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David C van der Zee,
Daniel C Aronson,
Gerard C Madern,
Zacharias J De Langen,
N Looyaard,
Marianne J G Hanneman,
Paul N M A Rieu, Esther E Hartman,
Mirjam A G Sprangers,
Frans J Oort,
L W Ernest van Heurn,
M van Silfhout-Bezemer
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ABSTRACT: To examine the outcome of biliary atresia (BA) and to identify prognostic factors using a national database.
All children born between January 1987 and December 2008 who underwent the Kasai surgical procedure for BA were retrieved from the Netherlands Study Group on Biliary Atresia Registry database. Outcomes were measured in terms of clearance of jaundice (bilirubin <1.17 g/dL, or 20 μmol/L, within 6 months after surgery) and 4-year transplant-free survival. Two cohorts, one from 1987-1997 and the other from 1998-2008, were compared. Survival rates were determined using Kaplan-Meier analysis, and prognostic factors were tested with univariate and multivariate analyses.
Between January 1987 and December 2008, 214 patients underwent Kasai surgery for BA. In this series, the 4-year transplant-free survival was 46%±4%, and 4-year overall survival was 73%±3%. Clearance of jaundice, surgery within 60 days, and postoperative antibiotic prophylaxis use were independently associated with increased transplant-free survival. The yearly caseload per center (range, 0.5-2.1) was not correlated with transplant-free survival (r=0.024; P=.73).
During the past 2 decades, outcome parameters have remained constant and are comparable with those reported from other Western countries, despite a relatively low annual caseload per center. Timely surgical correction and postoperative antibiotic therapy were associated with a higher transplant-free survival rate.
The Journal of pediatrics 11/2011; 160(4):638-644.e2. · 4.02 Impact Factor
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ABSTRACT: In order to further improve the outcome of BA, we characterized the mortality of BA patients who did not undergo OLT in the Netherlands, and compared our results with international data. For this purpose, we analyzed the causes of mortality of non-transplanted BA patients before the age of five yr, using the NeSBAR database. To evaluate trends in mortality, we compared the cohort 1987-1996 (n=99) with 1997-2008 (n=111). We compared clinical condition at OLT assessment with available international data, using the PELD-score. Mortality of non-transplanted BA children was 26% (26/99) in 1987-1996 and 16% (18/111) in 1997-2008 (p=0.09). Sepsis was the prevailing direct cause of death (30%; 13/44). PELD-scores at the time of assessment were higher in non-transplanted BA patients (median 20.5; range 13-40) compared with international data (mean/median between 11.7 and 13.3). Based on our national data, we conclude that pretransplant mortality of BA patients is still considerable, and that sepsis is a predominant contributor. Our results strongly indicate that the prognosis of patients with BA in the Netherlands can be improved by earlier listing of patients for OLT and by improving pretransplant care.
Pediatric Transplantation 03/2011; 15(2):176-83. · 1.48 Impact Factor
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Esther E Hartman,
Frans J Oort,
Mirjam A G Sprangers,
Marianne J G Hanneman,
L W Ernest van Heurn, Zacharias J de Langen,
Gerard C Madern,
Paul N M A Rieu,
David C van der Zee,
Nic Looyaard,
Marina van Silfhout-Bezemer,
Daniel C Aronson
[show abstract]
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ABSTRACT: First, to compare the quality of life (QL) and perceived self-competence of children and adolescents with anorectal malformations or Hirschsprung disease with that of reference groups. Second, to identify predictors of QL.
A total of 491 patients with anorectal malformations or Hirschsprung disease were sent a questionnaire, which assessed QL (mental, physical), disease-specific functioning (defecation-related), perceived self-competence (self-esteem, athletic competencies, school attitude), and demographic characteristics (sex, age). The clinical characteristics (disease severity, presence of congenital anomalies) were extracted from medical records.
