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SBL Midterm > Clinical Medicine > Flashcards

Clinical Medicine Flashcards

1
Q

CC: 72 yo white woman presents with fatigue

  • Onset: 2 months
  • Increases w/ activity, better w/ rest
  • Not improved w/ sleep
  • ADLs not affected
  • Wt loss of 5 lbs in last 2 mo
  • HTN (ACEi), mild OA, ibuprofen, APAP
  • Appendectomy, tonsillectomy
  • No allergies, no alcohol, no smoking
  • Fam hx: HTN, Type 2 DM, long lived
  • Enalapril, ASA, ibu, APAP
  • Sore throat for 2 weeks amoxicillin
  • Easily bruises, petechiae, purpura on skin
  • Conjunctival pallor (pale), palatal petechiae

Tests?

  • Pancytopenia (all WBC down)
  • High MCV, MCH
  • Retic LOW
  • LDH

Next…

  • Check B12 & folate=normal
  • Bone marrow biopsy (pancyto but hypoproliferative w/ macrocytosis)

-Concern of what?? What causes it? Tx?

A

-Aplastic anemia
(pancytopenia + aplastic bone marrow)

Causes:

  • Parvo B19 (aplastic anemia and pure red cell aplasia)
  • Other virus
  • Autoimmune (maybe suppress immune sys to tx)
  • Meds (know meds that cause marrow hypoplasia/aplasia)

Treatment:

  • Transfusion support
  • Antibiotics
  • Cyclosporin + prednisone
  • Thymoglobulin (T3 lymphocyte removal)
  • Bone marrow transplant/stem cells

Tests: CBC, CMP (BUN, creatine, bilirubin, LDH), Retic count

Sore throat for 2 weeks amoxicillin (normally 1 wk)
Pos symptoms for anemia
Thrombocytopenia symptoms

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2
Q

Meds that cause marrow hypoplasia/aplastic anemia? (2)

A
  • Chloramphenicol

- Quinacrine

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3
Q

CC: 45 yo white female with fatigue that developed over the last month. Mildly SOB with exertion, no chest pain. No bright red blood in stool or melena, but heavy menstrual periods for a year. Treated for anemia following 3rd pregnancy 10 years ago. No meds. Italian parents died in grade school

  • Aspirin for several months (81 mg…GI bleed? Anti-platelet)
  • Vegetarian who eats a lot of cereal (no meat)
  • NO cravings for starch or talc or craving for ice-pagophagia, celery, carrots, beet-uria (All Fe def)
  • Pale
  • Pale conjunctiva
  • No hepatosplenomegaly
  • Grade I systolic ejection murmur
  • No masses, heme negative brown stool
  • Heart rate and BP normal

SS of anemia?
Labs:
-Low hb, MCV low, MCH low, RDW high, High platelet count, Incr. reticulocyte count, LDH normal (hemolysis)

Causes of microcytic?

What tests are useful in hyperprol and hypoprol?
High TIDC=incr binding of transferrin to Fe b/c less Fe

Dx?

Tx?

A

SS of anemia?
-Fatigue, heavy menstrual periods, pale conjunctiva, SEM with heart rate/BP normal due to compensation of anemia

Microcytic causes:

  • Fe def
  • Thalassemia (Italian heritage)

Tests:
LDH, indirect bilirubin, reticulocyte count, transfusion requirement, serum haptoglobin

DX: Iron-def anemia due to vaginal bleeding

Tx: remove fibroid that causes excessive menstrual bleeding, oral iron therapy until Hb normal

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4
Q

What is the key in anemic pts?

A

History!

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5
Q

A heterogeneous group of disorders in which enzymes in the heme synthetic pathway are absent or defective. This permits a buildup of heme precursors which have toxic effects

A

Porphyrias

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6
Q

Cutaneous photosensitivity and neurological effects are most assoc. with what disease group?

A

Porphyrias

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7
Q

Acute Intermittent Porphyria (AIP):

  • Most common in peoples of Scandinavian, British, and eastern European descent
  • Increased incidence in psychiatric patients

Due to deficiency of what enzyme?


A

Porphobilinogen (PBG) deaminase

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8
Q

In AIP, resultant excretion of increased amounts of ____ and ____ in urine

A

ALA and PBG

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9
Q
  • No symptoms prior to puberty
  • Various severity
  • Abd pain
  • Occasional attacks followed by clinical improvement
  • Sometimes 1 attack in a lifetime
  • N/V,constipation
  • Tachycardia, HTN
  • Neuro changes: peripheral neuropathy, anxiety, insomnia, depression,hallucinations/paranoia (usually worse during attacks), seizures
A

AIP

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10
Q

What are some precipitating factors of AIP?

