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AM- STEP1 > DIT-Things to know 2 > Flashcards

DIT-Things to know 2 Flashcards

1
Q

MEN1

A 
Parathyroid adenoma
Pituitary adenoma
Pancreatic tumor
- Gastrinoma
- Insulinoma (hypoglycemia)
- Glucagonoma (hyperglycemia and diabetes)
- VIPoma (diarrhea)
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2
Q

MEN2A

A

Medullary thyroid cancer
Pheochromocytomas
Parathyroid hyperplasia

RET oncogene

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3
Q

MEN2B

A

Medullary thyroid cancer
Pheochromocytoma
Mucosal neuromas

RET oncogene

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4
Q

What might a lab detect in the urine or plasma of a patient with pheochromocytoma?

A

Plasma

  • Metanephrine
  • Normetanephrine

Urine
- Vanillylmandelic acid (VMA)

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5
Q

Most common tumor the adrenal gland

Most common tumor of the adrenal medulla (in adults)

Most common tumor of the adrenal medulla (in children)

Medical treatment for pheochromocytoma

Pheochromocytoma, medullary thyroid cancer, hyperparathyroidism

Pheochromocytoma, medullary thyroid cancer, mucosal neuromas

A
  1. Benign, non-functioning adrenal adenoma
  2. Pheochromocytoma
  3. Neuroblastoma
  4. Non-selective alpha blocker
  5. MEN2A
  6. MEN2B
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6
Q

Which cell wall inhibitor matches each of the following?

  1. Next step in tx of otitis media if amoxicillin resistant
  2. Prophylaxis against bacterial endocarditis
  3. Sufficient for the treatment of syphilis
  4. Single-dose treatment for gonorrhea
A
  1. Amoxicillin + Clavulonic avid
  2. Penicillin V, Aminopenicillins, 1st gen cepahlosporins
  3. Penicillin G
  4. Ceftriaxone
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7
Q

Equation for confidence interval

A

CI= mean +- Z (SEM)

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8
Q

Stages of grief

A 
Denial
Anger
Bargaining
Grief
Acceptance
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9
Q

In what gestational week does they thyroid begin to develop?

Embryonic origin?

A

Third week of gestation

Derived from endoderm from the floor of the primitive pharynx

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10
Q

Remnants of thyroid development

A

Thyroglossal duct

  • connection from tongue to thyroid
  • infection risk

Foreamen cecum

  • more common
  • middle of tongue
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11
Q

What is the most common site of ectopic thyroid tissue?

A

Tongue

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12
Q

Hypothyroidism will cause elevation in what labs?

A

LDL and Total cholesterol

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13
Q

Formation of thyroid hormone

A
  1. Na gradient brings Iodide (I-) into follicle
  2. Iodide is oxidized by Peroxidase to iodine (I2)
  3. Organification= Iodine binds to thyroglobulin (Tg)
  4. Thyroid hormone is formed inside they thyroglobulin by union of tyrosine and iodine
  5. Coupling or condensation of MIT and DIT –> T3 and T4
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14
Q

What converts T4 –> T3 in periphery

A

Propylthiouracil

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15
Q

Labs seen in Hypothyroidism

A

Increased TSH

Decreased T3 T4

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16
Q

Baby with impaired growth
Intellectual disability
Enlarged tongue
Distended abdomen

What could of fixed this

A

Iodine to mother during development

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17
Q

Hashimoto thyroiditis

  • type of rxn
  • infiltrate
  • genes
  • increased risk of
A

Type IV hypersensitivity
Lymphocytic infiltrate
HLA-DR5 and HLA-B5

Risk: B cell lymphoma of thyroid

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18
Q

Painful Goiter

  • infiltration
  • triggered by
  • Tx
A

Subacute (granulomatous, de Quervain) thyroiditis

Granulomatous infiltration

Viral infection

  • Coxsackievirus
  • Echovirus
  • Adenovirus
  • Measles
  • Mumps

NSAIDS
Corticosteroids

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19
Q

Fixed, hard painless goiter

  • tissue
  • infiltrate
  • may see
A

Riedel’s thyroiditis

Chronic inflammation of thyroid –> replaced by fibrous tissue

Hypothyroid
Euthyroid

Macrophages and eosinophils

Extension of fibrosis into local structures (airway)

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20
Q

Pregnancy affect on serum thyroid hormone levels

A

Pregnancy = increased estrogen

Estrogen increases thyroid binding globulin (TBG)

Binds up circulating thyroid hormone, thyroid senses T3/T4 dropping so releases more

Total T3/T4 increased
Free T3/T4 normal

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21
Q

Hyperthyroidism vs thyrotoxicosis

A

Hyperthyroidism: thyroid is making too much thyroid hormone

Thyrotoxicosis: too much thyroid hormone from any reason

  • Exogenous hormone
  • Thyroid inflammation
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22
Q

Graves disease MOA

  • Associated with
  • key features
  • Tx
A

Autoimmune

TSI binds to the TSH receptor and stimulates the thyroid gland to secrete T3 and T4

HLA-DR3 and HLA-B8

Exophthalmos
Pretibial myxedema
- Abnormal connective tissue deposit in orbit, extraocular muscle or skin on front of shins

Methimazole (preferred)
Propylthiouracil (PTU)
Beta blocker (tachycardia and anxiety)

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23
Q

Benign tumor that can present with hyperthyroidism

A

Struma ovarii teratoma

Contains functional thyroid tissue

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24
Q

Most likely diagnosis of patient with hyperthyroidism?

  1. Extremely tender thyroid gland
  2. Pretibial myxedema
  3. Pride in recent weight loss, medical professional
  4. Palpation of single thyroid nodule
  5. Palpation of multiple thyroid nodules
  6. Recent study using IV contrast (iodine)
  7. Eye changes: proptosis, edema, injection
  8. History of thyroidectomy or radioablation of thyroid
A
  1. Subacute (de Quervain) thyroiditis
  2. Graves disease
  3. Thyroid hormone abuse
  4. Toxic thyroid adenoma
  5. Multinodular goiter
  6. Jed0Basedow phenomen
  7. Graves disease
  8. Too much exogenous thyroid hormone
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25
Q

A Tall thin male teenager has abrupt onset of dyspnea and left-sided chest pain. Percussion on the affected side reveals hyper-resonance, and breath sounds are diminished. What is most likely?

A

Spontaneous pneumothorax

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26
Q

Attributable risk

A

(A/ A+B) - (C/ C+D)

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27
Q

Ground glass appearance of cytoplasm of thyroid

A

Papillary thyroid cancer

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28
Q

Orphan annie eyes

A

Papillary thyroid cancer

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29
Q

Concentric calcification

A

Psammoma bodies

Papillary thyroid cancer

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30
Q

Mutation of papillary thyroid cancer

A

RET gene mutation

BRAF

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31
Q

Uniform cuboidal cells lining follicles and invading capsule

  • spread
  • associated with
A

Follicular thyroid carcinoma

Hematogenously
RAS mutation
PAX8-PPAR gamma 1 rearrangment

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32
Q

Tumor of thyroid
Increased level of calcitonin

Associated with
Mutation

A

Medullay thyroid carcinoma

Proliferation of parafollicular C cells

RET gene mutation
MEN 2A and MEN2B

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33
Q

Tumors that activate tyrosine kinase receptor

A

Follicular thyroid carcinoma

Medullary thyroid carcinoma

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34
Q

Rock hard thyroid with local extensions

Elderly patient

A

Anaplastic thyroid carcinoma

Undifferentiated

Rapidly fatal

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35
Q

What type of cancer in thyroid?

1) Arises from parafollicular C cells
2) RAS mutation or a PAX8-PPARy-1 rearrangment
3) Rearrangement in RET oncogene
4) Mutation in BRAF gene
5) Enlarged thyroid cells with ground-glass nuclei

A

1) Medullary thyroid carcinoma
2) Follicular thyroid carcinoma
3) Medullary thyroid carcinoma, papillary carcinoma
4) Papillary carcinoma
5) Papillary thyroid cancer

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36
Q

What nerve can be damaged in thyroid surgery

Result?

A

Recurrent laryngeal n.

Hoarseness

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37
Q

Number needed to harm

A

1/ AR

AR= (A/A+B) - (C/C+D)

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38
Q

Cell types and secretion in pancreas

A

Alpha= Glucagon

Beta= Insulin

Delta= somatostatin

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39
Q

Glucagon does what

A

Stimulates

  • Gluconeogenesis
  • Glycogenolysis
  • Lipolysis (break down of fat adn TG to produce ketones)
  • Insulin secretion
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40
Q

GLUT-2

GLUT-4

GLUT-1

A

GLUT-2 (independent)

  • beta cells
  • Liver
  • Small intestine
  • Renal cells

GLUT-4 (Insulin dependent)

  • Adipose tissue
  • Skeletal muscle

GLUT-1 (independent)
- Brain and RBCs

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41
Q

What glut transport is dependent on insulin

A

GLUT-4

  • Adipose tissue
  • Skeletal muscle
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42
Q

Insulin binds to what of fat and muscle cells

Does what

A

Tyrosine kinase receptor

Causes cell to insert more GLUT-4 transporters into membrane

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43
Q

Type 1 DM

  • Ab against
  • Association
A

Islet cell antibody against glutamic acid decarboxylase (GAD)

HLA-DR3-DQ2
HLA- DR4- DQ8

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44
Q

Hemoglobin A1C

A

Moiety on hemoglobin molecule as it floats around, glucose sticks to it in an non-enzymatic reaction called glycation

More glucose in blood the more it sticks to hemoglobin

Measure percentage of surface area covered in glucose= A1C

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45
Q

Kimmelstiel wilson nodules

A

Acellular nodules in glomerulus

Diabetes

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46
Q

Too much glucose causes what complications

A

Leaky vessels

Retinopathy –> hemorrhage –> blindness

Nephropathy –> progressive proteinuria

Vascular disease –> astherosclerosis

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47
Q

Alcohol sugar
Alcohol form of glucose

Glucose –> (this alcohol sugar)

A

Sorbitol

Aldose reductase

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48
Q

Sorbital –> Fructose

A

Sorbitol dehydrogenase

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49
Q

Increased glucose –> sorbitol effect on tissues

A

Some tissues dont have srbitol dehydrogenase

Trapped in cells

  • Schwann cells
  • Lens
  • retina
  • kidney

Causes osmotic pressure that draws in free water causes swelling and damage to cell

Motor neuropathy
Sensory neuropathy
Autonomic neuropathy

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50
Q

How is hemoglobin glycosylated in DM to form HgbA1C

A

Nonenzymatic glycation

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51
Q

Diabetes with a strong genetic predisposition

A

Type II DM

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52
Q

Polymyositis vs Polymyalgia rheumatica

A

Polymyositis

  • Muscle weakness
  • Elevated Ck and aldolase
  • ANA
  • Anti-Jo 1

Polymyalgia rheumatica

  • Joint pain
  • Normal Ck and aldolase
  • No muscle pain or weakness
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53
Q

Diabetic ketoacidosis

  • values
  • complications
A 
Hyperglycemia > 150
Hyperkalemia
- Excess H, pull H out of cell in exchange for K
- Dump K in urine
-Total K low

Arrhythmias
- serum hyperkalemia, total body potassium depletion
V tach
Torsades

Invasive fungal infections
- Mucormycosis (brain abscess)

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54
Q

Extreme hyperglycemia > 800

A

Hyperosmolar Hyperglycemic state

Type II DM

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55
Q

How do acidosis and alkalosis affect extracellular K concentrations?

A

Acidosis

  • Increases extracellular K concentration
  • Pushes H into cells

Alkalosis
- Decreases extracellular K concentration

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56
Q

What is Budd-Chiari syndrome

A

Occlusion of IVC or hepatic veins –> hepatic congestion

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57
Q

Which ECG leads will show evidence of ischemia in an inferior wall MI?

A

II, III, aVF

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58
Q

What are the findings of Brown-Sequard syndrome

A

Hemisection of spinal cord

Ipsilateral UMN signs below lesion (lateral corticospinal tract)

Ipsilateral loss of proprioception, vibration sense, and fine touch below lesion
(dorsal columns)

Contralateral loss or pain and temperature sensation 2-3 segments below lesion
(lateral spinothalamic tract)

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59
Q

What produces hromone leptin

A

Adipocytes

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60
Q

Hypothalamic nuclei that regulate hunger

A
  1. Paraventricular nucleus
  2. Dorsal medial nuclei
  3. Arcuate nucleus
  4. Lateral hypothalamus
    - Stimulated –> hunger
    - Inhibted by leptin
    - Lesion –> anorexia
  5. Ventromedial nuclei
    - Stimulated by leptin

Lateral likes lunch but lesion (or leptin) lateral you get little

Ventromedial nuclei –> go medially or thinner

Leptin makes you thin

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61
Q

Lipodystrophy

Caused by

A

Distortions in the structure or function of adipose tissue

Buffalo hump

Leptin deficiency
HIV medication
- Protease inhibitors

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62
Q

5 categories of criteria for the diagnosis of metabolic syndrome

A 
Abdominal obesity
Elevated triglycerides
Low HDL
Elevated BP
Elevated glucose
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63
Q

Liver disease associated with obesity

A

Nonalcoholic steatohepatitis (NASH)

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64
Q

BMI to be obese

A

> = 30

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65
Q

Substances known for causing methemoglobinemia

A

Nitrates

Antimalarial drugs

  • Chloroquine
  • Primaquine

Dapsone

Local anesthetics
- Lidocaine

Sulfonamides

Metoclopramide

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66
Q

Parathyroid gland cells

A

Chief cells: PTH

Oxyphil cells

  • packed with mitochondria
  • appear at pubery
  • increase with age
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67
Q

Parathyroid innervation

A

Cervical sympathetic ganglia

Doesnt directly innervated parathyroid

Controls blood flow to parathyroid gland

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68
Q

PTH on bone cells

A

Increase bone turn over
Ramps up osteoclasts

Osteoclasts: dont have PTH recpetors

Osteoblasts: have PTH receptor
- make cytokine RANKL –> activates osteoclasts

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69
Q

Subperiosteal bone reabsorption on radial aspect of medial phalanges

Tapering of distal clavicles

Salt and pepper appearance skull

Also see?

A

Osteitis Fibrosis Cystica

Brown tumors of long bones

Primary hyperparathyroidism

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70
Q

Secondary hyperparathyroidism from renal failure values

A

Increased BUN and Cr

Increased Phosphate

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71
Q

Values for secondary hyperparathyroidism from Vit D deficiency

A

Increased PTH
Decrased Ca
Decreased or normal phosphate
Decreased Vit D

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72
Q

Pseudohypoparathyroidism

A

Mutations in gene called GNAS-1

Encodes G protein at PTH receptor in kidney

Increase PTH
Decrease Ca

AD

Albright hereditary osteodystrophy

  • Short stature
  • Obesity
  • Undevelopment of 4th and 5th digits
  • Ostitis fibrosa cystica
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73
Q

WAGR complex

A

Wilms tumor
Aniridia (no iris)
Genitourinary malformation
Retardation (mental and motor)

WT1 deletion

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74
Q

Extrinsic coagulation pathway

A

Tissue factor pathway

VII
X

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75
Q

Intrinsci coagulation pathway

A

Contact activation pathway

XII
XI
IX
X

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76
Q

Deficient in factor IX

A

Hemophilia B

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77
Q

Deficient in Factor VIII

A

hemophilia A

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78
Q

Accelerating factors for coagulation pathways

A

Factor V

Factor VIII

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79
Q

What inhibits the accelerating factors for the coagulation pathways

A

Protein C

Protein S

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80
Q

RL step of coagulation pathway

A

Factor X

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81
Q

Common Final pathway in coagulation

A

Factor X –> Xa
Xa + Va activate prothrombin
Thrombin cleaves fibrinogen –> fibrin
Fibrin + XIIIa –> mesh

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82
Q

Warfarin inhibits

A 
Factor II (thrombin)
Factor VII (extrinsic)
Factor IX (intrinsic) 
Factor X
Protein C 
Protein S
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83
Q

First seen with starting Warfarin

A

Inhibit Protein C and Protein S

Transient hyeprcoagulable state

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84
Q

What cleaves Fibrinogen to fibrin

A

Thrombin (Factor II)

Coagulase from S. Aureus

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85
Q

What is essential for coagulation cascade

A

Calcium

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86
Q

Factor XI

A

Kallikrein

Activates plasminogen –> plasmin

Produces Bradykinin

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87
Q

Plasmin

A

Breaks down fibrin mesh

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88
Q

Prothrombin time (PT)

A

Add tissue factor and see how long for clot to form

Test

  • Tissue factor pathway
  • Final common pathway

Factor VII
Factor X
Factor V
Factor II (prothrombin)

Resported with INR

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89
Q

Partial thromboplastin time (PTT)

A

25-40 seconds

Taking plasma add material to activate contact activation pathway

Factor XII
Factor XI
Factor IX
Factor VIII
Factor X
Factor V
Factor II (prothrombin)
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90
Q

Hemophilia A and B

  • deficiency
  • increases
A

Hemophilia A
- Factor VIII deficiency

Hemophila B
- Factor IX deficiency

Increase PTT

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91
Q

Coagulation disorders vs platelet disorders cause what type of bleeding

A

Coagulation disorders
- macrohemorrhages

Platelet disorders
- Microhemorrhages

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92
Q

Vit K deficiency

A

Cofactor for several clotting factors

Factor II, VII, IX, X, C and S

diSCo started in 1972

Increase PT and PTT

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93
Q

Factor V Leiden

A

Hypercoagulable

Makes factor Va resistant to inactivation by protein C

Stays active longer –> more coagulation

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94
Q

Prothrombin G20210A mutation

A

Hypercoagulable

Predisposes to thrombosis

Mutation Guanine –> Adenine at 20210

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95
Q

Antithrombin deficiency

  • resistant to
A

Hypercoagulable

Unable to inactivate thrombin

Resistant to heparin

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96
Q

Protein C deficiency

A

Hypercoagulable

Unable to inactivate factor V and VIII

or deficiency in protein S

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97
Q

Which glomerular disease should be suspected most in patients with each of the following findings?

  1. IF: granular pattern of immune complex deposition; LM; diffuse capillary thickening
  2. IF: granular pattern of immune complex deposition; LM: hypercellular
  3. IF: linear pattern of immune complex deposition
  4. EM: subendothelial humps and “tram track” appearance
  5. Nephritis, deafness, cataracts
  6. LM: crescent formation in the glomeruli
  7. LM: segmetnal sclerosis and hyalinosis
  8. EM spiking on the GBM due to electron-dense subepthelial deposits
A
  1. Membranous GN
    Diffuse proliferative GN
  2. Acute post-streptococcal GN
  3. Goodpasture syndrome
  4. Membranoproliferative GN
  5. Alport syndrome
  6. Rapidly progressive GN
  7. Focal segmental glomerulosclerossi
  8. Membranous GN
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98
Q

Heparin induced thrombocytopenia

  • What happens
  • symptoms
  • Tx
A

Platelet drop

Heparin binds platelet factor 4 –> autoantibody-heparin PF4 complex –> activates platelets

Thrombocytopenia
Hypercoagulable state

Stop heparin
Start direct thrombin inhibitor until platelets back up
Start warfarin

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99
Q

What lab test is used to monitor adequate anticoagulation in a patient taking heparin?

A

PTT

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100
Q

What lab test is used to monitor adequate anticoagulation in a patient taking warfarin

A

PT (INR)

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101
Q

Which genetic syndrome is caused by?

