Effect of the Sharrard procedure on hip instability in children with Down syndrome: a retrospective study.

Purpose: The aim of this study was to retrospectively analyze the effect of the Sharrard procedure on hip instability in children with Down syndrome (DS), as measured by the migration index.

Methods: In total, 17 children (21 hips) were included from six hospitals in the Netherlands between 2003 and 2019. The primary outcome, hip instability, was assessed with the Reimers' migration index on preoperative and postoperative plain anteroposterior pelvic radiographs.

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

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.

Spinal cord transection before scoliosis correction in myelomeningocele may improve bladder function.

Aims: In patients with myelomeningocele (MMC) and coexistent scoliosis, a spinal cord transection (SC-transection) is sometimes performed before scoliosis correction to prevent traction on the myelum after stretching the spinal column. Performing a SC-transection may have positive effects on bladder function, especially in case of refractory detrusor-sphincter dyssynergia. This study investigates the effects of SC-transection on lower and upper urinary tract outcomes.

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

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.

Identification of PLOD2 as telopeptide lysyl hydroxylase, an important enzyme in fibrosis.

The hallmark of fibrotic processes is an excessive accumulation of collagen. The deposited collagen shows an increase in pyridinoline cross-links, which are derived from hydroxylated lysine residues within the telopeptides. This change in cross-linking is related to irreversible accumulation of collagen in fibrotic tissues.

Scoliosis in children with osteogenesis imperfecta: influence of severity of disease and age of reaching motor milestones.

We studied the relationship between the age of reaching motor milestones, especially anti-gravity activities, and the age of development of pathological spinal curvatures in children with osteogenesis imperfecta (OI). We hypothesized that earlier achievement of anti-gravity motor milestones predicts a later development of pathological spinal curvatures. Ninety-six children participated in this retrospective study.

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.

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.