{
  "abstract": "X-linked agammaglobulinaemia (XLA) is a rare congenital immunodeficiency caused by pathogenic mutations in the Bruton’s tyrosine kinase (BTK) gene, characterised by a reduction in mature B cells and low levels of immunoglobulins, which predispose patients to recurrent infections. However, renal involvement in adult XLA patients is extremely rare. Here, we report a man in his early 20s with XLA who experienced recurrent severe infections, resulting in elevated serum creatinine, nephrotic-range proteinuria and haematuria. Laboratory analysis revealed the absence of mature B cells and significantly reduced immunoglobulin levels. A kidney biopsy confirmed a diagnosis of membranoproliferative glomerulonephritis (MPGN), and genetic testing identified a BTK gene mutation. This is a rare case of XLA complicated by MPGN prior to intravenous immunoglobulin therapy, which expands the pathological spectrum of renal involvement.",
  "authors": [
    {
      "affiliations": [
        "Nephrology, Zhongshan Hospital Fudan University, Shanghai, China",
        "Nephrology, Zhejiang Provincial People's Hospital (Affiliated People's Hospital, Hangzhou Medical College), Hang Zhou, China"
      ],
      "name": "Luna He"
    },
    {
      "affiliations": [
        "Nephrology, Zhongshan Hospital Fudan University, Shanghai, China"
      ],
      "name": "Xiao Tan"
    },
    {
      "affiliations": [
        "Nephrology, Zhongshan Hospital Fudan University, Shanghai, China"
      ],
      "name": "Shi Jin"
    },
    {
      "affiliations": [
        "Nephrology, Zhongshan Hospital Fudan University, Shanghai, China"
      ],
      "name": "Yi Fang"
    },
    {
      "affiliations": [
        "Nephrology, Zhongshan Hospital Fudan University, Shanghai, China"
      ],
      "name": "Yiqin Shi"
    }
  ],
  "title": "Membranoproliferative glomerulonephritis in a young adult with X-linked agammaglobulinaemia",
  "uid": "5c60bbc0-7aee-54df-88ee-d00a0933a718"
}