Chapter17: GIT: Congenital DO

Question 1

\Congenital abnormalities GIT

Answer 1

AtresiaDuplicationsFistula

Question 2

Most common congenital is ______. Developmentally incomplete

Answer 2

Atresia

Question 3

It rsults to mechanical obstruction due to replacement of noncanalized cord.

Answer 3

Esophageal atresia

Question 4

Atresia most commonly occurs in the _______. It is usually assoc with a _____ connecting the upper and lower esophageal pouches to a bronchus or trachea.

Answer 4

Near tracheal BifucationFistula

Question 5

Is less common than esophageal atresia but it frequently involves the duodenum and is characterized by a segment of bowel lacking a lumen.

Answer 5

Intestinal Atresia

Question 6

It is an incomplete form of atresia in which the lumen is reduced in caliber as a result of fibrous thickening of the wall resulting in partial or complete obstruction.Most commonly affected is in the esophagus and small intestine.

Answer 6

Stenosis

Question 7

The most common congenital intestinal atresia due to failure of the cloacal diaphragm to involute.

Answer 7

Imperforate anus

Question 8

Are saccular or congenital cystic masses that contain redundant smooth muscle layers. May be present in the esophagus,intestines or colon.

Answer 8

Congenital duplication cyst

Question 9

What is the tx of ménétrier dse

Answer 9

1. Supportive 2. IV albumin 3. Parenteral nutritionSevere cases: gastrectomy

Question 10

A disease caused by gastrin-secreting tumors, gastrinomas, that are most commonly found in the small intestine and pancreas.Px often present with duodenal ulcer and chronic diarrhea.Within the stomach the most remarkable feature is DOUBLING OXYNTIC MUCOSAL THICKNESS DUE TO A FIVEFOLD INCREASE IN PARIETAL CELLS

Answer 10

Zollinger Ellison Syndrome

Question 11

Gastric polyps and tumors

Answer 11

1. Inflammatory and hyperplastic polyps2. Fundic gland polyps3. Gastric adenoma4.gastric adenocarcinoma5. Lymphoma6. Carcinoid tumor7. Gastrointestinal stromal tumor

Question 12

Nodules or masses that project above the level id the surrounding mucosa and are i.d in up to 5% of upper GI endoscopiesIt may develop as a result of epithelial or stromal cell hyperplasia, inflammation, ectopia or neoplasia.

Answer 12

Polyps

Question 13

Approx 75% of all gastric polypsAre most common in indiv bet 50-60 yo.Polyps that usually develop in assoc with chronic gastritis, which initiates the injury and reactive hyperplasia that leads to polyp growth.In indiv with H.pylori, regresses after removal of insult.

Answer 13

Inflammatory and hyperplastic polyps

Question 14

Occur sporadically and in indiv with FAP.Increased gastric secretion, in response to reduced gastric acidity, and the resulting glandular hyperplasia.5x more commom in women and an average of 50y0.May be asymptomatic or assoc with nausea, vomiting, or epigastric pain.Morphology:Occur in gastric body and fundusWell circumscribed lesions with smooth surfaceSingle or multipleCystically dilated irregular glands lined by flattened parietal and chief cellsInflammation is typically absent or minimal.

Answer 14

Fundic gland polyps

Question 15

10% of all gastric polyps.Incidence increases progressively with age. Bet 50-603x more in malesLike fundic gland polyp it is assoc with FAPRisk of gastric ca is related to the size of lesion and is elev in leasion greater than 2cm in diameter

Answer 15

Gastric adenoma

Question 16

Morphology:Usually solitary lesions less than 2cm in diameterMost commonly loc in the antrumMajority are composed of intestinal-type columnar epithelium.

Answer 16

Gastric adenoma

Question 17

Is the most common malignancy in the stomach.90% of all gastric ca.Early symptoms resemble those of chronic gastritis, including,Dysphagia, dyspepsia, and nausea.Often discovered at advanced stages when symptoms such as wt loss, anorexia, altered bowel ha its, anemia a s hemorrhage trigger further diagnostic evaluation.It is. Ore common in lower socioeconomic status and in indiv with multifocal mucosal atrophy and intestinal metaplasia. PUD doesnt impart as risk factor except to those who had partial gastrectomy for PUD.Majority is not inherited.

