UWorld 2 Flashcards

Question

Lynch Syndrome

Answer 1

Hereditary NonPolyposis Colorectal cancer Amsterdam Criteria 1: 1) At least 3 relatives with CRC, one of whom must be a first degree relative of the other two 2) Involvement of 2 or more generations 3) At least one case diagnosed before age 50 4) Familial adenomatous polyposis has been excluded 2 subgroups 1) Hereditary site specific colon cancer (Lynch 1) 2) Cancer family syndrome (Lynch 2) - really associated with more extracolonic tumors like endometrial* carcinoma, which develops in 43% of females in affected families.

Answer 2

Epigastric pain and intermittent melena. Pain is worse on empty stomach (due to unopposed gastric acid emptying into duodenum) and improves with food (due to alkaline fluid secretion into duodenum) - this is opposite of gastric ulcer pattern Majority of DUs are caused by either H Pylori or NSAIDs. If there is no history of NSAID use look for H Pylori. Can be confirmed by endoscopic bx or urea breath test. Management of DU due to H Pylori: 1) Antisecretory therapy - preferably PPI AND 2) ABx eradication - amoxicillin plus clarithromycin

Answer 3

Predominantly conjugated chronic hyperbilirubinemia that is NOT associated with hemolysis Benign. More in Sephardic jews but can be in anyone. Clinically, icterus is evident, though the physical exam is typically otherwise normal. Most patients are asymptomatic with some complaining of nonspecific issues (fatigue, ab pain, weakness). Icterus may be so mild as to only become evident in the context of a trigger such as illness, pregnancy, or OCP use Routine labs are unremarkable, including CBC and LFTs. Bilirubin usually ranges 2-5 but can be normal or very high (20-25). Urinary coproporphyrin studies. People with DJS have 80% Copro I whereas normal people have majority Copro III. Grossly, liver is black. Histo it's normal though, though dense pigment composed of epinephrine metabolites in lysosomes can be seen. "That DJ has got some dark ass beats"

Answer 4

Conjugated hyperbilirubinemia with a direct bilirubin fraction of at least 50% and an otherwise normal liver function panel must be present. Confirmation can be obtained by evaluating urinary coproporphyrin for unusually high Copro I No tx required and prognosis is great

Answer 5

Benign condition in which there is a defect of hepatic storage of conjugated bilirubin, resulting in its leakage into the plasma Chronic and mild hyperbilirubinemia of both conjugated and unconjugated forms then develops, without any sign of hemolysis Liver function tests are normal. Pigmented granules are not seen in hepatocytes

Answer 6

Occurs in patients with no apparent liver disease who have mild unconjugated hyperbilirubinemia thought to be provoked by one of the classic triggers (fasting, stress, illness)

Answer 7

Type 1 is more severe and often results in permanent neurologic impairment or death. Very rare, autosomal recessive. Significant unconjugated hyperbilirubinemia. Phototherapy or plasmapharesis are usually helpful in short term, but liver transplant is only curative option

Answer 8

1) Change in bowel habits 2) Abdominal pain 3) Weight loss 4) Fe deficiency anemia 5) FHx of CRC

Answer 9

Associated with chronic constipation. Usually ASx but potential complications include hemorrhage and diverticulitis. Risk of complications is lower with high intake of fruit and vegetable fiber and higher with heavy meat consumption, aspirin, or NSAID use, obesity, and possibly smoking

Answer 10

Always suspect Crohn Disease

Answer 11

1) Alcohol 2) Gallstone 3) Hypertriglyceridemia 4) Meds (Azathioprine, valproic acid, thiazides) 5) Infections (CMV) 6) Recent ERCP 7) Trauma

Answer 12

Over 1000. Look for eruptive xanthomas (yellow-red papules on arms and shoulders). These are due to subQ fat deposition and are associated with hypertriglyceridemia. This high of a level though usually is due to familial hyperTG. So look for a younger patient or FHx of an early cardiac death. Get a fasting lipid profile to check out this cause of pancreatitis.

