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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with myeloproliferative disorders were prospectively studied by in vitro agar-gel marrow culture technics to evaluate factors involved in the evolution of abnormal granulopoiesis. Marrow granulocytic colony-forming capacity was determined in 78 patients with chronic myeloid leukemia, subacute myeloid leukemia, preleukemia, Di Guglielmo's syndrome, polycythemia vera or essential thrombocythemia. A wide range of marrow colony-forming capacity values was noted early in disease courses; however, in 26 of 33 patients decreased colony-forming capacity was associated with disease transformation into acute myeloid leukemia or other clinically aggressive stages. An increased proportion of abnormally light buoyant density (less than 1.062 g/cm3) colony-forming cells was present in the marrow and peripheral blood of 15 of 16 patients with chronic myeloid leukemia, subacute myeloid leukemia, preleukemia or essential thrombocythemia; in seven of eight patients with greater than 35 per cent abnormally light colony-forming cells their disease subsequently underwent transformation. Elevated levels of urinary colony-stimulating factor output were noted in 17 of 31 patients, and in 10 of 12 patients whose disease subsequently underwent acute transformation within 10 months of study. In six of seven patients who simultaneously had an increased urinary output of colony-stimulating factor and low colony-forming capacity in marrow, transformation occurred within 10 months. These findings indicate that progressive abnormalities of both marrow clonal growth patterns and levels of possible humoral regulatory substances develop during evolution of these diseases. In contrast, patients with idiopathic sideroblastic ineffective erythropoiesis had normal values for marrow colony-forming capacity, proportion of light density colony-forming cells and urinary colony-stimulating factor output, and in none has their disease transformed into acute myeloid leukemia. These in vitro studies appear useful for clinical staging, evaluating prognosis and categorizing patients with myeloproliferative disorders.
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PMID:The myeloproliferative disorders. Correlation between clinical evolution and alterations of granulopoiesis. 108 34

A series of 454 hydrocephalic patients with and without myelomeningocele and with and without treatment is reviewed. The survival rates for hydrocephalus alone and for hydrocephalus with myelodysplasia are comparable. The authors reach the conclusion that treatment of the hycrocephalic process and its complications is the most critical therapeutic consideration. Mental retardation is the major unalterable cause for failure to develop independence; some lesser emotional causes can be modified by encouragement. Repeated reassessment of the patient's condition and adjustment are important. Before treatment is started parents or guardians should be fully informed of the child's future potential for independent life and mental development.
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PMID:Follow-up comparison of hydrocephalus with and without myelomeningocele. 108 37

Twelve children with lumbar-level myelodysplasia (average age, eight and three-quarter years) underwent twenty-one pelvic displacement osteotomies for subluxated or dislocated hips. Nineteen of the twenty-one hips remained reduced on three-year follow-up. Gains in gait pattern, ease of bracing, and reduced pelvic obliquity were noted. Active function about the hips was not improved, nor was there a decrease in the amount of bracing needed following the osteotomy. Pelvic displacement osteotomy can be utilized in selected cases as part of the over-all management of chronic hip dislocation in myelodysplasia.
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PMID:Pelvic displacement osteotomy for chronic hip dislocation in myelodysplasia. 108 69

The subject of occult spinal dysraphism or myelodysplasia is reviewed from standpoints of embryology, clinical manifestations, and treatment, and the management of 73 cases summarized. In general, these concealed lesions arise from developmental variants in the most distal part of the neural tube, a situation which may cause distortion or partial absence of neural tissues and also lead to damage from compression or traction. Lipomyelomeningocele and congenital dermal sinus are two exampled of the many types of such lesions, but some are more complicated and border-line myelomeningocele-like forms occur. Incontinence, deformity or weakness of the feet, impaired gait, and other difficulties may appear late and increase with growth. Surgical treatment is advised to reduce chances of delayed or progressive loss of function.
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PMID:Occult spinal dysraphism: a series of 73 cases. 109

Of a total of 143 myelodysplastic patients treated between 1928 and 1951, there were sixty-three patients with severe myelodysplasia whose records allowed long-term review. At the time of writing twenty-nine were alive and were twenty to forty-three years old. All were walking in the hospital while under an intensive physical therapy program. However only two of the nine with twelfth thoracic-second lumbar function were walking at final follow-up as adults, while nineteen of the twenty with function at the third lumbar level were doing so. The status of the hips did not correlate with the ability to walk. One-third of the survivors were self-supporting at the time of writing. About one-half had scoliosis and in one-third was greater than 20 degrees.
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PMID:Myelodysplastics--fate of those followed for twenty years or more. 109 48

Five cases of myelodysplasia with progressive paraparesis are presented. Three of the five patients developed spasticity, but dissociated sensory loss and loss of sphincter control was not a prominent feature. All were found to have compensated hydrocephalus and extensive communicating hydromyelia. The use of myelography and ventriculography in the diagnosis of hydromyelia is discussed. Ventricular drainage led to clinical improvement in two cases and radiological improvement in one. The relationship of compensated hydrocephalus, meningomyelocele, and progressive hydromyelia postnatally may support the hydrodynamic hypothesis of myelodysplasia.
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PMID:Meningomyelocele and progressive hydromyelia. Progressive paresis in myelodysplasia. 109 75

Many children with myelodysplasia are ideal candidates for intermittent nonsterile urethral catheterization to manage urinary incontinence. This method of urinary management becomes no permanent commitment and is preferred by patients and parents to supravesical diversion when successful.
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PMID:Intermittent catheterization in children. 111 57

In thirty-nine patients with myelodysplasia at the third, fourth, or fifth lumbar level and subluxation or dislocation of the hip (fifty-nine hips involved), the following surgical approaches were utilized to balance the muscles about the hip: In one group (twenty hips in eleven patients) the origins of the adductors longus, brevis, and gracilis were transferred to the ischial tuberosity, and in fifteen hips in that group the iliopsoas also was transferred to the greater trochanter when the abductors were severely weakened. In the second group (thirty-nine hips in twenty-eight patients), only the iliopsoas transfer was done. Group I patients demonstrated improved muscle balance with less severe flexion-adduction contractures; increased power in abduction and extension; and a higher incidence of stable hips at follow-up.
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PMID:Paralytic dislocation of the hip in myelodysplasia. The role of the adductor transfer. 114 Dec 60

In a group of 44 patients with primary and secondary aplastic anemia, four (9%) developed an acute leukemia. Cytological changes that pointed to a preleukemia were not observed during the preleukemic stage. The possibility is discussed whether a aplastic anemia and a preleukemia can be distinguished by cytological, cytochemical and cytogenetic tests, by agar-culture technique, liquid-culture and determination of reverse transcriptase.
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PMID:[Aplastic anemia-a preleukemic stage (author's transl)]. 119 58

The case of a 40-year-old patient with congenital trisomy 8 and sex chromosome mosaicism is discussed. The main clinical features were: mental retardation, thick and darkly pigmented skin, prominent forehead, convergent strabismus, high arched palate, flexion contractures of the extremities, and numerous skeletal abnormalities. The patient developed severe aplastic anemia followed by an interim period of preleukemia which developed into acute leukemia. Electron microscope examination of the white blood cells at the stage of the aplastic anemia showed ultrastructural abnormalities similar to those observed in other genetic disorders with a predisposition to leukemia, as well as in leukemia.
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PMID:Aplastic anemia followed by leukemia in congenital trisomy 8 mosaicism. Ultrastructural studies of polymorphonuclear cells in peripheral blood. 124 72


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