PEM Board Studying

Hypopigmented lesions associated with seiures

Tuberous sclerosis; associated with shagreen patches, ashleaf spots (hypopigmented lesions) and angiofibromas

Complication of over drainage of VP shunt

subdural hematoma

Goal of lowering BP in hypertensive emergency

Lower 25% in the first 8 hrs of the difference from current systolic BP - goal systolic BP (also get head imaging prior to lower BP).

Patient with programmable VP shunt presenting with vomiting after head imaging a few weeks ago

If not reprogrammed after imaging —> can increase risk of suboptimal drainage and increased ICP

Normal VP shunt pressure and if elevated

12 cm H20; if elevated then suspect distal shunt failure, if low then suspect proximal shunt failure

Hemiparesis, ataxia, dysarthria, oculomotor deficits after CHI

Suspect posterior circulation stroke (most common cause is vertebral artery dissection)

Age of Infantile spasms

90% < 12 months of age

Immune-mediated paraneoplastic presentation associated with neuroblastoma, sometimes others such as ganglioneuroblastoma

Opsoclonus Myclonus

Imaging to get in patients with suspected opsoclonus myoclonus

CT or MRI of chest, abdomen and pelvis

Medication of choice for neonate that prevents in severe hypoxemia with palpable liver edge

Prostaglandin gtt at 0.05 mcg/kg/min to maintain ductus arteriosus patency. Keeping of duct open allows mixing of pulmonary and arterial blood to the systemic circulation

First line therapy for patient (2 mo with sweating with feeds, liver edge palpated and delayed cap refill)

CHF —> Diuretics (Furosemide)

Older adolescent who presents with heart failure symptoms while completing drills at practice (associated JVD, CXR with cardiomegaly, murmur harsh systolic ejection murmur along the L sternal border.)

heart failure associated with hypertrophic cardiomyopathy

Treatment of heart failure associated with hypertrophic cardiomyopathy

Betabloockers (metoprolol 0.1 mg/kg) used for obstruction, diuretics contraindicated as they will worsen the LVOT obstruction)

EKG changes with pericarditis

widespread ST elevation*, PR segment depression

Elements of Pericarditis diagnosis

Pleuritic chest pain, pericardial friction rub, EKG changes, new or worsened effusion (need 2 of these)

Approach to drain pericardial effusion if able to visualize with US

L parasternal approach (5th or 6th rib space)

Diagnostic marker for Myocarditis

Troponin; EKG is low sensitivity (can show non specific ST segment or T wave changes and sinus tachycardia)

Sharp chest pain at rest or during mild exercise, localized with the finger tip at the L sternal border

Precordial catch aka Texidor’s twinge (*don’t order EKG; provide reassurance)

15 yo with acute onset neck and chest pain after inhaling helium and with exam noting neck tenderness/swelling along the SCM bilateral, fair aeration but decreased breath sounds

Spontaneous pneumomediastinum

Chest XR findings associated with spontaneous pneumomediastinum

shows air tracking around and outlining mediastinal structures

Treatment of spontaneous pneumomediastinum

self resolves and can provide oral analgesics

16 yo with flu-like symptoms, eyes “blood shot”, urine with pink twinge, febrile, has a heart murmur. What test is needed?

Concern for infective endocarditis —> blood culture

Rash in patient with heart murmur and arthritis

Concern for rheumatic fever, rash is erythema marginatum. Need 2 major criteria or 1 major and 2 minor criteria.

o   Major: carditis* (pansystolic cardiac murmur at apex indicating mitral valvulitis), arthritis, chorea, erythema marginata, subcutaneous nodules

o   Minor: arthralgia, fever, elevated inflammatory markers, elevated PR interval)

Patient overdosed on medications; what drug to give her?

Sodium bicarbonate due to concern for TCA ingestion; see prolonged PR and widened QRS

Massive hemoptysis definition

> 240 ml within 24 hrs or recurrent bleeding > 100 ml daily for several days

Vent goals in COVID PNA

Lower TV 4-6 ml/kg and optimize peep; keep O2 88-95%

Pulmonary hemorrhage vent strategy

low TV and high PEEP

Imaging of ? and Management?

