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Rapidly progressive pediatric pulmonaryrenal syndrome of ill-defined etiology and prompt response to immunosuppressive treatment (CROSBI ID 732216)

Prilog sa skupa u zborniku | sažetak izlaganja sa skupa | međunarodna recenzija

Matić, Vanja Ille ; Kifer, Nastasia ; Rubić, Filip ; Matić, Toni ; Dessardo, Sandro ; Cvitković, Miran ; Galić, Slobodan ; Vrljičak, Kristina ; Kos, Ivanka ; Lamot, Lovro Rapidly progressive pediatric pulmonaryrenal syndrome of ill-defined etiology and prompt response to immunosuppressive treatment // Pediatric Nephrology. 2022. str. 2945-2945 doi: 10.1007/s00467-022-05630-1

Podaci o odgovornosti

Matić, Vanja Ille ; Kifer, Nastasia ; Rubić, Filip ; Matić, Toni ; Dessardo, Sandro ; Cvitković, Miran ; Galić, Slobodan ; Vrljičak, Kristina ; Kos, Ivanka ; Lamot, Lovro

engleski

Rapidly progressive pediatric pulmonaryrenal syndrome of ill-defined etiology and prompt response to immunosuppressive treatment

Introduction: Pulmonary-renal syndrome (PRS) refers to the combination of acute kidney injury (AKI) caused by rapidly progressive glomerulonephritis, and lung involvement with severe respiratory failure, which often involves diffuse alveolar haemorrhage. It implies different rare conditions such as various forms of systemic vasculitis, Goodpasteur syndrome and systemic lupus erythematosus. Nevertheless, nonspecific conditions without vasculitis can also cause PRS. Material and methods: We describe a child with unknown cause of PRS who was successfully treated with immunosuppressive therapy. Results: A 16- year-old girl presented with abdominal pain, vomiting, high fever, cervical lymphadenopathy and splenomegaly. Initially, her laboratory findings indicated systemic inflammation, with increased inflammatory markers and transaminases. Urinalysis was positive for leukocytes, erythrocytes, nitrites and proteins with active sediment and significant number of E. coli in microbiology. Subsequently, nephrotic range proteinuria and AKI were observed. Seven days later she developed dyspnoea with respiratory insufficiency necessitating mechanical ventilation. Computed lung tomography revealed ground-glass opacities and crazy paving pattern. The broad differential diagnosis of PRS was considered, with alternative complement pathway activation, negative ANCA, antiGBM and ASO titre. Renal biopsy was suggestive of possible infectious cause, while microbiology revealed high circulating EBV DNA load with positive EBV serology. Steroid pulse therapy led to prompt improvement of lung and kidney function, with further amelioration and no relapses during the six months follow-up. Conclusions: Even though PRS is mostly caused by specific well defined immunological disorders, unknown and atypical causes can also lead to this life-threatening condition. Therefore, early clinical recognition and prompt immunosuppressive treatment are of greater importance than defining the cause which might remain inconclusive long after the full remission has been achieved.

pulmonary-renal syndrome ; acute kidney injury ; immunosupression ; congenital anomalies of kidneys and the urinary tract

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Podaci o prilogu

2945-2945.

2022.

objavljeno

10.1007/s00467-022-05630-1

Podaci o matičnoj publikaciji

Pediatric Nephrology

Podaci o skupu

54th ESPN Annual Meeting

poster

22.06.2022-25.06.2022

Ljubljana, Slovenija

Povezanost rada

Kliničke medicinske znanosti

Poveznice