published studies. The study was unique in that it established and compared between CN reference jitter values of two voluntarily activated facial muscles (V-OOc & V-FRO) in the same individual in large number of healthy subjects.

Reliability of physiology MCQs' examinations at the Faculty of Medicine, University of Khartoum

Background: One of the major challenges that face exam constructors is generating highly reliable exams. An assessment cannot be viewed as valid unless it is reliable. Test reliability, which is the best single measure of test accuracy, is the extent to which test results are consistent, stable, reproducible and free of error variance. Reliability isinfluenced by internal factors related to exam construction, as well as external factors which depend on the situation of test administration. Objective: To estimate the reliability of multiple choice questions (MCQs) of physiology exams as part of an overall quality assessment at the Faculty of Medicine, University of Khartoum.Methods:Reliability influential factors related to exam construction and administration were controlled and catered for by departmental and administrative staff according to the exam regulations of the faculty. Remark software was used for post-examination analysis of scores of ten consecutive summative physiology MCQ exams at the Faculty of Medicine, University of Khartoum. The number of the examinees who sat for each of the ten exams ranged from 332­359. In addition to reliability coefficients, item difficulty index (DIF I) and point-biserial correlation coefficient (rpbis)as a measure of item discrimination ability, were calculated as part of item analysis results.Results:The study revealed high exams' reliability (0.84-0.95) as measured by different formulas [Kuder-Richard-son Formulas (KR-20, KR-21) and Cronbach's-Alpha], and low standard error of measurement/SEM (3.073.80).Factorswhichcontributed to the high reliability of our ten exams were: their high discrimination power (0.32-0.47), their recommended mean difficulty (48.62-65.67%), and the relatively large numbers of items (60­80) per each exam.Conclusion:The high exams reliability of this study was an indicator of the precise control of external and internal factors influencing reliability. The most important contributing factor was the proper construction of exams with high quality items; in addition to careful exam administration and meticulous scoring system

Prevalence of toxoplasmosis in humans and domestic animals in Ahwaz, capital of Khoozestan Province, south-west Iran.

From 1984 to 1988 sera from 1806 apparently healthy persons and 1096 patients suspected of toxoplasmosis in and around Ahwaz city, capital of Khoozestan province, Iran were examined by immunofluorescent antibody test (IFA). Prevalence of Toxoplasma antibodies at a titre of < or = 1:20 was found in 49.6% of healthy persons compared to 72.3% in suspected patients. The seropositivity rate was lowest in the new-born and children under 6 months of age and highest in persons 14-19 years old. Blood samples from 142 cattle, 138 sheep and 130 goats, collected from Ahwaz abattoir, were tested by the latex-agglutination test (LAT). The infection rates were 14.8, 13.8 and 13.1% respectively. The consumption of uncooked meat seems to be the most probable cause of the high infection rate of toxoplasmosis in the area.