3 Flashcards
Cardiac involvement of amyloidosis typically presents with what type of cardiomyopathy?
Restrictive; may progress to dilated
Features of restrictive cardiomyopathy on echocardiogram?
Thickened ventricular walls and diastolic dysfunction
Dx amyloidosis?
Tissue biopsy
Alcohol-related heart disease typically presents with what type of cardiomyopathy?
Dilated
Restrictive cardiomyopathy typically presents with what type of heart failure?
Right-sided
Constrictive pericarditis typically presents with what type of heart failure?
Right-sided
In addition to amyloidosis, list two other infiltrative diseases that initially present with restrictive, then dilated, cardiomyopathy.
Hemochromatosis
Sarcoidosis
Other possible presenting symptoms/signs of amyloidosis?
Asymptomatic proteinuria or nephrotic syndrome Hepatomegaly Macroglossia Peripheral/autonomic neuropathy Waxy skin thickening, easy bruising
Symptoms of catatonia?
Immobility (or excessive purposeless activity)
Mutism, stupor (decreased alertness and response to stimuli)
Negativism (resistance to instructions and movement)
Posturing (assuming positions against gravity)
Waxy flexibility
Echophenomena
Catatonia most often presents in the context of what two psychiatric illnesses?
Bipolar disorder
MDD
(It may occur in schizophrenia, ASD, general medical illnesses, etc.)
Symptoms of akinetic mutism?
Similar to catatonia, but NO echophenomena
Cause of locked-in syndrome?
Pontine stroke
Symptoms of locked-in syndrome?
Paralysis, mutism, preservation of cognitive abilities
The numerous causes of anemia may be divided into what three categories?
- Decreased RBC production
- Increased RBC destruction
- Blood loss
Pathophysiology of anemia in lymphoproliferative disorders?
Bone marrow infiltration with cancerous cells
Which contraceptive has weight gain as a common side effect?
Medroxyprogesterone injections
Features of HELLP syndrome?
Preeclampsia N/V RUQ or epigastric pain microangiopathic Hemolytic anemia Elevated liver enzymes Low platelet count
Rx HELLP syndrome?
Delivery
Magnesium for seizure prophylaxis
Antihypertensives
Acute fatty liver of pregnancy overlaps with HELLP syndrome, but patients with AFLP are more likely to have additional extrahepatic complications such as…?
Leukocytosis
Hypoglycemia
Acute kidney injury
Sudden-onset dyspnea, hypoxia, and crackles in the setting of severe preeclampsia is most likely due to what?
Acute pulmonary edema
Pathophysiologic mechanism of acute pulmonary edema in severe pre-eclampsia?
Generalized arterial vasospasm (systemic HTN) -> increased afterload -> increased pulmonary capillary pressure -> pulmonary edema
Decreased albumin
Decreased renal function
Increased vascular permeability due to endothelial damage
Symptoms of magnesium sulfate toxicity?
Neuromuscular depression
Decreased respiratory effort/apnea, muscle paralysis, somnolence, visual disturbances, decreased/absent DTRs
Strenuous activity during hot and humid weather, dehydration, poor acclimatization, lack of physical fitness, and obesity are risk factors for exertional heat stroke. Several medications also increase risk - list them (4).
Anticholinergics
Antihistamines
Phenithiazines
Tricyclics
Clinical manifestations of exertional heat stroke?
Core temperature >40 (104 F) immediately after collapse AND CNS dysfunction (AMS, confusion, irritability, seizure), additional organ/tissue damage (renal/hepatic failure, DIC, ARDS)
Management of exertional heat stroke?
Rapid cooling - ice water immersion preferred; can consider high-flow cool water dousing, ice/wet towel rotation, evaporative cooling
Fluid resuscitation
Electrolyte correction
Management of end-organ complications
No role for antipyretic therapy
Features of non-exertional (or classic) heat stroke?
Similar clinical features and potential complications
Occurs in the absence of strenuous activity, typically effects elderly patients with many comorbidities that limit their ability to escape/cope with excessive heat
Management for non-exertional heat stroke?
Evaporative cooling (rather than ice-water immersion)
AE of aminoglycosides (eg, gentamicin)?
Ototoxicity to both the cochlea (sensorineural hearing loss) and vestibular system (imbalance)
Features of AG ototoxicity?
- Bilateral, but not necessarily symmetric, hearing loss (whisper test)
- Imbalance and oscillopsia (bilateral vestibular systems affected)
- Positive head thrust test (inability to maintain visual fixation during forced, rapid head movement, evaluates vestibulo-ocular reflex, which is affected by peripheral but not central vestibulopathies)
Distinguish BPPV from AG ototoxicity.
