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

Patients with neuropathic bladders who have intractable hyperreflexia often require cystoplasty and in patients adequately motivated and counselled, continence rates in excess of 90% can be achieved. Seventy-eight patients with neurological disease have undergone enterocystoplasty and are presented. Twenty-two patients had acquired cord lesions of which 17 were traumatic. Of the 56 patients with congenital myelodysplasia, 49 were meningomyelocele, 5 had sacral agenesis, and 2 lipoma of cauda equina. The mean age was 26 (range 13-61) years with 48 male and 30 females. Pre-operative video urodynamics showed sphincter weakness in 54 patients, all patients had intermediate type bladders; hyperreflexia in 52, reduced compliance in 17, and in 7 a combination of both. Forty-seven patients had ileal, 20 had sigmoid, and 11 had ileocaecal cystoplasty. Fifty-two patients had an artificial urinary sphincter inserted, 9 had a colposuspension, and 1 had both for concurrent sphincter weakness incontinence. Acceptable continence has been achieved in 73 (93.6%) patients, 65 claiming complete continence with no pads or incontinence episodes and the 8 others needing pharmacotherapy or pads. Two of our 5 failures have been converted to an ileal loop, 2 are awaiting further surgery, and 1 is wet despite normal urodynamics. Our threshold for initiating intermittent catheterisation is low and 63 (81%) of our patients catheterise. Complications in these patients are few and the only one of note is persistent bowel problems in 30% of our patients. Cystoplasty is recommended as safe and reliable in the treatment of neurogenic detrusor over activity with a continence rate of 93% being achieved.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Enterocystoplasty in the neuropathic bladder. 774 49

Calcaneus deformity in patients with neurologic disorders leads to gait disturbances, a high incidence of osteomyelitis, cellulitis, and skin breakdown, and a major problem in the fitting of shoes of orthoses. Correction of this deformity remains a difficult challenge for the orthopedic surgeon. Twelve tenodeses of the tendo Achillis to the fibula or tibia were performed in eight patients with myelodysplasia in an attempt to control calcaneus deformity. The average age of the patients at the time of surgery was 7.4 years (range, 3.1-13.5 years) with an average follow-up period of 6.3 years (range, two to 10.9 years). The clinical examination demonstrated definite improvement in the ability to brace, prevent skin breakdown, and improve gait in all but one patient who had progressive hindfoot valgus. Roentgenographically, there was improvement in the tibiocalcaneal angle in 67%, fibular station in 58%, talar tilt in 25%, and growth of the posterior aspect of the calcaneus in 58%.
 ...
PMID:Achilles tenodesis for calcaneus deformity in the myelodysplastic child. 851 15

Little has been written regarding the assessment and treatment of hip disorders in children who have underlying paralysis. Each year approximately 2000 people younger than 20 years of age suffer a spinal cord injury (SCI). This compares with a larger number of children who have other forms of neurologic disorders, such as myelodysplasia, which affects approximately 6000 newborns annually in the United States, and for which there is a large body of literature describing the natural history and treatment of hip disorders in children who have myelodysplasia. This article focuses on hip disorders in children who have SCI, although there is clearly commonality in hip disorders that transcends many neurologic disorders.
 ...
PMID:Hip disorders in children who have spinal cord injury. 1663 50

Neurological disorders induced by long-term exposure to organic solvents typically have a slowly progressive clinical course, which may be arrested or even reversed following discontinuation of exposure. We report an unusual case of rapidly progressive toxic leukoencephalomyelopathy in a 29-year-old man who had worked at a chemical factory that used toluene for the manufacture of nylon 66 for 5 years. He presented with progressive weakness of legs, recurrent seizures, and cognitive decline. Widespread white-matter changes in the brain and spinal cord, and myelodysplastic syndrome were noted. He died 6 months after the onset of his symptoms, and autopsy showed discrete multifocal demyelination and necrosis in the central nervous system, and dysplastic cells of erythroid, myeloid, and megakaryotic lineages in blood vessels. The co-occurrence of leukoencephalomyelopathy and myelodysplastic syndrome highlights the vulnerability of the white matter and bone marrow to injury from organic solvents. Intravascular congestion of dysplastic hematopoietic cells might have led to his unusually rapid progression of leukoencephalomyelopathy.
 ...
PMID:Rapidly progressive toxic leukoencephalomyelopathy with myelodysplastic syndrome: a clinicopathological correlation. 1951 42

The International Parkinson Disease and Movement Disorder Society PSP study group (IPMDS-PSP) recently published new clinical diagnostic criteria for progressive supranuclear palsy (PSP). Currently, there is no data regarding the accuracy of these sets of criteria for differentiating various PSP phenotypes. We discuss the accuracy of the IPMDS-PSP criteria for differentiation of patients with the PSP- Richardson phenotype (PSP-RS) from those with the PSP-Parkinsonism (PSP-P) using data from a sample of 274 clinically diagnosed PSP patients participating in the Environmental Genetic PSP (ENGENE-PSP) case control study. Using National Institute of Neurological Disorders and Stroke and the Society for PSP (NINDS-SPSP) criteria and the Williams criteria we categorized 259 of these patients as probable PSP-RS and 15 as PSP-P. The IPD-MDS PSP-RS and PSP-P criteria were unable to distinguish the PSP-RS from the PSP-P phenotypes in this sample. Nearly all (92.6%; 240 out of 259) the PSP-RS patients and over half (60%; 9 out of 15) of the PSP-P patients fulfilled both the IPMDS criteria for PSP-RS and PSP-P. Applying the newly proposed multiple allocation extinction rules decreased the number of overlapping diagnoses among the NINDS-SPSP PSP-RS patients, however problems remained in the PSP-P group. Diagnostic accuracy might be improved by modification of timelines for development of falls and other parkinsonian features.
 ...
PMID:Are the International Parkinson disease and Movement Disorder Society progressive supranuclear palsy (IPMDS-PSP) diagnostic criteria accurate enough to differentiate common PSP phenotypes? 3166 86