Prep 2019 Flashcards
What causes horner’s syndrome in a neonate?
injury to the sympathetic fibers of the 1st thoracic nerve
may also get delayed pigmentation of iris
(horners = ptosis, miosis, anhidrosis, enophthalmos)
Stepwise approach to epiglottitis + etiology
- 100% humidified O2
- Airway expert to stabilize airway
etiology used to be H.Flu (still in unimmunized)
but now Streptococcus pneumoniae, Staphylococcus aureus, β-hemolytic streptococci, other types of H influenzae (A, F, and nontypeable), H parainfluenzae, Klebsiella, Pseudomonas, and multiple viruses.
What is Tay Sach’s?
- Cause, testing
- Timing in life
- Exam
- Symptoms
- AR metabolic disorder caused by hexosaminidase A deficiency
- neurodegenerative disorder.
- Normal at birth then by 3-6 mo get - loss of motor skills, increased weakness, decreased alertness, and increased startle response
- Exam: cherry-red spot of the fovea centralis of the macula of the retina, normal-sized liver and spleen, hyperreflexia, ankle clonus, and diffuse muscular hypotonia.
- Dx by leukocyte enzyme testing == low to absent levels of β-hexosaminidase A enzymatic activity in the white blood cells, with normal to high levels of the β-hexosaminidase B isoenzyme.
What is Klippel-Feil Syndrome?
- Congenital fusions of the cervical spine
- Assoc - CHD, vertebral fusions at other levels, hearing loss, renal anomalies
- Sprengel deformity (15-20%) congenital elevation of the scapula
What age group can you not trust cdiff testing? why not?
<3yo b/c they are commonly colonized
What is a greenstick fracture? typical mechanism?
- up to 50% of fx in <12yo
- mechanism usually fall on outstretched hand
- angulated but not displaced b/c long bone a bends before it breaks, so thick periosteum remains intact on one side
Test of choice for proteinuria?``
First morning urine sample with urine protein:cr ratio of >0.2 == abnormal
How to accurately take BP?
- cuff bladder width that is 40% of arm circumference
- place middway between olecranon and acromion
- inflate to 20mmHG above point at which radial pulse is not palpated
- Deflate no faster than 2-3mmHg per second
MCC thyroid enlargement in children? Who has`` increased risk?
- Hashimoto’s thyroiditis
- Trisomy 21
How does classic galactosemia present?
- 1st week after birth
- feeding problems, failure to thrive, progressive hepatic dysfunction, bleeding, cataracts, and Escherichia coli sepsis in neonates after a lactose-containing diet is introduced.
- immediately change to lactose-restricted diet
- AR IEM
- Dx by elevated erythrocyte G1P conc and decreased GALT and biallelic gene mutations in GALT gene
Whats VACTERL?
vertebral defects anal atresia cardiac defects, tracheoesophageal fistula renal anomalies limb abnormalities
What is Potter Syndrome/sequence?
- Characteristics features caues by oligohydramnios
- ARPCKD
- pulmonary hypoplasia (presenting with respiratory distress in the newborn)
- facial appearance (pseudoepicanthus, flattened ears and nose, recessed chin)
- skeletal abnormalities (hemivertebrae, sacral agenesis)
- ophthalmologic malformations (eg, cataracts, lens prolapse), and limb abnormalities (club feet and hip dislocation).
Treatment of Ichthyosis Vulgaris (ugly fish skin) and appearance
- “dry skin with “thin scales that have a pasted on appearance”
- Tx keratolytic agents = ammonium lactate cream, alpha hydroxy acid, and urea-containing emollients
- 50% will also have atopic dermatitis
What is granuloma annulare? what other skin condition can it be confused with?
- Benign inflammatory condition “non-scaling annular lesions without epidermal involvement”
- unclear cause
- NON SCALING vs. ringworm which is scaling
Erythema Multiforme Minor associations
- Primary/recurrent HSV
- Medication
