Board Vitals #2 Flashcards
White mass behind tympanic membrane, no history of ear infections. Conductive hearing loss on audiogram.
Dx
Congenital choleastoma
Tx with surgical excision or risk hearing loss
Tell apart from acquired given neg history
What is the risk for devo of T1DM in the sibling of affected individual?
Affected parent?
Risk is 5% in siblings
6% if parent has it
50% in identical twins
What are other diseases associated with T1DM?
Autoimmune thyroid with 25% risk
5% risk of celiacs
RF = Vit D deficiency, cereal induction before 3 months
What is benign neonatal sleep myoclonus?
Seen more common in preterm infants, no seizure and see focal or multifocal myoclonic jerks during non-REM sleep with normal EEG, no neuro abnormalities
Teen with irregular patches of missing hair with short broken hair shafts of different lengths, see perifollicular involvement and the hair shafts are incompletely keritanized
Dx?
Associations?
Tx?
Trichtillomania
Behavior therapy
Associated with OCD, anxiety, may need SSRIs or stimulants
Patient with broken hair shafts with erythematous and scaly patch on head.
Dx and tx?
Tinea capitis
Tx is 6-8 weeks of oral griseofulvin or 2-4 weeks of oral itraconazole or terbinafine
How can minors become emancipated?
Joining military, getting married
Patient started on amoxicillin a few days ago, developed a erythematous itchy rash on trunk, arms, hands and soles. Cause of rash and treatment
Drug eruptions; delayed T cell mediated type IV immune response
different from viral xanthem bc on palms and soles also see low grade fever and eosinophilia
Patient present with fever, swollen LN, joint pain and hives. Recently started on new AED.
Serum sickness
type III hypersensitivity; antiB-antiG immune complex deposition
When can kids sit in front seat?
When they turn 13 years old. No specific weight recommendation and really should be over 4ft 9in
3 yo patient with limping and left knee swelling but no pain and now decreased ROM. No fevers. On exam swollen knee, pain with ROM. ESR is 40 and aspirated fluid is neg for organisms.
Dx and complications?
Juvenile idopathic arthritis
Common complication is uveitis–> lead to blindness; should see optho regularly.
Patient presents with UTI, they are 2 years old. This is third UTI. You get renal US and a it shows reflux to renal pelvis with dilation of ureter and distension of collecting system of right kidney.
What grade is reflux and what is this concerning for?
Patient with grave IV or V (only ones that show up on US, lower grades don’t show up on US).
Pt with vesicourethral reflux and needs to be on prophylaxsis
Patient with MRI showing downward displacement of cerebellum from small posterior fossa into cervical or occipital encephalocele
Chiari III malformation
Abnormal cerebllar tonsils displaced below foramen magnum. Pt with occipital HA, neck pain and gait abnormalities. Also having nystagmus and dysarthria
Chiari I malformation; can be associated with syringomyelia
Fluid filled cavity lined with gliotic tissue and confined to spinal cord. No involvement of central canal
Syringomelia
Patient ingests rat killer. He has BL intracranial hemorrhage and prolonged PT.
What happened and what is tx?
rodenticide ingestion (anticoagulation by inhibiting Vit K dependent coag factors)
Tx for symptomatic patients are FFP for emergent reversal.
If not as severe/asymptomatic then use Vitamin K
What do we give to reverse Heparin urgently?
FFP for symptoms and give Protamine for asymptomatic urgent reversal
Pathophys of CF and inheritance.
Defective transmembrane conductance regulator protein; chloride channel found in exocrine tissues. Get loss of Na in sweat = hypoNa
Auto Recessive
How is CF screened in newborn screen?
Screen for immunoreactive trypsinogen then do follow up testing for genetics and sweat chloride later
Cause of Duchennes
X linked disorder; muslce degeneration; dystrophin gene is mutated leading to muscle degeneration and see devo delay, intellectual impairment and elevated serum Cr.
