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1
Q

Hirshprung Disease

A

absence of ganglia that regulate peristalsis

problem w/ neural crest cell migration

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2
Q

Spore-forming bacteria

A

Bacillus anthracis & cereus, clostridium perfringens, botulinum & tetani, & Coxiella burnetti

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3
Q

Drugs that INDUCE cytochrome P450

A

=> decrease effectiveness of the metabolized drug

  1. Phenobarbitol/all barbiturates
  2. Rifampin
  3. Carbemazepine
  4. Phenytoin
  5. Griseofulvin *also: chronic EtOH abuse
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4
Q

Drugs that INHIBIT cytochrome P450

A

=> decreased metabolism of cyp450 dependent drugs

  1. Cimetadine
  2. Fluoxetine
  3. Antibiotics: Isoniazid, Erythromycin
  4. Antifungal/virals: Ketoconazole, Ritonavir
  5. Grapefruit juice
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5
Q

Case:
HIV + patient, with diffuse neuro Sxs (headache, stiff neck, fever…).
What is the DDx (& assoc. treatment)?

A

1. Toxoplasmosis (w/ ring-enhancing lesions on MRI)

    Tx: pyrimethamine & sulfasiazine #2. Primary CNS Lymphoma (B cell infiltrate & EBV DNA in CSF) #3. Cryptococcus (diffuse)
    Tx: Amphotericin B
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6
Q

Bacteria that causes infective endocarditis and is associated with colon cancer

A

Streptococcus BOVIS

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7
Q

Case:
Patient presents w/ mitral regurgitation and history of chorea in childhood.
Cause?

A

Strep pyogenes => Rheumatic fever!
= molecular mimicry problem after resolved strep infection.
Acutely: fever, chorea
Chronic/long-term: mitral regurgitation OR mitral stenosis

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8
Q

Causes of Renal Papillary Necrosis

A

(Sx = gross hematuria, w/ a predisposing factor below)

  1. Sickle cell disease OR trait
  2. Diabetes
  3. Severe obstructive pyelonephritis
  4. Analgesic nephropathy (ie: NSAIDs)
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9
Q

Bacillary Angiomatosis vs. Kaposi’s Sarcoma

A

Bacillary angiomatosis: benign, capillary skin papules.
- w/ AIDS, from bartonella.
Kaposi’s Sarcoma: malignant, endothelial on skin OR mouth/GI/Resp.
- w/ HHV-8 & HIV

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10
Q

Sturge Weber disease

A

Congenital disease of capillaries.

  • “port wine stain” on face
  • ipsilateral leptomeningeal angiomatosis => intracerebral AVM
  • seizures, early glaucoma
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11
Q

“Monday Disease” for Nitroglycerin exposure

A

develop tolerance during week of exposure (less vasodilation), but LOSE tolerance over weekend
=> tachycardia, dizziness, headache w/ re-exposure

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12
Q 
Meds for lowering lipids
(class name, biggest effect, major SEs)
A

All decrease LDL.
#1. Statins: (–l HMG CoA reductase) decrease LDL *rhabdomyolysis
2. Niacin: increase HDL, *flushing, hyperglycemia/urate
3. Bile acid resins: block bile reabs. *gallstones
4. Ezetimibe: block cholesterol reabs.
5. Fibrates: upreg. LPL = decrease TGs! *myositis, LFTs up, gallstones

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13
Q

Niacin Deficiency

A

“3 Ds” –> Diarrhea, Dementia, Dermatitis
= Vit B3 def., common in EtOH abuse
* needed as coenzyme for NAD & NADPH

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14
Q

Kussmaul Sign

A

= paradoxical RISE in JVP during inspiration,
indicates Constrictive Pericarditis
(also likely to hear “pericardial knock” later than S3)

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15
Q

Signs and causes of Infectious Arthritis

A

“STD”
S- Synovitis, T- tenosynovitis, D- Dermatitis
*often migratory, asymmetric Sxs!
Causes: S. aureus, Strep, Gonorrhea (“gonococcal arthritis”)

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16
Q

Potential causes of avascular necrosis/osteonecrosis

A
  1. trauma
  2. high-dose corticosteroids
  3. alcoholism
  4. sickle cell disease
    * usually @ femoral head.
17
Q

Disease associated with Elevated ACE serum levels

A

Sarcoidosis!

wide-spread, immune-mediated non-caseating granulomas

18
Q

Secondary causes of hypoxia => clubbing

A
  1. Lung disease (esp. supurative): lung cancer, TB, CF, bronchiectasis, pulm. HTN, empyema, etc.
  2. Heart disease: tetralogy of fallot/other congen. heart defects, bacterial endocarditis
  3. Other: IBD (crohns/Ulc. colitis), hypERthyroidism, malabsorption
19
Q

1 most noxious toxin of Clostridium perfringens

A

Lecithinase, aka: “Alpha toxin” or Phospholipase C
-> splits phospholipid molecs
=> cell lysis & RBC hemolysis, edema, tissue necrosis

