Prep 2 Flashcards
Why are there muddy brown casts in the urine in ATN?
because in acute tubular necrosis tubular cells necrose, and as part of the process red cells leak into the tubule as a response to the injury… and so then these dead tubular cells with hemoglobin look a muddy brown color
Muddy brown casts and epithelial casts, mild pyruia and mild proteinuria think
ATN
Is the urine concentrated or dilute in ATN?
dilute
and you are also losing lots of sodium in the urine
the kidenys basically just stop doing their job
Treatment for ATN
Supportive
Type of casts seen in acute glomerulopnephritis
red blood cell casts
along with hematuria, microscopic or gross
What is BP like in acute glomerulonephritis?
high. you get hypertensive.
The cause of acute interstitial nephritis is usually blank. And the associated symptoms you get with it are….
caused by an allergic reaction to a drug
will get associated fever, rash, and eosinophilia
Urine eosinophils think
acute interstitial nephritis
UA in acute instertitial nephritis
will show…
white cells
red cells
a little protein
and EOSINOPHILS
hemolytic anemia, thrombocytopenia and renal failure =
HUS
How to remove an aspirated foreign body
rigid bronch
A very very thin teen gymnast female has a low free T4, but normal TSH. What does she have?
euthyroid sick syndrome in the setting of being in a negative energy balance state and way, way too thin
Define primary amenorrhea
= no period by age 15
What is euthyroid sick syndrome?
It’s a physiologic adaptation to conserve energy when living in a negative energy balance state
you will have a normal TSH but low free T4
Blank is a condition where serum levels of free T4 are low in patients who have nonthyroidal systemic illness but who are actually euthyroid.
Euthyroid sick syndrome
Delayed puberty in girls is defined as
lack of breast development by age 13
Permanent hypoigonadotropic hypogonadism + anosmia =
Kallman syndrome
Hypergonadotropic hypogonadism is the result of
primary gonadal failure
Name some causes of hypergonadotropic hypogonadism
Turner syndrome (streak, dysfunctional ovaries)
Gonad injury for radiation or chemo
Klinefelter syndrome
Define delayed puberty in boys:
lack of testicular growth to 4 mL (2.5 cm in length) by age 14
Name some causes of hypogonadotropic hypogonadism
eating disorder
excessive exercise
systemic illness
Name the 4 categories of delayed puberty:
normal variant constitutional delay
hypogonadotropic hypogonadism
hypergonadotropic hypogonadism
Are x-rays good at picking up femoral neck stress fractures?
NO
Femoral neck stress fracture is a can’t miss diagnosis, but x-ray will usually be normal so you need an MRI
Who usually gets femoral neck stress fractures
teenage girls who do running sports
How to diagnose a femoral neck stress fracture
MRI
The danger of missing a femoral neck stress fracture
If the patient keeps walking on it, can progress to a complete hip fracture
What type of injury should be considered in all female athletes with groin pain?
femoral neck stress fracture
Initial management for teens where you suspect a possible femoral neck stress fracture:
crutches (do NOT bare weight)
MRI
if it’s a lateral stress fracture, will need surgery
if medial, will need prolonged non weigh bearing and gradual return to normal walking and sports as long as remains pain free
When should healthy children start having their blood pressure checked, and how often should it be checked?
at each annual WCC starting at age 3
Who should have blood pressure screenings start before age 3?
obese children
ALL congenital heart disease (even if fully repaired)
SGA babies
VLBW babies
ANYONE who had an umbilical line
complicated nicu course
sickle cell disease
renal disease or FH
prematurity LESS than 32 weeks
Blank is characterized by infrequent, brief focal motor seizures that occur predominantly during sleep
benign rolandic epilepsy
What is benign rolandic epilepsy?
