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Home , Alagille syndrome, Genodermatosis, Lymphedema, Polysplenia

Index

A Adrenoleukodystrophy, neonatal, Anisakis, as gastritis cause, 52 Aagenaes syndrome. See Cholestasis- 274-275 Anthraquinone laxatives, adverse 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 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 paucity associated with, 210 Apolipoproteins, 69 Acid maltase deficiency. See Glycogen -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 as mimic of, 172 (AIDSj, conditions associated as cause, 278 Appendix, carcinoid tumors of, 171 with disease associated with, 221 Arteriovenous malformations, hepatic, adenovirus , 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 -related, 214 hepatocellular carcinoma, 278 biliary, 203, 204, 205-212 colonic, 113-114, 170 , 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 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 , 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 syndrome, 173 Chest pain, infectious esophagitis• Bannayan-Riley-Ruvalcaba syndrome, B19 parvovirus infections, 253, 254 related, 41-42 158, 163. See also Ruvalcaba 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 -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 -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 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, -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 -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- 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 , 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 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, 238, 240, 242, 243, clinical presentation of, 109 clinical presentation of, 109 245-246, 247, 274 as colon cancer cause, 113-114 as colorectal cancer risk factor, 114 Cholestatic syndromes, familial differentiated from Crohn's differential diagnosis of, 104, 106, intrahepatic, 209, 274, 303, 305 disease, 110-113, 114 110-113, 114, lIS, 120 Cholesterol ester storage disease, 286 as gastritis cause, 56-57 esophageal disorders associated with, Chondrodysplasia, 200 genetic factors in, 108 44,55 Choriocarcinoma, 326 occludin in, 65 extensive ileal resection in, 71 Chronic granulomatous disease, 50, 55, pathology of, 109-112, 113 fat malabsorption in, 69 56, 83, 111-112, 115 primary sclerosing cholangitis gastritis associated with, 55-56 Chylomicron retention (Andersen's) associated with, 214, 215 genetic factors in, 108 disease, 69-70 zonulin in, 65 ileocecal, 168 Chylous fluid, 172 Collagen, in collagenous gastritis,S7 occludin in, 65 CIRHIA disease, 245, 246 Collagen bands, in colitis, 117-118 pathology of, 109-112, 114 Colon primary sclerosing cholangitis Alagille syndrome-related, 221, 226 atresia and stenosis of, 22 associated with, 214, 215 autoimmune hepatitis-related, 262 mucosal histogenesis in, 10 zonulin in, 65 biliary, 211, 213 Colon cancer. See also Colorectal Cronkhite-Canada syndrome, 71, 158, choledochal cyst-related, 213, 214 cancer 159, 162-163 rubella-related, 254 colloid, 170 Cryptosporidiosis, 81, 85-86, 220 biliary atresia-related, 205 mucinous, 170 Cushing's syndrome, hepatic endocrine cystic fibrosis-related, 291-292 sclerosing cholangitis-related, 113 carcinoma associated with, 332 Indian childhood, 273-274, 289 in solitary juvenile polyps, 158-159 Cyclins, role in hepatoblastoma, metabolic disorders-related, 276-277 ulcerative colitis-related, 113-114 307-308 North American Indian childhood, Colonic specimens, major histologic Cyclophosphamide 207, 210, 237, 274, 276 patterns in, 100-101 as hepatoblastoma treatment, 330 parenteral nutrition-related, 301 Colonopathy, fibrosing, 120 as rhabdomyosarcoma treatment, 334 Wilson's disease-related, 274 Colorectal cancer, 157, 169-170 Cystic fibrosis, conditions associated Cisplatinum Crohn's disease-related, 114 with as hepatic sarcoma treatment, 334 familial adenomatous polyposis- biliary proliferation, 209, 210 as hepatoblastoma treatment, 329, related, 163, 164, 165 cholestasis, 224, 276 330-331 incidence of, 169 fat malabsorption, 69 as rhabdomyosarcoma treatment, juvenile polyposis-related, 161 gastroesophageal reflux disease, 44 334 prevalence of, 158 liver disease, 290-292 Clindamycin, as esophagitis cause, 43 prognosis of, 169-170 Cysts Clostridium botulinum toxin signet ring mucinous, 170 choledochal, 197-198, 204, 208, injections, as anal achalasia Common variable immunodeficiency, 212-214, 215, 216 treatment, 137 80-81,82, 169 duplication, 25-26 Clostridium infections, 52, 106-107, Congestive failure esophageal, 169 118 choledochal cysts associated with, mesenteric, 172, 173 Cobalamin C disease, 55 198 presenting as neuroenteric remnants, Colitis hemangioendothelioma-related, 325 26-27 active, definition of, 100 hepatiC hemangioma-related, 333 Cytomegalovirus infections acute, 104 mesenchymal hamartoma-related, as bile duct paucity cause, 224-225 allergic, 78, 100, 101 322 as biliary atresia cause, 207 chronic, 100 Connective tissue disease, enteric as diarrhea cause, 86-87 chronic active, 100 muscular involvement in, 148 as esophagitis cause, 42 collagenous, 117-118 Constipation as gastritis cause, 52, 53 differential diagnosis of, 100, 101 intestinal dysmotility-related, 128 Hirschsprung's disease-related, 139 diversion, 118, 119 severe idiopathic, 138 as inflammatory bowel disease enterohemorrhagic, 105-106 slow-transit, 138 cause, 107, 108 350 INDEX