More than 50% (316, 64%) of patients with anorectal malformations or Hirschsprung disease completed the questionnaire. On average, children and adolescents in both patient groups reported no differences in QL domains compared with the reference groups. However, standard deviations revealed considerable individual variation, indicating the presence of patients with high levels of QL as well as patients with low levels of QL. Children and adolescents in both patient groups reported psychosocial problems in all domains, compared with the reference groups. Females, older patients, and those with a severe form of the disease reported lower levels of perceived self-competence and global disease-specific functioning, which in turn predicted QL.
Our results should alert clinicians to patients who are at risk for QL problems and may therefore be in need of extra care. Our findings illustrate the importance of both global disease-specific functioning and perceived psychosocial competencies for enhancing the QL of these patients.
Journal of pediatric gastroenterology and nutrition 11/2008; 47(4):463-71. · 2.18 Impact Factor
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Esther E Hartman,
Frans J Oort,
Mirjam AG Sprangers,
Marianne JG Hanneman,
LW Ernest van Heurn, Zacharias J de Langen,
Gerard C Madern,
Paul NMA Rieu,
David C van der Zee,
Nic Looyaard,
Marina van Silfhout-Bezemer,
Daniel C Aronson
[show abstract]
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ABSTRACT: Objectives: First, to compare the quality of life (QL) and perceived self-competence of children and adolescents with anorectal malformations or Hirschsprung disease with that of reference groups. Second, to identify predictors of QL.
Patients and Methods: A total of 491 patients with anorectal malformations or Hirschsprung disease were sent a questionnaire, which assessed QL (mental, physical), disease-specific functioning (defecation-related), perceived self-competence (self-esteem, athletic competencies, school attitude), and demographic characteristics (sex, age). The clinical characteristics (disease severity, presence of congenital anomalies) were extracted from medical records.
Results: More than 50% (316, 64%) of patients with anorectal malformations or Hirschsprung disease completed the questionnaire. On average, children and adolescents in both patient groups reported no differences in QL domains compared with the reference groups. However, standard deviations revealed considerable individual variation, indicating the presence of patients with high levels of QL as well as patients with low levels of QL. Children and adolescents in both patient groups reported psychosocial problems in all domains, compared with the reference groups. Females, older patients, and those with a severe form of the disease reported lower levels of perceived self-competence and global disease-specific functioning, which in turn predicted QL.
Conclusions: Our results should alert clinicians to patients who are at risk for QL problems and may therefore be in need of extra care. Our findings illustrate the importance of both global disease-specific functioning and perceived psychosocial competencies for enhancing the QL of these patients.
Journal of Pediatric Gastroenterology and Nutrition 09/2008; 47(4):463-471. · 2.30 Impact Factor
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ABSTRACT: Long-term functional sequelae after resection of sacrococcygeal teratoma (SCT) are relatively common. This study determines the incidence of these sequelae associated clinical variables and its impact on quality of life (QoL).
Patients with SCT treated from 1980 to 2003 at the pediatric surgical centers in The Netherlands aged more than 3 years received age-specific questionnaires, which assessed parameters reflecting bowel function (involuntary bowel movements, soiling, constipation), urinary incontinence, subjective aspect of the scar, and QoL. These parameters were correlated with clinical variables, which were extracted from the medical records. Risk factors were identified using univariate analysis.
Of the 99 posted questionnaires, 79 (80%) were completed. The median age of the patients was 9.7 years (range, 3.2-22.6 years). There were 46% who reported impaired bowel function and/or urinary incontinence (9% involuntary bowel movements, 13% soiling, 17% constipation), and 31% urinary incontinence. In 40%, the scar was cosmetically unacceptable. Age at completion of the questionnaire, Altman classification, sex, and histopathology were not risk factors for any long-term sequelae. Size of the tumor (>500 cm3) was a significant risk factor for cosmetically unacceptable scar (odds ration [OR], 4.73; confidence limit [CL], 1.21-18.47; P = .026). Long-term sequelae were correlated with diminished QoL.
