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51.
Indolent systemic mastocytosis with elevated serum tryptase, absence of skin lesions, and recurrent severe anaphylactoid episodes 总被引:2,自引:0,他引:2
Florian S Krauth MT Simonitsch-Klupp I Sperr WR Fritsche-Polanz R Sonneck K Födinger M Agis H Böhm A Wimazal F Horny HP Valent P 《International archives of allergy and immunology》2005,136(3):273-280
BACKGROUND: In contrast to aggressive mastocytosis, patients with indolent systemic mastocytosis (ISM) usually present with urticaria pigmentosa-like skin lesions. In those who lack skin lesions, mastocytosis is often overlooked or confused with endocrinologic, allergic, or other internal disorders. CASE REPORT AND RESULTS: We report on a 33-year-old male patient in whom severe hypotensive episodes occurred after contact with ants or yellow jackets. Since no specific IgE was detected, the serum tryptase concentration was measured and found to be clearly elevated (70 ng/ml). Consecutive staging and examination of the bone marrow revealed ISM. The patient was advised to circumvent insect contact, to take antihistamines on demand, and to carry an epinephrine self-injector for emergency events. In a retrospective analysis of 40 patients seen between 1988 and 2003, only 2 had a life-threatening mediator-related episode before ISM was diagnosed. CONCLUSIONS: Our report confirms the diagnostic value of tryptase in patients with suspected mastocytosis. In addition, the report suggests that the lack of typical skin lesions does not exclude an indolent form of mastocytosis even if the serum tryptase is clearly elevated. Finally, our case further shows that mastocytosis can be an important differential diagnosis to be considered in patients with unexplained anaphylactoid or other mediator-related symptoms. 相似文献
52.
Initial validation of the SLEEP-50 questionnaire 总被引:1,自引:0,他引:1
Initial psychometric properties of the SLEEP-50 questionnaire, designed to detect sleep disorders as listed in the Diagnostic and Statistical Manual of Mental Disorders (4th ed., Text Revision), were examined. The sample consisted of 377 college students, 246 sleep patients, 32 nightmare sufferers, and 44 healthy volunteers. The internal consistency was high (Cronbach's alpha = .85); test-retest correlations fell between .65 and .89. Principal component analysis with a direct oblimin rotation revealed a factor structure that closely matched the designed structure. Sensitivity and specificity scores were promising for all sleep disorders; the agreement between all clinical diagnoses and SLEEP-50-classifications was substantial (kappa = .77). These initial findings indicate that the SLEEP-50 seems able to detect a variety of sleep disorders. The SLEEP-50 can aid in screening for common sleep disorders in the general population. 相似文献
53.
Zeynep H. Coban‐Akdemir Wu‐Lin Charng Mahshid Azamian Ingrid S. Paine Jaya Punetha Christopher M. Grochowski Tomasz Gambin Santiago O. Valdes Bryan Cannon Gladys Zapata Patricia P. Hernandez Shalini Jhangiani Harsha Doddapaneni Jianhong Hu Fatima Boricha Donna M. Muzny Eric Boerwinkle Yaping Yang Richard A. Gibbs Jennifer E. Posey Xander H. T. Wehrens John W. Belmont Jeffrey J. Kim Christina Y. Miyake James R. Lupski Seema R. Lalani 《American journal of medical genetics. Part A》2020,182(6):1387-1399
54.
Placental inflammation and perinatal transmission of HIV-1 总被引:2,自引:0,他引:2
Mwanyumba F Gaillard P Inion I Verhofstede C Claeys P Chohan V Vansteelandt S Mandaliya K Praet M Temmerman M 《Journal of acquired immune deficiency syndromes (1999)》2002,29(3):262-269
The effect of placental membrane inflammation on mother-to-child transmission (MTCT) of HIV-1 is reported. Placentas from HIV-1-infected women were examined as part of a perinatal HIV-1 project in Mombasa, Kenya. Polymerase chain reaction analysis was used to test for HIV-1 in the infants at birth and at 6 weeks. The maternal HIV-1 seroprevalence was 13.3% (298 of 2,235). The overall rate of MTCT of HIV-1 was 25.4%; polymerase chain reaction analysis revealed that of the 201 infants 6.0% (12) were already HIV-1-positive at birth (intrauterine transmission) and 19.4% (39) were infected during the peripartum period or in early neonatal life (perinatal transmission). The prevalence of acute chorioamnionitis was 8.8%, that of deciduitis was 10.8%, and that of villitis was 1.6%. Acute chorioamnionitis was independently associated with peripartum HIV-1 transmission but not with in utero MTCT (17.9% vs. 6.7%, respectively; adjusted odds ratio, 3.9; 95% confidence interval, 1.2-12.5; p =.025). Other correlates of perinatal MTCT were presence of HIV in the genital tract and in the baby's oral cavity and a high maternal viral load in peripheral blood. The adjusted population attributable fraction of 12.8% (95% confidence interval, 1.5%-22.8%) indicated that approximately 3% of MTCT could be prevented if acute chorioamnionitis was eliminated. We suggest that further research on the role of antimicrobial treatment in the prevention of chorioamnionitis and the reduction of peripartum MTCT needs to be performed. 相似文献
55.
