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What deficiency will a pt have in Chronic granulomatous disease?
Inherited mutations in one of the 4 components of the NADPH oxidase complex
What test can screen for Inherited mutations in one of the 4 components of the NADPH oxidase complex?
NBT Test
- Decreased Respiratory Burst

-These patients are susceptible to respiratory infections with fungal (most notably invasive aspergillosis) and bacterial agents

-NOT more susceptible to viral infxns--> PMNs and Mɸs are not effective, thus viral defenses not affected
Chronic Granulomatous Dz
Oppurtunistic Fungal Infections (2)
Aspergillosis

Zygomycosis
Fungal species that causes "Sick Building Syndrome"
Aspergillus sp.
Patients most likely to get opportunistic fungal infections have:
PMNs and Mɸs that are not effective
Identify factors that predispose pt to LRT opportunistic fungal infections
-HIV and other immunocompromised patients

-Transplant patients

-Cancer Patients
What type of T cell helps overcome fungal infection?
Predominately Th1 helps overcome infxn
Innate defenses spores encounter when inhaled:
Phagocytes

Mucociliary defense***

Collectins
just know this
For most healthy people -->innate immunity sufficient to clear Aspergillus

**phagocytes are KEY**
What cells initiate the T-helper response?
DC’s ultimately come in and induce a T cell response—typically Th1

(When phagocytes aren't getting job done)
Identify the major Aspergillus species that causes 95% of human illness

**EXAM**
Aspergillus fumigates
This fungal species exhibits acute angle (<90°) branching hyphae
Aspergillus
What are the most common molds encountered by humans
Aspergillus
Aspergillus is dimorphic/non-dimorphic
NON-DIMORPHIC

-exist as a mycelium (mass of hyphae—multicellular branches that form a tangled mass called mycelia) in nature and in the body
What are the 3 clinical forms of Aspergillus infection?
hypersensitivity,

colonization

invasive
A patient presents with asthmatic symptoms and:

-Immediate cutaneous reactivity to Aspergillus skin test antigen

-High total serum IgE levels**

-Elevated Aspergillus specific IgG or IgE**

-Episodic asthma

-Sputum or BAL containing Aspergillus hyphae
Allergic bronchopulmonary aspergillosis (ABPA)

(hypersensitivity)

**Think IgE**
Aspergillus Colonization of mucosal surfaces occurs WITHOUT invasion. What happens?
colonizing existing cavity from pre-existing illness
HALLMARK of both colonization and invasive forms of Aspergillosis
Hemoptysis
Masses of fungal mycelia that grow in preexisting lung cavities

(ex. healed TB cavities, sarcoidosis lesions, CF scarring)
Colonization Aspergillosis
You are looking at a CXR and see:

Observation of mobile mass within cavity = highly suggestive radiographic finding**

Crescent sign or Monod's sign**
Colonization Aspergillosis
Demonstration of septate hyphae with acute-angle (<90°) branching in sputum or biopsy tissue is diagnostic of:
Invasive pulmonary aspergillosis
What is the greatest risk factor for Invasive pulmonary aspergillosis ?
Myelosuppression

(Prolonged neutropenia is also a risk factor)
What cells play a critical role in host defense for Invasive pulmonary aspergillosis?
Mɸ and PMNs
A "halo sign" on CXR is only SUGGESTIVE OF _______
Invasive pulmonary aspergillosis

**Demonstration of septate hyphae with acute-angle (<90°) branching in sputum or biopsy tissue is diagnostic**
In contrast to Aspergillus, these are non-septate with short stubby side branches at 90° angles
Zygomycosis

(Rhizopus, Mucor and Absidia sp.)
Predispositions to this dz are:

-Inhibition of PMN function (diabetic ketoacidosis)
•High blood sugar levels
•Low pH of tissues
•**Favor growth of organisms in lung

-Suppression of PMN and alveolar macrophage function (corticosteroids or cytotoxic agents)

-Immunodeficiency
Zygomycosis

(Rhizopus, Mucor and Absidia sp.)
Name the form of Zygomycosis:

• Occurs almost exclusively in Diabetic ketoacidosis**

• Infection usual begins in nose, spreads to eye orbits, sinuses, and eventually reaches into the cranium
rhinocerebral zygomycosis form
Zygomycosis can invade _____ -HIGH mortality rate when this occurs
Blood Vessels