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The challenge of managing hemiphilia A and STEC-induced hemolytic uremic syndrome
- Source :
- Pediatric Nephrology, 28, 349-352. Springer-Verlag, Pediatric Nephrology, 28, 349-52, Pediatric Nephrology, 28, 2, pp. 349-52
- Publication Year :
- 2013
-
Abstract
- Item does not contain fulltext BACKGROUND: The hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy leading to acute kidney injury in children. In most cases it is triggered by an infection caused by Shiga-like toxin-producing Escherichia coli (STEC). Endothelial damage plays a central role in the pathogenesis of disease. Hemophilia A is a genetic disorder leading to factor VIII (FVIII) deficiency, an important factor in the coagulation system. CASE: Here we describe a hemophilia A patient who developed HUS due to a STEC O26 infection. The patient developed not only acute kidney injury, but also severe gastro-intestinal and neurological complications. Increased amounts of recombinant FVIII (rFVIII) had to be administered during the acute phase of the disease to reach acceptable blood levels of FVIII, in order to control the hemorrhagic colitis and to prevent severe neurological complications. CONCLUSION: The patient's treatment schedule of rFVIII during the HUS period was a serious challenge, and we cannot exclude that it contributed to the severity of the HUS by enhancing the thrombotic microangiopathic process. The role of factor VIII administration in the severe outcome of this disease is discussed.
- Subjects :
- Nephrology
Male
medicine.medical_specialty
Thrombotic microangiopathy
Age-related aspects of cancer [ONCOL 2]
Genomic disorders and inherited multi-system disorders Energy and redox metabolism [IGMD 3]
Disease
Constriction, Pathologic
Anuria
Hemophilia A
Renal disorder Energy and redox metabolism [IGMD 9]
Pathogenesis
Ileus
Internal medicine
hemic and lymphatic diseases
medicine
Humans
Escherichia coli Infections
Renal disorder [IGMD 9]
Factor VIII
Sigmoid Diseases
Shiga-Toxigenic Escherichia coli
business.industry
Coagulants
Genetic disorder
Acute kidney injury
Infant, Newborn
Rectum
Infant
Mitochondrial medicine Energy and redox metabolism [IGMD 8]
Plasmapheresis
medicine.disease
Renal disorder Membrane transport and intracellular motility [IGMD 9]
Treatment Schedule
Child, Preschool
Pediatrics, Perinatology and Child Health
Immunology
Hemolytic-Uremic Syndrome
Epilepsy, Tonic-Clonic
Hemofiltration
Hemorrhagic colitis
business
Gastrointestinal Hemorrhage
Subjects
Details
- ISSN :
- 0931041X
- Volume :
- 28
- Database :
- OpenAIRE
- Journal :
- Pediatric Nephrology
- Accession number :
- edsair.doi.dedup.....eb9c6b5fa56b2bba0f51e0d582accd78