The role of MRI-R2* in the detection of subclinical pancreatic iron loading among transfusion-dependent sickle cell disease patients and correlation with hepatic and cardiac iron loading.

OBJECTIVES: Pancreatic reserve could be preserved by early assessment of pancreatic iron overload among transfusion-dependent sickle cell disease (SCD) patients. This study aimed to measure pancreatic iron load and correlate its value with patients' laboratory and radiological markers of iron overload. MATERIALS AND METHODS: Sixty-six SCD children and young adults underwent MRI T2* relaxometry using a simple mathematical spreadsheet and laboratory assessment. RESULTS: The results indicated moderate-to-severe hepatic iron overload among 65.2% of studied cases. None had cardiac iron overload. Normal-to-mild iron overload was present in the pancreas in 86% of cases, and 50% had elevated serum ferritin > 2500 ug/L. There was no significant correlation between pancreatic R2* level, serum ferritin, and hepatic iron overload. Patients with higher levels of hemolysis markers and lower pre-transfusion hemoglobin levels showed moderate-to-severe pancreatic iron overload. CONCLUSION: Chronically transfused patients with SCD have a high frequency of iron overload complications including pancreatic iron deposition, thereby necessitating proper monitoring of the body's overall iron balance as well as detection of extrahepatic iron depositions.

Impact of exercise training on myocardial contractile functions assessed by cardiac magnetic resonance in post-myocardial infarction patients.

BACKGROUND: Improvement of functional capacity and mortality reduction in post-MI patients were found to be associated with regular exercise training. The cardiac magnetic resonance (CMR) is considered the most accurate non-invasive modality in quantitative assessment of left ventricular (LV) volumes and systolic functions. Our main objective was to investigate the impact of exercise training on LV systolic functions in patients post anterior MI using CMR. 32 patients on recommended medical treatment 4 week after having a successful primary PCI for an anterior MI were recruited, between May 2018 and May 2019. They were divided into two groups, training group (TG): 16 assigned to a 12 week exercise training program and control group (CG): 16 who received medical treatment without participating in the exercise training program. Treadmill exercise using modified Bruce protocol was done to TG before and after the training program in order to record the resting and maximum HR, metabolic equivalent (MET), and calculate HR reserve. CMR was performed for all patients 4 weeks after PCI and was repeated after completion of the study period to calculate ejection fraction (EF), left ventricular end-systolic volume (LVESV), left ventricular end-diastolic volume (LVEDV), and wall motion score index (WMSI). RESULTS: 100% were males. 6 patients from CG dropped during follow-up, no statistically significant difference between the two groups regarding age, BMI, smoking status, hypertension, diabetes mellitus and dyslipidemia. Using the CMR, the TG showed significant improvement in EF (36.6 ± 14.2% to 43.1 ± 12.4%; P < 0.001) and WMSI (2.03 ± 0.57 to 1.7 ± 0.49; P < 0.001), without statistically significant change in LV volumes. Regarding CG no significant changes in EF, WMSI, LV volumes were found. There was significant improvement in EF and WMSI change before and after study in TG vs. CG [6.5 (2.3-9.0) vs. - 2.0 (- 6.8 to 1.3), P value < 0.001] and [- 0.3 (- 0.5 to 0.1) vs. 0.1 (- 0.1 to - 0.5), P value 0.001] respectively. CONCLUSIONS: 12 weeks of exercise training program in post-MI patients have a favorable impact on LV global and regional systolic functions without adversely affecting LV remodeling (as assessed by CMR).

Hidden brain iron content in sickle cell disease: impact on neurocognitive functions.

Children with sickle cell disease (SCD) are at a high risk for neurocognitive impairment. We aim to quantitatively measure cerebral tissue R2* to investigate the brain iron deposition in children and young adults with SCD in comparison to beta thalassemia major (BTM) and healthy controls and evaluate its impact on neurocognitive functions in patients with SCD. Thirty-two SCD, fifteen BTM, and eleven controls were recruited. Multi-echo fast-gradient echo sequence brain MRI was performed, and brain R2* values of both caudate and thalamic regions were calculated. SCD patients were examined for the neurocognitive functions. SCD had high iron overload 0.30 ± 0.12 mg/kg/day. 68.9% of SCD had under-threshold IQ, 12.5% had moderate to severe anxiety, and 60.8% had depression. There were no differences between SCD, BTM, and controls in brain MRI except that left thalamus R2* higher in BTM than both SCD and controls (p = 0.032). Mean right caudate R2* was higher in female than male (p = 0.044). No significant association between brain R2* and LIC or heart R2* values in SCD. Left caudate R2* directly correlate with age and HbS%, and negatively correlate with HbA% while right thalamus R2* negatively correlate with transfusion index and among SCD patients.Conclusion: Neurocognitive dysfunction in SCD could not be explained solely by brain iron overload. What is Known: • Children with sickle cell disease are at great risk of brain damage due to their irregularly shaped red blood cells that can interrupt blood flow to the brain. • There are a number of factors that have negative brain effects that result in learning difficulties, and this not only due to increase brain iron content. What is New: • Assessment of quantitative brain iron content using MRI R2* in children and young adults with SCD in comparison to beta thalassemia major and healthy controls. • Impact of brain iron content on neurocognitive functions of children and young adults with SCD.

Renal iron deposition by magnetic resonance imaging in pediatric ß-thalassemia major patients: Relation to renal biomarkers, total body iron and chelation therapy.