= a common childhood epilepsy where kids get focal seizures (often during sleep) that involve facial twitching, speech arrest, hypersalivation, and face/arm clonic movements. Kids will actually have preserved consciousness and remember these seizures.
some kids with BRE also will have generalized tonic clonic seizures
usually outgrow by age 16
EEG in benign rolandic epilepsy will show
biphasic independent bilateral centrotemporal spikes, often during light sleep
normal background
Juvenile myoclonic epilepsy is characterized by this triad of symptoms:
- myoclonic seizures in early morning
- generalized tonic clonic seizures
- absence seizures
persists into adulthood
What is a myoclonic seizure?
= brief, shock like jerks of a muscle or group of muscles
Panayiotopoulous syndrome
= a childhood focal epilepsy syndrome that presents between ages 1-4
these toddlers/young kids will get infrequent autonomic seizures (for ex, ictal vomiting, cyanosis, pallor….) while awake. seizures can be long and can evolve into status.
kids usually outgrow.
Panayiotopoulous syndrome
= a childhood focal epilepsy syndrome that presents between ages 1-4
these toddlers/young kids will get infrequent autonomic seizures (for ex, ictal vomiting, cyanosis, pallor….) while awake. seizures can be long and can evolve into status.
kids usually outgrow.
Most common sites for pedi IO:
proximal tibia (go slightly medial)
distal femur
Who usually gets an RPA? (retropharyngeal abscess)
kids ages 2-4 who have just had a URI
What will a lateral neck radiograph show in a kid with an RPA?
edema of the prevertebral soft tissues
(basically edema right behind the pharynx – hence the name retro pharyngeal abscess)
RPAs are usually polymicrobial, however the PREDOMINANT pathogens are…..
strep pyogenes (group A strep)
staph aureus
and respiratory anerobic bacteria, like peptostreptococcus, fusobacterium, and prevotella
ok so mainly remember: GAS and staph aureus
Name 3 bugs that do NOT cause RPAs:
strep pneumo
pseudomonas
morexlla
these bugs do lots of other things, but they do NOT cause RPAs
Major 2 bugs that cause RPAs
GAS and staph aureus
Precautions for mumps should be airborne, droplet or contact?
droplet
Name the 4 disease/bugs that required airborne precautions:
covid obviously
TB
measles
varicella
Precautions needed for neisseria meningitidis
droplet
how is mumps spread
respiratory droplets
Marked hypotonia and poor suck and swallow in the first few days of life in a term neonate think
Prader-Wili syndrome
There is a neonate in the NICU with multiple anomalies but they are not suggestive of any one specific genetic syndrome. In this case, the best first line genetic test to do is:
a CMA to look for deletions or duplications anywhere across all of the chromosomes
(CMA = chromosomal microarray)
What is a chromosomal microarray?
= a genetic test with high resolution that will look for “copy number changes” (aka either duplications or deletions) across the entire genome
FISH
= fluorescent in situ hybridization
this genetic test is good for when you suspect a specific genetic disorder, such as 22q11.2 deletion syndrome (DiGeorge syndrome)
rapid turn around time of 48 hours
basically uses fluorescence to detect the presence or absence of a specific region of the genome
so you have to know what you are looking for
The recommended first-line test for multiple congenital anomalies not suggestive of a specific syndrome is
CMA (chromosomal microarray)
this is also the recommended first line genetic test for autism and unexplained, non-syndromic developmental delay
What test will look for deletions or duplications anywhere along the entire genome?
a chromosomal microarray
How doe a CMA (chromosomal microarray) work?
the patient’s DNA is put onto a microchip and the computer analysis is used to compare the patient’s genetic material to that of a reference sample
(there is more to it but this is the basic idea – this his how theoretically any and all deletions or duplications that a patient might have can be picked up)
Best genetic test to order first when evaluating a child with intellectual disability of unclear cause
chromosomal microarray
Genetic tests that can be used to diagnose Williams Syndrome (which is caused by a 7q11.23 deletion)
either a chromosomal microarray (which would detect deletions or duplications anywhere)
or FISH using a probe targeted to 7q11.23 if you have a very strong clinical suspicion
Can a chromosomal microarray detect a balanced translocation?
no
Is whole exome sequencing ever first line?
no
Turn around time for whole exome sequencing
3-4 months
yikes that is a longg time
How does whole exome sequencing work?
the test sequences protein-coding regions of all genes to identify disease-causing change in the proband
Proband
= first person with a genetic condition in a family
or the person serving as the starting point
Fascination with water think
Angelman syndrome
MECP2 mutation causes this x-linked syndrome
Rett syndrome
According to PREP, should a 6 mo intubated bronchiolitic be fed via NG tube or NJ tube?