  1. Absence of HGPRTase
  2. Deficiency of aldolase B
  3. Deficiency of cystathionine synthase
  4. Galactose-1- phosphate uridyl transferase deficiency –> intellectual disability, hepatosplenomegaly, cataracts
  5. Deficiency of tyrosinase
A
  1. Lesch-Nyhan
  2. Fructose intolerance
  3. Homocystinuria
  4. Galactosemia
  5. Albinism
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102
Q

What gives RBC their shape

Average life span RBC

A

Spectrin

120 days

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103
Q

Anisocytosis

Poikilocytosis

Polycythemia

Erythrocytosis

Reticulocytes

A

Anisocytosis: RBCs of varying sizes

Poikilocytosis: RBCs of varying shapes

Polycythemia: too many RBCs

Erythrocytosis: too many RBCs

Reticulocytes: immature RBCs

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104
Q

Basophilic stippling

A

Lead poisoning

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105
Q

RBC with uniform spikes all over surface

A

Burr cell
Echinocyte

Uremia
Renal failure
Pyruvate kinase deficiency

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106
Q

RBC with irregular spikes in size and distribution

A

Spur cell
Acanthocyte

Abetalipoproteinemia

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107
Q

Spherical RBCs

A

Spherocytes

Hereditary sphenocytosis

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108
Q

Fragmented RBCs

A

Schistocytes

DIC
TTP, HUS

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109
Q

RBCs looks like bulls eye

A

Target cells

“HALT” said the hunter to his target

Hemoglobin C disease
Asplenia
Liver disease
Thalassemia

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110
Q

Cell type seen in abetalipoproteinemia

A

Spur cell
Acanthocytes

RBC with irregular spikes in size and distribution

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111
Q

Crescent shaped RBC

A

Sickle cell

Sickle cell anemia

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112
Q

RBC with basophilic remnant of a nucleus (purple spot in RBC)

A

Howell-Jolly body

Asplenia

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113
Q

RBC with white spot of oxidized hemoglobin

A

Heinz body

G6PD

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114
Q

RBC with little part taken out of it

A

Bite cell
Heinz body removed

G6PD

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115
Q

Tear drop RBCs

A

Myelofibrosis

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116
Q

Pencil or cigar shaped cells

A

Elliptocytes

Hereditary elliptocytosis

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117
Q

RBC covered in blue dots

A

Ringed Sideroblast

Granules of iron

Disorders of heme synthesis
Found in bone marrow

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118
Q

Cell type seen with?

  1. Lead poisoning
  2. G6PD deficiency
  3. DIC

4 Abetalipoproteinemia

  1. Asplenia
A
  1. Basophilic stippling
  2. Heinz body, Bite cells
  3. Schistocytes
  4. Spur cells (acantholysis)
  5. Howell-Jolly bodies, target cells
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119
Q

Types of Hemoglobin

  1. HbA
  2. HbA1c
  3. HbF
  4. HbS
  5. HbC
  6. Hb Bart’s
  7. HbH
A
  1. Normal
    (2 alpha 2 beta)
  2. Poorly controlled diabetes
    ( 2 alpha 2 beta-glucose)
  3. Fetal hemoglobin
    ( 2 alpha 2 gamma)
  4. Sickle cell hemoglobin
    ( 2 alpha 2betaS glu –> val)
  5. Hemoglobin C dis
    (2 alpha 2 betaC glu –> lys)
  6. Severe alpha thalassemia
    (4 gamma)
  7. Severe alpha thalassemia
    (4 beta)
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120
Q

RBC production locations

A

“Young Liver Synthesizes Blood”

Yolk sac
Liver
Spleen
Bone marrow

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121
Q

An 18-year-old woman presents to the emergency department with acute onset of severe abdominal pain. She says she had a similar attack 1 year earlier after taking some barbiturates.
At that time she underwent an exploratory laparotomy, which revealed nothing. The
patient no longer takes barbiturates but recently
started an extremely low-carbohydrate and low-calorie diet. She has a temperature of
37°C (98.6°F), a respiratory rate of 16/min, and a blood pressure of 128/83 mm Hg. Her
WBC count is normal. Laboratory studies reveal a sodium level of 127 mEq/L, and urinalysis
shows increased porphobilinogen levels.
The physician tells the patient that she has a genetic condition involving her RBCs. What
congenital disorder did the physician most likely tell the patient she has?
(A) Acute intermittent porphyria
(B) Fanconi’s anemia
(C) Hereditary spherocytosis
(D) Porphyria cutanea tarda
(E) Sickle cell disease

A

A. Acute intermittent porphyria

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122
Q 
A 17-year-old boy presents to the emergency department with severe abdominal pain. Laboratory tests show a deficit in uroporphyrinogen synthetase and excess δ-aminolevulinate and porphobilinogen in the urine. Which of the following symptoms would most likely also be
present in this patient?
(A) Chest pain
(B) Hypotension
(C) Neuropsychiatric disturbances
(D) Polyphagia
(E) Stiff neck
A

C. Neuropsychiatric disturbances

Acute intermittent porphyria

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123
Q

Dark red urine
Psychologic disturbances
Polyneuropathy
Abdominal pain

  • deficiency
  • drug that precipitated

Treatment

A

Acute intermittent porphyria

Uroporphyrinogen-1 synthase
(porphobilinogen deaminase)

Precipitated by

  • Barbiturates
  • Seizure durgs
  • rifampin
  • metoclopramide

Give Heme and Glucose
- down regulates delta-ALA synthase

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124
Q

A 27-year-old man walks into the emergency
department in an agitated state. He complains
of severe abdominal pain and eventually becomes
combative, requiring five-point restraint.
His vital signs show elevated blood pressure
and tachycardia. When a straight catheter is inserted,
reddish urine enters the Foley bag. The
urine is negative for RBCs, and a toxicity
screen is negative. His doctor suspects a porphyria;
laboratory tests for urine porphobilinogen
are positive. Which of the following enzyme
defi ciencies is responsible for this
patient’s disorder?
(A) Aminolevulinate dehydratase
(B) Aminolevulinate synthase
(C) Ferrochelatase
(D) Heme oxygenase
(E) Porphobilinogen deaminase
(F) Uroporphyrinogen decarboxylase
(G) Uroporphyrinogen III cosynthase

A

E. (E) Porphobilinogen deaminase

aka Uroporphyrinogen-1- synthase

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125
Q

Sun sensitivity –> blistering
Tea colored urine
Excess hair growth
Hyperpigmentation

  • associated with
  • increased lab
  • deficiency
A

Porphyria cutanea tarda

Associated with hepatitis C and alcoholism

Increase LFTs

Uroporphyrinogen decarboxylase

AD

Build up uroporphyrinogen III

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126
Q

Lead poisoning affects

A

delta-ALA dehydratase
Ferrochelatase

Increased levels of protoporphyrin

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127
Q

A 3-year-old boy presents to his pediatrician with irritability, an ataxic gait, and regression
of speech to single words. During the interview, the patient is constantly putting objects
in his mouth. Laboratory values are signifi cant for a hemoglobin level of 8.3 g/dL. Which of
the following etiologies should be suspected in this patient?
(A) Acetaminophen toxicity
(B) Aspirin toxicity
(C) Button battery ingestion
(D) Lead poisoning
(E) Organophosphate absorption
(F) Tricyclic antidepressant overdose

A

D. Lead poisoning

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128
Q 
Abdominal pain
Renal failure
Mental deterioration
Foot/ wrist drop
Memory loss

Tx

A

Lead poisoning

EDTA
Succinmer
Severe poisoning in child
- Dimercaprol + succimer

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129
Q

Tumors that cause erythrocytosis

A

“Potentially Really High Hematocrit”

Pheochromocytomas
Renal cell carcinoma
Hepatocellular carcinoma
Hemangioblastoma

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130
Q

RL step of Heme synthesis

A

delta-ALA synthase

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131
Q

Which substance and cofactor are required for generation of GABA

A

Glutamate

B6

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132
Q

Plummer-Vinson syndrome

A

Iron deficiency anemia
Esophageal webs
Dysphagia

+/- atrophic glossitis

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133
Q

Alpha thalassemia trait/ minor

A

2 abnormal alpha alleles

2 normal alpha

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134
Q

Hemoglobin H

A

3 abnormal alpha alleles

Lots of beta

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135
Q

Hemoglobin Bart

A

4 abnormal alpha

Hydrops fetalis
Death

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136
Q

Chipmunk face
Hair on end appearance on xray of skull
Target cells

A

Beta thalassemia Major

Increase Hemoglobin F
2 alpha, 2 gamma

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137
Q

Sideroblastic anemia Labs

Treatmetn

A

Increased serum iron
Increased ferritin

Tx: B6

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138
Q

Test for beta thalassemia minor

A

Hemoglobin electrophoresis

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139
Q

What is the RL step of beta oxidation of fatty acids

A

Carnitine acyltransferase I

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140
Q

Cofactors required for function of pyruvate dehydrogenase?

These cofactors are also required for?

A

Tender Loving Care For Noone

TPP
Lipoic acid
CoA
FAD
NAD

Alpha-ketoglutarate dehydrogenase

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141
Q

Hypersegmented neutrophils
Glossitis
Increased homocysteine
Normal methylmalonic acid

A

Folate deficiency

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142
Q 
Hypersegmented neutrophila
Anemia
Glossitis
Neurologic deficits
Increased homocysteine
INcreased MMA
A

B12 deficiency

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143
Q

Perncious anemia

A

B12 deficiency

Intrinsic factor def

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144
Q

Anemia
Increased CMV
Hypersegmented neutrophils Orotic acid in urine

-deficiency

A

Orotic aciduria

Deficiency of UMP synthase

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145
Q

Causes of aplastic anemia

A 
Radiation
Benzene
Drugs
- Chloramphenicol
- Cancer drugs
Viral infections
- ParvoB19
- EBV
- HIV
Fanconi anemia
- inherited defect of DNA repair
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146
Q

Megaloblastic anemia + peripheral neuropathy

A

B12 deficiency

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147
Q

HIV positive patient with macrocytic anemia

A

Zidovudine

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148
Q

What disorder is associated with each of the following?

  1. HTN + hypokalemia + metabolic alkalosis
  2. Fever + night sweats + WL
  3. Adrenal hemorrhage due to meningococcemia
  4. Blue sclerae
  5. Hyperphagia + hypersexuality + hyperorality + hyperdocility
  6. Nystagmus + intention tremor + scanning speech
  7. Lower extremity purpura + arthralgias + renal disease
A
  1. hyperaldosteronism
  2. B symptoms of lymphoma
  3. Waterhouse frridrichsen syndrome
  4. Osteogenesis imperfecta
  5. Kluver-Bucy syndrome
    (B/L amygdala)
  6. Charcot triad of MS
  7. Henoch-Schelein purpura
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149
Q

Cold agglutinins

  • what it is
  • nearly always
  • occur with
A

Ab against RBC that interact more strongly at low temps

IgM

Occur regularly in infections with EBV or mycoplasma

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150
Q

Warm agglutinins

  • what is it
  • nearly always
  • Seen in
A

Ab against RBC that react at body temp

IgG

Seen in:
- EBV, HIV
- Lupus
- Malignancies
 (CLL, non-hodgkin lymphoma)
- Congenital immune abnormalities
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151
Q

Direct coombs

A

Using Ab to detect Ab already bound to RBCs

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152
Q

Indirect coombs

A

New blood see if Ab bind

Test prior to transfusion and screen for maternal Ab to fetus blood

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153
Q

MCHC

A

mean corpuscular hemoglobin content

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154
Q 
Increased MCHC
Increased RDW
Splenomegaly
Jaundice
Pigmented gallstones

(+) osmotic fragility test
(+) Eosin-5- maleimide

A

Hereditary spheocytosis

Defect of proteins
- Ankyrin, spectrin, Band 3, Protein 4.2

Eosin-5- maleimide
- reduced binding to RBCs indicated band 3 deficiency

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155
Q

Associated with G6PD deficiency

A

“Spleen purges Nasty Inclusion From Damaged Cells”

Sulfonamides
Primiquine
Nitrofurantoin
Isoniazid
Fava Beans
Dapsone
Chloroquine
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156
Q

Paroxysmal nocturnal hemoglobinuria

A

RBCs missing surface markers
CD55 CD59

Complement attacks and lyses RBCs

Ham’s test

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157
Q

A 45-year-old woman arrives in the emergency department complaining of intense pain in her upper abdomen for the past 4 hours. She had a similar episode in the past, but it went away within an hour. Her history is significant for a recent flu-like infection and a prolonged feeling of fatigue and general exhaustion. Physical examination reveals that her sclerae are icteric, her palate is abnormally pigmented, and her skin has a yellow hue. Ultrasound shows radiopaque gallstones. A Coombs’ test is negative.
A peripheral blood smear shows small RBCs, several of which have no central pallor.
Which of the following is the most likely cause of this patient’s condition?
(A) A mutation in the gene encoding ankyrin
(B) A mutation in the glucose 6-phosphate dehydrogenase
gene
(C) Circulating antibodies targeted against
erythrocytes
(D) Iron deficiency anemia
(E) RBC hemolysis because of a mechanical
heart valve

A

(A) A mutation in the gene encoding ankyrin

Hereditary spheocytsosi

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158
Q

A 20-year-old African-American man develops
anemia after being treated for a urinary tract infection.
A peripheral blood smear shows RBC
lysis and precipitates of hemoglobin within the
RBCs. Which of the following drug classes
most likely caused his hemolytic anemia?
(A) Aminoglycosides
(B) Fluoroquinolones
(C) Macrolides
(D) Sulfonamides
(E) Tetracyclines

A

D. Sulfonamides

G6PD deficiency

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159
Q

A 30-year-old, previously healthy man from Lagos,
Nigeria, passes dark brown urine 2 days after starting the
prophylactic antimalarial drug primaquine. On physical examination,
he appears pale and is afebrile. There is no organomegaly.
Laboratory studies show that his serum haptoglobin
level is decreased. Which of the following is the most likely
explanation of these findings?
A Antibody-mediated hemolysis
B Impaired DNA synthesis
C Impaired globin chain synthesis
D Increased susceptibility to complement-induced lysis
E Mechanical fragmentation of RBCs as a result of
vascular narrowing
F Oxidative injury to hemoglobin
G Reduced deformability of RBC membrane

A

F Oxidative injury to hemoglobin

G6PD deficiency

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160
Q

A 16-year-old boy notes passage of dark urine. He has a
history of multiple bacterial infections and venous thromboses for the past 10 years, including portal vein thrombosis in the previous year. On physical examination, his right leg is swollen and tender. CBC shows hemoglobin, 9.8 g/dL; hematocrit,
29.9%; MCV, 92 μm3; platelet count, 150,000/mm3; and WBC
count, 3800/mm3 with 24% segmented neutrophils, 1% bands, 64% lymphocytes, 10% monocytes, and 1% eosinophils. He has a reticulocytosis, and his serum haptoglobin level is very
low. A mutation affecting which of the following gene products
is most likely to give rise to this clinical condition?
A β-Globin chain
B Factor V
C Glucose-6-phosphate dehydrogenase
D Phosphatidylinositol glycan A (PIGA)
E Prothrombin G20210A
F Spectrin

A

D Phosphatidylinositol glycan A (PIGA)

Paroxysmal nocturnal hemoglobinuria

PIGA gene mutation

Prevents expression of certain proteins that are required as glycolipid anchor

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161
Q
  1. A 32-year-old woman from Hanoi, Vietnam, gives birth
    at 34 weeks’ gestation to a markedly hydropic stillborn male
    infant. Autopsy findings include hepatosplenomegaly and
    cardiomegaly, serous effusions in all body cavities, and generalized
    hydrops. No congenital anomalies are noted. There
    is marked extramedullary hematopoiesis in visceral organs.
    Which of the following hemoglobins is most likely predominant
    on hemoglobin electrophoresis of the fetal RBCs?
    A Hemoglobin A1
    B Hemoglobin A2
    C Hemoglobin Bart’s
    D Hemoglobin E
    E Hemoglobin F
    F Hemoglobin H
A

C Hemoglobin Bart’s

alpha-thalassemia major

chr 16

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162
Q
  1. A 3-year-old boy from Sicily has a poor appetite and
    is underweight for his age and height. Physical examination shows hepatosplenomegaly. The hemoglobin concentration is 6 g/dL, and the peripheral blood smear shows severely hypochromic and microcytic RBCs. The total serum iron level is normal, and the reticulocyte count is 10%. A radiograph of the skull shows maxillofacial deformities and expanded marrow spaces. Which of the following is the most likely cause of this child’s illness?
    A Imbalance in α-globin and β-globin chain
    production
    B Increased fragility of erythrocyte membranes
    C Reduced synthesis of hemoglobin F
    D Relative deficiency of vitamin B12
    E Sequestration of iron in reticuloendothelial cells
A

A Imbalance in α-globin and β-globin chain
production

beta-thalassemia major

bronze diabetes

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163
Q

12 A 17-year-old girl has had a history of fatigue and weakness
for her entire life. She has not undergone puberty. On
physical examination, secondary sex characteristics are not
well developed. She has hepatosplenomegaly. CBC shows hemoglobin
of 9.1 g/dL, hematocrit of 26.7%, MCV of 66 μm3,
platelet count of 89,000/mm3, and WBC count of 3670/mm3.
The appearance of the peripheral blood smear is shown in the
figure. Additional laboratory findings include serum glucose
of 144 mg/dL, TSH of 6.2 mU/mL, and ferritin of 679 ng/mL.
A mutation in a gene encoding for which of the following is
most likely to be present in this girl?
A Ankyrin
B β-Globin
C G6PD
D HFE
E NADPH oxidase

A

B β-Globin

beta-thalassemia
- intermediate severity

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164
Q

10 A 10-year-old child has experienced multiple episodes
of pneumonia and meningitis with septicemia since infancy.
Causative organisms include Streptococcus pneumoniae and
Haemophilus influenzae. On physical examination, the child
has no organomegaly and no deformities. Laboratory studies
show hemoglobin of 9.2 g/dL, hematocrit of 27.8%, platelet
count of 372,000/mm3, and WBC count of 10,300/mm3. A hemoglobin
electrophoresis shows 1% hemoglobin A2, 7% hemoglobin
F, and 92% hemoglobin S. Which of the following is
the most likely cause of the repeated infections in this child?

(RBC white white center) 
A Absent endothelial cell expression of adhesion
molecules
B Diminished hepatic synthesis of complement
proteins
C Impaired neutrophil production
D Loss of normal splenic function
E Reduced synthesis of immunoglobulins
A

D Loss of normal splenic function

Sickle cell anemia

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165
Q

13 A 12-year-old boy has a history of episodes of severe abdominal,
chest, and back pain since early childhood. On physical
examination, he is afebrile, and there is no organomegaly.
Laboratory studies show hemoglobin of 11.2 g/dL, platelet
count of 194,000/mm3, and WBC count of 9020/mm3. The peripheral
blood smear shows occasional sickled cells, nucleated
RBCs, and Howell-Jolly bodies. Hemoglobin electrophoresis
shows 1% hemoglobin A2, 6% hemoglobin F, and 93% hemoglobin
S. Hydroxyurea therapy is found to be beneficial in
this patient. An increase in which of the following is the most
likely basis for its therapeutic efficacy?

