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Disease Of The Pancreas >
Hereditary  Pancreatitis
What is Hereditary
Pancreatitis?
What are
symptoms of Hereditary Pancreatitis?
What
procedures are used to diagnosis Hereditary Pancreatitis?
What
are the causes of Hereditary Pancreatitis?
What
treatment options are available for Hereditary Pancreatitis?
What is the
prognosis of Hereditary Pancreatitis?
About
Hereditary Pancreatitis
Hereditary Pancreatitis is a
rare inherited condition characterized by recurrent episodes of
acute pancreatitis attacks. In about half of these cases the
problem progresses to chronic pancreatitis, which is severe
scarring of the pancreas. Laboratory tests performed during an
attack usually detect high blood levels of amylase and lipase,
which are enzymes released from the pancreas. The first attack
typically occurs within the first two decades of life, but can
begin at any age. In the United States, it is estimated that at
least 1,000 individuals are affected with hereditary pancreatitis.
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Symptoms
of
Hereditary Pancreatitis
Patients with hereditary
pancreatitis may have chronic abdominal pain, diarrhea, nausea,
vomiting, malnutrition, or diabetes.
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Diagnosis
of
Hereditary Pancreatitis
The diagnosis of hereditary
pancreatitis by genetic testing can be a valuable tool.
Hereditary pancreatitis is a progressive disease with a high risk
of permanent problems.
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Causes,
incidence, and risk factors
of Hereditary Pancreatitis
Hereditary Pancreatitis is a genetic
disorder, which means that it is usually passed from one
generation to the next.
The symptoms of Hereditary
Pancreatitis are caused by a change to a specific gene.
Genes are the packages of information that control how our bodies
look and function. A single gene appears to be involved in 60-75%
of hereditary pancreatitis families. This gene produces the
"cationic trypsinogen" enzyme, which breaks down
the proteins present in the foods we eat. (In some research papers
cationic trypsinogen is called PRSS1).
When a change to a gene occurs,
the gene may no longer function properly. These gene changes are
called mutations. Currently, there are two common, and more
than 6 uncommon cationic trypsinogen gene mutations that are
associated with hereditary pancreatitis. The major mutations are
known as cationic trypsinogen "R122H", "N29I".
Families with Hereditary
Pancreatitis might carry one of these mutations, but usually not
more than one type of mutation. It is also possible that a family
with a strong history of Hereditary Pancreatitis may not carry any
of the currently known mutations. For this reason, it is believed
that additional genes and mutations that cause Hereditary
Pancreatitis are awaiting discovery.
Hereditary Pancreatitis has
also been linked to an increased lifetime risk of pancreatic
cancer. Pancreatic cancer is the 4th most leading cause of cancer
deaths among Americans.
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Treatment
of
Hereditary Pancreatitis
The treatment for hereditary
pancreatitis depends on the symptoms. However, the primary
treatment focuses on pain control and pancreatic enzyme
replacement. Surgery may be indicated to improve symptoms. Removal
of the entire pancreas can be performed to eliminate the source of
the problem, however in most cases this results in permanent
insulin-dependent diabetes.
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Prognosis
of
Hereditary Pancreatitis
As with any chronic disease,
the effects may last for months or years. Follow your physician's
suggested diet, prescribed medications, and other medical orders.
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lori.hagerty@ucphysicians.com