Med Study 5 Flashcards
(199 cards)
1
Q
What is a cholesteatoma?
A
= a lesion consisting of built of squamous epithelium and keratin trapped within the middle ear
needs to be surgically removed
2
Q
How does a cholesteatoma present?
A
painless otorrhea
often in a kid with ear tubes or history of AOM
3
Q
Why does a cholesteatoma need to be surgically removed?
A
Because if it is left untreated, it can cause hearing loss, vertigo, CN palsies, or even predispose to brain abscess
4
Q
Blank is an abnormal growth of squamous epithelium in the middle ear that often presents with otorrhea
A
cholesteatoma
5
Q
If you don’t take a cholesteatoma out it can
A
grow and destroy the ossicles
thus obviously causing hearing loss
6
Q
According to med study, by which age can most children walk up and down stairs with 1 foot on each step?
A
4 years
not til 4 years old wow ok
7
Q
By this age, most children can jump off the ground with 2 feet up
A
30 mo
8
Q
By this age, most children can skip
A
6
9
Q
The only type of vascular anomaly where the “misplaced” vessel courses between the trachea and the esophagus is…
A
a pulmonary sling
it’s where the LPA comes off the RPA, not what it’s supposed to do, and ends up coursing between the trachea and the esophagus
10
Q
If a barium swallow shows anterior indentation of the esophagus in an infant, the diagnosis is
A
a pulmonary sling
11
Q
What is DRESS?
A
= stands for drug reaction with eosinophilia and systemic symptoms
can be caused by viruses (HHV 6 and 7, EMV/CMV) or drugs (sulfonamides, antiseizure meds)
12
Q
Do you get eosinophilia in mycoplasma pneumoniae infection?
A
nope
13
Q
Parvovirus B19 causes a febrile exanthematous illness in young children known as
A
erythema infectiosum
14
Q
When adolescents or adults get Parvovirus B19 infection, they are likely to get this symptom
A
arthritis of the fingers
15
Q
Treat DRESS syndrome with
A
steroids
16
Q
A 14 yo girl presents with a 2 day history of a rash and swollen face. She also has a fever. Labs show eosinophilia. She started a new seizure med 6 weeks ago. You should suspect this diagnosis
A
DRESS syndrome
17
Q
Reactivation of blank virus may play a role in the development of DRESS syndrome
A
Human herpesvirus 6/7, CMV/EBV
18
Q
Reactivation of this virus is frequently seen in DRESS syndrome
A
Human herpesvirus 6
19
Q
What is papular acrodermatitis of childhood?
A
Also called Gianotti-Crosti syndrome
it’s a self limited, uncommon reaction that can happen as a response to some viruses or vaccines
pts get a symmetric, papular eruption
20
Q
Symmetric papules on the arms/face/butt on a 2 yo with a URI =
A
probably papular acrodermatitis
21
Q
Papular acrodermatitis usually spares the
A
trunk
22
Q
How do patients with Truncus Arteriosus present?
A
Because pulmonary blood flow is typically increased, they actually don’t always present with cyanosis right away, or only present with mild cyanosis. But then in the first few weeks, due to PVR falling and tons of pulmonary blood flow, they will quickly present with signs of HR (tachypnea, FTT, diaphoresis) and mild cyanosis. The heart will be hyperdynamic and the infant will have strong, bounding peripheral pulses.
23
Q
Exam in infant presenting at a few weeks of life found to have Truncus Artertiosus
A
The heart will be hyperdynamic and the infant will have strong, bounding peripheral pulses. Mild cyanosis, but may look more like a HF picture. Widened pulse pressure. Single S2 (only one valve to create the S2 sound!!) w/ejection click typically.