NJ due to “risk of aspiration”
OK PREP sure.
Which type of abuse is MOST likely to be associated with attachment disorder/inability for the child to form normal emotional connections
psychological abuse
According to PREP, the only time you should use a CT scan to diagnose appendicitis in peds is
when the ultrasound results are indeterminant
or you suspect an abscess
People with G6PD deficiency should not eat
fava beans
this will cause oxidative stress that their red cells can’t handle and they will get increased hemolysis and jaundice
According to PREP, when does physiologic jaundice peak?
at 4-5 days of life
In this disease, reduced glutathione is not available to stabilize RBCs in times of oxidative stress, leading to hemolysis and elevated bilirubin levels
G6PD deficiency
aka glucose-6-phosphate dehydrogenase deficiency
This condition results from the inhibition of bilirubin uridine diphosphate glucuronosyltransferase
breast milk jaundice
Why should you avoid bactrim under 2 months of age?
because bactrim intereferes with hepatic conjugation and increases the risk of hyperbili
nitrofurantoin does the same, so also avoid it under age 2 mo
Which babes/toddlers/children with vesicoureteral reflux should be on antibiotic prophylaxis to help prevent UTIs?
anyone with grade 3 reflux or higher
any grade VUR in a baby/toddler who is not yet toilet trained
or low grade VUR but with recurrent UTIs
Preferred drug for prophylaxis against UTIs in children with VUR
bactrim
becuase it is bacteriocidal
should still use bactrim even if child has a history of a previous UTI that was resistant to bactrim
If an infant under 2 months needs prophylaxis against UTIs due to having VUR, which drug should you use?
amox
Oral drug of choice for treating cellulitis caused by mammal (dog/cat) bite
Augmentin
Can you give doxy to a child under 8? Why or why not?
NOPE
BEcause of risk of dental enamel hypoplasia and yellow tooth discoloration
Don’t use doxy in children under age
8
How to treat a cellulitis surrounding a dog bite in a child who is allergic to penicillin:
clinda + bactrim
or
clinda + 3rd gen cephalosporin
Which kids and teens should be screened for prediabetes and type 2 diabetes?
-those who are overweight (aka BMI 85th percentile or higher) who also have at least 1 other risk factor (for ex, signs of insulin resistance on exam, FH of type 2 diabetes, or maternal history of gestational diabetes when pregnant with this patient)
Screening should start at age 10 or after puberty has begun, whichever comes first.
How to screen adolescents for prediabetes and type 2 diabetes (like which tests):
-fasting plasma glucose
-2 hour plasma glucose during a 75 g oral glucose tolerance test
-or a Hb A1C
Clinical signs/conditions associated with insulin resistance:
PCOS
acanthosis nigricans
hypertension
dyslipidemia
SGA
Normal fasting plasma glucose result
< 100
(100-125 is prediabetes; 126 and up is diabetes)
Normal Hb A1c
< 5.7%
5.7-6.4% is prediabetes
6.5% and up is diabetes
What is Pityriasis alba?
= hypopigmented macules with scale that represent a post-inflammatory state, aka they often follow eczema
treat with emollient or low potentcy topical corticosteroid
lesions are NOT sharply demarcated
Is topical triamcinolone low, mid or high potency?
mid
Blank is a form of postinflammatory hypopigmentation that occurs in children who have a history of atopic dermatitis
Pityriasis alba
What is Tinea versicolor?