BPPV: brief (<1 minute) episodic vertigo stimulated by specific head movements, no hearing loss, confirmed by Dix-Hallpike (not head thrust)
Features of Meniere disease?
Discrete episodes of spinning vertigo accompanied by hearing loss and tinnitus or aural fullness
Features of tinea capitis?
Most common in AA children and immunocompromised
Causes a scaly erythematous patch that can progress to alopecia with inflammation, LAD, and scarring +/- black dots in affected area
Rx tinea capitis?
Oral griseofulvin or terbinafine
Characteristic lab findings of HUS?
Microangiopathic hemolytic anemia
Thrombocytopenia
AKI
Lab findings indicating RBC destruction?
Increased LDH Low haptoglobin (binds free Hgb normally; if free Hgb increases, haptoglobin decreases) Increased unconjugated bilirubin Hemoglobinemia Hemoglobinuria
Decreased ferritin?
Iron deficiency anemia
Compare clinical presentation of transient synovitis and septic arthritis in children.
TS: well-appearing, afebrile or low-grade fever, able to bear weight
SA: ill-appearing, febrile, non-weight bearing
Compare diagnostic work-up of TS vs. SA in children.
TS: normal or mildly elevated WBC, ESR/CRP, unilateral or bilateral U/S effusion, Dx of exclusion (key)
SA: moderately elevated WBC, ESR, CRP, +/- positive blood culture, unilateral U/S effusion, synovial fluid WBC >50,000
In patients with overlapping clinical features of TS and SA (for example, low grade fever, mildly elevated ESR, severe pain, inability to ambulate, leukocytosis), what should be done?
Bilateral hip U/S - if unilateral, arthrocentesis performed
Characteristic finding aplastic anemia?
Decreased reticulocyte count in the presence of anemia
Expected acid-base disturbance in acute asthma exacerbation?
Respiratory alkalosis with a low PaCO2 due to hyperventilation
What is an alarming finding suggesting impending respiratory failure in patients with an acute asthma exacerbation?
Normal or elevated PaCO2
Most common pediatric bony malignancy?
Osteosarcoma
Second most common pediatric bony malignancy?
Ewing sarcoma
Clinical features of Ewing sarcoma?
Chronic, localized pain and swelling
Long-bone diaphyses and axial skeleton (eg, pelvis)
+/- systemic findings
Early mets
X-ray findings of Ewing sarcoma?
Central lytic lesion
Onion skinning (lamelleted periosteal reaction)
Moth-eaten appearance
Periosteal elevation (Codman triangle -> non-specific finding)
2 bone tumors located in the epiphysis?
- Giant cell tumor
2. Chondroblastoma
2 bone tumors located in the metaphysis?
- Chondrosarcoma
2. Osteosarcoma
6 bone tumors located in the diaphysis?
Round cell lesions: Ewing tumor, malignant lymphoma, myeloma
Fibrosarcoma
Non-ossifying fibroma
Bone cyst
Bone tumor presenting with a painful mass in patients age >30, X-ray shows osteolytic lesion with well-defined or ragged moth-eaten margins
Fibrosarcoma
Bone tumor presenting in young adults with pain and swelling, X-ray shows lytic area with a “soap bubble” appearance
Giant cell tumor
Bone tumor presenting in adolescent boys, commonly located in the proximal femur, causes pain at night that responds to NSAIDs, small round lucency on X-ray
Osteoid osteoma
Presents in children and young adults, typically located at the proximal femur or humerus, pathologic fracture is the common presentation, cystic lesion with well-defined margins
Unicameral bone cysts
Presentation of hereditary spherocytosis in an infant?
Prolonged neonatal jaundice
Findings of hemolysis
Elevated MCHC
(Low to normal MCV, negative direct Coombs, spherocytes on peripheral smear)
Rx hereditary spherocytosis?
Supportive
RBC or exchange transfusion if hemolysis is severe
Blood group A - antigens on RBC and antibodies in serum?
A-Ag
Anti-B
Blood group B - antigens on RBC and antibodies in serum?
B-Ag
Anti-A
Blood group AB - antigens on RBC and antibodies in serum?
A-Ag + B-Ag
None
Blood group O - antigens on RBC and antibodies in serum?
None
Anti-B + Anti-A
Timing of presentation of beta thalassemia?
After 6 months (after fetal Hgb decreases)
Ab found in Hashimoto thyroiditis?
