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)

Pseudomonas cepacia is a gram negative rod, having no fermentative activity on glucose. This organism was detected in the sputum, throat swab, or throat washing of 22 inpatients treated between January, 1990, and December, 1990, at the First Department of Internal Medicine, Kagawa Medical School. The primary diseases for which these 22 patients were hospitalized were leukemia in 12, malignant lymphoma in 5, lung cancer in 2, myelodysplastic syndrome in 1, and embryonal cell carcinoma in 1. Twelve of the 22 patients had episodes of pneumonia which complied clinically with the diagnostic criteria provided to facilitate the National Nosocomial Infection Study. The complication of pneumonia occurred in 7 patients with leukemia, 2 with malignant lymphoma, 2 with lung cancer, and 1 with myelodysplastic syndrome. In 10 of these 12 patients, the organism was detected before the onset of pneumonia. All 22 patients in whom the organism was demonstrated had received antibiotics. The antibiotics which was most frequently used to treat these patients 1 month before detection of Pseudomonas cepacia were amikacin and ceftizoxime, which were used in 13 patients. Of the antibiotics in which the susceptibility to Pseudomonas cepacia was, evaluated, minocycline was effective in 100% (21/21), ceftazidime in 50% (11/22), and ofloxacin in 27.3% (6/22). Physicians should be especially aware of the possibility of colonization and nosocomial respiratory infection by Pseudomonas cepacia in patients with severe underlying diseases.
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PMID:[Nosocomial respiratory infection caused by Pseudomonas cepacia in immunocompromised hosts]. 138 85

We studied the long-term in vivo effects of recombinant granulocyte-macrophage colony stimulating factor (rhGM-CSF) on granulocyte functions in nine patients with myelodysplastic syndrome (MDS). The treatment schedule consisted of a 14 d course of rhGM-CSF (250 micrograms/m2/d s.c.) for patients with refractory anaemia (RA) and refractory anaemia with ringed sideroblasts (RARS), while patients with refractory anaemia with excess of blasts (RAEB) and refractory anaemia with excess blasts in transformation (RAEBt) received a 14 d combination course of rhGM-CSF (250 micrograms/m2 s.c.) and low dose cytosine arabinoside (20 mg/m2 s.c.). rhGM-CSF increased the mean neutrophil count from 3.9 x 10(9)/l to 44 x 10(9)/l. Significant increases of myeloperoxidase content in granulocytes occurred during treatment (P = 0.003). Phagocytosis and killing of Staph. aureus by granulocytes was markedly enhanced during treatment. Microbicidal capacity normalized in four out of six patients during GM-CSF therapy. However, chemotaxis in response to zymosan-activated serum (ZAS) and f-Met-Leu-Phe (f-MLP), was further impaired on the last day of treatment, which was associated with a marked increase in the expression of the granulocyte adhesion receptors CD11a (P = 0.01), CD11b (P = 0.002), CD11c (P = 0.00015) and CD18 (P = 0.0014). GM-CSF therapy did not cause significant changes in hexose monophosphate (HMP)-shunt activity, chemiluminescence, nor superoxide production. The present results show that in vivo administration of GM-CSF is able to repair at least in part the neutrophil anomalies in patients with myelodysplastic syndrome (MDS), which might be useful in modulating host response to infections. However, increased adherence and impaired chemotaxis may explain some toxicities observed during treatment with GM-CSF.
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PMID:In vivo administration of granulocyte-macrophage colony stimulating factor enhances neutrophil function in patients with myelodysplastic syndromes. 195 74

Neutrophil function studies have been carried out in a series of 44 patients with primary myelodysplastic syndromes (MDS). In vitro tests of phagocytosis and killing of Candida guilliermondii and Staphylococcus aureus identified 13 patients with abnormal neutrophil function at presentation and a further 10 who developed abnormalities during the course of their disease. The incidence of defective function in the five disease categories in this series was: refractory cytopenia (RC) 8/17; refractory cytopenia with sideroblastic change (RC + SC) 5/8; acquired idiopathic sideroblastic anaemia (AISA) 2/4; refractory anaemia with excess blasts (RAEB) 7/11; chronic myelomonocytic leukaemia (CMML) 1/4. Eleven of 23 patients with defective neutrophil function experienced severe infective complications; in only three of these patients were neutrophil counts less than 1 X 10(9)/l and susceptibility to infection was considered to reflect, at least partially, qualitative neutrophil abnormalities. There was no correlation between absolute neutrophil count and defective function. Abnormal overall neutrophil microbicidal activity was equally associated with impaired and normal phagocytosis. Some patients with intracellular killing defects had reduced myeloperoxidase (MPO) activities and one had reduced hexose monophosphate shunt (HMPS) activity. In two patients, whose neutrophils showed markedly impaired candidacidal activity, levamisole corrected function when added in vitro at 10(-7) M and also when administered in therapeutic dosage. It is suggested that deranged function, probably reflecting abnormalities in maturation of the granulocyte series, occurs across the myelodysplastic spectrum and that several microbicidal mechanisms may be defective.
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PMID:Defective neutrophil function and microbicidal mechanisms in the myelodysplastic disorders. 631 78

