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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-one patients with the diagnosis of mucopolysaccharidosis or mucolipidosis and a history of respiratory complaints or thorough respiratory evaluation were studied retrospectively. Anatomic factors affecting respiratory status included: (i) upper airway narrowing by hypertrophied tongue, tonsils, adenoids, and mucous membranes; (ii) lower airway narrowing by glycosaminoglycan deposition within the tracheobronchial mucosa; (iii) decreased thoracic dimensions due to scoliosis and thoracic hyperkyphosis; and (iv) decreased abdominal dimensions due to lumbar hyperlordosis, gibbus formation and hepatosplenomegaly. Cardiac and neurologic involvement, while present, did not play primary roles in the development of respiratory disease. The functional consequences of these findings included increased risk of developing: (i) respiratory tract infections; (ii) airway compromise during or after anesthesia or sedation; (iii) dyspnea on exertion; (iv) obstructive lung disease; (v) obstructive sleep apnea; and (vi) cor pulmonale. A management approach is presented which can reduce the morbidity and mortality experienced by these patients.
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PMID:Respiratory complications of mucopolysaccharide storage disorders. 313 89

Adolescent idiopathic scoliosis is the single most common form of spinal deformity seen in orthopedic practice. Our knowledge about the epidemiology, etiology, natural history, and treatment has recently increased dramatically. The incidence of small curves is rather high (2% of the population), whereas severe curves are much less common (<0.1%), but we cannot always predict which curve will progress. Abnormalities of the neuromuscular system and of calcium metabolism, and certain growth, genetic, and mechanical factors may all play roles in the pathogenesis of the disorder. The physiologic secondary effects of severe scoliosis relate to restrictive lung disease, but most patients do not have a deformity great enough to affect their cardiorespiratory function. The psychological and social effects of scoliosis are significant for patients but difficult to quantitate. For most patients with moderate scoliosis-that is, more than 25 to 30 degrees-treatment with an underarm brace or electrical stimulation is adequate to "control" progression of the curve. Surgical fusion allows actual correction of the curve but is indicated in only a small percentage of patients-usually those with more than 50 degrees of deformity.
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PMID:Adolescent idiopathic scoliosis. 327 8

The anterior approach to the spine is necessary for correction of some congenital spinal deformities and other conditions, including spinal trauma, infection, and tumor. The morbidity associated with this procedure has not been extensively reviewed in the literature. Between 1981 and 1986, 85 patients (41 male and 44 female) aged 1 to 77 years underwent anterior spinal fusion by an orthopedic or general surgery team (33 pediatric patients and 52 adult patients). Thirty-four patients had scoliosis, 8 had kyphosis, 24 had spinal trauma, 9 had tumor, and 10 had infection. Fifteen patients had restrictive lung disease diagnosed by pulmonary function testing (10 children and 5 adults). The thoracoabdominal approach was used in 50 patients, thoracotomy in 22 patients, and the lumbar approach in 10 patients. Two incisions were used in three patients. Correction was accomplished by interbody fusion in 36 patients (17 with instrumentation) and strut graft in 49 patients (6 with instrumentation). Twelve strut grafts were vascularized ribs and 37 were free ribs. Eighty-two patients survived (96 percent). Seventy-four complications occurring in 50 patients all resolved prior to discharge. These included 28 pulmonary complications, 27 urinary complications, and 5 gastrointestinal complications. Three patients required prolonged mechanical ventilation. Solid fusion was seen in 78 of 85 patients, whereas pseudoarthrosis developed in 7.
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PMID:Exposure of the anterior spine. Technique, complications, and results in 85 patients. 342 22

Twenty-seven patients with CF and joint complaints were reviewed. Twelve patients had arthritis, 11 had HPO, and four had symptoms of mechanical neck and back pain. We have confirmed the association of CF with an episodic form of arthritis. For the first time, we have reported that a subgroup of these patients later develop persistent synovitis with progressive asymmetric, erosive disease. The etiology of CF arthritis is unclear, but may relate to chronic infection and immune complex mechanisms. CF arthritis can be contrasted with CF induced HPO, which is associated with worse lung disease, a male predominance, and an older mean age of onset of symptoms. Mechanical neck and back pain may relate to an associated scoliosis or kyphosis in CF.
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PMID:The musculoskeletal manifestations of cystic fibrosis. 351 62

