First Aid

Question 1

Ehlers-Danlos syndrome is typically caused by a defect in what collagen type?

What about the vascular subtype?

Answer 1

V; III

Question 2

McCune-Albright syndrome is caused by a _____-activating mutation.

Answer 2

McCune-Albright syndrome is caused by a G_{<strong>s</strong>}-activating mutation.

Question 3

A patient goes into anaphylaxis following a blood transfusion. What do you suspect?

Answer 3

IgA deficiency

Question 4

Job syndrome (hyper-____ syndrome) is characterized by cold abscesses, eczema, eosinophilia, and defects in ____________ chemotaxis.

Answer 4

Job syndrome (hyper-IgE syndrome) is characterized by cold abscesses, eczema, eosinophilia, and defects in neutrophil chemotaxis.

Question 5

A patient presents with chest pain, ST depressions, and elevated troponins. What is the diagnosis?

Answer 5

NSTEMI

Question 6

A patient presents with chest pain, ST depressions, and no increase in troponins. What is the diagnosis?

Answer 6

Angina

Question 7

Osler nodes are caused by ____________, and Janeway lesions are caused by ____________.

Answer 7

Osler nodes are caused by immune complexes, and Janeway lesions are caused by septic emboli.

Question 8

Painless jaundice is associated with what?

Answer 8

Pancreatic malignancy

Question 9

A patient presents with jaundice and a palpable / distended / non-tender gallbladder.

What do you suspect?

Answer 9

Distal malignant obstruction (e.g., gallbladder adenocarcinoma)

(Courvoisier sign)

Question 10

A patient presents with arthralgias, adenopathy, cardiac and neurological symptoms, and diarrhea.

What do you suspect?

Answer 10

Whipple disease (Tropheryma whipplei)

Question 11

Severe jaundice in a neonate is likely the result of what?

Answer 11

Crigler-Najjar syndrome

(congenital, unconjugated hyperbilirubinemia)

Question 12

A patient presents with short stature, café-au-lait spots, and thumb/radial defects.

The patient is now presenting with S/Sy of leukemia.

What do you suspect?

Answer 12

Fanconi anemia

(genetic loss of DNA crosslink repair; often progresses to AML)

Question 13

What is the name of a cutaneous T-cell lymphoma characterized by microabscesses and skin patches with atypical T cells?

Answer 13

Mycosis fungoides

Question 14

What syndrome is characterized by mycosis fungoides plus malignant T cells in the blood?

Answer 14

Sézary syndrome

Question 15

What are the S/Sy of Klüver-Bucy syndrome?

Answer 15

Hyperphagia

Hypersexuality

Hyperorality

Question 16

What is the name of the benign vascular mark that is sometimes associated with Sturge-Weber syndrome?

Answer 16

Nevus flammeus

Question 17

What are the S/Sy of Meniere disease?

Answer 17

Episodic vertigo

Tinnitus

Hearing loss

Question 18

A patient presents with polyuria, renal tubular acidosis (type II), growth failure, and hypophosphatemic ricketts.

What do you suspect?

Answer 18

Fanconi syndrome

Question 19

Describe the differences between renal tubular acidoses types I, II, and IV.

Answer 19

Question 20

Which congenital immunodeficiency is characterized by large granules in phagocytes?

Answer 20

Chédiak-Higashi disease

(impaired phagolysosome formation)

Question 21

Where are Negri bodies found in patients with rabies?

Answer 21

The cytoplasm of hippocampal and cerebellar neurons

Question 22

Which tumors are associated with psammoma bodies?

Answer 22

Papillary thyroid carcinomas

Papillary serous carcinomas of the endometrium or ovary

Meningiomas

Mesotheliomas

Question 23

What disease can result from granulomatous heart infections?

Answer 23

Rheumatic fever

Question 24

A patient presents with alternating amplitude on ECG (electrical alternans).

What do you suspect?

Answer 24

Cardiac tamponade

Question 25

Wilson disease is associated with _____ serum ceruloplasmin.

Answer 25

Wilson disease is associated with **_low_** serum ceruloplasmin.

Question 26

What is the key factor causing idiopathic thrombocytopenic purpura?

Answer 26

Antiplatelet antibodies

Question 27

Which diseases are associated with MPO-ANCA / p-ANCA?

Answer 27

Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis Primary sclerosing cholangitis

Question 28

Which diseases are associated with PR3-ANCA / c-ANCA?

Answer 28

Granulomatosis with polyangitis

Question 29

What is the typical outcome/etiology of monoclonal gammopathy of undetermined significance?

Answer 29

Consequence of aging

Question 30

'Smudged' white blood cells in an older individual are an indication of what?

