UWorld - All Subjects Flashcards
Your patient presents with irritability, abdominal cramps, anorexia, perioral dermatitis, and neurological signs (hand tremors and globally decreased reflexes). What is the most likely explanation?
inhalant abuse (glue, shoe polish, gasoline, spray paint)
What are the acute symptoms of inhalant use?
neurologic - tremors, disorientation, headaches, slurred speech, hallucinations
gastrointestinal - stomach cramps, nausea
cardiovascular - arrhythmia
respiratory - wheezing, coughing
psychiatric - irritability, mood swings, neurocognitive impairment, grandiosity
What are the symptoms of chronic inhalant use?
weight loss, anorexia, neurocognitive impairment, cerebellar dysfunction, and peripheral neuropathy (decreased reflexes)
What is an adverse effect of treatment with calcineurin inhibitors (tacrolimus, cyclosporine). When are these medications prescribed?
acute calcineurin inhibitor renal toxicity -> HTN and AKI caused by constriction of the afferent and efferent renal arterioles. (This is usually reversible with a decrease in the blood levels of the drugs)
These drugs are prescribed for organ transplant immunosuppression and inhibit lymphocyte-mediated rejection via inhibition of IL-2 production.
What adverse effect is shared by beta-lactam antibiotics as well as NSAIDs? What would you see on diagnostic tests?
both can trigger AKI due to acute interstitial nephritis (AIN). Evidence of inflammation (positive leukocyte esterase and elevated white blood cells with casts) is expected on urine studies.
In what time frame is acute tubular necrosis a concern following renal transplantation? What would you see on urine microscopy?`
most common in the first week following transplantation, usually resulting from preoperative graft ischemia.
You would expect to find muddy brown casts on urine microscopy.
PKU inheritance, deficiency, accumulated product, symptoms, diagnosis
inheritance: autosomal recessive
deficiency: phenylalanine hydroxylase
accumulation: phenylalanine
symptoms: severe intellectual disability, seizures, musty body odor, hypo pigmentation involving skin, hair, eyes, and brain nuclei
diagnosis: newborn screening exam OR quantitative amino acid analysis (if did not receive newborn screening)
Fructose intolerance deficiency, accumulated product, symptoms
deficiency: aldolase B
accumulated product: fructose-1-phosphate
symptoms: vomiting, poor feeding, lethargy; seizures/encephalopathy follow if fructose is not removed from the diet
galactosemia deficiency, symptoms, and time of presentation
deficiency: galactose-1-phosphate uridyl transferase
symptoms: jaundice, hepatomegaly, and failure to thrive after consumption of breast milk or infant formula
time: within the first few days of life
What are the levels of HgA, HgA2, HgF, and HgS in a normal patient?
HgA - 95-98%
HgA2 - ~2/5%
HgF - <1%
HgS - absent
What are the levels of HgA, HgA2, HgF, and HgS in a patient with beta-thalassemia minor?
HgA - decreased
HgA2 - increased
HgF - near normal
HgS - absent
What are the levels of HgA, HgA2, HgF, and HgS in a patient with beta-thalassemia major?
HgA - absent
HgA2 - significantly elevated
HgF - significantly elevated
HgS - absent
What are the levels of HgA, HgA2, HgF, and HgS in a patient with sickle cell trait?
HgA - significantly decreased
HgA2 - near normal
HgF - near normal
HgS - elevated
What are the levels of HgA, HgA2, HgF, and HgS in a patient with sickle cell disease?
HgA - absent
HgA2 - near normal
HgF - significantly elevated
HgS - significantly elevated
When do patients with beta thalassemia major present? What symptoms do they have? What symptoms can develop if this condition remains untreated?
- patients present around 6-12 months of life (due to HbF predominating in the first few months of life)
- present with fatigue and pallor due to microcytic anemia. splenic hemolysis can cause jaundice, dark urine, and splenomegaly.
- if left untreated, patients can develop skeletal abnormalities due to extramedulllary hematopoiesis.
What CXR findings are suggestive of foreign body aspiration?
unilateral lung hyperinflation with mediastinal shift to the unaffected side
BUT a normal radiograph does not rule out FBA because at least 30% of radiographs are normal
What procedure is used to confirm the diagnosis of foreign body aspiration and remove the object?
bronchoscopy
What is a common sequelae of congenital CMV?
sensorineural hearing loss
What are 2 common sequelae of congenital toxoplasmosis?
chorioretinitis and obstructive hydrocephalus
What is a common sequelae of congenital syphilis?
osteoarticular destruction (saber shins, Hutchison teeth, saddle nose)
What are 2 common sequelae of congenital rubella?
cataracts and sensorineural hearing loss (also present with PDA)
What 5 signs/symptoms would you expect in a congenital CMV infection?
- hepatosplenomegaly
- jaundice
- periventricular calcifications
- microcephaly
- thrombocytopenia
What is the treatment for congenital hearing loss?
antiviral therapy (valganciclovir, ganciclovir) - can preserve hearing function and improve neurocognitive development
What is the difference between the brain findings of congenital toxoplasmosis vs congenital CMV?
Toxo - obstructive hydrocephalus as well as parenchymal calcifications
CMV - hydrocephalus ex vacuo (due to parenchymal volume loss) and periventricular calcifications