Flashcards in firecrack feb 1 Deck (297):
less than 60% (need to minimze oxygen radicals)
tidal volume =
8-10ml/kg of ideal body wt
how to change patient's arterial pco2
by modifying TV or RR
high resp rates use to
blow off excess pco2
ex severe metabolic acidosis
low resp rates use to
how to change pts arterial po2
modify fio2 or peep
most common complication of mechanical ventilation
risk increase with PEEP
alveolar pressure above atomspheric pressure found in lung at end of expiration
keeps alveoli from collpasing on expiration
complications of peep
increased intracranial pressure
inrecased risk of barotrauma
hypotension from diminished venous return
classic presentation of pheo
diaphoresis, headaches, palpitations
long standing undiganosed pheo causes
radiology for pheo
CT scans - adrenal adenoma
MIBG scans, pheo not on adrenal gland
adrenergic hypertensive crisis leads to multiple system organ faliure
conditions that can induce pheo crisis
anesthesia induction agents
iv urographic contrast
pheo treatment - first choice
pheo medical management
alpha blocker: phenoxybenzamine
constiutively active tyrosine kinase
median age of onset for CML
CML presents with
nonspecific - fatigue, fever, wt loss
early satiety (enlarged spleen)
LUQ pain - spleen infarction or spleenomegaly
CML & LAP
low leukocyte alkaline phosphatase
tyrosine kinase inhibitors - imatinib
CML with blast crisis
need hematopoietic stem cell transplant
CML can conver to
ALL or AML in a blast crisis
myeloid/lymphoid blasts proliferate
How is genetic sideroblastic anemia treated?
iron levels managed by transfusions, chelation, phlebotomy
What metabolic defect causes sideroblastic anemia?
defective heme synthesis
iron can't combine with heme
unused iron accumulates in mitochondria and around nucleus
hat is a potential long-term complication of acquired, irreversible sideroblastic anemia?
What vitamin can treat sideroblastic anemia?
vit b6 (pyridoxine)
What stain is used to diagnose sideroblastic anemia?
Prussian Blue of bone marrow
what is a ringed sideroblast?
unused iron encircling nucleus
How is molluscum contagiosum diagnosed?
What histological findings are associated with molluscum contagiosum?
giemsa or wright stains - inclusion bodies
What treatment options are available for patients with molluscum contagiosum?
topical trichloroacetic acid
What are five reversible causes of sideroblastic anemia?
lead, zinc, alcohol tox
diagnosis of CMV infection
culture buffy coat WBC
PCR or serology
What effect can CMV infection have on the adrenal glands?
addison's dz (primary adrenal insuff)
What is the classic description of microscopy findings in CMV infection?
large intranuclear basophilic inclusion surrounded by a halo and small intracytoplasmic basophilic inclusion
What are the most significant adverse effects of ganciclovir?
hematologic - neutropenia, etc
What are the signs/symptoms of congenital CMV?
What s/sx of CMV may be present at birth, but resolve within the first few weeks of life?
thrombocytopenia purpura - blueberry muffin rash (also in rubella)
What medication is used to treat ganciclovir-resistant CMV?
Why is congenital CMV important?
- #1 congenital infection in the developed world
- #1 viral cause of mental retardation in the U.S.
- #1 cause of sensorineural hearing loss
How does CMV infection manifest in immunocompetent vs immunocompromised hosts?
asymptomatic, mono vs
What are the GI sequelae of CMV infection?
esophagitis (AIDs, painful swallowing)
What infection does CMV cause in the lung?
How does CMV affect the kidneys?
progressive renal failure
will see intranuclear inclusions
Why does presence of HbS result in vaso-occlusive phenomena?
HbS - poorly soluble when deoxygenated
polymerize within RBCs, impaired RBC deformability - vaso occlusions
Sickle Cell Disease Lab Findings
elevated reticulocyte index 3-15%
elevated LDH, decrease haptoglobin
sickle cells, polychormasis, howell-jolly bodies
Why do children with sickle cell disease experience delayed growth and development?
primary hypogonadism, hypopituitarism, hypothalamic insuff
sickle cell bacteremia
sickle cell meningitis
sickle cell pneumonia
mycoplasma, chlamydia, legionella
What screening test regimens should sickle cell patients begin?
