Department of Pediatrics Renal Section, Baylor College of Medicine, MC 3-2482, 6621 Fannin St, Houston, TX 77030-2399, USA.
Pediatr Nephrol. 2010 Mar;25(3):535-8. doi: 10.1007/s00467-009-1382-8. Epub 2009 Dec 3.
Pulmonary renal syndromes are unusual, but frequently life-threatening manifestations of a distinct group of disorders in the pediatric age group. Although IgA nephropathy is a common cause of hematuria, it is an extremely rare cause of pulmonary renal syndrome, causing high mortality, and has mostly been reported in adult patients. We describe the youngest patient with this presentation reported to date, a 14-year-old male, who presented with end stage renal disease and pulmonary hemorrhage and was found to have IgA nephropathy by renal biopsy and pulmonary capillaritis by open lung biopsy. His lung disease was successfully treated with immunosuppressive medications. Despite this being a rare manifestation of IgA nephropathy, clinicians need to be aware of this presentation as it is potentially fatal, but amenable to aggressive immunosuppression.
肺-肾综合征是儿科中一组罕见但常危及生命的疾病的表现。虽然 IgA 肾病是血尿的常见原因,但它是肺-肾综合征的极罕见病因,导致高死亡率,并且主要在成年患者中报道。我们描述了迄今为止报告的该表现的最年轻患者,一名 14 岁男性,表现为终末期肾病和肺出血,肾活检显示 IgA 肾病,开胸肺活检显示肺毛细血管炎。他的肺病通过免疫抑制药物成功治疗。尽管这是 IgA 肾病的一种罕见表现,但临床医生需要意识到这一表现可能是致命的,但可通过积极的免疫抑制治疗。