A child with anemia, thrombocytopenia, renal failure and elevated amylase, and lipase enzymes: Questions

Source: Pediatric Nephrology - Category: Urology & Nephrology Source Type: research

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Source: Pediatric Nephrology - Category: Urology & Nephrology Source Type: research
Mauro Nicola Laforgia Shiga toxin-producing Escherichia Coli (STEC) infections routinely run as a common gastroenteritis, but in many cases they may evolve towards hemolytic uremic syndrome (HUS). HUS is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Gut microorganisms have a fundamental impact on human physiology, because they modulate normal intestinal functions and play a pivotal role in influencing the local and systemic immune responses. Despite surveillance established in many countries and major progresses in the understanding of STEC-HUS mechanism...
Source: International Journal of Environmental Research and Public Health - Category: Environmental Health Authors: Tags: Review Source Type: research
In conclusion, we have confirmed in our cohort of patients, the strong association between FHR1 deficiency and aHUS with anti-FH autoantibodies. Through an innovative approach based on the comparison with “supercontrols” carrying the homozygous CFHR1 deletion, identified by screening a large number of healthy adult subjects, we have documented that patients with anti-FHs aHUS are enriched in complement gene LPVs. This observation indicates that the pathogenesis of anti-FHs aHUS is complex and multiple “hits” are required for its clinical manifestation. We also document that the CFH H3 and the CD46GG...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Conclusion: Our findings describe the most reported risk factors for preventability of oral anticoagulant-induced bleedings. These factors may be useful for targeting interventions to improve pharmacovigilance activities in our regional territory and to reduce the burden of medication errors and inappropriate prescription. Introduction Oral anticoagulant therapy is widely used for the prevention of stroke and systemic embolism in patients with atrial fibrillation, or for the prevention and treatment of deep vein thrombosis and pulmonary embolism (Raj et al., 1994; Monaco et al., 2017). Oral anticoagulants can be di...
Source: Frontiers in Pharmacology - Category: Drugs & Pharmacology Source Type: research
A 6-month-old boy presented with acute renal failure, thrombocytopenia, and severe non-immune hemolytic anemia. Infection by Shiga-like toxin-producingEscherichia coli and other causes of microangiopathic hemolysis were ruled out, leading to a diagnosis of atypical hemolytic uremic syndrome (aHUS). Neither pathogenic variants in HUS-associated genes nor anti-factor H antibodies were identified. Copy number variation analysis uncovered 4 copies of complement factor H related genes,CFHR1-CFHR4, conceivably leading to higher than normal levels of the corresponding proteins. However, this abnormality was also found in the heal...
Source: Nephron - Category: Urology & Nephrology Source Type: research
The objective of this study is to study the clinical and histopathological profile of LN and the response to treatment with cyclophosphamide. This was a retrospective study conducted in a tertiary care center in Assam, India, where 176 LN patients who underwent renal biopsy were included. The presenting features, laboratory parameters such as proteinuria, hematuria, and the histopathological class of the patients were studied. Among the 176 patients, 89.8% were female and 10.2% were male and maximum patients (61.3%) were in the age group of 20-40 years. Pedal edema was present in 100% of the patients, decreased urine outpu...
Source: Saudi Journal of Kidney Diseases and Transplantation - Category: Urology & Nephrology Authors: Tags: Saudi J Kidney Dis Transpl Source Type: research
Influenza is a generally self-limited infection agent that only rarely causes severe complications. To increase awareness about its serious complications, we report three cases of influenza A (H1N1) infection complicated with hemolytic uremic syndrome, myocarditis and acute necrotizing encephalopathy. In all three cases, nasopharyngeal samples confirmed influenza A (H1N1) infection by antigen test and multiplex PCR detection. The first case, a 3-year-old girl, had respiratory distress, anemia, thrombocytopenia and renal failure at admission, and was diagnosed with hemolytic uremic syndrome. Supportive treatment and oseltam...
Source: The Journal of Infection in Developing Countries - Category: Infectious Diseases Source Type: research
Conclusions:While disease-directed therapy in atypical HUS (i.e. eculizumab) is required to effectively manage acute, complement-mediated hemolytic anemia and renal failure, correction of the calculated iron deficit in co-existing iron deficiency anemia, if present, can result in gradual but marked improvement in hemoglobin. Conversely, patients with atypical HUS who are actively hemolyzing with a normocytic anemia should still be evaluated for iron deficiency and undergo iron transfusions for any calculated deficit.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - Category: Hematology Authors: Tags: 311. Disorders of Platelet Number or Function Source Type: research
Conclusions: In this Phase 1 study, VICER shows encouraging antilymphoma activity in r/r DLBCL, including double hit/double expressor lymphomas, with high rates of complete metabolic response (69% CR by PET), which is higher than historical levels reported with R-ICE alone (CR typically
Source: Blood - Category: Hematology Authors: Tags: 626. Aggressive Lymphoma (Diffuse Large B-Cell and Other Aggressive B-Cell Non-Hodgkin Lymphomas)-Results from Prospective Clinical Trials: New Agents Source Type: research
Complement-mediated thrombotic microangiopathy (TMA), also known as atypical hemolytic uremic syndrome (aHUS) is a rare, hereditary, progressive, life-threatening disorder caused by a disruption in regulation of the alternative pathway of the complement system. Eculizumab, a terminal complement inhibitor, has emerged as a first-line therapy, however data are limited to small case series (Brocklebank et al., 2017). Here, we present a diagnostically challenging case of complement-mediated TMA, who received eculizumab therapy with excellent hematologic response.A 68-year-old female with history of possible Sjogren's syndrome,...
Source: Blood - Category: Hematology Authors: Tags: 311. Disorders of Platelet Number or Function Source Type: research
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