Internal Medicine

0521779407-10 CUNY1086/Karliner 0 521 77940 7 June 7, 2007 18:40

822 Immunodeficiency Disorders Impetigo

➣Early component (C1, C4, C2) deficiency associated w/ SLE

➣Late components: C5–C9; recurrent Neisseria infections

management

What to Do First

■Determine presence & type of immunodeficiency disorder

General Measures

■Appropriate antibiotic therapy for infections

specific therapy

■IVIg replacement therapy

➣Treatment of choice for common variable, hyper-IgM syndrome,

Bruton’s disease

NOT indicated in IgA or IgG subclass deficiency

Maintain trough IgG level >600 mg/dL

➣Side effects: chills, fever, nausea, vomiting, headache, aseptic

meningitis (related to rate of infusion)

No risk of HIV; small risk of hepatitis

■Stem cell transplantation (bone marrow, cord blood)

➣Treatment of choice for hyper-IgM, complete DiGeorge, Wiskott-

Aldrich, leukocyte adhesion deficiency

■Replacement of specific proteins

➣IFN ?subcutaneously for CGD

follow-up

■IgG trough levels, blood count & differential, liver enzymes every 3–6

months

■Blood & urine culture when febrile

complications & prognosis

■Echovirus encephalitis in agammaglobulinemia

■Autoimmune diseases: neutropenia, inflammatory bowel disease,

arthritis

■Chronic lung disease if not treated early

■Lymphoid malignancies

Impetigo...........................................

ALFRED L. KNABLE, MD

history & physical

History

■Seen in all age groups, but MOST common in infants and children