Rubins - combined Flashcards
- acidophilic (eosinophilic / pink-staining on H&E) globule of cells that represents a dying hepatocyte often surrounded by normal parenchyma.
- They are found in the liver of individuals suffering from viral hepatitis (acute), yellow fever, or other viral syndromes.
What are some diseases associated with chronic Hep B?
Manifestations due to circulating immune complexes which can cause
- serum sickness-like syndrome
- glomerulonephritis
- cryoglobulinemia
- polyarteritis nodos
Massive hepatic necrosis due to herpes virus. What is the most likely culprit? (A-E)
Hep B most likely to cause this. Other viruses are not associated with massive hepatic necrosis
Primary biliary sclerosis - classic presentation
- fatigue
- anorexia
- jaundice
- xanthomas of the skin
- pruritus
First 3 are liver findings
Last 2 are skin findings
Clinical hallmarks of advanced hereditary hemochromatosis? (3)
Presence of other diseases such as
- diabetes
- skin pigmentation
- cardiac failure
These globular inclusions are misfolded alpha1-antitrypsin proteins in hepatocytes. They stain red after PAS staining
Andersen disease
Glycogen storage disease type IV
Genetic disease caused by deficiency of glycogen-branching enzyme
- results in accumulation of abnormal glycogen (amylopectin) in the liver, muscle and other tissues
- failure to thrive and hepatosplenomegaly
- progressive hepatic fibrosis leading to cirrhosis and liver failure
Most common genetic porphyria
Porphobilinogen deaminase – autosomal dominant
Clinically: colicky abdominal pain and neuropsychiatric symptoms
Neonatal hepatitis - classic feature on histology
Giant cell formation
Clinically will present with prolonged cholestasis, inflammation and cell injury
Neonatal hepatitis - causes?
About 50% of the cases are discernible
- alpha1-antitrypsin deficiency (30%)
- chromosomal abnormalities
- intrauterine infections
- biliary atresia
Scleroderma
characterized by systemic fibrosis with deposition of collagen affecting most organs.
Unknown etiology
- Diffuse disease –> Progressive Sclerosis Scleroderma (PSS)
- More benign disease –> CREST syndrome
Staining:
- Anti-Scl-70 positive
- ANA positive
Scleroderma - PSS
PSS = Progressive Sclerosis Scleroderma
- rapid progression and early visceral iinvolvement
- skin
- swelling that starts at fingers and continues proximally
- “tightened” facial features
- Raynaud’s phenomenon
- polyarthralgia
- GI
- heartburn + dysphagia for solids and liquids
- reflux due to esophageal dysmotility
- due to smooth muscle conversion to collagen
- malabsorbtion + bacterial overgrowth
- loss of villi with diverticula formation
- heartburn + dysphagia for solids and liquids
- respiratory
- dyspnea (due to interstitial fibrosis)
- renal
- vasculitides, infarctions, malignant hypertension
Scleroderma - CREST syndrome
Calcinosis (subcutaneous calcifications)
Raynaud’s phenomenon
Esophageal dysfunction (due to LES sclerosis likely resulting in dysphagia)
Sclerodactyly
Telangiectasia
dyspepsia
indigestion
hiatal hernia - symptoms
- heart burn
- regurgitation
mainly attributed to the reflux of stomach contents due to incompetence of the LES
What kind of necrosis is seen with the ingestion of acids?
akaline solutions?
acid –> coagulative necrosis (of the esophagus)
alkaline –> liquefactive –> inflammation and saponification thereafter
- much more dangerous/insidious than acidic, b/c they are generally odorless and tasteless and therefore easily swallowed before protective reflexes come into play
reflux esophagitis
common complication of hiatal hernia
- thickening of the basal layer of the squamous epithelium
- extension of the papillae of the lamina propria
- increased number of neutrophils and lymphocytes
- areas of gastric reflux are susceptible to erosions and ulcers and appear as linear vertical streaks
Candida esophagitis
Commonplace in immunocompromised persons.
- pseudomembranes are formed composing of fungal mycelia, fibrin and necrotic debris
- involvement of deeper layers of esophageal wall can lead to disseminated candidiasis as well as fibrosis which can create esophageal strictures
Classical symptoms
- dysphagia
- odynophagia (pain on swallowing)
Occurs in
- immunocompromised
- diabetics
- those receiving antibiotic therapy
odynophagia
pain on swallowing
Populations most susceptible to Candida infections (3)
- immunocompromised
- diabetics
- those receiving antibiotic therapy
Acute hemorrhagic gastritis
Commonly associated with intake of:
- aspirin or NSAIDs
- alcohol
- ischemic injury
Thought to result int he breakdown of the mucosal barrier, which permits acid-induced injury (ie ulcers –> black stools)
- necrosis is accompanied by an acute inflammatory response and hemorrhage –> exsanguination and hypovolemic shock (if severe enough)
Ménétrier disease
Ménétrier’s disease causes the ridges along the inside of the stomach wall—called rugae—to enlarge, forming giant folds in the stomach lining.
- rugae enlarge due to an overgrowth of mucous cells in the stomach wall.
In a normal stomach, mucous cells in the rugae release protein-containing mucus. The mucous cells in enlarged rugae release too much mucus, causing proteins to leak from the blood into the stomach. This shortage of protein in the blood is known as hypoproteinemia.
Ménétrier’s disease also reduces the number of acid-producing cells in the stomach, which decreases stomach acid.
Ménétrier disease - classical presentation
Severe loss of plasma proteins (including albumin) from the altered gastric mucosa
- increased mucous production
- decreased acid production
Can include weight loss.
