Pancreatitis

1 General Considerations: Embryology.- 1.1 Normal Development.- 1.2 Congenital Abnormalities.- 1.2.1 Aplasia, Hypoplasia, and Dysplasia.- 1.2.1.1 Definition.- 1.2.1.2 Clinical Presentation.- 1.2.1.3 Diagnosis.- 1.2.1.4 Treatment.- 1.2.2 Pancreas Divisum.- 1.2.2.1 Definition.- 1.2.2.2 Pathogenetic Implications.- 1.2.2.3 Clinical Presentation.- 1.2.2.4 Diagnosis.- 1.2.2.5 Treatment.- 1.2.3 Heterotopic Pancreas.- 1.2.3.1 Definition.- 1.2.3.2 Clinical Presentation.- 1.2.3.3 Diagnosis.- 1.2.3.4 Treatment.- 1.2.4 Annular Pancreas.- 1.2.4.1 Definition.- 1.2.4.2 Clinical Presentation.- 1.2.4.3 Diagnosis.- 1.2.4.4 Treatment.- 1.2.5 Congenital Cyst(s).- 1.2.5.1 Definition.- 1.2.5.2 Clinical Presentation.- 1.2.5.3 Diagnosis.- 1.2.5.4 Treatment.- References.- 2 General Considerations: Anatomy.- References.- 3 General Considerations: Physiology.- 3.1 Function of the Exocrine Pancreas.- 3.2 Pancreatic Enzymes.- 3.3 Enzyme Synthesis.- 3.4 Protective Mechanisms to Prevent Autodigestion.- 3.5 Pancreatic Secretagogues.- 3.5.1 Secretin.- 3.5.2 Cholecystokinin.- 3.5.3 Cholinergic Influence.- 3.6 Phases of Pancreatic Secretion.- 3.7 Ductular Secretion.- 3.8 Acinar Secretion.- 3.9 Action of Pancreatic Lipase.- 3.10 What Turns the Pancreas Off?.- References.- 4 General Considerations: Classification.- 4.1 Marseille Classification of 1963.- 4.2 Cambridge Classification of 1983.- 4.3 Revised Pancreatitis Classification of Marseille 1984.- 4.4 Pancreatitis Classification of Marseille-Rome 1988.- 4.5 Pancreatitis Classification — Cambridge vs. Marseille: Features in Common and Differences.- 4.6 Classification System for Acute Pancreatitis of Atlanta 1992.- 4.7 Comment.- References.- 5 Acute Pancreatitis: Etiology.- 5.1 Biliary Tract Disease.- 5.2 Alcoholism.- 5.3 Obstruction of Pancreatic Ducts.- 5.3.1 Tumors.- 5.3.2 Duodenal Disorders.- 5.3.3 Pancreas Divisum.- 5.3.4 Helminthic Obstruction.- 5.3.5 Foreign Body Obstruction.- 5.4 Infections.- 5.5 Drugs.- 5.6 Toxins.- 5.7 Endocrine and Metabolic Disorders.- 5.7.1 Primary Hyperparathyroidism and Hypercalcemia.- 5.7.2 Pregnancy.- 5.7.3 Hyperlipemia.- 5.7.4 Uremia.- 5.7.5 Acute Intermittent Porphyria.- 5.7.6 Hypothermia.- 5.8 Vascular Disease.- 5.9 Trauma.- 5.10 Medical Procedures.- 5.10.1 Pancreatic Biopsy.- 5.10.2 Endoscopic Retrograde Cholangiopancreatography and Endoscopic Sphincterotomy.- 5.10.3 Sphincter of Oddi Manometry.- 5.11 Surgical Procedures.- 5.12 Cystic Fibrosis.- 5.13 Inborn Errors of Metabolism.- 5.14 Reye’s Syndrome.- 5.15 Kawasaki Disease.- 5.16 Hereditary Acute Pancreatitis.- 5.17 Idiopathic Acute Pancreatitis.- References.- 6 Acute Pancreatitis: Pathophysiology.- 6.1 Mechanisms of Pancreatic Injury.- 6.2 Gallstone-induced Acute Pancreatitis.- 6.3 Alcohol-induced Acute Pancreatitis.- 6.4 Concept of Oxidative Stress.- 6.5 Theories Explaining the Pathogenetic Mechanism of Acute Biliary Pancreatitis.- 6.6 The Pros and Cons of the Theories.- References.- 7 Acute Pancreatitis: Pathology.