Test 41: Pathology Flashcards
16-yr old boy confusion, fatigue, abdominal pain, excessive thirst, and polyuria.
Urine dipstick: positive for glucose and ketones
What does he have
diabetic ketoacidosis
Diabetic ketoacidosis commonly affects patients with what type of diabetes
Type 1 diabetes mellitus
What happens in type I diabetes
autoimmune response against pancreatic beta cells
- destruction of beta cells through cell-mediated immunity
insulitis
infiltration of islets by inflammatory cells
Insulin resistance accompanied by relative insulin deficiency is main cause of what
type 2 diabetes mellitus
Cystinuria
hereditary defect in renal PCT and intestinal amino acid transporter
cystinuria prevents absorption of what
COLA cystine ornithine lysine arginine
how does cystinuria impact intestines and kidney
intestine: do not develop deficiency, aa absorbed in sufficient quantities as oligopeptides
kidney: formation of cystine kidney stones (ornithine, lysine and arginine soluble in urine)
What are risk factors for cysteine precipitation
- low urine pH
- presence of preexisting crystal nidus and urine supersaturation
aortic dissection is a well-known complication of what disease
Marfan syndrome
how is cystinuria inherited
autosomal recessive
What hormones cannot be synthesized in congenital adrenal hyperplasia caused by 21-hydroxlase deficiency
aldosterone and cortisol
What hormone and over synthesized in 21-hydroxylase deficiency and why?
adrenal androgens
- low cortisol level increases ACTH, since aldosterone and cortisol not made, increase androgen
- increase 17-hydroxyprogesterone
clinical manifestations of 21-hydroxylase
female: virillization
made: normal genitalia
Both: salt-wasting, hypoglycemia,
1 week old boy: vomiting, poor feeding, dehydrated, hypotensive, hyperkalemia, hyponatremia. What does he have
21-hydroxylase deficiency
What is the strongest risk factor for cervical dysplasia and carcinoma
infection with human papillomarvirus (HPV) type 16 or 18
- need immune system to fight off
27 yr old man: muscle weakness, high blood pressure, very low plasma renin activity? what does he have
primary hyperaldosteronism
What causes primary hyperaldosteronism
- increase secretion of mineralocorticoids from bilateral nodular hyperplasia of adrenal zona glomerulosa
- aldosterone-producing adrenal adenoma (Conn syndrome)
What causes muscle weakness in hyeraldosteronism
hypokalemia
Why is there no edema in hyperaldosteronism
aldosterone escape
- increase renal blood flow and release of atrial natriuretic peptide –> increase sodium excretion
where does pheochromocytoma occur
- chromatin cells of adrenal medulla or extra-adrenal sympathetic chain
Kimmelsteil-Wilson nodules are diagnostic for what
nodular glomerulosclerosis
Characterize Kimmelsteil-Wilson
- peripheral mesangium
- ovoid or spherical
What is the most common cause of nodular glomerulosclerosis
Diabetic nephropathy
What is nodular glomerulosclerosis
- glomerular basement membrane thickening
- increased mesangial matrix deposition
nodular glomerulosclerosis leads to what
nephrotic syndrome to renal failure
minimal change disease on electron microscopy
podocyte foot process effacement
Minimal change disease can occur secondary to what
allergic reaction due to pollen/dust, insect stings, or immunization
Hep C is associated with what kidney problem
membranoproliferative glomerulonephritis
Focal segmental glomerulosclerosis can develop secondary to what
HIV
heroin
severe obesity
in poststreptococal glomerulonephritis, light microscopy shows
diffuse glomerular hypercellularity
What happens in amniotic fluid embolism
fetal squamous cells in maternal pulmonary arteries
Hallmark of respiratory distress syndrome
hyaline membranes living alveolar ducts
Where is a common lung location for aspiration pneumonia
lower lobe of right lung
Common sings for amniotic fluid embolism
hypoxia
hypotensive shock
DIC
3week-old male: projectile non-bilious vomiting after every meal, prominent peristalsis in epigastrium, olive-sized mass on deep palpation of right upper abdomen?