Cytomegalovirus infections (continued) mesenteric, 28 Encoporesis, intestinal dysmotility• as liver disease cause, 253 omphalomesenteric, 29-30 related, 128 as neonatal hepatitis cause, 2510252 presenting as neuroenteric remnants, Endocrine carcinoma, hepatic, as sclerosing cholangitis cause, 220 26-27 323-324, 331, 332 DNA (deoxyribonucleic acid) depletion, Endoscope cleansing solutions, as D mitochondrial, 280, 281 mucosal alterations cause, 100 Dactinomycin, as rhabdomyosarcoma Doxorubicin Endoscopy, gastrointestinal treatment, 334 as hepatic sarcoma treatment, 334 lower, 47-48 Delta virus, 261 as hepatoblastoma treatment, 329, upper, 37-38 Dermatitis herpetiformis, 75 330-331 Enemas, as colonic mucosa alteration Dermatologic disorders as rhabdomyosarcoma treatment, cause, 100 esophageal involvement in, 44 334 Enteritis, autoimmune, 80 inflammatory bowel disease-related, Doxycycline, as esophagitis cause, 43 Enteritis cystica profunda, 161-162 116-117 Drug toxicity Enterocolitis Dermatomyositis, juvenile, 148 as esophageal disorders cause, 43 Hirschsprung disease-related, 100, Desmoblastic tumors, differentiated as gastritis cause, 58 103, 104 from hepatoblastoma, 335 hepatic effects of, 226, 279 intestinal dysmotility-related, 128 Desmoid tumors, 163, 179 as steatosis cause, 279 necrotizing, 100, 101-103 Desmoplastic small round cell tumor Dubin-Johnson syndrome, 237, 238, neutropenic, 118 (DSRCT), 181 246, 248, 289, 291 Enterocyte structure, primary disorders Desmosis, intestinal, 145 Duchenne muscular dystrophy, 148 of,71-73 Desmosomes, 65 Ductal plates Enteropathies Diarrhea development of, 192, 193 AIDS-related, 84 bloody, milk allergy-related, 101 malformations of, 210, 212 allergic, 75-78 chronic, 63-99 definition of, 194 autoimmune, 63, 78-80 anatomical disorders-related, disorders associated with, 194-200 gluten-sensitive. See Celiac disease 87-88 Ductopenia, 277-278 post-enteritis, 84-85, 87 cancer-related, 89-90 idiopathic adult, 228 -losing, 71, 88 congenital enzymatic disorders• Duodenum tufted, 63, 72-73 related, 67-71 atresia and stenosis of, 10, 16, 19-20 Environmental exposure, as definition of, 63 normal histology of, 66-67 hepatoblastoma cause, 301 enterocyte structure disorders- Duplications, 25-26, 168 Enzymatic disorders, congenital, as related, 71-73 cystic, 25-26 diarrhea cause, 67-71 genetic causes of, 67-71 esophageal, 26 Eosinophilia infections-related, 84-87 gastric, carcinoid tumors in, 172 esophageal, 40-41, 43 inflammatory and immune- rectal, carcinoid tumors in, 172 gastric, 47, 48, 49 mediated disorders-related, tubular, 26 gastritis-related, 49, 50, 54-55 73-84 Dysganglionosis, type 4 non gastroenteritis-related, 41, 54-55 intestinal transport and processing classifiable, 145 Stevens-Johnson syndrome-related, disorders-related, 67-71, 128 , 169 43 intractable, of infancy, 63 Dysmotility, intestinal. See also Motor Eosinophilia counts, quantification of, metabolic diseases-related, 88-89 disorders, enteric 49, 55 nonspecific, 69 definition of, 128 , esophageal mechanisms of, 64-66 Dysplasia involvement in, 44, 45 osmotic, 65-66 arteriohepatic, 221 Episcleritis, inflammatory bowel persistent, 63 colitis-related, 113-114, 116 disease-related, 117 secretory, 66 epithelial, 72-73 Epithelial tumors, as diarrhea cause, syndromatic intractable, 83 intestinal neuronal lIND), 140, 89 toddler's, 69 141-142 Epstein-Barr virus infections, 261 types of, 65-66 renal-pancreatic-hepatic, 200 Erythema multiforme, esophageal, Dientoamoeba fragilis infections, 86, Dysplasia-associated lesion or mass 44-45 101 (DALM), 113-114, 116 Erythema nodosum, inflammatory Diffuse lymphoplasmacytic infiltration bowel disease-related, 116-117 of the small intestine (DLPISI), E Escherichia coli, types of, 105 145-146 Echoviral infections, as neonatal liver Escherichia coli infections, 52, 85, DiGeorge syndrome, 81 disease cause, 252-253, 256 105-106, 118 Disaccharidase deficiency, 65-66, 69 Electrolyte malabsorption disorders, 68 Escherichia coli 0157:H7, 105-106 Diversion procedures, as colitis cause, Electron transport disorders, 281 Esophageal cancer, epithelial, 169 118, 119 Encephalopathy Esophageal disorders, 37-46 Diverticula, 28-30 hepatic, 255, 256-257 Barrett's esophagus, 39-40, 169 antimesenteric, 28 mitochondrial neurogastrointestinal, esophagitis, 37 Meckel's, 28, 29, 30, 49, 160, 171 144 allergic, 37, 38, 40-41, 43 INDEX 351

