Kaplan1 Flashcards
1
Q
Primary infection with Mycobacterium tuberculosis presents as what?
A
Primary infection with Mycobacterium tuberculosis is most frequently located in the lung parenchyma and hilar lymph nodes, often generating a granulomatous reaction called the Ghon complex.
2
Q
Chronic severe hypertension can produce the following changes in the vasculature: (4)
A
- Decreased number of arterioles
- Thick-walled arterioles with increased arteriolar wall-to-lumen ratio
- Increased vascular resistance of end organs
- Ischemic and/or hemorrhagic end organ damage
3
Q
Cytotoxic T cells require _________ molecules on the surface of tumor cells to eliminate them.
A
MHC class I
4
Q
What are the causes of early feeding difficulties in a neonate that presents in the first few days of life?
A
- Annular Pancreas
- Trancheoesophageal Fistula
- Duodenal atresia
5
Q
Neonate presents with early feeding difficulties and CT shows a mass lesions surrounding the duodenum.. What is the most likely issue?
A
Annular Pancreas
6
Q
What is an Annular Pancreas and what causes it.
A
The pancreas is formed from fusion of the dorsal and ventral buds, which develop from the embryologic foregut in the region that will form the duodenum.
At approximately the eighth intrauterine week of life, the ventral pancreas rotates around the duodenum to reach the dorsal bud and then fuse with it.
Normally, the ventral pancreas rotates around the right side of the duodenum. In annular pancreas, the ventral pancreas divides into two parts and one part rotates around the right side of the duodenum and the other part rotates around the left side of the duodenum.
This results in a encircling of the duodenum and may cause an obstruction. If the annular pancreas encircles the duodenum but does not constrict it, it is asymptomatic.
The figure below shows normal development of the pancreas and duodenum.
7
Q
Acute intermittent porphyria (AIP) can manifest clinically as ?
A
acute attacks with abdominal symptoms such as pain and constipation, peripheral neuropathy, and psychiatric manifestations.
Attacks can be precipitated by drugs such as sulfonamides and phenobarbital.
8
Q
What is the inheritance pattern of Acute intermittent porphyria (AIP)?
A
Acute intermittent porphyria (AIP) is an autosomal dominant genetic disorder.
9
Q
Corneal reflex:
Afferent limb = ???
Efferent limb = ???
A
Corneal reflex:
Afferent limb of the corneal blink reflex is by ipsilateral CN V.
Efferent limb of the corneal blink reflex is by bilateral CN VII.
10
Q
What syndrome presents as esophageal webs, dysphagia, and iron deficiency anemia.
A
Plummer-Vinson syndrome
11
Q
Plummer-Vinson syndrome -ptients are at risk for developing what?
A
squamous cell cancer in the upper two-thirds of the esophagus.
12
Q
What are the most common sequela associated with reflux esophagitis?
A
- Adenocarcinoma
- Barrett esophagus
13
Q
Loss of elastic recoil in the lung ~w/ ?
A
COPD.
decrease lung elasticity –> decrease FEV1, increase residual & Total lung volume.
14
Q
Lowing a cutoff point for a screening test towards normal would increase the SENSITIVY, what does this do to the Negative Predictive Value of the test?
A
Increases NPV, b/c of the DECREASE in FALSE NEGATIVES.
15
Q
The __________ sinus is located in the sphenoid bone under the pituitary gland.
A
sphenoid sinus
16
Q
The ________ can be approached transnasally via the sphenoid sinus for hypophysectomy.
A
pituitary
17
Q
How does administering Triiodothyronine (T3) affect TSH, serum T3, and Serum T4?
A
Decreases TSH
Increases T3 (duh…)
Decreases T4, d/t negative feedback.
That’s why the standard Tx for Hypothyroidism is T4, which has a longer halg-life than T3, and can get converted to active T3 by the body, so that both T4 and T3 levels increase.
18
Q
What is McCune-Alright Syndrome?
A
Herefitary disorder also known as POLYOSTOTIC FIBROUS DYSPLASIA.
auto stimulation of aromastase enzyme production of estrogen by the overies.
~ cyctic bone lesions
~ café-au-lait skin spots
19
Q
cyctic bone lesions, café-au-lait skin spots, with or without precocious puberty == ?
A
McCune-Alright Syndrome
20
Q
During the period of time between discontinuing the PTU and RAI therapy, the patient needs to have her hyperthyroid condition appropriately managed wiith what drug?
A
Propranolol is a nonselective beta-adrenergic receptor antagonist that may also mildly block the conversion of thyroxine (T4) to triiodothyronine (T3).
Beta-blockers can be used before, during, and after RAI therapy without interfering with radioiodine uptake into the thyroid gland.
