Pachyonychia congenita associated with renal artery stenosis and bronchiectasis
Pachyonychia congenita (PC) is a rare hereditary disease, mainly characterized by a painful palmoplantar keratoderma, thickened nails, cysts and white lesions of the oral mucosa. Its clinical manifestations are very variable, it may appear from birth to adulthood. This study report the case of a chi...
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| Veröffentlicht in: | The Pan African medical journal 2016, Vol.24, p.183-183 |
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| container_title | The Pan African medical journal |
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| creator | Fatiha, El Alaoui Ismaili Abdeljalil, Chemlal Ilham, Karimi Nawal, Benabdellah Yassamine, Bentata Nada, Zizi Nafissa, Benajiba Intissar, Haddiya |
| description | Pachyonychia congenita (PC) is a rare hereditary disease, mainly characterized by a painful palmoplantar keratoderma, thickened nails, cysts and white lesions of the oral mucosa. Its clinical manifestations are very variable, it may appear from birth to adulthood. This study report the case of a child with pachyonychia congenita associated with bronchiectasis and renal artery stenosis. The diagnosis of pachyonychia congenita was retained based on clinical and histological data. However the presence of renal artery stenosis and bronchiectasis suggests a possible association as part of a particular syndromic group. |
| doi_str_mv | 10.11604/pamj.2016.24.183.9284 |
| format | Article |
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Its clinical manifestations are very variable, it may appear from birth to adulthood. This study report the case of a child with pachyonychia congenita associated with bronchiectasis and renal artery stenosis. The diagnosis of pachyonychia congenita was retained based on clinical and histological data. However the presence of renal artery stenosis and bronchiectasis suggests a possible association as part of a particular syndromic group.</description><identifier>EISSN: 1937-8688</identifier><identifier>DOI: 10.11604/pamj.2016.24.183.9284</identifier><identifier>PMID: 27795780</identifier><language>fre</language><publisher>Uganda</publisher><subject>Bronchiectasis - etiology ; Bronchiectasis - pathology ; Child ; Female ; Humans ; Pachyonychia Congenita - diagnosis ; Pachyonychia Congenita - physiopathology ; Renal Artery Obstruction - etiology ; Renal Artery Obstruction - pathology ; Syndrome</subject><ispartof>The Pan African medical journal, 2016, Vol.24, p.183-183</ispartof><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,864,4022,27922,27923,27924</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/27795780$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Fatiha, El Alaoui Ismaili</creatorcontrib><creatorcontrib>Abdeljalil, Chemlal</creatorcontrib><creatorcontrib>Ilham, Karimi</creatorcontrib><creatorcontrib>Nawal, Benabdellah</creatorcontrib><creatorcontrib>Yassamine, Bentata</creatorcontrib><creatorcontrib>Nada, Zizi</creatorcontrib><creatorcontrib>Nafissa, Benajiba</creatorcontrib><creatorcontrib>Intissar, Haddiya</creatorcontrib><title>Pachyonychia congenita associated with renal artery stenosis and bronchiectasis</title><title>The Pan African medical journal</title><addtitle>Pan Afr Med J</addtitle><description>Pachyonychia congenita (PC) is a rare hereditary disease, mainly characterized by a painful palmoplantar keratoderma, thickened nails, cysts and white lesions of the oral mucosa. 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However the presence of renal artery stenosis and bronchiectasis suggests a possible association as part of a particular syndromic group.