- 7.1 Gross Pathology.- 7.2 Histopathology.- 7.3 Sequential Changes and Outcome.- References.- 8 Acute Pancreatitis: Epidemiology.- References.- 9 Acute Pancreatitis: Diagnosis.- 9.1 Clinical Manifestation.- 9.1.1 Signs and Symptoms.- 9.1.2 Physical Examination.- 9.2 Laboratory Investigations.- 9.2.1 Enzymes.- 9.2.1.1 Serum Amylase.- 9.2.1.2 Macroamylasemia.- 9.2.1.3 Pancreatic Isoamylase.- 9.2.1.4 Urinary Amylase.- 9.2.1.5 Amylase-Creatinine Clearance Ratio.- 9.2.1.6 Serum Lipase.- 9.2.1.7 Macrolipasemia.- 9.2.1.8 Other Enzymes: Trypsin, Elastase-1, Phospholipase A.- 9.2.2 Other Laboratory Investigations.- 9.3 Imaging Procedures.- 9.3.1 Survey Film of the Abdomen.- 9.3.2 Chest Radiography.- 9.3.3 Barium Studies.- 9.3.4 Abdominal Ultrasound.- 9.3.5 Endoscopic Ultrasound.- 9.3.6 Computed Tomography.- 9.3.7 Magnetic Resonance Imaging.- 9.3.8 Endoscopic Retrograde Cholangiopancreatography.- 9.4 Synopsis of Diagnostic Procedures and Grading of Severity.- 9.4.1 Introduction.- 9.4.2 Early Prognostic Signs.- 9.4.2.1 Ranson’s Signs.- 9.4.2.2 Imrie’s Signs.- 9.4.2.3 Other Prognostic Systems.- 9.4.2.4 Peritoneal Lavage.- 9.4.2.5 Clinical Scoring Systems.- 9.4.2.6 APACHE-II Scores.- 9.4.2.7 Serum Markers.- 9.4.2.8 Urine Tests.- 9.4.2.9 Obesity.- 9.4.2.10 Chest X-ray.- 9.4.2.11 Summary.- 9.4.3 Organ Failure.- 9.4.4 Local Complications.- 9.4.5 Mortality.- References.- 10 Acute Pancreatitis: Complications.- 10.1 Local Complications.- 10.1.1 Acute Fluid Collection.- 10.1.2 Pancreatic Necrosis.- 10.1.3 Acute Pseudocyst.- 10.1.4 Pancreatic Abscess.- 10.2 Systemic and Other Extrapancreatic Complications.- 10.2.1 Shock.- 10.2.2 Renal Insufficiency.- 10.2.3 Respiratory Insufficiency.- 10.2.4 Cardiac Complications.- 10.2.5 Common Bile Duct Obstruction.- 10.2.6 Gastrointestinal Bleeding.- 10.2.7 Stenosis of an Adjacent Hollow Organ (Duodenum, Colon, Ureter).- 10.2.7.1 Stenosis of the Duodenum.- 10.2.7.2 Stenosis of the Colon.- 10.2.7.3 Stenosis of the Ureter.- 10.2.8 Ileus of the Small Intestine.- 10.2.9 Fat Necrosis.- 10.2.10 Pancreatic Encephalopathy.- 10.2.11 Cutaneous Manifestations.- 10.2.12 Ophthalmological Changes.- References.- 11 Acute Pancreatitis:Treatment.- 11.1 Basic Treatment.- 11.1.1 Goals.- 11.1.1.1 Limitation of Systemic Complications.- 11.1.1.2 Prevention of Pancreatic Necrosis.- 11.1.1.3 Prevention of Pancreatic Infection.- 11.1.2 Medical Management of Mild Pancreatitis.- 11.1.2.1 Supportive Care.- 11.1.2.2 Other Measures.- 11.1.2.3 Refeeding.- 11.1.3 Medical Management of Severe Pancreatitis.- 11.1.3.1 Fluid Resuscitation.- 11.1.3.2 Respiratory Care.- 11.1.3.3 Cardiovascular Care.- 11.1.3.4 Relief of Pain.- 11.1.3.5 Limitation of Systemic Complications.- 11.1.3.6 Treatment of Infection.- 11.1.3.7 Metabolic Complications.- 11.1.3.8 Nutritional Support.- 11.2 Treatment of Local Complications.- 11.2.1 Necrotizing Pancreatitis.- 11.2.1.1 Infected Necrosis of the Pancreas.- 11.2.1.2 Sterile Necrosis of the Pancreas.