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

Sixty-seven children (40 girls and 27 boys) suffering from primary mitral valve prolapse (MVP) were studied. The age of these subjects was 5 to 18 years with the mean 12.8 +/- 3.3 years. MVP was diagnosed on the basis of physical and echocardiographic examination. In every case following studies were performed: routine and 24-hours ECG (according to Holter method), echocardiography, and physical performance test (according to Bruce protocol). The cause of referring the child to cardiologist were: cardiac murmur with/or midsystolic click in 85%, chest pain in 31%, feeling of cardiac palpitations in 40%, loss of consciousness in 40%, increased fatigability in 22%, feeling of dyspnoea in 10.5% (there were often more than one symptom in the same subject). Familial inheritance of MVP was suggested in over 20% of our patients. Asthenic constitution with orthopedic disorders (e.g. important scoliosis, pectus excavatum etc.) was found in about 20% of studied children. In 15% so called silent MVP was recognized. In 19% of subjects mitral valve leaflets inspection indicated their thickening and redundancy. In 67% routine ECG showed abnormalities of cardiac rhythm, conduction or repolarization. During Holter monitoring rhythm disturbances were detected in 22% of patients. Physical performance test results were normal in every case.
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PMID:Clinical characteristics of primary mitral valve prolapse syndrome in children. 761 Jul 38

A 13-yr-old male with Marfan's syndrome underwent surgical correction of severe scoliosis. He had not manifested dyspnea previously in any position. Under anesthesia with thiamylal and vecuronium, his trachea was intubated with a reinforced endotracheal tube without any difficulty. Anesthesia was maintained with nitrous oxide and fentanyl, 8 micrograms.kg-1. The patient was placed in a prone position. Thirty min after the start of operation, when orthopedists compressed the thoracic vertebrae vertically, positive pressure ventilation became impossible abruptly, even with a high airway pressure. Three min later, ventilation became possible after cessation of compression and by mouth-to-tube insufflation. SpO2 monitored with a pulse oximeter recovered immediately from 61% to 99%. A capnogram showed a lengthy retardation of an inspiratory phase. Emergency fibreoptic bronchoscopy revealed that the trachea had been compressed vertically; the compression was reduced by moving the chest supporters laterally. After the apneic episode, the operation continued uneventfully, and he was discharged a month later. A severe deformity of the thorax due to severe scoliosis and weak tracheal tissue due to connective tissue defect caused partial tracheal compression before the surgery, and made his trachea susceptible to complete obstruction by vertical external compression on the thorax. Patients with Marfan's syndrome and scoliosis should have careful preoperative airway evaluation. The selection and positioning of endotracheal tubes should be done with care. During surgery, the patient's body position and the condition of the trachea should be checked frequently. Capnography and fiberoptic bronchoscopy seem to be mandatory for early detection of tracheal stenosis and prevention of tracheal obstruction.
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PMID:[Transient tracheal obstruction during surgical correction of scoliosis in a patient with Marfan's syndrome]. 763 68

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

An atypical case of hereditary motor and sensory neuropathy (HMSN) type 2 with cerebellar ataxia, hand tremor and bilateral recurrent nerve palsy was described. The patient was a 57-year-old man who complained of dyspnea, stridor , hoarseness during exercise and snored heavily during sleep since he was 20 years old. These symptoms and signs were slowly progressive. He had difficulty in breathing even at resting state when he was 54 years old and since then, he noticed muscle wasting of his hands and feet. Neurological examinations on admission revealed pes cavus, scoliosis, distal muscular atrophy in his four extremities, especially severe in bilateral lower limbs. Deep tendon reflexes were diffusely depressed. The fiberscopic examination demonstrated the limitation of bilateral vocal cord abduction and his tongue was slightly atrophic. Fine postural tremor was found in bilateral hands. Mild limb and truncal ataxias were also noted. Blood pCO2 level was elevated to 66% although FEV1.0% and vital capacity were within normal limits. Peripheral nerve conduction velocities were almost normal, though distal terminal latencies were slightly prolonged and amplitudes of evoked potentials were markedly decreased. The sural nerve biopsy studies revealed the chronic axonal or neuronal degeneration of both large and small myelinated fibers. From the clinical, electrophysiologic and histopathologic findings, the diagnosis of HMSN type 2 with bilateral recurrent nerve palsy and other atypical neurological findings was made. It is practically important to evaluate the presence or absence of vocal cord paralysis in the patients with HMSN from clinical viewpoints.
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PMID:[A case of hereditary motor and sensory neuropathy (HMSN type 2) with bilateral recurrent nerve palsy]. 829 75