= a fungal infection caused by the Malassezia species that causes hypo or hyper pigmented lesions
the lesions have WELL defined borders
and it actually is NOT common in kids
Describe the lesions in vitiligo
depigmented macules and patches with WELL DEFINED sharp borders
1st line tx for vitiligo
= topical corticosteroid or a calcineurin inhibitor
(other txs also exist tho– like UV B light therapy, and more)
How to treat pityriasis alba
Treatment of pityriasis alba is with an emollient or a short course (7 days) of a low-potency topical corticosteroid.
HBIG is most effective if given when
within the first 12 hours following birth
Who should get HBIG?
infants born to moms who are Hep B positive OR who are Hep B unknown
A premature infant is born weighing 1.8 kg to a hep B negative mom. When should this infant get the Hep B vaccine?
at 1 month of age or when being discharged, whichever comes first
(don’t give at birth if infant is < 2 kg)
What to do if an infant is born to a Hep B positive mom
give the HBV vaccien and HBIG within 12 hours
do this no matter what the size or GA of the baby
A term baby weighing 3.5 kg is born to a Hep B negative mom. The standard of care is for this infant to receive the Hep B vaccines within what time frame?
Within the first 24 hours of life
How to manage an infant born to a mom who is Hep B unknown:
It depends on whether the infant is > 2 kg or not.
If > 2 kg, give HBV vaccine right away but ok to wait for mom’s Hep B test to come back, as long as HBIG is given within 7 days
but for an infant < 2kg, no matter what if mom is unknown then HBIG has to be given within 12 hours
Decreased FVC (forced vital capacity) represents
restrictive lung disease
FEV1 and FEV1/FVC are measures of
airflow
so if they are decreased, it would indicate obstruction
A normal FEV1/FVC in kid is at least
80%
The most common malformation that accompanies a myelomeningocele is
a chiari 2 malformation
Symptoms of a chiari 2 malformation:
swallowing difficulties
breat holding spells
apnea
stridor
hoarse voice
choking
or, can be asymptomatic
Drug to give ASAP if you suspect malignant hyperthermia post anesthesia
dantrolene
Dantrolene MOA
stops muscle contraction
so is used to treat malignant hyperthemia
What is Hurler syndrome?
It’s a lysosomal storage disorder. Also known as mucopolysaccharidosis type 1.
Infants are completely normal at birth, but then have gradual progression of coarse facial features, global developmental delay, progressive intellectual disability, hearing loss, corneal clouding and hepatosplenomegaly.
What are mucopolysaccharidoses?
= lysosomal storage disorders where there is accumulation of glycosaminoglycans in multiple tissues and organs
For ex– Hurler syndrome (AR) and Hunter syndrome (x-linked recessive) are lysosomal storage disorders/mucopolysaccharidoses
The main function of lysosomes is to
digest non functional cell materials and debris
What are the mucopolysaccharidoses?
= a certain kind (group) of lysosomal storage disorders
In mucopolysaccharidoses, an enzyme is missing in the lysosomes that is needed to break down glycosaminoglycans, which are long changes of sugars/carbs
What are lysosomal storage disorders?
= inherited metabolic diseases where there is abnormal build-up of various toxic chemicals, becuase the lysosome can’t get rid of them.
3 types:
1. Lipidoses (can’t break down lipis)
2. Mucopolysaccharidoses (can’t break down long chains of sugars/carbs, aka glycosaminoglycans)
3. Sphingolipidoses (can’t break down sphingolipids)
*sphingolipid = a lipid attached to something else, like a carb, it sounds like
Name two mucopolysaccharidoses:
Hunter syndrome
Hurler syndrome
A 2 year old boy is having TONS of URIs, has coarse facial features, contractures of hte hands, a gibbus deformity (kyphosis of the spine), hepatomegaly, FTT and corneal clouding on exam. What is his diagnosis?
