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Papers by eric sulkers
The American Journal of Clinical Nutrition, 2000
Background: Currently available preterm formulas with energy contents of 3350 kJ (800 kcal)/L pro... more Background: Currently available preterm formulas with energy contents of 3350 kJ (800 kcal)/L promote weight and length gain at rates at or above intrauterine growth rates but disproportionately increase total body fat. Objective: The objective of this study was to determine whether fat accretion in formula-fed, very-low-birth-weight (VLBW) infants could be decreased and net protein gain maintained by reducing energy intakes from 502 kJ (80 kcal) • kg Ϫ1 •d Ϫ1 [normal-energy (NE) formula] to 419 kJ (100 kcal) • kg Ϫ1 •d Ϫ1 [low-energy (LE) formula] while providing similar protein intakes (3.3 g • kg Ϫ1 •d Ϫ1). Design: The study was a randomized, controlled trial enrolling 20 appropriate-for-gestational-age (AGA) and 16 small-for-gestational-age (SGA) VLBW infants (mean birth weight: 1.1 kg; mean gestational age: 31 wk); energy expenditure and nutrient balance were measured at 4 wk of age and anthropometric measurements were made when infants weighed 2 kg. Results: The percentage of fat in newly formed tissue was significantly lower in AGA infants fed the LE formula (n = 9) than in those fed the NE formula (n = 10) (9% compared with 23%; analysis of variance, P = 0.001). Energy expenditure was higher in AGA infants fed the NE formula than in those fed the LE formula. Skinfold thickness was markedly lower in AGA infants fed the LE formula than in those fed the NE formula, resulting in a lower estimated percentage body fat (8.0 ± 1.9% and 10.8 ± 3.5%, respectively; P < 0.05). Three of 6 SGA infants fed the LE formula were excluded during the study because of poor weight gain. Conclusions: Body composition can easily be altered by changing the energy intakes of formula-fed VLBW infants. Energy intakes in these infants should be > 419 kJ (100 kcal) • kg Ϫ1 • d Ϫ1 .
The American Journal of Clinical Nutrition, 1990
Parenterally fed preterm neonates are known to be at risk for carnitine deficiency. We studied su... more Parenterally fed preterm neonates are known to be at risk for carnitine deficiency. We studied substrate utilization in low-birth-weight infants receiving total parenteral nutrition (TPN) with (A) and without (B) supplementation of 48 mg carnitine. kg '. d ' on days 4-7 (birth weights 1 334 ± 282 vs 13 18 ± 248 g, gestational age 32 ± 2 vs 32 ± 2 wk, A vs B, respectively). TPN consisted of 1 1 g glucose. kg '. d and 2.4 g. kg '. d ' of both protein and fat. Plasma carnitine concentrations at day 7 were for free carnitine 1 1 .8 ± 5.0 vs 164 ± 56 zmol/L and for acyl carnitine 3.8 ± 2.0 vs 33.9 ± 1 5.4 tmol/ L, respectively. Indirect calorimetry at day 7 showed a higher fat oxidation (0.21,-0.31 to +0.60 vs 1.18, 0.70 to 1.95 g. kg .d , respectively, P < 0.02, median and interquartile range) in group B and a higher protein oxidation (0.37, 0.30-0.43 vs 0.63, 0.53-0.88 g.kg1 .d', P < 0.001). The time to regain birth weight was also higher in group B (7, 5.5-9 vs 9, 7-14 d, P < 0.05). Carnitine supplementation and calorie intake were the best explanatory variables for metabolic rate (R2 = 0.45, P < 0.002). We conclude that carnitine supplementation ofTPN in this dosage does not seem advisable.
Comparison of two preterm formulas with or without addition of medium-chain triglycerides (MCTs) ... more Comparison of two preterm formulas with or without addition of medium-chain triglycerides (MCTs) II: effects on mineral balance.
