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Platelet transfusion in hemolytic uremic syndrome

Donavan de Souza Lúcio, Jacqueline Foelkel Pignatari, Marcelo Gil Cliquet, Henri Augusto Korkes
CONTEXT: Thrombotic microangiopathy syndrome or thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) describes distinct diseases sharing common pathological features: microangiopathic hemolytic anemia and thrombocytopenia, without any other apparent cause. CASE REPORT: An 18-year-old second-trimester primigravida presented with a history of fifteen days of intense weakness, followed by diarrhea over the past six days. She reported having had low platelets since childhood, but said that she had never had bleeding or menstrual abnormalities...
September 2017: São Paulo Medical Journal, Revista Paulista de Medicina
Jan Beneke, Aleksej Sartison, Jan T Kielstein, Hermann Haller, Martin Nitschke, Ulrich Kunzendorf, Sebastian Loos, Markus J Kemper, Rolf A K Stahl, Jan Menne
Recent studies suggest that platelet transfusions are harmful in patients with thrombotic thrombocytopenic purpura, an entity of thrombotic microangiopathies. As the typical or Shiga toxin-producing Escherichia coli-induced hemolytic uremic syndrome (STEC-HUS) is also classified as thrombotic microangiopathy, we complement these data with an analysis of 250 patients from the German O104:H4 STEC-HUS outbreak. The effect of platelet transfusion in 44 patients who received platelet transfusions vs 206 control patients was investigated...
January 2017: Transfusion Medicine Reviews
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
Peng Fu, Ai-hong Yuan, Chun-hua Wang, Xin Li, Hai-yang Wu
BACKGROUND: Acute kidney injury in patients with acute pancreatitis carries a poor prognosis. Hemolytic uremic syndrome (HUS) is characterized by non-immune hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney, and though rare in adults it is associated with high mortality and a high rate of chronic renal failure. CASE PRESENTATION: Herein, we report a case of alcohol-induced acute pancreatitis in a 38-year-old Chinese female complicated by HUS...
2014: BMC Nephrology
Shan-shan Meng, Qing Yang, Guo-qiang Han, Jin-hong Yang, Hai-lin Zhang, Le-ping Ye, Yun-chun Luo, Chang-chong Li
OBJECTIVE: To study the clinical characteristics of Streptococcus pneumonia-associated hemolytic uremic syndrome (SP-HUS) in children. METHOD: Clinical and laboratory data of a pediatric case of SP-HUS were retrospectively analyzed and the key points of diagnosis and therapy were reviewed. RESULT: An 18-month old girl was admitted with chief complaint of fever and cough for 5 days combined with mild labored breath. Breath sound was found weakened in right lung with lower lobe dullness on percussion...
July 2013: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Mélanie Rinaudo-Gaujous, Emilie Talagrand, Paul O Verhoeven, Olivier Garraud, Françoise Flourié
Pediatric hemolytic uremic syndrome (HUS) is a rare complication of infections usually caused by Escherichia coli; Streptococcus pneumoniae may be a causative agent in 5% of cases and is often more serious in terms of morbidity and mortality. We report a case of pediatric HUS following an infection by a serotype of S. pneumoniae not included in the vaccine administered to the child. Bacterial neuraminidase revealed a T-antigen and a Tk-antigen and red blood cells polyagglutinability in the laboratory test. Transfusion has been reoriented by an indication of secondary preparations: deplasmatisation of red blood cells and platelets and abstention of therapeutic plasma administration...
March 2013: Annales de Biologie Clinique
Alejandro Balestracci, Sandra Mariel Martin, Ismael Toledo, Caupolican Alvarado, Raquel Eva Wainsztein
BACKGROUND: Platelet transfusions should be avoided in children with post-diarrheal hemolytic uremic syndrome (D + HUS) because they might increase microthrombi formation, thereby aggravating the disease. As this possibility has not yet been explored, we investigated whether platelet transfusion in patients with D + HUS would lead to a worse disease course compared to that in patients who did not receive platelet transfusion. METHODS: This was a case-control study in which data from D + HUS children who received platelet transfusions (cases, n  =  23) and those who did not (controls, n  =  54) were retrospectively reviewed and compared...
