Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Of 41 patients with Duchenne muscular dystrophy, no ambulatory patient had scoliosis greater than 19 degrees. Non-ambulatory patients were prophylactically placed in body jackets, which kept the spine flexible and provided adequate support for sitting in the majority of patients. Ten patients had posterior spine fusion for progressive spinal collapse. The procedure was extensive with significant blood loss but boney fusion was achieved in every case. Pulmonary complications were minimized by performing preoperative tracheostomy on all patients who had vital capacities less than 40% and or non-functional coughs. Spinal fusion permitted long-term sitting stability despite the progression of the disease.
 ...
PMID:Stabilization of the collapsing spine in duchenne muscular dystrophy. 34 97

Discitis was diagnosed in 5 children under 3 years of age, the initial clinical manifestations being difficulty in walking and abdominal pains in one case. Diagnosis was not made before periods varying from 8 days to 3 months, and no etiological basis for the disease was discovered. Pinching of the disc was always present in the first radiographic image, and the vertebral plate was ill-defined in 3 cases. Repeat radiological examinations were carried out in 3 children after 6 months, 2 and 5 years respectively. There was partial restauration of the disc space in 2 cases; the last one presented signs of late collapse after early recuperation. Early perilesional bone sclerosis was noted in 2 cases, while it was posterior and late in one child. There were no sequelae (fusion, vertebra plana, scoliosis). Two investigations are essential if a disc lesion is suspected: -- radiography of the spinal column, even if there are no disturbances in walking or abdominal pains. -- scintigraphy with technitium 99, which is the only means of establishing an early diagnosis.
 ...
PMID:[Discitis in small children (author's transl)]. 52 26

Calssical cervical syringomyelia was found in 3 members of one family. All 3 underwent air myelogram, and a Chiari malformation type I and postural collapse of the spinal cord was found in each case. An affected 7-year-old boy was discovered after a clinical and radiological survey of 8 first-degree relatives on the basis of mild scoliosis, pyramidal tract signs in the lower limbs and enlarged sagittal diameter of the cervical canal. One other member had basilar impression of the skull but no neurological abnormalities. No positive correlation was found between either the size of the cystic cord enlargement or descent of the ectopic tonsils with the duration or severity of the neurological findings. Suboccipital decompressive craniotomy and upper cervical laminectomy in one case was followed by improvement in strength and sensation 1 year later. Progression in familial syringomyelia appears to occur through a mechanism identical to that in the sporadic form and surgery is therefore also indicated. In affected families, routine survey of close relatives for abnormal neurological signs, and radiological evidence of scoliotic deformity of the spine, enlarged cervical canal and bone abnormalities at the craniovertebral junction may prove valuable for early detection. A dominantly inherited, genetically determined malformation seems to be the probable mechanism of inheritance in this family.
 ...
PMID:Familial communicating syringomyelia. 65 Feb 52

Idiopathic scoliosis is a complex three-dimensional deformity and in the thoracic region the essential lesion lies in the sagittal plane in the form of an area of inappropriate lordosis. The thoracic kyphosis is normally protected from buckling by being behind the axis of spinal column rotation but when the thoracic lordosis develops it brings the apical region anterior to this axis and thus under compression with resultant buckling failure of the spinal column. The condition of idiopathic thoracic scoliosis is the opposite to idiopathic hyperkyphosis (Scheuermann's disease), the latter being rotationally stable and not moving out of the sagittal plane. The two frequently co-exist in the same spine with thoracic hyperkyphosis above an area of lumbar lordo-scoliosis. There is a spectrum of normal lateral profile and flat backs at the one end are in danger of buckling (lordo-scoliosis) while round backs at the other end of the spectrum are in danger of being defined as Scheuermann's disease. There is no requirement for a specific pathological process. Engineers describe only two ways in which a flexible column can fall into mechanically-angular collapse (kyphosis) and column buckling (lordo-scoliosis). A number of factors favour column buckling (Euler's law) and thus the bigger a deformity the more likely it will be to continue progressing and the taller and more slender the column the more likely it will be to fail and this we see in our patients with idiopathic scoliosis. Not only is lordosis the essential lesion but it is also the primary abnormality which can be demonstrated in children before lateral curvature and rotation develop.
 ...
PMID:The etiology and pathogenesis of idiopathic scoliosis. 145 9

Transiliac limb lengthening osteotomy is a modified Salter innominate osteotomy which uses a trapezoidal interposition bone graft instead of the usual triangular graft to achieve up to 3 cm of intrapelvic lengthening. It allows correction of certain forms of postural imbalance and pelvic obliquity, as well as allowing an optimal and variable amount of acetabular redirection. This review of 105 patients who have undergone the procedure at our institution revealed the following indications: decompensated scoliosis, 26 patients; acetabular dysplasia with ipsilateral femoral shortening, 34 patients; intrapelvic asymmetry, 7 patients; pure limb length inequality, 38 patients. Intrapelvic lengthening averaged 2.5 cm. Correction of decompensated scoliotic curves averaged 7 degrees reduction in Cobb angle. Improvement in center-edge angle in patients with hip dysplasia averaged 17 degrees. Reduction in size of shoe lift required to balance the trunk in all cases was correlated closely with the intrapelvic lengthening achieved. Complications included two transient neurapraxias (one femoral, one femoral and sciatic), two cases of sacroiliac subluxation (one patient with myelodysplasia, and one patient with polio), three cases of partial graft collapse, and six instances of broken fixation pins, and three deep wound infections. No patient had chondrolysis or avascular necrosis of the femoral head. After average follow-up of nearly 5 years (minimum follow-up 2 years), patients who underwent transiliac limb lengthening osteotomy for correction of postural imbalance for various causes retained satisfactory correction.
 ...
PMID:[Transiliac leg lengthening: experiences with a modified Salter osteotomy]. 223 60

