Otologic disease among patients with primary ciliary dyskinesia - an international observational study (preprint)

ABSTRACT Importance Otologic disease is common among people with primary ciliary dyskinesia, yet little is known about its spectrum and severity. Objective We characterized otologic disease among participants with primary ciliary dyskinesia using data from the Ear-Nose-Throat Prospective International Cohort of PCD patients (EPIC-PCD). Design Cross-sectional analysis of baseline cohort data. Setting Twelve specialized primary ciliary dyskinesia centers in 10 countries. Participants We prospectively included children and adults with primary ciliary dyskinesia diagnoses, routine ENT examinations, and completed symptom questionnaires at the same visit or within 2 weeks. Exposures Potential risk factors associated with increased risk of ear disease. Main outcomes and measures We describe the prevalence and characteristics of patient-reported otologic symptoms and findings from otologic examinations; we identify potential factors associated with increased risk of ear inflammation and hearing impairment. Results We included 397 (211 males) participants with median age 15 (range 0–73). A total of 204 (51%) reported ear pain, 110 (28%) ear discharge, and 183 (46%) hearing problems. Adults reported ear pain and hearing problems more frequently when compared with children. Otitis media with effusion—usually bilateral—from otoscopy was most common among 121 (32%) of 384 participants. Retracted tympanic membrane and tympanic sclerosis were more commonly seen among adults. Tympanometry was performed on 216 participants and showed pathologic type B results for 114 (53%). Audiometry was performed on 273 participants and showed hearing impairment in at least 1 ear, most commonly mild. Season of visit was the strongest risk factor for problems related to ear inflammation (autumn compared with spring odds ratio, 95% confidence interval: 2.4, 1.5–3.8) and age 30 and older of hearing impairment (age 41–50 compared with age 10 years and younger odds ratio, 95% confidence interval: 3.3, 1.1–9.9). Conclusion and relevance Many people with primary ciliary dyskinesia suffer from ear problems yet frequency varies, highlighting disease expression differences and possible clinical phenotypes. Understanding differences in otologic disease expression and progression during lifetime may inform clinical decisions about follow-up and medical care. We recommend multidisciplinary primary ciliary dyskinesia management includes regular otologic assessments for all ages even without specific complaints. Key Points Question What are the characteristics of otologic disease among patients with primary ciliary dyskinesia (PCD)? Findings Baseline data from a large multicenter cohort of patients with PCD showed frequent reports of ear pain and reduced hearing with age as the main factor associated with hearing impairment. Otitis media with effusion was the most common otoscopic finding; adults often presented with tympanic sclerosis following history of previous ear infections. Meaning Since otologic disease is an important yet underreported part of PCD’s clinical expression, we recommend otologic assessments for all age groups as part of regular clinical follow-up.

COVID-19 disease characteristics in different pediatric age groups.

INTRODUCTION: Little is known about the COVID-19 disease characteristics and differences between different pediatric age groups. This study aimed to investigate the disease characteristics according to age groups. METHODOLOGY: We conducted a retrospective, single-center study of pediatric COVID-19 in a tertiary care hospital in Turkey. The patients were divided into three groups: 15 days-24 months old (Group 1), 25-144 months old (Group 2), and 145-210 months old (Group 3) according to age. RESULTS: A total of 139 pediatric patients with COVID-19 were examined. Twenty-nine patients (20.9%) were in Group 1, 52 (37.4%) were in Group 2, 58 (41.7%) were in Group 3. Thirty-nine patients (28.1%) were hospitalized. The most common symptoms were cough (55.4%) and fever (51.8%). The median chest X-ray (CXR) score of hospitalized patients was 1 (min 0-max 7), and the median CXR score of outpatients was 1 (min 0-max 6). Fever was significantly more frequent in Group 1, and chest pain was more frequent in Group 3. Group 1 had significantly higher WBC, lymphocyte, thrombocyte counts, AST, LDH, D-dimer, and Troponin T levels but lower hemoglobin, total protein, and albumin levels. The treatment included antibiotics, oseltamivir, hydroxychloroquine, and supportive therapy. Only one patient (0.7%) received non-invasive mechanical ventilatory support. CONCLUSIONS: As we know the clinical course of COVID-19 in children is less severe than in adults. We also found significant differences in both clinical and laboratory findings between different pediatric age groups which supports the theory that disease pathogenesis is highly variable according to age.

CF GLOBAL CARE: Collaboration Between Two CF Centers; University of Michigan, USA and Marmara University, Istanbul, Turkey before and during the COVID-19 Pandemic (preprint)

Solving the world’s health challenges requires multidisciplinary collaborations that bring together the talents, experiences, resources, and ideas from multiple sectors in low and middle-income countries (LMIC) and high -income countries (HIC). Cystic Fibrosis (CF) was thought to be a disease of Caucasian populations from European decent. However, it has been shown to affect people from all ethnic backgrounds. CF care varies significantly for people with CF (pw CF) from HIC with median survival approaching 50 years of age, to LMIC with pw CF dying in infancy or early childhood. To address the discrepancy in quality of care and outcomes, we report on a collaboration between our team at the University of Michigan cystic fibrosis center (UoM CFC), through support from the Middle East CF Association (MECFA) and the CF Foundation (CFF), and a CF center in Turkey (Marmara University CF Center, Istanbul) to address deficiencies and improve quality of care in that center. The collaboration has been successful in improving Ma CFC data and patient care. This partnership can be viewed as a model of collaboration to be duplicated in other Middle East Countries and LMIC to deliver optimal CF care.