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uremic hemolytic syndrom

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https://www.readbyqxmd.com/read/29331477/atypical-presentation-of-pregnancy-related-hemolytic-uremic-syndrome
#1
Salim Baghli, Catherine Abendroth, Umar Farooq, Jennifer A Schaub
The cause of acute kidney injury during pregnancy and in the postpartum period can be particularly challenging to diagnose, especially when it is necessary to differentiate among preeclampsia; eclampsia; hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome; and thrombotic microangiopathies (TMAs). All these disease entities can present with kidney failure, microangiopathic hemolytic anemia, and thrombocytopenia. We present a teaching case of atypical hemolytic uremic syndrome in the postpartum period in a young woman who was found to have mutations of uncertain clinical significance in the complement cascade, including in C3, CFH, and CFI...
January 10, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29328045/assessment-of-the-level-of-knowledge-of-medical-personnel-in-diarrhea-and-hemolytic-uremic-syndrome
#2
O Chokoshvili, N Vepkhvadze, T Tsertsvadze, T Gabrichidze, M Khorbaladze
Survey have been conducted among medical professionals to test knowledge level of HUS and diarrheal diseases and to identify predictor variables for better knowledge. Cross-sectional survey have been conducted among medical personnel at different clinics in Tbilisi and in regions of Georgia. Participants were selected from different clinics in Tbilisi and in three biggest regional cities (Zugdidi, Batumi and Kutaisi) of Georgia. A total of 12 clinics were selected from them 6 were in Tbilisi and 2 at each regional cities...
December 2017: Georgian Medical News
https://www.readbyqxmd.com/read/29327071/diseases-of-complement-dysregulation-an-overview
#3
REVIEW
Edwin K S Wong, David Kavanagh
Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation. Although complement overactivation is common to all, cell surface alternative pathway dysregulation (aHUS), fluid phase alternative pathway dysregulation (C3G), or terminal pathway dysregulation (PNH) predominates resulting in the very different phenotypes seen in these diseases. The mechanism underlying the dysregulation also varies with predominant acquired autoimmune (C3G), somatic mutations (PNH), or inherited germline mutations (aHUS) predisposing to disease...
January 11, 2018: Seminars in Immunopathology
https://www.readbyqxmd.com/read/29326853/purtscher-like-retinopathy-associated-with-atypical-hemolytic-uremic-syndrome
#4
Melih Ustaoğlu, Feyza Önder, Nilgün Solmaz, Savaş Öztürk, Mesut Ayer
A 25-year-old woman presented with acute bilateral blurred vision and history of headache, dizziness, and syncope for three days. Her visual acuity was 20/60 in both eyes. Fundoscopy revealed multiple bilateral peripapillary yellow-white patches like cotton wool spots, intraretinal hemorrhages and macular edema. The patient was diagnosed with Purtscher-like retinopathy based on the retinal findings and lack of trauma history. She was urgently admitted to the nephrology clinic due to thrombotic microangiopathy findings (hemoglobinemia, thrombocytopenia, and acute renal failure)...
December 2017: Turkish Journal of Ophthalmology
https://www.readbyqxmd.com/read/29322327/combined-and-sequential-liver-kidney-transplantation-in-children
#5
Ryszard Grenda, Piotr Kaliciński
Combined and sequential liver-kidney transplantation (CLKT and SLKT) is a definitive treatment in children with end-stage organ failure. There are two major indications: - terminal insufficiency of both organs, or - need for transplanting new liver as a source of lacking enzyme or specific regulator of the immune system in a patient with renal failure. A third (uncommon) option is secondary end-stage renal failure in liver transplant recipients. These three clinical settings use distinct qualification algorithms...
January 10, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29301877/draft-genome-sequences-of-enterohemorrhagic-and-enteropathogenic-escherichia-coli-strains-isolated-from-alpacas-in-peru
#6
Lenin Maturrano, Marjorie Aleman, Dennis Carhuaricra, Jorge Maximiliano, Juan Siuce, Luis Luna, Raul Rosadio
The draft genome sequences of two strains of Escherichia coli, isolated from alpacas in Peru, are reported here. ECA1 has been determined to be a strain of enterohemorrhagic E. coli and ECB1 a strain of enteropathogenic E. coli These pathogens are responsible for hemolytic-uremic syndrome in humans and diarrhea in different mammals, respectively.
