Summary
Definition
History and exam
Diagnostic investigations
1st investigations to order
- FBC
- reticulocyte count
- blood smear
- serum bilirubin
- serum aminotransferases
- direct anti-globulin test (DAT)
Investigations to consider
- eosin-5-maleimide binding test
- acidified glycerol lysis test
- sodium dodecyl sulfate-polyacrylamide gel electrophoresis
Treatment algorithm
Contributors
Authors
Associate Professor of Pediatrics
University of Arkansas for Medical Sciences
Little Rock
AR
Disclosures
SC is on an advisory board for Genentech for a hemophilia product (unrelated to this article).
Dr Shelley Crary would like to gratefully acknowledge Dr Paula Bolton-Maggs, a previous contributor to this topic. PB-M has received travel and accommodation payments to give a series of lectures on paediatric haematology, one of which was on HS. She also was an expert witness in a legal case concerning a child with HS. PB-M is an author of some references cited in this topic.
Peer reviewers
Professor of Medicine Emeritus
School of Medicine and Public Health
University of Wisconsin-Madison
Madison
WI
Disclosures
RS is an author of a number of references cited in this topic.
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