臨床小児科: オープンアクセス
オープンアクセス

概要

Pediatric Thrombotic Microangiopathy with AKI Secondary to aHUS : A Case Report

Jyoti Walia, Shahbaj Ahmad, Preeti Uniyal and Aditya Bedi

TMA is a pathological process involving thrombocytopenia,microangiopathic hemolytic anemia and microvascular occlusion. TMA belongs to  thrombocytopenia associated multi-organ failure(TAMOF) syndromes and therefore its diagnosis should be considered in critically ill children. MA is life threatening resulting from ischemic multiorgan failure and characterized by its diversity and high ICU mortality rate ~ 20%,despite appropriate treatment. It can manifest in diverse range of conditions and presentations, but AKI is a common prominent feature because of apparent propensity of the glomerular circulation to endothelial damage and occlusion. The most frequent TMA syndromes reported in children are haemolytic uraemic syndrome(HUS), in which renal impairment is the prominent clinical feature. Thrombotic thrombocytopenic purpura (TTP), another TMA syndrome also occurs in children often associated with cerebral involvement.(Secondary TMA are defined as TMA occurring with other co-morbidities serving as the triggering events. These include severe infections, autoimmunity, hematopoietic progenitor cells or solid organ transplantation, malignancy and drugs. Therefore the diagnosis of secondary TMA can be extremely challenging.

Here we present a diagnostic approach to a previously healthy 13Yrs old male child who presented in emergency with breathing difficulty, decreased oral acceptance, lethargy and altered sensorium for past one day, later found to have azotemia and anion gap metabolic acidosis.   

TMA is a pathological process involving thrombocytopenia,microangiopathic hemolytic anemia and microvascular occlusion. TMA belongs to  thrombocytopenia associated multi-organ failure(TAMOF) syndromes and therefore its diagnosis should be considered in critically ill children. MA is life threatening resulting from ischemic multiorgan failure and characterized by its diversity and high ICU mortality rate ~ 20%,despite appropriate treatment. It can manifest in diverse range of conditions and presentations, but AKI is a common prominent feature because of apparent propensity of the glomerular circulation to endothelial damage and occlusion. The most frequent TMA syndromes reported in children are haemolytic uraemic syndrome(HUS), in which renal impairment is the prominent clinical feature. Thrombotic thrombocytopenic purpura (TTP), another TMA syndrome also occurs in children often associated with cerebral involvement.(Secondary TMA are defined as TMA occurring with other co-morbidities serving as the triggering events. These include severe infections, autoimmunity, hematopoietic progenitor cells or solid organ transplantation, malignancy and drugs. Therefore the diagnosis of secondary TMA can be extremely challenging.

Here we present a diagnostic approach to a previously healthy 13Yrs old male child who presented in emergency with breathing difficulty, decreased oral acceptance, lethargy and altered sensorium for past one day, later found to have azotemia and anion gap metabolic acidosis.