Review of Topics

Question 1

Gliobastoma multiforme: incidence, pathology, location

Answer 1

• Most common primary brain tumor of adults
• Astrocytes stain for GFAP
• Found in cerebral hemispheres and can cross the corpus callosum (butterfly glioma)

Question 2

Meningioma: incidence, pathology, location

Answer 2

• second most common adult brain tumor
• arachnoid cells: spindle cells concentrically arranged in a whorled pattern w psammoma bodies
• in cerebral hemispheres

Question 3

Schwannoma: incidence, pathology, location

Answer 3

• third most common adult brain
• S-100 positive
• often localized to CN VIII (acoustic neuroma- bilateral in NF 2)

Question 4

oligodendroglioma: incidence, pathology, location

Answer 4

• rare, slow growing adult brain tumor
• chicken wire capillary pattern with fried egg cells
• most often in frontal lobes

Question 5

4 common childhood tumors in order of prevalence?

Answer 5

1. Medulloblastoma: cerebellar tumor- can compress 4th ventricle, can send drop metastases
2. Pilocytic astrocytoma: posterior fossa (cerebellum), benign, good prognosis
3. Brainstem gliomas
4. Ependymoma: found in 4th ventricle

Question 6

most common SUPRATENTORIAL childhood tumor?

Answer 6

craniopharyngioma

Question 7

what is contraction alkalosis?

Answer 7

metabolic alkalosis that occurs as a result of fluid/volume losses
- most likely due to increase in Renin, angiotensin II and aldosterone (accounts for hypokalemia seen in contraction alkalosis)

Question 8

Describe the childhood rash: rubella

Answer 8

rash begins at head and moves down: fine truncal rash

Question 9

Describe the childhood rash: measles

Answer 9

• paramyxovirus, begins at head and moves downward

- PRECEDED by cough, coryza, conjunctivitis, and blue-white (Koplik) spots on buccal mucosa

Question 10

Describe the childhood rash: Roseola

Answer 10

• HHV-6, macular rash over body appears AFTER several days of high fevers (can have seizures)

Question 11

Describe the childhood rash: Erythema infectiosum

Answer 11

• aka 5th disease by parvovirus B19

- slapped cheek race on face (can cause hydrops fetalis in pregnant women)

Question 12

By 12 months, a child’s weight should ___ and height should ____

Answer 12

weight should triple

height should increase by 50%

Question 13

what does DAF deficiency lead to?

Answer 13

complement mediated lysis of RBCs and proxysmal nocturnal hemoglobinuria

Question 14

C5-C9 deficiencies lead to?

Answer 14

recurrent neiserria bacteremia (bc neisseria requires complement host defenses

Question 15

C1 esterase inhibitor deficiency leads to what?

Answer 15

hereditary angioedema: autosomal dominant disorder causing episodes of painless, non pitting well circumscribed edema of the face, neck, lips, tongue
- ACE inhibitors are contraindicated

Question 16

what cytokine mediates septic shock?

Answer 16

TNF alpha: causes cachexia, activates endothelium, causes leukocyte recruitment and vascular leak

Question 17

what two cytokines are involved in inhibiting inflammation?

Answer 17

IL-10 and TGF-B

Question 18

For knowledge, type IV hypersensitivity reactions:

Answer 18

1. Multiple sclerosis
2. Guillain-Barre syndrome
3. PPD
4. Contact dermatitis

Question 19

For knowledge, type III hypersensitivity reactions:

Answer 19

1. SLE
2. Polyarteritis nodosa
3. Post strep Glomernephritis
4. Serum Sickness
5. Arthus Reaction
6. Rheumatoid arthritis

Question 20

mnemonic for hyper IgE syndrome?

Answer 20

FATED:
coarse Facies
cold staph Abscesses
retained primary Teeth
Increased IgE
Dermatologic problems (doughy skin)
= inability of neutrophils to respond to chemotactic stimuli

Question 21

The A’s of Ataxia Telangiectasia

Answer 21

defect in ATM gene (encodes for DNA repair enzymes)
IgA deficiency
Ataxia within first year of life
Autosomal recessive
Increased AFP
spider ANgiomatas
*increased risk of cancer ie leukemias and lymphomas

Question 22

absent pus formation and delayed separation of umbilical cord + recurrent bacterial infections

Answer 22

Leukocyte adhesion deficiency

Question 23

recurrent pyogenic infections by staph and strep, partial albinism, peripheral neuropathy

Answer 23

Chediak Higashi syndrome: autosomal recessive, defect in LYST gene, microtubule dysfunction

Question 24

mnemonic for wiskott aldrich syndrome?

Answer 24

WAITER
Wiskott Aldrich
Immunodeficiency
Thrombocytopenia/purpura
Eczema
Recurrent pyogenic infections
*X linked, WASP gene on x chromosome

Question 25

difference between caput succedaneum and cephalohematoma?

Answer 25

Caput succ: diffuse swelling or edema of the scalp that crosses the midline and is benign
Cephalohematoma: subperiosteal hemorrhages that are sharply limited by sutures and do not cross the midline, usually benign but may indicate an underlying skull fracture so get a XR of CT to rule out

Question 26

when should the anterior fontanelle close and what disorders should u suspect in delayed closure?

Answer 26

by 18 months closes

• should suspect:
  1. Hypothyroidism
  2. Hydrocephalus
  3. Rickets
  4. IUGR

Question 27

If only one umbilical artery is present instead of two, what should you suspect?

Answer 27

congenital renal malformations