FUENTES BUSTOS, MARIA IGNACIA
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FUENTES BUSTOS, MARIA IGNACIA
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mifuentes@udd.cl
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57193308856

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Immune cell profiling of wounds from Epidermolysis bullosa patients

2019 , FUENTES BUSTOS, MARIA IGNACIA , C. Guttmann-Gruber , J. Piñon Hofbauer , A. South

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Longitudinal study of wound healing status and bacterial colonisation of Staphylococcus aureus and Corynebacterium diphtheriae in epidermolysis bullosa patients

2022 , FUENTES BUSTOS, MARIA IGNACIA , YUBERO GONCALVEZ, MARIA JOAO , Pilar Morandé , Carmen Varela , Karen Oróstica , Francisco Acevedo , REBOLLEDO JARAMILLO, BORIS EDUARDO , Esteban Arancibia , PORTE TORRE, LORENA ISABEL , PALISSON ETCHARREN, FRANCIS

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Characterization of the epidermal-dermal junction in hiPSC-derived skin organoids

2022 , Veronika Ramovs , Hans Janssen , FUENTES BUSTOS, MARIA IGNACIA , Amandine Pitaval , Walid Rachidi , Susana M. Chuva de Sousa Lopes , Christian Freund , Xavier Gidrol , Christine L. Mummery , Karine Raymond

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Collagen VII maintains proteostasis in dermal fibroblasts by scaffolding TANGO1 cargo

2022 , Qingqing Cao , Grace Tartaglia , Michael Alexander , Pyung Hung Park , Shiv Poojan , Mehdi Farshchian , FUENTES BUSTOS, MARIA IGNACIA , Mei Chen , John A. McGrath , PALISSON ETCHARREN, FRANCIS , Julio Salas-Alanis , Andrew P. South

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Orofacial Anomalies in Kindler Epidermolysis Bullosa

2024 , Susanne Krämer , Anna Lena Hillebrecht , Yao Wang , Mihail-Alexandru Badea , Jose Ignacio Barrios , Sorina Danescu , FUENTES BUSTOS, MARIA IGNACIA , Demet Kartal , Alfred Klausegger , Enrique Ponce de León , Reinhard Schilke , Ivelina Yordanova , Agnes Bloch-Zupan , Cristina Has

ImportanceKindler epidermolysis bullosa is a genetic skin-blistering disease associated with recessive inherited pathogenic variants in FERMT1, which encodes kindlin-1. Severe orofacial manifestations of Kindler epidermolysis bullosa, including early oral squamous cell carcinoma, have been reported.ObjectiveTo determine whether hypoplastic pitted amelogenesis imperfecta is a feature of Kindler epidermolysis bullosa.Design, Settings, and ParticipantsThis longitudinal, 2-center cohort study was performed from 2003 to 2023 at the Epidermolysis Bullosa Centre, University of Freiburg, Germany, and the Special Care Dentistry Clinic, University of Chile in association with DEBRA Chile. Participants included a convenience sampling of all patients with a diagnosis of Kindler epidermolysis bullosa.Main Outcomes and MeasuresThe primary outcomes were the presence of hypoplastic pitted amelogenesis imperfecta, intraoral wounds, gingivitis and periodontal disease, gingival hyperplasia, vestibular obliteration, cheilitis, angular cheilitis, chronic lip wounds, microstomia, and oral squamous cell carcinoma.ResultsThe cohort consisted of 36 patients (15 female [42%] and 21 male [58%]; mean age at first examination, 23 years [range, 2 weeks to 70 years]) with Kindler epidermolysis bullosa. The follow-up ranged from 1 to 24 years. The enamel structure was assessed in 11 patients, all of whom presented with enamel structure abnormalities. The severity of hypoplastic pitted amelogenesis imperfecta varied from generalized to localized pitting. Additional orofacial features observed include gingivitis and periodontal disease, which was present in 90% (27 of 30 patients) of those assessed, followed by intraoral lesions (16 of 22 patients [73%]), angular cheilitis (24 of 33 patients [73%]), cheilitis (22 of 34 patients [65%]), gingival overgrowth (17 of 26 patients [65%]), microstomia (14 of 25 patients [56%]), and vestibular obliteration (8 of 16 patients [50%]). Other features included chronic lip ulcers (2 patients) and oral squamous cell carcinoma with lethal outcome (2 patients).Conclusions and RelevanceThese findings suggest that hypoplastic pitted amelogenesis imperfecta is a feature of Kindler epidermolysis bullosa and underscore the extent and severity of oral manifestations in Kindler epidermolysis bullosa and the need for early and sustained dental care.

