Ahmed El-Refaey

Psychiatric disorders in children with chronic renal failure

Psychiatric assessment was done according to the DSM-IV TR criteria in 19 children with predialysis chronic renal failure (CRF) and 19 children with end-stage renal disease on regular hemodialysis. The prevalence rate of psychiatric disorders in all the studied patients was 52.6%. Adjustment disorders were the most common disorders (18.4%), followed by depression (10.3%) and neurocognitive disorders (7.7%). Anxiety and elimination disorders were reported in 5.1 and 2.6%, respectively. The disorders were more prevalent (P=0.05) in dialysis (68.4%) than in predialysis patients (36.8%). The presence of psychiatric disorders was not significantly correlated with sex, severity of anemia, duration of CRF or the efficiency or the duration of hemodialysis. In conclusion, psychiatric disorders were prevalent in our patients, especially in those on hemodialysis. Both adjustments with depression and depressive disorders were the most common psychiatric disorders. This array of disorders was more likely explained by the difficulties encountered in living with CRF rather than by demographic or physical factors.

Comparison of intravenous immunoglobulin and plasma exchange in treatment of mechanically ventilated children with Guillain Barré syndrome: a randomized study

IntroductionRespiratory failure is a life threatening complication of Guillain Barré syndrome (GBS). There is no consensus on the specific treatment for this subset of children with GBS.MethodsThis was a prospective randomized study to compare the outcome of intravenous immunoglobulin (IVIG) and plasma exchange (PE) treatment in children with GBS requiring mechanical ventilation. Forty-one children with GBS requiring endotracheal mechanical ventilation (MV) within 14 days from disease onset were included. The ages of the children ranged from 49 to 143 months.Randomly, 20 children received a five-day course of IVIG (0.4 g/kg/day) and 21 children received a five-day course of one volume PE daily. Lumbar puncture (LP) was performed in 36 patients (18 in each group).ResultsBoth groups had comparable age (p = 0.764), weight (p = 0.764), duration of illness prior to MV (p = 0.854), preceding diarrhea (p = 0.751), cranial nerve involvement (p = 0.756), muscle power using Medical Research Council (MRC) sum score (p = 0.266) and cerebrospinal fluid (CSF) protein (p = 0.606).Children in the PE group had a shorter period of MV (median 11 days, IQR 11.0 to 13.0) compared to IVIG group (median 13 days, IQR 11.3 to 14.5) with p = 0.037.Those in the PE group had a tendency for a shorter Pediatric Intensive Care Unit (PICU) stay (p = 0.094).A total of 20/21 (95.2%) and 18/20 (90%) children in the PE and IVIG groups respectively could walk unaided within four weeks after PICU discharge (p = 0.606).There was a negative correlation between CSF protein and duration of mechanical ventilation in the PE group (p = 0.037), but not in the IVIG group (p = 0.132).ConclusionsIn children with GBS requiring MV, PE is superior to IVIG regarding the duration of MV but not PICU stay or the short term neurological outcome.The negative correlation between CSF protein values and duration of MV in PE group requires further evaluation of its clinical usefulness.Trial RegistrationClinicaltrials.gov Identifier NCT01306578

Fifteen years of kidney biopsies in children: A single center in Egypt

This study retrospectively investigates the indications and results of renal biopsy in children to determine the patterns of childhood kidney disease in a single tertiary childrens hospital in Egypt. We included all the patients who underwent ultrasound-guided renal biopsy from 1998 to 2012. All the kidney biopsies were studied under light microscopy, while immunofluorescence and electron microscopy were performed when indicated. A total of 1246 renal biopsies were performed over 15 years, on 1096 patients. The mean age of the patients at the time of biopsy was 9.2±3.7 years. The main indication for a biopsy was the steroid-resistant nephrotic syndrome (n=354, 28.4%), followed by the atypical nephrotic syndrome (n=250, 20.1%), and renal abnormalities in the systemic diseases (n=228, 18.3 %). In the 1226 pathologically diagnosed specimens, primary glomerulonephritis was the most common finding (n=826, 67.4%), followed by secondary glomerulonephritis (n=238, 19.4%). The most common causes of primary glomerulonephritis were Minimal Change Disease (MCD) (n=267, 21.8%), diffuse proliferative glomerulonephritis (n=188, 15. 3%), and focal proliferative glomerulonephritis (n=164, 13.3%). Lupus nephritis (n=209, 17%) was the most common cause of secondary glomerulonephritis. We conclude that the steroid-resistant nephrotic syndrome was the most frequent indication for biopsy and minimal change disease was the most common histopathological finding in our population.