We reviewed the clinical charts and spinal radiographs of fifty-one boys who had Duchenne muscular dystrophy, had not had surgical treatment of the spine, and had been followed until death. All had scoliosis. None of the following variables was useful in predicting which curves would become severe: age when the patient initially walked, age when he ceased walking, age at onset of spinal collapse, surgical release of the iliotibial bands, or age at the time of death. Radiographs were made within eighteen months before death for thirty-three patients; in thirty-one of them, the final curve exceeded 40 degrees and in seventeen, 90 degrees. For the remaining eighteen patients, final radiographs were made more than eighteen months before death; at that time, eight of them already had a curve of more than 90 degrees. Although there was a relationship between extension of the lumbar spine and severity of scoliosis at the time of final follow-up, early maintenance of the lumbar spine in extension rarely prevented the development of a severe curve. For most of the patients who had a severe curve, sitting was difficult and was accompanied by breakdown of the skin and pain. When a patient's curve exceeded 35 degrees, the vital capacity usually was less than 40 per cent of the predicted normal value. Therefore, when walking becomes impossible for boys who have Duchenne muscular dystrophy, routine spinal arthrodesis should be considered.
 ...
PMID:Progression of scoliosis in Duchenne muscular dystrophy. 276 82

Anaesthetic complications such as obstruction of airways by submucosal cartilage-bone protuberances, immobility of the neck or instability of the atlanto-axial joint have been described earlier in paediatric patients with congenital osteochondral disorders. This report concerns a case in which tracheal collapse due to tracheobronchial malacia in an adult patient with metaphyseal chondrodysplasia evidently caused severe ventilatory difficulties in the induction of anaesthesia. The management of the patient on three subsequent occasions is described. During the first operation, support of the upper respiratory tract was performed. For this procedure, awake tracheal intubation with local anaesthesia applied to the larynx, vocal cords and trachea was used. After surgical correction of the bronchus of the right upper lobe and the stem bronchus, subsequent anaesthesias for surgical treatment of scoliosis could be conducted safely. The possibility of co-existing tracheobronchial malacia in patients with osteochondrodysplasias should be considered and tracheal intubation under local anaesthesia is recommended.
 ...
PMID:Tracheobronchomalacia in an adult with metaphyseal chondrodysplasia. 381 48

In an attempt to find prognostic factors for mild scoliosis, the first clinical and radiologic data of 159 subjects with idiopathic scoliosis less than 30 degrees were correlated with the annual speed of progression estimated graphically by further examinations. Thoracic and double major are more progressive than thoracolumbar and lumbar. The correlation with rate of progression is better for supine angle than for standing angle. Curves with low supine angles are those that have great relative collapse and low risk of progression. Only the rib hump of thoracic and thoracolumbar are correlated with evolutivity. Predictive equations give less approximation for individual prognosis than a previous graphic method, so they are not used. Approximately 73% of scoliosis have evolutivities less than 6 degrees per year and required nocturn corrective treatment.
 ...
PMID:Scoliosis at less than 30 degrees. Properties of the evolutivity (risk of progression). 404 8

This work is a critical analysis of the term "flexibility of scoliosis." The authors demonstrate that this measure includes two very different and noncorrelated phenomena: the collapse and the reducibility of the structural curve. In 228 paralytic curves, these two phenomena were quantified and correlated with different parameters of scoliosis: sex, stage of maturation, curve pattern, number of vertebrae in the curve, spinal and abdominal test values, and the scoliotic evolution rate. Reducibility can be predicted since it is an elasticity process. Collapse is more complex, but it is a prognostic factor for evolution and effectiveness of treatment. The preliminary results of a similar study of idiopathic scoliosis are the same.
 ...
PMID:Flexibility of scoliosis. What does it mean? Is this terminology appropriate? 404 10

Using computerized spinal analysis, a new top view was developed that displays the spine as if the observer were above and looking down on the patient. Serial top views were obtained of 12 patients with idiopathic scoliosis. In five patients with clinically stable curves, the top views showed no change. One patient with an enlarging rib hump was seen on the top view to have progressive kyphosis but stable scoliosis. Six patients with progressive scoliosis all demonstrated collapse of the thoracic curve in the anteroposterior direction. Five of these six patients had associated lumbar curves. Three lumbar curves demonstrated collapse in the anteroposterior direction similar to the collapse of the thoracic curves, and the other two curves appeared elongated in the anteroposterior direction.
 ...
PMID:The top view for analysis of scoliosis progression. 634 Jan 56


1 2 3 Next >>