Germline biallelic mutations in one of the mismatch repair genes, mutS homolog 2, mutS homolog 6, mutL homolog 1, or postmeiotic segregation increased 2, result in one of the most aggressive cancer syndromes in humans termed as constitutional mismatch repair deficiency (CMMRD). Individuals with CMMRD are affected with multiple tumors arising from multiple organs during childhood, and these individuals rarely reach adulthood without specific interventions. The most common tumors observed are central nervous system, hematological, and gastrointestinal malignancies.
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December 2017
Cancer is a global health problem particularly in developing countries where the burden of cancer is ever increasing and claiming the lives of about 100,000 children under the age of 15 years every year. Majority of these occur in the Low and Middle Income Countries (LMICs) where 90% of world children live. Contributing factors to this trend is the reduction of communicable diseases and emergence of new infections, improvement of nutrition and socio-economic conditions, industrialization and urbanization.
View Article and Find Full Text PDFBackground: Little is known about childhood ALL in the Middle East. This study was undertaken by MECCA as initial efforts in collaborative data collection to provide clinical and demographic information on children with ALL in the Middle East.
Procedure: Clinical and laboratory data for patients with ALL between January 2008 and April 2012 were prospectively collected from institutions in 14 Middle East countries and entered into a custom-built-database during induction phase.
Background And Objectives: Hydroxyurea (HU) is the standard treatment for severely affected children with sickle cell disease (SCD). Starting dose is 15-20 mg/kg/day that can be escalated up to 35 mg/kg/day. Ethnic neutropenia is common in this area of the world that requires judicious usage of myelosuppressive drugs.
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