An unusually high prevalence (10%) of scoliosis is described in a series of 151 patients aged four years and older with cystic fibrosis. The scolioses were of the late onset (juvenile and adolescent) type, being typically thoracic with the curve convex to the right, although there was no significant preference for either sex. No direct relationship was found between the spinal curvature and the severity or distribution of the lung disease, although the worse scolioses tended to occur in patients with relatively severe pulmonary involvement. There was no evidence of metabolic bone disease as a predisposing cause. Some indication of a familial tendency towards scoliosis was apparent, and a genetic or constitutional basis is postulated with an unknown precipitating factor.
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PMID:Scoliosis in cystic fibrosis--an appraisal. 707 87

Forty-five individuals with spinal muscular atrophy (SMA) types II and III were evaluated prospectively over a 10-yr period to develop an impairment and disability profile. SMA II subjects showed marked weakness and progressive decline of strength. Mean manual muscle test (MMT) score for all muscles combined was 2.3 +/- 0.6, with a decline in strength of -0.24 MMT units per decade. SMA III individuals had a relatively static or very slowly progressive course and were far stronger. Mean MMT score for all muscles combined was 3.8 +/- 0.7, and the decline in strength per decade was not significant. In both types proximal weakness was greater than distal, but there was greater involvement of the lower extremities and the extensor muscle groups only in SMA II. Contractures, progressive scoliosis, and restrictive lung disease (RLD) were present in most of the SMA II individuals, but these complications were rare in SMA III. Maximal expiratory pressures were affected earlier and to a greater degree than vital capacity. Seventy-eight percent of those with SMA II had scoliosis with a mean Cobb angle of the primary curve of 62 +/- 37 degrees. Forty-one percent had severe RLD, and 17% had moderate RLD. In both types, 63% had abnormal electrocardiograms although most had minor findings. Timed motor performance and functional evaluations indicated that muscle weakness translated to substantial disability in both SMA II and III, with more severe impairment noted in SMA II. Neither type was associated with abnormal means scores on intellectual and neuropsychologic test batteries.
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PMID:Profiles of neuromuscular diseases. Spinal muscular atrophy. 757 22

Ninety-two individuals with myotonic dystrophy (MD) were evaluated prospectively over a 10-yr period and separated into two types, 75 noncongenital (NC-MD) and 17 congenital (C-MD) MD. Muscle weakness was relatively mild and similar in both types, 4.0 +/- 0.7 manual muscle test (MMT) scores for NC-MD and 3.8 +/- 0.7 in C-MD. However, weakness was progressive in the former, -0.36 MMT units per decade, and nonprogressive in C-MD. Weakness was usually generalized in both types, with no significant differences between upper and lower extremities or the proximal and distal muscles. Flexor and extensor differences were variable. Quantitative strength measurements showed a similar pattern but were more sensitive showing marked strength losses of 40-50% in muscle groups with MMT scores of four or more. There was a high frequency (47%) of relatively mild, nonprogressive scoliosis in C-MD, whereas spine deformity was unusual in NC-MD. Contractures, usually at the ankles, were also more common in C-MD. In NC-MD and C-MD, respectively, there was a low frequency of severe restrictive lung disease (14 and 20%) but a high percentage of significant electrocardiographic (ECG) abnormalities (75 and 81%), including conduction defects. There was a marked difference between the two types of MD in intellectual and cognitive function. Seventy-five percent of C-MD subjects showed impairment, frequently severe, compared with 35% impairment, usually mild, for NC-MD individuals. Functional evaluation was not markedly affected, but timed motor performance showed significant disability especially for individuals with C-MD.
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PMID:Profiles of neuromuscular diseases. Myotonic dystrophy. 757 18

Sixty-six individuals with Limb-Girdle Syndrome (LGS) were evaluated over a 10-yr period and classified into three types: 19 severe autosomal recessive muscular dystrophy of childhood (ARMDC), alternatively referred to by some as SCARMD, 18 autosomal dominant late onset (ADLO), and 29 pelvifemoral (PF) individuals. ARMDC subjects showed the greatest weakness, 2.5 +/- 1.0, mean Manual Muscle Test (MMT) grade for all muscles combined, and the only significant progression of loss of strength, -0.59 MMT unit decline per decade. Strength loss in ADLO and PF types was about the same, 3.7 +/- 0.7 and 4.0 +/- 0.7 grades, respectively. Quantitative strength measurements in ADLO and PF types were more sensitive than MMTs, showing losses of 30-40% strength in muscle groups with MMT grades of 4 or higher. All three types showed greater proximal and lower extremity weakness but usually no difference between flexor and extensor strength. There was a high percentage (44%) of mild very slowly progressive scoliosis in ARMDC, but spine deformity was unusual in ADLO and PF (11%) LGS. Contractures were few, slowly progressive, and usually mild in severity in all types, although more frequent in ARMDC. There also was a low frequency of severe restrictive lung disease in all types (10%) but a high percentage of electrocardiogram abnormalities (62-73%). The most common electrocardiogram abnormalities were increased R/S ratio in V1 and infranodal conduction defects. Intellectual and cognitive functions were within normal limits. Mobility and extremity function reflected the strength differences between the ARMDC and other types of LGS. Eight-five percent of ARMDC individuals relied on a wheelchair for all or part of their mobility, and all were unable to complete timed motor performance tests within the 99th percentile range for controls.
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PMID:Profiles of neuromuscular diseases. Limb-girdle syndromes. 757 19

