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| 1 | Therapy-refractory gastrointestinal motility disorder in a child with c-kit mutations显示文摘Constipation and fecal impaction are frequent and distressing complaints in pediatric gastroenterology. Especially in neurologically handicapped children, treatment of severe forms of slow-transit constipation (STC) can be difficult. In the majority of cases, STC is of unknown etiology. However, in recent years, there is growing evidence that interstitial cells of Cajal (ICCs), which serve as electrical pacemakers and generate spontaneous electrical slow waves in the gastrointestinal tract, might play an important role in the pathophysiology of STC. It remains unclear whether morphological ICC alterations seen in affected patients are based on congenital developmental anomalies, or whether they are a consequence of long-term constipation with secondary damage of the gastrointestinal nervous system. To the best of our knowledge, we present the first case of a patient with histological alterations in ICC morphology who displayed multiple alterations of c-kit at the level of mRNA. The protein encoded by c-kit is the receptor tyrosine kinase Kit (CD117), which is crucial for development and function of ICCs. Therefore, these findings provide a new explanation for congenital alterations of ICC development that result in gastrointestinal motility disorders. | Christian Breuer Jun Oh Gerhard J Molderings Michael Schemann Birgit Kuch Ertan Mayatepek Rüdiger Adam | 2010 | World Journal of Gastroenterology2010，16，34： | 7 |
| 2 | A concise, practical guide to diagnostic assessment for mast cell activation disease显示文摘As recognition of mast cell(MC) involvement in a range of chronic inflammatory disorders has increased, diagnosticians' suspicions of MC activation disease(MCAD) in their chronically mysteriously inflamed patients have similarly increased. It is now understood that the various forms of systemic mastocytosis- diseases of inappropriate activation and proliferation of MCs seemingly driven by a small set of rare, usually constitutively activating mutations in assorted MC regulatory elements-comprise merely the tip of the MCAD iceberg, whereas the far larger and far more clinically heterogeneous(and thus more difficult to recognize) bulk of the iceberg consists of assorted forms of MC activation syndrome(MCAS) which manifest little to no abnormal MC proliferation and may originate from a far more heterogeneous set of MC mutations. It is reasonable to suspect MCAD when symptoms and signs of MC activation are present and no other diagnosis better accounting for the full range of findings is present. Initial laboratory assessment should include not only routine blood counts and serum chemistries but also a serum total tryptase level, which helps direct further evaluation for mastocytosis vs MCAS. Appropriate tissue examinations are needed to diagnose mastocytosis, while elevated levels of relatively specific mast cell mediators are sought to support diagnosis of MCAS. Whether assessing for mastocytosis or MCAS, testing is fraught with potential pitfalls which can easily yield false negatives leading to erroneous rejection of diagnostic consideration of MCAD in spite of a clinical history highly consistent with MCAD. Efforts at accurate diagnosis of MCAD are worthwhile, as many patients then respond well to appropriately directed therapeutic efforts. | Lawrence B Afrin Gerhard J Molderings | 2014 | World Journal of Hematology2014，3，1： | 3 |
| 3 | Omalizumab treatment of systemic mast cell activation disease:experiences from four cases显示文摘 | Molderings GJ Raithel M Kratz F | | 0，，06： | 1 |
| 4 | Busemman Inlet for Hypersonic Speeds显示文摘 | Molder S Szpiro J | 1966 | Journal of Spacecraft and Rockets1966，3，8： | 1 |
| 5 | Busemann inlet for hypersonic speeds显示文摘 | Molder S Szpiro E J | 1966 | Journal of Spacecraft and Rockets1966，3，8： | 1 |
| 6 | Disparate Findings on the Role of Virulence Factors of Enterococcus faecalis in Mouse and Rat Models of Peritonitis 显示文摘 | Dupont H Montravers P Molder J | 1998 | Infection Immunity1998，66，6： | 1 |
| 7 | A review of systems and networks of the limbic forebrain/limbic midbrain 显示文摘 | Morgane P J Galler J R Molder D J | 2005 | Prog Neurobiol2005，75，2： | 1 |
| 8 | The occurrence of volatile N-nitrosaminesin Estonian meat products显示文摘 | YURCHENKO S MOLDER U | 2007 | Food Chemistry2007，100，： | 1 |
| 9 | Mast cell activation disease: A concise practical guide for diagnostic workup and therapeutic options显示文摘 | Molderings GJ Homann J Brettner S | 2014 | Dtsch Med Wochenschr2014，139，30： | 1 |
| 10 | The occurrence of volatile N-nitrosamines in Estonian meat products显示文摘 | Yurchenko Y MOlder | 2007 | Food Chemistry2007，100，： | 1 |
| 11 | Unsteady starting of high Mach number air inlets-A CFD study显示文摘 | Tahir R B and molder S | 2003 | AIAA2003，5191，： | 1 |
| 12 | Uncertainty in liquid chromatographic analysis of pharmaceutical product: Influence on various uncertainty sources显示文摘 | Signe Leito Kadi Molder Allan Kunnapas | 2006 | Journal of Chromatography A2006，，1121： | 1 |
| 13 | Anti-Mullerian hormone serum concentrations in normoovulatory and anovulatory women of reproductive age显示文摘 | Laven JS Molders AG Visser JA | 2004 | J Clip Endoerinol Metab2004，89，1： | 1 |
| 14 | Estonian Oil Shale Retorting Industry at a Cross-roads显示文摘 | MOLDER L | 2004 | Oil Shale2004，21，2： | 1 |
| 15 | Uncertainty in liquid chromatographic analysis of pharmaceutical product:Influence of various uncertainty sources显示文摘 | LEITO S MOLDER K KUNNAPAS A | 2006 | Journal of chromatography A2006，，1121： | 1 |
| 16 | Flux-profile relation- ships over a boreal forest-roughness sublayer corrections显示文摘 | Molder M Grelle A Lindroth A | 1999 | Agricultural and forest meteorology1999，98，： | 1 |
| 17 | Herb-layer di- versity in deciduous forests: Raised by tree richness or beaten by beech? 显示文摘 | Molder A Bemhardt-RSmermann M Schmidt W | 2008 | Forest Ecology and Management2008，256，3： | 1 |
| 18 | The occurrence of volatile N-nitrosamines in Estonian meat products显示文摘 | YURCHENKO S MOLDER U | 2007 | Food Chemistry2007，100，4： | 1 |
| 19 | Contractile hyporesponsiveness of hepatic arteries in humans with cirrhosis: Evidence for a receptor-specific mechanism显示文摘 | Michael Schepke J?rg Heller Sebastian Paschke Julia Thomas Martin Wolff Markus Neef Max Malago Gerhard J. Molderings Ulrich Spengler Tilman Sauerbruch | 2001 | Hepatology2001，，5： | 1 |
| 20 | Moldulation of noradrenaline release in human corpus cavernosum by presynaptic prostaglandin receptors显示文摘 | van Ahlen H Gothert M | 1992 | Int J Impot Res1992，4，： | 1 |
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