Review on Hydroxyurea Usage in Young Children with Sickle Cell Disease: Examining Hemoglobin Induction, Potential Benefits, Responses, Safety, and Effectiveness

Sickle cell disease (SCD) is a prevalent condition, particularly in the countries of sub-Saharan Africa, where the presence of specific genes associated with Malaria contributes to its high prevalence. Patients with sickle cell disease frequently experience painful episodes necessitating hospitalization, and their hemoglobin levels are typically lower than those of the general population. There are different treatment options available to manage complications, such as transfusing blood, hydroxyurea, and strong anti-pains. However, with all these treatments, patients still commonly experience pain crises and suffer from organ damage. Hydroxyurea, the sole approved medication for sickle cell anemia in developed and developing countries, is widely used in children despite being primarily indicated for adults. Multiple studies have demonstrated the efficacy of hydroxyurea in inducing HbF production in young children with SCD. Elevated HbF levels have been associated with improved clinical outcomes, including a reduction in vaso-occlusive crises, acute chest syndrome, and the need for blood transfusions. Furthermore, increased HbF levels have been shown to ameliorate disease-related organ damage, such as pulmonary hypertension and sickle cell retinopathy. The response to hydroxyurea treatment in young children with SCD is variable. Some patients achieve substantial increases in HbF levels and experience significant clinical benefits, while others may have a more modest response. Factors influencing the response include baseline HbF levels, genetic modifiers, treatment adherence, and dose optimization. Safety is a crucial consideration when using hydroxyurea in young children. Studies have shown that hydroxyurea is generally well-tolerated, with the most common adverse effects being myelosuppression, gastrointestinal symptoms, and dermatological manifestations. However,long-term effects and potential risks, such as renal dysfunction and reproductive impacts, require further investigation. The effectiveness of hydroxyurea in young children with SCD has been demonstrated in various clinical trials and observational studies. These studies have shown a significant reduction in disease-related complications and improved quality of life. However, optimal dosing, treatment duration, and long-term outcomes are still areas of ongoing research. This review focuses on recent studies investigating the benefits, effectiveness, responses, and safety of hydroxyurea in pediatric individuals diagnosed with sickle cell disease.

Analysis of the Effect of High-Dose Segmental Citrate Anticoagulation in High Flux Hemodialysis

Objective:To analyze the clinical effect of high-dose citrate in segmental extracorporeal anticoagulation for high-throughput hemodialysis.Methods:The subjects included in this study were admitted to the hospital for maintenance hemodialysis treatment from January 2021 to January 2023.All patients had a high risk of bleeding and received 4%trisodium citrate anticoagulant treatment,administered at a rate of 200 mL/h before and after the dialyzer.The anticoagulant effects achieved by the patients were observed and analyzed.Results:The total number of patients who received high-dose segmented citrate extracorporeal anticoagulation dialysis treatment was 50,with each patient undergoing 100 treatments.During the treatment,2 patients had to end the treatment early due to transmembrane pressure exceeding 30 mmHg and an increase in venous pressure exceeding 250 mmHg;the treatment times for these patients were 20 minutes and 200 minutes,respectively.The remaining patients successfully completed the 4-hour treatment.Blood pH and calcium ion concentration in the venous pot were monitored.It was observed that before dialysis,after 2 hours of dialysis,and at the end of dialysis,the blood pH of the patients remained within a relatively normal range.Although some patient levels changed after dialysis,they remained within the normal range.No adverse reactions(such as numbness of the limbs or convulsions)were observed during the anticoagulant treatment.Conclusion:Administering 4%trisodium citrate at a rate of 200 mL/h before and after the dialyzer achieves a good anticoagulant effect,maintains the patient’s blood gas levels within the normal range at the end of dialysis,and causes no adverse reactions.

