[Wegener's granulomatosis. Report of a pediatric case and review of the literature].
Wegener's granulomatosis is a form of systemic vasculitis of unknown etiology that affects the small blood vessels. The disease is characterized by the formation of granulomas on small and medium blood vessels, and often affects the respiratory tract and renal system. In 86% of diagnosed cases, renal compromise is in the form of glomerulonephritis. Wegener's granulomatosis can occur at any age, principally during the 4th and 5th decades of life, and 85% of cases occur over the age of 19 years. In the United States about 3 per 100,000 cases are reported, with a major occurrence in males. The present report is about a case of a 12 year old female with Wegener's granulomatosis of 18 months of evolution, with symptoms such as epistaxis, generalized edema, cough, dyspnea, hemoptysis, high blood pressure and macroscopic hematuria. Out of the five criteria for the diagnosis of Wegener's granulomatosis, she had perinuclear anti-neutrophil cytoplasmic antibody positive to serum p-ANCA mark, which is the least frequent, which makes more difficult the diagnosis. Over a period of five years, in our hospital, we have diagnosed 11 patients with Wegener's granulomatosis, and the present case is the only pediatric case.
Ayala de la Cruz
María del Carmen
Mdel C
Servicio de inmunología pediátrica, Hospital Regional de Especialidades Núm. 25, IMSS, Av. Lincoln y Gonzalitos, CP 64400, Monterrey, NL.
González Díaz
Roberto
R
López Lara
Norma Deyanira
ND
D000276:Adjuvants, Immunologic / Q000627:therapeutic use; D017668:Age of Onset; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000628:therapy; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005260:Female; D005512:Food Hypersensitivity / Q000150:complications; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000628:therapy; D006417:Hematuria / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D007676:Kidney Failure, Chronic / Q000209:etiology; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D012141:Respiratory Tract Infections / Q000188:drug therapy / Q000209:etiology; D014165:Transfer Factor / Q000627:therapeutic use; D015662:Trimethoprim, Sulfamethoxazole Drug Combination / Q000627:therapeutic use; D014481:United States
D000276:Adjuvants, Immunologic; D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D014165:Transfer Factor; D015662:Trimethoprim, Sulfamethoxazole Drug Combination; D003520:Cyclophosphamide; D011241:Prednisone