Thrombosis or compression of hepatic veins with centrilobular congestion and necrosis congestive liver disease (hepatomegaly, ascites, varices, abdominal pain, liver failure). Absence of JVD. Associated with hypercoagulable states, polycythemia vera, postpartum state, HCC. May cause nutmeg liver (mottled appearance). Clinical Presentation: Abdominal pain, ascites, hepatomegaly Lab Diagnostic/Finding: “Nutmeg” appearance of liver (because of chronic passive congestion of liver due to right heart failure or Budd-Chiari syndrome)  

Clinical Presentation: Abdominal pain, diarrhea, leukocytosis, recent antibiotic use Common Treatments: Oral metronidazole; if refractory, oral vancomycin Spore-forming bacteria (Page 127) Some bacteria can form spores at the end of the stationary phase when nutrients are limited. Spores are highly resistant to heat and chemicals. Have dipicolinic acid in their core. Have no metabolic activity. Must autoclave to potentially kill spores (as is done to surgical equipment) by steaming at 121°C for 15 minutes. Nosocomial Infections (Page 181) E coli (UTI) and S aureus (wound infection) are the two most common causes. Risk Factor: Antibiotic use Unique Signs/Symptoms: Watery diarrhea, leukocytosis Vancomycin (Page 186)  Mechanism: Inhibits cell wall peptidoglycan formation by binding D-ala D-ala portion of cell wall precursors. Bactericidal against most bacteria (bacteriostatic against C difficile). Not susceptible to β-lactamases.  Clinical Use: Gram ⊕ bugs only—serious, multidrug-resistant organisms, including MRSA, S epidermidis, sensitive Enterococcus species, and Clostridium difficile (oral dose for pseudomembranous colitis). Adverse Effects: Well tolerated in general—but NOT trouble free. Nephrotoxicity, Ototoxicity, Thrombophlebitis, diffuse flushing—red man syndrome (largely preventable by pretreatment with antihistamines and slow infusion rate).

Familial hypercholesterolemia (Decreased LDL receptor signaling) Clinical Presentation: Achilles tendon xanthoma Autosomal Dominant Diseases (Page 56) Familial Dyslipidemias (Page 90)  Type II - Familial hypercholesterolemia; Inheritance: AD; Pathogenesis: Absent or defective LDL receptors   High Blood Level: IIa: LDL, cholesterol IIb: LDL, cholesterol,VLDL   Clinical: Heterozygotes (1:500) have cholesterol ≈ 300mg/dL; homozygotes (very rare) have cholesterol ≈ 700+ mg/dL. Accelerated atherosclerosis (may have MI before age 20), tendon (Achilles) xanthomas, and corneal arcus. Hyperlipidemia Signs (Page 291)  Tendinous xanthoma: Lipid deposit in tendon, especially Achilles. 

Waterhouse-Friderichsen syndrome—acute 1° adrenal insufficiency due to adrenal hemorrhage associated with septicemia (usually Neisseria meningitidis), DIC, endotoxic shock. (Page 324) Classic Presentation: Adrenal hemorrhage, hypotension, DIC Neisseria / Meningococci (Page 138) Meningococci: Polysaccharide capsule. Maltose fermentation.  Vaccine (type B vaccine not widely available).  Transmitted via respiratory and oral secretions. causes meningococcemia with petechial hemorrhages and gangrene of toes B , meningitis, Waterhouse-Friderichsen syndrome (adrenal insufficiency, fever, DIC, shock). Rifampin, ciprofloxacin, or ceftriaxone prophylaxis in close contacts. Treatment: ceftriaxone or penicillin G.  Primary Adrenal Insufficiency (Page 324)  Deficiency of aldosterone and cortisol production due to loss of gland function leading to hypotension (hyponatremic volume contraction), hyperkalemia, metabolic acidosis, skin and mucosal hyperpigmentation (due to increased MSH, a byproduct of ACTH production from proopiomelanocortin [POMC]). Acute—sudden onset (eg, due to massive hemorrhage). May present with shock in acute adrenal crisis.      

Clinical Presentation: Anaphylaxis following blood transfusion Selective IgA deficiency (Page 112)  Defect: Unknown. Most common 1° immunodeficiency.  Presentation: Majority Asymptomatic. Can see Airway and GI infections, Autoimmune disease, Atopy, Anaphylaxis to IgA-containing products. Findings: Decreased IgA with normal IgG, IgM levels. Increased susceptibility to giardiasis.  Ataxia-telangiectasia (Page 113)  Defect: Defects in ATM gene lead to failure to repair DNA double strand breaks leads to cell cycle arrest.  Presentation: Triad - cerebellar defects (Ataxia), spider Angiomas (telangiectasia A ), IgA deficiency.  

Clinical Presentation: Anterior "drawer sign" + Knee Exam (Page 424) ACL: extends from lateral femoral condyle to anterior tibia. Test: Anterior drawer sign; Procedure: Bending knee at 90° angle, increased anterior gliding of tibia due to ACL injury. Lachman test is similar, but at 30° angle. Common Knee Conditions (Page 425) Unhappy Triad: Common injury in contact sports due to lateral force applied to a planted leg. Classically, consists of damage to the ACL , MCL, and medial meniscus (attached to MCL); however, lateral meniscus injury is more common. Presents with acute knee pain and signs of joint injury/instability.

Clinical Presentation: Arachnodactyly, lens dislocation (upward), aortic dissection, hyperflexible joints Disease/Finding: