Pulmonary, Hematologic, and Rheumatic Disease Flashcards
pulmonary function testing cutoffs for obstructive and restrictive pulmonary disease
obstructive: FEV1/FVC < 70%
restrictive: total lung capacity < 70% of predicted
ophthalmic disease processes associated with OSA?
floppy eyelid syndrome, keratoconus, NAION
ophthalmic side effects of CPAP?
increased IOP and dry eye
what immune modulating drugs are useful in acute asthma exacerbation?
systemic steroids. NOT inhaled steroids.
single most cost-effective and efficacious intervention to reducing risk and slowing progression of COPD?
smoking cessation
MOA of cromolyn sodium
mast cell stabilizer
COPD patients who use inhaled corticosteroids are at an increased risk for____
pneumonia
myeloid stem cells are precursors to____
erythrocytes, granulocytes, monocytes, and platelets
most common anemia worldwide?
iron deficiency
pathophys and best screening test for paroxysmal nocturnal hemoglobinuria?
RBCs are extra-sensative to complement-mediated cell lysis. flow cytometry
Treatment of choice for beta-thalassemia major?
allogeneic stem cell transplant
life span of circulating RBC?
120 days
cutoffs for microcytic, normocytic, and macrocytic anemia?
microcytic: MCV < 70
macrocytic: MCV > 100
what is hepcidin, and what is its relative value in iron-deficiency anemia?
peptide hormone that inhibits iron transport across intestinal epithelium and inhibits iron transport out of macrophages, thus decreasing serum iron. Hepcidin is compensatorily low in iron deficiency anemia
genetic mechanism and phenotype of alpha- and beta-thalassemia
alpha-thal: gene deletion leading to no synthesis of hemoglobin alpha chain
beta-thal: point mutation leading to impaired beta-chain synthesis
type of anemia described by defective incorporation of iron into hemoglobin
sideroblastic anemia
what type of anemia are alpha- and beta-thalassemia?
hypochromic, microcytic
source, mechanism and site of absorption, site and length of storage of vitamin B12
all animal products. binds with intrinsic factor produced by gastroepithelial cells and this complex is absorbed in terminal ileum. stored in liver, takes 3 years to deplete stores.
atrophic gastritis leading to decreased intrinsic factor production
pernicious anemia
type of anemia for B12 and folate deficiency?
megaloblastic (subset of macrocytic). Caused by impaired DNA synthesis
most sensitive and specific lab test for B12 deficiency (excluding blood smear)
serum methylmelonic acid (will be ELEVATED)
macrocytic anemia with peripheral neuropathy?
B12 deciciency
hypersegmented neutrophils in peripheral smear
folic acid deficiency
effect of folate supplementation for patient with B12 deficiency?
will correct anemia but will not affect neuropathy
lab features of hemolytic anemia
increased reticulocyte count, increased indirect bilirubin, increased LDH, decreased haptoglobin
what is haptoglobin and in what pathologic state is it reduced?
protein that binds free hemoglobin released by erythrocytes. this complex is then removed by the reticuloendothelial system. haptoglobin is low in hemolytic anemia
treatment indicated for all types of hemolytic anemia?
folate supplementation
inheritance, pathophys and definitive treatment for hereditary spherocytosis?
AD. RBC membrane is abnormal and cell is shaped like a sphere which ruptures more easily, especially in the spleen, leading to hemolytic anemia. splenectomy
inheritance and pathophys of G6PD deficiency?
XR. RBCs unable to deal with oxidative stress, leading to hemolytic anemia in setting of infection or precipitating drug. Heinz bodies = hemoglobin precipitates in RBCs
inheritance, pathophys, and definitive diagnosis of sickle cell anemia
AR causing amino acid substitution on beta-chain of hemoglobin which leads to erythrocyte cell membrane instability and sickling. hemoglobin electrophoresis
positive Coombs test?
autoimmune hemolytic anemia
platelet level where minor bleeding can occur? spontaneous bleeding?
what are the vitamin K-dependent coagulation factors?
factors, II, VII, IX, and X
ophthalmic side effects of CPAP?
increased IOP and dry eye
what immune modulating drugs are useful in acute asthma exacerbation?
systemic steroids. NOT inhaled steroids.
single most cost-effective and efficacious intervention to reducing risk and slowing progression of COPD?
smoking cessation
MOA of cromolyn sodium
mast cell stabilizer
COPD patients who use inhaled corticosteroids are at an increased risk for____
pneumonia
myeloid stem cells are precursors to____
erythrocytes, granulocytes, monocytes, and platelets
most common anemia worldwide?
iron deficiency
pathophys and best screening test for paroxysmal nocturnal hemoglobinuria?
RBCs are extra-sensative to complement-mediated cell lysis. flow cytometry
Treatment of choice for beta-thalassemia major?
allogeneic stem cell transplant
life span of circulating RBC?