Beta-blockers, such as propranolol and atenolol, are the drugs of choice for symptomatic therapy to improve adrenergic symptoms (tachycardia, tremor, and anxiety).
These agents do not affect the underlying hypermetabolic state. If the use of a beta-blocker is contraindicated, diltiazem or verapamil can be used for acute symptomatic treatment.
21
Q
What drug decreases the uptake of 131-Iodine by the thyroid follicular cell sodium-iodine symporter?
A
Potassium Iodine;
drug of choice for treatment of hyperthyroidism in preoperative thyroidectomy patients, because this agent decreases the vascularity of the thyroid gland, thereby increasing the chance for a more successful surgery.
22
Q
Post MI:
ventricular wall rupture leads to ?
Papillary muscle rupture leads to ?
A
Post MI:
ventricular wall rupture leads to cardiac tamponade.
Papillary muscle rupture leads to acute mitral valve prolapse.
23
Q
When do you see a True Ventricular Aneurysms?
A
seen several weeks post-MI.
Aneurysm is produced prom an outpouching of damage tissue that in lined by fibrotic tissue.
24
Q
ST elevation in leads: II, II, aVF, V5 and V6 ===> ?
A
Inferior myocardial infarction; d/t occulusion in RIGHT CORONARY a.
25
What are the potential complications of an Inferior myocardial infarction; d/t occulusion in RIGHT CORONARY a.?
- rupture of posterior left ventricular wall, leading to hemopericardium and cardiac tampnade (pulses paradoxus).
- rupture of interventricular septum
- rupture of the posterior papillary muscle --> acute mitral valve prolapse.
26
___________ is a feared complication of a ruptured ectopic pregnancy.
Exsanguination
27
_______________ is a very serious complication of mucinous tumors of the ovary or appendix.
Pseudomyxoma peritonei
28
What should be suspected in a patient with lower abdominal pain, vaginal discharge, adnexal tenderness, and tenderness with cervical motion.
pelvic inflammatory disease
29
Pelvic inflammatory disease may be due to infection with what?
N. gonorrhoeae, C. trachomatis, vaginal anaerobes, and/or facultative gram-negative rods.
30
What are complications of Pelvic inflammatory disease?
Complications include sterility, ectopic tubal pregnancy, bacteremia, peritonitis, and intestinal obstruction.
31
What can lithium therapy inhibit and what labs are needed for monitoring?
Lithium can inhibit thyroid function, which should be monitored by measuring thyroid-stimulating hormone (TSH) every 6-12 months of therapy.
32
In a patient with amenorrhea, a very shallow vagina and no obvious uterus and cervix, what should be suspected?
testicular feminization and look for palpable masses in the labia that might be abnormal testes.
Patients with testicular feminization have 46,XY genetics and a disorder of the androgen receptor that prevents normal male external genitalia from developing.
Secretion of Müllerian inhibiting factor and testosterone still occurs in these patients.
33
What cell releases MIF, and what does MIF do?
Sertoli Cells release MIF.
MIF prevents that development of the Paramesonephric duct.
34
What is a sign of stage 2 sleep?
K-complexes (choice B) are a sign of stage 2 sleep, the most common stage of sleep.
Although more sleep time for the elderly person is spent in Stage 2 sleep, the decline in total sleep time also means that Stage 2 sleep and K-complexes would also decline.
35
REM or Rapid Eye Movement sleep is characterized by what?
an aroused EEG pattern, sexual arousal, saccadic eye movements, and elaborate visual imagery (dreaming). The figure below summarizes the different types of sleep.
36
- Low-grade tumor in children and young adults
- Usually in the posterior fossa in children
- Cyst with a mural nodule
- Pathology: Rosenthal fibers, immunostaining with GFAP
Pilocytic astrocytoma
37
Pathology: Rosenthal fibers, immunostaining with GFAP
is what ???
Pilocytic astrocytoma
38
The presence of corkscrew-shaped, intensely eosinophilic structures deriving from accumulation of αβ-crystallin within astrocytic processes is an important histopathologic clue for pilocytic astrocytoma.
This are aka ???
Rosenthal fibers
39
cells organized around small vessels (perivascular pseudorosettes) or around small lumina (true ependymal rosettes), mimicking the primordial ependymal canal == ???
Ependymoma
40
What presents as a tumor that is solid (not cystic) and consists of undifferentiated small cells arranged around neuropil (Homer-Wright rosettes).
Note: these structures are actually considered “pseudorosettes” since they do not surround a lumen (definition of a true rosette).
Medulloblastoma
41
____________ is a defense mechanism in which the unacceptable is transformed into its opposite, i.e., the patient manifests the opposite of what she feels.
