Peds Uworld review 2 Flashcards
What is the weber and Rinne test?
Weber = tuning fork on top of head, detects bone conduction
Rinne = on mastoid process, then air
What determines an abnormal Rinne test?
-If bone conduction is heard longer than air conduction in affected ear (normal is reversed)
What sort of study design would be used to determine incidence of a disease?
Cohort study
What sort of study would be used to determine a disease prevalence?
Cross-section study
What is involved with a cross-sectional study?
Sample of individuals from a population at a given point in time to determine prevalence
What is the cause of physiologic jaundice of the newborn, and when does it typically appear/resolve?
- Increased RBC breakdown and Decreased hepatic UDP glucuronosyltransferase activity.
- Appears day 2-3, and resolves by 1-2 weeks.
At what level of bili is exchange transfusion indicated for physiologic jaundice of the newborn?
25 mg/dL
For how long is nocturnal enuresis normal?
Until age 5
Which gender typically requires more time to be fully toilet trained?
Boys
How can you differentiate between CVID and SCID?
CVID is due to abnormal maturation of B cells, but B and T cell counts are normal
SCID is due to T cell defects causing a low T cell count, and thus a low B cell count
Which gets better with ice pack application: myasthenia gravis or Lambert-Eaton syndrome?
MG, since this decreases breakdown of ACh in the synapse
What heart sound can classically be heard at onset of a new MI?
S4
What immunologic process is impaired with a splenectomy?
Antibody-facilitated phagocytosis, due to a loss of splenic macrophages
Which is associated with CF and which with down syndrome: meconium ileus vs Hirschsprung
CF = meconium ileus Hirschsprung's = DS
What is the level of obstruction seen with meconium ileus vs Hirschsprung’s disease?
Hirschsprung’s = Rectosigmoid
Meconium ileus = Ileum
What is the “squirt sign” associated with Hirschsprung’s disease?
Rectal exam produces an explosive discharge of stool and flatus
What associated conditions develop with Tourette syndrome? (2)
OCD and ADHD
What is the most common cause of lymphadenitis in children?
Staph Aureus
What gyn cancer is associated with Lynch syndrome?
Endometrial cancer
What is the general physiologic process that is impaired with Lynch syndrome?
DNA mismatch repair
What is the cause of a Meckel’s diverticulum?
Failure to obliterate the vitelline (omphalomesenteric) duct
What is the diagnostic test for a Meckel’s diverticulum?
Tch-99 pertechnetate scan
What are three conditions that can lead to pellagra (1 medication, 1 inherited disease, 1 acquired disease)?
- Isoniazid (interference of Y metabolism)
- Hartnup disease (Lack of Y uptake)
- Carcinoid syndrome (consumption of Y)
What is the pharmacotherapy for Tourette’s disorder? (2)
- Alpha-2-agonists if mild
- antipsychotics
What is the classic x-ray appearance of an osteosarcoma? What 2 labs are elevated?
- Sunburst appearance on xray
- Elevated alk phos and LDH
What are the classic findings of fetal hydantoin syndrome?
- Hypoplastic fingers/nails
- Cleft lip/palate
- MR
What is the role of x-rays in diagnosing osteonecrosis of the femoral head?
Will detect later findings, but not sensitive enough for early disease–need MRI
How long should pts with mono avoid contact sports?
Minimum of 3-4 weeks
What CT findings highly suggest a hydatid cyst 2/2 echinococcus granulosus?
Eggshell calcifications
What are simple hepatic cysts? S/sx? Imaging findings/?
- Congenital cyst related to fluid secretion by epithelial lining
- Dull RUQ pain, early satiety
- No calcifications
What are the upper endoscopy findings of ZES?
Prominent gastric folds, multiple duodenal/jejunal ulcers
What is the treatment for persistent enuresis?
- Bedwetting alarms/rewards
- Desmopressin if refractory
What are the adverse effects of desmopressin?
Since it is an ADH analogue, isovolemic hyponatremia
What is the appropriate diagnostic test for a Zenker’s diverticulum?
