[Treatment of cartilaginous defects in the knee: recommendations from the Dutch Orthopaedic Association].

Cartilaginous defects in the knee occur frequently and can cause the patient considerable limitations. They are diagnosed and classified by means of MRI and arthroscopy. The surgical options available to treat deep chondral lesions include bone marrow stimulation techniques (microfracture treatment), chondrocyte therapies (autologous chondrocyte implantation) and tissue replacement therapies (osteochondral autologous transplantation).

SUSHI: the Super Simple Hip score for younger patients.

We describe the development of a simple patient-based score for young patients with hip problems which concentrates on activities that are difficult for someone with a hip problem and includes an activity rating scale that measures the highest level of physical activity reached during the past year. We compared the super simple hip score (SUSHI) with the more extensive hip osteoarthritis outcome score (HOOS) and evaluated the validity, sensitivity to change and floor and ceiling effects of the SUSHI score. We found that the SUSHI score is an adequate score to measure hip problems and that this score was preferred to the HOOS score by patients.

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

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.

Postural instability in Charcot-Marie-Tooth type 1A patients is strongly associated with reduced somatosensation.

In order to determine the influence of somatosensory impairments, due to the loss of large myelinated fibres, on the postural stability of Charcot-Marie-Tooth 1A (CMT) patients, a cross-sectional balance assessment was done. Nine CMT patients were compared with eight patients with a distal type of Spinal Muscular Atrophy (SMA), and 11 healthy control subjects. The balance assessment consisted of four tasks: quiet barefoot standing on a stable versus compliant surface, with eyes opened or closed.

Fast responses to stepping on an unexpected surface height depend on intact large-diameter nerve fibers: a study on Charcot-Marie-Tooth type 1A disease.

The contribution of reflexes from the large myelinated afferents in the control of normal and perturbed gait in humans is a highly debated issue. One way to investigate this topic is by studying normal and perturbed gait in patients lacking large myelinated fibers in the distal limb (Charcot-Marie-Tooth [CMT] type 1A disease). Such patients should have delayed and decreased reflexes if the latter depend on these large myelinated fibers.

Hitting a support surface at unexpected height during walking induces loading transients.

The impact phase during walking is sometimes characterised by an early loading peak, termed 'transient', followed by a brief decline in the force profile, termed 'unloading phase'. It was hypothesized that transients occur more frequently when subjects are unaware of the landing condition, and that the unloading phase represents a yield of the leg. This was tested experimentally by introducing an unexpectedly lowered or level support surface height during walking.

Muscle reflexes and synergies triggered by an unexpected support surface height during walking.

An important phase in the step cycle is foot contact. When the moment of foot contact differs from the one expected, a fast response is needed. Such a mismatch can be caused by hitting a support surface earlier or later than expected.

Ambulatory disabilities and the use of walking aids in patients with hereditary motor and sensory neuropathy type I (HMSN I).

Purpose: To determine the level of ambulatory disability and the use of walking aids in well-ambulant Hereditary Motor and Sensory Neuropathy type I (HMSN I) patients, and to identify the related demographic, physical and psychological variables.

Methods: Seventy-five well-ambulant HMSN I patients, aged 20-58 years, were measured in a cross-sectional assessment, addressing disability of ambulation and mobility (Sickness Impact Profile), demographics, muscle strength (Medical Research Council), use of walking aids, physical activity (actometer), fatigue (Checklist Individual Strength), and quality of life (EuroQoL).

Results: Seventy-two percent of the patients perceived a significant amount of ambulatory disability.