Public Effective Dose Assessment Using Gross Alpha and Beta Radioactivity Levels of Tap Drinking Water in the District of Abidjan, Cote d’Ivoire

In order to assess public effective dose due to gross alpha and beta in water, 43 tap water samples were collected from different areas in the District of Abidjan. Using the low background Gas-less Automatic Alpha/Beta counting system (Canberra iMaticTM) for analysis, the gross alpha and beta concentrations found varied from 0.001 ± 0.002 to 0.063 ± 0.050 Bq/l with an average of 0.013 ± 0.012 Bq/l and from 0.067 ± 0.080 to 0.320 ± 0.120 Bq/l with an average of 0.174 ± 0.076 Bq/l, respectively in samples. The public effective dose assessment showed values of dose to ingestion of alpha and beta emitter radionuclides lower than the recommended value of dose for drinking water 0.1 mSv/y, except in 30% of the samples. These results show the need for additional studies to be conducted in order to clarify the hazardousness of these water samples. However, this study still remains important because it has provided necessary data for future tap water quality monitoring studies in the District of Abidjan.

Effectiveness of COVID-19 Vaccine Doses in Children: Case of Lake Region Economic Bloc-Kenya

Introduction: Vaccination of children has experienced delays due to paucity of information regarding safety, effectiveness, immunogenicity, and reactogenicity. Age wise approval prioritized 12 - 17 years and later 5 - 11 years. Those below 5 years possess na?ve immunity and not considered. In Lake Region Economic Bloc children aged 12 - 17 variably received 1, 2, and 3 doses of vaccine. This analysis looks into effectiveness of the doses administered. Method: Data providers from 84 LREB facilities submitted patients’ vaccination data to Power BI supported dashboard between June 24, 2021 and July 30, 2022. Data of 12 - 17 years old was mined, analyzed and visualized. Sample sizes considered for analysis were 0 dose, n = 8132;1 dose, n = 271;2 doses, n = 402, and 3 doses, n = 90. Data used in the analysis was facility operational and not from experimental design. Relative risk analysis of children who received 0, 1, 2, and 3 doses was done using Odds Ratio run on R software. Results: The relative risk of infection to a child with one dose against unvaccinated counterpart is 0.92 (95% CI, 0.61 - 1.43). Likewise the relative risk of infection to a child aged 12 - 17 years with 2 doses against another who received no dose is 0.87 (95% CI, 0.63 - 1.24). A child with 3 doses is 46% (95% CI, 27% - 84%) less likely to get infected compared to another not vaccinated. Also, the relative risk between having 2 doses and 1 dose for a child aged 12 - 17 years is 0.95 (95% CI, 0.55 - 1.6). For the same age group the relative risk of having 3 doses of vaccines against 1 dose is 51% (95% CI, 26% - 100%). In addition, a child who receives 3 doses of vaccine is 53% (95% CI, 28% - 100%) less likely to experience breakthrough infection compared to another with 2 doses. Whereas 1st dose offers (5%) marginal protection advantage over the 2nd dose, the 3r dose offers 49% and 47% more protection over 1st and 2nd doses, respectively, because of incremental reduced risk of infection gained from previous doses. During the period, 15 children at risk were admitted with COVID-19 infections in various regional hospitals, one had 3 doses but confounded with severe comorbidity. Conclusion: We found that 2nd dose had marginal protection over the 1st dose. However, the 3rd dose offers extensive protection compared to 1st and 2nd doses, and protects more against hospitalization. Children at risk should receive 3 doses of vaccines.

Effective Dose Levels from Computed Tomography of the Head during Contrast Studies in Nigeria

Background: Diagnostic reference levels for a number of common diagnostic radiological examinations against which individual centres could compare their performance have been recommended by relevant international agencies. Due to variations in different populations globally, local and national diagnostic reference levels are more reliable. To the best of our knowledge, no centre-specific study has been carried out and national surveys are not available. Objective: To establish a preliminary local and national diagnostic reference level in Nigeria. Methods: A pro-spective and cross-sectional study involving 30 conscious paediatrics and adult patients referred for head computed tomography scan. They were positioned supine and scanned according to the standard protocol for head computed tomography with manual mA selection. The total dose-length products were recorded at the end of the pre-contrast and post-contrast sequences respectively. The pre-contrast dose was taken into cognizance in the determination of the post-contrast value. The effective dose was established by multiplying the dose-length product by 0.0023 mSv.mGy-1.cm-1, a conversion coefficient for brain tissue adopted from the European Commission. Statistical Package for Social Sciences version 17.0 was used to analyze the data. Results: 30 paediatrics and adult patients of mixed gender participated in the study. Their ages ranged from 1 to 74 years with a mean age of 41.47 ± 23.30 years. The pre-contrast effective dose ranged from 1.93 mSv to 3.32 mSv with mean of 2.56 ± 0.51 mSv and 75th percentile of 3.11 mSv while the post-contrast effective dose ranged from 4.06 mSv to 6.97 mSv with mean of 5.27 ± 0.97 mSv and 75th percentile of 6.13 mSv. The mean effective dose from this work and two other isolated studies was 3.0 mSv. Conclusion: Although our quantified doses are below threshold limits for occupational exposures they are higher than the recommended level for the public. A further optimization of scanning protocols by the radiographers could lower the effective dose for patients undergoing contrast head computed tomography in our centre and in the country.

