Immunology and Resp

Question 1

When is DTaP vaccine contraindicated?

Is seizure when receving last vaccine a contracintidation?

Answer 1

ALL kids should get 5 doses of DTaP at ages.
2,4,6 months, 15-18 months and 4-6 years.

Previous seizure when receiving an earlier dose is self limited and IS NOT a contraindication.

The contraindications are:

1. ANaphylaxis following DTAP (patients should not get future dose)
2. Unstable neuro disorders like an existing history of uncontrolled epilepsy
3. Encephalopathy (coma, decreased level of onsciousness, prolonged seizures) within a week of DTaP admin are all contraindications to future doses.

Question 2

When should you give prophylactic antibiotics to people exposed to Pertussis?

Answer 2

ANYONE exposed to pertussis within the last 21 days.

if after that.. then dont give… it wont be effective.

Question 3

Severe Combined ImmunoDeficiency

Answer 3

Gene defect causing absence of leading to FAILURE of T CELL DEVELOPMENT.

B CELL DYSFUNCTION due to absent T cells.

X-linked recessive or autosomal recessive. (can have either inheritance pattern).

Recurrent SEVERE viral, fungal, or Opportunistic infections.

Failure to thrive (low weight and height at birth)

Chronic Diarrhea.

Treatment = STEM CELL TRANSPLANT

ALL IMMUNOGLOBULINS WILL BE LOW (IgG, IgA, IgM) and all cell counts (CD4 and CD8) will be low.

Question 4

What are the sequale of absent T cells vs absent B cells?

Answer 4

Absent T cells = defects in CELLULAR immunity –> increased risk of Viral, Fungal and Opportunistic pathogens (P. Jirovecii).

Absent B cells = Humoral immunity dysfunction –> increased risk of SINOPULMONARY and GI BACTERIAL infections.

Question 5

Bruton (x-linked) AGAMMAGLOBULINEMIA

Answer 5

Characterized by ABSENT B CELLS –> LOW serum IMMUNOGLOBULINS and NORMAL T CELL concentrations.

Absent B cells = Recurrent SINOPULMONARY and GI infections.

Question 6

Chronic Granulomatous Disease

Answer 6

Defect of the NADPH oxidase in Phagocytic cells –> Impaired killing of CATALASE-POSITIVE organisms (S. Aureus, Serratia Marcescens).

Affected patients have Recurrent SKIN and SOFT TISSUE infections but NORMAL LYMPHOCYTE and IMMUNOGLOBULIN concentrations

Question 7

Common Variable Immunodeficiency (CVID)

Answer 7

HYPOGAMAGLOBULINEMIA (not agammaglobulinemia like in brutons).

This is a very mild form of brutons.

Patients will have less severe infections and it will present at a later age in life with NORMAL T and B cell counts.

Patients will have Recurrent sinopulmonary or GI infections due to the HYPOgammaglobulinemia.

Diagnosis - QUANTATATIVE MEASUREMENT OF IMMUNOGLOBULIN LEVELS (Significantly reduced serum IgG, IgA and or IgM)

Question 8

Wiskott-Aldrich Syndrome

Answer 8

x-linked recessive disease.

Presents in EARLY INFANCY with ECZEMA and BLEEDING (post circumcision, bleeding from umblical stump when cutting cord) due to THROMBOCYTOPENIA.

Tcell dysfunction and HYPOgammaglobulienia worsen with age and present s BACTERIAL, VIRAL ND opportunistic INFECTIONS.

Question 9

What vaccines are recommended for north africa or developing countries?

Answer 9

Hep A, Hep B, Typhoid and Polio Booster.

Question 10

if you see a pleural effusion in a patient.. and on paracentesis you find pus what is the diagnosis and what do you do?

Answer 10

PUS on Paracentesis = EMPYEMA

Must DRAIN with chest tube and also give ABX

Question 11

Exudative effusions labs

Answer 11

Pleural fluid protein / serum protein ratio >.5

Pleural fluid LDH / serum LDH ratio >.6

Plueral fluid LDH >2/3 of upper limit of normal for serum LDH

Question 12

Adenosine deaminase deficency

Answer 12

an autosomal recessive form of severe combined immunodeficiency –> leads to deficient formation of Mature B and T lymphocytes –> sever infections and failure to thrive.

Question 13

Leukocyte Adhesion Deficiency

Answer 13

Defective integrins on the leukocyte surface, which normally allow neutrophils to adhere to vascular endothelium, exit the vasculature, and migrate to areas of infection or inflammation.

Lack of Neutrophil migration –> Recurrent SKIN (cellulitis, abscess, omphalitis) and MUCOSAL (periodontal) infections as well as POOR WOUND HEALING.