The Journal of Clinical Endocrinology & Metabolism, 2015
Context: Previously we showed that pubertal children born small for gestational age (SGA) with a ... more Context: Previously we showed that pubertal children born small for gestational age (SGA) with a poor adult height (AH) expectation can benefit from treatment with GH 1 mg/m 2 per day (ϳ0.033 mg/kg/d) in combination with 2 years of GnRH analog (GnRHa) and even more so with a double GH dose. GnRHa treatment is thought to have negative effects on body composition and blood pressure. Long-term effects and GH-dose effects on metabolic health in children treated with combined GH/GnRHa are unknown. Objective: This study aimed to investigate body composition, blood pressure, and lipid profile during GH treatment, either with or without 2 years of additional GnRHa. To assess whether GH 2 mg/m 2 per day (ϳ0.067 mg/kg/d) results in a similar or even more favorable metabolic health at AH than GH 1 mg/m 2 per day. Methods: This was a longitudinal, randomized, dose-response GH trial involving 107 short SGA children (58 girls) treated with GH until AH (GH randomized 1 or 2 mg/m 2 /d during puberty). Sixty-four children received additional GnRHa. At AH, metabolic parameters were compared between children treated with combined GH/GnRHa and those with only GH. The GH dose effect on metabolic health was evaluated in a subgroup of 47 children who started GH treatment in early puberty (randomized 1 or 2 mg/m 2 /d) with 2 years of GnRHa. Results: At AH, fat mass percentage (FM%) SD score (SDS), lean body mass (LBM) SDS, blood pressure SDS, and lipid profile were similar between children treated with combined GH/GnRHa and those with only GH. In the pubertal subgroup, FM% SDS was lower during treatment with GH 2 mg/m 2 per day. There was no GH dose-dependent effect on LBM SDS, blood pressure, and lipid profile. Conclusions: Combined GH/GnRHa treatment has no long-term negative effects on metabolic health compared with only GH. Started in early puberty, a GH dose of 2 mg/m 2 per day results in a similar metabolic health at AH and a more favorable FM% than GH 1 mg/m 2 per day. (J Clin Endocrinol Metab 100: 3725-3734, 2015) B eing born small for gestational age (SGA) has been associated with a higher prevalence of diabetes mellitus type 2, hypertension, and hyperlipidemia at a relatively young adult age (1). Because 10% of children born SGA have persistent short stature (2-4), many of them are treated with GH to increase adult height (AH) (5-10). Long-term GH treatment results in an increase in lean body mass (LBM), a decline in fat mass (FM), and a decrease in blood pressure (BP) and lipid profile (11-13).
Nederlands tijdschrift voor geneeskunde, 2009
Adherence to medication is a common problem in chronically ill patients. Three patients are prese... more Adherence to medication is a common problem in chronically ill patients. Three patients are presented who did not or did not fully adhere to their medication: a 3-year-old girl with asthma, a 57-year-old man with COPD and a 16-year-old boy with diabetes. This illustrates that adherence is not determined by age or illness. Known determinants of adherence are the patient's beliefs about illness and medication. Since these beliefs differ from patient to patient, dealing with non-adherence requires patient-centred care characterized by concordance, i.e. shared decision-making about therapy by doctors and patients. Appropriately targeted interventions, such as motivational interviewing, may influence patient beliefs and improve adherence.
Maatwerk, 2009
ABSTRACT Andrew Turnell is samen met Steve Edwards ontwerper van Signs of Safety, een praktijkger... more ABSTRACT Andrew Turnell is samen met Steve Edwards ontwerper van Signs of Safety, een praktijkgerichte benadering voor het werken in gezinnen met meervoudige problemen. Eric Sulkers, vertrouwenarts bij het AMK Zeeland en lector Sietske Dijkstra spraken Turnell in maart toen hij in Nederland een masterclass gaf.
Pediatric Research, 1989
Medium-chain triglycerides, with a chain length of eight and 10 carbon atoms, form up to 50% of t... more Medium-chain triglycerides, with a chain length of eight and 10 carbon atoms, form up to 50% of the total fat content in some preterm infant formulas. In 20 small preterm infants (birthwt: 1153 f 227 g; mean 2 SD) fed a special formula containing 40% MCT, a primed constant oral infusion study of l-'3C-potassium octanoate was conducted to quantify the oxidation of MCT. A plateau in 13C enrichment in breath COz was reached in all patients within 1-3 h. Simultaneously, substrate utilization was measured using a closed system indirect calorimeter. No significant difference was found between appropriate for gestational age (n = 8) and small for gestational age (n = 12) infants in the percentage of the administered tracer that was oxidized (44.9 f 9.1 % versus 48.5 f 11.0%). In all patients, the recovery was calculated to be 47.1 f 10.2%, which is less than previously estimated and corresponds to a mean MCT oxidation of 1.26 f 0.27 g/kg/d. With indirect calorimetry, a total fat oxidation of 1.42 f 0.84 g/kg/d in appropriate for gestational age and 2.00 2 0.85 g/kg/d in small for gestational age infants was found, indicating that MCT accounted for around 85% of the total fat oxidation in appropriate for gestational age versus 65% in small for gestational age infants.
