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Biomedical subjects

F D Muskiet

Publications and source records attributed to F D Muskiet.

16 recordsLinked to original sources

[Salmonella osteomyelitis in a child with sickle cell disease].

In an eight-months-old girl with sickle cell disease, osteomyelitis due to Salmonella arizona was diagnosed. Osteomyelitis caused by Salmonella species is rare in children. However, in patients with sickle cell disease it is the responsible pathogen in more than 50% of cases. The differentiation between, the much more common, bone crisis and osteomyelitis in sickle cell patients is often difficult. Ultrasound and bone marrow scans may be helpful. It is not known why Salmonella causes osteomyelitis in patients with sickle cell disease. What is clear, however, is that osteomyelitis usually occurs shortly after a preceding bone crisis. Empiric antibiotic treatment of osteomyelitis in patients with sickle cell disease should include coverage for Salmonella species. The patient described was initially treated with cefuroxime and gentamicin, but once the culture result was known this was switched to amoxicillin. As new infection foci later occurred in the bone the treatment was switched to ceftriaxone i.v. which was later substituted by ciprofloxacin orally. With this all of the skeletal abnormalities were fully corrected.

Anemia, Sickle Cell↗

Respiratory distress syndrome in Curaçao. Conventional versus surfactant treatment.

The aim of this study was to determine the incidence of Respiratory Distress Syndrome (RDS) and to evaluate the efficacy of surfactant treatment at the Neonatal Intensive Care Unit (NICU) at the St Elisabeth Hospital, Curaçao, Netherlands, Antilles. This was a retrospective cohort study of 86 infants, with moderate to severe RDS, out of 877 newborns admitted to the NICU between 1991 and 1998. Results of conventional RDS treatment between 1991 and 1994 (n = 54, group 1) were compared to results of treatment between 1994 and 1998 (n = 32, group 2) with surfactant and increased prenatal steroids. The incidence of RDS in group 1 was 12%, and 7.5% in group 2. Use of prenatal steroids increased from 7.3% (group 1) to 47% in group 2 (p < 0.05). Twenty-five infants died, 17 (31.5%) in group 1 and 8 (25%) in group 2. The complication most frequently found in both study groups was Bronchopulmonary Dysplasia (BPD): sixteen infants (30%) in group 1 and 9 infants (28%) in the surfactant-treated group. BPD was significantly associated with time on the ventilator in both groups (p < 0.05). We found no cases (0%) of Retinopathy of Prematurity (ROP) in group 1, and 3 cases (9%, p < 0.05) in group 2. We found no differences in other complications between group 1 and 2. The mean time between birth and the first surfactant treatment in group 2 was more than nine hours. Surfactant rescue treatment in combination with prenatal steroids results in lower incidence of RDS and in lower mortality than conventional RDS treatment in this study. The increased incidence of ROP in the surfactant-treated group was probably the result of better detection. BPD and other complications remained unchanged. Earlier surfactant administration is suggested to reduce mortality and morbidity in the future.

Biological Products↗

Surfactant treatment in premature infants with Respiratory Distress Syndrome in Curaçao.

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 Curaçao, 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 Curaçao between 1994 and 1998. Of 429 infants admitted to the study hospital in this period, 7.5% (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%) 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 newborns. This pilot study shows that surfactant treatment of premature infants is feasible in Curaçao. Earlier administration of surfactant, preferably within 2-3 hours after birth, is expected to lower the risk of death and oxygen dependence.

Bronchopulmonary Dysplasia↗

Elevated homocysteine levels indicate suboptimal folate status in pediatric sickle cell patients.