A Erythrocyte production
B Overall globin chain synthesis
C Oxygen affinity of hemoglobin
D Production of hemoglobin A
E Production of hemoglobin F
A

E Production of hemoglobin F

Sickle cell anemia

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166
Q

19 34-year-old woman reports becoming increasingly tired
for the past 5 months. On physical examination, she is afebrile
and has mild splenomegaly. Laboratory studies show a hemoglobin
concentration of 10.7 g/dL and hematocrit of 32.3%. The
peripheral blood smear shows spherocytes and rare nucleated
RBCs. Direct and indirect Coombs test results are positive at
37° C, although not at 4° C. Which of the following underlying
diseases is most likely to be diagnosed in this patient?
A Escherichia coli septicemia
B Hereditary spherocytosis
C Infectious mononucleosis
D Mycoplasma pneumoniae infection
E Systemic lupus erythematosus

A

E Systemic lupus erythematosus

Warm autoimmune hemolytic anemia secondary to SLE

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167
Q

20 A 22-year-old woman has experienced malaise and a
sore throat for 2 weeks. Her fingers turn white on exposure
to cold. On physical examination, she has a temperature of
37.8° C, and the pharynx is erythematous. Laboratory findings
include a positive monospot (heterophile antibody) test
result. Direct and indirect Coombs test results are positive at
4° C, although not at 37° C. Which of the following molecules
bound on the surfaces of the RBCs most likely accounts for
these findings?
A α2-Macroglobulin
B Complement C3b
C Fibronectin
D Histamine
E IgE

A

B Complement C3b

Cold agglutinin disease

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168
Q

38 A 28-year-old, previously healthy man has noted
increasing fatigue for the past 6 months and formation of bruises
after minimal trauma. Over the past 2 days, he has developed
a cough. On physical examination, his temperature is 38.9° C,
and he has diffuse rales in both lungs. He has no hepatosplenomegaly
and no lymphadenopathy. Laboratory findings include
a sputum culture positive for Streptococcus pneumoniae,
hemoglobin of 7.2 g/dL, hematocrit of 21.7%, platelet count of 23,400/mm3, WBC count of 1310/mm3, prothrombin time of 13 seconds, partial thromboplastin time of 28 seconds, and total bilirubin of 1 mg/dL. The ANA test result is negative. What is the most likely explanation of these findings?
A Hematopoietic stem cell defect
B Hemolysis of antibody-coated cells
C Increased susceptibility to lysis by complement
D Metastatic adenocarcinoma to bone marrow
E Secondary hypersplenism

A

A Hematopoietic stem cell defect

Aplastic anemia

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169
Q

47 A 37-year-old woman has noted an excessively heavy
menstrual flow each of the past 6 months. She also has noticed
increasing numbers of pinpoint hemorrhages on her lower extremities
in the past month. Physical examination shows no organomegaly
or lymphadenopathy. CBC shows hemoglobin of
14.2 g/dL, hematocrit of 42.5%, MCV of 91 μm3, platelet count
of 15,000/mm3, and WBC count of 6950/mm3. On admission
to the hospital, she has melena and after a transfusion of platelets,
her platelet count does not increase. Which of the following
describes the most likely basis for her bleeding tendency?
A Abnormal production of platelets by
megakaryocytes
B Defective platelet-endothelial interactions
C Destruction of antibody-coated platelets by the spleen
D Excessive loss of platelets in menstrual blood
E Suppression of pluripotent stem cell division

A

C Destruction of antibody-coated platelets by the spleen

Bleeding due to low platelet count

Idiopathic chronic immune thrombocytopenia purpura (ITP)

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170
Q

48 A 9-year-old boy has developed prominent bruises on
his extremities over the past week. On physical examination,
he has ecchymoses and petechiae on his arms and legs.
Laboratory studies show hemoglobin, 13.8 g/dL; hematocrit,
41.9%; MCV, 93 μm3; platelet count, 11,300/mm3; and WBC
count, 7720/mm3. He had respiratory syncytial virus pneumonia
3 weeks ago. His condition improves with corticosteroid
therapy. Which of the following abnormalities is most likely to
cause his hemorrhagic diathesis?
A Antiplatelet antibodies
B Bone marrow aplasia
C Glycoprotein IIb/IIIa dysfunction
D Vitamin C deficiency
E Von Willebrand factor metalloproteinase deficiency

A

A Antiplatelet antibodies

Acute immune thrombocytopenic purpura (ITP) and chronic ITP

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171
Q

49 A 21-year-old woman known to have a protein C deficiency
develops recurrent pulmonary thromboembolism and
is placed on anticoagulant therapy. Two weeks after initiation
of this therapy, she has a sudden change in mental status and
experiences difficulty speaking and swallowing. A cerebral
angiogram shows a left middle cerebral artery occlusion. Laboratory
studies show hemoglobin of 13 g/dL, platelet count of
65,400/mm3, WBC count of 5924/mm3, prothrombin time of
12 seconds, and partial thromboplastin time of 51 seconds. The
anticoagulant therapy is discontinued. Which of the following
pharmacologic agents used as an anticoagulant in this patient
is most likely to have caused these findings?
A Acetylsalicylic acid (aspirin)
B Heparin
C Tissue plasminogen activator
D Urokinase
E Warfarin

A

B Heparin

Heparin-induced thrombocytopenia

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172
Q

45 A 23-year-old woman in her 25th week of pregnancy has
felt no fetal movement for the past 3 days. Three weeks later,
she still has not given birth and suddenly develops dyspnea
with cyanosis. On physical examination, her temperature is 37°
C, pulse is 106/min, respirations are 23/min, and blood pressure
is 80/40 mm Hg. She has large ecchymoses over the skin
of her entire body. A stool sample is positive for occult blood.
Laboratory studies show an elevated prothrombin time and
partial thromboplastin time. The platelet count is decreased,
plasma fibrinogen is markedly decreased, and fibrin split products
are detected. A blood culture is negative. Which of the following
is the most likely cause of her bleeding diathesis?
A Consumption of coagulation factors
B Defects in platelet aggregation
C Increased vascular fragility
D Reduced production of platelets
E Toxic injury to the endothelium

A

A Consumption of coagulation factors

Thrombocytopenia
Increased PT and PTT
Fibrin split products
Low fibrinogen

Disseminated intravascular coagulation (DIC)

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173
Q

50 A 56-year-old woman suffers the sudden onset of headache
and photophobia, and her condition worsens for the next 2
days. On physical examination, she has a temperature of 38° C
and is disoriented. CBC shows hemoglobin of 11.2 g/dL, hematocrit
of 33.7%, MCV of 94 μm3, platelet count of 32,000/
mm3, and WBC count of 9900/mm3. The peripheral blood
smear shows schistocytes. The serum urea nitrogen level is 38
mg/dL, and the creatinine level is 3.9 mg/dL. Which of the
following is the most likely diagnosis?
A Autoimmune hemolytic anemia
B β-Thalassemia major
C Disseminated intravascular coagulation
D Idiopathic thrombocytopenic purpura
E Paroxysmal nocturnal hemoglobinuria
F Thrombotic thrombocytopenic purpura

A

F Thrombotic thrombocytopenic purpura

TTP

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174
Q

54 A clinical study is performed involving adult patients
diagnosed with microangiopathic hemolytic anemia. A subgroup
of patients who had fever or diarrhea preceding the
initial diagnosis of anemia were excluded. The patients had
schistocytes present on peripheral blood smears. Some of these
patients were found to have a deficiency of a metalloproteinase
known as ADAMTS13. Which of the following conditions
were the patients with this deficiency most likely to have?
A Disseminated intravascular coagulation (DIC)
B Hemolytic-uremic syndrome (HUS)
C Heparin-induced thrombocytopenia (HIT)
D Idiopathic thrombocytopenic purpura (ITP)
E Thrombotic thrombocytopenic purpura (TTP)

A

E Thrombotic thrombocytopenic purpura (TTP)

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175
Q

55 A 45-year-old woman has had episodes of blurred vision
and headaches for the past 6 months. She has had worsening
confusion with paresthesias over the past 3 days. On
physical examination, she has a temperature of 39.6° C, pulse
of 100/min, respiratory rate of 20/min, and blood pressure
of 80/50 mm Hg. Petechial hemorrhages are noted over her
trunk and extremities. Laboratory findings include hemoglobin,
10.9 g/dL; hematocrit, 34%; MCV, 96 μm3; platelet count,
28,000/mm3; and WBC count, 8500/mm3. Fragmented RBCs
are noted on her peripheral blood smear. Blood urea nitrogen
is 40 mg/dL, and serum creatinine is 3.1 mg/dL. Which of the
following is the most likely underlying cause for her findings?
A Circulating toxin that injures capillary endothelium
B Decreased factor VIII activity
C Defective ADP-induced platelet aggregation
D Formation of autoantibodies to platelet glycoproteins
IIb/IIIa and Ib-IX
E Inappropriate release of thromboplastic substances
into blood
F Presence of antibodies against ADAMTS13
metalloproteinase

A

F Presence of antibodies against ADAMTS13

Neurlogic abnromalities
Fever
Thrombocytopenia
microangiopathic hemolytic anemia
Renal failure

Thrombotic thrombocytopenic purpura (TTP)

deficiency of von Willebrand factor (vWF) metalloproteinase (ADMTS13)

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176
Q

52A 23-year-old woman has a history of easy bruising.
Physical examination shows multiple bruises ranging in color
from red to blue to purple on her arms and legs. There is no organomegaly,
and no deformities are noted. Laboratory studies
show hemoglobin, 9.5 g/dL; hematocrit, 28.2%; platelet count,
229,300/mm3; WBC count, 7185/mm3; prothrombin time, 12
seconds; and partial thromboplastin time, 38 seconds. A 1:1
dilution of the patient’s plasma with normal pooled plasma
corrects the partial thromboplastin time. Ristocetin-dependent
platelet aggregation in patient plasma is markedly reduced.
Factor VIII activity is 30% (reference range 50% to 150%).
Which of the following is the most likely potential consequence
of this disease?
A Bone marrow failure from aplasia
B Excessive bleeding after oral surgery
C Increasing difficulty with joint mobility
D Myeloproliferative disorder
E Recurrent deep venous thrombosis

A

B Excessive bleeding after oral surgery

Normal platelet count
Prolonged bleeding time

Von Willebrand disease

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177
Q

58 A 15-year-old girl has a history of easy bruising and
hemorrhages. Since menarche at the age of 13 years, she has had menometrorrhagia. On physical examination, she displays joint deformity and has decreased mobility of the ankles, knees, and wrists. Laboratory studies show hemoglobin, 11.8 g/dL; hematocrit, 35.1%; platelet count, 267,000/mm3;
WBC count, 5960/mm3; prothrombin time, 13 seconds; and
partial thromboplastin time, 60 seconds. A 1:1 dilution of the patient’s plasma with normal pooled plasma corrects the partial thromboplastin time. Which of the following is the most
likely diagnosis?
A Antiphospholipid syndrome
B Hemophilia B
C Idiopathic thrombocytopenic purpura
D Thrombotic thrombocytopenic purpura
E Von Willebrand disease

A

B Hemophilia B

PTT corrected by nromal pooled plasma

Factor IX deficiency

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178
Q

56 A 12-year-old boy has had worsening problems with joint
mobility involving his arms and legs, particularly his knees and
ankles, for the past 6 years. He has been receiving therapy for
this condition. His grandfather had a similar condition and died
at age 25 years. On physical examination, he has no visible petechiae
or areas of purpura. Laboratory studies show that prothrombin
time is 12 seconds, and partial thromboplastin time
is 52 seconds. After addition of an equivalent aliquot of normal
plasma, the partial thromboplastin time is 30 seconds. Hemoglobin
is 12.9 g/dL, platelet count is 238,500/mm3, and WBC
count is 6620/mm3. His platelet function studies are normal.
What is the most likely inheritance pattern for his condition?
A Autosomal dominant
B Autosomal recessive
C Confined placental mosaicism
D Germline mutation
E X-linked recessive

A

E X-linked recessive

Hemophilia A
monitored with Factor VIII

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179
Q

59 A 16-year-old girl has had frequent nosebleeds since
childhood. Her gums bleed easily, even with routine tooth
brushing. She has experienced menorrhagia since menarche
at age 13 years. On physical examination, there are no abnormal
findings. Laboratory studies show hemoglobin,
14.1 g/dL; hematocrit, 42.5%; MCV, 90 μm3; platelet count,
277,400/mm3; and WBC count, 5920/mm3. Her platelets fail
to aggregate in response to ADP, collagen, epinephrine, and
thrombin. The ristocetin agglutination test result is normal.
There is a deficiency of glycoprotein IIb/IIIa. Prothrombin time is 12 seconds, and partial thromboplastin time is 28 seconds.
What is the most likely diagnosis?
A Disseminated intravascular coagulation
B Glanzmann thrombasthenia
C Immune thrombocytopenic purpura
D Vitamin C deficiency
E Von Willebrand disease

A

B Glanzmann thrombasthenia

AR

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180
Q

An 8-year-old boy is brought to the pediatrician
after his parents noticed very dark urine in the
toilet earlier that morning. Initial laboratory
studies show intravascular hemolysis; further
testing shows that his RBCs are susceptible to
complement-mediated lysis. This patient most
likely has which of the following conditions?
(A) Autoimmune hemolytic anemia
(B) Common bile duct stricture
(C) Hereditary spherocytosis
(D) Paroxysmal nocturnal hemoglobinuria
(E) Thrombotic thrombocytopenic purpura

A

(D) Paroxysmal nocturnal hemoglobinuria

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181
Q
  1. Low-molecular-weight heparins (LMWH) are
    distinct from unfractionated heparin in several
    ways. Which of the following is the primary
    target of LMWH?
    (A) Antithrombin III
    (B) Factor IIa
    (C) Factor VII
    (D) Factor Xa
    (E) Factors II, IX, and X
A

(D) Factor Xa

LMWH act on Xa

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182
Q
  1. A 7-year-old African-American boy is brought
    to see his pediatrician. His father says he has
    noticed that the boy has been complaining of
    right knee pain for the past week. On physical
    exam, multiple ecchymoses are noted on both
    upper and lower extremities. His father claims
    that the boy has always bruised easily, and he
    has recently learned how to ride a bicycle.
    Which of the following elements of the coagulation
    cascade is most likely to be missing in
    this child?
    (A) Antithrombin III
    (B) Factor VII
    (C) Factor VIII
    (D) Factor IX
    (E) Protein C
A

(C) Factor VIII

Hemophilia A
X linked

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183
Q
  1. A 16-year-old boy is brought to the emergency
    department because of the acute onset of fever,
    chills, and a productive cough. X-ray of the
    chest shows an infi ltrate restricted to the left
    lower lobe. Samples taken of the sputum show
    α-hemolytic gram-positive cocci in pairs. The
    patient says that he has had similar infections
    over the past year. A peripheral blood smear is
    done, and results show several sickle-shaped
    RBCs. Which of the following explains why
    this patient is susceptible to this particular type
    of infection?
    (A) Bone marrow infi ltration resulting in neutropenia
    and compromised immune function
    (B) Large vessel occlusions in the cerebral vasculature
    resulting in neurologic events and
    aspiration pneumonia
    (C) Microvascular infarcts resulting in pulmonary
    failure
    (D) Microvascular infarcts resulting in splenic
    dysfunction
    (E) Vaso-occlusion in the renal medulla
A

(D) Microvascular infarcts resulting in splenic
dysfunction

Sickle cell disease

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184
Q

14.A child is brought to the pediatrician because
her parents are concerned about lead poisoning
since their house is known to contain leadbased
paint. A complete blood cell count reveals
anemia. Lead poisoning causes anemia
because it does which of the following?
(A) Disrupts heme synthesis by causing decreased
iron absorption from the gut
(B) Disrupts heme synthesis by increasing the
activity of aminolevulinate dehydratase
(C) Disrupts heme synthesis by inhibiting ferrochelatase
(D) Disrupts hemoglobin function by binding
to hemoglobin with high affi nity, preventing
oxygen binding
(E) Disrupts RBC DNA synthesis, causing
megaloblastic changes in RBCs

A

(C) Disrupts heme synthesis by inhibiting ferrochelatase

Lead inhibits (ALA) dehydratase
Ferrochelatase
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185
Q

27 A 3-year-old girl was in her usual state of good
health 1 month ago when she developed an
acute viral upper respiratory infection. She
now presents to the emergency department
with nonblanching purple skin lesions. The
rest of her physical examination is unremarkable.
The complete blood count demonstrates
a low platelet count, while the peripheral
blood smear is notable only for large platelets.
Which of the following laboratory fi ndings
would most likely be present in this patient?
(A) Antiplatelet antibodies
(B) Decreased megakaryocytes on bone marrow
biopsy
(C) IgA defi ciency
(D) Increased fi brin split products
(E) Vitamin K defi ciency

A

(A) Antiplatelet antibodies

Idiopathic thrombocytopenic purpura (ITP)

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186
Q

36 Hydrops fetalis occurs in the setting of a certain
type of thalassemia. What is the underlying
mechanism leading to this event?
(A) Excess α-globin chains binding tighter to
oxygen
(B) Excess α-globin chains binding weaker to
oxygen
(C) Excess β-globin chains binding tighter to
oxygen
(D) Excess β-globin chains binding weaker to
oxygen
(E) Excess gamma-globin chains binding
tighter to oxygen
(F) Excess gamma-globin chains binding
weaker to oxygen

A

(E) Excess gamma-globin chains binding
tighter to oxygen

alpha thalassemia

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187
Q

40 A 28-year-old woman comes to the physician
concerned about an excessive amount of bleeding
from her gums when she brushes her teeth.
Her laboratory results show an increased partial
thromboplastin time and an increased bleeding
time, but are otherwise unremarkable.
Which of the following treatments will most
likely alleviate this patient’s symptoms?
(A) Cryoprecipitate
(B) Factor VIII concentrate
(C) Fresh frozen plasma
(D) Low-molecular-weight heparin
(E) Protamine sulfate

A

(A) Cryoprecipitate

Von Willebrand disease

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188
Q

42 A 62-year-old woman presents to the clinic
complaining of frequent bleeding while brushing
her teeth and easy bruising. She reports
she recently had pneumonia and was treated
with a broad-spectrum antibiotic. Laboratory
tests show:
Prothrombin time: 18 seconds
Partial thromboplastin time: 37 seconds
Platelet count: 231,000/mm³
Hematocrit: 37%
WBC count: 4800/mm³
The cofactor that is defi cient in this patient is
needed for the carboxylation of glutamate residues
of which of the following?
(A) Factors II, VII, VIII, and X
(B) Factors VII, VIII, IX, and XII
(C) Proteins C and S and factors IX, X, XI, and
XII
(D) Proteins C and S and factors XII, IX, and X
(E) Proteins C and S, prothrombin, and factors
VII, IX, and X

A

(E) Proteins C and S, prothrombin, and factors
VII, IX, and X

Prolong pT
Deficiency of factor in extrinsic pathways

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189
Q
  1. A 20-year-old African-American man develops
    anemia after being treated for a urinary tract infection.
    A peripheral blood smear shows RBC
    lysis and precipitates of hemoglobin within the
    RBCs. Which of the following drug classes
    most likely caused his hemolytic anemia?
    (A) Aminoglycosides
    (B) Fluoroquinolones
    (C) Macrolides
    (D) Sulfonamides
    (E) Tetracyclines
A

(D) Sulfonamides

G6PD deficiency

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190
Q

44 Several drugs are used to prevent myocardial
infarction in patients with acute coronary syndrome.
One class of drugs binds to the glycoprotein
receptor IIb/IIIa on activated platelets,
thereby interfering with platelet aggregation.
This prevents renewed formation of clots that
could block the lumen of the cardiac vessels.
Which of the following is an example of this
class of drug?
(A) Abciximab
(B) Clopidogrel
(C) Leuprolide
(D) Selegiline
(E) Ticlopidine

A

(A) Abciximab

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191
Q

47 A 70-year-old man comes to his physician for a
routine physical examination. Although he is
asymptomatic, a blood test shows an abnormal
level of immunoglobulin. After further testing,
he is diagnosed with monoclonal gammopathy
of undetermined signifi cance. Which of the following is the current treatment for monoclonal
gammopathy of undetermined signifi -
cance?
(A) Alendronate
(B) Anticoagulation
(C) High-dose steroids
(D) No treatment
(E) Vinca alkaloids

A

(D) No treatment

Monoclonal gammopathy of undetermined significant (MGUS)

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192
Q

48 An 8-year-old boy has a chronic history of severe
hemolytic anemia, hepatosplenomegaly,
and maxillary overgrowth. He has received
blood transfusions since early infancy but has
not received a transfusion in over 4 months. A
hemoglobin electrophoresis shows marked elevation
of HbF, increased HbA2, and absence of
HbA1. Which of the following diagnoses is
most consistent with this patient’s electrophoresis?
(A) α-Thalassemia minor
(B) β-Thalassemia major
(C) β-Thalassemia minor
(D) Glucose 6-phosphate dehydrogenase defi -
ciency
(E) HbH disease
(F) RBCs containing hemoglobin Barts

A

(B) β-Thalassemia major

Thalassemias

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193
Q

Sickle cell disease

  • mutation
  • xray
  • tx
A

HbS mutation
Point mutation on beta globulin
Glu –> Val

Hair on end appearance on Xray skull
– bone marrow hyperplasia

Hydroxyurea
-increases production of HbF

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194
Q

Order of bands on electrophoresis

A 
Left to right
C 
S
F
A
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195
Q

Ham’s test

Osmotic fragility test

A

Paroxysmal nocturnla hemoglobinuria

Hereditary spherocytosis

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196
Q

Women develops muscle cramps and darkening of urine after exercising. Diagnosis?