24
Q
Heart defects typically seen in DiGeorge syndrome:
A
TOF
interrupted aortic arch
VSD
Truncus Arteriosus
25
Absent thymus think
DiGeorge Syndrome
26
depression + liver failure + abnormal eye exam think
Wilson Disease
due to copper accumulation
27
psych symptoms, tremor, and elevated serum aminotransferases think
Wilson disease
copper accumulation
28
Aplasia cutis congenita think
Trisomy 13
(tho can also be an isolated defect)
29
In a child with NF1, what is the average age that they would present with an optic glioma?
age 4
30
A 4 yo presents with precocious puberty and 20 cafe au lait spots, all of which have smooth borders. Most likely diagnosis is
NF1
(In McCune-Albright syndrome, the cafe au lait spots would be fewer and would have irregular borders)
31
In children with NF1 who develop an optic glioma, how do they usually present?
vision loss
or precocious puberty
in a child under age 6 typically
32
Which vaccines are administered subQ?
MMR and varicella
33
If a child has a mild, acute illness with OR without a fever (like AOM or gastroenteritis), should you defer vaccines that day?
Nope, still give them. do NOT defer.
34
What is Moyamoya disease?
= a chronic disease where cerebral vessels stenose over time to the point where an individual often has an ischemic stroke
can be associated with SCD, Trisomy 21, NF1
35
Moyamoya means
"puff of smoke"--refers to all the extensive small collateral vessels seen on imaging as a result of narrowing of the major cerebral vessels over time
36
How do kids with Moyamoya disease present clinically?
with ischemic stroke
or new seizures
or TIA
or chronic headaches actually
37
On CT scan, blood is
bright
so a bleed will look hypERdense
38
HypOdensity on CT scan think
ischemic infarct
39
How to calculate midparental height for a girl:
(Mom's height + (dad's height - 5 inches) / 2
40
How to calculate midparental height for a boy:
((mom's height + 5 inches) + dad's height)/2
41
Tx for Chlamydia
doxy 100 mg BID x 7 days
42
How to treat Gonorrhea
Ceftriaxone 500 mg single dose IM
(use 1 g if pt is > 150 kg or 300 lbs)
43
What is metatarsus adductus?
= in-toeing present at birth that is caused by intrauterine crowding
resolves on its own within the 1st year of life
44
Metatarsus adductus occurs from
intrauterine crowding
45
In this condition in babies, the forefoot is adducted, and may be flexible or rigid
metatarsus adductus
needs referral to ortho if rigid
otherwise should resolve on its own
46
What is the most common cyanotic heart defect that presents with cyanosis in the immediate few hours after birth?
TGA
47
What is epidermolysis bullosa simplex?
= a type of epidermolysis bullosa that is genetic (AD), and is caused by defects in keratin. It presents at birth or soon after with bullae following only minor trauma.
Luckily, pts usually mostly grow out of it when they get older.
48
What is epidermolysis bullosa? What are the different types?
= a group of genetic diseases where there is a mutation in skin anchoring proteins, resulting in skin blistering and fragility
presents in the newborn
the types are: Epidermolysis bullosa simplex (most common and least severe), and then junctional epidermolysis bullosa and dystrophic epidermolysis bullosa (both very severe)
49
wood lamp is used to search for
fungi
50
If you suspect that an infant has epidermolysis bullosa, you should get this test to determine the diagnosis
need a skin biopsy
51
If a mom has mastitis can she still breastfeed from the affected breast?
yes
unless horrible abscess with visible pus
otherwise, apparently fine to continue
52
What is alopecia areata?
= an autoimmune process where the immune system attacks the hair follicles
presents with sudden onset round or oval patches of hair loss on the scalp
associated with other autoimmune diseases
53
The most common cause of dysphagia and food impaction in children is
EoE
54
"Snowman sign"
= a rounded, figure 8 like cardiac contour with a large supracardiac shadow in the mediastinum
seen in TAPVR and PAPVR (partial anomalous pulmonary venous return), where apparently there is also a left sided SVC (in addition to the right sided SVC), creating the snowman sign
FYI--can be hidden in a neonate by the thymic shadow
55
What is L-TGA?
it's where the morphological RV/pulm artery and LV/aorta are switched
so like morphologically, RA is attached to LV
but blood circulation pathway is normal
babies and kids can be asymptomatic it sounds like
56
Straight left heart border on CXR think
L-TGA
57
Classic CXR finding in patients with supracardiac anomolous pulmonary venous return
snowman sign
58
"discordant atrioventricular and ventriculoarterial relationships" =
L-TGA
59
When the RV is where the LV should be and the LV is where the RV should be but everything else is ok, that's
L-TGA
60
Downward displacement of an abnormal tricuspid valve into the right ventricle is found in
Ebstein anomoly
61
Massive cardiomegaly-- a "wall to wall heart" on CXR in a neonate with cyanosis think
Epstein anomaly
62
Anterior deviation of the infundibular septum describes the anomaly seen in which types of congenital heart disease?
