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Biomedical subjects

R H Engelbert

Publications and source records attributed to R H Engelbert.

16 recordsLinked to original sources
PubMed2001

Paediatric rehabilitation.

More and more researchers are questioning the theoretical and scientific foundations as well as the efficacy and effects of many physiotherapy interventions. The same applies for many of the neurophysiological based interventions that are being used in paediatric rehabilitation. Opinions and views regarding the development of motor behaviour of infants and children are significantly changing. Paediatric interventionists should consider bringing their interventions and focus of treatment into agreement with changed scientific knowledge. Moreover, for almost all other medical problems in childhood, paediatric rehabilitation has little to offer but mostly miniaturized forms of adult treatment. It not only means that we have to make a paradigm shift, but also are in the need of a broader view on paediatric rehabilitation as a specialized professional activity.

Child↗
PubMed2001

Osteogenesis imperfecta in childhood: perceived competence in relation to impairment and disability.

OBJECTIVES: To examine the perceived competence of children with different types of osteogenesis imperfecta (OI) and to investigate the possible relationships between their perceived competence and impairment parameters. DESIGN: Cross-sectional study. SETTING: National referral center (hospital) for the treatment of children with OI. PATIENTS: Forty children with OI (type I = 17; type III = 11; type IV = 12) with a mean age +/- standard deviation of 12.6 +/- 3.2 years. INTERVENTIONS: Measured joint range of motion (ROM) in the upper extremities (UEs), and lower extremities (LEs), muscle strength, functional skills, ambulation, and perceived competence. MAIN OUTCOME MEASURES: Joint ROM in UE and LE; muscle strength (using the manual muscle testing criteria of the Medical Research Council); functional skills using the Pediatric Evaluation of Disability Inventory in 3 domains (self-care, mobility, social function). Ambulation (according to Bleck and classified as nonwalking, therapy walking, household walking, neighborhood walking, community walking with or without the use of crutches), and perceived competence (using the Harter Self-Perception Profile for Children, which was cross-culturally validated for Dutch children). RESULTS: In children with type I, joint ROM and muscle strength were almost comparable to the healthy population. In children with type III, a severe decrease in joint ROM was measured, especially in the LEs, and muscle strength was severely decreased in the UEs and LEs. In children with type IV, joint ROM and muscle strength decreased, especially in the LEs. In all types, fairly to strongly positive perceived competence was measured except for fairly negative perceived competence in the athletic performance subscale in type I and a fairly negative perceived competence in the romance subscale in type III. No correlations were found between (1) joint ROM and athletic performance and physical appearance, (2) muscle strength and athletic performance or physical appearance, or (3) the functional skills, concerning self-care and mobility, with the subscales of the perceived competence. CONCLUSIONS: Although joint ROM, muscle strength, and functional and walking ability were related to the severity of the disease and differed significantly between the different types of OI, overall perceived competence in children with OI was fairly to strongly positive, without significant differences between the different types of OI.

Adolescent↗
PubMed2000

Osteogenesis imperfecta: profiles of motor development as assessed by a postal questionnaire.

UNLABELLED: This study was performed to achieve more detailed information regarding the age and sequence in the development of motor milestones in the different types of osteogenesis imperfecta (OI). The parents of 98 patients with a diagnosis of OI were sent a questionnaire regarding the age at which patients achieved motor milestones. All patients were attending the outpatient clinic for children with OI at the Wilhelmina Children's Hospital. The motor milestones were classified into static motor milestones and dynamic motor milestones and all data were checked with health care records. The age of development of motor milestones was compared to reference values of the healthy population. The severity of the disease was classified according to Sillence based on clinical, genetic and radiological data. The age of intramedullary rodding of the first nail in the lower and upper extremity and the localisation was noted. A total of 76 parents responded to the 98 questionnaires (78%). In OI type I, a delay exists in achieving motor milestones, comparable to the 95th percentile of the normal population. In type III, the development of all motor milestones was significantly delayed compared to types I and IV with a discrepancy between static and dynamic milestones. In OI type IV, a retardation in motor development developed after the milestone 'sitting without support' was achieved. Motor development in types I and IV was not influenced by intramedullary rodding of the lower extremities, since rodding was rarely performed before the milestone 'unsupported standing' was achieved. In type III, the influence of intramedullary rodding on the age of achieving motor milestones remains questionable. CONCLUSION: The severity of osteogenesis imperfecta has a large influence on the age and sequence in the development of motor milestones. No influence of intramedullary rodding of the lower extremities on motor development was found in osteogenesis imperfecta types I and IV, whereas the influence in type III remains questionable.

