Diastolic funciton correlates with the degree of iron overload in patients with haemoglobin H disease

published_or_final_versio

Bronchiolitis obliterans with organising pneumonia (BOOP) following allogeneic bone marrow transplant - a common pulmonary conplication following BMT?

published_or_final_versio

Bone angiogenesis and vascular niche remodeling in stress, aging, and diseases

The bone marrow (BM) vascular niche microenvironments harbor stem and progenitor cells of various lineages. Bone angiogenesis is distinct and involves tissue-specific signals. The nurturing vascular niches in the BM are complex and heterogenous consisting of distinct vascular and perivascular cell types that provide crucial signals for the maintenance of stem and progenitor cells. Growing evidence suggests that the BM niche is highly sensitive to stress. Aging, inflammation and other stress factors induce changes in BM niche cells and their crosstalk with tissue cells leading to perturbed hematopoiesis, bone angiogenesis and bone formation. Defining vascular niche remodeling under stress conditions will improve our understanding of the BM vascular niche and its role in homeostasis and disease. Therefore, this review provides an overview of the current understanding of the BM vascular niches for hematopoietic stem cells and their malfunction during aging, bone loss diseases, arthritis and metastasis

Helper T-lymphocyte precursor frequency (HTLPf) predicts the occurrence of graft-versus-host disease (GVHD) and disease relapse after allogeneic bone marrow transplantation

published_or_final_versio

MR screening for iron overload in non-transfusion dependent haemoglobin H disease

published_or_final_versio

Movements of genes between populations: are pollinators more effective at transferring their own or plant genetic markers?

The transfer of genes between populations is increasingly important in a world where pollinators are declining, plant and animal populations are increasingly fragmented and climate change is forcing shifts in distribution. The distances that pollen can be transported by small insects are impressive, as is the extensive gene flow between their own populations. We compared the relative ease by which small insects introduce genetic markers into their own and host-plant populations. Gene flow via seeds and pollen between populations of an Asian fig species were evaluated using cpDNA and nuclear DNA markers, and between-population gene flow of its pollinator fig wasp was determined using microsatellites. This insect is the tree's only pollinator locally, and only reproduces in its figs. The plant's pollen-to-seed dispersal ratio was 9.183-9.437, smaller than that recorded for other Ficus. The relative effectiveness of the pollinator at introducing markers into its own populations was higher than the rate it introduced markers into the plant's populations (ratio = 14 : 1), but given the demographic differences between plant and pollinator, pollen transfer effectiveness is remarkably high. Resource availability affects the dispersal of fig wasps, and host-plant flowering phenology here and in other plant-pollinator systems may strongly influence relative gene flow rates

Genetic and clinical features of hemoglobin H disease in Chinese patients

Background. Normally, one pair of each of the two α-globin genes, α1 and α2, resides on each copy of chromosome 16. In hemoglobin H disease, three of these four α-globin genes are affected by a deletion, a mutation, or both. We studied the α-globin gene abnormalities and the clinical and hematologic features of Chinese patients with hemoglobin H disease in Hong Kong. Methods. We assessed the clinical features, hematologic values, serum ferritin levels, and liver function of 114 patients with hemoglobin H disease. We also performed echocardiography and magnetic resonance imaging of the liver and examined the two pairs of α-globin genes. Results. Hemoglobin H disease in 87 of the 114 patients (76 percent) was due to the deletion of three of the four α-globin genes (--/-α), a combination termed the deletional type of hemoglobin H. The remaining 27 patients (24 percent) had the nondeletional type of hemoglobin H disease, in which two α-globin genes are deleted and a third is mutated (--/αα(T)). All 87 patients with the deletional type of hemoglobin H were double heterozygotes in whom there was a deletion of both α-globin genes from one chromosome, plus a deletion of the α1 or α2 gene from the other chromosome (--/α- or --/-α). A variety of mutated α-globin genes was found in the patients with nondeletional type of hemoglobin H disease. Patients with the nondeletional type of the H disease had more symptoms at a younger age, more severe hemolytic anemia, and larger spleens and were more likely to require transfusions than patients with deletional hemoglobin H disease. The severity of iron overload was not related to the genotype. Conclusions. Chinese patients in Hong Kong with the nondeletional type of hemoglobin H disease have more severe disease than those with the deletional type of the disease. Iron overload is a major cause of disability in both forms of the disease. (C) 2000, Massachusetts Medical Society.published_or_final_versio