Wessels MW Berger RM Frohn-Mulder IM Roos-Hesselink JW Hoogeboom JJ Mancini GS Bartelings MM Krijger Rd Wladimiroff JW Niermeijer MF Grossfeld P Willems PJ 《American journal of medical genetics. Part A》2005,(2):171-179
Most nonsyndromic congenital heart malformations (CHMs) in humans are multifactorial in origin, although an increasing number of monogenic cases have been reported recently. We describe here four new families with presumed autosomal dominant inheritance of left ventricular outflow tract obstruction (LVOTO), consisting of hypoplastic left heart (HLHS) or left ventricle (HLV), aortic valve stenosis (AS) and bicuspid aortic valve (BAV), hypoplastic aortic arch (HAA), and coarctation of the aorta (CoA). LVOTO in these families shows a wide clinical spectrum with some family members having severe anomalies such as hypoplastic left heart, and others only minor anomalies such as mild aortic valve stenosis. This supports the suggestion that all anomalies of the LVOTO spectrum are developmentally related and can be caused by a single gene defect. 相似文献
56.
Transgenic rat model of Huntington's disease 总被引:12,自引:0,他引:12
von Hörsten S Schmitt I Nguyen HP Holzmann C Schmidt T Walther T Bader M Pabst R Kobbe P Krotova J Stiller D Kask A Vaarmann A Rathke-Hartlieb S Schulz JB Grasshoff U Bauer I Vieira-Saecker AM Paul M Jones L Lindenberg KS Landwehrmeyer B Bauer A Li XJ Riess O 《Human molecular genetics》2003,12(6):617-624
Huntington's disease (HD) is a late manifesting neurodegenerative disorder in humans caused by an expansion of a CAG trinucleotide repeat of more than 39 units in a gene of unknown function. Several mouse models have been reported which show rapid progression of a phenotype leading to death within 3-5 months (transgenic models) resembling the rare juvenile course of HD (Westphal variant) or which do not present with any symptoms (knock-in mice). Owing to the small size of the brain, mice are not suitable for repetitive in vivo imaging studies. Also, rapid progression of the disease in the transgenic models limits their usefulness for neurotransplantation. We therefore generated a rat model transgenic of HD, which carries a truncated huntingtin cDNA fragment with 51 CAG repeats under control of the native rat huntingtin promoter. This is the first transgenic rat model of a neurodegenerative disorder of the brain. These rats exhibit adult-onset neurological phenotypes with reduced anxiety, cognitive impairments, and slowly progressive motor dysfunction as well as typical histopathological alterations in the form of neuronal nuclear inclusions in the brain. As in HD patients, in vivo imaging demonstrates striatal shrinkage in magnetic resonance images and a reduced brain glucose metabolism in high-resolution fluor-deoxy-glucose positron emission tomography studies. This model allows longitudinal in vivo imaging studies and is therefore ideally suited for the evaluation of novel therapeutic approaches such as neurotransplantation. 相似文献
57.
Roy O. Weller ; Malavika Subash ; Stephen D. Preston ; Ingrid Mazanti ; Roxana O. Carare 《Brain pathology (Zurich, Switzerland)》2008,18(2):253-266
Alzheimer's disease is the commonest dementia. One major characteristic of its pathology is accumulation of amyloid-β (Aβ) as insoluble deposits in brain parenchyma and in blood vessel walls [cerebral amyloid angiopathy (CAA)]. The distribution of Aβ deposits in the basement membranes of cerebral capillaries and arteries corresponds to the perivascular drainage pathways by which interstitial fluid (ISF) and solutes are eliminated from the brain—effectively the lymphatic drainage of the brain. Theoretical models suggest that vessel pulsations supply the motive force for perivascular drainage of ISF and solutes. As arteries stiffen with age, the amplitude of pulsations is reduced and insoluble Aβ is deposited in ISF drainage pathways as CAA, thus, further impeding the drainage of soluble Aβ. Failure of perivascular drainage of Aβ and deposition of Aβ in the walls of arteries has two major consequences: (i) intracerebral hemorrhage associated with rupture of Aβ-laden arteries in CAA; and (ii) Alzheimer's disease in which failure of elimination of ISF, Aβ and other soluble metabolites from the brain alters homeostasis and the neuronal environment resulting in cognitive decline and dementia. Therapeutic strategies that improve elimination of Aβ and other soluble metabolites from the brain may prevent cognitive decline in Alzheimer's disease. 相似文献
58.