Contrast esophagram
What are the five components of metabolic syndrome?
- Abdominal obesity
- Impaired fasting BG
- HTN
- High triglycerides
- Low HDL
What is the pathogenesis of polycythemia in an infant?
- In utero hypoxia causing increased RBC production
- Delayed cord clamping causing excessive placental blood transfer
What are the s/sx of polycythemia in a neonate?
RUddy complexion
-Respiratory distress and hypoxia d/t increased viscosity of the blood
What level of hematocrit is diagnostic of polycythemia in the neonate?
Over 65%
What are the s/sx of contact lens associated keratitis?
- Conjunctival injection
- Pain
- Purulent d/c
- Hazy cornea
What is the treatment for keratitis?
Emergent abx and removal of contact lens d/t risk of corneal perforation and loss of vision
What is disruptive mood dysregulation disorder?
-Adolescent mental disorder, characterized by persistently irritable or angry mood and frequent temper outbursts that are disproportionate to the situation
What is the pathophysiology for Osgood-Schlatter disease? What is the other term for this?
- Traction apophysitis
- Periods of rapid growth cause tension on the quadriceps tendon and thus the apophysis of the tibial tubercle.
What is the common presentation of patellofemoral stress syndrome?
Overuse injury in runners characterized by anterior knee pain that worsens upon descending steps of hills.
What is the common presentation of patellar tendonitis?
- Overuse syndrome resulting from repetitive jumping or kicking.
- Anterior knee pain after exercise.
- Point tenderness at the inferior pole of the patella
What are the five s/sx or lab results that indicate septic arthritis over transient tenosynoviits?
- Fever over 101
- Inability to bear weight
- WBC over 12
- ESR over 40
- CRP over 2 mg/dL
What are the specific s/sx of congenital syphilis?
- Snuffles
- maculopapular rash that desquamates
- Abnormal long bone radiographs
What are the cranial manifestations of CMV?
Ventricular calcifications
What lung manifestations may occur with exertional heat stroke?
ARDS
What is the difference in treatment in exertional vs nonexertional heat stroke?
- Exertional = ice water bath immersion
- Non-exertional = evaporative cooling
What are the pupils like with anticholinergic toxicity?
nonreactive mydriasis
how long do abscence seizures typically last? What sort of movements may be seen?
- Less than 20 seconds
- Simple automatisms (but no more complex motor movements)
What is the treatment for mil, moderate, and severe hypothermia?
- Mild = Passive external warming (90 F+)
- Moderate = active external warming (warm baths, heating pads) (82-90 F)
- Severe = Peritoneal irrigation with warm fluids (less than 82 F)
What vitamin has been shown to decrease mortality in Measles?
Vitamin A
How does the rash progress with Measles?
Cephalocaudal
What is the emergent treatment for uterine bleeding in a hemodynamically stable pt?
High dose oral estrogen/progestin contraceptive pills
What is the emergent treatment for uterine bleeding in a hemodynamically unstable patient?
D and C and/or pRBC infusion
What happens to the following with Bruton’s agammaglobulinemia:
- T cell count
- B cell count
- IgA levels
- IgG levels
- IgE levels
- IgM levels
- T cell count elevated
- B cell count markedly reduced
- All Ig levels markedly reduced
What happens to the following with CIVD:
- T cell levels
- B cell levels
- IgA levels
- IgG levels
- IgE levels
- IgM levels
- T cell counts normal
- B cell counts normal
- All Ig levels markedly reduced
What is the cause of SCID, and what happens to T cells, B cells, and Ig levels because of this?
-Impaired T cell development and thus subsequent B cell dysfunction
Both B and T cell counts reduced, and thus Ig levels down
What is transient hypogammaglobulinemia of infancy?
- transient decrease in IgG levels (variable IgM levels)
- Normal B and T cell concentrations
- Basically worse infections until 9 ish months, but not life threatening
What happens to H and K with high aldosterone levels?
