Publications by authors named "Ralph J B Sakkers"

21 Publications

  • Page 1 of 1

Quantifying the Effects of Hip Surgery on the Sphericity of the Femoral Head in Patients with Mucopolysaccharidosis Type I.

J Bone Joint Surg Am 2021 Mar;103(6):489-496

Departments of Orthopedic Surgery (E.L.v.d.V., H.W., and R.J.B.S.), Radiology (E.J.B.), and Pediatrics (P.M.v.H.), University Medical Center Utrecht, Utrecht, the Netherlands.

Background: The introduction of stem cell transplantation has improved life expectancy and cognitive outcome in patients with mucopolysaccharidosis I, but this condition remains associated with substantial residual disease in several parts of the body. Many patients have hip dysplasia with progressive medial flattening of the femoral head. Quantitative evidence on the effect of surgery on remodeling to sphericity of flattened femoral heads is lacking. In the present study, we used statistical shape modeling to quantify the effect of hip surgery on the sphericity of the femoral head in patients with mucopolysaccharidosis I.

Methods: We performed a retrospective case control study involving a series of 23 patients with hip dysplasia due to mucopolysaccharidosis I. Surgery was not offered to the first 11 children (control group). Following a change in treatment protocol, the next 12 children underwent bilateral proximal femoral varus derotation osteotomy and Pemberton osteotomy for the treatment of acetabular dysplasia with progressive femoral head flattening (surgery group). The surgery and control groups were compared with a reference group of patients with normal hips. Statistical shape modeling was used to quantify the shape of the femoral head (i.e., flattening and/or roundness of the epiphysis).

Results: The mean age at the time of stem cell transplantation in the surgery and control groups was comparable (1.2 years). The mean age at the time of surgical intervention was 5.5 years, and mean duration of postoperative follow-up was 3.3 years. Statistical shape modeling showed variations within the total group in terms of medial indentation, width, height, and sphericity of the femoral heads. In contrast to the progressive femoral head flattening in the control group, the surgery group showed improvement of the sphericity of the femoral head after surgery. The overall shape characteristics of the femoral head in the surgery group were similar to those of the reference group of patients with normal hips.

Conclusions: To our knowledge, this is the first study in patients with mucopolysaccharidosis I that has shown quantitative remodeling of the dysplastic, flattened femoral head to normal sphericity after increasing containment of the femoral head.

Level Of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.
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http://dx.doi.org/10.2106/JBJS.20.00515DOI Listing
March 2021

Combined massive allograft and intramedullary vascularized fibula transfer: the Capanna technique for treatment of congenital pseudarthrosis of the tibia.

Acta Orthop 2020 10 8;91(5):605-610. Epub 2020 Jun 8.

University Medical Center Utrecht, The Netherlands.

Background and purpose - Congenital pseudarthrosis of the tibia (CPT) is caused by local periosteal disease that can lead to bowing, fracturing, and pseudarthrosis. Current most successful treatment methods are segmental bone transport and vascularized and non-vascularized bone grafting. These methods are commonly hampered by discomfort, reoperations, and long-term complications. We report a combination of a vascularized fibula graft and large bone segment allograft, to improve patient comfort with similar outcomes.Patients and methods - 7 limbs that were operated on in 6 patients between November 2007 and July 2018 with resection of the CPT and reconstruction with a vascularized fibula graft in combination with a bone allograft were retrospectively studied. The mean follow-up time was 5.4 years (0.9-9.6). Postoperative endpoints: time to discharge, time to unrestricted weight bearing, complications within 30 days, consolidation, number of fractures, and secondary deformities.Results - The average time to unrestricted weight bearing with removable orthosis was 3.5 months (1.2-7.8). All proximal anastomoses consolidated within 10 months (2-10). 4 of the 7 grafts fractured at the distal anastomosis between 6 and 14 months postoperatively. After reoperation, consolidation of the distal anastomosis was seen after 2.8 months (2-4). 1 patient required a below-knee amputation.Interpretation - This case series showed favorable results of the treatment of CPT through a combination of a vascularized fibula graft and large bone segment allograft, avoiding the higher reintervention rate and discomfort with ring frame bone transport, and the prolonged non-weight bearing with vascularized fibula transfer without reinforcement with a massive large bone segment allograft.
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http://dx.doi.org/10.1080/17453674.2020.1773670DOI Listing
October 2020

Long-term outcomes of the hip shelf arthroplasty in adolescents and adults with residual hip dysplasia: a systematic review.

Acta Orthop 2020 08 2;91(4):383-389. Epub 2020 Apr 2.

Department of Orthopedics, University Medical Center Utrecht, Utrecht.