Hormones, drugs, etc

A
  • Hormones: AIP in women over men, attacks w/ pregnancy
  • Drugs: anticonvulsants (Dilantin, Tegretol, Valproic acid), barbiturates, sulfonamides, alcohol
  • Low caloric intake—carbohydrate reduction can increase ALA and PBG levels and precipitate an attack
  • Infection
  • Surgery
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11
Q

How do we treat acute attacks of AIP?

A
  • Hydration with carbohydrate solutions (D5 or D10)
  • Hemin infusion—similar molecule to heme which decreases porphyrin production (negative feedback effect) and terminates an attack
  • Beta blockers—for tachycardia and hypertension
  • Analgesics
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12
Q

How do we treat chronic long-term management of AIP?

A
  • Avoid medications known to precipitate attacks
  • Avoid fasting or caloric deprivation
  • Prompt treatment of intercurrent diseases or infections
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13
Q

What is the most common of porphyrias?

A

Porphyria Cutanea Tarda (PCT)

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14
Q

PCT is due to deficiency of what enzyme in the liver?

A

Uroporphyrinogen decarboxylase

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15
Q

TQ
What is the major clinical feature of PCT?

Which pt population does it tend to occur in?

A

Cutaneous photosensitivity

Alcohol consumers

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16
Q

T/F: We see neuro effects in PCT

A

FALSE

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17
Q

Pt presents with…dx?
Cutaneous bulla formation on sun-exposed areas of skin:
-occur after minor trauma
-skin may tighten diffusely —”pseudoscleroderma”
-Exacerbations after alcohol ingestion in some
-Occasional outbreaks after exposure to petrochemicals—dioxin, trichlorophenols

A

PCT

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18
Q

TQ What are the clinical features that are assoc with PCT? (Increased risk for?)

A
  • May occur in end stage renal dz

- Increase risk for hepatoma

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19
Q

TQ: When may you have a PCT outbreak?

A

After alcohol ingestion!

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20
Q

What may you see in PCT lab work?

A
  • Abnormal liver function tests—due to buildup of porphyrins in liver
  • Increased iron buildup in liver
  • Increased porphyrins in urine—ALA primarily; only slight increase
  • Increased porphyrins in stool (buildup in liver)
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21
Q

Tx of PCT?

A
  • Discontinue exacerbating factors—alcohol, suspicious drugs
  • Phlebotomy for iron overload
  • Chloroquin—may complex with excess porphyrins and facilitate excretion
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22
Q

TQ

  • A group of disorders resulting from an inherited abnormality of globin production
  • The major focus is a decreased rate of production of a specific globin molecule
A

Thalassemias

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23
Q

-Microcytic hypochromic anemia
-Hypoproliferative, pale target cells
-Decreased reticulocyte count
-Decreased levels of Hb A1 with increased levels of Hb A2
-May have increased levels of Hb F in some cases
-In homozygous cases:
chronic tissue hypoxia, disordered iron metabolism, expansion of marrow mass causing skeletal deformities
-Transfusion dependent
-Abnormal Hb electrophor.

A

β thalassemia

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24
Q

How do we manage homozygous B thalassemia?

Die of disease in mid-20s

A
  • Transfusions are the mainstay of tx— decr. erythropoiesis & avoids disfiguring complications
  • Tx for iron overload (desferrioxamine) is required in most cases
  • Genetic counseling
  • Bone marrow transplantation
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25
Q

In alpha thalassemia, 2 genes are inherited per halpotype

So, we have __alpha genes, two genes are active during fetal development, and go off- line as the other two genes become active at the time Hb A is produced

A

4

this leaves 4 chances to go bad

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26
Q
  • Complete absence of α chain production (α° thalassemia) or reduced α chain production (α+ thalassemia)
  • In fetal life, homozygous α° thalassemia results in γ chain tetramers resulting in what?
A

Hb Barts or hydrops fetalis.

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27
Q

TQ:

-In adult life, homozygous α° thalassemia results in β chain tetramers resulting in what disease?