Increased renal secretion (contraction alkalosis)
What is “contraction alkalosis”?
Loss of fluids cause aldosterone release, which promotes H and K excretion.
What are the s/sx of CN poisoning?
- Almond breath
- AMS
- Lactic acidosis
- Seizures
- Coma
When does EtOH withdrawal set in?
12-48 hours
In whom is CN poisoning 2/2 Nitroprusside use more common in?
Renal insufficiency
What are the s/sx of HTN encephalopathy?
Encephalopathy without focal symptoms (diffuse brain edema)
What are the urinary metabolites seen with a neuroblastoma?
Homovanillic acid
vanillylmandelic acid
What is the standard test for lactose intolerance?
Hydrogen breath test
What is the medical term for strawberry hemangiomas? What is the treatment for these lesions if they’re in a life threatening place?
- Infantile hemangiomas.
- Beta blockers
What is the highest predisposing factor associated with aortic dissection?
Systemic HTN
What is the relationship between atherosclerosis and aortic dissection?
Associated, but not causative.
what are the common symptoms of cataracts?
Halos around lights
Decreased visual acuity
Glare
What is the underlying pathophysiology of cataracts?
Oxidative damage from things like DM, smoking, aging, or glucocorticoid use
What are the s/sx of Chikungunya fever? (5)
- High fevers
- Polyarthralgias
- HA
- Conjunctivitis
- Maculopapular rash
What are three lab manifestations of Chikungunya?
- elevated LFTs
- Lymphopenia
- Thrombocytopenia
How is Chikungunya spread?
Mosquito bites
What is pyoderma gangrenosum?
Neutrophilic ulcerative skin disease usually associated with an underlying systemic disorder (e.g. IBD) that starts as an inflammatory papule, pustule, or nodule, and progresses to an expanding ulcer with a purulent base and ragged violaceous borders
What is the treatment for pyoderma gangrenosum?
Corticosteroids
How is pyoderma gangrenosum diagnosed?
Skin Bx
What is ecthyma gangrenosum?
Hemorrhagic pustules with surrounding erythema that evolve into necrotic ulcers
usually due to pseudomonas infection
What is the best test to assess for the risk of foot ulceration in pts with diabetic neuropathy?
Monofilament testing
What is enthesitis?
Inflammation at tendon/ligament insertion points
Diffuse enthesitis is suspicious for what disease?
Ankylosing spondylitis
How does hypomagnesemia cause hypocalcemia?
Induces PTH resistance, and decreasing PTH secretion
What is the first line medication to reduce the reoccurance of sickle cell crises? What is the side effect of this?
Hydroxyurea
Myelosuppression
Can the CENTOR criteria be used to empirically treat strep in preadolescents?
No–must do a RSAT
What type of fluids should be used in the initial resuscitation of a pt with hypernatremia with hypovolemia
NS
What are the skin manifestations of Sturge-Weber syndrome?
Port wine stain
What are the head XR findings of sturge-weber syndrome?
Gyriform intracranial calcifications that resemble a tramline
Angiofibromas in a butterfly pattern on a child’s face = ?
Tuberous sclerosis
What are the UA findings of renal papillary necrosis? Why?
- Normal appearing RBCs in the urine. Nothing else
- Extraglomerular problem
When is myringotomy with tympanostomy tube placement indicated?
If AOM develops 3x/6 months OR
4x/12 months
What is the underlying cause of dysmenorrhea?
Prostaglandin release
When does the pain occur with primary dysmenorrhea vs endometriosis?
Primary = at onset of menses Endo = few days prior
What happens to thirst mechanism with DI?
Central = no thirst peripheral = thirst intact
What is the treatment for SIADH? MOA?
Demeclocycline
Inhibits ADH-mediated aquaporin insertion
What is the preferred treatment for nephrogenic DI?
Thiazides
What is the screening test for Conn syndrome? What is the f/u test?
Plasma aldosterone / renin concentration
Adrenal suppression testing f/u
What are the four major s/sx that distinguish orbital cellulitis vs preseptal cellulitis?
