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Atypical hemoglobin uremic syndrome

Douggl G N Bailey, Hans Fuchs, Roland Hentschel
BACKGROUND: Neonatal hyperbilirubinemia is influenced by a wide variety of factors, one of which is hemolysis. Serious hyperbilirubinemia may lead to a kernicterus with detrimental neurologic sequelae. Patients suffering from hemolytic disease have a higher risk of developing kernicterus. Carbon monoxide (CO), a byproduct of hemolysis or heme degradation, was described by Sjöstrand in the 1960s. It is transported as carboxyhemoglobin (COHb) and exhaled through the lungs. We were interested in a potential correlation between COHb and total serum bilirubin (TSB) and the time course of both parameters...
January 18, 2017: Journal of Perinatal Medicine
Fadi Fakhouri, Maryvonne Hourmant, Josep M Campistol, Spero R Cataland, Mario Espinosa, A Osama Gaber, Jan Menne, Enrico E Minetti, François Provôt, Eric Rondeau, Piero Ruggenenti, Laurent E Weekers, Masayo Ogawa, Camille L Bedrosian, Christophe M Legendre
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare genetic life-threatening disease of chronic uncontrolled complement activation leading to thrombotic microangiopathy (TMA) and severe end-organ damage. Eculizumab, a terminal complement inhibitor approved for aHUS treatment, was reported to improve hematologic and renal parameters in 2 prior prospective phase 2 studies. This is the largest prospective study of eculizumab in aHUS to date, conducted in an adult population. STUDY DESIGN: Open-label single-arm phase 2 trial...
July 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Johan Vande Walle, Yahsou Delmas, Gianluigi Ardissino, Jimmy Wang, John F Kincaid, Herman Haller
BACKGROUND: Eculizumab is approved for atypical hemolytic uremic syndrome (aHUS). Guidelines discuss the importance of prompt treatment. We report a post hoc analysis investigating the effect of baseline factors, including patient characteristics and time from the latest aHUS manifestation to eculizumab initiation, on change from baseline in estimated glomerular filtration rate (eGFR) and other outcomes. METHODS: Data were pooled from four phase 2, open-label, single-arm, prospective clinical studies of eculizumab for patients with aHUS...
March 19, 2016: Journal of Nephrology
K Anastaze Stelle, E Gonzalez, A Wilhelm-Bals, P-R Michelet, C M Korff, P Parvex
Hemolytic uremic syndrome (HUS) is rare in neonates. We report the case of atypical HUS (aHUS) revealed by neonatal seizures. This 18-day-old baby presented with repeated clonus of the left arm and eye deviation. Four days earlier, she had suffered from gastroenteritis (non-bloody diarrhea and vomiting without fever). Her work-up revealed hemolytic anemia (120 g/L), thrombocytopenia (78 g/L), and impaired renal function (serum creatinine=102 μmol/L) compatible with the diagnosis of HUS. Levels of C3 and C4 in the serum were normal...
March 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Claudia Iglesias Teixeira, Roberta Gonçalves Mota, Bruna Gabriela Vieira Afonso, Tatiana Vieira Carneiro, Geraldo Sérgio Gonçalves Meira, Divino Urias Mendonça
To report the use of Eculizumab in atypical hemolytic uremic syndrome (aHUS) after renal transplantation. A 16 year-old patient diagnosed with chronic kidney disease since 2010, due to aHUS, under dialysis. kidney transplantation by deceased donor: February/2012. She showed good clinical evolution until the 14th postoperative day, when he developed a fever, oliguria, worsening of renal function [serum creatinine (CRs): 4.0 mg/dl] and signs of hemolysis [platelets: 110,000 mm3; hemoglobin (Hb): 4.5 g/dL; LDH: 3366 U/L]...