A large proportion of the patients with SCT have problems with defecation, urinary incontinence, or a cosmetically unacceptable scar that affects QoL. Patients who are at higher risk for the development of long-term sequelae cannot be clearly assessed using clinical variables.
Journal of Pediatric Surgery 06/2007; 42(6):1122-6. · 1.45 Impact Factor
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Esther E Hartman,
Frans J Oort,
Daniel C Aronson,
Marianne J G Hanneman,
Ernest van Heurn, Zacharias J de Langen,
Gerard C Madern,
Paul N M A Rieu,
David C van der Zee,
Nic Looyaard,
Marina van Silfhout-Bezemer,
Mirjam A G Sprangers
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ABSTRACT: The purpose of this work was to examine changes in quality of life, disease-specific functioning, and psychosocial competencies of children and adolescents (8-16 years of age) with anorectal malformations or Hirschsprung disease and to identify predictors of change in quality of life by testing an explanatory model in which background variables explained changes in quality of life via changes in disease-specific functioning and psychosocial competencies.
Questionnaires were administered to 129 patients with anorectal malformations and 121 patients with Hirschsprung disease within a 3-year interval. Clinical and sociodemographic background variables were measured on the first occasion. Quality of life (physical and mental), disease-specific functioning (defecation-related), and psychosocial competencies (self-esteem, athletic competencies, and school attitude) were measured on both occasions.
Patients improved in disease-specific functioning and mental quality of life. Changes in quality of life were indeed explained by the explanatory model. Among other things, the results indicated that patients with a severe form of the disease or with additional congenital diseases showed worsening of school attitude, which in turn affected change in mental quality of life negatively.
Children and adolescents with anorectal malformations or Hirschsprung disease reported better quality of life over time. To improve and maintain an optimal level of children's and adolescents' quality of life, it is important to direct treatment both to reducing symptoms and enhancing psychosocial competencies, in particular by paying attention to school attitude.
PEDIATRICS 03/2007; 119(2):e374-83. · 4.47 Impact Factor
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Esther E Hartman,
Mirjam A G Sprangers,
Mechteld R M Visser,
Frans J Oort,
Marianne J G Hanneman,
L W Ernest van Heurn, Zacharias J de Langen,
Gerard C Madern,
Paul N M A Rieu,
David C van der Zee,
Marina van Silfhout-Bezemer,
Daniel C Aronson
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ABSTRACT: The objective of this study was to examine the types of healthcare services used by children, adolescents, and adults with Hirschsprung Disease (HD) in relation to the severity of the initial defect (mild, severe), whether additional care was needed, the provision of information, transfer to adult care, and satisfaction with the care provided.
Three hundred twenty (71%) HD patients, ages 6 to 54, completed a questionnaire that assessed the use of healthcare services, the need for more healthcare, the provision of information, the transfer to adult care and satisfaction with the provided care.
In 6 months, 45% of the children, 14% of the adolescents, and 15% of the adults consulted a medical specialist. Compared with patients with a mild form of HD in the age range of 6 to 16 years, only the more severely afflicted adult patients visited medical professionals more often (10% vs 29%) (P < .05). Of the children, the adolescents, and the adults 23%, 8%, and 6% respectively consulted a nonmedical professional. Less than 15% of all patients whould have liked more treatment. In 6 months 51% of the children, 24% of the adolescents, and 21% of the adults received treatment information, of which respectively 14, 8, and 20% wished they had received more information. Three (12%) patients who needed adult care encountered problems with the transfer. Almost all patients were satisfied with the care provided.
There is good access to medical healthcare services, especially for children. The only lacuna in the healthcare system we revealed was a lack of information, particularly for adult patients. Most parents and patients reported to be very satisfied with the provided care.