A

Hb H disease

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28
Q

TQ

  • These patients have a clinical course similar to β thalassemia major with lifelong anemia, skeletal changes, splenomegaly, and transfusion dependence
  • What is seen on electrophoresis?
A

-Homozygous α° thalassemia

  • Electrophoresis shows Hb A as major component, with Hb H levels varying from 5-30%;
  • May be small levels of Hb Barts as well
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29
Q

-Patients with __________ α° thalassemia usually have a much milder disease course with a trace of Hb Barts or Hb H found on electrophoresis

A

heterozygous

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30
Q

Patients with ___________ α+ thalassemia have a thalassemic picture with 5-10% Hb Barts at birth and findings similar to _________ α° thalassemia in adult life

A

homozygous

heterozygous

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31
Q

A group of diseases in which the structure of the globin molecule is altered such that conformational changes in RBC morphology occur with deoxygenation

A

Sickling Syndromes

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32
Q

What are the 4 sickling syndromes?

A

Sickle cell anemia

Sickle cell trait

Hemoglobin C disease

Hemoglobin D disease

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33
Q

Sickling:

  • Hemoglobin normally exists in two conformations—oxy (relaxed, R) and deoxy (tense, T).
  • Amino acid substitutions cause conformational changes in globin morphology resulting in hemoglobin molecules that tend to aggregate when ___________–>sickled RBCs overtime
A

deoxygenated (T)

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34
Q

TQ

  • Symptoms begin early in life, levels of Hb F protect newborns
  • Due to homozygous state for hemoglobin S (SS)
  • What is the mutation involved in this dz?
A

Sickle cell anemia

Valine is substituted for glutamic acid at the 6th amino acid residue on the β globin chain
Glu–>Val

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35
Q

Even though most sickle cell anemia patients are in reasonably good health much of the time, this may be suddenly interrupted by a crisis which is usually severe and often fatal

What is the most common form of the crisis? What is it due to? What is the result?

A
  • Infarctive crises are the most common form of crisis
  • Due to obstruction of blood flow by sickled cells
  • Results in tissue hypoxia and death
36
Q

What is the primary manifestation of an infarctive crisis? What are the primary organs involved?

A
  • Pain=major manifestation
  • Bone
  • Lung: occasional infiltrates and fever (“acute chest syndrome”)
  • Spleen: many pts asplenic after age (“autosplenectomy”)
  • CNS (CVAs not unheard of in children!)
37
Q

What are aplastic crisis of sickle cell anemia pts usually associated with?

A

-Parvovirus suppressing erythropoiesis

May also be substrate deficiency (folate during pregnancy)

38
Q

What are is the major cause of death of infants and young children with sickle cell anemia?

hint: serum Hb<6gm

A

Sequestration crises due to massive pooling of RBCs in spleen

(Major crisis: serum Hb < 6gm or drop > 3gm from baseline)

39
Q

TQ
Growth abnormalities may occur in sickle cell children where they may be shorter than peers, but catch up in late adolescence

What other abnormalities might be seen?

A

Bone abnormalities!

  • Sickle cell dactylitis: swelling of the dorsal surfaces of the hands and feet (infants/young children)
  • Medullary expansion w/ thinning of bone cortex
  • Infarctive crises–>osteosclerosis
40
Q

TQ

For sickle cell anemia pts, there is an increased rate of what infections in necrotic bone marrow?

A

Salmonella

41
Q

What lab results may we see in sickle cell anemia pts?

A
  • Anemia: Hb 5-11gm/dl; normocytic normochromic
  • Signs of hemolysis: incr. serum bilirubin &retic. count, nucleated RBCs on smear
  • Sickled RBCs on PS
  • Pos. sickle cell test: suspend RBCs in oxygen poor environment and observe for sickling
  • BEST: Hb electrophoresis-Hb S comprises >50% of Hb in the RBC
42
Q

What situations should a person with sickle cell anemia avoid?

A

-changes in oxygenation (if going in for surgery, give transfusion 1 wk prrior to decr. sickled cells); airplanes!!

43
Q

How should you treat a sickle cell pt with an infection?

A
  • Aggressive antibiotics
  • Pneumococcal vaccine (during infancy b/c need spleen)
  • Prophylactic penicillin
44
Q

How do we handle painful infarctive crises of sickle cell pts? Splenic sequestration crises?

A
  • Hospitalization, hydration, and analgesics (try nonnarcotics first) to alleviate pain
  • Splenic sequestration should be managed by transfusion
45
Q

What are some experimental sickle cell anemia treatments?

A
  • Antisickling agents such as Hydroxyurea but is myelosuppressive & pt compliance required
  • Bone marrow transplantation
  • Hypertransfusion—transfuse to keep Hb S levels <50% to reduce sickling (Fe overload risk)
46
Q

Sickle cell trait:

  • Represents carrier state for sickle cell anemia
  • <50% of Hb in RBC is Hb S
  • No clinical abnormalities
  • Patients should still avoid high altitude

Tx?