congenital pyloric stenosis
congenital pyloric stenosis arises secondary to what problem
hypertrophy of pyloric muscular mucosae
Free wall rupture of the heart is a catastrophic mechanical complication of what
transmural MI
when does Free wall rupture of the heart typically occur? what does it look like
first 5-14 days after MI
- slit-like tear
Abrupt rupture of left ventricle leads to what
cardiac tamponade –> profound shock
What is the most common cause of sudden cardiac death within the first 48-72 hours after an MI
ventricular arrhythmias
What is pulses paradoxus
exaggerated drop ( less than 10 mmHg) in systolic blood pressure during inspiration
how is pulses paradoxus calculated
- diference in systolic pressure at which Korotokoff sounds are first audible during expiration AND pressure when Korotkoff sounds are heard throughout all phases of respiration
how does inspiration impact blood vessels
increase systemic venous retune
impair expansion into pericardial space of heart, increased right ventricular volume with inspiration leads to what
bowing of inter ventricular septum toward left ventricle
Pulsus paradoxus is seen in what conditions
cardiac tamponade, asthma, obstructuve sleep apnea, pericarditis, croup
Patient presents with dyspnea, tachypnea, prolonged expiration, bilateral wheezing
obstructive pulmonary disease
What is acute obstructive pulmonary disease treated with
beta-adrenergic agonists
MOA for beta-adrenergic agonists
Gs protein
- adenylyl cyclase
- increase cCAMP
role of Cromolyn sodium
inhibits mast cell degranulation and release of histamine and leukotrienes
acute synovitis is best evaluated with what
diagnostic arthrocentesis and synovial fluid analysis
- fluid sent for crystal analysis, cell count, gram stain/culture
patient with well-demarcated, scaly erythematous plaques involving extensor surfaces of extremities has features of what
psoriasis
Additional complications of psoriasis
- arthritis
- nail changes: yellow-brown discoloration, pitting
- inflammatory disorder of eye: uveitis
What characterizes dermatitis herpetiformis
- erythematous prussic papules, vesicles, and bull on extensor surfaces, upper back, and buttocks.
Dermatiti herpetiformis is associated with what
celiac disease and IgA antibodies against gliadin
What causes Gaucher disease
autosomal recessive
- beta-glucocerebrosidase deficiency
- accumulation of glucocerebroside and glycolipid component of leukocyte and erythrocyte membranes
Describe Gaucher cells
-lipid-laden macrophages
“wrinkled tissue paper”
symptoms of gaucher
- abdominal dissension due to hepatosplenomegaly
- fatigue due to pancytopenia
Clinical presentation for Hodgkin lymphoma
- nontender lymphadenopathy
- B symptoms ( fevers, night sweats, weight loss)
What age group does Hodgkin lymphoma attack
bimodal
20’s then 60’s
in lymph node biopsy, what distinguishes Hodgkin Lymphoma
Reed-Sternberg cells
Burkitt lymphoma presents a mass where
abdomen, pelvis, or jaw
Histo for Burkitt lymphoma
- “starry sky” appearance
- sheets of lymphocytes with interspersed “tangible Body” macrophages
clinical presentation for follicular lymphoma
“waxing and waning” lymphadenopathy
2 important causes of megaloblastic anemia
folic acid and B12 deficiency
- required for DNA synthesis for erythropoiesis
Which megaoblastic anemia has neurological dysfunction
B12
Treating megaloblastic anemia due to vit B12 deficiency with folate does what
worsens the neurological dysfunction
What type of neutrophils are seen in megaloblastic anemia
hypersegmented neutrophils
what is MCV value for Vitamin B12 and folic anemias
greater than 110 fL
CXR: bilateral and diffuse pattern of small irregular (reticulonodular) opacities that are most pronounced in the lower lobules
pulmonary fibrosis
clinical presentation for pulmonary fibrosis
gradual-onset dyspnea
PE for pulmonary fibrosis
end-inspiratory crackles
Advanced pulmonary fibrosis shows up how on
honeycomb
What drug is used for rheumatoid arthritis that can cause interstitial pneumonitis and fibrosis
Methotrexate
Atelectasis
diminished air volume in part of lung due to obstruction
- opaque on CXR
Interstitial fibrosis with cystic air space enlargement
idiopathic pulmonary fibrosis
What causes honeycomb appearance for pulmonary fibrosis
collapse of alveolar
FEV1/FVC ratio for restrictive disease
greater than 80%
Most common benign tumor of the breast
Fibroadenoma
histo for fibroadenoma
myxoid storm that encircles epithelium-lined glandular and cystic spaces
How does estrogen impact fibroadenoma
increases with size with increased estrogen
myasthenia gravis
autoantibodies directed against nicotinic acetylcholine receptors on postsynaptic membrane
- complement-mediated damage to postsynaptic membrane
clinical presentation for myasthenia gravis
fluctuating weakness worsens over course of day
- extra ocular weakness
- may have thymoma
pathophysiology of Lambert-Easton