Transcranial Doppler ultrasound for evaluation of cerebral blood flow, starting at age 2 and repeated every 1-2 years until age 16
Retinal evaluation should start at age 10 and repeated routinely
How is sickle cell disease diagnosed in the prenatal period? How is it diagnosed after birth?
prenatal: dna pcr testing
newborn: electrophoresis (HbF, HbS but no HbA)
What antimicrobial prophylaxis is recommended for sickle cell patients?
vaccianations: s pneumo/h flu/mengitis
3 months - 5 years: penicillin or erythromycin
What medication & supplements can be provided for patients with symptomatic sickle cell disease?
folic acid supplementation
What are 6 common neurological complications of sickle cell disease?
TIA, ischemic stroke
sensory hearing loss
What are 3 common skeletal complications of sickle cell disease?
What cardiac complication are sickle cell patients at risk for?
increased cardiac output to compensate for anemia
What type of gallstones are typically seen in patients with sickle cell disease?
acute chest syndrome
appearance of new infiltrate on CXR along with pulm symptoms
can be due to infarction or infection
What are the most common causes of malignant solitary pulmonary nodules?
primary lung cancer
Following an episode of pharyngitis, a 7-year-old child develops periorbital edema and hematuria. Explain the pathophysiology of this patient’s condition.
type III hypersensitivity
Infections of what organ systems can result in PSGN (post streptococcal glomerulonephritis)?
skin or URI
What is the confirmatory test for PSGN?
igg and c3 positive bumpy deposits on renal basement membrane
What are symptoms of PSGN (post streptococcal glomerulonephritis) aside from the classic triad?
brown urine, renal failure
What lab findings are common in PSGN (post streptococcal glomerulonephritis) other than hematuria and proteinuria
postivie anti-dnase b titer
What is the classical clinical presentation of post-strep glomerulonephritis?
edema, esp periorbital
What levels of hematuria and proteinuria are found in PSGN (post streptococcal glomerulonephritis)?
4+ blood on urine dipstick
treatment for psgn
only for complications (diuretics, acei)
What is the most common anatomic location for a carcinoid tumor?
In addition to octreotide, what therapies are indicated for the treatment of metastatic carcinoid disease?
chemoembolization of hepatic artery
What is a carcinoid tumor?
derived from primitive stem cells in gut wall
What are some potential complications of a carcinoid tumor?
carcinoid syndrome and carcinoid crisis
carcinoid heart disease
Describe the constellation of symptoms seen in a patient with carcinoid syndrome.
tricuspid/pulm valvular dz
What is the best screening method for carcinoid tumor?
urine 5 HIAA
What parameters are associated with a poor prognosis in a patient with a carcinoid tumor?
tumors greater than 2 cm in size
positive lymph nodes
What substance secreted by carcinoid tumors is responsible for the symptoms seen in carcinoid syndrome?
Carcinoid tumors are most likely to be found in what patient population?
rare in kids
Under what conditions can a carcinoid tumor cause carcinoid syndrome?
GI tumor metastasizes to liver
primary tumor outside of GI system
In a patient with carcinoid syndrome, what abnormalities may be present on cardiopulmonary exam?
rs heart murmur
What is the standard treatment for a localized carcinoid tumor?
What pharmacologic agent may be administered for symptomatic relief in a patient with carcinoid syndrome?
kidney biopsy for iga nephropathy
increased prolif of mesangial cells
iga nephropathy classic presentation
low grade fever
What is the basic pathophysiology of IgA nephropathy?
igA immune complexes deposit in mesangial cells of kidneys, cause damage to glomeruli
diseases iga nephropathy is associated with
inflamm disorders - sarcoidosis, IBD
What is the most common cause of glomerular hematuria world-wide?
