Impact of educational programme regarding chelation therapy on the quality of life for B-thalassemia major children.

Objectives Thalassemia is the most common genetic disorder in Egypt, with an estimated carrier rate of 9-10%. It is a genetic blood disorder which can be fatal if proper chelation is not received. The introduction of chelating agents capable of removing excessive iron from the body has dramatically increased life expectancy and improved the overall quality of life. The aim of this study was to assess the impact of educational programmes regarding chelation therapy on the quality of life of thalassemic children. Methods The study was carried out at the Mansoura University Children's Hospital in the period between March 2010 and May 2011. It included 173 B-thalassemia children (84 boys and 89 girls) with age ranging between 8-18 years. The researcher used a predesigned interviewing questionnaire to collect data regarding children's knowledge about thalassemia and its management, especially regarding chelation therapy. The paediatric quality-of-life inventory tool (Peds QL 4.0 generic core) was also used to assess the studied children's quality of life. Results There was a significant statistical difference of the studied children's knowledge regarding chelation therapy and their quality of life. Conclusion There was a positive effect of the educational programme in improving children's knowledge score and their quality of life. Application of educational programmes for thalassemic children and their nurses regarding chelation therapy and its importance in preventing thalassemia complications is established.

Adenocarcinoma of the colon in children: case series and mini-review of the literature.

Colon cancer is extremely rare in children. This article reports three cases of adenocarcinoma of the colon. A 12-year-old boy, a 13-year-old boy, and a 13-year-old girl presented with constipation and abdominal enlargement over a two-month duration. Abdominal ultrasound and barium enema confirmed a stenotic segment at the rectum with obvious shouldering. Adenocarcinoma was diagnosed following colonoscopic biopsy and laparotomy. We conclude that any child presenting with unexplained persistent constipation, abdominal distension or bleeding per rectum, colon cancer should be suspected and investigated with endoscopy or barium enema.

Vitamin e and N-acetylcysteine as antioxidant adjuvant therapy in children with acute lymphoblastic leukemia.

Although cancer therapies have experienced great success nowadays, yet the associated toxic response and free radicals formation have resulted in significant number of treatment-induced deaths rather than disease-induced fatalities. Complications of chemotherapy have forced physicians to study antioxidant use as adjunctive treatment in cancer. This study aimed to evaluate the antioxidant role of vitamin E and N-acetyl cysteine (NAC) in overcoming treatment-induced toxicity in acute lymphoblastic leukaemia (ALL) during the intensive period of chemo-/radiotherapy, almost the first two months of treatment. Forty children newly diagnosed with ALL were enrolled in this study. Twenty children (group I) have taken vitamin E and NAC supplementations with chemotherapy and the other twenty children (group II) have not taken any adjuvant antioxidant therapy. They were evaluated clinically for the occurrence of complications and by the laboratory parameters (blood levels of glutathione peroxidase (Glu.PX) antioxidant enzyme, malondialdehyde (MDA), tumor necrosis factor-alpha (TNF-alpha), liver enzymes, and bone marrow picture). Results revealed reduced chemotherapy and radiotherapy toxicity as evidenced by decreasing level of MDA, increasing level of Glu.Px and decreased occurrence of toxic hepatitis, haematological complications, and need for blood and platelet transfusions in group I compared to group II. We can conclude that vitamin E and NAC have been shown to be effective as antioxidant adjuvant therapy in children with ALL to reduce chemo-/radiotherapy-related toxicities during the initial period of treatment.

Unbalanced bone turnover in children with beta-thalassemia.

The life expectancy of patients with thalassemia has greatly improved over the last decade as a result of regular transfusions and increased compliance with iron chelation therapy, however, this improvement is often accompanied by a series of serious complications including osteopenia and osteoporosis. The pathogenesis of these skeletal disorders is multifactorial which may be due to hormonal deficiency, compromised nutritional status, bone marrow expansion due to erythroid hyperplasia, increased iron stores or desferrioxamine toxicity. The non invasive assessment of bone turnover has markedly improved with the development of specific and sensitive markers of bone formation. The aim of this work is to assess the value of bone formation markers in patients with beta-thalassemia. To achieve this goal, 36 patients with thalassemia were recruited in this study. There were 20 males (56.6%) and 16 females (44.4%) and their ages ranged from 3 to 18 years. A control group of 20 apparently healthy subjects of matched age and sex was used. The patients were selected from the outpatient clinic and inpatients of the Hematology/Oncology Unit of Mansoura University Children's Hospital (MUCH). The selected subjects were subjected to thorough history taking, clinical examination, radiological evaluation and laboratory investigations in the form of: complete blood count, serum iron, serum ferritin, total iron binding capacity, serum calcium, serum phosphorus and estimation of bone formation markers as alkaline phosphatase and osteocalcin. The results were as follows: serum calcium level was within normal range and showed no statistical significance (p = 0.176) when compared to the control group, while serum phosphorus level was significantly higher in thalassemic patients than the controls (p = 0.002); this may reflect hypoparathyroidism. Analysis of the level of bone formation markers showed serum alkaline phosphatase levels slightly higher in patients than controls but not significant (p = 0.055), and this elevation can be referred to associated liver disease in these patients. On the other hand, osteocalcin level was significantly lower in patients than controls (p = 0.011), and this may be due to osteoblast poisoning by iron overload. In conclusion, thalassemic patients have unbalanced bone turnover between the bone formation and resorption markers and this is evidenced by non significant changes or decreased levels of bone formation markers, while bone resorption is an active process.