autoantibodies to presynaptic Ca 2+ channel
- proximal muscle weakness improves with use
Leading medical cause of death in athletes age less than 35
sudden cardiac death
- hypertrophic cardiomyopathy
- ventricular fibrillaiton or v-tach
histo for hypertrophic cardiomyopathy
massive myocyte hypertrophy and myofiber disarray
complications resulting from poor blood flow with varicose veins are
- stasis dermatitis
- skin ulcerations
- poor wound healing
- superficial infections
Claudication
pain and weakness associated with exertion
What is phlegmasia alba dozens? who gets it
- painful white leg “milk leg”
- consequence of iliofemoral venous thrombosis occurring in permpartum women
what characterizes small patent ductus arteriosus
continuous machinelike murmur
PDA clinical features
clubbing and cyanosis without blood pressure or pulse discrepancy
what is pseudo gout made of
calcium pryophosphate disposition disease
where does pseudo gout occur and regular gout
pseudo: joints
gout: first metatarsophalangeal joint
What is in synovial fluid for pseudogout
- elevated WBC
- neutrophilic predominance
- Rhomboid-shaped calcium pyrophosphate crystals
one of most common constituents of renal calculi
calcium oxalate
What is deposited in gout
monosodium urate
birefringment for gout and pseudogout
gout: negativiely birefringent
pseudogout: positiviely birefringent
Fever, dyspnea, new blowing holosystolic murmur best heard at cardiac apex? what does patient have
mitral regurgitation likely due to infective endocarditis
Describe Janeway lesions
nontender, macular, erythematous lesions
- typically on palms and soles
what can cause laneway lesion
septic embolization from valvular vegetarians
-composed of bacteria, neutrophils, necrotic material, subcutaneous hemorrhage
pathogenesis for Osler nodes
immune-complex deposition in skin
granuloma serves to do what
wall off offending agent
Th1 CD4 + cells secrete what? what does it activate
interferon-gamma
activates macrophages
Marjolin’s ulcer
aggressive, ulcerating squamous cell carcinoma that presents in areas of previously traumatized, chronically inflamed, or scarred skin
how do keloids form
excessive granulation tissue formation during tissue repair`
Ascending paralysis that starts a few weeks after a febrile illness is a typical presentation of what? usually precedes what?
Guillain-Barre syndrome
- usually precedes upper respiratory or GI infection
What happens in guillain-Barre
acute demyelinating disease with autoantibodies cross react with the myelin of spinal roots and peripheral nerves
- “endoneural inflammatory infiltrate” consisting of lymphocytes and macrophages
Toxin penetration through the Blood-nerve barrier occurs in what
diphtheria
Hyalinization of nerve arterioles is seen in what
diabetes mellitus
Polymyositis
inflammatory disease of unknown etiology that affects skeletal muscles
Pilocytic astrocytomas and medullublastomas arise where
cerebellum
Sheets of primitive cells with many mitotic figures describes what cerebellar tumor
Medulloblastoma
most common location of medulloblastoma
cerebellar vermis
symptoms for medulloblastoma
- increased intracranial pressure ( morning headache, voting, lethargy)
- cerebellar dysfuction
Most common brain neoplasm of childhood
pilocytic astrocytoma
histo for pilocytic astrocytoma
astrocytes and Rosenthal fibers
histo for ependymoma
rosettes
compare prognosis for pilocytic astrocytoma and medulloblastoma
- pilocytic astrocytoma: low-grade tumor, better prognosis
- medulloblastoma: bad prognosis and poorly differentiated
pseudocyst consists of what
granulation tissue and no epithelial lining
Glycogen-rich cuboidal epithelium is seen in what pancreas problem
serous pancreatic neoplasm
columnar mutinous epithelium is seen in what pancreas problem
mucinous cystic neoplasms of pancreas
late phase of an atopic asthma attack involves what
mucosal infiltration by eosinophils, basophils, neutrophils
What protein do basophils release
anti-helminthic toxin
- can damage epithelial and endothelial cells
What is Metalloproteinase
zinc-containing enzyme: degrease components of ECM and basement membrane
how do tumor cells detach from each other
decrease expression of E-cadherins
Most common cause of xanthelasmas
LDL receptor abnormality
15 yr old girl: gait instability, mild weakness, “clumsiness” in lower limbs, hold on to rails when walking down stairs due to several recent falls, kyphoscoliosis, pets cavus, joint position and vibration impaired
Fredreich ataxia
heat like in frediech ataxia
hypertrophic cardiomyopathy
degeneration of spinocerebellar tracts
gait ataxia
degeneration of posterior columns and dorsal root ganglia
impaired joint and vibration sense
lymphoblast in precursor B-ALL are
TdT +
CD10
CD19
lymphoblast in precursor T-ALL are
tDt
CD1a
CD2,3,4,5,7,8
What is the most common predisposing condition for infective endocarditis in developed nations? developing nations?
