Q 1:
You are asked to evaluate a 5-year-old boy for dry skin that has been present since infancy and has been resistant to treatment with emollients. The boy's father also has dry, scaly skin and allergic rhinitis.
The physical examination demonstrates generally dry skin, with thin scales that have a "pasted-on" appearance on the extensor surfaces of the legs and buttocks.
Of the following, the MOST appropriate therapy is
A. ammonium lactate topically
B. cephalexin orally
C. isotretinoin orally
D. tacrolimus topically
E. triamcinolone topically
The Answer :
A
Explanation :
The child described in the vignette has features of ichthyosis vulgaris, an autosomal dominant disorder that usually becomes apparent between 3 months and 5 years of age.
Affected individuals develop thin scales that have elevated edges, creating the appearance that the scales have been “pasted-on
.” The extensor surfaces of the lower extremities are affected primarily, although the trunk and upper extremities may be involved.
Typically, the face and antecubital and popliteal fossae are spared.
Ichthyosis vulgaris is a chronic disorder that tends to improve over time.
Although it can be managed effectively, there is no cure, and scaling returns if therapy is discontinued.
Initially, treatment involves frequent emollient application that prevents evaporation of moisture from the skin. If the response to this treatment is unsatisfactory, a product containing an alpha hydroxy acid (eg, ammonium lactate, citric acid, lactic acid, and glycolic acid) may be applied to hydrate the skin and cause skin cells to detach.
Alternately, a urea-containing emollient or propylene glycol may be applied to enhance water binding or a product that contains salicylic acid used to reduce scale.
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Q 2:
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Q 6
Answer
E
The Answer :
A
Explanation :
The child described in the vignette has features of ichthyosis vulgaris, an autosomal dominant disorder that usually becomes apparent between 3 months and 5 years of age.
Affected individuals develop thin scales that have elevated edges, creating the appearance that the scales have been “pasted-on
.” The extensor surfaces of the lower extremities are affected primarily, although the trunk and upper extremities may be involved.
Typically, the face and antecubital and popliteal fossae are spared.
Ichthyosis vulgaris is a chronic disorder that tends to improve over time.
Although it can be managed effectively, there is no cure, and scaling returns if therapy is discontinued.
Initially, treatment involves frequent emollient application that prevents evaporation of moisture from the skin. If the response to this treatment is unsatisfactory, a product containing an alpha hydroxy acid (eg, ammonium lactate, citric acid, lactic acid, and glycolic acid) may be applied to hydrate the skin and cause skin cells to detach.
Alternately, a urea-containing emollient or propylene glycol may be applied to enhance water binding or a product that contains salicylic acid used to reduce scale.
----------------------------------------
Q 2:
You
are evaluating a 4-week-old boy for tearing of the right eye that has worsened
over the past week. Physical examination reveals slight tearing but no evidence
of purulent exudate or conjunctival erythema. All other findings are normal.
Of
the following, the MOST appropriate initial management is:
A. administration
of amoxicillin
B. endoscopic
dacrocystorhinostomy
C. instillation
of silver nitrate into the eyes
D. observation
with intermittent massage of the duct
E. surgical
dilation of the nasolacrimal duct
Answer :
D
--------------------------
Q 3
Answer :
D
Congenital
nasolacrimal duct obstruction is the most common abnormality of the infant’s
lacrimal system.
Almost 5% of infants manifest nasolacrimal duct obstruction,
and 30% of affected infants have bilateral obstruction. The obstruction is
usually at the distal end of the nasolacrimal system.
Most typically, infants
present with tearing and crusting of the eyelashes. Massage of the duct will
reveal clear-to-mucopurulent material. Conjunctival inflammation, photophobia,
blepharospasm, corneal clouding, or other eye abnormalities are notably absent.
Natural
resolution rates as high as 95% have been reported by 13 months of age.
Complications are rare and include development of nasolacrimal duct cysts with nasal obstruction or dacryocystitis.
Because
of the favorable natural history, recommended treatment is conservative.
Digital massage of the duct two to three times a day may help to open the
occluded system.
Topical
antibiotics are indicated only for mucopurulent conjunctivitis. Silver nitrate
is indicated for the prevention of gonococcal ophthalmia, but may cause
chemical conjunctivitis. Systemic antibiotics may be necessary for treatment of
dacryocystitis, a bacterial infection of the lacrimal sac manifested by edema,
erythema, and tenderness over the sac.
Nasolacrimal
duct probing is appropriate if symptoms persist beyond 13 months of age.
Probing usually is performed under general anesthesia and generally is
curative. Tube placement or dacrocystorhinostomy (surgical opening of the
lacrimal system into the lateral nasal wall) are indicated for
persistent duct obstruction that does not respond to conservative medical and
surgical management.
