Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Seventy-six consecutive surgical cases of paralytic neuromuscular spinal deformity were retrospectively analyzed. Posterior arthrodesis with segmental spinal stabilization with Luque L-rods, sometimes preceded by anterior release, was done in all cases. The infection rate of 14.5% was observed to be markedly higher in patients with myelodysplasia. Deep placement of the rods lateral to the spine and well beneath full-thickness skin is recommended to reduce the incidence of this complication.
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PMID:Segmental spinal instrumentation for neuromuscular spinal deformity. 270 47

The results of a prospective study of the surgical treatment of paralytic scoliosis and congenital lumbar kyphosis in 19 children with myelodysplasia and one child with sacral agenesis indicate that improved results can be achieved with the use of staged anterior and posterior fusions, more rigid spinal instrumentation, and bank and autogenous bone graft. No wound infections occurred as a result of preoperative treatment of urinary infection and with the use of prophylactic antibiotics. Improved posterior spinal fixation was accomplished with a double square end sacral hook in paralytic scoliosis and pelvic obliquity and with a cable-hook compression system in congenital lumbar kyphosis. Posterior lumbopelvic fusion with cable-hook compression instrumentation gave acceptable results in lumbopelvic kyphosis from complete sacral agenesis.
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PMID:Severe spine deformity in myelodysplasia and sacral agenesis: an aggressive surgical approach. 730 85

In this review, basic knowledge and recent innovation of surgical treatment for scoliosis will be described. Surgical treatment for scoliosis is indicated, in general, for the curve exceeding 45 or 50 degrees by the Cobb's method on the ground that:1) Curves larger than 50 degrees progress even after skeletal maturity.2) Curves of greater magnitude cause loss of pulmonary function, and much larger curves cause respiratory failure.3) Larger the curve progress, more difficult to treat with surgery.Posterior fusion with instrumentation has been a standard of the surgical treatment for scoliosis. In modern instrumentation systems, more anchors are used to connect the rod and the spine, resulting in better correction and less frequent implant failures. Segmental pedicle screw constructs or hybrid constructs using pedicle screws, hooks, and wires are the trend of today.Anterior instrumentation surgery had been a choice of treatment for the thoracolumbar and lumbar scoliosis because better correction can be obtained with shorter fusion levels. Recently, superiority of anterior surgery for the thoracolumbar and lumbar scoliosis has been lost. Initial enthusiasm for anterior instrumentation for the thoracic curve using video assisted thoracoscopic surgery technique has faded out.Various attempts are being made with use of fusionless surgery. To control growth, epiphysiodesis on the convex side of the deformity with or without instrumentation is a technique to provide gradual progressive correction and to arrest the deterioration of the curves. To avoid fusion for skeletally immature children with spinal cord injury or myelodysplasia, vertebral wedge ostetomies are performed for the treatment of progressive paralytic scoliosis. For right thoracic curve with idiopathic scoliosis, multiple vertebral wedge osteotomies without fusion are performed. To provide correction and maintain it during the growing years while allowing spinal growth for early onset scoliosis, technique of instrumentation without fusion or with limited fusion using dual rod instrumentation has been developed. To increase the volume of the thorax in thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis, vertical expandable prosthetic titanium ribs has been developed.
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PMID:Surgical treatment of scoliosis: a review of techniques currently applied. 1842 27

Posterior reversible encephalopathy syndrome (PRES) is an uncommon but distinctive clinical-radiologic entity characterized by headache, seizures, visual disturbance, and altered mental function associated with reversible white matter edema affecting the posterior parietal and occipital lobes of the brain. Although PRES is caused by a variety of conditions, acute elevation of blood pressure, fluid retention, and treatment with immunosuppressive drugs and/or anti-neoplastic agents are the main causes. A few cases of PRES associated with hematopoietic stem cell transplantation (HSCT) in children have been reported. Early recognition of PRES and appropriate management are needed to reduce the risk of permanent neurologic disability. The authors report a case of PRES in a girl who received an HLA-identical sibling bone marrow transplantation for myelodysplastic syndrome to emphasize the importance of early recognition and institution of appropriate management of PRES during HSCT.
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PMID:A case of posterior reversible encephalopathy syndrome in a child with myelodysplastic syndrome following allogenic bone marrow transplantation. 2012 56

Posterior reversible encephalopathy syndrome (PRES) is one of the most serious complication after allogeneic stem cell transplantation in paediatric setting. It is most commonly reported as adverse event of immunosuppressive strategies during transplantation. We present a case of a 7 years old girl with myelodysplastic syndrome (MDS) treated with allogeneic stem cell transplantation (ASCT) at our department. Diagnosis of PRES was confirmed by imaging techniques during the first month after transplant and it was very likely connected with cyclosporine neurotoxicity. The aim of this article is to present our first experience in diagnosing and treating PRES in paediatric stem cell transplantation. Our experience showed that PRES is one of the reasons for higher transplant related mortality in children. Early prediction of factors contributing to PRES and closely monitoring of patient's vital signs, especially blood pressure, neurological status and vision are the main contributors for challenging the patient with another immunosuppressive agent that has less neurological toxicity. Still studies have to be initiated to confirm the influence of PRES on transplant outcome.
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PMID:Posterior Reversible Encephalopathy Syndrome (PRES) in Children Undergoing Allogeneic Stem Cell Transplantation. 3115 38