McCoy Jennifer L, Kaffenberger Thomas M, Yang Tiffany S, Shaffer Amber D, Dohar Joseph E
Division of Pediatric Otolaryngology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
Department of Otolaryngology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
Int J Pediatr Otorhinolaryngol. 2020 Jul;134:110032. doi: 10.1016/j.ijporl.2020.110032. Epub 2020 Apr 1.
Historically cystic fibrosis (CF), in contrast to primary ciliary dyskinesia (PCD), has been considered protective of the middle ear from otitis media and rarely were such patients "severely affected" to require tympanostomy tube placement (BMT). Previously the Pittsburgh Otitis Media Research group reported a 10% prevalence of otitis media in the pediatric CF population which is significantly lower than age matched non-CF children. We studied the prevalence of OM in pediatric CF patients to assess if the otologic phenotype has changed in parallel with changes in the diagnosis itself.
The medical records of 133 CF patients seen either inpatient or outpatient from one of the largest tertiary pediatric CF centers in the world from 2010 to 2019 were reviewed for demographics, acute otitis media (AOM) episodes, risk factors for AOM, placement of myringotomy tubes, genotype, BMI, pancreatic status, respiratory culture results, and pulmonary exacerbations.
Just over half (52.6%) the patients were male. A median age for CF diagnosis was 11 days old (range 0 days-16 years). The most common genotype (49.6%) was homozygous for ΔF508 mutation. Fifty-five (41.4%) patients had 1-2 episodes of AOM, and 15 (11.3%) were severely affected (i.e. ≥3 episodes/6 months or ≥4 episodes/year). COME was diagnosed in 4 (3.0%) of the patients. Twelve (9.0%) patients had tympanostomy tubes at least once, including 3 patients with multiple sets of tubes. Having at least one AOM episode was not predicted by genetic mutation groups, BMI, age at diagnosis, or comorbidities, p > .05.
The time-honored adage of CF protecting against otitis media is no longer true and pediatric otolaryngologists must now prioritize the management of middle ear disease as highly as sino-nasal and pulmonary disease. Future study into mechanisms explaining the change is needed.
从历史上看，与原发性纤毛运动障碍（PCD）相比，囊性纤维化（CF）被认为可保护中耳免受中耳炎影响，这类患者很少“受到严重影响”而需要放置鼓膜造孔管（BMT）。此前，匹兹堡中耳炎研究小组报告称，儿科CF人群中中耳炎的患病率为10%，显著低于年龄匹配的非CF儿童。我们研究了儿科CF患者中耳炎的患病率，以评估耳科表型是否与诊断本身的变化同步改变。
回顾了2010年至2019年期间，来自世界上最大的三级儿科CF中心之一的133例住院或门诊CF患者的病历，内容包括人口统计学资料、急性中耳炎（AOM）发作情况、AOM的危险因素、鼓膜切开置管情况、基因型、体重指数（BMI）、胰腺状态、呼吸道培养结果和肺部加重情况。
略超过一半（52.6%）的患者为男性。CF诊断的中位年龄为11天（范围0天至16岁）。最常见的基因型（49.6%）是ΔF508突变的纯合子。55例（41.4%）患者有1 - 2次AOM发作，15例（11.3%）受到严重影响（即≥3次发作/6个月或≥4次发作/年）。4例（3.0%）患者被诊断为慢性中耳炎（COME）。12例（9.0%）患者至少有一次鼓膜造孔管置入，其中3例患者有多套置管。基因突变组、BMI、诊断时年龄或合并症均不能预测是否至少有一次AOM发作，p>0.05。
CF可预防中耳炎这一由来已久的说法已不再成立，儿科耳鼻喉科医生现在必须将中耳疾病的管理与鼻窦和肺部疾病的管理置于同等重要的位置。需要对解释这种变化的机制进行进一步研究。