Anti-TPO (antithyroid peroxidase)
Anti-thyroglobulin may also be present
High titers of anti-TPO are associated with increased risk of ___ in both euthyroid and hypothyroid women.
Miscarriage
Compare thyroid size in hypothyroidism with anti-TPO vs. anti-TSH receptor Ab.
Anti-TPO: enlarged
Anti-TSH receptor: atrophy
Features of primary syphilis?
Painless genital ulcer (chancre)
Features of secondary syphilis?
Diffuse maculopapular rash beginning on the trunk and extending to the extremities (also involves palms and soles), LAD (epitrochlear -> pathognomnoic), condyloma lata (raised gray genital papules), oral lesions, hepatitis
Features of latent syphilis?
Asymptomatic
Features of tertiary syphilis?
CNS (tabes dorsalis, dementia)
CV (aortic aneurysm/insfufficnecy)
Cutaneous (gummas)
Dx syphilis?
Combination of non-treponemal (RPR) and treponemal-specific (T. pallidum immunoassay) tests
Rx primary and secondary syphilis?
One dose IM penicillin G benzathine
Confirm adequate treatment by a 4-fold decrease in titers at 6-12 months
Presentation - painless vaginal bleeding after 20 weeks gestation?
Placenta previa
3 risk factors for placenta previa?
Prior placenta previa
Prior C-section
Multiple gestation
Smoking
Dx placenta previa?
Transabdominal, then TVUS
Management of placenta previa?
No intercourse
No digital cervical exam
Inpatient admission for bleeding episodes
Most resolve by T3
If persistent previa, deliver via C-section at 36-37 weeks
Define pre-term labor.
Cervical dilation of 3+ cm or effacement with regular painful uterine contractions at <37 weeks
Both transfusion-associated circulatory overload and transfusion-associated acute lung injury may present with acute dyspnea and diffuse bilateral infiltrates on CXR. How can they be distinguished?
TRALI: absent JVD, crackles/rales on auscultation, normal EF, normal BNP
TACO: present JVD, crackles/rales +/- S3, decreased EF, high BNP
General causes of thrombocytopenia?
- Decreased platelet production
- Increased platelet destruction
- Dilutional
- Splenic sequestration
DDx - thrombocytopenia due to decreased production
- Viral infections (EBV, hepatitis C, HIV, etc.)
- Chemo
- Myelodysplasia (especially if >60 years old)
- Alcohol use
- Congenital (eg, Fanconi syndrome)
- Vitamin B12 or folate deficiency
DDx - thrombocytopenia due to increased destruction
- SLE
- Medications (eg, heparin)
- ITP, DIC, TTP, HUS
- Antiphospholipid syndrome
Schilling test?
Determine whether a patient had vitamin B12 deficiency due to pernicious anemia or a malabsorption syndrome
ITP is a diagnosis of exclusion.
Don’t forget it.
Mainstay of therapy in patients with alcoholic cardiomyopathy (can lead to normalization of LVF over time)?
Complete cessation of alcohol consumption
AFib is most commonly caused by ectopic foci in what location?
Within the pulmonary veins
Atrial flutter commonly involves a re-entrant circuit around what structure?
Tricuspid annulus
AV nodal re-entry tachycardia results from a re-entrant circuit formed by what structure?
2 separate conducting pathways within the AV node
EKG findings of AFib?
Absent P waves (replaced by tiny chaotic fibrillatory waves)
Irregularly irregular R-R intervals
Narrow QRS
Characteristic EKG finding of WPW?
Slurred upsloping R waves (aka delta waves)
EKG findings of AV nodal re-entry tachycardia?
Sudden onset and termination
Rapid (140-250/min) regular rhythm
Narrow QRS
Absence of definite P waves
EKG findings of sinus tachycardia?
Rate 100-180/min
Regular R-R intervals
Normal P waves and QRS complexes
EKG findings of atrial flutter?
Sawtooth waves
What is the major cause of morbidity and mortality in patients with PAD?
CV disease
Patients with PAD and intermittent claudication have an estimated 20% 5-year risk of ___ and ___, and a 15-30% risk of death due to CV causes. Only 1-2% of patients progress to develop ___.
Non-fatal MI; stroke
Critical limb ischemia with risk of limb amputation
Initial treatment of chronic venous insufficiency?
Conservative measures (leg elevation, exercise, compression therapy)
If no response -> venous duplex U/S to confirm diagnosis by identifying venous reflux
Estimate CVP = ?
JVP + depth to R atrium (5 cm)
Normal CVP?
6-8 cm H2O (elevated >8)
Splenic infarction usually occurs in what settings?
