Disorders of the Exocrine Pancreas Flashcards
What is the most common cause of pancreatic insufficiency in children?
Cystic fibrosis
What is Schwachmann-Diamond syndrome?
It is an autosomal recessive disorder which is characterized by short stature, intermittent or persistent neutropenia, skeletal abnormalities, and exocrine pancreas insufficiency. It is the 2nd most common cause of exocrine pancreas insufficiency, after cystic fibrosis.
What are the (4) commonly identified causes of acute pancreatitis in children?
Biliary obstruction from gallstones, idiopathic (many of these are probably viral), blunt abdominal trauma, and multisystem disease.
List four drugs which have been implicated in the development of acute pancreatitis.
Valproate, asparaginase, azathioprine, and didanosine (for HIV).
Describe the typical presentation of acute hemorrhagic pancreatitis (necrotizing pancreatitis).
It is rare in children, but is life-threatening. Patients are acutely ill with vomiting, abdominal pain, high fever, and shock. The Cullen sign and Grey Turner sign are classic on exam.
What is the typical progression of acute hemorrhagic pancreatitis (necrotizing pancreatitis)?
Over time the pancreas becomes necrotic and eventually, without therapy, transforms into an inflammatory hemorrhagic mass. The mortality rate is > 50%.
What is the Cullen sign?
It is one of the classic physical exam findings in acute hemorrhagic pancreatitis (necrotizing pancreatitis). It is characterized by bluish patchy discoloration around/near the umbilicus.
What is the Grey Turner sign?
It is one of the classic physical exam findings in acute hemorrhagic pancreatitis (necrotizing pancreatitis). It is characterized by bluish discoloration of the flanks from retroperitoneal hemorrhage.
What is the classic laboratory finding in acute pancreatitis?
Serum lipase levels are highly sensitive and specific for acute pancreatitis if elevated > 4x the upper limit of normal (i.e. in the hundreds). Higher lipase levels indicate more severe pancreatic inflammation.
Which radiologic test is usually most helpful in diagnosing acute pancreatitis?
Ultrasound of the abdomen is the easiest and best imaging study in children because it can directly visualize the pancreas, showing peripancreatic fluid or an increase in pancreatic size, which supports the diagnosis of acute pancreatitis.
When is endoscopic retrograde cholangiopancreatography (ERCP) most useful in the diagnosis and management of acute pancreatitis?
ERCP can be helpful in rarer cases, such as a stone in the bile duct, autoimmune hepatitis, sphincter of Oddi dysfunction, and other anatomic biliary causes.
What is the recommended management of acute pancreatitis?
IV hydration, pain control, and fasting, with resumption of enteral nutrition once the patient desires to eat. Antibiotics are not recommended, even in the most severe cases.
What is the typical management of pancreatic pseudocysts?
They typically resolve spontaneously; rarely, they require percutaneous drainage or endoscopic placement of a cystogastrostomy.
What is the most common cause of chronic pancreatitis?
It is rare in children, and some cases are due to AD hereditary pancreatitis, but most patients have idiopathic disease.
List three potential complications of chronic pancreatitis.
Pancreatic insufficiency develops in 50% of patients, diabetes mellitus develops in 25%, and there is an increased risk for development of pancreatic adenocarcinomas as well.