Pediatric Research, 1990
During total parenteral nutrition in preterm infants, glucose may be infused at high rates, but i... more During total parenteral nutrition in preterm infants, glucose may be infused at high rates, but it is not known if the endogenous glucose production is fully suppressed under these circumstances. Eight preterm appropriate for gestational age (AGA) (birth wt: 1613 f 151 g, gestational age: 31.1 f 1.5 wk) and eight preterm small for gestational age (SGA) newborn infants (1 185 f 241 g, 32.9 2 2.6 wk) receiving a glucose infusion rate of 7.55 2 0.56 and 8.16 f 0.65 mg/kg. min, respectively, were studied during continuous total parenteral nutrition at postnatal d 8. Glucose oxidation rate was determined with a primed constant infusion of [U-"q glucose, measuring the '"02 production in breath gas by isotope ratio mass spectrometry and the glucose production rate in plasma by gas chromatography mass spectrometry. In breath gas of AGA and SGA infants, 60 and 6576, respectively, of the infused tracer appeared as ' T O 2. The glucose production rates were 7.97 2 1.61 and 8.1 2 f 1.84 mg/kg. min in AGA and SGA infants, respectively, indicating that no significant endogenous glucose production occurred. The glucose oxidation calculated from the glucose production and I3CO2 production was 4.74 f 0.99 mg/kg.min in AGA infants and was significantly different from the carbohydrate oxidation rate of 6.62 f 1.23 mg/kg. min measured by simultaneous indirect calorimetry. In SGA infants, however, the glucose and carbohydrate oxidation rates were not significantly different at 5.33 f 1.56 and 6.16 f 2.45 mg/kg min. It is concluded that 1-wk-old AGA or SGA preterm infants receiving total parenteral nutrition of 80 kcallkg. d produce no endogenous glucose and their glucose oxidation rates are similar at 63-65% of the glucose infused. It is suggested that the significant difference between glucose and carbohydrate oxidation rates observed in AGA but not in SGA infants is due either to a higher rate of lipogenesis from carbohydrates, or, less likely, to a higher rate of glycogen oxidation. (Pediatr Res 28: 153-157, 1990) Abbreviations AGA, appropriate for gestational age R,, rate of glucose appearance in plasma RQ, respiratory quotient SGA. small for eestational aee PATIENTS AND METHODS TPN; total pare;;teral nutritik Patients. Eight AGA and eight SGA preterm newborn infants
Pediatric Research, 1991
Ventilated VLBW infahts usually receive only glucose during the first day of life. These infants ... more Ventilated VLBW infahts usually receive only glucose during the first day of life. These infants are prone to hypo-and hyper glycemia, and it is unknown if this is due to changes in glucose oxidation(G1uox) or non-oxidative disposal (NOD). We studied glucose turnover and oxidation in 7 ventilated VLBW infante (birthweight 1.25 * 0.30 kg; gestional age 32 t 2 weeks; postnatal
Pediatric Research, 1991
An energy intake of 120 kcal1kg.d in preterm infants resulted in a higher fat accretion than in u... more An energy intake of 120 kcal1kg.d in preterm infants resulted in a higher fat accretion than in utero. To study if a reduced energy intake would not only reduce fat accretion, but also influence proteinlprol turnovcrlOl, we measured leucine(leu1 kinetics in 4 wk old, orally fed VLBW infants, receiving 3.2 g pro1kg.d. They were fed either 120-In= l2,bw= 1.1 i 0. 2 kg,ga=30* 2 wk) or 100
Pediatric Research, 1991
An energy intake of 120 kcal1kg.d in prelerm infants resulted in a higher fat accretion than in u... more An energy intake of 120 kcal1kg.d in prelerm infants resulted in a higher fat accretion than in utero. To study if a reduced energy inlake would not only reduce fat accretion, but also influence protein(pro1 turnoverlOl, we measured leucine(leu1 kinetics in 4 wk old, orally fed VLBW infants, receiving 3.2 g pro1kg.d. They were fed either 120-In= 12,bw= 1.1 20.2 kg,ga=30+2 wkl or 100
Pediatric and Developmental Pathology, 1999
We report a novel case of partial trisomy 19q and concomitant partial monosomy 21q, segregated fr... more We report a novel case of partial trisomy 19q and concomitant partial monosomy 21q, segregated from a maternal translocation (19;21) (q13.1;q22.3), identified by spectral karyotyping. Clinical examination revealed dysmorphic features of the face and limbs, cleft palate, bilateral colobomas with associated bilateral colobomatous optic nerve cysts, hearing loss, and a cardiac anomaly. At autopsy, the dysmorphic features and cleft palate were confirmed. The ocular histopathology is described in detail and the cardiac anomaly was further specified. The combination of phenotype features is diagnostic of the CHARGE ( coloboma, heart malformation, atresia choanae, retarded growth and development, and/or CNS anomalies, genital hypoplasia, ear anomalies and/or deafness) association. This case also has some phenotypic features in common with previous cases of partial trisomy 19q. The importance of a complete autopsy in cases with multiple congenital anomalies and/or genetic abnormalities is e...