We investigated whether pediatric patients with sickle cell disease (SCD) (9 +/- 4 years; 27 homozygous SCD [HbSS]; 19 sickle-C disease [HbSC]) have different folate status compared with age-, sex-, and race-matched normal hemoglobin (HbAA) controls (n = 20), and whether their folate status can be improved by folate supplementation. The patients were supplemented with vitamins B6 and B12 during one week and with folate during the following week. Circulating folate, homocysteine, vitamin B6 and vitamin B12 levels were measured at baseline (patients and controls), after one week and after two weeks (patients). The patients had similar folate, vitamin B6, and vitamin B12, but higher homocysteine levels compared with HbAA controls (12.7 +/- 4.5 vs. 10.9 +/- 3.5 micromol/l; P = 0.04). Vitamin B6 and B12 supplementation did not change their homocysteine levels, but folate supplementation caused a 53% reduction (to 5.7 +/- 1.6). We conclude that patients with SCD have adequate vitamin B6 and B12 status, but suboptimal folate status, leading to elevated plasma homocysteine levels. They may therefore benefit from folate supplementation to reduce their high risk for endothelial damage.

Adolescent↗

Serum calcium and vitamin D status of patients with sickle cell disease in Curaçao.

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.

Adolescent↗

[High percentage of antibiotic resistance in Shigella infections in children in Curaçao].

OBJECTIVE: To evaluate antimicrobial treatment and resistance in clinical childhood shigellosis. DESIGN: Retrospective. SETTING: St. Elisabeth Hospital, Willemstad, Curaçao, Dutch Antilles. METHOD: From September 1991 through August 1995 shigellosis was diagnosed in 93 children out of 456 hospitalised with gastroenteritis (S. flexneri in 60, S. sonnei in 32, S. dysenteriae in 1). From hospital and laboratory records, the clinical presentation, antibiotic treatment and duration of hospitalization were indexed as well as the antibacterial resistance pattern of shigellae. RESULTS: Of the hospitalised children 52 (56%) were treated with antibiotics. Ampicillin was given most frequently (71%), followed by the combination trimethoprim-sulfamethoxazole (25%). Isolated shigellae were resistant to ampicillin in 52% and to trimethoprim-sulfamethoxazole in 34%; 42% of the antibiotic treatments were in accordance with susceptibility of the isolated Shigella. CONCLUSION: A high percentage of shigellae isolated on Curaçao was resistant to the most frequently used antibiotics ampicillin and trimethoprim-sulfamethoxazole.

Ampicillin↗

[Inadequate screening for congenital syphilis on Curaçao; 1987-1991].

OBJECTIVE: To evaluate if routine antenatal screening for congenital syphilis (CS) was adequately implemented. DESIGN: Retrospective study. SETTING: Curaçao, St. Elisabeth Hospital. METHOD: From 1987-1991 16 infants were treated for congenital syphilis in the paediatric department of the St. Elisabeth Hospital. From hospital and lab records, syphilis serology of their mothers before and during pregnancy and at delivery were indexed as well as cord blood values. The response in case of positive syphilis serology was traced. RESULTS: During the evaluation period the congenital syphilis incidence was 1.1/1000 life born infants. 9 pregnant women avoided prenatal care. Despite positive syphilis serology in the 1st (1 patient) and 3rd trimester (4 patients) no action was undertaken. In 4 neonates with congenital syphilis no cord blood sample for screening was taken. On 2 occasions the cord blood RPR was false-negative. (Re)screening was not performed at delivery in 3 mothers although positive serology was found during pregnancy. CONCLUSION: Screening for congenital syphilis was not always applied. Insufficient action was noted if positive syphilis serology was detected. Intensification of screening for congenital syphilis in Curaçao is necessary especially for mothers with poor prenatal care. The need for immediate post partum screening for mother and child is stressed.

Adult↗

Screening cord blood for hemoglobinopathies and thalassemia by HPLC.

