Surfactant treatment in premature infants with respiratory distress syndrome in Curacao

Surfactant replacement therapy for Respiratory Distress Syndrome (RDS) in premature neonates has been established as an effective treatment, although significant mortality and morbidity remain. In Curacao, surfactant became available as a therapeutic option in 1994. A retrospective cohort study was performed to describe the results of surfactant treatment in premature newborns with RDS in Curacao between 1994 and 1998. Of 429 infants admitted to the study hospital in this period, 7.5 percent (n=32) developed RDS and were treated with surfactant. Twenty-five per cent (n=8) of these infants died, most of them in the first year of surfactant treatment. Twenty-eight per cent (n=9) developed bronchopulmonary dysplasia (BPD), the most frequently observed complication. The highest incidence of BPD (44 percent) was found in the very low birth weight infants (750-1500 g); all infants with BPD were 27-30 weeks of gestational age. The duration of ventilator dependence was significantly associated with the development of BPD (p < 0.05). No other risk factors for complications during the treatment course could be identified. The mean time between birth and the first surfactant treatment was more than nine hours. In this study, we found low incidence rates of RDS and BPD, and a considerable mortality in surfactant treated surfactant treatment newborns. This pilot study shows that surfactant treatment of premature infants is feasible in Curacao. Earlier administration of surfactant, preferably within 2-3 hrs after birth, is expected to lower the risk of death and oxygen dependence.(Au)

Familial partial monosomy 5p and trisomy 5q; three cases due to paternal pericentric inversion 5 (p151q333).

A family is described in which the mother's 9 pregnancies ended in the birth of 2 healthy girls, 4 spontaneous abortions and 3 infants with multiple congenital malformations as bird-headed appearance, pre- and postnatal growth deficiency, microcephaly, micrognathia with small mouth and cat-like cry. Two of the three affected sibs had complex cardiac malformations incompatible with life; the third had a bicuspid aortic valve. Chromosomal investigation revealed an abnormal karyotype: 46,XX,rec(5),dupq,inv(5)(p151q333)pat, leading to a partial monosomy 5p and partial trisomy 5q. A large pericentric inversion of chromosome 5 was found in the father: 46,XY,inv(5)(p151q333) as well as in the firstborn healthy female sib. The clinical features partly fit the partial monosomy 5p as well as the partial trisomy 5q syndrome.