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| 1 | Juvenile polyposis syndrome显示文摘Juvenile polyposis syndrome is a rare autosomal dominant syndrome characterized by multiple distinct juvenile polyps in the gastrointestinal tract and an increased risk of colorectal cancer.The cumulative life-time risk of colorectal cancer is 39% and the relative risk is 34.Juvenile polyps have a distinctive histology characterized by an abundance of edematous lamina propria with inflammatory cells and cystically dilated glands lined by cuboidal to columnar epithelium with reactive changes.Clinically,juvenile polyposis syndrome is defined by the presence of 5 or more juvenile polyps in the colorectum,juvenile polyps throughout the gastrointestinal tract or any number of juvenile polyps and a positive family history of juvenile polyposis.In about 50%-60% of patients diagnosed with juvenile polyposis syndrome a germline mutation in the SMAD4 or BMPR1A gene is found.Both genes play a role in the BMP/TGF-beta signalling pathway.It has been suggested that cancer in juvenile polyposis may develop through the socalled 'landscaper mechanism' where an abnormal stromal environment leads to neoplastic transformation of the adjacent epithelium and in the end invasive carcinoma.Recognition of this rare disorder is important for patients and their families with regard to treatment,follow-up and screening of at risk individuals.Each clinician confronted with the diagnosis of a juvenile polyp should therefore consider the possibility of juvenile polyposis syndrome.In addition,juvenile polyposis syndrome provides a unique model to study colorectal cancer pathogenesis in general and gives insight in the molecular genetic basis of cancer.This review discusses clinical manifestations,genetics,pathogenesis and management of juvenile polyposis syndrome. | Lodewijk AA Brosens Danielle Langeveld W Arnout van Hattem Francis M Giardiello G Johan A Offerhaus | 2011 | World Journal of Gastroenterology2011，17，44： | 7 |
| 2 | Autoantibody profiles in autoimmune hepatitis and chronic hepatitis C identifies similarities in patients with severe disease显示文摘AIM To determine how the auto-antibodies(Abs) profiles overlap in chronic hepatitis C infection(CHC) and autoimmune hepatitis(AIH) and correlate to liver disease.METHODS Levels of antinuclear Ab, smooth muscle antibody(SMA) and liver/kidney microsomal-1(LKM-1) Ab and markers of liver damage were determined in the sera of 50 patients with CHC infection, 20 AIH patients and 20 healthy controls using enzyme linked immunosorbent assay and other immune assays. RESULTS We found that AIH patients had more severe liver disease as determined by elevation of total Ig G, alkaline phosphatase, total serum bilirubin and serum transaminases and significantly higher prevalence of the three non-organ-specific autoantibodies(auto-Abs) than CHC patients. Antinuclear Ab, SMA and LKM-1 Ab were also present in 36% of CHC patients and related to disease severity. CHC cases positive for auto-Abs were directly comparable to AIH in respect of most markers of liver damage and total Ig G. These cases had longer disease duration compared with auto-Ab negative cases, but there was no difference in gender, age or viral load. KLM-1+ Ab CHC cases showed best overlap with AIH. CONCLUSION Auto-Ab levels in CHC may be important markers of disease severity and positive cases have a disease similar to AIH. Auto-Abs might have a pathogenic role as indicated by elevated markers of liver damage. Future studies will unravel any novel associations between these two diseases, whether genetic or other. | Kawa Amin Aram H Rasool Ali Hattem Taha AM Al-Karboly Taher E Taher Jonas Bystrom | 2017 | World Journal of Gastroenterology2017，23，8： | 5 |
| 3 | Topical silverfor treating infected wounds 显示文摘 | Vermeulen H Van Hattem JM Storm Versloot MN | 2007 | Cochrane Database Syst Rev2007，24，1： | 1 |
| 4 | Large genomic deletions of SMAIM, BMPRIA and PTEN in juvenile polyposis 显示文摘 | van Hattem WA Brosens LA de Leng WW | 2008 | Gut2008，57，5： | 1 |
| 5 | Increased cyclooxygenase -2 expression in juvenile polyposis syndrome 显示文摘 | van Hattem WA Brosens LA Marks SY | 2009 | Clin Gastroenterol Hepatol2009，7，1： | 1 |
| 6 | Design temperaturedata for surface water heating and cooling systems 显示文摘 | HATTEMER B KAVANAUGH S P | 2005 | ASHRAE Transactions2005，，1： | 1 |
| 7 | Family Relations 显示文摘 | Leone Poindexter Hummer and Alberta Koch - Hattem | 1986 | 35 (4) : 523 - 5291986，35，4： | 1 |
| 8 | Redox homeostasis, antioxidant defense, and methylglyoxal detoxification as markers for salt tolerance in Pokkali rice显示文摘 | Hattem El-Shabrawi Bhumesh Kumar Tanushri Kaul Malireddy K. Reddy Sneh L. Singla-Pareek Sudhir K. Sopory | 2010 | Protoplasma2010，，1： | 1 |
| 9 | Gastrointestinal polyposis syndrome显示文摘 | Brosens L A van Hattem W A Jansen M | 2007 | Curr Mol Med2007，7，： | 1 |
| 10 | Design temperature data for surface water heating and cooling systems 显示文摘 | HATTEMER B KAVANAUGH S P | 2005 | ASHRAE Transactions2005，111，1： | 1 |
| 11 | Inheritance of allozymes and hybridization in two European Tilia species显示文摘 | Fromm M Hattemer H H | 2003 | Heredity2003，91，： | 1 |
| 12 | HIF-1A gene muta- tions associated with higher microvessel density in endometrial car- cinomas 显示文摘 | Horree N Groot AJ van Hattem WA | 2008 | Histopathology2008，52，5： | 1 |
| 13 | Large genomic deletions of SMAD4,BMPR1A or PTEN in juvenile polyposis显示文摘 | van Hattem WA Brosens AA de Leng WW | 2007 | Gut2007，57，5： | 1 |
| 14 | Systemic therapy with immunosuppressive agents and retinoids in hidradenitis suppurativa:a systematic review显示文摘 | Blok JL van Hattem S Jonkman MF | | 0，，02： | 1 |
| 15 | Risk of colorectal cancer in juvenile polyposis显示文摘 | Brosens LA van Hattem A Hylind LM | 2007 | Gut2007，56，7： | 1 |
| 16 | Design temperature data for surface water heating and cooling systems 显示文摘 | Hattemer B Kavanaugh S P | 2005 | ASHRAE Transactions2005，111，1： | 1 |
| 17 | Risk of eolorectal cancer in juvenil epolyposis 显示文摘 | Brosens LA van Hattem A Hylind LM | 2007 | Gut2007，56，7： | 1 |
| 18 | Syslemic therapy with immunosuppressive agents and retinoids in hidradenitis sup- purativa: a systematic review 显示文摘 | Blok JL van Hattem S Jonkman MF | 2013 | Br J Dermatol2013，168，2： | 1 |
| 19 | Design temperature data for surface water heating and cooling systems 显示文摘 | Hattemer B Kavanaugh S P | 2005 | ASHRAE Transaction2005，，： | 1 |
| 20 | Topical silver for treating infected wounds显示文摘 | Vermeulen H van Hattem JM Storm- Versloot MN | 2007 | Coehrane Data- base Syst Rev2007，24，00： | 1 |
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