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1.
Calciphylaxis – a topical overview 总被引:3,自引:0,他引:3
G Arseculeratne† AT Evans‡ SM Morley† 《Journal of the European Academy of Dermatology and Venereology》2006,20(5):493-502
'Calciphylaxis', a calcification syndrome associated with ischaemic cutaneous necrosis, is acquired naturally in humans in disease states. It is a life and limb-threatening complication, usually observed in patients with renal disease and secondary hyperparathyroidism, but known to occur in the absence of renal or parathyroid disease. The reported mortality rate, which ranges from 60-80%, relates to wound infection, sepsis and organ failure. It is a small-vessel vasculopathy, which is estimated to occur in about 4% of haemodialysis patients. Clinically, violaceous, reticulate areas of cutaneous necrosis and eschar may be evident, particularly in the extremities. In addition to the clinical picture, a raised calcium phosphorous product, an elevated parathyroid hormone level, radiographic evidence of vessel and soft-tissue calcification and the finding of mural calcification affecting small arteries and arterioles on histopathology help to confirm the diagnosis of this entity which generally has a poor prognosis. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital in the management of these patients. In this overview, we discuss the pathophysiology, clinical features and associations, risk factors, diagnosis and management issues relating to calciphylaxis. 相似文献
2.
3.
What is the prognostic value of myocardial perfusion imaging using rubidium-82 positron emission tomography? 总被引:5,自引:0,他引:5
Keiichiro Yoshinaga Benjamin J W Chow Kathryn Williams Li Chen Robert A deKemp Linda Garrard Alexander Lok-Tin Szeto May Aung Ross A Davies Terrence D Ruddy Rob S B Beanlands 《Journal of the American College of Cardiology》2006,48(5):1029-1039
OBJECTIVES: The objective was to determine the prognostic value of rubidium-82 (82Rb) positron emission tomography (PET) myocardial perfusion imaging (MPI). BACKGROUND: 82Rb PET MPI accurately diagnoses coronary artery disease (CAD). However, there are limited data evaluating its prognostic value. METHODS: Follow-up (3.1 +/- 0.9 years) was obtained in 367 patients who underwent dipyridamole 82Rb PET MPI. Patients were divided into groups based on their summed stress score (SSS): group I, normal (<4); group II, mild (4 to 7); and group III, moderate (8 to 11) to severe (> or =12). RESULTS: There were significant differences among patients in the 3 SSS groups for hard events (cardiac death and myocardial infarction [MI]) (p < 0.001) and total cardiac events (hard events, revascularization and hospitalization) (p < 0.001). The annual hard events rates were 0.4%, 2.3%, and 7.0% in the normal, mild, and moderate-severe groups, respectively. In adjusted survival models, 82Rb PET SSS was the strongest predictor of total cardiac events and a significant predictor of hard events. Among patients referred for PET after 99mTc single-photon emission computed tomography, the annual total event rate was higher with abnormal versus normal SSS on PET (15.2% vs. 1.3%, p < 0.001). In patients with obesity, the annual total event rate was 11.1% with an abnormal scan and 1.5% with a normal scan (p < 0.001). CONCLUSIONS: This study shows that 82Rb PET MPI has significant prognostic value for predicting cardiac events, including death and MI. It also seems to have prognostic value in patients whose diagnosis remains uncertain after single-photon emission computed tomography MPI and in obese patients. The prognostic value of PET MPI may improve the management of cardiac patients. 相似文献
4.
Effect of peri-operative red blood cell transfusion on 30-day and 1-year mortality following coronary artery bypass surgery. 总被引:2,自引:0,他引:2
Manoj Kuduvalli Aung Y Oo Nick Newall Antony D Grayson Mark Jackson Michael J Desmond Brian M Fabri Abbas Rashid 《European journal of cardio-thoracic surgery》2005,27(4):592-598
OBJECTIVE: The purpose of this study was to examine the effect of peri-operative red blood cell (RBC) transfusion on 30-day and 1-year mortality following coronary artery bypass grafting (CABG). METHODS: We retrospectively analysed 3024 consecutive patients who underwent isolated CABG between January 1999 and December 2001. Patient records were linked to the National Strategic Tracing Service, which records all mortality in the UK. Thirty-day and 1-year mortality were derived from Kaplan-Meier curves. Confounding variables were controlled for by constructing a propensity score for the probability of receiving a transfusion from core patient characteristics including the lowest recorded laboratory haemoglobin (LL Hb) from a clinical chemistry database (C statistic 0.81). The propensity score and the comparison variable (transfusion versus no transfusion) were included in a Cox proportional hazards analysis, allowing calculation of adjusted hazard ratios (HR) and Kaplan-Meier survival curves. RESULTS: Nine hundred and forty (31.1%) patients received RBC transfusion during or within 72h of surgery. Predictors of the need for transfusion were LL Hb and lower body mass index, use of cardiopulmonary bypass, female sex, number of grafts, renal dysfunction, increased age, extent of disease, and prior CABG; these factors were all included in the propensity score. After 1-year of follow-up, 122 (4.03%) deaths occurred. The crude HR for 1-year mortality in patients transfused was 3.0 (P<0.001). After adjusting for the propensity score, re-operation for bleeding, peri-operative blood loss and post-operative complications, the adjusted 30-day mortality was 1.9% in transfused patients compared to 1.1% in patients not transfused (P<0.05). The adjusted HR for 1-year mortality in patients transfused was 1.88 (P<0.01). CONCLUSIONS: Peri-operative RBC transfusion after CABG is associated with an increased risk of mortality during a 1-year follow-up period, with a large proportion of deaths occurring within 30-days. 相似文献
5.