A

McArdle disease

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197
Q

Von Willebrand factor (vWF)

  • fxn
  • works with
  • defieicny
A

Complexes with and stabilizes factor VIII

Platelet adhesion to vessel wall and other platelets

Deficiency increase PTT
Increase bleeding time

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198
Q

Life span platelets

A

8-10 days

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199
Q

Platelet Adhesion steps

A

Endothelial damage

Exposure underlying collagen

Damaged cells release contents

vWF binds exposed collagen and bind glycoprotein receptor GpIb on surface of platelet

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200
Q

Platelet Activation

Platelet Aggregation

A

Activation

  • platelets grow pseudopods help bind in injury site
  • secrete substances

Aggregation

  • Activated platelet secretes ADP (adenosine diphosphate)
  • ADP binds to receptor on surface of platelet
  • Triggers expression of glycoprotein IIB/IIIA to come to surface of platelet
  • binds fibrinogen, forms series of crosslinks between platelets
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201
Q

Branching rods on oral infection

A

Actinomyces israelii

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202
Q

Eczema
Recurrent infections
Thrombocytopenia

A

Wiskott-aldrich syndrome

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203
Q

Hemosiderinuria

Thrombosis

A

Paroxysmal nocturnal hemoglobinuria

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204
Q

Deficiency of metalloprotease ADAMTS13

A

Thrombotic thrombocytopenic purpura (TTP)

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205
Q

Thrombotic thrombocytopenic purpura (TTP)

A

Deficiency of metalloprotease ADAMTS13

ADAMTS13 cleaves vWF into active units

Unregulated platelet aggregation –> thrombosis

Consumption of paltelets –> thrombocytopenia

Microangiopathic hemolytic anemia

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206
Q

Platelet disorder presentation

A

Bleeding from mucous membranes
Epistaxis (nose bleeds)
Petechiae
Purpura

Increased bleeding time

Normal PT and PTT

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207
Q

Immune thrombocytopenia (ITP)

  • labs
  • tx
A

Autoantibodies bind glycoprotein IIb/IIIa on surface platelets

Decreased platelets
Increased megakaryocytes
(trying to make more platelets)

Steroids
IVIG
Splenectomy

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208
Q

Thrombotic thromocytopenic purpura symptoms

A 
"HUNT For The TPP"
Hemolysis
Uremia
Neurological symptoms
Thrombocytopenia
Fever 
TTP
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209
Q

Hemolytic uremic syndrome associated with

features

A

E.coli 0157:H7

Children

Hemolysis
Renal insufficiency
Thromobytopenia

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210
Q

Bernard-Soulier syndrome

A

Defect of glycoprotein Ib

Platelet unable to bind collagen

Moderate thrombocytopenia

Platelet number only slightly low
Increased bleeding time

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211
Q

Glanzmann thrombasthenia

A

Defect in glycoprotein IIb/IIIa

Abnormal platelet aggregation

Normal platelet count
Increased bleeding time

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212
Q

Increased PTT

Increased bleeding time

A

Von Willebrand disease

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213
Q

Ristocetin cofactor assay

A

Causes platelets to agglutinate if vWF is present

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214
Q

Von Willebrand disease

tx

A

DDAVP
- desmopressin (ADH analog)

ADH increase release of vWF from storage sites in endothelial cells

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215
Q 
Decreased platelets
Increased bleeding time
Increased PT and PTT
Decreased fibrinogen
Increase fibrin split products (d-dimer)
A

Disseminated intravascular coagulation (DIC)

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216
Q

Which matches?

  1. Anti-gp IIb/IIIa antibodies
  2. ADAMTS-13 deficiency
  3. gp Ib defect
  4. gp IIb/ IIIa defect
  5. Von Willebrand factor defect
  6. Widespread activation of platelets and coagulation cascade
A
  1. ITP
  2. TTP-HUS
  3. Bernard-Soulier syndrome
  4. Glanzmann thromasthenia
  5. von Willebrand disease
  6. DIC
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217
Q

Antiplatelet antibodies

A

Immune thrombocytopenia

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218
Q

Large cells with bi-lobed nucleus
Prominent nucleoli with clearing around nucleoi
Clearing around cell

A

Reed Sternberg cells

Hodgkin lymphoma

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219
Q 
48 A 35-year-old woman presents to her primary care physician with a fever of 38.3º C (101º F), night sweats, and fatigue. The patient says that she has lost about 6.7 kg (15 lb) over the past year. A CT scan demonstrates mediastinal lymphadenopathy. Biopsy of the nodes shows a small number of large cells with “owl-eye” nucleoli, multiple nuclei, and an abundance of pale cytoplasm on a background of many reactive lymphocytes, macrophages, and granulocytes.
Which of the following drugs could be
used to treat this condition?
(A) Azathioprine
(B) Cisplatin
(C) Doxorubicin
(D) Paclitaxel
(E) β-Interferon
A

C. Doxorubicin

Hodgkin lymphoma

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220
Q
  1. A 34-year-old man presents to his primary care physician with night sweats, a fever of 38º C (100.2º F), and weight loss of 5 kg (12 lb) over the last 3 months. A CT scan demonstrates mediastinal
    lymphadenopathy, and results of a biopsy
    of the node are shown in the image.
    Which of the following drugs is part of the
    multidrug regimen that would be used to manage this patient’s disease?
(A) Cyclosporine
(B) Hydroxyurea
(C) Imatinib
(D) Isoniazid
(E) Vinblastine
A

E.(E) Vinblastine

ABVD
Adriamycin
Bleomycin
Vinblastine
Decarbazine
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221
Q

Hodgkin lymphoma in younger male

A

Lymphocyte-prdominant type

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222
Q

Few reed sterberg to lymphocytes
Bands of collagen, sclerosis and fibrosis
Women

A

Nodular sclerosing

Hodgkin lymphoma

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223
Q

t(14;18)

A

Diffuse large B cell lymphoma
-Elderly

Follicular lymphoma

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224
Q

t (8;14)

A

Burkitt lymphoma

c-MYC

Starry sky appearance

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225
Q

Type of burkitt lymphoma

A

Endemic

  • Africa
  • involves mandible

Sporadic
- pelvis or abdomen

Immunodeficiency assoc
- HIV

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226
Q

t(11;14)

A

Mantle cell lymphoma

Disruption of regulation of cyclin D

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227
Q

Non-hodgkin lymphoma associated with sjogren, hashimoto and h. pylori

A

Marginal cell MALToma

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228
Q

Most common lymphoma in U.S

A

Diffuse large B cell lymphoma

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229
Q

Owl eye nuclei

A

Reed-sternberg cels

Hodgkin lymphoma

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230
Q

Lymphoma associated with EBV

A

Burkitt lymphoma

Hodgkin lymphoma

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231
Q

Over 20% blasts in bone marrow

A

AML or ALL

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232
Q 
Child
Bone pain
terminal deoxynucleotidy transferase (TdT) 
20% blasts
t(9;22)
A

ALL

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233
Q

Auer rods
20% blasts
Adult

A

AML

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234
Q

Elderly adult
CD19 CD 20
Smudge cells

A

CLL

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235
Q 
t(9;22)
Fatigue
Abdominal pain
Splenomegaly
Middle aged
A

CML

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236
Q

t(9;22)

  • is what
  • creates
  • associated with
  • tx
A

Philadelpha chromosome

Mutation bcr-abl
Oncogene
Activated tyrosine kinase always on

CML: always present
ALL: Sometimes present
AML: rarely present

Tx: imatinib
- inhibits bcr-abl tyrosine kinase

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237
Q

t(15;17)

tx

A

M3 AML

Treat w/ all-trans retinoic acid

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238
Q

Acute luekemia positive for peroxidase

A

AML

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239
Q

Bence Jones proteins

A

Multiple myeloma

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240
Q

Rouleaux formation

A

RBC stacked like coins

MM

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241
Q

33 32-year-old woman presents to the emergency
department with a temperature of 38.7º
C (101.7º F), petechiae covering her abdomen,
and mental status changes. On questioning,
her husband states that “she had been fi ne
3 weeks ago but got sick real fast.” Physical examination
shows severe weakness, petechiae
and ecchymoses on the abdomen and back,
and pale conjunctiva. A laboratory study shows
severe anemia and thrombocytopenia with leukocytosis.
The peripheral blood smear demonstrates
the presence of abundant myeloblasts.
Which of the following chromosomal translocations
is most likely involved in this disorder?
(A) t(8:14) c-myc activation
(B) t(9:22), bcr-abl
(C) t(11:14), BCL1/PRAD1
(D) t(11:22), EWS gene
(E) t(15:17), PML-RAR-α

A

(E) t(15:17), PML-RAR-α

AML

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242
Q

15 A 70-year-old man has experienced increasing fatigue
for the past 6 months. On physical examination, he has nontender
axillary and cervical lymphadenopathy, but there is no
hepatosplenomegaly. The CBC shows hemoglobin, 9.5 g/dL;
hematocrit, 28%; MCV, 90 μm3; platelet count, 120,000/mm3;
and WBC count, 42,000/mm3. His peripheral blood smear
shows a monotonous population of small, round, maturelooking
lymphocytes. Flow cytometry shows these cells to
be CD19+, CD5+, and deoxynucleoti dyl transferase negative
(TdT−). Cytogenetic and molecular analysis of the abnormal
cells in his blood are most likely to reveal which of the following
alterations?
A Clonal rearrangement of immunoglobulin genes
B Clonal rearrangement of T-cell receptor genes
C t(8;14) leading to c-MYC overexpression
D t(9;22) leading to BCR-ABL rearrangement
E t(14;18) leading to BCL2 overexpression

A

A Clonal rearrangement of immunoglobulin genes

CLL

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243
Q

17 A 4-year-old boy has appeared listless during the
past week. He exhibits irritability when his arms or legs are
touched. In the past 2 days, large ecchymoses have appeared
on the right thigh and left shoulder. CBC shows hemoglobin,
9.3 g/dL; hematocrit, 28.7%; MCV, 96 μm3; platelet count,
45,000/mm3; and WBC count, 13,990/mm3. Examination of
the peripheral blood smear shows blasts that lack peroxidasepositive
granules, but contain PAS-positive aggregates and
stain positively for deoxynucleoti dyl transferase negative
(TdT−). Flow cytometry shows the phenotype of blasts to be
CD19+, CD3−, and sIg−. Which of the following is the most
likely diagnosis?
A Acute lymphoblastic leukemia
B Acute myelogenous leukemia
C Chronic lymphocytic leukemia
D Chronic myelogenous leukemia
E Idiopathic thrombocytopenic purpura

A

A Acute lymphoblastic leukemia

ALL

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244
Q

8 A 36-year-old woman has a cough and fever for 1 week.
On physical examination, her temperature is 38.3° C. She has
diffuse crackles in all lung fields. A chest radiograph shows
bilateral extensive infiltrates. CBC shows hemoglobin, 13.9 g/
dL; hematocrit, 42%; MCV, 89 μm3; platelet count, 210,000/
mm3; and WBC count, 56,000/mm3 with 63% segmented neutrophils,
16% bands, 7% metamyelocytes, 3% myelocytes, 1%
blasts, 8% lymphocytes, and 2% monocytes. A bone marrow
biopsy is obtained and shows normal maturation of myeloid
cells. Which of the following is the most likely diagnosis?
A Chronic myelogenous leukemia
B Hairy cell leukemia
C Hodgkin lymphoma, lymphocyte depletion type
D Leukemoid reaction
E Myelodysplastic syndrome

A

D Leukemoid reaction

CML

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245
Q

14 A 14-year-old boy complains of discomfort in his chest
that has worsened over the past 5 days. On physical examination,
he has generalized lymphadenopathy. A chest radiograph
shows clear lung fields, but there is widening of the mediastinum.
A chest CT scan shows a 10-cm mass in the anterior
mediastinum. A biopsy specimen of the mass is obtained and
microscopically shows effacement by lymphoid cells with
lobulated nuclei having delicate, finely stippled, nuclear chromatin.
There is scant cytoplasm, and many mitotic figures are
seen. The cells express deoxynucleoti dyl transferase negative
(TdT−), CD2, and CD7 antigens. Molecular analysis reveals a
point mutation in the NOTCH1 gene. The oncologist tells the
parents that chemotherapy can be curative in vast majority of
such cases. What is the most likely diagnosis?
A Burkitt lymphoma
B Follicular lymphoma C Hodgkin lymphoma, nodular sclerosing type
D Lymphoblastic lymphoma
E Mantle cell lymphoma
F Small lymphocytic lymphoma

A

D Lymphoblastic lymphoma

ALL

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246
Q

18 A 7-year-old boy has complained of a severe headache
for the past week. On physical examination, there is tenderness
on palpation of long bones, hepatosplenomegaly, and
generalized lymphadenopathy. Petechial hemorrhages are
present on the skin. Laboratory studies show hemoglobin,
8.8 g/dL; hematocrit, 26.5%; platelet count, 34,700/mm3; and
WBC count, 14,800/mm3. A bone marrow biopsy specimen
shows 100% cellularity, with almost complete replacement
by a population of large cells with scant cytoplasm lacking
granules, delicate nuclear chromatin, and rare nucleoli. His
oncologist is confident that chemotherapy will induce a complete
remission. Which of the following combinations of phenotypic
and karyotypic markers is most likely to be present in
marrow cells from this boy?
A Early pre-B CD19+ hyperdiploidy
B Early pre-B CD20+ t(9;22)
C Pre-B CD5+ normal karyotype
D Pre-B CD23+ 11q deletion
E T cell CD2+ numerous blasts
F T cell CD3+ MLL gene translocation

A

A Early pre-B CD19+ hyperdiploidy

ALL

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247
Q

20 The figure skater Sonja Henie, who won gold medals
at the 1928, 1932, and 1936 Winter Olympic Games, became
progressively fatigued in her late 50s. On physical examination,
she had palpable nontender axillary and inguinal lymph
nodes, and the spleen tip was palpable. Laboratory studies
showed hemoglobin, 10.1 g/dL; hematocrit, 30.5%; MCV, 90
μm3; platelet count, 89,000/mm3; and WBC count, 31,300/
mm3. From the peripheral blood smear shown in the figure,
which of the following is the most likely diagnosis?
A Acute lymphoblastic leukemia
B Chronic lymphocytic leukemia
C Infectious mononucleosis
D Iron deficiency anemia
E Leukemoid reaction

A

B Chronic lymphocytic leukemia

CLL

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248
Q

24 A 62-year-old man has experienced vague abdominal
discomfort accompanied by bloating and diarrhea for the past
6 months. On physical examination, there is a midabdominal
firm mass. The stool is positive for occult blood. An abdominal
CT scan shows an 11 × 4 cm mass involving the wall of the
distal ileum and adjacent mesentery. A laparotomy is performed,
and the mass is removed. Microscopically, the mass is
composed of sheets of large lymphoid cells with large nuclei,
prominent nucleoli, and frequent mitotic figures. The neoplastic
cells mark with CD19+ and CD20+ and have the BCL6 gene
rearrangement. Which of the following prognostic features is
most applicable to this case?
A Aggressive, can be cured by chemotherapy
B Aggressive, often spreads to liver, spleen, and marrow
C Aggressive, often transforms to acute leukemia
D Indolent, can be cured by chemotherapy
E Indolent, often undergoes spontaneous remission
F Indolent, survival of 7 to 9 years without treatment

A

A Aggressive, can be cured by chemotherapy

Diffuse large cell lymphoma of B cells

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249
Q

22 A 12-year-old boy has had increasing abdominal distention
and pain for the past 3 days. Physical examination of his
abdomen shows lower abdominal tenderness with tympany
and reduced bowel sounds. An abdominal CT scan shows a
7-cm mass involving the region of the ileocecal valve. Surgery
is performed and the resected mass microscopically shows
sheets of intermediate-sized lymphoid cells, with nuclei having
coarse chromatin, several nucleoli, and many mitotic figures.
A bone marrow biopsy sample is negative for this cell population.
Cytogenetic analysis of the cells from the mass shows
a t(8;14) karyotype. Flow cytometric analysis reveals 40% of
the cells are in S phase. The tumor shrinks dramatically after
a course of chemotherapy. Which of the following is the most
likely diagnosis?
A Acute lymphoblastic leukemia/lymphoma
B Burkitt lymphoma
C Diffuse large B-cell lymphoma
D Follicular lymphoma
E Plasmacytoma

A

B Burkitt lymphoma

Burkitt lymphoma
Sporadic

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250
Q

25 A 9-year-old boy living in Uganda has had increasing
pain and swelling on the right side of his face over the past 8
months. On physical examination, there is a large, nontender
mass involving the mandible, which deforms the right side of his face. There is no lymphadenopathy or splenomegaly, and
he is afebrile. A biopsy of the mass is performed and microscopic
examination shows intermediate-sized lymphocytes
with a high mitotic rate. A chromosome analysis shows a
46,XY,t(8;14) karyotype in these cells. The hemoglobin concentration
is 13.2 g/dL, platelet count is 272,000/mm3, and WBC
count is 5820/mm3. Infection with which of the following viruses
is most likely to be causally related to the development
of these findings?
A Cytomegalovirus
B Epstein-Barr virus
C Hepatitis B virus
D HIV
E Human papillomavirus
F Respiratory syncytial virus

A

B Epstein-Barr virus

Endemic african variety of burkitt lymphoma

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251
Q

23 A 55-year-old man felt a lump near his shoulder 1 week
ago. On physical examination, there is an enlarged, nontender,
supraclavicular lymph node and enlargement of the Waldeyer
ring of oropharyngeal lymphoid tissue. There is no hepatosplenomegaly.
CBC is normal except for findings of mild anemia.
A lymph node biopsy specimen shows replacement by a
monomorphous population of lymphoid cells that are twice the
size of normal lymphocytes, with enlarged nuclei and prominent
nucleoli. Immunohistochemical staining and flow cytometry
of the node indicates that most lymphoid cells are CD19+,
CD10+, CD3−, CD15−, and terminal deoxynucleotidyl transferase
negative (TdT−). A BCL6 gene mutation is present. Which of
the following is the most likely diagnosis?
A Acute lymphoblastic lymphoma
B Chronic lymphadenitis
C Diffuse large B-cell lymphoma
D Hodgkin lymphoma
E Small lymphocytic lymphoma

A

C Diffuse large B-cell lymphoma

Diffuse B cell lymphoma

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252
Q

30 A 62-year-old man has had fever and a 4-kg weight loss
over the past 6 months. On physical examination, his temperature
is 38.6° C. He has generalized nontender lymphadenopathy, and
the spleen tip is palpable. Laboratory studies show hemoglobin,
10.1 g/dL; hematocrit, 30.3%; platelet count, 140,000/mm3; and
WBC count, 24,500/mm3 with 10% segmented neutrophils, 1%
bands, 86% lymphocytes, and 3% monocytes. A cervical lymph
node biopsy specimen microscopically shows a nodular pattern
of small lymphoid cells. A bone marrow biopsy specimen shows
infiltrates of similar small cells having surface immunoglobulins
that are CD5+, but CD10−. Cytogenetic analysis indicates t(11;14)
in these cells. What is the most likely diagnosis?
A Acute lymphoblastic lymphoma
B Burkitt lymphoma
C Follicular lymphoma
D Mantle cell lymphoma
E Small lymphocytic lymphoma

A

D Mantle cell lymphoma

Mantle cell lymphoma

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253
Q

42 A 33-year-old man has experienced multiple nosebleeds
along with bleeding gums for the past month. On examination,
his temperature is 37.3° C. He has multiple cutaneous ecchymoses.
Laboratory studies show hemoglobin, 8.5 g/dL; hematocrit,
25.7%; platelet count, 13,000/mm3; and WBC count,
52,100/mm3 with 5% segmented neutrophils, 5% bands, 2%
myelocytes, 83% blasts, 3% lymphocytes, and 2% monocytes.
Examination of his peripheral blood smear shows the blasts
have delicate nuclear chromatin along with fine cytoplasmic
azurophilic granules. These blasts are CD33+. Which of the
following morphologic findings is most likely to be present on
his peripheral blood smear?
A Auer rods
B Döhle bodies
C Hairy projections
D Heinz bodies
E Sickle cells
F Toxic granulations

A

A Auer rods

AML

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254
Q

43 A 38-year-old man experiences sudden onset of a severe
headache. Physical examination shows no localizing neurologic
signs and no organomegaly. A stool sample is positive
for occult blood. Areas of purpura appear on the skin of his
extremities. Laboratory studies show hemoglobin of 9.6 g/dL,
hematocrit of 28.9%, platelet count of 16,400/mm3, and
WBC count of 75,000/mm3. The peripheral blood smear
has the appearance shown in the figure; schistocytes also
are seen. The plasma D-dimer level (fibrin degradation products),
prothrombin time, and partial thromboplastin time
all are elevated. Cytogenetic analysis of cells from a bone marrow
biopsy specimen is most likely to yield what karyotypic
abnormality?
A t(8;14)
B t(8;21)
C t(9;22)
D t(14;18)
E t(15;17)

