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• W2023000951 abstract "To the Editors: A 3-year-old girl presented with epigastric pain, vomiting, diarrhea, and anorexia. Blood results showed a serum lipase value of 10.784 U/L (normal 0–200 U/L), amylase 150 U/L (normal 0–85 U/L), alkaline phosphatase 218 U/L, ALT 23 U/L, AST 32 U/L, LD 695 U/L, and a total bilirubin of 3 μmol/L. Serologic tests showed a high antibody titer to Coxsackie virus type B5 (1:2048). IgM antibody for mumps, varicella zoster, rubella, measles, para-influenza, and cytomegalovirus were all undetectable. After a second episode of acute pancreatitis, a mutation in the serine protease inhibitor kazal type I (SPINK-1) was identified. Endoscopic ultrasonography (EUS) and retrograde cholangiopancreaticography (ERCP) showed a short distal stenosis in the pancreatic head. A 5 cm 7 Fr endoprosthesis was placed in the pancreatic duct. Exocrine pancreatic function remained undisturbed (elastase-1 levels >0.500). The child's mother also had a SPINK-1 mutation. SPINK-1 mutations are also found in 2% of patients without chronic pancreatitis.1,2 Therefore, the mutation by itself does not cause pancreatitis. Other factors (like in this case a Coxsackie type B5 infection3) must be present to cause pancreatitis. SPINK-1 mutation might predispose to chronic pancreatitis. Under normal conditions, SPINK-1 inhibits the transformation of trypsinogen in trypsin in the pancreas, by which auto-digestion is prevented. A SPINK-1 gene mutation leads to an increased trypsin activity in the pancreas, causing auto-digestion.2 In the case of a Coxsackie type B5 induced acute pancreatitis, screening for a SPINK-1 mutation is indicated if episodes of acute pancreatitis recur. Endoscopic ultrasonography is a useful diagnostic technique to visualize pancreatic duct abnormalities in children with chronic pancreatitis.4,5 Michael Groeneweg, MD, PhD Dept of Paediatrics, Sophia Children's Hospital Erasmus Medical Center Rotterdam Rotterdam, the Netherlands Dept of Paediatrics Maasstad Hospital Rotterdam, the Netherlands Jan W. Poley, MD, PhD Dept of Gastroenterology and Hepatology Erasmus Medical Center Rotterdam Rotterdam, the Netherlands Marlies Dansen, MD Dept of Paediatrics, Sophia Children's Hospital Erasmus Medical Center Rotterdam Rotterdam, the Netherlands Jan G. Brinkman, MD Dept of Paediatrics Maasstad Hospital Rotterdam, the Netherlands Johanna C. Escher, MD, PhD Edward E. S. Nieuwenhuis, MD, PhD Dept of Paediatrics, Sophia Children's Hospital Erasmus Medical Center Rotterdam Rotterdam, the Netherlands" @default.
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• W2023000951 date "2009-02-01" @default.
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• W2023000951 title "Chronic Hereditary Pancreatitis in a Girl With a Serine Protease Inhibitor Kazal Type I (SPINK-1) Gene Mutation and a Coxsackie Type B5 Infection" @default.
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• W2023000951 doi "https://doi.org/10.1097/inf.0b013e318193e7e4" @default.
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