School of Dentistry, Hainan Medical University, Haikou, PR China.
Children's Hospital, Nanjing Medical University, Nanjing, PR China.
Medicine (Baltimore). 2024 Jul 26;103(30):e39086. doi: 10.1097/MD.0000000000039086.
Severe congenital neutropenia (SCN) is a rare and heterogeneous genetic disease. By describing the diagnosis and treatment of a child with SCN and periodontitis, this case provides a reference for the oral health management of a child with SCN and periodontitis.
We describe a boy with clinical manifestations of oral bleeding, neutropenia, recurrent fever, and other recurrent infections. The absolute neutrophil count (ANC) was <0.50 × 109/L most of the time. Morphological examination of bone marrow cells showed active granulocyte hyperplasia and dysmaturation.
According to the clinical manifestations, hematological examination and gene detection results, the child was diagnosed as SCN with chronic periodontitis.
Periodontal treatment was performed after informed consent was obtained from the child guardian. These included supragingival and subgingival cleaning, hydrogen peroxide and saline irrigation, placement of iodoglycerin in the gingival sulcus, and oral hygiene instruction. Hematopoietic stem cell transplantation (HSCT) was performed later.
One month after initial periodontal treatment, oral hygiene was well maintained and gingival swelling had subsided. Probing depth (PD) index on periodontal probing and bleeding was significantly reduced. However, there was no significant change in blood routine and other indicators before and after periodontal treatment.
Once SCN is diagnosed, individualized treatment plans can be developed according to the characteristics of the disease and its impact on oral health, which can effectively control the interaction between SCN and periodontal disease and reduce the occurrence of serious infection.
严重先天性中性粒细胞减少症(SCN)是一种罕见且异质性的遗传疾病。通过描述 1 例 SCN 合并牙周炎患儿的诊治经过,为 SCN 合并牙周炎患儿的口腔健康管理提供参考。
我们描述了 1 例以口腔出血、中性粒细胞减少、反复发热和其他反复感染为临床表现的男孩。患儿中性粒细胞绝对计数(ANC)大部分时间<0.50×109/L。骨髓细胞形态学检查显示粒细胞增生活跃伴成熟障碍。
根据临床表现、血常规检查和基因检测结果,患儿被诊断为 SCN 合并慢性牙周炎。
在获得患儿监护人知情同意后,进行牙周治疗,包括龈上洁治、龈下刮治、双氧水和生理盐水冲洗、碘甘油龈沟内上药、口腔卫生指导。后行造血干细胞移植(HSCT)。
初次牙周治疗 1 个月后,患儿口腔卫生维护良好,牙龈肿胀消退,牙周探诊深度(PD)指数及出血情况显著改善。但牙周治疗前后血常规等其他指标无明显变化。
一旦确诊 SCN,可根据疾病特点及其对口腔健康的影响制定个体化治疗方案,有效控制 SCN 与牙周病的相互作用,减少严重感染的发生。