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Clinical features in 17 paediatric patients with Wegener granulomatosis
- Source :
- Pediatric nephrology (Berlin, Germany). 17(9)
- Publication Year :
- 2001
-
Abstract
- The aim of this report was to describe childhood patients with Wegener granulomatosis (WG) from one centre, to analyse the variety of clinical manifestations seen and compare the data with other published paediatric and adult series. The records of 17 patients with WG who were under the care of Great Ormond Street Hospital for Children (GOSH) from 1981 to 1998 were reviewed. We analysed presenting features before admittance to GOSH and the clinical signs observed whilst the children were under the care of the hospital. Of 17 patients, 13 were females and there was a male/female ratio of 1:3.25. Among the patients there were 2 sisters. The age of the patients at disease onset varied from 2 weeks to 14 years. The median/mean age was 6/6.3 years. American College of Rheumatology criteria for diagnosing WG were fulfilled in 11 of 17 patients. The frequency of different system involvement was: respiratory 87%, kidneys 53%, sinuses 35%, joints 53%, eyes 53%, nervous system 12%, skin 53%. cANCA was positive in 10 patients (59%), but pANCA was negative in all measured sera. Kidneys were involved in 2 of 8 patients (25%) with the disease onset from 0 to 5 years and in 7 of 9 patients (78%) with the disease onset from 6 to 14 years ( P
- Subjects :
- Nephrology
Adult
Male
medicine.medical_specialty
Systemic disease
Adolescent
Eye Diseases
Respiratory Tract Diseases
Antibodies, Antineutrophil Cytoplasmic
Internal medicine
medicine
Humans
Child
Anti-neutrophil cytoplasmic antibody
business.industry
Vascular disease
Granulomatosis with Polyangiitis
medicine.disease
Rheumatology
Surgery
Treatment Outcome
El Niño
Pediatrics, Perinatology and Child Health
Prednisolone
Female
Kidney Diseases
business
medicine.drug
Kidney disease
Subjects
Details
- ISSN :
- 0931041X
- Volume :
- 17
- Issue :
- 9
- Database :
- OpenAIRE
- Journal :
- Pediatric nephrology (Berlin, Germany)
- Accession number :
- edsair.doi.dedup.....d8b1501e299ce2acc84dc809da642f09