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11.
N. Matamoros Florí J. Mila Llambi T. Español Boren S. Raga Borja G. Fontan Casariego 《Journal of clinical immunology》1997,17(4):333-339
The Spanish Registry for Primary Immunodeficiency Diseases (REDIP) was organized in 1993. One thousand sixty-nine cases of primary immunodeficiency diseases (PID) were registered in patients diagnosed between January 1980 and December 1995. PID diagnosis was made according to the World Health Organization criteria. The most frequent disorders were IgA deficiency (n = 394) and common variable immunodeficiency (n = 213), followed by severe combined immunodeficiency (n = 61), C1 inhibitor deficiency (n = 52), X-Iinked agammaglobulinemia (n = 49), IgG subclass deficiency (n = 48), and chronic granulomatous disease (n = 32). A comparative study between REDIP and data recently obtained from the European registry (ESID Report, 1995) revealed important differences between phagocytic disorders and complement deficiencies reported in both registries, 4.9 vs 8.7 and 6.0 vs 3.6, while percentages of predominantly antibody deficiencies and T cell and combined deficiencies concurred with those reported in the European registry, 69.3 vs 64.7 and 14.7 vs 20.2, respectively. The heterogeneous nature of the geographical distribution of cases submitted may indicate underdiagnosis of PID in some country areas; surprisingly, the interval between the onset of clinical symptoms and diagnosis was significant, even in immunodeficiency diseases, such as IgA deficiency, which are easy to diagnose. 相似文献
12.
Canfield MC; Tamarappoo BK; Moses AM; Verkman AS; Holtzman EJ 《Human molecular genetics》1997,6(11):1865-1871
Congenital nephrogenic diabetes insipidus (NDI) is a rare disease caused
most often by mutations in the vasopressin V2 receptor (AVPR2). We studied
a family which included a female patient with NDI with symptoms dating from
infancy. The patient responded to large doses of desmopressin (dDAVP) which
decreased urine volume from 10 to 4 I/day. Neither the parents nor the
three sisters were polyuric. The patient was found to be a compound
heterozygote for two novel recessive point mutations in the aquaporin-2
(AQP2) gene: L22V in exon 1 and C181W in exon 3. Residue Cys181 in AQP2 is
the site for inhibition of water permeation by mercurial compounds and is
located near to the NPA motif conserved in all aquaporins. Osmotic water
permeability (Pf) in Xenopus oocytes injected with cRNA encoding C181W-AQP2
was not increased over water control, while expression of L22V cRNA
increased the Pf to approximately 60% of that for wild-type AQP2.
Co-injection of the mutant cRNAs with the wild-type cRNA did not affect the
function of the wild-type AQP2. Immunolocalization of AQP2-transfected CHO
cells showed that the C181W mutant had an endoplasmic reticulum-like
intracellular distribution, whereas L22V and wild-type AQP2 showed endosome
and plasma membrane staining. Water permeability assays showed a high Pf in
cells expressing wild-type and L22V AQP2. This study indicates that AQP2
mutations can confer partially responsive NDI.
相似文献
13.
Russell EJ; Geremia GK; Johnson CE; Huckman MS; Ramsey RG; Washburn-Bleck J; Turner DA; Norusis M 《Radiology》1987,165(3):609-617
Sixteen patients with suspected cerebral metastases were studied with magnetic resonance (MR) imaging before and after the intravenous administration of 0.1 mmol/kg of gadolinium diethylenetriaminepenta-acetic acid. The images were interpreted blindly by two neuroradiologists; all clinical, radiologic (computed tomographic and MR imaging), and pathologic data were reviewed to arrive at a final "best diagnosis," which was then compared with the prior blinded interpretations. Of seven patients found to have multiple metastases, six (86%) had at least one tumor nodule depicted by postinfusion MR imaging that was missed by one or both observers on review of preinfusion images alone. Lesions missed on preinfusion studies were usually small nodules hidden by or not detected next to regions of high-signal edema thought to be related to the adjacent tumor nodule. The authors believe that contrast enhancement improves detection of metastatic foci with MR imaging and that the findings indicate broader implications for the detection of multiple lesions from other causes. 相似文献
14.
