Introduction: Previous small-scale, single-center investigations of associated hemolytic uremic syndrome (SpHUS) have shown increased disease severity among SpHUS relative to non-SpHUS patients. Our study compares the impact of on patient outcomes between SpHUS cases and non-SpHUS controls using the national, multicenter retrospective Pediatric Health Information Systems (PHIS) Database.
Methods: Children <18 years of age with a diagnosis of HUS were included. Univariate analyses and multivariable linear and logistic regressions were utilized to assess the impact of on mortality, length of stay (LOS), intensive care unit admission (ICU), and mechanical ventilation use. Models were adjusted for demographic and clinical characteristics, including cardiac, neurologic, pulmonary, gastrointestinal, immunologic and renal clinical complications.
Results: Of 3,952 index HUS hospitalizations, 231 (5.8%) were due to SpHUS. SpHUS patients had worse outcomes, including longer hospital stays, increased rate of ICU admission, and increased use of mechanical ventilation ( < 0.001 for all). There was a strong positive relationship between clinical complications and adverse outcomes. After adjusting for covariates, SpHUS was associated with an increase in hospital LOS by 3.47 days ( = 0.009) and overall ICU-LOS by 4.21 days ( < 0.001). SpHUS was also associated with increased likelihood of mechanical ventilation (OR: 3.08; < 0.001), with no increase in ICU admission ( = 0.070) and in-hospital mortality ( = 0.3874).
Discussion: Our study highlights that SpHUS patients are at increased risk of multiple adverse outcomes likely due to the summative impact of pneumococcal infection and HUS as well as more frequent clinical complications.
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http://dx.doi.org/10.3389/fped.2023.1268971 | DOI Listing |
Introduction: Thrombotic microangiopathies (TMA) represent distinct pathological and clinical entities with known chronicity and recurrence. Kidney biopsy is the gold standard to diagnose TMA in patients with renal manifestations but the prognostic significance of acute or chronic phase of the disease has not been well studied. We examined the clinical characteristics, management, and predictors of acute vs.
View Article and Find Full Text PDFCureus
December 2024
Internal Medicine, Hospital Beatriz Ângelo, Lisboa, PRT.
Vitamin B12 deficiency is a potentially severe condition with clinical manifestations ranging from nonspecific symptoms, such as asthenia and glossitis, to severe hematological problems, including pancytopenia and megaloblastic anemia. One of the rare phenomena associated with this condition is pseudo-thrombotic microangiopathy (pseudo-TMA), which can mimic diseases such as thrombotic thrombocytopenic purpura (TTP), leading to possible misdiagnosis and inappropriate treatment. In this article, we present the case of a 62-year-old man with a history of intravenous drug use, untreated hepatitis C, smoking, and alcoholism.
View Article and Find Full Text PDFJ Tradit Complement Med
January 2025
School of Chemical & Biotechnology, SASTRA Deemed University, Thanjavur, India.
Background & Aim: Hemolytic anemia is a blood disorder whose incidence is increasing in the world in recent years especially after the pandemic. Conventional treatments include use of steroids and immunosuppresants that are accompanied by numerous adverse effects. With growing interest in using complex multi-component formulations for multi-targeted therapy, the present study aims to investigate the therapeutic efficacy of a traditional herbomineral preparation, , which has been traditionally used as a supplement in iron-deficiency anemia, against phenylhydrazine-induced hemolytic anemia in rodent models.
View Article and Find Full Text PDFTissue Cell
January 2025
Department of Biology, Universidade Estadual Paulista (UNESP), São Paulo, Brazil; Campus de Três Lagoas, Universidade Federal de Mato Grosso do Sul (CPTL/UFMS), Mato Grosso do Sul, Brazil. Electronic address:
Sickle cell disease (SCD) is a hereditary hemolytic anemia associated with the alteration of the membrane composition of the sickle erythrocytes, the loss of glycolysis, dysregulation of the pyruvate phosphatase pathway, and changes in nucleotide metabolism of the sickle red blood cell (RBC). This review provides a comprehensive overview of the impact of the presence of Hb S, which leads to the disruption of the normal RBC metabolism. The intricate interplay between the redox and energetic balance in erythrocytic cells, where the glycolysis, pentose phosphate pathway, and methemoglobin reductase pathways are all altered in sickle RBC, is a key focus.
View Article and Find Full Text PDFJ Thromb Haemost
January 2025
Department of Pathology and Laboratory Medicine; Institute of Reproductive Medicine and Developmental Sciences, The University of Kansas Medical Center, Kansas City, KS 66160. Electronic address:
Background: A loss-of-functional mutation (W1183R) in human complement factor H (CFH) is associated with complement-associated hemolytic uremic syndrome; mice carrying a similar mutation (W1206R) in CFH also develop thrombotic microangiopathy but its plasma von Willebrand factor (VWF) multimer sizes were dramatically reduced. The mechanism underlying such a dramatic change in plasma VWF multimer distribution in these mice is not fully understood.
Objective And Methods: To determine the VWF and CFH interaction and how CFH proteins affect VWF multimer distribution, we employed recombinant protein expression, purification, and various biochemical and biophysical tools.
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