</description><subject>Bronchiectasis - etiology</subject><subject>Bronchiectasis - pathology</subject><subject>Child</subject><subject>Female</subject><subject>Humans</subject><subject>Pachyonychia Congenita - diagnosis</subject><subject>Pachyonychia Congenita - physiopathology</subject><subject>Renal Artery Obstruction - etiology</subject><subject>Renal Artery Obstruction - pathology</subject><subject>Syndrome</subject><issn>1937-8688</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2016</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo1kE1LxDAYhIMg7rr6F5YcvbQmadokR1n8goX1oOfyNnnrdmnTmmSR_nsL6mlgeGYYhpAtZznnFZP3EwynXDBe5ULmXBe5EVpekDU3hcp0pfWKXMd4YqyqdMGuyEooZUql2Zoc3sAe59HP9tgBtaP_RN8loBDjaDtI6Oh3l440oIeeQkgYZhoT-jF2kYJ3tAmjX8JoEyzWDblsoY94-6cb8vH0-L57yfaH59fdwz6buOQp0wKaypRtwRRDsBIlSCEaBcoxwUvbyGUqcGucEaVruDSicUppbA26VqpiQ-5-e6cwfp0xpnroosW-B4_jOdbLCyVjUopiQbd_6LkZ0NVT6AYIc_1_QvEDjCpfuA</recordid><startdate>2016</startdate><enddate>2016</enddate><creator>Fatiha, El Alaoui Ismaili</creator><creator>Abdeljalil, Chemlal</creator><creator>Ilham, Karimi</creator><creator>Nawal, Benabdellah</creator><creator>Yassamine, Bentata</creator><creator>Nada, Zizi</creator><creator>Nafissa, Benajiba</creator><creator>Intissar, Haddiya</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>2016</creationdate><title>Pachyonychia congenita associated with renal artery stenosis and bronchiectasis</title><author>Fatiha, El Alaoui Ismaili ; Abdeljalil, Chemlal ; Ilham, Karimi ; Nawal, Benabdellah ; Yassamine, Bentata ; Nada, Zizi ; Nafissa, Benajiba ; Intissar, Haddiya</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p141t-82ab695f3070eac4e4a422b7a7d0215cb4668a1c9d925db1492bd778ef9edf473</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>fre</language><creationdate>2016</creationdate><topic>Bronchiectasis - etiology</topic><topic>Bronchiectasis - pathology</topic><topic>Child</topic><topic>Female</topic><topic>Humans</topic><topic>Pachyonychia Congenita - diagnosis</topic><topic>Pachyonychia Congenita - physiopathology</topic><topic>Renal Artery Obstruction - etiology</topic><topic>Renal Artery Obstruction - pathology</topic><topic>Syndrome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Fatiha, El Alaoui Ismaili</creatorcontrib><creatorcontrib>Abdeljalil, Chemlal</creatorcontrib><creatorcontrib>Ilham, Karimi</creatorcontrib><creatorcontrib>Nawal, Benabdellah</creatorcontrib><creatorcontrib>Yassamine, Bentata</creatorcontrib><creatorcontrib>Nada, Zizi</creatorcontrib><creatorcontrib>Nafissa, Benajiba</creatorcontrib><creatorcontrib>Intissar, Haddiya</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><jtitle>The Pan African medical journal</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Fatiha, El Alaoui Ismaili</au><au>Abdeljalil, Chemlal</au><au>Ilham, Karimi</au><au>Nawal, Benabdellah</au><au>Yassamine, Bentata</au><au>Nada, Zizi</au><au>Nafissa, Benajiba</au><au>Intissar, Haddiya</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Pachyonychia congenita associated with renal artery stenosis and bronchiectasis</atitle><jtitle>The Pan African medical journal</jtitle><addtitle>Pan Afr Med J</addtitle><date>2016</date><risdate>2016</risdate><volume>24</volume><spage>183</spage><epage>183</epage><pages>183-183</pages><eissn>1937-8688</eissn><abstract>Pachyonychia congenita (PC) is a rare hereditary disease, mainly characterized by a painful palmoplantar keratoderma, thickened nails, cysts and white lesions of the oral mucosa. 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| source | MEDLINE; DOAJ Directory of Open Access Journals; PubMed Central Open Access; African Journals Online (Open Access); EZB-FREE-00999 freely available EZB journals; PubMed Central |
| subjects | Bronchiectasis - etiology Bronchiectasis - pathology Child Female Humans Pachyonychia Congenita - diagnosis Pachyonychia Congenita - physiopathology Renal Artery Obstruction - etiology Renal Artery Obstruction - pathology Syndrome |
| title | Pachyonychia congenita associated with renal artery stenosis and bronchiectasis |
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