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1
Q

What is the posterior neck mass in Turner’s syndrome composed of? What is it called?

A

Cystic hygroma, consisting of cystic spaces separated by connective tissue rich in lymphoid aggregates

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2
Q

Infants with Turner’s syndrome usually have what two manifestations due to abnormalities in lymphatic outflow?

A

Cystic hygromas

Lymphedema in the hands and feet

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3
Q

Another word for reliable is?

A

Precise

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4
Q

On what type of cells would one find P antigen (globoside)?

A

Mostly on mature erythrocytes and erythroid progenitors

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5
Q

Why does parvovirus B19 target the bone marrow?

A

Due to the high concentration of P antigen (B19 receptor) on erythrocytes and erythroblasts

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6
Q

What is the acute, flu-like, self-limiting disease of Legionella called?

A

Pontiac fever

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7
Q

What are some common exposure sources for Legionnaire’s disease?

A

Cruise ships, spas, hospitals, hotels

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8
Q

What is suggested by patchy lung infiltrates, high fever, relative bradycardia, neuro sx, GI sx, with no organisms shown on gram stain?

A

Legionnaire’s disease

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9
Q

What type of medium does Legionella grow on?

A

BCYE

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10
Q

What is the most common lab abnormality in Legionnaire’s disease?

A

HypoNa

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11
Q

What is the first line tx for myoclonic sz?

A

Valproic acid

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12
Q

What treatment is recommended for Tourette syndrome?

A

Typical and atypical neuroleptics (Haloperidol)

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13
Q

How to differentiate between a myoclonic sz and Tourette syndrome?

A

Myoclonic: brief, symmetric contractions with loss of body tone causing fall or slumping. Precipitated by stress and sleep deprivation, esp in the morning.

Tourette: Nonrhythmic, suppressible motor tics preceded by prodromal sensation.

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14
Q

What is the first line treatment for partial sz?

A

Carbamazepine

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15
Q

Prophylactic medication given to traveler to Africa causing anemia, hematuria, and Heinz bodies is indicative of what disease?

A

G6PD deficiency unmasked by anti-malarial drugs

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16
Q

Inheritance of G6PD deficiency?

A

X linked recessive

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17
Q

What class of drugs will mask the sx of hypoglycemia?

A

Non selective beta blockers (propranolol, timolol, nadolol)

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18
Q

Vascular intimal thickening in response to injury is mediated by what process?

A

Migration of medial Smooth Muscle Cells (SMC) across internal elastic lamina and into the intima

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19
Q

What are the positive waves in a JVP?

A

A wave: atrial contraction (late diastole)
C wave: tricuspid bulging into atrium (early systole)
V wave: atrial filling (late systole, early diastole)

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20
Q

What are the negative waves in a JVP?

A

X descent: relaxation of the R atrium (mid systole)

Y descent: tricuspid opens and passive filling of R ventricle (early diastole)

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21
Q

What is a normal A-a gradient?

A

10-15 mmHg

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22
Q

What is the dequence of events in base excision repair?

A

Glycosylase, endonuclease/lyase, polymerase, ligase

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23
Q

In what cases of atherosclorotic disease would ACE-I be contraindicated?

Study These Flashcards
A

Renal artery stenosis

24
Q

Mallory-Weiss tears are associated with what anatomic abnormality?

Study These Flashcards
A

Hiatal hernias

25
Thick rope-like cords of mycobacteria indicate presence of what factor? What is its significance?
Cord factor, which increases virulence by inactivating neutrophiles, damaging mitochondria, and increasing release of TNF
26
What is the most common cause of death, post-MI?
Ventricular arrhythmia
27
What antiarrhythmic do you administer for post-MI ventricular arrhythmias?
Lidocaine
28
What is the preferred treatment for ventricular tachycardia?
Amiodarone
29
What are the short acting benzodiazepines (<10 hours)? Pros/Cons?
``` Pro: less daytime drowsiness Con: more addictive - Alprazolam - Triazolam - Oxazepam "AlTO is addicting and reduces daytime drowsiness" ```
30
What are the medium duration benzodiazepines (10-20 hrs)?
Estazolam Lorazepam Temazepam
31
What are the long acting benzodiazepines (days)? Pros/cons?
``` Pro: less addictive Con: severe daytime drowsiness - Chlordiazepoxide - Clorazepate - Diazepam - Flurazepam "Chloe Feels like Dying" ```
32
What hormone level should be monitored in patients on long term Lithium therapy?
TSH (hypothyroidism is common)
33
What are some side effects of Lithium treatment?
Hypothyroidism, nephropathy, nephrogenic DI, fetal cardiac malformations (Ebstein's anomaly of the tricuspid: "atrialization of the R ventricle")
34
What pathology shows on radiography as diffuse gallbladder calcifications? Is it bad?
"Porcelain gallbladder": bluish, brittle, calcified gallbladder wall. Yes! 11-33% progress to gallbladder carcinoma
35
What type of capsule does S. pneumo have?
Polysaccharide capsule (preventing phagocytosis)
36
What toxicity is a risk from nitroprusside administration?
Cyanide!
37
What two coumpounds is nitroprusside metabolized into?
CN and NO
38
What to give for CN toxicity?
Sodium thioSULFATE (which donates sulfur to liver rhodanase, detoxifying CN to thiocyanate)
39
What liver enzyme detoxifies CN?
Rhodanase
40
What are sx of CN toxicity?
AMS and lactic acidosis
41
What are the "fatty streaks" on the inner surface of aortas in children after age 10?
Intimal lipid-filled foam cells, derived from macrophages and smooth muscle cells that engulf LDL and enter the intima through the endothelium
42
What is another name for epithelioid macrophages without central necrosis?
Noncaseating granuloma
43
Which IBD is indicated by presence of noncaseating granulomas?
Crohn's disease
44
Cobblestone appearance of the colon with skip lesions indicate what disease?
Crohn's disease
45
Where do very long chain and some branched chain fatty acids get metabolized?
In peroxisomes (not mitochondria, like other fatty acids)
46
What is a branched chain fatty acid that cannot undergo mitochondrial beta-oxidation?
Phytanic acid (defect in its peroxisomal alpha oxidation = Refsum disease)
47
How do you treat Refsum disease?
Strict avoidance of chlorophyll in the diet
48
Cushing syndrome in SLE patient is caused by what?
Iatrogenic, from exogenous glucocorticoids. This can lead to HPA suppression and bilateral adrenal atrophy
49
In what pathology would you see unilateral adrenal atrophy? Bilateral?
Unilateral atrophy: Adrenocortical adenoma suppressing contralateral adrenal Bilateral atrophy: Exogenous or extra-adrenal glucocorticoids
50
What liver pathology would be seen on acetaminophen toxicity?
Centrilobular hepatic necrosis and liver failure (treat with NAC)
51
When would you see HIGH Leukocyte Alkaline Phosphatase?
Polycythemia vera Essential thrombocytosis Primary myelofibrosis Leukomoid reaction
52
When would you see LOW Leukocyte Alkaline Phosphatase?
CML, AML, paroxysysmal nocturnal hemoglobinuria
53
What differentiates CML from Leukemoid reaction?
Leukocyte (neutrophil) alkaline phosphatase: CML: Low LR: High Similarities: Leukocytosis, few blasts
54
What differentiates AML from CML?
AML has increased blasts (>20% of total nucleated cells) Both have low neutrophil alk phos
55
What is CREST syndrome?
``` Calcinosis Raynaud Esophageal dysmotility Sclerodactyly Telangiectasias ``` Syndrome of systemic sclerosis

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