Hugo Heymans - Profile on Academia.edu (original) (raw)
Papers by Hugo Heymans
The American Journal of Clinical Nutrition, 2005
Background: Stunted children with cystic fibrosis (CF) have less net protein anabolism than do ch... more Background: Stunted children with cystic fibrosis (CF) have less net protein anabolism than do children without CF, and the result is retarded growth in the CF patients. It is not known whether protein intake above that recommended by the Cystic Fibrosis Foundation would further stimulate whole-body protein synthesis. Objective: We studied the effects of 3 amounts of protein intake on whole-body protein synthesis and breakdown by using isotopic infusion of [1-13 C]valine and [ 15 N 2 ]urea in children with stable CF who required tube feeding. Design: In 8 pediatric CF patients, we administered 3 randomly allocated isocaloric diets with normal (NP), intermediate (IP), and high (HP) amounts of protein (1.5, 3, and 5 g • kg Ҁ1 • d Ҁ1 , respectively) by continuous drip feeding during a 4-d period at 6-wk intervals. Each patient acted as his or her own control. On the fourth day of feeding, whole-body protein synthesis and breakdown were measured. Results: Protein synthesis was significantly higher in the HP group (x Ȁ SEM: 1.78 Ȁ 0.07 mol • kg Ҁ1 • min Ҁ1) than in the IP (1.57 Ȁ 0.08 mol • kg Ҁ1 • min Ҁ1 ; P ҃ 0.001) and NP (1.37 Ȁ 0.07 mol • kg Ҁ1 • min Ҁ1 ; P 0.001) groups. There were no significant differences in protein breakdown. Net retention of nitrogen was significantly higher in the HP group (12.93 Ȁ 1.42 mol • kg Ҁ1 • min Ҁ1) than in the IP (7.61 Ȁ 1.40 mol ⅐ kg Ҁ1 ⅐ min Ҁ1 ; P ҃ 0.01) and HP (2.48 Ȁ 0.20 mol • kg Ҁ1 • min Ҁ1 ; P 0.001) groups. Conclusion: In stunted children with CF requiring tube feeding, the highest stimulation of whole-body protein synthesis was achieved with a short-term dietary protein intake of 5 g • kg Ҁ1 • d Ҁ1 .
Kidney International, 2002
Mortality and causes of death of end-stage renal disease in treatment for children with end-stage... more Mortality and causes of death of end-stage renal disease in treatment for children with end-stage renal disease children: A Dutch cohort study. (ESRD). Improvement in dialysis techniques and sup-Background. To establish mortality rates, causes of death, portive therapies over the last two decades has placed and determinants of mortality in children with end-stage renal chronic RRT in most children beyond controversy. Notadisease (ESRD), we performed a national long-term follow up study. bly, the introduction of bicarbonate buffer replacing Methods. Mortality rate was determined in all Dutch paacetate in hemodialysis, continuous cycling peritoneal tients with onset of ESRD at ages 0 to 14 years in the period dialysis (CCPD), and the use of recombinant human between 1972 and 1992. Causes of death and mortality determierythropoietin (EPO) and growth hormone (GH) thernants were investigated in all patients of this cohort who were apy have been associated with a decrease in acute morborn before 1979. Data were derived from the Dutch Registry for patients on renal replacement therapy (RRT), medical bidity and mortality. Although treatment of infants is charts and National Health Database. still being debated, recent studies advocate the initiation Results. Of all 381 patients 85 had died. The overall mortality of RRT even in this group [1]. rate (MR) was 1.57/100 patient-years, and the standardized Nevertheless, detailed information about the longmortality rate (SMR) was 31.0. The MR for patients 0 to 5 and 6 to 10 years old at onset of ESRD decreased from, respec- term outcomes of RRT in children is lacking. Previous tively, 7.0 (range 0-14.9) to 3.9 (1.2-6.7) and 4.3 (1.1-7.5) to studies on morbidity and mortality in these children have 1.6 (0.3-2.8) between the periods 1972-1981 and 1982-1992. relatively short follow-up times, show only crude and The mortality hazard ratio of relatively long standing dialysis incomplete registry data, or describe isolated groups of and of long standing hypertension were, respectively, 7.2 (4. 4-11.8) and.1 (2.1-4.6), of cyclosporine-introduction in trans-transplanted children or children on dialysis [2-9]. Thereplanted patients 0.3 (0.1-0.4). Overall cerebrovascular accifore, we conducted a national retrospective and prospecdents (24%) and infections (21%) were the most common tive cross-sectional study to evaluate the Late physical, causes of death; after 10 years of RRT cardiac death (7/21) social and psychological Effects of Renal InsuffiCiency was most prevalent. Cardiovascular death was most prominent (LERIC) in all Dutch children who started RRT bein dialysis as well as transplanted patients. Conclusion. Survival in children with ESRD has increased tween 1972 and 1992. This article reports the mortality over the last 20 years, but the SMR remains high. Early transand causes of death. plantation and a more vigorous approach toward hypertension and infection may be mandatory in order to further reduce mortality. Participants: The total cohort and the LERIC cohort Since the early seventies of the last century, renal The LERIC study was designed to examine the physireplacement therapy (RRT) has become an established cal, social and psychological consequences of pediatric ESRD patients who were at an adult age. In 1972 the first Dutch center for pediatric dialysis was officially opened.
