P1: RLJ/OZN P2: KUF
0521779407-D-01 CUNY1086/Karliner 0 521 77940 7 June 13, 2007 7:41
Diphtheria Disorders of Neutrophil Function 489■Patients should be immunized with diphtheria toxoid, since toxin is
so potent that disease itself may not be immunizing.
■Close contacts should receive therapy with erythromycin for 7 d and
have cultures 2 wks after completion of therapy to ensure eradica-
tion of the carrier state; close contacts should also be immunized if
immunization status unclear or immunizations not up to date.
complications and prognosis
■Prognosis depends on immunization status and prompt institution
of therapy.
■Full or partial immunization associated with improved outcome
■Early administration of antitoxin improves outcome.
■Cardiac disease associated with higher mortality
■Neuropathy may be slow to resolve, but recovery usually complete.
■Prevention depends on adequate immunization; following primary
immunization in childhood, adults should receive booster doses of
Td, tetanus and diphtheria toxoid (dose of diphtheria toxoid lower
in the adult than the pediatric preparation) every 10 y.Disorders of Neutrophil Function........................
NANCY BERLINER, MD
history & physical
■Severe bacterial and fungal infections from early childhood
■Family history of the disorder: usually X-linked, but autosomal reces-
sive inheritance also occurs
■Examine for evidence of infection. Most common infections: pneu-
monia, lymphadenitis, cutaneous infections, hepatic abscesses,
osteomyelitis, aphthous ulcers, perirectal abscesses
tests
■Diagnosis confirmed by tests of neutrophil oxidative metabolism:
nitroblue tetrazolium (NBT) slide test or measurements of superox-
ide or peroxide production.
differential diagnosis
■Heterogeneous group of rare disorders
■Defective production of superoxide (O− 2 ) by neutrophils, monocytes,
and eosinophils
■Caused by mutations in any of four genes encoding the respiratory
burst oxidase.