1/29. Spontaneous regression (autoinfarction) of proliferative sickle retinopathy.Of 45 patients with proliferative sickle retinopathy in stages III, IV, and V, nine patients (eight with hemoglobin sc disease, one with sickle cell thalassemia) showed spontaneous regression (autoinfarction) of retinal sea fans. One mechanism involved in autoinfarction of neovascular tissue is progressive, centripetal retraction of the anterior vascular arcade of the peripheral retina. In addition, vitreous traction on feeder vessels may result in sluggish blood flow and occlusion of these vessels, or may tear the sea fan completely away from its feeder vessels. In view of the many incidences of vitreous hemorrhages that occur in patients with proliferative retinopathy, however, we recommend treatment of neovascularization rather than prolonged observation.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/29. Rationale and results of in utero transplants of stem cells in humans.Following 18 years experience in postnatal fetal liver transplantation (FLT), we have developed a new therapeutical method, namely the in utero transplantation of stem cells from the human fetal liver. This early transplant takes advantage of the immunological tolerance that exists in young fetal recipients. The four fetuses that we treated were 28, 26, 17 and 12 weeks of age (weeks after fecundation). The first two patients had immunodeficiencies, the two other had thalassemia major. Donor cells were obtained from 7- to 12-week-old fetuses, with conditions approved by the National Committee for bioethics. Donors and recipients were not matched. The fetal cells were infused through the umbilical vein of three patients and injected intraperitoneally into the other one, under ultrasonic visualization. The first patient, bone in 1988, has evidence of engraftment and reconstitution of cell-mediated immunity: initially 10% then 26% of lymphocytes of donor origin (with distinct phenotype), T cell responses to tetanus toxoid and candida antigens. This child, who had bare lymphocyte syndrome, has no clinical manifestation of the disease and lives normally at home. The second child was born in 1989; donor cell engraftment has been proven (y chromosome in this female patient) and immunological reconstitution is in progress, allowing a normal life at home. The third patient has also evidence of donor cell take (y chromosome in a female patient) and a partial effect on thalassemia has been documented (donor haemoglobin present in small quantity). In all three cases, no side effect of any kind developed in the mother nor in the fetus.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 0.16666666666667keywords = life (Clic here for more details about this article) |
3/29. Skeletal manifestations in beta-thalassaemia intermedia.Thalassaemia intermedia with distressing complications is reported from india. An 11 year old boy presented with recurrent fractures since 6 years of age. Investigations revealed that he had multiple healed fractures of long bones, hypersplenism, iron overload and a red blood cell half life (t1/2) of 17 1/2 days. splenectomy was planned.- - - - - - - - - - ranking = 0.16666666666667keywords = life (Clic here for more details about this article) |
4/29. Spinal extradural hematopoiesis in adolescents with thalassemia. Report of two cases and a review of the literature.Extramedullary hematopoietic tissue occurring within the extramedullary space and causing cord compression is an unusual occurrence in childhood and adolescence, and only four cases have been reported during the first two decades of life. We had the opportunity to treat a 16-year-old girl and a 14-year-old boy with paraparesis secondary to extradural extramedullary hematopoiesis. Findings in these cases are discussed, and an attempt is made to stress once more the possibility of congenital hematopoietic rests as the origin of such hyperplastic tissue. The various modes of therapy are discussed and related literature is reviewed.- - - - - - - - - - ranking = 0.16666666666667keywords = life (Clic here for more details about this article) |
5/29. bone marrow transplantation in an hiv positive thalassemic child following therapy with azidothymidine.The authors report their experience on allogeneic bone marrow transplant in an hiv seropositive thalassemic child. Before transplant a treatment with Azidothymidine was performed with the aim of reducing the viral load. Engraftment took place but, later, an explosive upsurge of viral disease occurred with encephalitis, positivation of the P24 antigen, proliferation of opportunistic infections and an increase of the IgG level. Furthermore the failure of CD4 cell recovery was also observed. This case underlines that bone marrow transplantation can have a successful engraftment in hiv seropositive patients, but this doesn't modify the course of the infection.- - - - - - - - - - ranking = 0.16666666666667keywords = life (Clic here for more details about this article) |