Answer 30

**CLL** | (typically B cell)

Question 31

A 'soap bubble' lesion seen in an X-ray of the tibia or femur might be an indication of what?

Answer 31

A giant cell tumor (osteoclastoma) | (typically benign)

Question 32

An 'onion skin' periosteal reaction might be an indication of what?

Answer 32

Ewing sarcoma

Question 33

Pseudogout calcium pyrophosphate crystals are _______ birefringent and \_\_\_\_\_\_\_\_\_-shaped.

Answer 33

Pseudogout calcium pyrophosphate crystals are **_positively_** birefringent and **_rhomboid_**-shaped.

Question 34

Which antibodies are indicators of SLE?

Answer 34

Antinuclear antibodies | (anti-Smith, anti-dsDNA)

Question 35

Which antibodies are indicators of drug-induced SLE?

Answer 35

Anti-histone

Question 36

What mnemonic is useful in remembering the drugs associated with drug-induced SLE?

Answer 36

It isn't **HIPP** to do drugs. **H**ydralazine, **i**soniazid, **p**henytoin, **p**rocainamide

Question 37

Which antibodies are indicators of diffuse scleroderma?

Answer 37

Anti-topoisomerase

Question 38

What nephropathy appears as 'tram-track' lesions on light microscopy?

Answer 38

Membranoproliferative glomerulonephritis

Question 39

What nephropathy appears as 'wire loop' lesions on light microscopy?

Answer 39

Diffuse proliferative glomerulonephritis (lupus)

Question 40

What tumor type is a glomerulus-like structure with vessels and germ cells?

Answer 40

Yolk sac tumor (Schiller-Duval bodies)

Question 41

What medication type is used for treating both hepatitis B and C?

Answer 41

IFN-alpha

Question 42

Homer-Wright rosettes (circular grouping of dark cells around pale neurofibrils) are seen in what malignancies?

Answer 42

Neuroblastomas Medulloblastomas

Question 43

What medications are used in treating hepatitis C?

Answer 43

Ribavirin Simeprevir Sofosbuvir (-**vir-**)

Question 44

How are *Toxoplasma gondii* infections treated?

Answer 44

Sulfadiazine + pyrimethamine

Question 45

What drugs are used to treat influenza?

Answer 45

Oseltamivir; zanamivir | (**neuraminidase** **inhibitors**)

Question 46

What is the best medication category for treating isolated hypertriglyceridemia?

Answer 46

Fibrates

Question 47

How is nephrogenic diabetes insipidus managed?

Answer 47

Hydrochlorothiazide Indomethacin Amiloride

Question 48

How is SIADH managed?

Answer 48

Water restriction IV hypertonic saline Demeclocycline Tolvaptan, conivaptan

Question 49

How is diabetic ketoacidosis managed?

Answer 49

Fluids, insulin, K⁺

Question 50

Is mesalamine (5-ASA preparation) used to treat ulcerative colitis or Crohn disease?

Answer 50

Ulcerative colitis

Question 51

How are the positive symptoms of schizophrenia managed? And the negative symptoms?

Answer 51

Typical or atypical antipsychotics Atypical antipsychotics

Question 52

What medications are used to treat idiopathic pulmonary arterial hypertension?

Answer 52

Bosentan Sildenafil Epoprostenol

Question 53

What is the most common helminthic infection in the U.S.?

Answer 53

*Enterobius vermicularis*

Question 54

Name two exotoxin-mediated etiologies of food poisoning.

Answer 54

* S. aureus* * B. cereus*

Question 55

What is the most common etiology of osteomyelitis in a patient with sickle cell anemia?

Answer 55

*Salmonella*

Question 56

**True/False**. Patients with *C. trachomitis* are typically coinfected with *N. gonorrhoeae*.

Answer 56

True.

Question 57

Name three causes of holosystolic murmurs.

Answer 57

VSD TR MR

Question 58

Marfan syndrome is associated with aortic aneurysms in what aortic section?

Answer 58

Thoracic

Question 59

Which are the culture-negative causes of endocarditis?

Answer 59

*Coxiella*, *Bartonella*, HACEK

Question 60

What are the most common sources of metastases to the brain?

Answer 60

Lung \> breast \> melanoma/colon/kidney

Question 61

What are the most common sources of metastases to the liver?

Answer 61

Colon \>\> stomach \> pancreas

Question 62

What are the most common sources of metastases to the bone?

Answer 62

Prostate/breast \> kidney/thyroid/lung

Question 63

Are neuroblastomas typically benign or malignant? Are pheochromocytomas typically benign or malignant?

Answer 63

Malignant Benign

Question 64

What is the most common cause of esophageal cancer worldwide? And in the U.S.?

Answer 64

Squamous cell carcinoma Adenocarcinoma

Question 65

What is a Cushing ulcer?

