BACKGROUND: Ireland has been relatively free of sickle-cell disease (SCD) and a care policy for the disease has not been established. AIM: To determine the prevalence of childhood SCD in Ireland and to predict requirements for a comprehensive care and assessment programme. METHODS: We retrospectively analysed the data of children with SCD presenting with sickle-cell crisis to one institution from January 1999 to April 2001. We also determined the nature and severity of the presenting haemoglobinopathy phenotype. RESULTS: Ninety-two patients with haemoglobinopathy have been registered with the Paediatric Haematology Service. The majority are from Nigeria with a smaller number from Angola and the Congo. Sixty have sickle-cell trait, 23 SCD, four haemoglobin SC disease and two haemoglobin E (HbE). There have been 32 sickle-cell crises. The majority were haemolytic or splenic sequestration events with a smaller number of aplastic and vaso-occlusive events and one osteomyelitis. CONCLUSION: The increasing number of children presenting with SCD as a result of the increasing refugee numbers requires a comprehensive care approach similar to that required for paediatric haemophilia to ensure optimum care.
BACKGROUND: Ireland has been relatively free of sickle-cell disease (SCD) and a care policy for the disease has not been established. AIM: To determine the prevalence of childhood SCD in Ireland and to predict requirements for a comprehensive care and assessment programme. METHODS: We retrospectively analysed the data of children with SCD presenting with sickle-cell crisis to one institution from January 1999 to April 2001. We also determined the nature and severity of the presenting haemoglobinopathy phenotype. RESULTS: Ninety-two patients with haemoglobinopathy have been registered with the Paediatric Haematology Service. The majority are from Nigeria with a smaller number from Angola and the Congo. Sixty have sickle-cell trait, 23 SCD, four haemoglobin SC disease and two haemoglobin E (HbE). There have been 32 sickle-cell crises. The majority were haemolytic or splenic sequestration events with a smaller number of aplastic and vaso-occlusive events and one osteomyelitis. CONCLUSION: The increasing number of children presenting with SCD as a result of the increasing refugee numbers requires a comprehensive care approach similar to that required for paediatric haemophilia to ensure optimum care.
Authors: A S Prasad; F W Beck; J Kaplan; P H Chandrasekar; J Ortega; J T Fitzgerald; P Swerdlow Journal: Am J Hematol Date: 1999-07 Impact factor: 10.047
Authors: T R Kinney; L A Sleeper; W C Wang; R A Zimmerman; C H Pegelow; K Ohene-Frempong; D L Wethers; J A Bello; E P Vichinsky; F G Moser; D M Gallagher; M R DeBaun; O S Platt; S T Miller Journal: Pediatrics Date: 1999-03 Impact factor: 7.124
Authors: Bukola Salami; Higinio Fernandez-Sanchez; Christa Fouche; Catrin Evans; Lindiwe Sibeko; Mia Tulli; Ashley Bulaong; Stephen Owusu Kwankye; Mary Ani-Amponsah; Philomina Okeke-Ihejirika; Hayat Gommaa; Kafuli Agbemenu; Chizoma Millicent Ndikom; Solina Richter Journal: Int J Environ Res Public Health Date: 2021-03-28 Impact factor: 3.390