What will the urine dipstick show for IgA nephropathy?
pos for blood and protein
RBC and RBC casts can be present
What is seen on light microscopy in IgA nephropathy?
normal-appearing glomeruli or mesangial widening due to mesangial proliferation
What are the medical treatment options for IgA nephropathy?
acei/arb with statins
When are steroids indicated for treating IgA nephropathy?
nephrotic range proteinuria
What are some features that distinguish IgA nephropathy from Post-Streptococcal Glomerulonephritis?
iga within one week of URI, psgn several weeks latera
normal complement in iga nephropathy
positive throat culture in PSGN
cell that accumulates in CLL
functionally incompetent, but mature appearing lymphocytes
Rai staging system
stage 0 - lymphocytosis
I, II, LAD, organomegaly
III, IV: anemia, thrombocytopenia
median survival for CLL pts
mite sarcoptes scabiei
populations affected by CLL
caucasions, male, elderly (70)
next step in treatment of localized CLL
two staging systems for CLL
Rai and Binet
most common PE findings for CLL
infection, anemia, thrombocytopenia
tumor lysis syndrome
Binet staging system
a: fewer than 3 LN
b: 3 or more LN
c: anemia or thrombocytopenia
CLL pts that should receive chemo
scabies presents with
worse at night and after a hot bath
presenting symptoms of CLL
manifestaton of leukemia
topical permethrin cream or oral ivermectin
graves dz highest risk
women, age 20-40
Graves disease cellular infiltrate
lymphocytic infiltrate, germinal centers
RA first line DMARDS
NSAIDS/glucticorticoids in RA
adjuntive antiinflamm agents
RA clinical diagnosis, next step
x ray imaging:
joint space narrowing
drugs for prinzmetals
joint space aspiration in RA
increased leukocyte count
RA lab findings
increase ESR, CRP
prinzmetal complication that occurs with coronary relaxation
severe life threatening arrhythmias (v fib, vtach, etc)
prinzmetal contraindicated drug
RA - systemic manifestations
management of hyperthyroidism during pregnancy
PTU in first trimester
methimazole after that
meth - teratogenic, PTU - liver injury
gold standard test for prinzmetal's angina
coronary angiography with provocative test for vasospasm (acetylcholine infusion directly into the vessel)
RA - classic hand deformitites
swan-neck deform, boutonnier deform
mcp joint hypertrophy
graves dz antibodies
can have anti-TPO and anti-thyroglobulin
RA - joints affected first
hands and feet
rheumatoid factor is..
IgM against the constant region of patient's own IgG antibodies
LLQ or diffuse abd pain
diverticulitis with abscess greater than 4cm
CT guided percutaneous drainage
fecal materal or perforation --> surgery
CT with oral and IV contrast
treatment of diverticulitis
exposure to lead in children, adults
lead paint in kids
occupational in adults
bone lead level indicative of
total lifetime lead exposure
lead poisoning treatment
dimercaprol, EDTA, succimer
use of x-ray in children with lead poisoning
looking for foreign body containing lead
hemochromatosis - HLA ?
hemochromatosis when i s surgery indicated
end stage liver dz (transplant)
severe arthropathy (arthroplasty)
presentation of acute lead poisoning
abdominal and neurological symptoms
ineffective erythropoiesis (thalassemia, sideroblastic anemia)
chronic liver disease
lead toxicity pts peripheral smear
microcytic, sideroblastic, hypochromic anemia
triad of adult onset hemochromatosis
bronze skin pigmentation
hemochromatosis other symptoms
amenorrhea, impotence, hypogonadism
lead toxicity effects on fetus
miscarriages, stillbirths, low birth wt
liver biopsy in hemochromatosis
high iron content seen with prussian blue staining
most accurate test for lead toxicity
blood lead level
chronic lead exposure symptoms
lead-induced peripheral neuropathy
wrist or foot drop in adults
hand-eye coordination in children
serum iron increased
transferrin sat increase
screening text for hemochromatosis
lead poisoning anemia - age group
under 6, BBB not fully formed
areas of intrinsic weakness inside colon --
vasa recta enters muscularis externa
treatment of trichinella
how do humans acuqire trichinellosis
undercooked infected meat
antibiotic for cellulitis
acne vulgaris is inflamm of
hair follicules, sebaceous glands
common preseentation of trichinellosis
muscle pain, tenderness, swelling, weakness
cutaneous structures affected by cellulitis