caustic, 43 Fibromatosis, intraabdominal, 179 increased numbers of. See drug-induced, 43 Fibrosarcoma Hyperganglionosis eosinophilic, 40-41 congenital hepatic, 304-305 reduced numbers of. See histologic diagnosis of, 37 -related, 335 Hypoganglionosis infectious, 38, 41-43 Fibrosis retarded maturation of, 142-143 inflammatory bowel disease- congenital hepatic, 194, 195, 196, Gangliosidoses, 284, 286 related, 113 197 Gardner's syndrome, 158, 163, reflux, 37, 38-39 with , 198 165-166 viral, 41, 42, 43 focal biliary, 292 Gastric folds, enlarged, differential fibromuscular hamartomas, 168 pancreatic, autosomal polycystic diagnosis of, 57 immunodeficiency disorders-related, disease-related, 197 Gastric lesions, stress-related, 57-58 43 Filamin-l mutations, as intestinal Gastritis strictures, 43-44 dysmotility cause, 138 atrophic, 49, 50, 169 systemic diseases-related, 44-45, 55 Fissures, perianal, Crohn's disease• autoimmune, 49, 50, 54 upper endoscopic-based diagnosis of, related, 109 autoimmune atrophic, 51 37-38 Fistulae chronic not otherwise specified, 50, varices, 195 anorectal, 22-24 57 Esophagitis intestinal, Crohn's disease-related, chronic varioliform, 53, 54 allergic, 37, 38, 40-41, 43 110 collagenous, 57 caustic, 43 omphalomesenteric diverticula• eosinophilic, 49, 50, 54-55 drug-induced, 43 related,30 focal active, 57 eosinophilic, 40-41 perianal, Crohn's disease-related, 109 focal chronic, 57 histologic diagnosis of, 37 presenting as neuroenteric remnants, granulomatous, 49, 55-56 infectious, 38, 41-43 26-27 Helicobacter pylori-related, 49-52, inflammatory bowel disease-related, tracheoesophageal, 14-17 53 113 Floxuridine, as sclerosing cholangitis lymphocytic, 52-53 reflux, ulcerative colitis-related, 113 cause, 219, 220 nonatrophic, 50 viral, 41, 42, 43 Fluorouracil, as hepatoblastoma patterns of, 49 Esophagogastroduodenoscopy, 37-38 treatment, 329, 331 Gastroautonomic nerve tumors, 178 Esophagus Food allergy Gastroduodenal disease, Crohn's atresia and stenosis of, 14-17 as colitis cause, 101 disease-related, 109 development of, 6, 8-9 as diarrhea cause, 76-78 Gastroenteritis, eosinophilic, 41, 54-55 duplications, 26 differentiated from food intolerance, Gastroenteropathy, eosinophilic, 77, 78 as mesenteric cyst origin site, 172 76 Gastroesophageal junction Estrogens Fructose-l,6-diphosphate deficiency, histology of, 40 as hepatic adenoma cause, 324 281 inflammatory polyp-fold complex of, as hepatocellular cancer cause, 302 Fructose intolerance, hereditary, 204, 166-167 Etoposide, as rhabdomyosarcoma 281, 282 Gastroesophageal reflux, 37, 38-39 treatment, 334 Fructose malabsorption, 69 chronic gastritis not otherwise Ewing's sarcoma, 304-305 Fucosidosis, 284, 287 specified-related, 57 Fumaryl acetoacetate hydrolase Gastroesophageal reflux disease F deficiency. See Tyrosinemia, (GERDj, 39, 44 Fabry's disease, 89, 284, 286 type 1 Facies, of Alagille syndrome patients, Fungal infections. See also specific congenital anomalies of, 4, 13-31,21 222, 223 fungal infections abnormalities of rotation and Farber's disease, 284, 286 as gastritis cause, 52 fixation, 30-31 Fas ligand, in biliary atresia, 208 atresia and stenosis, 14-24 Fat malabsorption disorders, 68, 69 G diverticula, 28-30 Fatty acid transport and mitochondrial Galactosemia, 204, 278-279, 281-282 duplications and neuroenteric oxidation (FATMOj disorders, Galactosialodosis, 283, 284 cysts, 25-26 280-281 Gallbladder, agenesis of, 193-194 neuroenteric remnants, 26-28 Fatty liver. See Steatosis Ganglioneuroblastoma, 90, 145 developmental anatomy of, 3-13 Ferrous sulfate, as esophagitis cause, Ganglioneuroma, 140, 179-180 blastogenesis, 3-4, 5 43 Ganglioneuromatosis, diffuse, 140-141, embryology, 3-7 a-Fetoprotein, as hepatic tumor 179-180 histogenesis, 3, 8-11 marker, 315, 318, 319 Ganglioneuromatous polyposis, 140, rotation and fixation, 7-8 Fibrolamellar carcinoma 179, 180 developmental control mechanisms differentiated from focal nodular Ganglionitis, 145 of, 11-13 , 318, 321 Ganglion nerve cells, enteric non-neoplastic conditions of, 168 genetic diseases-related, 305 absence of. See Aganglionosis Gastrointestinal tract tumors/cancer, microscopic pathology of, 330 distribution in terminal , 137, 168-181 sex steroids-related, 302 138 carcinoid tumors, 170-1 72 352 INDEX

Gastrointestinal tract tumors/cancer, type la, 288-289 Hemangioma (continued) type Ib, 88 cavernous, 172, 173 epithelial neoplasms, 169-170 type II, 284, 286-287, 288 hepatic, 300, 311, 314, 333 fibromatosis, 179 type III, 278, 288 Hemangiomata, molecular genetics of, ganglioneuroma, 179-180 type IV, 278, 288, 289, 290 311 gastrointestinal stromal tumors, type IX, 288 Hemangiopericytoma, 173 176-178 Glycolipid storage diseases, 89 Hemochromatosis HIV-associated smooth muscle Glycoproteinoses, 284, 287 hereditary, 289-290 tumors, 176 Glycosylation, congenital disorders of, juvenile, 290 inflammatory myofibroblastic 88-89 liver involvement in, 276-277 tumors, 178-179 Godin's syndrome, 159 neonatal, 258-259, 277, 289 leiomyomas and leiomyomatosis, GRACILE syndrome, 237, 238, 245 Hemolytic uremic syndrome, 105-106 176 Graft-versus-host disease, 44, 45, 78, Hemophagocytic syndromes, 251, 252, lymphomas, 173-175 82, 119-120, 225 261 myofibromatosis, 179 Granuloma Henoch-Sch6nlein purpura, 50, 53, 54, neurofibroma, 180 antral, Crohn's disease-related, 103 Peutz-Jeghers syndrome-related, 162 55-56 Hepatic artery abnormalities, as biliary prevalence of, 157 chronic granulomatous disease• atresia cause, 207 stromal tumors, 175-176 related, 55 Hepatic ducts, accessory, 194 vascular tumors, 172-173 colonic, differential diagnosis of, 114 Hepatic tumors, 300-346 Gastropathies, 47-62 Crohn's disease-related, 55-56, age of presentation of, 315 classification of, 48, 50 111-112, 114, 115 biochemical tests for, 315, 317, 319 gastritides of unknown cause, 50 pyogenic, 166, 167 clinical features of, 313-317 chronic gastritis not otherwise Granulomatous disease, chronic, 50, epidemiology of, 300-301 specified, 50, 57 55, 56, 83, 111-112, 112, 115 etiology