Children with urea cycle disorders present with hyperammonaemiaand its non-specific symptoms. Acute hyperammonaemia is a medicalemergency as the developmental and neurological outcome dependson the duration of hyperammonaemic coma [1]. The longer endogenousprotein catabolism continues, the more ammonia will be producedand accumulate and the greater is the risk of coma. To minimizepermanent brain damage, early diagnosis and appropriate therapyis mandatory. With diagnosis of hyperammonaemia, it is essentialto differentiate between urea cycle defects and other causesof encephalopathy. In Figure 1 a practicable flowchart for establishingthe correct diagnosis is depicted [2]. The emergency therapyin children with inborn metabolic disorders presenting  相似文献   
32.
Aktuelles zum Thema Latex-Allergie     
Angelika Heese  Ulrike Lacher  Hans Uwe Koch  Janna Kubosch  Yasmin Ghane  Klaus-Peter Peters 《Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und verwandte Gebiete》1996,47(11):817-824
Zusammenfassung Die Typ I-Allergien gegen Latex sind in den vergangenen Jahren zu einem zunehmenden berufsdermatologischen Problem geworden, zumal mindestens 10% der Angestellten im Gesundheitswesen betroffen sind. In der Dermatologischen Klinik der Universit?t Erlangen-Nürnberg stieg die Anzahl der j?hrlich diagnostizierten Patienten mit Latexallergien von 1989 bis 1995 auf das 12fache, wobei der Anteil der schweren, generalisierten Formen der Erkrankung von 10,7% (1989/1990) auf 44% (1994/1995) zunahm. Unter den m?glichen Ausl?sern der Latexallergie (wasserl?sliche Proteine mit Molekulargewichten von 2 bis 200 kD) sind mindestens 5 Hauptproteine mit bereits bekannter Prim?rstruktur zu berücksichtigen. Zus?tzlich gibt es Hinweise für Markerproteine, die in bestimmten Risikogruppen geh?uft zur Ausl?sung spezifischer IgE-Antik?rper führen (z.B. 46 kD-Protein in medizinischen Berufen, 14,6 kD- und 27 kD-Proteine bei Kindern mit Spina bifida). Das Vorkommen von Kreuzreaktionen zwischen Latex und unterschiedlichen Früchten (besonders Avocado, Kiwi, Banane, E?kastanie) bei 60 bis 70% der Latexallergiker ist bei der allergologischen Abkl?rung und Beratung dieser Patienten zu beachten. Wesentliche Aspekte der Prophylaxe umfassen die konsequente Umstellung medizinischer Einrichtungen auf ungepuderte Latexhandschuhe mit niedrigem Proteingehalt. Eine Zusammenstellung von OP- und Untersuchungshandschuhen, welche Angaben über die von uns ermittelten Proteinkonzentrationen (modifizierte Lowry-Methode und Hochdruck-Flüssigkeits-Chromatographie, HPLC) enth?lt, soll ein Leitfaden bei der Auswahl allergologisch geeigneter Handschuhe sein. Eingegangen am 10. August 1996 Angenommen am 21. August 1996  相似文献   
33.
A newly established murine immature dendritic cell line can be differentiated into a mature state, but exerts tolerogenic function upon maturation in the presence of glucocorticoid          下载免费PDF全文
Bros M  Jährling F  Renzing A  Wiechmann N  Dang NA  Sutter A  Ross R  Knop J  Sudowe S  Reske-Kunz AB 《Blood》2007,109(9):3820-3829
The phenotype and function of murine dendritic cells (DCs) are primarily studied using bone-marrow-derived DCs (BM-DCs), but may be hampered by the heterogeneous phenotype of BM-DCs due to their differential state of maturation. Here we characterize a newly established murine DC line (SP37A3) of myeloid origin. During maintainance in the presence of granulocyte-macrophage colony-stimulating factor (GM-CSF) and M-CSF, SP37A3 cells resemble immature DCs characterized by low expression of major histocompatibility complex (MHC) II and costimulatory molecules and low T-cell stimulatory capacity. Upon stimulation, SP37A3 cells acquire a mature phenotype and activate naive T cells as potently as BM-DCs. Similar to BM-DCs, SP37A3 cells activated in the presence of dexamethasone-induced regulatory T cells, which were anergic upon restimulation and suppressed proliferation of naive T cells. This tolerogenic state was reflected by lower expression levels of costimulatory molecules and proinflammatory cytokines compared with mature cells, as well as up-regulated expression of FcgammaRIIB and interleukin-1RA (IL-1RA). SP37A3 cells were responsive to dexamethasone even when applied at later time points during activation, suggesting functional plasticity. Thus, DC line SP37A3 represents a suitable model to study functions of immature and mature as well as tolerogenic myeloid DCs, circumventing restrictions associated with the use of primary DCs and BM-DCs.  相似文献   
34.