Ménétrier disease - types/forms (2)
- A childhood form due to CMV infection
- An adult form attributed to overexpression of TGF-alpha
Gastrointestinal stromal tumor (GIST)
- Derived from pacemaker cells of Cajal
- Comprises the vast majority mesenchyme-derived stromal tumors of the entire GI track.
- Usually submucosal and covered by intact mucosa
- Microscopically: spindle cells with vaculoated cytoplasms
- Low malignant potentional
- Surgically removed
Gastrointestinal stromal tumor (GIST) - what cells are they derived from?
Derived from pacemaker cells of Cajal
Bezoar - what are they?
What are their sub categories?
Bezoar are foreign bodies in the stomach composed of food or hair that have been altered by the digestive process
-
Trichobezoar – hairball within a gelatinous matrix
- seen in long-haired girls or young women who eat their own hair as a nervous habit
- trichotillomania or Rapunzel syndrome
-
Phytobezoar – concretions of plant material
- usually occuring in persons with conditions that interfere with gastric emptying
trichotillomania
compulsive disorder characterized by the compulsive urge to pull out one’s hair, leading to hair loss and balding, distress, and social or functional impairment
Can result in bezoar (trichobezoar)
Rapunzel syndrome
an extremely rare intestinal condition in humans resulting from ingesting hair (trichophagia)
Carcinoid tumors - most common location
Submucosa of the intestines (appendix, terminal ileum, and rectum)
Carcinoid tumors - clinical features
- Flushing
- bronchial wheezing
- watery diarrhea
- abdominal colic
Presumably due to the release of 5HT, bradykinin and histamine
Spontaneous bacterial peritonitis
Can occur without obvious perforation
In children
- most have a nephrotic syndrome
- most have a systemic infection that seeds the ascitic fluid with bacteria
In adults
- feared complication of cirrhosis
Pseudomembranous colitis - what is it?
What is typically found on colonoscopy or gross exam?
Pseudomembranous colitis – is a generic term for an inflammatory disease due to an overgrowth of Clostridium dificile. Typically caused by antibiotic therapy.
On colonoscopy or gross exam, it is characterized by exudative plaques.
Anorectal malformations
Most common anomalies and vary from minor narrowing to serious and complex effects
- classification is based on relation of terminal bowel ot the levator ani muscle
Result from arrested development of the caudal region of the gut in the first 6 months of fetal life.
- cause is unknown
- fistulas between the malformation and the bladder, urethra, vagina or skin may occur in all types of anorectal anomalies
Anorectal malformations - cause?
Result from arrested development of the caudal region of the gut in the first 6 months of fetal life.
cause is unknown
Anorectal malformations - complications
fistulas between the malformation and the bladder, urethra, vagina or skin may occur in all types of anorectal anomalies
Stercoral ulcers
an ulcer of the colon due to pressure and irritation resulting from severe, prolonged constipation due to large bowel obstruction.
It is most commonly located in the rectum.
Complications: bleeding and perforation
Mucocele
Refers to a dilated mucous-filled appendix. Pathogenesis may be neoplastic or nonneoplastic
non-neoplastic
- chronic obstruction leads to retention of mucus in the appendiceal lumen
neoplastic (mucious cystadenoma/cystadenocarcinoma)
- dilated appendix is lined by villous adenomatous mucosa
- mucocele may become infected and rupture
- rupture may see the peritoneal cavity w/ mucous secreting tumor cells –> pseudomyxoma peritonei
pseudomyxoma peritonei
seeding of the peritoneal cavity with mucus-secreting tumor cells (typically from a mucinous adenocarcinoma)
- will cause fibrosis of tissues and impede digestion or organ function
- if left untreated, tumor and mucin will fill the abdominal cavity –> compression of organs –> destroy function of colon, small intestine, stomach and other organs
Curling ulcers - most common site
stomach
Small (<1cm) and shallow, never penetrating the muscularis propria, but they can bleed profusely
What is the pathogenesis of H. pylori? (what is the gene associated with damage)
It does not invade but rather hangs out on the mucosal surfaces producing cytotoxin-associated gene A (CagA)
gastric polyps – most comon type?
why do they arise? complications?
Gastric inflammatory/hyperplastic polyps – most common
Arise in the setting of H. pylori infection
May be precursors to gastric adenocarcinomas particularly if lesions > 1.5cm with high-grade dysplasia
Fundic gland polyps – associations
associated with the use of proton pump inhibitors and also with FAP
increased gastrin (remember g-cells in the fundus which are stimulated w/ the use of proton pump inhibitors) –> drives glandular hyperplasia
t(11;18) - what do you suspect?
Found in all MALTomas (probably associated with some H. pylori infection)
Will regress with anti-biotic therapy
Most common cause of acute pancreatitis (assuming everything normal)
cholelithiasis (~45% of patients with acute pancreatitis have this)
Glucagonomas - what is its assocation with skin?
Necrotizing migratory erythema (necrotizing erythematous skin rash)
“Pancreatic cholera”
Intractable diarrhea, hypokalemia nad low levels of chloride in gastric juice constitutes this syndrome
- always secondary to the secretion of vasoactive intestinal polypeptide
- stimulated adenylyl cyclase activity –> increased cAMP –> secretion of K+ and water into the intestinal lumin
Delta cell tumors - what do they produce?
How do they present?
Somatostatinomas – produce a syndrome consisting:
- diabetes mellitus
- gallstones
- steatorrhea
- hypochlorhydria
Result from the inhibitory action of somatostatin shutting down secretion of hormones from
- endocrine pancreas –> DM
- acinar cells of pancreas –> stones, steatorrhea
- certain hormone secreting cells of GI –> hypochlorhydria
