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Aagenaes Syndrome. See Cholestasis- Lymphedema Syndrome ABCB4

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Aagenaes Syndrome. See Cholestasis- Lymphedema Syndrome ABCB4 Index A Adrenoleukodystrophy, neonatal, Anisakis, as gastritis cause, 52 Aagenaes syndrome. See Cholestasis- 274-275 Anthraquinone laxatives, adverse lymphedema syndrome Adriamycin murine model, 13, 17, 27 effects of, 139, 149 ABCB4 disease, 244-245 Agammaglobulinemia, X-linked, 80, 169 Antiangiogenic agents, as ABCBll disease, 240-241 Aganglionosis. See also Hirschsprung's hepatoblastoma treatment, 331 Abetalipoproteinemia, 69, 70 disease Antibiotics, as bile duct injury and Abscesses acquired, 145 loss cause, 226-227 crypt, ulcerative colitis-related, Ill, colonic atresia as diagnostic marker Anus 113 for, 22 atresia and stenosis of, 22-24 intestinal, Crohn's disease-related, 109 segmental, differentiated from development of, 25 perianal, 168 "skip" lesions, 137 fissures of, 109 Crohn's disease-related, 109 Alagille syndrome, 209, 221-224, 274 APECED syndrome, 83-84 Achalasia, anal, 137 bile duct paucity associated with, 210 Apolipoproteins, 69 Acid maltase deficiency. See Glycogen biliary atresia-related, 210 Appendicitis storage diseases, type II differentiated from nonsyndromic Burkitt's lymphoma-related, 174 Acquired immunodeficiency syndrome paucity of bile ducts, 227-228 mesenteric cysts as mimic of, 172 (AIDSj, conditions associated as hepatocellular carcinoma cause, 278 Appendix, carcinoid tumors of, 171 with liver disease associated with, 221 Arteriovenous malformations, hepatic, adenovirus infections, 107 liver histopathology in, 222-224, 314 anti-enterocyte antibodies, 78, 79-80 225, 226 Arthritis, inflammatory bowel disease­ chronic diarrhea, 84 as liver tumor cause, 305 related, 116 cryptosporidiosis, 86 Allergies Arthrogryposis-renal dysfunction­ cytomegalovirus infections, 86-87 as colitis cause, 100, 101 cholestasis (ARC) syndrome, esophageal disorders, 42, 43 as enteropathy cause, 75-78 237, 238, 242, 243 esophageal ulcers, 42 as esophagitis cause, 37, 38, 4Q-41, 43 Aspergillus, as chronic granulomatous infectious cholangiopathies, 220 Alpha-I-antitrypsin deficiency, disease cause, 83 neutropenic enterocolitis, 118 conditions associated with Aspirin toxicity, 43, 283 pseudo-Gaucher cells, 285 bile duct paucity, 224 Ataxia-telangiectasia, 81, 169,305,315 Acrodermatitis enteropathica, 71 biliary proliferation, 209, 210 ATP8Bl disease, 237-240 Adenocarcinoma cholestasis, 204, 276 Atresia in the cardia, 52 hepatitis, 270 anorectal, 22-24 choledochal cysts-related, 214 hepatocellular carcinoma, 278 biliary, 203, 204, 205-212 colonic, 113-114, 170 liver disease, 272-273, 276-277 classification of, 208 esophageal, 39, 40, 169 Alpha chain disease, 175 clinical findings in, 205 gastric, 52, 169 Alprenolol, as esophagitis cause, 43 differentiated from Alagille Peutz-Jeghers syndrome-related, 162 Amebiasis, 107-108, 168 syndrome, 221 prevalence of, 158 Amino acids malabsorption disorders, 68 embryonic/fetal form of, 207, 210 small-bowel, 169 Amino acids transport disorders, 70 etiology of, 206-208 Adenoma Amyloidosis, inflammatory bowel extrahepatic, 245, 247, 303 biliary, genetic diseases-related, 305 