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21.
Hematopoietic defects in mice lacking the sialomucin CD34 总被引:9,自引:5,他引:9
Cheng J; Baumhueter S; Cacalano G; Carver-Moore K; Thibodeaux H; Thomas R; Broxmeyer HE; Cooper S; Hague N; Moore M; Lasky LA 《Blood》1996,87(2):479-490
Although the pluripotent hematopoietic stem cell can only be definitively identified by its ability to reconstitute the various mature blood lineages, a diversity of cell surface antigens have also been specifically recognized on this subset of hematopoietic progenitors. One such stem cell-associated antigen is the sialomucin CD34, a highly O-glycosylated cell surface glycoprotein that has also been shown to be expressed on all vascular endothelial cells throughout murine embryogenesis as well as in the adult. The functional significance of CD34 expression on hematopoietic progenitor cells and developing blood vessels is unknown. To analyze the involvement of CD34 in hematopoiesis, we have produced both embryonic stem (ES) cells and mice that are null for the expression of this mucin. Analysis of yolk saclike hematopoietic development in embryoid bodies derived from CD34- null ES cells showed a significant delay in both erythroid and myeloid differentiation that could be reversed by transfection of the mutant ES cells with CD34 constructs expressing either a complete or truncated cytoplasmic domain. Measurements of colony-forming activity of hematopoietic progenitor cells derived from yolk sacs or fetal livers isolated from CD34-null embryos also showed a decreased number of these precursor cells. In spite of these diminished embryonic hematopoietic progenitor numbers, the CD34-null mice developed normally, and the hematopoietic profile of adult blood appeared typical. However, the colony-forming activity of hematopoietic progenitors derived from both bone marrow and spleen is significantly reduced in adult CD34-deficient animals, and these CD34-deficient progenitors also appear to be unable to expand in liquid cultures in response to hematopoietic growth factors. Even with these apparent progenitor cell deficiencies, CD34- null animals showed kinetics of erythroid, myeloid, and platelet recovery after sublethal irradiation that are indistinguishable from wild-type mice. These data strongly suggest that CD34 plays an important role in the formation of progenitor cells during both embryonic and adult hematopoiesis. However, the hematopoietic sites of adult CD34-deficient mice may still have a significant reservoir of progenitor cells that allows for normal recovery after nonmyeloablative peripheral cell depletion. 相似文献
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Hiroko Morisaki Gretchen MacCarrick Mark Lindsay David Liang Sarju G. Mehta Jennifer Hague Judith Verhagen Ingrid van de Laar Marja Wessels Yvonne Detisch Mieke van Haelst Annette Baas Klaske Lichtenbelt Kees Braun Denise van der Linde Jolien Roos‐Hesselink George McGillivray Josephina Meester Isabelle Maystadt Paul Coucke Elie El‐Khoury Sandhya Parkash Birgitte Diness Lotte Risom Ingrid Scurr Yvonne Hilhorst‐Hofstee Takayuki Morisaki Julie Richer Julie Désir Marlies Kempers Andrea L. Rideout Gabrielle Horne Chris Bennett Elisa Rahikkala Geert Vandeweyer Maaike Alaerts Aline Verstraeten Hal Dietz Lut Van Laer Bart Loeys 《Human mutation》2018,39(5):621-634
The Loeys–Dietz syndrome (LDS) is a connective tissue disorder affecting the cardiovascular, skeletal, and ocular system. Most typically, LDS patients present with aortic aneurysms and arterial tortuosity, hypertelorism, and bifid/broad uvula or cleft palate. Initially, mutations in transforming growth factor‐β (TGF‐β) receptors (TGFBR1 and TGFBR2) were described to cause LDS, hereby leading to impaired TGF‐β signaling. More recently, TGF‐β ligands, TGFB2 and TGFB3, as well as intracellular downstream effectors of the TGF‐β pathway, SMAD2 and SMAD3, were shown to be involved in LDS. This emphasizes the role of disturbed TGF‐β signaling in LDS pathogenesis. Since most literature so far has focused on TGFBR1/2, we provide a comprehensive review on the known and some novel TGFB2/3 and SMAD2/3 mutations. For TGFB2 and SMAD3, the clinical manifestations, both of the patients previously described in the literature and our newly reported patients, are summarized in detail. This clearly indicates that LDS concerns a disorder with a broad phenotypical spectrum that is still emerging as more patients will be identified. All mutations described here are present in the corresponding Leiden Open Variant Database. 相似文献
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J. S. Hague M. R. Kaur J. Hafiji R. A. Carr H. Lewis R. Charles‐Holmes A. Heagerty S. D. Orpin 《Clinical and experimental dermatology》2011,36(1):42-45
Toxic epidermal necrolysis (TEN) is a life‐threatening, immune‐mediated reaction, characterized by severe cutaneous and mucosal blisters and erosions. It often presents with flu‐like symptoms, followed by a maculopapular, urticarial, purpuric or erythema multiforme‐like eruption, which then evolves into blisters and sheet‐like erosions. Presentation with pustules, however, is not well described in the English literature, and may lead to delayed diagnosis. We present two unusual cases of TEN that initially presented with pustular lesions. 相似文献
26.
