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The Hunger Project Bolen Report
Ohm Society
Severe Congenital Neutropenia (SCN) Print E-mail

Dr. Kennedy Children born with severe congenital neutropenia lack neutrophils (a type of white blood cell that is important in fighting infection). These children suffer frequent infections from bacteria which in the past led to death in three-quarters of cases before 3 years of age. The gene involved in many cases has been identified. It is called ELA2. Males and females are affected equally. The treatment of children affected by severe congenital neutropenia with G-CSF (a white cell growth factor) strongly reduces the risk of sepsis by reversing neutropenia. However, SCN patients who respond to this treatment still have an elevated risk of succumbing to sepsis because the disease is usually caused by heterozygous mutations of ELA2. Treatment with G-CSF may not be sufficient to correct all of the functional deficiency of neutrophils, and this might account for the consistent risk of infections observed in SCN patients. Neverhteless, survival is greatly enhanced by G-CSF. G-CSF stands for "granulocyte-colony stimulating factor." It is an haematopoietic growth factor that works by encouraging the bone marrow to produce more white blood cells. Trade names for G-CSF are Neupogen®, Granocyte®, and Neulasta®.

The information in this article is not meant to be medical advice.�Treatment for a medical condition should come at the recommendation of your personal physician.

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