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)

This report evaluates the efficacy of extensive chest wall resection and prosthetic reconstruction in 15 children with chest wall malignancies. There were nine boys and six girls, with a mean age of 9.6 years. Eleven patients had primary chest wall tumors including Ewing's sarcoma (ES), six; rhabdomyosarcoma (RH), two; chondrosarcoma (CS), one; Askin's malignant neuroectodermal tumor, one; and mesenchymal sarcoma, one. Four children had metastases to chest wall and lung from Wilms' tumor (WT), two; osteogenic sarcoma (OS), one; and neuroblastoma (NB), one. Chest wall resection of two to six ribs and reconstruction with Marlex mesh (seven), lattisimus flap (two), prolene mesh (one), and more recently, a Gortex patch (five), was performed. Eight of the patients required concomitant en-bloc pulmonary resection (wedge, five; lobectomy, two; pneumonectomy, one) and two required resection of diaphragm. Fourteen received adjunctive therapy (chemotherapy, 14; irradiation, eight [preoperative, five; postoperative, three]. Six patients had second-look resections after chemotherapy. There was no operative mortality. Early pulmonary function was normal; however, pulmonary restrictive disease and scoliosis occurred with growth. One ES patient developed a radiation-induced second malignant tumor at age 10 and one ES child died at age 6 (no evidence of disease) of meningitis. Average survival length for ES patients was 77 months (range, 18 to 132 months.) Currently, eight patients are alive and five are free of disease. Extensive chest wall resection and reconstruction is useful in the treatment of primary chest wall tumors, but is palliative in metastatic cases. The Gortex patch is the current prosthetic of choice.
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PMID:Chest wall resection and reconstruction for malignant conditions in childhood. 320 68

The most difficult aspect of the surgical treatment of chest wall tumors is reconstruction of the large residual defect. Materials that have been used include Marlex, Goretex, Vicryl, bone, metal, and fascia. The authors' successful experience with dehydrated human dura (Tutoplast) for moderate-size defects is described. A large Askin's tumor in a 13-year-old boy required resection of the right posterior aspect of the 9th to 11th ribs and the transverse process of T-10, the 12- x 12-cm thoracic defect was closed with dura. Partial soft-tissue coverage was obtained with the latissimus dorsi muscle. Although a scoliosis secondary to paraspinal muscle resection has developed, the chest wall is stable, without evidence of a flail chest, at 18 months of follow-up. A 6-year-old girl underwent left anterior chest wall resection of three ribs for an epithelioid sarcoma. Human dura and a myocutaneous flap were used for reconstruction, with good functional and cosmetic results at 16 months of follow-up. Dura is simple to use, has low antigenicity, and in experimental studies appears to be incorporated into the tissues, acting as a collagen matrix. For moderate-size chest wall defects, it appears to be an excellent alternative to synthetic prosthesis.
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PMID:Use of human dura in pediatric chest wall reconstruction after tumor resection. 780 42

Radiography can be used to aid in the diagnosis and treatment of pet fish diseases. Handling, restraint and radiographic technique for the radiographic examination of pet fish is described. Quality diagnostic images can be obtained with standard radiographic equipment and radiographic techniques. Fishes with undifferentiated sarcoma, swim bladder herniation and scoliosis are three clinical examples that are described where radiography was used in the management of the patient. Conventional radiography appears to be best for evaluating skeletal and swim bladder diseases. Alternate imaging techniques such as computed tomography and magnetic resonance imaging may enhance the evaluation of coelomic soft tissue structures.
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PMID:Pet fish radiography: technique and case history reports. 923 66

