Estimated incidence of sickle-cell disease in Aruba and St Maarten suggests cost-effectiveness of a universal screening programme for St Maarten / Incidencia estimada de la enfermedad de células falciformes en Aruba y St Marteen sugiere costo-efectividad para un programa de pesquisaje universal para St Marteen

OBJECTIVE: To estimate the incidence of Sickle-Cell Disease (SCD) in Aruba and St Maarten and to determine whether universal screening would be costeffective according to United Kingdom criteria. METHODS: Consecutive cord blood samples were collected in Aruba and the Dutch part of St Maarten during 3 and 4 months, respectively. Samples were subjected to High Performance Liquid Chromatography (HPLC) screening of haemoglobin variants. RESULTS: Of the 368 samples (87.6% of all registered births) collected in Aruba, 10 (2.72%; CI 1.3, 4.9%) tested heterozygous for the Sickle-cell gene (HbAS) and 7 (1.90%; CI 0.8, 3.9%) for the haemoglobin C gene (HbAC). Of the 193 samples (83.5%) collected in St Maarten, 14 (7.25%; CI 4.0, 11.9%) contained HbAS and 10 (5.18%; CI 2.5, 9.3%) HbAC. HardyWeinberg equilibrium predicted an incidence of 2.65% for HbAS and 1.86% for HbAC in Aruba and 6.80% for HbAS and 4.86% for HbAC in St Maarten. These figures imply a newborn rate of about 2 SCD patients per 3 years in Aruba and 2 SCD patients per year in St Maarten. CONCLUSIONS: Universal screening of newborns for SCD seems costeffective for St Maarten.

OBJETIVO: Estimar la incidencia de la enfermedad de células falciformes (ECF) en Aruba y St Marteen y determinar si una pesquisaje universal sería costoefectivo de acuerdo con los criterios del Reino Unido. MÉTODOS: Se recogieron muestras de sangre de cordón umbilical en Aruba y en la parte holandesa de St Maarten durante 3 y 4 meses, respectivamente. Las muestras fueron sometidas a pesquisaje de variantes de hemoglobina mediante cromatografía líquida de alta eficiencia (CLAE). RESULTADOS: De las 368 muestras (87.6% de todos los nacimientos registrados) recogidas en Aruba, 10 (2.72%; CI 1.3, 4.9%) resultaron heterocigóticos para el gen de la célula falciforme (HbAS) y 7 (1.90%; CI 0.8, 3.9%) para el gen de la hemoglobina C (HbAC). De las 193 muestras (83.5%) recogidas en St Maarten, 14 (7.25%; CI 4.0, 11.9%) contenían HbAS y 10 (5.18%; CI 2.5, 9.3%) HbAC. El equilibrio de HardyWeinberg predijo una incidencia de 2.65% para HbAS y 1.86% para HbAC en Aruba y 6.80% para HbAS y 4.86% para HbAC en St Maarten. Estas cifras implican una tasa de recién nacidos de alrededor de 2 pacientes ECF por año en St Maarten. CONCLUSIONES: El pesquisaje universal para la detección de recién nacidos ECF parecer ser costoefectivo para St Maarten.

Estimated incidence of sickle-cell disease in Aruba and St. Maarten suggests cost-effectiveness of a universal screening programme for St. Maarten.

OBJECTIVE: To estimate the incidence of Sickle-Cell Disease (SCD) in Aruba and St. Maarten and to determine whether universal screening would be cost-effective according to United Kingdom criteria. METHODS: Consecutive cord blood samples were collected in Aruba and the Dutch part of St. Maarten during 3 and 4 months, respectively. Samples were subjected to High Performance Liquid Chromatography (HPLC) screening of haemoglobin variants. RESULTS: Of the 368 samples (87.6% of all registered births) collected in Aruba, 10 (2.72%; CI 1.3, 4.9%) tested heterozygous for the Sickle-cell gene (HbAS) and 7 (1.90%; CI 0.8, 3.9%) for the haemoglobin C gene (HbAC). Of the 193 samples (83.5%) collected in St. Maarten, 14 (7.25%; CI 4.0, 11.9%) contained HbAS and 10 (5.18%; CI 2.5, 9.3%) HbAC. Hardy-Weinberg equilibrium predicted an incidence of 2.65% for HbAS and 1.86% for HbAC in Aruba and 6.80% for HbAS and 4.86% for HbAC in St. Maarten. These figures imply a newborn rate of about 2 SCD patients per 3 years in Aruba and 2 SCD patients per year in St. Maarten. CONCLUSIONS: Universal screening of newborns for SCD seems cost-effective for St. Maarten.

Left ventricular assist system support is associated with persistent inflammation and temporary immunosuppression.

BACKGROUND: In patients undergoing left-ventricular assist system support, it has not been elucidated to which extent mechanical circulatory support itself as opposed to the underlying condition of endstage heart-failure contributes to perturbation of immune homeostasis. METHODS: In eleven heart transplant candidates who had to undergo Novacor left-ventricular assist device bridging, we prospectively sampled interleukin-6, T-cell and monocyte subsets and compared them to fifteen UNOS status II patients awaiting cardiac transplantation on medical heart failure treatment as outpatients at the time of LVAS implantation/listing decision as well as 2.0+/-1.2 months and 4.5+/-2.3 months later. In order to assess deviations in both groups from normal values, thirty-two healthy subjects served as reference group. RESULTS: Patients undergoing Novacor bridging had higher C-reactive protein, leukocyte, neutrophil, and monocyte levels at all three times, and exhibited lower CD3 +, CD4+, CD3+/CD45 RO T-cell and natural killer cell counts than medically treated patients awaiting transplantation 2 months after the LVAS implantation/listing decision. In comparison to controls, both groups had higher levels of inflammatory activation and lower levels of immunocompetence at all three times. CONCLUSIONS: While both groups of endstage heart failure patients show immunological alterations compared to controls, patients who have to be bridged by the Novacor LVAS exhibit a more pronounced activation of inflammatory markers. This may be due to more advanced heart failure but the device itself also may contribute to more pronounced inflammation and a temporary suppression of immunocompetent cells.