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

Most, but not all, megaloblastic anemia is produced by "ineffective erythropoiesis" in the bone marrow due to either folic acid or vitamin B12 deficiency. In folic acid deficiency the cause frequently is inadequate dietary intake, whereas vitamin B12 deficiency is almost always conditioned by some specific type of malabsorption. Anemia with oval macrocytes, few reticulocytes, moderate leukopenia, and thrombocytopenia is typical of both. Aplastic anemia, refractory anemias with cellular marrow, preleukemia, aleukemia, and erythroleukemia may have somewhat similar blood findings but are usually recognizable from bone marrow biopsy. Decreased levels of folate or vitamin B12 are the most reliable criteria of megaloblastic anemia. With these available in advance, therapy with the appropriate vitamin can be begun at once. If serum levels are unavailable or available only in retrospect, initial treatment, especially of severe anemia, should be with both vitamins. Differentiation between folate and vitamin B12 deficiency is important but impossible by blood and bone marrow morphology alone. Thus, if serum levels are unavailable, the distinction must be made, sometimes retrospectively, on the basis of other laboratory examinations, such as gastric analysis, small-bowel x-ray films, and the Schilling test.
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PMID:Megaloblastic anemia. 70 1

Conditions that are often associated with an increased incidence of renal anomalies include imperforate anus, congenital vertebral abnormalities, and Fanconi anemia; excretory urography should be done if such a condition is present. Urography is also useful to provide baseline data in conditions associated with later development of urinary problems, such as myelodysplasia, prune-belly syndrome, and exstrophy of the bladder. In addition, urography serves as a periodic check for complications of treatment (hydronephrosis, obstruction) in patients with urinary diversion. Certain signs, eg, dribbling, hematuria after mild trauma, unexplained pneumothorax or pneumomediastinum in a neonate, and neonatal abdominal mass, call for immediate urography. In many conditions that were formerly thought to be associated with major urinary abnormalities, urography is not called for. Such is the case in hypospadias, deformities of the external ear alone, and undescended testes. Dehydration is the only absolute contraindication to urography.
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PMID:Urography in children: when should it be done? 2. Conditions other than infection. 71 36

The prognostic value of marrow chromosome studies was examined in 112 "preleukemic" patients followed for at least one year or until death. Based on recent definitions, 49 patients were classified as myeloproliferative disorders (MPD) (polycythemia vera, myelofibrosis, undifferentiated myeloproliferative disorder, essential thrombocythemia), and 58 as cytopenic states (refractory anemia, pancytopenia). In each group, approximately one-third had a chromosomally-abnormal clone. For MPD, this had little predictive value, but in the cytopenias, 77% with a cytogenetic abnormality developed leukemia versus 39% without. Twelve cytopenic patients had multiple alterations involving more than 2 chromosomes and 11 died within 6 months, 9 with leukemia. Such patients may warrant consideration for aggressive chemotherapy before the appearance of clinical leukemia. Banding studies did not reveal any specific chromosome abnormalities consistently associated with these various preleukemic disorders, or with progression to leukemia, but nonrandom alterations were noted involving chromosomes 1, 5, 7-9, and 20 in the MPD group, and chromosomes 6 and 16 in the cytopenic patients. Correlation of these data with other reports indicates that certain cytogenetic abnormalities involving specific segments of the human genome confer a selective growth advantage on hemic clones which may present clinically as either preleukemia or leukemia.
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PMID:Chromosome studies in preleukemic states. IV. Myeloproliferative versus cytopenic disorders. 71 5

Computerized axial tomography is a safe, non-invasive technique for measurement of ventricular size. A review of 44 children without myelodysplasia who were evaluated for inappropriately enlarging heads, documented a group of 20 children presenting at five months to two years of age with megalencephaly or mild communicating hydrocephalus. Of these, only two required shunts. At their most recent examination, the great majority of these children are developmentally normal. Serial neurological examination, developmental evaluation and head-circumference measurements, in association with computerized axial tomography, have proved effective in the assessment and management of this particular group of children with large heads.
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PMID:Re-evaluation of the approach to an enlarging head in infancy. 72 27

A Sharrard posterior iliopsoas muscle-transfer procedure was done in forty-two hips of twenty-four children with myelodysplasia. Folow-up ranged from twenty-nine months to approximately ten years. An over-all success rate of 50 per cent was achieved which included all hips operated on for potential and existing instability. The success rate for surgery in hips with an existing instability was 29.6 per cent. Factors predisposing to a poor result were: age more than five years old, dysplastic acetabulum (index more than 30 degrees), prior hip surgery, and inequality of limb length. This operation is recommended primarily for hips not previously operated on in children less than five years old who have a normal acetabular index. If the child is more than five years old or if acetabular dysplasia exists, reconstruction of the acetabulum should precede or accompany the tendon transfer for best results. Iliopsoas muscle transfer is unlikely to be successful if used as a salvage procedure.
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PMID:Posterior iliopsoas muscle transfer in myelodysplasia. 75 34

