title: A massive intestinal vaso-occlusive crisis or "girdle syndrome" in a 6-year-old boy observed as a first manifestation of sickle cell disease.
abstract: Sickle cell disease is a chronic hematologic disease with variable but often severe systemic symptoms. In this report, we describe a 6-year-old boy presenting with acute bowel pseudo-obstruction. During this episode, previously undiagnosed sickle cell disease was discovered upon peripheral blood smear analysis. The condition was therefore interpreted as a massive intestinal vaso-occlusive crisis or "girdle syndrome". Conservative treatment with hydration therapy, analgesia and a manual partial exchange transfusion was initiated. The patient fully recovered within 5 days.
  
  
  CONCLUSIONS
  Girdle syndrome is a rare but severe adverse event associated with sickle cell disease that must be considered as differential diagnosis in patients with sickle cell disease.
authors.affiliation: Pediatric Hematology/Oncology, Universitätsklinikum Essen, Essen, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Knorr
authors.affiliation: Department of General Pediatrics, Elisabeth-Krankenhaus Essen, Essen, Germany.
authors.forename: K
authors.initials: K
authors.lastname: Bienemann
authors.affiliation: Pediatric Hematology/Oncology, Universitätsklinikum Essen, Essen, Germany.
authors.forename: G
authors.initials: G
authors.lastname: Walde
authors.affiliation: Pediatric Hematology/Oncology, Universitätsklinikum Essen, Essen, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Kaufhold
authors.affiliation: Pediatric Hematology/Oncology, Universitätsklinikum Essen, Essen, Germany.
authors.forename: M M
authors.initials: MM
authors.lastname: Schündeln
mesh_terms: D000006:Abdomen, Acute / Q000209:etiology*; D000755:Anemia, Sickle Cell / Q000175:diagnosis*; D001157:Arterial Occlusive Diseases / Q000175:diagnosis* / Q000628:therapy; D002648:Child; D054242:Emigrants and Immigrants; D005078:Exchange Transfusion, Whole Blood; D005858:Germany; D005869:Ghana / Q000208:ethnology; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000175:diagnosis* / Q000628:therapy; D007421:Intestine, Small / Q000098:blood supply*; D008297:Male