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Objectives Although the painful and disabling features of early diffuse cutaneous SSc (dcSSc) have an inflammatory basis and could respond to corticosteroids, corticosteroids are a risk factor for scleroderma renal crisis. Whether or not they should be prescribed is therefore highly contentious. Our aim was to examine safety and efficacy of moderate-dose prednisolone in early dcSSc. Methods PRedSS set out as a Phase II, multicentre, double-blind randomized controlled trial, converted to open-label during the Covid-19 pandemic. Patients were randomized to receive either prednisolone (∼0.3 mg/kg) or matching placebo (or no treatment during open-label) for 6 months. Co-primary endpoints were the HAQ Disability Index (HAQ-DI) and modified Rodnan skin score (mRSS) at 3 months. Over 20 secondary endpoints included patient reported outcome measures reflecting pain, itch, fatigue, anxiety and depression, and helplessness. Target recruitment was 72 patients. Results Thirty-five patients were randomized (17 prednisolone, 18 placebo/control). The adjusted mean difference between treatment groups at 3 months in HAQ-DI score was −0.10 (97.5% CI: −0.29, 0.10), P  = 0.254, and in mRSS −3.90 (97.5% CI: −8.83, 1.03), P  = 0.070, both favouring prednisolone but not significantly. Patients in the prednisolone group experienced significantly less pain (P  = 0.027), anxiety (P  = 0.018) and helplessness (P  = 0.040) than control patients at 3 months. There were no renal crises, but sample size was small. Conclusion PRedSS was terminated early primarily due to the Covid-19 pandemic, and so was underpowered. Therefore, interpretation must be cautious and results considered inconclusive, indicating the need for a further randomized trial. Trial registration ClinicalTrials.gov , https://clinicaltrials.gov , NCT03708718. [ABSTRACT FROM AUTHOR]

Battery thermal management systems (BTMS) in hybrid electric vehicles can be complex and heavy. They tend to increase energy consumption, leading to higher carbon dioxide emissions. In this study, a new approach was investigated for the potential use of four fuel components as coolants for direct liquid-cooled (LC)-BTMS, N-Pentane, N-Hexane, N-Butane, and Cyclo-Pentane. The performance of the fuel components was numerically analysed and CFD modelled using ANSYS Fluent software. Several meshing iterations of the lithium-ion battery (LIB) module were performed to conduct mesh independence check for higher accuracy and less computational time. The LIB module was simulated, in comparison to a free air convection (FAC)-BTMS as a benchmark, at three discharge rates (1C, 1.5C, 2C) for each of the inlet velocity values (0.1, 0.5, 1 m/s). Results show that FAC-BTMS exceeded the LIB module optimal operating temperature range (293–313 K) at 2C. On average, at the worst condition (lowest inlet velocity and highest discharge rate), all fuel components of the LC-BTMS were able to maintain the LIB module temperature below 288 K. That is at least 4.7% cooler compared to FAC-BTMS, which renders the new approach viable alternative to the conventional BTMS. [ABSTRACT FROM AUTHOR]

Background: Organic acidurias are a group of inborn errors of metabolism. They present a significant diagnostic challenge and are associated with serious morbidity and mortality. They are considered the most frequent inborn errors of metabolism among high‐risk children. Gas chromatography–mass spectrometry is a reliable diagnostic technique for organic acidurias. This hospital‐based study aimed to quantify the frequency of organic acidurias among a group of high‐risk Egyptian pediatric patients and to highlight the importance of high‐risk screening for such disorders. Methods: One hundred and fifty high‐risk children who presented to the inherited metabolic disease unit and the pediatric intensive care units of Cairo University Children Hospital were tested for urine organic acids using gas chromatography–mass spectrometry. Results: Thirty percent (45/150) of the patients were confirmed as having an altered organic acids profile. Neurological manifestations were the most common presentation. Glutaric aciduria type I and maple‐syrup urine disease were the most common disorders encountered among the group that was studied. Conclusion: Organic acid detection by gas chromatography–mass spectrometry is key to the diagnosis of many metabolic disorders. Until a national expanded newborn screening program is established, high‐risk screening is strongly encouraged for the early detection of, and proper intervention for such disorders among Egyptian children. [ABSTRACT FROM AUTHOR]