A retrospective analysis of 77 children treated between 1974 and 1996 was undertaken to evaluate morbidity and the evolution of therapy. A Wilms' tumor (WT) was present in 73 children. 74% of patients (pats.) with WT survived (54 of 73 pats.). Histological specimens of 67 patients were re-evaluated, including 4 children with non-WT histology. Among patients with Wilms' tumors (WT), nephroblastoma (NB) of intermediate risk predominated (73%; 46 of 63 pats.). Low-risk tumors occurred in 5 of 63 children (8%; mesoblastic nephroma 3, cystic partially diff. NB 1, completely necrotic NB 1). High-risk WT were diagnosed in 12 of 63 patients (19%) (NB with anaplasia 10, clear cell sarcoma 1, malignant rhabdoid tumor 1). Nephrogenic rests were present in 14 cases. We observed 3 children of school age with renal carcinoma and one patient with an intrarenal neuroblastoma. WT histology was the most important factor determining prognosis (p = 0.018). The risk for relapses was 2.6-fold higher in patients with high-risk WT compared to the standard risk group. Stages were re-evaluated according to SIOP 93-01. Comparing relapse-free survival of stages I, II and III, respectively, there was a reduced survival rate for stage III (p=0.019). According to the SIOP/GPOH protocol in 1989, the regimen was switched from primary surgery to preoperative chemotherapy without biopsy in 1989 (11 pats.). Compared to earlier years, survival improved (n.s.). In 3 patients preoperative diagnosis by means of imaging failed. During preoperative chemotherapy a venous occlusive disease of the liver occurred in 2 patients. Preoperative chemotherapy led to an impressive tumor shrinkage in the majority of patients. 2 patients of the preoperative group died (focal anaplastic NB). Long-term morbidity was analysed in 49 patients and included radiation-induced scoliosis (35), chest-wall deformity (3), congestive cardiomyopathy after relapse (1) and arterial hypertension (2). Over the years there was a trend to reduce frequency and dose of irradiation. Prognosis of WT is excellent but unfavorable histology (high risk) predicts a poor prognosis. In our experience, reduction of tumor volume due to preoperative chemotherapy facilitates tumor removal by surgery and may prevent tumor spillage and the deleterious effects of radiation in young children. Surgery without delay is necessary if the diagnosis is unclear or the tumor fails to respond to preoperative chemotherapy.
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PMID:A 23-year experience with malignant renal tumors in infancy and childhood. 1137 Oct 43

Neurofibromatosis 1 is an autosomal dominant disorder characterized by distinctive clinical problems including scoliosis and malignant peripheral nerve sheath tumors. We present two cases of retroperitoneal malignant peripheral nerve sheath tumor associated with scoliosis in neurofibromatosis. Presence of spinal deformity resulted in delay of the diagnosis of the sarcoma.
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PMID:Retroperitoneal malignant peripheral nerve sheath tumor associated with scoliosis in neurofibromatosis. 1138 79

This study aims to investigate features of different diseases with low back pain misdiagnosed as spondyloarthropathy so as to improve the accuracy of diagnosis for spondyloarthropathy. The clinical and laboratory data of 24 cases misdiagnosed as spondyloarthropathy in recent 3 years were comparatively and retrospectively analyzed. The diagnostic accuracy of the European Spondyloarthropathy Study Group (ESSG) criteria, Amor criteria, and the combination of them in these misdiagnosed cases was also analyzed. The final diagnoses of these 24 cases were listed below: four malignant tumors (retroperitoneal adipose sarcoma, advanced gastric carcinoma, ovarian papillary epithelioma, acute lymphocytic leukemia), six benign tumors (two parathyroid adenoma with hyperparathyroidism, one intraspinal lipoma, intraspinal ependymomas, sacral tubulocyst, and intraspinal schwannoglioma, respectively). The other 14 cases included fibromyalgia syndrome (3), osteitis condensans (3), diffuse idiopathic skeletal hyperostosis (2), lumbar intervertebral disk protrusion (1), congenital scoliosis (1), Wilson's disease (1), ochronosis (1), Fanconi syndrome (1) and hypophosphatemic rachiopathy (1). Among patients with tumor, all except three patients had persistent low back pain without morning stiffness, which aggravated at night and could not be relieved by rest or exercise. The symptoms could not be relieved by administration of multiple nonsteroidal anti-inflammatory drugs. Eleven patients had inflammatory low back pain defined by Calin. Of the total misdiagnosed cases, 54.17-83.33% could be prevented by application of ESSG criteria or Amor criteria, or a combination of them. From the data, we could see that the clinical features of different diseases with low back pain were different from each other and from those of spondyloarthropathy. The various criteria for spondyloarthropathy may be more effective in combination, along with other clinical information like these clinical features.
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PMID:Comparative study on low back pain misdiagnosed as spondyloarthropathy. 1944 45