An autopsy case of 63-year old male of atypical mycobacteriosis with severe scoliosis was reported. He was diagnosed as atypical mycobacteriosis in 1981 and controlled by antimycobacterial treatment. But since 1989, dyspnea and sputum have been increasing, new abnormal shadow in company with progression of curvature of the scoliosis was revealed on the chest roentgenogram. In spite of various treatments for mycobacteriosis, he died of respiratory failure. The aggravation of this mycobacteriosis brought on advancement of malnutrition and impairment of cell mediated immunity. Findings of autopsy showed the remarkable chest wall deformity and tuberculous change of the lung parenchyma. In this case, chest deformity was thought as the major risk factor of onset of mycobacterial mycobacteriosis.
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PMID:[An autopsy case of atypical mycobacteriosis accompanied with chest wall deformity]. 830 19

A 13-year-old boy with juvenile-onset acid alpha glucosidase deficiency was reported. Proximal muscle weakness including respiratory muscles and scoliosis progressed since nine year of age. He developed nocturnal dyspnea and daytime somnolence at age 13. His arterial blood gas analysis showed hypoxemia (PO2 54.1 mmHg) and hypercapnia (PCO2 72.3 mmHg), and spirometry showed significantly decreased vital capacity (% VC 21%). He was treated with nocturnal NIPPV employing a device for delivering bilevel positive airway pressure (Bi-PAP). Nocturnal dyspnea and daytime somnolence rapidly disappeared with nocturnal ventilatory support. Daytime arterial PO2 and PCO2 improved after the therapy, namely 74.8 mmHg and 64.1 mmHg respectively. We conclude that NIPPV is a noninvasive and effective therapy for respiratory failure in patients with chronic progressive neuromuscular disorder including acid alpha glucosidase deficiency.
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PMID:[Chronic respiratory failure in a case with juvenile-onset acid alpha-glucosidase deficiency; successful therapy with nasal intermittent positive pressure ventilation (NIPPV)]. 898 97

A 54-year-old man presented with osteogenesis imperfecta complicated with both aortic regurgitation due to annuloaortic ectasia and mitral regurgitation secondary to tendon rupture. He had spinal and carpal deformities in his childhood, and heart murmurs were identified at the age of 25. He was admitted complaining of dyspnea on effort. His height was 142 cm and his weight was 46 kg. He had kyphosis, scoliosis and carpal deformity. Blue sclera was not observed. Chest radiography showed cardiomegaly and lung congestion. Echocardiography showed annuloaortic ectasia, mild aortic regurgitation, and serious mitral regurgitation due to postero-apical tendon rupture. Bone deformity and his statues were indicative of osteogenesis imperfecta. He received modified Bentall and mitral valve replacements.
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PMID:[Successful surgical treatment of aortic regurgitation due to annuloaortic ectasia and mitral regurgitation caused by tendon rupture in a case of osteogenesis imperfecta]. 921 Nov 8