Hurler syndrome
A 2 year old boy is having TONS of URIs, has coarse facial features, contractures of hte hands, a gibbus deformity (kyphosis of the spine), hepatomegaly, FTT and corneal clouding on exam. What is his diagnosis?
Hurler syndrome
How to differentiate between Hurler syndrome and Hunter syndrome:
children with Hunter syndrome do NOT have corneal clouding, and have better cognitive outcomes
Mucopolysaccharidosis type 1 is also called blank. Its pattern of inheritance is blank.
Hurler syndrome
AR
Mucopolysaccharidosis type 2 is also called blank. Its pattern of inheritence is blank.
Hunter syndrome
x-linked recessive
standard of care for severe Hurler syndrome
stem cell transplant
but doesn’t fix everything
also give enzyme replacement therapy
Skeletal manifestations of Hurler syndrome
Gibbus deformity (thoracolumbar kyphosis)
joint contractures
In Hurler syndrome and Hunter syndrome, these two glycosaminoglycans build up in various tissues and organs over time
dermatan and heparan sulfate
Hunter syndrome
= mucopolysaccharidosis type 2
not quite as severe as Hurler syndrome, but is otherwise similar. has better cognitive outcome though and no eye involvement (no corneal clouding).
Treat with enzyme replacement therapy.
In Fabry disease, lysosomse are missing the enzymes to break down
lipids/fats
recycling centers within cells are called
lysosomes
What is Fabry disease?
= a lysosomal storage disease where you are missing the enzyme alpha-galactosidase A, and so cannot break down fats in the lysosome
Fats therefore collect in the blood vessels and tissues, and patients get periodic pain crises, strokes, LVH, renal disease, angiokeratomas (bumps on the skin), eye isseus and sweating abnormalities
x-linked
treat with enzyme replacement therapy
Marked hypotonia, FTT, and hypertrophic cardiomyopathy in the first year of life think
Pompe disease
What is Pompe disease?
It’s a GLYCOGEN storage disease. It’s classified as a lysosomal storage disorder where there is a lack of the enzyme alpha-glucosidase. AR. Has infantile and late onset forms. Treat with enzyme replacement.
glycogen builds up becuase you can’t break it down
causes problems with muscles (skeletal and cardiac)
In the PREP hospital, do ALL kids admitted with influenza get antiviral therapy as in tamiflu aka oseltamivir?
Yup
even give it empirically if you suspect it
(wtf)
Kids under this age are at especially high risk of complications from influenza and according to PREP, should probs get tamiflu
2
Mineral oil (used to treat constipation) should not be used in children under age blank, due to blank
1
becuase it’s associated with lipid pneumonitis
Why do kids with post infectious glomerulonephritis get edema?
because of salt and water retention
Do kids who present with post strep glomerulonephritis always have a positive ASO (antistreptolysin O) titer?
No, not always
typically will be positive following strep pharyngitis, but, in the case of a strep skin infection the stretolysin may be bound to lipids in the skin so kind of is stuck there and you don’t get a positive ASO
Inhalants that teens may use to get high include
spray paint
hair spray
paint thinners and glue
gases like propane
A teen comes into the ED after being found by a neighbor. The teen is agitated and confused, hallucinating, has nystagmus, slurred speech and excessive salivation, clearly altered mental status, and normal sized pupils. On the monitor, he has some irregular heart beats. The toxidrome is
acute inhalant intoxication
How a teen with acute inhalant toxicity presents:
euphoria
agitated/confused
hallucinations
nystagmus
skin flushing/excessive salivation
normal sized pupils apparently
Most common cause of death from inhalant abuse is
cardiac arrhythmias
Congenital scoliosis is associated with
genitourinary anomolies
so if you discover that someone has congenital scoliosis, a renal ultrasound should be performed
Is bracing an effective treatment for congenital scoliosis?
nope not at all
becuase bracing won’t correct a structural defect such as a wedge or hemivertebrae that is causing the congenital scoliosis
Congenital scoliosis is caused by
structural vertebral malformations
tho sometimes is not picked up until adolescense
Which kids/teens with scoliosis should be referred to an orthopedic surgeon?
kids with congenital scoliosis
anyone with scoliosis with a curve of 20 degrees or greater
When should you use azithro to treat cambylobacter gastroenteritis?
only if it’s really severe.
otherwise, in an otherwise healthy child, just do supportive care.