Kidney International, 2003
Cardiac disease in young adult patients with end-stage renal its late sequelae. Improvement in di... more Cardiac disease in young adult patients with end-stage renal its late sequelae. Improvement in dialysis technique and disease since childhood: A Dutch cohort study. supportive therapy over the last two decades, such as Background. Cardiovascular disease is the most important the introduction of bicarbonate buffer or peritoneal dialcause of death in patients with pediatric end-stage renal disease ysis and the use of recombinant erythropoietin (EPO), (ESRD). Yet, few data exist on cardiac function in these pahave been associated with a decrease in morbidity and tients. We assessed the extent of cardiac abnormality and analyzed its association with potential determinants in young adult mortality [1-3]. Nevertheless, detailed information of patients with pediatric ESRD in a long-term follow-up study. long-term outcomes of renal replacement therapy (RRT) Methods. All Dutch living adult patients with ESRD onset in children is lacking. at age of 0 to 14 years between 1972 and 1992 were invited In adults, cardiovascular disease has been recognized for echocardiography and blood pressure assessment. Special attention was paid to evidence of left ventricular hypertrophy as the most important cause of death in patients with ESRD (LVH), diastolic dysfunction, and aortic valve calcification. We [4-8]. Left ventricular hypertrophy (LVH) is the most collected data on determinants by review of all medical charts. frequently observed alteration in these patients and has Results. Of all the 187 living patients, 140 participated in been directly associated with acute death [5, 7]. Chronic the study. Of those, 110 patients had received a transplant and 30 patients were on dialysis. Mean age was 29.2 (20.7 to 41.8) pressure and volume overload, as a result of water and years. Left ventricular mass index (LVMI) exceeded 150 g/m 2 salt retention and hypertension, and the use of cyclospoin 47% of all male patients and 120 g/m 2 in 39% of all female rine in transplantation have been regarded as potential patients, both consistent with LVH. Diastolic dysfunction, derisk factors in the development of LVH in older adult fined as an early over atrial transmitral blood flow velocity patients with ESRD [6,. Enhanced calcification of the (E/A ratio) Ͻ1, was found in 18 (13%) patients; 27 (19%) had aortic valve calcification. Multiple regression analysis revealed aortic valve as a result of chronic volume overload and the following: a high LVMI was associated with a current high a high serum calcium-phosphate product has recently blood pressure ( ϭ 0.46, P Ͻ 0.001) and male gender ( ϭ been recognized as another independent risk factor for 0.21, P ϭ 0.009), a low E/A ratio with aging ( ϭ Ϫ0.28, P Ͻ acute death in adult patients with ESRD [10, 11]. 0.001) and a glomerular filtration rate (GFR) Ͻ25 mL/min/ 1.73 m 2 ( ϭ Ϫ0.29, P Ͻ 0.001), and aortic valve calcification To date, few data exist on cardiac disease in young with prolonged peritoneal dialysis ( ϭ 0.36, P Ͻ 0.001). adults with ESRD since childhood. There are concerns Conclusion. Young adult patients with pediatric ESRD are that, as in patients with adult onset of ESRD, these at risk for LVH caused by hypertension and for aortic valve patients are at risk for cardiovascular death at a very calcification. Diastolic function decreases with age and is enhanced by a current low GFR. Prolonged peritoneal dialysis young age. We conducted a national long-term followmay enhance aortic valve calcification. up study to evaluate late effects of renal insufficiency in children (LERIC). The cohort consisted of all Dutch adult patients that commenced RRT between 1972 and As life expectancy has increased in children with end-1992 at age 0 to 14 years. Recently, we reported on morstage renal disease (ESRD), concern has arisen about tality and causes of death . Cardiovascular disease was found to be the cause of death in more than 40% of
Kidney International, 2003
disease is a common, often disabling, complication of nile renal failure. end-stage renal disease... more disease is a common, often disabling, complication of nile renal failure. end-stage renal disease (ESRD) [1-4]. Chronic acidosis Background. Little is known about the late effects of juveand hypovitamin-D-and phosphate-induced secondary nile end-stage renal disease (ESRD) on bone integrity. To hyperparathyroidism can lead to disturbed bone modelestablish clinical manifestations of metabolic bone disease and bone mineral density (BMD) in young adult patients with juve-ing with high or low bone turnover in patients on chronic nile ESRD, we performed a long-term outcome study. dialysis [5-8]. In addition, inactivity during chronic dial-Methods. A cohort was formed of all Dutch patients with ysis and corticosteroids, mainly given chronically as antionset of ESRD between 1972 and 1992 at age 0 to 14 years, rejection therapy after transplantation, may lead to osborn before 1979. Data were collected by review of medical charts, current history, physical examination, and performing teoporosis [9-14]. In children with ESRD, malnutrition, dual energy x-ray absorptiometry (DEXA) of the lumbar spine reduced bioactivity of insulin-like growth hormones and and the femoral neck. delayed puberty can aggravate these effects, disturbing Results. Clinical information was retrieved in 247 out of 249 normal bone formation and growth [15-20]. Little is known patients. Of all of these patients, 61.4% had severe growth retardation (ϽϪ2 SD), 36.8% had clinical symptoms of bone about the effects of ESRD of childhood on bone formadisease, and 17.8% were disabled by bone disease. Growth tion at adult age, and about the extent of clinical bone retardation and clinical bone disease were associated with a disease in long-term survivors of juvenile ESRD. long duration of dialysis. DEXA