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Q 3
A
mother brings her 4-month-old infant to the emergency department because the
infant has been excessively irritable for 10 hours. She has been feeding
normally. The mother reports that the infant seems to be rubbing her right eye.
On physical examination, the child is afebrile, interactive, and soothes
temporarily in her mother's arms, but she has frequent outbursts of crying.
There is tearing in the right eye, with scant conjunctival injection.
Of
the following, the evaluation that is MOST likely to aid in the diagnosis is:
A. conjunctival
swab for Chlamydia trachomatis
B. conjunctival
swab for Gram stain
C. fluorescein
staining of both eyes
D. funduscopic
examination
E. measurement
of intraocular pressure
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Q4
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Q4
You
are asked to evaluate a 1-day-old healthy term African-American infant who has
a rash. Findings on physical examination include scattered pustules without
surrounding erythema involving the trunk and forehead and several small
hyperpigmented macules, some of which possess a collarette of scale.
Of
the following, the MOST likely diagnosis is:
A. candidiasis
B. erythema
toxicum
C. neonatal
herpes simplex virus infection
D. Staphylococcus
aureus infection
E. transient
neonatal pustular melanosis
Answer
E
Answer
E
The physical findings described for the infant in the vignette are
those of transient neonatal pustular melanosis. Pustules and small (2 to 3 mm in diameter) hyperpigmented
macules are present at birth. The macules are surrounded by a
collarette of scale that represents the remnant of a pustule roof. Although the
clinical diagnosis usually is straightforward, if there is uncertainty, a Gram
or Wright stain of pustule fluid will reveal polymorphonuclear neutrophils
(PMNs) without organisms.
Candidiasis
in the neonate may be acquired in utero or at the time of delivery. Infants who
have congenital candidiasis exhibit diffuse scaling and erythema or
erythematous papules and pustules at birth. Those who acquired infection
natally have similar physical findings that begin days to weeks after delivery.
A potassium hydroxide preparation performed on scale or a pustule roof
demonstrates pseudohyphae and spores.
Erythema
toxicum is a benign, self-limited eruption that occurs in
approximately 50% of term newborns. It is characterized by erythematous macules
2 to 3 cm in diameter that have a central papule, vesicle, or pustule. The
eruption typically begins at 24 to 48 hours of life and lasts for 4 to 5 days.
A Wright stain of the contents of a vesicle or pustule reveals
numerous eosinophils.
Neonatal
herpes simplex virus infection usually is acquired from passage through an
infected birth canal. The clinical hallmark of infection is clustered vesicles
on an erythematous base. Although lesions may be present at birth, more often
they appear during the first week of life.
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Q 5
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Q 5
An
8-year-old girl is having trouble keeping up with her class in reading. She
holds her book closely while reading, but has normal distance vision. Results
of the physical examination are normal.
Of
the following, the most appropriate intervention is to:
A. arrange
for large print books to be available in her classes
B. limit
the amount of time she spends reading
C. make
sure lighting is appropriate when she is reading
D. refer
her for ophthalmologic examination
E. refer
for educational evaluation
Answer
E
Answer
E
The
most common ophthalmologic problem in young children is myopia or
near-sightedness. Many children at the age of the girl described in the
vignette hold books closely and have no difficulty focusing at close distances.
By age 40, most people have lost the ability to read at near distance and begin
to use reading glasses. The normal results on a physical examination of the eye
to exclude inflammation and normal distance vision reported for this girl make
an ophthalmologic etiology unlikely for her school difficulty. Accordingly,
arranging for large print books, limiting the amount of time she spends
reading, making sure lighting is appropriate for reading, or referring her for
ophthalmologic examination are not appropriate interventions. The most likely
cause of this child’s difficulty is educational. She should be referred for
achievement cognitive testing to help clarify her academic strengths and
weaknesses.
The
eye evaluation for children changes from birth to school age. From birth to 2
years of age, it should include examination of the eyelids and orbits for
symmetry and function; external evaluation of the conjunctiva, sclera, cornea,
and iris; assessment of ocular motility and muscle balance with the corneal
light reflex; and examination of the posterior segment of the eye with the red
reflex. The unilateral cover test is useful in infants and toddlers who are
able to fixate on an object. If the infant becomes fussy when one eye is
covered, poor vision in the uncovered eye is a possibility.
Examination
of children ages 2 to 4 years includes the previously noted tests and two
additional measures. Ophthalmoscopic examination may be possible in very
cooperative 3- to 4-year-old children. Vision testing should be attempted with
pictures by 3 years of age, although some younger children may be able to
perform the Allen card test. The Allen card test is known for its commonly used
symbols of a truck, house, birthday cake, bear, telephone, horse, and tree.