- Ophthalmoplegia
- Pain with EOMs
- Proptosis
- Vision impairment
True or false: pts with exertional heat stroke are often volume depleted
False–more often NOT volume depleted. Rather, it’s a failure of the thermoregulatory system
Which has significant CNS dysfunction: volume depletion 2/2 heat or exertional heat stroke?
Exertional heat stroke
What is the inheritance pattern and defect of hereditary spherocytosis? What is the treatment?
AD loss of ankyrin
Splenectomy or folic acid supplementation
What happens to Mean corpuscular Hgb concentration with hereditary spherocytosis?
Increases due to a lack of RBC membrane.
What is the classic symptom of ABO incompatibility hemolytic transfusion reaction?
Flank pain
Fever
What are the s/sx of IgA deficiency as a cause of transfusion reaction?
Anaphylaxis, with angioedema, hypotension, and dyspnea
What disease causes a albuminocytologic dissociation on CSF?
Guillain -barre
What is the classic sign of leukocyte adhesion deficiency (outside of the neonatal period)?
Inflammation with a lack of purulence
What one UA finding distinguishes glomerular causes of hematuria vs nonglomerular?
Proteinuria = glomerular
What is the gene defect in osteogenesis imperfecta?What is the mode of inheritance?
AD mutation in the COL1A1
What is eczema herpeticum?
Superinfection of eczema with herpes, causing a vesicular eruption on pre existing inflamed skin
Type 2 RTA is associated with what disease?
Fanconi syndrome
What are the causes of type 1, 2, and 4 RTA?
1 = inability to secrete H+ into urine
2 = inability to reabsorb HCO3
4 Aldosterone deficiency
What are the lab findings common to all 3 types of RTA? (2)
Hyperchloremic
Non-anion gap acidosis
What is Juvenile myoclonic epilepsy?
Myoclonic jerks that usually occur in the morning. Not loss of tone, but dropping things common.
What are the EEG findings of Lennox-gastaut syndrome?
2.5 Hz Slow spike and wave pattern
What are the EEG findings of Infantile spasms?
Hypsarrhythmia
What is the classic triad of Hemolytic uremic syndrome?
- Microangiopathic hemolytic anemia
- Thrombocytopenia
- AKI
What are the UA findings of multiple myeloma?
Bland UA that may show evidence of granular casts
What sort of animal bite always requires abx prophylaxis?
cat bites since they’re so deep
What areas of the body that, when bitten by an animal, require abx prophylaxis?
Hands
Feet
Genitalia
What are the s/sx of atlantoaxial instability that is characteristic of Down syndrome?
-Similar to normal pressure hydrocephalus
What are the characteristic AXR findings of jejunal atresia?
Triple bubble sign
What is the enzyme that is deficient in lesch-Nyhan syndrome?
HGRPT
When is bicarb indicated for the treatment of acidosis? Why not sooner?
If pH less than 7.1
Risk of impairing cardiac conduction
What position is stridor worse with laryngomalacia?
Supine makes it worse
What happens to MCV with thalassemias?
Normal
What happens to total RBC numbers in thalassemia?
Normal, despite low Hgb
What are the classic signs of amyloidosis?
- Macroglossia
- LVH
- Waxy skin
- Anemia
- neuropathy
- Ecchymoses
- Proteinuria
What are all the s/sx of anticholinergic toxicity? (5)
Blind as a bat Hot as a hare Dry as a bone Red as a beet Full as a flask
What are the s/sx of theophylline? (4)
Seizures
Hyperthermia
Cardiac arrhythmias
Hypotension
What CNS pathology is a complication of mumps?
Aseptic meningitis
True or false: facial nerve palsy is a possible consequence of mumps infx
false
what are the classic PBS findings of G6PD deficiency?
Heinz bodies and bite cells
Heinz bodies are oxidized Hgb
When are growing pains classically the worst?
At night
What happens to the peak of the murmur of aortic stenosis as the disease progresses?
