Oxidative Stress in Hemodialysis Pediatric Patients.

BACKGROUND: Oxidative stress may play a role in complications of hemodialysis patients as atherosclerosis, thrombosis, and inflammation. AIM: The aim of the study was to evaluate the oxidative stress in hemodialysis pediatric patients through measurement of oxidative stress enzymes as paraoxanase activity (PON), arylesterase activity (ASA), superoxide dismutase (SOD) and also non-enzymatic antioxidant vitamins as vitamins A, C and E levels. METHODS: The study included 50 hemodialysis pediatric patients with mean age 11.4 ± 5.4 years and 30 normal children of matched sex and age as a control group. Assessment of oxidative stresses was done using ELIZA technique. RESULTS: SOD, ASA, and vitamin C were significantly lower among hemodialysis patients in comparison to control group (p = 0.004, 0.004, > 0.001 respectively). CONCLUSION: The study concluded that oxidative stress was common finding in hemodialysis pediatric patients which may play a role in complications encountered among these patients.

Low-grade Nonrhabdomyosarcoma Soft Tissue Sarcoma: What is Peculiar for Childhood.

BACKGROUND: Nearly half of soft tissue sarcomas are nonrhabdomyosarcomas (NRSTSs). The low-grade (LG) form comprises a heterogenous group of diseases that rarely metastasize but are known for local recurrence. AIM OF THE STUDY: The aim of the study was to retrospectively evaluate pediatric LG-NRSTS with regard to demography, survival, and factors affecting outcome in Egyptian patients. PATIENTS AND METHODS: The study reviewed 66 NRSTS patients who presented to the Pediatric Oncology Department, National Cancer Institute, Cairo University, between January 2008 and December 2013. RESULTS: Out of the reviewed cases 32 patients had LG tumors and were eligible for analysis. The male to female ratio was 1:1 and the median age was 7.5 years (range, 1 mo to 18 y). Desmoid fibromatosis (N=18) showed frequent local recurrence and nearly half of this group was alive without disease. No recurrence of the disease occurred in the nonfibromatosis group (n=14) and all patients were alive and free of disease. The 5-year overall survival was 88% for the entire group of study patients versus 45% for event-free survival. Tumors >5 cm in diameter and fibromatosis histology subtype were associated with lower EFS. CONCLUSIONS: LG-NRSTS generally has good prognosis, with overall survival reaching 90%. However, aggressive fibromatosis usually runs a poorer course in the form of high incidence of local recurrence and lower survival rates. This needs to be further assessed in larger prospective studies including novel therapies in addition to the current conventional modalities.

Cardiovascular Abnormalities in Egyptian Children with Mucopolysaccharidoses.

INTRODUCTION: The Mucopolysaccharidoses (MPS) are rare inherited metabolic disorders. They are characterized by the progressive systemic deposition of Glycosaminoglycans (GAGs). GAGs accumulate in the myocardium and the cardiac valves. Enzyme Replacement Therapy (ERT) is available for MPS I, II, and VI. However, ERT does not appear to improve cardiac valve disease in patients with valve disease present at the start of ERT. AIM: To evaluate the cardiac involvement in Egyptian children with MPS. MATERIALS AND METHODS: Echocardiograms (ECG) were done for 34 patients. Both quantitative and qualitative Glycosaminoglycans (GAGs) in urine and enzyme assay confirmed the diagnosis. Mitral, tricuspid and aortic valves were evaluated for increased thickness, regurgitation and/or stenosis, left ventricular chamber dimensions, septal and posterior wall thicknesses. RESULTS: The patients' age ranged from 0.9-16 years (median age 4 years). They included 19 cases of MPS I (55.9%), 3 cases of MPS II (8.8%), 2 cases of MPS III (5.9%), 6 cases of MPS IV (17.6%) and 4 cases of MPS VI (11.8%). Heart murmur was heard in 9 of the participants (9/34) (26%). However, 15 patients (15/34) (44%) revealed cardiac lesions on ECG examinations. Mitral regurge (47%), followed by pulmonary hypertension (40%), were the most frequent findings. CONCLUSION: The absence of Cardiac murmurs does not exclude the heart involvement. Cardiac valve dysfunction may not be reversible. Regular ECG should be routinely warranted in children with MPS and early ERT are recommended.

Limitations of Living Donor Liver Transplantation in Egyptian Children.

BACKGROUND/AIMS: In Egypt, the liver transplantation (LTx) program that became available since 2001 is a living donor program. We aimed to assess the obstacles to pediatric LTx. METHODS: Over a six-month-period, 41 pediatric patients were indicated for LTx; their ages ranged between 1.5 months to 17 years. Patients and potential donors were evaluated according to the program protocol. RESULTS: The obstacles for performing LTx were classified into recipient, donor and program obstacles or limitations. Each patient may have more than one limitation. Late presentation and co-morbid conditions were on the top of the recipient list of obstacles. Refusal of potential donors to donate was the commonest limitation on the donor side (33%). The commonest program limitations were young age and small size of the recipient. CONCLUSIONS: Limitations in recipient characteristics as well as donor shortage are still the main obstacles for living donor liver transplantation (LDLT) in our pediatric liver disease patients. Small weight and young age of potential LDLT candidates are the principle causes for delaying this life saving procedure. Increasing community awareness about living organ donation and nutritional support for end stage liver disease (ESLD) babies is pivotal, given our limitation to a living donor program.

Relationship between Biochemical Bone Markers and Bone Mineral Density in Patients with Phenylketonuria under Restricted Diet.