Fifteen subjects with Becker's muscular dystrophy (BMD) were studied prospectively over a 10-yr period to provide a profile of impairment and disability. Proximal lower extremity musculature (particularly the hip and knee extensors), ankle dorsiflexors, and neck flexors showed significantly early weakness. Extensor muscle groups were weaker than flexor muscles at the elbow and knee. The mean manual muscle test (MMT) strength grade for all muscle groups combined was 3.7 +/- 0.8 MMT units. There was a slowly progressive decline in strength, only -0.31 MMT units per decade, and the decline was relatively equal in all muscle groups. There was not side dominance. Severe contractures did not appear to be a problem until after transition to a wheelchair, and scoliosis was rare. Restrictive lung disease occurred as a late complication in a small percentage of cases; however, maximal expiratory pressure was significantly reduced early in the disease. Only two individuals (19%) had severe restrictive lung disease and a history of significant respiratory complications. There was a slight but significant decline in forced vital capacity and maximal expiratory pressure with age and disease duration. The cardiomyopathy in BMD appeared to be disproportionately severe in some cases. Eleven individuals (73%) had abnormal electrocardiograms, and five (25%) had a history of significant cardiovascular complication, with no age or disease duration effect. Functional evaluations and timed motor performance tests showed only mild disability in most individuals. Mean intellectual and neuropsychologic function was within normal limits, but with a large variability in intelligence quotient scores. This report and others suggest a tremendous heterogeneity of severity among males with BMD.
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PMID:Profiles of neuromuscular diseases. Becker's muscular dystrophy. 757 25

In early phases of neuromuscular disease, patients are either free of respiratory symptoms or have exertional dyspnea not explained by obvious obstructive or restrictive lung disease. Physical examination may be negative because generalized muscle weakness does not correlate with the degree of respiratory muscle involvement. When the diaphragm is involved, one may detect the absence of outward excursion during inspiration or even paradoxic inward inspiratory movement of the abdomen on one side. A substantial loss of respiratory muscle strength is typically accompanied by little or no change in spirometry or arterial blood gas composition. Other characteristics are moderate loss of maximal voluntary ventilation and an increase in residual volume, yet PImax and PEmax may be as low as 50% of the predicted value. In more advanced neuromuscular disease, patients may have severe symptoms if the onset is acute or subacute; however, patients with chronic advanced generalized muscle weakness do not exercise and, therefore, may not be breathless. Many patients with advanced neuromuscular disease present with daytime somnolence as a manifestation of a sleep-related breathing disorder. Physical examination may reveal generalized muscle weakness and difficulty with speech or swallowing. Signs specific to respiratory involvement include tachypnea, use of neck inspiratory muscles and abdominal expiratory muscles, and loss of chest-abdomen synchrony. Sometimes paradoxic bilateral inward movement of the abdomen with inspiration is overt. Patients may be unable to cough effectively, have scoliosis, and lack a gag reflex. At this advanced stage, PImax and PEmax are lower than 50% of the predicted value, and the vital capacity is reduced. Maximal voluntary ventilation increases, and residual volume increases further. Patients may not yet exhibit CO2 retention during the day and may even have a low PaCO3. A sleep study may reveal significant hypopneas with severe desaturation and hypercapnia, especially during REM sleep. It is important to be aware that overt ventilatory failure can occur abruptly and that measurement of arterial blood gas composition is not a reliable indicator of this danger. Therefore, it is critically important to heed clinical phenomena, such as increasing dyspnea and tachypnea, and symptoms of sleep disturbance, such as morning headache and daytime somnolence. Physicians should make serial measurements of VC and respiratory muscle strength in patients considered to be at risk for further deterioration.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Assessment of ventilatory function in patients with neuromuscular disease. 786 89


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