Background and purpose - The shelf arthroplasty was the regular treatment for residual hip dysplasia before it was substituted by the peri-acetabular osteotomy. Yet, evidence regarding the survival of shelf arthroplasty surgery has never been systematically documented. Hence, we investigated the survival time of the shelf procedure until revision to THA in patients with primary hip dysplasia. Factors that influenced survival and complications were also examined, along with the accuracy of correcting radiographic parameters to characterize dysplasia.Material and methods - The inclusion criteria were studies of human adolescents and adults (> 16 years) with primary or congenital hip dysplasia who were treated with a shelf arthroplasty procedure. Data were extracted concerning patient characteristics, survival time, complications, operative techniques, and accuracy of correcting radiographic parameters.Results - Our inclusion criteria were applicable to 9 studies. The average postoperative Center-Edge Angle and Acetabular Head Index were mostly within target range, but large variations were common. Kaplan-Meier curves (endpoint: conversion to THA) varied between 37% at 20 years' follow-up and 72% at 35 years' follow-up. Clinical failures were commonly associated with pain and radiographic osteoarthritis. Only minor complications were reported with incidences between 17% and 32%.Interpretation - The shelf arthroplasty is capable of restoring normal radiographic hip parameters and is not associated with major complications. When carefully selected on minimal osteoarthritic changes, hip dysplasia patients with a closed triradiate cartilage may benefit from the shelf procedure with satisfactory survival rates. The importance of the shelf arthroplasty in relation to peri-acetabular osteotomies needs to be further (re)explored.
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http://dx.doi.org/10.1080/17453674.2020.1747210DOI Listing
August 2020

Club foot in association with the 22q11.2 deletion syndrome: An observational study.

Am J Med Genet A 2018 10;176(10):2135-2139

Division of Human Genetics and 22q and You Center, The Children's Hospital of Philadelphia (CHOP), Philadelphia, Pennsylvania.

The 22q11.2 Deletion Syndrome (22q11.2DS) occurs in ~1:3,000-6,000 individuals. Features less typically associated with 22q11.2DS, such as orthopedic manifestations, may be overlooked or may not lead to appropriate diagnostic testing. Club foot has a general population prevalence of ~1:1,000 and has been occasionally described in association with 22q11.2DS. Our hypothesis is that the prevalence of club foot is higher in patients with 22q11.2DS. We performed a retrospective review in two specialized 22q11.2DS centers to determine the prevalence of club foot. "True club foot" requires treatment (either conservative or surgical), therefore we only included those patients with proof of treatment. We investigated whether congenital heart disease (CHD) and/or cleft palate were associated with the presence of club foot within 22q11.2DS. The records of 1,466 patients were reviewed. Of these, 48 (3.3%) had confirmation of club foot (95% Confidence Interval: 2.4-4.3): 22 (46%) had a bilateral, 12 (25%) left, and 14 (29%) right club foot. Within our study, neither a CHD and/or a cleft palate were associated with a club foot. The prevalence of club foot in 22q11.2DS is 30 times higher than that observed in the general population. This suggests the diagnosis of club foot, especially in the face of other typically associated abnormalities of 22q11.2DS, should provoke consideration of 22q11.2DS as an underlying diagnosis, particularly in the neonatal setting.
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http://dx.doi.org/10.1002/ajmg.a.40649DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6221052PMC
October 2018

Guideline on the diagnosis and treatment of primary idiopathic clubfoot.

Acta Orthop 2017 Jun 7;88(3):305-309. Epub 2017 Mar 7.

d Knowledge Institute of Medical Specialists (Kennisinstituut van Medisch Specialisten).

- A delegation of 6 pediatric orthopedic surgeons from the Dutch Orthopedic Association (NOV) and 2 members of the board of the Dutch Parents' Association for children with clubfoot created the guideline "The diagnosis and treatment of primary idiopathic clubfeet" between April 2011 and February 2014. The development of the guideline was supported by a professional methodologist from the Dutch Knowledge Institute of Medical Specialists. This evidence-based guideline process was new and unique, in the sense that the process was initiated by a parents' association. This is the first official guideline in pediatric orthopedics in the Netherlands, and to our knowledge it is also the first evidence-based guideline on clubfoot worldwide. The guideline was developed in accordance with the criteria of the international AGREE instrument (AGREE II: Appraisal of Guidelines for Research and Evaluation II). The scientific literature was searched and systematically analyzed. In the second phase, conclusions and recommendations in the literature were formulated according to the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) method. Recommendations were developed considering the balance of benefits and harms, the type and quality of evidence, the values and preferences of the people involved, and the costs. The guideline is a solid foundation for standardization of clubfoot treatment in the Netherlands, with a clear recommendation of the Ponseti method as the optimal method of primary clubfoot treatment. We believe that the format used in the current guideline sets a unique example for guideline development in pediatric orthopedics that may be used worldwide. Our format ensured optimal collaboration between medical specialists and parents, and resulted in an important change in clubfoot care in the Netherlands, to the benefit of medical professionals as well as parents and patients. In this way, it is possible to improve professional collaboration between medical specialists and parents, resulting in an important change in clubfoot care in the Netherlands that will benefit medical professionals, parents, and patients. The guideline was published online, and is freely available from the Dutch Guideline Database ( www.richtlijnendatabase.nl ).
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http://dx.doi.org/10.1080/17453674.2017.1294416DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5434600PMC
June 2017