of, 301-304 collagenous colitis, 57 familial adenomatous polyposis• Crohn's disease, 55-56 H related, 163, 164 Menetrier's disease, 57 Hamartoma imaging and staging of, 313-315, ulcerative colitis, 56-57 Brunner's gland, 168 316,317 hyperplastic, Menetrier's disease- fibromuscular, of the esophagus, 168 incidence of, 300-301 related, 57 mesenchymal hepatic, 310, 322 misdiagnosis of, 327-328 hypertrophic, 49, 51 age of presentation of, 315 molecular and cytogenetics of, 300, infectious, 49-52 cytogenetic abnormalities 304-313 metabolic/genetic disorders- associated with, 313 pathology ot 317-328 associated gastritis, 50, 55, 56 imaging of, 316, 334 gross, 318 noninfectious, 50 incidence of, 301 microscopic, 318-328 autoimmune gastritis, 54 microscopic pathology of, 322, 325 prognostic factors in, 328-335 celiac disease, 52-53, 54 omental-mesenteric-myxoid, 168 research goals and prospects eosinophilic gastritis, 54-55 rectosigmoid, 168 regarding, 335-337 Henoch-Sch6nlein purpura, 50, 53, HB. See Hepatoblastoma treatment of, 328-335, 336-337 54 HCC. See Hepatocarcinoma tumor markers for, 315, 317, 318 polyarteritis, 53, 54 Helicobacter heilmannii infections, as Hepatitis proton pump inhibitors-related, 58 gastritis cause, 50, 52 alpha-I-antitrypsin deficiency• reactive, 49, 58 Helicobacter pylori, 175 related, 270 stress-related, 57-58 detection in biopsy samples, 48 autoimmune, 217, 257, 262-264, Gaucher's disease, 284, 285 structure of, 51 263,264 , 163 Helicobacter pylori infections as cholestasis cause, 204 Germ cell tumors, 181,326 as gastritis cause, 49-52, 54 chronic, 260-266 Giant-cell transformation, of as respiratory allergic reaction cause, giant cell, 303 hepatocytes, 263, 264, 272 76-77 inflammatory bowel disease-related, Giardiasis, 52, 85, 86 ulcerative colitis-associated, 113 17 Gilbert disease/syndrome, 237, 238, Hemangioendothelioma, 311 metabolic causes of, 271-272 246,289 Beckwith-Wiedemann syndrome• neonatal, 203, 251, 271-272 Gliosis, enteric, 150-151 related, 305, 309 idiopathic, 204 Glomus tumors, 173 cytogenetic abnormalities associated Wilson's disease-related, 274 Glucose-galactose malabsorption, 69 with,313 Hepatitis A, 254 Glucose-6-phosphate deficiency. See genetic diseases-related, 305 Hepatitis B, 254, 260, 261, 262 Glycogen storage diseases, type I imaging of, 314, 317, 334 as hepatic tumor cause, 331 Glycogen storage diseases, 288-289 microscopic pathology of, 309, 325, as hepatocellular carcinoma cause, as hepatocarcinoma risk factor, 302, 333 300, 301-302, 305, 315 303-304 misdiagnosis ot 327 lack of association with biliary type I, 270, 278, 288, 305 treatment of, 334 atresia, 206-207 INDEX 353

Hepatitis B vaccination, as Hepatocellular carcinoma ileoanal pull-through procedure in, hepatocarcinoma prophylaxis, Alagille syndrome-related, 224 150 301, 333 clinical features of, 313 neuronal hypertrophy associated Hepatitis C, 260-261, 262, 263 differentiated diagnosis of, 314, 319, With, 132, 133, 134, 135, 136 acute leukemia-related, 265 327 as primary myopathy mimic, 148 as hepatocellular carcinoma cause, disseminated, 317 ultrashort-segment, 137, 138 315 familial adenomatous polyposis- Histiocytosis, Langerhans' cell, 55, 89, lack of association with biliary related, 163, 307 175, 219-220 atresia, 207 gender factors in, 302 Histogenesis, 3, 8-11 perinatal, 254 genetic disease-related, 302, 303, 305 HNPCC (hereditary nonpolyposis as steatosis cause, 278-279 hepatitis B virus-related, 300, colorectal cancer syndrome), Hepatitis C vaccination, as 301-302,315 169, 170 hepatocarcinoma prophylaxis, hereditary hemochromatosis-related, Hodgkin's disease, 173, 228 333 289 Hormones, gastrointestinal, as diarrhea Hepatitis 0, 261 incidence of, 300, 301 cause, 89 Hepatitis G, 261 metabolic disease-related, 278, Human immunodeficiency virus (HIVI Hepatobiliary disease, inflammatory 303-304 infection . See also Acquired bowel disease-related, 117 microscopic pathology of, 321-322, immunodeficiency syndrome Hepatoblastoma, 300 330 (AIDS) age of presentation of, 315 molecular and cytogenetics o( esophageal disorders associated With, Beckwith-Wiedemann syndrome• 309-310,311-313,331-334,336 43 related, 305, 309 prevention of, 3m, 331-333 liver involvement in, 262, 263 cholangioblastic differentiation in, prognosis of, 331-334 smooth muscle tumors associated 328 progressive familial intrahepatic With, 176 clinical features of, 313 cholestasis-related, 241 Human papillomavirus, as biliary congenital, 314 screening for, 333-334 atresia cause, 207 cytogenetic abnormalities in, treatment of, 331-334, 337 Hunter's syndrome, 284, 287 305-309 tumor markers for, 331 Hurler's syndrome, 278, 284, 287, 288 differentiated diagnosis of, 335 tyrosinemia-related, 277 Hydrops fetalis, 253, 254 embryonal, 336 Hepatocytes, giant-cell transformations Hyperbilirubinemia, 203, 204, 205, familial adenomatous polyposis- of, 263, 264, 272 213-214 related, 163, 305, 306-307 Hereditary nonpolyposis colorectal Hypercholanemia, familiat 238, a-fetoprotein as marker for, 315 cancer syndrome (HNPCCl, 169, 241-242 gene expression in, 307-309 170 Hypercholesterolemia, genetic diseases-related, 305 Hernia hepatoblastoma-related, 317, 330 hepatocarcinoma-related, 302 hiatal, genetic factors in, 39 Hyperganglionosis, 139-142 imaging o( 314 