Background: The incidence of Down syndrome (DS) in Egypt varies between 1∶555 and 1∶770 and its screening by triple test is becoming increasingly popular nowadays. Results, however, seem inaccurate due to the lack of Egyptian-specific information needed for risk calculation and a clear policy for programme implementation. Our study aimed at calculation and validation of the triple marker medians used in screening Egyptian females as well as to recommend programme conventions to unify screening in this country. Methods: The study was conducted on 668 Egyptian women, in weeks 15–20 of pregnancy as proven by sonar. Chorionic gonadotropin (CG), α-fetoprotein (AFP) and unconjugated oestriol (uE3) were measured on Siemens Immulite analyzer. Medians of the three parameters were calculated, regressed against gestational age (GA) and weighted by the number of participants/week. Equations were derived to adjust each parameter to the maternal weight and were centered on the median Egyptian weight. Prisca software was fed with the above data, multiples-of-median (MoM) and DS risks were calculated and the screening performance was evaluated at a mid-trimester risk cutoff of 1∶270. Results: Log-linear [AFP/uE3 = 10(A+B*GA)] and exponential equations [CG = A*e(B*GA)] were derived and the regressed medians were found to follow similar patterns to other Asian and Western medians. Oestriol was always lowest (even halved) while CG and AFP were intermediate. A linear reciprocal model best fitted weight distribution among Egyptians and successfully adjusted each parameter to a weight of 78.2 kg. Epidemiological monitoring of these recommendations revealed satisfactory performance in terms of 6.7% initial positive rate and 1.00 grand MoM. Conclusions: Adoption of the above recommendations is hoped to pave the way to a successful DS screening programme tailored to Egyptian peculiarities. [ABSTRACT FROM AUTHOR]

The etiology of Systemic lupus erythematosus (SLE) seems to be multifactorial including environmental as well as genetic factors. Major histocompatibility complex (MHC) genes especially HLA-DRB1 and HLA-DQB1 are strongly implicated in susceptibility to SLE. Moreover ethnicity has been found to have a significant role in both disease susceptibility and disease expression. This study was carried out to determine HLA-DRB1 allele association with SLE susceptibility and disease presentation in Egyptian children with juvenile onset SLE. HLA-DRB1 allele typing was done using polymerase chain reaction-sequence-specific oligonucleotide probe for 65 juvenile Egyptian SLE patients and 150 healthy controls. p-values were corrected for the number of the alleles tested (Pc). HLA-DRB1*15 g allele was significantly increased in SLE children versus controls (OR = 4.76; 95% CI = 1.83–12.4; p = 0.001 and Pc = 0.012). No HLA-DRB1 allele was found to be statistically significant associated with musculoskeletal, cutaneous, hematologic, cardiac or neuropsychiatric manifestations in SLE patients (p > 0.05). Moreover no statistically significant association was found between HLA-DRB1 alleles and clinical presentation or histologic classes of lupus nephritis. The current work suggests that HLA-DRB1*15g allele may be a susceptibility allele in Egyptian children with SLE but is not related to clinical presentation of SLE. [ABSTRACT FROM AUTHOR]

Background: Atrial fibrillation represents a common complication after coronary artery bypass and valvular surgery, although it is a benign arrhythmia it may contribute to the morbidity, high cost and prolonged ICU stay. Objectives: The purpose of this study is to investigate and analyze the incidence and risk factors associated with postoperative atrial fibrillation (POAF) and its impact on intensive care unit (ICU) and postoperative hospital stay in patients undergoing coronary artery bypass graft surgeries (CABG) at Ain Shams University hospitals using the medical records of patients who underwent(CABG) surgeries from July 2018 to July 2019. Patients and Methods: Our study was conducted in Ain Shams University hospitals from July 2018 to July 2019. This study was a retrospective non randomized on total 660 patients who undergone isolated Coronary artery bypass graft surgeries (CABG) during this period, of them we targeted 100 cases who developed POAF after surgery. Results: Our results show that age, history of hypertension,smoking, history of renal impairment and recent myocardial infarction were all predictors of atrial fibrillation after cardiac surgery. POAF developed more frequent in patients who had larger left atrium diameter and lower ejection fraction. Our present results indicate that there is significant association between longer bypass and cross clamping time and development of POAF. We also found that patients who had postoperative lower serum potassium experienced more frequent POAF than who had normal potassium levels. In our study, we found that use of preoperative beta blocking drugs reduces risk of developing POAF. Early electrocardiographic ischemic postop. changes was also associated with high risk to develop POAF. No significant relation between use of intra-aortic balloon pump and development of POAF. Also, no significant relation between POAF and development of stroke or thromboembolic manifestations as POAF is usually self limiting,transient and resolves spontaneously in most of cases. Conclusion: The current study found that low ejection fraction, dilated left atrium, hypertension, smoking, old age, male gender, prolonged bypass time, prolonged cross clamping time, renal impairment, previous myocardial infarction, ungrafted dominant right coronary artery, low temperatures on bypass, early ischemic changes postoperative, hypokalemia, use of adrenaline and nor adrenaline, all of these factors were a significant predictors for development of atrial fibrillation after cardiac surgery. [ABSTRACT FROM AUTHOR]

