TY - CHAP M1 - Book, Section TI - Hereditary Pancreatitis A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - Genetic condition characterized by recurrent episodes of painful pancreatic attacks, which can progress to chronic pancreatitis. Symptoms include abdominal/epigastric pain, nausea, and vomiting. Onset may begin as early as birth, but most often the attacks typically occur between the first two decades of life. The presentation is associated with chronic pancreatitis, failure of the endocrine and exocrine functions with an increased risk of mortality caused by pancreatic cancer. Lifetime risk of cancer is estimated around 35 to 54% by the age of 75 years. Although there are numerous individuals that choose to have the pancreas surgically removed to prevent pancreatic cancer, a study of 189 patients in 2009 show no increased in mortality following conservative medical treatment. The anesthesia considerations include an increased risk of intra- and postoperative uncontrolled diabetes necessitating the administration of short-acting insulin. Also, a high incidence of nausea and vomiting must be taken into consideration at the time of induction of anesthesia and postoperatively. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/11/05 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164072631 ER -