Complete evaluation of a child should be attempted by age 3 and repeated in 6
months if not initially successful. If the repeat examination also is
unsuccessful, the child should be referred to a pediatric ophthalmologist for
testing. Similar testing should occur at age 5 years, but children ages 5 to 10
years should be tested annually due to the high frequency of refractive errors
noted in this age group. After age 10 years, children and adolescents should
have vision screening every 2 to 3 years.
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Q 6
During
your evaluation of an infant in the nursery, you note that the red reflex in
one eye seems paler than in the other.
Of
the following, the MOST appropriate next step is to:
A. apply
a cycloplegic to the infant's eyes for examination before discharge
B. perform
the corneal light reflex test
C. perform
the cover-uncover test
D. re-evaluate
the red reflex in 1 to 2 weeks
E. refer
the infant to a pediatric ophthalmologist
Answer
E
The
red reflex is evaluated using a direct ophthalmoscope that is
held 12 to 24 inches from the eyes. In an alert infant, both eyes may be
visualized at once. The reflected light from the retina will be orange-red and
should be of equal color and intensity in each eye. Abnormalities in the
pupillary red reflex suggest cataracts or intraocular pathology and warrant an
immediate ophthalmologic referral.
Leukocoria (white pupillary
reflex) can be caused by cataract, tumor (retinoblastoma), chorioretinitis,
retinopathy of prematurity, or a persistent hyperplastic vitreous.
It
is not necessary to dilate the infant’s eyes with a cycloplegic to examine the
light reflex. If the infant closes his or her eyes during the examination,
holding him or her and tipping the head forward and backward will take
advantage of the tonic labyrinthine and neck reflexes. Most infants will open
their eyes during this maneuver. The red reflex should be examined at each
visit during infancy because leukocoria may develop after the newborn period.
Because there is no expected change in the red reflex during infancy, waiting
to re-examine the infant’s eye until 2 to 3 weeks of age is not recommended.
The
corneal light reflex test screens for ocular alignment and is performed by
shining a light on both eyes at the same time. The reflected light noted on the
corneal surface should be in the same position on each pupil. A deviation of 2
mm or more is indicative of ocular misalignment.
The
cover-uncover test evaluates for eye muscle imbalance and visual acuity. The
infant or child must be able to fixate on an object, usually a toy or a light.
The examiner first moves the object in front of the child to gain his or her
interest and to see whether the eyes move together. The examiner then covers
one eye with his or her hand and moves the toy to see if the child can focus
and track with the uncovered eye. A child who consistently objects to one eye
being covered should be suspected of having decreased visual acuity in the
uncovered eye and referred for evaluation.
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Q 7
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Q 7
At
a 4-month health supervision visit, a mother expresses concern that her infant
son does not see well. Birth and postnatal history are unremarkable. On
physical examination, you note horizontal nystagmus with jerking eye movement
when the child looks to the left. Occipitofrontal circumference is 43.0 cm
(75th percentile). Red reflex is normal, as are findings on brain magnetic
resonance imaging.
Of
the following, the MOST likely diagnosis is:
A. congenital
nystagmus
B. neuroblastoma
C. retinoblastoma
D. seizures
E. spasmus
nutans
Answer
A
Answer
A
Nystagmus
is a rapid, rhythmic oscillation of the eyes that usually is bilateral. It can
be a worrisome sign of serious eye or central nervous system pathology and may
warrant referral to a neurologist. Congenital nystagmus is characterized by
pendular nystagmus in primary gaze that becomes jerkier with lateral gaze, as
described for the infant in the vignette. Frequently, there is a null point at
which nystagmus is least and vision is best. Visual acuity often is diminished.
Typically the child’s distance vision is worse than near vision because
nystagmus is dampened with convergence. Congenital nystagmus may be associated
with ocular albinism and head titubation (bobbing). Onset of the disorder is
typically between 2 and 6 months of age. Many of these children will have
lifelong visual impairment.
Spasmus
nutans is a triad of nystagmus, head nodding, and head tilt. The nystagmus may
be quick and pendular (shimmering) and asymmetric. Onset is typically at 4 to
12 months, and it resolves by 4 years. Rarely, patients have an associated
optic pathway pilocytic astrocytoma.
Nystagmus
can be seen during a seizure, but it is absent interictally. Downbeating
vertical nystagmus is pathognomonic of disease at the cervicomedullary
junction, particularly a Chiari malformation. Upbeat nystagmus can be seen with
lesions in the low brainstem or cerebellum.
Opsoclonus
is distinct from nystagmus, lacking the latter’s rhythmicity and regularity.
Rather, it is characterized by irregular, chaotic, conjugate rapid eye
movements in all planes. It can be associated with encephalitis, ingestion of
toxins, or hydrocephalus, but the finding should trigger a search for occult
neuroblastoma. Retinoblastoma is characterized by leukocoria from the tumor
producing loss of the red reflex.
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