Search

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.

We measured parameters of calcium homeostasis and vitamin D status in HbSS patients (median age 8 years, range 3-19; 8 females, 10 males) and matched HbAA controls living in the tropical island of Curaçao. Serum calcium concentration in HbSS patients [2.32(0.07)mmol/L] was lower (ANCOVA, P = 0.002) than that of HbAA controls [2.44(0.14)]. None of the subjects had hypocalcaemia. There were no differences in serum concentrations of phosphate, total protein, albumin, intact parathyroid hormone (PTH), 25-hydroxyvitamin D [87(27) nmol/L in patients, 86(15) nmol/L in controls) and 1,25-dihydroxyvitamin D. There were no significant relations between PTH and 25(OH)D. We conclude that vitamin D status of HbSS patients in Curaçao is adequate.

Plasma cholesterol ester and triglyceride fatty acid compositions of 38 singleton deliveries (23-42 wk), three twins (32, 39, and 40 wk), and their mothers were investigated. No gestational age-dependent changes occurred in maternal fatty acid compositions. Long-chain polyunsaturated fatty acids in fetal cholesterol esters and triglycerides were higher, and their precursors (18:2c,omega 6 and 18:3c,omega 3) were lower than those in corresponding maternal lipid classes. Except for 22:6c,omega 3,20:2c,omega 6, and 22:5c,omega 6, all long-chain polyunsaturated fatty acids in fetal triglycerides increased with advancing gestation. Fetal triglyceride 22:6c,omega 3/22:5c,omega 3 ratio decreased, whereas 22:5c,omega 6/22:4c,omega 6 remained constant. Fetal cholesterol ester and triglyceride 20:3c,omega 9 contents were higher than those of corresponding maternal fractions and did not change with gestation. Triglyceride 18:2c,omega 6 contents of babies with gestational ages of more than 34 wk were linearly related to those of their mothers. The data suggest that increasing triglyceride long-chain polyunsaturated fatty acid content with advancing gestation is partially caused by delta 6- and delta 5-desaturase maturation in the liver. Constancy of 22:6c,omega 3 and 22:5c,omega 6/22:4c,omega 6 and decrease of 22:6c,omega 3/22:5c,omega 3 in triglycerides may point to low hepatic delta 4-desaturation. Transplacental transport of 20:3c,omega 9, following by fetal conservation, should be considered. High 18:2c,omega 6 and low 18:3,omega 3 intakes by the mother may unfavorably influence fetal production of 22:6c,omega 3 in the liver. Because of low hepatic delta 4-desaturation capacity the influence may be small, however.

Triglycerides, cholesterol, fatty acid composition, and tocopherols were determined in colostrum, transitional milk, and mature milk in St Lucia. With progress of lactation, triglycerides and percentage medium-chain fatty acids increased whereas tocopherols, cholesterol, and percentage long-chain polyunsaturated fatty acids decreased. These changes reflect augmented de novo synthesis of fatty acids (8:0, 10:0, 12:0, and 14:0) in the mammary gland and a tendency of increasing fat-globule size as milk matures. Transitional and mature milks, but particularly colostrum, contained higher concentrations of components considered to be derived from the fat-globule membrane (cholesterol, tocopherols, percentage long-chain polyunsaturated fatty acids) compared with those reported for Western countries. Percentage medium-chain fatty acids in mature milk was two to three times higher than in developed countries. Differences from data from studies in Western countries are discussed in relation to analytical methods and possible consequences for lipid digestion, lipid absorption, growth, and brain development.

We isolated phospholipid (PL) subclasses from milk of women in Dominica and Belize. Fatty acid (FA) compositions of PLs and total lipids were determined. In the total-lipid fraction Dominican milk showed higher relative amounts of medium-chain saturated fatty acids (MC-SAFAs; 6:0-14:0) and 22:6n-3 and lower amounts of long-chain saturated fatty acids (LC-SAFAs) and monounsaturated fatty acids (MUFAs). There was a positive relationship between the MC-SAFA content in total lipids and total PLs. Incorporation of MC-SAFAs in PLs occurred at the expense of LC-SAFAs, MUFAs, polyunsaturated fatty acids (PUFAs), and long-chain PUFAs with greater than or equal to 20 carbon atoms (LC-PUFAs greater than or equal to C20). Previous studies from Western countries revealed low amounts of MCSAFAs and high amounts of PUFAs and LC-PUFAs greater than or equal to C20 in milk PLs. Our data show that carbohydrate-rich diets give rise to incorporation of MC-SAFAs in PLs at the expense of PUFAs and LC-PUFAs greater than or equal to C20. The data are discussed in relation to the presumed origin of fat-globule membrane phospholipids.