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Intestines I, Intestines I Peritoneum MISC CONDITIONS Sclerosing Retroperitonitis-dense, fibrosing -can involve mesentery Mesenteric Cysts- From: sequestered lymphatic channels, pinched-off diverticula, UG ridge, walled-off infx or pancreatitis (pseudocysts), malignant TUMORS-almost all malignant Primary-from mesothelium (mesotheliomas) -like in pleural cavity-ASBESTOS Secondary-COMMON-in any form of adv CA -penetration to serosa or mets seeding -esp OVARIAN, PANCREATIC, Intestines I Malabsorption Syndromes DISACCHARIDASE (LACTASE) DEF Congenital rare, but acquired common -esp N. American BLACKS -can’t break lactose->gluc, galactose ->osmotic diarrhea ->bacterial fermentation of lactose->gas Infants: inherited enz deficiency Sx-infants w/ explosive, watery, frothy stools, Abd distention Tx-stop giving baby milk! Adults-can be acquired w/ viral, bac infx, Intestines I Malabsorption Syndromes TROPICAL (POSTINFX) SPRUE Celiac-Like dz in ppl visiting/living in Tropics -Caribbean (not Jamaica), C., S. Africa, India, SE Asia, C. S. America -Endemic and Epidemic forms -poss from E.coli, Hamophilus overgrowth MORPH-injury at ALL levels of small int. -often w/ folate, B12 def ->megaloblastic changes (like PernA) CLINICAL-acute diarrheal infx -days-wks later, apparent malabsorption -persists if unTx Tx-broad-spectrum ABX -no CA risks like Celiac Sprue, Intestines I Megacolon CONGENITAL AGANGLIONIC MEGACOLON-HIRSCHSPRUNG DZ No ganglion cells in LARGE bowel -Auerbach AND Meissner plexuses -via failure of neural crest cell migration ->obstruction, dilatation proximally MORPH-rectum always afftected -most w/ rectum and sigmoid only ->megacolon -thinned wall, risk rupture near cecum -or shallow STERCORAL ulcers possible CLINICAL-fam forms, but mostly sporadic -short segments in BOYS, long in GIRLS -10% of HD in Down’s; 5% w/ neuro ABNL Threats to life: enterocolitis, perforation, Intestines I Megacolon Acquired megacolon (latter 3 not neural): 1. Chagas’ Dz (tryps destroy plexus) 2. Obstruction by neoplasm, inflam 3. Toxic megacolon (UC or Crohn’s) 4. Psychosomatic d/o, Intestines I Enterocolitis VIRAL ENTEROCOLITIS -shortened, blunt villi, lymphos infiltrate lam.prop. Rotavirus most common-esp kids -outbreaks in hospitals, daycares -incubate 2 days-> watery D/V for days -can produce flat mucosa like Sprue Caliciviridae (Noro)-most common in adults -1-2d incubation->watery D/N/V ~2d, Intestines I Malabsorption Syndromes CELIAC SPRUE Chronic dz, lesion of small int. -in WHITES PATHO-sensitivity to gliadin (gluten) -CMI: intraepith CTL, -gluten-specific Th in lam.prop ->cytokines dmg int mucosa HLA-DQ a/b (w2) interacts w/ gliadin -also many w/ HLA B8 -poss due to exposure to adenovirus MORPH-flat, scalloped, or NL mucosa -Bx-diffuse enteritis, vacuolar degen -loss of brush border -inc. mitotic activity in crypts -overall mucosal thickness equal -more evident in prox SI -lam prop w/ plasmas, Th, macs, eos, mast ->NL after exclusion of gluten CLINICAL-variable: diarrhea, FTT, flatulence, wt loss, fatigue -ANTIGLIADIN, ANTIENDOMESIAL ABs Definitive Dx-1. malabsorption, 2. small bowel Bx, 3. improvement w/ gluten w/drawal Risk: int.lymphomas(esp T cell), GI, BR CA, Intestines I Congenital RARE: Duplication Malrotation Omphalocele-abd wall mm doesn’t form -herniation of abd contents into sac Gastroschisis- abd wall doesn’t form -extrusion of intestines, Intestines I Enterocolitis Both uncommon, benign, both w/ 3-20 stools/d COLLAGENOUS COLITIS Chronic watery diarr, coll bands under epith Middle-aged, older WOMEN LYMPHOCYTIC COLITIS Chronic watery diarr, lymphos in epith MEN and WOMEN Ass’d w/ AI dzs: Celiac Sprue, thyroiditis, arthritis, AI gastritis, Intestines I Malabsorption Syndromes ABETALIPOPROTEINEMIA Can’t synthesize ApoB-auto recessive -defective export from enterocytes -FFAs re-esterified but can’t be put into CMs ->TGs stored in cells->vacuolation -also no CMs, VLDLs, LDLs ->lipid membrane ABNLities->burr cells ->FTT, diarrhea, steatorrhea in infancy