Answer 17

Gastric adenocarcinoma

Question 18

What is the pathogenesis in gastric adenocarcinoma?

Answer 18

Germline mutations in CDH1 which encodes E-cadherin , a protein that contributes to epithelial intercellular adhesion are associated with familial gastric ca of which is a diffuse type.

Question 19

What is the pathogenesis of intestinal-type gastric ca?

Answer 19

Mutation in beta-catenin, a protein that binds to both E-cadherinand adenomatous polyposis coli ( APC ), as well as microsatellite instability and hypermethylationof several genes including TGF B RII, BAX, IGFR II and p16 /INK4.

Question 20

Morphology charac:Irreg enlargement of gastric rugae.Some areas appear polypoid.Enlarged rugae present in body and fundus except antrum( gen spared)Most characteristic feature: HYPERPLASIA OF FOVEOLAR MUCOUS CELLS.THe glands are elongated with CORK-SCREW appearance and cystic dilation is common.Inflammation is only modest although some cases show marked intraepithelial lymphocytosis.Diffuse or patchy glandular atrophy, evident as hypoplasia or parietal and chief cell is typical

Answer 20

Ménétrier dse

Question 21

2 diseases associated in hypertrophic gastrophaties

Answer 21

1. Ménétrier dis2. Zollinger ellison syndrome

Question 22

Uncommon diseases charac by GIANT CEREBRIFORM ENLARGEMENT OF THE RUGAL FOLDS due to epithelial hyperplasia W/O inflammation.As expected they are linked to excessive GF release

Answer 22

HYPERTROPHIC GASTROPHATIES

Question 23

Rare d/oCaused by x’ss secretion TGF-alphaCharac by: diffuse hyperplasia of foveolar epithelium of the. Body and fundus and hyperproteinemia due to protein losing enteropathySecondary symptom: wt loss, diRrhea, peripheral edema.Pathologic feature in children same in adult but pedia dse is self- lim and often follows respi infection.Risk of gastric adenocarcinoma is ⬆️ in adult.

Answer 23

Ménétrier dse

Question 24

Intestinal obstruction occurs at any level but which is commonly affected and why?

Answer 24

Small intestine because of its narrow lumen

Question 25

what are the collection of intestinal obstruction?

Answer 25

1. hernias2. intussuception3. volvulus4. adhesions

Question 26

this account for 80 % of intestinal obstructions

Answer 26

volvulus

Question 27

how many percent does tumors account for intestinal obstruction?

Answer 27

10-15%

Question 28

what are the clin manifestation of intestinal obstruction?

Answer 28

abdomina pain and distentionvomitingconstipation

Question 29

What is the weakness or defect in the wall of the peritoneal cavity that may permit protrusion of a serosa-lined pouch of peritoneum

Answer 29

hernial sac

Question 30

Acquired hernias most commonly occured in the?It is via?

Answer 30

anterioringuinal, femoral canals, umbilicus, or sites of surgical scars

Question 31

The resultant stasis and edema increases the bulk of the herniated loop, leading to permanent entrapment called _______. In which overtime, arterial, and venous compromise ______ develpps that can result in infarction.`

Answer 31

incarcerationstrangulation

Question 32

Surgical procedures, infections, or other causes of peritoneal inflammation, such as endometriosis, may result in development of _______ between bowel segmens, the abdomina wall and operative sites.

Answer 32

adesions

Question 33

Complete twisting of a loop bowel about its mesenteric base of attachment is called _____ and produces both luminal and vascular compromise. Thus presentation includes features of obstruction and infarction. It occurs most often in large redundant loops of sigmoid colon, followed in frequency by the :cecumsmall bowelstomachor rarely transverse colon.Because this is rare, it is often missed clinically

Answer 33

volvulus

Question 34

It occurs when a segment of the intestine, constricted by a wave of peristalsis, telescopes into te immediately distal segment. Once trapped, the invaginated segment is propelled by peristalsis and pulls the mesentery along. If left untreated may result in or progress into intestinal obstruction, compression of mesenteric vessels and infarction.When encountered in infants no underlying cause but come cases are associated with_______.In older children and adults an intraluminal mass or tumor generally serves as the point of traction that causes this.

Answer 34

IntussusceptionRota virus

Question 35

Acute compromise of any major vessel can lead to infarction of several meters of intestine. Damage can range from______.

