Physician end-of-life decision-making in newborns in a less developed health care setting: insight in considerations and implementation.

BACKGROUND: A substantial proportion of the decisions to withhold or withdraw life-prolonging treatment are based on the newborn's predicted poor quality of life. All previous studies on end-of-life decisions were done in countries with adequate support for disabled neonatal intensive care units (NICU) survivors. Data on quality-of-life considerations in countries with developing health care are not available yet. AIM: The aim of the study was to examine the considerations of physicians taking end-of-life decisions in sick newborns and how those decisions are carried out in practice in a less developed health care setting. METHOD: Thirty-two deaths over 18 months in a neonatal unit were retrospectively analyzed. RESULTS: Twenty-four deaths (75%) were attributable to withholding or withdrawing of treatment. In 7 of these cases (29%), the decisions were based on quality-of-life considerations, mostly predicted suffering and expected hospital dependency. For the majority of paediatricians, end-of-life decision making was not influenced by legal or economic considerations or by considerations regarding availability of supportive care after discharge. CONCLUSION: Our study suggests that physician end-of-life decision making in this unit in a less developed health care setting is found to be similar to that in developed health care settings and is independent of availability of supportive care after discharge for infants with disabilities.

[Salmonella osteomyelitis in a child with sickle cell disease]. / Salmonella-osteomyelitis bij een patiëntje met sikkelcelanemie.

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.

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.

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.

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.

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)

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.

Optimization of daily folate, vitamin B12 and vitamin B6 supplements in paediatric patients with sickle cell disease

We determined optimal folate, vitamin B12 and vitamin B6 dosages in 21 sickle cell disease (SCD) patients (11 HbSS, 10 HbSC; mean 7 years, range 7-16), using plasma homocysteine (Hcy) as functional marker. They received daily 400 g (0-3 weeks), 700 g (3-6) and 1000 g (6-70) folate; 1 (0-21), 3 (21-45 and 5 RDA (45-70) vitamin B12; and 1 RDA vitamin B6 (0-70). Blood was taken at baseline (P0) and after 3 (PI), 6 (P2), 9 (P3), 21 (P4), 33 (P5), 45 (P6), 57 (P7) and 70 (P8) weeks for measurement of erythrocyte (RBC), serum folate, plasma vitamin B12, whole blood vitamin B6 and plasma Hcy. Vitamin B6 increased from P0 to P1 and P1 to P2; vitamin B12 from P4 to P8; serum folate from P0 to P1 and P1 to P2; RBC folate from P0 to P1, P1 to P2 and P2 to P3. Hcy decreased from P1 to P2 and P4 to P6. Most pronounced Hcy decreases occurred from P0 to P1 (43 percent of patients), P1 to P2 (14 percent) and P4 to P5 (24 percent). Haematological indices did not change. Patients with HbSS had higher RBC folate at P1, P2 and P8. The entire group exhibited inverse relations between RBC folate and haemoglobin on P1, P2, P3, P6, P7 and P8. We conclude that RBC folate is less valuable for folate status assessment in SCD patients. The optimal daily supplement is 700 g folate (3.5-7 RDA vitamin B12 (4.2-6.0 g) and 1 RDA vitamin B6 (1.4-2.0 mg). This combination causes Hcy levels that do not decrease further upon higher dosages and may reduce by simple and relatively inexpensive means their inherently high risk of endothelial damage.(Au)

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.