Name an ACE inhibitor
enalapril
For kids with structural heart disease and asymptomatic heart failure (for ex, s/p AV canal repair years ago now regurg and depressed cardiac function on echo but no symptoms), what drug should be started?
an ace inhibitor
like enalapril
allergen immunotherapy for treating asthma has been shown to decrease
the amount of INHALED corticosteroids a child gets
hasn’t been shown necessarily to do other good things yet
Does immunotherapy for asthma improve lung function, excercise capacity, or decrease need for rescue medication?
No, but it does decrease the amount of inhaled corticosteroids used
Most effective way to decreased violence between students in school is
not to target certain groups of students
but instead to do universal school-based violence prevention programs
What is tick paralysis? What causes it?
It’s a crazy phenomenon where a rapid ascending paralysis (that can mimic other things like GBS or botulism) is caused by a neurotoxin released by an attached tick, that actually blocks the release of acetylcholine.
The treatment is to remove the tick and the patient will rapidly improve.
When someone presents with acute onset ascending paralysis, you should always do a skin exam to look for
tick paralysis
Who should NOT be given lasix (furoesmide)?
It’s actually a sulfonamide, so do NOT give it to people with a sulfa allergy
If you are allergic to bactrim, avoid this diuretic
lasix
(they are both sulfa drugs!)
Antimicrobial therapy for nontyphoidal salmonella should be reserved for…. which patients?
-infants under 3 mo of age
-immunocompromised children
-anyone with a chronic GI disease
otherwise DO NOT TREAT unless it’s like SEVERE
Do kids with nontyphoidal salmonella gastroenteritis need to be completely asymptomatic to return to daycare?
Nope!
Just need to have “less than five stools per day and the stool needs to stay contained in the diaper”
or
when a toilet trained kid is no longer having accidents
or
ïf stool frequency is no more than 2 stools above normal for that child”
so basically PREP wants to send the backkkkk to daycare
What finding is the hallmark of fatty acid oxidation disorders?
hypoketotic hypoglycemia
The key lab finding in an infant presenting with a fatty acid oxidation disorder is
hypoketotic hypoglycemia
A neonate shows up to the ED SICK and has a high anion gap metabolic acidosis and hypoketotic hypoglycemia. What is the diagnosis?
Most likely a fatty acid oxidation disorder
Medium-chain acyl-CoA dehydrogenase deficiency, very long-chain acyl-CoA dehydrogenase deficiency, carnitine palmitoyltransferase 1 and 2 deficiency, and carnitine transporter defects are all examples of what kind of disorder?
Fatty acid oxidation disorders
When is the earliest that glycogen storage disorder might present?
Usually no symptoms in the neonatal period.
Earliest presentation would be 3-6 months
How do organic acidemias present?
In the neonatal period with hyperammonemia, anion gab metabolic acidosis, and positive presence of urine ketones
This type of metabolic disorder presents in the neonatal period iwth hyperammonemia and respiratory alkalosis
urea cycle defects
Can neonates with a cleft lip but an INTACT palate attempt to breast feed?
yup!
Pierre Robin sequence
= micrognathia, posterior tongue (glossoptosis), and u shaped cleft palate
can present with airway obstruction shortly after birth
Cleft lip repair timing
2-6 months
Timing of cleft palate repair
9-19 mo
(before this is associated with causing mid face hypoplasia)
cleft palate is associated with common AOM because
it’s associated with abnormal eustachian tube anatomy
Who can evaluate for and make a diagnosis of autism?
a developmental psychologist
developmental-behavioral pediatrician
neurologist (ok)