Efficacy and Safety of Bisphosphonate Therapy in Children with Osteogenesis Imperfecta: A Systematic Review.

Horm Res Paediatr 2015 23;84(1):26-42. Epub 2015 May 23.

Child Development & Exercise Center, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.

Background/aims: To systematically assess contemporary knowledge regarding the effectiveness and safety of bisphosphonates (BPs) in children with osteogenesis imperfecta (OI).

Methods: PubMed/MEDLINE, Embase, and Cochrane were searched for eligible articles up to June 2014. Studies eligible for inclusion were (randomized) controlled trials assessing the effects of BPs in children with OI. Methodological quality was assessed independently by 4 reviewers using the Cochrane Collaboration's tool for risk of bias.

Results: Ten studies (519 children) were included. Four studies (40%) showed a low risk of bias. All studies investigating lumbar spine areal bone mineral density indicated a significant increase as a result of BP treatment. Most studies observed a significant decrease in fracture incidence. The most frequently reported adverse events were gastrointestinal complaints, fever, and muscle soreness. A significant decrease in (bone) pain due to BP treatment was observed in more than half of the studies. Most studies measuring urinary markers of bone resorption reported a significant decrease. The majority of studies with intravenous treatment showed a significant increase in lumbar projection area, whereas studies with oral treatment did not.

Conclusions: Treatment with oral or intravenous BPs in children with OI results in an increase in bone mineral density and seems to be safe and well tolerated.
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http://dx.doi.org/10.1159/000381713DOI Listing
May 2016

Bone mineral density in developing children with osteogenesis imperfecta: a longitudinal study with 9 years of follow-up.

Acta Orthop 2013 Aug;84(4):431-6

Department of Orthopaedic Surgery, Wilhelmina Children's Hospital, University Medical Center Utrecht, the Netherlands.

Background And Purpose: Osteogenesis imperfecta (OI) is a heritable disorder of connective tissue caused by a defect in collagen type I synthesis. For bone, this includes fragility, low bone mass, and progressive skeletal deformities, which can result in various degrees of short stature. The purpose of this study was to investigate development of bone mineral density in children with OI.

Patients And Methods: Development of lumbar bone mineral density was studied retrospectively in a cohort of 74 children with OI. Mean age was 16.3 years (SD 4.3). In 52 children, repeated measurements were available. Mean age at the start of measurement was 8.8 years (SD 4.1), and mean follow-up was 9 years (SD 2.7). A longitudinal data analysis was performed. In the total cohort (74 children), a cross-sectional analysis was performed with the latest-measured BMD. Age at the latest BMD measurement was almost equal for girls and boys: 17.4 and 17.7 years respectively.

Result: Mean annual increase in BMD in the 52 children was 0.038 g/cm(2)/year (SD 0.024). Annual increase in BMD was statistically significantly higher in girls, in both the unadjusted and adjusted analysis. In cross-sectional analysis, in the whole cohort the latest-measured lumbar BMD was significantly higher in girls, in the children with OI of type I, in walkers, and in those who were older, in both unadjusted and adjusted analysis.

Interpretation: During 9 years of follow-up, there appeared to be an increase in bone mineral density, which was most pronounced in girls. One possible explanation might be a later growth spurt and older age at peak bone mass in boys.
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http://dx.doi.org/10.3109/17453674.2013.831321DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3768047PMC
August 2013

Psychosocial predictors of parental participation in ultrasound screening for developmental dysplasia of the hip.

Fam Syst Health 2013 Jun 3;31(2):218-29. Epub 2013 Jun 3.

Department of Health Technology and Services Research, University of Twente, the Netherlands.