Moves later in systole
What happens to S2 with AS and why?
Soft and single S2 due to reduced mobility of the valve
A loud S1 is typically heard with what valvular disease?
Mitral stenosis
What is the most sensitive indicator for dehydration?
Decrease urine levels
Why is a head CT not required prior to performing an LP in infants?
Their open fontanelles relieve intracranial pressure
What are the top three modifiable risk factors for pancreatic cancer, in order?
- Smoking
- Chronic pancreatitis
- Obesity
What sort of lesions are present in bone with langerhans cell histiocytosis?
Lytic bone lesions, typically in long bones, with systemic hypercalcemia
What is the defect an inheritance pattern of Kallmann syndrome?
XLR disorder of migration of fetal gonadotropin-releasing hormone and olfactory neuron
What is the diagnostic test of choice for meconium ileus? Treatment?
- Contrast enema after pneumoperitoneum has been excluded by AXR
- This may break up the insuppsated meconim
What is the classic appearance of meconium ileus on contrast enema?
Microcolon
What is the diagnostic test of choice for malrotation?
Upper GI series
What is the test used to differentiate meconium ileus and Hirschsprung’s disease? What will this test show with each of these?
Contrast enema
- microcolon = ileus
- Rectosigmoid transition zone = Hirschsprung’s
What is the treatment for seborrheic dermatitis in children?
Gentle emollients and non-medicated shampoos.
Weak corticosteroids or ketoconazole are second line
Peak airway pressure = ? (looking for equation)
Resistive pressure + plateau pressure
Plateau pressure = ? (equation)
Elastic pressure + PEEP
newborn baby with cyanosis that is better with crying, but worse when feeding = ?
Choanal atresia
What are the components of CHARGE syndrome?
Coloboma Heart defects Atresia, choanae Retardation GU anomalies Ear abnormalities
When is vaccination against Yellow fever indicated?
Sub-saharan africa or south america trips
What are the lab findings of diamond-blackfan anemia?
ONLY RBCs are affected. No pancytopenia
What cell lines are decreased with transient erythroblastopenia?
Only RBCs
Which test is prolonged with factor 8 deficiency?
aPTT
What is the MOA of tetanospasmin?
Prevents release of GABA/glycine from UMN. This causes uninhibited LMN, leading to spasticity/hypertonicity
What is needed to make a diagnosis of acute liver failure? (3)
- Elevated LFTs
- Features of hepatic encephalopathy
- INR at/over 1.5
What are the classic stool findings of a dehydrated infant?
Brick-red urate crystals
During the first week of life, how many wet diapers/day should there be?
Same as age in days (e.g. 4 day old should have 4 wet diapers)
What is the most common cause of sepsis in the sickle cell population?
Strep pneumoniae
What is the major difference between Meniere’s disease and labyrinthitis?
Meniere’s disease is recurrent and progressive. Labyrinthitis is not
What causes the secondary metabolic acidosis with ASA intoxication?
Uncoupling of Mitochondrial ETC, causing lactic acidosis
What is the progression of viral bronchitis?
Dry cough, followed by yellowish purulence 2/2 epithelial cell sloughing, and possibly mild hemoptysis
When is chronic LAD significant?
If more than 2 cm, firm/hard, and immobile
What is the MOA and use of bethanechol?
Cholinergic agent used to treat urinary retention
What is the MOA and use of Oxybutynin?
Anticholinergic agent used to treat urinary incontinence
What is neuropraxia?
Transient Weakness d/t nerve injury, common with elbow fractures.
How does GERD worsen asthma? What is the treatment for this?
Microaspiration of gastric contents, leading to an increase in vagal tone and bronchial reactivity.
Treatment is with PPIs.
