Specific temperament in patients with nevoid basal cell carcinoma syndrome.
Background: Nevoid basal cell carcinoma syndrome (NBCCS) is a neurocutaneous disease, characterized by tumorigenesis and developmental anomalies due to aberrant sonic hedgehog (Shh) signaling. Patients with NBCCS typically appear calm and carefree, suggesting that a specific personality in these pat...
| Publicado en: | Pediatrics International Vol. 63; no. 2; pp. 177 - 183 |
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| Autores principales: | , , , , , , |
| Formato: | diagnostic images research tables/charts Journal Article |
| Publicado: |
Wiley-Blackwell
Feb2021
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=148947733&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 148947733 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 13288067 FS4 jtl: Pediatrics International issn: 13288067 maglogo: Y pubinfo: dt: Feb2021 vid: 63 iid: 2 pid: 480 pub: Wiley-Blackwell place: Malden, Massachusetts artinfo: ui: 148947733 148466762 148947733 148947733 10.1111/ped.14419 148947733 ppf: 177 ppct: 6 formats: tig: atl: Specific temperament in patients with nevoid basal cell carcinoma syndrome. aug: au: Uchikawa, Hideki Fujii, Katsunori Shiohama, Tadashi Nakazato, Michiko Shimizu, Eiji Miyashita, Toshiyuki Shimojo, Naoki affil: Department of Pediatrics, Chiba University Graduate School of Medicine, Chiba Japan sug: subj: Basal Cell Nevus Syndrome Cancer Patients Temperament Human Male Female Adult Psychological Tests Behavioral Symptoms Amygdala Anatomy and Histology Magnetic Resonance Imaging Image Processing, Computer Assisted Software Risk Taking Behavior Avoidance (Psychology) Abnormalities Hedgehog Signaling Proteins Signal Transduction Serotonin Adult: 19-44 years Male Female ab: Background: Nevoid basal cell carcinoma syndrome (NBCCS) is a neurocutaneous disease, characterized by tumorigenesis and developmental anomalies due to aberrant sonic hedgehog (Shh) signaling. Patients with NBCCS typically appear calm and carefree, suggesting that a specific personality in these patients may be associated with an enhanced hedgehog pathway. Our study aimed to determine the personality type in these patients. Methods: We enrolled 14 mentally normal patients with genetically confirmed NBCCS (seven males and seven females; mean age = 25.2 years) and 20 controls (10 males and 10 females; mean age = 27.9 years). The patients were assessed with the Japanese version of the Temperament and Character Inventory, based on the seven‐dimensional model of temperament and character, and their clinical symptoms were evaluated. The amygdala volumes of six patients with NBCCS were measured using magnetic resonance imaging with image‐processing software. Results: Patients with NBCCS scored significantly lower on harm avoidance (0.89) than controls (1.00; P = 0.0084). Moreover, patients with NBCCS and developmental malformations such as rib anomalies, who may have experienced Shh signaling enhancement from the prenatal period, scored significantly lower on harm avoidance (0.80 [P = 0.0031]). The left amygdala volume was also significantly reduced in patients with NBCCS (P = 0.0426). Conclusions: Patients with NBCCS who experienced increased Shh signaling from the prenatal period showed significantly lower harm avoidance related to serotonin. The left amygdala volume was significantly reduced in these patients. Our results indicate that Shh signaling may influence the human personality. pubtype: Academic Journal doctype: diagnostic images research tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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