developed: mitral valve prolapse
developing: rheumatic heart disease
During healing process, fibroblast migration and proliferation are controlled by what growth factors
- platelet-derived growth factor (PDGF)
- transforming growth factor-beta (TGF-beta)
how is hypertrophic cardiomyopathy inherited and what happens
autosomal dominant
- mutations in cardiac sarcomere proteins
left ventricular outlaw tract obstruction in heart results in what
- systolic anterior motion of anterior leaflet of mitral valve toward inter ventricular septum
- mitral regurgitation
bcl-2 normally inhibits what
cell death cascade
Follicular lymphoma has what translocation
t (14;18)
14: heavy-chain Ig
18: bcl-2
What is primary biliary cirrhosis
autoimmune destruction of intrahepatic bile ducts and cholestasis
who gets primary biliary cirrhosis
middle-aged women
first symptom of biliary cirrhosis
pruritus
paradoxical emboli
venous thromboembolic that enter systemic arterial circulation via intracardiac or intrapulmonary shunt. (patent foramen oval, atrial petal defect
atrial septal defects with left-to-right shunting typically causes what sounds
wide and fixed splitting (no change with respiration) of 2nd heart sound
What is diastolic decrescendo murmur
aortic regurgitation
systolic ejection murmur that increases with intensity with standing is in who
hypertophic cadriomyopathy
1 week old boy: normal till now. Grade II/VI harsh, holosystolic murmur best heard at the left mid to lower sternal border ? what does he have
small ventricular septal defect
when do small ventral septal defects show on babies and why
4-10 days after born
- pulmonary vascular resistance declines, enabling left-to-right shunting
clinical presentation of Ebstein Anomaly
- cyanosis
- heart failure from severe tricuspid regurgitation
Atrial septal defects and patent ductus arterioles are what type of heart defects?
non-cyanotic congenital heart disease
What is Ebstein anomaly
displacement of malformed tricuspid valve into right ventricle
patient with history of rheumatic fever now presenting with exertion dyspnea and diastolic murmur at cardiac apex most likely has what
- mitral stenosis
PE for mitral stenosis
- loud first heart sound S1
- Early diastolic opening snap
- low-pitched diastolic rumble
Where is mitral valve best heard
cardiac apex
The opening snap sound in mitral stenosis is caused by what
sudden opening of mitral valve leaflets when left ventricular pressure falls below he left arterial pressure at beginning of diastole
Patient with hypertrophic cardiomyopathy has increased intensity of murmur in what position
standing up (Valvsalva) nitroglycerin administration - b/c they both decrease preload therefore increase murmur intensity
Phenylphrine
alpha 1 agonist
patient presents with bitemporal hemianopsia and hypogonadism ( erectile dysfunction and decreased libido)
prolactin-secreting pituitary adenoma
- prolactinoma
what negatively regulates prolactin
dopamine from hypothalamus
pituitary tumor can give what eye problems
bitemporal hemainopsia
Prolactin surpasses what hormone
GnRH
What increases prolactin recreation
TRH