Answer 35

1. mucosal infarction2. mural infarction3. transmural infarction

Question 36

Infarction extending no deeper than te muscularis mucosa

Answer 36

mucosal infarction

Question 37

Infarction extending of mucosa to submucosa

Answer 37

mural infarction

Question 38

infarction involving all three wall layers

Answer 38

transmural infarciton

Question 39

Mucosal or mural infarctions are often secondary to____

Answer 39

acute or chronic hypoperfusion

Question 40

transmural infarciton is generally caused by _____

Answer 40

acute vascular obstruction

Question 41

what are the two major variables in IBD?

Answer 41

1. Time frame which it develops2. vessels affected

Question 42

Intestinal segments at the end of their respective arterial supplies are particularly susceptible to iscemia. These are called_______ which includes:__________

Answer 42

Watershed zonesplenic flexure where the superior and inferior mesenteric arterial circulation endsto a lesser extent the sigmoid colon andrecutm where inf mesenteric, pudendal and iliac arteries circulations end.

Question 43

What is a morphologic signature of iscemic intestinal disease? why?

Answer 43

Surface epithelial atrophy or even necrosis and sloughing with normal or hyperproliferative crypts .The reason is intestinal capillaries run alongside the glands, form the crypt to surface, before making a hairpin turn at the surface to empty into the post-capillary venules. This allows oxygenated blood to supply crypts but leaves the surce epithelium vulnerable to ischemic injury. This anatomy protects the crypts which contain the epithelial stem cells.

Question 44

Microscopic examination showing ATROPHY OR SLOUGHING OF SURFACE EPITHELIUM. In contrast, crypts may be hyperproliferative. Inflammatory infiltrates are initially absent in acute ischemia nut neutrophils are recruited within hours of reperfusion. Chronic ischemia is accompanied by fibrous scarrring of the lamina propria and uncommonly stricture forms.

Answer 44

IBD

Question 45

In acute phase of IBD bacterial superinfection and enterotoxin release may induce pseudomembrane formation that can resemble ______

Answer 45

C. difficile- associated pseudomembranous colitis

Question 46

It tends to occur in older individual with coexisting cardiac or vulvular disease. Acute transmural infarction typically presents wit sudden abdominal pain and tenderness, sometimes accompanied by nausea, vomiting, bloody diarrhea, or grossly melanotic stool.Patients may progress to shockand vascular collapse within hours as a result of blood loss. Peristaltic sound diminished or disappear and muscular spasm creates board- like rigidity of the abdomina wall.These physical signs overlaps with other abdominal emergencies that results in delayed or missed diagnosis.

Answer 46

IBD

Question 47

These themselves may not be fatal. However, may progress to more extensive infarction if the vascular supply is not restored by correction of the insult or in chronic dse by dev of adequate collateral supplies. Diagnosis is partially difficult since there may be a confusing array of nonspecific abdominal symptoms including intermittent bloody diarrhea and intestinal psuedo-obstruction.

Answer 47

Mucosal and mural infarction

Question 48

May masquarade as IBD with episodes of bloody diarrhea interspesed with periods of healing

Answer 48

chronic ischemia

Question 49

This causes iscemic GI dsew due to viral tropism for and infection of endothelial cells. This is a complication of an immunosuppressive therapy

Answer 49

CMV

Question 50

This occurs when the GI tract is irradiated. In add to epitelial damage, radiation-induced vascular injury may be significant and produce changes that are similar to ischemic disease. The presence of BIZARRE “ RADIATION FIBROBLAST” within the stroma may provide important clue to the etiology. It manifest as anorexia, abdominal cramps, and a malabsorptive diarrhea while chronic radiation enteritis or colitis is often more indolent and may present as an inflammatory colitis.

Answer 50

Radiation enterocolitis

Question 51

Is an acute do of the small and large intestines that can result in transmural necrosis. It is the most common acquired GI emergency of neonates, particularly those who are premature of LBW and occurs most oftenat the time of oral feeding.

Answer 51

NEC

Question 52

Characterized by malformed submucosal and mucosal blood vessels. It occurs most often in the CECUM or RIGHT colon because cecum has the largest diameter that develops tha greatest wall tension. It is usally after the 6th decade of life. Prevalence is < 1 % in adult, it accounts for 20 % major episodes of lower intestinal bleeding; intestinal hemorrage may be chronic and intermittent, acute and massive. Pathogenesis is attributed to mechanical and congenital factors.