vit a - birth defects, hepatotoxicity
where does trichinella encyst
antibiotic for cellulitis, febrile pts
IV ceftriaxone or cefazolin
clinical, confirm with serology
severe acne treatment
trichinella neuro symptom
ha worse with movement
erysipelas affects what cutaneous structures
trichenella - cardiac
erysipelas specific symptoms
systemic - fever and chills
cellulitis with systemic toxicity, suspect
toxic shock syndrome
AML - pt population
elderly (65), female
anemia infection, bleeding
leukostasis, metabolic abn, CNS involvement
pathophysiology of AML
failure of bone marrow stem cells to mature
suppression of all cell lines
rapid prolif of immature myeloid cells
cholangiocarcinoma age group
AML presenting symptoms
infections, fatigue, pallor, bone pain, bleeding
AML bone marrow biopsy
greater than 20% blasts
symptoms of cholangiocarcinoma
jaundice, pruritis, wt loss
cancer of bile ducts
AML lab tests
risk factor for cholangiocarcinoma
auer rods - what subtype
cholangiocarcinoma most common location
proximal common bile duct
aeur rods =
treatmetn for M3 subtype of AML or APL
all trans retinoic acid (vit A)
AML supportive therapy
platelet, RBC transfusions
uric acid lowering agents
t 15 17 translocation
ITP associated with what conditions
acquire thrombocytopenia mediated by antibodies
ITP diagnosed by
ITP: when to transfuse platelets
severe bleeding (GI or intracranial)
ITP treatment guided by
presence of bleeding
mild ITP vs severe ITP
mild - clinically silent/mild drop in platelets
severe - bleeding
ITP First line treatment
corticosteroids and/or IVIG
ITP level above to not treat
ITP second line treatment
polymyalgia rheumatica common in what populations
what is polymyalgia rheumatica
autoimmune dz with several sites of muscle and joint pain
associated with temporal arteritis
5 PE findings in polymyalgia rheumatica
- edema of extremities
- tenderness to palpitation
- movement limited by pain
- normal muscle strength
polymyalgia rheumatica MRI
increased signal at tendon sheaths and tissue outside of joints
polymyalgia rheumatica lab findings
clinical presentation of polymyalgia rheumatica
morning muscle stiffness
pain in neck, shoulders, hips
treatment for polymyalgia rheumatica
low dose corticosteroids
alport's underlying defect
type IV collagen
sensorineural hearing loss
alports electron microscopy
thickeninning/thinning of GBM
alports mode of inheritance
who is most commonly affected by polymyositis and dermatomyositis?
poly - adults
derm - children
Raynaud + myositisi
1/3 will develop it
myosititis immune cells
poly - CD8 T cells
derm - B and T cells
how do polymyositis and dermatomyositis present?
symmetrical proximal limb and neck weakness over 3 to 6 months
life threatening complications associated with myositis
myoglobinuric renal failure
myositis - HLA subtypes
non-pharmacologic treatment may be required if there is severe inflammation associated with polymyositis and dermatomyositis?
bed rest with PT to prevent contractures
What are the first line treatments for polymyositis and dermatomyositis?
topical steroids for derm
What is the age distribution of patients commonly affected by polymyositis and dermatomyositis?
What is the second line therapy for polymyositis and dermatomyositis?
MTX,aza, other immunosuppressants
In addition to symmetrical proximal limb weakness, what symptoms are associated with polymyositis and dermatomyositis?
What limitations associated with polymyositis and dermatomyositis may cause patients to first visit a physicians office?
trouble combing hair or climbing stairs
How are polymyositis and dermatomysitis diagnosed?
- symmetric proximal muscle weakness
- elevated skeletal muscle enzymes
- myopathic changes on EMG
- inflamm on muscle biopsy
lacy purple rash around MCP or PIP joints
pathophysiology of polycythemia vera
JAK 2 mutation
stimulation of EPO receptor
polycythemia vera imaging
look for tumor that produce EPO (pheo, RCC, hemangioblastoma, HCC)
what is polycythemia vera
increased RBC volume
What are some complications of polycythemia vera NOT related to hyperviscosity syndrome?
What lab findings are required to make the diagnosis of polycythemia vera?
elevated hgb, hct
What are some complications of polycythemia vera related to hyperviscosity syndrome?
What is the life expectancy of treated & untreated polycythemia vera patients?