A 67-year-old woman investigated because of 'myelodysplastic syndrome' was found to have a 4-fold increase in G-6-PD activity in her erythrocytes. The enzyme was partially purified and characterized. On grounds of: (a) reduced electrophoretic mobility, (b) abnormal cathodic band(s) in isoelectrofocusing, (c) increased Michaelis constant for glucose 6-phosphate, (d) abnormal thermostability, and (e) abnormal interaction with the ligand NADPH, we conclude that this is a new structural variant which we designate G-6-PD Verona. G-6-PD Verona was the sole apparent source of G-6-PD activity in the patient's erythrocytes; by contrast, the patient's fibroblasts had only normal G-6-PH (type B). The patient's haematological course terminated into acute myeloid leukaemia. We believe G-6-PD Verona was the result of a somatic mutation in an X-chromosome which took place in a haemopoietic cell clone which subsequently underwent neoplastic transformation.
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PMID:A new glucose 6-phosphate dehydrogenase variant (G-6-PD Verona) in a patient with myelodysplastic syndrome. 657 92

A 33 year-old female patient presented with apparent skin pigmentation, sustained liver dysfunction and impaired glucose tolerance. She had received blood transfusions totalling more than 40,000 ml for myelodysplastic syndrome and an allogeneic bone marrow transplant from her HLA-matched sister at the age of 31. Ferrokinetic data showed a significant iron overload. Magnetic resonance imaging suggested excessive iron deposition in the liver. The patient was diagnosed as having secondary hemochromatosis. She was given subcutaneous injections of 6,000 units of recombinant human erythropoietin initially twice a week and then weekly, and phlebotomies were performed to maintain her hemoglobin level above 10 g/dl. Three years later, the total volume of phlebotomized blood reached 24,000 ml, and her ferrokinetic data, serum transaminase levels, glucose tolerance and skin color were significantly improved.
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PMID:[Successful treatment using iron depletion phlebotomy combined with recombinant erythropoietin after allogeneic bone marrow transplantation for myelodysplastic syndrome complicated by secondary hemochromatosis]. 1152 49

We report here a patient who suffered from PCR-confirmed human herpesvirus type 6 (HHV-6) encephalitis following reduced intensity stem cell transplantation (RIST) from her HLA-matched sibling donor. A 66-year-old woman with MDS-RA underwent RIST from her HLA-matched brother. Engraftment was favorable and grade 2 GVHD (skin and intestine) was observed with good response to 60 mg of prednisolone. On day 162, she developed fever, headache, diplopia, disorientation and abnormal neurological findings including cervical stiffness and nystagmus. An analysis of cerebrospinal fluid (CSF) revealed 80 cells/microl, a glucose level of 50 mg/dl and a protein level of 97 mg/dl on day 162. Although computed tomography (CT) of the brain and electroencephalography (EEG) were nonspecific, HHV-6 was detected in the CSF using polymerase chain reaction (PCR) techniques and the patient was diagnosed as having encephalitis due to local reactivation of HHV-6. Administration of ganciclovir (GCV) and acyclovir (ACV) were started from day 162. Treatment with antiviral agents was effective, with total resolution of her symptoms and the DNA of this virus disappeared from the CSF after 23 days of treatment. This case shows that HHV-6 infection has to be considered in patients with neurological symptoms following stem cell transplantation, and suggests the necessity of PCR for HHV-6 virus from the CSF.
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PMID:[Successful treatment with antiviral agents for human herpesvirus type 6 encephalitis following reduced intensity stem cell transplantation in a patient with myelodysplastic syndrome]. 1528 24

We report a fatal case of disseminated zygomycosis due to Cunninghamella bertholletiae in a 68-year-old man with myelodysplasia and type II diabetes mellitus, receiving desferrioxamine therapy for iron overload secondary to multiple transfusions. It is thought that he acquired the infection through the use of blood glucose self-monitoring equipment.
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PMID:A fatal case of disseminated zygomycosis associated with the use of blood glucose self-monitoring equipment. 1590 67