Answer 52

angiodysplasia

Question 53

Presents most commonly as chronic diarrhea characterized by defective absorption of fats, fat- and water- soluble vit ,proteins, carbohydrates,electrolytes and mineral and water.

Answer 53

Malabsorption

Question 54

It is accompanied by wt loss, anorexia, abdominal distention, borborygmi and muscle wasting.

Answer 54

chronic malabsorption

Question 55

Steatorrhea is the hallmark of this condition which is characterized by excessive fecal fat and bulky, frothy greasy, yellow or clay colored stool.

Answer 55

Malabsorption

Question 56

Malabsorption results from distrubance of the ff phases:

Answer 56

1. intraluminal digestion2. terminal digestion3. transepithelial digestion4. lymphatic transport

Question 57

The phase of digestion in which fats, protein, CHOH are broken down intor forms suitable for absorption.

Answer 57

intraluminal digestion

Question 58

The phase that involves the hydrolysis of carboydrates and peptieds by dissacharides and peptidases, respectively in the brush boder of the mall intesinal mucosa.

Answer 58

terminal digestion

Question 59

Phase where nutritiens, electrolytes are transported across and processed within the small intetinal epithelium.

Answer 59

transepithelial digestion

Question 60

transport of absorbed lipids

Answer 60

lymphatic transport

Question 61

What are the general sypmtoms of malabsorption

Answer 61

diarrea ( nutrient malabsorption and excessive intestinal secretion)flatusabdominal painand weight loss.

Question 62

It is defined as an increase in stool mass, frequency or fluidity, typically greater than 200g per day. In severe cases, stool volume can exceed 14 L per day and without fluid resusscitation can result in death.

Answer 62

diarrhea

Question 63

Painful, bloody, small volume diarrhea is known as _____

Answer 63

dysentery

Question 64

What are the 4 major categories of diarrhea?

Answer 64

1. secretory diarrhea2. osmotic diarrhea3. malabsorptive diarrhea4. exudative diarrhea

Question 65

Isotonic stool and persist during fasting

Answer 65

secretory diarrhea

Question 66

Excessive osmotic forces exerted by unabsorbed luminal solutes. The diarrhea fluid is more than 50 mOsm more concentrated than plasma and abates with fasting. An example is lactase deficiency.

Answer 66

Osmotic diarrhea

Question 67

It follows generalized failures of nutient absorption and is associated with steatorrhea and is relieved by fasting

Answer 67

Malabsorptive diarrhea

Question 68

It is due to inflammatory disease and characterized by purulent, bloody stools tat continue during fasting.

Answer 68

Exudative diarrhea

Question 69

This is due to absence of CFTR, individuals have defects in intestinal chloride ion secretion. This interferes with bicarb, sodium and water secretion, ultimately resulting in defective luminal hydration.

Answer 69

Cystic Fibrosis

Question 70

It is an immune-mediated enteropathy triggered by te ingestion of gluten-containing cereals, such a wheat, rye or barley, in genetically predisposed individuals. It is common in European and caucasian descent with a prevalence rate of 0.5% to 1%.

Answer 70

Celiac disease, celiac sprue or gluten-sensitive enteropathy.

Question 71

This is a malabsorption that occurs almost exclusively in people living in or visiting the tropics, including Puerto Rico, the Caribbean, northen South America, West Africa, India and Southeast Asia. Histologic changes are similar to celiac disease, but toal villous atrophy is uncommon and tends to inolve the distal small bowel. The latter probably explains the freq of folate and vit B12 deficiencies with megaloblastic anemia. There is no definite casual organism but various infections including Cyclospora or enterotoxigenic bacteria have been suggested as etiologic factors.

Answer 71

Tropical Sprue

Question 72

It is an X-linked do characterized by severe diarrhea and autoimmune disease that occurs most often in young children. There is a germline mutation in te FOXP3 gene which is located on the X chromosome.

Answer 72

Autoimmune enteropathy

Question 73

What does IPEX stands for?

Answer 73

Immune dysregulation, polyendocrinopathy, enteropathy and X- linkage

Question 74

What is the pathophysio of IPEX?

