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Rupture of an intra-aortic balloon counterpulsator (IABCP) demands immediate removal. We report a case of thrombus formation within a Datascope IABCP secondary to IABCP rupture, necessitating surgical exploration for removal. There is a disturbing pattern of balloon ruptures with this type of IABCP. 相似文献
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Congenital erythropoietic porphyria (CEP) is a rare disorder of heme biosynthesis that results in the production of large quantities of photoactive porphyrins. The clinical syndrome is dominated by extreme photosensitivity with mutilation of light exposed extremities and hemolytic anemia. Bone disease has been occasionally noted, but is not well characterised. We describe a man with CEP who developed bone pain and spinal crush fractures at the age of 22. Skeletal radiographs revealed features typical of other severe hemolytic anemias, but in addition there was loss of the terminal phalanges of the hand as a result of photomutilation. Spinal bone density (assessed by DPA) was reduced and at the hip bone density was at the lower limit of normal. The metacarpal cortical bone density was 2.9 standard deviations below normal. Biochemical and histological studies accelerated bone turnover. Although the serum 250H vitamin D concentration was very low (because of light avoidance) there was no evidence that the bone disease was a consequence of this. Treatment for one year with clodronate and a high transfusion regime was associated with small reductions in serum alkaline phosphatase and urine hydroxyproline excretion, but there was no improvement in bone mineral density. We conclude that CEP has a distinctive osteodystrophy comprising osteolysis of light-exposed extremities and a high turnover type of osteoporosis. Privational vitamin D deficiency may also occur. The effect upon bone of the new therapies for CEP should be considered. 相似文献
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Bellingham R Cohen B Jones T Spaniol le R 《American journal of health promotion : AJHP》1989,4(1):18-31
Abstract This article explores the relationship between connectedness and spiritual health. Three forms of connectedness are discussed: connecting with oneself, with others, and with a larger meaning or purpose. Eight connectedness skills are suggested. Combined, these skills may lead to an enhanced sense of spiritual well-being. 相似文献
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Verschuren MC; Blom B; Bogers AJ; Spits H; van Dongen JJ 《International immunology》1998,10(12):1873-1880
Recombination of deltaRec to psiJalpha will delete the TCR delta gene,
which is thought to play an important role in the bifurcation of the TCR
alphabeta versus TCR gammadelta differentiation lineages. We recently
detected a DNA-binding protein in human thymocytes, the so- called PJA-BP,
which recognizes the psiJalpha gene segment and might be one of the factors
involved in the regulation of preferential deltaRec- psiJalpha
rearrangements. We now investigate PJA-BP expression and its correlation
with TCR delta gene deletion in thymocytes. Our electrophoretic mobility
shift assay experiments showed that the PJA-BP is evolutionary conserved in
human, murine and simian thymocytes. Using a large series of human
hematopoietic malignancies (n = 30), we conclude that PJA-BP expression is
thymocyte specific and seems to be restricted to thymocytes committed to
the TCR alphabeta lineage. Analysis of seven well-defined human thymocyte
subpopulations showed that preferential deltaRec-psiJalpha rearrangements
as well as PJA-BP expression can be detected from the immature
CD34-/CD1+/CD3- /CD4+/CD8alpha+beta- thymocyte differentiation stage
onwards. These experiments indicate that expression of PJA-BP in human
thymocytes starts simultaneously with preferential deltaRec-psiJalpha
rearrangements, which supports our hypothesis that PJA-BP is one of the
factors involved in the preferential recombination of deltaRec to
psiJalpha.
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