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Old 06-21-2011
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Kids Pediatric immune deficiency disease

A 14-year-old girl is seen in the pediatric clinic with a sinus infection. The pediatrician notes this is her fourth such infection this year, and that she had bacterial pneumonia 9 months ago. The patient had been healthy until these infections first appeared. Physical examination is consistent with bacterial sinusitis; otherwise, it is normal. A complete blood count with differential and serum immunoglobin panel is ordered and shows: white blood cell count 14,000/mm3, hemoglobin 14 g/dL, hematocrit 38%, platelets 300,000 mm3, immunoglobulin M 20 mg/dL, immunoglobulin G 250 mg/dL, and immunoglobulin A 20 mg/dL. Which of the following is the most likely diagnosis?

Answer Choices
A. C6 deficiency
B. Chronic granulomatous disease
C. Common variable immunodeficiency
D. Hyperimmunoglobulinemia E syndrome
E. Lazy leukocyte syndrome
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Old 06-21-2011
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go with C not sure
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Old 06-22-2011
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I will go with "Common Variable Immunodeficiency" :sorry:
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Old 06-22-2011
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Thumbs Up Common Immunoglobulins Value for Adults

I have attached a table (very interesting), that Shows the Normal Values for Adults' Immunoglobulines.







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Pediatric immune deficiency disease-common-immunoglobulins-values-adults.jpg  
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Old 06-22-2011
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Option D (Hyperimmunoglobulinemia E syndrome) is correct. Although immunoglobulin (Ig) E levels are not shown, this syndrome manifests at a much earlier age and is unmistakable for the severity of associated bacterial skin infections, which gives the syndrome its alternative designation of “Job’s disease.” Also, other Ig levels are not typically depressed.

Option A (C6 deficiency) is incorrect. C6 deficiency is associated with recurrent meningococcal meningitis and gonococcemia.

Option B (Chronic granulomatous disease) is incorrect. Chronic granulomatous disease is associated with normal antibody levels.

Option C (Common variable immunodeficiency) is incorrect. The patient’s history of previous good health does not point toward a primary immunodeficiency but does not entirely rule it out. Quantitation of serum immunoglobulins reveals diminished IgG, IgM, and IgA levels. This is a good case of common variable immunodeficiency with the reminder that primary immunodeficiencies may be undetected until later in life.

Option E (Lazy leukocyte syndrome) is incorrect. Myeloperoxidase deficiency in leukocyte results in some increased susceptibility to fungal infections but not in increased sinopulmonary bacterial infections. Immunoglobin levels are not depressed.

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.

Taken from Cecil Textbook of Medicine by Goldman.
High-yield Hit 2
HYPERIMMUNOGLOBULINEMIA E SYNDROME (HIE).
page 1602

page 1603
This syndrome is a well described but poorly understood defect that leads to recurrent staphylococcal abscesses and markedly elevated serum IgE concentrations. Patients develop severe staphylococcal abscesses predominantly involving the skin and lungs. Pulmonary involvement is very common and often results in pneumatocoeles. The syndrome can sometimes be confused with severe atopic dermatitis, but HIE has a much earlier onset and almost always includes lung involvement. Patients with HIE may develop cutaneous (and nail) candidal infections, which is usually not a feature of atopy. Other clinical features of HIE include hyperextensible joints, scoliosis, easy breakage of bones, osteoporosis, midline anomalies, craniosynostosis, delayed shedding of primary teeth due to a lack of root resorption, unusual facies with a broad nasal bridge, rough facial skin, and a tubular and prominent nose. Most cases are sporadic, but an autosomal-dominant form of inheritance with variable expressivity has been described. Laboratory features include high levels of serum IgE (<2000, but perhaps reducing with age), normal levels of IgG, IgA, and IgM, eosinophilia, and reduced anamnestic antibody responses to some vaccine antigens. The nature of this disorder remains unknown. The only therapy is prophylactic therapeutic doses of a penicillinase-resistant antibiotic and treatment of other infections as appropriate.
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