Shete Mona, Thompson Jerome W, Naidu Sri I, Stocks Rose Mary S, Wang Winfred C
University Tennessee Health Science Center, Department of Otolaryngology, Head & Neck Surgery, 910, Madison Ave, Suite # 428, Memphis, TN 38163, United States.
Int J Pediatr Otorhinolaryngol. 2012 Mar;76(3):392-5. doi: 10.1016/j.ijporl.2011.12.018. Epub 2012 Jan 16.
Chronic neutropenia of childhood (CNC) is a rare disorder in which the absolute neutrophil count is below 1500/μL over an extended period of time. The objective of this study is to describe the otolaryngolgic manifestations associated with CNC to facilitate diagnosis and treatment of this condition.
We performed a retrospective chart review of patients with the diagnosis of CNC between 1970 and 2005 at a tertiary pediatric hematology center. After Institutional Review Board approval, 43 patients were evaluated. The average age at hematologic diagnosis was 49 months (range: 1 month - 15 years with 35% <1 year, 44% 1-10 years, 21% >10 years). A total of 2049 encounters were analyzed from the hospital charts.
Twenty four subjects (56%) presented with recurrent otitis media (ROM), sinusitis or pharyngo-tonsillitis, while 11% presented with oral mucosal lesions. After diagnosis, otolaryngologic problems persisted, including ROM (81%), viral upper respiratory tract infection (67%), oral ulcers or gingivitis (53%), tonsillitis (39%) and sinusitis (37%) and were more common than other systemic infections. Myringotomy tube placement, endoscopic sinus debridement, adeno-tonsillectomy or tracheostomy were required in 42% of patients. After G-CSF (granulocyte colony-stimulating factor) became available in the early 1990s, the infection rate markedly decreased. Five deaths occurred (12% mortality) including one due to sepsis from otolaryngologic infection.
The majority of children with CNC had otolaryngologic problems at presentation and these continued after diagnosis. While managing common otolaryngologic infections in children, a high index of suspicion for chronic neutropenia is necessary. An otolaryngologist is frequently one of the first physicians to encounter children with this condition. Awareness of CNC and its management will enhance earlier diagnosis and more effective treatment for these children.
儿童慢性中性粒细胞减少症（CNC）是一种罕见疾病，其绝对中性粒细胞计数在较长时间内低于1500/μL。本研究的目的是描述与CNC相关的耳鼻喉科表现，以促进对该疾病的诊断和治疗。
我们对1970年至2005年在一家三级儿科血液学中心诊断为CNC的患者进行了回顾性病历审查。经机构审查委员会批准，对43例患者进行了评估。血液学诊断时的平均年龄为49个月（范围：1个月至15岁，其中35%<1岁，44%为1至10岁，21%>10岁）。共分析了2049份医院病历中的就诊情况。
24名受试者（56%）出现复发性中耳炎（ROM）、鼻窦炎或咽扁桃体炎，而11%出现口腔黏膜病变。诊断后，耳鼻喉科问题持续存在，包括ROM（81%）、病毒性上呼吸道感染（67%）、口腔溃疡或牙龈炎（53%）、扁桃体炎（39%）和鼻窦炎（37%），且比其他全身感染更为常见。42%的患者需要进行鼓膜切开置管、鼻内镜鼻窦清创、腺样体扁桃体切除术或气管切开术。20世纪90年代初粒细胞集落刺激因子（G-CSF）问世后，感染率显著下降。发生了5例死亡（死亡率12%），其中1例死于耳鼻喉科感染引起的败血症。
大多数CNC患儿在就诊时存在耳鼻喉科问题，诊断后这些问题仍持续存在。在处理儿童常见的耳鼻喉科感染时，必须高度怀疑慢性中性粒细胞减少症。耳鼻喉科医生常常是首批接触到患有这种疾病儿童的医生之一。了解CNC及其治疗方法将有助于对这些儿童进行更早的诊断和更有效的治疗。
