Nowzari H, Jorgensen M G, Ta T T, Contreras A, Slots J
University of Southern California School of Dentistry, Los Angeles, USA.
J Periodontol. 2001 Nov;72(11):1601-6. doi: 10.1902/jop.2001.72.11.1601.
Fanconi's anemia is an autosomal recessive disease associated with chromosomal breakage as well as pancytopenia, skin pigmentation, renal hypoplasia, cardiac defects, microcephaly, congenital malformations of the skeleton, hypogonadism, and increased risk of leukemia. The present report describes the periodontal clinical and microbiological status of an 11-year old male having Fanconi's anemia.
Polymerase chain reaction analysis to detect human cytomegalovirus (HCMV), Epstein-Barr type 1 virus, and herpes simplex virus (HSV) was performed on paper-point samples pooled from either 3 periodontal sites with advanced attachment loss or 3 gingivitis sites with no clinical attachment loss. Anaerobic bacterial culture examination was performed on the pooled periodontitis sample.
The patient suffered from pancytopenia, allergy, asthma, hearing impairment, and mental retardation. Dentition consisted of 7 primary teeth, 11 erupted permanent teeth, and 14 unerupted permanent teeth. Most erupted teeth showed severe gingival inflammation with some gingival overgrowth and various degrees of periodontal attachment loss. Genomes of HCMV and HSV were detected in the pooled periodontitis sample and HCMV in the pooled gingivitis sample. The periodontitis sample but not the gingivitis sample revealed HCMV mRNA of major capsid protein, suggestive of active viral infection. The periodontitis sample also yielded Actinobacillus actinomycetemcomitans (1.1% of total isolates), FusobActerium species (7.9%), Campylobacter species (2.2%), Peptostreptococcus micros (3.4%), and Candida albicans (0.3%).
Oral features of Fanconi's anemia may include increased susceptibility to periodontitis. It is likely that underlying host defense impairment coupled with periodontal infection by HCMV and A. actinomycetemcomitans contribute to the severe type of periodontitis associated with Fanconi's anemia.
范可尼贫血是一种常染色体隐性疾病，与染色体断裂以及全血细胞减少、皮肤色素沉着、肾发育不全、心脏缺陷、小头畸形、骨骼先天性畸形、性腺功能减退和白血病风险增加有关。本报告描述了一名患有范可尼贫血的11岁男性的牙周临床和微生物学状况。
对从3个有严重附着丧失的牙周部位或3个无临床附着丧失的牙龈炎部位采集的纸尖样本进行聚合酶链反应分析，以检测人巨细胞病毒（HCMV）、1型爱泼斯坦-巴尔病毒和单纯疱疹病毒（HSV）。对合并的牙周炎样本进行厌氧菌培养检查。
该患者患有全血细胞减少、过敏、哮喘、听力障碍和智力迟钝。牙列包括7颗乳牙、11颗已萌出恒牙和14颗未萌出恒牙。大多数已萌出牙齿表现出严重的牙龈炎症，伴有一些牙龈增生和不同程度的牙周附着丧失。在合并的牙周炎样本中检测到HCMV和HSV的基因组，在合并的牙龈炎样本中检测到HCMV。牙周炎样本而非牙龈炎样本显示主要衣壳蛋白的HCMV mRNA，提示病毒活跃感染。牙周炎样本还分离出伴放线放线杆菌（占总分离株的1.1%）、梭杆菌属（7.9%）、弯曲杆菌属（2.2%）、微小消化链球菌（3.4%）和白色念珠菌（0.3%）。
范可尼贫血的口腔特征可能包括对牙周炎易感性增加。宿主防御功能受损以及HCMV和伴放线放线杆菌的牙周感染可能导致与范可尼贫血相关的严重牙周炎。