Ultrasound screening for developmental dysplasia of the hip (DDH) is an innovation in preventive child health care in the Netherlands. Parental participation in the screening will be essential for the success of implementation of the screening. The aim of the current study was to investigate whether psychosocial factors (attitude, subjective norm, self-efficacy, perceived susceptibility, perceived severity, perceived effectiveness) predicted parental participation in the screening. A cross-sectional survey was conducted. Using a questionnaire, several background variables (organization, sociodemographic variables, and knowledge) and psychosocial variables were collected. Blockwise logistic regression was used to analyze the relations. A total of 703 questionnaires of participating parents (response 61.7%) and 393 questionnaires of nonparticipating parents were received (response 37.2%). When controlling for the background variables, attitude, subjective norm, self-efficacy, perceived susceptibility, and perceived effectiveness predicted parental participation in the screening (p < .05). Perceived severity of the dysplasia did not predict participation (p > .05). Psychosocial determinants influenced parental participation in the ultrasound screening for DDH. Emphasizing the positive aspects of the screening, highlighting the effectiveness, removing practical barriers, and being conscious of the influential role of child health care professionals on decision making are areas to focus on when organizing the ultrasound screening for DDH. Health care policy decision makers and child health care professionals should consider these determinants in order to stimulate parental participation.
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http://dx.doi.org/10.1037/a0032393DOI Listing
June 2013

Determinants of parental satisfaction with ultrasound hip screening in child health care.

J Child Health Care 2012 Jun 22;16(2):178-89. Epub 2012 Feb 22.

University of Twente, The Netherlands.

Prior research has shown ultrasound (US) screening for developmental dysplasia of the hip (DDH) in preventive child health care to be more effective than the current screening method. In the present study, 3-month-old infants were screened for DDH with US. The objective of this study was to examine parental satisfaction with the screening and determinants that affect satisfaction. Parental satisfaction was measured using a questionnaire. Independent variables included socio-demographic determinants, structure, process and outcome-related determinants and the meeting of expectations. Satisfaction with the screening was high. Parents who perceived the screener as competent, had enough time to ask questions, perceived the proceeding as fluent, perceived a low burden on their infant and whose expectations were met, were more likely to be satisfied. Satisfaction was influenced by process-related factors and not by factors related to the structure and the outcome of the screening. Good information provision before the screening and communication during the screening are means by which parental satisfaction can be influenced positively.
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http://dx.doi.org/10.1177/1367493511426280DOI Listing
June 2012

Predicting participation in ultrasound hip screening from message framing.

Health Commun 2012 15;27(2):186-93. Epub 2011 Aug 15.

Department of Health Technology and Services Research, University of Twente, Netherlands.

The use of ultrasound (US) screening for developmental dysplasia of the hip (DDH) is an innovation in preventive child health care in the Netherlands. What is not known is whether parents will accept this screening method and will actually participate in it. It is widely known that health behaviors can be influenced by the framing of information. The objective of this study was to examine the influence of a gain- versus loss-framed brochure on parental participation in US screening for DDH. In total, 4150 parents of infants born between August 2007 and December 2008 received either a gain-framed or a loss-framed brochure. Parents could participate in the screening when their infant was 3 months old. The participation rate in the US screening was 74.3%. In contrast to the predictions of prospect theory, the results indicated that parents who had received the gain-framed message were more likely to participate in the screening compared to parents who had received the loss-framed message. This effect may be explained by the low risk perception of parents and by the possibility that the screening was perceived as a health-affirming behavior rather than an illness-detecting behavior. To increase participation rates, it is recommended that parents be informed about the positive aspects of partaking in screening for DDH.
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http://dx.doi.org/10.1080/10410236.2011.571760DOI Listing
May 2012

Orthopaedic management of Hurler's disease after hematopoietic stem cell transplantation: a systematic review.

J Inherit Metab Dis 2011 Jun 17;34(3):657-69. Epub 2011 Mar 17.

Department of Orthopaedics, University Medical Center Utrecht, HP G 05.228, Postbus 85500, 3508 GA Utrecht, The Netherlands.

Objective: The introduction of hematopoietic stem cell transplantation (HSCT) has significantly improved the life-span of Hurler patients (mucopolysaccharidosis type I-H, MPS I-H). Yet, the musculoskeletal manifestations seem largely unresponsive to HSCT. In order to facilitate evidence based management, the aim of the current study was to give a systematic overview of the orthopaedic complications and motor functioning of Hurler's patients after HSCT.

Methods: A systematic review was conducted of the medical literature published from January 1981 to June 2010. Two reviewers independently assessed all eligible citations, as identified from the Pubmed and Embase databases. A pre-developed data extraction form was used to systematically collect information on the prevalence of radiological and clinical signs, and on the orthopaedic treatments and outcomes.