OBJECTIVE: Most of phenylketonuria (PKU) develops bone turnover impairment and low bone mineral density (BMD). Measurements of BMD reflect only bone mineral status but not the dynamics of bone turnover. Bone markers are a noninvasive tool useful for the assessment of bone formation and bone resorption processes. Our study was to assess the levels of bone markers in PKU in order to select a screen marker and detect the most specific marker which can be combined with BMD for appropriate follow up. METHODS: Thirty three classic PKU patients were studied. BMD and bone mineral content (BMC) were measured. Total alkaline phosphatase (ALP), osteocalcin (OC) and carboxy-terminal propeptide of type I collagen (CICP), osteoprotegerin (OPG), receptor activator of nuclear factor κß ligand (RANKL) and Deoxypyridinoline (DPD) were measured. Findings : Nineteen (57.6%) male and fourteen (42.4 %) female PKU patients were involved in the current study. Their mean age was 8.4±4.6 yrs and the age range 3-19 yrs. The control group consisted of twenty two (52.4%) males and twenty (47.6%) females. Their mean age was 8.5±3.3 yrs and th age range 2-17 yrs. Using the Z score values, there was a significant decrease of total BMC (TBMC-Z), BMD of the femoral neck BMD-FN-Z, BMD of lumbar vertebrae (BMD-L-Z), BMD-FN and DPD while RANKL increased. There was a negative correlation between CICP and TBMC and between CICP and BMD-L in these patients. Also, a negative correlation between ALP and TBMC and between ALP and BMD-L was observed. It was concluded that the ALP provides a good impression of the new bone formation in the PKU patients and it has a highly significant negative correlation with the many parameters of the bone mineral status beside the wide availability of inexpensive and simple methods. So a screening test and/or follow up for the PKU patients using ALP would be available. Once the level of ALP decrease is detected, one can combine it with BMD to explore the bone mineral status and with specific bone markers (OC, RANKL and DBD), to verify the dynamics of bone turnover. CONCLUSION: This schedule will reduce the risk of exposure of these patients to the risk hazards of DXA and limit its use only to a limited number of the highly suspected cases.

Comparative study of two mechlorethamine, vincristine, procarbazine, and prednisone derived chemotherapeutic protocols for the management of pediatric Hodgkin lymphoma (HL): single-center 5-year experience.

We aimed for the comparison of two protocols (OAP and COMP) as chemotherapy treatment in children with Hodgkin lymphoma (HL). A total of 119 children newly diagnosed with HD were divided to receive either the anthracycline-based OAP protocol or the alkylating-agent-based COMP protocol. Sixty patients received the OAP protocol and 59 patients received the COMP protocol. Complete response was achieved for 81.4% of patients treated with the COMP protocol versus 53.3% for those who received the OAP treatment. Toxic hepatitis or liver cell failure was recorded in 5% of patients treated with the COMP protocol. Complications were more frequent in those treated with the OAP protocol, as 6.8% developed heart failure and 20% showed toxic hepatitis or liver cell failure. The relapse rate was almost equal in both treatment arms. Patients treated with the COMP protocol achieved a better response and less toxicity but with similar survival to those given the OAP protocol.

Mycotoxins and child health: the need for health risk assessment.

The occurrences of mycotoxins as food contaminants in different localities particularly in developing countries and the inevitable exposure of populations and children to these toxins with probable adverse outcomes need be scientifically and systematically assessed. Health risk assessment developed in the 1980s is separate from risk management, both with risk communication form the risk analysis framework adopted by the World Health Organization. The process contributes increasingly to policy development, public health decision making, the establishment of mycotoxin regulations and research planning. However, the exercise of the risk assessment structured approach is not simple and is faced up to lack of data, capable infrastructure facilities and need for trained personnel and resources. Furthermore, adopted methodologies need be developed focusing on child characteristics and health concerns.

The spectrum of inherited bleeding disorders in pediatrics.

Inherited bleeding disorders (IBDs) are caused by quantitative and qualitative alterations of either platelets or plasma proteins involved in coagulation and fibrinolysis. Hemophilias are the most frequent IBDs; however, accumulated data from various studies reported that von Willebrand disease (VWD) is the most common cause of IBD, with an increased incidence of platelet function defects, mostly due to the increased rate of consanguinity in some communities. VWD is an inherited disorder of homeostasis due to quantitative or qualitative defect of von Willebrand factor. Data on its epidemiology and impact in developing countries are limited. The objective of this study was to assess the local prevalence of some IBD and establish the clinical and historical variables that are predictive for those bleeding disorders in pediatrics. The study involved 43 children with various bleeding manifestations and 15 age- and sex-matched controls, recruited from the Pediatrics Hematology Clinic at the National Research Centre, Sausan Mubarek children's hospital in Cairo, Egypt and the King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia. Hematological profile included platelet counts and function, prothrombin time, partial thromboplastin time, factor VIII antigen and its activity, factor IX antigen and its activity, von Willebrand factor and its activity assayed with multimeric analysis. A total of 12 (27.9%) children had VWD, 11 (25.5%) had hemophilia A, three (7%) had hemophilia B, seven (16.3%) had platelet dysfunction and 10 (23.3%) had bleeding with undiagnosed cause. Two of the VWD cases had type I, three had type II, four had type III and one case appeared to have type IIM and another to have IIB VWD. Bruising and epistaxis were the main symptoms in all children with VWD The majority of platelet dysfunction disorders were diagnosed as Glanzmann's thrombasthenia. VWD and Glanzmann's thrombasthenia should be considered not uncommon causes of IBDs in children in Egypt and Kingdom of Saudi Arabia. Routine hematological screening should be mandatory in children with positive family history of bruising and bleeding as a predictor for IBD.