- 11.2.1.2.1 Necrotizing Pancreatitis With Clinical Improvement..- 11.2.1.2.2 Necrotizing Pancreatitis Without Clinical Improvement.- 11.2.2 Treatment of Pancreatic Pseudocyst.- 11.2.2.1 Medical Treatment.- 11.2.2.2 Surgical Treatment.- 11.2.2.3 Radiologic Treatment.- 11.2.2.4 Endoscopic Treatment.- 11.2.2.5 Pancreatic Pseudocysts Associated With Pancreatic Necrosis.- 11.2.2.6 Pancreatic Abscess.- 11.2.2.7 Smoldering Pancreatitis.- 11.3 Treatment of Systemic Complications.- 11.3.1 Shock.- 11.3.2 Renal Insufficiency.- 11.3.3 Respiratory Insufficiency.- 11.3.4 General Systemic Therapy (Removal of Activated Pancreatic Enzymes).- 11.4 Treatment of Metabolic Complications.- 11.4.1 Hyperglycemia.- 11.4.2 Hypocalcemia.- 11.4.3 Hypomagnesemia.- 11.5 Treatment of Extrapancreatic Complications.- 11.5.1 Obstruction of Common Bile Duct, Duodenum, Colon, and Ureter.- 11.5.1.1 Pancreatic Pseudocyst.- 11.5.2 Gastrointestinal Bleeding.- References.- 12 Acute Pancreatitis: Prognosis.- 12.1 Introduction.- 12.2 Etiology.- 12.3 Initial or Repeated Episodes.- 12.4 Severity.- 12.4.1 General.- 12.4.2 Clinical Parameters.- 12.4.3 Biochemical Severity Predictors.- 12.4.3.1 Scoring Systems.- 12.4.3.1.1 Ranson’s Prognostic Criteria.- 12.4.3.1.2 Imrie’s Prognostic Criteria.- 12.4.3.1.3 Bank’s Prognostic Criteria.- 12.4.3.1.4 Agarwal’s and Pitchumoni’s Simplified Prognostic Criteria.- 12.4.3.1.5 Hong Kong Criteria.- 12.4.3.1.6 APACHE-II Scores.- 12.4.3.1.7 Comparison of Multiple-Parameter Prognostic Systems.- 12.4.3.2 Peritoneal Lavage for Prognosis.- 12.4.3.3 Single Markers.- 12.4.4 Imaging Procedures.- 12.5 Systemic Complications.- 12.6 Mortality.- 12.7 Late Morphological and Functional Consequences of the Pancreas.- 12.8 Quality of Life.- References.- 13 Chronic Pancreatitis: Etiology.- 13.1 Alcohol-induced Chronic Pancreatitis.- 13.2 Idiopathic Chronic Pancreatitis.- 13.3 Tropical Pancreatitis.- 13.4 Hereditary Diseases of the Pancreas.- 13.4.1 Hereditary Pancreatitis.- 13.4.2 Cystic Fibrosis.- 13.5 Congenital Abnormalities Including Pancreas Divisum.- 13.6 Obstructive Chronic Pancreatitis.- 13.7 Drug-induced Chronic Pancreatitis.- 13.8 Stress-induced Chronic Pancreatitis.- 13.9 Radiation-induced Chronic Pancreatitis.- 13.10 Hyperparathyroidism.- References.- 14 Chronic Pancreatitis: Pathophysiology.- 14.1 Concept of Primary Intraductal Obstruction.- 14.2 Concept of Primary Toxic-Metabolic Action of Alcohol.- 14.3 Concept of Oxidative Stress.- 14.4 Concept of Necrosis-Fibrosis Sequence.- References.- 15 Chronic Pancreatitis: Pathology.- 15.1 Gross Pathology.- 15.2 Histopathology.- 15.3 Chronic Pancreatitis With Focal Necrosis.- 15.4 Chronic Pancreatitis With Segmental or Diffuse Fibrosis.- 15.5 Obstructive Chronic Pancreatitis.- References.- 16 Chronic Pancreatitis: Epidemiology.- References.- 17 Chronic Pancreatitis: Diagnosis.- 17.1 Clinical Manifestation.- 17.1.1 Signs and Symptoms.- 17.1.2 Physical Examination.- 17.2 Laboratory Investigations.