January 4, 2018: Genome Announcements
https://www.readbyqxmd.com/read/29288280/is-eculizumab-efficacious-in-shigatoxin-associated-hemolytic-uremic-syndrome-a-narrative-review-of-current-evidence
#7
REVIEW
Werner Keenswijk, Ann Raes, Johan Vande Walle
Severe complications due to Shigatoxin-associated hemolytic uremic syndrome (STEC-HUS) currently present a serious challenge since no specific treatment for this condition is available. Eculizumab, a terminal complement inhibitor, has been used especially in STEC-HUS patients with severe neurological involvement, but the efficacy remains undetermined. In order to determine its efficacy, we searched the databases Pubmed, Web of Science, Embase, and LiLACS for reports describing outcomes of eculizumab administration in STEC-HUS...
December 29, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29282226/maternal-and-fetal-outcomes-of-pregnancies-in-women-with-atypical-hemolytic-uremic-syndrome
#8
Martina Gaggl, Christof Aigner, Dorottya Csuka, Ágnes Szilágyi, Zoltán Prohászka, Renate Kain, Natalja Haninger, Maarten Knechtelsdorfer, Raute Sunder-Plassmann, Gere Sunder-Plassmann, Alice Schmidt
Atypical HUS (aHUS) is a disorder most commonly caused by inherited defects of the alternative pathway of complement, or the proteins that regulate this pathway, and life-threatening episodes of aHUS can be provoked by pregnancy. We retrospectively and prospectively investigated 27 maternal and fetal pregnancy outcomes in 14 women with aHUS from the Vienna Thrombotic Microangiopathy Cohort. Seven pregnancies (26%) were complicated by pregnancy-associated aHUS (p-aHUS), of which three appeared to be provoked by infection, bleeding, and curettage, and three individuals were considered to have preeclampsia/HELLP syndrome before the definitive diagnosis of p-aHUS was made...
December 27, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29280264/anti-shigatoxin-2-antibodies-in-ehec-o104-h4-infected-patients-may-predict-hemolytic-uremic-syndrome
#9
Werner Dammermann, Valentin Mihajlov, Barbara Middendorf, Alexander Mellmann, Helge Karch, Stefan Lüth, Sebastian Ullrich
BACKGROUND AND AIM: An outbreak of Shigatoxin 2 (Stx2) producing enterohemorrhagic and enteroaggregative E.coli (EHEC) O104:H4 infection in May 2011 in Germany caused enterocolitis and an unprecedented high 22% rate of hemolytic uremic syndrome (HUS). We hypothesized that anti-Stx2 IgM or IgG titers might predict HUS development. METHODS: 32 patients infected with EHEC O104:H4 (HUS: n=23;non-HUS: n=9) were retrospectively screened for anti-Stx2 IgM/IgG and matched with clinical data regarding HUS development, fever, superinfection, dialysis, neurological symptoms, intensive care, antibiotic treatment, and plasmapheresis...
December 27, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29260749/-difficulties-in-the-diagnosis-of-diarrhea-associated-hemolytic-uremic-syndrome-in-adults
#10
V A Malov, V V Maleev, N L Kozlovskaya, N A Tsvetkova, S V Smetanina, A N Gorobchenko, V V Serova, V B Chentsov, A G Volkov, A P Faller
Hemolytic uremic syndrome (HUS) is a rare, but menacing condition registered mainly in children. The paper gives a detailed description and analysis of a clinical case of HUS with a favorable outcome in an adult woman who developed the syndrome in the presence of bloody diarrhea. It considers an update on the etiology, pathogenesis, and clinical features of HUS associated with diarrheal syndrome and discusses differential diagnostic features, diagnostic problems, and characteristics of management tactics for patients...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/29250893/atypical-hemolytic-uremic-syndrome-a-meta-analysis-of-case-reports-confirms-the-prevalence-of-genetic-mutations-and-the-shift-of-treatment-regimens
#11
Vinod Krishnappa, Mohit Gupta, Mohamed Elrifai, Bahar Moftakhar, Michael J Ensley, Tushar J Vachharajani, Sidharth Kumar Sethi, Rupesh Raina
Atypical hemolytic uremic syndrome (aHUS) is a rare life-threatening thrombotic microangiopathy (TMA) affecting multiple organ systems. Recently, aHUS has been shown to be associated with uncontrolled complement activation due to mutations in the alternative pathway of complement components paving the way for targeted drug therapy. By meta-analysis of case reports, we discuss the impact of new treatment strategies on the resolution time of aHUS symptoms and mortality, and the distribution of genetic mutations...
