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Old 07-31-2011
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Default impaired defence mechanisms

How to differentiate between impaired chemotaxis
destruction of CD4 T lymphocytes
defective opsonization
developmental arrest of maturation of B lymphocytes
impaired phagocytic oxidative metabolism??? i always get this mcq wrong??
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Old 04-12-2016
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Default

I wonder if there is an age difference between them.
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Old 04-12-2016
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High-yield Hit 1
HYPERIMMUNOGLOBULINEMIA E WITH IMPAIRED CHEMOTAXIS (JOB'S SYNDROME).
Features of this syndrome are eczema, "cold" staphylococcal skin abscesses, sinusitis, and otitis media. Originally described in fair-skinned, red-headed women, it has now been described in blacks and males as well. Recurrent pneumonia and mucocutaneous candidiasis are common, as are elevated IgE and eosinophil levels. Levamisole, once touted highly as a treatment, did not work in a well-performed, randomized trial, reducing therapeutic options to symptomatic therapy.

Job's is your chemotaxis (since people with Job's pay taxes)
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Old 04-12-2016
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Default Here you go. Maybe this will help.

Chronic glaucomatous disease is a defect of phagocytic cells due to dysfunction of the NADPH oxidase enzyme complex, leading to recurrent infections from catalse positive organisms. They include staph aureus, serratia marcescens,, burkholderia, klebsiella, and aspergillus. The dx is made by nitro blue tetrazolium slide test, flow cytometry, or cytochrome C reduction.
Tx includes prevention of infection with daily TMP-SMX and gamma-interferon 3 times a week. Bone marrow transplantation is experimental but curative.

Wiskott-Aldrich syndrome is an x-linked recessive disease caused by a defective gene encoding for wiskott-Aldrich syndrome protein. The classic presentation involves a young boy with eczema, thrombocytopenia, and recurrent infections with encapsulated germs. Immunologic findings include low IgM, high IgA and IgE, poor antibody responses to polysaccharide antigens, and moderately reduced number of T cells and platelets.

Chediak-higashi is characterized by decreased degranulation, chemo taxis, and granulopoiesis. It is a multisystem disorder with mild coagulopathy, peripheral and cranial neuropathy, hepatosplenomegaly, pancytopenia, partial oculocutaneous albinism, frequent bacterial infections (usually staph aureus) and progressive lymphoproliferative syndrome.
The findings of neutropenia and giant lysosomes in neutrophils will confirm the dx.
Tx includes prevention of infection with daily TMP-SMX and daily ascorbic acid.

Leukocyte adhesion defect: results from failure of host defenses due to defective tethering, adhesion, and targeting of myeloid leukocytes to sites of microbial invasion. Pts have Europhilia without polymorphs in the infected tissue or pus. Patients will have a history of delayed separation of the umbilical cord, recurrent bacterial infections, necrotic skin lesions, periodontitis, and alveolar bone loss leading to early loss of deciduous and permanent teeth.

Hyper-IgE (job’s) syndrome: characterized by chronic pruritic dermatitis, recurrent staph infections (skin and respiratory tract), markedly elevated serum IgE levels, eosinophilia, and coarse facial features.
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