title: Haptoglobin therapy for acute favism: a Japanese boy with glucose-6-phosphate dehydrogenase Guadalajara.
abstract: We report the case of a 2-year-old Japanese boy with acute favism who was treated with human haptoglobin products. He had been exhibiting chronic nonspherocytic haemolytic anaemia until the diagnosis of glucose-6-phosphate dehydrogenase (G6PD) deficiency when 14 months old. He suffered a favic crisis at 24 months of age, when the administration of haptoglobin was effective for relieving bilirubinaemia and haemoglobinuria. Serum-free Hb rapidly decreased to normal levels despite the sustained level of serum lactate dehydrogenase. His G6PD gene was G6PD Guadalajara. This is the first application of haptoglobin therapy for acute favism and the first reported case of Japanese G6PD deficiency with typical favic crisis. Haptoglobin treatment might be helpful for managing the haemolytic crisis in the disease.
authors.affiliation: Department of Paediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Ohga
authors.forename: E
authors.initials: E
authors.lastname: Higashi
authors.forename: A
authors.initials: A
authors.lastname: Nomura
authors.forename: A
authors.initials: A
authors.lastname: Matsuzaki
authors.forename: A
authors.initials: A
authors.lastname: Hirono
authors.forename: S
authors.initials: S
authors.lastname: Miwa
authors.forename: H
authors.initials: H
authors.lastname: Fujii
authors.forename: K
authors.initials: K
authors.lastname: Ueda
mesh_terms: D002675:Child, Preschool; D005236:Favism / Q000150:complications / Q000628:therapy*; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000150:complications / Q000628:therapy*; D006242:Haptoglobins / Q000627:therapeutic use*; D006454:Hemoglobins / Q000032:analysis; D006456:Hemoglobinuria / Q000628:therapy; D006801:Humans; D007564:Japan; D008297:Male
chemical_list: D006242:Haptoglobins; D006454:Hemoglobins