Splenic artery (or sub-branch) occlusion due to hypercoagulable states, embolic disease, or hemoglobinopathy
Although patients with sickle cell trait are generally aymptomatic, they occasionally develop splenic infarct and intravascular hemolysis in what settings?
Flying at high altitude
Dehydration due to alcohol consumption
Treatment with vitamin ___ reduces morbidity and mortality rates in children with severe measles.
A
How is measles transmitted?
Airborne
Clinical presentation of measles?
Prodrome (eg, cough , coryza, conunctivitis, fever, Koplik spots)
Maculopapular exanthem (cephalocaudal and centrifugal spread, spares palms and soles)
Features of hereditary hemorrhagic telangiectasia (aka Osler-Weber-Rendu syndrome)?
Diffuse telangiectasis
Recurrent epistaxis
Widespread AV malformations (tend to occur in mucus membranes, skin, GI tract; may be present in liver, brain, lung)
AV malformations in the lungs cause what findings?
Chronic hypoxemia
Digital clubbing
Reactive polycythemia
Can present as massive, sometimes fatal, hemoptysis
Findings of polycythemia vera?
Increased production of all 3 blood cell lines, splenomegaly, >60 y/o
Renal transplant dysfunction in the early post-operative period manifests as oliguria, HTN, and increased creatinine/BUN. It can be explained by many causes, including what?
Ureteral obstruction (renal U/S) Acute rejection (biopsy -> lymphocyte infiltrate) Cyclosporine toxicity Vascular obstruction ATN And more
Rx acute rejection?
High-dose IV steroids
Characteristic features of child abuse in the form of deliberate scald injuries?
- Burns with sharp lines of demarcation
- Uniform depth
- Lack of splash marks
- Spared flexural creases (due to ankle, knee, and hip flexion at the time of forced immersion)
In DKA, measured serum K levels are often initially high - why?
Extracellular shift of K driven by increased plasma tonicity
Loss of insulin-dependent potassium uptake across cell membranes
Rx acute exacerbations of MS?
Glucocorticoids
Consider plasmapheresis if refractory
Maintenance therapy in patients with relapsing-remitting or secondary, progressive forms of MS?
Interferon beta
Glatiramer acetate
Why may baclofen be used in MS?
Treatment of muscle spasticity
___ is a myeloproliferative disorder marked by erythrocytosis. It is almost always caused by a mutation of ___ in a myeloid precursor cell.
Polycythemia vera; JAK2
Symptoms of polycythemia vera?
Increased blood viscosity -> HTN, erythromelalgia (burning cyanosis in hands/feet), transient visual disturbances
Increased RBC turnover -> gouty arthritis
Aquagenic pruritus (itching after bathing)
Bleeding
Exam findings in polycythemia vera?
Facial plethora (ruddy cyanosis) Splenomegaly
Lab findings in polycythemia vera?
Elevated Hgb
Leukocytosis and thrombocytosis
Low EPO level**
JAK2 mutation positive
Complications of polycythemia vera?
Thrombosis
Myelofibrosis and acute leukemia
Treatment of polycythemia vera?
Phlebotomy
Hydroxyurea (if increased risk of thrombus)
BCR-ABL fusion protein?
CML
Severe nodulocystic acne may be the initial sign of hyperandrogenism in patients with ___.
PCOS
DDx of hyperandrogenism?
PCOS
Androgen-secreting tumor
Cushing syndrome
Non-classic CAH
What statistical method is used to compare the means of two groups of subjects?
Two-sample t test
The t statistic is obtained to calculate what value?
P value
How does a two-sample z test differ from the t test?
Used to compare two means, but uses population variances, not sample variances
What statistical test is used to compare 3+ means?
ANOVA
What statistical test is appropriate for categorical data and proportions?
Chi-square test
Pediatric chronic rhinosinusitis with nasal polyposis + exocrine pancreatic dysfunction = ?
Cystic fibrosis
Features of CF?
Sinusitis Barrel chest Pneumonia/bronchiectasis Cachexia Biliary cirrhosis Pancreatic exocrine insufficiency Diabetes Digital clubbing Inspissated stool Absent vas deferens
How does primary ciliary dyskinesia differ from CF in its presentation?
No GI symptoms
In patients with upper GI bleeding, ongoing hematemesis, and depressed level of consciousness, what key step must be done?
Intubation to protect the airway prior to endoscopic treatment
What is the most common cardiac manifestation of SLE?
Pericarditis
EKG findings of pericarditis?
Diffuse ST elevation and PR depression, eventual diffuse T wave inversion