Klinische Pädiatrie: Offener Zugang
Offener Zugang

Abstrakt

Paediatric Thrombotic Microangiopathy with AKI secondary to aHUS

Turner*

TMA is a pathological process involving thrombocytopenia, microangiopathic haemolytic 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. TMA is life threatening resulting from ischemic multiorgan
failure and characterized by its diversity and high ICU mortality rate ~ 20%, despite appropriate treatment [1,2].
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 impairement 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 trigerring events. These include severe infections, autoimmunity, haematopoietic
progenitor cells or solid organ transplantations, 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.