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Chronic pancreatitis in children

Chronic pancreatitis in children






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    Chronic pancreatitis in children Chronic pancreatitis in children Presentation Transcript

    • 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.