TOF
63
CXR findings in Truncus Arteriosus
cardiomegaly and increased pulmonary vascular markings with pulmonary edema
64
Narrow mediastinum with egg shaped heart on CXR, or "egg on a string" appearance =
D-TGA
65
Common cyanotic congenital heart disease with LVH and left axis deviation:
Tricuspid atresia
66
Supracardiac TAPVR will present with blank on CXR
snowman sign
67
What is the ONE cyanotic congenital heart disease that gets WORSE when you start prostaglandins?
The infradiaphragmatic form of TAPVR when there is severe obstruction to pulmonary venous return
the neonate will have severe pulmonary edema and cyanosis
(Can probs think of as increases R to L shunt?)
68
How do neonates with TAPVR present?
It will depend on how obstructed the pulmonary venous return is. If not too obstructed, they might actually do ok and get elective surgery. IF severely obstructed, super bad lung white out and cyanosis.
69
Auscultation findings in neonate presenting with HLHS
no murmur
single, loud S2
70
A random plasma glucose > blank, along with the classic symptoms of diabetes meets criteria for a diagnosis of DM
>200
71
The first screening test to use to look for a complement deficiency is
the CH50 assay
(this measures total complement activity of the classic pathway, and a normal result would show that all factors, C1-C9, are present)
(If CH50 is low or zero, then do tests looking at each specific complement level)
72
Name two deficiencies that are associated with fulminant meningococcal disease
Terminal complement deficiency (C5-C9 deficiency)
Properdin deficiency
73
Early complement deficiencies refer to a deficiency in
C1,2,3 or 4
74
Symptoms of an early complement deficiency
pyogenic infections from encapsulated bacteria (aka neisseria meningitidis, strep pneumo, and h flu) PLUS tendency to have autoimmune diseases, such as lupus
75
Why are early complement deficiencies associated with also having autoimmune disease?
Because without complement functioning properly, immune complexes end up being deposited in different organs and joints
76
Early complement deficiencies are associated with autoimmune diseases, especially
Lupus
77
Lupus is especially associated with this complement deficiency
C2 complement deficiency
78
What is properdin?
= a positive complement regulator
so if you are deficient, you are immune compromised
Properdin deficiency is a rare, hereditary, primary immunodeficiency due to a complement cascade protein anomaly characterized by significantly increased susceptibility to Neisseria species infections.
79
MOA of montelukast
leukotriene receptor antagonist
80
The iliotibial (IT) band goes from where to where?
lateral hip to the lateral lower femoral condyle and lateral aspect of the proximal tibia, just below the knee
81
What is iliotibial band syndrome (also called iliotibial band friction syndrome)?
= when the IT band gets inflamed as it passes over the lateral distal femoral condyle
presents with lateral knee pain DURING activity, commonly seen in athletes like long distance runners
82
According to med study, if a sickle cell pt has osteomyelitis, the organism causing it is most likely to be
salmonella
83
Most common cause of osteomyelitis in sickle cell patients
salmonella
84
Psoriasis is typically present on blank surfaces
extensor
like elbows and knees
85
A 5 yo with a known metabolic disorder is started on 500 mg/day of pyridoxine. What metabolic disorder do they have, and how can you assess response to treatment?
Homocystinuria
assess response to tx by monitoring homocysteine levels
86
Finding alloisoleucine is diagnostic for this metabolic disease
Maple Syrup Urine Disease
87
In Maple Syrup Urine Disease, levels of blank, blank and blank are elevated
leucine
isoleucine
valine
88
Blank is a metabolic disorder where methionine metabolism is disrupted and not able to happen normally
Homocystinuria
89
In homocystinuria, these two levels are elevated
homocystine level, duh
and methionine level
since the whole problem with this disease is that you can't break down methionine normally
90
How to test a child for homocystinuria if you suspect this clinically:
homocysteine level
and amino acids in plasma (to look at methionine level)
91
Tx for homocystinuria
LARGE doses of pyridoxine (B6)
because, pyridoxine causes a decrease in the total plasma level of homocysteine
+ also will need to eat low methionine diet (methionine gets broken down into homocysteine)
92
Methionine (a dietary amino acid) normally gets broken down to homocysteine in the body, which is then converted to cystathionine and then cysteine. In the metabolic disease homocystinuria, which step in this process is not able to happen?
