Chronic pancreatitis in children
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Transcript of Chronic pancreatitis in children
Chronic Pancreatitis in Children: Focus on Pancreatic Divisum & Genetic Pancreatitis
Case Conference Joanna Yeh
October 27, 2011
Case Presentation
• 7 yo girl admit to CHLA in April 2011 for acute onset emesis and pain
• Pain was periumbilical, causing her to be sent home from school
• Pain seems to be worse with food and liquids
• She had low grade temperatures x 1 day at presentation
• Patient has had abdominal pain episodes for 2 years; diagnosed with “constipation”
• Prior to admission, patient had been seen by PMD and told she had “stomach flu”
• When family history was elicited, dad has hereditary pancreatitis (PRSSI and CFTR)
• Remaining H&P was non contributory
Further History
Physical Exam
• Temp 100.1 HR 121 BP 102/58 RR 20 Sat 99% RA
• Wt. 20 kg
• Abd: soft, non tender to deep palpation throughout but endorses intermittent subjective pain. Not distended, no masses. No HSM.
• Skin: no rashes, no papules
• Remaining exam normal
• At CHLA, labs notable for normal CBC, normal CMP, lipase 1030
• Abdominal ultrasound notable for prominent pancreatic duct, 3-4 mm, common bile duct 1 mm
• Abdominal CT was done with IV and PO contrast that showed no pancreatic inflammation
• MR cholangiopancreatography (MRCP) revealed pancreas divisum
Labs & Imaging
Chronic Pancreatitis Definition
• Progressive inflammatory disease of pancreas
• Characterized by irreversible structural changes that result in irreversible exocrine and endocrine pancreatic insufficiency
Etiology
• Congenital anomalies
• Genetic
• Toxic/metabolic
• Autoimmune
• Idiopathic
Congenital anomalies
• Pancreatic divisum • Choledochal cysts • Pancreatic duct duplication • Renal cysts • Congenital pancreatic cysts • Annular pancreas
Embryology
Anatomy
Different types of pancreas divisum
Divisum
• Diagnosis: – Endoscopic ultrasonography
– ERCP (endoscopic retrograde cholangiopancreatography)
– MRCP (magnetic resonance cholangiopancreatography)
• Divisum can be the sole etiology of acute or chronic pancreatitis, but not always
• Incidental divisum: 5-10% lifetime risk of developing pancreatitis
• Malignancy risk highest in dorsal pancreas
Divisum Treatment
• Endoscopy / Therapeutic ERCP
– Spincterotomy
– Papillary dilation
– Stent insertion
• Surgical
– Minor papilla spincterotomy
– Puestow procedure (pancreaticojejunostomy)
– Pancreatectomy
Genetic Pancreatitis
• PRSS1
cationic trypsinogen
• SPINK1
serine protease inhibitor Kazal type 1
• CFTR cystic fibrosis transmembrane conductance regulator
• Chymotrypsin C
Who should be tested?
– A family history of recurrent acute pancreatitis, idiopathic chronic pancreatitis, or childhood pancreatitis without a known cause
– Relatives known to carry mutations associated with hereditary pancreatitis
– Recurrent acute attacks of pancreatitis for which there is no identifiable cause
– An unexplained documented episode of pancreatitis as a child
– Idiopathic chronic pancreatitis, particularly when the onset of pancreatitis occurs before age 25
Genetic Pancreatitis: Management
• Small meals, low fat, good hydration
• Avoidance of alcohol and tobacco
• Monitor exocrine and endocrine function
– Enzyme supplementation
– Diabetes management
• Pancreaticojejunostomy / Pancreatectomy
• Islet cell autotransplantation
Pancreatic Enzymes
• Child<4 yo: 1000 units lipase/kg/meal
• Child>4 yo: 500 units lipase/kg/meal
• Max: 2500 units lipase/kg/meal
• Snack dose is about ½ of meal dose
• Enteric coated microspheres
• At UCLA inpatient, we have Zenpep (5) and Zenpep (20)
• Others: Creon, Pancreacarb, Pancreaze
• If sprinkle into food, should be relatively acidic to avoid dissolving enteric coating (applesauce, banana, sweet potato)
Patient Update
• Hospitalized in Oct 2011 to Santa Monica for another episode of pancreatitis
• Father has since undergone pancreatectomy and islet cell transplantation
• Her genetic testing was positive for CFTR (dad is positive for CFTR and PRSSI)
• Dr. Farrell is following her and may consider EUS in future. No plans for ERCP.
References • Takuma, et al, “Pancreatic disease associated with pancreas divisum,” Digestive Surgery, 2010, 27,
pg 144-8.
• Dumont, et al, “Pancreas divisum and the dominant dorsal duct syndrome,” Annals of Surgery, Jan 2005, 130, 1, pg 5-14.
• Bellin, et al, ‘Outcome After Pancreatectomy and Islet Autotransplantation in a Pediatric Population,” Journal of Pediactirc Gastroenterology and Nutrition, 2008, 47, p 37-44.
• “Surgical Treatment of Pancreas Divisum Causing Chronic Pancreatitis,” Journal of Gastrointestinal Surgery, May 2005, 9, 5, pg 710-5.
• Dray, et al, “Association of Pancreas Divisum and Recurrent Acute Pancreatitis,” Pancreas, 2007, 35, pg 90-3.
• Bhardwaj, et al, “A RCT of antioxidant supplementation for pain relief in patients with chronic pancreatitis,” Gastroenterology, 2009, 136, 1, pg 149.
• Kamisawa, et al, “Pancreatitis associated with congenital abnormalities of the pancreaticobiliary system,” Hepatogastroenterology, 2005, 52, 61, pg 223-9.
• Rosendahl, et al, “Hereditary chronic pancreatitis,” Orphanet Journal of Rare Diseases,” Jan 2007, 2, 1.
• Witt H, “Genetics of pancreatitis: a guide for clinicians,” Digestive Diseases, 2010, 28, 6, pg 702-8.
• Ferrone, et al, “Panreatic enzyme pharmacotherapy,” Pharmacotherapy, 2007, 27, 6, pg 910-20.