Abstract
Good pasture syndrome (GPS) has been paid much attention recently for the dangerous illness and high mortality. To investigate the efficiency of plasma exchange (PE) to treat Goodpasture syndrome (GPS) in children associated with Turner's syndrome. We report a case of a 15 year old female with GPS and Turner's syndrome. The patient has intermittent fever and cough for 45 days and oliguria for 6 days. Turner's syndrome was determined through blood karyotype analysis, and GPS was diagnosed because the patient was negative for antinuclear antibodies and antineutrophil cytoplasmic antibodies (ANCA), but positive for anti-glomerular basement membrane (anti-GBM) antibodies (200 RU/ml). PE was carried out in combination with immunosuppression therapy. The results show PE treatment can efficiently decrease the levels of anti-GBM antibodies. The antibody levels were >200 RU/ml and 184 RU/ml before and after the first PE treatment, respectively. The removal efficiency were 40%, 47%, 42%, 54%, 52% for the fifth, sixth, seventh, eighth and ninth PE procedures, respectively. The therapy with PE, hemodialysis, pulse methylprednisolone followed by oral prednisone and cyclophosphamide greatly contributed to improvement of this patient's condition, and resolved the patient's pulmonary haemorrhage. All these results demonstrate that PE contributed efficiently to the treatment for GPS in children.
Highlights
Goodpasture syndrome (GPS) is rare in children
The role of anti-glomerular basement membrane antibodies in the pathogenesis of GPS is well-known, and plasma exchange (PE) has been used for antibody removal 1-2 .We reported the first case of GPS associated with Turner’s syndrome in children
GPS is characterized by rapidly progressive glomerulonephritis, pulmonary haemorrhage and the presence of anti-GBM antibodies
Summary
Goodpasture syndrome (GPS) is rare in children. The role of anti-glomerular basement membrane (anti-GBM) antibodies in the pathogenesis of GPS is well-known, and plasma exchange (PE) has been used for antibody removal 1-2 .We reported the first case of GPS associated with Turner’s syndrome in children. The patient was treated with a combination of PE, steroids and immunosuppression, which has previously been reported to be effective. The levels of anti-GBM antibodies were tested pre- and postPE, and the efficiency of removal was calculated to evaluate the efficiency of PE therapyfor treatment of GPS
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