Résumé
The triggering receptor expressed on myeloid cells (TREM) family, which is abundantly expressed in myeloid lineage cells, plays a pivotal role in innate and adaptive immune response. In this study, we aimed to identify a novel receptor expressed on hematopoietic stem cells (HSCs) by using in silico bioinformatics and to characterize the identified receptor. We thus found the TREM-like transcript (TLT)-6, a new member of TREM family. TLT-6 has a single immunoglobulin domain in the extracellular region and a long cytoplasmic region containing 2 immunoreceptor tyrosine-based inhibitory motif-like domains. TLT-6 transcript was expressed in HSCs, monocytes and macrophages. TLT-6 protein was up-regulated on the surface of bone marrow-derived and peritoneal macrophages by lipopolysaccharide stimulation. TLT-6 exerted anti-proliferative effects in macrophages. Our results demonstrate that TLT-6 may regulate the activation and proliferation of macrophages.
Sujets)
Humains , Immunité acquise , Biologie informatique , Simulation numérique , Cytoplasme , Cellules souches hématopoïétiques , Immunoglobulines , Macrophages , Macrophages péritonéaux , Monocytes , Cellules myéloïdesRésumé
BACKGROUND/AIMS: Fabry disease is an X-linked recessive and progressive disease caused by alpha-galactosidase A (alpha-GaL A) deficiency. We sought to assess the prevalence of unrecognized Fabry disease in dialysis-dependent patients and the efficacy of serum globotriaosylceramide (GL3) screening. METHODS: A total of 480 patients of 1,230 patients among 17 clinics were enrolled. Serum GL3 levels were measured by tandem mass spectrometry. Additionally, we studied the association between increased GL3 levels and cardiovascular disease, cerebrovascular disease, or left ventricular hypertrophy. RESULTS: Twenty-nine patients had elevated serum GL3 levels. The alpha-GaL A activity was determined for the 26 patients with high GL3 levels. The mean alpha-GaL A activity was 64.6 nmol/hr/mg (reference range, 45 to 85), and no patient was identified with decreased alpha-GaL A activity. Among the group with high GL3 levels, 15 women had a alpha-GaL A genetics analysis. No point mutations were discovered among the women with high GL3 levels. No correlation was observed between serum GL3 levels and alpha-GaL A activity; the Pearson correlation coefficient was 0.01352 (p = 0.9478). No significant correlation was observed between increased GL3 levels and the frequency of cardiovascular disease or cerebrovascular disease. CONCLUSIONS: Fabry disease is very rare disease in patients with end-stage renal disease. Serum GL3 measurements as a screening method for Fabry disease showed a high false-positive rate. Thus, serum GL3 levels determined by tandem mass spectrometry may not be useful as a screening method for Fabry disease in patients with end stage renal disease.
Sujets)
Adulte , Sujet âgé , Femelle , Humains , Mâle , Adulte d'âge moyen , Maladie de Fabry/sang , Défaillance rénale chronique/sang , Dialyse rénale , Trihexosylcéramide/sang , alpha-Galactosidase/génétiqueRésumé
With the wide application of ultrasonography and CT scanning, the incidental finding of a radiologic abnormality, apparently adrenal origin, has presented a problem for clinical management. The prevalence of these clinically silent tumors has been reported as 0.6 to 1.3% of upper abdominal CT scans performed for other reasons.Once identified, an adrenal lesion must characterized as to its functional status and malignant potential. A thorough approach that initially excludes biochemical hypersecretion, then considers characteristics of anatomy and the functional nature of an adrenal mass, will allow a rational and cost-effective evaluation and management of these lesions.We present out experience of eleven adrenal incidentalomas with a review of literatures.
Sujets)
Étude clinique , Résultats fortuits , Prévalence , Tomodensitométrie , ÉchographieRésumé
Osteoporosis is a common clinical problem with high risk of fractures in old age, especially postmenopausal women.Secondary causes of osteoporosis can be identified in 20% of women and 40% of men with vertebral fractures. One of the causes of secondary osteoporosis is endocrine disease such as hypogonadism, ovarian agenesis, hyperadrenocorticism, hyperthyroidism, hyperparathyroidism and diabetes mellitus. Patients with Sheehan's syndrome have deficiency of multiple hormones which may cause bone loss.To determine changes in the bone mineral density in women with Sheehan's syndrome and to compare clinical and biochemical characteristics between the patients with osteoporosis and the patients without osteoporosis, we measured the bone mineral density(BMD) of the lumber spine and midradius by dual energy X-ray absortiometry(DEXA) and the serum levels of estrogen and osteocalcin in 11 patients of Sheehan's syndrome.The results were as follows;1) The BMDs of the lumbar spine were significantly decreased in patients with Sheehan's syndrome when compared with those of age-matched control.2) The prevalence of osteoporosis in patients with Sheehan's syndromes was 55%. Between the patients with osteoporosis and the patients without osteoporosis, there were no difference in the onset age of amenorrhea, the duration of amenorrhea, and the serum levels of osteocalcin and alkaline phosphatase.3) Serum estradiol levels were decreased uniformly in the patients with Sheehan's syndrome except three patients with estrogen replacement, but the concentration of estradiol was not correlated with the degree of the decrease in bone mass.In conclusion, the patients with Sheehan's syndrome have an increased prevalence of osteoporosis. But the effect of each anterior pituitary hormone deficiency on bone loss should be clarified in the futher prospective study.
Sujets)
Femelle , Humains , Mâle , Hypercorticisme , Âge de début , Aménorrhée , Densité osseuse , Diabète , Maladies endocriniennes , Oestradiol , Oestrogénothérapie substitutive , Oestrogènes , Hyperparathyroïdie , Hyperthyroïdie , Hypogonadisme , Hypopituitarisme , Mineurs (métier) , Ostéocalcine , Ostéoporose , Prévalence , Études prospectives , RachisRésumé
No abstract available.