BACKGROUND: The reciprocal of multiecho gradient-echo (ME-GRE) T2* magnetic resonance imaging (MRI) R2*, rises linearly with tissue iron concentration in both heart and liver. Little is known about renal iron deposition in ß-thalassemia major (ß-TM). AIM: To assess renal iron overload by MRI and its relation to total body iron and renal function among 50 pediatric patients with ß-TM. METHODS: Serum ferritin, serum cystatin C, urinary albumin creatinine ratio (UACR), and urinary ß2-microglobulin (ß2 M) were measured with calculation of ß2 M/albumin ratio. Quantification of liver, heart and kidney iron overload was done by MRI. RESULTS: Serum cystatin C, UACR and urinary ß2 microglobulin as well as urinary ß2m/albumin were significantly higher in ß-TM patients than the control group. No significant difference was found as regards renal R2* between Patients with mean serum ferritin above 2500 µg/L and those with lower serum cutoff. Renal R2* was higher in patients with poor compliance to chelation therapy and positively correlated to indirect bilirubin, LDH, cystatin C and LIC but inversely correlated to cardiac T2*. CONCLUSION: kidney iron deposition impairs renal glomerular and tubular functions in pediatric patients with ß-TM and is related to hemolysis, total body iron overload and poor compliance to chelation.

Three Dimensional (3D) Echocardiography as a Tool of Left Ventricular Assessment in Children with Dilated Cardiomyopathy: Comparison to Cardiac MRI.

BACKGROUND: Left ventricular (LV) volumes and ejection fraction (EF) is Strong prognostic indicators for DCM. Cardiac MRI (CMRI) is a preferred technique for LV volumes and EF assessment due to high spatial resolution and complete volumetric datasets. Three-dimensional echocardiography is a promising new technique under investigations. AIM: Evaluate 3D echocardiography as a tool in LV assessment in DCM children about CMRI. PATIENTS AND METHODS: A group of 20 DCM children (LVdiastolic diameter < 2 Z score, LVEF < 35%) at Children s Hospital, Ain-Shams University (gp1) (mean age 6.6 years) were compared to 20 age and sex-matched children as controls (gp2). Patients were subjected to: clinical examination, conventional echocardiography, automated 3D LV quantification, 3D speckle tracking echocardiography (3D-STE) (VIVID E9 Vingmed, Norway) and CMRI (Philips Achieva Nova, 1.5 Tesla scanner) for LV end systolic volume (LVESV), LVend diastolic volume (LVEDV) that were indexed to body surface area, EF% and wall motion abnormalities assessment. RESUTS: No statistically significant difference was found between automated 3D LV quantification echocardiography, 3D-STE, and CMRI in ESV/BSA and EDV/BSA assessment (p = 1, 0.99 respectively), between automated LV quantification echocardiography and CMRI in EF% assessment (p = 0.99) and between CMRI and 3D-STE in LV Global hypokinesia detection (P = 0.255). As for segmental hypokinesia CMRI was more sensitive [45% of patients vs. 40%, (P = 0,036), basal septal hypokinesia 85% vs. 75%, (p = 0.045), mid septal hypokinesia 80% vs. 65%, (p = 0.012) and lateral wall hypokinesia 75% vs. 65%, (p = 0.028)]. CONCLUSION: Automated 3D LV quantification echocardiography and 3D-STE are reliable tools in LV volumetric and systolic function assessment about CMRIas a standard method. 3D speckle echocardiography is comparable to CMRI in global wall hypokinesia detection but less sensitive in segmental wall hypokinesia which mandates further studies.

MRI Surface-Based Brain Morphometry in Egyptian Autistic and Typically Developing Children.

OBJECTIVES: The verbal abilities of autistic children differ from those of typically developing ones and they also differ among autistic children themselves. Neuroanatomical changes and an abnormal organization of functional networks are expected to accompany such a neurodevelopmental disorder. The aim of this study was to delineate the brain neuroanatomical changes in Egyptian children with autism and to compare them with previous studies in order to add more insight into the global brain imaging deviations linked to autism. PATIENTS AND METHODS: Twenty-five autistic children and 25 typically developing children underwent MRI. Further analysis was performed using surface-based morphometry to obtain cortical thickness, brain volume, and cortical complexity. RESULTS: MRI analysis results revealed significantly greater cortical thickness, cortical complexity, and gray matter volume in the autistic as compared to the control group. On the other hand, the white matter volume was significantly smaller. CONCLUSION: These findings generally align with findings in previous studies, except for occasional differences.

Cognition and lobar morphology in full mutation boys with fragile X syndrome.

The aims of the present study are twofold: (1) to examine cortical morphology (CM) associated with alterations in cognition in fragile X syndrome (FXS); (2) to characterize the CM profile of FXS versus FXS with an autism diagnosis (FXS+Aut) as a preliminary attempt to further elucidate the behavioral distinctions between the two sub-groups. We used anatomical magnetic resonance imaging surface-based morphometry in 21 male children (FXS N=11 and age [2.27-13.3] matched controls [C] N=10). We found (1) increased whole hemispheric and lobar cortical volume, cortical thickness and cortical complexity bilaterally, yet insignificant changes in hemispheric surface area and gyrification index in FXS compared to C; (2) linear regression analyses revealed significant negative correlations between CM and cognition; (3) significant CM differences between FXS and FXS+Aut associated with their distinctive behavioral phenotypes. These findings are critical in understanding the neuropathophysiology of one of the most common intellectual deficiency syndromes associated with altered cognition as they provide human in vivo information about genetic control of CM and cognition.