CANDIDA ENDOCARDITIS IN A CHILD WITH HYPERIMMUNOGLOBULINEMIA-E SYNDROME

Hyperimmunoglobulin E syndrome (HIE) is a disorder characterized by ex tremely elevated serum levels of IgE and recurrent infections, Patient s are particularly predisposed to have staphylococcal abscesses, usual ly involving skin, lungs, and joints; but they are also at risk for in fections with other bacteria and fungi. We report the case of a 46-mon th-old bog with HIE who had Candida endocarditis and sepsis with a lar ge fungal mass extending through the tricuspid valve and into the surr ounding heart tissue, requiring surgical Excision and replacement with a prosthetic valve. He had an indwelling central line for previous an tibiotic therapy and had oral thrush for a month before presentation, which had been treated with oral nystatin. He was first seen with very dark urine, a new murmur, petechial rash, in shock, and disseminated intravascular coagulation. The white blood cell count was 35,700 with 70% segmented neutrophils, 9% banded neutrophils, 15% lymphocytes, 4% monocytes, and 2% eosinophils. Hemoglobin was 7.1, and platelet count was 14,000. Prothrombin time was 15.5, and partial thromboplastin time was 31; fibrinogen level was 110 mg/ml, and fibrin degradation produc ts were greater than 40 mg/ml, Serum IgE was 38,664 and 44,510 on repe at measurement, He has had recurrent staphylococcal pneumonias with pn eumatoceles, twice requiring segmental lung resection. Blood and tricu spid valve cultures grew Candida albicans. He was treated with amybote ricin and flucytosine, and later smirched to fluconazole, with good re sponse to therapy, A literature search revealed no other reported case of Candida endocarditis in patients with HIE. Fungal endocarditis is a rare complication, which may occur in patients with HIE and indwelli ng central catheters.