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Introduction: Myotonic dystrophy type 1 (DM1) is a multisystemic genetic disorder caused by the increased number of CTG repeats in 3' UTR of Dystrophia Myotonia Protein Kinase (DMPK) gene. DM1 patients experience conduction abnormalities as well as atrial and ventricular arrhythmias with increased susceptibility to sudden cardiac death. The ionic basis of these electrical abnormalities is poorly understood. Methods: We evaluated the surface electrocardiogram (ECG) and key ion currents underlying the action potential (AP) in a mouse model of DM1, DMSXL, which express over 1000 CTG repeats. Sodium current (INa), L-type calcium current (ICaL), transient outward potassium current (Ito), and APs were recorded using the patchclamp technique. Results: Arrhythmic events on the ECG including sinus bradycardia, conduction defects, and premature ventricular and atrial arrhythmias were observed in DMSXL homozygous mice but not in WT mice. PR interval shortening was observed in homozygous mice while ECG parameters such as QRS duration, and QTc did not change. Further, flecainide prolonged PR, QRS, and QTc visually in DMSXL homozygous mice. At the single ventricular myocyte level, we observed a reduced current density for Ito and ICaL with a positive shift in steady state activation of L-type calcium channels carrying ICaL in DMSXL homozygous mice compared with WT mice. INa densities and action potential duration did not change between DMSXL and WT mice. Conclusion: The reduced current densities of Ito, and ICaL and alterations in gating properties in L-type calcium channels may contribute to the ECG abnormalities in the DMSXL mouse model of DM1. These findings open new avenues for novel targeted therapeutics. [ABSTRACT FROM AUTHOR]

Purpose: to evaluate the Retinopathy of prematurity prevalence and screening outcome in a tertiary hospital in Cairo, Egypt. Also, to assess the risk factors for its development and to suggest modifications to the international guidelines for screening for Retinopathy of prematurity to fit the Egyptian population. Methods: A prospective observational study was carried out in Neonatal Intensive Care Unit (NICU) in Ain Shams University Hospital. In this study 159 premature infants born in the period between 1 September 2018 to 1 September 2021 were screened for Retinopathy of Prematurity (ROP). Screening was based on the most inclusive criteria reported to date. All premature infants with gestational age (GA) of ≤34 weeks or birth weight (BW) of ≤2000 grams were included. Infants were also included if GA>34 weeks or BW>2000 grams, but multiple co-morbidities existed. The prevalence of retinopathy of prematurity and plus disease and their correlation with risk factors of interest were studied. Results: The gestational age of the included infants ranged from 27 to 36 weeks, with a mean (SD) of 31.87 (‡ 1.81) weeks. Infants had birth weight ranging from 640 to 3900 grams, with a mean (SD) of 1784.71 (± 560.30) grams. The prevalence of ROP changes more than stage 0 in the screened infants was 25.8% (41 infants) with 7.3% of cases (11 infants) showing plus disease and 6.3% of cases (10 infants) showing severe changes that needed treatment. Of those, 2 cases (20%) fell outside the British Guideline's criteria for Screening. there was a highly significant (p<0.0001) correlation between appearance of Retinopathy of prematurity changes more than stage 0 and low gestational age, low birth weight of the screened infants, receiving mechanical ventilation, respiratory distress syndrome stage, presence of Necrotizing enterocolitis, Intraventricular haemorrhage and blood transfusion. On the other hand, no significant correlation was found between appearance of Retinopathy of prematurity changes more than stage 0 and gender (p=0.911), presence of PDA (p=0.187), or sepsis (p=0.998). Conclusion: Retinopathy of prematurity is a significant problem in the premature infants in Cairo, Egypt. Extremely premature infants with lower birth weight are more prone to develop this disease. However, cases with higher gestational age and birth weight than mentioned in the British guidelines screening criteria especially with multiple comorbidities showed severe Retinopathy of prematurity changes that needed intervention, which implies the need to develop a screening guideline for the Egyptian population. [ABSTRACT FROM AUTHOR]