January 2015: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
B Thajudeen, A Sussman, E Bracamonte
Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy (TMA) characterized by the triad of hemolytic anemia, thrombocytopenia, and acute renal failure. Eculizumab, a monoclonal complement C5 antibody which prevents the induction of the terminal complement cascade, has recently emerged as a therapeutic option for aHUS. We report a case of aHUS successfully treated with eculizumab. A 51-year-old male was admitted to the hospital following a mechanical fall. His past medical history was significant for rheumatic valve disease and mitral valve replacement; he was on warfarin for anticoagulation...
July 2013: Case Reports in Nephrology and Urology
Jeffrey Laurence
Atypical hemolytic uremic syndrome (aHUS) is a major thrombotic microangiopathy (TMA). A TMA is recognized by the laboratory signs of microangiopathic hemolysis, as indicated by schistocytes, elevated lactate dehydrogenase, low haptoglobin, and low hemoglobin, plus thrombocytopenia and accompanying signs and symptoms of organ system involvement. aHUS results from chronic, uncontrolled activity of the alternative complement pathway. In most patients, this defect is related to a genetic deficiency in one or more soluble and/or membrane-bound complement regulatory proteins...
October 2012: Clinical Advances in Hematology & Oncology: H&O
F Semsa Cayci, Nilgun Cakar, Veysel Sabri Hancer, Nermin Uncu, Banu Acar, Gokce Gur
BACKGROUND: Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in childhood. It usually occurs after a prodromal episode of diarrhea and it leads to significant morbidity and mortality during the acute phase. However, cases that start as diarrhea-positive HUS whose renal function fail to recover should be screened for genetic disorders of the complement system, which is called atypical HUS (aHUS). CASE-DIAGNOSIS/TREATMENT: We herein report a 10-year-old girl, who initially came with bloody diarrhea and had features of HUS with delayed renal and hematological recovery despite plasma therapy...
December 2012: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Gema Ariceta, Beatriz Arrizabalaga, Mireia Aguirre, Elvira Morteruel, Margarita Lopez-Trascasa
A 28-day-old male newborn weighing 3.6 kg was given a diagnosis of atypical hemolytic-uremic syndrome, new-onset thrombotic microangiopathy (TMA; hemoglobin, 7.7 g/dL; schistocytes, 9%), thrombocytopenia (platelets, 49 × 10(3)/μL [49 × 10(9)/L]), and acute kidney failure (serum creatinine, 1.13 mg/dL [99.8 μmol/L], corresponding to estimated glomerular filtration rate [eGFR] of 15 mL/min/1.73 m(2) [0.25 mL/s/1.73 m(2)]). Repeated high-volume plasma infusions were ineffective. Plasma exchange was attempted, but not tolerated...
May 2012: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Bérengère Koehl, Olivia Boyer, Nathalie Biebuyck-Gougé, Manoelle Kossorotoff, Véronique Frémeaux-Bacchi, Nathalie Boddaert, Patrick Niaudet
We report the case of a 4-year-old boy, diagnosed with atypical hemolytic uremic syndrome (HUS) due to a hybrid factor H. He progressed to end-stage renal failure despite plasmatherapy and underwent bilateral nephrectomy because of uncontrolled hypertension. Three days after, he had partial complex seizures with normal blood pressure, normal blood count and normal magnetic resonance imaging (MRI), which recurred 1 month later. Eight months later, he had a third episode of seizures, with hemoglobin of 10 g/dl without schizocytes, low haptoglobin of 0...
December 2010: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
A Y Elzouki, K Mirza, A Mahmood, A M Al-Sowailem
Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. There are two main subgroups: the typical form of HUS follows a diarrheal prodrome (D+HUS) and the atypical form is without the diarrheal prodrome (D-HUS). We have studied 28 children with HUS over a period of 15 months between 1992 and 1993. The median age was 2.2 years (range from six months to six years). All children had prodromal diarrhea. Hypertension was present in 71% and neurological complications in 39%...
March 1995: Annals of Saudi Medicine
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