Can't convert homocysteine to cystathionine, because the enzyme Cystathionine B-synthase is missing.
so pts get elevated levels of homocysteine and methionine
93
Marfan like habitus + downward lens dislocation + blood clots think
Homocystinuria
94
Homocystinuria pattern of inheritance
AR
95
Elevated methionine and homocystinuria levels think
Homocystinuria
96
Tx for Homocystinuria
Pyridoxine (vitamin B6)
bcuz this helps convert homocysteine to cysteine (basically it enhances the activity of the deficient enzyme)
97
If a patient with homocystinuria does not respond to pyridoxine supplementation, what should you do?
give folic acid and B12
bcuz the pt won't respond to pyridoxine if they are folate deplete
98
A child has impetigo caused by strep. They then get post strep (post-infectious) glomerulonephritis. Which antibody will tell you if indeed they recently had a SKIN strep infection?
Antibodies to deoxyribonuclease B
(anti-deoxyribonuclease B = anti DNase B for short)
99
This antibody will be positive after a recent strep infection of the SKIN
Anti-deoxyribonuclease B
(this is the most sensitive and specific)
100
Syndactly =
webbed fingers or toes
101
Meds you an use to treat an acute dystonic reaction
benztropine or benadryl
102
Patients with Type 1 diabetes have this HLA type
HLA-DQ2-DQ8
103
Do patients presenting with celiac have an elevated CRP?
Nope not always
104
The best initial screening test for Celiac is
Serum tissue transglutaminase (tTG)-IgA antibodies WITH total IgA
105
Pattern of distribution of Dermatitis herpetiformis (celiac rash):
symmetrically, on the extensor surfaces of the arms, legs and butt
vesicular, itchy
106
How do you test for celiac in a child under age 2?
tTG IGA and total IgA
AND DEAMINATED gliadin IgG
107
Is serum gliadin IgG a good test to screen for Celiac?
nope
bcuz it's not sensitive or specific
however, deaminated gliadin peptide IgG is useful when testing children < 2 yrs of age
108
CO MOA of poisoning
binds super tight, way more tightly than oxygen does, to heme and impairs oxygen delivery
109
Cyanide MOA of poisoning
cyanide interferes with oxygen utilization by mitochondria
110
In cyanide poisoning, what will a venous blood gas show?
the venous blood gas will be bright red
like it will look arterial
and the O2 content will be high (even of the venous blood), because the O2 is not getting extracted at the capillary level
not good
111
2 ways someone might get cyanide poisoning
smoke inhalation, or a nitroprusside drip (bcuz nitroprusside is metabolized to cyanide)
112
How to treat cyanide poisoning
patients will be VERY sick with severe lactic acidosis
treat with O2, hydroxocobalamin (a precursor of vitamin B12), or sodium thiosulfate. can also add amyl nitrite. okk.
113
What happens to total body water, intracellular water, and extracellular water as a kid goes from newborn to 10 years old?
Total body water decreases, from like 80% as a preemie to 70% as a term baby to 60% of body weight as a 10 year old.
Extracellular water also decreases, like majorly. Babies are born with a lot of extracellular water. Then that decreases.
INTRACELLULAR WATER however INCREASES with ages. Think more cells and cells grow and a bigger PERCENTAGE of total body water is intracellular in a 10 yo compared to an infant.
114
Is more of the body's fluid intracellular or extracellular?
Intracellular (2/3)
extracellular is only 1/3 (in an adult. it's higher in a baby).
115
What is Scheuermann kyphosis?
