Prognosis of chronic granulomatous disease.

Abstract

The records of 28 patients with chronic granulomatous disease born over a 32 year period were reviewed. The characteristics of the group, and the frequency with which various clinical and laboratory features had been recorded, was assessed. Nine patients were known to have died, in most cases of progressive suppurative infection. Actuarial analysis showed 50% survival through the third decade of life. The long term survival of patients developing symptoms after the end of the first year of life was significantly better than that of patients whose illness started in infancy. Our data confirm that the severity of chronic granulomatous disease is not uniform, and that the prognosis for long term survival is better than that suggested in earlier reports. Early onset may be a poor prognostic sign and invasive aspergillosis is a life threatening complication. In the absence of curative treatment, trials to assess the effectiveness of interferon gamma are necessary and early antenatal diagnosis should be offered to as many affected families as possible.

Monocyte/macrophage-Specific NADPH Oxidase Contributes to Antimicrobial Host Defense in X-CGD

“…Mortality of CGD patients is high especially during the first two decades of life [15]. Invasive fungal infections, principally aspergillosis, increase with age and account for one-third to half of all deaths [2,[16][17][18][19].…”

Successful unrelated bone marrow transplantation for a patient with chronic granulomatous disease and associated resistant pneumonitis and Aspergillus osteomyelitis

“…[1][2][3][4] A report of 368 patients with CGD showed a significantly higher mortality in patients with the X-linked recessive form of the disease. 4 It is difficult to predict which patient will experience severe infections repeatedly and have a poor prognosis.…”

“…IFN-␥, supportive therapy and prophylactic administration of therapeutic agents such as sulfamethoxazole-trimethoprim have improved the prognosis. [1][2][3][4] However, control of therapy- There has been an increase in the number of reports of successful hematopoietic stem cell transplantation (HSCT) for patients with CGD. [5][6][7][8][9][10][11][12] Most of these patients receive transplants from HLA-identical siblings during the first decade.…”

Glomerulonephritis associated with chronic granulomatous disease and systemic lupus erythematosus

“…Aspergillus pneumonia is the leading cause of death [5]. Under continuous prophylaxis using intracellularly active antimicrobials the survival rate at age 20 has increased to 50% [4]. This report describes a boy with CGD with an unusual systemic and renal complication, namely lupus erythematosus with diffuse proliferative glomerulonephritis.…”

“…Clinical manifestations start in 80% before the second birthday [4] and consist of recurrent pyodermia, suppurative lymphadenitis, pneumonia, enteritis, liver abscess, osteomyelitis, and failure to thrive. Aspergillus pneumonia is the leading cause of death [5].…”