Results: A total of 32 studies, including 399 patient reports were identified. The most frequent musculoskeletal abnormalities were odontoid hypoplasia (72%), thoracolumbar kyphosis (81%), genu valgum (70%), hip dysplasia (90%) and carpal tunnel syndrome (63%), which were often treated surgically during the first decade of life. The overall complication rate of surgical interventions was 13.5%. Motor functioning was further hampered due to reduced joint mobility, hand dexterity, motor development and longitudinal growth.

Conclusion: Stem cell transplantation does not halt the progression of a large range of disabling musculoskeletal abnormalities in Hurler's disease. Although prospective data on the quantification, progression and treatment of these deformities were very limited, early surgical intervention is often advocated. Prospective data collection will be mandatory to achieve better evidence on the effect of treatment strategies.
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http://dx.doi.org/10.1007/s10545-011-9304-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3109254PMC
June 2011

Transinguinal sonographic determination of the position of the femoral head after reposition and follow-up in a spica cast.

Pediatr Radiol 2010 Nov 16;40(11):1794-9. Epub 2010 Jun 16.

Department of Radiology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, E.01.231, Heidelberglaan 100, 3584 Utrecht, The Netherlands.

Background: Transinguinal sonography can be used to demonstrate the position of the femoral head after reduction of a dislocated hip.

Objective: To determine whether transinguinal sonography can replace radiography and CT in the follow-up after reduction of a hip dislocation?

Materials And Methods: Thirty-three children with 39 dislocated hips were followed up with sonography after reduction and immobilization in a spica cast. In cases of an abnormal position a CT scan was advised. A pelvic radiograph at the end of treatment served as an indicator that no dislocations were missed during the previous sonographic examinations.

Results: The repositioned hips were examined on 138 occasions. Twenty-four examinations were abnormal and CT scanning was performed on 11 occasions. In four children additional CT was done because a recurrent dislocation was suspected or because sonography was difficult to perform. No dislocations were demonstrated. In five children a recurrent dislocation was suspected, on one or more occasions. In all but one child a CT scan was performed that confirmed the dislocation.

Conclusion: Transinguinal sonography is well-suited to demonstrate a normal position of the femoral head in a spica cast. Transinguinal sonography decreases the number of radiographs and CT scans and reduces the exposure to ionizing radiation.
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http://dx.doi.org/10.1007/s00247-010-1726-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2950267PMC
November 2010

Implementation by simulation; strategies for ultrasound screening for hip dysplasia in the Netherlands.

BMC Health Serv Res 2010 Mar 23;10:75. Epub 2010 Mar 23.

Department Orthopaedics, University Medical Center Utrecht, Utrecht, Netherlands.

Background: Implementation of medical interventions may vary with organization and available capacity. The influence of this source of variability on the cost-effectiveness can be evaluated by computer simulation following a carefully designed experimental design. We used this approach as part of a national implementation study of ultrasonographic infant screening for developmental dysplasia of the hip (DDH).

Methods: First, workflow and performance of the current screening program (physical examination) was analyzed. Then, experimental variables, i.e., relevant entities in the workflow of screening, were defined with varying levels to describe alternative implementation models. To determine the relevant levels literature and interviews among professional stakeholders are used. Finally, cost-effectiveness ratios (inclusive of sensitivity analyses) for the range of implementation scenarios were calculated.

Results: The four experimental variables for implementation were: 1) location of the consultation, 2) integrated with regular consultation or not, 3) number of ultrasound machines and 4) discipline of the screener. With respective numbers of levels of 3,2,3,4 in total 72 possible scenarios were identified. In our model experimental variables related to the number of available ultrasound machines and the necessity of an extra consultation influenced the cost-effectiveness most.

Conclusions: Better information comes available for choosing optimised implementation strategies where organizational and capacity variables are important using the combination of simulation models and an experimental design. Information to determine the levels of experimental variables can be extracted from the literature or directly from experts.
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http://dx.doi.org/10.1186/1472-6963-10-75DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2851713PMC
March 2010

A tutorial on discrete-event simulation for health policy design and decision making: optimizing pediatric ultrasound screening for hip dysplasia as an illustration.

Health Policy 2009 Dec 11;93(2-3):143-50. Epub 2009 Aug 11.

University Medical Center Utrecht, Department of Orthopaedics, 3508 GA Utrecht, Netherlands.

Background: It is increasingly recognized that healthcare is a complex system with limited resources and many interacting sources of both positive and negative feedback. Discrete-event simulation (DES) is a tool that readily accommodates questions of capacity planning, throughput management and interacting resources. As a result the use of DES in informing healthcare decision making is increasing. However, understanding when and how to build a DES model and use it for policy making is not yet a common knowledge.

