Abstract:
:Malignant osteopetrosis (MOP) is an autosomal recessive disease in which osteoclast dysfunction results in excessive bone deposition and early infant death. Thirteen children suffering from MOP from four related families all belonging to one Bedouin tribe, were studied. The disease was diagnosed as early as at a few days postnatal to 5 months. Nine children underwent BMT, four of whom are still alive; one is blind and two have markedly reduced vision. Four children who did not undergo BMT died between 4 and 6 months of age. Recently, the gene for MOP has been mapped for this Bedouin tribe allowing prenatal diagnosis. Seven pregnancies were subsequently prenatally diagnosed and two fetuses were found to be affected. Pregnancy was electively terminated in one case. In the other case the parents refused and after establishing the diagnosis, the newborn was transplanted at the age of 7 days.
journal_name
Bone Marrow Transplantjournal_title
Bone marrow transplantationauthors
Kapelushnik J,Shalev C,Yaniv I,Aker M,Carmi R,Cohen Z,Mozer A,Schulman C,Stein G,Or Rdoi
10.1038/sj.bmt.1702743subject
Has Abstractpub_date
2001-01-01 00:00:00pages
129-32issue
2eissn
0268-3369issn
1476-5365journal_volume
27pub_type
杂志文章abstract::The effect of prophylactic intravenous administration of a cytomegalovirus (CMV) hyperimmune globulin with a high titer of neutralizing antibodies plus oral acyclovir was studied in 93 consecutive bone marrow transplant recipients. In spite of receiving blood products unscreened for CMV only six patients developed CMV...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:
更新日期:1988-11-01 00:00:00
abstract::One hundred patients with chronic myeloid leukemia (CML) submitted to bone marrow transplantation (BMT) were included in a cooperative cytogenetic study. Relapse (defined on the basis of hematological and cytogenetic findings) occurred in 24 (24%) patients at different intervals after BMT. In 18 of these patients (stu...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:
更新日期:1988-09-01 00:00:00
abstract::Thirty adults with leukemia or lymphoma transplanted with marrow or blood stem cells from 1-antigen mismatched related donors received tacrolimus and minidose methotrexate to prevent acute graft-versus-host disease (GVHD). The group had a median age of 42 years (range 18-56 years). Twenty-seven patients had advanced d...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/sj.bmt.1701983
更新日期:1999-10-01 00:00:00
abstract::Whereas patients with multiple myeloma continue to relapse after autologous transplantation and are unlikely to be cured, the probability of progression is less after allogeneic transplantation and a proportion of patients may be cured. This is attributable to an immunologically mediated graft-versus-myeloma (GVM) eff...
journal_title:Bone marrow transplantation
pub_type: 杂志文章,评审
doi:10.1038/sj.bmt.1701459
更新日期:1998-11-01 00:00:00
abstract::Forty-eight adult patients with recurrent or refractory intermediate grade or immunoblastic lymphoma received high-dose carmustine (BCNU), etoposide, Ara C and cyclophosphamide (BEAC), followed by autologous bone marrow transplantation (BMT). Median follow-up is 906 days (range 613-2067 days). The complete remission r...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:
更新日期:1995-04-01 00:00:00
abstract::Sequential administration of high-dose chemotherapy courses possibly allows extensive in vivo purging before circulating progenitor collection for autograft. To evaluate whether progenitor cell mobilization was negatively affected by repeated high-dose chemotherapy courses, we studied 23 lymphoma patients undergoing t...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:
更新日期:1995-08-01 00:00:00
abstract::The Japan Marrow Donor Program (JMDP), established in 1991, has continued to grow in its capacity to facilitate unrelated bone marrow (BMT) and peripheral blood stem cell transplantation (PBSCT) for the past 25 years in Japan. The current donor pool is 463,465 (as of 31 December 2016) and 20,237 transplants were perfo...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/s41409-017-0084-2
更新日期:2018-05-01 00:00:00
abstract::Basic fibroblast growth factor (bFGF) inhibits radiation-induced apoptosis, and radioprotects haematopoietic, cartilage growth plate, pulmonary and gastrointestinal tissues. Conversely, chronic overexpression of bFGF may promote fibrosis. We measured the endogenous circulating bFGF in blood of patients undergoing cond...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/sj.bmt.1701778
更新日期:1999-06-01 00:00:00
abstract::Abnormalities in liver function tests are common in hematopoietic SCT (HSCT) recipients. We retrospectively investigated the role of liver biopsy in determining the cause of elevated liver enzymes and its impact on the management of patients in the post-HSCT setting. A total of 24 consecutive liver biopsies were obtai...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/bmt.2008.193
更新日期:2008-10-01 00:00:00
abstract::We investigated the effect of 0.05% topical cyclosporine (Cys) on the ocular surface and tear functions in dry eye patients with chronic GVHD (cGVHD) in a prospective comparative study. Thirty eyes of 15 patients refractory to baseline treatment were recruited and the patients assigned for topical Cys treatment group ...