A

E t(15;17)

Acute promyelocytic leukemia (M3)

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255
Q

47 In an experiment, cell samples are collected from the
bone marrow aspirates of patients who were diagnosed with
lymphoproliferative disorders. Cytogenetic analyses are performed
on these cells, and a subset of the cases is found to have
the BCR-ABL fusion gene from the reciprocal translocation
t(9;22)(q34;11). The presence of this gene results in increased
tyrosine kinase activity. Patients with which of the following
conditions are most likely to have this gene?
A Acute promyelocytic leukemia
B Chronic myelogenous leukemia
C Follicular lymphoma
D Hodgkin lymphoma, lymphocyte depletion type
E Multiple myeloma

A

B Chronic myelogenous leukemia

CML

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256
Q

49 A 60-year-old woman has had headaches and dizziness for
the past 5 weeks. She has been taking omeprazole for ulcers. On
physical examination, she is afebrile and normotensive, and her
face has a plethoric to cyanotic appearance. There is mild splenomegaly,
but no other abnormal findings. Laboratory studies
show hemoglobin, 21.7 g/dL; hematocrit, 65%; platelet count,
400,000/mm3; and WBC count, 30,000/mm3 with 85% polymorphonuclear
leukocytes, 10% lymphocytes, and 5% monocytes.
The peripheral blood smear shows abnormally large platelets
and nucleated RBCs. Which of the following is most characteristic
of the natural history of this patient’s disease?
A Development of a gastric non-Hodgkin lymphoma
B Increase in monoclonal serum immunoglobulin
C Marrow fibrosis with extramedullary hematopoiesis
D Spontaneous remissions and relapses without
treatment
E Transformation into acute B-lymphoblastic leukemia

A

C Marrow fibrosis with extramedullary hematopoiesis

Polycythemia vera (PCV)

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257
Q

50 A 45-year-old man has experienced a gradual weight
loss and weakness, anorexia, and easy fatigability for 9
months. Physical examination shows marked splenomegaly. CBC shows hemoglobin, 12.9 g/dL; hematocrit, 38.1%; MCV, 92 μm3; platelet count, 410,000/mm3; and WBC count,
168,000/mm3. The peripheral blood smear is depicted in the figure. Karyotypic analysis shows the Ph1 chromosome. The patient undergoes chemotherapy with imatinib mesylate (tyrosine kinase inhibitor). He remains in remission for 3 years and then begins to experience fatigue and an 8-kg weight loss. CBC shows hemoglobin, 10.5 g/dL; hematocrit, 30%; platelet count, 60,000/μL; and WBC count, 40,000/μL. Karyotypic analysis shows two Ph1 chromosomes and aneuploidy. Flow cytometric analysis of the peripheral blood shows CD19+,
CD10+, sIg−, and CD3− cells. Which of the following
complications of the initial disease did this patient develop
after therapy?
A Acute myeloblastic leukemia
B B-cell lymphoblastic leukemia
C Hairy cell leukemia
D Myelodysplastic syndrome
E Sézary syndrome

A

B B-cell lymphoblastic leukemia

CML

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258
Q

51 A 50-year-old man has had headache, dizziness, and
fatigue for the past 3 months. His friends have been commenting
about his increasingly ruddy complexion. He also has experienced
generalized and severe pruritus, particularly when
showering. He notes that his stools are dark. On physical examination,
he is afebrile, and his blood pressure is 165/95 mm Hg.
There is no hepatosplenomegaly or lymphadenopathy. A stool
sample is positive for occult blood. CBC shows hemoglobin,
22.3 g/dL; hematocrit, 67.1%; MCV, 94 μm3; platelet count,
453,000/mm3; and WBC count, 7800/mm3. What is the most
likely diagnosis?
A Chronic myelogenous leukemia
B Erythroleukemia
C Essential thrombocytosis
D Myelodysplastic syndrome
E Polycythemia vera

A

E

Polycythemia vera (PCV)

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259
Q

27 A 67-year-old man has had increasing weakness, fatigue, and weight loss over the past 5 months. He now has decreasing vision in both eyes and has headaches and dizziness. His hands are sensitive to cold. On physical examination, he has generalized lymphadenopathy and hepatosplenomegaly. Laboratory studies indicate a serum protein level of 15.5 g/dL and albumin concentration of 3.2 g/dL. A bone marrow biopsy is performed, and microscopic examination of the specimen
shows infiltration by numerous small plasmacytoid lymphoid cells with Russell bodies in the cytoplasm. Which of the following additional laboratory findings is most likely to be reported for this patient?
A Bence Jones proteinuria
B Hypercalcemia
C Karyotype with t(14;18) translocation
D Monoclonal IgM spike in serum
E WBC count of 255,000/mm3

A

D Monoclonal IgM spike in serum

Lymphoplamacytic lymphoma (waldenstrom macroglobulinema)

Hyperviscosity
Visual distrubances
Dizziness
Headache
Raynaud
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260
Q

48 A 63-year-old woman experiences a burning sensation
in her hands and feet. Two months ago, she had an episode
of swelling with tenderness in the right leg, followed by dyspnea
and right-sided chest pain. On physical examination, the
spleen and liver now appear to be enlarged. CBC shows hemoglobin,
13.3 g/dL; hematocrit, 40.1%; MCV, 91 μm3; platelet
count, 657,000/mm3; and WBC count, 17,400/mm3. The peripheral
blood smear shows abnormally large platelets. Which
of the following is the most likely diagnosis?
A Acute myelogenous leukemia
B Chronic myelogenous leukemia
C Essential thrombocytosis
D Myelofibrosis with myeloid metaplasia
E Polycythemia vera

A

C

Essential thrombocytosis

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261
Q

35 An 83-year-old man complains of worsening malaise
and fatigue over the past 5 months. On physical examination,
he is afebrile and normotensive. The spleen tip is palpable.
A CBC shows hemoglobin, 10.6 g/dL; hematocrit, 29.8%;
MCV, 92 μm3; platelet count, 95,000/mm3; and WBC count,
4900/mm3 with 63% segmented neutrophils, 7% bands, 2%
metamyelocytes, 1% myelocytes, 22% lymphocytes, 5% monocytes,
and 3 nucleated RBCs per 100 WBCs. The peripheral
blood smear shows occasional teardrop cells. An examination
of the bone marrow biopsy specimen and smear is most likely
to show which of the following findings?
A Erythroid hyperplasia
B Extensive fibrosis
C Fatty replacement
D Many megaloblasts
E Numerous myeloblasts

A

B

Myelofibrosis

Tear drop cells

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262
Q

52

A

A

Myeofibrosis

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263
Q

Waldenstrom Macroglobulinema

  • proliferation
  • associated with
  • dont have
A

Monoclonal proliferation of IgM

Assoc w/

  • Amyloidosis
  • Hyperviscosity

No lytic bone lesions

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264
Q

Myeloproliferative neoplasma

3 types

mutation

A

Monclonal proliferation of mature myeloid cells

Types

  • polycythemia vera
  • essential thrombocytosis
  • myelofibrosis

Mutation
- JAK2

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265
Q

JAK2 mutation in red cell precursor

Increased red cell mass
Decreased erythropoietin

A

Polycythemia vera

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266
Q 
Red face
Pruritis after hot shower
Splenomegaly hyperviscosity of blood
HA
Red hands and feet
A

Polycythemia vera

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267
Q

Increased platelets

Low thrombopoietin

A

Essential thrombocytosis

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268
Q

Fibrosis and olbiteration of marrow space

tear drop

A

Myelofibrosis

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269
Q

Monoclonal Ab spike

A

MM
MGUS
Waldenstrom macroglobulinemia

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270
Q

MM urine analysis

A

No protein in urine

Monoclonal Ab spike on UPEP

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271
Q

A patient presents with hypertension, hypokalemia, metabolic alkalosis and low plasma renin.

Diagnosis?
Treatment

A

Primary hyperaldosteronism (Conn syndrome)

Spironolactone

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272
Q

Stain for carcinoma

A

Cytokeratin stain

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273
Q

Epithelial vs mesenchymal tumors

A

Epithelial

  • adenoma
  • papilloma
  • carcinoma

Mesenchymal

  • sarcoma
  • oma
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274
Q

Liver metastasis

A

“Cancer Sometime Penetrates Benign Liver”

Colon
Stomach
Pancreas
Breast
Lung

Colon&raquo_space; stomach > pancreas

Elevated LFTs
Abdominal pain
Hepatomegaly
Ascites
Jaundice
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275
Q

Brain Metastasis

A

“Lots of Bad Stuff Kills Glia”

Lung
Breast
Skin (melanoma)
Kidney
GI tract

Lung > breast > melanoma, colon, kidney

HA
Neurological deficits
Cognitive function
Seizures

Well circumscribed tumors at grey/white matter junctions

276
Q

Bone Metastasis

A

“Permanently Relocated Tumors That Like Bone”

Prostate
Renal cell cancer
Testes
Thyroid
Lung 
Breast

Prostate, breast > lung, thyroid, kidney

277
Q

Hypercalcemia of malignancy

A

Squamous cell lung cancer

Secretes PTHrP

Head and neck squamous cancers
Breast cancer
Renal cell carcinoma

278
Q

Paraneoplastic of Small cell lung cancer

A

ACTH –> cushing syndrome

ADH –> SIADH
- hyponatremia

Lambert eaton syn

  • antibodies against presynaptic calcium channel at NMJ
  • weakness improve with use

Cerebellar degeneration

279
Q

Psammoma bodies

A

Lamellated concentric calcific spheruloes

Rings in a tree

PSaMMoMa bodies

  • Papillary thyroid cancer
  • Serous papillary cystadenocarcinoma of the ovary
  • Malignant mesothelioma
  • Meningioma
280
Q

What cancer is associated with Hashimoto thyroiditis

A

Marginal cell lymphoma

281
Q

To which main two molecules is iron bound in human cells?

To which two main molecules is iron bound in blood?

A

Human cells

  • Myoglobin
  • Ferritin

Blood

  • Hemoglobin
  • Transferrin
282
Q

Rb mutation

  • type of mutation
  • see in
  • sporadic vs inherited
  • Rb normal binds to
A

Tumor suppressor gene mutation

TWO mutations

Retinoblastoma
Osteosarcoma

White reflex in eye

60% sporadic (unilateral)
40% inherited (unilateral or bilateral)

E2F

283
Q

Mutation seen in more than 50% of human cancers

A

p53

causes cell cycle arrest normally

284
Q

Mutation in APC

  • type mutation
  • seen in
A

Tumor suppressor genes

Negative regulator of beta-catenin/WNT pathway

Familial adenomatous polyposis

Colon cancer

285
Q

BRCA mutation

- type of mutation

A

Tumor suppressor genes
- DNA repair protein

BReast CAncer (only 3% have this mutation)

Ovarian cancer

286
Q

DPC mutation

A

Tumor suppressor protein

Deleted in pancreatic cancer

287
Q

DCC mutation

A

Tumor suppressor protein

Deleted in colon cancer

288
Q

NF1 mutation

  • type of mutation
  • chromosome
  • presentation
A

Tumor suppressor protein
- Neurofibromin (Ras GTPase activating protein)

NF type 1

Neurofibromas, cafe au lait spots

Chr 17

289
Q

NF2 mutation

  • type of mutation
  • chromosome
  • presentation
A

Tumor suppressor protein
- Merlin (schwannomin) protein

NF type 2

Bilateral acoustic schwannoma

chr 22

290
Q

WT1 WT2 mutation on what chromosome

type of mutation

A

Tumor suppressor protein

chr 11

291
Q

Von Hippel Lindau mutation on what chromosome

type of mutation

A

Tumor suppressor protein

Chr 3

292
Q

Most common mutations

A

p53 and RAS

293
Q

RAS mutation

A

oncogene mutation

1 mutated allele

294
Q

K-RAS mutation

  • type
  • seen in
A

oncogene mutation

Colon
Lung
Pancreatic tumors

295
Q

H-Ras mutation

  • type
  • seen in
A

oncogene mutation

Bladder and kidney tumors

296
Q

N-RAS mutation

  • type
  • seen in
A

oncogene mutation

Melanomas
Hematologic malignancies
Follicular thyroid cancer

297
Q

BCR-ABL

  • type
  • normal fxn
  • what occurs
  • seen in
A

oncogene mutation
- tyrosine kinase

ABL codes for tyrosine kinase

ABL translocated from 9 to 22

Philadelphia chromosome

CML

298
Q

RET mutation

  • type mutation
  • associated with
A

oncogene mutation
- receptor tyrosine kinase

MEN 2A and 2B

  • Pheochromocytoma
  • Medullary thyroid cancer

Papillary carcinoma of thyroid

299
Q

HER2/neu mutation

  • type mutation
  • seen in
A

oncogene mutation
- receptor tyrosine kinase

ERBB2

Breast cancer
Ovarian cancer
Gastric cancer

300
Q

c-myc

  • type mutation
  • seen in
A

oncogene mutation
- transcription factor

burkitt lymphoma

301
Q

L-myc

A

Lung tumors

302
Q

N-myc

A

Neuroblastoma (adrenal medulla)

303
Q

What is the most common urea cycle disorder

A

Ornithien transcarbamylase deficiency

304
Q

Aflatoxins

A

Toxins produced by aspergillus mold

Causes mutation in p53

305
Q

Nitrosamines

  • found in
  • assoc with
A

Found in smoked foods

Assoc w/

  • Gastric cancer
  • Squamous cell esophageal cancer
306
Q

Arsenic causes

A

Lung cancer
Squamous cell skin cancer
angiosarcoma- Liver

307
Q

Vinyl chloride causes

A

angiosarcoma- liver

308
Q

Alkylating agents assoc with

A

Leukemia

Lymphoma

309
Q

Benzene assoc with

A

Leukemia

Hodgkin lymphoma

310
Q

Naphthalene causes

A

Transitional cell bladder cancer

311
Q

HHV-8

A

Kaposi sarcoma

312
Q

EBV associated iwth

A

Hodgkin lymphoma
(Reed-sternberg cells)

Burkitt lymphoma
(starry sky, 8;14)

Nasopharyngeal carcinoma

313
Q

Streptococcus bovis assoc with

A

Colon cancer

314
Q

Schistosoma haematobium assoc with

A

Squamous cell cancer of bladder

315
Q

Clonorchis sinensis assoc with

A

Biliary cancer

316
Q

Tuberous sclerosis associated with what neoplasms

A

Cardiac rhabdomyoma
Renal angiomyolipoma
Astrocytoma

317
Q

Ionizing radiation associated with what neoplasms

A

AML
CML
Papillary thyroid cancer

318
Q

What UV radiation most problematic

A

UVB

319
Q

What is the RL enzyme in purine synthesis?

RL enzyme in pyrimidine synthesis?

A

Purine: Glutamine PRPP amidotransferase

Pyrimidine: Carbamoyl phosphate synthase 2

320
Q

PSA tumor marker

A

Prostate cancer

321
Q

CA-125

A

Ovarian cancer

322
Q

Alkaline phosphatase

A

bone metastasis
Biliary disease
paget disease of bone

323
Q

alpha-fetoprotein

A

hepatocellular carcinoma

Testicular tumors

324
Q

CA 19-9

A

pancreatic cancer

325
Q

CEA

Calcitonin

Chromogranin

A

Colon
Pancreatic cancer

Medullary thyroid cancer
(alone and with MEN2A, MEN2B)

Neuroendocrine tumors

326
Q

S-100

A

Melanoma

Schwannoma

327
Q

Tartrate-resistant acid phosphatase (TRAP)

A

Hairy cell leukemia

328
Q

Which tumor marker would you use to monitor each?

  1. Hepatocellular carcinoma
  2. Ovarian cancer
  3. Pancreatic cancer
  4. Melanoma
  5. Colon cancer
A
  1. AFP
  2. CA-125
  3. CA 19-9, CEA
  4. S-100
  5. CEA
329
Q

RL enzymes for each?

  1. Urea cycle
  2. Hexose monophosphate pathway
  3. Fatty acid synthesis
  4. Beta-oxidation of fatty acids
  5. Ketone body synthesis
  6. Cholesterol synthesis
  7. Bile acid synthesis
  8. Heme synthesis
A
  1. Carbamoyl phosphate synthetase I
  2. Glucose 6 phosphate dehydrogenase
  3. Acetyl CoA carboxylase
  4. Carnitine acyltransferase
  5. HMG CoA synthase
  6. HMG CoA reductase
  7. 7-alpha hydroxylase
  8. Aminolevulinate synthase
330
Q

What are the irreversible enzymes involved in gluconeogenesis?

A

Pyruvate carboxylase (pyruvate –> oxaloacetate)

PEP carboxykinase
(oxaloacetate –> phosphoenolpyruvate)

Fructose 1,6 bisphosphatase
(Fructose 1,6- bisphosphate –> Fructose 6 phosphate)

Glucose 6 phosphate
(Glucose 6 phosphate –> glucose)

331
Q

Enveloped viruses spread

A

Droplets perientrally or sexually

332
Q

Non-enveloped viruses spread

A

Fecal oral spread

333
Q

Non-enveloped RNA viruses

A

“my Real Heavy Picture of California was Ruined without an Envelope”

Reovirus
Hepevirus
Picornavirus
Calicivirus

334
Q

Non-enveloped DNA viruses

A

“PaPa and Aunt Poly didnt have an Envelop”

Parvovirus
Papillomavirus
Adenovirus
Polyomavirus

335
Q

DNA viruses typically replicate

A

In the nucleus

336
Q

RNA viruses typically replicate

A

in the cytoplasm

337
Q

DNA viruses that replicate in the cytoplasm

A

Smallpox virus

Molluscum contagiosum

338
Q

RNA viruses that replicate in the nucleus

A

Influenza virus

Retroviruses

339
Q

Viral recombination where two viruses exchange entire segment

  • example
  • only what
A

Viral reassortment

RNA viruses only

Influenza virus

340
Q

When two different viruses infect same host a genes are exchanged from two different chromosomes

A

Viral recombination

341
Q

When two viruses infect a cell and one is non-functional so other virus makes a functional protein that serves both viruses

A

Viral Complementation

342
Q

When two viruses infect the same host and the progeny receive surface proteins from either parent

A

Phenotypic mixing

343
Q

Ploidy of viruses

A

All RNA and DNA viruses are haploid

Except: retroviruses (2) of ssRNA

344
Q

DNA viruses

  • examples
  • typical features
  • exceptions
A

HAPPy HiPPo Dung

Herpesvirus
Adenovirus
Parvovirus
Papillomavirus
Hepadnavirus
Polyomavirus
Poxvirus
DNA

Linear dsDNA
Icosahedral shape
Replicate in nucleus

Only parvoviridae (ssDNA)

Papillomarvirus (circular)
Polyomavirus (circular)
Hepadnavirus (circular)

345
Q

RNA viral genomes

A

(+) stranded RNA viruses

I went to a RETRO TOGA party, where I drank FLAvored CORONA and ate HIPPIE CALIfornia PICkles

Retrovirus
Togavirus
Flavivirus
Coronavirus
Hepevirus
Calicivirus
Picornavirus

All ssRNA except reoviridae (dsRNA)

346
Q

Negative-sense ssRNA

A

Not immediately translated

Can only be transcribed and not directly translated

Bring along viral transcriptase

Always Bring Polymerase Or Fail Replication

Arenaviruses
Bunyaviruses
Paramyxoviruses
Orthomyxoviruses
Filoviruses
Rhabdoviruses
347
Q

dsRNA brings along what

A

Polymerase

348
Q

Live attenuated vaccine

A

“Attention Teachers” Please Vaccinate Small Beautiful Young Infants with MMR Regularly

Adenovirus
Typhoid
Polio (Oral)
Varicella
Small pox
BCG
Yellow fever
Influena (intranasal)
MMR
Rotavirus
349
Q