The purpose of this study was to compare the diagnostic accuracy of fluorine-18 fluorodeoxyglucose (FDG) images obtained with (a) a dual-head coincidence gamma camera (DHC) equipped with 5/8-inch-thick NaI(Tl) crystals and parallel slit collimators and (b) a dedicated positron emission tomograph (PET) in a series of 28 patients with known or suspected malignancies. Twenty-eight patients with known or suspected malignancies underwent whole-body FDG PET imaging (Siemens, ECAT 933) after injection of approximately 10 mCi of 18F-FDG. FDG DHC images were then acquired for 30 min over the regions of interest using a dual-head gamma camera (VariCam, Elscint). The images were reconstructed in the normal mode, using photopeak/photopeak, photopeak/Compton, and Compton/photopeak coincidence events. FDG PET imaging found 45 lesions ranging in size from 1 cm to 7 cm in 28 patients. FDG DHC imaging detected 35/45 (78%) of these lesions. Among the ten lesions not seen with FDG DHC imaging, eight were less than 1.5 cm in size, and two were located centrally within the abdomen suffering from marked attenuation effects. The lesions were classified into three categories: thorax (n=24), liver (n=12), and extrahepatic abdominal (n=9). FDG DHC imaging identified 100% of lesions above 1.5 cm in the thorax group and 78% of those below 1.5 cm, for an overall total of 83%. FDG DHC imaging identified 100% of lesions above 1.5 cm, in the liver and 43% of lesions below 1.5 cm, for an overall total of 67%. FDG DHC imaging identified 78% of lesions above 1.5 cm in the extrahepatic abdominal group. There were no lesions below 1.5 cm in this group. FDG coincidence imaging using a dual-head gamma camera detected 90% of lesions greater than 1.5 cm. These data suggest that DHC imaging can be used clinically in well-defined diagnostic situations to differentiate benign from malignant lesions. 相似文献
15.
Inhibition of growth and induction of apoptosis in human cancer cell lines by tea polyphenols 总被引:47,自引:7,他引:47
In order to study the biological activities of tea preparations and
purified tea polyphenols, their growth inhibitory effects were investigated
using four human cancer cell lines. Growth inhibition was measured by
[3H]thymidine incorporation after 48 h of treatment. The green tea
catechins (-)-epigallocatechin-3-gallate (EGCG) and (-)- epigallocatechin
(EGC) displayed strong growth inhibitory effects against lung tumor cell
lines H661 and H1299, with estimated IC50 values of 22 microM, but were
less effective against lung cancer cell line H441 and colon cancer cell
line HT-29 with IC50 values 2- to 3- fold higher.
(-)-Epicatechin-3-gallate, had lower activities, and (-)- epicatechin was
even less effective. Preparations of green tea polyphenols and theaflavins
had higher activities than extracts of green tea and decaffeinated green
tea. The results suggest that the growth inhibitory activity of tea
extracts is caused by the activities of different tea polyphenols. Exposure
of H661 cells to 30 microM EGCG, EGC or theaflavins for 24 h led to the
induction of apoptosis as determined by an annexin V apoptosis assay,
showing apoptosis indices of 23, 26 and 8%, respectively; with 100 microM
of these compounds, the apoptosis indices were 82, 76 and 78%,
respectively. Incubation of H661 cells with EGCG also induced a
dose-dependent formation of H2O2. Addition of H2O2 to H661 cells caused
apoptosis in a manner similar to that caused by EGCG. The EGCG-induced
apoptosis in H661 cells was completely inhibited by exogenously added
catalase (50 units/ml). These results suggest that tea polyphenol-induced
production of H2O2 may mediate apoptosis and that this may contribute to
the growth inhibitory activities of tea polyphenols in vitro.
相似文献
16.
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18.
Kory Floyd Alice E. Veksler Bree McEwan Colin Hesse Justin P. Boren Dana R. Dinsmore 《Health communication》2017,32(8):1039-1042
Loneliness has been shown to have direct effects on one’s personal well-being. Specifically, a greater feeling of loneliness is associated with negative mental health outcomes, negative health behaviors, and an increased likelihood of premature mortality. Using the neuroendocrine hypothesis, we expected social inclusion to predict decreases in both blood glucose levels and low-density lipoproteins (LDLs) and increases in high-density lipoproteins (HDLs). Fifty-two healthy adults provided self-report data for social inclusion and blood samples for hematological tests. Results indicated that higher social inclusion predicted lower levels of blood glucose and LDL, but had no effect on HDL. Implications for theory and practice are discussed. 相似文献
19.