A

NONE! no therapy needed! normal lifepan

47
Q

In Hb C disease, ______ replaces_____ ____ at position 6 on the β globin chain

Hb C produces fragile RBCs in patients ________ for the trait

> 50% of the Hb in RBCs is Hb C

A

lysine–> glutamic acid

homozygous

48
Q

Pt presents with…dx?

  • Splenomegaly
  • Fleeting abdominal pain on occasion
  • Anemia
  • Target cells on PS, a few sickled cells may be present

Tx?

A

Hemoglobin C Disease so no tx needed

Just beware of worsening anemia with infection!

49
Q

Grade of anaphylaxis?
-Generalize erythema, urticaria,
periorbital edema, angioedema

A

Mild (Cutaneous only)

50
Q

Grade of anaphylaxis?

-Dyspnea, Stridor, Wheeze, Nausea, Vomiting, dizziness, diaphoresis, chest or throat tightness, abd pain

A

Moderate ( Respiratory, CV

or GI involvement)

51
Q

Grade of anaphylaxis?

-Cyanosis, Sat <90) confusion, collapse, LOC,incontinence

A

Severe (Hypoxia,
hypotension or neuro
compromise)

52
Q

What are some indications for IM administration of epinephrine in a cutaneous anaphylaxis rxn?

hint:
Goals include
-Maintain Airway patency
-Reduce Fluid extravasations

A
  • Bronchospasm
  • Laryngeal edema
  • Hypotension
  • Urticaria
  • angioedema
53
Q

What are some indications for I.V. H1 and H2 inhibitors (Diphenhydramine, 25‐50mg and Ranatidine 50mg) in a cutaneous anaphylaxis rxn?

hint: 
Goals include
-Reduce pruritis
-Antagonize histamine
-Assist in pressure support
A
  • Urticaria

- Hypotension

54
Q

Which drug reduces late phase rxn in bronchospasm of cutaneous anaphylaxis rxn?

A

Methylprednisolone

55
Q

In cardiovascular anaphylaxis rxns, what are some treatments? When is IV fluid indicated?

A
  • Epinephrine/Norepi
  • IV ranitidine

IV fluids indicated for hypotension

56
Q

How long must you observe a pt post anaphylaxis rxn? Why?

A

-Should be observed for at least 4 hours (90% reactions occur during this time period)
-20%‐28% of cases have recurrence several hours
after treatment
-Can occur after symptoms completely resolved

57
Q

Fatal anaphylactic events often result from _____ _____ as the primary cause of death

A

cardiac arrest

(peripheral pooling of
circulating volume or enters the interstitial space. Therefore, Heart has no blood to pump…Empty heart syndrome, Hypotension)

58
Q

T/F: Blockage ofβ‐receptors decreases effectiveness of

epinephrine

A

TRUE!

also avoid ACEi

59
Q

What type of reaction is anaphylaxis?

A

Type 1 hypersensitivity

60
Q

4 manifestations of anaphylaxis?

A
  • Mucocutaneous
  • Cardiovascular
  • Respiratory
  • Gastrointestinal
61
Q

Most common cause of anaphylaxis?

A

foods! (peanuts, tree nuts, shellfish, gelatin, fish, eggs, milk, soy, wheat, etc)

62
Q

What is the primary mediator of anaphylatic shock?

A 
histamine! 
– H1
• Pruritis
• Rhinorrhea
• Tachycardia
• Bronchospasm
– H1 & H2
• Headache
• Flushing
• Hypotension
63
Q

Richet and Portier in 1902 defined a “super sensitivity” reaction while attempting to
produce tolerance to sea anemone venom in
dogs
-Dogs who completely recovered from initial dose of the toxic venom died a few minutes
after given a second, very small dose
-What is this called?

A

anaphylaxis

64
Q

Pt presents with…dx?

  • Rash is spreading
  • Itches
  • Ate peanut butter cupcake after getting kindergarten shots
  • Rash is red, raised, itches (erythematous & urticarial)

Dx? Differentials?

Tx?

A

Peanut allergy

Differentials:

  • Immunization shots
  • Contact dermatitis
  • Vericella
  • Latex
  • Infx (fever?)

Tx: Histamine antag (diphenhydramine)

WATCH for 4 hours (late phase rxn risk)

65
Q

Common causes of anaphylatic rashes? Just name a few…

A 
Peanuts (foods)
Latex
Exercise
Insect sting (Hymenoptera)
Shots (eggs)
Meds
Shellfish
Cinnamon
Plants
Detergent
66
Q

Kindergarten shots?