left mesocolic internal, 30-31 Hyperimmunoglobulinemia E chemotherapy based on, 330-331 Herpes simplex virus infections, 41, syndrome (Job's syndromeI, 83 incidence of, 300, 301 42,43,262 Hyperplasia macrotrabecular, 319, 327, 336 Herpes virus infections, as enteric adenomatous, 303 misdiagnosis of, 327 ganglionitis cause, 139 nodular mitotically-active fetal, 336 Heteropias, 168, 193 differentiated from fibrolamellar mixed, 317, 319-320, 325 pancreatic, 17, 18 carcinoma, 318, 321 molecular and cytogenetics of, Hirschsprung's disease focal, 278, 303, 318, 321, 324 304-309 biopsy findings in Hypobetalipoproteinemia, 69 pathology of from resected aganglionic gut, 134, Hypoganglionosis, 138 gross, 317, 318 136 anthraquinone laxatives-related, 139 microscopic, 318-321, 324, 328, seromucosal rectal, 134, 136 differentiated from Hirschsprung's 330 submucosal rectal, 132, 133, 134, disease, 132 prognosis of, 328-331 135 Hirschsprung's disease-related, 136, staging of, 317 classification of, 128, 129 137, 138 correlated with histopathology, as colitis cause, 101 postinfectious, 139 336 colonic atresia associated With, 22 X-linked neurogenic intestinal teratoid, 320-321, 327, 336 cytomegalovirus infections pseudo-obstruction-related, 138 treatment of, 313, 328-331, 334-335, associated with, 139 336 definition of, 132 I undifferentiated, 323 diversion procedure for, as colitis I-cell disease, 287 small cell, 307, 319, 326, 328-329, cause, 118 Ifosfamide 335-336, 336 enterocolitis associated With, 103, 104 as hepatic sarcoma treatment, 334 well-differentiated fetal, 336 esophageal congenital anomalies as hepatoblastoma treatment, Hepatocarcinoma. See Hepatocellular associated With, 16 329-330 carcinoma fibrosing colonopathy-related, 120 as rhabdomyosarcoma treatment, 334 354 INDEX

Ileal pouch reservoirs Inflammatory bowel disease-like Jejunum, atresia and stenosis of, 20-22 inflammation (pouchitis) of, 118-119 presentation, Jeune syndrome, 200 in inflammatory bowel disease immunodeficiencies-related, 109 Job's syndrome, 83 patients, 116 Inflammatory bowel disorders, chronic, Ileoanal pull-through procedures, effect 100-127 K on contractile protein allergic, 101 Kabuki make-up syndrome, 219, 246 expression, 150 chronic inflammatory bowel disease, Kaposi sarcoma, gastric, 173 Ileum, atresia and stenosis of, 20-22 108-118 Kidney transplantation, 332 Imaging, of hepatic tumors, 313-315, clinical manifestations of, 109 Klippel-Trenauney syndrome, 173 316,317 extraintestinal manifestations of, Immune dysfunction, as biliary atresia 116-117 L cause, 207-208 fulminant colitis, 114-115, 116 Lactase deficiency, 69 Immune-mediated disorders, as genetic factors in, 108-109 Lafora's disease, 289 chronic diarrhea cause, 73-84 indeterminate colitis, 115-116 Laxatives, anthraquinone Immunocompromised patients lymphocytic and collagenous as intestinal pseudo-obstruction esophagitis in, 41 colitis, 117-118 cause, 139 mucormycoses in, 52 pathology of, 109-114 as melanosis coli cause, 149 Immunodeficiencies. See also Acquired Hirschsprung disease-related Leiomyoma, 175-176, 176 immunodeficiency syndrome enterocolitis, 103 Leiomyomatosis, diffuse, 176 (AIDS); Human histologic patterns in, 100, 101 Leiomyosarcoma, 158, 175-176, 176, immunodeficiency virus (HIV) infection-related, 101, 103-108 335 infection ischemic, 101-103 Leukemia as chronic diarrhea cause, 80-84 neuromuscular, 101, 103 acute, 118, 265 common variable, 80-81, 82, 169 treatment-related disorders, 118-120 megakaryoblastic, differentiated with inflammatory bowel disease- differential diagnosis of, 101 from hepatoblastoma, 328, 335 like presentation, 109 Inflammatory cell population counts, Leukocyte adhesion deficiency, 83, 84 as sclerosing cholangitis cause, 219 gastric, 48 Leukodystrophy, metachromatic, 284, severe combined, 81-82, 83 Inflammatory disorders, as chronic 286 Immunoglobulin A deficiency, 78, 80, diarrhea cause, 73-84 Li-Fraumeni syndrome, as liver tumor 81 Inflammatory myofibroblastic tumors, cause, 305 Immunoglobulin G subclass 178-179,314,326 Lipid storage diseases, 284 deficiency, 81 Intestinal neuronal dysplasia (IND), Lipid transport disorders, differentiated Immunoproliferative small intestinal 141-142 from peroxisomal disorders, 89 disease (IPSIDl, 175 Intestinal neuronal dysplasia (IND)• Lipoma, 181 Infections like pathology, 136, 142 Liposarcoma, 181 as chronic diarrhea cause, 84-87 Intestinal processing and transport Listeriosis, 254-255 as esophagitis cause, 41-43 disorders, as diarrhea cause, 67-71 Liver as gastropathy cause, 49-52 Intussusception, 30, 168, 174 agenesis of, 193 as hepatitis cause, 251, 252 Ion transport, intestinal, 70-71 development of as hypoganglionosis cause, 139 IPEX syndrome, 80, 81, 83 anomalous, 193 as inflammatory bowel disease IPSID (immunoproliferative small normal, 6, 7-8, 191, 192, 193 cause, 103-108 intestinal disease), 175 fatty. See Steatosis Infectious mononucleosis, 261 Iron poisoning, as gastritis cause, 58 as hematopoiesis site, 193 Inflammatory bowel disease. See also Iron storage disease, neonatal, 258-259 involvement in metabolic disorders, Colitis, ulcerative; Crohn's Iron supplementation, as gastritis 270 disease cause, 58 Liver cancer. See Hepatic tumors chronic, 108-118 Ischemia, as sclerosing cholangitis Liver disease