Killed vaccine

A

RIP Always

Rabies
Influenza (injection)
Polio (injectable)
Hepatitis A

350
Q

Subunit vaccines

A

Use specific viral antigens to stimulate immune response

hepatitis B
HPV

351
Q

Egg allergy

A

Yellow fever
Influenza

(MMR)

352
Q

When should live virus vaccines be considered for HIV positive patients

A

All infants with HIV
- Rotavirus vaccine

CD4 count > 200

  • MMR
  • Varicella vaccine

Not recommended

  • Live virus influenza vaccine
  • Herpes zoster vaccine
353
Q

What condition is suggested by each of the following

  1. Signet ring cells
  2. Nutmeg liver
  3. RBC casts in urine
A
  1. Gastric adenocarcinoma, lobular carcinoma in situ of breast
  2. Right sided heart failure and budd-chiari
  3. Acute glomerulonephritis
354
Q

Infects spindle cells

A

HHV-8 Kaposi sarcoma associated herpesvirus (KSHV)

355
Q

Temporal lobe encephalitis

A

Herpes simplex virus 1 (HSV-1)

356
Q

Tzanck smear

A

Multinucleated giant cells positive

HSV-1, HSV-2, VZV

Low specificity
Low sensitivity

357
Q

Intranuclear eosinophilic inclusions

A

Type A Cowdry bodies

HSV, VZV, CMV

358
Q

EBV infects what cells

causes what

cell type seen

A

Infects B cells

Causes T cell response

Downey cells
- Foamy appearing basophilic cytoplasm

359
Q

Foamy appearing cells with basophilic cytoplasm

A

Downey cells

T cells

EBV

360
Q

Fever
Sorethroat
LAD of posterior cervical nodes
Splenomegaly

(+) monospot test

A

Infectious mononucleosis

EBV

361
Q

Ab against antigens found on horse, sheep and beef RBCs

A

Monospot test

Detects heterophiles

EBV

362
Q

Heterophile (-) mononucleosis

A

CMV infection

Acute HIV

363
Q

Owl eye nuclear inclusions

A

CMV

364
Q

Retinitis blind within days

A

CMV w/ AIDS

365
Q

HIgh fever (102) 3-5 days
Fever breaks
Maculopapular rash all over body
Seizures

A

Roseola
Sixth disease

Human Herpes virus -6

366
Q

What group of patients is prone to esopahgitis caused by CMV

A

HIV patients (CD4 < 50)

367
Q

Hypoglycmeia
Jaundice
Cirrhosis

A

Fructose intolerance

368
Q

Child with bright red rash on cheeks

A 
Slap cheek rash
Erythema infectiosum (fifth disease)

Parvovirus B19

369
Q

Upper respiratory infection
Then conjunctivitis
Diarrhea

A

Adenovirus

dsDNA virus

370
Q

Variola poxvirus vs vaccinia pox virus

A

Variola poxvirus
- smallpox

Vaccinia poxvirus
- cowpox

371
Q

Flesh colored dome shaped lesions with central dimple (umbilication)

A

Molluscum contagiosum

In immunocompetent will resolve in less than a year

372
Q

Demyelination of CNA due to destruction of oligodendrocytes

AIDS

Progressive

Visial symptoms

A

Progressive Multifocal leukoencephalopathy

JC virus
POlyomavirus

373
Q

Only Hepatitis virus dsDNA

A

Hepatitis B virus

374
Q

Hepatitis B virus is what type of virus

A

Hepadnavirus

375
Q

Hepatitis B virus replication

A

dsDNA virus

Partially double-stranded circular DNA enter nucleus

Host polymerase makes RNA intermediate from dNA

Viral reverse transcriptase makes DNA from RNA intermediate

376
Q

Hides in sensory ganglia of S2 S3

Hides in trigeminal ganglia

Hides in dorsal root ganglia

A

HSV-2

HSV-1

VZV

377
Q

Opacities seen on X ray on both sides of carina

A

Bilateral hilar lymphadenopathy

Sarcoidosis

378
Q

Common cold

A

Rhinovirus

Coronavirus

379
Q 
Severe diarrhea and vomiting
Winter months
Daycare center
Non-enveloped
dsRNA virus
A

Rotavirus

type of reovirus

380
Q

Western US/ canada
Hiker, Fisherman, camping
Flu- like

A

Coltivirus
(type of reovirus)

Self limiting

381
Q

Types of picornaviruses

A

Enterovirus

  • Poliovirus
  • Coxsackievirus
  • Echovirus
  • Hepatitis A virus

Rhinovirus

382
Q

Types of enteroviruses

A

Enterovirus falls under Picornaviruses

Spread through enteric system

4 types

  • Poliovirus
  • Coxsackievirus
  • Echovirus
  • Hepatitis A virus
383
Q

Poliovirus

A

Infects grey matter of the anterior horn of the spinal cord and motor neurons of the pons of the medulla

Causes paralysis

384
Q

Aseptic meningitis
Myocarditis
URIs

Summer

-Diagnosis?

A

Echovirus

PCR of CSF

385
Q

Hand foot and mouth disease

A

Coxsackievirus

386
Q

Most common cause of viral myocarditis in US

A

Coxsackievirus

Dilation of all chambers
Dyspnea
Chest pain
Fever
Arrhythmias
387
Q

Mouth blisters, fever

A

herpangina

Coxsackievirus

388
Q

Coxsackievirus can cause

A 
Myocarditis
Aseptic meningitis
Pericarditis
herpangina (blisters mouth and fever)
Hand, foot and mouth disease
Febrile pharngitis
389
Q

Picornavirus that is not an enterovirus

A

Rhinovirus

Common cold

390
Q

Vomiting and diarrhea

Cruise ship

A

Norovirus (caliciviruses)

Fecal oral route

391
Q

Types of flaviviruses

A

Yellow fever
Dengue fever
West nile virus
St. Louis Encephalitis virus

Most are arboviruses
(spread by arthropod)

392
Q 
High fever, HA
Epistaxis
Gum bleeding
petechial and purpura
Black vomit
Jaundice
Africa

Spread by?

A

Yellow Fever

Aedes mosquitoes

Liver damage

393
Q

Fever
Severe musculoskeletal pain
Retro-orbital headache

Spread by?
Can get?
Test?

A

Dengue fever

Mosquito

Hemorrhagic fever

Tourniquet test

  • field test for hemorrhagic fever
  • BP inflated leave 5 min
  • Excess petechiae
394
Q 
Headache
Malaise
Backpain
Myalgia
Anorexia
Alteration in consciousness
Neck stiffness
A

West Nile virus (WNV)

Mosquito

Serology for IgM anti-WNV antibodies

Tx supportive

395
Q

A 26-year-old woman has headaches for 4 weeks
along with increasing malaise. Physical examination yields
no remarkable findings. CT scan of the head shows no
abnormalities. A lumbar puncture yields clear, colorless CSF
with a normal opening pressure. Laboratory analysis of the
CSF shows a normal glucose concentration and a minimally
increased protein level. A few lymphocytes are present,
but there are no neutrophils. A Gram stain and India ink
preparation of the CSF are negative. Her condition gradually
improves over the next 6 months. Serum serologic tests are
most likely to show an elevated titer of antibodies to which
of the following infectious agents?
A Cryptococcus neoformans
B Echovirus
C Listeria monocytogenes
D Neisseria meningitidis
E Toxoplasma gondii

A

B. Echovirus

Acute lymphocytic meningitis

West nile
Echovirus

396
Q

Togaviruses

A

Rubella virus

397
Q

Fever Lymphadenopathy
Arthralgia
Macuopapular rash

A

Rubella or german measles

398
Q

Congenital rubella

A

Cardiac defects

  • PDA
  • Pulmonic stenosis

Cataracts
Deafness

399
Q

11 A 6-year-old girl has a blotchy, reddish-brown rash
on her face, trunk, and proximal extremities that developed
over the course of 3 days. On physical examination, she has
0.2-cm to 0.5-cm ulcerated lesions on the oral cavity mucosa
and generalized tender lymphadenopathy. A cough with
minimal sputum production becomes progressively worse
over the next 3 days. Which of the following viruses is most
likely to produce these findings?
A Epstein-Barr
B Mumps
C Rubella
D Rubeola
E Varicella zoster

A

D Rubeola

Measles (rubeola)

Rash
Koplik spots

Rubella: german measles, is a much milder infection than rubeola

400
Q

7 term infant has initial Apgar scores of 8 and 10 at 1
and 5 minutes. On auscultation of the chest, a heart murmur
is audible. There is hepatosplenomegaly. Cataracts of the crystalline
lens are noted. The infant is at the 30th percentile for
height and weight. Echocardiography shows a patent ductus
arteriosus. Which of the following events is the most likely
risk factor for the findings in this infant?
A Congenital rubella infection
B Dietary folate deficiency
C Dispermy at conception
D Erythroblastosis fetalis
E Maternal thalidomide use
F Paternal meiotic nondisjunction

A

A

Rubella in first trimester

401
Q

38 A family who recently emigrated from Romania
brings their 7-year-old child to the pediatrician
with complaints of conjunctivitis and periorbital
swelling. The child has had coughing
with a runny nose and high fever for 3 days.
Small lesions with blue-white centers are seen
in his oral cavity. Which of the following is the
most likely cause of this child’s symptoms?
(A) Diphtheria
(B) Pertussis
(C) Roseola
(D) Rubella
(E) Rubeola

A

E

Rubeola

402
Q

Retroviruses

A

Use reverse transcriptase

HIV
Human T cell Luekemia virus (HTLV)

403
Q

What is the number one cause of fatal infantile gastroenteritis

A

Rotavirus

404
Q

Posterior cervical adenopathy

A

EBV mononucleosis
Cat scratch disease (bartonella)
Acute otitis media

405
Q

Avian influenza

A

H5N1

Spread only from bird to human

60% mortality

Severe rapidly progressive couse

Fever cough myalgias
diarrhea, pancytopenia

Tx: Oseltamivir

406
Q

Swine flu

A

H1N1

Two swine strain
One human strain
One avian strain

Flu and GI symptoms

Tx: Oseltamivir or zanamivir

407
Q

Influenza virus

  • type of virus
  • description of virus
  • two key features
A

Orthomyxoviruses

Enveloped ssRNA

Hemagglutinin
- viral attachment to host cell
Neuraminidase
- release progeny virions

408
Q

Barking seal cough child

A

Croup

Parainfluenza virus
- enveloped

409
Q

Steeple sign

A

Croup

Parainfluenza virus
- enveloped

410
Q

Winter months viruses

A

Influenza
Rotavirus
RSV

411
Q

Summer months enterovirus

A

Poliovirus
Echovirus
Coxsackievirus

412
Q

Summer months arbovirus

A

West Nile virus

413
Q

Bronchiolitis
Child
Pneumonia in child

A

Infection of bronchioles

RSV

414
Q

Primary cause of penumonia in children

A

RSV

415
Q

RSV virus has what

A

Transmembrane protein called Fusion protein (F protein)

This allows infected cells to bond to near by uninfected cells

Multinucleated giant cells

416
Q

55 A 3-year-old boy has had a cough, headache, and slight
fever for 5 days. He is now having increasing respiratory difficulty.
On physical examination, his temperature is 37.8° C,
pulse is 81/min, respirations are 25/min, and blood pressure
is 90/55 mm Hg. On auscultation, there are inspiratory crackles,
but no dullness to percussion or tympany. Respiratory
syncytial virus is isolated from a sputum sample. Which of
the following chest radiographic patterns is most likely to be
present?
A Hilar lymphadenopathy
B Hyperinflation
C Interstitial infiltrates
D Lobar consolidation
E Pleural effusions
F Upper lobe cavitation

A

C Interstitial infiltrates

RSV

417
Q

Paramyxoviruses

A

RSV
Parainfluenza virus
Rubeola virus (measles)
Mumps

418
Q

Runny nose
cough
Conjunctivitis
Blue grey specks on buccal mucosa surround by base of red
Wide spread maculopapular rash that started on head and spread

A

Measles
Enveloped

Koplik spots

419
Q

Parotits
Orchitis
Meningitis

A

Mumps enveloped

Inflammation of parotid gland

420
Q

Viral meningitis

A

Coxsackievirus
Echovirus
Enterovirus
Mumps virus

421
Q 
Negri bodies
Fever
Nausea vomiting
Strange behavior
Hallucinations
Hydrophobia
Coma 
Death
A

Rabies virus
(Rhabdovirus)
Enveloped RNA

422
Q

Eosinophilic cytoplasmic inclusions that contain viral nucleocapsids

A

Negri bodies

Rabies

423
Q

Bullet shaped capsid

A

Rabies

424
Q

Fever
Progressive pulmonary edema
Respiratory failure

Exposure to mouse urine

A

Hantavirus

425
Q

A 27-year-old man dies of acute respiratory distress
syndrome 1 day after presenting to the
hospital with shortness of breath and a fever of
38° C (100.4° F). On the second hospital day,
he developed extreme pulmonary edema and
hypotension before he died. His family says
that he had recently gone hiking and caving in
an area known to be heavily populated with rodents.
Which of the following is the most likely
cause of death?
(A) Dengue virus
(B) Ebola virus
(C) Hantavirus
(D) Marburg virus
(E) Rhabdovirus

A

C. Hantavirus

426
Q

Illnesses spread through animal urine

A

Hantavirus
Lassa virus
LCM virus
Leptospirosis

427
Q 
Fever HA myalgias
Followed by vomiting and diarrhea
Multiorgan failure
Shock 
Death
A

Ebola virus

428
Q

A patient present with fever, vomiting and diarrhea. She was in Sierra Leone two weeks visiting family. What viral infection? Type of isolation

A

Ebola

Contact and droplet precautions

429
Q

Viral proteins of HIV

A

Gp120

Gp41

430
Q

Gp120

A

Grabs onto CD4 surface receptor on T cells and macrophages

Once connected, changes shape and allows it to bind to coreceptor (CCR5 or CXCR4)

431
Q

Tropsim

A

Which coreceptor HIV uses to enter cell

only CXCR4: X4 viruses
Only CCR5: R5 viruses
Both : dual tropism

432
Q

GP41

A

Fusion and entry into host cells

433
Q

Three main structural genes in HIV genoma

A

Gag: encodes p24 capsid protein

Env: gp120 and gp41

Pol: codes for reverse transcriptase

434
Q

Diagnosis of HIV check

A

P24 antigen adn HIV antibodies

Viral load (PCR test)

435
Q

AIDS diagnosis

A

CD4 < 200
< 14% lymphocytes are Cd4

AIDs defining condition
- Pneumocystis jirovecii pneumonia

436
Q

Ring enhancing lesions

A

HIV associated Primary CNS lymphoma
(solitary lesion)

Toxoplasmosis
(multiple lesions)

437
Q

HIV < 200 opportunistic infections

A

Pneumocystis jirovecii pneumonia

438
Q

HIV < 100 opportunistic infections

A

Histoplasmosis capsulatum

Candida albicans

439
Q

HIV < 50 opportunistic infections

A

Mycobacterium tuberculosis

Mycobacterium avium complex

CMV retinitis

440
Q

Hepatosplenomegaly
Fever
Cough
CD4 < 150

A

Histoplasmosis capsulatum

441
Q

Chronic watery diarrhea
HIV patient

diagnosis

A

Cryptosporidium spp. (c.parvum)

Oocytes on modified acid-fast stain of stool turns bright pink or red

442
Q

HIV patient covered in what look likes cherry angiomas

A

Bacillary angiomatosis

Bartonella henselae

443
Q

What four molds are considered dermatophytes (fungal species that invade superficial skin)

A

Trichophyton
Microsporum
Epidermophyton
Malassezia furfur

444
Q

Diaper rash

Satellite lesions

A

Candida albicans

Shiny skin or eroded

445
Q

Blood culture

Germ tubes have small projections on side of cell

A

Candida albicans

446
Q

Narrow based budding yeast

A

Cryptococcal neoformans

447
Q

India ink

Clear circle

A

Cryptococcal neoformatns

448
Q

Soap bubble lesion on imaging

A

Cryptococcal meningoencephalitis

449
Q

Silver stain

Ground glass appearance of interstital infiltrates

A

Pneumocystis jirovecii (PCP)

450
Q

Narrow septate hyphae that branch at acute angles (45 degrees)

A

Aspergillus fumigatus

451
Q

Broad irregularly shaped, nonseptate hyphae branching at right angles (90 degrees)

A

Mucor and Rhizopus

452
Q 
Facial pain 
HA
Large necrotic lesion, black escar
Blindness
CN deficits
A

Rhinocerebral mucomycosis

453
Q

Most common type of dermatophytes with animal reservoir

A

Microsporium (dog or cat)

454
Q

Hypopigmented or hyperpigmented lesion on body
Scaling plaque
Yeast

A

Tinea versicolor

Malassezia furfur

455
Q

KOH prep spaghetti and meatball appearance

A

Tinea versicolor

Malassezia furfur

456
Q

What is associated iwth each clue

  1. Cat scratch
  2. Cat bite
  3. Cat feces
  4. Puppy feces
  5. Animal urine
A
  1. Bartonella henselae
  2. Pasteurella multocida
  3. Toxoplasma
  4. Yersinia enterocolitica, campylobacter jejuni
  5. Leptospira, hantavirus
457
Q

Leptin on hypothalamus

A

Inhibits lateral area and stimulates ventromedial area of hypothalamus

= satiety

458
Q

Organisms associated with birds

A 
Histoplasma capsulatum
Cryptococcus neoformans
Chlamydophilia psittaci
H5N1 influenza
West Nile virus
459
Q

Mississippi ohio river valley
Bird and bat droppings
Spelunkers

  • Imaging
  • what happens to it
A

Histoplasma capsulatum

Enters through lung
Engulfed by macrophages

Hyphae and spores on imaging

Macrophage filled w/ 100 spores

460
Q

Mississippi and ohio river basins

Thick refractile wall around yeast

broad based budding

A

Blastomyces dermatitidis

Granulomatous nodules

461
Q

Southwestern U.S
Mexico

Pneumonia

Spherule
-filled with endospores

A

Coccidioides immitis

462
Q

A 46-year-old man from northern Mexico has had fever,
nonproductive cough, and weight loss for 2 months. On examination
his temperature is 37.5 ° C. A chest radiograph shows
a miliary pattern of small nodules in all lung fields. Bronchoalveolar
lavage is performed and microscopic examination of
the fluid shows organisms averaging 50 microns in diameter
with thick walls and filled with endospores. Which of the following
infections is he most likely to have?
A Blastomycosis
B Coccidioidomycosis
C Histoplasmosis
D Mycobacteriosis
E Nocardiosis
F Paracoccidioidomycosis

A

B. Coccidioidomycosis

463
Q

Pneumonia
Granulomatos changes in mucous membranes

Captain wheel

Latin america

A

Paracoccidioides brasiliensis

Paracocci parasails with a captains wheel all the way to Latin america

464
Q

Gardner
Dimorphic fungus

Pustule on puncture site

Nodules follow draining lymphatics

Tx

A

Sporothrix schenckii

Tx: Itraconazole (first line)
potassium iodine

465
Q

Mold form contains barrel shaped arthroconidia

A

Coccidioides immitis

466
Q

Associated iwth dust storms

A

Coccidioides immitis

467
Q

Multiple budding of yeast form

A

Paracoccidioides brasillensis

468
Q

Councilman bodies

A

Apoptotic liver cells

Viral hepatits
yellow fever

469
Q

Acute fatty foul smelling diarrhea
Abdominal bloating

  • Diagnosis
    Tx
A

Giardia lamblia

Pear-shaped trophozoites or cysts in stool

Metronidazole
Tinidazole

470
Q

Pear-shaped trophozoites

A

Giardia lamblia

471
Q

Bloody diarrhea
Flask shaped ulcers
RUQ pain

  • type organism
  • transmittered
  • diagnosis
  • tx
A

Entamoeba histolytica

Liver abscesses

Fecal-oral route
- eating food or water contaminated with feces that contain cysts

Trophozoites or cysts in stool

Treatment

  • trophozoites= metronidazole or tinidazole
  • cysts= iodoquinol or paromomycin
472
Q

