3

Question 1

immediate management and long term treatment of SCD vaso-occlusive pain crisis

Answer 1

• IVF and possible narcotics (morphine or hydromorphone)
• Administration of hydroxyurea will increase the concentration of fetal hemoglobin, thus reducing the frequency of sickle cell crisis episodes.

Question 2

risk factors and triggers for sickle cell pain crisis

Answer 2

increasing age and high baseline hemoglobin level

infection, stress, cold temperatures, or high altitude, often no trigger can be found

Question 3

most common sites of sickle cell pain crisis

Answer 3

extremities or back

Question 4

vaso-occlusive crisis

Answer 4

an episode of severe pain caused by increased sickling of RBCs, which leads to bone marrow ischemia and infarction.

Question 5

acute chest syndrome (ACS)

treatment?

Answer 5

A new pulmonary infiltrate on chest x-ray (CXR) in addition to one of the following signs: fever, chest pain, shortness of breath, tachypnea, or low oxygen saturations.
-empiric antibiotics, supplemental oxygen, pain medications, and IVF

Question 6

aplastic crises: etiology?

Answer 6

Infection with parvovirus B19 (most commonly), which leads to temporary cessation of RBC formation. Reduced lifespan of RBCs in SCD coupled with reduced production may result in profound anemia.

Question 7

why is a pt with SCD + fever greater than 38.5°C an emergency?
how to dx/tx

Answer 7

SCD causes functional asplenia and predisposes patients to invasive encapsulated organisms (typically pneumococcal disease)
get CBC and blood culture
initiate empiric abx if no source found (i.e. ceftriaxone)

Question 8

a child with SCD who has abdominal pain, distension, or acute enlargement of the spleen likely has acute splenic sequestration and requires hospitalization, possibly in the intensive care unit, to observe for ?
management may require ?

Answer 8

cardiovascular collapse
Blood transfusions

-spleen typically auto-infarcts with age

Question 9

10% of kids with SCD have acute strokes, so routine well-child care may involve this screening and treatment?

Answer 9

transcranial Doppler (TCD) ultrasonography  to identify those with increased flow velocity in the large cerebral blood vessels, and thus are at high-risk for developing a first stroke
-Routine chronic transfusion

Question 10

child with SCD who presents with a significant increase in pallor, fatigue, or lethargy may be exhibiting signs of ?
labs?

Answer 10

aplastic crisis
hgb level below their normal baseline and a low reticulocyte count
observe for CV collapse, may need transfusion

Question 11

management of boy with SCD who has a priapism episode persisting for more than 3 to 4 hours

Answer 11

IVF, pain control, don’t use ice, urologist may need to aspirate and irrigate the corpora cavernosa to achieve detumescence, Failure of 3 or 4 aspirations in the outpt setting requires more extensive inpt care, including exchange blood transfusions, further pain control, and sx intervention.

Question 12

prophylactic care for kids with SCD

Answer 12

prophylactic penicillin and folate, pneumococcal PCV13 series at 2, 4, 6, and 12 to 15 months, pneumococcal and meningococcal vaccines starting at 2 years of age

Question 13

routine care for kids with SCD

Answer 13

Frequent CBCs are performed, and renal, liver, and lung function are monitored annually beginning at 1 year of age, Routine spleen palpation should be performed at home

Question 14

Children with SCD who have ? (many conditions) must be evaluated urgently

Answer 14

fever (risk of sepsis), pallor (aplastic crisis), abdominal pain or distension (splenic sequestration), pain crisis, evidence of lower respiratory disease (acute chest syndrome) priapism, new neurologic findings (stroke), or dehydration

Question 15

hyperammonemia and elevated urine orotic acid are diagnostic of ?

Answer 15

OTC deficiency, an x-linked condition, the most common urea cycle disorder.

Question 16

Lesions within the vermis (midline cerebellum) cause ?

Answer 16

dysarthria, truncal ataxia, and gait abnormalities

Question 17

Cerebellar hemispheric lesions cause ?

