Hydroxychloroquine (HCQ) is an antimalarial agent used to treat mucocutaneous, musculoskeletal, constitutional manifestations of systemic lupus erythematosus (SLE). This study assessed the efficacy and side effects of HCQ in children with proliferative lupus nephritis (LN). This double-blind, randomized, placebo-controlled trial study was conducted on 60 children with proliferative LN classes III and IV treated with steroids and a mycophenolate (MMF) regimen. Patients were categorized into two groups, the HCQ group (n = 30) and the placebo group (n = 30). They were evaluated initially at 6- and a 12-month follow-up by mucocutaneous, ophthalmological examination, and investigations (BUN, creatinine, 24 h proteinuria, triglycerides (TG), cholesterol, Antids-DNA, C3, C4). Disease activity was assessed using the SLE disease activity index (SLEDAI-2 k). After 12 months, TG, cholesterol, 24 h proteinuria, Antids-DNA, and SLEDAI score were significantly decreased in the HCQ group (P: 0.002, 0.012, 0.031, 0.001, respectively). After 12 months, the cumulative probabilities of developing primary end-points (LN partial and complete remission) were 40% and 60% in the HCQ group versus 53.3% and 36.7% in the placebo group (P: 0.002). After 12 months, the HCQ group experienced mucocutaneous alopecia (3.3%), hyperpigmentation (10%), and ophthalmological mild retinal changes (6.7%), but they did not differ significantly from the placebo group. Cunclusion: HCQ improved the disease and LN activity in children with proliferative LN, with documented skin hyperpigmentation and mild retinal changes following HCQ use in a few cases. This study was registered on http://www.clinicaltrials.gov/ with trial registration number (TRN): NCT03687905, September 2018 “retrospectively registered.” What is Known: • Hydroxychloroquine (HCQ) is documented as an adjunctive treatment in children with systemic lupus erythematosus (c-SLE) LN with efficacy in improving lupus musculoskeletal and mucocutaneous manifestations. • Due to the paucity of studies, its effects and side effects in children with LN remain unclear. What is New: • This pilot randomized clinical trial assessed the efficacy and adverse effects of HCQ in children with proliferative LN. • HCQ had numerous advantages for LN, including rapid and sustained remission, antilipidemic effect, and rapid improvement of kidney functions.
Background: Dilated cardiomyopathy (DCM) refers to dilating the ventricles and dysfunction of their systolic functions (predominantly the left ventricle) with or without congestive heart failure. In children, it is the most common form of heart muscle disease. We aimed to evaluate the right ventricular functions and structure using speckling tracking echocardiography in children with dilated cardiomyopathy and correlate this parameter with other echocardiographic findings. Methods: This observational Case-Control Study was carried out on 75 subjects. They were subdivided into two groups: Group 1: 50 patients with dilated cardiomyopathy Group 2: 25 healthy children matched for age and sex. Patients were evaluated by M-mode echocardiography, Transthoracic 2DE Examination (TTE), Tissue Doppler Examination (TDE) and Speckling Tracking Technique. Results: Left ventricle (LV) and right ventricle (RV) systolic dysfunction was evidenced by a significant decrease of mitral and tricuspid annular systolic velocities and a significant decrease of LV and RV global systolic strain and a significant decrease of LV and RV Ejection fraction (EF). LV and RV diastolic dysfunction were evidenced by a significant decrease of mitral and tricuspid annular diastolic velocities (E’/A’) and a significant increase of LV and RV Myocardial Perfusion Imaging (MPI). LV and RV global strains were significantly reduced in comparison to controls, suggesting that the dilated cardiomyopathy is a diffuse disease. Conclusion: In DCM patients, RV had significant systolic and diastolic dysfunction mainly elicited by the Tissue Doppler imaging (TDI) beside LV affection secondary to the interventricular interaction. TDI and 2D-STE add value to interpreting the findings and the dependency of RV systolic and diastolic functions on each other in DCM patients.
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