Neonatology, 1991
The accuracy of 8-hour indirect calorimetry (IDC) as an estimate of energy expenditure was invest... more The accuracy of 8-hour indirect calorimetry (IDC) as an estimate of energy expenditure was investigated in 8 healthy preterm infants (birth weight 1,270 ± 193 g, gestational age 32 ± 3 weeks, mean ± SD) in comparison with an analysis over 5 days using the doubly-labeled water (2H218O) method (DLW). The infants that were fed continuously by nasogastric drip with 120 kcal/kg/day of special infant formula were measured twice under thermoneutral conditions in a closed system indirect calorimeter during 8 h with a 4-day interval; simultaneously isotope decay was measured by isotope ratio mass spectrometry in urine samples collected daily during 5 days from 6 h after an oral dose of 2H2180 on the first day of IDC, all during the 4th postnatal week. The mean differences between carbon dioxide production rate (rCO2) measured either by single 8-hour IDC or by duplicate 8-hour IDC and the 5-day DLW method, using the two-point analysis or the multipoint analysis were not significantly differen...
Journal of Pediatric Gastroenterology & Nutrition, 1996
The structure of the triglycerides (TG) in human milk (HM) differs from those of vegetable oils u... more The structure of the triglycerides (TG) in human milk (HM) differs from those of vegetable oils used in infant formulas. In HM, palmitic acid is predominantly esterified to the center or beta-position of the TG, in vegetable oil, it is mainly at the external or alpha-positions. These differences in configuration affect intestinal fat absorption. Fat and mineral balances were investigated in three groups of 9 healthy term infants aged 5 weeks. Infants were randomly assigned to receive one of the three study formulas from birth: (a) formula beta, resembling the structure of HM fat most closely (24% palmitic acid, 66% esterified to beta-position), (b) formula intermediate (24% palmitic acid, 39% esterified to the beta-position), and (c) regular formula (20% palmitic acid; 13% esterified to the beta-position). Fat absorption was highest in infants fed the beta formula (97.6 +/- 0.9%), intermediate in those fed with the intermediate formula (93.0 +/- 1.8%), and lowest in infants receiving the regular formula (90.4 +/- 4.6%). Fecal calcium excretion was significantly lower in the beta group than in the other two groups (43.3 +/- 18.1 vs. 59.9 +/- 15.1 vs. 68.4 +/- 22.3 mg.kg-1.day-1 for beta, intermediate, and regular respectively). Dietary TG containing palmitic acid predominantly at the beta-position, as in HM, have significant beneficial effects on the intestinal absorption of fat and calcium in healthy term infants.
The Journal of Clinical Endocrinology & Metabolism, 2012
Context: GH treatment is effective in improving height in short children born small for gestation... more Context: GH treatment is effective in improving height in short children born small for gestational age (SGA). GH is thought to have limited effect when started during adolescence. Objective: The aim of this study was to investigate GH treatment efficacy in short SGA children when treatment was started during adolescence; to assess whether GH 2 mg/m 2 ⅐ d during puberty improves adult height (AH) compared with 1 mg/m 2 ⅐ d; and to assess whether an additional 2-yr postponement of puberty by GnRH analog (GnRHa) improves AH in children who are short at the start of puberty (Ͻ140 cm), with a poor AH expectation. Patients and Design: In this longitudinal, randomized, dose-response GH trial, we included 121 short SGA children (60 boys) at least 8 yr of age. We performed intention-to-treat analyses on all children and uncensored case analyses on 84 children who reached AH. Besides, we evaluated growth during 2 yr of combined GH/GnRHa and subsequent GH treatment until AH in a subgroup of 40 pubertal children with a height of less than 140 cm at the start. Results: Short SGA children started treatment at a median age of 11.2 yr, when 46% had already started puberty. Median height increased from Ϫ2.9 at start to Ϫ1.7 SD score (SDS) at AH (P Ͻ 0.001). Treatment with GH 2 vs. 1 mg/m 2 ⅐ d during puberty resulted in significantly better AH (P ϭ 0.001), also after correction for gender, age at start, height SDS at start, treatment years before puberty, and target height SDS. AH was similar in children who started puberty at less than 140 cm and received GH/GnRHa, compared with children who started puberty greater than 140 cm and received GH only (P ϭ 0.795). Conclusion: When started in adolescence, GH treatment significantly improves AH in short SGA children, particularly with GH 2 mg/m 2 ⅐ d during puberty. When SGA children are short at the start of puberty, they can benefit from combined GH/GnRHa treatment.
