5/26 Mixed

Question 1

ALL

Answer 1

The most common malignancy of childhood
B-cell ALL is responsible for 70-80% of all cases of ALL
T-cell ALL accounts for 15-17% of all ALL
T-cell ALL often presents as a mediastinal mass that can cause respiratory symptoms, dysphagia, or superior vena cava syndome (young male)

Question 2

Graft vs. host disease

Answer 2

can occur following transplantation of organs rich in lymphocytes (e.g. liver)
T lymphocytes found in the donor organ become sensitized against MHC antigens of the recipient and subsequently attack the host’s tissues
The skin, liver and GI tract are most frequently affected (diffuse maculopapular rash, diarrhea, intestinal bleeding, abdominal pain)

Question 3

Lesions of the femoral nerve

Answer 3

Can occur due to trauma, nerve compression, stretch injury, or ischemia
Patients develop weakness of the quadriceps muscle, loss of the patellar reflex, and loss of sensation over the anterior and medial thigh and medial leg

Question 4

Specific features of hyperthyroidism

Answer 4

Pretibial myxedema and exopthalmous are specific features of hyperthyroidism due to Graves disease
They are caused by an autoimmune response directed against the TSH receptor that results in accumulation of glycosaminoglycans within the affected tissue

Question 5

“Red ragged” Muscle fibers

Answer 5

Mitochondrial disease
“Blotchy red muscle fibers on Gomori trichrome stain”
Muscle fibers have this appearance because abnormal mitochondria accumulate under the sarcolemma.
Mitochondrial dx show maternal inheritance
Ex. Myoclonic epilepsy, Leper optic neuropathy, Mitochondrial encephalopathy with stroke-like episodes and lactic acidosis

Question 6

Osteogenesis Imperfecta

Answer 6

Results from defective synthesis of type 1 collagen
Clinical findings include a history of fractures after only minimal trauma, abnormal (blue) sclerae, and small malformed teeth
Problem with bone matrix formation

Question 7

Kawasaki disease

Answer 7

Vasculitis of medium sized arteries
Presents with persistent fever for > 5 days, bilateral conjunctivitis, cervical lymphadenopathy, mucocutaneous involvement
Coronary artery aneurysms are a serious complication

Question 8

Most common cause of calcium kidney stone disease

Answer 8

Idiopathic hypercalciuria

Normal serum calcium levels with hypercalciuria

Question 9

Polyhydramnios (excessive accumulation of amniotic fluid)

Answer 9

Presents with increase abdominal circumference out of proportion to gestational age
Etiology is decreased fetal swallowing or increased fetal urination
Fetal anomalies associated with impaired swallowing include gastrointestinal obstruction (eg, duodenal, esophageal, intestinal atresia) and anencephaly

Question 10

Alzheimer’s Disease

Answer 10

Beta-AMYLOID deposits in the brain parenchyma (neuritic plaques) and walls of cerebral vessels (amyloid angiopathy)

Question 11

Individual subunits of hemoglobin

Answer 11

Structurally analogous to myoglobin

Monomeric subunits will demonstrate a hyperbolic oxygen- dissociation curve similar to that of myoglobin

Question 12

COX-2

Answer 12

An inducible enzyme that is upregulated to increase synthesis of pro-inflammatory arachidonic acid metabolites
Selective COX-2 inhibitors (Celecoxib) decrease inflammation by inhibiting COX-2 but have no effect on COX-1 (minimizing gastroduodenal toxicity)

Question 13

Non-pharmacologic treatments for insomnia

Answer 13

Sleep hygiene
Stimulus control
Relaxation
Sleep Restriction
Cognitive behavioral therapy

Stimulus control focuses on eliminating stimulating bedroom activities and getting into bed only when sleepy

Question 14

Rheumatoid arthritis

Answer 14

Progressive joint destruction involving the hands, wrists, elbows, knees. Cervical spine involvement can lead to spinal instability and cord compression

Question 15

Acetazolamide in the treatment of open-angle and angle-closure glaucoma

Answer 15

Carbonic anhydrase in proximal tubule
Acetazolamide inhibits CA, blocks NAHCO3 and water reabsoprtion in PT, resulting in urinary bicarbonate wasting
Relieve intraocular pressure in open-angle and angle-closure glaucoma

Question 16

B12 deficiency myelopathy

Answer 16

Subacute combined degeneration
Degeneration of both the dorsal columns and corticospinal tract
Loss of position and vibration sensation, ataxia, and spastic paresis are common manifestations

Question 17

Auer rods

Answer 17

Deformed azurophilic granules found in the cytoplasm of myeloblasts that stain positively for myeloperoxidase (PEROXIDASE)
Auer rods are found in abundance in AML M3 (APL)

Question 18

Meningococcal lipooligosaccharide (LOS)

