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
Background: The skull base and the hard palate contain many anatomical features that make them rich in information which are useful in sex differentiation; in addition to that they have the ability to resist the hardest environmental conditions that support them in making sex differentiation. Three dimensional computed tomographic techniques has important role in differentiation between sex since it offers images with very accurate data and details of all anatomical structures with high resolution. This study was made to study sex variations among Iraqi sample by craniometric linear measurements of the hard palate and the skull base using 3D reconstructed Computed Tomographic scan. Materials and methods: This study composed of 100 Iraqi su
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Reducing of ethyl 4-((2-hydroxy-3-methoxybenzylidene)amino)benzoate (1) afford ethyl 4-((2-hydroxy-3-methoxybenzyl)amino)benzoate (2). Reaction of this compound with Vilsmeier reagent affords novel 2-chloro-[1,3] benzoxazine ring (3). The corresponding acid hydrazide of compound 3 was synthesized from reaction of compound (3) with hydrazine hydrate. Newly series of hydrazones (5a–i) were synthesized from reaction of acid hydrazide with various aryl aldehydes. Antibacterial activity of the hydrazones was secerned utilizing gram-negative and gram-positive bacteria. Compound (5b) and (5c) exhibited significant antibacterial ability against both gram-negative and gram-positive bacteria, while the compounds (5a) showed mild antibacteri
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Activated carbon derived from Ficus Binjamina agro-waste synthesized by pyro carbonic acid microwave method and treated with silicon oxide (SiO2) was used to enhance the adsorption capability of the malachite green (MG) dye. Three factors of concentration of dye, time of mixing, and the amount of activated carbon with four levels were used to investigate their effect on the MG removal efficiency. The results show that 0.4 g/L dosage, 80 mg/L dye concentration, and 40 min adsorption duration were found as an optimum conditions for 99.13% removal efficiency. The results also reveal that Freundlich isotherm and the pseudo-second-order kinetic models were the best models to describe the equilibrium adsorption data.
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A phytoremediation experiment was carried out with kerosene as a model for total petroleum hydrocarbons. A constructed wetland of barley was exposed to kerosene pollutants at varying concentrations (1, 2, and 3% v/v) in a subsurface flow (SSF) system. After a period of 42 days of exposure, it was found that the average ability to eliminate kerosene ranged from 56.5% to 61.2%, with the highest removal obtained at a kerosene concentration of 1% v/v. The analysis of kerosene at varying initial concentrations allowed the kinetics of kerosene to be fitted with the Grau model, which was closer than that with the zero order, first order, or second order kinetic models. The experimental study showed that the barley plant designed in a subsu
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Reducing of ethyl 4-((2-hydroxy-3-methoxybenzylidene)amino)benzoate (1) afford ethyl 4-((2-hydroxy-3-methoxybenzyl) amino)benzoate (2). Reaction of this compound with Vilsmeier reagent affords novel 2-chloro-[1,3] benzoxazine ring (3). The corresponding acid hydrazide of compound 3 was synthesized from reaction of compound (3) with hydrazine hydrate. Newly series of hydrazones(5a–i) were synthesized from reaction of acid hydrazide with various aryl aldehydes. Antibacterial activity of the hydrazones wassecerned utilizing gram-negative and gram-positive bacteria. Compound (5b) and (5c) exhibited significant antibacterial ability against both gram-negative and gram-positive bacteria, while the compounds(5a) showed mild antibacterial activit
... Show MoreThe research included the preparation of cyclic compounds from thiazoles, imidazoles and oxazepines from the reaction of cyclization starting material that acts Schiff bases, which is a raw material in the formation of cyclic compounds from Schiff's(B1) by reaction of 4- aminobenzenesulfonylamide with 4-hydroxyacetophenone which can used to synthesized two lines. The first introducing the preparation of pyrazoles [B4, B5] from ester [B2], which derived to acid hydrazide[B3] with hydrazine hydrate and final pyrazoles obtained by the reaction with diethylmalonate and acetylacetone. The second including prepared the new 1,3-oxazepine1,5-dione derivatives[B6,B7,B8] from adding different anhydrides to the base[B1] as a seven membered ring ; te
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In this work ,pure and doped(CdO)thin films with different concentration of V2O5x (0.0, 0.05, 0.1 ) wt.% have been prepared on glass substrate at room temperature using Pulse Laser Deposition technique(PLD).The focused Nd:YAG laser beam at 800 mJ with a frequency second radiation at 1064 nm (pulse width 9 ns) repetition frequency (6 Hz), for 500 laser pulses incident on the target surface At first ,The pellets of (CdO)1-x(V2O5)x at different V2O5 contents were sintered to a temperature of 773K for one hours.Then films of (CdO)1-x(V2O5)x have been prepared.The structure of the thin films was examined by using (XRD) analysis..Hall effect has been measured in orded to know the type of conductivity, Finally the solar cell and the effici
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The research examines the mechanism of application of )ISO 21001: 2018( in the Energy Branch- Electromechanical Engineering at the University of Technology to achieve the quality of the educational service to prepare the branch to obtain the certificate of conformity with the requirements of) ISO 21001: 2018(, the necessary data were collected Depending on the (CHEKLIST) of (ISO 21001: 2018), field interviews and records of the concerned department, The researchers reached a number of results, the most prominent of which was the adoption of high quality leadership leaders and their willingness to implement the standard requirements, The university has a basic structure that qualifies it to implement the international standard, as
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Natural Bauxite (BXT) mineral clay was modified with a cationic surfactant (hexadecy ltrimethy lammonium bromide (BXT-HDTMA)) and characterized with different techniques: FTIR spectroscopy, X-ray powder diffraction (XRD) and scanning electron microscopy (SEM). The modified and natural bauxite (BXT) were used as adsorbents for the adsorption of 4- Chlorophenol (4-CP) from aqueous solutions. The adsorption study was carried out at different conditions and parameters: contact time, pH value, adsorbent dosage and ionic strength. The adsorption kinetic (described by a pseudo-first order and a pseudo-second order), equilibrium experimental data (analyzed by Langmuir, Freundlich and Temkin isotherm models) and thermodynamic parameters (change in s
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