Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:2.1.1.69 (BMT)
 2,655 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this paper, we present a method that permits the application of a direct digital receiver based on undersampling techniques to NMR and MRI scanners working with incoherent excitation pulses, and we evaluate the performance of such receivers in the acquisition and reconstruction of images. The method has been tested on a BRUKER BIOSPEC BMT 47/40, and the results show that undersampling can be used to process NMR and MRI signals, and that it correctly reconstructs images without loss of information, extending the range of applications of 'digital radio' techniques to NMR and MRI systems working with high-intensity magnetic fields. We also describe a series of tests performed to validate the application of undersampling to NMR systems and an algorithm to compensate the phase fluctuations due to the incoherent excitation pulses.
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PMID:Undersampling to acquire nuclear magnetic resonance images. 1523 88

We reported a case of adolescent type adrenoleukodystrophy. A 20-year old man noticed slight hemiparesis on his right side. After admission, high level of serum VCLFA and ACTH and a point mutation in ALDP codon 606 were recognized along with white matter lesions in the left medulla, basal ganglia, splenium of the corpus callosum on brain MRI. Then we diagnosed the patient as having adolescent type adrenoleukodystrophy. We started Lorenzo's oil and seaching for BMT donor. Six months later, he received allo-BMT at our hospital. His clinical symptoms gradually deteriorated during waiting allo-BMT, and just before allo-BMT, right hemiparesis, gait disturbance, exaggerated deep tendon reflexes on his right side, right lower quadrantanopia and mild cognitive deterioration. Two months after allo-BMT, gait disturbance and right hemiparesis were alleviated. We also observed improvement of abnormal findings on brain MRI and magnetic stimulation. We believed that allo-BMT is effective for the treatment of adolescent type adrenoleukodystrophy.
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PMID:[A case of adolescent adrenoleukodystrophy with clinical improvement after allogeneic bone marrow transplantation (allo-BMT)]. 1556 81

We report a 10-yr-old boy who developed CIPS after a second allogeneic BMT for severe aplastic anemia. He received the second BMT from the same HLA-matched sibling donor 16 months after the first BMT due to secondary graft failure. The preparative regimen for the second BMT consisted of fludarabine, cyclophosphamide, and anti-thymocyte globulin. Prophylaxis for acute GVHD was tacrolimus and oral PSL. Engraftment was achieved on day 15. On day 19, he suddenly complained of intermittent pain in the bilateral lower limbs. Electric shock-like pain continued for a few minutes in succession. This intractable pain was not ameliorated by various analgesic drugs including pentazocine. MRI demonstrated bone marrow edema with high T2 signal intensity in the femur. He was diagnosed as CIPS based on his symptoms and MRI findings. The trough concentration of tacrolimus (10.1 ng/mL) at the onset of CIPS was within the therapeutic range. Bouts of severe pain naturally resolved after day 43 without the discontinuation of tacrolimus. CIPS is a rare complication in HSCT. This is the first non-malignant, and the first pediatric, case who developed CIPS after HSCT.
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PMID:Calcineurin-inhibitor-induced pain syndrome after a second allogeneic bone marrow transplantation for a child with aplastic anemia. 1878 10

The childhood cerebral form of X-linked ALD is a demyelinating disorder of the central nervous system, which rapidly leads to total disability and death. Allogeneic stem cell transplantation benefits patients who show early evidence of the demyelination. We report here a one-yr-old boy with ALD who received HLA-matched unrelated BMT in an early stage of the disease after careful planning and observation since his birth. BMT was performed when MRI began to show slight signal intensity changes in the white matter of the brain. Pretransplant conditioning consisted of fludarabine, l-PAM and TBI (2 Gy). GVHD prophylaxis consisted of cyclosporine A and short-course methotrexate. The patient showed an uneventful BMT course with fast and stable engraftment. Following BMT, the plasma levels of VLCFA decreased gradually and MRI changes improved. The patient did not have any evidence of further neurological deterioration 22 months following the transplant. Although this is still a short follow-up, it has been shown that BMT should be considered when a child has a biochemical diagnosis and MRI findings of ALD without any neurological signs. RIST should be considered as a pretransplant conditioning for ALD.
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PMID:Successful allogeneic unrelated bone marrow transplantation using reduced-intensity conditioning for the treatment of X-linked adrenoleukodystrophy in a one-yr-old boy. 1882 5

ADEM is a rare inflammatory demyelinating disease of the CNS, which usually presents after a viral infection or a vaccination. We report a 15-yr-old boy who was diagnosed with ADEM after an HLA-identical sibling allogeneic BMT for transfusion-dependent PRCA. His course was complicated with GVHD affecting the skin and lungs. Five months post-BMT, he developed neurological symptoms including sudden mental status alteration, dysarthria, facial nerve palsy, and acute paraplegia. The MRI revealed multifocal hyperintense lesions mainly in the subcortical white matter of the cerebrum, the brainstem, the basal ganglia, and the thalami. CSF examination was normal. There was no laboratory evidence of infection. The typical MRI findings and an acute monophasic clinical course were consistent with the diagnosis of ADEM. Clinical and radiological improvement was observed after treatment with high-dose steroids and IVIG. Complete neurologic recovery was achieved six months after the onset of symptoms. We present a rare case of ADEM post-BMT and review of the literature.
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PMID:Acute disseminated encephalomyelitis after allogeneic bone marrow transplantation for pure red cell aplasia - a case report and review of the literature. 2321 73


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