10 or more years vs 6-18 months
What are the 5 aspects of medical treatment of polycythemia vera?
myelosuppresive agents - hydroxyurea
uric acid lowering agents
What are the 5 chief presenting symptoms of polycythemia vera?
burning pain in hands & feet
easy bleeding and bruising
most common causes of death in polycythemia vera?
polycytehmia vera oxygen sat
otherwise hypoxia-induced erythrocytosis
EPO in polycythemia vera
should eb low (negative feedback)
polycythemia vera bone marrow biopsy
polycythemia vera PE
TdT & CD10
prophylatic intrathecal chemo with MTX
T cell ALL symptoms
SOB due to mediastinal mass
effect of ALL on other cell lines
decreases other cell lines
thrombocytopenia, anemia, neutropenia
ALL peripheral smear
cured in children
worse in adults
ALL cell of origin
B cells > T cells
children age 2-5
aplastic anemia - 2 broad mechanisms
injury to pluripotent stem cells
aplastic anemia =
diminshed or abscent hematopoietic precursors in bone marrow
aplastic anemia drugs/toxins
aplastic anemia symptoms
anemia -- fatigue
leukopenia - recurrent infections
thrombocytopneia - mucosal hemorrhage
aplastic anemia caused by what viruses
aplastic anemia hereditary cause
fanconi anemia (impaired dna repair mechanisms)
aplastic anemia bone marrow biopsy
no fibrotic infiltration or malignant cells
aplastic anemia treatment
removal of offending agent
transplant or immunosuppressive thearpy
hodgkin lymphoma cell type
hodgkin lymphoma histo findings
Reed-Sternberg cells in inflamm background
4 types of hodgkin lymphoma
hodgkin lymphoma pt risk factors
age 20 & 65
stages I-IV of hodgkin lymphoma
1 - single LN or site
2 - 2 or more LN, site on same side of diaphargm
3 - both sides of diaphragm
4 - outside of LN
CD 15 and CD 30
presenting symptoms of hodgkin lymphoma
nonspecific b symptoms
early vs late hodgkin lymphoma
early - cure (chemo + rad)
late - prognosis by IPS (chemo only)
hodgkin lymphoma treatment
early - chemo and rad
late - chemo only
cause of vit D def
impaired 1-alpha hydroxy
target organ resistance
treatment of osteomalacia due to poor diet
50,000 IU weekly for 3-12 weeks
osteomalacia and rickets
impaired calcification of newly formed bone matrix
treatment of osteomalacia due to increased metabolism or impaired hydroxylation
50,000 IU weekly
impaired 25-hydroxylation due to
treatment for osteomalacia due to imapired 1alpha hydroxylation
give active form (1,25)oh2 vit d
What causes of vitamin D deficiency are associated with impaired 1alpha-hydroxylation?
What changes to bone structure occur in rickets?
changes in growth plate (hypertrophic cartilage) and bone bowing/bone cortical thinning
What is meant when osteomalacia and rickets are called a “qualitative” defect?
process of bone mineralization is abnormal
How much calcium should patients with vitamin D deficiency supplement per day?
How is iatrogenic osteomalacia managed?
withhold offending med
What causes of vitamin D deficiency are associated with increased loss of vitamin D?
imapired enterohepatic circulation
rickets lab values
decreased 25 vit d
decreased ca, phosphate
increased alk phos, PTH
rickets x ray findings
Widening of physes
Bowing of long bones
Translucent lines in bones
Flattening of the skull
Enlarged costal cartilages
What results from the hypocalcemic state seen in osteomalacia and rickets?
What causes of vitamin D deficiency are associated target organ resistance?
vit d receptor mutation
internal organs affected by scleroderma
organ system most often involved with systemic sclerosis
thick, tight, shiny skin
populations affected by scleroderma
pulmonary complications in systemic sclerosis
how to treat esophageal conditions of scleroderma
strictures may need surgical correction
what causes the GI symptoms of scleroderma
overgrwoth of bacteria and malabsorption
scleroderma age range
anti-centromere Ab, skin calcification, raynaud phenomenon, esophageal dysmotility, sclerodactly, telangiectasia
esophageal conditions associated with scleroderma
scleroderma - treatment of joint contractures
what three processes contribute to scleroderma pathogenesis
overproduction of ECM components
dysfxn cell/humoarl immunity
other diseases associated with scleroderma
scleroderma & the heart
myocardial fibrosis --> CHF, arrhythmias
systemic sclerosis antibodiy
scleroderma GI symptoms
diarrhea, constipation, esophageal disease
GI symptoms treatment in scleroderma
treatment for scleroderma renal crisis
treatment for sclerotic vessels in scleroderma
scleroderma renal crisis
sudden onset of hypertension and renal insuff