Management and outcomes of childhood Goodpasture's disease
| Autor: | Gianluigi Ardissino, Carmela P Menzi, Mario G. Bianchetti, Giacomo D. Simonetti, Barbara S. Bucher |
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| Rok vydání: | 2017 |
| Předmět: |
Nephrology
Male medicine.medical_specialty Pediatrics Adolescent Anti-Glomerular Basement Membrane Disease medicine.medical_treatment Biopsy 030232 urology & nephrology Disease 03 medical and health sciences 0302 clinical medicine Adrenal Cortex Hormones Risk Factors Internal medicine Surveys and Questionnaires medicine Prevalence Humans Medical history Renal replacement therapy Child Cyclophosphamide Retrospective Studies 030203 arthritis & rheumatology medicine.diagnostic_test business.industry Incidence Retrospective cohort study Plasmapheresis Renal Replacement Therapy Treatment Outcome Child Preschool Pediatrics Perinatology and Child Health Cohort Kidney Failure Chronic Female business Immunosuppressive Agents |
| Zdroj: | Pediatric research. 83(4) |
| ISSN: | 1530-0447 |
| Popis: | BackgroundIn an attempt to improve knowledge about childhood Goodpasture's disease, we performed a retrospective analysis of patients with Goodpasture's disease from several pediatric nephrology centers.MethodsWe analyzed the responses to 27 questions that elicited information about the following: incidence, demographics, patient history and clinical presentation, diagnostics performed, acute and chronic therapy, course of disease, and outcome.ResultsGoodpasture's disease, which is extremely rare in this age group, may manifest in 2-year-old toddlers and does not typically present with pulmonary findings before puberty. Goodpasture's disease has a poor outcome with more than 50% of patients progressing to end-stage renal disease. No deaths were reported in this cohort, and renal improvement was observed in children with severe biopsy findings who required renal replacement therapy during the acute phase.ConclusionThe present investigation gives detailed information about childhood Goodpasture's disease under real-life conditions and reveals that very few pediatric cases have been reported. Nearly 50% of children progressed to end-stage renal disease. However, long-term outcome in children might be better than in adults. Aggressive immunosuppressive therapy might be necessary for all affected children, even in patients who require renal replacement therapy or have severe biopsy findings. |
| Databáze: | OpenAIRE |
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