Methods: The steps in building a DES model will be demonstrated using a real-world example, i.e., pediatric ultrasound screening for hip dysplasia. The main components of a DES model such as entities, resources and queues will be introduced and we will examine questions such as referral schedule, number of ultrasound machines and type of screeners and how these entities interact. Finally a review of the statistical techniques appropriate to DES will be provided.

Conclusion: Discrete-event simulation is a valuable tool in the policymakers armentarium. It can be used effectively to analyze and understand complex healthcare systems and policy problems such as population screening.
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http://dx.doi.org/10.1016/j.healthpol.2009.07.007DOI Listing
December 2009

Quality of life in children with osteogenesis imperfecta treated with oral bisphosphonates (Olpadronate): a 2-year randomized placebo-controlled trial.

Eur J Pediatr 2007 Nov 17;166(11):1155-61. Epub 2007 Feb 17.

Department of Orthopaedic Surgery, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands.

Unlabelled: In this double-blind randomised placebo-controlled trial it was investigated during a two-year follow-up whether oral bisphosphonates (Olpadronate 10 mg/m2/day) influence quality of life in children with osteogenesis imperfecta (OI). Thirty-four children with OI (classified according to Sillence criteria), aged 3 to 18 years of age, with a restricted level of ambulation were included. Randomisation was performed using a list of computer generated random numbers to allocate patients to receive Olpadronate or placebo. Quality of life was measured using self-perception profile for children (SPPC) and health-utility index (HUI). Differences between baseline measurements and measurements at two years follow-up were analysed within the Olpadronate and placebo group using a student's t-test. Differences in HUI and SPPC regression coefficients were analysed by random-effects repeated measures analysis (SAS, Proc Mixed, version 8.2), adjusted for age, gender and type of OI. Within the Olpadronate group there was a significant decrease in pain utility; however, difference in six months' regression coefficients between the placebo and Olpadronate group were not significant. Within the placebo group there was a significant increase in scholastic competence and behavioural conduct. The item behavioural conduct showed a steeper annual regression coefficient favourable for the placebo group. In the other SPPC items none of the annual regression coefficients showed a significant difference between the Olpadronate and the placebo group.

Conclusion: We found only slight differences in quality of life in favour of the bisphosphonate group. A small but not significant decrease in pain was detected in the bisphosphonate group.
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http://dx.doi.org/10.1007/s00431-006-0399-2DOI Listing
November 2007

Cardiopulmonary exercise capacity, muscle strength, and physical activity in children and adolescents with achondroplasia.

J Pediatr 2007 Jan;150(1):26-30

Department of Pediatric Physical Therapy and Exercise Physiology and the Department of Pediatric Orthopedics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.

Objective: To study in children with achondroplasia the response to exercise and muscle strength compared with healthy peers and to describe the relation between exercise capacity, anthropometric factors, and physical activity.

Study Design: Patients (7 boys and 10 girls; mean age, 11.8 +/- 3.3 years) with achondroplasia performed a maximal treadmill exercise test. Anthropometric variables and muscle strength were measured and compared with the general population. The level of everyday physical activity was measured by using a diary. Functional ability was measured by using the Activity Scale for Kids.

Results: The exercise capacity of the children with achondroplasia was significantly reduced compared with reference values. All anthropometrical measurements differed significantly from reference values. There was a decrease in muscle strength in almost all muscle groups. We found a reduced physical activity level and impairments in functional ability.

Conclusions: Cardiopulmonary exercise capacity and muscle strength in children with achondroplasia was reduced compared with reference values. Children with achondroplasia have a unique response to exercise. Clinicians should take these characteristic differences into account when the exercise capacity of subjects with achondroplasia is being tested.
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http://dx.doi.org/10.1016/j.jpeds.2006.10.058DOI Listing
January 2007

Pediatric generalized joint hypomobility and musculoskeletal complaints: a new entity? Clinical, biochemical, and osseal characteristics.

Pediatrics 2004 Apr;113(4):714-9

Department of Pediatric Physical Therapy, University Medical Center, Wilhelmina Children's Hospital, Utrecht, The Netherlands.

Objective: To describe the clinical features, osseal characteristics, and collagen biochemistry in children who attended our clinic with predominantly generalized hypomobility of the joints, in combination with musculoskeletal complaints or abnormal walking, and no known syndrome or known rheumatic, neurologic, skeletal, metabolic, or connective tissue disorder was present.

