Slone's disease

Slone’s disease is a specific form of hereditary pancreatitis. It is a rare inherited condition characterized by recurrent episodes of acute pancreatitis attacks.

In 1972, Robert McElroy and Philip A. Christiansen reported a large pedigree known as the Slone family, residing in the neighbouring state of Kentucky, that was affected with a rare autosomal dominant form of hereditary pancreatitis.^[1]

In the United States, the majority of Slones patients have a lineage which can be traced back to Appalachia. It is estimated that at least 1,000 individuals are newly diagnosed with hereditary pancreatitis each year. As genetic testing increases, these numbers may escalate.

In about half of these cases the problem progresses to chronic pancreatitis, which is severe scarring of the pancreas. The first attack typically occurs within the first two decades of life, but can begin at any age.

Patients with Slone’s disese may have chronic abdominal pain, diarrhea, nausea, vomiting, malnutrition, or diabetes.

Laboratory tests performed during an attack usually detect high blood levels of amylase and lipase, which are enzymes released from the pancreas.

One patient in western Kentucky was diagnosed with pancreatitis at 6 weeks of age. She continued to have severe episodes of pancreatitis throughout her childhood. In 1990 at the age of 5, she had a Peustow procedure performed at the Mayo Clinic by Dr. Christopher Moir. Despite repeated procedures, she continues to have episodes of pancreatitis.

• http://freepages.genealogy.rootsweb.com/~fmitchel/sloan/traits.html
• http://www.pancreas.org/assets/hp_presentation/sld001.htm

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