Activities of Daily Living↗
PubMed2000

Operative treatment of severe scoliosis in osteogenesis imperfecta: results of 20 patients after halo traction and posterior spondylodesis with instrumentation.

Correction and stabilisation of the scoliotic spine in osteogenesis imperfecta is difficult. The optimal technique has yet to be determined, since no large series in which a single procedure has been carried out by a single surgeon using a single protocol has yet been described. The charts of 20 patients with osteogenesis imperfecta who had undergone halo gravity traction (HGT) and a posterior spondylodesis with Cotrel-Dubousset (n = 18) or Harrington (n = 2) instrumentation were reviewed. No correction was made at the time of the surgical spondylodesis. The average follow-up was 4.8 years (range 2-10.5 years). The preoperative traction improved the Cobb angle of the scoliosis by 32% (from a mean of 78.5 degrees to a mean of 53.3 degrees) and improved the kyphosis by 24% (from a mean of 56.0 degrees to mean of 42.5 degrees). This correction deteriorated slightly at final follow-up, for both the scoliosis and the kyphosis (mean 57.6 degrees and 44.4 degrees respectively). Few complications were encountered during the HGT period. In 16 cases no complications occurred during the follow-up period. Ambulation and functional ability were upgraded for 7 of 20 patients.

Adolescent↗
PubMed2000

Osteogenesis imperfecta in childhood: prognosis for walking.

OBJECTIVES: We studied the predicted value of disease-related characteristics for the ability of children with osteogenesis imperfecta (OI) to walk. STUDY DESIGN: The severity of OI was classified according to Sillence. The parents were asked to report the age at which the child achieved motor milestones, the fracture incidence, and the age and localization of the first surgical intervention. The present main means of mobility was classified according to Bleck. RESULTS: There were 76 replies to the 98 questionnaires, of which 70 were included (type I, 41; type III, 11; type IV, 18). The type of OI was strongly associated with current walking ability, as was the presence of dentinogenesis imperfecta. Patients with type III and IV had a lower chance of ultimately walking compared with those with type I. Children with more than 2 intramedullary rods in the lower extremities had a reduced chance of walking than patients without rods. Rolling over before 8 months, unsupported sitting before 9 months, the ability to get in sitting position without support before 12 months, and the ability to get in a standing position without support before 12 months showed positive odds ratios. In Bleck > or = 4, multivariate analysis revealed that only the presence of rodding (yes/no) in the lower extremities had additional predictive value to the type of OI. The presence of dentinogenesis imperfecta and rodding (yes/no) had additional value in Bleck > or = 5. CONCLUSION: The type of OI is the single most important clinical indicator of the ultimate ability to walk. Information about motor development adds little. The early achievement of motor milestones contributes to the ability of independent walking when the type of OI is uncertain. Intramedullary rodding of the lower extremities is primarily related to the severity of the disease and in this way provides consequences for the ability to walk.

Adolescent↗
PubMed1999

Osteogenesis imperfecta in childhood: impairment and disability--a follow-up study.

OBJECTIVES: To evaluate differences over time (mean follow-up, 14 months) on impairment parameters (range of joint motion and muscle strength), functional limitation parameters (functional ability), and disability parameters (caregiver assistance in achieving functional skills) in osteogenesis imperfecta (OI), related to the different types of the disease. DESIGN: A prospective, descriptive study. MATERIALS AND METHODS: Fifty-four children with OI and their parents participated at the start of the study. At the end, 44 children participated in the assessment of functional skills and 42 of them participated in clinical assessment (OI type I, n = 19; OI type III, n = 13; OI type IV, n = 10). Range of joint motion was measured by means of goniometry. Generalized hypermobility was scored according to Bulbena. Manual muscle strength was scored by means of the MRC grading system. The level of ambulation was scored according to Bleck, and functional skills and caregiver assistance were scored with the Pediatric Evaluation of Disability Inventory. RESULTS: The different types of OI have impact on impairment, functional limitation, and disability. Almost all impairment parameters did not change significantly over time, whereas some disability parameters seemed to improve significantly. CONCLUSIONS: Impairment parameters in OI are presumably not always preconditions for functional limitation and disability. A 1-year follow-up revealed no significant changes in impairment parameters, whereas some disability parameters improved. Treatment strategies in OI should, therefore, focus primarily on improving functional ability, with respect to the natural course of the disease, and not only on impairment parameters.

