Systemic lupus erythematosus children in Egypt: Homeland spectrum amid the global situation.

OBJECTIVES: This study aims to present the manifestations of juvenile systemic lupus erythematosus (JSLE) across Egypt, to focus on age at onset and gender-driven influence on disease characteristics, and to compare findings to other countries. METHODS: The study included 404 Egyptian children with systemic lupus erythematosus (SLE) presenting to one of the specialized rheumatology centers corresponding to 13 major governorates. Juvenile cases age was ≤ 16°years at the time of recruitment. The SLE Disease Activity Index (SLEDAI) and damage index (DI) were assessed. RESULTS: The mean age was 13.2 ± 2.4°years; 355 females and 49 males (7.2:1), and the disease duration was 2.3 ± 1.6 years, while age at disease onset was 11.1 ± 2.5°years. Their SLEDAI was 13.5 ± 12.3, and DI, 0.36 ± 0.78. The overall estimated prevalence of childhood-SLE patients in the recruited cohort in Egypt was 1/100,000 population (0.24/100000 males and 1.8/100000 females). 7.4% developed pre-pubertal SLE (≤ 7 years); 73.3%, peri-pubertal; and 19.3% during early adolescence. The differences according to age group were equal for gender and clinical manifestations except skin lesions present in 59.3% of pre-pubertal onset, 74.6% of peri-pubertal, and 84.2% of adolescents (p = 0.029), and renal involvement in 73.8% of peripubertal, 62.1% of pre-pubertal and 58.9% of adolescents (p = 0.03). Laboratory investigations, SLEDAI, and DI were similar among age categories. Lupus nephritis was more common in Egypt compared to JSLE from other countries. CONCLUSION: Our large multicenter study identified that female gender influenced disease characteristics with more frequent skin involvement. Skin lesions were significantly higher in adolescents, while renal involvement in peri-pubertal children.

Differentiation of Bilayer interventricular septum function in children with systemic lupus erythematosus by tissue Doppler and speckle tracking imaging.

BACKGROUND: The ventricular septum (VS) is considered a single functional wall. However, a line with high echogenicity in the middle of the VS could be detected with the recent echocardiographic modalities which separate the left from the right side of the VS. Our study aimed to evaluate functional differences between both sides of VS by tissue Doppler and Speckle Tracking Imaging (STI) in children with Systemic lupus erythematosus (SLE). SUBJECTS AND METHODS: This is a case-control study that included 40 children divided into 20 active SLE patients and 20 healthy children matched for age and sex as controls. Tissue Doppler imaging from the VS was performed with the region of interest was positioned in both sides at the mid-part of the septum. Longitudinal strain of both sides of the VS was obtained by (STI). Ejection fraction (EF) of the right and left ventricles were assessed by real-time 3-dimensional echocardiography. RESULTS: There was significant impaired S`, È, Á, compared with controls. STI of the VS showed significant affection of the left side regarding systolic strain (SS) and strain rate late diastole (SRDA). However, strain rate systole (SRS) and strain rate early diastole (SRED) are affected on both sides of VS compared with control. Á and SRDE of the left VS correlated significantly with SLEDAI (P ≤ .05). È of the right VS correlated with the duration of the disease (P ≤ .05). CONCLUSION: Bilayer analysis of VS could helps in the evaluation of right and left ventricular functions in subclinical myocarditis in SLE patients.

Predictive risk factors of steroid dependent nephrotic syndrome in children.

