Tamara Pawlaczyk-Kamieńska1, Hanna Winiarska2, Tomasz Kulczyk3, Szczepan Cofta2. 1. Department of Risk Group Dentistry, Poznan University of Medical Sciences, Bukowska 70, 60-812 Poznań, Poland. 2. Department of Pulmonology, Allergology and Respiratory Oncology, Poznan University of Medical Sciences, Szamarzewskiego 82/84, 60-569 Poznań, Poland. 3. Section of Dental Radiology, Department of Biomaterials and Experimental Dentistry, Poznan University of Medical Sciences, Bukowska 70, 60-812 Poznań, Poland.
Abstract
BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare, ciliopathic disorder. In many ciliopathies, dental anomalies are observed alongside other symptoms of the disease. To date, there are no published reports concerning the dental developmental problems that are associated with ciliary defects in PCD patients. METHODS: Patients suffering from PCD underwent dental clinical examination, which included the assessment of developmental disorders regarding the number and morphological structure of the teeth (size and shape) as well as developmental disorders of mineralised dental tissues. Then, three-dimensional radiographic examination was performed utilising Cone Beam Computed Tomography (CBCT). RESULTS: Four PCD patients, aged 31-54, agreed to enter the study. Dental examinations showed the presence of dental developmental disorders in three of them. Additionally, CBCT showed abnormalities in those patients. CONCLUSIONS: 1. The dental phenotype in PCD patients seems to be heterogeneous. Tooth developmental disorders resulting from abnormal odontogenesis may be a symptom of PCD that is concomitant with other developmental abnormalities resulting from malfunctioning primary cilia. 2. Patients with ciliopathies are likely to develop dental developmental defects. Therefore, beginning in early childhood, they should be included in a targeted specialised dental programme to enable early diagnosis and to ensure dedicated preventive and therapeutic measures.
BACKGROUND:Primary ciliary dyskinesia (PCD) is a rare, ciliopathic disorder. In many ciliopathies, dental anomalies are observed alongside other symptoms of the disease. To date, there are no published reports concerning the dental developmental problems that are associated with ciliary defects in PCDpatients. METHODS:Patients suffering from PCD underwent dental clinical examination, which included the assessment of developmental disorders regarding the number and morphological structure of the teeth (size and shape) as well as developmental disorders of mineralised dental tissues. Then, three-dimensional radiographic examination was performed utilising Cone Beam Computed Tomography (CBCT). RESULTS: Four PCDpatients, aged 31-54, agreed to enter the study. Dental examinations showed the presence of dental developmental disorders in three of them. Additionally, CBCT showed abnormalities in those patients. CONCLUSIONS: 1. The dental phenotype in PCDpatients seems to be heterogeneous. Tooth developmental disorders resulting from abnormal odontogenesis may be a symptom of PCD that is concomitant with other developmental abnormalities resulting from malfunctioning primary cilia. 2. Patients with ciliopathies are likely to develop dental developmental defects. Therefore, beginning in early childhood, they should be included in a targeted specialised dental programme to enable early diagnosis and to ensure dedicated preventive and therapeutic measures.
Authors: Alexandria L Irace; Anne Koivuholma; Eero Huotilainen; Jaana Hagström; Katri Aro; Mika Salmi; Antti Markkola; Heli Sistonen; Timo Atula; Antti A Mäkitie Journal: Int J Environ Res Public Health Date: 2021-01-21 Impact factor: 3.390
Authors: Christina Torres Kozycki; Shilpa Kodati; Laryssa Huryn; Hongying Wang; Blake M Warner; Priyam Jani; Dima Hammoud; Mones S Abu-Asab; Yingyos Jittayasothorn; Mary J Mattapallil; Wanxia Li Tsai; Ehsan Ullah; Ping Zhou; Xiaoying Tian; Ariane Soldatos; Niki Moutsopoulos; Marie Kao-Hsieh; Theo Heller; Edward W Cowen; Chyi-Chia Richard Lee; Camilo Toro; Shelley Kalsi; Zohreh Khavandgar; Alan Baer; Margaret Beach; Debra Long Priel; Michele Nehrebecky; Sofia Rosenzweig; Tina Romeo; Natalie Deuitch; Laurie Brenchley; Eileen Pelayo; Wadih Zein; Nida Sen; Alexander H Yang; Gary Farley; David A Sweetser; Lauren Briere; Janine Yang; Fabiano de Oliveira Poswar; Ida Vanessa D Schwartz; Tamires Silva Alves; Perrine Dusser; Isabelle Koné-Paut; Isabelle Touitou; Salah Mohamed Titah; Petrus Martin van Hagen; Rogier T A van Wijck; Peter J van der Spek; Hiromi Yano; Andreas Benneche; Ellen M Apalset; Ragnhild Wivestad Jansson; Rachel R Caspi; Douglas Byron Kuhns; Massimo Gadina; Hidetoshi Takada; Hiroaki Ida; Ryuta Nishikomori; Elena Verrecchia; Eugenio Sangiorgi; Raffaele Manna; Brian P Brooks; Lucia Sobrin; Robert B Hufnagel; David Beck; Feng Shao; Amanda K Ombrello; Ivona Aksentijevich; Daniel L Kastner Journal: Ann Rheum Dis Date: 2022-07-22 Impact factor: 27.973