Laser-assisted Simulation of Dose Rate Effects of Wide Band Gap Semiconductor Devices

Laser-assisted simulation technique has played a crucial role in the investigation of dose rate effects of silicon-based devices and integrated circuits,due to its exceptional advantages in terms of flexibility,safety,convenience,and precision.In recent years,wide band gap materials,known for their strong bonding and high ionization energy,have gained increasing attention from researchers and hold significant promise for extensive applications in specialized environments.Consequently,there is a growing need for comprehensive research on the dose rate effects of wide band gap materials.In response to this need,the use of laser-assisted simulation technology has emerged as a promising approach,offering an effective means to assess the efficacy of investigating these materials and devices.This paper focused on investigating the feasibility of laser-assisted simulation to study the dose rate effects of wide band gap semiconductor devices.Theoretical conversion factors for laser-assisted simulation of dose rate effects of GaN-based and SiC-based devices were been provided.Moreover,to validate the accuracy of the conversion factors,pulsed laser and dose rate experiments were conducted on GaN-based and SiC-based PIN diodes.The results demonstrate that pulsed laser radiation andγ-ray radiation can produce highly similar photocurrent responses in GaN-based and SiC-based PIN diodes,with correlation coefficients of 0.98 and 0.974,respectively.This finding reaffirms the effectiveness of laser-assisted simulation technology,making it a valuable complement in studying the dose rate effects of wide band gap semiconductor devices.

Synergistic effect of total ionizing dose on single-event gate rupture in SiC power MOSFETs

The synergistic effect of total ionizing dose(TID) and single event gate rupture(SEGR) in SiC power metal–oxide–semiconductor field effect transistors(MOSFETs) is investigated via simulation. The device is found to be more sensitive to SEGR with TID increasing, especially at higher temperature. The microscopic mechanism is revealed to be the increased trapped charges induced by TID and subsequent enhancement of electric field intensity inside the oxide layer.

Estimation of Annual Effective Dose Due to Ingestion of Natural Radionuclides in Cattle in Tin Mining Area of Jos Plateau, Nigeria

Contamination of land could occur during the extraction and handling of materials containing high levels of naturally occurring radionuclide materials. These radionuclides find their ways into the food chain. The activity concentrations of natural radionuclides 40K, 226Ra, and 232Th in heart, liver, kidney, lungs, spleen and beef of cattle slaughtered and consumed in a tin mining area of Jos Plateau, Nigeria were determined by gamma ray spectroscopy method. The activity concentration of 40K is the highest in all the samples. The annual effective dose to man through the ingestion of the radionuclides in the organs was estimated. The mean annual effective doses calculated are 35.35 ± 13.84, 57.89 ± 38.27 and 46.93 ± 10.28 μSv?y?1 for heart, liver and kidney, respectively. Those of lungs, spleen and meat are 28.44 ± 15.70, 48.34 ± 28.85 and 41.24 ± 3.56 μSv?y?1, respectively. These are of the order of two magnitudes higher than those obtained for food in Abeokuta, Nigeria.

Effects of Food Diet Preparation Techniques on Radionuclide Intake and Its Implications for Individual Ingestion Effective Dose in Abeokuta, Southwestern Nigeria