Answer 13

For biliary pancreatitis. These patients may have normal US. They usually have ALT above 150. Also consider ERCP if patient has had more than 1 episode of acute pancreatitis of unknown cause

Answer 14

100% without elective proctocolectomy

Answer 15

1) Viral hepatitis (HSV, CMV, Hep A, B, D, E) 2) Drug tox - acetaminophen OD and idiosyncratic 3) Ischemia - shock liver, Budd Chiari 4) Autoimmune hepatitis 5) Wilson Disease 6) Malignant infiltration

Answer 16

1) Generalized symptoms (fatigue, lethargy, anorexia, nausea) 2) RUQ abdominal pain 3) Pruritus and jaundice due to hyperbilirubinemia 4) Renal insufficiency 5) Thrombocytopenia 6) Hypoglycemia

Answer 17

1) Severe acute liver injury (ALT and AST often above 1000) - often 10x normal 2) Signs of hepatic encephalopathy (confusion, asterixis) 3) Synthetic liver dysfunction (INR at least 1.5)

Answer 18

PT Patients who improve typically have decreasing transaminases, bilirubin and PT/INR BUT, patients with rapidly worsening ALF can also have decreasing transaminases but with WORSENING PT/INR Worsening PT/INR and bilirubin suggest worsening synthetic function.

Answer 19

Acute onset of severe liver injury with encephalopathy and impaired synthetic function in a patient without cirrhosis or underlying liver disease

Answer 20

Decreased synthesis of serum binding proteins for triiodothyronine (T3) and thyroxine (T4) such as thyroxine binding globulin, albumin and lipoproteins. This lowers the TOTAL T3 and T4. Free T3 and T4 is usually unchanged as nearly all T3 and T4 is bound to the serum binding proteins.

Answer 21

May present with fatigue, weakness, anorexia and weight loss. Androgen production occurs in adrenal glands in women. who can develop decreased axillary and pubic hair and loss of libido Men lack these findings bc androgen production is in the testes.

Answer 22

Absence of food buffer (2-5h after meals, on an empty stomach or at night)

Answer 23

Melena (upper GI bleed above ligament of Treitz)

Answer 24

One of the most dangerous complications of end stage liver disease. Occurs in up to 10% of patients with cirrhosis. Characterized by decreased glomerular filtration in absence of shock, proteinuria, or other clear cause of renal dysfunction, and a failure to respond to a 1.5L normal saline bolus Thought to be from renal vasoconstriction in response to decreased total renal blood flow and vasodilatory substance synthesis. Type 1 - rapidly progressive. Most patients die within 10w without treatment Type 2 - More slow. Average survival 3-6 months. Most common causes of death are infection and hemorrhage. No med is consistently helpful. Mortality for these patients placed on dialysis is very high. Liver transplant is only intervention with established benefit. Kidneys of HRS patients are essentially normal on bx and renal function improves with liver transplant.

Answer 25

RUQ US helps distinguish intrahepatic (no biliary tract dilation) from extrahepatic (biliary tract dilation like due to gallstones) cholestasis. If US suggests intrahepatic process the next step is to get serum anti-mitochondrial antibody titers which have high sensitivity for primary biliary cholangitis/cirrhosis (same thing, new name)

Answer 26

chronic liver disease characterized by autoimmune destruction of the intrahepatic bile ducts with resulting cholestasis. presents most commonly in middle aged women and is insidious in onset. As disease progresses, jaundice, hepatomegaly, high alk phos, steatorrhea, and portal HTN may develop. Additional complications can include severe hyperlipidemia (with xanthelasma) and metabolic bone disease. PBC is often associated with other autoimmune diseases like thyroid diseases.

Answer 27

Associated with elevated titers of ANA and anti-smooth muscle antibodies. Characterized by fluctuating hepatocellular injury (elevated transaminases) rather than cholestasis First line treatment is steroids

Answer 28

Drug induced pancreatitis makes up 5% of acute pancreatitis. 1) Anti-seizure drugs (Valproic acid) 2) Drugs for IBD (sulfasalazine, 5-ASA) 3) Immunosuppressive agents (Azathioprine) 4) HIV related meds (didanosine, pentamidine) 5) ABx (metronidazole, tetracycline) 6) Diuretics (Furosemide, thiazides)

Answer 29

Usually mild. N/V, and abd pain radiating to back. Labs show elevated serum amylase and lipase. CT shows swelling of the pancreas with prominent peripancreatic fluid and fat stranding. Supportive treatment with fluids and lyte replacement is recommended.

Answer 30

Supportive care (nothing by mouth, IV ABx, analgesics) Lap Chole is recommended shortly after hospitalization and should be performed immediately in cases of perforation or gangrene.

Answer 31

The first serologic marker detected in the serum after inoculation. It precedes the onset of clinical symptoms and elevation of serum aminotransferases. Also remains detectable during entire symptomatic phase of acute hep B and suggests infectivity

Answer 32

Appears in serum after either successful HBV vaccination or clearance of HBsAg, and remains detectable for life in most patients. Indicates non-infectivity and immunity. However, there is a time lag btw the disappearance of HBsAg and the appearance of anti-HBs called the window period.