Child↗
PubMed1999

Influence of infant-walkers on motor development: mimicking spastic diplegia?

We discuss two patients, who used an infant walker during the period in which they learned to walk. The influence on qualitative and quantitative motor development is illustrated in this report. A disharmonic and delayed motor development, contractures of the calf-muscles and motor development mimicking spastic diplegia are considered to be caused by the early use of infant walkers. As illustrated by the literature, infant walkers do not have any positive effect on improving motor development. Vulnerability of infants with regards to accidents is increased. In our opinion, the use of infant-walkers should be discouraged.

Cerebral Palsy↗
PubMed1999

Complications of the Bailey-Dubow elongating nail in osteogenesis imperfecta: 34 children with 110 nails.

The Bailey-Dubow nail, inserted in the femur or tibia of 34 children with osteogenesis imperfecta (OI), was studied retrospectively. Comparing the various groups of OI, no significant difference was found. Location of the nail (tibia or femur) did not influence the complication rate significantly. The reoperation rate was 29%, a rate comparable to that reported in earlier studies. The part of the nail located around the knee had a significantly higher migration rate (P = 0.005 at obturator ends and P = 0.007 at sleeve ends). Migration of the nail was the reason to reoperate in 50% of the patients. Better anchoring of the T-piece will substantially decrease the complication rate. In consideration of the different functional capacities of the OI population, the complications are likely related more to the hardware than to the patient.

Adolescent↗
PubMed1999

Temporary muscle weakness in the early phase of distraction during femoral lengthening. Clinical and electromyographical observations.

Limb lengthening by distraction osteogenesis has a high complication rate. Much of the response of muscle and nerve to distraction is still unknown. Thirteen children, mean age 12.6 yr (8.4-17.3) were surgically treated by the Ilizarov procedure for acquired and congenital femoral limb-length discrepancy. All children showed a decrease in muscle strength in the quadriceps, shortly after the operation, followed by an improvement before distraction started. After an elongation in the early phase of distraction (1 to 2 cm), muscle weakness was again observed and the muscle strength gradually increased after ending of distraction. To provide an explanation for this clinical observation, in one patient (limb lengthening of 4.1 cm) muscle strength measurements were extended with investigations of Hoffman (H) reflex of m. vastus medialis and determination of muscle-fiber conduction velocity of m. vastus lateralis by using the invasive method (IMFCV). The examinations were performed every two weeks during 20 weeks and 12 weeks after removing the cast. A severe decrease in muscle strength of the corrected limb was found after 1.2 cm of distraction with a recovery in muscle strength before lengthening was ended. EMG study showed the same tendency. Denervation was observed as evidence by positive sharp waves and reduced IMFCV findings. Evidence for reinnervation before lengthening was ended, was found by an increased range of velocities consisting of a combination of slow potentials and gradual increase of the velocity of reinnervated fibers (increased Fast/Slow ratio). The latencies of M waves and H-M interval from both legs separated as well after 2.25 cm of distraction. At the end of the follow-up period, the H-M interval reached the preoperative value. It is suggested that these neurogenic changes are an effect of axonal dysfunction and the local effect due to intraoperative trauma and stretching might affect nerve blood flow adversely.

Adolescent↗
PubMed1998

Osteogenesis imperfecta in childhood: treatment strategies.

Osteogenesis imperfecta (OI) is a skeletal disorder of remarkable clinical variability characterized by bone fragility, osteopenia, variable degrees of short stature, and progressive skeletal deformities. Additional clinical manifestations such as blue sclerae, dentinogenesis imperfecta, joint laxity, and maturity onset deafness are described in the literature. OI occurs in about 1 in 20,000 births and is caused by quantitative and qualitative defects in the synthesis of collagen I. Depending on the severity of the disease, a large impact on motor development, range of joint motion, muscle strength, and functional ability may occur. Treatment strategies should primarily focus on the improvement of functional ability and the adoption of compensatory strategies, rather than merely improving range of joint motion and muscle strength. Surgical treatment of the extremities may be indicated to stabilize the long bones to optimize functional ability and walking capacity. Surgical treatment of the spine may be indicated in patients with progressive spinal deformity and in those with symptomatic basilar impression.

Activities of Daily Living↗
PubMed1998

Spinal complications in osteogenesis imperfecta: 47 patients 1-16 years of age.