Journal of Pediatric Surgery 09/2006; 41(8):1420-4. · 1.45 Impact Factor
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Esther E Hartman,
Frans J Oort,
Mechteld R Visser,
Mirjam A Sprangers,
Marianne J Hanneman, Zacharias J de Langen,
L W Ernst va Heurn,
Paul N M A Rieu,
Gerard C Madern,
David C van der Zee,
Nic Looyard,
Marina van Silfhout-Bezemer,
Daniel C Aronson
[show abstract]
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ABSTRACT: The aim of this study was to examine changes in the quality of life of adult patients with anorectal malformations or Hirschsprung's disease over a three-year interval and to identify demographic, clinical, and psychosocial variables that explain possible quality-of-life changes. Understanding the factors that affect changes in quality of life over time is particularly important to provide adequate care.
Questionnaires were administered to 261 patients (77 percent), with a three-year interval. Background characteristics, including demographic and clinical variables, and psychosocial variables (i.e., self-esteem, mastery, social support, disease cognition) were measured on one occasion. Generic and disease-specific quality of life were measured twice.
On average patients indicated no change in quality-of-life level after three years. However, variance in the change scores revealed individual variation, indicating the presence of patients who improved and patients who deteriorated. Patients who were female, older, have other congenital diseases, or a stoma reported poorer quality of life over time. The psychosocial variable "disease cognition" most strongly affected the change in quality of life of patients with anorectal malformations or Hirschsprung's disease.
Our results could alert clinicians to patients who are at risk for quality-of-life deterioration and might therefore be in need for extra care. Our findings illustrate the importance of psychosocial functioning for enhancing the quality of life over time of these patients.
Diseases of the Colon & Rectum 02/2006; 49(1):96-103. · 3.13 Impact Factor
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ABSTRACT: Esophageal cancer development after previous atresia repair is extremely rare in young patients. We present the clinical course of a patient who developed an adenocarcinoma of the esophagus at the age of 22 years, after repair of a tracheoesophageal fistula with esophageal atresia in the neonatal period. She developed a stricture of the esophageal anastomosis requiring frequent dilatations. Six years after an antireflux procedure because of a difficult treatable severe gastroesophageal reflux, an advanced adenocarcinoma was detected at the site of the end-to-end anastomosis of the previous atresia.
Journal of Pediatric Surgery 01/2006; 40(12):e1-4. · 1.45 Impact Factor
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Esther E. Hartman,
Frans J. Oort,
Mechteld R. Visser,
Mirjam A. Sprangers,
Marianne J. G. Hanneman, Zacharias J. de Langen,
L. W. Ernest van Heurn,
Paul N. M. A. Rieu,
Gerard C. Madern,
David C. van der Zee,
Nic Looyaard,
Marina van Silfhout-Bezemer,
Daniel C. Aronson
[show abstract]
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ABSTRACT: PurposeThe aim of this study was to examine changes in the quality of life of adult patients with anorectal malformations or Hirschsprung's
disease over a three-year interval and to identify demographic, clinical, and psychosocial variables that explain possible
quality-of-life changes. Understanding the factors that affect changes in quality of life over time is particularly important
to provide adequate care.
MethodsQuestionnaires were administered to 261 patients (77 percent), with a three-year interval. Background characteristics, including
demographic and clinical variables, and psychosocial variables (i.e., self-esteem, mastery, social support, disease cognition) were measured on one occasion. Generic and disease-specific quality
of life were measured twice.
ResultsOn average patients indicated no change in quality-of-life level after three years. However, variance in the change scores
revealed individual variation, indicating the presence of patients who improved and patients who deteriorated. Patients who
were female, older, have other congenital diseases, or a stoma reported poorer quality of life over time. The psychosocial
variable “disease cognition” most strongly affected the change in quality of life of patients with anorectal malformations
or Hirschsprung's disease.
ConclusionsOur results could alert clinicians to patients who are at risk for quality-of-life deterioration and might therefore be in
need for extra care. Our findings illustrate the importance of psychosocial functioning for enhancing the quality of life
over time of these patients.
