Q Banks: Week of 03/20/17 Flashcards
What does opsoclonus-myoclonus syndrome likely portend in a young child?
Neuroblastoma
With a mean age of onset of 2 years, neuroblastoma is the most common extracranial tumor of childhood. It causes a disorder in which children can have non-rhythmic nystagmus with myoclonus.
True or false: tardive dyskinesia specifically refers to tongue movements.
False. Tardive dyskinesia refers to any purposeless repetitive movements such as writhing fingers or facial grimacing.
How is acute dystonia different from tardive dyskinesia?
Acute dystonia is sustained muscle contraction, whereas tardive dyskinesia is just brief movements.
Explain the phenomenon of orthopnea.
Orthopnea is a sign of left-heart failure. Left-heart failure will lead to edema throughout the body due to inadequate circulation. Much of the fluid pools in the lower extremities due to the force of gravity. When a person with LH failure lies supine, that fluid quickly returns to the right heart via increased venous return. If the left heart cannot match the output of the right heart, then fluid will pool in the lungs.
Paresthesia in the extremities and a papular rash on the groin area are indicative of a disorder that leads to the accumulation of what metabolite?
Globotriaosylceramide
Note: this accumulates in muscle tissue. The earliest sign of Fabry disease is inability to sweat due to loss of smooth muscles in sweat glands.
What demyelinating disorder that results from a missing enzyme presents with ataxia, seizures, and hypotonia?
Metachromatic leukodystrophy
This is a defect in arylsulfatase
What kind of pneumonia produces foul-smelling sputum?
Aspiration pneumonia
In addition to alcohol overdose, drug overdose, and neurologic disorders, _____________ can also produce aspiration pneumonia.
seizure disorders
Explain the utility of paraaminohippuric acid (PAH).
PAH is both filtered by and excreted by the kidneys. Also, it is NOT REABSORBED AT ANY POINT and so provides an approximation of renal blood perfusion.
What does this mean? Well, filtration into Bowman’s capsule is not saturable and is only limited by GFR. As such, the more PAH you put into the glomerulus, the more will be filtered. PAH is also excreted into the PCT. This is a carrier-protein mediated process that can be saturable. So if you increase the concentration of PAH, you will see the extraction ratio increase up to the point at which the carrier is saturated.
If a patient is hypoxemic but his A-a gradient is normal (i.e., 10 or less), then the etiology has to be one of two things: __________________.
hypoventilation or high altitude (or any other low-oxygen air inhalation)
PAH concentration is likely to be lowest in which part of the nephron?
Bowman’s space (because it is secreted into the PCT and not reabsorbed at any point)
True or false: in hypertrophic cardiomyopathy, the interventricular septum overrides the aortic valve and blocks the outlet.
False-ish. The mitral valve can randomly get pushed back toward the aortic valve in those with HOCM under stress (say, while exercising). When this happens, the mitral valve AND the interventricular septum block the aortic valve.
Give a rundown on the most important points about PCOS.
- The main abnormality is an elevated LH:FSH ratio. This leads to increased androgen production by theca cells.
- The androgens lead to hirsutism and infertility. Estrone levels rise when androgens are converted to estrogen peripherally.
- The elevated estrogen shuts off FSH production and thus follicles fail to develop.
- Unopposed estrogen leads to endometrial hyperplasia.
- Elevated LH leads to hypertrophy of the ovaries.
What are some key treatments for PCOS?
- Weight loss can often revert the whole axis back to normal.
- Diabetes treatment (metformin) can usually restore ovulatory function if a patient wishes to conceive.
- Another route to restore fertility is to use the SERM clomiphene! It blocks estrogen selectively at the pituitary/hypothalamus and thus tricks the body into secreting more FSH.
- In those who do not want any of the above, it is recommended that they take dual hormone OCPs which will decrease LH secretion and stop the unopposed estrogen high (to reduce risk of endometrial cancer).
_____________ make S. viridans able to adhere to fibrin.
Dextrans (synthesized from sucrose)
M. leprae invades which cells?
Skin and nerves
Cardiomegaly and hepatomegaly in a five-month-old will likely be present in a disorder that causes ________-positive samples on biopsy.
acid-Schiff (PAS)
This stains for glycogen and will be found in kids with Pompe disease.
What histologic pattern will contact dermatitis present with?
Spongiosus – accumulations of fluid in between cells of the epidermis
The right atrium and SVC are in very similar spots in an x-ray of the heart. What landmarks are they normally near?
- RA: below the right bronchi and roughly one or two ribs above the resting diaphragm
- SVC: about where the right bronchi is and three or four ribs above the resting diaphragm
Explain how UAA, UGA, and UAG stimulate release of mRNA from the ribosome.
The three stop codons bind to releasing factors in the cytosol that trigger the release of mRNA from the ribosome.
What cellular process leads to cavitation formation in lung diseases?
Release of lysosomal enzymes from macrophages and neutrophils
Which two vasculitis disorders present with granulomas of the media?
Takayasu and giant cell
Imagine a giant cell destroying a Japanese city called Takayasu –that would get a lot of MEDIA coverage.
People who get ABPA will typically have high levels of what two serum markers?
- IgE
* Eosinophils
Patients with membranous nephropathy often have antibodies to what?
Phospholipase A2 receptor (PLA2R) on podocytes
Note: this is what causes the immune deposits in MN.