A 42-year-old Caucasian woman with SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) refractory to non-steroidal anti-inflammatory drugs, sulfasalzine, methotrexate, bisphosphonates and steroids was successfully treated with antitumour necrosis factor therapy (infliximab). This case was a diagnostic challenge leading to extensive investigations for infection and malignancy and delayed diagnosis for several years. We report the significant improvement in clinical, radiological and laboratory markers of disease activity on infliximab and the steroid sparing effect of such therapy. [ABSTRACT FROM AUTHOR]

Abstract: Objective: To study the role of VDR polymorphisms as risk factor for RA and osteoporosis, and whether osteoporosis complicating RA is due to RA or VDR polymorphisms. Methods: VDR gene polymorphisms ApaI, TaqI, BsmI and FokI were typed by RFLP for 128 RA patients, 30 postmenopausal osteoporotic females and 150 healthy controls. Results: Significant differences were found between patients and healthy controls in the frequency of BsmI and TaqI (Pc<0.05) but no significant associations were found for FokI and ApaI polymorphisms except for aa genotype (Pc<0.001). Titers of RF were higher with aa and bb genotypes. Anti-CCP and CRP levels were higher with aa genotype and more bone loss was associated with Bb genotype. Ff genotype frequency was higher in RA patients with osteoporosis than those without osteoporosis. Conclusions: The ApaI, BsmI and TaqI polymorphisms may be a susceptibility risk factors for RA and the Ff genotype may be responsible for development of osteoporosis in RA Egyptian patients. However, the present study needs to be replicated in a large number of patients from allover the Egypt and also in multi-ethnic populations. [Copyright &y& Elsevier]

• VDR gene Fok I AA (rs2228570) is associated with Hashimoto's thyroiditis. • VDR gene FokI AA (rs2228570) variant could have normal levels of 25−OH-vitamin D3. • Thyroid volume of cutoff ⋟ 4.8 ml can help in diagnosis of Hashimoto's thyroiditis. Hashimoto's thyroiditis (HT) is considered the predominant cause of hypothyroidism in iodine sufficient countries. The deficiency of 25−OH-vitamin D3 serum level and the variation of vitamin D receptor (VDR) gene were implicated in a number of autoimmune disorders. This study aimed to test the hypothesis linking between VDR Fok I and BsmI variants and HT, in addition to explain their impact on 25−OH-vitamin D3 serum level. Cross sectional study included 160 hypothyroid subjects, 112 patients with HT and 48 hypothyroid non-HT controls. They were diagnosed based on anti-TPO Ab and or anti-TG Ab results. All cases were subjected to full history taking, thyroid ultrasound examination and a panel of assays (TSH, f.T3, f.T4, anti-TPO Ab, anti-TG Ab, calcium, alkaline phosphatase and phosphate). Serum 25−OH-vitamin D3 was assayed using HPLC-UV method. VDR variants (FokI and BsmI) were genotyped using real-time PCR. FokI AA genotype was statistically higher in HT patients than control group (P value = 0.02) with subsequently higher serum 25−OH-vitamin D3 level in comparison to all other genotypes (P value = 0.039). Serum 25−OH-vitamin D3 level was statistically indifferent between HT and control group (P value = 0.223). A statistically significant increase in total thyroid volume was observed in HT group (P value = 0.002). FokI AA genotype is more associated with HT in Egyptian patients compared to hypothyroid non-HT controls. Moreover, patients with FokI AA genotype have statistically higher levels of 25−OH-vitamin D3 suggesting VDR dysfunction even in patients expressing normal level of vitamin D. [ABSTRACT FROM AUTHOR]