The American Journal of Clinical Nutrition, 2000
Background: Currently available preterm formulas with energy contents of 3350 kJ (800 kcal)/L pro... more Background: Currently available preterm formulas with energy contents of 3350 kJ (800 kcal)/L promote weight and length gain at rates at or above intrauterine growth rates but disproportionately increase total body fat. Objective: The objective of this study was to determine whether fat accretion in formula-fed, very-low-birth-weight (VLBW) infants could be decreased and net protein gain maintained by reducing energy intakes from 502 kJ (80 kcal) • kg Ϫ1 •d Ϫ1 [normal-energy (NE) formula] to 419 kJ (100 kcal) • kg Ϫ1 •d Ϫ1 [low-energy (LE) formula] while providing similar protein intakes (3.3 g • kg Ϫ1 •d Ϫ1). Design: The study was a randomized, controlled trial enrolling 20 appropriate-for-gestational-age (AGA) and 16 small-for-gestational-age (SGA) VLBW infants (mean birth weight: 1.1 kg; mean gestational age: 31 wk); energy expenditure and nutrient balance were measured at 4 wk of age and anthropometric measurements were made when infants weighed 2 kg. Results: The percentage of fat in newly formed tissue was significantly lower in AGA infants fed the LE formula (n = 9) than in those fed the NE formula (n = 10) (9% compared with 23%; analysis of variance, P = 0.001). Energy expenditure was higher in AGA infants fed the NE formula than in those fed the LE formula. Skinfold thickness was markedly lower in AGA infants fed the LE formula than in those fed the NE formula, resulting in a lower estimated percentage body fat (8.0 ± 1.9% and 10.8 ± 3.5%, respectively; P < 0.05). Three of 6 SGA infants fed the LE formula were excluded during the study because of poor weight gain. Conclusions: Body composition can easily be altered by changing the energy intakes of formula-fed VLBW infants. Energy intakes in these infants should be > 419 kJ (100 kcal) • kg Ϫ1 • d Ϫ1 .
The American Journal of Clinical Nutrition, 1990
Parenterally fed preterm neonates are known to be at risk for carnitine deficiency. We studied su... more Parenterally fed preterm neonates are known to be at risk for carnitine deficiency. We studied substrate utilization in low-birth-weight infants receiving total parenteral nutrition (TPN) with (A) and without (B) supplementation of 48 mg carnitine. kg '. d ' on days 4-7 (birth weights 1 334 ± 282 vs 13 18 ± 248 g, gestational age 32 ± 2 vs 32 ± 2 wk, A vs B, respectively). TPN consisted of 1 1 g glucose. kg '. d and 2.4 g. kg '. d ' of both protein and fat. Plasma carnitine concentrations at day 7 were for free carnitine 1 1 .8 ± 5.0 vs 164 ± 56 zmol/L and for acyl carnitine 3.8 ± 2.0 vs 33.9 ± 1 5.4 tmol/ L, respectively. Indirect calorimetry at day 7 showed a higher fat oxidation (0.21,-0.31 to +0.60 vs 1.18, 0.70 to 1.95 g. kg .d , respectively, P < 0.02, median and interquartile range) in group B and a higher protein oxidation (0.37, 0.30-0.43 vs 0.63, 0.53-0.88 g.kg1 .d', P < 0.001). The time to regain birth weight was also higher in group B (7, 5.5-9 vs 9, 7-14 d, P < 0.05). Carnitine supplementation and calorie intake were the best explanatory variables for metabolic rate (R2 = 0.45, P < 0.002). We conclude that carnitine supplementation ofTPN in this dosage does not seem advisable.
Comparison of two preterm formulas with or without addition of medium-chain triglycerides (MCTs) ... more Comparison of two preterm formulas with or without addition of medium-chain triglycerides (MCTs) II: effects on mineral balance.
