title: [Amyloidosis associated with chronic granulomatous disease in a patient with a renal transplant and recurrent urinary tract infections].
abstract: Chronic granulomatous disease is a group of syndromes which share a defect in a component of the phagocyte NADPH-oxidase complex. Without this enzyme activity, phagocytic cells cannot produce superoxide, peroxide, and other potent microbicidal radicals, and are less able to kill ingested pathogens. The clinical picture is characterised by recurrent life-threatening bacterial and fungal infections and abnormal tissue granuloma formation. On the other hand, amyloidosis is a systemic disease with renal involvement occurring in the majority of cases. Recurrent amyloidosis is a rare but well documented event in renal transplant recipients. However, graft loss secondary to amyloidosis has been noted infrequently. In addition, de novo amyloidosis has not been previously associated with graft loss. We report here a renal transplant recipient with chronic granulomatous disease and history of recurrent urinary tract infections, who developed nephrotic syndrome and progressive renal insufficiency secondary to de novo AA amyloidosis leading to graft loss 66 months after transplantation.
authors.affiliation: Servicio de Nefrología, Hospital Central de Asturias, Oviedo. cpeces@correo.varnet.com
authors.forename: R
authors.initials: R
authors.lastname: Peces
authors.forename: P
authors.initials: P
authors.lastname: Ablanedo
authors.forename: M
authors.initials: M
authors.lastname: Seco
mesh_terms: D000686:Amyloidosis / Q000209:etiology* / Q000473:pathology; D002545:Brain Ischemia / Q000150:complications; D004697:Endocarditis, Bacterial / Q000209:etiology; D004927:Escherichia coli Infections / Q000150:complications; D017809:Fatal Outcome; D005260:Female; D016908:Gram-Positive Bacterial Infections / Q000150:complications; D006105:Granulomatous Disease, Chronic / Q000150:complications*; D006801:Humans; D006937:Hypercholesterolemia / Q000150:complications; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000601:surgery; D016030:Kidney Transplantation; D008875:Middle Aged; D011183:Postoperative Complications / Q000209:etiology*; D011425:Propionibacterium acnes; D011507:Proteinuria / Q000209:etiology; D011512:Proteus Infections / Q000150:complications; D011513:Proteus mirabilis; D012008:Recurrence; D018805:Sepsis / Q000150:complications; D019737:Transplants; D014552:Urinary Tract Infections / Q000150:complications* / Q000382:microbiology