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6 Cards in this Set
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Wiskott Aldrich
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bloody nature of the diarrhea may help distinguish from SCID... The big prob¬lem with platelet transfusions is that the patient may develop platelet antibodies, making subsequent transfusions less useful... Standard atopic care with vigorous use of moisturizers first and foremost ... Defer topical steroids unless necessary... Crust with eczema can be hemorrhagic ... Failure to thrive can be seen as with all primary immunodeficiencies in infancy. Often, early death without BMT although longer survival without BMT may occur...
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Chronic Granulomatous Disease
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A couple of kids have had resistant bacterial scalp infections... I have never seen the periorificial dermatitis... NBT is a simple test to do... Can be done right in the doctor's off ice ... May pick up tender joints by observing the kid's positioning of the arms and legs ... One quarter of a cup of clorox to a full bath of water is a useful, cheap way to keep up with recurrent skin infections... I tend to not restrict patient's activity... Subcutaneous gamma interferon three times a week has provided clinical improvement... Patients with chronic granulornatous disease (CGD) tend to be better targets for prophylactic antibiotics...
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Hyper IgE Syndrome
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I lump this syndrome with job syndrome. . . You're talking about a child with diffuse dermatitis and deep seated pyogenic infection... Not just your casual impetigo but deep seated abscesses, chronic severe ear infections... Remember atopics can have IgE in the thousands as well... This is typically not a syndrome of infancy... Large pneumatoceles will be amenable to lobectomy...
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Severe Combined Immunodeficiency
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Some cases may present with GVHD resulting from packed red blood cell transfu¬sions or in neonates because of maternal fetal transfusion... Most institutions are irradiating blood products... Ideal ly you'd see an absent thymic shadow on x ray... Virtually every medical center in the United States will be able to do lymphocyte assays... A limited number will do ADA assays... ADA and gamma C have been tar¬geted for gene therapy ...
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SCID
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BMT once diagnosis is made... Approximately 50% survival with transplant ... Once the diagnosis is made, they are isolated with total reverse isolation. . . Like any chronic illness with kids, there are tremendous psy-chosocial issues... Multidisciplinary care centers with social workers and child life workers are ideal
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Hereditary Angioedema
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We have used Stanazolol to induce activity of the enzyme... it seems to have a shorter half life and to be better tolerated by the patients than Danazol ... Use with caution in growing children... Kids can start getting attacks at any time ... To me, the classic occurrence is a fixed, indurated swelling of the lip without erythema... Angioedema is not responsive to histarnines... These patients should be wearing medic alert bracelets... Death from laryngeal obstruction still occurs
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