The Journal of Clinical Endocrinology & Metabolism, 2015
Context: Previously we showed that pubertal children born small for gestational age (SGA) with a ... more Context: Previously we showed that pubertal children born small for gestational age (SGA) with a poor adult height (AH) expectation can benefit from treatment with GH 1 mg/m 2 per day (ϳ0.033 mg/kg/d) in combination with 2 years of GnRH analog (GnRHa) and even more so with a double GH dose. GnRHa treatment is thought to have negative effects on body composition and blood pressure. Long-term effects and GH-dose effects on metabolic health in children treated with combined GH/GnRHa are unknown. Objective: This study aimed to investigate body composition, blood pressure, and lipid profile during GH treatment, either with or without 2 years of additional GnRHa. To assess whether GH 2 mg/m 2 per day (ϳ0.067 mg/kg/d) results in a similar or even more favorable metabolic health at AH than GH 1 mg/m 2 per day. Methods: This was a longitudinal, randomized, dose-response GH trial involving 107 short SGA children (58 girls) treated with GH until AH (GH randomized 1 or 2 mg/m 2 /d during puberty). Sixty-four children received additional GnRHa. At AH, metabolic parameters were compared between children treated with combined GH/GnRHa and those with only GH. The GH dose effect on metabolic health was evaluated in a subgroup of 47 children who started GH treatment in early puberty (randomized 1 or 2 mg/m 2 /d) with 2 years of GnRHa. Results: At AH, fat mass percentage (FM%) SD score (SDS), lean body mass (LBM) SDS, blood pressure SDS, and lipid profile were similar between children treated with combined GH/GnRHa and those with only GH. In the pubertal subgroup, FM% SDS was lower during treatment with GH 2 mg/m 2 per day. There was no GH dose-dependent effect on LBM SDS, blood pressure, and lipid profile. Conclusions: Combined GH/GnRHa treatment has no long-term negative effects on metabolic health compared with only GH. Started in early puberty, a GH dose of 2 mg/m 2 per day results in a similar metabolic health at AH and a more favorable FM% than GH 1 mg/m 2 per day. (J Clin Endocrinol Metab 100: 3725-3734, 2015) B eing born small for gestational age (SGA) has been associated with a higher prevalence of diabetes mellitus type 2, hypertension, and hyperlipidemia at a relatively young adult age (1). Because 10% of children born SGA have persistent short stature (2-4), many of them are treated with GH to increase adult height (AH) (5-10). Long-term GH treatment results in an increase in lean body mass (LBM), a decline in fat mass (FM), and a decrease in blood pressure (BP) and lipid profile (11-13).
Nederlands tijdschrift voor geneeskunde, 2009
Adherence to medication is a common problem in chronically ill patients. Three patients are prese... more Adherence to medication is a common problem in chronically ill patients. Three patients are presented who did not or did not fully adhere to their medication: a 3-year-old girl with asthma, a 57-year-old man with COPD and a 16-year-old boy with diabetes. This illustrates that adherence is not determined by age or illness. Known determinants of adherence are the patient's beliefs about illness and medication. Since these beliefs differ from patient to patient, dealing with non-adherence requires patient-centred care characterized by concordance, i.e. shared decision-making about therapy by doctors and patients. Appropriately targeted interventions, such as motivational interviewing, may influence patient beliefs and improve adherence.
Maatwerk, 2009
ABSTRACT Andrew Turnell is samen met Steve Edwards ontwerper van Signs of Safety, een praktijkger... more ABSTRACT Andrew Turnell is samen met Steve Edwards ontwerper van Signs of Safety, een praktijkgerichte benadering voor het werken in gezinnen met meervoudige problemen. Eric Sulkers, vertrouwenarts bij het AMK Zeeland en lector Sietske Dijkstra spraken Turnell in maart toen hij in Nederland een masterclass gaf.
Pediatric Research, 1989
Medium-chain triglycerides, with a chain length of eight and 10 carbon atoms, form up to 50% of t... more Medium-chain triglycerides, with a chain length of eight and 10 carbon atoms, form up to 50% of the total fat content in some preterm infant formulas. In 20 small preterm infants (birthwt: 1153 f 227 g; mean 2 SD) fed a special formula containing 40% MCT, a primed constant oral infusion study of l-'3C-potassium octanoate was conducted to quantify the oxidation of MCT. A plateau in 13C enrichment in breath COz was reached in all patients within 1-3 h. Simultaneously, substrate utilization was measured using a closed system indirect calorimeter. No significant difference was found between appropriate for gestational age (n = 8) and small for gestational age (n = 12) infants in the percentage of the administered tracer that was oxidized (44.9 f 9.1 % versus 48.5 f 11.0%). In all patients, the recovery was calculated to be 47.1 f 10.2%, which is less than previously estimated and corresponds to a mean MCT oxidation of 1.26 f 0.27 g/kg/d. With indirect calorimetry, a total fat oxidation of 1.42 f 0.84 g/kg/d in appropriate for gestational age and 2.00 2 0.85 g/kg/d in small for gestational age infants was found, indicating that MCT accounted for around 85% of the total fat oxidation in appropriate for gestational age versus 65% in small for gestational age infants.
