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)

In patients with idiopathic aplastic anaemia (n = 34) and Fanconi's anaemia (n = 8), sampled once or on several occasions, serum erythropoietin (Epo) increased with increasing severity of anaemia with apparently similar rates of increase in each group. However, after adjustment for Hb, log Epo values for the Fanconi's anaemics tended to be greater than those for the idiopathic aplastic anaemics (P < 0.01). Erythropoietin concentrations in serum samples from patients with Fanconi's and idiopathic aplastic anaemias tended to be greater than in samples from patients with anaemias from protein energy malnutrition, myelodysplasia and iron deficiency. The results suggest that there is no deficiency of erythropoietin in Fanconi's and idiopathic aplastic anaemias and that if exogenous erythropoietin is of any benefit it would need to be administered in doses large enough to induce a significant increase in log Epo. Results of the study illustrate the need to take account of the assumptions which underlie interpretation of the statistical analysis. Use of erythropoietin values in place of log Epo gives misleading conclusions demonstrable as invalid as the conditions for normality of distribution of the data and homogeneity of variances were not satisfied.
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PMID:Serum immunoreactive erythropoietin in patients with idiopathic aplastic and Fanconi's anaemias. 148 41

20 cases of myelodysplastic syndrome (MDS) patients were treated with Yisuikang (YSK) including MDS-RA 17 cases, MDS-RAEB 2 cases, MDS-RAEB-T 1 case. 14 patients were treated with YSK only, 6 cases were treated with both YSK and Western medicine. The result showed that 4 cases were remitted, 5 cases markedly improved, 6 cases improved and 5 cases ineffective, the total effective rate was 75.0%. It was found that YSK was effective in the treatment of MDS-RA or Qi-Blood Deficiency and Yu-Re Syndrome in TCM, while any other types were ineffective. They also found that types of TCM might be correlated with MDS.
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PMID:[Clinical observation on treatment of myelodysplastic syndromes with yisuikang]. 778 94

Forty two patients who were seen and satisfied the French-American-British (FAB) diagnostic criteria for myelodysplastic syndromes (MDS) over a 6-year period at the University of Zimbabwe's Department of Haematology, Harare, are presented. Their overall ages ranged from 29 to 75 years with a mean +/- SD of 57.8 +/- 11.2 years. Males outnumbered females with a male to female ratio of 1.2:1. Refractory anaemia (RA) occurred in 33.3%; refractory anaemia with ringed sideroblasts (RARS) in 16.7%; refractory anaemia with excess blasts (RAEB) in 21.4%; refractory anaemia with excess blasts in transformation (RAEB-T) in 16.7% and chronic myelomonocytic leukaemia (CMML) in 11.9% of the patients. In 90.5% the disease was primary and in 9.5% prior exposure to myelotoxic agents resulted in secondary MDS. The study reveals that MDS as a cause of anaemia in the African population is usually hidden in the big number of well known anaemias due to rampant malaria, malnutrition and a host of nutritional deficiencies. There is therefore the need to increase diagnostic awareness among our clinicians about the existence of these disorders.
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PMID:Myelodysplastic syndromes (MDS) in Central Africans. 816 30

I retrospectively reviewed the records of 111 patients who had pyogenic vertebral osteomyelitis unrelated to an open procedure on the spine. The mean age at the time of the diagnosis was sixty years (range, eighteen to eighty-four years); sixty-one patients (55 per cent) were sixty years old or more. Forty-four patients (40 per cent) had an impaired immune system secondary to diabetes mellitus, the use of corticosteroids, chemotherapy for cancer, rheumatic or immunological disease, renal or hepatic failure, malnutrition, or myelodysplasia. Magnetic resonance imaging, critical for the determination of an early diagnosis, was performed for 103 patients (93 per cent). The infection in sixty-eight patients (61 per cent) was diagnosed within one month after the onset of symptoms. The most frequent infecting organism was Staphylococcus aureus (forty patients; 36 per cent). The infection in forty-one patients (37 per cent) was caused by organisms, such as Staphylococcus epidermidis, Propionibacterium acnes, and diphtheroid species, that are traditionally considered to be of low virulence. The urinary tract was the most frequent source of infection (confirmed in thirteen patients and suspected in twenty-one). The success of non-operative treatment was predicted by four independent variables: an age of less than sixty years, the immune status, infection with Staphylococcus aureus, and a decreasing erythrocyte sedimentation rate. Forty-two patients were managed with debridement and arthrodesis. Fourteen of these patients also had instrumentation of the spine, in the presence of infection, without compromise of the outcome. Eighteen patients died by the time of the latest follow-up evaluation at a mean of four years (range, two years and two months to six years and six months): seven who had been managed non-operatively died in the first month after the diagnosis was made, three died in the acute postoperative period, three died of late complications of paraplegia, and five died of unrelated causes. None of the eighty-nine patients who were seen at a minimum of two years postoperatively had had late recurrence of infection. Chronic, severe back pain was noted in only seven patients.
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PMID:Pyogenic vertebral osteomyelitis. 961 Oct 38

Azacitidine pharmacokinetic parameters in adolescent patients with renal compromise are not available from the medical literature. We describe a 14-year-old with myelodysplastic syndrome treated with subcutaneous 5-azacitidine for disease relapse 2 years after hematopoietic stem cell transplant. Because of renal compromise, malnutrition, and poor functional status, pharmacokinetic parameters were projected from existing literature data to select the patient's first azacitidine treatment course (1.5 mg/kg/d for 7 d). Posttreatment azacitidine plasma concentrations used to calculate patient-specific pharmacokinetic parameters corroborated initial estimates and systemic exposure associated with therapeutic benefit in adults and permitted individualization of treatment.
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PMID:Azacitidine pharmacokinetics in an adolescent patient with renal compromise. 1748 13