clinical manifestations of, 109 cause, 219, 220 Alagille syndrome-related, 221 extraintestinal manifestations of, Ivemark syndrome, 200 metabolic. See Metabolic disorders, 116-117 as liver disease cause fulminant colitis, 114-115 J neonatal, congenital infections• genetic factors in, 108-109 related, 251-255 indeterminate colitis, 115-116 acute -related, 256 total parenteral nutrition-related, lymphocytic and collagenous biliary atresia-related, 207, 208 103 colitis, 117-118 choledochal cysts-related, 213 Liver failure pathology of, 109-114 cholestatic, 203, 205, 226 acute, 244, 255-259 colorectal cancer associated with, 170 hepatic tumors-related, 313 differential diagnosis of, 256, 258 cytomegalovirus infections inflammatory myoblastic tumors- Coxsackie B virus infections-related, associated with, 86-87 related, 314 253 polyps associated with, 166 neonatal, evaluation of, 203, 205 echoviral infections-related, 252 primary sclerosing cholangitis primary sclerosing cholangitis• Liver transplantation, 331-332 associated with, 217 related, 214 as acute liver failure treatment, 256 INDEX 355

as Alagille syndrome treatment, 221 Meckel-Gruber syndrome, 24, 199-200 enteric neuropathies, 128-145 bile duct damage in, 207 Medium-chain acyl-CoA aganglionosis (Hirschsprung's as biliary agenesis treatment, 208 dehydrogenase deficiency, 280, disease), 132-138 as biliary atresia treatment, 211 281 cytoarchitectural, 144-145 chronic allograft rejection in, Megacolon, congenital. See cytological, 142-144 225-226 Hirschsprung's disease hyperganglionosis, 139-142 as eosinophilic gastroenteritis cause, Megacystis-microcolon-intestinal hypoganglionosis, 138-139 54 hypoperistalsis syndrome inflammatory, 145 as hepatocarcinoma risk factor, 303 (MMIHS), 146-147 quantitative, 128-132 as neonatal iron storage disease Melanosis coli, 149 mitochondrial neuromyopathies• treatment, 259 Menetrier's disease, as gastritis cause, related, 144 orthotopic, 334-335 57 nonspecific, 148-151 as graft-versus-host disease cause, Menkes' disease, 83 primary connective tissue disease- 225 Mesenchymal tumors, hepatic, related, 148 as primary sclerosing cholangitis 310-311 Mucolipidoses, 284, 287 treatment, 218 Metabolic disorders Mucopolysaccharidoses, 284, 287 Liver tumors. See Hepatic tumors as bile duct paucity cause, 224, 276 Mucormycoses, as gastritis cause, Long-chain 3-hydroxyacyl-CoA as chronic diarrhea cause, 88-89 52 dehydrogenase deficiency, 281 as gastritis cause, 55 Mucosa-associated lymphoid tissue Lymphadenitis, mesenteric, 168 as hepatocarcinoma cause, 303-304 (MALT) lymphoma, 175 , intestinal, 87-88 as liver disease cause, 270-299, Mucosa-associated lymphoid tissue Lymphangioma, 172 271-292 (MALT) system, 49, 52, 66-67 cystic, 172, 173 cholestatic histologic pattern in, development of, 10-11, 12 Lymphedema-cholestasis syndrome, 276 Mucosal development 237, 238, 240, 242, 243, 244, cirrhotic histologic pattern in, colonic, 10 245-246,247, 274 276-277 esophageal, 8-9 Lymphohistiocytosis, hemophagocytic, cytoplasmic histologic pattern in, gastric, 6, 9, 12 264, 266 288-292 mucosa-associated lymphoid tissue, Lymphoma, 173-175 diagnostic tests for, 270-271 10-11, 12 Burkitt's, 173-174 ductopenic histologic pattern in, small intestine, 9-10 enteropathy-associated T cell, 175 277-278 Multidrug resistance 3 (MDR3) gastric, Helicobacter pylori hepatitic histologic pattern in, disease, 237 infection-related, 49, 52 271-276 Multiple endocrine neoplasia (MEN), intestinal, 157 technique in, 271 type 2B, 141, 179, 180 large-cell, 174-175 as neonatal hepatitis cause, 251 Multiple gastrointestinal anomalies misdiagnosis of, 327 neoplastic histologic pattern in, syndrome, 16 mucosa-associated lymphoid tissue 278 Multiple hamartoma-neoplasia (MALT), 175 steatotic histologic pattern in, syndrome. See Cowden's disease prevalence of, 158 278-283 Muscular coats, development of, II, Lymphoproliferative syndrome, X• storage histologic pattern in, 13 linked, 261 283-288 Muscular dystrophies, 147-148 Lysinuric protein intolerance, 70, 83 Metaplasia, Helicobacter pylori Muscularis propria, Lysosomal storage diseases, 283-288 infection-related, 49, 51 vacuolization/fibrosis of, 146, Metastatic tumors, 181 147, 148, 149 M Microvillus inclusion disease, 63, Mycobacterium avium complex Maffucci's syndrome, 173 71-72, 237, 242-244 infections, 85 Major histocompatibility complex Milk allergy, 100, 101 Mycobacterium avium-intracellulare class II deficiency, 83 Milroy disease, 87 infections, 285 Malabsorption, 63 Mitochondrial disorders, 279-281 Mycobacterium tuberculosis celiac disease-related, 73 Mitochondrial mutations, as diarrhea infections, 83, 85 classification of, 64 cause, 89 Myoblastic tumors, inflammatory, Crohn's disease-related, 109 Mitochondrial neurogastrointestinal 314 genetic causes of, 67-71 encephalomyopathy (MNGIE), Myofibroblastic tumors, inflammatory, necrotizing fasciitis-related, 103 144 178-179,313 Malabsorption syndrome, 63-64 Mixed connective tissue disorders, 148 Myofibromatosis, 89-90, 179 Malrotation, 30-31 Motor disorders, enteric, 128-156 Myopathies MALT (mucosa-associated lymphoid as diarrhea cause, 66 familial visceral (hereditary hollow tissue) lymphoma, 175 enteric myopathies visceral), 147 MALT (mucosa-associated lymphoid inflammatory, 145-146 inflammatory, 145-146 tissue) system, 49, 52, 66-67 primary visceral, 146-148 primary visceral (noninflammatory), development of, 10-11, 12 enteric quantification in, 146-148 Mannosidosis, 284, 287 128, 130-132 Myositis, idiopathic, 146 356 INDEX