Foul smelling vaginal discharge
Motile

-transmission
- wet mount
- vaginal pH
- tx
-

A

Trichomonas vaginalis

Sexual transmission

Pear shaped appearance

Increase vaginal pH

Metronidazole

473
Q

Ring enhancing lesions in brain

A

Toxoplasma gondii

474
Q

Chorioretinitis
Hydrocephalus
Intracranial calcifications

A

Toxoplasma gondii

475
Q

Toxoplasma gondii acquired how

A

Cat feces or litter

Eating cyst in infected meat

476
Q

Rapidly fatal meningoencephaltiis

Swimming

A

Naegleria fowleri

Swimming in freshwater lakes

Enters nose passes through cribriform plate

477
Q

A 40-year-old man goes on a camping vacation
with his family. One day after swimming in a
freshwater lake near the camp site, he develops
nausea and vomiting and starts to behave irrationally.
His family takes him to the emergency
department, where blood samples are taken
and a spinal tap is performed. He is diagnosed
with a rapidly progressing meningoencephalitis
and dies shortly thereafter. Which of the following
protozoa was most likely the cause of
the man’s illness?
(A) Cryptosporidium species
(B) Entamoeba histolytica
(C) Leishmania donovani
(D) Naegleria fowleri
(E) Plasmodium falciparum

A

(D) Naegleria fowleri

478
Q

55 A 9-year-old child who is living in a mud hut in Paraguay
has a sore persisting on her face for 4 days. Physical examination
shows an indurated area of erythema and swelling just
lateral to the left eye, accompanied by posterior cervical lymphadenopathy.
She has unilateral painless edema of the palpebrae
and periocular tissues. Two days later, she has malaise, fever,
anorexia, and edema of the face and lower extremities. On
physical examination 1 week later, there is hepatosplenomegaly
and generalized lymphadenopathy. Which of the following
pathologic findings is most likely to develop in this patient?
A Cerebral abscesses
B Chronic arthritis
C Dilated cardiomyopathy
D Meningitis
E Mucocutaneous ulcers
F Paranasal bony destruction

A

C Dilated cardiomyopathy

Trypanosoma cruzi

479
Q

Trypanosoma brucei spp

A

African sleeping sickness

tsetse fly

Fever
Enlarged LN
Sleepiness

Encephaltiis
Coma and death

Blood smear: long whispy organisms in blood

Early: Suramin
CNS involved: Melarsoprol

480
Q

Dilated cardiomyopathy
Megesophagus
Megacolon

A

Trypanosoma cruzi

Chagas disease

Reduviid bug

  • painless bit
  • Feces scrated into skin

Benznidazole
Nifurtimox

481
Q

Spiking fevers
Hepatosplenomegaly
Pancytopenia

  • transmission
  • diagnosis
  • tx
A

Leishmania donovani

Visceral leishmaniasis

Sandfly

Amastigotes inside macrophages

Liposomal amphotericin B

482
Q

Ulcerating papules that are slow to heal

A

Leishmania donovani
- Cutaneous leishmaniasis

Sandyfly

Sodium stibogluconate

483
Q

Cyclic fever
Headache
Anemia
Splenomegaly

A

Malaria
Plasmodium species

Mosquito

484
Q

Malaria fever due to

A

RBCs rupture and release merozoites

485
Q

Malaria that fever spikes every 48 hrs

Every 72 hrs

A

48 hr

  • P. ovale
  • P. vivax

72 hr
- P. malariae

486
Q

Malaria

  • subtype
  • tx
  • Diagnosis
A

Plasmodium species

P. vivax and P. ovale
- dormant infection in liver
Tx primaquine

P. falciparum
- most severe
- no dormant form
- sequestration of RBCs --> occlusion of small vessels
Tx: Chloroquine

Blood smear

  • Merozoites
  • Trophozoite= diamond ring
  • Gametocytes: banana shaped
487
Q

Two ring forms inside one erythrocyte

A

Trophozoite form

Babesia Microti

488
Q

Maltese cross

A

inside RBC
merozoites

Babesia Microti

489
Q

Babesia Microti

  • symptoms
  • blood smear
  • transmission
  • tx
A

Fever
Hemolytic anemia

Two rings inside RBC
Maltese cross

Ixodes tick
coinfection w/ lyme disease

Tx
Quinine + Clindamycin

490
Q

Names of following stages of malaria life cycle

  1. Looks like diamond ring
  2. Ruptures the cell host
  3. Replicating intracellularly
  4. Form injected from the anopheles mosquito
  5. banana shaped
A
  1. Early trophozoite
  2. Merozoite
  3. Schizont
  4. Sporozoite
  5. Gametocyte
491
Q

Ingest helminths

A

“EAT”

Enterobius vermicularis
Ascaris lumbricoides
Trichinella spiralis

492
Q

Penetrating helminths

A

“Sand”

Stronglyoides
Ancylostoma
Necator
D

493
Q

Worm lays eggs at anus

tx

A

Enterobius vermicularis
Nematode (round worm)

Benzimidazoles
- Albendazole
- mebendazole
Pyrantel pamoate

494
Q

Giant round worm

Eosinophilic pneumonitis

  • diagnosis
  • tx
A

Ascaris lumbricoides

Ingest eggs
Hatch in intestines
Release larvae penetrate bowel --> lungs
Migrate up trachea and swallowed
Mature worms in intestine

Eggs released in stool

Benzimidazoles
- Albendazole
- mebendazole
Pyrantel pamoate

495
Q

Myositis
Fever
Eosinophilia
Periorbital edema

  • acquired
  • diagnosis
  • tx
A

Trichinella spiralis

Undercooked meat

GI –> muscles

Muscle biopsy: cysts with larvae in muscle tissues

Bendzimidazoles

496
Q

A 28-year-old woman from rural Guyana with a history
of rheumatoid arthritis develops painful swelling of her
hands and feet. She is treated with corticosteroid therapy.
A month later, she develops profuse, watery diarrhea along
with fever and cough. On examination, she has a temperature
of 37.3° C. Laboratory studies show WBC count, 12,900/
mm3; and the WBC differential count shows 57% segmented
neutrophils, 5% bands, 16% lymphocytes, 8% monocytes,
and 14% eosinophils. Microscopic examination of a stool
specimen shows ova and small rhabditoid larvae. Similar
larvae are present in a sputum specimen. Which of the following
infectious diseases is most likely to produce these
findings?
A Cysticercosis
B Onchocerciasis
C Schistosomiasis
D Strongyloidiasis
E Trichinosis

A

D. Strongyloidiasis

497
Q

Rhabditiform larvae in feces

tx

A

Strongyloides
Threadworm

Ivermectin
Albendazole

498
Q

Wound on feet
Anemia
Abdominal discomfort

tx

A

Ancylostoma
Necator

Hook worms

Benzimidazoles
- Albendazole
- mebendazole
Pyrantel pamoate

499
Q

Taenia solium

  • transmission
  • symptoms
  • tx
A

Cestodes (tapoworms)

Ingestion of alrvae in undercooked port
- GI tract infection by adult worm

Ingestion of eggs in contaminated food or water
- brain filled w/ cysts

Tx
Albendazole
Praziquentel
Dexamethaone: keep CNS symptoms from getting worse

500
Q

Cestodes

A

Tape worms

Taenia solium
Diphyllobothrium latum
Echinococcus granulosus

501
Q

Brain filled with cysts

Seizures

A

Taenia solium

Ingestion of eggs in contaminated food

502
Q

Giant limbs

  • type
  • transmitted
  • tx
A

Wuchereria bancrofti

Helminth, neomatoid
Round worm in GI

Mosquitoes

Migrate to lymphatics

Elephantiasis of limbs
Hydrocele of scrotum

Tx
Diethylcarbamazine

503
Q

Raw fish
Megaloblastic anemia

-def
tx

A

Diphyllobothrium latum

B12 deficiency

Praziquantel

504
Q

Hydatid cyst lesion in liver
RUQ pain
Well circumscribed circular lesion

  • transmitted
  • can find where in body
  • symptom
  • tx
A

Echinococcus granulosus

dog feces

Form mature larval cysts in liver and lungs

Anaphylaxis

Surgical removal of cysts

505
Q

A 23-year-old man presents to the physician
with abdominal distention and tenderness with
no vomiting or diarrhea. Physical examination
shows hepatosplenomegaly. Bowel sounds are
normal. On questioning, the patient says that
he traveled to eastern South America 1 year
ago. Several weeks after returning from his trip,
he remembers having fever, diarrhea, weight
loss, and “funny looking stools.” Ultrasonography
shows ascites and hepatic periportal fi brosis.
Which of the following is most likely
responsible for this patient’s present symptoms?
(A) Appendicitis
(B) Bowel obstruction
(C) Enterocolitis
(D) Portal hypertension
(E) Ruptured viscus

A

D. Portal hypertension

Schistosoma mansoni

506
Q

A 29-year-old man has had hematuria for the past
month. On physical examination, he is afebrile. There is diffuse
lower abdominal tenderness, but no palpable masses. An
abdominal radiograph shows a small bladder outlined by a
rim of calcification. Cystoscopy is performed, and the entire
bladder mucosa is erythematous and granular. Biopsy samples
are taken. Which of the following histologic findings is
most likely to be seen in these samples?
A Acid-fast bacilli of Mycobacterium avium complex
B Eggs of Schistosoma haematobium
C Larvae of Trichinella spiralis
D Migrating Ascaris lumbricoides
E Taenia solium cysts

A

B. Eggs of schistosoma haematobium

507
Q

Deals with snails

A

Schistosoma

508
Q

Portal HTN
Splenomegaly
Egyptian immigrant

tx

A

Schistosoma
- blood fluke

Praziquantel

509
Q

Chronic bronchitis
Hemoptysis

acquired
-t x

A

Paragonimus westermani

Adult fluke in lung

Undercooked crab meat

Praziquantel

510
Q

Pigmented gallstones
Cholangiocarcinoma

  • transmission
  • lives
  • Tx
A

Clonorchis sinensis
- liver fluke

undercooked fish

Lives in biliary tract

Praziquantel
Albendazole

511
Q

Swimmers itch

A

Schistosoma

512
Q

Fluke associated iwth hematuria and bladder cancer

A

Schistosoma haematobium

513
Q

Burns all over the body what will occur over the 7 day?

A

Increased metabolic rate

Not

  • Increased extracellular volume
  • Not increase in serum cholesterol concentration
  • Not loss of B vitamins in skin
514
Q

Testicular tumor with friend egg appearance

A

Seminoma

Germ cell tumor

515
Q

Umbilical cord compression of infant, monitoring shows a normal fetal heart rate of 150/ min with variable spontaneous decelerations to 110/min. Why the decrease in fetal heart rate

A

Increased fetal systemic vascular resistance

Compressing vessel, decreases radius increase in resistance

Cause reflex bradycardia

516
Q

20 yr history of T1D uses insulin

why hypoglycemia

A

Impaired release of glucagon

517
Q

Subscapularis does what

A

internal rotation

518
Q

Parathyroid effects on parts of kidney

A

Increase reabsorption of Ca in distal convoluted tubule

Decreased reabsorption of phosphate in proximal convoluted tubule

Increase 1,25 OH2 D3 production by stimulating kidney in 1alpha-hydroxylase in PCT

519
Q

Transfusion reactions

A

Type II hypersensitivity

520
Q

Bone conduction > Air conduction

A

Conductive hearing loss

Otosclerossi

521
Q

Pregnancy, pituitary enlargement

A

Lactotroph hyperplasia due to estrogen increase

522
Q

Lupus

  • type of rxn
  • deficiency of
A

type II hypersensitivity

C1q, C4, C2

523
Q

Genetic component to hypertrophic obstructive cardiomyopathy

A

Mutations in sarcometric proteins

  • myosin binding protein C
  • beta-myosin heavy chains
524
Q

Papilledema

increased

A

HTN

Increased angiotensin

525
Q

Primary adrenal insufficiency labs

A

Hyponatremia
Hyperkalemia
Metabolic acidosis

Hyperkalemia leads to K entering all cells (via H/K exchanger in exchange for H exiting cells)

526
Q

Fixed split S2

Systolic murmur at left sternal border

A

Atrial septal defect

527
Q

Panic attack what will the labs show

A

Hyperventilating
Decrease PCO2

Respiratory alkalosis

528
Q

Bioavailability on graph

A

area under the curve

529
Q

Inject 5% saline what changes

A

Hypertonic into extracellular space

water from intracellular into extracellular

Decrease IC vol
Increase IC osm
Increase EC vol
Inc osm vol

530
Q

Hypotonic saline

Isotonic saline

Hypertonic saline

A

.45% saline= hypo

.9%= isotonic

3%= hypertonic

531
Q

Explains impaired action potential in axons of people with MS

A

Increased axonal capacitance

532
Q

Prostate cancer spread to

by means of

A

Spine

Prostatic venous plexus to vertebral venous plexus

533
Q

Mechanical ventilation on

  • peak inspiratory alveolar
  • end-tidal alveolar
  • peak inspiratory intrapleural
  • end-tidal intrapleursal
A

Positive on all

534
Q

Pap smear columnar cells seen where?

A

Cervical canal

Doesn’t reach endometirum

535
Q

Histology of female parts

A

Labia major= SS keratinized
Labia minor= SS nonkeratinized

Vagina= ss nonkeratinized
Ectocervix= ss nonkeratinized

Endocervix= simple columnar

Body uterus= simple columnar

536
Q

Fluoxetine affects neurons arising from

A

SSRI

From Raphe nucleus

537
Q

NE is from

A

Locus cereuleus

538
Q

GABA from

A

Nucleus accumbens

539
Q

Dopamine from

A

Ventral tegmentum SNc

540
Q

Acetylcholine from

A

Basal nucleus of Meynert

541
Q

Erythematous scaly rings with central clearing

From pet

A

Tinea corporis

542
Q

Leukomoid vs CML

A

Leukomoid
- high alkaline phosphate (LAP)

CML
- low leukocyte alkaline phospahtase

543
Q

Strawberry hemangioma

A

Benign capillary hemangioma of infancty

544
Q

Renal artery aneurysn due to

A

Fibromuscular dysplasia

545
Q

Silicosis, asbestosis, coal workers lung

FEV1/FVC
FVC
Diffusion capacity of lung for carbon monoxide

A

Interstitial lung diseases

FEV1/FVC: normal

FVC: Decreased

Diffusing capacity: Decreased
(short swallow breaths)

546
Q

White plaque on side of tongue

what happens if dont tx

A

Hairy leukoplakia

EBV

Progress into submucosa

547
Q

Black escar nose

A

Mucor

548
Q

Ixodes deer tick

A

Borrelia burgdorferi
Anaplasma
Babesia

549
Q

Part of eye with least impact on vision

A

Iris

550
Q 
Fever
Diarrhea
Splenomegaly
Muscle wasting
Pancytopenia
A

Bitten sand fly

Leishmania spp

551
Q

What shows up right after a burn with a blister

A

Histamine

552
Q

Phototherapy does what

A

Increased conversion of bilirubin to water soluble isomers

553
Q

18 month old failure to thrive and frequent wet diapers. 3rd percentile for length and weight. Physical examination shows dehydration, decreased muscle tone, and bowing of legs. Slit lamp examination shows crystals in corneas. Hypophosphatemia, hypokalemia, nonanion gap metabolic acidosis. pH of 5, glucosuria, phorphaturia, and generalized aminoaciduria. Cuase of metabolic acidosis in this patient

A

Decreased sodium bicarbonate reabsorption in the proximal tubule

554
Q

Bulimia Nervosa

  • potassium
  • chloride
  • bicarbonate
  • acidosis/ alkalosis
A

K
- decreased

Chloride
- increased

Bicarbonate
- decreased

Alkalosis

555
Q

New born 2 weeks old, yellow skin for 7 days. At birth, lethargic and hypoglycemia. Vomits at feedings. PE shows jaundice. There is no glucosuria but reducing substances are detected in the urine. Direct bilirubin concentration is increased. An assay for what will give a diagnosis

A

Galactose-1-phosphate uridyltransferase

556
Q

Normoblast

Megakaryocyte

A

Normoblst: production of RBC

Megakaryocyte: production of platelet

557
Q

Leukotrienes do what

A

LTC4
LTD4
LTE4

Increase bronchial tone

LBT4
- increase neutrophil chemotaxis

558
Q

Prostacyclin do what

A

Decrease platelet aggregation

Decrease vascular tone

559
Q

Prostaglandins do what

A

Decrease vascular tone

Increase uterine tone

560
Q

Thromboxane do what

A

Increase platelet aggregation

Increased vascular tone

561
Q

Small cell lung cancer
- secretes

tx

A

SIADH

Demeclocycline

562
Q

Split S1. First component of sound

A

Closure of mitral valve

563
Q

Cerebellar hemisphere lesion on right see deficits on

A

Right

564
Q

Lateral eye movement

A

CN VI

Pons

565
Q

Stellate gangion stimulation

A

heart rate

566
Q

Chronic renal failure

  • Prosphate
  • Ca
  • PTH
A

Phosphate increase

Ca decrease

Parathyroid increase

567
Q

Crash crash
Fractures and lacerations

Tachypnea, tachycardia and cyanosis

Diffuse consolidation of both lungs

what is seen on autopsy

A

Hyaline membranes

acute respiratory distress

568
Q

Leukocyte differential

A

WBC

no T cell or B cell

569
Q

How are integral membranes anchored

A

Extensive hydrophobic interactions between the amino acid side chain of the protein and the membrane phospholipid tails

570
Q

Bronze diabetes

A

Hemochromatosis

HFE gene mutation chr 6
HLA-A3

Increased ferritin
Increased iron
Decreased TIBC
Increased transferrin saturation

571
Q

Microcytic anemia types

A

Thalassemia
Anemia of chornic disease
Iron deficiency
Lead poisoning

TAIL

572
Q

Microcytic anemia

Asain

A

Alpha thalassemia

573
Q

Most common lung cancer

A

adenocarcinoma

574
Q

Disease form bed bugs

A

Staph aureus

575
Q

Erectile dysfunction nerve

A

Cavernous n.
Inferior hypogastric form
pelvic splanchnic

576
Q

Sperm missing tails

A

Primary ciliary dyskinesia (PCD)

AR

Bronchiectasis another symptoms
Situs inversus

577
Q

E coli makes many proteins how

A

mRNA contains sequence for many proteins

578
Q

Scratches
Vetarianian
Parrot

Tender regional lymphadenopathy

A

Bartonella henselae

579
Q

Bartonella henselae can cause what in immunocompromised

A

Bacillary angiomatosis

Red-purple papular skin lesions

580
Q

Pulmonary embolism does what to pH, PaO2, PaCO2, Serum HCO3

A

pH increase
PaO2 decrease
PaCO2 decrease
Serum HCO3 Normal

PE occludes parenchyma resulting in increased dead space ventilation

Hyperventilation –> hypocapnia and respiratory alkalosis

Acute so no bicarb involvement yet

581
Q

Fever myalgias, malaise, and progressive fatigue over 2 week period

No sore throat

Splenomegaly

No Lad or jaundice

Fails to agglutinate horse erythrocytes

A

Mono

(normally EBV0

but since no agglutination
CMV

582
Q

Regulates glucose homeostasis during prolonged fasting by altering transcription of many key enzymes

A 
Cortisol (cytoplasm)
Growth hormone ( membrane-bound)
583
Q

Splenomegaly on histology

A

Red pulp expansion

584
Q

Additional thing to check if alkalosis

A

Urine Cl

  • checks vomiting
  • checks diuretic
585
Q

Noscomal infections

A

Coag neg staph
Staph aureus
Enterococci
Candida

586
Q

Increase in use of what has increased staph noscomal infections

A

Intravascular catheters

587
Q

Strongest factor for risk of completed suicide

A

Past suicide attempt

588
Q

Arthritis
Drug for treat causes
Mouth ulcers
Elevates AST ALT

A

Methotrexate

589
Q

Most infections that arise after chemotherapy are from

A

Patients own endogenous bacterial flora

590
Q

Tetralogy of fallot squatting does what

A

Ventricular septal defect
Overriding aorta

Increases systemic vascular resistance

Increase SVR causes a higher proportion of right ventricular output into pulmonary circulation rather than into left ventricle

591
Q

Foot drop
Loss sensation on dorsum of foot

Nerve compressed where

A

Fibular neck

592
Q

Mycoplasma and anemia

A

Cross rxn of IgM antibodies to red blood cells, activate complement and cause erythrocyte lysis

Cold agglutinins

Hemolytic anemia

IgM titers fall with infection resolution

593
Q

Brain natriuretic peptide levels secreted from

A

Ventricular myocytes

Due to increased ventricular wall stress

594
Q

5 y.o to ER iwth 2 days of dark, low-volume urine and decreased energy. Had abdominal pain, fever and bloody diarrhea after eatting hamburgers 3 days ago. No edmea or rashes. Anemia, thormocytopenia and elevated BUN and creatine.

