{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T04:19:33Z","timestamp":1770956373834,"version":"3.50.1"},"reference-count":26,"publisher":"Wiley","issue":"21","license":[{"start":{"date-parts":[[2008,10,16]],"date-time":"2008-10-16T00:00:00Z","timestamp":1224115200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J of Med Genetics Pt A"],"published-print":{"date-parts":[[2008,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Three siblings from Morocco consanguineous family presented with cutaneous poikiloderma following postnatal ichthyosiform lesions, associated with papillomatous lesions, palmoplantar keratoderma, pachyonychia of toenails, fragile carious teeth, and lachrymal duct obstruction. Photosensitivity and blistering improved with age. Atrophic scars were prominent on the limbs. Neutropenia developed in the first year secondary to dysmyelopoiesis affecting the granulocyte lineage, associated with a polyclonal hypergammaglobulinemia. Several broncho\u2010pulmonary infectious episodes complicated the evolution, and cystic fibrosis was first considered on the basis of repeated abnormal sweat chloride tests but not confirmed by molecular analyses. This autosomal recessive disorder matches that described originally as poikiloderma with neutropenia\u2010Clericuzio type in Navajo Indians (OMIM 604173). It is discussed within the group of the major hereditary poikiloderma disorders, that is, Rothmund\u2013Thomson syndrome, dyskeratosis congenita, and Kindler syndrome. \u00a9 2008 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.a.32524","type":"journal-article","created":{"date-parts":[[2008,10,17]],"date-time":"2008-10-17T01:01:25Z","timestamp":1224205285000},"page":"2762-2769","source":"Crossref","is-referenced-by-count":40,"title":["Poikiloderma with neutropenia, Clericuzio type, in a family from Morocco"],"prefix":"10.1002","volume":"146A","author":[{"given":"Rahima","family":"Mostefai","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fanny","family":"Morice\u2010Picard","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Franck","family":"Boralevi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michel","family":"Sautarel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Didier","family":"Lacombe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marie Jos\u00e9","family":"Stasia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John","family":"McGrath","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alain","family":"Ta\u00efeb","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2008,10,16]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-1759(89)90395-5"},{"key":"e_1_2_5_3_1","first-page":"77","article-title":"Isolation of mononuclear cells and granulocytes from human blood","volume":"21","author":"B\u00f6yum A","year":"1968","journal-title":"Scan J Clin Lab Invest"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(96)06337-4"},{"key":"e_1_2_5_5_1","first-page":"A661","article-title":"Immune deficient poikiloderma: A new genodermatosis","volume":"49","author":"Clericuzio C","year":"1991","journal-title":"Am J Hum Genet"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2005.1.89"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/00125817-199905000-00007"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.65.9.942"},{"key":"e_1_2_5_9_1","first-page":"583","article-title":"Epidermodysplasia verruciformis as a model in studies on the role of papovaviruses in oncogenesis","volume":"32","author":"Jablonska S","year":"1972","journal-title":"Cancer Res"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/8788"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1525-1470.2001.018003210.x"},{"key":"e_1_2_5_12_1","doi-asserted-by":"crossref","first-page":"1299","DOI":"10.5858\/2005-129-1299-MSCFPA","article-title":"Myelodysplastic syndromes: Clinicopathologic features, pathobiology, and molecular pathogenesis","volume":"129","author":"Nishino HT","year":"2005","journal-title":"Arch Pathol Lab Med"},{"key":"e_1_2_5_13_1","first-page":"1074","article-title":"Characteristics of the lesions and risk of malignant conversion associated with the type of human papillomavirus involved in epidermodysplasia verruciformis","volume":"39","author":"Orth G","year":"1979","journal-title":"Cancer Res"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.2174\/156652307780859044"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1525-1470.2001.01971.x"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/1096-8628(20001127)95:3<204::AID-AJMG4>3.0.CO;2-Q"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.1086\/301874"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-199602000-00020"},{"key":"e_1_2_5_19_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.12.4.339"},{"key":"e_1_2_5_20_1","doi-asserted-by":"publisher","DOI":"10.1159\/000017888"},{"key":"e_1_2_5_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1985.tb00192.x"},{"key":"e_1_2_5_22_1","doi-asserted-by":"publisher","DOI":"10.1086\/379035"},{"key":"e_1_2_5_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.30430"},{"key":"e_1_2_5_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/0190-9622(92)70249-F"},{"key":"e_1_2_5_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/1096-8628(20010722)102:1<11::AID-AJMG1413>3.0.CO;2-A"},{"key":"e_1_2_5_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.10057"},{"key":"e_1_2_5_27_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnci\/95.9.669"}],"container-title":["American Journal of Medical Genetics Part A"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajmg.a.32524","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.a.32524","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,18]],"date-time":"2023-10-18T09:20:10Z","timestamp":1697620810000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.a.32524"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,10,16]]},"references-count":26,"journal-issue":{"issue":"21","published-print":{"date-parts":[[2008,11]]}},"alternative-id":["10.1002\/ajmg.a.32524"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.a.32524","archive":["Portico"],"relation":{},"ISSN":["1552-4825","1552-4833"],"issn-type":[{"value":"1552-4825","type":"print"},{"value":"1552-4833","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,10,16]]}}}