was performed in 140 out of We conducted a national long-term follow up study in 187 living patients. Mean BMD Ϯ SD corrected for gender order to evaluate Late physical, social and psychological and age (Z score) of the lumbar spine was Ϫ2.12 Ϯ 1.4 and of the femoral neck was Ϫ1.77 Ϯ 1.4. A low lean body mass Effects of Renal Insufficiency in Children (LERIC Study). was associated with a low lumbar spine and a low femoral The cohort consisted of all Dutch children who had comneck BMD; male gender, physical inactivity and aseptic bone menced RRT between 1972 and 1992 and who had reached necrosis were associated with a low lumbar spine BMD. Conclusion. Bone disease is a major clinical problem in young (or would have reached if still alive) adulthood at the adults with pediatric ESRD. Further follow-up is needed to time of investigation. This article reports the impact on establish the impact of the low bone mineral densities found growth, the incidence of fractures, clinically apparent bone in these patients. disease and bone densities measured by dual energy x-ray absorptiometry (DEXA) in these young adults with pediatric onset of ESRD. As life expectancy has increased in children with chronic renal replacement therapy (RRT), concern has arisen about its late complications. In adults, metabolic bone METHODS
Journal of the American Society of Nephrology, 2002
Increased arterial stiffness is a risk factor for mortality in adults over 40 yr of age with end-... more Increased arterial stiffness is a risk factor for mortality in adults over 40 yr of age with end-stage renal disease (ESRD). As no data exist on vascular changes in young adults with ESRD since childhood, a long-term outcome study was performed. All living Dutch adult patients with onset of ESRD between 1972 and 1992 at age 0 to 14 yr were invited for carotid artery and cardiac ultrasound and BP measurements. Data on clinical characteristics were collected by review of all medical charts. Carotid ultrasound data were compared with those of 48 age-matched and gender-matched healthy controls. Carotid artery and cardiac ultrasound was performed in 130 out of 187 eligible patients. Mean age was 29.0 (20.7 to 40.6) yr. Compared with controls, patients had a similar intima media thickness but a reduced mean arterial wall distensibility DC
The daily gluten intake in relatives of patients with coeliac disease compared with that of the general Dutch population
European Journal of Gastroenterology & Hepatology, 1997
It has been suggested that the amount of gluten intake in populations offers an explanation for d... more It has been suggested that the amount of gluten intake in populations offers an explanation for differences in the epidemiology of coeliac disease. Investigations into first-degree relatives of coeliac disease patients have often shown that relatives exhibit intermediate features of coeliac disease, possibly due to a low gluten intake. The aim of this study was to investigate the pattern of gluten consumption in the general Dutch population for different age and sex groups and for different product groups, and to investigate the daily gluten intake of first-degree relatives of coeliac disease patients. Questionnaires concerning the gluten intake of 55 first-degree relatives of coeliac disease patients were analysed. To determine the gluten intake of the general Dutch population, the results of a mass investigation were used. The amount of gluten in the gluten-rich products was estimated by multiplying the amount of vegetable proteins by 0.8. The median daily gluten intake of the relatives was 12.9 g (range: 3.8-31.3). The mean daily gluten intake of the study population in the Netherlands was 1 3.1 g. The gluten intake of first-degree relatives of coeliac disease patients was the same as that of the general population. Thus, a low gluten intake apparently does not explain the aspecific presentation and prevalence of coeliac disease in first-degree relatives of coeliac disease patients.
BDJ, 2007
Defects were much commoner than in a control group, and most were of a characteristic type.
Thorax, 1996
Hepatitis C virus in pulmonary fibrosis genotypes were identified had genotype II alone or in com... more Hepatitis C virus in pulmonary fibrosis genotypes were identified had genotype II alone or in combination with other types, and the high frequency of type II genotype is in keeping with the prevalence in Italy.'3 Thus, HCV infection in patients with IPF is not associated with specific genotypes. Thirdly, in two patients a combination of two and three genotypes was present, a rare association in a single patient and almost exclusively confined to cases who have received multiple transfusions.'2
Cancer in children in the Netherlands (1989-1997). – In a recent report by the Society of Integra... more Cancer in children in the Netherlands (1989-1997). – In a recent report by the Society of Integral Cancer Centres in the Netherlands, attention was devoted to the incidence of cancer in children and the mortality arising from this. In recent years the growing diagnostic and therapeutic possibilities have changed the perspective of childhood cancer enormously. Based on a careful classification and clinical staging, national and internaNed Tijdschr Geneeskd 2001 28 juli;145(30) 1443 tional investigations have resulted in new and successful therapeutical strategies. Overall prognoses of childhood cancer have improved dramatically from a 5-year survival rate in the 1960s and 1970s of less than 30%, to an 8-year survival rate of more than 70% between 1989 and 1997. However, this success means that more investigations into the long-term effects of childhood cancer and its treatment are needed. In a study at the Amsterdam University Hospital, 700 adult survivors of childhood cancer were re...
Should relatives of coeliacs with mild clinical complaints undergo a small-bowel biopsy despite negative serology?
