Nalazite se na CroRIS probnoj okolini. Ovdje evidentirani podaci neće biti pohranjeni u Informacijskom sustavu znanosti RH. Ako je ovo greška, CroRIS produkcijskoj okolini moguće je pristupi putem poveznice www.croris.hr
izvor podataka: crosbi !

Autoimmune Pulmonary Alveolar Proteinosis (PAP) in a pediatric patient-case report (CROSBI ID 598708)

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

Ferenčić, Željko ; Navratil, Marta ; Petković, Giorgie ; Erceg, Damir ; Turkalj, Mirjana. Autoimmune Pulmonary Alveolar Proteinosis (PAP) in a pediatric patient-case report. 2013

Podaci o odgovornosti

Ferenčić, Željko ; Navratil, Marta ; Petković, Giorgie ; Erceg, Damir ; Turkalj, Mirjana.

engleski

Autoimmune Pulmonary Alveolar Proteinosis (PAP) in a pediatric patient-case report

Pulmonary alveolar proteinosis, (PAP) is a rare disease of unknown etiology, characterized by accumulation of lipoproteinaceous material within alveoli. The prognosis is highly variable, and for over three decades the pathophysiology and treatment of this disease remained a mystery. With recent developments in molecular genetics, our understanding of the pathogenesis of PAP has improved significantly.Four forms of PAP are recognised in children: congenital, acquired, secondary and idiopathic. In adults, and occasionally in older children and adolescents, PAP is usually an autoimmune disease ; antibodies to granulocyte macrophage colony-stimulating factor (anti-GM-CSF) are present in 90 percent of cases. Autoimmune PAP with anti-GM-CSF antibodies has been reported in only a few children, who presented in late childhood or adolescence. Thus, unlike adults with PAP, it appears that the majority of infants and children with PAP do not have the autoimmune form of the disease. As an example, in a study of 15 children with PAP, none had anti-GM-CSF antibodies in the serum, and only one had anti-GM-CSF antibodies in bronchoalveolar lavage fluid. We present a rare case of autoimmune PAP in a 10-year old male patient, diagnosed by HRCT, lung biopsy and lab analyses (positive anti- GM-CSF antibodies in serum). Patient was treated with corticosteroids, hidrochloroquine, GM-CSF and supportive therapy. Lung transplantation was performed one and half year after diagnosis.

Pulmonary alveolar proteinosis; children; autoimmune disease; Lung transplantation

nije evidentirano

nije evidentirano

nije evidentirano

nije evidentirano

nije evidentirano

nije evidentirano

Podaci o prilogu

2013.

nije evidentirano

objavljeno

Podaci o matičnoj publikaciji

Podaci o skupu

ISABS conference on Forensic, Anthropologic and Medical Genetics and Mayo Clinic Lectures in Translation Medicine

poster

24.06.2013-28.06.2013

Split, Hrvatska

Povezanost rada

Kliničke medicinske znanosti