Answer 74

There is a germline mutation of FOXP3 which is located in X chromosome. FOXP3 is a transcriptor factor expressed in CD4+ regulatory T cells and individuals with IPEX and FOXP3 mutations have defective T-regulatory function.

Question 75

Where is the disaccharide lactase located?

Answer 75

apical brus-border membrane of the villous

Question 76

It is caused by mutation in the gene encoding lactase, is an autosomal recessive disorder. The disease is rare and presents as EXPLOSIVE DIARRHEA with watery, FROTHY stools and abdomina distention upon milk ingestion.

Answer 76

Congenital Lactase deficiency

Question 77

It is a down regulation of lactase gene expression and is particularly common among native Americans, African Americans and Chinese populations. Disease presents after childhood, peraps reflecting the fact that , before farming of dairy animals, lactase was unnecessary after children stopped drinking mother’s milk

Answer 77

acquired lactase deficiency

Question 78

This is a rare autosomal recessive disease characterized by an inability to secrete triglyceride-rich lipoprotein due to mutation in the microsomal triglyceride transfer protein ( MTP) that catalyzes transport of triglycerides, cholesterol esters and phospholipids. MTP-deficient are unable to export lipoproteins and free faty acids as a result that monoglycerides cant be assembled as chylomicrons and TG accumulate within the epithelial cells. This presents in infancy and clinical picture is dominated by failure to thrive, diarrhea and steatorrhea. There is presence of acanthocytic red cells (burr cells) in PBS.

Answer 78

ABETALIPOPROTEINEMIA

Question 79

What are the infectious Enterocolitis?

Answer 79

1. Cholera2. Campylobacter Enterocolitis3. Shigellosis4.Salmonellosis5. Typhoid Fever6. Yersina7. Eschericia Coli8. Pseudomembranous Colitis9. Whipple Disease10. Viral Enterocolitis11. Parasitic Enterocolitis

Question 80

It is a comma -shaped , Gr negative bacteria, a diseases that as been endemic in the Ganges Valley of India and Bangladesh for all recorded history. It is primarily transmitted by contaminated drinking water. However, it can also be present in food and causes sporadic cases of sea-food associated disease in North America.

Answer 80

Vibrio cholera

Question 81

There is a marked seasonal variation in most climates due to rapid growth of vibrio bacteria at warm temperature; the only animal resovoirs are______

Answer 81

shellfish and planktons

Question 82

It is the most common cause of seafood- associated gastroenteritis in North America.

Answer 82

V. Parahaemolyticus

Question 83

What virulence factor in V. cholerae is responsible for te bacterial detachment and shedding in stool.

Answer 83

Hemaglutinin

Question 84

It is the most common bacterial enteric patogen in developed countires and is an important cause of traveler’s diarrea. Most infections are associated wit ingestion of improperly cooked chicken but outbreaks can also be caused by unpasteurized milk or contaminated water. The clinical presentation watery diarrhea , either acute or following an influenza-like prodrome is the primary symptom and dysentery develops in 15 % of patient.

Answer 84

Campylobacter enterocolitis

Question 85

True or False. In Campylobacter enterocolitism, antibiotic therapy is required?

Answer 85

FALSE

Question 86

Gr. Neg bacilli that were initially isolated during the Japanese red diarrhea epidemic of 1897. These are unencapsulated, nonmotile, facultative anaerobes that belong to the Enterobacteriacea and are closely related to enteroinvasive E.coli. Humans are the only known reservoir and they are the most common cause of bloody diarrhea.

Answer 86

Shigella

Question 87

What are the four majour subgroup of E.coli

Answer 87

ETEC ( Enterotoxigenic E.coli)EHEC ( Enterohemorrhagic E.coli)EIEC ( Entero invasive E.coli)EAEC ( Enteroaggregative E.coli)

Question 88

These are the principal cause of traveller’s diarrhea that is most common un underdeveloped regions affecting children younger than 2 years of age are susceptible.

Answer 88

ETEC

Question 89

In developed countries assoctiate with the consumption of inadequately cooked ground beef, sometimes from fast food. Contaminated milk and vegetables are also vehicles.

Answer 89

E.coli 0157:H7

Question 90

These are organism that are bacteriologic similar to Shigella and are transmitted by person to person, food and water. These dont produce toxins but instead they cause to invade epithelial cells and cause nonspecific features of acute limited colitis.