European Journal of Gastroenterology & Hepatology, 2000
Small intestinal lesions in coeliac disease (CD) have a variable severity. Early diagnosis of CD ... more Small intestinal lesions in coeliac disease (CD) have a variable severity. Early diagnosis of CD is important because treatment allows a normal psycho-physical development, especially in children, and can avoid associated disorders. The aim of this study was to evaluate the predictive value of screening parameters for the detection and estimation of CD prevalence in first-degree relatives. The screening was performed in 338 first-degree relatives of 134 coeliac families. Questionnaires and a physical examination followed by haematological analyses and serologyfor IgA anti-endomysium (EMA)/IgA antigliadin (AGA) antibodies were used in orderto selectthe candidates for small-bowel biopsy. The small-bowel biopsy was indicated on the basis of clinical complaints, laboratory tests and serology performed in 96 (28%) of the study group. CD was diagnosed in 17/96 cases. Six of the 17 showed total villous atrophy (VA) (Marsh IIIc), five subtotal VA (Marsh IIIb) and six partial VA (Marsh IIIa). EMA and AGA were strongly positive in the six patients whose intestinal biopsy showed total VA. However, only one coeliac out of the six patients with partial VA had positive EMA and AGA. A significant proportion of coeliacs may be missed if cases are screened by serology only. Although endomysial antibody assay has been reported as a highly sensitive and specific test for detection of CD, we argue that using only EMA and AGA in screening is not enough for investigation of the true prevalence of CD. A combination of clinical parameters as described in this study and laboratory/serological tests is an important and practical contribution to improving the detection rate of CD.
Screening for child abuse using a checklist and physical examinations in the emergency department led to the detection of more cases
Acta paediatrica (Oslo, Norway : 1992), Jan 10, 2018
We studied the accuracy of a screening checklist (SPUTOVAMO), complete physical examination (top-... more We studied the accuracy of a screening checklist (SPUTOVAMO), complete physical examination (top-to-toe inspection, TTI) and their combination in detecting child abuse in the emergency department (ED). Consecutive patients admitted to the ED of the Academic Medical Center in Amsterdam between January 2011 and 1 July 2013 were included. An Expert Panel assigned a consensus diagnosis to positive cases. For all other and missed cases, the Child Abuse Counselling and Reporting Centre diagnosis was used. We included 17 229 admissions of 12 198 patients. In 46%, SPUTOVAMO was performed, in 33% TTI; 421 children (4.3%) tested positive on either or both, with 68 positive consensus diagnoses. In eight children not reported to the Expert Panel, the Child Abuse Counselling and Reporting Center diagnosis was positive. Ten of 3519 (0.3%) children testing negative on both were child abuse cases; 0.88% of the study group had a final child abuse diagnosis. The estimated PPV was 0.46 for SPUTOVAMO, ...
Assessments carried out by a child abuse and neglect team in an Amsterdam teaching hospital led to interventions in most of the reported cases
Acta paediatrica (Oslo, Norway : 1992), Jan 30, 2016
This study described cases of child abuse and neglect (CAN) that were reported to the multiagency... more This study described cases of child abuse and neglect (CAN) that were reported to the multiagency CAN team at the Emma Children's Hospital in Amsterdam and the resulting interventions. We carried out a retrospective review of all cases that were reported to the CAN team from 1 January 2010 to 31 December 2012. There were 27 prenatal cases, 92 referrals based on parental characteristics and 523 children. Overall, 1.2% of the children visiting the emergency department of our hospital, attending the outpatients department or being admitted were reported to the team. More than half of the referrals (55.1%) were confirmed as CAN. The most common diagnoses were as follows: witnessing intimate partner violence, physical neglect and emotional abuse. If CAN was confirmed an intervention was offered in 98.3% of cases. If a CAN diagnosis was undetermined or rejected, the figures were still 83.5% and 64.2%, respectively. Our results showed that CAN affected more than one in every 100 childr...
Comparing the effect of Livercell Transplantation (LCTX) in Gunn rats using either immunesuppresive therapy or immunetolerance inducing techniques
J Pediat Gastroenterol Nutr, 1998
The pharmacokinetics of nelfinavir in HIV-1-infected children
Therapeutic Drug Monitoring, Aug 1, 2002
The authors investigated the steady-state plasma pharmacokinetics of nelfinavir in HIV-1-infected... more The authors investigated the steady-state plasma pharmacokinetics of nelfinavir in HIV-1-infected children in a dosage of 30 mg/kg every 8 hours and 45 mg/kg every 12 hours in 12 HIV-1-infected children (aged 2.1 to 10.8 years) participating in an open-label study of nelfinavir in combination with stavudine and lamivudine. Median values of the primary pharmacokinetic parameters of nelfinavir 30 mg/kg every-8-hours (n = 8) and 45 mg/kg every 12 hours (n = 10) were, respectively, for the area under the plasma concentration-time curve over 24 hours, 90.5 and 71.9 h x microg/mL, for the trough concentration 1.9 and 1.0 microg/mL, and for the maximal concentration 6.3 and 5.2 microg/mL. Overall, a 7-fold interpatient variability was observed for the exposure to nelfinavir. Nelfinavir 30 mg/kg every-8-hours or 45 mg/kg every 12 hours in HIV-1-infected children generally resulted in plasma concentrations higher than those obtained in adults. However, due to a large interpatient variability in the exposure, individual dosage adjustments based on plasma concentrations may be necessary for both dosing regimens to ensure optimal treatment.
