Chronic Granulomatous Dz
VP Shunts
Congenital ENT
DHE
Pierre-Robin
100
This is the defect that causes CGD
What is a defect in NADPH oxidase? Results in the inability of phagocytes to destroy certain microbes.
100
In an internalized shunt, this is where is the proximal end is located and where the distal end is located.
What is the proximal end is in the ventricle and the distal end is in the peritoneum?
100
This is the most common type of branchial cleft cyst (also must give location of lesion)
What is 2nd branchial cleft. Usually located inferior to the angle of the mandible and anterior to the sternocleidomastoid.
100
This is what DHE stand for.
What is dihydroergotamine?
100
These are the clinical features.
What are micrognathia, cleft palate and glossoptosis?
200
Infectious pathogens in CGD usually have this common microbiological feature.
What is they are catalase positive?
200
These are the 4 main components of a VP shunt.
What are ventricle catheter, reservoir, valve and distal tubing?
200
This is the name for a mucocoele or retention cyst arising from obstruction of the sublingual glands in the floor of the mouth.
What is a ranula?
200
This is the mechanism of action of DHE.
What is ergot alkaloid alpha adrenergic blocker? Causes direct vasoconstriction.
200
You have a baby with Pierre-Robin in your clinic. What change would you make to your normal well child anticipatory guidance?
What is the child should sleep prone?
300
X-linked CGD differs clinically from non-X-linked CGD. How?
What is earlier age of onset and more severe?
300
Though not universal, many children with VP shunts are told to restrict this type of activity.
What are contact sports?
300
This rare finding is the reason excised thyroglossal duct cysts require careful histologic investigation.
What is thyroid carcinoma?
300
This is the time for onset of action in IV formulation.
What is immediate?
300
This is the name of the special nipple put on bottles to feed children with cleft palate.
What is the Haberman nipple or Haberman feeder?
400
Name the 3 most common infections in CGD in order of most-->least common.
What is 1) Pneumonia 2) abscesses 3) adenitis
400
In a non-scientific study done by me 1 hour ago, numerous web posts (about 1 in 2) about VP shunts by people in support groups deal with this non-medical issue
What is the partial head shaving required for the procedure?
400
True or false: The treatment of lingual thyroid is excision.
False: May be the only functioning thyroid tissue.
400
These are the two most commonly reported side effects of DHE
What are nausea and anxiety?
400
Give any feature of the Haberman feeder
1) One way valve prevents air entry 2) slit valve opening closes so baby is not overwhelmed 3) usually there are markings on the bottle to adjust flow as needed
500
The overwhelming majority of infections are caused by these 5 organisms (name 4)
What are staph aureus, burkholderia cepacia, nocardia, aspergillus and serratia?
500
This is how an Ommaya reservoir differs from an externalized shunt.
What is in the Ommaya reservoir, the reservoir is under the scalp?
500
Give two antibiotics for treatment of an infected thyroglossal duct cyst.
What are amoxicillin/clav, cephalexin, clindamycin
500
You have a pediatric cardiology patient s/p something complicated who is being considered for DHE. Thoughts?
What is be careful and probably don't do it! This is a potent vasoconstrictor and an constrict the coronary arteries and is contraindicated in adults with ischemic heart disease.
500
Pierre Robin is associated with many conditions. On the Pierre Robin support group website, the site informs parents to look for signs of Stickler Syndrome. These are the characteristics of this syndrome.
What is craniofacial abnormalities, but also joint and eye problems, which can lead to blindness.
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