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  1. Article: Atypical Hemolytic Uremic Syndrome: A Brief Review.

    Zhang, Kuixing / Lu, Yuxin / Harley, Kevin T / Tran, Minh-Ha

    Hematology reports

    2017  Volume 9, Issue 2, Page(s) 7053

    Abstract: Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad ... of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include ...

    Abstract Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majority of aHUS is caused by complement system defects impairing ordinary regulatory mechanisms. Activating events therefore lead to unbridled, ongoing complement activity producing widespread endothelial injury. Pathologic mutations include those resulting in loss-of-function in a complement regulatory gene (
    Language English
    Publishing date 2017-06-01
    Publishing country Italy
    Document type Journal Article ; Review
    ZDB-ID 2586645-X
    ISSN 2038-8330 ; 2038-8322
    ISSN (online) 2038-8330
    ISSN 2038-8322
    DOI 10.4081/hr.2017.7053
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Pregnancy-associated atypical hemolytic uremic syndrome. Case report.

    Barrera-Hoffmann, Christopher / Mariaca-Ortíz, Yadira / Ruiz-Villa, Josué Giovani / Cuevas-Cruz, Lesllie Eugenia / López-Mendoza, María Del Rosario / Briones-Garduño, Jesús Carlos

    The journal of obstetrics and gynaecology research

    2024  

    Abstract: Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare disease. There are only ... sessions and eculizumab. We present here a brief review of the case since it is an entity that needs to be ... characterized for microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction. We report the case ...

    Abstract Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare disease. There are only few reports in the literature, and most are in the puerperium period. It is a thrombotic microangiopathy (TMA) characterized for microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction. We report the case of a pregnant patient at 26.3 gestation weeks, who developed clinical features of TMA, neurological alterations, and septic shock; then after fetus and placental delivery, no clinical improvement was observed; a diagnostic protocol was performed due to suspicion of P-aHUS, showing improvement after the plasma exchange sessions and eculizumab. We present here a brief review of the case since it is an entity that needs to be suspected during pregnancy when TMA features and requires an immediate diagnosis to provide timely treatment.
    Language English
    Publishing date 2024-05-09
    Publishing country Australia
    Document type Case Reports
    ZDB-ID 1327307-3
    ISSN 1447-0756 ; 1341-8076
    ISSN (online) 1447-0756
    ISSN 1341-8076
    DOI 10.1111/jog.15958
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1810: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  3. Article ; Online: PURTSCHER-LIKE RETINOPATHY ASSOCIATED WITH ATYPICAL HEMOLYTIC UREMIC SYNDROME: CASE REPORT AND REVIEW OF OUTCOMES.

    Gange, William S / Haghighi, Arezu / Toy, Brian C

    Retinal cases & brief reports

    2021  Volume 17, Issue 2, Page(s) 154–159

    Abstract: ... diagnosed atypical hemolytic uremic syndrome (aHUS) and Purtscher-like retinopathy.: Methods: This is ... an observational case report and review of literature. A 38-year-old woman presented with 3 months of rashes ... fevers, arthralgias, and abdominal pain. Initial workup was suggestive of hypereosinophilic syndrome or ...

    Abstract Purpose: The purpose of this study was to report the case of a patient presenting with newly diagnosed atypical hemolytic uremic syndrome (aHUS) and Purtscher-like retinopathy.
    Methods: This is an observational case report and review of literature. A 38-year-old woman presented with 3 months of rashes, fevers, arthralgias, and abdominal pain. Initial workup was suggestive of hypereosinophilic syndrome or adult-onset Still's disease. The patient developed acute renal failure and progressively blurry vision bilaterally over the course of 5 days. Funduscopic examination was notable for numerous Purtscher flecken and cotton-wool spots, with rare intraretinal hemorrhages at the posterior pole. The constellation of renal failure, hemolytic anemia, and thrombocytopenia prompted a workup for thrombotic microangiopathy that was remarkable for a mutation in the gene coding for complement protein C9.
    Results: The patient was diagnosed with aHUS and treated with intravenous pulse dose steroids for 3 days and an extended course of eculizumab. The patient's renal failure resolved, and her visual acuity improved, although she had residual visual field constriction and developed bilateral optic atrophy. Outcomes of other cases of Purtscher-like retinopathy related to aHUS are reviewed.
    Conclusion: Purtscher-like retinopathy is a rare but severe ophthalmic complication of aHUS. Eculizumab is an effective treatment for the systemic illness caused by aHUS, and anatomical resolution of Purtscher-like retinopathy may follow, although visual prognosis remains guarded. Recovery of visual acuity may lag behind resolution of macular edema in these patients.
    MeSH term(s) Adult ; Female ; Humans ; Atypical Hemolytic Uremic Syndrome/complications ; Atypical Hemolytic Uremic Syndrome/therapy ; Retinal Diseases/diagnosis ; Retinal Hemorrhage/etiology ; Eye ; Renal Insufficiency
    Language English
    Publishing date 2021-01-24
    Publishing country United States
    Document type Case Reports ; Journal Article
    ISSN 1937-1578
    ISSN (online) 1937-1578
    DOI 10.1097/ICB.0000000000001126
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: A case report of late-onset atypical Hemolytic Uremic Syndrome during interferon beta in multiple sclerosis: Open issues in literature review.