In adolescent idiopathic thoracic scoliosis (ITS) working capacity may be reduced during exercise. Despite concern about its usefulness, bracing is still being used in ITS. Thus the effects of bracing on exercise performance need to be examined. We studied six females, ages 12-15 years who had mild ITS (Cobb angle range 20-35 degrees). Pulmonary volumes, maximal voluntary ventilation (MVV), breathing pattern, the lowest (most negative in sign) pleural pressure during sniff maneuver (Pplsn), and pleural pressure swings (Pplsw) were measured first. Then, Pplsw, O2 uptake (VO2), CO2 output (VCO2), heart rate (HR) at rest and during progressive incremental exercise on a cycling ergometer (10 watts/min) were recorded. The exercise test was performed under control conditions without bracing (C) and after 7 days of bracing with the braced on (B). Dyspnea was measured by a modified Borg scale. At rest, bracing mildly affected total lung capacity and forced vital capacity (p < 0.03 for both) but not breathing pattern, Pplsn, or Pplsw (%Pplsn), a measure of respiratory effort. Furthermore, bracing did not consistently affect maximum work rate (WRmax). In both B and C VO2 was below (< 70%) the predicted value, VE was below (< 45%) MVV, and HR reserve was < 15 beats/min, indicating some cardiovascular deconditioning. On the other hand, respiratory frequency (Rf) increased more in B than in C (p < 0.03). In addition, Pplsw, Pplsw (%Pplsn), and Pplsw (%Pplsn)/VT, an index of neuroventilatory dissociation (NVD) of the respiratory pump, were greater in B (p < 0.03 for all). At a similar work rate, the Borg rating score was greater with bracing on than off, and the difference (delta Borg) tended to relate to concurrent changes in Pplsw (%Pplsn)/VT (r2 = 0.71; p < 0.07). We conclude that bracing affects respiratory effort, NVD, and dyspnea score during progressive exercise. These effects are consistent with increased lung elastance. Diminished exercise tolerance in patients with mild ITS probably reflects impaired physical fitness but is not affected by bracing. Training programs proposed for this subset of patients to increase peripheral muscle performance might also consider NVD of the respiratory pump.
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PMID:Short-term effects of bracing on exercise performance in mild idiopathic thoracic scoliosis. 927 Sep 87

As thalassemia patients age, bone disease becomes a serious cause of morbidity. The frequency and type of bone disease is affected by the underlying type of thalassemia and its treatment. Problems include rickets, scoliosis, spinal deformities, nerve compression, fractures and severe osteoporosis. In early stages, patients may be asymptomatic but can present with back pain, a limp, dyspnea, neurological emergencies, or sudden fractures. The etiologies are often multifactorial, culminating with increased bone resorption and remodeling. They include hormonal deficiency, bone marrow expansion, nutritional deficiency, or desferal toxicity. Particular risk factors include older patients, low baseline hemoglobin, delayed puberty, hormonal failure, and high iron stores. Nutritional deficiencies may further compound the patient's risk for bone disease. Increasing evidence suggests that these complications and their associated long-term morbidity can be prevented if an annual screening is done, followed by long-term intervention. Patients treated with amino biphosphonates inhibit bone resorption and may demonstrate rapid healing. Intra-nasal calcitonin has also been successful in treating osteopenia. Early use of estrogen and testosterone appears to markedly lower the risk for selective patients. Both transfused and non-transfused patients should be educated about risk factors and early symptoms. All patients should be screened annually for bone disease. Once adolescence occurs, annual testing in selected cases should include bone density studies with X-ray absorptiometry.
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PMID:The morbidity of bone disease in thalassemia. 966 56

We experienced a case of difficult tracheal intubation in a 15-year-old boy with von Recklinghausen disease scheduled for resection of a right neck tumor. His scoliosis made it difficult to intubate and to manage airway because he easily developed dyspnea. We tried nasotracheal intubation with the patient awake under sedation using a bronchofiberscope, but we found an unexpected tumor jeopardizing his airway patency near his vocal cord. Preoperative examination of a tumor in the airway is essential in the anesthetic management of the patients with von Recklinghausen disease.
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PMID:[Difficult tracheal intubation with von Recklinghausen disease]. 978 92


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