Gastrointestinal System Flashcards

1
Q

Auto immune hepatitis

A

Female > male
Associated other autoimmune disorders
Hepatocellular injury (↑ transaminases)
Anti–smooth muscle & Antinuclear Antibodies
Histology: Interface hepatitis (portal & periportal lymphoplasmacytic infiltrate)

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2
Q

Primary biliary cholangitis

A

Female&raquo_space; male
Asossiated other autoimmune disorders
↑ alkaline phosphatase , ↑ Bilirubin
Autoimmune destruction of intralobular bile ducts.
Complication includes Cirhosis & Liver failure
Malnutrition
Bile aids in absorbtion of Fat-Sol vitamins thus patient can have Deficiency of Fat sol vitamins( A,D, E &K)
Antimitochondrial & Antinuclear Antibodies
Histology: Florid duct lesion (granulomatous destruction of small bile ducts)

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3
Q

Primary sclerosing cholangitis

A

Male > female
Asossiated with IBD (particularly UC)
↑ alkaline phosphatase
± p-ANCA*
Histology: Fibrous obliteration of bile ducts with concentric periductal deposition of connective tissue (onion skin–like pattern )

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4
Q

Reye Syndrome

A

Acute liver failure
Hepatomegaly
Elevated transaminases; coagulopathy
Rapidly progressive encephalopathy
Vomiting, lethargy, seizures, coma following salicylate (aspirin) administration in children.
Aspirin-induced mitochondrial dysfunction causes impaired fatty acid metabolism and microvesicular steatosis of the liver

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5
Q

Carcinoid syndrome

A

Diarrhea, Flushing, Wheezing

– Carcinoid Heart Disease (Right sided endcardial fibrosis and thickening of heart valves secondary to exposure to serotonin)

– Vitamin B3 deficiency (Pellagra > Diarhea, Dermatitis, Dementia), Vit-B3 def is because Tryptophan which is also a precursor for Vit-B3 gets shunted more towards production of serotonin therefore Vit-B3 level decrease.

– Elevated 24-hr urinary excretion of 5-HIAA.

– Octreotide for symptomatic patients.

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6
Q

Familial adenomatous polyposis

A

APC gene mutation
Colorectal cancer
Desmoids & osteomas
Brain tumors

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7
Q

AAA

A

abdominal aortic aneurysm
risk factors
Age>65yrs, Male, Smoking

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8
Q

Kawasaki disease

A

Spiking Fever for 5 days or more.
Cervical lymphadenopathy, desquamating rash, coronary aneurysms, red conjunctivae and tongue (Strawberry tounge), hand-foot changes
(mucocutaneous lymph node syndrome,
treat with IVIG and aspirin)

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9
Q

Sjögren syndrome

A

(autoimmune destruction of exocrine glands)
Dry eyes, dry mouth, arthritis

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10
Q

Plummer-Vinson syndrome

A
  • Dysphagia (esophageal webs),
  • Glossitis,
  • Iron deficiency anemia
    (may progress to esophageal
    squamous cell carcinoma)
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11
Q

Zollinger-Elison Syndrome
(ZES)

A

Traid of:
Pancreatic/Duodenal tumor (Gastrinoma)
Increase Gastric secretion (Non responsive to antiacids)
Peptic Ulcer (Distal Duodenal Ulcer)

Other symptoms
Diarrhea
Abdominal pain
Malabsorbtion
Post-prandial pain

Gastrin increases in response to exogenus Secretin (normally secretin inhibits gastrin production in ZES effect is opposite)

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12
Q

Zenkers Diverticulum

A

False Diverticulum
Develops immideately above UES
Posterior mucosal herniation between fibers of Circophyrngeus muscle

Pathophysiology : (diminished relaxtion of circopharengeal muscle during swallowing leads to incr intraluminal pressure eventually causing mucosa to herniate through a zone of muscle weakness in posterior hypopharynx)

Presents as:

  • Dysphagia
  • Regurgitation/ Halitosis
  • Aspiration
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13
Q

Meckels Diverticulum

A

True diverticulum
all 3 layer: mucosa, submucosa, muscularis
Outpouching from ileum w fibrous band connected to umbilicus
Congenital anamoly due to incomplete obliteration of Vitelline (omphalomesenteric) duct

Classically present with painless hematochezia +/– Iron def anemia.
Abdominal pain if inflamed (i.e Diverticulitis)

Often contain Heterotrophic gastric mucosa secreting HCl and causing local ulceration & bleeding of small bowel.
Rule of 2: Present by age 2, <2 inches long, Located with 2 feet of Ileocecal valve

Complications: Intussuception, Volvulus, Bowel Perforation

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14
Q

Scleroderma/ Systemic Sclerosis

A

It can be localized called as localized scleroderma or diffuse/systemic scleroderma called as systemic sclerosis.

AKA “ C R E S T syndrome ”

C = Calcinosis (Ca+2 deposit in skin)
R = Raynauds Phenomenon
E = Esophageal dysfunction(acid reflux,↓motility)
S = Sclarodactyly (thickening & tightening of
skin on fingers & hands)
T = Telangectasias

– Anti-Centromere Antibodies are classic for CREST syndrome.
– Anti-Topoisomerase (Anti-Scl-70) are seen in diffuse systemic sclerosis.

Pulmonary fibrosis can be caused secondary to CREST syndrome, both limited and diffuse systemic sclerosis can lead to pulmonary fibrosis.

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15
Q

Peutz-Jeghers

A

Combo of Perioral Melanosis and Hamartomatous colonic polyps
Start colonoscopy at age 5 then do it every 1-2yrs

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16
Q

Duodenal Atresia

A

Bilious vomitting in Neonate.
Obstruction at level of duodenum or lower.
Assossiated with Down syndrome
Double Bubble sign on X-ray.

