An Unusual Presentation of Chest Pain in a Patient With Cystic Fibrosis: A Case Report.

PURPOSE: This case report describes the identification and treatment of costochondritis with suspected neural entrapment in a 14-year-old individual diagnosed with cystic fibrosis. SUMMARY OF KEY POINTS: The individual discussed in this report had resolution of his chest pain with additional improvement in pulmonary function test results. STATEMENT OF CONCLUSION AND RECOMMENDATIONS FOR CLINICAL PRACTICE: This case supports the need for musculoskeletal and neuromuscular screening and intervention for patients with cystic fibrosis. The success of the intervention suggests that when traditional approaches to treatment of costochondritis fail, use of myofascial release at the accessory muscles of breathing could be beneficial.

Recognition of the peripheral airway impairment phenotype in children with well-controlled asthma.

BACKGROUND: Peripheral airway impairment, although frequently unrecognized, is a risk factor for poor asthma control, loss of control, increased exacerbations, airway hyperresponsiveness, and loss of lung function with age, even in patients with well-controlled asthma. OBJECTIVE: To determine the presence of peripheral airway impairment by impulse oscillometry and forced expiratory flow between 25% and 75% (FEF25%-75%) in children whose asthma is well controlled by National Asthma Education and Prevention Program (NAEPP) guidelines. METHODS: In this retrospective, cross-sectional analysis, outcomes were evaluated across 192 encounters in 139 patients with moderate to severe asthma, ages 4-18 years. Receiver operator characteristic curves were created and oscillometry thresholds determined by maximizing the sum of sensitivity and specificity to identify those whose condition is not well controlled. Impairment was then identified for those whose condition was well controlled when these age-dependent oscillometry thresholds were met for each IOS measure or FEF25%-75% < 65% of predicted. RESULTS: Reactance at 5 Hz (X5) appeared most robust to identify peripheral airway impairment. In 96 well-controlled asthma encounters, impairment was identified by X5 in approximately 20% and 45% for those younger than 12 years and adolescents, respectively, compared with a maximum of 10% with FEF25%-75% in the adolescent cohort (P < .05). CONCLUSION: We conclude that peripheral airway impairment, determined by oscillometry, is common in patients with well-controlled asthma across age cohorts. X5 with optimal cut points ≤ -3.8, ≤ -2.5, and ≤ -1.5 cmH2O/L/s for ages 4-7, 8-11, and >12 years, provides the clinician with a practical tool to identify the presence of the peripheral airway impairment phenotype that is consistently superior to FEF25%-75%. This recognition, if confirmed, may reduce the risk of asthma-associated consequences with earlier and more targeted therapy.

External validation can provide the clinician with IOS equations that best predict the risk of uncontrolled asthma in their population.

BACKGROUND: Peripheral airway impairment (PAI) has been shown to have a close association to risk of uncontrolled asthma in children. However, clear methods have not been established for the clinician to select impulse oscillometry (IOS) reference equations best suited for their population. Our study aimed to develop a practical external validation analytic approach for the clinician to determine which of the available reference equations best predicts uncontrolled asthma for their patients. METHODS: This is a post hoc analyses of data collected at baseline in a randomized controlled study that occurred from March 2016 to 2018. The study population consisted of 227 children, ages 4-18 years, with moderate to severe asthma. Discrimination and calibration predictive performance of available and suitable IOS equations were assessed by using uncontrolled asthma as the criterion outcome. Discrimination statistics of accuracy, sensitivity, and specificity served as the primary performance indicators. Rank scores were determined by the number of acceptable limit thresholds met for these measures (≥60%, ≥50%, and ≥60%, respectively) across IOS metrics (R5, R5-R20, AX, and X5) resulting in a total possible score of 12. RESULTS: External validity assessment determined the rank order of best to worst equations as being Gochicoa-Rangel (rank score = 10) > Nowowiejska (rank score = 9) > Assumapcao (rank score = 6) > Amra (rank score = 2). Gochicoa-Rangel reference equations provided the best option for universal application with accuracy of 73.1%, 72.2%, 76.7%, and 66.2% for R5, R5-R20, AX, and X5, respectively. CONCLUSIONS: External validation, particularly discrimination in asthmatic children, offers the clinician a practical approach to selecting the most suitable predictive equations for their patients.

Standardized IOS Reference Values Define Peripheral Airway Impairment-Associated Uncontrolled Asthma Risk Across Ethnicity in Children.

BACKGROUND: Although clinically useful in identifying peripheral airway impairment (PAI), impulse oscillometry (IOS) has not received universal acceptance. This may be due to variable cutoff points, and the perception that available standardized reference values may not be clinically relevant in all populations. OBJECTIVE: To establish the relationship between PAI, based on standardized IOS reference values, and uncontrolled asthma as well as interchangeability between Hispanic and white reference algorithms. METHODS: IOS reference values were established for upper and lower limits of normal (>95th and <5th percentile, respectively) using published algorithms in Hispanic and white children. Values exceeding normal limits (PAI) were compared in those uncontrolled and controlled for significance of differences. Probability estimates and odds ratio of uncontrolled asthma were determined for R5, R5-R20, AX, and X5 using adjusted generalized estimating equation analyses. Intraclass correlation coefficients determined interchangeability of Hispanic and white reference algorithm values. RESULTS: Those with uncontrolled asthma had significantly greater PAI and a higher frequency of PAI than those well controlled (P < .05), whereas odds of uncontrolled asthma increased with increasing PAI (P < .001) for all IOS measures, particularly for X5, where odds ratios ranged from 2.70 to 11.01.There was good to excellent (>70%) agreement between Hispanic and white algorithms, except for R5-R20 (<70%). CONCLUSIONS: PAI, defined by IOS reference values, not central airway markers (R20), is consistently related to the risk of uncontrolled asthma. This is true whether using Hispanic or white reference algorithms. Thus, standardized reference values in children offer the clinician readily available IOS cutoff points that are clinically relevant across ethnicity.

Chronic Eosinophilic Pneumonia: A Pediatric Case with Idiopathic Bronchiectasis.

Chronic eosinophilic pneumonia (CEP) is a rare lung disease and is especially uncommon in children. It presents with subacute respiratory symptoms of hypoxemia and dyspnea, peripheral infiltrates on imaging, and eosinophilia. However, it can be difficult to diagnose, as there is no strict diagnosis criteria and the clinical findings may be very nonspecific. In this case report, we describe a 14-year-old female with an unusual presentation of asymptomatic bronchiectasis, and the role of surgical lung biopsy in the diagnosis of CEP when no peripheral or alveolar eosinophilia is seen. It suggests that perhaps bronchiectasis can also be an unusual presenting sign of CEP and that there may be more asymptomatic cases of CEP with the true incidence being underreported, especially in the pediatric population.