Answer 17

ipsilateral limb abnormalities, nystagmus, tremor/dysmetria and tend to spare speech.
-fall towards the side of the lesion and have worse nystagmus when they look towards the side of the lesion.

Question 18

Lesions of the deep cerebellar nuclei cause ?

Answer 18

resting tremor, myoclonus, and opsoclonus such as that seen in children with a neuroblastoma.

Question 19

Infratentorial lesions usually present with ?

Answer 19

cerebellar signs and signs of raised intracranial pressure (ICP).

Question 20

Cerebellar hemispheric lesions can cause changes in ?

Answer 20

muscle tone and DTRs, usually hypotonia and hyporeflexia.

Question 21

Supratentorial lesions more commonly lead to ?

Answer 21

focal motor and sensory abnormalities on the side opposite to the lesion.

Question 22

Brain stem tumors will often be associated with ?

Answer 22

cranial nerve and gaze palsies.

Question 23

Post-infectious cerebellitis

Answer 23

typically presents in a younger child with ataxia, nystagmus, vomiting and sometimes dysarthria
-believed to be an autoimmune response leading to demyelination of the cerebellum occurring several weeks after a viral infection such as varicella or coxsackie virus.

Question 24

(A, E, I, O, U) and TIPS is often helpful in remembering the causes of altered mental status:

Answer 24

Alcohol, ingested toxins
Epilepsy, encephalitis, endocrine, electrolytes
Infection, insulin
Overdose, opiates, oxygen deprived
Uremia (renal failure)
Trauma, temperature
Insulin, infection
Psychosis
Stroke, shock, space occupying lesions

Question 25

Immediate treatment to address hypoglycemia, hypotension, and tachycardia would include ?

Answer 25

giving boluses of 20cc/kg normal saline and 25% dextrose. These can be followed by administration of fluids with 10% dextrose at maintenance.

Question 26

possible antidote for hypoglycemia caused by antiglycemic OD

Answer 26

Octreotide: a somatostatin analog—inhibits insulin release and may be indicated as an antidote in dextrose-refractory sulfonylurea overdose

Question 27

kid vital signs

Answer 27

http://app1.med-u.org/player/data/db/image/258458.pdf

Question 28

by 12 mos how should weight and height change from birth?

Answer 28

weight should triple and height increase by 50%

Question 29

transposition of the great vessels presents how?

Answer 29

presents in 1st few hours with cyanosis and single loud S2
most common congenital cyanotic heart defect in neonatal period
initiate prostaglandins to keep PDA open to facilitate blood mixing

Question 30

first-line management of epiglottis with severe respiratory distress

Answer 30

endotracheal intubation
then consider abx and lateral neck films
steroids and racemic epi are for croup

Question 31

Diamond-Blackfan Syndrome (DBS)

Answer 31

congenital hypoplastic anemia
macrocytic anemia with NO hyperseg. neutros, low retic, congenital anomalies (short, web neck, cleft lip, shield chest, triphalangeal thumb), pallor in neonate, elevated Hgb F
tx: corticosteroids, transfusion therapy

Question 32

refeeding syndrome

Answer 32

increased insulin results in increase in intracellular uptake of phosphorus, K+, Mg+, thiamine, can result in low serum levels leading to arrhythmia, CHF, seizures, Wernicke’s
-always supplement refeeding with electrolytes

Question 33

cerebral palsy

Answer 33

A disorder of nonprogressive movement and posture that results from an insult to or anomaly of the developing central nervous system (CNS)

Question 34

how common is CP? comorbidities?

Answer 34

CP is the most common childhood movement disorder. about 1/3 also have seizures, about 60% of affected children are mentally retarded
-Deafness, visual impairments, swallowing difficulty with concomitant aspiration, limb and sensory impairments, and behavioral disturbances

Question 35

proposed causes of CP

Answer 35

antenatal insults, and subsequent difficulties during the pregnancy, delivery, and perinatal period increase insult
-10% have family history (chromosomal or metabolic disorder)

Question 36

Acquired conditions that carry a risk for CP are

Answer 36

infection (cytomegalovirus, group B Streptococcus, herpes simplex virus), intraventricular hemorrhage, especially if it extends into the white matter and causes cystic periventricular leukomalacia (PVL), acute bilirubin encephalopathy (kernicterus), stroke, and brain trauma.

