Step 1a Flashcards
What is mycophenolate mofetil?
Drug used to treat CF & prophylaxis of organ rejection.
MOA: Converted to active metabolite mycophenolic acid (selective inhibitor of inosine monosphosphate dehydrogenase [IMPDH]) –> inhibits denovo synthesis of guanine nucleotide –> (blockage of purine synthesis) –> inability for lymphocyte proliferation
Common side effects: HTN, infection, peripheral edema, hypercholestermia
Serious side effects: thrombocytopenia, leukopenia, neutropenia, immunosuppresion –> opportunistic infections
Tuberous sclerosis, what is it?
AD neurocutaneous disorder caused by mutations to TSC1 (hamartin) & TSC2 (tuberin) genes, which are TSGs.
This disease affects cellular differentiation, proliferation, and migration during development –> hamartomatous lesions affecting nearly every organ system.
Findings include:
- Cortical hamartomas known as tubers
- Cutaneous findings: facial angiofibromas (adenoma sebaceum), shagreen patches (leathery skin patches), “ash-leaf” spots (hypopigmented patches on the skin)
- Cardiac rhabdomyomas (which can cause arrhythmias, syncope, and sudden death)
- Seizures
- Mental retardation
- Retinal hamartomas (phakomas)
- Renal angiomyolipomas
- Pulmonary hamartomas
- Astrocytomas
Patent ductus arteriosus
Connects the left pulmonary artery to the aortic arch.
Closes shortly after birth usually under the influence of increased O2 tension, release of bradykinin, and decreased PGE levels.
Characterized by a “machinery” murmur.
Streptokinase drug, what it do?
Causes thrombolysis through formation of an activator complex with plasminogen (bound and free) leading to the release of plasmin.
Plasmin degrades fibrin and fibrinogen as well as other plasma proteins.
CML and the Philadelphia chromosome
Philly chromosome = t(9;22) –> results in BCR-ABL fusion gene –> constitutively activates tyrosine kinase –> development of CML
Findings: increased mature neutrophils, immature netrophils (metamyelocytes), and basophilia [granulocytes]
Tx: imatinib –> TK inhibitor
Visual field stuff
http://puu.sh/nTMJx/0329b741c0.jpg
Upper quadrantanopia –> temporal lobe (Meyer’s loop)
Lower quadrantanopia –> parietal lobe
Colonic polyp types
Hyperplastic –> sawtooth glandular epithelium with proliferation of goblet and columnar epithelial cells (no atypia) - no progression to malignancy
Tubular adenoma –> pedunculated - 4% progress to malignancy
Tubulovillous adenoma –> combines both - intermediate level of malignancy
Villous –> sessile - 30% progress to malignancy
Congestive heart failure signs
Holosystolic ejection murmur best heard over the apex –> mitral valve regurgitation
Hemosiderin-laden macrophages in the lungs –> heart-failure cells due to increased pulmonary capillary pressure causing erythrocytes to escape in the alveoli & being phagocytosed by macrophages
Adenovirus information
Non-enveloped (naked), linear, icosahedral, naked double-stranded DNA virus
Clinical syndromes: ARD, pharyngoconjunctivitis, epidemic keratoconjunctivitis, gastroenteritis
Commonly spread by contamined swimming pools
Pineal gland tumors (germinoma)
Compress the verticle gaze center in the tectum of the midbrain
- -> can result in Parinaud syndrome:
- eye movement & pupil dysfunction
- vertical gaze palsy & light-near disocciation
Aortic regurgitation murmur
Widened arterial pulse pressure (>100 mm Hg often)
Diastolic murmur –> high pitched, blowing, decrescendo murmur heard best along the lower left sternal border
Large stroke volume
Aortic stenosis
Auscultation: crescendo-decrescendo systolic murmur
In elderly: most common cause of aortic stenosis is calcification
Aortic stenosis increases afterload, causing left ventricular hypertrophy
Adult (autosomal domination) polycystic kidney disease (ADPKD)
Characteristics:
- AD inheritance of chromosome 16 –> mutations in PKD1 & PKD2 –> encode polycystins
- Present (usually 4th decade of life) with renal insufficieny, hematuria, flank pain, and HTN
- kidney develops marked enlargement bilaterally with large cysts bulging through the surface (autosomal recessive form dose not distort overlaying renal capsule)
Wiskott-Aldrich syndrome
Characteristics:
- X-linked, partial combined immunodeficiency disorder
- decreased serum IgM
- elevated IgA/IgE
- Prone to devloping non-Hodgkin lymphoma
Classic triad:
1) thrombocytopenic purpura
2) eczema
3) recurrent opportunistic infections of encapsulated organisms
Aspirin-induced asthma
MOA: inhibits cox pathway without affecting the lipoxygenase pathway –> leads to decreased ratio of PGs (bronchodilators) to leukotrienes (bronchoconstrictors)
