Pompe disease is a progressive, multisystemic, debilitating, often fatal neuromuscular disease caused by a pathogenic variant in the acid α-glucosidase gene leading to GAA enzyme deficiency and lysosomal glycogen accumulation. Objectives: This study aimed to determine the prevalence of early onset Pompe disease in Basra, using the dried blood spot (DBS) as a screening tool, also to determine the spectrum of presentation. Materials and Methods: In a prospective study conducted in Basrah, Iraq, from October 2021 to September 2023, all infants with a family member diagnosed as a case of Pompe disease, hypotonia, or ventricular hypertrophy referred to the pediatric cardiology unit in Basra Cardiac Hospital were subjected to echocardiographic examination and assessment of GAA enzyme level, and genetic study by dried blood spot. Results: Thirty patients with confirmed Pompe disease were evaluated (12 males and 18 females), and the mean age of presentation was 3.7 months. The level of CK raged from 123 to 1471 (mean 614.3 ± 247), and the level of GAA activity ranged from 0.0 to 0.3 (mean 0.123 ± 0.07). All infants were homozygous for mutations in GAA. The most commonly encountered mutation was c.1314C>A. Conclusion: Pompe disease is an underestimated disease in Iraq, and the delay in the diagnosis results in established, irreversible myopathic changes even with enzyme replacement therapy and results in high mortality, so a high index of suspicion and early diagnosis will help to provide proper therapy and will help to provide a better quality of life for such patients
A new derivatives of Schiff bases connected with 5H-thiazolo[3,4-b][1,3,4]thiadiazole ring 5a-c were prepared via many reactions starting by treating 1,4-phenylene diamine 1 with chloroacetylchloride to prepared compound 2, then reaction with p-hydroxybenzaldehyde to synthesize compound 3 then, this was reacted with thioglycolic acid and thiosemicarazide to giveN,N-(1.4-phenylene)bis(2-(4-(2-amino-5Hthiazolo[4,3-b][1,3,4]thiadiazol-5-yl)phenoxy)acetamide) 4. Compound 4 was treated with different aromatic aldehydes to give a new derivatives of Schiff bases containing 5H-thiazolo[3,4-b][1,3,4]thiadiazole ring 5a-c. The synthesized compounds were characterized using FTIR spectrophotometer and 1H NMR spectroscopy and the biological activity of
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Objective: This in vitro study is aimed to compare and evaluate the cyclic fatigue of four varying NiTi rotary instrumentation systems. Method: In this study, four types of rotary files were used in four groups (10 files for each group), namely, Group A: Wave One Gold; Group B: AF Blue R3; Group C: One Curve; Group D: F6 SkyTaper. These groups were evaluated by a cyclic fatigue apparatus to measure cyclic fatigue resistance within the artificial metallic simulating canal that has a 60 angle of curvature, the curvature radius was 5 mm, whereas the inner diameter of the canal was 1.5 mm. All the files were rotated in artificial canals until they fracture. The resistance to cyclic fatigue was determined by counting the number of cycles to frac
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Angle of arrival (AOA) estimation for wideband signal becomes more necessary for modern communication systems like Global System for Mobile (GSM), satellite, military applications and spread spectrum (frequency hopping and direct sequence). Most of the researchers are focusing on how to cancel the effects of signal bandwidth on AOA estimation performance by using a transversal filter (tap delay line) (TDL). Most of the researchers were using two elements array antenna to study these effects. In this research, a general case of proposed (M) array elements is used. A transversal filter (TDL) in phase adaptive array antenna system is used to calculate the optimum number of taps required to compensate these effect. The propo
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