PSEUDOMONAS CEPACIA PNEUMONIA IN A CHILD WITH CHRONIC GRANULOMATOUS DISEASE AND SELECTIVE IgA DEFICIENCY

Abstract

A 6 1/2 year-old boy with chronic granulomatous disease (CGD) and selective IgA deficiency developed a chronic progressive pneumonia which failed to respond to several conventional combinations of antimicrobial therapy. On lung biopsy, Pseudomonas cepacia was obtained in pure culture, sensitive to chloramphenicol, tetracycline, kanamycin and nalidixic acid. With specific therapy, he slowly recovered. P. cepacia has not been previously described as a cause of persistent pneumonia in immunodeficient children. The occurrence of CGD and selective IgA deficiency together is a very rare combination of immunodeficiencies.

Therapeutic options for Burkholderia cepacia infections beyond co-trimoxazole: a systematic review of the clinical evidence

“…The median age of the included patients was 35 years (range 0-78 years). Seven (12.3%) of the 57 patients suffered from CF [14,39,42,50,60,67,74] and 5 (8.8%) suffered from CGD [4,46,66].…”

“…In heroin addicts it has been reported to cause endocarditis [54], whilst pneumonia is frequent in CGD [4,66]. It is also common in immunocompromised patients suffering from malignancies [58,62,68], haemoglobinopathies [35] and other types of immunodeficiency [41,52,70].…”

“…Nine (15.8%) of 57 cases were treated with chloramphenicol, either as monotherapy in 4 cases (44.4%) [37,64,69,72] or as part of combination therapy with sulfisoxazole in 3 cases (33.3%) [4], sulfadiazine in 1 case (11.1%) [69] and tetracycline/kanamycin/nalidixic acid in the last case (11.1%) [66]. Six (66.7%) of them were cured and clinical improvement was noted in two (22.2%), one of whom died of a reason irrelevant to the infection.…”

Burkholderia glumae Infection in an Infant with Chronic Granulomatous Disease

“…It has been associated with epidemics of rice panicle blight in the southeastern United States (29), producing yield losses as high as 40% (27). There is some evidence to suggest that this disease is seed borne, transmitted from plants infected the previous year (30). Two toxins, fervenulin and toxoflavin, appear to be involved in the phytopathology of B. glumae (28).…”

“…Patients with CGD are at increased risk for invasive infections caused by various catalase-positive bacteria including Staphylococcus aureus, Aspergillus spp., Nocardia spp., and Serratia marcescens (12,20). Certain Burkholderia species, particularly those within the B. cepacia complex, are wellrecognized pathogens in CGD patients (1,2,19,30). In fact, these species are especially virulent in CGD, causing sepsis and being responsible for a significant proportion of fatalities in these patients (38).…”