الفهرس | Only 14 pages are availabe for public view |
Abstract Background: Langerhans cell histiocytosis (LCH) is a challenging disease and may be manifested in a variety of ways, ranging from a spontaneously regressing solitary lesion of bone to a multisystem, lifethreatening disorder. Some forms require little if any treatment, and others need aggressive therapy. It is estimated that one in 200.000 children are affected each year. Over 75% of cases occur before the age of 10 years. However, epidemiological data are sparse and only one national incidence estimate (5.4 per million) has been reproted for Denmark, during the 1980?s (Carstensen H, Ornvold K,1993). It is estimated that there are between 50100 new cases per year in children in UK which has a childhood population of around 12 millions, and possibly an equal number of cases in adults. There are few epidemiological studies of LCH. Two large casecontrol studies have been reported from the USA, in which risk factors for the development of LCH were investigated. No unifying hypothesis regarding risk factors for LCH has emerged from these studies (Bhatia S et al., 1997). Aim of work: Clinico epidemiological study of cases of histiocytosis in children at Mansoura University Children?s Hospital, which drains Governorates of North East Egypt to throw a beam of light on this rare disease in this geographical area Methods: This study had been carried on children with Langerhans Cell Histiocytosis attending the oncology outpatient clinic whether taking their chemotherapy or finishing their treatment on follow up visits. A sheet for each of the patients will be done including the following: Age at presentation.,Sex.,Residence, Mode of presentation., Histological diagnosis., Radiological diagnosis. Laboratory investigations. ,The final out come of the disease. , Therapy given. Results: The highest incidence was reported in Dakahlia our main locality. In different districts of Dakahlia. We noticed increased incidence in Mansoura City. the most common clinical presentation was lymphadenopathy (65%), skin lesion and endocrinal lesions were less common (10% each) as regard other reported studies. Pulmonary lesions, haemopoietic lesions and hepatosplenomegaly were the least common (5% each). The vast majority of patients? We found that bone defect was reported in (40%) of the studied cases, represented as (skull defect, otitis media, tibial defect, proptosis; 25%, 10%, 5%, 5%, respectively). Other studies reported much higher incidence of bone lesions. Most of our studied cases showed good response to chemotherapy (65%). We found that, organ dysfunction was reported in (10%) of cases in the form of hepatic and pulmonary failure. We found that the overall survival rate was more than the overall mortality rate. Conclusion: Proper documentation, data collection and analysis of histiocytosis patients from different localities and all over the country. Cooperation and exchange of information between different oncology centers and via special recording system. Annual review of statistical data in different aspects for future evaluation of pediatric malignancy in Egypt. |