Assessment of myocardial ischemia and viability is now mandatory for treatment decisions in patients with coronary artery disease (CAD). The high subjectivity of the noninvasive imaging tools such as dobutamine stress cardiovascular magnetic resonance (DS-CMR) in viability and ischemia assessment and late gadolinium enhancement (LGE) in scar transmurality detection, as well as the risks imposed by the contrast administration in patients with renal affection, paved the way for the evolution of more objective and safer techniques. Strain analysis provides quantifiable measurable values of the degree of myocardial deformation. Feature tracking-cardiac magnetic resonance (FTCMR) is a novel strain analysis technique that uses only routinely acquired cine images in strain analysis. Our study aimed to assess the quantitative ability of the CMR-FT in assessment of myocardial ischemia and viability in patients with CAD. We investigated 20 patients (n=320 myocardial segments), known or suspected CAD. DS-CMR and LGE were used to identify the viable non-ischemic, ischemic and non-viable myocardial segments. Then calculations of the rest segmental radial (Err), circumferential (Ecc) and longitudinal (Ell) strain were done by manual contouring of endocardial and epicardial borders using Segment Software. Results: Based on the results of both DS-CMR and LGE of the 320 myocardial segments, 210 segments were defined as viable non-ischemic (remote), 71 segments were viable ischemic and 39 segments were non-viable. Rest segmental Ecc, Err and Ell values were statistically significantly reduced in the non-viable (mean6 SD = -3.98 6 5.08%, 12.26 6 12.62% and - 7.39 6 7.05%, respectively) compared to both viable groups, p<0.001. Furthermore, segmental Ecc and Err significantly differentiated between non-ischemic and ischemic group (mean6 SD = -18.60 6 7.09% vs -13.49 6 8.53% and 44.09 6 20.38% vs 32.21 6 16.92% respectively), p1<0.001. However, Ell was weak to find a statistical significance between them, despite showing lower values in the ischemic group (mean6 SD = -16.17 6 8.86% vs -15.27 6 9.82%, p=0.741). Conclusions: FT-CMR strain analysis can provide a more objective metric in myocardial strain analysis to differentiate between viable and non-viable as well as ischemic and nonischemic myocardial segments. Therefore, such technique has a promising objective role in ischemia and viability assessment in conjunction with CMR or even may replace it in the future. [ABSTRACT FROM AUTHOR]

Background: Liver transplantation is the treatment of choice for end-stage hepatic diseases. The most important factor responsible for the success of the transplant is the size of the graft and the remnant liver volume in the donor. A small graft may not meet the metabolic demands of the recipient resulting in impaired liver functions such as hyperbilirubinemia, prolonged prothrombin time (PT), ascites and portal hypertension. Patient and Methods: This comparative study was conducted on manual contrast enhanced hepatic CT scans of 40 potential living liver donors (29) male and (11) female Age range from (18 to 50) in Ain shams specialized hospital and some private centers during the period from May 2021 till July 2022. The potential donors were investigated using 16 channel multi-detector row CT scanner (Alexion; Toshiba medical systems). All potential donors underwent 1st step laboratory investigations to enter the 2nd step investigations for living donor liver transplantation operations. All potential donors were of average weight and physically fit for operation with no history of any medical diseases. Results: We correlated the results of CT volumetry of right lobe graft weight with the intra-operative weight of liver graft in patients undergoing Living Donor Liver Transplantation(LDLT). CT Volumetry is an efficient and a reliable tool for assessing potential donors undergoing liver transplant, and this information is essential for donor selection and pre operative surgical planning as well as it ensure that the liver remnant volume is at least 30 % which guarantee the safety of the donor. Conclusion: The size of the right lobe graft for LDLT can be precisely calculated from pre-operative CT Volumetry with confidence and accuracy up to 95%. [ABSTRACT FROM AUTHOR]

The T2* technique, used for quantifying myocardial iron content (MIC), has limitations in detecting early myocardial iron overload (MIO). The in vivo mapping of the myocardial T1 relaxation time is a promising alternative for the early detection and management of MIO. 32 β-thalassemia major (βTM) patients aged 11.5 ± 4 years and 32 healthy controls were recruited and underwent thorough clinical and laboratory assessments. The mid-level septal iron overload was measured through T1 mapping using a modified Look-Locker inversion recovery sequence with a 3 (3 s) 3 (3 s) 5 scheme. Septum was divided at the mentioned level into 3 zones corresponding to segments 8 and 9 in the cardiac segmentation model. 21.9 % of βTM had clinical cardiac morbidity. The cut-off of T1 mapping of hepatic and myocardium to differentiate between the patients and control groups was ≤466 and ≥ 923 ms respectively. The T1 technique was able to detect 4 patients with high MIC, two of them were not detected by the T2* technique. There was a statistically significant correlation between the average T1 values of the studied zones in patients with βTM and the liver iron content (LIC), the T1 values within segment 8 of the liver, age of patients, the age at first transfusion, age of splenectomy and serum ferritin value. The addition of the T1 mapping sequence to the conventional T2* technique was able to increase the efficacy of the MIC detection protocol by earlier detection of MIO. This would guide chelation therapy to decrease myocardial morbidity. [ABSTRACT FROM AUTHOR]