Enhanced sVCAM-1 levels in paediatric patients with sickle cell disease

Recently, several reports have shown endothelial activation to play an important role in the pathophysiology of sickle cell vaso-occlusion. We measured serum soluble VCAM-1 and soluble ICAM-I levels in steady state paediatric sickle cell patients. TNF, an endothelial activating cytokine, was also measured. sVCAM-I levels were increased as compared to age, sex and race-matched controls (p=0.002), whereas sICAM-I levels were not significantly enhanced. TNF levels were also elevated in paediatric sickle cell patients as compared to controls (p=0.01). These results show that endothelial activation is already manifest at a very young age in sickle cell patients, probably resulting from enhanced endothelial activating cytokines, such as TNF.(AU)

Folic acid status of paediatric patients with sickle cell disease

We investigated whether paediatric patients with sickle cell disease (9ñ4 years; 27 HbSS; 19 HbSC) have different folic acid status compared with age-, sex-and race-matched HbAA controls (n=20), and whether their folic acid status can be improved by folic acid supplementation. The patients were supplemented with vitamins B6 and B12 during one week and with folic acid during the next week. Circulating folic acid, homocysteine, vitamin B6 and vitamin B12 levels were measured at baseline (patients and controls), after 1 and 2 weeks (patients). The patients had similar folic acid, vitamin B6 and vitamin B12, but higher homocysteine levels, compared with HbAA controls (12.7ñ4.5 vs 10.9ñ3.5 mmol/l;p=0.04). Vitamin B6 and B12 supplementation did not change their homocysteine levels, but folic acid supplementation caused a 52 percent reduction (to 5.7ñ1.6). We conclude that patients with sickle cell disease have adequate vitamin B6 and B12 status, but suboptimal folic acid status. They may benefit from folic acid supplementation to reduce their high risk for endothelial damage.(AU)

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.

[High percentage of antibiotic resistance in Shigella infections in children in Curaçao]. / Hoog percentage antibioticumresistentie bij Shigella-infectie van kinderen op 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.

Serum calcium and vitamin D status of patients with sickle cell disease in Curacao: comparison with Saudi Arabia

Data from Saudi Arabia suggest that low vitamin D status is involved in skeletal abnormalities of patients with homozygous sickle-cell disease (HbSS). 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 Curacao. Serum calcium of HbSS patients (2.32 ñ 0.07 mmol/l) was lower (ANCOVA, p = 0.002) than that of HbAA controls (2.44 ñ 0.14). None of the subjects had hypocalcaemia. There were no differences in phosphate, total protein, albumin, intact parathyroid hormone (PTH), 25-hydroxyvitamin D [25(OH)D; HbSS 87 ñ 27, HbAA 86 ñ 15 nmol/l] and 1,25-dihydroxyvitamin D. There were no significant relationships between serum calcium and albumin, calcium and total protein, and PTH and 25 (OH)D. Our data suggest that hypocalcaemia and hyperparathyroidic tendencies in Saudi Arabian HbSS patients are likely to be caused by the locally poor vitamin D status, attributable to insufficient exposure to direct sunlight (AU)

[Inadequate screening for congenital syphilis on Curaçao; 1987-1991]. / Onvoldoende screening op congenitale lues op 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.

Low sickle-cell linoleic acid content is related to young erythrocyte age - abstract

Erythrocyte (RBC) fatty acids (FA) and polyamines were determined in subjects with HvM (n=29), HbAC (3), HbAS (41), HbSC (25) and HbSS (19). FA of plasma cholesterol esters (CE), plasma phosphatidylcholines (PC), RBC PC, and plasma and RBC PC-species were studied in subgroups. RBC of patients with HbSS and HbSC had abnormal FA, PC-FA, PC-species and polyamines. There were no major differences in plasma CE-FA, PC-FA and PC-species. Low 18:2U6 in RBC, RBC PC and RBC PC-species of patients with HbSC and HbSS are related to RBC polyamines. Low RBC 18:2U6 is almost stoichiometrically compensated for by higher stearic and palmitic acids. Circulating RBC from patients with HbSC and HbSS have normal total polyunsaturated FA with 20 carbons or more. Low RBC 18:2U6 of patients with HbSS and HbSC is rather related to young RBC-age (RBC polyamines) than to diet (plasma CE-FA). Their rapid RBC turnover causes incomplete RBC-FA exchange with plasma species (AU)