Endoscopic Balloon Dilatation as Treatment of Gastric Outlet Obstruction in Infancy and Childhood: 79
Pediat Res, 1987
ABSTRACT
De ziekte van Fabry: etiologie, kliniek en behandeling
Kindermishandeling, diagnostische en therapeutische dilemma's
Tijdschrift Voor Kindergeneeskunde, 2005
Kanker op de kinderleeftijd in Nederland (1989-1997)
Functionele gastro-intestinale ziektebeelden op de kinderleeftijd. II. Obstipatie en solitaire encopresis; fysiologie en pathofysiologie
Nederlands Tijdschrift Voor Geneeskunde, 2003
The American Journal of Clinical Nutrition, 2005
Background: Stunted children with cystic fibrosis (CF) have less net protein anabolism than do ch... more Background: Stunted children with cystic fibrosis (CF) have less net protein anabolism than do children without CF, and the result is retarded growth in the CF patients. It is not known whether protein intake above that recommended by the Cystic Fibrosis Foundation would further stimulate whole-body protein synthesis. Objective: We studied the effects of 3 amounts of protein intake on whole-body protein synthesis and breakdown by using isotopic infusion of [1-13 C]valine and [ 15 N 2 ]urea in children with stable CF who required tube feeding. Design: In 8 pediatric CF patients, we administered 3 randomly allocated isocaloric diets with normal (NP), intermediate (IP), and high (HP) amounts of protein (1.5, 3, and 5 g • kg Ҁ1 • d Ҁ1 , respectively) by continuous drip feeding during a 4-d period at 6-wk intervals. Each patient acted as his or her own control. On the fourth day of feeding, whole-body protein synthesis and breakdown were measured. Results: Protein synthesis was significantly higher in the HP group (x Ȁ SEM: 1.78 Ȁ 0.07 mol • kg Ҁ1 • min Ҁ1) than in the IP (1.57 Ȁ 0.08 mol • kg Ҁ1 • min Ҁ1 ; P ҃ 0.001) and NP (1.37 Ȁ 0.07 mol • kg Ҁ1 • min Ҁ1 ; P 0.001) groups. There were no significant differences in protein breakdown. Net retention of nitrogen was significantly higher in the HP group (12.93 Ȁ 1.42 mol • kg Ҁ1 • min Ҁ1) than in the IP (7.61 Ȁ 1.40 mol ⅐ kg Ҁ1 ⅐ min Ҁ1 ; P ҃ 0.01) and HP (2.48 Ȁ 0.20 mol • kg Ҁ1 • min Ҁ1 ; P 0.001) groups. Conclusion: In stunted children with CF requiring tube feeding, the highest stimulation of whole-body protein synthesis was achieved with a short-term dietary protein intake of 5 g • kg Ҁ1 • d Ҁ1 .
Kidney International, 2002
Mortality and causes of death of end-stage renal disease in treatment for children with end-stage... more Mortality and causes of death of end-stage renal disease in treatment for children with end-stage renal disease children: A Dutch cohort study. (ESRD). Improvement in dialysis techniques and sup-Background. To establish mortality rates, causes of death, portive therapies over the last two decades has placed and determinants of mortality in children with end-stage renal chronic RRT in most children beyond controversy. Notadisease (ESRD), we performed a national long-term follow up study. bly, the introduction of bicarbonate buffer replacing Methods. Mortality rate was determined in all Dutch paacetate in hemodialysis, continuous cycling peritoneal tients with onset of ESRD at ages 0 to 14 years in the period dialysis (CCPD), and the use of recombinant human between 1972 and 1992. Causes of death and mortality determierythropoietin (EPO) and growth hormone (GH) thernants were investigated in all patients of this cohort who were apy have been associated with a decrease in acute morborn before 1979. Data were derived from the Dutch Registry for patients on renal replacement therapy (RRT), medical bidity and mortality. Although treatment of infants is charts and National Health Database. still being debated, recent studies advocate the initiation Results. Of all 381 patients 85 had died. The overall mortality of RRT even in this group [1]. rate (MR) was 1.57/100 patient-years, and the standardized Nevertheless, detailed information about the longmortality rate (SMR) was 31.0. The MR for patients 0 to 5 and 6 to 10 years old at onset of ESRD decreased from, respec- term outcomes of RRT in children is lacking. Previous tively, 7.0 (range 0-14.9) to 3.9 (1.2-6.7) and 4.3 (1.1-7.5) to studies on morbidity and mortality in these children have 1.6 (0.3-2.8) between the periods 1972-1981 and 1982-1992. relatively short follow-up times, show only crude and The mortality hazard ratio of relatively long standing dialysis incomplete registry data, or describe isolated groups of and of long standing hypertension were, respectively, 7.2 (4. 4-11.8) and.1 (2.1-4.6), of cyclosporine-introduction in trans-transplanted children or children on dialysis [2-9]. Thereplanted patients 0.3 (0.1-0.4). Overall cerebrovascular accifore, we conducted a national retrospective and prospecdents (24%) and infections (21%) were the most common tive cross-sectional study to evaluate the Late physical, causes of death; after 10 years of RRT cardiac death (7/21) social and psychological Effects of Renal InsuffiCiency was most prevalent. Cardiovascular death was most prominent (LERIC) in all Dutch children who started RRT bein dialysis as well as transplanted patients. Conclusion. Survival in children with ESRD has increased tween 1972 and 1992. This article reports the mortality over the last 20 years, but the SMR remains high. Early transand causes of death. plantation and a more vigorous approach toward hypertension and infection may be mandatory in order to further reduce mortality. Participants: The total cohort and the LERIC cohort Since the early seventies of the last century, renal The LERIC study was designed to examine the physireplacement therapy (RRT) has become an established cal, social and psychological consequences of pediatric ESRD patients who were at an adult age. In 1972 the first Dutch center for pediatric dialysis was officially opened.
