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Published on: March 9, 2018
Para Vertebral Abscess and Rib Osteomyelitis due to Aspergillous Fumigatus in a Patient with Chronic Granulomatous
A Farhoudi1, A Siadati, Lila Atarod
1Department of Immunology and Allergy, Department of Pediatric infectious disease, Children Medical Centre, Tehran University of Medical Sciences, Tehran, Iran.
Abstract:
Chronic granulomatous disease is an infrequent primary immunodeficiency characterized by defective intracellular killing of ingested microorganisms thereby making patients highly susceptible to recurrent lite threatening bacterial and fungal infections. In this study, we review the medical course of an 8 yrs old girl with AR-CGD. She suffered from recurrent dermal and deep abscesses, retractable salmonellosis, disseminated BCGosis, recurrent aspergillus infection presenting as mandibular osteomyelitis and pulmonary involvement with invasion to rib and vertebral bodies. Despite of longterm IV amphotricin B, itraconazole and IFN-gamma administration, and surgical interventions (drainage and resection), she died in spite of long term antibiotic anti fungal prophylaxis and interferon-gamma administrations, invasive aspergillosis resistant to current conventional therapies is the cause of 1/2 to 1/3 of CGD deaths.
Insights
Chronic granulomatous disease (CGD) patients face severe infections due to impaired microorganism killing. This case highlights invasive aspergillosis as a fatal complication, even with aggressive treatment.
Area of Science:
- Immunology
- Infectious Diseases
- Genetics
Background:
- Chronic granulomatous disease (CGD) is a primary immunodeficiency.
- Patients experience recurrent, life-threatening bacterial and fungal infections due to defective intracellular killing.
- Autosomal recessive CGD (AR-CGD) presents unique challenges in management.
Purpose of the Study:
- To review the clinical course of a pediatric patient with AR-CGD.
- To illustrate the severe manifestations and treatment complexities of CGD.
- To emphasize the significant mortality associated with invasive fungal infections in CGD.
Main Methods:
- Case report review of an 8-year-old girl with AR-CGD.
- Documentation of clinical presentation, infections, and treatment interventions.
- Analysis of treatment outcomes, including antifungal therapy, IFN-gamma, and surgical procedures.
Main Results:
- The patient presented with recurrent abscesses, salmonellosis, BCGosis, and invasive aspergillosis affecting the mandible and pulmonary system.
- Despite long-term intravenous amphotericin B, itraconazole, IFN-gamma, and surgical interventions, the patient's condition deteriorated.
- Invasive aspergillosis, resistant to conventional therapies, was identified as the cause of death.
Conclusions:
- Invasive aspergillosis remains a major cause of mortality in CGD patients.
- Treatment resistance in invasive fungal infections poses a significant challenge in managing CGD.
- Improved therapeutic strategies are crucial for improving outcomes in CGD, particularly for invasive fungal infections.
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