Answer 90

Enteroinvasive E. coli

Question 91

Identified on the absis of their uniques pattern of adherence to epithelial cells. It also cause traveller’s diarrhea. They attach to epithelial cell via ADHERENCE FIMBRIAE and are aided by dispersin, a bacterial surface protein that neutralizes the negative surface charge of LPS. It produces enterotoxin related to SHIGELLA and ETEC ST toxin

Answer 91

EAEC

Question 92

Generally caused by ________________, is also known as antibioticassociated colitis or antibiotic-associated diarrhea. The latter terms apply to diarrhea developing during or after a course of antibiotic therapy and may be due to C. difficile as well as Salmonella, C. perfringens type A, or Staphylococcus aureus. The latter two organisms produce enterotoxins and are common agents of food poisoning.

Answer 92

Pseudomembranous colitis

Question 93

Morphology. Fully developed_____ is accompanied by formation of pseudomembranes , made up of an adherent layer of inflammatory cells and debris at sites of colonic mucosal injury. While pseudomembranes are not specific and may occur in ischemia and necrotizing infections, the histopathology of this is striking. The surface epithelium is denuded, and the superficial lamina propria contains a dense infiltrate of neutrophils and occasional fibrin thrombi within capillaries. Superficially damaged crypts are distended by a mucopurulent exudate thatforms an eruption reminiscent of a volcano ( These exudates coalesce to form the pseudomembranes

Answer 93

pseudomembranous colitis

Question 94

a rare, multivisceral chronic disease first described as intestinallipodystrophy. original case report described an individual with malabsorption, lymphadenopathy,and arthritis of undefined origin.Postmortem examination demonstrated the presence of foamy macrophages and large numbers of argyrophilic rods in the lymph nodes, providing evidence that the disease was infectious. Clinical symptoms occur because organism-laden macrophages accumulate within the small intestinal lamina propria and mesenteric lymph nodes, causing lymphatic obstruction. Thus, the malabsorptive diarrhea of Whipple disease is due to impairedlymphatic transport.

Answer 94

Whipples Disease

Question 95

The Gram-positive actinomycete, named ________, that is responsible for Whipple disease was identified by PCR in 1992 and finally

Answer 95

Tropherymawhippelii

Question 96

the malabsorptive diarrhea of Whipple disease is due to impaired_________.

Answer 96

lymphatic transport

Question 97

This is a disease is a dense accumulation of distended, foamy macrophages in the small intestinal lamina propria ( Fig. 17-30A ).The macrophages contain periodic acid–Schiff (PAS)-positive, diastase-resistant granules that represent lysosomes stuffed with partially digested bacteria ( Fig. 17-30B ). Intact rodshaped bacilli can also be identified by electron microscopy ( Fig. 17-30C ). A similar infiltrate of foamy macrophages is present in intestinal tuberculosis ( Fig. 17-30D ), and in both diseases the organisms are PAS-positive. The villous expansion caused by the dense macrophage infiltrate imparts a shaggy gross appearance to the mucosal surface. Lymphatic dilatation and mucosal lipid deposition account for the common endoscopic detection of white to yellow mucosal plaques. In this disease, bacteria-laden macrophages can accumulate within mesenteric lymph nodes, synovial membranes of affected joints, cardiac valves, the brain, and othersites

Answer 97

Whipple disease

Question 98

The morphologic hallmark of Whipple disease is a ________

Answer 98

dense accumulation ofdistended, foamy macrophages in the small intestinal lamina propria

Question 99

a rare, multivisceral chronic disease first described as intestinal lipodystrophy. Its original case report described an individual with malabsorption, lymphadenopathy,and arthritis of undefined origin. Postmortem examination demonstrated the presence of foamymacrophages and large numbers of argyrophilic rods in the lymph nodes, providing evidence Infectious Enterocolitis that the disease was infectious. Clinical symptoms occur because organism-laden macrophagesaccumulate within the small intestinal lamina propria and mesenteric lymph nodes, causing lymphatic obstruction. Thus, the malabsorptive diarrhea is due to impairedlymphatic transport

Answer 99

Whipple’s Disease

Question 100

The clinical presentation of is usually a triad of diarrhea, weight loss, andmalabsorption. Extraintestinal symptoms, which can exist for months or years beforemalabsorption, include arthritis, arthralgia, fever, lymphadenopathy, and neurologic, cardiac, orpulmonary disease.

