Management dilemma of anti-GBM disease and p-ANCA-associated vasculitis with necrotizing skin lesions in a pediatric patient.
Diffuse alveolar hemorrhage and acute glomerulonephritis characterize pulmonary-renal syndrome, in which anti-glomerular basement membrane antibodies (anti-GBM) and anti-neutrophil cytoplasmic antibodies (ANCA) are often assessed. The treatment is complex, requiring a multidisciplinary approach, bas...
| Publicado en: | Pediatric Nephrology Vol. 40; no. 8; pp. 2691 - 2703 |
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| Autores principales: | , , , , , , , , , |
| Formato: | case study diagnostic images pictorial tables/charts Journal Article |
| Publicado: |
Springer Nature
Aug2025
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=186136025&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 186136025 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 0931041X EF1 jtl: Pediatric Nephrology issn: 0931041X maglogo: N pubinfo: dt: Aug2025 vid: 40 iid: 8 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 186136025 183404895 186136025 186136025 10.1007/s00467-025-06721-5 186136025 ppf: 2691 ppct: 12 formats: fmt: – @attributes: type: T – @attributes: type: P tig: atl: Management dilemma of anti-GBM disease and p-ANCA-associated vasculitis with necrotizing skin lesions in a pediatric patient. aug: au: Chirico, Valeria Silipigni, Lorena Tripodi, Filippo Conti, Giovanni Rulli, Immacolata Granata, Francesca Cinquegrani, Antonella Santoro, Domenico Gitto, Eloisa Chimenz, Roberto affil: https://ror.org/03tf96d34 Pediatric Nephrology and Dialysis Unit, University Hospital "G. Martino", 98124, Messina, Italy sug: subj: Goodpasture's Syndrome Therapy Antibodies, Antineutrophil Cytoplasmic Adverse Effects Vasculitis Risk Factors Skin Diseases, Vascular Risk Factors Necrosis Risk Factors Risk Assessment Vasculitis Therapy Skin Diseases, Vascular Therapy Necrosis Therapy Kidney Failure, Acute Lung Diseases Hemorrhage Nervous System Diseases Posterior Leukoencephalopathy Syndrome Therapy Female Child Immunosuppressive Agents Therapeutic Use Plasma Exchange Dialysis Biopsy Adrenal Cortex Hormones Therapeutic Use Cyclophosphamide Therapeutic Use Rituximab Therapeutic Use Brain Diseases Upper Extremity Surgery Amputation Death Child: 6-12 years Female ab: Diffuse alveolar hemorrhage and acute glomerulonephritis characterize pulmonary-renal syndrome, in which anti-glomerular basement membrane antibodies (anti-GBM) and anti-neutrophil cytoplasmic antibodies (ANCA) are often assessed. The treatment is complex, requiring a multidisciplinary approach, based on immunosuppressant therapies, mechanical ventilation, plasma exchange (PLEX), and kidney replacement therapy. This clinical case describes a 7-year-old female hospitalized for edema, hypertension, and acute kidney failure. Laboratory tests showed the coexistence of ANCA and anti-GBM autoantibodies. A kidney biopsy revealed necrotizing crescentic glomerulonephritis with linear deposits of IgG along the GBM. Corticosteroids and cyclophosphamide, followed by rituximab and PLEX represented the therapeutical strategies. Hemodialysis was needed for the fluid overload and acute kidney injury (AKI) management. Posterior reversible leukoencephalopathy syndrome (PRES), hemorrhagic alveolitis, and cutaneous necrotizing skin lesions requiring the amputation of the limb complicated the clinical course, until the death of the patient. Early diagnosis and multidisciplinary and personalized therapies represent the management dilemmas for this disease. The evaluation of new treatments, such as avacopan and imlifidase, is much needed in pediatric-onset disease. pubtype: Academic Journal doctype: case study diagnostic images pictorial tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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