The natural course of disease was followed in 33 patients with so-called preleukemia by a prospective multicentric protocol. Patients with the following criteria were included: Anaemia with granulocytopenia and/or thrombocytopenia, normal or increased cellularity of the bone marrow and exclusion of a known diagnosis of underlying disease. Follow up after assumption of "preleukemia" was 3 years or more. Median survival was 26 months after diagnosis of preleukemia and 36 months after the first unequivocal symptoms of the blood dyscrasia. 40% of the patients changed to the picture of leukemia within 2 years. The most important parameters suggesting subsequent transition to overt leukemia where chromosomal abberations and an increased blast count of the bone marrow.
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PMID:[A prospective study on course and prognostic criteria in "preleukemia" (author's transl)]. 75 14

Problems of ninety-eight patients with myelodysplasia, ages 13 to 72, were reviewed. They were grouped as follows: Those having thoracic and high lumbar level (L(2) upward arrow) lesions and confined to wheel chairs, those with intermediate paralysis (L(3-5) nerve roots) as walking with aids and those with less paralysis (S(1) downward arrow) as fully ambulatory. Fifty-two percent of the L(2) upward arrow and only 15 percent of of the less severely paralyzed patients were retarded below an IQ level 70 (P<0.01). Thirty-six patients (62 percent) were fully and 26 partially, but appropriately, self-sufficient. Thirty-six patients were found in some form of dependent care. Two of the 71 more paralyzed patients (L(3-5) and L(2) upward arrow) and five of the 28 S(1) downward arrow patients were "naturally continent" but reported stress incontinence of urine. Thirteen of 23 female and five of 28 male patients between ages 16 and 72 years reported sexual activity and accounted for 17 normal offspring. All 23 retarded patients were in some form of custodial care. Dependency among the normal intellect patients could be attributed to neglect of physically deforming complications and emotional disorders, primarily low self-esteem centering around social and sexual identity problems associated with excrement soiling.
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PMID:Myelodysplasia. Problems of long-term survival and social function. 80 42

The distribution of various types of leukemia due to chronic exposure to benzene is described in a series comprising 34 cases. The incidence of leukemia among 31 show-workers was 13.5/100,000. Acute myeloblastic leukemia was the most frequent type, followed by preleukemia, acute erythroleukemia and acute lymphoblastic leukemia. The extreme rarity of chronic myeloid leukemia was a noteworthy finding. The differences and similarities between the distribution of various types of leukemia in different series of patients with chronic exposure to benzene and ionizing radiation are discussed.
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PMID:Types of leukemia in chronic benzene poisoning. A study in thirty-four patients. 81 44

The triple osteotomy of the innominate bone is a total redirectional reconstruction and is obtained by circum-acetabular osteotomies through the ilium, ischium and pubis. The method achieves coverage of a dislocated or subluxated femoral head where other iliac osteotomies are ineffective or incomplete. Triple osteotomy was performed on 175 hips including 121 congenital dislocation-subluxations. The patients' ages ranged from 6 to 35, with 70 per cent falling between 9 and 12. Eighty-six per cent had a satisfactory result. Most of the unsatisfactory results were in cerebral palsy, myelodysplasia and peroneal muscular atrophy and associated with uncontrolled muscle contractures or progressive disease.
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PMID:Triple osteotomy of the innominate bone. A procedure to accomplish coverage of the dislocated or subluxated femoral head in the older patient. 83 97

Urodynamic evaluation was performed in 46 children with myelodysplasia, spinal cord injury, enuresis, postoperative incontinece, sacral agenesis and recurrent urinary tract infection. The basic study consisted of voiding cystourethrography followed by the simultaneous recording of intra-abdominal and intravesical pressure with external urethral sphincter electromyography. Urethral pressure profile, urinary flow rate and anal sphincter electromyography were performed selectively. There was no correlation between the clinical neurological level and the cystometric pattern in patients with spinal cord lesions. Bladder-external sphincter dyssynergia was associated with a high post-void residual urine in children with hypertonic bladders, while in children with hypotonic bladders dyssynergia did not affect the post-void residual. Since, urinary symptoms bore little relationship to urodynamic findings in our study we recommend complete evaluation of children who have persistent disturbances of micturition.
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PMID:Changing concepts in the urodynamic evaluation of children. 87 55


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