disease-related, 17 familial, 246 hepatic Andersen's (chylomicron retention) as hyperbilirubinemia cause, 204 Fanconi's anemia-related, 302, 303 disease, 69-70 intrahepatic, 221 glycogen storage disease-related, Anemia noncorrectable, 208 303-304 collagenous gastritis-related, 57 portoenterostomy treatment of, 205, hepatocellular, 320, 324 Fanconi's, 302, 303, 305, 324 207,208,209,210,211-212,213 incidence of, 301 iron-deficiency, 159, 172 rubella-related, 254 metabolic disease-related, 278, Angiofibroma, nasopharyngeal, 163 colonic, 22 303-304 Angioma, cerebral cavernous, 311 congenital duodenal, 10 Adenomatous polyposis coli (APC) Angiomyolipoma, hepatic, 305, 326 duodenal, 16, 19-20 gene mutations, 163-164 Angiosarcoma esophageal, 14-17 Adenovirus infections, 107, 108, 220 colonic, 173 gastric, 17-18 Adrenocorticotropic hormone-secreting hepatic, 305, 311, 316, 325, 333, 334 jejunal-ileal, 20-22 tumors, 323 microscopic pathology of, 323 multiple, 15 347 348 INDEX Autoimmune disorders extrahepatic Celecoxib, as hepatoblastoma as biliary atresia cause, 207-208 anomalous development of, treatment, 331 as chronic diarrhea cause, 78-80 193-194 Celiac disease, 50, 65, 73-75, 76 as primary sclerosing cholangitis normal development of, 191, 192 as gastritis cause, 52-53, 54 cause, 215 intrahepatic, normal development of, relationship with lymphocytic and Autoimmune polyglandular syndrome, 191-193 collagenous colitis, 118 83-84 Biliary tree carcinoma, 213 serologic testing in, 76 Biopsy, intestinal, normal histology in, Cerebrohepatorenal syndrome of B 66-67 Zellweger, 224, 227, 274-275 Bacterial infections. See also specific Blastocystis hominis infections, 86 Chagas' disease, 139 bacteria Blastogenesis, 3-4, 5 Chemotherapy, as esophagitis cause, as chronic diarrhea cause, 84-85 errors of, 12 43 as neonatal liver disease cause, 254 Blue rubber bleb nevus syndrome, 173 Chest pain, infectious esophagitis­ Bannayan-Riley-Ruvalcaba syndrome, B19 parvovirus infections, 253, 254 related, 41-42 158, 163. See also Ruvalcaba Bone marrow transplantation, 82, 107, Cholangiocarcinoma, 303, 304 syndrome 118, 225 choledochal cysts-related, 214 Barrett's esophagus, 39-40, 169 Botryoid rhabdomyosarcoma, 313 congenital hepatic fibrosis-related, B-cell disorders, primary, as chronic Bowel wall, generalized inflammation 195 diarrhea cause, 80-81 of, 145 genetic diseases-related, 305 Beckwith-Wiedemann syndrome, Brancher enzyme deficiency, 304 microscopic pathology of, 322-323 305-306, 309 BRCA-2, expression in primary sclerosing cholangitis- Bile, "Byler," 239, 240, 242 hepatoblastoma, 308-309 related, 218 Bile acid malabsorption disorders, 68 Breast milk, allergenic substances in, Cholangitis Bile acid synthesis disorders, 244-246, 76 congenital hepatic fibrosis-related, 275 BRIC. See Cholestasis, benign 195, 197 Bile ducts recurrent intrahepatic Cryptosporidium infection-related, common, duplicated, 194 Brunner's gland, hamartomas of, 168 81 dilatation of, 198, 212-214, 216 BSClL disease, 245, 246 cytomegalovirus infection-related, large, 192 Burkholderia infections, as chronic 224 paucity of, 220-224, 277-278 granulomatous disease cause, 83 sclerosing, 214-220 in biliary atresia, 211-212, 213 Byler disease, 237, 274, 303 biliary atresia associated with, 