The radioactivity measurements in food crops and their diet derivatives and farm soil samples from Abeokuta, one of the elevated background radiation areas in Nigeria have been carried out in order to determine the concentration levels of natural radionuclides (40K, 226Ra and 232Th). The activity concentrations of the natural radionuclides in the samples were determined via gamma-ray spectrometry using a 76 mm × 76 mm NaI(Tl) detector. Different common food crops representing the major sources of dietary requirements to the local population were collected for the measurements. The collected food crops were prepared into their different derivable composite diets using preparation techniques locale to the population. Using available food consumption data and the activity concentrations of the radionuclides, the ingestion effective doses were evaluated for the food crops and diet types per preparation techniques. For the tuberous food crop samples, the annual ingestion effective doses in the raw and different composite diets were 0.02 - 0.04 μSv and cumulatively 0.04 - 0.05 μSv while in the non-tuberous crops the doses were 0.44 - 0.70 μSv and cumulatively greater than 1 μSv respectively. Results of the study indicate that method of diet preparation is seen to play a major role in population ingestion dose reduction especially for tuberous crops than in non-tuberous crops. The study also showed that more ingestion dose could be incurred in diets prepared by roasting techniques. The result of the study will serve as a useful radiometric data for future epidemiological studies in the area and for food safety regulations and policy implementations in the country.

Development of a scintillation detector for real-time measurement of space proton effective dose

In this study, a scintillation detector was developed to measure the space proton effective dose for astronauts based on the proton effective dose conversion coefficients provided by International Commission on Radiological Protection Report No. 116. In the Monte Carlo N-Particle Transport Code X(version 2.6.0) simulation process, by modulating the depth and solid angle of truncated conical holes in an iron shell from lower-energy protons to higher-energy protons, the energy deposited in the scintillator by isotropic protons was nearly proportional to the corresponding effective dose, with a maximum relative deviation of 13.28% at thirteen energy points in the energy range of 10–400 MeV. Therefore, the detector can monitor proton effective dose indirectly in real time by measuring the deposited energy. We calibrated the photoelectric conversion efficiency of the detector at the cobalt source, tested the response of the detector in the energy range of 30–100 MeV in unidirectional proton field, and validated the simulation with the experimental results.

Assessment of Organ Dose and Effective Dose from Head CT Examination in a Large Hospital in South-Southern Nigeria

The magnitude of radiation dose imparted to patients who underwent Head Computed Tomography examination in a large tertiary hospital in South-Southern Nigeria has been estimated. CT-ExPO dosimetry software was used to determine the effective and organ doses to the head region of 40 adult patients. Scanning parameters were retrieved from the CT monitor for both contrast-enhanced and non-contrast head CT examinations. The tube potential ranged from 100 kVp to 120 kVp, while the mAs ranged from 127 mAs to 202 mAs. The mean values of the volume Computed Tomography Dose Index (CTDIvol) and Dose Length Product (DLP) for the contrast-enhanced Head CT examination were 166.4 ± 39.6 mGy and 3568.6 ± 756.1 mGy·cm, respectively, while for the non-contrast examination they were 86.6 ± 30.4 mGy and 2102.3 ± 870.3 mGy·cm, respectively. The effective doses were higher for the contrast-enhanced study than for the non-contrast study by a factor of 1.6. Results were compared with the European Union reference doses and other published data and were found to be higher. Doses to the organs which comprise of brain, red bone marrow, thyroid and eye lens were also estimated. The high variation in the doses in this study may be due to differences in imaging protocols such as large range of mAs and scan lengths and also the algorithm of the scanner.

Indoor^(222)Rn Levels and Effective Dose Estimation of Academic Staff in izmir-Turkey

Objective To investigate the annual effective doses from indoor radon received by academic staff in the Faculty building. Methods Measurements of indoor radon concentrations were performed in the Arts and Sciences Faculty of Dokuz Eylul University for two surveys of about 1 month duration respectively using the SSNTD （Solid State Nuclear Track Detectors） method with LR115 detectors. Time integrated measurements comprised different locations inside the faculty building： classrooms, toilets, canteen and offices. Homes of academic staff were also tested for radon. Results The aritthmetic mean radon concentration is 161 Bq m-3 with a range between 40 and 335 Bq m-3 in the Faculty. Six offices and three classrooms have a radon concentration above 200 Bq m-3. The results show that the radon concentration in classrooms is generally higher than in offices. Based on the measured indoor radon data, the annual effective doses received by staff in the Faculty were estimated to range from 0.79 to 4.27 mSv, according to UNSCEAR methodology. The annual effective doses received by staff ranged from 0.78 to 4.20 mSv in homes. On average, the Faculty contributed 56% to the annual effective dose. Conclusion Reported values for radon concentrations and corresponding doses are within the ICRP recommended limits for workplaces.