June 2013: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
L Barry Seltz, Leigh Anne Bakel, Jennifer Tiehen, Dexiang Gao, Melissa A Cadnapaphornchai, Gary Lum, Douglas Ford
INTRODUCTION: Hemolytic uremic syndrome is a thrombotic microangiopathy. Clopidogrel, a recently developed platelet aggregation inhibitor, has not been previously reported as a treatment for this illness. Our study's objective was to explore the efficacy and safety of clopidogrel in children with diarrhea associated hemolytic uremic syndrome. MATERIALS AND METHODS: We performed a retrospective chart review of all children (≤ 18 years) hospitalized with diarrhea associated hemolytic uremic syndrome...
September 2012: Thrombosis Research
Chad S Kessler, Bilal A Khan, Katie Lai-Miller
BACKGROUND: Thrombotic thrombocytopenic purpura is a hematological emergency and diagnostic challenge. The critical determinant of outcome is timely diagnosis and treatment. OBJECTIVES: Describe the pathophysiology, presentation, diagnosis, and treatment of thrombotic thrombocytopenic purpura. DISCUSSION: Thrombotic thrombocytopenic purpura has a varied presentation and a tendency to mimic several disorders. However, it may be at least provisionally diagnosed in the patient with thrombocytopenia and microangiopathic hemolytic anemia without alternate cause...
September 2012: Journal of Emergency Medicine
Alisa A Acosta, Ronald J Hogg
BACKGROUND: Acute kidney injury (AKI) with elevated serum uric acid (UA) levels has been reported in patients with hemolytic uremic syndrome (HUS). AKI is thought to result from tubular obstruction by UA crystals. Inducing a diuresis may ameliorate the oligoanuria in such patients. We describe a child with HUS in whom reducing UA with fluids and rasburicase appeared to accelerate the recovery of renal function. CASE-DIAGNOSIS/TREATMENT: A 9-month-old Caucasian male infant presented with 6 days of diarrhea, 3 days of vomiting, and 24 h of oliguria...
February 2012: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Sadaya Matano, Katsuhisa Inamura, Michio Konishi, Toshiya Okumura, Hiroshi Kawai, Toshiyuki Okamura, Yoshiko Takata, Keiko Yamada, Misato Obata, Hajime Nagata, Yoshiko Muramoto, Tatsuho Sugimoto
An outbreak of enterohemorrhagic Escherichia coli (EHEC) occurred in Toyama and other prefectures in Japan during 2011. Some patients, including adults, showed complications such as encephalopathy, disseminated intravascular coagulation, and hemolytic-uremic syndrome, and the disease course was extremely aggressive. This report describes the clinical features of four patients infected with Escherichia coli (E. coli) O111 who developed very severe to fatal complications. The initial symptoms in all patients included abdominal pain, diarrhea, and bloody stools, and neurological abnormalities started to appear from 1 to 3 days after admission...
August 2012: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
Alex Koyfman, Elizabeth Brém, Vincent W Chiang
In 1924, Dr Eli Moschcowitz described a 16-year-old adolescent girl with abrupt onset of petechiae, hemolytic anemia, followed by paralysis, coma, and death. Autopsy showed widespread hyaline thrombi in the terminal arterioles and capillaries of various organs. The syndrome described by Moschowitz is now known as thrombotic thrombocytopenic purpura.
November 2011: Pediatric Emergency Care
Dániel Deme, Márton Ragán, Katalin Kalmár, Lajos Kovács, Erzsébet Varga, Tünde Varga, Ervin Rakonczai
Disseminated intravascular coagulopathy (DIC) is characterized as activation of the clotting system resulting in fibrin thrombi, gradually diminishing levels of clotting factors with increased risk of bleeding. Basically two types of DIC are distinguished: (1) chronic (compensated) - with alteration of laboratory values and (2) acute (non-compensated) - with severe clinical manifestations: bleeding, shock, acute renal failure (ARF), transient focal neurologic deficit, delirium or coma. Chronic DIC related to metastatic neoplasia is caused by pancreatic, gastric or prostatic carcinoma in most of the cases...
