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Unlabelled: The most frequent cause of nephritic syndrome in the pediatric population is acute post-infectious glomerulonephritis (PIGN). A rare complication is posterior reversible encephalopathy syndrome (PRES), characterized by subcortical vasogenic cerebral edema associated with variable neurological symptoms. The development of autoimmune hemolytic anemia is an atypical clinical presentation.
View Article and Find Full Text PDFMultigene Panel Testing Revealed Novel Variants in Hereditary Spherocytosis Patients in Türkiye.
Turk J Haematol
January 2025
Marmara University Faculty of Medicine, Department of Medical Genetics, İstanbul, Türkiye.
Objective: This study aims to determine the genotypic characteristics of Hereditary Spherocytosis (HS) patients in Turkiye and to examine the correlation between genotype and phenotype.
Materials And Methods: Herein we had 18 patients who were admitted to pediatric hematology outpatient clinic with hemolytic anemia, jaundice, cholelithiasis, and splenomegaly. According to the Eber's classification, the patients' clinical presentations were categorized as mild, moderate, and severe.
Severe childhood SLE with refractory autoimmune hemolytic anemia-a therapeutic challenge.
BMJ Case Rep
January 2025
Dermatology, Venereology and Leprosy, AIIMS Rishikesh, Rishikesh, Uttarakhand, India
Paediatric Systemic lupus erythematosus (SLE) constitutes 10 to 20% of cases of SLE with more severe disease and higher mortality. We report a case of an adolescent girl with SLE with multisystem involvement who was started on hydroxychloroquine and oral prednisolone. However, due to persistent worsening of skin lesions and falling cell counts, pulsed dexamethasone was initiated which showed improvement in the skin lesions, cell counts, proteinuria and pleural effusion but there was a persistent fall in the haemoglobin.
View Article and Find Full Text PDFObjective: The objective of this study was to identify serum complement factor-based biomarkers indicative of clinical efficacy in patients with first-episode schizophrenia (SCZ) following treatment with aripiprazole.
Methods: The retrospective study cohort comprised 40 patients diagnosed with first-episode SCZ (SCZ group) and 40 healthy individuals (control group). Quantitative analyses were conducted on five complement factors, namely complement component 1 (C1), C2, C3, C4, and the 50% hemolytic complement (CH50).
Plasma Exchange and N-Acetylcysteine Therapy in a Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting With Acute Renal Failure.
J Pediatr Hematol Oncol
January 2025
Departments of Pediatric Hematology.
Congenital thrombotic thrombocytopenic purpura (cTTP), which is associated with mutations in the gene for a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 (ADAMTS13), is a chronic and lifelong disease. The clinical course is variable. Regularly using ADAMTS13-containing products such as fresh frozen plasma (FFP) for long-term prophylaxis is the most important treatment to prevent thrombotic microangiopathy (TMA) episodes.
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