207 in cystic fibrosis, 291-292 as colon cancer risk factor, 113 nonsyndromatic, 210, 224-225 C inflammatory bowel disease- proliferation of, in neonatal Campylobacter infections, lOS, 112 related, 17 hepatitis, 251 Cancer. See also specific types of cancer neonatal, 218-219, 237, 246, 247 quantification of, 238, 240 as chronic diarrhea cause, 89-90 primary, 214-218 small, 192 as ganglionitis cause, 145 primary small-duct, 244 Bile duct to portal tract (BD/PT) ratio, Peutz-Jeghers syndrome-related, 162 secondary causes of, 219-220 in Alagille syndrome, 220, 222 Candidiasis Cholecystitis, 205 Bile reflux, 213 as chronic granulomatous disease Choledocholithiasis, as sclerosing Biliary agenesis, 208 cause, 83 cholangitis cause, 219 Biliary tract diseases, 203-236 esophageal, 41, 42 Cholelithiasis, cholesterol-gallstone, extrahepatic, 203-220 Carbohydrate-deficient glycoprotein 244 biliary atresia, 205-212, 213, syndrome, type C, 88 Cholestasis, 203-205 214 Carbohydrate malabsorption disorders, Alagille syndrome-related, 221 cholestasis, 203-205, 206 68-69 benign recurrent intrahepatic, 237, congenital dilation of the bile Carcinoid tumors, 170-172 238, 240 ducts, 198, 212-214, 216 appendiceal, 171 choledochal cyst-related, 214 sclerosing cholangitis, 214-220 choledochal cysts-related, 214 cystic fibrosis-related, 224 intrahepatic, 220-228 prevalence of, 158 cytomegalovirus infection-related, Alagille syndrome, 221-224, 225 primary hepatic, 323, 331 251-252 inflammatory and immune rectal, Peutz-Jeghers syndrome- differential diagnosis of, 206 disorders, 225-228 related, 162 extrahepatic, 206 nonsyndromic paucity of Cardia, gastric as hepatocarcinoma risk factor, 303 intrahepatic bile ducts, 210, anatomy of, 47, 48 high-y-glutamyl transpeptidase 224-225 in Helicobacter pylori infections, 52 forms of, 244 paucity of intrahepatic bile ducts, Carditis, chronic, 52 intrahepatic, 206 220-224, 277-278, 291-292 Caroli's disease, 197-198, 212 of pregnancy, 240 Biliary tree. See also Bile ducts Caroli's syndrome, 197, 212 low-y-glutamyl transpeptidase forms anomalies of, 193-194 Caustic ingestions of, 237, 238, 240, 242, 243, 244 dilatation of, biliary atresia as esophagitis cause, 43 metabolic disorders-related, 276 associated with, 207 as gastritis cause, 58 neonatal and infantile, 203-205, 270 INDEX 349 neonatal hepatitis-related, 251 eosinophilic, 86, 100 Contractile proteins, in idiopathic Norwegian. See Cholestasis­ fulminant, 114-115, 116 intestinal pseudo-obstruction, lymphedema syndrome Hirschsprung disease-related, 101 150 in older children, 205 infectious, polyps associated with, Corticosteroids, as primary sclerosing cholangitis­ 166 hemangioendothelioma related, 217, 218 interdeterminate, 115-116 treatment, 334 progressive familial intrahepatic, lymphocytic, 117, 118 Cortisone, as gastritis cause, 58 274,278,322-324 microscopic, 117-118 Cowden's disease, 158, 159, 160, 163 ABCBll disease form oC 240-241 pseudomembranous, 100, 104, Cow's milk allergy, 73, 76, 78 ATP8Bl disease form of, 237-240 106-107 Coxsackie B virus infections, 253 toxoplasmosis-related, 255 quiescent, 113, 116 Crigler-Najjar syndrome, 237, 238, 246 Cholestasis-Iymphedema syndrome, ulcerative Crohn's disease, 50 237,
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