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Management of pediatric hemolytic uremic syndrome
Turk J Pediatr. 2024;66(1):1-16. doi: 10.24953/turkjped.2023.596.ABSTRACTClassical clinical triad of hemolytic uremic syndrome (HUS) is microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury associated with endothelial cell injury. Several situations, including infections, medications, malignancies, and transplantation can trigger endothelial damage. On the HUS spectrum, atypical hemolytic uremic syndrome (aHUS) deserves special attention in pediatric patients, as it can cause endstage kidney disease and mortality. A dysfunction in the alternative complement pathway, either acquired or genetic, has be...
Source: The Turkish Journal of Pediatrics - March 25, 2024 Category: Pediatrics Authors: Bora G ülhan Fatih Özaltın Kibriya Fidan Zeynep Birsin Özçakar O ğuz Söylemezoğlu Source Type: research

Use of eculizumab in pediatric patients with high-risk transplant-associated thrombotic microangiopathy: outcomes and risk factors associated with response and survival. A retrospective study on behalf of the Spanish Group for Hematopoietic Transplantation and Cellular Therapy (GETH-TC)
Transplant-associated thrombotic microangiopathy (TA-TMA) is a major complication of hematopoietic stem cell transplantation (HSCT) associated with high morbidity and mortality1 –3. It is characterized by an endothelial damage consisting of microangiopathic hemolytic anemia and microvascular thrombosis, which ends up causing tissue ischemia and organ damage 4–7. In TA-TMA, the complement activation and endothelial damage are triggered by multiple insults after transplan tation: chemotherapy, total body irradiation (TBI), human leukocyte antigen (HLA) mismatch, calcineurin inhibitors (CNIs), graft versus host disease (G...
Source: Biology of Blood and Marrow Transplantation - March 21, 2024 Category: Hematology Authors: MI Ben ítez-Carabante, D Bueno, L Alonso García, I López Torija, J Marsal, Fernandez NavarroJM, ML Uria Oficialdegui, M Panesso, B Molina, C Beléndez Bieler, P Palomo, A Pérez Martínez, C Diaz-de-Heredia Source Type: research

Use of Eculizumab in Pediatric Patients with High-Risk Transplantation-Associated Thrombotic Microangiopathy: Outcomes and Risk Factors Associated with Response and Survival. A Retrospective Study on Behalf of the Spanish Group for Hematopoietic Transplantation and Cellular Therapy (GETH-TC)
Transplantation-associated thrombotic microangiopathy (TA-TMA) is a major complication of hematopoietic stem cell transplantation (HSCT) associated with high morbidity and mortality [1 –3]. TA-TMA is characterized by endothelial damage consisting of microangiopathic hemolytic anemia and microvascular thrombosis, leading to tissue ischemia and organ damage [4–7]. In TA-TMA, complement activation and endothelial damage can be triggered by multiple insults after transplantation, including chemotherapy, total body irradiation (TBI), HLA mismatch, calcineurin inhibitor (CNI) therapy, graft-versus-host disease (GVHD), and vi...
Source: Biology of Blood and Marrow Transplantation - March 21, 2024 Category: Hematology Authors: MI Ben ítez-Carabante, D. Bueno, L. Alonso García, I. López Torija, J. Marsal, JM Fernandez Navarro, ML Uria Oficialdegui, M. Panesso, B. Molina, C. Beléndez Bieler, P. Palomo, A. Pérez Martínez, C. Diaz-de-Heredia Tags: Full Length Article Source Type: research

Microangiopathic Haemolytic Anaemia in Metastatic Angiosarcoma: A Grave Marker of Bone Marrow Infiltration
(Source: Indian Journal of Hematology and Blood Transfusion)
Source: Indian Journal of Hematology and Blood Transfusion - March 14, 2024 Category: Hematology Source Type: research

STEC colitis mimicking acute severe colitis with life-threatening consequences: a case report
We present a patient with a medical history of refractory ulcerative colitis, where making the diagnosis of STEC-HUS was challenging since the initial clinical presentation was difficult to differentiate from a flare of ulcerative colitis. This case illustrates that STEC induced colitis can mimic acute severe ulcerative colitis. This finding is of utmost clinical importance because of the potential life-threatening complications of STEC-HUS. Therefore it should be excluded promptly in patients with acute severe ulcerative colitis by using multiplex-PCR assay on a faecal sample.PMID:38431789 | DOI:10.51821/87.1.11652 (Sourc...
Source: Acta Gastro-Enterologica Belgica - March 3, 2024 Category: Gastroenterology Authors: T Hendrickx M Peetermans A D'Hoore K Claes A Van Hootegem J Sabino Source Type: research