Randomized Clinical and Biochemical Study Comparing the Effect of L-arginine and Sildenafil in Beta Thalassemia Major Children With High Tricuspid Regurgitant Jet Velocity.

BACKGROUND: Pulmonary hypertension (PHT) is common in ß-thalassemia patients due to hemolysis, iron overload and diminished nitric oxide (NO) levels. Biochemical markers can help to understand the pathophysiology and to introduce new therapies for this condition. AIM: This study aimed to evaluate the effectiveness of L-arginine and sildenafil in thalassemia children with PHT at both clinical and biochemical levels. METHODS AND RESULTS: In a randomized controlled study, 60 ß-thalassemia major children with PHT were divided into 3 equal groups; Control group (Conventional thalassemia and PHT management), L-arginine group (Conventional + Oral L-arginine 0.1 mg.kg-1 daily), and sildenafil group (Conventional + Oral sildenafil 0.25 mg.kg-1 two times a day) for 60 days. Tricuspid Regurgitant Jet Velocity (TRJV) with Doppler echocardiography along with serum levels of NO, asymmetric dimethylarginine (ADMA), interleukin 1-beta (IL-1ß), E-selectin, and visfatin were followed-up at baseline, 30, and 60 days after treatment. Both drugs reduced the TRJV significantly. NO was significantly higher in both L-arginine and sildenafil groups after 60 days compared to baseline, while visfatin levels were lower. Only L-arginine reduced ADMA levels compared to baseline, while sildenafil did not. E-selectin and IL-1ß levels did not change remarkably by both drugs. NO and TRJV showed significant negative correlations in both treatment groups. CONCLUSION: L-arginine and sildenafil could clinically ameliorate chronic PHT whereas, L-arginine showed superiority to sildenafil on some biochemical markers.

Trichrome-positive intrahepatic cytoplasmic globules are potential histopathological clue for COVID-19-induced hepatitis: a case report.

BACKGROUND: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection mainly affects respiratory system. Later, liver affection has also been reported in the form of marked elevated liver enzymes. However, the association of coronavirus disease-19 (COVID-19) and autoimmune diseases is not clear. CASE PRESENTATION: A female patient with a known history of autoimmune hemolytic anemia (AIHH) for which she was treated with prednisolone was admitted for uncontrolled anemia followed by fever and elevated liver enzymes. All the laboratory and radiological investigations were not typical for COVID-19 or any other etiology. Liver biopsy revealed numerous pale eosinophilic trichrome-positive intracytoplasmic globules. The pathology raised the suspicion for SARS-CoV-2-associated hepatitis, which was confirmed by a positive IgG titer. The patient showed a dramatic improvement on the maintenance dose of prednisolone. CONCLUSIONS: AIHA patients co-infected with SARS-CoV-2 may be at risk of uncontrolled disease and should continue their treatment regimen. Histopathology has a role in the diagnosis of liver affection due to SARS-CoV-2 infection.

Left Ventricular Regional Function in Children with Beta Thalassemia with No Cardiac Manifestations (Four-Dimensional Echocardiographic Study).

Early detection of myocardial dysfunction is essential for the management of patients with thalassemia. Four-dimensional echocardiography imaging technique may be useful for detecting subclinical cardiovascular disease. To evaluate the 4-dimensional echocardiographic strain in children with beta thalassemia major with no cardiac manifestation and correlate it with other echocardiographic parameters. This is a prospective cross-sectional cohort Study included 200 children, 1-18 years-old. They were divided into: One hundred children with p-Thalassemia major with no clinical cardiac manifestations and 100 healthy children as a control group. They were subjected to the following investigations: Complete blood count, serum ferritin and Four-dimensional echocardiographic strains (Longitudinal, Circumferential, Radial and Area strains). There was no significant difference between the two groups as regard mitral annulus systolic velocity (S wave), E/A ratio and iso-volumic acceleration, but there was a significant difference as regard to ejection fraction, left ventricle mass, sphericity index and myocardial performance index. The mean values of Left Ventricular Strains (Longitudinal, Circumferential, Radial and Area strains) were significantly lower in patients with thalassemia (- 14.86 ± 12.13, - 8.01 ± 3.829, 33.13 ± 10.61, - 19.45 ± 6.866) than controls (- 19.13 ± 1.502, - 16.32 ± 1.34, 37.28 ± 4.209, - 22.94 ± 3.06) than controls respectively with a positive correlation with 2-Dimensional strain. Strain parameters of the left ventricle obtained by four-dimensional.

Speckle tracking evaluation of right ventricular functions in children with sickle cell disease.

BACKGROUND: Cardiac dysfunction is a risk factor for death in patients with sickle cell disease (SCD). AIM OF THE WORK: Aim of the work is to evaluate the right ventricular systolic and diastolic functions by tissue Doppler and speckling tracking imaging in children with SCD. SUBJECTS AND METHODS: Thirty children with SCD and thirty controls were subjected to clinical, laboratory evaluations, and echocardiographic study using GE Vivid 7 (GE Medical System, Horten, Norway with a 3.5-MHz multifrequency transducer) including; Two-dimensional and tissue Doppler echocardiographic study (lateral tricuspid valve annulus peak E' velocity, lateral tricuspid valve annulus peak A' velocity, E'/A' ratio, isovolumetric relaxation time, lateral tricuspid valve annulus S' and septal S' waves and peak longitudinal systolic strain [PLSS] and time to PLSS) were done in six right ventricular segments. RESULTS: There was a significant decrease in right ventricular systolic and diastolic function in patients group when compared to controls. CONCLUSIONS: Children with SCD have impaired right ventricular systolic and diastolic functions when compared to healthy children with early evaluation of the systolic dysfunction by speckle tracking imaging technique.