True or false: pts who are newly diagnosed with rheumatic fever do not need abx unless they have pharyngitis at the time
False–always give PCN to any newly diagnosed rheumatic fever
What is the duration of PCN therapy in the following pts:
- Rheumatic fever without carditis
- Rheumatic fever with carditis, but no residual heart or valvular disease
- Rheumatic fever with carditis and persistent valvular disease
- 5 years or until 21 years (whichever is longer) for rheumatic fever without carditis or 10 years carditis w/o valvular disease
- 10 years until 40 years old if have valvular disease
What are the two hemoglobins present with sickle cell trait? Disease?
- HbA and HbS = trait
- HbS + HbF = disease
What are the classic hand findings associated with Trisomy 18? Feet?
- closed fist with fingers overlapping
- Rocker bottom feet
What are the characteristics of the head with trisomy 18 (2)?
- micrognathia
- Prominent occiput
What are the classic head findings of trisomy 13?
- Microphthalmia
- Cleft lip/palate
- Cutis aplasia
What are the characteristic hand findings of trisomy 13? Feet?
Polydactyly
Rocker bottom feet
Isolated thrombocytopenia in a child after recent viral illness = ? What is the treatment?
- ITP
- Observation if not bleeding. If they are bleeding, then IVIG and corticosteroids
What is obesity hypoventilation syndrome, and how does it differ from OSA?
-OSA is only at night, whereas OHS is persistent always. Due to chronic hypercapnia in OHS, there is increased biacarb, and resultant decrease in Chloride
Why does squatting relieve Tet spells?
Increases afterload, forcing more blood through stenotic pulmonary artery
Does strongyloides produce diarrhea?
Not typically, but if so, not profuse
What are the effects of exogenous androgen use in men? (besides obvious, 4)
Gynecomastia
Polycythemia
Hyperlipidemia
Possibly hepatotoxic
What animal is histoplasma associated with?
Bird/bat droppings
Hilar adenopathy with noncaseating granulomas is suspicious for what infectious process?
Histoplasmosis
What normally happens to SBP with inspiration? What changes can occur to this?
Falls by less than 10 mmHg
-If falls by more than 10 mmHg, then pulsus paradoxus
What is the pathophysiology behind pulsus paradoxus?
-If due to tamponade, then L and R ventricles compete for limited space in sac. Increased venous return from inspiration increases RV volume, and thus decreasing space in the LV. This reduces EDV in the LV, and thus reducing SBP
What causes pulsus paradoxus with asthma or COPD?
The drop in thoracic pressure is greatly exaggerated with these, causing a much larger volume to be maintained in the lungs, reducing LV EDV, and thus SBP
What, generally, is Werdnig-Hoffman syndrome?
AR loss of anterior parts of the spinal cord, and CN motor nuclei, causing a “floppy baby”
Large amount of blood on UA, but very few RBC = ?
Rhabdomyolysis (AU detecting myoglobin as blood)
Why should plain water never be given to infants under 6 months?
Can dilute out electrolytes since kidneys not yet fully functional
What are the s/sx of cavernous sinus thrombosis?
- HA
- binocular palsies
- periorbital edema
- Hypoesthesia
- Hyperesthesia in V1, V2
- CN III, IV, and VI impingement
What are the biologic DMARDs for RA?
- Etanercept
- Infliximab
- Adalimumab
- Tocilizumab
- Rituximab
When should pts with RA be started on DMARDs? Why?
ASAP to reduce bony erosions and disease progression
What is the first line DMARD for RA?
Methotrexate
What are the head imaging findings of JC virus?
Patchy white areas c/w demyelination
What lab test can differentiate between chloride responsive and unresponsive metabolic alkalosis?
Urinary Cl excretion will be high is unresponsive, and low if responsive
What happens to urinary Cl with hyperaldosteronism?
Increased, since there is no stimulus to hold on to Cl
What is the most common bodily secretion that transmits CMV?
Saliva (think mono)
What is the mechanism of a subdural bleed with shaken baby syndrome?
Shearing of the subdural veins
When is rolling over achieved in development?
4-6 months
What virus most commonly causes myocarditis, especially in children?
Coxsackie virus
What is the acute treatment for myocarditis?
Diuretics and inotropes