Fibroblast strains derived from six patients with maple syrup urine disease have been investigated for their requirements of the cofactors NAD, CoASH, Mg++ and TPP in comparison with 10 normal control strains. The reconstitution of the decarboxylase function of branched chain α-keto acid (BCKA) dehydrogenase complex in lysed cells was studied with respect to the substrates u-keto-isocaproic acid, α-keto-isovaleric acid, and α-keto-β-methylvaleric acid (KIC, KIVA, MEVA). The enzyme activity of all normal control strains for the substrates KIC and KIVA was not reconstituted by TPP + Mg++ alone, but CoASH + NAD could reconstitute the enzyme activity with KIC and KIVA in different degrees. Only two control strains were tested with MEVA as substrate, and these showed in contrast that TPP + Mg++ could partly reconstitute the enzyme activity. In contrast to the relative homogeneiy in the reconstitution profiles of normal strains, the five classical and one intermittent MSUD strains showed heterogeneity in cofactor requirements.
Complementation analysis using heterokaryons prepared from fibroblasts of four patients with classical MSUD and one patient with intermittent MSUD showed, in contrast to experiments with normal controls, a partial amelioration of the defect in two combinations; it is suggested that the defect in these strains is located at different functional subunits of the multienzyme complex. 相似文献
Complementation analysis using heterokaryons prepared from fibroblasts of four patients with classical MSUD and one patient with intermittent MSUD showed, in contrast to experiments with normal controls, a partial amelioration of the defect in two combinations; it is suggested that the defect in these strains is located at different functional subunits of the multienzyme complex. 相似文献
59.
Ingrid V. Allen 《Immunology》1965,8(5):475-483
The effect of total body irradiation on the development of delayed hypersensitivity and on the febrile response to specific antigen has been studied in guinea-pigs with the following results:
1. 200 R. whole body irradiation in guinea-pigs, while suppressing circulating antibody response, did not prevent the development of delayed hypersensitivity.
2. Irradiated and non-irradiated hypersensitive animals had an equal febrile response to systemic challenge with specific antigen.
3. Serum from antigen-challenged, irradiated, hypersensitive animals contained a pyrogenic factor of the endogenous serum type capable of producing fever in normal recipients.
These results support the conclusion that production of circulating specific antibody is not essential either for development of delayed hypersensitivity or for the febrile response of the hypersensitive animal to specific antigen.
相似文献60.
Studies on the mechanism of phorbol ester-induced inhibition of intercellular junctional communication 总被引:1,自引:0,他引:1
Intercellular gap-junctional communication was measured using[14C]citrulline incorporation in co-cultures of argininosuccinatelyase-deficient human fibroblasts and argininosuccinate synthetase-deficientChinese Hamster V79 cells. As previously shown, in this systemjunctional communication is completely inhibited by the tumorpromoting phorbol ester 12-O-tetra-decanoylphorbol-13-acetate(TPA). In the absence of extracellular calcium, TPA inhibitionwas less pronounced. However, synergism with calcium ionophoreA23187 could not be demonstrated. Chlorpromazine, trifluoperazineand 3,4,5-trimethoxybenzoic acid 8-(diethylamino)octyl esterpartially antagonised the effect of TPA. No antagonism was demonstrablebetween calmidazolium and TPA. Treatment of co-cultures withexogenous phospholipase C or 1-oleoyl-2-acetylglycerol (OAG)resulted in communication inhibition, suggesting that proteinkinase C activation is involved in the mechanism of phorbolester-mediated communication inhibition. However co-cultureswhich had been made refractory to TPA by prolonged exposureto high concentrations remained sensitive to inhibition by phospholipaseC and OAG. These results suggest either that diacylglycerolcan produce other effects independent of protein kinase C activation,or that refractoriness to phorbol esters is not simply due toa decrease in the amount of protein kinase C. 相似文献