17
Q

Pyloric Stenosis

A

Forceful/ Projectile Non-bilious vomitting in neonates.
Obstruction is above the level of duodenum.
Hypertrophic pylorous “ Olive shaped mass “ in abdomen.
Ultrasound for diagnosis.

18
Q

Celiac disease

A

Intolerence to Gluten ( i.e gliadin protiens found in wheats, oats, rye and barley, but not in rice )
Causes Type-IV hypersenstivity response where T-cells attack the small intestinal villi, resulting in flattening of villi and malabsorbtion.
Anti-Endomysial ( AKA Anti-Gliadin ) antibody
Anti-Tissue Transglutaminase IgA
Patients with IgA deficiency have greater chance of developing celiac disease
Celiac disease present as vague bloating and diarrhea but can also cause Iron Def Anemia as iron absorbtion is impaired by flattening of villi.

Celiac disease has low Hb ( HY differential )

D-xylose test is abnormal as intestinal lining architecture is abnormal.

Assossiated with dermatitis herpitiformis and also cause increased risk of gastrointestinal T-cell Lymphoma ( Enteropathy- Assosiated T-cell Lymphoma EATL )

19
Q

Whipple Disease

A

Caused by bacterium Troponema Whipplei.
Causes PAS positive macrophages in lamina propria of small bowel.
Can cause Arthritis and Diarrhea.
Tx: Antibiotics ( Doxycycline )

20
Q

Spontaneous Bacterial Peritonitis (SBP)

A

Cirrhosis + Fluid wave + Fever + Abdominal pain

Diabetic + undergoing peritoneal dialysis + Fever + Abdominal pain

TX: Ceftriaxone.

21
Q

Charcots Triad

A

Jaundice , Fever (Rigors) , RUQ pain
For Ascending Colangitis

22
Q

Reynolds Pentad

A

Charcots Triad ( Jaundice , Fever (Rigors) , RUQ pain ) with Hypotension and Altered mental status.
For Ascending Cholangitis

23
Q

Criggler Najjar Type-I

A
  • Unconjugated bilirubin buildup;
  • In newborn or infants;
  • No UDP-GT;
  • Phenobarbital yields no change
24
Q

Congenital diaphragmatic
hernia

A
  • Caused by failure of formation of pleuroperitoneal membranes.
  • Always occurs on the left, where bowel from the abdomen can herniate up into the left-chest.
  • Can present as ↓ bowel sounds in the abdomen + ­↑ bowel sounds in the left hemithorax.
25
Q

Somatostatinoma

A

Delta-cell tumor&raquo_space; Somatostatin.

  • Global GI inhibition.
  • Steatorhea.
  • Gallstones.
  • Mild diabetes.

Tx: Oral hypoglycemics

26
Q

MEN Syndromes
(Multiple Endocrine Neoplasia)

A

Autosomal dominant syndromes

– MEN 1 : Menin gene
“ P P P “
Pituitary adenoma ,
Parathyroid adenoma ,
Pancreatic adenoma.

– MEN 2A : Marfanoid habitus
“ P M P “
Parathyroid adenoma,
Medullary thyroid carcinoma,
Pheochromocytoma.

– MEN 2B : Marfanoid habitus
“ P M N “
Pheochromocytoma,
Medullary thyroid carcinoma,
Neuromas (mucosal).

Both MEN 2A and 2B have RET gene mutation

27
Q

Neuroblastoma

A
  • Young child with large abdominal mass.
  • Urine shows elevated catecholamine metabolites.
  • Biopsy shows small, round, blue tumor cells.
  • This tumor is of neural crest cell origin.
28
Q

A middle-aged woman with joint pain, dry eyes, and dry mouth has extensive lymphoid infiltrate with germinal centers on excision of a salivary gland. What is the diagnosis?

A

Sjogren syndrome.

Positive anti-Ro (SSA) &/or anti-La (SSB)

29
Q

CREST Syndrome

A
  1. Calcinosis: calcium deposits in the skin
  2. Raynaud phenomenon: blood vessel spasms in response to cold or stress
  3. Esophageal dysfunction: acid reflux +/- decrease esophageal motility
  4. Sclerodactyly: thickening of skin over hands and fingers
  5. Telangiectasia: dilation of capillaries on surface of skin
30
Q

Reye Syndrome

A

Acute liver failure
Hepatomegaly
Elevated transaminases; coagulopathy
Rapidly progressive encephalopathy
Vomiting, lethargy, seizures, coma following salicylate (aspirin) administration in children.
Aspirin-induced mitochondrial dysfunction causes impaired fatty acid metabolism and microvesicular steatosis of the liver

31
Q

Familial adenomatous polyposis

A

APC gene mutation
Colorectal cancer
Desmoids & osteomas
Brain tumors

32
Q

Lynch syndrome

A

Autosomal dominant
Inherited DNA mismatch repair defect
MSH2, MLH1, MSH6, PMS2 genes
Colorectal cancer
Endometrial cancer
Ovarian cancer
Patient with postive family history are at risk should start colonscopy by age 5

33
Q

Wilsons Disease

A

AR, Chromosome 13
- Defective Copper transport protien ATP7B.
- Impaired biliary excretion of Copper accumulation of Cu in hepatocytes cause oxidative stress and apoptosis (Cirrhosis).
- Extra hepatic Cu accumulation in basal ganglia (Encephalopathy), Cornea (Kayser-Fischer Ring).

Classical Symptoms Traid:
1-Hepatic (Cirhosis, Liver failure)
2-Neurological (Confusion, Disorientation)
3-Psycological (Depression)

Parkinsonism in young patient is Wilson disease.
Putamen in brain is damaged in wilson disease.

Tx: Penicillamine ( Copper chelator )