What is the classic presentation of PCOS
hyperadrnogenism (hirsutism and acne) oligomenorrhea or amenorrhea, polycystic ovaries and LH elevated to FSH: 3:1
What neonates should get the VZV immune globulin
Mom with symptoms 5 days before or 2 days after birth OR hospitalized premature infants >28 wks in mom’s w/o immunity OR
<28 weeks that weight less 1000g no matter what
Cause of congenital diaphragmatic hernia
embryologic defect around 6-7 weeks gestation
more common in teen mom, smoking
What electrolyte abnormalities are seen with salicylate toxicity?
Anion Gap metabolic acidosis with respiratory alkalosis (increased RR)
Hypoglycemia
Hypokalemia
Tx alkalinize urine with sodium bicarb
What are SIRS criteria in peds?
- Temp >38.5 or < 36
- Pulse >2 SD above norm
- RR >2 SD above norm
- Leukocyte abnormalities
* 2/4 must be met and one must be either temp or leuks
Whats the most important initial therapy for Peds in septic shock?
FLUIDS; 60ml/kg and then antibiotics
What type of imaging is best to assess for retropharngyeal abscess?
Full extension during inspiration
Patient with single oval red plaque on chest that became scaly. In teens, no other symptoms. What may happen next?
Pityriasis rosea; can progress to similar legions concentrated on trunk in christmas tree distribution
Tx for bulemia
CBT and fluoxetine
This bug causes diarrhea, rash, pharyngeal or conjuctival erythema, rhinorrhea and fevers. See outbreaks in NICUs, daycares and lots of different buts in this family.
Enterovirus
see Coxsackie A and B
Delayed cord separation and omphalitis and nonpustular lesions are associated with this immune defect
LAD; leukocyte adhesion defect; neutrophils don’t adhere to vascular endothelium due to defects in integrin CD18 (key for neutrophil
Male patient who is 2 yo, recurrent s.pneumo and H.influenza as well as S. aureas infections. No Ig’s on testing
Agammaglobulinemia from mutation in Btk gene on chrom Xq22; arrest B cell devo at pre-B cell state.
X linked more common than AR form
2 week old infant with low set ears and flat nasal bridge present with tachypnea, crackles, puffy hands and feet and poor eating. Femoral pulses are barely palpable when compared to radial.
Dx and treatement
Coarct in setting of Turners
prostaglandin E1 to keep the PDA open until surgery can be done and give inotropic meds.
Serum sickness is a ____ hypersensitivity from ____.
See arthralgias as well as uticaria and fever 1-3 weeks after starting drug
Type III
Immune complex deposition
type III = C for immune Complex
How do we treat hypernatremic dehydration?
restore intravascular volume with isotonic fluids until adequate perfusion has been restored. Then you can use D5 0.2NS at a constant rate. Goal is not to drop Na by more than 12 in 24 hours to minimize risk of cerebral edema
parent interview form to screen for developmental and behavioral problems that need to be evaled. Works for kids 0-8 and only 10 questions
PEDS (parents evaluation of developmental status)
good to screen for academic, behaviors, fine motor, ect.)
What are the CHAT and M-CHAT used for?
screen for autism
Modified Checklist for Autism in Toddlers
M-CHAT: 16-48 months
CHAT; 18-24 months
What is the Ages and Stages or ASQ used for
parent completed for communication, gross motor, fine motor, problem solving and personal adaptive skills for 4-60 months
What three things are seen in septo-optic dysplasia?
- optic nerve hypoplasia
- midline devo defects (dysgenesis or absence of septum pelludicum, thinning of corpus collosum)
- Hypopit (see GH deficiency as most common)
Constellation of findings in MEN2B
Medullary thyroid carcinoma
Marfanoid
Mucosal neuromas and distinct face~ large lips and ganglioneuromatosis
Pheochromocytoma
Findings in MEN1
Hyperparathyroid
Islet cell tumor
Pituitary tumors
Findings in MEN 2A
Medullary thyroid
Pheochromocytoma
Parathyroid adenoma
In preterm infant what is most common cause of hyponatremia?
