Ashraf Bakr

Low turnover bone disease in Egyptian children with acute leukemia

Abstract The aim of this work was to study bone turnover markers, calcium homeostasis and bone mineral density (BMD) in children with acute leukemia at diagnosis, after induction chemotherapy, and during maintenance therapy to delineate abnormalities present. After evaluation of L2-L4 BMD using dual-energy X-ray absorptiometry in patients with acute myeloid and lymphoid leukemia at presentation and after treatment, the results were compared to 352 healthy age- and sex-matched Egyptian controls. Calcium homeostasis parameters and bone turnover biochemical markers (serum osteocalcin and urinary deoxypyridinoline) were also assayed and the results were compared to 12 healthy age- and sex-matched controls. Osteopenia was observed at diagnosis and during treatment in patients with acute leukemia. At diagnosis osteopenia was observed in 27 patients (62.8%): 10 (23.3%) had non severe osteopenia and 17 (39.5%) had severe osteopenia. This low BMD persisted in those who were followed up. Parathyroid hormone (PTH) (pg/ml) levels demonstrated non significant differences between children with acute leukemia at different stages of therapy and controls, while, 25 (OH) D3 (ng/ml) was significantly lower in acute leukemia patients at different stages of therapy compared to controls (p < 0.001). Osteocalcin (ng/ml) is significantly lower in patients at different stages of the disease compared to controls (p < 0.001) but there was no significant difference between patients at different stages of therapy. Deoxy-pyridoline cross links showed non-significant difference between the different types of acute leukemia and with controls. Osteopenia is a significant problem in children with acute leukemia at presentation and after chemotherapy. Osteopenia in acute leukemia appears to be of the low turnover type (decreased osteoblastic activity and decreased bone mineralization).

Low expression of glucocorticoid receptors in children with steroid-resistant nephrotic syndrome

BackgroundAbout 10–20 % of children with idiopathic nephrotic syndrome (NS) are steroid-resistant (SR). Low expression of glucocorticoid receptors (GRs) has been associated with poor response to steroids in a variety of autoimmune diseases. This study was done to assess the expression of cytoplasmic GRs for CD3 and CD14 in children with NS.MethodsExpression of cytoplasmic GRs in lymphocytes (CD3+/GR) and monocytes (CD14+/GR) in the peripheral blood were assessed in 51 children with NS before the start of therapy by flow cytometry. Patients were divided into two groups: 30 children who were steroid-sensitive (SSNS) and 21 children who had initial steroid resistance (SRNS). Twenty age- and sex-matched healthy children served as controls.ResultsExpression of CD3+/GR was significantly lower in SRNS in comparison to SSNS patients and controls (p < 0.0001). Similarly, expression of CD14+/GR was significantly lower in SRNS in comparison to SSNS patients (p < 0.0001) and controls (p = 0.002). CD3+/GR and CD14+/GR expression were not significantly different in SSNS patients compared with controls (p = 0.06 and 0.07 respectively).ConclusionsPatients with initial SRNS showed decreased GR expression in peripheral blood mononuclear cells (PBMC) before starting therapy, and this low expression may be one of the pathophysiological mechanisms of steroid resistance in these children.

LOW BONE MASS IN CHILDREN WITH MALIGNANT LYMPHOMA

The aim of this work was to study the effect of disease process on bone mass and calcium homeo-stasis in children with malignant lymphoma at diagnosis, 3 months after starting chemotherapy, and after 1 year. Evaluation of lumber vertebrae (L2–L4) bone mineral density using dual-energy X-ray absorptiometry and calcium homeostasis parameters and bone turnover biochemical markers (serum osteocalcin and urinary deoxypyridinoline) had been assayed in twenty lymphoma patients at presentation and after treatment. Low bone mass for chronological age was observed in 4 patients (20%) at diagnosis and persisted after 3 months and 1 year. Parathyroid hormone level demonstrated no differences between children with lymphoma at different stages of therapy and controls, while 25(OH) D3 was significantly lower in lymphoma patients at different stages of therapy as compared to controls (p <. 001). Osteocalcin was significantly lower in lymphoma patients at different stages of therapy. Deoxypyridinoline showed only significant higher values after 3 months of therapy compared to controls (p =. 01). In conclusion, low bone mass was observed in children with lymphoma and is related to decreased osteoblastic activity and decreased mineralization of bone.

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.

Assessing the Intelligence of Children with Chronic Kidney Diseases

To evaluate the level of intelligence in children with chronic kidney diseases (CKDs), 12 children with CKD stage 5 on regular hemodialysis, 12 children in the predialysis stage, and 12 as controls were assessed using the Wechsler Intelligence Scale for Children (WISC). Mean scores on the verbal, performance, and full scale IQ were significantly lower in the predialysis and dialysis children than in the healthy children, while the mean score on the arithmetic subtests was significantly lower in the predialysis children than in the dialysis group. In conclusion, a better understanding of the neurocognitive function in children with CKDs is a critical element to be ascertained early with proper assessment programs so as to design appropriate educational interventions for this handicapping illness.

Assessment of Nutritional Status in Children With Chronic Kidney Disease Using Hand Grip Strength Tool

OBJECTIVE Muscle status assessment is crucial for diagnosis of protein energy wasting PEW/cachexia in chronic kidney disease (CKD) population. Hand grip strength (HGS) has been used in muscle power assessment in adult CKD. However, no data is available about its usefulness in children with CKD. Hence, we aimed to study the reliability of HGS in reflecting the muscle power and thus, nutritional status in children with CKD. DESIGN AND METHODS In this Observational cross sectional study we enrolled 73 CKD children; 45 had end stage kidney disease (ESKD) on hemodialysis (HD) and 28 had CKD but not on dialysis yet. Assessment of childrens nutritional status was done through biochemical variables (serum albumin and serum cholesterol) and anthropometric measures (height and BMI). Body composition monitor (BCM) device was used for lean tissue mass (LTM) assessment whilst muscle power was tested by HGS tool. RESULTS The study showed that 69.8% of CKD patients had HGS values below 10th percentile for age and sex. Moreover, HGS was observed to be more affected in CRI patients and those with non - glomerular causes. HGS was also found to be positively correlated to height but not to lean tissue mass or serum albumin. CONCLUSION HGS tool can be used as a reliable bedside tool for nutritional assessment in children with CKD.

A novel fibrillin-1 mutation in an egyptian marfan family: A proband showing nephrotic syndrome due to focal segmental glomerulosclerosis.

Marfan syndrome (MFS), the founding member of connective tissue disorder, is an autosomal dominant disease; it is caused by a deficiency of the microfibrillar protein fibrillin-1 (FBN1) and characterized by involvement of three main systems; skeletal, ocular, and cardiovascular. More than one thousand mutations in FBN1 gene on chromosome 15 were found to cause MFS. Nephrotic syndrome (NS) had been described in very few patients with MFS being attributed to membranoproliferative glomerulonephritis secondary to infective endocarditis. Focal segmental glomerulosclerosis (FSGS) had been reported in NS in conjunction with MFS without confirming the diagnosis by mutational analysis of FBN1. We hereby present an Egyptian family with MFS documented at the molecular level; it showed a male proband with NS secondary to FSGS, unfortunately, we failed to make any causal link between FBN dysfunction and FSGS. In this context, we review the spectrum of renal involvements occurring in MFS patients.