N p as small bowel adenocarcinoma Neonatal iron storage disease, 258-259 Pancreaticobiliary junction, abnormal, risk factor, 169 Nephronophthisis, congenital hepatic 194, 207, 213 surgical treatment of, 118-119 fibrosis-related, 198 Pancreatitis, choledochal cyst-related, variants of, 158, 163, 165-166 Neuroblastoma 213 ganglioneuromatous, 140, 179, 180 as diarrhea cause, 90 Panhypopituitarism, bile duct paucity juvenile, 160-161 differentiated diagnosis of, 327, 335 associated with, 224 Polyposis syndromes, 158, 162-168 intestinal, 141 Parasitic infections. See also specific Polyps Neuroenteric remnants, 26-28 parasitic infections adenomatous, ureterosigmoidoscopy• Neurofibroma, 180 as eosinophilic colitis cause, 100 related, 118 plexiform, 140, 141 Parenteral nutrition, as cirrhosis cause, fundal gland, 164, 166 Neurofibromatosis, 140, 141, 305 301 hamartomatous, 157, 161-162, type I, 166 Peptic disease, Helicobacter 162-163, 163 Neuroglial tumors, 139 pylori infection-related, 49-50 hyperplastic, 166 Neuromyopathies, mitochondrial, Perianal disease, Crohn's disease• inflammatory, 166-167 144 related, 109 juvenile (retention), 158-159 Neuronal intranuclear inclusion Peribiliary glands, 192-193 lymphoid, 167-168 diseases, 143-144 Peripheral neuroectodermal tumors of Peutz-Jeghers syndrome, 159-160, , enteric (PNET), 181 161-162, 163 counting of, 128, 130-132 Peristalsis, impaired, 128 sequence/syndrome, 207 development and distribution of, Peritonitis, 102, 107, 114 Pompe's disease. See Glycogen storage 128 Perivascular epitheloid tumors, diseases, type 11 Neuropathies, enteric, 128-145 hepatic, 325-326 Porphyria, 289, 290 aganglionosis (Hirschsprung's Peroxisomal disorders, 89, 274-275 Porphyria cutanea tarda, 290 disease), 132-138 Peutz-Jeghers syndrome, 159-160, Portal vein cytoarchitectural, 144-145 161-162, 169 congenital absence of, 193 cytological, 142-144 p53, 308, 331 hepatic tumor dissemination within, hyperganglionosis, 139-142 Phagocyte function disorders, 83-84 315-317 hypoganglionosis, 138-139 Phosphomannose isomerase deficiency, Portoenterostomy inflammatory, 145 88 as Alagille syndrome treatment, 223 quantitative, 128-132 Pigment storage disease, 289 as biliary atresia treatment, 205, Niemann-Pick disease, 285-286 Pills, entrapment in the esophagus, 43 207, 208, 209, 210, 211-212, type 11,275-276 Plexosarcomas, 178 213 2-Nitro-4-trifluoromethylbenzoyl-1,3• PNET (peripheral neuroectodermal Potassium chloride, as esophagitis cyclohexanedione, as tumors), 181 cause, 43 hepatocarcinoma inhibitor, 277, Pneumatosis intestinalis, 102-103 Pouchitis, 118-119 331-333 Pneumocystis carinii infections, 81 Proctitis, allergic, 78 Non-steroidal anti-inflammatory drugs POLIP (polyneuropathy, Proctocolitis. See Colitis, allergic as esophagitis cause, 43 ophthalmoplegia, Proteus syndrome, 163 as gastritis cause, 58 leukoencephalopathy, and Proton pump inhibitors, as gastritis Noonan's syndrome, 87 intestinal pseudo-obstruction) cause, 58 syndrome, 144 Proton pumps, in Helicobacter py10ri• o Polyarteritis nodosa, 53-54 related gastritis, 51 Obstruction, intestinal, partial, 149 Polycystic diseases Protozoan infections, as chronic Odynophagia, infectious esophagitis- genetics of, 195 diarrhea cause, 85-86 related, 41-42 isolated hepatic, 199 Prune-belly syndrome, 224 Omenn's syndrome, 82-83 Polycystic Pseudo-Hurler's syndrome, 287 Organogenesis, gastrointestinal, 3, 4, autosomal dominant, 194, 198-199 Pseudomembranes, colitides associated 6-7, 8, 9, 10, 11 autosomal recessive, 194, 195, 197 with, 106-107 . See also Bone Polymyositis, 148 Pseudomonas infections, as marrow transplantation; Kidney Polyneuropathy, ophthalmoplegia, neutropenic enterocolitis cause, transplantation; Liver leukoencephalopathy, and 118 transplantation; Small bowel intestinal pseudo-obstruction Pseudo-obstruction, intestinal transplantation (POLIPI syndrome, 144 anthraquinone laxatives-related, 139 as adenovirus infection risk factor, Polyposis cancer-related, 145 107 familial, as hepatocarcinoma risk classification of Ornitine transcarbamylase deficiency, factor, 302 clinical subclassification, 128, 129 283 familial adenomatous, 160, 163-166 pathological, 128, 130 Osler-Weber-Rendu disease, 173 as hepatocellular carcinoma risk connective tissue disease-related, Osteosarcoma, metastatic, 181 factor, 307 148 Ovarian sex cord tumors with annular as liver tumor risk factor, 305, contractile muscle protein tubules (SCTAT), 162 306-307 expression in, 150 INDEX 357

