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| 1 | Juvenile hemochromatosis:HAMP mutation and severe iron overload treated with phlebotomies and deferasirox显示文摘Juvenile hemochromatosis(JH) is a rare condition classified as an autosomal recessive disorder that leads to severe iron absorption. JH usually affects people under the age of 30 and presents symptoms such as chronic liver damage,hypogonadotropic hypogonadism,cardiac diseases and endocrine dysfunctions. The present case reports a 29-year-old Brazilian woman with JH condition due to HAMP mutation(g.47G>A),treated with phlebotomies and deferasirox. She presented symptoms such as weakness,skin hyperpigmentation,joint pain in the shoulders and hands and amenorrhea. First laboratory tests showed altered biochemical parameters [serum ferritin(SF): 5696 ng/mL,transferrin saturation(TS): 85%]. After sessions of phlebotomies(450 mL every 15 d),the patient presented partial symptomatic improvements and biochemical parameters(SF: 1000 ng/mL,Hb: 11 g/dL). One year later,deferasirox(15 mg/kg per day) was introduced to the treatment,and the patient showed total symptomatic improvement,with significant clearing of the skin,SF: 169 ng/mL,and TS: 50%. Furthermore,after the combined deferasirox-phlebotomy therapy,magnetic resonance imaging measurements revealed normalized level for liver iron(30 μmol/g; reference value < 36 μmol/g). In conclusion,combined deferasirox-phlebotomy treatment was able to normalize iron levels and improve symptoms. | Manuel A Lescano Letícia C Tavares Paulo C J L Santos | 2017 | World Journal of Clinical Cases2017，5，10： | 2 |
| 2 | Antioxi- dant strategies for preventing oxidative flavor deterioration of foods enriched with n-3 polyunsaturated lipids: A comparative evaluation 显示文摘 | JACOBSEN C LET M B NIELSEN N S | 2008 | Trends Food Sci Technol2008，19，： | 1 |
| 3 | cobalt catalysts显示文摘 | Luwei C Weng K Let aLHydrogen orsynthesis gas production via the partial oxidation ofmethane over supported nickel | 2007 | Int J Hydrogen Energy2007，32，6： | 1 |
| 4 | Ascorbyl palmitate,y- tocopherol, and EDTA affect lipid oxidation in fish oil enriched salad dressing differently显示文摘 | Let M B Jacobsen C Meyer A S | 2007 | Journal of Agricuhural and Food Chemistry2007，55，6： | 1 |
| 5 | A ubiqui- tin C-terminal hydrolase gene on the proximal short arm of the X chromosome: implications for X-linked retinal disorders显示文摘 | SWANSON D A FREUND C L PLODER Let a/ | 1996 | Human Molecular Genetics1996，5，4： | 1 |
| 6 | Proeoagulants and osteonecrosis显示文摘 | JONES L C MONT M A LET B | 2003 | J Rheumatol2003，30，4： | 1 |
| 7 | Development of biode- gradable films from whey proteins by cross-linking and entrapment in cellulose显示文摘 | LET C LETENDRE M ISPAS S P | 2000 | J Agric Food Chem2000，48，11： | 1 |
| 8 | A study on the thermal conductivity of compact-ed bentonites显示文摘 | TANG A M C UI Y J LET T | 2008 | Applied Clay Science2008，41，3： | 1 |
| 9 | Functional reorganization of the brain in recovery from stfiatocapsular infarction in man 显示文摘 | Weiller C Chol let F Friston KJ | 1992 | Ann Neurol1992，3，1： | 1 |
| 10 | Sulfide from anaerobic waste treatment effluent of a papermill显示文摘 | Buisman C J Letting A G | 1990 | Wat Res1990，24，3： | 1 |
| 11 | Sulfide from anaerobic waste treatment effluent of a papermill显示文摘 | Buisman C J Letting A G | 1990 | Wat Res1990，24，3： | 1 |
| 12 | Use of enzymetic transesterified palm stearin- sunflower oil blends in the preparation of table margarine 显示文摘 | MING L O GHAZALI M LET C C | 1999 | Food Chemistry1999，64，1： | 1 |
| 13 | Homogenization conditions affect the oxidative stability of fish oil enriched milk emulsions:lipid oxidation显示文摘 | Let MB Jacobsen C Sφrensen AD | 2007 | J Agric Food Chem2007，55，： | 1 |
| 14 | Use of -irradia- tion to produce films from whey, casein and soya proteins: structure and functionals characteristics显示文摘 | LACROIX M LET C OUATTARA B | 2002 | Radiat Phys Chem2002，63，36： | 1 |
| 15 | Action of beta-amyloid peptidel-40 on IHVA and its modulation by ginkgolide B显示文摘 | Jurgen H Let C ChangJin LIU | 2006 | Aeta Physiologica Sinica2006，58，1： | 1 |
| 16 | Capsaicin-induced apoptosis of FaDu human pharyngeal squamous carcinoma cells显示文摘 | LET D JIN D C RHO S | 2012 | Yonsei Medical Jour- nal2012，53，4： | 1 |
| 17 | Overweight, obesity, diabetes, and risk of breast cancer' interlocking pieces of the puzzle显示文摘 | Let Vecchia C Giordano SH Hortobag yi GN | 2011 | Oncologist2011，16，6： | 1 |
| 18 | Fluid mannge- ment in acute lung injury and ards显示文摘 | Roch A Guervilly C Papazian Let | 2011 | Ann Intensive Care2011，1，1： | 1 |
| 19 | Sefer ha-Middot: A mid-twelfth-cen- tury text on arithmetic and geometry attributed to Abraham Ibn Ezra 显示文摘 | LET BURNETr C | 2006 | Aleph2006，，6： | 1 |
| 20 | Sensory stability and oxidation of fish oil enriched milk is affected by milk storage temperature and oil quality显示文摘 | Let M B Jacobsen C Meyer A S | 2005 | International Dairy Journal2005，15，2： | 1 |
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