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2019, Number 4

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Rev Cubana Pediatr 2019; 91 (4)

Goodpasture´s syndrome in a pre-school girl

Martínez SM, Navarro PJE, Duarte PMC, Acosta MEC, Salabarria GJR

Language: Spanish
References: 13
Page: 1-11
PDF size: 345.02 Kb.


ABSTRACT

Introduction: Goodpasture´s syndrome is a rare autoimmune disease that is part of the lung-kidney syndrome’s sprectrum.
Objective: To present the case of a pediatric patient with an atypical Goodpasture´s syndrome.
Case presentation: Six years old female patient under follow-up in the Nephrology service. She was admitted when she was four years old by presenting macroscopic hematuria, respiratory symptoms and a history of high titers of anti-glomerular basement membrane antibodies. She was under research and was diagnosed with the Goodpasture´s symdrome. After being under immunosupressive treatment with cyclophosphamide and methylprednisolone followed by oral prednisone, the patient presented a decrease in the titers of anti-glomerular basement membrane antibodies, and an improvement of the respiratory symptoms; however, proteinuria kept increasing in the last months. For the diagnosis, there were taken into account the clinical manifestations of the girl, the high titers of anti-glomerular basement membrane antibodies and the confirmations of glumerulopathy by renal biopsy. The treatment was effective quickly with an inmmediate decrease of the titers of anti-glomerular basement membrane antibodies and an evident improvement of the clinical condition of the patient. This is the first pediatric case presenting Goodpasture´s syndrome that has been published in Cuba.
Conclusions: As this is a rare entity in Pediatrics, it is required an early diagnosis and an aggressive treatment to improve the patient’s prognosis. In the follow-up are needed a prolonged pharmacological therapy, an adequate adherence to the proposed treatment and a close clinical and analytic monitoring from which will depend the progression of the lung and renal injury.


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