Santos S, Marques S, Golper T, Langone A, Fogo A B
Department of Nephrology, Centro Hospitalar do Porto, Porto, Portugal; Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Department of Nephrology, Hospital São João, Porto, Portugal; Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Transplant Proc. 2017 Dec;49(10):2384-2387. doi: 10.1016/j.transproceed.2017.07.006.
Alport syndrome (AS) and thin basement membrane lesions are caused by various mutations in type IV collagen genes. Although AS is considered a rare disease, thin basement membrane is a frequent pattern, especially in families with a history of persistent hematuria. We report a patient with a diagnosis of AS who developed end-stage kidney disease (ESKD) and received a kidney transplant from a living unrelated donor. The graft biopsy specimen surprisingly showed a pattern of thin basement membranes.
奥尔波特综合征（AS）和薄基底膜病变是由IV型胶原基因的各种突变引起的。尽管AS被认为是一种罕见疾病，但薄基底膜是一种常见表现，尤其是在有持续性血尿病史的家族中。我们报告了一名诊断为AS的患者，该患者发展为终末期肾病（ESKD）并接受了来自非亲属活体供体的肾移植。移植肾活检标本令人惊讶地显示出薄基底膜的表现。
