title: Asplenia in children with congenital heart disease as a cause of poor outcome.
abstract: The absence of a spleen is a well-known risk factor for severe bacterial infections, especially due to encapsulated bacteria. Congenital asplenia can be part of multiple congenital abnormalities as in heterotaxy including Ivemark syndrome with congenital anomalies of the heart or great vessels, or it can be isolated, which is extremely rare. In these cases, asplenia is an important factor effecting mortality. In this report, the clinical courses of five children with asplenia and concomitant minor or complex cardiac anomalies are presented. The ages of the children ranged between 1.5 and 17 months at the time of diagnosis. All of the cases had had a history of hospitalisation for infectious diseases before the diagnosis. The patient who was diagnosed at 17 months old had a history pneumonia, urinary tract infection, and bacterial meningitis beginning at five months old. Three children had complex cardiac anomalies, one child had ventricular septal defect, and one child had atrial septal defect. Howell-Jolly bodies were determined in peripheral blood smear in all of the patients. The diagnoses of asplenia were confirmed with spleen scintigraphy. One of the patients with complex cardiac anomalies died a short time after diagnosis, because of cardiac failure. The rest of the four patients were vaccinated for encapsulated bacteria and were taken under antibiotic prophylaxis. These children did not need hospitalisation for infectious diseases during the follow-up period (5-40 months). In asplenic children, early diagnosis, antibiotic prophylaxis, and immunisation for encapsulated bacteria can decrease the risk of morbidity and mortality.
authors.affiliation: Department of Pediatric Allergy and Immunology Department, Dr. Behcet Uz Children Training and Research Hospital, İzmir, Turkey.
authors.forename: Semiha Bahceci
authors.initials: SB
authors.lastname: Erdem
authors.affiliation: Department of Pediatric Allergy and Immunology Department, Dr. Behcet Uz Children Training and Research Hospital, İzmir, Turkey.
authors.forename: Ferah
authors.initials: F
authors.lastname: Genel
authors.affiliation: Department of Pediatric Allergy and Immunology Department, Dr. Behcet Uz Children Training and Research Hospital, İzmir, Turkey.
authors.forename: Baris
authors.initials: B
authors.lastname: Erdur
authors.affiliation: Department of Pediatric Allergy and Immunology Department, Dr. Behcet Uz Children Training and Research Hospital, İzmir, Turkey.
authors.forename: Erhan
authors.initials: E
authors.lastname: Ozbek
authors.affiliation: Department of Pediatric Allergy and Immunology Department, Dr. Behcet Uz Children Training and Research Hospital, İzmir, Turkey.
authors.forename: Nesrin
authors.initials: N
authors.lastname: Gulez
authors.affiliation: Department of Pediatric Cardiology, Dr. Behcet Uz Children Training and Research Hospital, İzmir, Turkey.
authors.forename: Timur
authors.initials: T
authors.lastname: Mese
keywords: asplenia; cardiac anomalies; child; heterotaxy syndrome; immunisation; infection