Background: Hemolytic uremic syndrome (HUS) is one of the main causes of acute renal failure in the Chilean pediatric population.
Aim: To report the features of patients with HUS, admitted to the pediatric ward of a clinical hospital.
Material And Methods: Retrospective review of medical records of patients admitted with the diagnosis of HUS between 1995 and 2002.
Results: During the period, 58 patients were admitted with the diagnosis of HUS but only 43 (age range 1 month to 6 years, 22 females) had complete medical records for review. Ninety five percent presented with prodromic diarrhea, mainly dysenteric. Antibiotics were administered to 70%, in the previous days. Acute renal replacement, mainly peritoneal dialysis, was required in 40%. The clinical signs and laboratory parameters that correlated better with the indication for dialysis were anuria, hypertension, initial and permanently high serum creatinine and blood urea nitrogen. Four patients with blood urea nitrogen over 100 mg/dl but without anuria or hyperkalemia, were treated conservatively, and experienced an uneventful course (permissive azotemia). Hospital stay was almost 3 times greater in dialyzed than in non dialyzed children. No deaths related to HUS were reported in the study period. In an average follow up of 54 months, 11.6% of the patients developed chronic renal failure of diverse magnitude.
Conclusions: Despite the fact that our study group behaved clinically similar to published HUS patients in other series, no mortality was observed in a retrospective analysis of patients with this disease.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.4067/s0034-98872005000700005 | DOI Listing |
Children (Basel)
January 2025
Division of Critical Care Medicine, Nicklaus Children's Hospital, 3100 SW 62nd Avenue, Miami, FL 33155, USA.
Thrombocytopenia frequently occurs in patients before, during, and after admission to Pediatric Intensive Care Units (PICUs). In critically ill children, it is often due to multifactorial causes and can be a sign of significant organ dysfunction. This review summarizes the potential causes/mechanisms of thrombocytopenia in acutely ill children, their identification, and treatments, with special attention paid to septic patients.
View Article and Find Full Text PDFFront Pharmacol
January 2025
The First Department of Specialty Medicine, Inner Mongolia Corps Hospital of The Chinese People's Armed Police Force, Hohhot, China.
Introduction: Eculizumab is a C5 complement inhibitor approved by the FDA for the targeted treatment of four rare diseases, paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), generalized myasthenia gravis (gMG), and aquaporin-4 immunoglobulin G-positive optic neuromyelitis optica spectrum disorders (AQP4-IgG+NMOSD). The current study was conducted to assess real-world adverse events (AEs) associated with eculizumab through data mining of the FDA Adverse Event Reporting System (FAERS).
Methods: Disproportionality analyses, including Reporting Ratio Ratio (ROR), Proportional Reporting Ratio (PRR), Bayesian Confidence Propagation Neural Network (BCPNN), and Multi-Item Gamma Poisson Shrinker (MGPS) algorithms were used to quantify the signals of eculizumab-associated AEs.
Kidney Int
January 2025
Complement Therapeutics Research Group, Newcastle University Translational and Clinical Research Institute, The Medical School, Newcastle-upon-Tyne, UK; National Renal Complement Therapeutics Centre, The Royal Victoria Infirmary, Newcastle-upon-Tyne, UK.
Introduction of complement (C) inhibition into clinical practice has revolutionized the treatment of patients with complement-mediated atypical hemolytic syndrome (aHUS). Our C3 mouse model, engineered around a gain of function point mutation in C3, is associated with complement mediated aHUS in man, allowing us to study the clinical disease in a preclinical model. Backcrossing our model onto C7 deficient and C5aR1 deficient mice enabled further determination of the roles of the C5a-C5aR1 axis and C5b-9 (the membrane attack complex) on kidney disease.
View Article and Find Full Text PDFFront Med (Lausanne)
January 2025
Department of Hematology, The Second Affiliated Hospital of Nanchang University, Nanchang, China.
Congenital thrombotic thrombocytopenic purpura (cTTP) is a thrombotic microangiopathy (TMA) characterized by severe hereditary ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motifs 13) deficiency caused by mutations. This rare autosomal recessive genetic disorder is often misdiagnosed as immune thrombocytopenia (ITP) or hemolytic uremic syndrome (HUS). Here, we report a 21-year-old male cTTP patient with a compound heterozygous mutation.
View Article and Find Full Text PDFInn Med (Heidelb)
January 2025
MVZ Nephrocare Mettmann GmbH, Gartenstr. 4-8, 40822, Mettmann, Deutschland.
Recognizing anemia and thrombpenia in acute and emergency medicine is easy. Acute (microangiopathic hemolytic) anemia and thrombopenia can be a sign of thrombotic microangiopathy (TMA). TMA syndromes are potentially life-threatening diseases.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!