120 days
cutoffs for microcytic, normocytic, and macrocytic anemia?
microcytic: MCV 100
what is hepcidin, and what is its relative value in iron-deficiency anemia?
peptide hormone that inhibits iron transport across intestinal epithelium and inhibits iron transport out of macrophages, thus decreasing serum iron. Hepcidin is compensatorily low in iron deficiency anemia
genetic mechanism and phenotype of alpha- and beta-thalassemia
alpha-thal: gene deletion leading to no synthesis of hemoglobin alpha chain
beta-thal: point mutation leading to impaired beta-chain synthesis
type of anemia described by defective incorporation of iron into hemoglobin
sideroblastic anemia
what type of anemia are alpha- and beta-thalassemia?
hypochromic, microcytic
source, mechanism and site of absorption, site and length of storage of vitamin B12
all animal products. binds with intrinsic factor produces by gastroepithelial cells and this complex is absorbed in terminal ileum. stored in liver, takes 3 years to deplete stores.
atrophic gastritis leading to decreased intrinsic factor production
pernicious anemia
type of anemia for B12 and folate deficiency?
megaloblastic (subset of macrocytic). Caused by impaired DNA synthesis
most sensitive and specific lab test for B12 deficiency (excluding blood smear)
serum methylmelonic acid (will be ELEVATED)
macrocytic anemia with peripheral neuropathy?
B12 deciciency
hypersegmented neutrophils in peripheral smear
folic acid deficiency
effect of folate supplementation for patient with B12 deficiency?
will correct anemia but will not affect neuropathy
lab features of hemolytic anemia
increased reticulocyte count, increased indirect bilirubin, increased LDH, decreased haptoglobin
what is haptoglobin and in what pathologic state is it reduced?
protein that binds free hemoglobin released by erythrocytes. this complex is then removed by the reticuloendothelial system. haptoglobin is low in hemolytic anemia
treatment indicated for all types of hemolytic anemia?
folate supplementation
inheritance, pathophys and definitive treatment for hereditary spherocytosis?
AD. RBC membrane is abnormal and cell is shaped like a sphere which ruptures more easily, especially in the spleen, leading to hemolytic anemia. splenectomy
inheritance and pathophys of G6PD deficiency?
XR. RBCs unable to deal with oxidative stress, leading to hemolytic anemia in setting of infection or precipitating drug. Heinz bodies = hemoglobin precipitates in RBCs
inheritance, pathophys, and definitive diagnosis of sickle cell anemia
AR causing amino acid substitution on beta-chain of hemoglobin which leads to erythrocyte cell membrane instability and sickling. hemoglobin electrophoresis
positive Coombs test?
autoimmune hemolytic anemia
platelet level where minor bleeding can occur? spontaneous bleeding?
PTT test useful for assessing response to what drug?
heparin
what are the vitamin K-dependent coagulation factors?
factors, II, VII, IX, and X
diagnosis and inheritance of localized dilation of capillaries and venules of skin and mucous membranes including conjunctiva
Osler-Weber-Rendu (hereditary hemorrhagic telangiectasia). AD. worsens with age, can lead to profuse bleeding
diagnosis, inheritance, and ophthalmic findings of patient with hyperplastic skin, hyperextensible joints and easy bruising
Ehlers-Danlos. AD. angioid streaks, microcornea, myopia, RD, ectopia lentis
diagnosis, inheritance, and ophthalmic findings of patient with bone fragility, deafness, and easy bruising
osteogenisis imperfecta (deafness from otosclerosis). AD. blue sclera
more common cause of bleeding disorders: platelet abnormalities or coagulation abnormalities?
platelet disorders by far
mechanism and first line treatment of ideopathic thrombocytopenic purpura
autoantibodies against platelets, often occurring after a viral illness. steroids.
low platelet counts with thrombotic occlusions of microcirculation and hemolytic anemia
TTP (thrombotic thrombocytopenic purpura)
mechanism and effective life span of aspirin?
irreversibly inhibits platelet aggregation, usually lasting 48-72 hours
most common inherited coagulation factor deficiency? Inheritance? Coag study findings?
factor VIII (hemophilia A). XR. Prolonged PTT, normal PT/INR
most common inherited bleeding disorder?
Von Willebrand disease
cause of hemorrhagic disease of the newborn?
normal, mild-deficiency of vitamin-K dependent factors in the newborn. Now very rare with treatment of all newborns with vitamin K
widespread activation of coagulation and fibrinolytic systems causing bleeding and clotting?
DIC
most common inherited thrombophilia?
factor V Ledein
Which of the following does NOT cause a hypercoagulable state?
Antithrombin III deficiency, Protein C deficiency, Protein S deficiency, factor V Leiden, Von Willebrand disease, prothrombin gene mutation, hyperhomocysteinemia, antiphospholipid antibody syndrome
Von Willebrand
mechanism of acquired hyperhomocysteinemia?
nutritional deficiency of pyridoxine (B6), B12, or folate
Which of the following is not appropriate method for vitamin K supplementation?
subcutaneous, intramuscular, or intravenous
IV vitamin K can lead to anaphylaxis and death
mechanism of action of heparin
binds to antithrombin III and helps catalyze its antithrombin and anti-Xa activity
Which of the following is NOT a benefit of low molecular weight heparin compared to unfractionated heparin?
(a) Aspirin can be safely coadministered
(b) Greater bioavailability when given subQ
(c) laboratory monitoring is not necessary
(d) lower risk of heparin-induced thrombocytopenia
(a). Aspirin should not be given to patients on unfractionated or LMWH heparin due to the risk of bleeding
Which of the following is FALSE regarding direct thrombin inhibitors and factor Xa inhibitors?
(a) Neither requires direct laboratory monitoring
(b) Due to early onset of action (1-4 hours), neither requires bridging when switching to these from initial treatment such as heparin
(c) A major disadvantage is that there are no available antidotes for bleeding events
(d) Fondaparinux exclusively inhibits factor Xa and is eliminated by the liver
(d) Fondaparinux exclusively inhibits factor Xa and is eliminated by the KIDNEY and should be used cautiously in patients with renal disease