Pediatric Research, 1990
During total parenteral nutrition in preterm infants, glucose may be infused at high rates, but i... more During total parenteral nutrition in preterm infants, glucose may be infused at high rates, but it is not known if the endogenous glucose production is fully suppressed under these circumstances. Eight preterm appropriate for gestational age (AGA) (birth wt: 1613 f 151 g, gestational age: 31.1 f 1.5 wk) and eight preterm small for gestational age (SGA) newborn infants (1 185 f 241 g, 32.9 2 2.6 wk) receiving a glucose infusion rate of 7.55 2 0.56 and 8.16 f 0.65 mg/kg. min, respectively, were studied during continuous total parenteral nutrition at postnatal d 8. Glucose oxidation rate was determined with a primed constant infusion of [U-"q glucose, measuring the '"02 production in breath gas by isotope ratio mass spectrometry and the glucose production rate in plasma by gas chromatography mass spectrometry. In breath gas of AGA and SGA infants, 60 and 6576, respectively, of the infused tracer appeared as ' T O 2. The glucose production rates were 7.97 2 1.61 and 8.1 2 f 1.84 mg/kg. min in AGA and SGA infants, respectively, indicating that no significant endogenous glucose production occurred. The glucose oxidation calculated from the glucose production and I3CO2 production was 4.74 f 0.99 mg/kg.min in AGA infants and was significantly different from the carbohydrate oxidation rate of 6.62 f 1.23 mg/kg. min measured by simultaneous indirect calorimetry. In SGA infants, however, the glucose and carbohydrate oxidation rates were not significantly different at 5.33 f 1.56 and 6.16 f 2.45 mg/kg min. It is concluded that 1-wk-old AGA or SGA preterm infants receiving total parenteral nutrition of 80 kcallkg. d produce no endogenous glucose and their glucose oxidation rates are similar at 63-65% of the glucose infused. It is suggested that the significant difference between glucose and carbohydrate oxidation rates observed in AGA but not in SGA infants is due either to a higher rate of lipogenesis from carbohydrates, or, less likely, to a higher rate of glycogen oxidation. (Pediatr Res 28: 153-157, 1990) Abbreviations AGA, appropriate for gestational age R,, rate of glucose appearance in plasma RQ, respiratory quotient SGA. small for eestational aee PATIENTS AND METHODS TPN; total pare;;teral nutritik Patients. Eight AGA and eight SGA preterm newborn infants
Pediatric Research, 1991
Ventilated VLBW infahts usually receive only glucose during the first day of life. These infants ... more Ventilated VLBW infahts usually receive only glucose during the first day of life. These infants are prone to hypo-and hyper glycemia, and it is unknown if this is due to changes in glucose oxidation(G1uox) or non-oxidative disposal (NOD). We studied glucose turnover and oxidation in 7 ventilated VLBW infante (birthweight 1.25 * 0.30 kg; gestional age 32 t 2 weeks; postnatal
Pediatric Research, 1991
An energy intake of 120 kcal1kg.d in preterm infants resulted in a higher fat accretion than in u... more An energy intake of 120 kcal1kg.d in preterm infants resulted in a higher fat accretion than in utero. To study if a reduced energy intake would not only reduce fat accretion, but also influence proteinlprol turnovcrlOl, we measured leucine(leu1 kinetics in 4 wk old, orally fed VLBW infants, receiving 3.2 g pro1kg.d. They were fed either 120-In= l2,bw= 1.1 i 0. 2 kg,ga=30* 2 wk) or 100
Pediatric Research, 1991
An energy intake of 120 kcal1kg.d in prelerm infants resulted in a higher fat accretion than in u... more An energy intake of 120 kcal1kg.d in prelerm infants resulted in a higher fat accretion than in utero. To study if a reduced energy inlake would not only reduce fat accretion, but also influence protein(pro1 turnoverlOl, we measured leucine(leu1 kinetics in 4 wk old, orally fed VLBW infants, receiving 3.2 g pro1kg.d. They were fed either 120-In= 12,bw= 1.1 20.2 kg,ga=30+2 wkl or 100
Pediatric and Developmental Pathology, 1999
We report a novel case of partial trisomy 19q and concomitant partial monosomy 21q, segregated fr... more We report a novel case of partial trisomy 19q and concomitant partial monosomy 21q, segregated from a maternal translocation (19;21) (q13.1;q22.3), identified by spectral karyotyping. Clinical examination revealed dysmorphic features of the face and limbs, cleft palate, bilateral colobomas with associated bilateral colobomatous optic nerve cysts, hearing loss, and a cardiac anomaly. At autopsy, the dysmorphic features and cleft palate were confirmed. The ocular histopathology is described in detail and the cardiac anomaly was further specified. The combination of phenotype features is diagnostic of the CHARGE ( coloboma, heart malformation, atresia choanae, retarded growth and development, and/or CNS anomalies, genital hypoplasia, ear anomalies and/or deafness) association. This case also has some phenotypic features in common with previous cases of partial trisomy 19q. The importance of a complete autopsy in cases with multiple congenital anomalies and/or genetic abnormalities is e...