Background: While neonatal jaundice is generally a common benign condition; severe hyperbilirubinemia has a devastating potential for brain injury.Aim: To detect the impact of severe neonatal hyperbilirubinemia on motor and mental development and its progress over time in the first year of life using the Bayley scales of infant development (BSID) II.Study Design and Patients: 177 term/near-term infants admitted for neonatal hyperbilirubinemia to the NICU of Cairo University Children's Hospital were enrolled. Clinical examination, BIND score and laboratory tests were performed at admission. Neurodevelopmental assessment using BSIDΙΙ was performed at 3 months for 147/177 neonates, and at 6 months and 12 months for 139/177 neonates. Auditory brainstem evoked potential was recorded at 3 months of age and repeated if abnormal.Outcome Measures: Psychomotor (PDI) and mental developmental indices (MDI) using BSIDII. Auditory impairment using Auditory Brainstem Response (ABR).Results: TSB levels ranged from 10 to 63 mg/dL (179.6-1077 μmol/L) with a mean of 25.52 ± 6.5 mg/dL (436 ± 112.9 μmol/L) and BIND scores ranged from 0 to 7. By one year of age, 19/139 patients were affected; 8 had classic kernicterus, 3 had isolated auditory impairment, 1 had severe motor and mild mental delay and 7 had mild motor delay. TSB level and BIND score had positive correlation with auditory impairment and lower scores for PDI (which improved with time) and MDI (which remained stationary). Duration of exposure to hyperbilirubinemia negatively affected neurodevelopmental scores.Conclusion: The impact of severe hyperbilirubinemia is mainly on motor and auditory impairment. Mild mental delay was detected by BSIDII in few patients. Neurodevelopmental outcome improves over time. [ABSTRACT FROM AUTHOR]

Background and Objective: Bilirubin/albumin ratio (B/A) may provide a better estimate of free bilirubin than total serum bilirubin (TSB), thus improving identification of newborns at risk for bilirubin encephalopathy. The objective of the study was to identify thresholds and compare specificities of TSB and B/A in detecting patients with acute and posttreatment auditory and neurologic impairment., Methods: A total of 193 term/near-term infants, admitted for severe jaundice to Cairo University Children's Hospital, were evaluated for neurologic status and auditory impairment (automated auditory brainstem response), both at admission and posttreatment by investigators blinded to laboratory results. The relationships of TSB and B/A to advancing stages of neurotoxicity were compared by using receiver operating characteristic curves., Results: TSB and B/A ranged from 17 to 61 mg/dL and 5.4 to 21.0 mg/g, respectively; 58 (30%) of 193 subjects developed acute bilirubin encephalopathy, leading to kernicterus in 35 infants (13 lethal). Auditory impairment was identified in 86 (49%) of 173 infants at admission and in 22 of 128 at follow-up. In the absence of clinical risk factors, no residual neurologic or hearing impairment occurred unless TSB exceeded 31 mg/dl. However, transient auditory impairment occurred at lower TSB and B/A (22.9 mg/dL and 5.7 mg/g, respectively). Intervention values of TSB and B/A set at high sensitivity to detect different stages of neurotoxicity had nearly the same specificity., Conclusions: Both TSB and B/A are strong predictors of neurotoxicity, but B/A does not improve prediction over TSB alone. Threshold values detecting all affected patients (100% sensitivity) increase with advancing severity of neurotoxicity., (Copyright © 2014 by the American Academy of Pediatrics.)

Objective: Accelerated atherosclerosis and premature coronary heart disease (CHD) are recognized complications of systemic lupus erythematosus (SLE), but the exact etiology remains unclear and is likely to be multifactorial. We hypothesized that SLE patients with CHD have increased exposure to traditional risk factors as well as differing disease phenotype and therapy-related factors compared to SLE patients free of CHD. Our aim was to examine risk factors for development of clinical CHD in SLE in the clinical setting., Methods: In a UK-wide multicenter retrospective case-control study we recruited 53 SLE patients with verified clinical CHD (myocardial infarction or angina pectoris) and 96 SLE patients without clinical CHD. Controls were recruited from the same center as the case and matched by disease duration. Charts were reviewed up to time of event for cases, or the same "dummy-date" in controls., Results: SLE patients with clinical CHD were older at the time of event [mean (SD) 53 (10) vs 42 (10) yrs; p < 0.001], more likely to be male [11 (20%) vs 3 (7%); p < 0.001], and had more exposure to all classic CHD risk factors compared to SLE patients without clinical CHD. They were also more likely to have been treated with corticosteroids (OR 2.46; 95% CI 1.03, 5.88) and azathioprine (OR 2.33; 95% CI 1.16, 4.67) and to have evidence of damage on the pre-event SLICC damage index (SDI) (OR 2.20; 95% CI 1.09, 4.44). There was no difference between groups with regard to clinical organ involvement or autoantibody profile., Conclusion: Our study highlights the need for clinical vigilance to identify modifiable risk factors in the clinical setting and in particular with male patients. The pattern of organ involvement did not differ in SLE patients with CHD events. However, the higher pre-event SDI, azathioprine exposure, and pattern of damage items (disease-related rather than therapy-related) in cases suggests that a persistent active lupus phenotype contributes to CHD risk. In this regard, corticosteroids and azathioprine may not control disease well enough to prevent CHD. Clinical trials are needed to determine whether classic risk factor modification will have a role in primary prevention of CHD in SLE patients and whether new therapies that control disease activity can better reduce CHD risk.