This section contains 561 words (approx. 2 pages at 300 words per page) |
Shwachman-Diamond Syndrome
Shwachman-Diamond syndrome found in 1964, also known as Shwachman Syndrome (SDS), is a rare autosomal recessive, multi-systemic disorder affecting the pancreas, bone marrow, and skeleton. SDS is found in 1 out of every 500,000 children. When a child has SDS, the pancreas fails to produce the enzymes that are necessary to digest foods properly. Due to the exocrine pancreatic dysfunction (malabsorption), the child does not absorb enough nutrients, very often the fat-soluble vitamins, to grow and develop normally. Other problems occur like hematologic abnormalities, including increased risk of horrible transformation, skeletal abnormalities, short stature, and low neutrophil. The low counts of white blood cells (below 1500 cells/mm3) Neutropenia. Some children are affected in all bloodlines and some are not affected at all. Some have Cyclic Neutropenia and some have Chronic Neutropenia. Cyclic Neutropenia makes the bloodline cycle occur every 28 days, were as children with Chronic Neutropenia never have a...
This section contains 561 words (approx. 2 pages at 300 words per page) |