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Background: Despite advances in immunosuppression, acute allograft rejection remains one of the key factors affecting patient and graft survival in pediatric kidney transplantation. The aim of the study is to evaluate the role of serum soluble IL2 receptor (sIL2R) level as a noninvasive assessment parameter of acute rejection (AR). Method: Serum sIL2R level was measured (using enzyme-linked immune-sorbent assay technique) in 60 pediatric kidney transplant recipients (30 recipients with AR and 30 transplant recipients with stable graft function). Results: The mean values of sIL2R level in patients experiencing AR (14.8 ± 6.54) ng/mL were significantly higher than that in patients with stable graft functions (6.44 ±1.95) ng/mL (p = 0.0001). In addition; patients with AR proved by graft biopsy had their mean values of sIL2R level (16.19 ± 7.48) ng/mL significantly higher than that of other recipients in the study population (p = 0.032). Conclusion: Serum sIL2R level may serve as a noninvasive diagnostic indicator in pediatric kidney transplant recipients experiencing AR.

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This prospective study was undertaken to focus on the clinicopathological aspects as well as the value of brain magnetic resonance imaging (MRI) in children suffering from mitochondriopathies. Thirty-one patients [15 males (48.4%) and 16 females (51.6%)] gathered from the neuropediatric and metabolic clinic of Cairo University Children Hospital were included in this study. All patients were subjected to a thorough history and a meticulous clinical examination, computerized tomography and (MRI), electromyography and nerve conduction velocity. Muscle biopsies were performed in 18/31 patients and were subjected to both cytochrome c oxidase (COX) staining and electron microscopic (EM) examination. Laboratory tests including serum lactate and pyruvate, serum creatine kinase (CK), urine and plasma aminogram, urine organic acid profile, acyl carnitine profile, enzymatic analysis for arylsulfatase, galactocerebrosidase, very long chain fatty acids, acetyl aspartase were also performed when needed. Patients were classified according to their clinical phenotypes into: mitochondrial leukoencephalopathy (3/31), Leigh syndrome (7/31), neuropathy, ataxia and retinitis pigmentosa (2/31), mitochondrial encephalomyopathy (6/31), Leber?s hereditary opticneuropathy (4/31), mitochondrial encephalopathy, red ragged fibers (MERRF) (1/31). Mitochondrial encephalopathy, lactic acidosis (MELAS) (1/31), familial dystonia (4/31), and mitochondrial myopathy (3/31). The main neurological manifestations in their order of frequency were global developmental delay (20; 64.5%), spasticity (16; 51.6%), hypotonia (12; 38.7%), convulsions (10; 32.3%), sensory neural deafness (7; 22.6%), dystonia (7; 22.6%) optic atrophy (5; 16.1%), proximal muscle weakness (3; 9.7%), retinitis pigmentosa (2; 6.5%) and ataxia (2; 6.5%). Electromyography and nerve conduction study were normal in 18/31 (58%), myopathic in 7/31 (22.6%) and neuropathic in 6/31 (19.4%) patients. CK was significantly increased in only 3/31 (9.7%) patients. The main MRI findings were basal ganglia abnormalities appearing as bilateral and symmetrical high signal intensity lesions on T2 weighted MRI (11/28; 39.3%). Magnetic resonance spectroscopy (MRS) was performed in 7 patients and all were showing an increase lactate peak. EM examination revealed a subsarcolemmal aggregation of mitochondria in (12/18; 66.7%) and or a large and bizarre shaped mitochondria in (8/18; 44.4%), nonspecific myopathic changes in (3/18; 16.7%). Histochemical analysis of muscle biopsies using COX staining revealed a COX- vein (5/11; 45.5%), COX + vein (6/11; 54.5%), and myopathic changes in (3/18; 16.7%) patients. In conclusion, MRI and MRS are proved a promising tool in the diagnosis of mitochondrial cytopathies. MRS is a sensitive, non-invasive tool to evaluate the pathological lactate production in metabolic brain disease with disturbed energy production.

Background Cystic fibrosis (CF) is the most common life-limiting autosomal recessive disease among people in the USA and Europe with increased prevalence in Egypt. Affected children are in danger of acute kidney injury and the development of chronic renal disease through exposure to multiple nephrotoxic agents. N-acetyl beta-D-glucosaminidase (NAG) is a lysosomal enzyme present in high concentrations in the proximal tubular cells, thus raising urinary NAG levels to reflect tubular dysfunction. The aim of our study is to detect the role of the urinary NAG enzyme in the early detection of renal impairment in CF patients. This cross-sectional study enrolled 40 CF patients diagnosed in the CF Clinic in Children’s Hospital of Cairo University. They were age- and sex-matched to 40 healthy controls. All patients had glomerular filtration rate (GFR), serum creatinine, blood urea nitrogen (BUN), albumin/creatinine (A/C) ratio measured, urine analysis, urinary NAG enzyme using enzyme-linked immunosorbent assay (ELISA), and renal ultrasound (U/S) were done. Results Our study showed high levels of urinary NAG in cases with a significant difference between cases and controls (P value < 0.001). There was a significant correlation between urinary NAG enzyme elevation and A/C ratio in urine, nephrotoxic drugs administration, and duration of disease (P value = 0.002, 0.005, 0.019), respectively. Conclusion Our study suggested that the NAG enzyme is a good early detector of renal impairment in CF patients before the conventional laboratory assays become deranged.