Kidney International, 2003
Cardiac disease in young adult patients with end-stage renal its late sequelae. Improvement in di... more Cardiac disease in young adult patients with end-stage renal its late sequelae. Improvement in dialysis technique and disease since childhood: A Dutch cohort study. supportive therapy over the last two decades, such as Background. Cardiovascular disease is the most important the introduction of bicarbonate buffer or peritoneal dialcause of death in patients with pediatric end-stage renal disease ysis and the use of recombinant erythropoietin (EPO), (ESRD). Yet, few data exist on cardiac function in these pahave been associated with a decrease in morbidity and tients. We assessed the extent of cardiac abnormality and analyzed its association with potential determinants in young adult mortality [1-3]. Nevertheless, detailed information of patients with pediatric ESRD in a long-term follow-up study. long-term outcomes of renal replacement therapy (RRT) Methods. All Dutch living adult patients with ESRD onset in children is lacking. at age of 0 to 14 years between 1972 and 1992 were invited In adults, cardiovascular disease has been recognized for echocardiography and blood pressure assessment. Special attention was paid to evidence of left ventricular hypertrophy as the most important cause of death in patients with ESRD (LVH), diastolic dysfunction, and aortic valve calcification. We [4-8]. Left ventricular hypertrophy (LVH) is the most collected data on determinants by review of all medical charts. frequently observed alteration in these patients and has Results. Of all the 187 living patients, 140 participated in been directly associated with acute death [5, 7]. Chronic the study. Of those, 110 patients had received a transplant and 30 patients were on dialysis. Mean age was 29.2 (20.7 to 41.8) pressure and volume overload, as a result of water and years. Left ventricular mass index (LVMI) exceeded 150 g/m 2 salt retention and hypertension, and the use of cyclospoin 47% of all male patients and 120 g/m 2 in 39% of all female rine in transplantation have been regarded as potential patients, both consistent with LVH. Diastolic dysfunction, derisk factors in the development of LVH in older adult fined as an early over atrial transmitral blood flow velocity patients with ESRD [6,. Enhanced calcification of the (E/A ratio) Ͻ1, was found in 18 (13%) patients; 27 (19%) had aortic valve calcification. Multiple regression analysis revealed aortic valve as a result of chronic volume overload and the following: a high LVMI was associated with a current high a high serum calcium-phosphate product has recently blood pressure ( ϭ 0.46, P Ͻ 0.001) and male gender ( ϭ been recognized as another independent risk factor for 0.21, P ϭ 0.009), a low E/A ratio with aging ( ϭ Ϫ0.28, P Ͻ acute death in adult patients with ESRD [10, 11]. 0.001) and a glomerular filtration rate (GFR) Ͻ25 mL/min/ 1.73 m 2 ( ϭ Ϫ0.29, P Ͻ 0.001), and aortic valve calcification To date, few data exist on cardiac disease in young with prolonged peritoneal dialysis ( ϭ 0.36, P Ͻ 0.001). adults with ESRD since childhood. There are concerns Conclusion. Young adult patients with pediatric ESRD are that, as in patients with adult onset of ESRD, these at risk for LVH caused by hypertension and for aortic valve patients are at risk for cardiovascular death at a very calcification. Diastolic function decreases with age and is enhanced by a current low GFR. Prolonged peritoneal dialysis young age. We conducted a national long-term followmay enhance aortic valve calcification. up study to evaluate late effects of renal insufficiency in children (LERIC). The cohort consisted of all Dutch adult patients that commenced RRT between 1972 and As life expectancy has increased in children with end-1992 at age 0 to 14 years. Recently, we reported on morstage renal disease (ESRD), concern has arisen about tality and causes of death . Cardiovascular disease was found to be the cause of death in more than 40% of
Kidney International, 2003
disease is a common, often disabling, complication of nile renal failure. end-stage renal disease... more disease is a common, often disabling, complication of nile renal failure. end-stage renal disease (ESRD) [1-4]. Chronic acidosis Background. Little is known about the late effects of juveand hypovitamin-D-and phosphate-induced secondary nile end-stage renal disease (ESRD) on bone integrity. To hyperparathyroidism can lead to disturbed bone modelestablish clinical manifestations of metabolic bone disease and bone mineral density (BMD) in young adult patients with juve-ing with high or low bone turnover in patients on chronic nile ESRD, we performed a long-term outcome study. dialysis [5-8]. In addition, inactivity during chronic dial-Methods. A cohort was formed of all Dutch patients with ysis and corticosteroids, mainly given chronically as antionset of ESRD between 1972 and 1992 at age 0 to 14 years, rejection therapy after transplantation, may lead to osborn before 1979. Data were collected by review of medical charts, current history, physical examination, and performing teoporosis [9-14]. In children with ESRD, malnutrition, dual energy x-ray absorptiometry (DEXA) of the lumbar spine reduced bioactivity of insulin-like growth hormones and and the femoral neck. delayed puberty can aggravate these effects, disturbing Results. Clinical information was retrieved in 247 out of 249 normal bone formation and growth [15-20]. Little is known patients. Of all of these patients, 61.4% had severe growth retardation (ϽϪ2 SD), 36.8% had clinical symptoms of bone about the effects of ESRD of childhood on bone formadisease, and 17.8% were disabled by bone disease. Growth tion at adult age, and about the extent of clinical bone retardation and clinical bone disease were associated with a disease in long-term survivors of juvenile ESRD. long duration of dialysis. DEXA