Reaction formation
42
What are the S/Sx of Multiple Myeloma?
```
mnemonic CRAB:
Calcium
Renal failure
Anemia
Bone lytic lesions
```
Patients also have increased infections due to secondary deficiency/defective immunoglobulin secretion.
43
______________ is a monoclonal neoplastic proliferation of plasma cells, which involves the vertebral column, skull, ribs, pelvis, and other bones. The bone marrow is expanded and replaced by proliferating neoplastic plasma cells.
Multiple myeloma
44
What is common in pt. with Multiple Myeloma?
Compression fractures of vertebral bodies, such as the one depicted in the middle of this gross photograph, are frequently encountered in patients with multiple myeloma and manifest with pain. Note: Blue arrows indicate lytic areas of vertebral bone softening and hemorrhage.
45
_______________ is a suppurative infection of the bone and medullary cavity, usually affecting long bones or vertebrae.
Pyogenic osteomyelitis.
Bone destruction surrounded by a rim of bone condensation is a characteristic pathologic and radiologic finding.
Ninety percent of cases are due to Staphylococcus aureus
46
_____________ is characterized by repetitive cycles of osteolysis and disorganized osteoblastic activity, leading to thickened bone with a haphazard lamellar orientation.
Paget disease
47
A histologic jigsaw puzzle or mosaic pattern appearance of bone lamellae is pathognomonic of what disease?
Paget disease.
Clinical manifestations include bone pain, bone deformities, and chalk stick-type fractures. There is increased risk for development of osteosarcoma and fibrosarcoma.
48
90% of Pyogenic osteomyelitis is caused by ????
Staphylococcus aureus
49
Bone destruction surrounded by a rim of bone condensation is a characteristic pathologic and radiologic finding of what disease pathology?
Pyogenic osteomyelitis.
50
How do the kidneys handle the filtered load of phosphate?
The PCT reabsorbs most of the filtered load of phosphate, mainly via 3 or more Na+/Pi cotransporter located in the apical membrane.
51
What increases and decreases Phosphate reabsorption at the PCT?
PTH decreases Pi reabsorption by down-regulation of Na+/Pi cotransporter expression.
Calcitriol increases transporter expression and INCREASES Pi reabsorption.
** Calcitonin is NOT a major regulator of Pi reabsorption.
52
What is the site of action of Furosemide? what is its effects?
Furosemide, a loop diuretic, is used in congestive heart failure to decrease edema. It inhibits the Na+-K+-2Cl- cotransporter, weakens the corticopapillary osmotic gradient, and thereby causes diuresis.
53
When does the max velocity of muscles shortening occur?
when there is no afterload on the muscle. (force = 0)
54
Maximum velocity of shortening of a muscle is greater with what?
maximum velocity of shortening will be greater if ATPase activity is high.
55
In muscle, what has a profound effects on the rate of relaxation?
Sarco/endoplasmic reticulum calcium ATPase (SERCA) activity is responsible for renormalization of intracellular calcium concentrations following contraction.
Altered SERCA activity would have a profound effect on rate of relaxation, but less effect on maximum velocity of contraction.
56
Force–velocity relationships are created by measuring muscle contraction velocity against varying _________.
afterloads.
Contraction is fastest (Vmax) when there is no afterload, because all available ATP is applied toward moving the thick filaments (myosin) and thin filaments (actin) against each other.
As afterload increases, energy is expended in lifting the load, so a fewer number of cross-bridge cycles are available for muscle shortening. When the afterload becomes so great that all cross-bridge cycles are engaged in attempting to lift the load, shortening is no longer possible and Vmax = 0.
57
The pressure at the point of aortic valve opening is equal to what?
Diastolic BP.
Pressure here is equal to aortic end-diastolic pressure.
58
A person with cholera without fluid replacement undergoes what changes body fluid compartments?
A person with cholera without fluid replacement undergoes isosmotic volume contraction caused by loss of isotonic fluid.
A decrease in extracellular volume without significant change in intracellular volume occurs.
59
What ion exchanger is located in cardiac muscle, moves a net positive charge into the sarcoplasm ==> causes sarcolemmal depol.
A plasma Na+-Ca2+ exchanger,
60
change in volume/change in pressure = ?
Compliance.
61
A decrease in compliance, such as that caused by age-related arteriosclerosis, will cause what?
an increase in systolic pressure and a decrease in diastolic pressure, leading to a widened pulse pressure. (SP - DP = PP)
62
What causes widened pulse pressure?
decrease in arterial compliance
63
What is the effects of acetazolamide on H+ at the PCT?
Carbonic anhydrase inhibitors decreases H+ secretion, which decreases NH4+ secretion.