December 17, 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/29248444/excitotoxicity-in-encephalopathy-associated-with-stec-o-157-infection
#12
Shigenobu Ishida, Kumi Yasukawa, Mai Koizumi, Katsuhiro Abe, Nozomi Hirai, Takafumi Honda, Hiroshi Sakuma, Hiroko Tada, Jun-Ichi Takanashi
Cytokines play an important role in the pathogenesis of the severe complications of Shiga toxin-producing Escherichia coli (STEC) infection, such as hemolytic uremic syndrome (HUS) and acute encephalopathy. A 3-year-old boy with acute encephalopathy associated with STEC O-157 HUS showed increased levels of IL-6 and IL-10, which normalized after methylprednisolone pulse therapy, and additionally exhibited a transient increase of glutamine on MR spectroscopy. This finding suggests that excitotoxicity, in addition to hypercytokinemia, may play an important role in the pathogenesis of HUS encephalopathy...
December 13, 2017: Brain & Development
https://www.readbyqxmd.com/read/29248304/glucose-6-phosphate-dehydrogenase-deficiency-mimicking-atypical-hemolytic-uremic-syndrome
#13
Patrick R Walsh, Sally Johnson, Vicky Brocklebank, Jacobo Salvatore, Martin Christian, David Kavanagh
A 4-year-old boy presented with nonimmune hemolysis, thrombocytopenia, and acute kidney injury. Investigations for an underlying cause failed to identify a definitive cause and a putative diagnosis of complement-mediated atypical hemolytic uremic syndrome (aHUS) was made. The patient was started initially on plasma exchange and subsequently eculizumab therapy, after which his kidney function rapidly improved. While on eculizumab therapy, despite adequate complement blockade, he presented 2 more times with hemolytic anemia and thrombocytopenia, but without renal involvement...
December 13, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29243465/atypical-hemolytic-uremic-syndrome
#14
Kati Kaartinen, Leena Martola, Seppo Meri
Atypical hemolytic-uremic syndrome (aHUS) is a rare form of thrombotic microagiopathy caused dysregulation of the alternative pathway of the complement resulting in tissue. In aHUS, activation of the alternative pathway of the complement is in an aberrant way directed against endothelial cells and blood cells. This is either due to a mutation in a complement factor, most commonly factor H, or an autoantibody against a complement regulator. In some patients the underlying disorder is not identified despite thorough examinations...
2017: Duodecim; Lääketieteellinen Aikakauskirja
https://www.readbyqxmd.com/read/29241200/atypical-hemolytic-uremic-syndrome-associated-with-complement-factor-h-mutation-and-iga-nephropathy-a-case-report-successfully-treated-with-eculizumab
#15
Hironori Nakamura, Mariko Anayama, Mutsuki Makino, Yasushi Makino, Katsuhiko Tamura, Masaki Nagasawa
We present a rare case of IgA nephropathy in a patient who developed atypical hemolytic uremic syndrome (aHUS) associated with a complement factor H (CFH) gene mutation, and who was successfully treated with eculizmab. A 76-year-old man was admitted as the patients had thrombotic microangiopathies findings. The patient was treated with plasma exchange, hemodialysis and methylprednisolone. A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 level was not decreased. Light microscopy findings were consistent with hemolytic uremic syndrome and immunofluorescence analysis revealed IgA and C3 were detected...
