Respiratory Delights
Genetic Disorders
Formula Fantasia
Neurology Nuances
Potpourri
100
Congenital absence of which surfactant protein (SP) is most likely to be lethal in the neonatal period?
What is surfactant protein B?
100
A syndrome resulting from a deletion of 7q11.23 and characterized by supravalvular subaortic stenosis and hypoplastic nails.
What is Williams syndrome?
100
P = 2T / r
What is Laplace's Law?
100
This nerve root injury may involve the T1 nerve root, resulting in Horner's syndrome
What is Klumpke's Palsy (C8-T1)
100
These events on a fetal HR monitor are thought to result from compression of the fetal head.
What are early decelerations?
200
This measure of respiratory mechanics is the product of the Resistance X Compliance?
What is the time constant?
200
An AGA term male infant has hypoglycemia, a large tongue, exophthalmos and a prominent occiput. Although most cases of this syndrome are sporadic, 15% of cases are inherited. What is the most likely pattern of familial inheritance for this infant's syndrome?
What is autosomal dominant?
Beckwith-Wiedemann syndrome
200
For a clinical trial, this is defined as 1-type II Error
What is Power?
200
This neurocutaneous syndrome is characterized by irregular brown pigmentation, fibrous dysplasia of bones, and hyperparathyroidism and precocious puberty.
What is McCune Albright syndrome?
200
A subgaleal bleed occurs in this compartment
What is C?
300
A decrease in 2,3 disphosphoglycerate will shift the Oxyhemoglobin Dissociation Curve to this direction.
What is to the left?
300
This syndrome is characterized by syndactyly of the 2nd and 3rd toes, anteverted nostrils, and hypospadias and cryptorchidism.
What is Smith-Lemli-Opitz syndrome?
300
True Negative / (False Positive + True Negative)
What is specificity?
300
Which of the two most commonly used methods to screen for hearing loss in newborns is more likely to affected by fluid in the ear canal?
What is Evoked Otoacoustic Emissions (EOAE)?
300
This statistical test is most appropriate for comparing continuous variables among 3 or more study groups.
What is the Kruskal-Wallis test?
400
According to the oxygen content equation, how many ml of oxygen are carried per gram hemoglobin in newborns?
What is 1.34?
400
This rare, lethal, autosomal recessive disorder characterized by the following triad of clinical findings: occipital encephalocele, polycystic kidneys, and polydactyly.
What is Meckel-Gruber Syndrome (MGS)
400
Fill in the missing value:
OI = [(MAP X FiO2) / ( ) ] X 100
What is the postductal paO2?
400
This antiepileptic drug can increase the risk of cardiac arrhythmias.
What is phenytoin?
400
This category of metabolic disorders may be suspected in an fetus when diagnosis of acute fatty liver of pregnancy is made.
What are long-chain fatty acid oxidation disorders?
500
This pulmonary vasodilator is phosphodiesterase-3 inhibitor that results in increased concentrations of cyclic adenosine monophosphate (cAMP)
What is milrinone?
500
A syndrome characterized by hypertelorism, maxillary hypoplasia, shallow orbits and premature craniosynostosis
What is Crouzon syndrome?
500
(tidal volume-dead space volume) X resp rate
Alveolar Ventilation (VA)
500
This neuromuscular disorder is caused by expanded CPG repeats on chromosome 19.
What is congenital myotonic dystrophy?
500
This reflex describes the increase in ventilatory rate assoiate with abrupt delfation of lungs (such as occurs with a pneumothorax).
Hering-Breuer Deflation Reflex
600
An infant is born with tracheo-esophageal fistula. This defect likely occurred during this stage of lung development?
What is the embryonic stage (1st seven weeks)?
600
Infants with this syndrome may have this ocular manefestation characterized by prominent Schwalbe's line
What is Alagille syndrome (posterior embryotoxon)
600
Using variables (abcd), define sensitivity:
What is: a/(a+c)?
aka: TP/ (TP + FN)
600
This reflex appears at 28 weeks' gestational age, is established by 32 weeks' gestation & dissappears at 2-4 months. If it persists, it is characteristic of athetoid cerebral palsy.
What is the palmar grasp reflex?
600
An infant is admitted with ambiguous genitalia. Which of the following etiologies is least likely to be attributed to an overvirilized female?
A. 5-alpha reductase deficiiency. B. 11-beta hydroxylase deficiency C. 21-hydroxylase deficiency D. Aromatase deficiency
What is A) 5-alpha reductase deficiency?