journal_title:Bone marrow transplantation
pub_type: 临床试验,杂志文章
doi:10.1038/sj.bmt.1705900
更新日期:2008-02-01 00:00:00
abstract::Allogeneic hematopoietic stem cell transplantation (SCT) from unaffected donors remains the only modality for the correction of hematological abnormalities in Fanconi anemia (FA) patients. We performed four HLA-matched related donor SCT using a novel irradiation and cyclophosphamide-free conditioning regimen. The prot...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/sj.bmt.1704580
更新日期:2004-08-01 00:00:00
abstract::In recent years, there has been an increasing interest in non-hematopoietic pluripotent progenitor cells that are found in the bone marrow. Mesenchymal stem cells (MSCs) are the first non-hematopoietic progenitors to be isolated from the bone marrow and extensively characterized. In addition to their ability to suppor...
journal_title:Bone marrow transplantation
pub_type: 杂志文章,评审
doi:10.1038/sj.bmt.1702791
更新日期:2001-02-01 00:00:00
abstract::The introduction of hematopoietic growth factors (HGFs) offers new opportunities for autologous transplantation by facilitating and enriching collection of circulating progenitor cells from peripheral blood as a source of stem cell rescue. Substitution of peripheral blood progenitor cells (PBPC) from bone marrow in au...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:
更新日期:1994-12-01 00:00:00
abstract::A monoclonal antibody of the IgM class, reacting with the CD9 (p24) antigen is described. The antibody (FMC27) is cytotoxic against cells of the common type of acute lymphoblastic leukaemia (c-ALL), giving killing at higher dilutions than an IgG antibody (FMC8) against the same antigen. FMC27 and FMC8 recognise differ...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:
更新日期:1987-02-01 00:00:00
abstract::One of the major aims of allogeneic haemopoietic stem cell transplantation has been the effective suppression of graft-versus-host disease (GVHD) without loss of a graft-versus-leukaemia effect. For GVHD suppression, one of the most frequently used regimens has been the combination of cyclosporin (CsA) and a short cou...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/sj.bmt.1701396
更新日期:1998-09-01 00:00:00
abstract::An infant who received haploidentical BM for severe combined immunodeficiency (SCID) developed acute, reversible complete heart block in association with an exacerbation of GVHD. Respiratory distress and myocardial dysfunction were also seen with this and previous GVHD exacerbations. The patient had not received chemo...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/sj.bmt.1701038
更新日期:1998-01-01 00:00:00
abstract::A phase I/II trial evaluated early administration and dose escalation of interleukin (IL)-2 with granulocyte macrophage colony stimulating factor (GM-CSF) post-transplant. Following melphalan (200 mg/m(2)) and an autologous transplant, IL-2 was initiated (day 0) and continued for 4 weeks. GM-CSF (250 mcg/m(2)/day) beg...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/sj.bmt.1705665
更新日期:2007-06-01 00:00:00
abstract::Myeloablative chemotherapy with autologous hematopoietic progenitor cell rescue has been evaluated in the treatment of children and young adults with brain tumors for whom conventional therapy is either too toxic (for example, radiotherapy in infants) or ineffective (for example, recurrent malignant tumors). With this...