Sleep quality assessment using polysomnography in children on regular hemodialysis

Studies examining sleep patterns in children on hemodialysis (HD) are lacking. This cross-sectional, control-matched group study was performed to assess the sleep quality in children on HD. The assessment was made using a subjective sleep assessment and sleep questionnaire and objective analysis was made using full night polysomnography. A total of 25 children with end-stage renal disease (ESRD) on HD were compared with 15 age- and sex-matched controls. The average age of the cases was 14 ± 4 years, 52% were males and the mean body mass index was 20 ± 3.8 kg/m². The average duration on dialysis was 2.6 ± 2 years. Analysis of subjective data revealed markedly affected sleep quality in HD patients, as evidenced by excessive day time sleepiness (P <0.005), night awakening (P <0.005), difficult morning arousal (P <0.005) and limb pains (P <0.005). Objective analysis showed differences in sleep architecture, less slow wave sleep in HD children, similar rapid eye movement and non-rapid eye movement, more sleep disordered breathing (P <0.0001) and more periodic limb movement disorders (P <0.0001). Our study suggests that children on regular HD have markedly affected objective as well as subjective quality of sleep.

The emergence of systemic lupus erythematosus in hypothyroid patients: two case reports and mini review.

Systemic lupus erythematosus (SLE) is a multi-systemic autoimmune disease that involves almost all the organs in the human body and is characterized by auto antibodies formation. Autoimmune thyroid diseases (AITD) are organ-specific diseases that are associated with a production of a variety of antibodies such as antinuclear antibodies, anti-double-stranded DNA, anti-Ro antibodies, anti-cardiolipin antibodies, and others. The diagnosis of AITD in patients with SLE is well known, but the reverse is rarely reported. We present two cases of adolescent girls in whom SLE evolved one year after being diagnosed with hypothyroidism.

Montelukast as an add-on treatment in steroid dependant nephrotic syndrome, randomised-controlled trial

BackgroundThe underlying mechanisms of nephrotic syndrome (NS) are still under debate and the need for more effective and less toxic treatment is challenging. We aimed to evaluate the efficacy of montelukast, a leukotriene receptor antagonist as an add-on therapy, and to explore the leukotriene (LT) biology in steroid-dependent nephrotic syndrome (SDNS).MethodsA randomized controlled trial was conducted including 32 patients with SDNS who were randomly assigned to receive standard steroid treatment only [low-dose steroid (LDS) group] or standard steroid therapy plus montelukast (Montelukast group). Urine protein/creatinine ratio, serum albumin, creatinine, cholesterol, and plasma LTs (LTB4/C4/D4/E4) were evaluated in all patients before and after treatment.ResultsAfter treatment, both groups showed a significant decrease of LTB4 and LTC4/D4/E4. Further, the Montelukast group showed a significant decrease in serum creatinine and a significant increase in diastolic blood pressure and protein/creatinine ratio. It also showed a marked decrease in plasma LTC4/D4/E4 compared to the LDS group, although not statistically significant.ConclusionsOur findings highlight the effect of montelukast on renal function, but suggest that the clinical and laboratory efficacy of the addition of montelukast to steroids in maintenance treatment of SDNS is debatable.

Schimke immune-osseous dysplasia: A case report.

Schimke immune-osseous dysplasia (SIOD) is a rare autosomal recessive disorder characterized by spondylo-epiphyseal dysplasia (SED), progressive renal insufficiency beginning as steroid-resistant nephrotic syndrome (SRNS) and defective cellular immunity. This article reports a case from Egypt with a mild form of SIOD. A 14.5-year-old male patient presented with disproportionate short stature, SRNS (focal and segmental glomerulosclerosis), laboratory evidence of cellular immune deficiency and radiologic characteristics of SED. He died at the age of 16.5 years with bone marrow failure and severe pneumonia. To the best of our knowledge, this is the first case of SIOD to be reported from Egypt.

Serum cystatin-C and BETA 2-microglobulin as accurate markers in the early diagnosis of kidney injury in neonates: A single center study

Early detection of kidney injury in neonates is very important for appropriate management and prevention of serious complications; however, commonly used detectors as creatinine and blood urea nitrogen (BUN) do not directly reflect early renal cell injury. Serum cystatin-C (Cys-C) and beta 2-microglobulin (Β2M), serum creatinine and BUN were assessed in 20 neonates who developed renal impairment after admission to the neonatal intensive care unit and 10 healthy neonates. The means of serum Cys-C, Β2M and creatinine on Day 1 of admission in the cases and control groups were 2.15 ± 0.52 vs 0.45 ± 0.19, 7.18 ± 2.36 vs 1.92 ± 0.41 and 0.81 ± 0.07 vs 0.53 ± 0.20, respectively, with P-value <0.05 in only Cys-C and Β2M. We conclude that serum Cys-C and Β2M are suggested as simple and accurate markers for the early diagnosis of kidney injury in neonates than serum creatinine.