64
If the blood sample for an ABG is mistakenly exposed to atmospheric air, what would be the sample's Po2, PCO2, and pH?
Higher PO2 (b/c PO2 in air is 160mg)
Lower PCO2 (b/c CO2 is near 0mmHg in air)
Higher pH (Basic); lower CO2 = low carbonic acid = basic
65
At high altitude, the reduced atmospheric pressure does what to the alveoli and arterials?
alveolar and arterial hypoxia.
66
Alveolar hypoxia is a stimulus for pulmonary ___________ ;
vasoconstriction.
therefore, the diameter of the pulmonary vessels of a person who lives at sea level would be greater than that of a person who lives at high altitude.
67
When is the sarcomere length of the left ventricular muscle fibers the longest?
when ventricular volume is maximal (end diastolic volume = preload)
it is shortest at the end of systole.
68
w.r.t. muscle:
Active tension = ?
Active tension = total tension - passive tension
69
What do spindle afferent detect?
muscle stretch
70
________ involves loss of inhibitory pathways that normally prevent excessive responses to movement.
Spasticity
71
_____________ constitute the bulk of the contracting muscle belly. They produce muscle tension but do not initiate the reflex arc.
Extrafusal fibers
72
____________ are small diameter motor neurons that innervate the intrafusal muscle fibers found in the muscle spindle. The regulation of the contraction of these intrafusal muscle fibers regulates the sensitivity of the muscle spindle and thereby determines how much stretch is needed to initiate the myotatic reflex.
Gamma motor neurons
73
What is the function of the Golgi tendon organs in muscle?
Initiation of the inverse stretch reflex in which muscle contraction in INHIBITIED in response to the excessive stretch.
These receptors are found in the tendon near the muscle-tendon junction. They have a very high threshold such that excessive stretch is needed to cause them to fire. When they do, an inhibitory reflex is initiated that prevents muscle contraction.
74
A commonly cited example of this is the weight lifter who attempts to lift a very heavy weight and suddenly drops the weight because the muscle contraction is inhibited by the reflex response initiated by what in the muscle.
the Golgi tendon organ
75
________ is neither reabsorbed nor secreted in the nephron, so its clearance accurately measures glomerular filtration rate (GFR).
Inulin
76
__________ is freely filtered and slightly secreted in the proximal tubule.
Creatinine
**Serum creatinine and renal clearance are common clinical tools to estimate GFR.
77
What happens to Myoglobin oxygen saturation one hour after exercise?
One hour after exercise, myoglobin oxygen saturation decreases and PO2 decreases, which stimulates VEGF upregulation.
78
What happens to bFGF mRNA levels after exercise?
bFGF mRNA levels are not increased following exercise.
79
When the blood supply from the hypothalamus to the pituitary is interrupted, what happens to prolactin?
Prolactin release is increased
80
What hormone is the first marker of trophoblastic differentiation, initially doubles every two days, reaching a maximum around week 10 of gestation, and then declines.
Human chorionic gonadotropin (hCG)
81
What hormone's concentration rises in the first trimester and then declines, similar to hCG.
17-OH progesterone.
17-OH progesterone is formed in the corpus luteum as a result of 17-alpha hydroxylase actions on progesterone.
82
What hormone rises throughout pregnancy in proportion to fetal mass.
Human placental lactogen (hPL)
83
What hormone rises sharply around week 28, and may play a role in initiating labor and stimulating fetal lung maturation.
Placental corticotropin-releasing hormone (CRH)
84
What is the Alveolar ventilation (V_A)?
is the volume of gas that is available for gas exchange per minute.
85
How do you measure Interstitial volume?
You can't;
It must be measure Indirectly by calculation:
Interstitial volume = ECFV - Plasma_vol.
86
How do you measure the following:
Total body water:
ECF volume:
Plasma Volume:
Total body water can be measured with heavy water (contains D2O);
extracellular fluid volume (ECFV) with radioactive sodium;
plasma volume with radio-iodinated serum albumin (RISA).
87
The histopathological hallmark of what neurological pathology is an intracytoplasmic eosinophilic inclusion containing alpha-synuclein or ubiquitin?
Lewy Body dementia;
S/Sx:
- Memory loss
- Visual hallucinations
- Parkinsonism
88
Neocorticcal neurons with round intracytoplasmic inclusions that can contain neurofilaments, tau, and ubiquitin are known as what?
Pick Bodies
89
intracytoplasmic inclusions of Pick Bodies can contain what?
Pick bodies can contain neurofilaments, tau, and/or ubiquitin.
90
what is a frontaltemporal dementia that leads to extreme atrophy of frontal and temporal cortex?