Answer 100

Whipple disease

Question 101

is a common cause of nonbacterial infectious gastroenteritis. [87] These are small icosahedral viruses with a single-stranded RNA genome that forms a genus within the Caliciviridae family. Norovirus causes approximately half of all gastroenteritis outbreaks worldwide and is a common cause of sporadic gastroenteritis indeveloped countries. Local outbreaks are usually related to contaminated food or water, but person-to-person transmission underlies most sporadic cases.Infections spread easily within schools, hospitals, nursing homes, and, most recently, cruise ships. Following a short incubation period, affected individuals develop nausea, vomiting, watery diarrhea, and abdominal pain.Biopsy morphology is nonspecific. When detected, abnormalities are most evident in the small intestine and include mild villous shortening, epithelial vacuolization, loss of the microvillus brush border, crypt hypertrophy, and lamina propria infiltration by lymphocytes (Fig. 17-31A ). The disease is self-limited.

Answer 101

Norovirus

Question 102

This encapsulated virus with a segmented double-stranded RNA genome infects 140 million people and causes 1 million deaths each year, making rotavirus the most common cause of severe childhood diarrhea and diarrheal mortality worldwide. Children between 6 and 24 months of age are most vulnerable.Protection in the first 6 months of life is probably due to the presence of antibodies to rotavirus in breast milk, while protection beyond 2 years is due to immunity that develops following the first infection. [88] Outbreaks in hospitals and daycare centers are common, and infection spreads easily; the estimated minimal infective inoculum isonly 10 viral particles.It selectively infects and destroys mature enterocytes in the small intestine, and the villus surface is repopulated by immature secretory cells. This results in loss of absorptive function and net secretion of water and electrolytes that is compounded by an osmotic diarrhea from incompletely absorbed nutrients. Like norovirus, rotavirus this has short incubation period followed by several days of vomiting and watery diarrhea.

Answer 102

Rotavirus.

Question 103

The second most common cause of pediatric diarrhea (after rotavirus), _______ also affectsimmunocompromised patients. [89]Small intestinal biopsy specimens can show epithelial degeneration but more often exhibit nonspecific villous atrophy and compensatory crypt hyperplasia. Viral nuclear inclusions are uncommon. Disease typically presents after an incubation period of 1 week with nonspecific symptoms that include diarrhea, vomiting, andabdominal pain. Fever and weight loss may also be present. Symptoms generally resolve within 10 days.

Answer 103

adenovirus

Question 104

It is characterized by chronic, relapsing abdominal pain, bloating,and changes in bowel habits. Despite very real symptoms, the gross and microscopicevaluation is normal in most IBS patients. Thus, the diagnosis depends on clinical symptoms.

Answer 104

Irritable Bowel Disease

Question 105

Explain the pathophysio of IBS

Answer 105

The pathogenesis of IBS remains poorly defined, although there is clearly an interplay between psychologic stressors, diet, and abnormal GI motility. Data showing disturbances of intestinal motility and enteric sensory function suggest that impairment of signaling in the brain-gut axis contributes to IBS. A small subgroup of IBS patients also relate onset to a bout of infectious gastroenteritis, suggesting an immune or neuroimmune contribution.

Question 106

This is a chronic condition resulting from inappropriate mucosalimmune activation.

Answer 106

Inflammatory Bowel Disease ( IBD )

Question 107

The two disorders that comprise IBD are :

Answer 107

Crohn’s diseaseulcerative colitis

Question 108

The distinction between ulcerative colitis and Crohn disease is based, in large part, on the _________.

Answer 108

distribution of affected sitesmorphologic expression of diseaseSTUDY( Table17-8 ) at those sites.

Question 109

Molecular linkage analyses of affected families have identified _______ as a susceptibility gene in Crohn disease. This encodes a protein that binds to intracellular bacterial peptidoglycans and subsequently activates NF-κB. It has been postulated thatthis variants are less effective at recognizing and combatingluminal microbes, which are then able to enter the lamina propria and triggerinflammatory reactions. Other data suggest that it may regulate immune responsesto prevent excessive activation by luminal microbes. Whatever the mechanism by which thiscontribute to Crohn disease pathogenesis, it should beremembered that fewer than 10% of individuals carrying this mutations developdisease.