Neonatology, 1991
The accuracy of 8-hour indirect calorimetry (IDC) as an estimate of energy expenditure was invest... more The accuracy of 8-hour indirect calorimetry (IDC) as an estimate of energy expenditure was investigated in 8 healthy preterm infants (birth weight 1,270 ± 193 g, gestational age 32 ± 3 weeks, mean ± SD) in comparison with an analysis over 5 days using the doubly-labeled water (2H218O) method (DLW). The infants that were fed continuously by nasogastric drip with 120 kcal/kg/day of special infant formula were measured twice under thermoneutral conditions in a closed system indirect calorimeter during 8 h with a 4-day interval; simultaneously isotope decay was measured by isotope ratio mass spectrometry in urine samples collected daily during 5 days from 6 h after an oral dose of 2H2180 on the first day of IDC, all during the 4th postnatal week. The mean differences between carbon dioxide production rate (rCO2) measured either by single 8-hour IDC or by duplicate 8-hour IDC and the 5-day DLW method, using the two-point analysis or the multipoint analysis were not significantly differen...
Journal of Pediatric Gastroenterology & Nutrition, 1996
The structure of the triglycerides (TG) in human milk (HM) differs from those of vegetable oils u... more The structure of the triglycerides (TG) in human milk (HM) differs from those of vegetable oils used in infant formulas. In HM, palmitic acid is predominantly esterified to the center or beta-position of the TG, in vegetable oil, it is mainly at the external or alpha-positions. These differences in configuration affect intestinal fat absorption. Fat and mineral balances were investigated in three groups of 9 healthy term infants aged 5 weeks. Infants were randomly assigned to receive one of the three study formulas from birth: (a) formula beta, resembling the structure of HM fat most closely (24% palmitic acid, 66% esterified to beta-position), (b) formula intermediate (24% palmitic acid, 39% esterified to the beta-position), and (c) regular formula (20% palmitic acid; 13% esterified to the beta-position). Fat absorption was highest in infants fed the beta formula (97.6 +/- 0.9%), intermediate in those fed with the intermediate formula (93.0 +/- 1.8%), and lowest in infants receiving the regular formula (90.4 +/- 4.6%). Fecal calcium excretion was significantly lower in the beta group than in the other two groups (43.3 +/- 18.1 vs. 59.9 +/- 15.1 vs. 68.4 +/- 22.3 mg.kg-1.day-1 for beta, intermediate, and regular respectively). Dietary TG containing palmitic acid predominantly at the beta-position, as in HM, have significant beneficial effects on the intestinal absorption of fat and calcium in healthy term infants.
The Journal of Clinical Endocrinology & Metabolism, 2012
Context: GH treatment is effective in improving height in short children born small for gestation... more Context: GH treatment is effective in improving height in short children born small for gestational age (SGA). GH is thought to have limited effect when started during adolescence. Objective: The aim of this study was to investigate GH treatment efficacy in short SGA children when treatment was started during adolescence; to assess whether GH 2 mg/m 2 ⅐ d during puberty improves adult height (AH) compared with 1 mg/m 2 ⅐ d; and to assess whether an additional 2-yr postponement of puberty by GnRH analog (GnRHa) improves AH in children who are short at the start of puberty (Ͻ140 cm), with a poor AH expectation. Patients and Design: In this longitudinal, randomized, dose-response GH trial, we included 121 short SGA children (60 boys) at least 8 yr of age. We performed intention-to-treat analyses on all children and uncensored case analyses on 84 children who reached AH. Besides, we evaluated growth during 2 yr of combined GH/GnRHa and subsequent GH treatment until AH in a subgroup of 40 pubertal children with a height of less than 140 cm at the start. Results: Short SGA children started treatment at a median age of 11.2 yr, when 46% had already started puberty. Median height increased from Ϫ2.9 at start to Ϫ1.7 SD score (SDS) at AH (P Ͻ 0.001). Treatment with GH 2 vs. 1 mg/m 2 ⅐ d during puberty resulted in significantly better AH (P ϭ 0.001), also after correction for gender, age at start, height SDS at start, treatment years before puberty, and target height SDS. AH was similar in children who started puberty at less than 140 cm and received GH/GnRHa, compared with children who started puberty greater than 140 cm and received GH only (P ϭ 0.795). Conclusion: When started in adolescence, GH treatment significantly improves AH in short SGA children, particularly with GH 2 mg/m 2 ⅐ d during puberty. When SGA children are short at the start of puberty, they can benefit from combined GH/GnRHa treatment.