How should eosinophilic cystitis be treated in patients with chronic granulomatous disease?
Pediatr Nephrol
; 29(11): 2229-33, 2014 Nov.
Article
en En
| MEDLINE
| ID: mdl-25037864
ABSTRACT
BACKGROUND:
Chronic granulomatous disease (CGD) is a primary immunodeficiency resulting from the absence or malfunction of oxidative mechanism in phagocytic cells. The disease is due to a mutation in one of four genes that encode subunits of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Affected patients experience severe infections and granuloma formation due to exuberant inflammatory responses. Some evidence suggests that eosinophilic cystitis (EC) is included in the spectrum of inflammatory manifestations. EC is an inflammatory disease, rare in childhood, which may require different, nonstandardized therapeutic approaches, ranging from antihistamines to cyclosporine. CASE-DIAGNOSIS/TREATMENT Herein we describe the cases of two CGD patients with CGD who experienced EC during hospitalization for a severe infection.CONCLUSIONS:
EC in immunocompetent children seems to have a self-limiting course, unlike in CGD patients, in whom it presents a prolonged and recurrent course. We focus on the effective therapy administered to our patients with CGD and review the corresponding literature.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Contexto en salud:
3_ND
Problema de salud:
3_neglected_diseases
/
3_zoonosis
Asunto principal:
Cistitis
/
Enfermedad Granulomatosa Crónica
Límite:
Child, preschool
/
Humans
/
Male
Idioma:
En
Revista:
Pediatr Nephrol
Asunto de la revista:
NEFROLOGIA
/
PEDIATRIA
Año:
2014
Tipo del documento:
Article
País de afiliación:
Italia