- 17.2.1 Basic Laboratory Tests.- 17.2.2 Tests of Exocrine Pancreatic Function.- 17.2.2.1 General Remarks.- 17.2.2.2 Direct Pancreatic Function Tests.- 17.2.2.2.1 Determinations in Duodenal Juice.- 17.2.2.2.2 Secretin Test.- 17.2.2.2.3 Secretin-Cholecystokinin-Pancreozymin (Cerulein) Test.- 17.2.2.2.4 Lundh Test.- 17.2.2.3 Indirect Pancreatic Function Tests.- 17.2.2.3.1 Parotid Saliva Test.- 17.2.2.3.2 Serum Tests.- 17.2.2.3.3 Fecal Tests.- 17.2.2.3.4 Urine Tests.- 17.2.2.3.5 Value of Indirect Pancreatic Function Tests to Confirm or to Excluce Exocrine Pancreatic Insufficiency.- 17.2.2.3.6 Plasma Amino Acid Consumption Test.- 17.2.2.3.7 Breath Analysis Tests.- 17.2.2.3.8 Dual-Label Schilling Test.- 17.2.2.3.9 Pancreatic Polypeptide.- 17.2.3 Tests of Endocrine Pancreatic Function.- 17.3 Imaging Procedures.- 17.3.1 Conventional Radiology.- 17.3.2 Ultrasound and Computed Tomography.- 17.3.3 Magnetic Resonance Imaging.- 17.3.4 Endoscopic Retrograde Cholangiopancreatography.- 17.3.5 Endoscopic Ultrasound.- 17.3.6 Angiography.- 17.3.7 Pancreatic Duct Manometry.- 17.4 Synopsis of Diagnostic Procedures.- 17.4.1 General.- 17.4.2 Case History.- 17.4.3 Physical Examination.- 17.4.4 Basic Laboratory Tests.- 17.4.5 Basic Imaging Procedures.- 17.4.6 Gastroscopy.- 17.4.7 ERCP or Function Test: Which Step Next?.- References.- 18 Chronic Pancreatitis: Complications.- 18.1 General.- 18.2 Intrapancreatic Complications.- 18.2.1 Pancreatic Calcifications.- 18.2.1.1 Treatment.- 18.2.2 Pancreatic Pseudocysts.- 18.2.2.1. Treatment.- 18.2.3 Abscess.- 18.2.3.1 Treatment.- 18.2.4 Acute Necrotizing Pancreatitis.- 18.2.4.1 Treatment.- 18.2.5 Pancreatic Carcinoma.- 18.2.5.1 Treatment.- 18.3 Extrapancreatic Complications.- 18.3.1 Stenosis of Adjacent Viscera.- 18.3.1.1 Duodenal Stenosis.- 18.3.1.1.1 Treatment.- 18.3.1.2 Colonic Stenosis.- 18.3.1.2.1 Treatment.- 18.3.2 Stenosis of the Common Bile Duct.- 18.3.2.1 Treatment.- 18.3.3 Peptic Ulcer.- 18.3.3.1 Treatment.- 18.3.4 Gastrointestinal Bleeding.- 18.3.4.1 Treatment.- 18.3.5 Pleural Effusion.- 18.3.5.1 Treatment.- 18.3.6 Ascites.- 18.3.6.1 Treatment.- 18.3.7 Splenic Changes.- 18.3.7.1 Treatment.- 18.3.8 Osseous Lesions.- 18.3.8.1 Treatment.- 18.3.9 Metabolic Consequences.- 18.3.9.1 Treatment.- 18.3.10 Associated Diseases.- 18.3.10.1 Treatment.- 18.3.11 Extrapancreatic Carcinomas.- 18.3.11.1 Treatment.- References.- 19 Chronic Pancreatitis: Treatment.- 19.1 Pain.- 19.1.1 Definition and Mechanisms.- 19.1.1.1 Pancreatic Causes.- 19.1.1.1.1 Acute Inflammation.- 19.1.1.1.2 Increased Intrapancreatic Pressure.- 19.1.1.1.3 Parenchymal Abnormalities.- 19.1.1.1.4 Pancreatic Pseudocysts.- 19.1.1.1.5 Neural Inflammation.- 19.1.1.2 Extrapancreatic Causes.- 19.1.2 Conservative Treatment.- 19.1.2.1 Oral Pancreatic Enzyme Therapy.- 19.1.2.2 Endoscopic Treatment.- 19.1.2.3 Nerve Block.- 19.1.3 Surgical Treatment.- 19.2 Exocrine Pancreatic Insufficiency.- 19.2.1 Definition and Mechanisms.