= when 3 verebrae in a row have endplate abnormalities with anterior wedging in the thoracic spine
cause is unknown
presents in teenage boys with back stiffness and kyphosis that the patient can't correct
116
Does alcohol increase your risk of kidney stones?
Nope!
117
Do coffee and tea increase your risk of kidney stones?
Nope!
118
The most important steps for preventing kidney stones are:
1. generous fluid intake
2. Decreased salt intake
3. treat with a thiazide diuretic (like hydrochlorothiazide) when needed to decrease the amount of calcium in the urine
119
Most kidney stones are made of calcium oxalate. Should people with kidney stones eat less calcium?
Nooo!!! They should actually eat MORE calcium! Eating more calcium will make their GI tract absorb less oxalate, so will actually decrease the incidence of kidney stones.
120
The problem with eating too much supplemental vitamin C is .....
it can cause kidney stones
because vitamin C increases the amount of oxalate in the urine, predisposing you to the formation of calcium oxalate stones
121
RsR' means
volume overloaded RV
seen in a kid with an ASD
122
Acute angle closure glaucoma is a rare but dangerous side effect of this antiepileptic drug
Topiramate
123
How does acute-angle closure glaucoma present?
Eye pain, swelling and redness
124
Side effects of Topiramate
fatigue
psychomotor slowing
acute close angle glaucoma yikes
nephrolithiasis (kidney stones)
125
What is X-linked adrenoleukodystrophy?
= disease that presents in boys around age 7, with ADHD like symptoms/ seizures/ loss of gait and vision and then death over the course of a few years, due to the accumulation of very long chain fatty acids in the white matter of the CNS.
126
What x-linked disease is caused by the body's inability to break down very-long-chain fatty acids in peroxisomes, leading to toxic accumulation in the adrenal glands and the brain?
adrenoleukodystrophy
127
Imaging findings in a boy presenting with seizure, found to have new diagnosis of adrenaleukodystrophy
symmetric periventricular demyelination in the posterior regions of the cerebral white matter
128
Tx for dilated cardiomyopathy in childhood:
diuretics
ACE inhibitors
129
Should a 7 yo get Tdap or Dtap?
Tdap
!
130
Hib is not recommended for healthy children > blank years of age
5
specifically, 60 months
131
If a child who has never gotten any vaccines who is now 5 years old or older and has asplenia or HIV, should they get a Hib vaccine?
yes, one dose
132
A child has a phyisican-reported history of chicken pox, do they need the varicella vaccine?
nope!!
133
A 7 yo has never received any vaccines. They are previously healthy. What vaccines should you give them?
MMR, followed by a 2nd dose at least 1 mo later.
IPV (polio) series.
Hep A and Hep B.
134
Sotos syndrome
aka "cerebral gigantism"
patients are big and tall (>97th percentile) until age 5, when growth normalizes
often have low IQ and behavioral issues and are clumpsy
these children apparently speak in a monotone voice
it's a spont mutation genetic condition. does NOT have any endocrine abnormalities.
135
blank is a genetic syndrome where there is rapid growth in early childhood, but no evidence of an endocrine disorder
Sotos Syndrome
136
Growth hormone levels in Sotos syndrome
normal
137
If a fetus is exposed to this drug in utero (specifically between 6 and 9 weeks gestation), they get nasal hypoplasia and stippled epiphyses
Warfarin
138
What are stippled epiphyses?
A congenital abnormality of the epiphyses marked by multiple ossification centers that severely deform the long bone and give it a stippled appearance and a thickened shaft.
139
Fetal Warfarin Syndrome
nasal hypoplasia and stippled epiphyses
140
What are ash leaf spots and what should they make you think of?
= hypopigemted macules
seen in Tuberous Sclerosis
141
If a patient seizes and you find 3 or more ash leaf (hypopigmented) spots on their skin, they probably have this diagnosis
Tuberous Sclerosis
142
Are scorpion bites visible?
no, they leave no mark!
yikes
can be tender, but won't be visible
143
Necrosis, ulceration and eschar formation at the site of the bite happens with this type of spider
brown recluse spider
144
What is Smith-Lemli-Opitz syndrome?
= an AR disorder of cholesterol metabolism
Due to deficiency of 7-dehydrocholesterol reductase, causing an increase in plasma 7-dehydrocholesterol and a decrease in plasma cholesterol.