BACKGROUND: Development of steroid dependency is one of the difficult problems in the management of children with idiopathic nephrotic syndrome, leading to increased morbidity, complications and cost of treatment. Thus, predicting early in the disease course will be useful in counseling parents and may improve treatment strategy. OBJECTIVES: To determine the clinical characteristics that can predict the development of steroid dependency early in the initial episodes of steroid sensitive nephrotic syndrome (SSNS). PATIENTS AND METHODS: The study included 52 children with SSNS. Their ages ranged from 3 to 16 years. Patients were divided into two groups. Group A consisted of 24 patients with steroid dependency or frequent relapses nephrotic syndrome and group B consisted of 28 patients with complete remission or recurrent nephrotic syndrome. Data obtained retrospectively from patients' files. RESULTS: Children who require a cumulative steroid dose equal or more than 140 mg/kg to maintain remission during the first 6 months of the disease are at high risk to require steroid sparing agents (SSA) for disease control, and who did not achieve remission by day 20 of the initial prednisone course became steroid dependent with 96% specificity but with low sensitivity (50%). All steroid dependent children in this study showed relapses associated significantly with upper respiratory tract infections. CONCLUSIONS: Cumulative steroid dose in the first 6 months of treatment and the need of more than 20 days to achieve initial remission can predict steroid dependency in children with nephrotic syndrome.

Histopathological patterns of renal diseases in egyptian children: A single-center experience.

The present cross-sectional, retrospective study was aimed to determine the histopathological spectrum of renal diseases in Egyptian children and to evaluate the indications, safety, and efficacy of percutaneous renal biopsy (PRB) in a large tertiary center in Egypt. PRBs performed at the Department of Pediatrics, Tanta University Hospital over a period of nine years (from January 2007 to December 2015) were included. Light microscopic (LM) examination was performed in all cases while immunofluorescence and electron microscopic examination were performed in selected cases. Two hundred and thirty renal biopsies were performed during the study duration. Nine biopsies were excluded from the study due to insufficient sample (<7 glomeruli per specimen) giving a PRB efficacy rate of 96.1%. Results of 221 renal biopsies performed on 210 patients from native kidneys were described. Ninety-seven patients were male (46.19%) and 113 were female (53.81%) with age ranging from three months to 18 years (mean 10.51 ± 3.81 years). The main indications of renal biopsy were nephrotic syndrome (NS) (43.89%), lupus nephritis (23.53%), and recurrent or persistent hematuria (10.41%). The most common finding on LM examination of renal biopsies from children with NS was minimal change disease (22.17%). Secondary nephropathies were mostly due to lupus (23.53%). IgA nephropathy was found in eight patients (3.62%). Local pain at the site of biopsy was the most common minor complication seen postbiopsy (60.58%). Transient gross hematuria was seen in 13 patients (5.88%) without urinary retention. Major complications that required surgical intervention or blood transfusion did not occur. NS was the main indication and minimal change disease was the most common histological finding of renal biopsies from Egyptian children. Complications and efficacy of renal biopsy procedure were comparable to that reported from developed countries.

Sleep disorders in children with chronic kidney disease.

BACKGROUND AND OBJECTIVES: This case-control study assessed the prevalence of sleep disorders among Egyptian children with chronic kidney disease (CKD), either maintained or not maintained on hemodialysis (HD), and compared them with healthy age and sex-matched children. PATIENTS AND METHODS: The total study population included 95 children, 54 of whom were CKD patients, 22 maintained on HD and 32 not maintained on HD; 41 healthy children of matched age and sex composed the control group.Subjective impairment of sleep quality was assessed using the Arabic version of the Children's Sleep Habits Questionnaire (CSHQ). Daytime sleepiness and restless leg syndrome (RLS) were assessed using a pediatric modification of the Epworth sleepiness scale (ESS) and RLS Questionnaire, respectively. RESULTS: Sleep disturbances were detected in 75.9% of the studied children with CKD: 81.8% in children with CKD undergoing dialysis, and 71.8% in children with CKD not on dialysis. Excessive daytime sleepiness (EDS) and RLS symptoms were reported in 22% and 20.4% of the studied children with CKD, respectively. CONCLUSIONS: Sleep disturbances are very common among children with CKD. Sleep disturbances in patients with CKD include restless legs syndrome (RLS), excessive daytime sleepiness (EDS), sleep-disordered breathing (SDB), behavioral insomnias, and parasomnias.