Disorder of Immunity II

Question 0

HIGH YEILD

Mikulicz Disease vs. Mikulicz Syndrome

Answer 0

anything involving the lacrimal/salivary glands. Whatever causes problem of the glands leads to enlargement of the gland.

The syndrome means the glands are enlarged.

Question 1

Describe the symptoms dealing with Sjogren Syndrome

Answer 1

Involvement of the lacrimal and salivary glands. Patients present with xerostomia (dry mouth), keratoconjunctivitis (dry eyes), rheumatoid factor may be seen in 75% of patients. Patients will have chipmunk appearance.

Question 2

What is the most common cause of death for Lupus patients

Answer 2

renal failure and intercurrent infections

Question 3

What is essential for the diagnosis of Sjogren Syndrome

Answer 3

biopsy of the lip

Question 4

What cell is causing the problem in fibrosis?

Answer 4

Fibroblast are the bad cells because they are the ones that makes collagen

Question 5

Scleroderma is also known as? Is it an acute or chronic disease? What causes this disease? What is it characterized by?

Answer 5

Systemic Sclerosis
Chronic
Etiology unknown
Characterized by abnormal accumulation of fibrosis in skin and multiple organs

Question 6

What are the two major categories of Systemic Sclerosis? How do you tell them apart?

Answer 6

Diffuse & Limited which are associated with the CREST syndrome
The prognosis is different between the two, and 2 separate antibodies are associated with them

Question 7

Involvement of the heart, kidney, and lungs with scar tissue is associated with which form of Scleroderma?

Answer 7

Diffuse bka SCL 70

Local - is limited to where it is in the body and has a better prognosis than diffuse

Question 8

CREST stands for

Answer 8

C = Calcinosis - calcium deposits in the body 
R = Raynaud's phenomenon - fingers and toes get cold, ischemic, brought on by cold waterr, gets vasospasms of fingers and toes 
E = Esophageal dismotility - problems swallowing 
S = Sclerodactyly - claw hand deformity 
T = Telangiectasia - small dilated little blood vessels

Question 9

An older women around the age of 56 complains skin is tight and shiny with no hair follicles. The patient has malignant hypertension, respiratory difficulties, arrhythmias and reflux in the GI tract. What does this patient have

Answer 9

Diffuse form of Scleroderma

Question 10

Primary Immunodeficiencies is commonly seen in?

Answer 10

Infants and children. Normally congenital or genetic

The infant will have recurrent infections because they do not have the defense to fight that particular infection

Question 11

Secondary will be acquired and commonly seen in?

Answer 11

Adults for ex: HIV

Question 12

An 8th mouth baby boy has recurrent bacterial infections such as gastritis, pneumonia etc.. Test results showed no antibodies present in the blood. What is the name of this disorder? Is it primary or secondary immunodeficiency?

Answer 12

It is primary.

X-Linked Bruton Agammaglobulinemia

Question 13

What is the underling problem in X-Linked Bruton Agammaglobulinemia?

Answer 13

Failure of pre B-cells to differentiate into B-cells

B-cell problem only
T-cells are okay so the boy can fight viral infections
ONLY seen in boys

Question 14

What is the most common of ALL the primary immunodeficiency disorders

Answer 14

IgA deficiency - European descent, rare in blacks and asians
due to the failure of selective IgA B-cells to differentiate into plasma cells.

Most patients are asymptomatic

Question 15

CVI (Common Variable Immunodeficiency) is due to? Onset occurs? The patients will have an increase in which type of disorders? Will antibodies be present in the patients blood? How is it different from X-Linked Bruton?

Answer 15

B-cells fail to mature to plasma cells
Onset occurs in childhood or adolescence
Increase in autoimmune disorders
Antibodies will be present, but at low amount
CVI is not an X-linked so it can occur in male and female. Some antibodies are present unlike in Bruton

Question 16

In DiGeorge Syndrome which pharyngeal pouches are undeveloped?

Answer 16

3rd and 4th

Question 17

Thymic hypoplasia is the same thing as

Answer 17

DiGeorge

Question 18

A patient with tetany has hypocalcemis, recurrent viral infections and fungal infections, and abnormal facial features. What is the immunodeficiency

Answer 18

DiGeorge
deficiency on T-cells
no thymus

Question 19

A male patient has poor response to polysaccharide antigen due to antibody problem. T-cells deficient, male with viral infections, bleeding, and skin rash. What disorder is associated with these symptoms?

Answer 19

Wiscott Aldrich Syndrome

Question 20

Accumulation of toxic products in T and B cells –> defective lymphocytic function. What is the name of the defective enzyme.

Answer 20

ADA deficiency

Adenosine DeAminase

Question 21

Defect in humoral and T-cell mediated immunity is associated with? What is the treatment

Answer 21

SCID
Sever Combined Immunodeficiency Disease

bone marrow transplant

Question 22

What is the infective portion of the AIDs virus and which cells do it mainly affects?

Answer 22

gp120
CD4+ Tcells
normal ratio CD4 : CD8 = 2:1

abnormal ratio = 1:8
CD8 will out number the CD4 cells due to AIDS

Question 23

Cryptococcal is the most common fungal infection. What is its vector?

Answer 23

Pigeon droppings

Question 24

In the oral cavity how do you differentiate between oral thrush (oral candidiasis) and leukoplakia

Answer 24

Oral thrush also referred to as Oral Candidiasis is commonly seen with people with AIDs. White spots in the oral cavity that can be rubbed off

Leukoplakia cannot be rubbed off and it is not associated with AIDs

Question 25

Kaposi’s Sarcoma has to deal with? KNOW 4 Boards —-> What is it associated with?

Answer 25

homosexual/ bisexual males
It is associated with Herpes type 8

KS has to deal with skin lesions, can involve the liver, stomach, mmeninges,

Question 26

In dealing with AIDS what test is used to monitor for disease?

Answer 26

VIRAL LORD!!

Question 27

In diagnosing Amyloidsis what stain is used?

Answer 27

Congo Red staining it will be pink due to the beta-pleated sheets
Apple green birefringence on polarization***** NBQ [National board question]

Question 28

Describe the difference between primary and secondary types of amyloid

Answer 28

AL - Primary - amyloid of light chains. Kappa and Lambda

AA- Secondary - associated with chronic infections like Tb or rheumatoid arthritis

Question 29

What are some of the clinical features dealing with Amyloidosis

Answer 29

Macroglossia - enlargement on the tongue
heart - congestive heart failure
kidney - nephrotic syndrome
GI tract- malabsorption