{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T16:47:40Z","timestamp":1790959660946,"version":"4.1.0"},"reference-count":50,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2019,3,28]],"date-time":"2019-03-28T00:00:00Z","timestamp":1553731200000},"content-version":"vor","delay-in-days":55,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J cachexia sarcopenia muscle"],"published-print":{"date-parts":[[2019,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Cachexia has significant impact on the patients' quality of life and prognosis. It is frequently observed in patients with cancer, especially in advanced stages, but prevalence data for the overall population are lacking. Good quality estimates of cancer cachexia in general and for each of the major cancer types would be highly relevant for potential treatment development efforts in this field. Both the USA and European Union (EU) have implemented special clinical development rules for such rare disorders what are called \u2018orphan diseases\u2019. The cut\u2010off level for a disease to be considered an orphan disease in the USA is 200\u00a0000 people (0.06% of the population) and EU is 5 per 10\u00a0000 people (0.05% of the population).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>For this systematic review, we searched at PubMed (from inception to 31 January 2018) to identify clinical studies that assessed the prevalence of cachexia in cancer patients at risk. Studies reporting the prevalence of either cancer cachexia or wasting disease in the top\u201010 cancer types and 4 other selected cancer types known to be particularly commonly complicated by cachexia were included in this analysis (i.e. prostate cancer, breast cancer, colorectal cancer, melanoma, endometrial cancer, thyroid cancer, urinary bladder cancer, non\u2010hodgkin lymphoma, lung cancer, kidney and renal pelvis cancer, head and neck cancer, gastric cancer, liver cancer, and pancreatic cancer). We calculated the current burden of cancer cachexia, disease by disease, in the USA and in the EU and compared them to the current guidelines for the definition of orphan disease status.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We estimate that in 2014 in the USA, a total of 527\u00a0100 patients (16.5 subjects per 10\u00a0000 people of the total population), and in 2013 in the EU, a total of 800\u00a0300 patients (15.8 subjects per 10\u00a0000 people of the total population) suffered from cancer cachexia (of any kind). In the 14 separately analysed cancer types, the prevalence of cancer cachexia in the USA ranged between 11\u00a0300 (0.4\/10\u00a0000, gastric cancer) and 92\u00a0000 patients (2.9\/10\u00a0000, lung cancer) and in the EU between 14\u00a0300 (0.3\/10\u00a0000, melanoma of the skin) and 150\u00a0100 (3.0\/10\u00a0000, colorectal cancer).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The absolute number of patients affected by cancer cachexia in each cancer group is lower than the defined thresholds for orphan diseases in the USA and EU. Cancer cachexia in each subgroup separately should be considered an orphan disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jcsm.12402","type":"journal-article","created":{"date-parts":[[2019,3,28]],"date-time":"2019-03-28T11:30:26Z","timestamp":1553772626000},"page":"22-34","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":190,"title":["Orphan disease status of cancer cachexia in the USA and in the European Union: a systematic review"],"prefix":"10.1002","volume":"10","author":[{"given":"Markus S.","family":"Anker","sequence":"first","affiliation":[{"name":"Division of Cardiology and Metabolism, Department of Cardiology &amp; Berlin\u2010Brandenburg Center for Regenerative Therapies (BCRT) DZHK (German Centre for Cardiovascular Research), Partner Site Berlin, Charit\u00e9\u2010Universit\u00e4tsmedizin Berlin (CVK)  Berlin Germany"},{"name":"Department of Cardiology Charit\u00e9 Campus Benjamin Franklin  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Holcomb","sequence":"additional","affiliation":[{"name":"Independent biostatistician  Minneapolis MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurizio","family":"Muscaritoli","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine Sapienza University of Rome  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephan","family":"von Haehling","sequence":"additional","affiliation":[{"name":"Department of Cardiology and Pneumology, DZHK (German Center for Cardiovascular Research) University of G\u00f6ttingen Medical Center (UMG)  G\u00f6ttingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wilhelm","family":"Haverkamp","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Cardiology Charit\u00e9 University Medicine  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aminah","family":"Jatoi","sequence":"additional","affiliation":[{"name":"Mayo Clinic  Rochester MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John E.","family":"Morley","sequence":"additional","affiliation":[{"name":"Division of Geriatric Medicine Saint Louis University School of Medicine  Saint Louis MO 63104 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florian","family":"Strasser","sequence":"additional","affiliation":[{"name":"Oncological Palliative Medicine, Clinic Medical Oncology and Haematology, Department of Internal Medicine Cantonal Hospital, St Gallen and Integrated Cancer Rehabilitation  Klinik Gais Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulf","family":"Landmesser","sequence":"additional","affiliation":[{"name":"DZHK (German Centre for Cardiovascular Research), Partner Site Berlin Berlin Institute of Health (BIH)  Berlin Germany"},{"name":"Department of Cardiology Campus Benjamin Franklin Charit\u00e9 Universit\u00e4tsmedizin Berlin  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew J.S.","family":"Coats","sequence":"additional","affiliation":[{"name":"IRCCS San Raffaele Hospital, Pisana  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan D.","family":"Anker","sequence":"additional","affiliation":[{"name":"Division of Cardiology and Metabolism, Department of Cardiology &amp; Berlin\u2010Brandenburg Center for Regenerative Therapies (BCRT) DZHK (German Centre for Cardiovascular Research), Partner Site Berlin, Charit\u00e9\u2010Universit\u00e4tsmedizin Berlin (CVK)  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,3,28]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13539-013-0111-0"},{"key":"e_1_2_8_3_1","article-title":"Management of anorexia\u2010cachexia in late stage lung cancer patients","volume":"14","author":"Ferraro CD","year":"2012","journal-title":"J Hosp Palliat Nurs"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcsm.12167"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcsm.12106"},{"key":"e_1_2_8_6_1","unstructured":"https:\/\/www.statista.com\/statistics\/183457\/united\u2010states\u2010\u2010resident\u2010population\/"},{"key":"e_1_2_8_7_1","unstructured":"https:\/\/www.gpo.gov\/fdsys\/pkg\/PLAW\u2010107publ280\/html\/PLAW\u2010107publ280.htm"},{"key":"e_1_2_8_8_1","unstructured":"https:\/\/www.statista.com\/statistics\/253372\/total\u2010population\u2010of\u2010the\u2010european\u2010union\u2010eu\/"},{"key":"e_1_2_8_9_1","unstructured":"http:\/\/www.ema.europa.eu\/ema\/index.jsp?curl=pages\/regulation\/general\/general_content_000029.jsp"},{"key":"e_1_2_8_10_1","unstructured":"HowladerN NooneAM KrapchoM MillerD BishopK AltekruseSF et al.SEER Cancer Statistics Review 1975\u20102013 National Cancer Institute. Bethesda MD http:\/\/seer.cancer.gov\/csr\/1975_2013\/ based on November 2015 SEER data submission posted to the SEER web site April 2016."},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ijc.27711"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.18632\/oncotarget.20168"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11605-008-0505-z"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ehf2.12184"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2017.73.0226"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.29395"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0149-2918(05)80001-3"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1155\/2009\/693458"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.juro.2010.09.021"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1177\/0148607113502674"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.juro.2006.06.015"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-017-2958-1"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.ju.0000092764.81308.6a"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.ju.0000121440.82581.d3"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1177\/026921639200600406"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00520-014-2340-y"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bjc.6605578"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1406470"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-15-1127"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/0261-5614(83)90012-2"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcsm.12220"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nut.2009.04.013"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clnu.2008.06.013"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(10)70218-7"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0959-8049(97)10090-9"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0167-8140(86)80176-1"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2273.1997.00872.x"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1016\/0885-3924(91)90027-2"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2354.1999.00156.x"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00435383"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1177\/026921639300700105"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clnu.2005.05.001"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/0885-3924(96)00042-5"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1080\/0742-969X.1988.11882634"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrdp.2017.105"},{"key":"e_1_2_8_46_1","unstructured":"https:\/\/www.cancer.gov\/about\u2010cancer\/treatment\/drugs\/cancer\u2010type"},{"key":"e_1_2_8_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.critrevonc.2014.10.014"},{"key":"e_1_2_8_48_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000441-193211000-00002"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.7573\/dic.212265"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.3322\/canjclin.52.2.72"},{"key":"e_1_2_8_51_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcsm.12261"}],"container-title":["Journal of Cachexia, Sarcopenia and Muscle"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jcsm.12402","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/jcsm.12402","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jcsm.12402","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,10]],"date-time":"2023-09-10T14:42:37Z","timestamp":1694356957000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jcsm.12402"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,2]]},"references-count":50,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2019,2]]}},"alternative-id":["10.1002\/jcsm.12402"],"URL":"https:\/\/doi.org\/10.1002\/jcsm.12402","archive":["Portico"],"relation":{},"ISSN":["2190-5991","2190-6009"],"issn-type":[{"value":"2190-5991","type":"print"},{"value":"2190-6009","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,2]]},"assertion":[{"value":"2018-12-21","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-01-09","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-03-28","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}