CPAM; congenital pulmonary airway malformation with cystic and adenomatous elements. Tx with surgical lobectomy in large CPAMs; consult surgery*

Chylothorax findings

triglycerides greater than 110, cholesterol lower than 200 along with lymphocyte predominance

“Kyle got 100% on his Test”

14 yo with URI symptoms with L sternal chest pain and localized area of tenderness and nonsuppurative edema over the costochondral junction

Tietz’s syndrome; -Benign, painful, nonsuppurative localized swelling of the costochondral joints, most often involving the 2^nd and third ribs; tx with NSAIDs

How is diagnosis of sarcoidosis made?

lymph node biopsy (noncaseating granulomas)

Earlier complication of lung transplant

Anastosis ( Donor lungs are attached at the level of the main bronchus which is a frequent site of complications)

Later complication of lung transplant

Bronchial stenosis (in Q stem surgery was 9 mo ago); dyspnea, stridor, wheezing, post obstructive PNA and require scope for stenosis dilation

Factor for Hemophila A and B

A; factor 8, B: Factor 9

PT/PTT and plts in Hemophilia A and B

normal plts, normal PT, abnormal PTT (intrinsic pathway including VIII and IX)

PT/PTT in Factor VII deficiency

abnormal PT, normal PTT

How much correction to give in patient with Hemophilia A and head trauma

100% correction in cases of severe bleeding or high risk of bleeding

Patient with Hemphilia and presents as abdominal pain, flexure contraction at the hip, with lower extremity paresthesia

Think Psoas abscess; compression of femoral nerve from abscess causes the paresthesia - diagnose with CT of abdomen/pelvis

PT/PTT in vWD

normal; note abnormal in hemphilias and factor VII deficiency

Plt level in ITP that increases risk of ICH

< 10,000

Treatment vs. supportive care for ITP

Mucosal bleeding or significant bleeding

First line therapy for autoimmune hemolytic anemia

Steriods for first line; note labs show: anemia, high retic, +coombs, elevated LDH/bili (all signs of hemolyic anemia)

Difference between FFP and Cryoprecipitate

FFP is more broad and has all components to help with bleeding; Cryoprecipitate is made from FFP and includes vWF, fibrinogen and coagulation factors

Electrolyte abnormalities when providing significant blood transfusions

Hypocalcemia from citrate, hypomagnesemia, hypo/hyperkalemia

Lab abnormalities associated with tumor lysis syndrome

Hyperkalemia, hyperphosphatemia, HYPOcalcemia and hyperurecemia

A neutropenic patient with RLQ pain

Consider typhlitis on differential (can influence the cecum and large intestine)

Imaging for Typhlitis

XR shows paucity of air in the RLQ and can get serial Xrs to look for development of free air (although technically US and CT can be more definitive for the diagnosis itself)

< 6 yr old with anemia, hypertension, hematuria and painless abdominal mass

Nephroblastoma/Wilms tumor (most common pediatric malignancy)

Location for most neuroblastomas

Can be 60% adrenals and 20% anterior mediastinum

Test to detect neuroblastoma

Urine catecholamines elevated in 95% of patients with this disease

Most common primary malignant bone tumor in pediatric patients and disposition if identified

osteosarcoma; need to be admitted as they are highly malignant

Age for Dtap vs. Tdap

< 6 should get Dtap

When to give Tetanus immunoglobulin

If unvaccinated with dirty wound; (if given > 3 doses of vaccine in general don’t need to give immunoglobulin)

When to give tetanus vaccine

If unvaccinated with clean or dirty wound, for dirty wound if > 5 years since last vaccine

Size of abscess for I&D of RPA

> 2.5 cm in diameter

At what age is the Monospot less sensitive and EBV titers more necessary

< 5

Rash spread in Measles

Head down toward the rest of the body

Most specific test of disseminated HSV

HSV PCR of blood, but in question stem it was LFTs (as the HSV PCR was not a possible response)

Difference between viral, bacterial and fungal meningitis labs

In fungal and viral ; you have > WBC, but not > 1000, in fungal you have elevated protein > 200 like bacterial (viral has < 200 protein), in fungal you have low glucose like bacterial, viral has normal protein

Age it is safe to give Doxy for Lyme disease

If > 8 yrs of age; less than 8 give amoxicillin

Labs in RMSF

Hyponatremia and thrombocytopenia

Rash spread in RMSF

starts on palms and soles and spreads centrally

How to test for malaria

Rapid antigen test has 85-95% sensitivity and specificity; thin and thick smear are confirmatory testing

Child with vomiting and elevated LFTs in daycare

Most likely Hepatitis A; need health department clearance prior to return to daycare (disease is self limited)

Post exposure ppx recommendations in immunocompromised patients exposed to flu?

Vaccine + osteltamivir daily x 7 days

Varecella zoster ppx in immunocompromised patients?

Should get varicella zoster immunoglobulin; NOT vaccine given that is a live vaccine

Electrolyte side effects of immunosuppressive meds (tacrolimus or mycophenolate)

hypomagnesemia; can present as spasm, tremor, EKG changes (pACs, QRS widening)

What medication can interact with tacrolimus and cause toxicity? symptoms of tacro toxicity?

azthromycin; if concerned get a tacolimus level (headache, hypertension, seizures, *hand tremors)

Presents with improved shortness of breath and oxygen saturation when lying flat (platypnea and orthodeoxia)

Hepatopulmonary syndrome (in Liver transplant patients); cause increased pulmonary capillary dilation and occasionally direct AV connections. To test get a contrast ECHO (aka bubble study)

What virus is typically positive in PTLD patients?