Babies have dysmorphic facial features, abnormal thumbs, overlapping fingers, syndactly, and ambiguous genitalia. This all happens because cholesterol is very important to embryogenesis, so without cholesterol you do not get normal embryogenesis.
145
2/3 toe syndactly think
Smith-Lemli-Opitz Syndrome
146
A newborn has microcephaly with narrow bifrontal diameter, cleft palate, hypospadias, and 2/3 syndactly. What syndrome does this baby have, and what lab abnormalities will you see?
Smith-Lemli-Opitz Syndrome
low total cholesterol
elevated 7-dehydrocholesterol
147
Glycogen storage disease type 1 is due to a deficiency of one of these two enzymes
glucose-6-phosphatase
glucose-6-phosphatase translocase
the problem is you can't break down glycogen
148
If you do a study where you divide groups by outcome and then compare the risk factors that exist between the two groups, that is a blank type of study
case-control study
149
In a blank study, the subjects are divided into groups based on the presence or absence of risk factors, and then are followed (either in the past or prospective follow in real time) to see if certain outcomes (like a certain disease) develops.
cohort study
150
In a study, if patients are grouped by outcome, that is a blank type of study
case-control
151
"Meth mouth" refers to
dry mouth leading to tooth decay in meth users
152
How to distinguish epiglottitis from bacterial tracheitis:
epiglottitis: drooling and difficulty swallowing from severe sore throat. would not have a prominent cough.
bacterial tracheitis: does have a prominent cough. is like viral croup presentation but then the child rapidly deteriorates.
both will have fever, stridor and resp distress
153
Epligottitis prominent symptom
drooling
154
Bacterial tracheitis prominent symptom
croup like cough
155
What is cutaneous larva migrans?
in Brazil it's called "geographic bug" "bishu geographico" because it looks like a map, the red serpiginous lines that you get on your feet
it's caused by dog and cat hookworms
often get when walking barefoot, like on the beach
treat with albendazole
156
Serpiginous map like red itchy rash that you get on your feet after walking barefoot at the beach =
Cutaneous larva migrans
aka dog and cat hookworm (ancylostoma braziliense, A. caninum)
157
Treat hookworm infections with
albendazole or pyrantel pamoate
158
How to treat cutaneous larva migrans (a type of hookworm infection)
albendazole or ivermectin
hookworm
think footworm
to help remember the rash on the feet
159
A 20 mo girl presents with regression of both motor and language milestones, stereotypic hand movements, and deceleration of head growth, all consistent with blank syndrome
Rett syndrome :(
160
Why are patients with Rett syndrome at higher risk for sudden cardiac death than the general population?
Becuase in Rett syndrome, there is abnormal regulation of the autonomic nervous system, and this leads to QTc prolongation and cardiac electrical instability.
161
Which drugs are known to decrease the efficacy of OCPs?
Antiseizure meds-- like:
carbamazapine
lamotrigine
oxcarbazepine
phenytoin
phenobarbital
topiramate
AND Rifampin and rifabutin (abx).
162
Which antibiotics are the only antibiotics known to decrease the effectiveness of OCPs?
Rifampin and rifabutin
163
Steeple sign on xray in croup is due to narrowing of what?
subglottic narrowing
164
Loss of normal shoulders of the subglottic airway refers to.....
subglottic narrowing seen in croup
aka steeple sign
165
When should babies with cryptorchidism (aka undescended testes) be referred for surgical consultation?
6 mo actually
166
When should babies with cryptorchidism (aka undescended testes) be referred for surgical consultation?
6 mo actually
167
Undescended testes have a risk of
cancer
and like a MINIMALLY increased risk of infertility
but the major issue is the increased risk of cancer
168
Are retractile testes fine?
yes
will almost always end up in the scrotum so no intervention is necessary
169
PCP (Phencyclidine) toxidrome
= a hallucinogen
causes hyperreflexia, clonus, muscle rigidity, nystagmus, and drooling. Also seizures.
Causes temp and BP instability. Pts can become acutely psychotic and have frightening hallucinations.