Picks disease
91
What disease is caused by degeneration of dopaminergic neurons in the substantia nigra and manifests with tremor, rigidity, and bradykinesia?
Parkinson disease
92
What disease is caused by degeneration of motor neurons in the anterior horns of the spinal cord (lower motor neurons; causing atrophy and fasciculations) and/or large pyramidal neurons in the primary motor cortex (upper motor neurons; causing spastic paresis and hyperreflexia). This condition thus manifests with progressive weakness involving lower and upper extremities.
ALS
Bulbar muscles are also similarly affected. Skeletal muscles become atrophic (hence the term amyotrophic) and show a denervation pattern on biopsy.
93
What are Bulbar muscles and in what diease are they defective in?
Muscles of the mouth, tongue and swallowing.
==> ALS
94
What neurodegenerative disease results in dementia (accounting for about half of all cases of dementia) and is characterized by high numbers of senile plaques and neurofibrillary tangles in the hippocampus, neocortex, and cholinergic nuclei of the basal forebrain.
Alzheimer disease
95
Is Alzheimer disease ~w/ with extrapyramidal Sx?
No!
the Substantia nigra is NOT involved.
96
How do you differentiate between Lewy body and Alzheimer disease symptomatically?
Lewy Bodies: Visual hallucinations, parkinsonian motor features, and cognitive fluctuations.
AD does not have any of those, and cognitive decline is slow and more gradual.
97
Fucked limbic cortex --> what Sx?
Hallucinations
98
Issues with neurons of the substantia nigra --> what Sx?
Parkinsonism
99
What does increases in peak systolic pressures do to the oxygen demand and work of the heart?
Oxygen demand is directly related to increased work, which is increased by the increased peak systolic pressure.
100
The area enclosed by the volume-pressure diagram of the left ventricle is equal to ???
the stroke work output of the heart during its cardiac cycle
101
What ABX inhibits peptidyl transferase at the 50S subunit in prokaryotes
Chloramphenicol
102
What ABX acts by binding to the beta subunit of DNA-dependent RNA polymerase, thus inhibiting RNA synthesis
Rifabutin
103
What ABX works by inhibition of cell wall mucopeptide synthesis?
Beta-Lactams; penicillin and cephalosporins
104
A prego has walking pneumonia, what ABX is counterindicated?
Doxycycline (AGs)
105
Infection with a positive cold (IgM) agglutinin test?
Mycoplasma pneumoniae (walking pneumoniae)
106
What is the patho-phys behnd hypothatlamic amenorrhea?
low gonadotropin estrogen.
The basic mechanism of amenorrhea and delayed puberty with exercise and chronic dieting is suppression of pulsatile release of gonadotropin-releasing hormone (GnRH); this is reflected in reduced pulsatility of the gonadotropins leading to low estrogen.
107
What should you suspect in a patient who has severe intermittent flank pain radiating to the groin, often with intermittent hematuria.
Suspect ureteral obstruction from renal stones in a patient who has severe intermittent flank pain radiating to the groin, often with intermittent hematuria.
108
What would the Complete removal of the zona glomerulosa would result in?
death due to salt wasting, hypovolemia, and hypotension.
109
List examples of lysogenic conversion:
```
"COBEDS":
C = Cholera toxin
O = O antigen of Salmonella
B = Botulinum toxin (phage CEβ and DEβ)
E = Erythrogenic toxin of Streptococcus pyogenes
D = Diphtheria toxin (Corynephage β)
S = Shiga toxin
```
COBEDS, when 2 people share a bed, somebody gets prego (w/phage) hahaha
110
CGD is the absence of NADPH oxidase. What are the common bugs that causes infections?
susceptible to catalase-positive infections, particularly those caused by:
- S. aureus
- Aspergillus
- Nocardia
- Salmonella
- Candida.
111
A facial or scalp port-wine stain (a facial angioma, or nevus flammeus) should specifically suggest evaluation for what disease?
Sturge-Weber disease, a slowly progressive neurocutaneous disorder.
112
Sturge-Weber disease?
a slowly progressive neurocutaneous disorder.
Port-wine nevi present as large flat, pink-red birth marks, usually located on the face and in the distribution of a cranial nerve [usually V1 (ophthalmic) and V2 (maxillary) distribution of the trigeminal nerve]. The lesions persist throughout life and may darken as the child becomes older. When biopsied, these lesions appear to be capillary malformations.
113
S/Sx of Sturge-Weber disease?
Port-wine nevi present as large flat, pink-red birth marks, usually located on the face and in the distribution of a cranial nerve [usually V1 (ophthalmic) and V2 (maxillary) distribution of the trigeminal nerve].
- Absent red-reflex on physical examination, known as leukocoria. Though such a finding should always prompt evaluation for retinoblastoma.