Hematopoietic progenitor cell colony growth differentiates chronic myelomonocytic leukemia from reactive monocytosis     
Birrer A  Jusufi F  Bernimoulin M  Tichelli A  Gratwohl A  Nissen-Druey C  Dirnhofer S  Meyer-Monard S  Stern M 《European journal of haematology》2008,81(4):267-272
In the absence of a cytogenetic abnormality or overt dysplasia, chronic myelomonocytic leukemia (CMML) may be difficult to be distinguished from reactive monocytosis. We have previously described a typical growth pattern in CMML patients, i.e., 'pseudonormal' colonies resembling granulocytic colonies but consisting entirely of monocytic cells when stained. To study the utility of the colony forming unit cell assay (CFU-C) as a diagnostic tool in patients with monocytosis, we analyzed a cohort of 48 consecutive patients referred to our institution with peripheral blood monocytosis. Thirty-six patients fulfilled the WHO criteria for CMML; 12 were diagnosed with reactive monocytosis. Of the patients with CMML, 28 showed pseudonormal growth with or without leukemic cluster growth, another four showed exclusively leukemic growth. None of the patients with reactive monocytosis showed either leukemic or pseudonormal growth. With a specificity of 100% and a sensitivity of 89%, the CFU-C assay has a unique potential to distinguish CMML from reactive monocytosis.  相似文献   
35.
In vivo regulation of inducible no synthase in immune-mediated liver injury in mice     
Koerber K  Sass G  Kiemer AK  Vollmar AM  Tiegs G 《Hepatology (Baltimore, Md.)》2002,36(5):1061-1069
Inducible nitric oxide synthase (iNOS) has been shown to play an important role in the development of liver injury. iNOS deficiency protects mice from hemorrhage/resuscitation as well as from cytokine-mediated liver injury, for example, after administration of concanavalin A (con A). Here we investigated the in vivo effects of tumor necrosis factor (TNF)-alpha and/or interferon (IFN)-gamma, two mediators of con A-induced liver injury, the TNF receptor (TNFR) usage leading to iNOS expression, and its connection with nuclear factor kappaB (NF-kappaB) activation. In conclusion, iNOS expression in vivo is dependent on both TNF-alpha and IFN-gamma. Although con A-induced liver injury depends on both TNFR1 and TNFR2, TNF-dependent iNOS expression is mediated exclusively by TNFR1 and requires NF-kappaB activation.  相似文献   
36.
37.
38.
Experiences of Urban African Americans with Cancer Pain     
Angelika Maly  Navdeep Singh  April Hazard Vallerand 《Pain Management Nursing》2018,19(1):72-78
The experience of cancer pain is poorly understood from the perspective of African Americans, who experience higher levels of pain, more pain-related distress, and poorer function than Caucasians. Decreased perceived control over pain may play a greater role for African American patients, affecting pain-related distress and function. The purpose of this study was to add to the understanding of cancer pain and perceived control over pain in African Americans, from the patients’ perspective. This qualitative inquiry was part of a larger mixed-methods study testing an intervention to improve pain, pain-related distress, and functional status through increasing perceived control over pain. Participants were recruited from the waiting room of an urban comprehensive cancer and interviewed in their homes. Interviews with 18 adult cancer patients who self-identified as African American and reported experiencing moderate to severe pain (>4 on a 0–10 scale) within the past two weeks were included. Qualitative interviews were audiotaped, transcribed, and analyzed using a constant comparative method. Two major themes emerged from this qualitative inquiry: struggles of the chronic pain experience and benefits of perceived control over pain. Each theme contained several categories. The study unveiled the participants account of both struggles of the chronic pain experience and barriers of perceived control that can be assessed for and targeted in nursing intervention. Benefits to having perceived control over pain were also illustrated in the participants’ narratives.  相似文献   
39.
40.
Screening for impaired liver function as a risk factor for drug safety at hospital admission of surgical patients     
Strobach  Dorothea  Poppele  Angelika  Mannell  Hanna  Andraschko  Monika  Schiek  Susanne  Bertsche  Thilo 《International journal of clinical pharmacy》2020,42(1):124-131
International Journal of Clinical Pharmacy - Background Hepatic insufficiency can affect patient safety and should therefore be considered during drug therapy. Hospital admission offers an ideal...  相似文献   
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31.
   Introduction
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