Answer 18

Responsible for many of the toxic effects observed in meningitis and meningococcemia
Blood levels of LOS correlate closely with morbidity and mortality

Question 19

Ischemic-hypoxic encephalopathy

Answer 19

Profound cerebral hypoperfusion may lead to global cerebral ischemia (also called ischemic-hypoxic encephalopathy)
Watershed infarcts occurs between the zones of perfusion of the anterior, middle, and posterior cerebral arteries
These infarcts typically appear as bilateral wedge-shaped strips of necrosis over the cerebral convexity, parallel and adjacent to the longitudinal cerebral fissue

Question 20

Transference

Answer 20

The unconscious shifting of emotions associated with a significant person from one’s past to a person in the present

Question 21

Poor long-term prognostic factor for PSGN?

Answer 21

AGE. Most children recover completely but adult patients have a relatively poor prognosis and higher risk of chronic HTN and renal insufficiency

Question 22

Synaptophysin

Answer 22

Protein found in the presynaptic vesicles of neurons, neuroendocrine and neuroectodermal cells
CNS tumors of neuronal origin frequently stain positively for synaptophysin on immunohistology
Neoplasms of glial origin (astrocytomas, ependymomas, and oligodendrogliomas) strain for GFAP

Question 23

Sheehan Syndrome

Answer 23

High estrogen levels during pregnancy cause enlargement of the pituitary gland without a proportional increase in blood supply
Peripartum hypotension can cause ischemic necrosis of the pituitary leading to panhypopituitarism (Sheehan syndrome)
Patients commonly develop failure of lactation due to deficiency of prolactin

Question 24

Factor V Leiden

Answer 24

One to nine percent of Caucasians worldwide are heterozygote carriers of factor V Leiden, which is modified to resist activated protein C
The resulting hypercoagulable state predisposes to deep vein thromboses, which are the source of most pulmonary emboli

Question 25

SGLT2 Inhibitors (Canagliflozin, dapagliflozin)

Answer 25

Oral anti-diabetic agents Decrease proximal tubular reabsorption of glucose, promoting urinary glucose loss *Recommended to monitor serum creatinine before and after initiating therapy with SGLT2 inhibitors

Question 26

Maternal serum quadruple screen

Answer 26

Performed to assess risk of congenital defects in fetuses Accurate dating is important for determining whether levels of alpha-fetoprotein and other analytes are abnormal for gestational age Patients with a history of irregular menses are at risk for inaccurate pregnancy dating

Question 27

A yellowish eyelid papule or plaque containing lipi-laden macrophages= xanthelasma

Answer 27

Xanthelasma may occur in association with primary or secondary hyperlipidemia; cholestatic conditions such a primary biliary cirrhosis are a potential cause of hypercholesterolemia leading to xanthelasma

Question 28

Atherosclerotic plaque formation

Answer 28

Vascular smooth muscle cells are the only cells within the atherosclerotic plaque capable of synthesizing structurally important collagen isoforms and extracellular matrix Progressive enlargement of the plaque results in remodeling of the extracellular matric and VSMC death, promoting development of vulnerable plaques with an increased propensity for rupture

Question 29

First generation H1-histamine receptor antagonists (Diphenhydramine and chlorpheniramine)

Answer 29

Can cause significant sedation, especially when used with other medications that cause CNS depression (such as benzodiazepines)

Question 30

Parotid gland tumors

Answer 30

The facil nerve exists the stylomastoid foramen and courses through the substance of the parotid gland, where it divides into its 5 terminal branches that innervate the muscles of facial expression. Malignant tumors of the parotid gland often compress and disrupt the facial nerve causing ipsilateral facial droop

Question 31

Ethylene glycol ingestion

Answer 31

Leads to toxic, acute tubular necrosis with vacuolar degeneration and ballooning of the proximal tubular cells Typical clinical findings include high anion gap metabolic acidosis, increased osmolar gap, and calcium oxalate crystals in the urine

Question 32

Acute renal allograft rejection

Answer 32

Can be an antibody or cell mediated process Acute cellular rejection is associated with diffuse lymphocytic infiltration of the renal vasculature (endotheliitis), tubules, and interstitium

Question 33

Expanding space occupying lesions within the temporal lobe can cause elevated intracranial pressure with transtentorial herniation of the uncus

Answer 33

Uncal herniation often compresses the ipsilateral third cranial nerve as it exits the midbrain, resulting in oculomotor nerve palsy with a fixed dilated pupil (eg due to preganglionic parasympathetic fiber damage) These fibers run on the outer portion of the third cranial canal

Question 34

Fick principle = used to calculate cardiac output using the rate of oxygen consumption and the AV oxygen content difference

Answer 34

Cardiac output= rate of O2 consumtion/ AV O2 content difference