- Leukocoria may also present in patients with glaucoma.
- Ipsilateral leptomeningeal angiomatosis (vascular malformations within the meninges), intellectual disability, and focal or generalized seizures, sometimes with hemiparesis, sensory changes, or hemianopia.
- Patients may also develop pheochromocytomas.
114
Is Sturge-Weber disease genetic or sporadic?
Sturge Weber is usually sporadic!
As a caveat, Sturge-Weber is part of a family of neurocutaneous disorders known as phakomatoses. Other phakomatoses include neurofibromatosis, Von Hippel-Lindau syndrome, and tuberous sclerosis. As the name would suggest, these patients usually have both cutaneous and neurologic findings, with increased incidence of certain tumors. Unlike most phakomatoses, Sturge Weber is usually sporadic rather than genetic in origin.
115
- Cerebellar hemangioblastomas
- Bilateral renal cell carcinoma
- Retinal hemangioblastoma
- pheochromocytoma
are a feature of Features of what disease?
von Hippel-Lindau syndrome, which is an autosomal dominant condition resulting from mutation in the VHL gene on chromosome 3.
116
Subependymal and cortical tubers are found in what disease process?
tuberous sclerosis
117
What type of infarction is this:
- d/t a state of hypotension.
- Elevation of the creatinine kinase, MB fraction (CK-MB) or troponin confirms that an infarction has occurred.
- ECG may show ST-segment depression or flattening on various leads.
subendocardial infarction
118
ECG may show ST-segment depression or flattening on various leads, with elevations in CK-MB and/or Troponin == ?
subendocardial infarction
119
proliferative synovitis with many lymphocytes, macrophages, and plasma cells == ???
Rheumatoid Arthritis;
symmetric, inflammatory, peripheral polyarthritis that primarily involves the synovial membranes and articular structures of multiple joints.
intense inflammatory infiltrate that is present is typically composed of plasma cells, lymphocytes, and macrophages, indicative of chronic inflammation.
120
Most spec. test for RA?
Anti-citullinated peptide/protein (CCP)
121
Pyruvate carboxylase is a mitochondrial enzyme requiring what cofactor?
Biotin
122
Gluconeogenesis, what enzyme produces oxaloacetate (OAA) that can ultimately be converted to glucose.
Pyruvate carboxylase (PC)
123
A deficiency of pyruvate carboxylase leads to accumulation of what?
Gluconeogenic precursors, alanine, pyruvate and lactate.
Pyruvate carboxylase deficiency is a rare autosomal recessive disorder characterized by elevated levels of alanine, pyruvate, lactate, and ketoacids
124
The recurrent laryngeal nerve is a branch of the vagus nerve that innervates all of the intrinsic laryngeal muscles except the ________________.
cricothyroid muscle
125
The cricothyroid muscle is innervated by the ???
external branch of the superior laryngeal nerve.
126
The recurrent laryngeal nerve is a branch of the vagus nerve that innervates what muscles?
all of the intrinsic laryngeal muscles except the cricothyroid muscle.
127
a tumor suppressor gene that regulates the bifunctional protein catenin is called?
APC
loss of function I s d/t to a GENE deletion
128
The superficial and deep peroneal nerves provide sensory innervation to the dorsum of the foot:
The superficial peroneal nerve provides sensory innervation to most of: ??
The deep peroneal nerve provides sensory innervation to the: ?
The superficial peroneal nerve provides sensory innervation to most of the dorsum of the foot.
The deep peroneal nerve providessensory innervation to the webspace between the first and second digit.
129
HSV-1 encephalitis has a propensity for what areas of the brain.
temporal lobes of the brain
130
Unilateral temporal lobe abnormalities (hypodense lesions) on brain imaging (MRI or CT scan) are considered strong evidence for infection by what?
herpes simplex encephalitis.
131
What type of inclusions can be seen in neurons and glia in HSV-1 encephalitis?
Cowdry type A inclusions can be seen in neurons and glia in HSV-1 encephalitis.
132
Greatly enlarged oligodendrocyte nuclei with glassy amorphic viral inclusions are a feature of what infectious process?
progressive multifocal leukoencephalopathy (PML).
This viral encephalitis usually occurs in immunosuppressed patients and is caused by the JC polyomavirus. PML is characterized by demyelination because it preferentially infects oligodendroglia.
133
Microglial nodules containing multinucleated giant cells are characteristic of what infectious pathology?
chronic HIV encephalitis, which tends to involve in particular the subcortical white matter, diencephalon, and brainstem.
134
The presence of both distinct nuclear and ill-defined cytoplasmic inclusions specifically suggests infection by what?
cytomegalovirus (CMV) infection.