December 14, 2017: Nephron
https://www.readbyqxmd.com/read/29226095/atypical-hemolytic-uremic-syndrome-due-to-complement-factor-i-mutation
#16
Abdullah H Almalki, Laila F Sadagah, Mohammed Qureshi, Hatim Maghrabi, Abdulrahman Algain, Ahmed Alsaeed
Atypical hemolytic-uremic syndrome (aHUS) is a rare disease of complement dysregulation leading to thrombotic microangiopathy (TMA). Renal involvement and progression to end-stage renal disease are common in untreated patients. We report a 52-year-old female patient who presented with severe acute kidney injury, microangiopathic hemolytic anemia, and thrombocytopenia. She was managed with steroid, plasma exchange, and dialysis. Kidney biopsy shows TMA and renal cortical necrosis. Genetic analysis reveals heterozygous complement factor I (CFI) mutation...
November 6, 2017: World Journal of Nephrology
https://www.readbyqxmd.com/read/29222317/thrombocytopenia-in-hospitalized-patients-approach-to-the-patient-with-thrombotic-microangiopathy
#17
REVIEW
Marie Scully
Thrombotic microangiopathies (TMAs), specifically, thrombotic thrombocytopenic purpura (TTP) and complement-mediated hemolytic uremic syndrome (CM-HUS) are acute life-threatening disorders that require prompt consideration, diagnosis, and treatment to improve the high inherent mortality and morbidity. Presentation is with microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) and variable organ symptoms resulting from microvascular thrombi. Neurological and cardiac involvement is most common in TTP and associated with poorer prognosis and primarily renal involvement in CM-HUS...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222249/thrombocytopenia-in-pregnancy
#18
REVIEW
Douglas B Cines, Lisa D Levine
Thrombocytopenia develops in 5% to 10% of women during pregnancy or in the immediate postpartum period. A low platelet count is often an incidental feature, but it might also provide a biomarker of a coexisting systemic or gestational disorder and a potential reason for a maternal intervention or treatment that might pose harm to the fetus. This chapter reflects our approach to these issues with an emphasis on advances made over the past 5 to 10 years in understanding and managing the more common causes of thrombocytopenia in pregnancy...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29218045/factor-h-c-terminal-domains-are-critical-for-regulation-of-platelet-granulocyte-aggregate-formation
#19
Adam Z Blatt, Gurpanna Saggu, Claudio Cortes, Andrew P Herbert, David Kavanagh, Daniel Ricklin, John D Lambris, Viviana P Ferreira
Platelet/granulocyte aggregates (PGAs) increase thromboinflammation in the vasculature, and PGA formation is tightly controlled by the complement alternative pathway (AP) negative regulator, Factor H (FH). Mutations in FH are associated with the prothrombotic disease atypical hemolytic uremic syndrome (aHUS), yet it is unknown whether increased PGA formation contributes to the thrombosis seen in patients with aHUS. Here, flow cytometry assays were used to evaluate the effects of aHUS-related mutations on FH regulation of PGA formation and characterize the mechanism...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29216383/genetic-susceptibility-to-hemolytic-uremic-syndrome-after-shiga-toxin-producing-escherichia-coli-stec-infection-a-centers-for-disease-control-and-prevention-cdc-foodnet-study
#20
Asha R Kallianpur, Yuki Bradford, Rajal K Mody, Katie N Garman, Nicole Comstock, Sarah L Lathrop, Carol Lyons, Amy Saupe, Katie Wymore, Jeffrey A Canter, Lana M Olson, Amanda Palmer, Timothy F Jones
Background: Post-diarrheal hemolytic-uremic syndrome (D+HUS) following Shiga toxin-producing Escherichia coli (STEC) infection is a serious condition lacking specific treatment. Host immune dysregulation and genetic susceptibility to complement hyperactivation are implicated in non-STEC-related HUS. However, genetic susceptibility to D+HUS remains largely uncharacterized. Methods: Patients with culture-confirmed STEC diarrhea, identified through the CDC FoodNet surveillance system (2007-2012), were serotyped and classified by laboratory and/or clinical criteria as suspected, probable, or confirmed D+HUS, or as controls and genotyped at 200 loci linked to non-diarrheal HUS or similar pathologies...
December 6, 2017: Journal of Infectious Diseases
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