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

Report on a follow-up of 38 children treated with surgery for bronchiectasis during the last 10 years at the Department of Paediatric Surgery at the Dresden Medical Academy. 25 patients could be followed-up. In 17 of them a thoracotomy was performed in an age below 10 years. Besides some minor changes like narrowing of the intercostal spaces several cases with scoliosis of the vertebral column could be found. The importance of early controls and intensive physiotherapy for these cases is stressed. A case with rib osteomyelitis after surgery, which made thoracoplasty necessary is discussed.
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PMID:[Thorax deformity after lung resection during childhood (author's transl)]. 89 60

Upward diaphragmatic displacement was performed in 53 patients after massive lung resection for tuberculosis or bronchiectasis and the data are presented. This technique provides ample room for expansion of the remainder of the lung; it also avoids overexpansion, activation of tuberculous foci, and relapse of bronchiectasis. Diaphragamatic displacement can be used for liquidation of a remaining infected cavity. In children with massive bronchiectasis it serves to conserve two unaffected segments and avoids pneumonectomy. During pneumonectomy in children it prevents significant shift of the mediastinum and progression of scoliosis.
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PMID:Upward displacement of the diaphragm after lung resection. 111 47

Thoracic computed tomography was performed in 32 patients who had undergone thoracoplasty as part of their treatment for pulmonary tuberculosis. Pleural thickening and the prevalence of bronchiectasis were more marked in the operated hemithorax. Bullae were more prevalent in the operated hemithorax but the difference was not statistically significant. In all but one patient, scoliosis was present. Illustrative examples are presented to demonstrate the range of appearances following this operation.
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PMID:Appearances on computed tomography following thoracoplasty for pulmonary tuberculosis. 326 11

Kartagener's syndrome (KS) is a clinical variant of primary ciliary dyskinesia involving situs inversus associated with chronic airway infections. We studied two sisters; the elder one had dextrocardia and scoliosis, and the younger one had situs inversus of the lung, liver, and stomach as well as dextrocardia. Both patients had chronic sinusitis and chronic bronchitis with bronchiectasis. In both cases, the ciliary defect associated with this syndrome is the absence of inner dynein arms.
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PMID:Ciliary ultrastructure in two sisters with Kartagener's syndrome. 1738 88

Hyper IgE syndrome (HIES) is a rare primary immunodeficiency characterized by the triad of elevated IgE and eosinophilia, eczema, and recurrent skin and pulmonary infections. The autosomal dominant (AD) form of HIES results from mutations in STAT3 and is characterized by disordered inflammation, connective tissue, and skeletal abnormalities. Tissue-specific STAT3 deficiency in animals, cytokine and transcriptional array data, and careful clinical phenotyping have explained some of the pathophysiology of the immunologic and non-immunologic abnormalities of AD-HIES. In depth study of the role of STAT3 mutations in specific aspects of HIES may lead to better understanding and new approaches to treatment of conditions intrinsic to HIES that are common in the general population, such as staphylococcal infections, scoliosis, osteoporosis, bronchiectasis, and arterial aneurysms. As the genotypes of STAT3 deficiency are further characterized, genotype-phenotype correlations may emerge that will be informative regarding specific molecular interactions. Autosomal recessive forms of hyper IgE (AR-HIES) have also been reported. A single case of homozygous deficiency of the signal protein Tyk2 has been reported as well as a recessive syndrome with some features overlapping AD-HIES, but for which the genetic etiology is unknown. Better understanding of the pathophysiology and mechanisms of dominant and recessive hyper IgE syndromes will shed light on somatic and immune biology and may improve quality of life and survival for HIES patients.
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PMID:Pathogenesis of hyper IgE syndrome. 1945 85

Pulmonary rehabilitation is a tool that is receiving more acceptance in chronic lung diseases. A retrospective study was made in Riyadh, Saudi Arabia, on the impact of pulmonary rehabilitation on respiratory parameters and health care utilization in a group of outpatients with chronic lung diseases other than chronic obstructive pulmonary disease. A group of 51 patients diagnosed with interstitial lung diseases, bronchiectasis, asthma and scoliosis were studied. Initial number of emergency department visits and hospital admissions and use of prednisone and antibiotics were significantly associated with adherence to the pulmonary rehabilitation programme. There was a significant improvement in functional exercise capacity as manifested on the 6-minute walking distance and distance on treadmill, bicycle and arm ergometer and significantly better utilization of health care resources (fewer emergency department and outpatient department visits) over the 12 months after completion of the programme.
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PMID:Impact of a pulmonary rehabilitation programme on respiratory parameters and health care utilization in patients with chronic lung diseases other than COPD. 2257 Oct 87

The disorder comprising Macrocephaly, Alopecia, Cutis laxa, and Scoliosis has been designated MACS syndrome. It is a rare condition, inherited in an autosomal recessive pattern. Three families from different ethnic origins have so far been reported and were all linked to homozygous mutations in RIN2, a gene encoding the Ras and Rab interactor 2 protein involved in cell trafficking. We describe herein the fourth family with MACS syndrome in two siblings carrying a novel homozygous mutation, c.1878_1879insC in exon 8 of the RIN2 gene, which predicts p.Ile627Hisfs*7. We also report on additional findings not previously described in MACS syndrome, including bronchiectasis and hypergonadotropic hypogonadism. Finally, our overall data support the argument that RIN2 syndrome is a more appropriate name for the disorder.
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PMID:Newly described clinical features in two siblings with MACS syndrome and a novel mutation in RIN2. 2444 1

Hemoptysis is defined as bleeding from the lower respiratory tract. It can be life-threatening and requires urgent investigation and intervention. Common causes of hemoptysis include bronchiectasis, tuberculosis, aspergilomas and malignancy. We present an unusual case of hemoptysis in a young woman with a history of surgery for scoliosis 18 years earlier. A 30-years-old woman was admitted to our institution for recurring hemoptysis since one year. She had a history of scoliosis and had undergone antero-lateral Th7 through Th12 spinal fusion surgery 18 years earlier. The hemoptysis was slight and resolved spontaneously or after empirical antibiotic therapy, and was attributed to bronchitis. Computed tomography revealed spinal rod penetration into the lung resulting in injury, while the caudal edge of the rod migrated into the liver and the joining screws had entered the mediastinum. Hemoptysis was due to penetration of the rod into the lung. The patient underwent extensive surgery, which was successful. The case highlights the need for thorough evaluation of patients with hemoptysis. Every incident of hemoptysis, even if minor, should be promptly investigated, because it can be life-threatening.
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PMID:Unusual cause of hemoptysis: A case report. 3292 71