A 
Polio
Diphtheria
Pertusus
Tetanus
Hep B
Varicella
Measles, Mumps, Rubella
67
Q

The mucosa is sky blue in the back of throat/nose is due to..

A

mast cells

68
Q

74 yo obese male admitted to ER w/ hypertensive crisis. Complains of pressure in chest and can’t breathe. Pt also cannot talk and has swollen lips and tongue

  • Meds: B-blocker, alpha-blocker, simvastatin (sulfa), lisinopril to help with HTN
  • HR, BP, RR, Pulse ox low
  • Cant see veins, cant hear breath sounds, abd distended, pitting edema, cool to touch

1st priority? Secure airway via intubation
What caused his tongue to swell?

Tx:
-Epi, albuterol, diphenydramine, ranitidine, IV fluids, norepinephrine, glucagon

Epi (to incr HR but on B-blocker…give glucagon)

A

ACEi–>bradykinin angioedema!!!!

69
Q

Hypersegmented neutrophils. Think:

A

Megaloblastic anemia

70
Q

Acanthocytes on peripheral smear can be attributed to:

A

Chronic kidney dz

71
Q

3 RBC morphologies in megaloblastic anemia:

A
  • Macro-ovalocytes
  • Cabot rings – remnants of the mitotic spindle apparatus
  • Howell-Jolly bodies – nuclear remnants
72
Q

Pernicious anemia (Abs specifically directed at intrinsic factor in the stomach) impairs absorption of nutrients, such as vitamin B12, leading to what type of anemia?

A

Megaloblastic macrocytic

73
Q

Clinical presentation:

  • Fatigue, lethargy, and exercise intolerance
  • Some pts will note unusual craving for ice chips, carrots, or other foods having a crunchy consistency when eaten
  • Pts may also crave dirt (pica)
A

Iron deficiency anemia

74
Q

What are some lab features in iron deficiency anemia?

A
  • Microcytic hypochromic anemia
  • Decreased serum iron
  • Decreased serum ferritin
  • Increased TIBC (transferrin is relatively unsaturated and available for iron binding)
75
Q

What are 4 pieces of evidence that hemolysis is occurring?

A
  • Decreased haptoglobin
  • Increased LDH and bilirubin
  • Urine hemosiderin elevated in some
  • Plasma Hb elevated if hemolysis severe
76
Q
  • Common in individuals of Northern European descent
  • Inherited defect in RBC cytoskeleton-membrane tethering proteins
  • Normocytic anemia with predominant extravascular hemolysis
  • Extravascular hemolysis leads to splenomegaly, jaundice, and increase risk of bilirubin gallstones
  • Increased for aplastic crisis w/ parvovirus B19 infection of erythroid precursors
A

Hereditary spherocytosis

77
Q

What is the test called to diagnose hereditary spherocytosis?

A

Osmotic fragility test

78
Q

How do you treat hereditary spherocytosis?

A

Splenectomy

79
Q

Blood smear shows:

-Normocytic, hypochromic, with prominent basophilic stippling in young polychromatophilic cells.

A

Lead intoxication

80
Q
  • MC cause of hemolytic anemia in the world
  • Transmitted by the bite of an infected female Anopheles mosquito
  • Hemolysis results from erythrocytic infestation by Plasmodium organisms
A

Malaria

81
Q
  • Protozoan infection through transmission by the bite of a tick
  • Infection leads to clinical syndrome of fever, lethargy, malaise, hemoglobinuria 1-4 weeks after the bite
  • Intravascular hemolysis occurs
  • Protozoa appears inside RBCs as “Maltese cross”
A

Babesiosis

82
Q

How do you treat babesiosis?

A

Atovaquone + Azithromycin

or clindamycin + quinine, but Hubbard doesn’t like quinine

83
Q
  • An acquired clonal hematopoietic stem cell disorder cause by a somatic mutation of the PIG-A gene that results in a lack of GPI anchor proteins (CD55 and CD59) ***
  • Complement-mediated lysis of RBCs
  • Patients may experience chronic hemolytic anemia, cytopenias, and/or thrombotic tendency
  • Occurs in both children and adults
A

Paroxysmal nocturnal hemoglobinuria (PNH)

84
Q

What is the treatment for autoimmune hemolytic anemia?

A

Prednisone

85
Q

Bite cells are a clinical feature of what?

A

Enzyme deficiency (both G6PD and PK)

SBL Midterm (6 decks)

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