170
Seeing smells and hearing colors think
LSD
artsy
think Lucy in the Sky with Diamonds
171
MDMA is known as
Molly or Ecstasy
172
How to distinguish PCP toxidrome from MDMA
PCP has nystagmus
(MDMA doesn't)
173
Perioral eczematous changes think
inhalants
174
Patients with this inherited disease have recurrent skin infections, pneumonias, and episodes of lymphadenitis caused by catalase positive organisms
CGD (chronic granulomatous disease)
ex of a catalase positive organism: staph aureus
175
Hereditary defect where you can't form reactive oxygen species =
CGD
176
Patients who get pneumonias and skin infections caused by catalase positive organisms think
CGD
177
What disease is due to a failure of the patient's phagocytic leukocytes to kill pathogens using the oxidative burst?
CGD
NADPH oxidase is defective in these patients
they can form granulomas around pathogens but then can't do anything else
178
Why are CGD patients prone to infections with catalase positive organisms? Name some catalase positive organisms.
Staph aureus
Nocardia
Aspergillus
Burkholderia cepacia
Serratia
Salmonella
179
The test of choice to determine if a patient has CGD is
the Dihydrorhodamine oxidation test
"DHR" oxidation test
180
A young child has recurrent otitis media, draining ears, eczema, and FTT. The diagnosis is likely....
Wiskott Aldrich Syndrome
181
Why do CGD patients get infections over and over again with catalase POSITIVE organisms, but do fine with catalase negative organisms?
It is believed that their phagocytes can use the hydrogen peroxide/oxidative burst materials supplied by catalase neg bacteria. Can't do this with catalase positive bacteria, since catalse I think must like destroy hydrogen peroxide.
182
Name 3 vaccines that can be administered subQ:
MMR
varicella
IPV
183
How to treat a patient with idiopathic hypercalciuria:
The patient should be treated, to avoid the risk of developing nephrocalcinosis (calcium building up in the kidneys which can eventually cause kidney failure). Tx means:
-push fluids
-limit sodium intake
-watch for signs of kidney stones
ok, cool...
184
What is rosacea?
= a chronic disorder where you get facial redness, often with a papulopustular component
temp changes, exercise, sun, alcohol, spicy foods, hot beverages, all trigger the redness
it's etiology is unclear. most likely to occur in fair skinned individuals.
185
How to treat papulopustular rosacea
topical metronidazole
186
Bones with "ground glass" appearance on xray think
Scurvy
187
Bone pain, widening of the wrist and knees, and bowed legs think
Rickets
188
Lucent metaphyseal band think
Syphillis
189
What is Treacher Collins syndrome?
Also called mandibulofacial dysostosis (meaning defective bone). You get midface hypopasia, choanal stenosis or atresia, cleft palate, hypoplastic lower eyelids and ABSENT lower lid lashes. Also get outer and middle ear malformations, so conductive hearing loss is common.
190
Choanal stensis, midface hypoplasia, downward slanting eyes, and absent lower eyelid lashes think
Treacher Collins Syndrome
191
Shagreen patches
= fleshy, irregular plaques often found on the lower back in patients with Tuberous sclerosis
192
Options for prophylaxis for neisseria meningitis for close contact:
CTX once
Rifampin BID x 2 days
or
Cipro once (can be used in pts > 1 month of age)
193
Kallman syndrome is frequently associated with what other defects?
midface defects like cleft lip and palate
also congenital heart disease and renal agenesis
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What is acrodermatitis enteropathica?
= an AR disorder where you can't absorb zinc from the GI tract
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Genetic disease where zinc is not adequately absorbed
Acrodermatitis enteropathica
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How does acrodermatitis enteropathica present?
In this rare AR disorder, the patient's GI tract is very bad at absorbing zinc.
However, breastmilk contains the missing zinc-binding factor necessary for absorption, so infants who are breast feeding will do just fine.
When they switch to formula, a few weeks later they will present with severe zinc deficiency: irritability, new FTT, diarrhea, and rash.
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How to treat Acrodermatitis enteropathica
give life long zinc supplementation
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Best treatment for comedones in acne
topical retinoids
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Which type of med is particularly effective for comedonal acne?
retinoids
