الفهرس 
Hematological and renal phenotypes in pediatrics primary thrombotic microangiopathy
List of ContentsOnly 14 pages are availabe for public view
 |  | from | 122 |  |  |
| | | from | 122 | | |
Abstract
Background:There has been a dramatic acceleration in the understanding of the pathogenesis of primary thrombotic microangiopathy (TMA) syndromes. This will create opportunities for specific treatments and the availability of specific treatments has created the need for rapid and specific diagnoses. Objective:was to provide a clinical description of primary thrombotic microangiopathy syndromes in the pediatric age group, and to identify the underlying immunological disturbances in such group of diseases. Methods: This cross sectional analytical study was conducted on 30 children with clinical and laboratory features suggestive of TMA at Cairo University Children Hospital at the period from August 2019 to February 2020. Shiga toxin screening (STEC),complement assay, ADAMT13 antigen concentration, homocysteine, CBC, urea&creatinine, coagulation profile and total& direct bilirubin were conducted to all patients enrolled in this study. Results:Nine (30%) patients had positive STEC test &nine (30%) patients had autoantibodies against CFH, and twelve (40%) patients with negative Shiga toxin test and no detectable autoantibodies against CFH.All patients presented with acute onset of intravascular hemolytic anemia, direct comb`s test was performed at onset and was negative in all patients. Almost all children had impaired Kidney function tests as evidenced by increased median urea&creatinine. Renal biopsy was indicted during the course of illness in 10 (33.3%) & Pathology of the studied renal biopsies was indicative of TMA in all specimens