We evaluated the use of an HPLC method for screening hemoglobins in cord blood. We studied the genotype frequencies of the structural hemoglobin variants HbS and HbC and the synthesis variants alpha- and beta(+)-thalassemia in babies born on Curaçao. During three months, 67.2% of all (748) newborns were screened: 122 (24.3%) had an abnormal hemoglobin pattern, of which 53 (43.4%) had a hemoglobinopathy (HbS or HbC), 64 (52.2%) had alpha-thalassemia (HbBarts greater than 0.5%, corresponding to heterozygous or homozygous alpha-thalassemia-2), and 5 (4.1%) had a hemoglobinopathy plus alpha-thalassemia. None of the newborns with heterozygous HbS and HbC had concomitant beta(+)-thalassemia. The population genotype frequency of heterozygous alpha-thalassemia-2 was calculated to be 30.7%. The data are in excellent agreement with those previously established for the adult population and those available from the black population in the United States and Jamaica. Based on the HPLC results, we estimate that 67.1% of newborns with heterozygous alpha-thalassemia-2 remain undetected. A coincidental finding was a relation between demonstrable alpha-thalassemia and short gestation. Because of its superior separating power and high sensitivity for quantifying relatively low percentages of hemoglobins in the presence of HbF0, the HPLC method was preeminently suitable for screening cord-blood samples.

Chromatography, High Pressure Liquid↗

Supplementation of patients with homozygous sickle cell disease with zinc, alpha-tocopherol, vitamin C, soybean oil, and fish oil.

Thirteen patients (aged 0.7-17.9 y) with homozygous sickle cell disease were supplemented with alpha-tocopherol, vitamin C, zinc, and soybean oil (suppl 1; for 8 mo) and alpha-tocopherol, vitamin C, and fish oil (suppl 2; for 7 mo). Urinary zinc (suppl 1), plasma vitamin C, plasma cholesterol ester and erythrocyte (RBC) omega 3 fatty acids (suppl 2), and plasma and RBC alpha-tocopherol (suppl 1 and 2) increased. Suppl 1 decreased irreversibly sickled cells by 37.5%, decreased RBC protoporphyrin and urinary porphyrins, and increased the RBC total fatty acid-cholesterol ratio. Suppl 2 decreased plasma triglycerides, further increased the RBC alpha-tocopherol, moderately increased the RBC double-bond index, but decreased the RBC total fatty acid-cholesterol ratio. Zinc, copper, and porphyrins showed prolonged changes. The supplements did not change hemoglobin concentrations, RBC age (reticulocytes, polyamines), or number of aplastic and vasoocclusive crises. Zinc reduces irreversibly sickled cells. Augmentation of RBC antioxidant status by alpha-tocopherol and vitamin C and incorporation of omega 3 fatty acids into RBCs do not affect hemolytic component. Effects on vasoocclusive component are unclear.

Adolescent↗

Lipids, fatty acids and trace elements in plasma and erythrocytes of pediatric patients with homozygous sickle cell disease.

Plasma and erythrocyte levels of zinc, copper, fatty acids, total cholesterol and cholesterol sulfate, and plasma vitamin E and free cholesterol were measured in six pediatric patients with HbSS sickle cell anemia, one adult patient in crisis and six age-, sex- and race-matched pediatric controls. Patient plasma zinc levels were significantly decreased, while erythrocyte zinc levels were normal. Although subject to a large range, plasma vitamin E levels were not significantly altered. For patients an increased plasma percentage of free cholesterol, increased levels of oleic acid and vaccenic acid, and decreased values for total cholesterol were found. Erythrocyte fatty acid analyses revealed a significant decrease in total polyunsaturated fatty acids which was 'compensated' for by increased total monounsaturated and saturated fatty acids. The low amounts of total polyunsaturated fatty acids could completely be ascribed to decreased levels of linoleic acid. Erythrocyte cholesterol levels were significantly increased, while the total fatty acid/cholesterol molar ratio was found to be subject to a relatively large range. Cholesterol sulfate determinations showed that patients had relatively low plasma or erythrocyte levels, or both. The present results are suggestive of a lipid peroxidation mediated, and hepatic and/or splenic dysfunction mediated lipid component in the rigidification of the sickle cell membrane.

Adolescent↗