Anemia signifies an underlying disease and is associated with poor clinical outcomes. In elderly patients, in whom anemia has a high prevalence (greater than 10 percent), neither the hemoglobin threshold for concern nor the identity of the anemia-causing disease is easily established. This is an important shortfall, because even mild anemia can compromise patient well-being and survival, regardless of the underlying cause. This review discusses definitions of "normal" hemoglobin levels in adults, common causes of anemia in people aged 65 years and older (eg, nutritional deficiency, renal insufficiency, inflammatory disorders, and myelodysplastic syndrome), and potential consequences of anemia in elderly patients (eg, poorer cognitive status, increased frailty, and an elevated risk of hospitalization and of complications during hospitalization). We also outline a practical initial diagnostic approach that helps determine appropriate treatment, and we weigh therapeutic options in light of new safety concerns regarding erythropoiesis-stimulating agents.
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PMID:Anemia in the elderly: how should we define it, when does it matter, and what can be done? 1831 6

More than 50% of children with cancer in Guatemala have some degree of malnutrition at diagnosis. In particular, the proportions of patients with non-lymphoblastic leukemias and myelodysplasia (MDS) with moderate and severe malnutrition were 73% and 9% respectively; higher than that in the population with acute lymphoblastic leukemia (ALL), P = 0.0493. There was no relationship of nutritional status to gender. Children with ALL and other leukemias + MDS who had severe malnutrition were significantly younger than such malnourished children with solid tumors (P = 0.0003). The relationship of malnutrition to race was mixed, perhaps reflecting the frequent difficulty of accurately categorizing race.
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PMID:Nutritional status at diagnosis in children and adolescents with cancer in the Asociacion de Hemato-Oncologia Pediatrica de Centro America (AHOPCA) countries: preliminary results from Guatemala. 1806 63

Mucormycosis (zygomycosis) is an acute and often fatal opportunistic fungal infection. Predisposing factors in the development of mucormycosis are nonspecific and include hyperglycemia, hematologic malignancies, neutropenia, pharmacologic immunosuppression, solid organ or bone marrow/stem cell transplantation, burns, trauma, malnutrition, and intravenous drug use. Mucormycosis has also been described in patients with iron and aluminum overload, patients on dialysis, and patients receiving iron chelating therapy. We describe a 75-year-old man with myelodysplastic syndrome and iron overload secondary to multiple red blood cell transfusions who had been treated with deferoxamine chelation therapy. He was admitted to the hospital for atrial fibrillation, developed multiple organ failure, and died. Pulmonary invasive mucormycosis was demonstrated at autopsy. This case further documents an association between invasive mucormycosis, iron overload, and deferoxamine therapy.
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PMID:Pulmonary invasive mucormycosis in a patient with secondary iron overload following deferoxamine therapy. 1898 78

Deficiency in glucose-6-phosphate dehydrogenase (G6PD), an X-linked recessive red cell enzymopathy, is endemic in Southern Chinese. Universal screening of newborn is done in Hong Kong, Taiwan and Singapore, among other places. In Hong Kong, 4.8% of males are affected and seven common G6PD alleles account for over 99% of all defects. Male hemizygotes suffer from severe deficiency, while female heterozygotes may also be affected. Deficiency of G6PD may affect haematopoietic stem cell transplantation (HSCT) recipients and donors, before and after HSCT. Female patients with clonal erythropoiesis (eg myelodysplasia/myeloproliferative diseases) will have the male population incidence of G6PD. Quantitative enzyme level screening is prudent for donors and recipients, and should be repeated after engraftment. Cotrimoxazole prophylaxis should be avoided in known male and female carriers, including those with low-normal G6PD enzyme levels. Our experience suggested that G6PD-deficient marrow, stem cell and cord blood donor units have no engraftment problems. Post-engraftment G6PD levels correlate with those in donors. An acquired change in G6PD status may serve as a surrogate marker for engraftment. For female heterozygote donors with normal G6PD levels, skewing of lyonized X-chromosome ratio during engraftment may result in over-expression of the deficient allele. This can result in unexpected significant G6PD deficiency. Hence, a repeat G6PD screening at stable engraftment is recommended, especially before commencement of oxidative medications.
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PMID:Glucose-6-phosphate-dehydrogenase deficiency and haematopoietic stem cell transplantation in Chinese patients. 1949 95

Anemia is common in the elderly, especially in very old patients who are often frail and may be institutionalized. Senescence, the ageing process, puts the elderly at risk of developing anemia for multiple reasons, but anemia may not be attributed to senescence unless a thorough diagnostic workup has excluded other etiologies. Nutritional deficiencies are common and need to be identified and treated appropriately. Inflammatory diseases and renal failure are also frequent etiological factors and tend to be chronic. Myelodysplastic syndromes increase in frequency with age and may be difficult to diagnose and only a minority of cases respond to appropriate treatment. Anemia is associated with poor outcome and symptomatic treatment with transfusions frequently has to be considered. Red blood cell transfusion has a high therapeutic index and is likely to be effective only if anemia is symptomatic or particularly severe, as a consequence, its use has been restricted to this group. Much of the evidence on usage is limited to younger adults and specific clinical situations. Geriatricians have to deal with a large number of patients with significant anemia but with an absence of well constructed guidelines for the frail and the very old. The object of the present article is to raise awareness that anemia in the geriatric group is multi-factorial and that the patients are more than merely older than those included in most studies, that the results of ongoing trials should be appropriately interpreted and will be important in guiding future practice.
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PMID:Anemia and transfusions in geriatric patients: a time for evaluation. 2042 72


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