Hemoglobin cast nephropathy: a rare but serious complication of hemolysis in a pediatric patient
Background. Intravascular hemolysis is a serious and rare condition in children and causes the release of hemoglobin and heme into circulation, which have proinflammatory properties. These substances lead to inflammation, oxidative stress, apoptosis, and organelle dysfunction that lead to acu...
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Hacettepe University Institute of Child Health
2023-10-01
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| Series: | The Turkish Journal of Pediatrics |
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| Online Access: | https://turkjpediatr.org/article/view/98 |
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| author | Demet Baltu Nihan Avcu Oral Selman Kesici Rezan Topaloğlu Osman İlhami Özcebe Tekin Aksu Diclehan Orhan Fatih Özaltın |
| author_facet | Demet Baltu Nihan Avcu Oral Selman Kesici Rezan Topaloğlu Osman İlhami Özcebe Tekin Aksu Diclehan Orhan Fatih Özaltın |
| author_sort | Demet Baltu |
| collection | DOAJ |
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Background. Intravascular hemolysis is a serious and rare condition in children and causes the release of hemoglobin and heme into circulation, which have proinflammatory properties. These substances lead to inflammation, oxidative stress, apoptosis, and organelle dysfunction that lead to acute kidney injury (AKI). We report a pediatric case diagnosed with hemolysis-associated hemoglobin cast nephropathy due to autoimmune hemolytic anemia.
Case. A 4-year-old boy, who was admitted to another hospital with complaints of fever and dark urine for one day, developed anemia and kidney failure in the follow-up, was referred to our hospital. In physical examination, pallor and icterus on the sclera were noted. The patient had low hemoglobin and haptoglobin levels concomitant with high levels of serum lactate dehydrogenase, urea and creatinine. A peripheral blood smear showed marked spherocytes without schistocytes. A kidney biopsy was performed due to ongoing overt hemolysis and dialysis requirement, which showed findings consistent with hemoglobin cast nephropathy. Although the initial polyspecific direct antiglobulin test (DAT) was negative, due to persistent intravascular hemolysis DAT was studied monospecifically and showed IgM antibody positivity. Therefore, a diagnosis of autoimmune hemolytic anemia was made, and corticosteroid treatment was started. Hemolysis immediately ceased and the need for erythrocyte transfusion and dialysis disappeared.
Conclusions. Acute kidney injury associated with hemoglobin cast nephropathy is an extremely rare condition in childhood. Although the initial course is severe and potentially life-threatening, the prognosis is favorable with the treatment of the underlying cause and management of AKI. Therefore, pediatricians should be aware of this rare clinical entity during clinical practice.
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| format | Article |
| id | doaj-art-5cfbba0d192046cfa8ae7f6de54903de |
| institution | OA Journals |
| issn | 0041-4301 2791-6421 |
| language | English |
| publishDate | 2023-10-01 |
| publisher | Hacettepe University Institute of Child Health |
| record_format | Article |
| series | The Turkish Journal of Pediatrics |
| spelling | doaj-art-5cfbba0d192046cfa8ae7f6de54903de2025-08-20T02:00:50ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212023-10-0165510.24953/turkjped.2022.1067Hemoglobin cast nephropathy: a rare but serious complication of hemolysis in a pediatric patientDemet Baltu0Nihan Avcu Oral1Selman Kesici2Rezan Topaloğlu3Osman İlhami Özcebe4Tekin Aksu5Diclehan Orhan6Fatih Özaltın7Division of Pediatric Nephrology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara.Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara.Division of Pediatric Intensive Care Unit, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara.Division of Pediatric Nephrology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara.Department of Internal Medicine, Hematology Unit, Hacettepe University Faculty of Medicine, Ankara.Division of Pediatric Hematology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara.Department of Pathology, Hacettepe University Faculty of Medicine, Ankara, Türkiye.Division of Pediatric Nephrology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara. Background. Intravascular hemolysis is a serious and rare condition in children and causes the release of hemoglobin and heme into circulation, which have proinflammatory properties. These substances lead to inflammation, oxidative stress, apoptosis, and organelle dysfunction that lead to acute kidney injury (AKI). We report a pediatric case diagnosed with hemolysis-associated hemoglobin cast nephropathy due to autoimmune hemolytic anemia. Case. A 4-year-old boy, who was admitted to another hospital with complaints of fever and dark urine for one day, developed anemia and kidney failure in the follow-up, was referred to our hospital. In physical examination, pallor and icterus on the sclera were noted. The patient had low hemoglobin and haptoglobin levels concomitant with high levels of serum lactate dehydrogenase, urea and creatinine. A peripheral blood smear showed marked spherocytes without schistocytes. A kidney biopsy was performed due to ongoing overt hemolysis and dialysis requirement, which showed findings consistent with hemoglobin cast nephropathy. Although the initial polyspecific direct antiglobulin test (DAT) was negative, due to persistent intravascular hemolysis DAT was studied monospecifically and showed IgM antibody positivity. Therefore, a diagnosis of autoimmune hemolytic anemia was made, and corticosteroid treatment was started. Hemolysis immediately ceased and the need for erythrocyte transfusion and dialysis disappeared. Conclusions. Acute kidney injury associated with hemoglobin cast nephropathy is an extremely rare condition in childhood. Although the initial course is severe and potentially life-threatening, the prognosis is favorable with the treatment of the underlying cause and management of AKI. Therefore, pediatricians should be aware of this rare clinical entity during clinical practice. https://turkjpediatr.org/article/view/98acute kidney injuryautoimmune hemolytic anemiahemoglobin cast nephropathyintravascular hemolysis |
| spellingShingle | Demet Baltu Nihan Avcu Oral Selman Kesici Rezan Topaloğlu Osman İlhami Özcebe Tekin Aksu Diclehan Orhan Fatih Özaltın Hemoglobin cast nephropathy: a rare but serious complication of hemolysis in a pediatric patient The Turkish Journal of Pediatrics acute kidney injury autoimmune hemolytic anemia hemoglobin cast nephropathy intravascular hemolysis |
| title | Hemoglobin cast nephropathy: a rare but serious complication of hemolysis in a pediatric patient |
| title_full | Hemoglobin cast nephropathy: a rare but serious complication of hemolysis in a pediatric patient |
| title_fullStr | Hemoglobin cast nephropathy: a rare but serious complication of hemolysis in a pediatric patient |
| title_full_unstemmed | Hemoglobin cast nephropathy: a rare but serious complication of hemolysis in a pediatric patient |
| title_short | Hemoglobin cast nephropathy: a rare but serious complication of hemolysis in a pediatric patient |
| title_sort | hemoglobin cast nephropathy a rare but serious complication of hemolysis in a pediatric patient |
| topic | acute kidney injury autoimmune hemolytic anemia hemoglobin cast nephropathy intravascular hemolysis |
| url | https://turkjpediatr.org/article/view/98 |
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