Human interleukin-5 (IL-5) regulates the production of eosinophils in human bone marrow cultures: comparison and interaction with IL-1, IL-3, IL-6, and GMCSF 总被引:33,自引:1,他引:33
Recombinant human interleukin-5 (rhIL-5), in either liquid or semi- solid cultures, selectively induced eosinophil production from normal human bone marrow, with no activity on other cell lineages. The time course of eosinophil production induced by murine IL-5, rhIL-3, and rh granulocyte-macrophage colony stimulating factor (GMCSF) was similar to rhIL-5. The rate of eosinophil maturation in vitro was independent of the stimulating cytokine, mature eosinophils being produced after 4 to 5 weeks in liquid culture with each of these cytokines. The eosinophils produced in response to each cytokine were morphologically indistinguishable, and had the ultrastructural features of maturity except that the electron-dense material in the granules had not formed into crystalline cores. Neither rhIL-1 nor rhIL-6 alone, or in combination with rhIL-5 or rhIL-3, induced eosinophil differentiation or proliferation under the conditions used. rhIL-3 and rhGMCSF induced more eosinophil colonies than rhIL-5, rhIL-5 had an additive, not synergistic, effect on eosinophil colony production when combined with either rhIL-3 or rhGMCSF, suggesting that rhIL-5 stimulates a smaller and possibly different population of eosinophil progenitors. However, rhIL-5 induced the greatest eosinophil production in liquid cultures, suggesting that although it may act on a smaller population of precursors, it is able to stimulate more proliferative steps than either rhIL-3 or rhGMCSF. 相似文献
20.
Charlotte W Ockeloen Marjolein H Willemsen Sonja de Munnik Bregje WM van Bon Nicole de Leeuw Aad Verrips Sarina G Kant Elizabeth A Jones Han G Brunner Rosa LE van Loon Eric EJ Smeets Mieke M van Haelst Gijs van Haaften Ann Nordgren Helena Malmgren Giedre Grigelioniene Sascha Vermeer Pedro Louro Lina Ramos Thomas JJ Maal Celeste C van Heumen Helger G Yntema Carine EL Carels Tjitske Kleefstra 《European journal of human genetics : EJHG》2015,23(9):1270-1185
Loss-of-function variants in ANKRD11 were identified as the cause of KBG syndrome, an autosomal dominant syndrome with specific dental, neurobehavioural, craniofacial and skeletal anomalies. We present the largest cohort of KBG syndrome cases confirmed by ANKRD11 variants reported so far, consisting of 20 patients from 13 families. Sixteen patients were molecularly diagnosed by Sanger sequencing of ANKRD11, one familial case and three sporadic patients were diagnosed through whole-exome sequencing and one patient was identified through genomewide array analysis. All patients were evaluated by a clinical geneticist. Detailed orofacial phenotyping, including orthodontic evaluation, intra-oral photographs and orthopantomograms, was performed in 10 patients and revealed besides the hallmark feature of macrodontia of central upper incisors, several additional dental anomalies as oligodontia, talon cusps and macrodontia of other teeth. Three-dimensional (3D) stereophotogrammetry was performed in 14 patients and 3D analysis of patients compared with controls showed consistent facial dysmorphisms comprising a bulbous nasal tip, upturned nose with a broad base and a round or triangular face. Many patients exhibited neurobehavioural problems, such as autism spectrum disorder or hyperactivity. One-third of patients presented with (conductive) hearing loss. Congenital heart defects, velopharyngeal insufficiency and hip anomalies were less frequent. On the basis of our observations, we recommend cardiac assessment in children and regular hearing tests in all individuals with a molecular diagnosis of KBG syndrome. As ANKRD11 is a relatively common gene in which sequence variants have been identified in individuals with neurodevelopmental disorders, it seems an important contributor to the aetiology of both sporadic and familial cases. 相似文献