    Parisi, Mosè / Manni, Alessia / Caputo, Francesca / Trojano, Maria / Paolicelli, Damiano

    Brain and behavior

    2020  Volume 11, Issue 1, Page(s) e01930

    Abstract: ... for relapsing-remitting multiple sclerosis (RRMS) patients and remains the most widely prescribed agent. Atypical hemolytic uremic syndrome ... review the literature on this topic.: Methods: We performed a systematic review of the literature using PubMed, and ... the case of the latest onset of IFNβ-related aHUS experienced by an MS patient and we briefly ...

    Abstract Background and aims: Interferon beta (IFNβ) is a well-established first-line therapy for relapsing-remitting multiple sclerosis (RRMS) patients and remains the most widely prescribed agent. Atypical hemolytic uremic syndrome (aHUS) represents a rare but severe adverse effect (AE) that could occur even after many years from the beginning of IFNβ therapy. Eculizumab is currently approved for treatment of aHUS and recently for neuromyelitis optica spectrum disorder (NMOSD) with aquaporin-4 antibodies (AQP4-IgG). In this article, we report the case of the latest onset of IFNβ-related aHUS experienced by an MS patient and we briefly review the literature on this topic.
    Methods: We performed a systematic review of the literature using PubMed, and we performed a retrospective analysis of RRMS patients that received IFNβ-1a in our center and developed thrombotic microangiopathy (TMA). From this search, we identified only one patient.
    Results: In the published literature, we identified 24 MS patients who received IFNβ as disease-modifying treatment (DMT) and then developed thrombotic microangiopathy with kidney injury. The aHUS has been diagnosed in 6, all received IFNβ-1a and the latest onset was after 15 years. We report a case of a 39-year-old man affected by RRMS who assumed IFNβ-1a since 1999. In July 2018, he developed an IFNβ-related aHUS. After the failure of plasma exchange, he underwent eculizumab, with an improvement of glomerular filtration rate and without new signs of MS activity.
    Conclusion: To our knowledge, this case represents the latest onset of IFNβ-related aHUS in MS patients. Up to now, there are not literary reports about the possibility to reintroduce a DMT as add-on therapy to eculizumab.
    MeSH term(s) Adult ; Atypical Hemolytic Uremic Syndrome ; Humans ; Interferon-beta/adverse effects ; Male ; Multiple Sclerosis/drug therapy ; Multiple Sclerosis, Relapsing-Remitting/drug therapy ; Retrospective Studies
    Chemical Substances Interferon-beta (77238-31-4)
    Language English
    Publishing date 2020-12-16
    Publishing country United States
    Document type Case Reports ; Journal Article ; Systematic Review
    ZDB-ID 2623587-0
    ISSN 2162-3279 ; 2162-3279
    ISSN (online) 2162-3279
    ISSN 2162-3279
    DOI 10.1002/brb3.1930
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Eculizumab in the treatment of atypical haemolytic uraemic syndrome and other complement-mediated renal diseases.

    Nester, Carla M / Brophy, Patrick D

    Current opinion in pediatrics

    2013  Volume 25, Issue 2, Page(s) 225–231

    Abstract: Purpose of review: This review considers the use of eculizumab in the treatment of atypical ... haemolytic uraemic syndrome (aHUS) as well as the other complement-mediated renal diseases, including ... the treatment of shiga toxin associated haemolytic uraemic syndrome (STEC HUS) is also provided.: Recent ...

    Abstract Purpose of review: This review considers the use of eculizumab in the treatment of atypical haemolytic uraemic syndrome (aHUS) as well as the other complement-mediated renal diseases, including dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). In addition, a brief discussion of the effectiveness of eculizumab for the prevention of antibody-mediated rejection (AMR) in the setting of renal transplant and the treatment of shiga toxin associated haemolytic uraemic syndrome (STEC HUS) is also provided.
    Recent findings: No randomized controlled trials exist to support the use of eculizumab in renal disease. The results of two unpublished, prospective adult and adolescent trials support its utility in aHUS, whereas retrospective data support the effectiveness in paediatric aHUS. These two data sets form the basis of the sole renal indication for eculizumab. One small, single-centre trial and a growing number of case reports support the use of eculizumab in C3 glomerulopathy (C3G). There are limited trial data in AMR and renal transplant. Finally, there are conflicting data for the use of eculizumab in STEC HUS.
    Summary: The cumulative published data establish the effectiveness of eculizumab in a select group of renal diseases that have at the centre of their disease either abnormal complement control or maladaptive complement activation.
    MeSH term(s) Antibodies, Monoclonal, Humanized/therapeutic use ; Atypical Hemolytic Uremic Syndrome ; Complement Activation/immunology ; Complement C3/immunology ; Complement Inactivating Agents/therapeutic use ; Glomerulonephritis/drug therapy ; Glomerulonephritis/immunology ; Graft Rejection/prevention & control ; Hemolytic-Uremic Syndrome/drug therapy ; Hemolytic-Uremic Syndrome/immunology ; Hemolytic-Uremic Syndrome/surgery ; Humans ; Kidney Transplantation
    Chemical Substances Antibodies, Monoclonal, Humanized ; Complement C3 ; Complement Inactivating Agents ; eculizumab (A3ULP0F556)
    Language English
    Publishing date 2013-04
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 1049374-8
    ISSN 1531-698X ; 1040-8703
    ISSN (online) 1531-698X
    ISSN 1040-8703
    DOI 10.1097/MOP.0b013e32835df4a3
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 3049: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)

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