ABSTRACT
Background:Early detection of neuropsychological changes in children with sickle cell disease (SCD) is essential to improve their quality of life. Aim of the Work: To assess neurological and psychological disorders in children with sickle cell disease (SCD) using multimodal approach through clinical, laboratory, neuroimaging and neurophysiological studies in a trial to detect etiological risk factors.Study Design: Cross-sectional study.Placeand Duration of Study: Sample: Department of Pediatric (Hematology Unit) and Department of Neurology, Tanta University Hospital Egypt, between April 2016 and April 2018.Methodology:This study was conducted on 50 children (27 male and 23 female; age range 2-18 years) with SCD and 25 healthy children matched age and sex. All subjects were subjected to full history taking, neurologic examination using pediatric neurological sheet, laboratory investigations, neuroimaging including: CT and /or MRI, MRA and/or CT angiography, also MR, EEG and Stanford-Binet Intelligence scales-Fifth Edition. Results:Most of patients presented with headache 66%, cognitive decline 48%, seizures 28%, and visual affection 24%. Less common presentations were, ischemic and hemorrhagic stroke 6%and 4% respectively. SCD children showed many abnormalities on neurological examination and on different modalities of MR imaging on the brain with positive correlation (X2=7.641, p-value <0.001*, r=0.248) with many risk factors. Prophylactic blood transfusion in SCD patients with abnormal TCD had a role in reducing the incidence of stroke. Conclusion: Children with SCD were presented with variable neuropsychological disturbance that correlated with the brain imaging.
ABSTRACT
This study aimed to investigate the value of conventional Doppler-echocardiography, Doppler tissue imaging [DTI], and serum cardiac troponin I [cTni] as early predictors of cardiotoxicity in children treated with doxorubicin for different hematological malignancies and to evaluate their feasibility as early screening tests in assessing the left as well as the right ventricular systolic and diastolic functions. This study included 19 clinically asymptomatic children aged 4.9 +/- 2.1 years with normal systolic function who were receiving doxorubicin chemotherapy [cumulative dose= 122.4 +/- 59.9 mg/m2] for different malignant neoplasms [16 children having acute lymphoblastic leukemia, 2 having acute myeloid leukemia and I having leukemic phase of lymphoma]. They were subjected to Doppler-echocardiographic and DTI examination of the right ventricular [RV] and left ventricular [LV] systolic and diastolic functions as well as estimation of serum levels of cTni by sandwich immunoassay after the last dose of doxorubicin during the induction-remission therapy. Another 20 healthy normal children were taken as a control group. Results showed that the LV systolic functions as well as LV and RV diastolic functions [assessed by Doppler study of mitral and tricuspid inflow, and mitral flow propagation velocity [MPW and myocardial performance index [MPI] were impaired in patients compared with controls. DTI study confirmed and disclosed such impairment in LV and RV systolic [decreased lateral mitral and tricuspid annulus systolic [Sa] velocities] and diastolic functions [decreased early diastolic tricuspid and lateral and septal mitral annulus [Ea] velocities and mitral Ea/Aa] in patients compared with controls. Serum cTni was statistically significantly increased in-patient as compared with the control group. There was a significant negative correlation between serum levels of cTni and Ea/Aa. On the other-hand, cumulative dose of doxorubicin was not correlated with either serum cTni or any systolic or diastolic cardiac functions
Conclusion: DTI confirmed and disclosed abnormal RV and LV systolic and diastolic functions reported by conventional Doppler-echocardiography in asymptomatic doxorubicin-treated children. DTI had more ability to detect abnormal RV and LV systolic and diastolic functions than conventional Doppler-echocardiography. Serum cTni, which is considered as a marker for myocardial cell injury significantly, correlates with the degree of diastolic dysfunction detected only by DTI [Ea/Aa]. The repetitive measurements of the new DTI-derived velocities, Doppler-derived indices [MPI], M-mode- derived MPV and serum cTni could add significant value in the early defection of doxorubicin-induced cardiotoxicity and enhance several studies to find suitable cardio protective free radical scavengers which can reduce the cardio toxic effects of doxorubicin including; dexrazoxane, exogenous melatonin, phosphodiesterase inhibitors type 4 or induction of metallothionein by zinc