Comparison of the ICRP 60 and ICRP 103 Recommendations on the Determination of the Effective Dose from Abdominopelvic Computed Tomography

The effective dose takes into consideration the amount of absorbed dose received by tissues, individual organs and also organ’s radiosensitivity. This study concentrates on the Commission’s regulations of 1991 and 2007 tagged ICRP 60 and ICRP 103 respectively, and seeks to suggest the better guideline for determination of detriment to patient especially from abdominopelvic computed tomography. With mean totals of 375.0 mSv for the ICRP 60 and 341.3 mSv for the ICRP 103 obtained from abdominopelvic computed tomography involving 20 different patients, a T-test calculated value of 6.716 was obtained and compared with the value in the T-table at 95% confidence limit and 18 degrees of freedom to confirm whether there is a significant difference in both ICRP 60 and 103 recommendations in the determination of the effective dose. Finally, it is concluded that there is a significant difference in the ICRP 60 and ICRP 103 as fewer effective doses are obtained from the ICRP 103 recommendations and this difference verifies that the ICRP 103 is more suitable for the determination of the effective dose.

Determination Uranium Concentrations and Effective Dose of Drinking Water for Nineveh Governorate—Iraq, Using Kinetic Phosphorescence Analyzer (KPA)

In this work, the concentrations of natural Uranium and the annual effective dose (ET in Sv·y-1) in drinking water for Nineveh Governorate in northern Iraq were determined using a pulsed-laser Kinetic Phosphorescence Analyzer (KPA). Furthermore, the relationship between pH for water samples and the concentration of Uranium was studied. The drinking water samples are taken approximately from all regions of Nineveh as;15 samples of tap water are taken from refinery stations, 13 samples of water samples are taken from wells that are used for drinking, and 18 samples of drinking water are withdrawn from wells in Adayyah region (this region is located near nuclear wastes site). Thus, the total studied samples are 46. The results of Uranium concentration for all samples ranged from 2.61 ± 0.08 to 9.14 ± 0.012 μg·L-1 with an overall average value about 5.87 ± 0.046 μg·L-1, and the total annual effective dose ranged from 2.3 to 8.04 μSv·y-1. The results also showed that the pH number increases with increasing Uranium concentration and have, in general, high values in well water.

Biologically-Effective-Dose of Tolpyralate and Tolpyralate plus Atrazine for Control of Multiple-Herbicide-Resistant Waterhemp [<i>Amaranthus tuberculatus</i>(Moq.) J. D. Sauer] in Corn

The biologically-effective-dose of tolpyralate, a new 4-hydroxyphenyl-pyruvate dioxygenase (HPPD)-inhibitor, applied alone or tank-mixed with atrazine, for the control of multiple-herbicide-resistant (MHR) waterhemp [Amaranthus tuberculatus (Moq.) J. D. Sauer] has not been studied in corn. Seven field experiments were conducted during a three-year period (2018, 2019, 2020) in Ontario, Canada with MHR waterhemp to determine: 1) the dose-response of MHR waterhemp to tolpyralate and tolpyralate plus atrazine, and 2) the relative efficacy of tolpyralate and tolpyralate plus atrazine to post-emergence corn herbicides, dicamba/atrazine (500/1000 g·ha−1) and mesotrione + atrazine (100 + 280 g·ha−1). Tolpyralate + atrazine (120 + 4000 g·ha−1) caused 13% corn injury at one site two weeks after application (WAA), which was observed as transient foliar chlorosis and bleaching of new leaves. At 12 WAA, the predicted dose of tolpyralate for 50% control of MHR waterhemp at Cottam and on Walpole Island was 8 and 2 g·ha−1, respectively;the predicted dose of tolpyralate + atrazine for 50% control of MHR waterhemp at Cottam and on Walpole Island was 5 + 160 and 1 + 21 g·ha−1, respectively. The difference in predicted dose at the two sites is likely due to differences in MHR density and resistance profile. Applied at the registered rate, tolpyralate (30 g·ha−1) and tolpyralate + atrazine (30 + 1000 g·ha−1) controlled MHR waterhemp similar to dicamba/atrazine and mesotrione + atrazine across sites. This study demonstrates that tolpyralate + atrazine, applied POST, provides season-long control of MHR waterhemp in corn.