December 2010: Magyar Onkologia
J W Oliver, R S Akins, M K Bibens, D M Dunn
Thrombotic microangiopathies are disorders resulting from platelet thromboses forming in the microvasculature with resultant schistocyte forms. Hemolytic uremic syndrome (HUS) is a microangiopathic hemolytic anemia often complicated by acute renal failure in children. HUS is typically caused by bacterial infection, most commonly enterohemorrhagic Escherichia coli. Neuraminidase-producing organisms, such as Streptococcus pneumoniae have also been reported as potential etiologies. The pathogenesis in these cases involves cleavage of sialic acid residues from the surfaces of erythrocytes, platelets, and glomerular capillary endothelial cells, exposing the Thomsen-Friedenreich antigen, a process known as T-activation...
2010: Clinical Medicine Insights. Pathology
Maria Goretti Polito, Gianna Mastroianni Kirsztajn
Thrombotic microangiopathies (TMAs) are pathological conditions characterized by generalized microvascular occlusion by platelet thrombi, thrombocytopenia, and microangiopathic hemolytic anemia. Two typical phenotypes of TMAs are hemolytic- uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Other disorders occasionally present with similar manifestations. Depending on whether renal or brain lesions prevail, two pathologically indistinguishable but somehow clinically different disorders have been described: HUS and TTP...
July 2010: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
James N George
Thrombotic thrombocytopenic purpura (TTP) is the common name for adults with microangiopathic hemolytic anemia, thrombocytopenia, with or without neurologic or renal abnormalities, and without another etiology; children without renal failure are also described as TTP. The diagnosis of TTP is an indication for plasma exchange treatment, but beginning treatment requires sufficient confidence in the diagnosis to justify the risk of plasma exchange complications. Documentation of a severe deficiency of plasma ADAMTS13 activity, defined as less than 10% of normal, is not essential for the diagnosis of TTP...
November 18, 2010: Blood
T Said, T Al-Otaibi, S Al-Wahaib, I Francis, M P Nair, M A Halim, A El-Sayed, M R N Nampoory
INTRODUCTION: Calcineurin inhibitor (CNI) induced HUS, although rare, can be a serious complication of renal transplantation. Classical syndrome of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal injury may not be fully manifested. METHODS: We retrospectively analyzed our data in 950 kidney recipients under follow-up in our center (1994-2008). We reviewed the kidney biopsies performed for these patients to exclude conflicting diagnoses like antibody mediated rejection...
April 2010: Transplantation Proceedings
Brent R Weil, Sharon P Andreoli, Deborah F Billmire
Children with hemolytic uremic syndrome (HUS) frequently develop acute kidney injury (AKI) requiring renal replacement therapy (RRT). Peritoneal dialysis (PD) is commonly used. Despite high rates of thrombocytopenia, there is concern that platelet transfusions may worsen HUS by exacerbating microthrombi formation. We evaluated bleeding risk for PD catheter placement with or without central venous catheter (CVC) placement in children with HUS. Records from 1998 to 2007 were searched. Data regarding patient demographics, PD catheter placement, CVC placement, occurrence of procedure-associated bleeding, and time from insertion to removal of PD catheter were collected...
September 2010: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Yoshihiro Fujimura, Masanori Matsumoto
BACKGROUND: Thrombotic microangiopathies (TMAs) are pathological conditions characterized by generalized microvascular occlusion by platelet thrombi, thrombocytopenia, and microangiopathic hemolytic anemia. Two typical phenotypes of TMAs are hemolytic-uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Severe deficiency of plasma ADAMTS13 activity (ADAMTS13: AC) is more specific for TTP, but not for HUS. Since 1998, our laboratory has functioned as a nationwide referral center for TMAs by analyzing ADAMTS13...
2010: Internal Medicine
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