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Sephardic Ancestry in Recessive Dystrophic Epidermolysis Bullosa Individuals Carrying the Prevalent c.6527insC Mutation

2022 , FUENTES BUSTOS, MARIA IGNACIA , Warshauer, E. M. , Roop, D. , Maier, P. , Runfeldt, G. , Gene Gene, Houston, TX USA

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Cells from discarded dressings differentiate chronic from acute wounds in patients with Epidermolysis Bullosa

2020 , FUENTES BUSTOS, MARIA IGNACIA , Christina Guttmann-Gruber , Birgit Tockner , Anja Diem , Alfred Klausegger , Glenda Cofré-Araneda , Olga Figuera , Yessia Hidalgo , Pilar Morandé , PALISSON ETCHARREN, FRANCIS , Boris Rebolledo-Jaramillo , YUBERO GONCALVEZ, MARIA JOAO , Raymond J. Cho , Heather I. Rishel , M. Peter Marinkovich , Joyce M. C. Teng , Timothy G. Webster , Marco Prisco , Luis H. Eraso , Josefina Piñon Hofbauer , Andrew P. South

AbstractImpaired wound healing complicates a wide range of diseases and represents a major cost to healthcare systems. Here we describe the use of discarded wound dressings as a novel, cost effective, accessible, and non-invasive method of isolating viable human cells present at the site of skin wounds. By analyzing 133 discarded wound dressings from 51 patients with the inherited skin-blistering disease epidermolysis bullosa (EB), we show that large numbers of cells, often in excess of 100 million per day, continually infiltrate wound dressings. We show, that the method is able to differentiate chronic from acute wounds, identifying significant increases in granulocytes in chronic wounds, and we show that patients with the junctional form of EB have significantly more cells infiltrating their wounds compared with patients with recessive dystrophic EB. Finally, we identify subsets of granulocytes and T lymphocytes present in all wounds paving the way for single cell profiling of innate and adaptive immune cells with relevance to wound pathologies. In summary, our study delineates findings in EB that have potential relevance for all chronic wounds, and presents a method of cellular isolation that has wide reaching clinical application.

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IMMUNE CELL PROFILING OF WOUNDS FROM EPIDERMOLYSIS BULLOSA PATIENTS

2020 , YUBERO GONCALVEZ, MARIA JOAO , FUENTES BUSTOS, MARIA IGNACIA , PALISSON ETCHARREN, FRANCIS , REBOLLEDO JARAMILLO, BORIS EDUARDO , Guttmann-Gruber, Christina , Tockner, Birgit , Anja Diem , Klausegger, Alfred , Cofre-Araneda, Glenda , Figuera, Olga , Hidalgo, Yessia , Morande, Pilar , Cho, Raymond J. , Rishel, Heather I , Marinkovich, M. Peter , Teng, Joyce , Webster, Timothy G. , Prisco, Marco , Eraso, Luis H. , Hofbauer, Josefina Pinon , South, Andrew P.

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Deciphering wound chronicity with a novel non-invasive sampling technology in Epidermolysis bullosa patients

2021 , FUENTES BUSTOS, MARIA IGNACIA , South, A.

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APOBEC mutation drives early-onset squamous cell carcinomas in recessive dystrophic epidermolysis bullosa

2018 , Raymond J. Cho , Ludmil B. Alexandrov , Nicoline Y. den Breems , Velina S. Atanasova , Mehdi Farshchian , Elizabeth Purdom , Tran N. Nguyen , Cristian Coarfa , Kimal Rajapakshe , Marco Prisco , Joya Sahu , Patrick Tassone , Evan J. Greenawalt , Eric A. Collisson , Wei Wu , Hui Yao , Xiaoping Su , Christina Guttmann-Gruber , Josefina Piñón Hofbauer , Raabia Hashmi , Ignacia Fuentes , Stephen C. Benz , Justin Golovato , Erik A. Ehli , Christel M. Davis , Gareth E. Davies , Kyle R. Covington , Dedee F. Murrell , Julio C. Salas-Alanis , Francis Palisson , Anna L. Bruckner , William Robinson , Cristina Has , Leena Bruckner-Tuderman , Matthias Titeux , Marcel F. Jonkman , Elham Rashidghamat , Su M. Lwin , Jemima E. Mellerio , John A. McGrath , Johann W. Bauer , Alain Hovnanian , Kenneth Y. Tsai , Andrew P. South

Early-onset squamous cell carcinoma in recessive dystrophic epidermolysis bullosa patients is characterized by APOBEC mutagenesis.

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