CD34(+) bone marrow blasts from high-risk myelodysplastic syndrome (MDS) patients as well as MDS patient-derived cell lines (P39 and MOLM13) constitutively activate the nuclear factor-kappaB (NF-kappaB) pathway and undergo apoptosis when NF-kappaB is inhibited. Here, we show that the combination of conventional chemotherapeutic agents (daunorubicin, mitoxantrone, 5-azacytidine or camptothecin) with the NF-kappaB inhibitor BAY11-7082 did not yield a synergistic cytotoxicity. In contrast, BAY11-7082 (which targets the NF-kappaB-activating I-kappaB kinase (IKK) complex) or knockdown of essential components of the NF-kappaB system (such as the IKK1 and IKK2 subunits of the IKK complex and the p65 subunit of NF-kappaB), by small interfering RNAs sensitized MDS cell lines to starvation-induced apoptosis. The combination of BAY11-7082 and nutrient depletion synergistically killed the acute myeloid leukemia (AML) cell line U937 as well as primary CD34(+) bone marrow blasts from AML and high-risk MDS patients. The synergistic killing by BAY11-7082, combined with nutrient depletion, led to cell death accompanied by all hallmarks of apoptosis, including an early loss of the mitochondrial transmembrane potential, the release of cytochrome c and apoptosis-inducing factor (AIF) from mitochondria, activation of caspase-3, phosphatidylserine exposure on the plasma membrane surface and nuclear chromatin condensation. Transmission electron microscopy revealed the presence of numerous autophagic vacuoles in the cytoplasm before cells underwent nuclear apoptosis. Nonetheless, cell death was neither inhibited by the pan-caspase inhibitor z-VAD-fmk nor by knockdown of AIF or of essential components of the autophagy pathway (ATG5, ATG6/Beclin-1, ATG10, ATG12). In contrast, external supply of glucose, insulin or insulin-like growth factor-I could retard the cell death induced by BAY11-7082 combined with starvation. These results suggest that in MDS cells, NF-kappaB inhibition can precipitate a bioenergetic crisis that leads to an autophagic stress response followed by apoptotic cell death.
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PMID:NF-kappaB inhibition sensitizes to starvation-induced cell death in high-risk myelodysplastic syndrome and acute myeloid leukemia. 1721 4

Glucose binds irreversibly to a variety of structures, including hemoglobin and proteins, by non-enzymatic glycosylation. Glycosylated Hemoglobin A1c (HbA1c) measures the blood glucose control over the lifespan of the RBCs. The importance of routinely assessing HbA1c in diabetic patients is well established. Both individual and institutional performance in the diabetes arena may be judged by the number of patients reaching target HbA1c values. In some patients, however, the HbA1c does not accurately portray glycemic control and may delay treatment for poorly-controlled diabetes. We report on a patient in whom the HbA1c values were falsely low as a result of hemolytic anemia associated with Myelodysplastic syndrome. The patient had consistent elevation of glucose values. Fructosamine measurement was able to confirm poorly-controlled diabetes and assist in improving diabetes control. Fructosamine is unaffected by disorders of red blood cells, which have a profound potential influence on HbA1c. Fructosamine also has the advantage of accurately reflecting shorter-term changes in glycemia that correspond to the half-life of albumin. In diabetic patients with HbA1c values below the lower limit of normal, a routine Fructosamine level should be performed. We recommend a Fructosamine level should be considered in all patients with red blood cell disorders or with discrepancies between glucose measurements and HbAlc values. Fructosamine, an inexpensive assay, is currently underused in the clinical practice. A guideline for using Fructosamine levels is included and some of the pitfalls in relying solely on the HbAlc are discussed.
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PMID:Fructosamine--an underutilized tool in diabetes management: case report and literature review. 1902 48

For the patients under long-term tube feeding, copper deficiency is known to be a cause of neutropenia and/or anemia. It is recognized as reversible myelodysplasia, since the condition improves by giving a copper supplementation. Myelodysplasia caused by copper deficiency is difficult to be diagnosed because it is not so common, and often it takes a long time to reach correct diagnosis. We reported usefulness of FDG-PET for the diagnosis of myelodysplasia caused by copper deficiency in early stage. The case was 46 y.o. male patient in vegetative state for 2 years after traumatic brain injury. Laboratory examination revealed slight leukopenia. PET/CT demonstrated high and diffuse FDG accumulation mainly in the vertebral bone marrow. Based on the lower levels of serum copper and ceruloprasmin, the patient was diagnosed as copper deficiency. After treatment of copper supplementation, FDG accumulation of the bone marrow disappeared, and the serum copper level has normalized. From the FDG-PET findings, even in the early stage of copper deficiency, high glucose metabolism of bone marrow was shown.
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PMID:[Usefulness of FDG-PET for the diagnosis of copper deficiency at an early stage: a case report]. 1959 8


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