Hereditary chronic pancreatitis: Implications for surgical treatment and follow-up

Hereditary pancreatitis is an uncommon cause of chronic pancreatitis in Wes tern society. It should be suspected when chronic pancreatitis presents in young adults. The diagnosis is made when chronic pancreatitis is present in several members of the same family who are determined not to have other ri sk factors for chronic pancreatitis. Molecular research focusing on mutatio ns in the trypsinogen gene has uncovered the genetic defects associated wit h hereditary pancreatitis, and this knowledge has suggested the possible pa thophysiologic mechanism of this disease, Because patients with hereditary pancreatitis develop their disease early in life they are very likely to re quire treatment for complications, As in patients with chronic pancreatitis of other etiologies those with hereditary pancreatitis should be treated m edically for acute exacerbations. When complications occur or when the dise ase causes intractable pain surgery is recommended. Surgical therapy is tai lored to the patient's pancreatic anatomy based on endoscopic retrograde ch olangiopancreatography or CT scan, The two patients described in this repor t underwent successful longitudinal pancreaticojejunostomy (Puestow procedu re) with good results, Finally it has been shown that patients with heredit ary pancreatitis are at increased risk for developing pancreatic adenocarci noma, Although not widely used pancreatic cancer screening programs have be en suggested for surveillance of these patients.