was performed in 140 out of We conducted a national long-term follow up study in 187 living patients. Mean BMD Ϯ SD corrected for gender order to evaluate Late physical, social and psychological and age (Z score) of the lumbar spine was Ϫ2.12 Ϯ 1.4 and of the femoral neck was Ϫ1.77 Ϯ 1.4. A low lean body mass Effects of Renal Insufficiency in Children (LERIC Study). was associated with a low lumbar spine and a low femoral The cohort consisted of all Dutch children who had comneck BMD; male gender, physical inactivity and aseptic bone menced RRT between 1972 and 1992 and who had reached necrosis were associated with a low lumbar spine BMD. Conclusion. Bone disease is a major clinical problem in young (or would have reached if still alive) adulthood at the adults with pediatric ESRD. Further follow-up is needed to time of investigation. This article reports the impact on establish the impact of the low bone mineral densities found growth, the incidence of fractures, clinically apparent bone in these patients. disease and bone densities measured by dual energy x-ray absorptiometry (DEXA) in these young adults with pediatric onset of ESRD. As life expectancy has increased in children with chronic renal replacement therapy (RRT), concern has arisen about its late complications. In adults, metabolic bone METHODS
Journal of the American Society of Nephrology, 2002
Increased arterial stiffness is a risk factor for mortality in adults over 40 yr of age with end-... more Increased arterial stiffness is a risk factor for mortality in adults over 40 yr of age with end-stage renal disease (ESRD). As no data exist on vascular changes in young adults with ESRD since childhood, a long-term outcome study was performed. All living Dutch adult patients with onset of ESRD between 1972 and 1992 at age 0 to 14 yr were invited for carotid artery and cardiac ultrasound and BP measurements. Data on clinical characteristics were collected by review of all medical charts. Carotid ultrasound data were compared with those of 48 age-matched and gender-matched healthy controls. Carotid artery and cardiac ultrasound was performed in 130 out of 187 eligible patients. Mean age was 29.0 (20.7 to 40.6) yr. Compared with controls, patients had a similar intima media thickness but a reduced mean arterial wall distensibility DC
The daily gluten intake in relatives of patients with coeliac disease compared with that of the general Dutch population
European Journal of Gastroenterology & Hepatology, 1997
It has been suggested that the amount of gluten intake in populations offers an explanation for d... more It has been suggested that the amount of gluten intake in populations offers an explanation for differences in the epidemiology of coeliac disease. Investigations into first-degree relatives of coeliac disease patients have often shown that relatives exhibit intermediate features of coeliac disease, possibly due to a low gluten intake. The aim of this study was to investigate the pattern of gluten consumption in the general Dutch population for different age and sex groups and for different product groups, and to investigate the daily gluten intake of first-degree relatives of coeliac disease patients. Questionnaires concerning the gluten intake of 55 first-degree relatives of coeliac disease patients were analysed. To determine the gluten intake of the general Dutch population, the results of a mass investigation were used. The amount of gluten in the gluten-rich products was estimated by multiplying the amount of vegetable proteins by 0.8. The median daily gluten intake of the relatives was 12.9 g (range: 3.8-31.3). The mean daily gluten intake of the study population in the Netherlands was 1 3.1 g. The gluten intake of first-degree relatives of coeliac disease patients was the same as that of the general population. Thus, a low gluten intake apparently does not explain the aspecific presentation and prevalence of coeliac disease in first-degree relatives of coeliac disease patients.
BDJ, 2007
Defects were much commoner than in a control group, and most were of a characteristic type.
Thorax, 1996
Hepatitis C virus in pulmonary fibrosis genotypes were identified had genotype II alone or in com... more Hepatitis C virus in pulmonary fibrosis genotypes were identified had genotype II alone or in combination with other types, and the high frequency of type II genotype is in keeping with the prevalence in Italy.'3 Thus, HCV infection in patients with IPF is not associated with specific genotypes. Thirdly, in two patients a combination of two and three genotypes was present, a rare association in a single patient and almost exclusively confined to cases who have received multiple transfusions.'2
Cancer in children in the Netherlands (1989-1997). – In a recent report by the Society of Integra... more Cancer in children in the Netherlands (1989-1997). – In a recent report by the Society of Integral Cancer Centres in the Netherlands, attention was devoted to the incidence of cancer in children and the mortality arising from this. In recent years the growing diagnostic and therapeutic possibilities have changed the perspective of childhood cancer enormously. Based on a careful classification and clinical staging, national and internaNed Tijdschr Geneeskd 2001 28 juli;145(30) 1443 tional investigations have resulted in new and successful therapeutical strategies. Overall prognoses of childhood cancer have improved dramatically from a 5-year survival rate in the 1960s and 1970s of less than 30%, to an 8-year survival rate of more than 70% between 1989 and 1997. However, this success means that more investigations into the long-term effects of childhood cancer and its treatment are needed. In a study at the Amsterdam University Hospital, 700 adult survivors of childhood cancer were re...
Should relatives of coeliacs with mild clinical complaints undergo a small-bowel biopsy despite negative serology?