Answer 65

Acute gastric ulcer associated with CNS injury (Increased ICP --\> Increased vagal stimulation)

Question 66

What is a Curling ulcer?

Answer 66

Acute gastric ulcer associated with severe burns (Greatly reduced plasma volume --\> gastric mucosal sloughing)

Question 67

What diseases are associated with autosplenectomy (with spleen fibrosis and atrophy)?

Answer 67

Sickle cell disease

Question 68

What is the most common cause of chronic pancreatitis in children?

Answer 68

Cystic fibrosis

Question 69

What is the cause of Bernard-Soulier syndrome?

Answer 69

GpIb deficiency

Question 70

Which has a normal platelet count, Glanzmann thrombasthenia or Bernard-Soulier syndrome?

Answer 70

Glanzmann thrombasthenia

Question 71

Burkitt lymphoma (t;\_\_\_,\_\_\_) is associated with _____ production.

Answer 71

Burkitt lymphoma (t;**_8_**,**_14_**) is associated with ***_c-Myc_*** production.

Question 72

What is the typical cause of death in patients with CML?

Answer 72

Blast crisis

Question 73

What is the typical cause of death in patients with SLE?

Answer 73

Lupus nephropathy

Question 74

What are the most common primary brain tumors in adults?

Answer 74

Astrocytomas (e.g., GM) Meningiomas Schwannomas

Question 75

What are the most common primary brain tumors in children?

Answer 75

If infratentorial: Medulloblastomas (cerebellum) If supratentorial: Craniopharyingiomas

Question 76

What are the characteristics of ALS?

Answer 76

Mixed UMN-LMN degeneration | (no sensory component)

Question 77

What is the most common cause of primary amenorrhea?

Answer 77

Turner syndrome

Question 78

A patient has hypogonadotropic hypogonadism and anosmia. What do you suspect?

Answer 78

Kallman syndrome

Question 79

**True/False**. _Placental alkaline phosphatase_ is often elevated in patients with testicular tumors (e.g., seminomas).

Answer 79

True.

Question 80

Which urinary stones are radiopaque?

Answer 80

Calcium, struvite, cysteine (faintly)

Question 81

Which urinary stones are radiolucent?

Answer 81

Uric acid

Question 82

Name some medications used for migraine prophylaxis.

Answer 82

Beta blockers, topiramate, CCBs, amitriptyline

Question 83

Name some medications used for migraine treatment.

Answer 83

Sumatriptan, NSAIDs

Question 84

Name some medications used for treating alcoholism.

Answer 84

Disulfiram, acamprosate, naltrexone

Question 85

Which alpha-2 agonists are indicated for ADHD treatment?

Answer 85

Guanfacine, clonidine

Question 86

Name some medications used for managing tonic-clonic seizures. **L-PVC** (Use 'el PVC' to fix the leaky brain!)

Answer 86

Levetiracetam, phenytoin, valproate, carbamazepine

Question 87

What is febuxostat?

Answer 87

A xanthine oxidase inhibitor (like allopurinol)

Question 88

Are probenecid and pegloticase used for treating acute gout or chronic gout?

Answer 88

Chronic

Question 89

How is volume of distribution calculated?

Answer 89

Total drug in body / plasma [drug]

Question 90

How is half-life calculated?

Answer 90

0.7 \* volume of distribution / clearance rate

Question 91

How is drug clearance calculated?

Answer 91

Rate_elimination \* plasma [drug]

Question 92

How is loading dose calculated?

Answer 92

Target steady-state [drug] \* volume_distribution / bioavailability Cp \* Vd / F

Question 93

How is maintenance dose calculated?

Answer 93

Target steady-state [drug] \* clearance \* half-life / bioavailability Cp \* CL \* t / F

Question 94

What is attributable risk? What is absolute risk reduction? What is relative risk reduction?

Answer 94

a/a+b - c/c+d c/c+d - a/a+b 1 - RR

Question 95

How is renal blood flow calculated?

Answer 95

RBF = RPF / (1 - hematocrit)

Question 96

What does Paco minus peco over paco tell us (multiply by tidal volume)?

Answer 96

Dead space

Question 97

What is the alveolar gas equation?

Answer 97

Pao2 = Pio2 - Paco2 / R

Question 98

What is Winter's formula?

Answer 98

P_CO2 = 1.5[HCO₃^-] + 8 +or- 2

Question 99

How is an anion gap calculated?

Answer 99

Na⁺ - (Cl^- + HCO₃^-)

Question 100

What is the Henderson-Hasselbalch equation (used for calculating the extracellular pH)?

Answer 100

pH = 6.1 + log [HCO₃^-] / 0.03 \* P_CO2