Mutations in the retinal guanylate cyclase (RETGC-1) gene in dominant cone-rod dystrophy 总被引:3,自引:0,他引:3
Kelsell RE; Gregory-Evans K; Payne AM; Perrault I; Kaplan J; Yang RB; Garbers DL; Bird AC; Moore AT; Hunt DM 《Human molecular genetics》1998,7(7):1179-1184
The dominant cone-rod dystrophy gene CORD6 has previously been mapped to
within an 8 cM interval on chromosome 17p12-p13. The retinal- specific
guanylate cyclase gene (RETGC-1), which maps to within this genetic
interval and previously was implicated in Leber's congenital amaurosis, was
screened for mutations within this family and in a panel of small families
and individuals with various cone and cone- rod dystrophy phenotypes. A
missense mutation (E837D) was identified in affected members of the CORD6
family, as well as a second missense mutation (R838C) in three other
families with dominant cone-rod dystrophy. RETGC-1 is only the fourth gene
to be implicated in cone-rod dystrophy and this is the first report of
dominant mutations in this gene.
相似文献
6.
Anatomical study of the obturator internus, gemelli and quadratus femoris muscles with special reference to their innervation 总被引:2,自引:0,他引:2
The manner of innervation of the obturator internus, superior and inferior gemelli, and the quadratus femoris in humans (101 pelvic halves) and in rhesus monkeys (Macaca mulatta: 8 pelvic halves) were investigated. In most specimens, the inferior gemellus originated from the lateral surface of the ischial tuberosity and also from the medial surface (intrapelvic origin) just beneath the obturator internus and was covered by the falciform process of the sacrotuberous ligament. The superior gemellus was frequently innervated by the nerve to the obturator internus and the nerve to the quadratus femoris (60.4%), and the inferior gemellus was innervated by the obturator internus nerve in two specimens. The quadratus femoris nerve originated from more cranial segments than the obturator internus nerve, however these nerves had various communication patterns inside and outside the muscles. According to the intramuscular nerve distribution, in some specimens the branches to the superior gemellus from the quadratus femoris nerve extended to the inferior gemellus, and the branches to the inferior gemellus were distributed to the obturator internus. The present findings revealed that the positional relationships among the branches to the obturator internus and gemelli muscles are relatively constant, although the branching patterns and innervation patterns were varied. The various patterns and routes are considered to reflect the variability of the differentiation patterns of the anlage of the muscles. A possible schematic model of the positional relationships between the muscles and the nerves is proposed. 相似文献
7.
A case of jejunal gangliocytic paraganglioma is reported in a 54 year old woman who presented with brisk melaena. The tumour was not encapsulated, involved the mucosa and submucosa, and was composed of epithelial nests, spindle cells, and ganglion cells. These cells were mixed, giving carcinoid-like, paraganglioma-like, and ganglioneuroma-like patterns in different areas of the tumour. The lesion was excised locally and recovery was uneventful. Only four previous cases have been reported at this site. 相似文献
8.
Anderson RA; Wallace AM; Kicman AT; Wu FC 《Human reproduction (Oxford, England)》1997,12(8):1657-1662
Administration of supraphysiological doses of testosterone to normal men
causes inhibition of spermatogenesis, but while most become azoospermic,
30-55% maintain a low rate of spermatogenesis. We have investigated whether
there are differences in endogenous androgen production, of testicular and
adrenal origin, which may be related to the degree of suppression of
spermatogenesis. Thirty-three healthy Caucasian men were given weekly i.m.
injections of 200 mg testosterone oenanthate (TE), 18 became azoospermic,
while 15 remained oligozoospermic. Urinary excretion of epitestosterone, a
specific testicular product, was reduced to <10% of pretreatment values,
with no differences between the groups. Similar results were obtained for
other markers of testicular steroidogenesis. Urinary and plasma adrenal
androgens were also reduced during TE treatment: a statistically
significant decrease in both (P < 0.001 and P < 0.05 respectively)
was seen in the azoospermic but not oligozoospermic responders. These
results suggest that testicular steroidogenesis is decreased to <10% by
the administration of supraphysiological doses of exogenous testosterone.
Differences in the degree of ongoing steroidogenesis in the testis do not
appear to account for incomplete suppression of spermatogenesis, thus
differences in androgen metabolism may underlie this heterogeneous
response. A small but significant reduction in secretion of adrenal
androgens was also detectable, the relevance of which is unclear.
相似文献
9.
Berry V; Ionides AC; Moore AT; Plant C; Bhattacharya SS; Shiels A 《Human molecular genetics》1996,5(3):415-419
Inherited cataract is a clinically and genetically heterogeneous disease.
Here we report the identification of a new locus for an autosomal dominant
anterior polar cataract on the short arm of chromosome 17. To map this new
locus we performed genetic linkage analysis with microsatellite markers in
a four-generation pedigree. After exclusion of seven candidate loci for
cataract, we obtained significant positive LOD scores for markers D17S849
(Z = 4.01 / theta = 0.05) and D17S796 (Z = 4.17 / theta = 0.05). Multipoint
analysis gave a maximum LOD score of 5.2 (theta max = 0.06) between these
two markers. From haplotype analysis, the cataract locus lies in the 13 cM
interval between markers D17S849 and D17S796. This study provides the first
genetic mapping of an autosomal dominant anterior polar cataract.
相似文献
10.
Increased replication of HIV-1 at sites of Mycobacterium tuberculosis infection: potential mechanisms of viral activation 总被引:2,自引:0,他引:2