From excessive renal loss of sodium
Most common inherited risk factor for thrombosis
Factor V Leiden
aids in clotting of injured vessels
mutation causes resistance to activated protein C (key for regulating amount of clotting)
Dx by DNA blood test to see if hetero (5-7 fold increase of thrmobosis) or homozygous (25-50 times increase)
What happens as a result of scorpion sting?
Catecholamine surge; see tachycardia, hypertension, hyperthermia, tachypnea
A complete series of Hep B vaccines requires ___ doses.
A dose does not count if the infant is
3 doses
<2.0kg
Patient with asthma symptoms LESS than ___ days per week and ___ nights per month have intermittent asthma
2
2
Patient with asthma more than 2x per week but less than once a day, flares affect activity. Night flares occur >2x/month but not weekly with lung function 80% or more of normal
Mild persistant asthma
Tx with low dose ICS
Symptoms of asthma daily, flares last several days and disrupt activity and frequent night time wakenings with lung function 60-80% normal
Moderate persistant asthma
Daily and frequent asthma symptoms, disrupt sleep and lung function <60% of normal
Severe persistant
Most common urea cycle defect with elevated ammonia and low citrulline, somnolence, temp instability, vomiting and seizures.
Ornithine transcarbomylase deficiency
X linked
usually nitrogen and ammonia–>urea cycle-> urea and excreted but doesn’t happen bc ornithine transcarbomylase is deficent
Urea cycle defect present with: \_\_\_\_\_ anion gap \_\_\_\_blood glucose \_\_\_\_ketones \_\_\_\_plasma ammonia
NORMAL anion gap
NORMAL blood glucose
NORMAL ketones
ELEVATED ammonia
Organic acidemias present with
____ ammonia
____ketones
____glucose
ELEVATED ammonia
ELEVATED ketones
LOW glucose
Patient is AA, 10 yo and coming in with fever, rash and swollen LN. Mom says he has joint pain and on exam rash looks like erythema nodosum. Eyes are red and injected and you note a cough on exam, mom says this has been going on for months
CXR with BL hilar lymphadenopathy.
Sarcoidosis
seen 8-15yo in kids, more in AA
Dx with CXR, labs (CBC, CMP with elevated Ca, TB test, PFT and eye exam with uveitis)
Gold standard dx is biopsy of lesion
Patient with flu like illness, HSP, hilar lymphadenopathy, pulmonary nodules, cavitary lung disease, joint pain.
Pt in Ohio
Histoplasmosis
-also see calcifications on CXR
Tx: amphotericin or fluconazole or ketoconazole
Patient recently started on AED about one month ago now has high fevers, swollen LN, rash and body aches. Mucosal membranes are not involved
Drug induced hypersensitivity syndrome
Seen in AEDs and sulfas
Mom noticed left sided mass in abdomen while giving a bath to 3 yo. Elevated BP on exam, normal HR. Most likely malignancy
Wilms
most common abdominal malignancy in kids.
RCC not until teens!
Tx with nephrectomy; good prognosis with favorable histology
Hemihypertrophy is associated with..
Beckwidth weidemann
Klippel Trenaunay
Russell Silver syndrome and Denys-Drash
Symptoms associated with accidental Ca Channel blocker
Tx
Hypotension, bradycardia and usually normal mentation. EKG with PR prolongation and hyperglycemia 2/2 decreased insulin release
Tx IV Calcium and glucagon (to improve inotropy and chronotropy) and IV fluids
Patients with CSF glucose <40 are most often associated with
bacterial meningitis
Ciprofloxacin is a FQ that works by_____
Best to treat _____
Can’t use with____
FQs work by inhibiting DNA gyrase and topiosomerase (enZ needed for DNA synthesis)
Use for gram negatives (like UTIs) and PSA
can’t use with zinc, calcium of mag (cations) bc decreases absorption. can’t use with antacids
SE are upset stomach, tendonopathy, HA
Necrotizing encephalopathy; mitochondrial disorder. Lesions in brain stem, thalamus, basal ganglia and cerebellum. Presents in infancy/early childhood with devo delay, ataxia, dystonia, seizures, lactic acidosis
Leigh disease; progressive