See elevated waht

A

HUS from E.coli

Shigella toxin

Decease in hemoglobin and haptoglobin

Increase lactate dehydrogenase and unconjugated bilirubin levels

595
Q

Bacteria virulence factor simlar to tropomyosin and myosin.

Fxn

A

M protein

Resist phagocytosis

Group A streptococcus

  • S pyogenes
  • cross rxn causes rheumatic carditis
596
Q

What contributes to hypocalcemia in patient with chronic renal disease

A

Hyperphosphatemia

Phosphate binds free calcium and precipitates in soft tissue

Triggers fibroblast growth factor, which lowers phospahte levels by inhibiting 1 alpha hydroxylsae

Decreases production of 1,25 hydroxyvitamin D

597
Q

Decreased pulmonary capillary wedge pressure

A

Right sided heart failure

Impaired blood flow from right ventricle to left side of heart

598
Q

Drugs and renal failure

A

Acute interstital nephritis

599
Q

Delayed puberty

Anosmia

A

Kallmann syndrome

Failure of GnRH secreting neurons to migrate from origin in olfactory placode to normal anatomic location in hypothalamus

Mutation KAL-1 gene

May have cleft lip or palate

600
Q

Antibodies to phospholipase A2 (PLA2R)

A

Membranous nephropathy

601
Q

Magnesium salts and aluminum hydroxide together for antiacid

A

Magnesium causes diarrhea

Aluminum hydroxide causes constipation

Both temporarily increase the gastric pH by neutralizing hydrochloric acid relieving reflux

602
Q

Common source of hemorrhage from penetrating gastric ulcers

A

Right and left gastric arteries that run along lesser curvature of stomach

603
Q

What if taking during pregnancy can cause oligohydramnios

A

ACE inhibitors and ARBs

604
Q

Pleural effusion

  • due to pressure changes
  • due to inflammation
A

Pressure changes

  • transudative
  • heart failure
  • cirrhosis
  • nephrotic syndrome

Inflammation
- Exudative
- increase vascular membrane permeability
(infection, malignancy, rheumatic disease)
- fluid to serum ratio of protein > 0.5

605
Q

3 week old
Abdominal distension
vomiting
blood streaked stools

Had normal stool and urine

X ray shows thin curvilinear areas of lucency that parallel the bowel of the lumen

A

Pneumatosis intestinalis (air in the bowel wall)

Necrotizing enterocolitis (NEC)

606
Q

Man with behavioral changes
Lack of focus and poor memory
Involuntary jerky movements

A

Huntington’s disease

CAG trinucleotide repeats

GOF mutation

Abnormal protein build up causes loss of inhibitory (GABA) neurons

Atrophy of caudate nucleus

607
Q

Hiatal hernia caued by

A

Circumferential laxity of the phrenoesophageal membrane

608
Q

IgA dn C3 deposition in vascular lesions of thigh

A

Henoch-Schonlein purpura (HSP)

609
Q

Saddle nose and oliguria

CXR?

A

Granulomatosis with polyangiitis (Wegeners)

Large nodular densities

610
Q

Which testicular tumor is associated with each?

1) Composed of cytotrophoblasts and syncytiotrophoblasts?
2) May present initially with gynecomastia
3) Elevated AFP
4) Elevated beta-hCG
5) Histologic appearance similar to koilocytes (cytoplasmic clearing)

A
  1. Choriocarcinoma
  2. Leydig or sertoli cell tumor
  3. yolk sac tumor
  4. choricocarcinoma, embryonal carcinoma
  5. Seminoma
611
Q

Which ovarian tumor matches each?

1) Estrogen secreting leading to precocious puberty
2) Produces AFP
3) Psammoma bodies
4) Testosterone secreting leading to virilization
5) Lined with fallopian tube like epithelium
6) Ovarian tumor + ascites + hydrothorax
7) Call-Exner bodies
8) Elevated beta-hCG
9) Resembles bladder epithelium

A

1) Granulosa theca cell tumor
2) Yolk-sac tumor
3) Serous cystadenocarcinoma
4) Sertoli-leydig cell tumor
5) Serous cystadenoma
6) Ovarian fibroma (meigs syn)
7) Granulosa cell tumors
8) Choriocarcinoma, dysgerminoma
9) Brenner tumor

612
Q

Which embryologic structure of the heart gives rise to each of the following?

1) Ascending aorta and pulmonary trunk
2) Coronary sinus
3) Superior vena cava

A

1) Truncus arteriosus
2) Left horn of sinus venosus
3) Right common cardinal vein and right anterior cardinal vein

613
Q

What is the antidote for

1) Iron
2) Lead
3) cyanide
4) Methomoglobin
5) Carbon monoxide
6) Methanol or ethylene glycol

A

1) Deferoxamine
2) EDTA , succimer, dimercaprol
3) Nitraites, hydroxycobalamin, thiosulfate
4) Methylene blue, vit C
5) 100% oxygen hyperbaric oxygen
6) Fomepizole, ethanol, hemodialysis

614
Q

Wound healing after 1 week

A

Fibroblasts

Type III collagen replaced by type I collagen

615
Q

Exudative vs transudate

  • Looks like
  • Lab
  • Due to
A

Exudate

  • Cellular (cloudy)
  • Increased protein > 2.9 , increased LDH (vs serum)
  • Lymphatic obstruction, inflammation, infection, malignancy

Transudate

  • Hypopcellular (clear)
  • Decrease protein < 2.5, decreased LDH vs serum
  • Increased hydrostatic pressure (HF, Na retention)
  • Decreased oncotic pressure (cirrhosis, nephrotic syndrome)
616
Q

Hematogenous spread of carcinomas

A

Four Carcinomas Route Hematogenously

Follicular thyroid carcinoma
Choriocarcinoma
Renal cell carcinoma
Hepatocellular carcinoma

617
Q

Low Vd

  • compartment
  • drug types
A

Intravascular

Large/ charged molecules
Plasma protein bound

618
Q

High Vd

  • compartment
  • drug types
A

All tissues including fat

Small lipophilic molecules, especially if bound to tissue protein

619
Q

Clearance (CL)=

A

Rate of elimination of drug/ plasma drug concentration

= Vd x Ke (elimination constant)

Ke= 0.7/t(1/2)

CL= (0.7 x Vd)/ t(1/2)

620
Q

Half life (t1/2) =

A
  1. 7 x Vd/ CL
  2. 3 half lives = 90% steady state
% remaining
1 half life= 50%
2= 25%
3= 12.5%
4= 6.25%
621
Q

Loading dose=

A

(Cp x Vd)/ F

F= bioavailability
IV F= 100%

Cp= target plasma conc at steady state

622
Q

Maintenance dose=

A

(Cp x CL x tau)/ F

tau= dosage interval (time between doses, if not administered continuously, in hours)

Maintenance dose for 1 day= 24 hrs

623
Q

Therapeutic index=

A

TD50/ ED50

Median toxic dose/ median effective dose

The higher the TI the safer the drug

624
Q

Drugs with low Therapeutic index

A

Warning! These Drugs are Lethal

Warfarin
Theophylline
Digoxin
Lithium

625
Q

Nicotinic ACh receptors are

Muscarinic ACh receptors are

A

Nicotinic ACh receptors are ligand gated Na/K channels

Muscarinic ACh receptors are G protein coupled receptors through second messengers

626
Q

Alpha 1 fxn (3)

A

Increase vascular smooth muscle contraction
Increase pupillary dilator muscle contraction (mydriasis, dilation)
Increase intestinal and bladder sphincter muscle contraction

627
Q

Alpha 2 fxn (5)

A 
Decrease sympathetic (adrenergic) outflow
Decrease insulin release
Decrease lipolysis
Increase platelet aggregation
Decrease aqueous humor production
628
Q

Beta 1 fxn (4)

A

Increase HR
Increase contractility (one heart)
Increase renin release
Increase lipolysis

629
Q

Beta 2 fxn ( 7)

A 
Vasodilation
Bronchodilation (two lungs)
Increase lipolysis
Increase insulin release
Decrease uterine tone **
Ciliary muscle relaxation
Increase aqueous humor production
630
Q

M1 fxn

M2 fxn

M3 Fxn

A

M1: mediates higher cognitive functions, stimulates enteric nervous system

M2: Decrease HR and contractility of atria

M3: Increase exocrine gland secretions, increase gut peristalsis, increase bladder contraction, bronchoconstriction, increase pupillary sphincter muscle contraction (miosis)

631
Q

Conotruncal abnormalities associated with failure of

A

Neural crest cells to migrate:

Transposition of great vessels
Tetralogy of fallot
Persistent truncus arteriosus

632
Q

Baby takes first breathe

A

Decrease resistance in pulmonary vasculature

Increase pressure in left atrium

633
Q

Preload

  • is what
  • approximated by
  • also measures
  • decreased by
A

Pressure at end of diastole (relaxation) when ventricles are the fullest right before contraction.

Approximated by ventricular End-diastolic volume

Ventricles fill from atria, atrial pressure= preload

Decreased by venous vasodilators

634
Q

Afterload

  • approximated by
  • decreased by
A

Approximated by Mean arterial pressure
Total peripheral resistance (TPR)

Decreased by arterial vasodilators

635
Q

EDV =

A

Right atrial pressure

636
Q

S1 heart sound

S2 heart sound

A

S1= mitral and tricuspid closing

S2= aortic and pulmonary closing

637
Q

Beside maneuver

1) Inspiration
2) Hand grip
3) Valsalva, standing up
4) Squatting

A

1) Inspiration
- increases venous return to right atrium
- Increase Intensity of right heart sounds

2) Hand grip
- Increase after load
- Increase intensity of MR, AR, and VSD
- Decrease AS murmur

3) Valsalva, standing up
- Decrease preload
- Decrease intensity of most murmurs
- Increase intensity of hypertropic cardiomyopathy

4) Squatting
- Increase venous return, increase preload, increase afterload
- Increase intensity of AS, MR, VSD

638
Q

Holosystolic murmur

A

Tricuspid regurg

Mitral regurg

639
Q

Slurred curved upstroke (delta wave) between P and R

A

Wolff parkinson white

Abnormal fast accessory conduction pathway from atria to ventricle (bundle of kent) bypassing AV node

May result in SVT

640
Q

Origination site of atrial fibrillation

A

Pulmonary vein ostia

641
Q

Atrial flutter caused by

A

Large reentrant circuit that transverses the cavotricuspid isthmus (region of R atrial tissue between IVC and tricuspid valve annulus)

642
Q

Atrial natriuretic peptide released due to

A

Increase in blood volume and atrial pressure

acts as cGMP

Vasodilation and Decreased Na reabsorption

643
Q

Pulmonary capillary wedge pressure approximation of

A

left atrial pressure

644
Q

Rheumatic fever

  • valve
  • tx
A

Early: mitral regurg
Late: mitral stenosis

Penicillin

645
Q

Innumerable renal microaneurysms and spasms on arteriogram

Fever, WL, Headache
Abdominal pain
HTN

A

Polyarteritis nodosa

646
Q

Drugs that cause pulmonary fibrosis

A

Amiodarone
Bleomycin
Busulfan

647
Q

Drugs taht cause flushing

A

Vancomycin
Niacin
Adenosine
Dihydropyridine Ca channel blockers

648
Q

Drugs that cause Torsades de pointes

A 
Potassium channel blockers 
Na channel blockers
Macrolides
Haloperidol
Chloroquine
Protease inhibitors
649
Q

Drugs that cause gingival hyperplasia

A

Phenytoin

Verapamil

650
Q

GI bleeding

Buccal pigmentation

A

Peutz jegher syndrome

651
Q

Tuberous Sclerosis

A

HAMARTOMASS

Hamartomas in CNS and skin
Angiofibromas
Mitral regurg
Ash lead spots
Cardiac rhabdomyoma
Tuberous sclerosis
autosomal dOminant
Mental retardation
renal Angiomyolipoma
Seizures
Shagreen patches
652
Q

78 y.o to office with one month history of progressive dyspnea, generalized weakness, fatigue and palpitations. He reports tingling and numbness in both lower limbs. Hes not been taking care of himself since wife died. CV exam shows displaced apical impulse at 6th intercostal space, a third heart sound, and high volume carotid pulses. Pedal edema, mild hepatomegaly. Normal blood counts. What deficiency?

A. Ascorbic acid
B. Cobalamin
C. Niacin
D. Pyridoxine
E. Retinol
F. Riboflavin
G. Thiamine
A

G. Thiamine

Wet beriberi

  • peripheral neuropathy
  • heart failure
653
Q

Person dies after 5 days from heart failure due to

A

Free wall rupture –> Profound hypotension and shock

654
Q

History of mitral valve prolapse. Temp of 100. Mid systolic click systolic murmur at apex. Gram positive bacteria that synthesizes dextrans from glucose. Adherence of bacteria would be where?

A. Circulating heparins
B. Endothelial surface glycoproteins
C. Fibrin platelet aggregates
D. Subendothelial collagen
E. Subendothelial glycosaminoglycans
A

C. Fibrin platelet aggregates

Virdians streptococci. Produce dextrans which facilitate adherence to fibrin.

Fibrin and platelets are deposited at sites of endothelial truma

655
Q

What is similar in parameters between systemic and pulmonary circulation

A. Arterial oxygen content
B. Arterial resistance
C. Blood flow per minutes
D. Diastolic arterial pressure
E. Driving pressure for blood flow
F. Mean arterial pressure
A

C. Blood flow per minute

656
Q

Superior gluteal and inferior gluteal come off

A

Internal iliac

also obturator comes off

657
Q

Chronic anemia on cardiac output and venous return curves

A

Increase in cardiac output

Somewhat increase in venous return

658
Q

Anaplylaxis effect on cardiac otuput and venous return curves

A

Widespread venous and arteriolar dilation

Drop in venous return (shifted down and leftward)

659
Q

1 week old infant
Discharged at 30 hrs
PE harsh, holosystolic murmur at left mid to lower sternal border

No murmur heard at birth

A

Non-cyanotic possibilities

  • Atrial septal defect
  • VSD
  • PDA
  • Coarctation of aorta

Patients presents w/ VSD
- loud blowing holosystolic murmur

660
Q

What chemical substance help decrease thrombus propagation by inhibiting platelet aggregation?

A

Prostacyclin

661
Q

Normal cardiac pressures

1) RA
2) RV
3) Pulmonary artery
4) Pulmonary artery wedge
5) LA
6) LV
7) Aortia

A

1) RA= < 5
2) RV= 25/5
3) Pulmonary artery= 25/10
4) Pulmonary artery wedge= < 12 (10)
5) LA= < 12 (10)
6) LV= 130/10
7) Aorta= 130/90

662
Q

Atrial myxoma on histology

A

Amorphous extracellular matrix

With scattered stellate or globular myxomas cells within abundant mucopolysaccharide (myxoid) ground substance containing chondroitin sulfate and hyaluronic acid

663
Q

Common cardinal veins form

A

Superior vena cava

664
Q

Disorder in which no finding can be found to support symptoms but recently had trauma or stress in life

A

Conversion disorder

665
Q

Cachectin caused by

A

TNF-alpha
IL-1
IL-6

666
Q

Liver mass that is a cystic lesion

Hepatomegaly

  • microscopic examination
  • complication of treatment
A

Echinococcus granulosus

Hydatid cysts

Sheep/ dog expsoure

Eggshell calcifications

Caution with removal –> spilling of cyst contents can cause anaphylactic shock

667
Q

Blistering in sun

Itchy

A

Porphyria cutaneous tardia

Uroporphyringoen decarboxylase

668
Q

Painful genital lesion, irregular ragged borders

Grey excudatecovering

Enlarged inguinal LN

A

Chancroid`

669
Q

Fatigue
Early satiety
Mucosal pallor with no scleral icterus. Lungs clear. Mild hepatomegaly. Can feel tip of spleen. Pancytopenia. Peripheral blood= immature granulocytes, nucleated erythrocytes and teardrop cells. BM aspiration is attempted but yields no marrow

  • disease
  • MOA
  • Secretes
  • Symptoms
  • Histopathylogical features
A

Primary myelofibrosis

Chronic myeloproliferative disorder with clonal megakaryocytes

Secrete TGF-beta –> fibroblasts to produce collagen and BM fibrosis

–> Extramedullary hematopoiesis

Massive splenomegaly
Tear drop cells
Dry tap

Large islands of hematopoietic progenitor cells

670
Q

Spleen histopathyology

1) Accumulation of macrophages with fibrillary cytoplasm
2) Diffuse neutrophilic infiltration and follicular necrosis
3) Dilated sinusoids and fibrosis nodules with hemosiderin

A

1) Gaucher disease
- AR lysosomal storage disease
- fibrils of glucocerebroside
(Crumped tissue paper)

2) Acute infection of spleen
Follicular necrosis= infection group A strep

3) Congestive splenomegaly due to portal HTN

671
Q

Dermatomyositis

  • biopsy shows
  • can affect
A

Proximal muscle weakness
Rash on eyelids

Affects striated muscles and skin

Oropharynx and upper esophagus

Perimysial inflammatory infiltrates and atrophy involving the fibers around the periphery of muscle fascicles (perifascicular atrophy)

672
Q

Eosinophilic esophagitis presents as

A

Dysphagia
Epigastric pain
Recurrent esophageal relfux
Food impaction

673
Q

NE leak into arm causes

Tx

A

Alpha 1 receptor mediated vasoconstriction which can lead to tissue necrosis

Tx Phentolamine (alpha receptor blocker)

674
Q

Thyroid Fine needle aspiration biopsy

Clusters of cells with large overlapping nuclei containing sparse, finely dispersed chromatin

Numerous intranuclear inclusion bodies and grooves are seen

A

Papillary carcinoma

Overlapping nuclei containing finely dispersed chromatic (empty or ground glass appearance= orphan annie eyes)

675
Q 
Unusually muscle movements of thumb
Hypoplastic mandible
Low set ears
Bifid uvula
Cleft palate
A

Physical features of DiGeorge syndrome

Hypocalcemia

676
Q

Heme synthesis occurs where

A

Mitochondria

677
Q

New born with drug addict mother. Irritable, crying, tremors, sneezing and diarrhea

Tx

A

Opioid withdrawal
- Neonatal abstinence syndrome

Morphine
Methadone

678
Q

COPD lung volumes

A

Air trapping and hyperinflation

Higher functional residual capacity
Higher reserve volume

Total lung capacity increases as well but to a lesser extent

High RV/ TLC ratio

Diffusing capacity of lung for carbon monoxide is decreased
- due to emphysematous destruction of the alveolar arterial membrane

FEV1 decreased
FVC decreased
FEV1/FVC decreased

679
Q

Loss of internal elastic lamina

A

Fibromuscular dysplasia

Luminal stenosis alternating with areas of dilation

Resistant HTN
Stroke
Ruptured aneurysm

Renal artery stenosis
Cerebrovascular involvment

680
Q

Other form of downs syndrome

A

Trisomy 21

46, XX t(14;21)
Robertsonian translocatoin

681
Q

HTN
Suppressed Renin

Na?
K?
Bicarb

A

Primary aldosteronism

High Na
Low K
Low Bicarb

682
Q

Sudden cardiac death due to

A

Ventricular fibrillation

- Lack of tissue perfusion due to blockage

683
Q

Horseshoe kidney associated iwth

A

Turner

684
Q

Schistocytes associated with

A

HUS, TTP, DIC and AORTIC STENOSIS

685
Q

Structure gives rise to heart structure

1) Smooth parts of left and right ventricles
2) Smooth part of right atrium
3) Trabeculated left and right atria
4) Trabeculated parts of the left and right ventricles

A

1) Smooth parts of left and right ventricles
- Bulbus cordis

2) Smooth part of right atrium
- Right horn of sinus venosus

3) Trabeculated left and right atria
- Primitive atria

4) Trabeculated parts of the left and right ventricles
- Primitive ventricle

AM- STEP1 (20 decks)

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