EBV+; labs also show elevated LDH and uric acid”

splenomegaly, thrombocytopenia and abdominal pain in patient with liver transplant

Portal vein thrombosis; need abdominal US with doppler to evaluate

Most frequent clinical manifestation of graft vs. host disease

Rash; especially seen on. palms and soles. Need skin biopsy to diagnose.

Test of Toxoplasmosis

PCR of Blood; Crypto is DFA

Test for Cryptosporidium

Stool direct immunofluorescent antibody (DFA); Toxo is blood PCR

Metabolic acidosis, hypoglycemia and NO ketones

Fatty acid disorders (MCADD); have abnormal urine organic acids and acyl carnitine profile

Metabolic acidosis, elevated lactate, + ketones

Organic aciduria; have abnormal urine organic acids

Test for PKU

abnormal serum amino acids

Labs for galactosemia

+ urine reducing substances

Neonate with lethargy, poor feeding, hypoglycemia, prolonged jaundice, cloudy corneas

Galactosemia (+ urine reducing substances)

2 yo with who presents after extended periods of fasting and present with ketonemia (high ketones) and symptoms of lethargy

Ketotic hypoglycemia (common form of childhood hypoglycemia);

different than organic acidemia who also present with hypoglycemia and ketones in that patients with ketotic hypoglycemia present between 18 mo - 5 yrs of age where as organic acidemia present in neonates

2 yo with more protein who presents with AMS and lethargy

Urea cycle disorder; high ammonia, diagnosis with: abnormal plasma amino acids, enzyme assay

Treatment of Urea cycle defect with elevated ammonia

IV fluids, sodium benzoate and arginine (scavengers); if ammonia > 200 likely need dialysis

Infant with regression of developmental milestones, difficulty feeding and failure to thrive, significant hepatosplenomegaly and cherry red spot on the macule

Lysosomal storage disorder; Gaucher is one of the most common; tested with lysosomal enzyme assay

neonate presents with lethargy, malodorous smell, hypoglycemia and metabolic acidosis

Organic acidemia;’ tested with urine organic acids

Neonate with seizures in the setting of microcephaly, eczema, unusual odor

PKU; test with serum amino acids; elevated phenylalanine

lethargy, hypoglycemia most often brought on by periods of fasting or vomiting without ketones, also have metabolic acidosis

Fatty acid oxidation disorders (MCADD); metabolic acidosis, hypoglycemia and NO ketones; as opposed to organic acidemias which have similar presentation but have ketones. Diagnosed with acylcarnitine profile and urine organic acids.

Patients present with increased thrombosis risk in the setting of learning disabilities, dislocated lens, poor growth and skeletal abnormalities

Homocysteinuria; have elevated stoke risk and thrombosis; test with homocysteine level

Adolescent presents with psychiatric and behavioral issues alone with movement abnormalities and elevated LFts

Wilsons disease; elevated ceruloplasmin level

Adolescent with depression and psychosis as well as abdominal pain, blistering with sun exposure, red or brown urine

Porphyria; test with protoporphyrin level

Lab findings in DiGeorge syndrome

normal neutrophils, platelets and immunoglobulins, + lymphopenia (are at high risk of SCID with T cell deficiency) and hypoplastic thymus

Child presenting with recurrent pneumonia, abscesses, adenitis, osteomyelitis, sepsis; lab to test?

Chronic granulomatous disease; increase risk of catalase produce organisms; test with neutrophil oxidative burst index

Patient with immunodeficiency, thrombocytopenia and eczema

Wiskott Aldridge syndrome

Patient with severe immunodeficiency and eczema but NO thrombocytopenia

Job syndrome (hyper IgE); they als have facial bossing, coarse facies and retained primary teeth

Patient without tonsil adenoids or palpable lymphoid tissue

X-linked agammaglobulinemia; have severe hypogammaglobuliinemia and antibdody deficiency

What to give for patients with refractory anaphylaxis 2/2 to betablockers

Glucagon; of note; glucagon is also what you give when there is beta blocker overdose

Abnormal labs in DRESS syndrome

eosinophilia, leukocytosis, neutrophilia, liver dysfunction

How to test for adrenal insufficiency?

AM cortisol level or ACTH stim test

Neonate with bradycardia and periorbital rash

neonatal lupus

Labs in lupus

hypocomplementemia (low C3, C4 or CH50), + ANA, dddDNA, antiSm antibodies

Patient with heliotrope rash, Gotton sign, difficulty stading up and lower body weakness requiring upper body to help

Dermatomyositis