Diseases of the Colon & Rectum 12/2005; 49(1):96-103. · 3.13 Impact Factor
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Esther E Hartman,
Mirjam A G Sprangers,
Mechteld R M Visser,
Frans J Oort,
Marianne J G Hanneman,
L W Ernest van Heurn, Zacharias J de Langen,
Gerard C Madern,
Paul N M A Rieu,
David C van der Zee,
N Looyaard,
Daniel C Aronson
[show abstract]
[hide abstract]
ABSTRACT: The first aim was to identify the types of healthcare services used by children, adolescents, and adults with anorectal malformation (ARM) in relation to the severity of their disease and to examine whether additional care was needed. The second aim was to evaluate specific areas in the healthcare system, including provided information, transfer from pediatric to adult care, and satisfaction with the provided care.
Three hundred eighty-six (61%) patients with ARM, ages 6 to 52, completed a questionnaire that assessed their use of healthcare services and the need for additional services. Also, questions were asked about specific areas in the healthcare system. Clinical and sociodemographic characteristics were extracted from medical records.
In the preceding 6 months 50% of the children, 24% of the adolescents, and 24% of the adults consulted a medical specialist. Compared with patients with a mild form of ARM in the age range of 6 to 16 years, the more severely afflicted patients visited medical professionals more often (18% vs. 32%). Particularly, adolescents in the age range of 12 to 16 years with a severe form of the disease more often visited the pediatric surgeon than their peers with a mild form (2% vs. 16%). Twenty-three percent of the children, 7% of the adolescents, and 8% of the adults consulted a nonmedical professional. Twenty percent of the children, 13% of the adolescents, and 17% of the adults would have liked additional or more treatment of a nonmedical professional. In 6 months, 40% of the children, 24% of the adolescents, and 20% of the adults received treatment information. One third of the adult patients who were transferred to "adult" surgeons encountered transfer problems. Almost all patients were satisfied with the care provided.
There is good access to medical healthcare services, especially for children. However, more psychosocial and paramedical care is considered necessary. As could be expected, children and adolescents with a severe form of the disease reported to have visited a medical specialist more often. Although healthcare for patients with ARM may be improved at certain points, most parents and patients were very satisfied with the care provided.
Journal of Pediatric Gastroenterology and Nutrition 09/2005; 41(2):210-5. · 2.30 Impact Factor
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Esther E Hartman,
Frans J Oort,
Daniel C Aronson,
Marianne J G Hanneman,
David C van der Zee,
Paul N M A Rieu,
Gerard C Madern, Zacharias J De Langen,
L W Ernest van Heurn,
M van Silfhout-Bezemer,
N Looyaard,
Mirjam A G Sprangers
[show abstract]
[hide abstract]
ABSTRACT: The first objective was to compare the quality of life of adult patients with anorectal malformations (ARM) or Hirschsprung's disease (HD) and to compare both groups with healthy people. And the second objective was to examine the factors that affect the quality of life of patients with ARM or HD, using a theoretical model in which patients' background characteristics explain quality of life via mediating disease-specific functioning and psychosocial functioning.
Three hundred and forty-one patients completed a questionnaire, which assessed sociodemographic characteristics, disease-specific and psychosocial functioning, and quality of life. Clinical factors were extracted from the medical records.
Patients with ARM or Hirschsprung's disease did not differ in their quality of life. Compared to healthy people, both patient groups reported more limitations in their "overall" physical quality of life, but only patients with ARM reported impaired quality of life on several specific domains (e.g., physical role-functioning, pain). The model was largely accepted. Most striking were the strong effects of the psychosocial functioning factors in contrast to weak effects of the disease-specific "constipation" and "fecal continence" factors.
The quality of life of patients with ARM or Hirschsprung's disease was found to be comparable. Compared to healthy people, both patient groups encountered "overall" physical health problems, but only patients with ARM reported additional pain and limitations in role functioning due to physical problems. It appeared that psychosocial functioning had the most important effect on the quality of life of patients with ARM or Hirschsprung's disease, while fecal incontinence and constipation had almost no effect on their quality of life.
The American Journal of Gastroenterology 06/2004; 99(5):907-13. · 7.28 Impact Factor