CMV infection of the brain characteristically occurs in severely immunosuppressed patients, in whom it tends to localize to the paraventricular subependymal regions of the brain.
135
What drug is the treatment of choice for HSV-1 encephalitis?
Acyclovir
Empiric therapy with IV acyclovir should be initiated as soon as the diagnosis is suspected.
136
Extracellular spirochetes can sometimes be demonstrated in patients with what infectious process?
- Neuroborreliosis, the brain infection seen in Lyme disease, and in neurosyphilis.
Neuroborreliosis can cause aseptic meningitis, facial nerve palsies, and encephalopathy. Neurosyphilis can occur in three basic forms: meningovascular syphilis (involving the base of the brain), general paresis (involving the frontal lobe and other areas of the neocortex), and tabes dorsalis (involving the dorsal roots and spinal cord).
137
What is the action of Aldosterone with regards to Na+ and K+ ?
Aldosterone works at Mineralocorticoid receptors to INCREASE:
Sodium Reabsorption & Potassium Secretion!
Aldosterone normally increases Na+-H+ exchange in the proximal tubule (PT) and H+-K+ ATPase activity in the distal segments.
138
The mineralocorticoid deficiency (following injury to the zona glomerulosa; see figure) causes urinary loss of sodium and water, and retention of potassium. The hypotonic dehydration reduces renal perfusion; what is the compensatory response to this?
increased secretion of renin, which elevates renin activity.
139
What thyroid mass presents as:
- Commonly benign lesion of the thyroid.
- Microscopic examination shows benign colloid follicles without capsular or vascular invasion.
- Patients are usually euthyroid.
Thyroid (follicular) adenoma
140
Destruction of the adrenal _______ produces a mineralocorticoid deficiency, causing a hypotonic dehydration.
Adrenal cortex
This leads to reduced renal perfusion, producing a compensatory response that includes increased renin secretion and activity.
141
Waterhouse-Friderichsen syndrome ???
Septic shock.
The pneumoperitoneum on CT indicates that bowel perforation occurred, for example from a ruptured diverticulum.
Severe septic shock can lead to destruction of the adrenal cortex, which causes loss of mineralocorticoids as well as glucocorticoids.
142
Neuropathy is very common among diabetics, and causes atrophy of _________ & _________ muscles.
lumbricals and interosseous.
This can lead to deformities such as claw toe or hammertoe. The lumbricals contribute to extension at the proximal and distal interphalangeal joints and flexion of the metatarsophalangeal joints. The interossei muscles contribute to flexion, abduction, and adduction at the metatarsophalangeal joints. The peripheral neuropathy causes loss of trophic factors from the nerves to the muscles and subsequent atrophy of the muscles.
143
What are Claw Toes?
Claw toes occurs when other digital flexors and extensors overpower the weakened lumbrical and interossei muscles, causing hyperextension of the metatarsophalangeal joint and flexion of the proximal and distal interphalangeal joints.
144
What is Hammertoes?
Hammertoes occurs when the flexor digitorum longus contracts and overpowers the weakened muscles, causing extension of the metatarsophalangeal joint, flexion of the proximal interphalangeal joints, and hyperextension of the distal interphalangeal joints. Claw toes and hammertoes can affect the lateral four toes.
145
Constriction of the efferent arterioles in the kidney, which increases peritubular colloid osmotic pressure, thereby enhancing reabsorption of salt and water from the proximal tubule;
Is an effect of what hormone?
AgII
146
Child with:
- Renal (and often hepatic) cysts and progressive renal failure.
- On Gross: bilaterally enlarged kidneys with smooth surfaces.
- On Cut section: sponge-like appearance with multiple small cysts in the cortex and medulla.
Childhood polycystic disease is a rare autosomal recessive disease that presents in infancy
ARPKD
147
What can be seen on peripheral blood smear in any condition that causes mechanical damage producing hemolysis (microangiopathic hemolytic anemia).
Hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), DIC, and trauma from mechanical heart valves are major causes of such RBC damage.
Fragmented red blood cells, helmet cells and schistocytes.
148
Patients with HUS and TTP will have what in terms of PT and PTT levels.
normal PT and PTT levels.
149
___________ is usually seen in children and associated with infection with enterohemorrhagic E coli O157: H7 strain, i.e. ingestion of contaminated burgers, which cause thrombi within glomerular capillaries and hematuria.
HUS
150
____________ is usually seen in women age 20-40 years who present with the classic pentad: fever, neurologic signs, thrombocytopenia, kidney dysfunction, and presence of schistocytes, TTP is due to the lack of the metalloprotease, ADAMTS13 which cleaves large von Willebrand multimers.