6/29. Bimaxillary hyperplasia: the facial expression of homozygous beta-thalassemia.A boy born in Curacoa, who was 6 years old at his initial visit and known to have homozygous beta-thalassemia, is described. Emphasis is directed to the typical facial expression. The possibility of surgical treatment of the maxillary hypertrophy is discussed. However, in view of the limited life expectancy of these patients, correction should not be performed before adolescence.- - - - - - - - - - ranking = 0.16666666666667keywords = life (Clic here for more details about this article) |
7/29. Acetabular protrusion in thalassemia. A report of four cases.Four female patients with thalassemia intermedia developed secondary acetabular protrusion. These cases could be the first reported cases of nontraumatic acetabular protrusion in patients with thalassemia. The rarified pelvic bone may be due to medullary hyperplasia, iron overload, or disordered calcium metabolism in conjunction with osteomalacia. The fact that currently patients with beta-thalassemia have a longer life expectancy may explain the recent observation of this entity, which should become more familiar to orthopedic surgeons who treat thalassemia patients in the future.- - - - - - - - - - ranking = 0.16666666666667keywords = life (Clic here for more details about this article) |
8/29. Pulmonary syndrome in patients with thalassemia major receiving intravenous deferoxamine infusions.Eight patients with transfusion-dependent thalassemia major were given continuous intravenous infusions of the chelator, deferoxamine mesylate, to reduce iron overload. Within 5 to 9 days of starting the infusions, four patients developed a pulmonary syndrome of moderate to life-threatening severity characterized by tachypnea, hypoxemia, and a diffuse interstitial pattern on chest roentgenogram. Pulmonary function studies showed restrictive dysfunction. lung biopsy showed diffuse abnormalities with alveolar damage, interstitial fibrosis, and inflammation. The inflammatory infiltrate comprised lymphocytes, eosinophils, and mast cells. Exposure of the biopsy specimen to fluorescein-conjugated anti-IgE antibody showed fixation of IgE to the mast cell surface, suggesting a hypersensitivity reaction. Detailed studies failed to identify an infectious agent. The temporal relationship between drug administration and lung disease, and the clinical similarities in the four affected patients, strongly suggested a cause and effect relationship. We recommend that therapy with continuous intravenous infusions of deferoxamine be monitored carefully with respect to pulmonary status.- - - - - - - - - - ranking = 0.16666666666667keywords = life (Clic here for more details about this article) |
9/29. A benign sickle-cell disease in a Saudi subject with beta zero-thalassemia and glucose-6-phosphate dehydrogenase deficiency.Sickle-cell disease with raised fetal hemoglobin is found relatively frequently in the eastern part of the Arabian Peninsula. In contrast to the severe and sometimes life-threatening complications of sickle-cell disease in the black population, Saudi arabs homozygotes for HbS gene exhibit a mild course for this disease. Here we present a Saudi sickle-cell patient with an unusually low fetal hemoglobin level. Moreover, this individual has beta 0-thalassemia and a deficiency in the enzyme glucose-6-phosphate dehydrogenase. Clinical and hematological examinations revealed a remarkably benign condition. This observation is potentially important since most of the mild clinical symptoms of sickle-cell disease have been attributed to high fetal hemoglobin. Clearly in this case, other factors are operating and may be also operating in those patients with high fetal hemoglobin.- - - - - - - - - - ranking = 0.16666666666667keywords = life (Clic here for more details about this article) |
10/29. Non-productive or thalassemic-like heavy-chain gene rearrangement in a K-chain CLL clone.Information obtained from the study of immunoglobulin (Ig) gene rearrangement in different types of lymphoproliferative disorders has led to the suggestion that Ig gene activation obeys a precise hierarchy in which heavy-chain gene rearrangement procedes light-chain gene recombination. However no information is available on the mechanism(s) controlling this sequential activation. We report here a case of CLL which suggests that the expression of the heavy-chain genes is not necessary for light-chain gene rearrangement, or that very low amounts of heavy-chain mRNA and/or heavy chains are sufficient to activate k-chain genes.- - - - - - - - - - ranking = 0.16666666666667keywords = life (Clic here for more details about this article) |
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