Methods: Nineteen children who attended the Children's Hospital of the University Medical Center Utrecht for generalized hypomobility of the joints (mean age: 11.6; standard deviation: 2.7), in combination with musculoskeletal complaints or abnormal walking as primary complaints (symptomatic generalized hypomobility [SGH]), were compared with an age-matched reference group of 284 healthy children with normal mobility of the joints. Anthropometrics, range of joint motion, muscle strength, exercise tolerance, motor development, quantitative ultrasound measurements of bone, and degradation products of collagen in urine were studied. Collagen modifications were determined in skin biopsies of 3 children and in hypertrophic scar tissue of another child, all with SGH.

Results: The range of joint motion was significantly decreased in almost all joints of all 19 children and after adjustment for age, gender, body weight, and height, significantly lower than that of the reference group (-108.3 degrees; 95% confidence interval [CI]: -136.9 to -79.8). Quantitative ultrasound measurements as well as urinary pyridinoline cross-link levels were, after adjustment for possible confounders, significantly lower in SGH children (broad-band ultrasound attenuation: -9.6 dB/MHz [95% CI: -17.4 to -1.9]; speed of sound: -25.0 m/s [95% CI: -39.7 to -10.3]; hydroxylysylpyridinoline: -50.1 micromol/mmol [95% CI: -87.6 to -12.6], lysylpyridinoline: -21.3 micromol/mmol [95% CI: -34.0 to -8.6]). An increased amount of pyridinoline cross-links per collagen molecule was observed in skin and hypertrophic scar tissue, in combination with increased amounts of collagen.

Conclusion: SGH in children is considered a new clinical entity with specific clinical characteristics and might be related to an increased stiffness of connective tissue as a result of higher amounts of collagen with increased cross-linking.
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http://dx.doi.org/10.1542/peds.113.4.714DOI Listing
April 2004

Pediatric generalized joint hypermobility with and without musculoskeletal complaints: a localized or systemic disorder?

Pediatrics 2003 Mar;111(3):e248-54

Department of Pediatric Physical Therapy, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands.

Objectives: Children with generalized hypermobility of the joints and musculoskeletal complaints frequently visit pediatric clinics, but many show no currently known collagen or other possibly related diseases. Whether the symptoms are confined to the musculoskeletal system is unknown. We assessed whether such children have detectable differences in laxity of connective tissue present in organ systems other than joints. We also assessed whether children with generalized joint hypermobility and musculoskeletal complaints have more profound systemic changes in connective tissue of various organ systems as compared with children with generalized joint hypermobility without musculoskeletal complaints.

Methods: Anthropometrics, range of joint motion, muscle strength, skin extensibility, blood pressure, quantitative ultrasound measurements of bone, and degradation products of collagen were studied in 15 prepubertal children with generalized joint hypermobility and musculoskeletal complaints and compared with a population-based reference group of 95 nonsymptomatic prepubertal children. Symptomatic hypermobile children were also compared with children of the population-based reference group who had asymptomatic hypermobility of the joints (n = 16).

Results: Children with symptomatic generalized joint hypermobility had significantly higher skin extensibility (5.6 mm/15 kPa, 95% confidence interval [CI]: 4.0-7.1), lower quantitative ultrasound measurements (speed of sound: -26.8 m/s; 95% CI: -41.1 to -12.6) in bone, and lower systolic and diastolic blood pressure (-8.0 mmHg, 95% CI: -13.3 to -2.8; and -6.0 mmHg, 95% CI: -10.0 to -2.2, respectively) as compared with the total reference group. Also, they had significantly lower excretion of urinary hydroxylysylpyridinoline cross-links (mean difference: -51.3 micro mol/mmol; 95% CI: -92.2 to -10.4) as well as lysylpyridinoline cross-links (-18.7 micro mol/mmol; 95% CI: -36.9 to -0.5). Age, gender, body weight, height, and particularly cross-links excretion did not explain group differences in clinical and bone characteristics. After adjustment for age, gender, body weight, and height, children with symptomatic generalized joint hypermobility (n = 15) had significantly higher total range of joint motion (117.8 degrees; 95% CI: 77.7-158.0), skin extensibility (3.5 mm/15 kPa; 95% CI: 1.6-5.3), lower quantitative ultrasound measurements in bone (speed of sound: -27.9 m/s; 95% CI: -48.4 to -7.5), borderline lower diastolic blood pressure (-4.9 mmHg; 95% CI: -10.7-0.9), and significantly higher degradation products in urine (hydroxyproline/creatinine: 21.2 micro mol/mmol; 95% CI: 2.3-40.1) as compared with asymptomatic hypermobile children of the total reference group (n = 16). After adjustment for possible confounders, children with generalized joint hypermobility without musculoskeletal complaints had a significantly higher total range of joint motion and more profound skin extensibility, as compared with the reference group (n = 79).