R A Hague P L Yap J Y Mok O B Eden N A Coutts J G Watson F D Hargreaves J M Whitelaw 《Archives of disease in childhood》1989,64(8):1146-1150
Eight children with symptoms of HIV infection were treated for 12-26 months (median 14 months) with infusions of intravenous immunoglobulin (200 mg/kg) every three weeks. Significant improvement was noted in all children in terms of weight gain, number of infectious episodes, and days spent in hospital. This resulted in a 49% saving in cost on treatment compared with costs accrued previously during inpatient admissions. Immunoglobulin concentrations, which were raised at the start of treatment were not altered, and T4 counts continued to decline slowly. HIV core antigen was detected in four children before treatment, but all became core antigen negative after treatment was commenced, this effect being sustained in three. Intravenous immunoglobulin therefore has major clinical benefit, and by reducing viral activity may delay disease progression. 相似文献
27.
Contact allergy in relation to hand eczema and atopic diseases in north Norwegian schoolchildren 总被引:3,自引:0,他引:3
Dotterud LK, Falk ES. Contact allergy in relation to hand eczema and atopic diseases in north Norwegian schoolchildren. Acta Psediatr 1995;84:402–6. Stockholm. ISSN 0803–5253
Patch testing was carried out in 424 schoolchildren (223M, 201F), aged 7–12 years, in northern Norway. In 99 (23.3%) of these children, one or more allergic patch test reactions were demonstrated; 30 children reacted to two and 6 to three or more substances; 53 irritant reactions were recorded in 33 (7.8%) of those tested. From a total of 144 positive tests, the most common allergen was nickel (14.9%), followed by cobalt (5.7%), kathon CG (5.2%), lanolin (1.7%) and neomycin (1.4%). Both allergic and irritant reactions were found twice as frequently in girls as in boys. Positive patch tests were significantly more frequent in atopic (28.8%) than in non–atopic (17.9%) children, being most pronounced in atopic girls (37.4%). Hand eczema was reported to have occurred or to be present in 6.5% of cases. Twenty–nine of 36 children reporting hand eczema participated in the clinical examination. Altogether 15 (3.5%) children had hand eczema at the time of the clinical examination but 12 of these children had no previous history of hand eczema. In 14 of these 15 subjects, the eczema was localized to the back of the hands, with 13 having atopic dermatitis. In 4 of these 15 children, an allergic patch test reaction was found; however, in only 2 of these 4 was the test considered to be clinically relevant for the diagnosis allergic hand eczema. In conclusion, irritant hand eczema may occur in early childhood and is most prevalent in children with atopic dermatitis 相似文献
Patch testing was carried out in 424 schoolchildren (223M, 201F), aged 7–12 years, in northern Norway. In 99 (23.3%) of these children, one or more allergic patch test reactions were demonstrated; 30 children reacted to two and 6 to three or more substances; 53 irritant reactions were recorded in 33 (7.8%) of those tested. From a total of 144 positive tests, the most common allergen was nickel (14.9%), followed by cobalt (5.7%), kathon CG (5.2%), lanolin (1.7%) and neomycin (1.4%). Both allergic and irritant reactions were found twice as frequently in girls as in boys. Positive patch tests were significantly more frequent in atopic (28.8%) than in non–atopic (17.9%) children, being most pronounced in atopic girls (37.4%). Hand eczema was reported to have occurred or to be present in 6.5% of cases. Twenty–nine of 36 children reporting hand eczema participated in the clinical examination. Altogether 15 (3.5%) children had hand eczema at the time of the clinical examination but 12 of these children had no previous history of hand eczema. In 14 of these 15 subjects, the eczema was localized to the back of the hands, with 13 having atopic dermatitis. In 4 of these 15 children, an allergic patch test reaction was found; however, in only 2 of these 4 was the test considered to be clinically relevant for the diagnosis allergic hand eczema. In conclusion, irritant hand eczema may occur in early childhood and is most prevalent in children with atopic dermatitis 相似文献
28.
Recombinant interferon gamma has been used prophylactically in children with chronic granulomatous disease, but its role in the treatment of acute infective episodes has not been defined. A 3 year old boy presented with multiple candidal liver abscesses and was given intravenous antifungal treatment and he showed initial improvement. After six weeks his erythrocyte sedimentation rate and C reactive protein remained raised, and a computed tomogram showed a single abscess in the left lobe of the liver from which pus was drained and Staphylococcus aureus isolated. During the next eight months the abscess persisted despite appropriate intravenous antibiotics and percutaneous drainage. Subphrenic extension precluded definitive surgery. Nine months after initial presentation recombinant interferon gamma 0.05 mg/m2 intravenously was commenced three times a week. Complete resolution occurred within two months. It is concluded that interferon gamma is useful in treating infective episodes, and further study of the use of prophylactic antimicrobial treatment and intermittent interferon gamma during acute episodes is now required. 相似文献
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