20
Q

Metabolism mech for drugs associated with drug-induced lupus

A

(example drugs: hydralazine, procainamide)
N-Acetylation by the Liver (phase II)
*slow acetylators have greatest risk of developing drug-induced lupus.
*anti-histone Ab = most specific for drug-induced Lupus

21
Q

Triad of Sxs/complications in Congenital Rubella infection

A
  1. “white pupils” (= congenital cataracts)
  2. Sensorineural hearing loss
  3. PDA/congen. heart defects
    *prevent by vaccinating NON-pregnant women of child-bearing age!
    (w/ live attenuated vaccine)
22
Q

toxins of Pseudomonas aeruginosa

A
  • gram + rod => “ecthyma gangrenosum” (purple cut. lesions, esp. if lacking humeral immunity)
    1. Exotoxin A –l protein synthesis
    2. Elastase –> blood vessel destruction
    3. Phospholipase C –> degrades cell membranes
    4. pyocyanin: makes ROS
23
Q

Odds Ratio vs. Relative Risk calculations

A

Odds ratio: (#Tx & disease/#Tx & no disease)/(#disease and NO Tx/#no Tx & no disease) = “(AxD/BxC)
aka: (%Tx w/ outcome)/(%not Tx w/ outcome)

Relative risk: (#Tx & outcome/all Tx)/(#noTx & outcome/all NOT Tx)

24
Q

mycobacterium avium

A

Mycobacterium (not TB),
causes disease in healthy OR immunosuppressed pts.
Sxs: hepatosplenomegaly & elevated AlkPhos, fever, weight loss, diarrhea… *clear chest Xray (vs. granulomas if TB)
**grows best at 41C! Prophylactic Tx w/ azithromycin (esp. if CD4 <50)

25
Q

Amino acids with 3 titratable protons

=> 3 points distinct points on titration

A

(7) Histidine, Lysine, Arginine,
Aspartic acid, Glutamic acid,
Cysteine, tyrosine.

26
Q

Fetal development timeline & landmarks

week 1, 2, …

A

wk 1: hCG secretion begins (can confirm on urine test)
wk 2: BiLaminar disc (epiblast/hypoblast)
wk 3: TriLaminar/gastrulation (endo/meso/ectoderm)
wk 4: 4 limb buds, heart beats
wk 8: fetal mvmt, wk 10: genitalia formed (complete @ wk 12)

27
Q

Fetal “triple test” components and significance

A
  1. AFP: increases w/ # wks => likely dating error if abnormal
  2. hCG: high => twins, hydatidiform mole, or choriocarcinoma
  3. Estriol: low = placental insufficiency
28
Q

Major Tissues that originate from surface ectoderm (5)

A
  1. Epidermis
  2. Ant. Pituitary (Adenohypophysis)
  3. Parotid, sweat, & mammary glands
  4. sensory organs of ear
  5. epithelial lining of mouth
29
Q

Major tissues that originate from the Neuroectoderm (3)

A
  1. Brain/CNS (includes spinal cord)
  2. Retina & optic nerve
  3. pineal gland
30
Q

Major tissues originating from Neural Crest (6)

A
  1. PNS
  2. Melanocytes
  3. Chromaffin cells of adrenal medulla
  4. Parafollicular (“C”) cells of thyroid
  5. skull bones, 6. Aorticopulmonary septum
31
Q

Major tissues that originate from the Mesoderm (6)

A
  1. Muscle *includes heart!
  2. bone
  3. CT & dermis
  4. serous lining of body cavities (peritoneum, spleen)
  5. Blood & lymphatics
  6. Testes/ovaries
32
Q

Major tissues derived from Endoderm (~2)

A
  1. gut epithelium

2. “Lumenal epithelium” => Lungs, Liver, gallbladder, pancreas, paraT, thymus…

33
Q

Deformation vs. Malformation

A

Deformation = EXtrinsic disruption of dvpt, AFTER embryonic period
vs.
Malformation = INtrinsic disruption, DURING embryonic period

34
Q

“Fetal Hydantoin Syndrome”

A

= results of phenytoin (teratogen).
=> microcephaly & dysmorphic craniofacial features, hypoplastic fingernails & distal phalanges, AND cardiac defects, mental retardation
(*similar to carbemazepine effects)

35
Q

Effects on Fetus of Nicotine vs. Cocaine vs. EtOH

…vs. Diabetes vs. Vitamin A excess

A

Nicotine: IUGR (small), placenta problems, ADHD, preterm labor
Cocaine: fetal addiction, placental abruption
EtOH: major birth defects & mental retardation (FAS)
*Diabetes: caudal regression, heart & neural tube defects
* VitA: cleft palate & heart defects

36
Q

Reyes Syndrome

A

mitochondrial damage in Children given ASPIRIN for viral infections.
=> fatty liver, hypoglycemia, vomiting, hepatomegaly, coma

*beta oxidation blocked by revers. inhibition of mitochondrial enzymes

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