- 19.2.2 Salient Clinical Features.- 19.2.3 Pancreatic Enzyme Replacement.- 19.2.3.1 Aim.- 19.2.3.2 Indications.- 19.2.3.3 Choice of Enzyme Preparations.- 19.2.3.4 Choice of Administration Schedule.- 19.2.4 Interactions, Contraindications and Side Effects.- 19.2.5 Control of Response.- 19.2.6 Failure to Respond.- 19.2.7 Gastric Acid Inactivation of Pancreatic Enzyme Preparations.- 19.2.8 Nonsimultaneous Gastric Emptying of Pancreatic Enzyme Preparations With the Food.- 19.2.9 Factors Preventing Total Elimination of Steatorrhea.- 19.2.10 Conclusions, Recommendations, and Future Aspects.- 19.3 Endocrine Pancreatic Insufficiency.- 19.3.1 Definition and Mechanisms.- 19.3.2 Treatment of Endocrine Pancreatic Insufficiency.- 19.4 Postoperative Syndromes.- 19.4.1 Definition.- 19.4.2 Disease-related Postoperative Pain Symptoms.- 19.4.3 Exocrine Pancreatic Insufficiency.- 19.4.3.1 Treatment.- 19.4.4 Endocrine Pancreatic Insufficiency.- 19.4.4.1 Treatment.- 19.4.5 Surgery-related Sequelae.- 19.4.5.1 Sequelae of Gastric Resection.- 19.4.5.2 Sequelae of Biliodigestive Anastomosis.- 19.4.5.3 Sequelae of Duct Drainage.- 19.5 Evaluation of Follow-up Results.- 19.5.1 Quality of Life.- 19.5.2 Evaluation of Conservative and Surgical Treatment Results.- 19.5.3 Outlook.- References.- 20 Chronic Pancreatitis: Prognosis.- 20.1 Introduction.- 20.2 Pain.- 20.2.1 Painless Pancreatitis.- 20.2.2 Does Pain Decrease Differently in the Alcohol-induced and Idiopathic Chronic Pancreatitis?.- 20.2.3 Does Pain Decrease With the Duration of the Disease?.- 20.2.4 Does Pain Decrease With Progressing Exocrine Pancreatic Insufficiency?.- 20.2.5 Does Pain Decrease When Pancreatic Calcifications and/or Duct Abnormalities Occur?.- 20.2.6 Does Pain Decrease With Alcohol Abstinence?.- 20.2.7 Does Pain Decrease After Surgery?.- 20.3 Exocrine Pancreatic Insufficiency.- 20.4 Endocrine Pancreatic Insufficiency.- 20.5 Complications.- 20.6 Pancreatic and Extrapancreatic Carcinomas.- 20.7 Quality of Life.- 20.8 Mortality.- 20.9 Factors of Prognosis.- References.- 21 Addendum: Hereditary Pancreatic Diseases.- 21.1 Hereditary Pancreatic Diseases Associated With Exocrine Pancreatic Insufficiency.- 21.1.1 Cystic Fibrosis.- 21.1.1.1 General.- 21.1.1.2 Pathology.- 21.1.1.3 Clinical Presentation.- 21.1.1.4 Diagnosis.- 21.1.1.5 Treatment.- 21.1.2 Shwachman Syndrome.- 21.1.2.1 General.- 21.1.2.2 Pathology.- 21.1.2.3 Clinical Presentation.- 21.1.2.4 Diagnosis.- 21.1.2.5 Treatment.- 21.1.3 Johanson-Blizzard Syndrome.- 21.1.3.1 General.- 21.1.3.2 Pathology.- 21.1.3.3 Clinical Presentation.- 21.1.3.4 Treatment.- 21.1.4 Sideroblastic Anemia.- 21.1.5 Isolated Defects.- 21.2 Hereditary Diseases of the Exocrine Pancreas Associated With Pancreatitis.- 21.2.1 Hereditary Pancreatitis.- 21.2.1.1. Definition.- 21.2.1.2 Pathology.- 21.2.1.3 Clinical Presentation.- 21.2.1.4 Diagnosis.- 21.2.1.5 Treatment.- 21.2.1.6 Prognosis.- 21.2.2 Antitrypsin Deficiency.- 21.2.3 Inborn Errors of Metabolism.- References.