Answer 33

This marker is not detectable in serum as it is normally sequestered within the HBsAg coat

Answer 34

IgM appears shortly after the emergence of HBsAg and may be the only diagnostic marker for acute HBV infection during the window period. IgG remains detectable during recovery from acute HBV infection or progression to chronic infection

Answer 35

This antigen is detectable shortly after the appearance of HBsAg and indicates active viral replication/infectivity. It is associated with presence of HBV DNA. HBeAg tends to disappear shortly after the aminotransferase levels peak and before HBsAg is eliminated. It is followed by appearance of anti-HBe

Answer 36

Marker suggests cessation of active viral replication and low infectivity

Answer 37

1) HBsAg positive 2) HBeAg positive 3) IgM anti-HBc positive 4) HBV DNA really positive

Answer 38

1) IgM anti-HBc positive | 2) HBV DNA positive

Answer 39

1) IgG anti-HBc positive 2) Anti-HBs positive 3) Anti-HBe positive 4) HBV DNA likely positive

Answer 40

1) HBsAg positive | 2) IgG Anti-HBc positive

Answer 41

1) HBsAg positive 2) HBeAg likely positive 3) IgM Anti-HBc positive 4) IgG Anti-HBc positive 5) HBV DNA positive Only scenario where both IgM and IgG for HBc are positive

Answer 42

Anti-HBs positive

Answer 43

1) IgG anti-HBc positive | 2) Anti-HBs positive

Answer 44

Reflects buildup of conjugated bilirubin Note: Positive urine urobilinogen assay reflects buildup of unconjugated bilirubin (hemolysis for example)

Answer 45

Conjugated bili (loosely bound to albumin - unconjugated is tightly bound) is water soluble so it can be excreted in urine. Normally bili doesnt make it to the urine though bc the intestines usually degrades it all. If there is hepatic dysfunction though, or biliary obstruction or a defect in hepatic bilirubin secretion there is plasma buildup of conjugated bilirubin which leaks into the urine, making it dark and causing a positive urine bilirubin assay

Answer 46

Risk factors 1) Smoking 2) Hereditary pancreatitis 3) Nonhereditary chronic pancreatitis 4) Obesity and lack of physical activity Clinical presentation 1) Systemic symptoms (weight loss, anorexia) 2) Abdominal pain/back pain (80%) 3) Jaundice (56%) 4) Recent onset atypical diabetes mellitus 5) Unexplained migratory superficial thrombophlebitis* 6) Hepatomegaly and ascites with mets Labs 1) Cholestasis (increased alk phos and direct bilirubin) 2) Increased CA 19-9 (not as screening tho) 3) Abdominal US (if jaundice) or CT (if no jaundice)

Answer 47

1) Cancers of the head of the pancreas (60-70%) typically present with jaundice (common bile duct obstruction, elevated alk phos and bilirubin) and steatorrhea (pancreatic exocrine insufficiency or pancreatic duct blockage). In patients with these findings, US is preferred for detecting pancreatic head tumors excluding other potential causes of biliary obstruction (choledocholithiasis) 2) Cancers in the body and tail usually do not present with obstructive jaundice. Abdominal CT scan is preferred (more sensitive and specific) and helps exclude other conditions. US is less sensitive for visualizing the pancreatic body and tail due to overlaying bowel gas and for detecting smaller tumors (less than 3cm)

Answer 48

Intubate! protect airway as part of initial stabilization and resuscitation.

Answer 49

1) Positive hydrogen breath test - rise in measured breath H after ingestion of lactose, thus indicating bacterial carb metabolism 2) Positive stool test for reducing substances 3) Low stool pH - due to fermentation products 4) Increased stool osmotic gap No steatorrhea

Answer 50

Osmotic gap is 290 - 2(stool Na plus stool K). Greater than 50 in all forms of osmotic diarrhea, including lactose intolerance

Answer 51

Acute inflammation of gallbladder without gallstones most commonly seen in hospitalized and severely ill patients Risk factors 1) Severe trauma, extensive burns, recent surgery (cardiopulmonary, aortic, abdominal) 2) Prolonged fasting or TPN 3) Critical illness (sepsis, ICU, mechanical vent) Clinical presentation 1) Unexplained fever, vague/RUQ ab discomfort, leukocytosis 2) Possible jaundice, RUQ mass, abnormal LFTs Dx 1) Ab US (preferred) 2) Cholescintigraphy (HIDA) or abdominal CT if US is not diagnostic

Answer 52

Keep acalculous cholecystitis in mind.