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Background <p>Acute kidney injury (AKI) is an understudied complication in pediatric Fanconi anemia (FA) patients undergoing hematopoietic stem cell transplantation (HSCT). This study evaluates AKI incidence, risk factors, renal trajectories, and survival impact in pediatric FA patients receiving uniform non-TBI conditioning.</p> Methods <p>This retrospective cohort study enrolled 37 pediatric patients (&lt; 18&#xa0;years) with confirmed FA who underwent allogeneic HSCT between April 2017 and June 2023 at Children's Medical Center, Tehran. AKI was defined and staged using KDIGO serum creatinine criteria, and renal function was tracked via estimated glomerular filtration rate (eGFR; bedside Schwartz equation).</p> Results <p>AKI developed in 13 of 37 patients (35.1%), mostly stage 1 (53.8%), with 92.3% of cases occurring within the first three months post-transplantation. Older age at HSCT (p = 0.023) and higher admission eGFR (p = 0.010) were associated with increased AKI risk. While binary CAKUT presence did not significantly correlate with AKI, subgroup analysis revealed single kidney or severe renal malrotation carried the highest risk. Longitudinal eGFR modelling showed a marked decline within one month post-HSCT and partial recovery by six months. Calcineurin inhibitor levels, conditioning intensity, GvHD grade, and CMV infection did not significantly correlate with AKI.</p> Conclusions <p>AKI is common, mostly mild, and predominantly occurs early after transplantation, highlighting the need for risk-stratified renal monitoring, tailored immunosuppressive management, and long-term nephrological follow-up in FA patients surviving HSCT.</p> Graphical abstract <p>A higher resolution version of the Graphical abstract is available as Supplementary information</p> <p></p>

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Early, frequent, yet reversible: acute kidney injury after hematopoietic stem cell transplantation in pediatric Fanconi anemia and long-term renal outcomes

  • 期刊论文

  • 发表日期：

    2026年6月16日

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  • Fahimeh Askarian,
  • Zahra Karimizadeh,
  • Amirali Kalantari,
  • Mahsa Mousakhan Bakhtiari,
  • Atieh Karimzadeh,
  • Mohammad Jahanpanah,
  • Shirin Eshghi,
  • Nyasha Sandaramu,
  • Leila Jafari,
  • Amir Ali Hamidieh,
  • Mohammad Taha Salmanifard Ardestani,
  • Maryam Behfar,
  • Farahnaz Pasha

摘要

Background

Acute kidney injury (AKI) is an understudied complication in pediatric Fanconi anemia (FA) patients undergoing hematopoietic stem cell transplantation (HSCT). This study evaluates AKI incidence, risk factors, renal trajectories, and survival impact in pediatric FA patients receiving uniform non-TBI conditioning.

Methods

This retrospective cohort study enrolled 37 pediatric patients (< 18 years) with confirmed FA who underwent allogeneic HSCT between April 2017 and June 2023 at Children's Medical Center, Tehran. AKI was defined and staged using KDIGO serum creatinine criteria, and renal function was tracked via estimated glomerular filtration rate (eGFR; bedside Schwartz equation).

Results

AKI developed in 13 of 37 patients (35.1%), mostly stage 1 (53.8%), with 92.3% of cases occurring within the first three months post-transplantation. Older age at HSCT (p = 0.023) and higher admission eGFR (p = 0.010) were associated with increased AKI risk. While binary CAKUT presence did not significantly correlate with AKI, subgroup analysis revealed single kidney or severe renal malrotation carried the highest risk. Longitudinal eGFR modelling showed a marked decline within one month post-HSCT and partial recovery by six months. Calcineurin inhibitor levels, conditioning intensity, GvHD grade, and CMV infection did not significantly correlate with AKI.

Conclusions

AKI is common, mostly mild, and predominantly occurs early after transplantation, highlighting the need for risk-stratified renal monitoring, tailored immunosuppressive management, and long-term nephrological follow-up in FA patients surviving HSCT.

Graphical abstract

A higher resolution version of the Graphical abstract is available as Supplementary information