Experimental and Simulated Effective Dose for Some Building Materials in France

The specific radioactivity of several building materials used in France, which is considered a direct exposure to radiation, has been assessed by γ-ray spectrometry. Corrected for coincidence summing and self-absorption effects, the values for 226Ra, 232Th and 40K were in the ranges 4 - 56, 3 - 72 and 9 - 1136 Bq·kg-1, respectively. The samples were found to have radium-equivalent activities between 5 and 245 Bq·kg-1. Values of 0.02 - 0.67 for the external and 0.03 - 0.82 for the internal hazard indexes were estimated. The calculated absorbed dose in air agrees closely with MCNPX simulations. The conversion of absorbed dose to annual effective dose gave values between 0.03 - 1.09 mSv·y-1. All these values are below action limits recommended by the International Commission on Radiological Protection. The materials examined would not contribute a significant radiation exposure for an occupant and thus are acceptable for construction.

Annual effective dose values from^137Cs activity concentrations in soils of Manisa,Turkey

In this study,the activity concentrations of the fallout radionuclide ^(137(Cs in 150 soil samples collected from 13 districts of Manisa Province were determined using a gamma ray spectrometer with a Na I(Tl) detector.The health risks to people resulting from external exposure were evaluated by estimating the outdoor absorbed gamma dose rate and annual effective dose rate(outdoor and indoor).The activity concentration of ^(137)Cs varied from 3.6 ± 2.0 to 124.1 ± 11.0 Bq kg^(-1),with an average value of43.3 Bq kg^(-1).The average values of the outdoor absorbed gamma dose rate and annual effective dose(outdoor plus indoor) were found to be 1.3 n Gy h^(-1),and 8.0 μSv y^(-1),(6.38 + 1.59 μSv y^(-1)),respectively.The results were compared with internationally recommended values.

Total Ionizing Dose Radiation Effects on MOS Transistors with Different Layouts

Both nMOS and pMOS transistors with two-edged and multi-finger layouts are fabricated in a standard commercial 0.6μm CMOS/bulk process to study their total ionizing dose （TID） radiation effects. The leakage current, threshold voltage shift, and transconductance of the devices are monitored before and after T-ray irradiation. Different device bias conditions are used during irradiation. The experiment results show that TID radiation effects on nMOS devices are very sensitive to their layout structures. The impact of the layout on TID effects on pMOS devices is slight and can be neglected.

Synergistic effect of total ionizing dose on single event effect induced by pulsed laser microbeam on SiGe heterojunction bipolar transistor

The synergistic effect of total ionizing dose（TID） on single event effect（SEE） in SiGe heterojunction bipolar transistor（HBT） is investigated in a series of experiments. The SiGe HBTs after being exposed to 60 Co g irradiation are struck by pulsed laser to simulate SEE. The SEE transient currents and collected charges of the un-irradiated device are compared with those of the devices which are irradiated at high and low dose rate with various biases. The results show that the SEE damage to un-irradiated device is more serious than that to irradiated SiGe HBT at a low applied voltage of laser test. In addition, the g irradiations at forward and all-grounded bias have an obvious influence on SEE in the SiGe HBT, but the synergistic effect after cutting off the g irradiation is not significant. The influence of positive oxide-trap charges induced by TID on the distortion of electric field in SEE is the major factor of the synergistic effect. Moreover, the recombination of interface traps also plays a role in charge collection.

Machine learning-based analyses for total ionizing dose effects in bipolar junction transistors

Machine learning methods have proven to be powerful in various research fields.In this paper,we show that research on radiation effects could benefit from such methods and present a machine learning-based scientific discovery approach.The total ionizing dose(TID)effects usually cause gain degradation of bipolar junction transistors(BJTs),leading to functional failures of bipolar integrated circuits.Currently,many experiments of TID effects on BJTs have been conducted at different laboratories worldwide,producing a large amount of experimental data which provides a wealth of information.However,it is difficult to utilize these data effectively.In this study,we proposed a new artificial neural network(ANN)approach to analyze the experimental data of TID effects on BJTs An ANN model was built and trained using data collected from different experiments.The results indicate that the proposed ANN model has advantages in capturing nonlinear correlations and predicting the data.The trained ANN model suggests that the TID hardness of a BJT tends to increase with base current I.A possible cause for this finding was analyzed and confirmed through irradiation experiments.