Answer 109

NOD2

Question 110

Crohn disease may occur in any area of the GI tract, but the most commonsites involved at presentation are the __________.Disease is limited to the small intestine alone in about 40% of cases; the small intestine and colon are both involved in 30% of patients; and the remainder have only colonicinvolvement. The presence of multiple, separate, sharply delineated areas of disease,resulting in skip lesions, is characteristic of Crohn disease and may help in thedifferentiation from ulcerative colitis. Strictures are common ( Fig. 17-34A )

Answer 110

terminal ileum, ileocecal valve, and cecum

Question 111

The earliest Crohn disease lesion, the ______, may progress, and multiple lesionsoften coalesce into elongated, serpentine ulcers oriented along the axis of the bowel.Edema and loss of the normal mucosal texture are common.

Answer 111

aphthous ulcer

Question 112

The earliest Crohn disease lesion, the _______, may progress, and multiple lesions often coalesce into elongated, serpentine ulcers oriented along the axis of the bowel. Edema and loss of the normal mucosal texture are common.

Answer 112

aphthous ulc

Question 113

This is most common in the colon but may occur in the esophagus, stomach, or smallintestine. Most, if not all, begin as small elevations of the mucosa. These are referred to as sessile, a term borrowed from botanists who use it to describe flowers and leaves that grow directly from the stem without a stalk.

Answer 113

polyps

Question 114

The most common neoplastic polyp is the______, which has the potential to progress to cancer.

Answer 114

adenoma

Question 115

The nonneoplastic polyps can be further classified as __inflammatory, hamartomatous, or hyperplastic________________.

Answer 115

inflammatory, hamartomatous, or hyperplastic

Question 116

The polyp that forms as part of the solitary rectal ulcer syndrome is an example of a purely type of this. Patients present with a clinical triad of rectal bleeding, mucus discharge,and an inflammatory lesion of the anterior rectal wall. The underlying cause is impaired relaxation of the anorectal sphincter that creates a sharp angle at the anterior rectal shelf and leads to recurrent abrasion and ulceration of the overlying rectal mucosa.This may ultimately form as a result of chronic cycles of injury and healing. Entrapment of this polyp in the fecal stream leads to mucosal prolapse. Thus, the distinctive histologic features are those of a typical with superimposed mucosal prolapse and include lamina propria fibromuscular hyperplasia, mixed inflammatory infiltrates, erosion, and epithelial hyperplasia

Answer 116

Inflammatory polyp

Question 117

Solitary rectal ulcer syndrome.A, The dilated glands, proliferativeepithelium, superficial erosions, and inflammatory infiltrate are typical of an ________However, the smooth muscle hyperplasia within the lamina propria suggests that mucosalprolapse has also occurred.B, Epithelial hyperplasia.C, Granulation tissue-like capillaryproliferation within the lamina propria caused by repeated erosion and re-epithelialization

Answer 117

inflamatory polyp.

Question 118

This occurs sporadically and in the context of various genetically determinedor acquired syndromes ( Table 17-9 ). Recall that these are tumor-like growthscomposed of mature tissues that are normally present at the site in which they develop.Although these syndromes are rare, they are important to recognizebecause of associated intestinal and extra-intestinal manifestations and the possibility that other family members are affected

Answer 118

HAMARTOMATOUS POLYPS

Question 119

Mean Age at Presentation (yr)— Mutated Gene : SMAD4, BMPR1AGastrointestinal Lesions : Juvenile polyps; risk of gastric, small intestinal,colonic, and pancreatic adenocarcinomaSelected Extra-Gastrointestinal Manifestations: Pulmonaryarteriovenousmalformations, digital clubbing

Answer 119

Juvenile polyposis

Question 120

Mean Age at Presentation (yr) :10–15 Mutated Gene :LKB1/STK11Gastrointestinal Lesions : Arborizing polyps; Small intestine > colon > stomach; colonic adenocarcinomaSelected Extra- Gastrointestinal Manifestations : Skin macules; increasedrisk of thyroid, breast, lung, pancreas, gonadal, and bladder cancers

Answer 120

Peutz-Jegherssyndrome

Question 121

Mean Age atPresentation (yr) :Mutated Gene :Gastrointestinal Lesions :Selected Extra- Gastrointestinal Manifestations :

Answer 121

Cowden syndrome,Bannayan- Ruvalcaba-Riley syndrome