diffuse lymphoplasmacytic S Steatohepatitis, idiopathic, of infiltration of the small Salicylates, as Reye's syndrome cause, childhood, 279 intestine (DLPISI)-related, 282 Steatosis, 278-279, 280, 282 145-146 Salmonella infections, 103-104, lOS, cystic fibrosis-related, 291-292 hypoganglionosis associated with, 112 enterocytic, 70 138 Sandhoff's disease, 286 inflammatory bowel disease-related, idiopathic, 150 Sanfilippo's syndrome, 88, 284, 2897 17 idiopathic myositis-related, 146 Sarcoma, hepatic Stenosis intranuclear inclusions associated incidence of, 301 colonic, 22 with, 143-144 mesenchymal hamartoma-related, duodenal, 19-20 mitochondrial neuromyopathies- 310 esophageal, 14-17 related, 144 prognosis of, 334 gastric, 17-18 muscular dystrophy-related, 148 treatment of, 334 jejunal-ileal, 20-22 neurofibromatosis typel-related, 141 undifferentiated, 305, 310, 313, 322, multiple, 15 X-linked neurogenic, 138 323, 324 Steroidal agents, as esophagitis cause, Pseudopolyps, inflammatory bowel Sclerosis 43 disease-related, 110-111, 112 progressive systemic, 148 Stevens-Johnson syndrome, 43 Pyloric outlet obstruction, 55, 56 tuberous, 163, 305, 326 Stomach , 16 SCTAT (ovarian sex cord tumors with atresia and stenosis o( 17-18 infantile hypertrophic, 18 annular tubulesL 162 inflammatory cell population of, 47, Pyoderma gangrenosum, inflammatory 48 bowel disease-related, 116, biliary proliferation associated with, mucosal histogenesis in, 6, 9, 12 117 209 normal anatomy of, 47 as cholestasis cause, 204 Stomach cancer, 49, 169, 194 Q Severe combined immunodeficiency, Storage diseases, 283-292. See also Quinidine, as esophagitis cause, 43 81-82, 83 Glycogen storage diseases Sex steroids, as hepatocellular cancer cytoplasmic, 288-292 R risk factor, 302 lysosomal, 283-288 Rectal prolapse, hamartomas Shigella infections, 104-105 Streptococcal infections, as gastritis associated with, 168 Short-chain 3-hydroxyacyl-CoA cause, 52 Rectum dehydrogenase deficiency, 281 Strictures atresia and fistulae o( 22-24 Short-gut syndrome, necrotizing biliary, 215-216, 218 development o( 25 fasciitis-related, 103 esophageal, 43-44 inflammatory cloacogenic polyps o( Sialidosis, 287 intestinal, necrotizing fasciitis• 167 Sialolipidosis, 287 related, 102, 103 leiomyoma of, 176 Skip lesions, 22 Stromal tumors, 175-176 lymphoid polyps of, 167-168 differentiated from segmental gastrointestinal (GIST), 176-178 terminal, ganglion cell distribution aganglionosis, 137 Sudden unexpected death syndrome in, 137, 138 Sly's syndrome, 287 (SUDS), 280-281 Refsum disease, 89, 274-275 Small bowel. See also Duodenum Syphilis, 52, 255, 256 Renal abnormalities, Alagille as mesenteric cyst origin site, 172 syndrome-related, 224, 227 mucosal histogenesis in, 9-10 T Reovirus type 3, as biliary atresia Small bowel carcinoma, Crohn's Tangier disease, 89 cause, 206 disease-related, 114 Tay-Sachs disease, 286 Respiratory chain disorders, Small bowel transplantation, 63, 72, 242 T-cell defects, primary, as chronic mitochondrial, 281 Smooth muscle tumors, 176, 178 diarrhea cause, 81 Reye's syndrome, 280-281, 282-283 Solitary rectal ulcer syndrome (SRUSI, Teratoma, 181, 326 Rhabdoid tumors, 323, 324, 329 167 Tetracycline, as esophagitis cause, 43 Rhabdomyosarcoma, 304-305 Sorbitol malabsorption, 69 Thalidomide, as hepatoblastoma choledochal cysts-related, 214 Soy allergy, 76, 78, 101 treatment, 331 familial adenomatous polyposis- Spindle cell tumors, 176 Thrush, oral, 41 related, 163 Split notochord syndrome, 26-28 Thymidine phosphorylase gene hepatic, 326, 334 Sprue, 63-64 mutations, 144 perianal, 181 refractory, 75, 78 TJP2-BAAT disease, 237, 241-242 peribiliary, treatment of, 334 Squamous cell carcinoma, choledochal Toxoplasmosis, congenital, 254, 255, primary embryonal, misdiagnosis of, cysts-related, 214 257 327 Squamous cell papilloma, esophageal, Trace metals malabsorption disorders, Rotavirus type C, as biliary atresia 169 68 cause, 206 SRUS (solitary rectal ulcer syndrome), Treponema pallidum infections, 254, Rotor syndrome, 237, 238, 246, 248 167 255 Rubella, congenital, 254, 255 Staphylococcal infections, as gastritis Trichorrhexis nodosa, 83 Ruvalcaba syndrome, 159, 160 cause, 52 Trisomy 2, 304-305 358 INDEX

Trisomy 8, 304-305 cycle disorders, 283 as chronic diarrhea cause, 85, Trisomy 18, 305 Ureterosigmoidoscopy, complications 86-87 Trisomy 20, 304-305 of, 118 Vitamin B12 transport disorders, 71 Trisomy 21, 224 Ureterosigmoidostomy, as colonic Vitamin malabsorption disorders, 68 Trisomy 26, 304-305 adenocarcinoma cause, 170 Volvulus, 30 Tuberculosis, as gastritis cause, 52 Uveitis, inflammatory bowel disease• von Hippel-Lindau syndrome, 311 Turcot's syndrome, 158, 163, 166 related, 117 Typhlitis, 118 W Typhoid fever, 104 v Watson-Alagille syndrome, 221 Tyrosinemia VACTERL association, 13, 17, 24 Whipple's disease, 85 as hepatocellular carcinoma risk Valproic acid, hepatotoxicity of, 265 Wilms' tumor, 181, 304-305, 327 factor, 278, 331-333 Vanishing bile duct syndrome, 226-227 Wilson's disease, 264, 274, 276-277, hereditary, 305, 315 Varicella infections, 52, 282 278-279, 289 liver involvement in, 277 Varicella-zoster virus infections, 42 Wiskott-Aldrich syndrome, 81 type 1,303 Varices, esophageal, 195 Wolman's disease, 88-89, 279, 286, Vascular tumors 287 U gastrointestinal, 172-173 Ulcers hepatic, 300, 301, 309, 325, 333, 334 Y aphthous, Crohn's disease-related, Very-long-chain 3-hydroxyacyl-CoA Yersinia infections, 106, 112 110 dehydrogenase deficiency, 281 Yolk sac tumors, 313, 315, 326 colonic, 107-108, 108 Villi duodenal, 51, 113 blunting of, 67, 68 Z esophageal, 42, 43, 44-45 normal appearance of, 66, 67 Zellweger syndrome, 224, 227, gastric, 51 Vincristine, 329, 330, 334 274-275 gastroduodenal, perforated, 54 Viral infections. See also specific Zinc absorption disorders, 71 peptic, 49-50 viruses Zinc deficiency, 71, 83