journal_title:Bone marrow transplantation
pub_type: 杂志文章,评审
doi:10.1038/sj.bmt.1705953
更新日期:2008-01-01 00:00:00
abstract::As low trough levels of mycophenolic acid (MPA) have been measured in recipients of allo-SCTs, we performed a pilot study targeting mycophenolate mofetil (MMF) doses according to the MPA area under the concentration (AUC) levels. Twenty-nine patients were transplanted from matched sibling (n=7) and unrelated donors (n...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/bmt.2008.85
更新日期:2008-07-01 00:00:00
abstract::Three patients developed abdominal pain and abnormal liver enzymes without hyperbilirubinemia, early after autografting for lymphoma. Two had received conditioning therapy with busulfan, cyclophosphamide and continuous infusion etoposide; the other had received busulfan and melphalan. Doppler ultrasound in all cases d...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:
更新日期:1996-11-01 00:00:00
abstract::We report an 11-year old female with myelodysplastic (refractory anemia with excess of blasts) presentation of Fanconi anemia. After failure of initial chemotherapy with low doses of 6-mercaptopurine and prednisolone she underwent allogeneic bone marrow transplantation (BMT) from her HLA-matched sibling. Busulfan 8 mg...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/sj.bmt.1700662
更新日期:1997-02-01 00:00:00
abstract::We retrospectively analyzed the outcome of 67 patients with breast (n=24), ovarian (n=11) or testicular cancer (n=32) treated for relapse after high-dose chemotherapy (HDC) and peripheral blood stem cell (PBSC) transplantation. Treatment, survival and toxicity were analyzed. Patients with breast, ovarian or testicular...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/sj.bmt.1704047
更新日期:2003-06-01 00:00:00
abstract::Current prophylaxis regimens do not provide satisfactory control of graft-versus-host disease (GVHD) in patients transplanted with unmodified marrow from an unrelated donor or from a related donor mismatched for more than one HLA antigen. Extensive T cell depletion of the donor marrow can eliminate the need for post-t...
journal_title:Bone marrow transplantation
pub_type: 杂志文章,评审
doi:
更新日期:1990-11-01 00:00:00
abstract::Recombinant human (rHu) G-CSF has been widely used to treat neutropenia and mobilize PBPCs for their autologous and allogeneic transplantation. It shortens neutropenia and thus reduces the frequency of neutropenic fever. We compared the efficiency of glycosylated rHu and non-glycosylated Hu G-CSF in mobilizing hematop...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/bmt.2009.150
更新日期:2010-02-01 00:00:00
abstract::Conditioning therapy in connection with haematopoietic SCT (HSCT) induces a disruption of the intestinal barrier function facilitating the permeation of bacteria and endotoxin through the bowel wall with subsequent increased risk of septicaemia and a worsening of GVHD in the allogeneic setting. Palifermin (recombinant...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/bmt.2008.388
更新日期:2009-05-01 00:00:00
abstract::Respiratory syncytial virus (RSV), a common cause of both upper and lower respiratory tract infection (LRTI) in infants and children, is rarely described as an infective agent in adults. It has been reported in bone marrow transplant (BMT) recipients and patients with malignancy immunosuppressed by chemotherapy. Such ...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/sj.bmt.1701091
更新日期:1998-02-01 00:00:00
abstract::Cord blood (CB) transplantation is an alternate source of human hematopoietic progenitor cells for allogeneic stem cell transplantation in children and adolescents with both malignant and nonmalignant diseases. Current limitations included delay in hematopoietic reconstitution, increased incidence of primary graft fai...
journal_title:Bone marrow transplantation
pub_type: 杂志文章,评审
doi:10.1038/bmt.2015.196
更新日期:2016-01-01 00:00:00
abstract::To evaluate the efficacy and toxicity of two different etoposide (VP-16) dosages (30 or 45 mg/kg) in combination with busulfan/cyclophosphamide as conditioning therapy followed by stem cell transplantation in acute myeloid leukemia (AML), 90 patients with AML received either 30 mg/kg (n = 60) or 45 mg/kg (n = 30) etop...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/sj.bmt.1702598
更新日期:2000-10-01 00:00:00
abstract::Extracorporeal photopheresis (ECP) was given to 23 patients with steroid-refractory acute GVHD (aGVHD, grade II (n=10), III (n=7) or IV (n=6)). The median duration of ECP was 7 months (1-33) and the median number of ECP cycles in each patient was 10. Twelve patients (52%) had complete responses. Eleven patients (48%) ...
journal_title:Bone marrow transplantation
pub_type: 杂志文章
doi:10.1038/bmt.2008.221
更新日期:2008-11-01 00:00:00
abstract::The management of acute leukaemia which relapses following an allogeneic stem cell transplant remains a major challenge. In this review we summarize the outcomes of the currently available treatment modalities and discuss emerging novel approaches. ...
journal_title:Bone marrow transplantation
pub_type: 杂志文章,评审
doi:10.1038/sj.bmt.1705888
更新日期:2008-03-01 00:00:00