1. Kidney complications in 107 Fanconi anemia patients submitted to hematopoietic cell transplantation
- Author
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Samantha Nichele, Adriana Mello, Lucimary de Castro Sylvestre, Fellype C. Barreto, Joanna Trennepohl, Tyane de Almeida Pinto Jardim, Mariana Faucz Munhoz da Cunha, Carmem Bonfim, Lisandro Ribeiro, Gisele Loth, Adriana Koliski, and Ricardo Pasquini
- Subjects
medicine.medical_specialty ,business.industry ,Urinary system ,Acute kidney injury ,Bone marrow failure ,Hematopoietic Stem Cell Transplantation ,Renal function ,Acute Kidney Injury ,medicine.disease ,Kidney ,Gastroenterology ,Transplantation ,Fanconi Anemia ,Fanconi anemia ,hemic and lymphatic diseases ,Internal medicine ,Pediatrics, Perinatology and Child Health ,medicine ,Humans ,business ,Complication ,Child ,Kidney disease ,Retrospective Studies - Abstract
Fanconi anemia (FA) is a rare disease characterized by progressive bone marrow failure, cancer predisposition, and multiple systemic malformations, including congenital abnormalities of the kidney and urinary tract (CAKUT). Hematopoietic cell transplantation (HCT), the only potentially curative treatment for the hematological complications of FA, may precipitate acute kidney injury (AKI) and hypertension. We retrospectively investigated 107 FA patients who underwent HCT between 2009 and 2017. We investigated the incidence and risk factors of AKI within 100 days after HCT in a cohort of FA patients, and kidney function and hypertension over 2-year follow-up.The incidence of AKI (mainly stage I) was 18.7%. Patients aged ≥ 11 years at transplantation showed a higher risk of AKI (OR 3.53). The eGFR was 60-90 mL/min/1.73 m2 in 53 (49.5%), 55 (51.4%), 50 (50.5%), 50 (51%), and 46 (59.7%) patients before HCT, at 100 days, 6 months, 1 year, and 2 years. Within the first 100 days after HCT, hypertension was observed in 72% of the patients and was associated with cyclosporine therapy. Most (62.3%) patients had stage 2 hypertension. CAKUT was observed in 33.7% of the patients and was associated with both hypertension (86%) and diminished kidney function but not with AKI.Conlusion: Although AKI, a commonly known HCT complication, was mild in this study, the prevalence of chronic kidney disease (CKD), as well as the high incidence of hypertension, specially associated with CAKUT point out the importance of kidney care in short and long-term follow up of FA patients. What is Known: • Fanconi anemia (FA) is the most frequent inherited bone marrow failure in children, and 30% of cases have congenital anomalies of kidney (CAKUT). • Acute kidney injury and hypertension after hematopoietic cell transplantation (HCT) may impact the outcomes.. What is New: • Despite the presence of CAKUT and stage 2 CKD in 33.7% and 50% of the patients, respectively, AKI was mild and transitory after HCT in FA patients. • CAKUT in FA patients was associated with lower kidney function and hypertension after HCT.
- Published
- 2021