Question 37

why is diagnosis of CP nearly impossible in a neonate and difficult in infants younger than 6 months

Answer 37

Immaturity of the CNS at birth, primitive reflexes may persist until 6 months

Question 38

imaging for CP

Answer 38

MRI, may show periventricular leukomalacia, atrophy, or focal infarctions

Question 39

concerning developmental findings in CP

Answer 39

A stepping response after the age of 3 months
A Moro reflex beyond 6 months
An asymmetrical tonic neck reflex beyond 6 months

Question 40

definitions hemiplegia, diplegia, spastic quadriplegia (topographic classification)

Answer 40

Hemiplegia: single lateral side of the body, UE worse than LEs
Diplegia: 4 limbs, LE worse than UE
Spastic quadriplegia: 4 limbs typically equal but LE may be worse than UE

Question 41

Physiologic classification of CP identify the major motor abnormality and are divided into ?

Answer 41

pyramidal (spastic) and extrapyramidal (nonspastic) categories. Extrapyramidal types can be subdivided further into choreoathetoid, ataxic, dystonic, or rigid types.

Question 42

functional classification of CP relies on a “motor quotient”

Answer 42

A motor quotient of 75 to 100 represents minimal impairment, 55 to 70 is mild impairment, 40 to 55 is moderate impairment, and lesser quotients indicate severe impairment.

Question 43

Identification of comorbid conditions with CP includes ?

Answer 43

cognitive testing for mental retardation and electroencephalography (EEG) for seizures, vision and hearing test

Question 44

cystic fibrosis

Answer 44

The major cause of chronic debilitating pulmonary disease and pancreatic exocrine deficiency in the first three decades of life. It is characterized by the triad of chronic obstructive pulmonary disease, pancreatic exocrine deficiency, and abnormally high sweat electrolyte concentrations. Characteristic pancreatic changes give the disease its name.

Question 45

most common mutation causing the abnormal CFTR (cystic fibrosis transmembrane conductance regulator) protein is known as CFTR (cystic fibrosis transmembrane conductance regulator) protein ?
which allows loss of ?

Answer 45

delta 508
altered CFTR protein allows excess loss of sodium and chloride. The loss from the eccrine glands of the skin causes a hyponatremic, hypochloremic alkalosis

Question 46

CF may result in fat-soluble vitamin deficiencies manifesting as ?

Answer 46

peripheral neuropathy and hemolytic anemia (vitamin E), night blindness (vitamin A), or mucosal bleeding (vitamin K)

Question 47

The diagnosis of CF requires

Answer 47

two positive sweat tests (60 mEq/L or greater) in conjunction with any of the characteristic respiratory, gastrointestinal, or genitourinary symptoms.
then DNA testing is then employed to detect the presence of mutations known to cause CF to confirm, even if sweat Cl- is negative (may be falsely neg)

Question 48

Routine screening for CF is performed on all newborns in the US with what test?

Answer 48

detection of pancreatic enzyme, immunoreactive trypsinogen (IRT), which is elevated in infants with CF.
if +, undergo sweat chloride testing for definitive diagnosis

Question 49

management of CF

Answer 49

Bronchodilators, inhaled corticosteroids, antibiotics, and inhaled recombinant human DNAse are used to minimize airway reactivity, infections, and secretions.
Optimal nutrition is dependent on pancreatic enzyme replacement and vitamin supplements.

Question 50

Extrapulmonary signs and symptoms, such as ? are clues to the diagnosis of CF.

Answer 50

digital clubbing, recurrent sinusitis, failure to thrive, fat malabsorption, bronchiectasis, and a history of meconium ileus