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| 1 | Primary intestinal lymphangiectasia in an adult patient:A case report and review of literature显示文摘BACKGROUND Primary intestinal lymphangiectasia(PIL),first described in 1961,is a rare disorder of unknown etiology resulting in protein-losing enteropathy.The disease is characterized by dilatation and leakage of intestinal lymph vessels leading to hypoalbuminemia,hypogammaglobulinemia,and lymphopenia.Since the severity and location of lymph vessels being affected can vary considerably,the range of associated symptoms is wide from mild lower-limb edema to generalized edema,abdominal and/or pleural effusion,and recurrent diarrhea,among others.Although usually developing in early childhood,we present the case of a 34-yearold woman with PIL.Moreover,we performed a literature review systematically assessing clinical presentation,and provide a practical approach to facilitate diagnosis and therapy of PIL in adults.CASE SUMMARY Our patient presented with unspecific symptoms of abdominal discomfort,fatigue,nausea,and recurrent edema of the lower limbs.Interestingly,a striking collinearity of clinical symptoms with female hormone status was evident.Additionally,polyglobulia,hypoalbuminemia,hypogammaglobulinemia,and transient lymphocytopenia were evident.Due to suspicion of a bone marrow disease,an extensive diagnostic investigation was carried out excluding secondary causes of polyglobulinemia and hypoalbuminemia.The diagnosis of primary intestinal lymphangiectasia was established after 22 wk by histological analysis of biopsy samples obtained via enteroscopy.Consecutively,the patient was put on a high-protein and low-fat diet with medium-chain triglycerides supplementation leading to significant improvement of clinical symptoms until 2 years of follow-up.CONCLUSION PIL can be the reason for cryptogenic hypoalbuminemia,hypogammaglobulinemia,and lymphopenia in adulthood.Due to difficulty in correct diagnosis,treatment initiation is often delayed despite being effective and welltolerated.This leads to a significant disease burden in affected patients.PIL is increasingly been recognized in adults since the majority of case reports were published within the last 10 years,pointing towards an underestimation of the true prevalence.The association with female hormone status warrants further investigation. | Rudolf Huber Georg Semmler Alexander Mayr Felix Offner Christian Datz | 2020 | World Journal of Gastroenterology2020，26，48： | 6 |
| 2 | Characterization and amino acid sequence of a fatty acid-binding protein from human heart显示文摘 | Offner GD Brecher P Sawlivich WB | 1988 | Biochem J1988，252，1： | 1 |
| 3 | Estrogen potentiates treatmentwith T-cell receptnr protein of female mice with experimental encephalomyelitis 显示文摘 | Offner H Adland K Zamora A | 2000 | J Clin Invest2000，105，10： | 1 |
| 4 | Induction of heat shock protein 70 by zine-bis-(DL-hydrogenaspartate) reduces cy- tokine liberation, apotosis, and mortality rate in a rate model of LDIO0 endotoxemia显示文摘 | Klosterhalfen B Hauptmann S Offner FA | 1997 | Shock1997，7，4： | 1 |
| 5 | Experimental stroke induces massive, rapid activation of the peripheral im- mune system显示文摘 | Offner H Subramanian S Parker SM | 2006 | Journal of Cerebral Blood Flow and Metabo- lism2006，26，5： | 1 |
| 6 | Identification of a 130-kilodalton human billaryconcanavalin a binding protein as aminopeptidase N显示文摘 | Offner GD Gong D Afdhal NH | 1994 | Gastroenterology1994，106，3： | 1 |
| 7 | Hemodynamic and oxygenation profiles in the early period after hyperbaric oxygen therapy:an observational study of intensivecare patients显示文摘 | Ratzenhofer-Komenda B Offner A Quehenberger F | 2003 | Acta Anaesthesiol Scand2003，47，5： | 1 |
| 8 | Comparison ofcystatin c versus creatinine for detection of mild renal failure显示文摘 | Chantrel F Agin A Offner M | 2000 | Clin Nephrol2000，54，5： | 1 |
| 9 | A retrospectiveanalysis of geriatric trauma patients: venous lactate is a betterpredictor of mortality than traditional vital signs显示文摘 | Salottolo KM Mains CW Offner PJ | 2013 | Scand JTrauma Resusc Emerg Med2013，21，： | 1 |
| 10 | Anatomic na- ture and surgical significance of anal sinus and anal in-tramuscular glands显示文摘 | Klosterhalfen B Offner F Vogel P | 1991 | Dis Colon Rectum1991，34，2： | 1 |
| 11 | Hemodynamic and oxygenation profiles in the early period after hyperbaric oxygen therapy: an observational study of intensive-care patients显示文摘 | Ratzenhofer-Komenda B Offner A Quehenberger F | 2003 | Acta Anaesthesiol Scand2003，47，5： | 1 |
| 12 | Avoidance of abdominal compartment syndrome in damage control laparotomy after trauma 显示文摘 | Offner PJ DeSouza AL Moore EE | 2001 | Arch Surg2001，136，： | 1 |
| 13 | General surgery residents improve efficiency but not outcome of trauma care显示文摘 | Offner PJ Hawkes A Madayag R | 2003 | J Trauma2003，55，1： | 1 |
| 14 | Bone marrow necrosis 显示文摘 | Janssens AM Offner FC VanHove WZ | 2000 | Cancer2000，88，8： | 1 |
| 15 | Human peritoneal mesothelial cells are more potent than ovarian cancer cells in producing tumor marker CA-125显示文摘 | Marth C Offner FA | 1996 | Gynecol Oncol1996，62，3： | 1 |
| 16 | New concepts in proeetion mask a- ligners显示文摘 | OFFNER A | 1975 | Optical Engineering1975，14，2： | 1 |
| 17 | Combined paravertebral and intrathecal vs thoracic epidural analgesia for post-thoracotomy pain relief显示文摘 | Dango S Harris S Offner K | 2013 | Br J Anaesth2013，110，3： | 1 |
| 18 | Comparison of cystatin C versus creatinine for detection of mild renal failure显示文摘 | Agin A Offner M | 2000 | Clin Nephrol2000，54，5： | 1 |
| 19 | IF-Nalpha activates dormant haematopoietic stem cells in vivo 显示文摘 | ESSERS M A OFFNER S BLANCO-BOSE W E | 2009 | Nature2009，458，： | 1 |
| 20 | The amino-terminal sequence of MUC5B contains conserved multifunctional D domains: implications for tissue-specific mucin functions显示文摘 | Offner GD Nunes DP Keates AC | 1998 | Biochem Biophys Res Commun1998，251，1： | 1 |
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