TTP
151
Functional asplenia is associated with conditions with increased extravascular hemolysis such as: (4)
sickle cell disease
hemoglobin SC disease
sickle cell hemoglobin (HbS)
beta thalassemia
152
What disease is characterized by spherocytes in the peripheral smear?
Hereditary spherocytosis (choice C) would
153
____________ is characterized by microspherocytes in the peripheral smear, where splenic macrophages partially remove affected red cell membrane coated by immunoglobulins.
Immune hemolysis
154
Immune hemolysis = _______ direct Coombs test.
Patients present with jaundice, acute onset anemia, decreased renal function, hemoglobinuria, decreased levels of haptoglobin, and a POSITIVE direct Coombs test. Platelet count would not be low.
155
What blood/heme disease has the following pathogenic mech?
Endothelial cell injury leading to a cyclical process involving a prothrombotic event with consumption of clotting factors and the breakdown of clots by the fibrinolytic system.
disseminated intravascular coagulation (DIC), which is a feared complication of many disorders, such as massive trauma, obstetric complications (i.e. abruptio placenta), malignancy (adenocarcinoma), pancreatitis, nephrotic syndrome (loss of antithrombin III into urine), and gram-negative bacterial sepsis.
156
____________ is characterized by bleeding from mucosal surfaces, thrombocytopenia, prolonged PT and PTT, decreased fibrinogen level (due to consumption of fibrin from the coagulation cascade), and elevated fibrin split products (breakdown of circulating fibrinogen and fibrin from blood clots).
DIC
157
DIC is characterized by ???
bleeding from mucosal surfaces
thrombocytopenia
prolonged PT and PTT
decreased fibrinogen level (due to consumption of fibrin from the coagulation cascade)
elevated fibrin split products (breakdown of circulating fibrinogen and fibrin from blood clots).
158
DIC is characterized by ???
bleeding from mucosal surfaces
thrombocytopenia
prolonged PT and PTT
decreased fibrinogen level (due to consumption of fibrin from the coagulation cascade)
elevated fibrin split products (breakdown of circulating fibrinogen and fibrin from blood clots).
159
Serology that results in Positive cold agglutinins (autoantibody to O+ red blood cells in cold) ==> ???
Mycoplasma pneumoniae is the most common cause of atypical pneumonia in adolescents and young adults.
Macrolides are used for treatment (azithromycin, clarithromycin).
160
Rupture of the middle meningeal artery causes what?
epidural hematoma.
CT scans show a lens-shaped hemorrhage.
161
__________________ are small, intraparenchymal aneurysms that are related to hypertension (most common), diabetes, and amyloid angiopathy. They usually occur at the sites of small vessels in the basal ganglia, thalamus, and internal capsule.
Charcot-Bouchard aneurysms
162
___________ located in the subdural space and result in subdural hematoma.
Bridging veins; are located in the subdural space and result in subdural hematoma.
Typically subdural hematomas result from trauma and can result in gradual onset headache and eventually transtentorial herniation due to elevated intracranial pressures.
The elderly, alcoholics, and those with atrophic brains are at risk for subdural hemorrhages. CT scans show a crescent-shaped hemorrhage.
163
___________ can produce both subarachnoid and parenchymal hemorrhage. They are typically located at the junction of the anterior cerebral artery and the anterior communicating artery in the Circle of Willis.
Berry aneurysms
164
Major risk factors for _______ aneurysms include hypertension, connective tissue disorders (Marfan and Ehler Danlos), and adult polycystic kidney disease. An AVM is more likely in an HHT patient.
berry aneurysms
165
What S/Sx points towards a diagnosis of hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome?
Key findings include a history of recurrent epistaxis (due to telangiectasias in nasal mucosa), melena (due to GI telangiectasias), and multiple cutaneous and mucosal vascular lesions.
These patients also have a propensity to develop large AVMs in the brain, lungs, and liver, and cerebral AVMs can produce seizures. Patients may also develop high-output heart failure due to the presence of AVMs. This patient has a secondary polycythemia due to erythropoietin secretion from chronic hypoxemia from a right-to-left shunt. The hypoxemia is caused by right-to-left shunting via pulmonary AVMs, which allows systemic venous blood to bypass the alveoli. In addition, patients may also have iron deficiency anemia is secondary to the patient's numerous bleeding episodes.
166
What two Vascular defects can cause a Subarachnoid hemorrhage?
- AV malformations ( hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome)
- Berry aneurysms (ADPKD)
167
What is the MOA and ADR of Vincristine?
MOA: vinca alkaloid that inhibits mitosis in a cell cycle-dependent manner by blocking the formation of microtubules, thereby inhibiting neoplastic cell growth.
ADR: Peripheral Neuropathy
