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| 1 | Metabolically based liver damage pathophysiology in patients with urea cycle disorders-A new hypothesis显示文摘The underlying pathophysiology of liver dysfunction in urea cycle disorders(UCDs) is still largely elusive. There is some evidence that the accumulation of urea cycle(UC) intermediates are toxic for hepatocyte mitochondria. It is possible that liver injury is directly caused by the toxicity of ammonia. The rarity of UCDs, the lack of checking of iron level in these patients, superficial knowledge of UC and an underestimation of the metabolic role of fumaric acid, are the main reasons that are responsible for the incomprehension of the mechanism of liver injury in patients suffering from UCDs. Owing to our routine clinical practice to screen for iron overload in severely ill neonates, with the focus on the newborns suffering from acute liver failure, we report a case of citrullinemia with neonatal liver failure and high blood parameters of iron overload. We hypothesize that the key is in the decreased-deficient fumaric acid production in the course of UC in UCDs that causes several sequentially intertwined metabolic disturbances with final result of liver iron overload. The presented hypothesis could be easily tested by examining the patients suffering from UCDs, for liver iron overload. This could be easily performed in countries with a high population and comprehensive national register for inborn errors of metabolism. Conclusion: Providing the hypothesis is correct, neonatal liver damage in patients having UCD can be prevented by the supplementation of pregnant women with fumaric or succinic acid, prepared in the form of iron supplementation pills. After birth, liverdamage in patients having UCDs can be prevented by supplementation of these patients with zinc fumarate or zinc succinylate, as well. | Ivan Ivanovski Milos Jesic Ana Ivanovski Livia Garavelli Petar Ivanovski | 2017 | World Journal of Gastroenterology2017，23，44： | 4 |
| 2 | Knowledge transfer and inter-firm relationships in industrial districts: the role of the leader finn 显示文摘 | Albino V Garavelli A C Schiuma G | 1998 | Teehnovation Journal1998，191，： | 1 |
| 3 | The Effect of Spatial Variables on the Economic and Environmental Performance of Bioenergy Production Chains显示文摘 | Yazan D M Garavelli A C Petruzzeli A M | 2011 | International Journal of Production Economics2011，131，1： | 1 |
| 4 | Knowledge transfer and inter-firm relationships in industrial districts: the role of the leader firm显示文摘 | Vito Albino A.Claudio Garavelli Giovanni Schiuma | 1998 | Technovation1998，，1： | 1 |
| 5 | The protein information resource(PIR)显示文摘 | Garavelli JS and Huang HZ | 2000 | Nucleic Acids Research2000，28，1： | 1 |
| 6 | Knowledge transfer and inter-finn relationships in industrial districts : The role of the leader firm 显示文摘 | Albino V A Garavelli C Schiuma G | 1998 | Technovation1998，19，1： | 1 |
| 7 | Knowledge TranMer and Inter-Firm Relationships in Industrial Districts: the Role of the Leader Firm显示文摘 | ALBINO V GARAVELLI A C SCHIUMA G | 1999 | Teehnovation1999，19，1： | 1 |
| 8 | Protein Information Resource: a commuity resource for expert annotation of protein data显示文摘 | Barker WC Garavelli JS Hou Z | 2001 | Nucleic Acids Res2001，29，1： | 1 |
| 9 | The RESID database of protein modifications: 2003 Developments显示文摘 | Garavelli J S | 2003 | Nucleic Acids Res2003，31，1： | 1 |
| 10 | Knowledge transfer and inter- firm relationships in industrial districts : The role of the leader firm 显示文摘 | Albino V Garavelli A C Schiuma G | 1998 | Technovation Journal1998，19，1： | 1 |
| 11 | Managing knowledge trans-fer by knowledge technologies显示文摘 | Garavelli A C Gorgoglione M S | | 0，，5： | 1 |
| 12 | Methods for modeling and supporting innovation processes in SMEs显示文摘 | SCOZZI B GARAVELLI C CROWSTON K | 2005 | European Journal of Innovation Management2005，8，1： | 1 |
| 13 | Operations Planning and Flexibility in a Supply Chain 显示文摘 | Aprile D Garavelli A C Giannoaacro I | 2005 | Production Planning and Control2005，，1： | 1 |
| 14 | A neural network application to subcontractor rating in construction forms显示文摘 | Albino V Garavelli A C | 1998 | International Journal of Project Manage- ment1998，16，1： | 1 |
| 15 | Knowledge Relationships in Industrial Districts : The Role of the Leader Firm 显示文摘 | Albino V Garavelli A Transfer and Inter-firm C Schiuma G | 1999 | Technovation1999，19，1： | 1 |
| 16 | Annotation of post-translational modifications in the Swiss-Prot knowledge base 显示文摘 | Farriol-Mathis N Garavelli J S Boeckmann B Duvaud S Gasteiger E Gateau A Veuthey A L Bairoch A | 2004 | Proteomics2004，4，6： | 1 |
| 17 | Hirschsprung disease, mental retardation, characteristic facial features, and mutation in the gene ZFHX1B (SIP1): confirmation of the Mowat-Wilson syndromme显示文摘 | Garavelli L Donadio A Zanacca C | 2003 | Am J Med Genet2003，116，4： | 1 |
| 18 | Knowledge transfer and inter-firm relationships in industrial districts:the role of the leader firm显示文摘 | Vito Albino A Claudio Garavelli Giovanni Schiuma | 1999 | Technovation1999，19，1： | 1 |
| 19 | Knowledge transfer and inter - firm relationships in industrial districts : the role of the leader firm显示文摘 | Albino V Garavelli A C Schiuma G | 1999 | Technovation1999，19，： | 1 |
| 20 | Knowledge transfer and inter- firm relations in industrial districts: the role of the leader firm显示文摘 | Albino V Garavelli A Sehiuma G | 1999 | Technovation1999，19，： | 1 |
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