We examined in a cross-sectional study, 47 children (mean age 7.7 (1-16) years) with osteogenesis imperfecta (OI) to find the prevalence of spinal deformities and to correlate these observations with anthropometry. The associations between dentinogenesis imperfecta, joint hypermobility and spinal deformities were also studied. Disproportion in stature in OI type I and type IV was mainly caused by spinal involvement, as evidenced by a greater decrease in body height than in leg length. In OI type I, the decrease in sitting height was mainly caused by platyspondyly, whereas in OI types III and IV, it was also caused by progressive scoliosis and kyphosis. Scoliosis was present in 22 children, and pathological kyphosis in 18, mainly in the severe OI types. Basilar impression was observed in 10 children, mainly in type III. Children with dentinogenesis imperfecta seemed to be prone to develop scoliosis, pathological kyphosis and basilar impression. Children with generalized joint hypermobility were less prone to develop scoliosis and basilar impression. Our observations may contribute to a better understanding of the risk factors for progressive spinal deformities in OI.

Adolescent↗
PubMed1998

Bruck syndrome: a rare combination of bone fragility and multiple congenital joint contractures.

Bruck syndrome manifests with combined features of arthrogryposis and osteogenesis imperfecta. It is a distinct autosomal recessive disorder with normal collagen I. The main features are osteoporosis, bowing of the long bones, scoliosis due to vertebral deformities, and congenital joint contractures. The presence of arthrogryposis differentiates this syndrome from "classical" osteogenesis imperfecta. A family with three affected children is presented with a review of the literature.

Arthrogryposis↗
PubMed1997

Osteogenesis imperfecta in childhood: impairment and disability.

OBJECTIVE: To determine clinical characteristics in children with osteogenesis imperfecta (OI) regarding impairment (range of joint motion and muscle strength) and disability (functional skills) in relation to the different types of the disease, and to study the correlation between characteristics of impairment and disability. METHODS: In a cross-sectional study 54 children with OI (OI type I: 24; OI type III: 15; OI type IV: 15), the range of joint motion, muscle strength, and functional ability were measured in a standardized way and analyzed statistically. RESULTS: The range of joint motion in almost all joints differed significantly with respect to the different disease types. In OI type I patients, generalized hypermobility of the joints was present, without decrease in joint motion. In OI type III the extremities were severely maligned, especially the lower limbs. In type IV the upper and lower extremities were equally maligned. Muscle strength differed significantly with respect to the different types of OI. In type I patients, muscle strength was normal except for the periarticular hip muscles. In type III, especially in the lower extremities, muscle strength was severely decreased, with a muscular imbalance around the hip joint. In type IV, muscle strength was mainly decreased in the proximal muscles of the upper and lower extremities. In children </=7.5 years of age, significant differences existed among the different disease types in functional skills regarding mobility. No significant difference was observed in self-care and social function, although the most severely affected children showed a tendency to score better with social function. Older children differed significantly concerning mobility and self-care items. In children </=7.5 years old, a correlation was sometimes observed between impairment and disability items, although in older children a moderate to good correlation was always present (r > .6). CONCLUSION: In OI, severity-related profiles exist, within the different subtypes of the disease, regarding range of joint motion, muscle strength, and functional skills. In younger children, impairment parameters do not sufficiently correlate for disability. Rehabilitation strategies in younger children should therefore focus on improvement of functional skills and not only on impairment parameters.

Activities of Daily Living↗
PubMed1995

Intramedullary rodding in type III osteogenesis imperfecta. Effects on neuromotor development in 10 children.

We studied retrospectively gross motor development and the impact of intramedullary rodding in 10 children with type III osteogenesis imperfecta (OI). There was a pronounced delay in motor development and the order in achieving gross motor milestones differed from the normal developmental sequence. Static milestones developed at an earlier stage than dynamic milestones. Intramedullary rodding of the lower extremities prior to the age of 3.5 years enhanced neuromotor development, especially regarding the milestones supported standing, rolling from prone to supine and crawling with abdomen on the floor. The different sequence in achieving gross motor milestones should have implications for future rehabilitation programs and for orthopedic surgery.

Body Height↗
PubMed1993

[2 children with discitis].

The childhood form of discitis was diagnosed in a 2-year-old girl and a 5-year-old boy. They presented with an antalgic posture, muscular defense and a positive Gowers sign. Characteristic symptoms of this childhood discitis form the triad: unexplained fever, increased erythrocyte sedimentation rate and symptomatic narrowing of an intervertebral space.

Braces↗