European Journal of Gastroenterology & Hepatology, 2000
Small intestinal lesions in coeliac disease (CD) have a variable severity. Early diagnosis of CD ... more Small intestinal lesions in coeliac disease (CD) have a variable severity. Early diagnosis of CD is important because treatment allows a normal psycho-physical development, especially in children, and can avoid associated disorders. The aim of this study was to evaluate the predictive value of screening parameters for the detection and estimation of CD prevalence in first-degree relatives. The screening was performed in 338 first-degree relatives of 134 coeliac families. Questionnaires and a physical examination followed by haematological analyses and serologyfor IgA anti-endomysium (EMA)/IgA antigliadin (AGA) antibodies were used in orderto selectthe candidates for small-bowel biopsy. The small-bowel biopsy was indicated on the basis of clinical complaints, laboratory tests and serology performed in 96 (28%) of the study group. CD was diagnosed in 17/96 cases. Six of the 17 showed total villous atrophy (VA) (Marsh IIIc), five subtotal VA (Marsh IIIb) and six partial VA (Marsh IIIa). EMA and AGA were strongly positive in the six patients whose intestinal biopsy showed total VA. However, only one coeliac out of the six patients with partial VA had positive EMA and AGA. A significant proportion of coeliacs may be missed if cases are screened by serology only. Although endomysial antibody assay has been reported as a highly sensitive and specific test for detection of CD, we argue that using only EMA and AGA in screening is not enough for investigation of the true prevalence of CD. A combination of clinical parameters as described in this study and laboratory/serological tests is an important and practical contribution to improving the detection rate of CD.
Screening for child abuse using a checklist and physical examinations in the emergency department led to the detection of more cases
Acta paediatrica (Oslo, Norway : 1992), Jan 10, 2018
We studied the accuracy of a screening checklist (SPUTOVAMO), complete physical examination (top-... more We studied the accuracy of a screening checklist (SPUTOVAMO), complete physical examination (top-to-toe inspection, TTI) and their combination in detecting child abuse in the emergency department (ED). Consecutive patients admitted to the ED of the Academic Medical Center in Amsterdam between January 2011 and 1 July 2013 were included. An Expert Panel assigned a consensus diagnosis to positive cases. For all other and missed cases, the Child Abuse Counselling and Reporting Centre diagnosis was used. We included 17 229 admissions of 12 198 patients. In 46%, SPUTOVAMO was performed, in 33% TTI; 421 children (4.3%) tested positive on either or both, with 68 positive consensus diagnoses. In eight children not reported to the Expert Panel, the Child Abuse Counselling and Reporting Center diagnosis was positive. Ten of 3519 (0.3%) children testing negative on both were child abuse cases; 0.88% of the study group had a final child abuse diagnosis. The estimated PPV was 0.46 for SPUTOVAMO, ...
Assessments carried out by a child abuse and neglect team in an Amsterdam teaching hospital led to interventions in most of the reported cases
Acta paediatrica (Oslo, Norway : 1992), Jan 30, 2016
This study described cases of child abuse and neglect (CAN) that were reported to the multiagency... more This study described cases of child abuse and neglect (CAN) that were reported to the multiagency CAN team at the Emma Children's Hospital in Amsterdam and the resulting interventions. We carried out a retrospective review of all cases that were reported to the CAN team from 1 January 2010 to 31 December 2012. There were 27 prenatal cases, 92 referrals based on parental characteristics and 523 children. Overall, 1.2% of the children visiting the emergency department of our hospital, attending the outpatients department or being admitted were reported to the team. More than half of the referrals (55.1%) were confirmed as CAN. The most common diagnoses were as follows: witnessing intimate partner violence, physical neglect and emotional abuse. If CAN was confirmed an intervention was offered in 98.3% of cases. If a CAN diagnosis was undetermined or rejected, the figures were still 83.5% and 64.2%, respectively. Our results showed that CAN affected more than one in every 100 childr...
Comparing the effect of Livercell Transplantation (LCTX) in Gunn rats using either immunesuppresive therapy or immunetolerance inducing techniques
J Pediat Gastroenterol Nutr, 1998
The pharmacokinetics of nelfinavir in HIV-1-infected children
Therapeutic Drug Monitoring, Aug 1, 2002
The authors investigated the steady-state plasma pharmacokinetics of nelfinavir in HIV-1-infected... more The authors investigated the steady-state plasma pharmacokinetics of nelfinavir in HIV-1-infected children in a dosage of 30 mg/kg every 8 hours and 45 mg/kg every 12 hours in 12 HIV-1-infected children (aged 2.1 to 10.8 years) participating in an open-label study of nelfinavir in combination with stavudine and lamivudine. Median values of the primary pharmacokinetic parameters of nelfinavir 30 mg/kg every-8-hours (n = 8) and 45 mg/kg every 12 hours (n = 10) were, respectively, for the area under the plasma concentration-time curve over 24 hours, 90.5 and 71.9 h x microg/mL, for the trough concentration 1.9 and 1.0 microg/mL, and for the maximal concentration 6.3 and 5.2 microg/mL. Overall, a 7-fold interpatient variability was observed for the exposure to nelfinavir. Nelfinavir 30 mg/kg every-8-hours or 45 mg/kg every 12 hours in HIV-1-infected children generally resulted in plasma concentrations higher than those obtained in adults. However, due to a large interpatient variability in the exposure, individual dosage adjustments based on plasma concentrations may be necessary for both dosing regimens to ensure optimal treatment.
Endoscopic Balloon Dilatation as Treatment of Gastric Outlet Obstruction in Infancy and Childhood: 79
Pediat Res, 1987
ABSTRACT
De ziekte van Fabry: etiologie, kliniek en behandeling
Kindermishandeling, diagnostische en therapeutische dilemma's
Tijdschrift Voor Kindergeneeskunde, 2005
Kanker op de kinderleeftijd in Nederland (1989-1997)
Functionele gastro-intestinale ziektebeelden op de kinderleeftijd. II. Obstipatie en solitaire encopresis; fysiologie en pathofysiologie
Nederlands Tijdschrift Voor Geneeskunde, 2003