CHaracteristic sign –> Inflammation WITHOUT PURULENCE (neutrophils cannot get there to make pus)

Will see very elevated neutrophils.. but neutrophils dont make it to site of infection

Question 14

recurrent skin and soft tissue abscesses with a variety of catalse positive organisims suggests what diagnosis?

Answer 14

Chronic Granulomatous Disease (CGD)

Test with Dihydrorhodamine 123 test or Nitroblue Tetrasolium Testing and CONFIRM with Gene testing.

Patients with CGD should receive antimicrobial prophylaxis with - TMP SMX and ITRACONAZOLE!

Question 15

Chediak-Higashi

Answer 15

characterized by partial OCULOCUTANEOUS ALBINISM and RECURRENT CUTANEOUS INFECTIONS.

Question 16

DiGeorge SYndrome (22q11.2 deletion)

Answer 16

Dysmorphic facies
developmental delays
HYPOCalcemia
and Decreased T cell concentration and function (due to THYMIC HYPOPLASIA)

Question 17

When does angioedema from ACE-Inhibitors occur?

Answer 17

ANY time.. doesnt have to be in first few weeks of starting the med.. cna occur decades later while ur on it.

First step –> CHeck for airway compromise –> if present give EPINEPHRINE.

If epinephrine doesnt correct the airway obstruction –> EMERGENT TRACHEOSTOMY.

Obviously stop the ACE -I

Question 18

Hereditary Angioedema - C1 inhibitor deficency .. what are the symptoms?

Answer 18

Characterized by Recurrent Edema without associated PURITUS or UTRICARIA.

the AUTSOMAL DOMINANT disorder is typically caused by a DEFICENCY in or dysfunction of C1 INHIBITOR –> leads to ELEVATED BRADYKYNIN –> EDEMA.

usually presents in late childhood after a DENTAL procedure, STRESS or TRAUMA.

The FACE, Limbs, and Gentialia are most commonly affected.

BOWEL WALL edema –> presents as colicky abdominal pain, vomiting and diarrhea.

The most threatening complications is LARYNGOSPASM –> airway obstruction.

Confirm diagnosis with COMPLEMENT TESTING –> the elevated C1 (due to lack of c1 inhibitor) results in excess cleavage of C4 and thus DEPRESSED LEVELS OF C4!!

Treatment –> Bradykinin antagonist (ICATIBANT).

Question 19

how do you make the diagnosis of wegners (GRanulomatosis with Polyangitis)

Answer 19

Diagnosis is made with Anitneutrophil cytoplasmic antibodies and definitive diagnosis is with TISSUE BIOPSY.

Treatment = High Dose Corticosteroids as well as Cyclophosphamide or Rituximab.

Presents with
Upper Resp tract (sinusitis, otitis, saddle nose deformity)
Lower Resp tract ( Lung Nodules / cavitations)
Renal (RPGN)
Skin (Livedo reticularis , non healing ulcers)

C-ANCA (PR3, MPO) positive

Question 20

What are the 2 types of contact dermatitis?

Answer 20

Allergic and Irritant

Allergic = Type 4 hypersensitivity –> metals, rubber / lates

Irritant = Physical or chemical irritation –> soaps / detergents / chemicals / cacid

Question 21

what immunoglobulin findings are in Hyper IgM syndrome

Answer 21

Normal B cells
DECREASED IgG and IgA

ELEVATED IgM.

Caused by an x-linked defect in the CD40 LIGAND.

Normally CD40 is expressed on B cells –> induces a change in B cell production of IgM to other immunoglobuoins (Class Switching).

Without CD40 there Is NO class switching and thus everything stays as IgM.

Recurrent sinopulmonary infections with ENCAPSULATED bacteria –> otitis media, pneumonia, sinusitis.

Question 22

What is the treatment for wiskott aldrich and SCID?

Answer 22

Hematopoietic stem cell transplantation.

Question 23

Which Immunodeficiency is associated with Atopiy (eczcema / asthma) and anaphylaxis during transfusions?

Answer 23

Selective IgA deficency.

Low or absent IgA
Normal IgG, IgM and Bcells

Recurrent sinopulmonary and GI infections

Treatment - Supportive care. Medical alert bracelet for transfusion reactions

Question 24

Which Immunodeficiency is associated with Atopy (eczcema / asthma) and anaphylaxis during transfusions?

Answer 24

Selective IgA deficency.

Low or absent IgA
Normal IgG, IgM and Bcells

Recurrent sinopulmonary and GI infections

Treatment - Supportive care. Medical alert bracelet for transfusion reactions

Question 25

When should premature infants receive vaccines?

Answer 25

Same schedule as if they were born at term.

Do not delay.

Vaccines are given based on CHRONOLOGICAL AGE (age since birth.. not corrected)