Conclusions: Clinically manifested symptoms in otherwise healthy children with generalized joint hypermobility are accompanied by increases in the laxity of other body tissues. Thus, generalized joint hypermobility with musculoskeletal symptoms does not seem to be restricted to joint tissues. In symptomatic hypermobile children, a more systemic derangement was also present as compared with asymptomatic hypermobile children.
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http://dx.doi.org/10.1542/peds.111.3.e248DOI Listing
March 2003

A relation between blood pressure and stiffness of joints and skin.

Epidemiology 2003 Mar;14(2):223-7

Julius Center for Health Sciences and Primary Care, University Medical Center, Wilhelmina Children's Hospital, Ulrecht, The Netherlands.

Background: Blood pressure, particularly pulse pressure, is associated with arterial wall stiffness, but little is known about its relation to stiffness of other parts of the body. We examined the extent to which blood pressure levels in young healthy children are related to stiffness of various tissues.

Methods: In November 2000, we studied 95 healthy prepubertal children (41 boys and 54 girls, within age range 8-10 years) from two primary schools in the city of Zeist, The Netherlands. Systolic and diastolic blood pressure and pulse pressure were analyzed in relation to various tissue indicators of stiffness, including active joint mobility and skin extensibility. All results were adjusted for age, sex, body height, body weight and muscle strength as possible confounders.

Results: Diastolic blood pressure was lower with increased active joint mobility (multivariate generalized linear regression coefficient = -4.5 mmHg per standard deviation [SD] joint mobility; 95% confidence interval [CI] = -7.8 to -1.2). Pulse pressure was lower with increased skin extensibility (-3.2 mmHg per SD skin extensibility; CI = -5.2 to -1.1), through a higher diastolic blood pressure (2.0 mmHg per SD skin extensibility; CI = 0.2-3.9) and possibly lower systolic blood pressure (-0.8 mmHg per SD skin extensibility; CI = -3.5 to 1.9). These associations were mutually independent. Additional adjustment for reported musculoskeletal problems or physical activity levels did not materially change the findings.

Conclusions: Our findings support the hypothesis that constitutional stiffness of body tissues may be associated with blood pressure levels and eventually cardiovascular risk.
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http://dx.doi.org/10.1097/01.EDE.0000040327.31385.9BDOI Listing
March 2003

Ultrasound in the management of the position of the femoral head during treatment in a spica cast after reduction of hip dislocation in developmental dysplasia of the hip.

J Bone Joint Surg Br 2003 Jan;85(1):117-20

Department of Pediatric Orthopaedic Surgery, Wilhelmina Children's Hospital, Utrecht, The Netherlands.

In this prospective study of 18 hips we compared the efficacy of ultrasound with CT in determining the position of the femoral head in a spica cast after closed or open reduction in children with developmental dysplasia of the hip. Ultrasound was performed through the perineal opening of the cast. With a transinguinal approach, the superior ramus of the pubis, the acetabulum, the femoral head and the femoral neck can be depicted in one plane. The CT and ultrasound images were blinded and reviewed by two of the authors. Ultrasound was inconclusive in the first two reductions since the perineal opening was too small to see all the landmarks in one plane. In the following 16 reductions the landmarks were well defined and interpretation of the CT and ultrasound was similar. The perineal opening in the spica cast should be made in such a way that the ultrasound probe can be positioned in the groin so that the landmarks can be shown in one plane.
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http://dx.doi.org/10.1302/0301-620x.85b1.12665DOI Listing
January 2003

Overgrowth and correction of rotational deformity in 12 femoral shaft fractures in 3-6-year-old children treated with an external fixator.

Acta Orthop Scand 2002 Apr;73(2):170-4

Department of Orthopaedic Surgery, University Medical Center, Utrecht, The Netherlands.

We treated 11 young children (3-6 years old) who had uncomplicated femoral shaft fractures primarily with an external fixator. 9 children were available for follow-up and were evaluated for the amount of overgrowth and rotational deformity. All underwent a clinical examination and an MRI after mean 21 (13-25) months. The mean overgrowth was 0.4 (-0.3-1.1) cm and the anteversion angle showed a mean increase of 12 degrees, as compared to the contralateral femur. In 5 children with an anteversion angle difference of 10 degrees or more, a second MRI was done 4 years after the trauma. The mean anteversion angle difference of the femora in these 5 children had diminished from 15 degrees on the first MRI to 7.4 degrees on the second. 3 of the 5 children had a full correction of their rotational deformity. Growth did not correct the rotational deformity in the oldest child in this group.
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http://dx.doi.org/10.1080/000164702753671759 DOI Listing
April 2002