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Arthritis & Rheumatology (Hoboken, N.J.) Aug 2020Relapsing polychondritis (RP) is a systemic disease. Failure to recognize RP can lead to diagnostic delay and further complications, including death. This study was... (Observational Study)
Observational Study
OBJECTIVE
Relapsing polychondritis (RP) is a systemic disease. Failure to recognize RP can lead to diagnostic delay and further complications, including death. This study was undertaken to identify clinical patterns in a prospective cohort of patients with RP.
METHODS
Patient subgroups were identified using latent class analysis based on 8 clinical variables: saddle-nose deformity, subglottic stenosis, tracheomalacia, bronchomalacia, ear chondritis, tenosynovitis/synovitis, inflammatory eye disease, and audiovestibular disease. Model selection was based on Akaike's information criterion.
RESULTS
Seventy-three patients were included in this study. Patients were classified into 1 of 3 subgroups: type 1 RP (14%), type 2 RP (29%), and type 3 RP (58%). Type 1 RP was characterized by ear chondritis (100%), tracheomalacia (100%), saddle-nose deformity (90%), and subglottic stenosis (80%). These patients had the shortest median time to diagnosis (1 year), highest disease activity, and greatest frequency of admission to the intensive care unit and tracheostomy. Type 2 RP was characterized by tracheomalacia (100%) and bronchomalacia (52%), but no saddle-nose deformity or subglottic stenosis. These patients had the longest median time to diagnosis (10 years) and highest percentage of work disability. Type 3 RP was characterized by tenosynovitis/synovitis (60%) and ear chondritis (55%). There were no significant differences in sex, race, or treatment strategies between the 3 subgroups.
CONCLUSION
Our findings indicate that there are 3 subgroups of patients with RP, with differences in time to diagnosis, clinical and radiologic characteristics, and disease-related complications. Recognizing a broader spectrum of clinical patterns in RP, beyond cartilaginous involvement of the ear and upper airway, may facilitate more timely diagnosis.
Topics: Adult; Delayed Diagnosis; Female; Humans; Latent Class Analysis; Male; Middle Aged; Polychondritis, Relapsing; Prospective Studies; Symptom Assessment; Syndrome
PubMed: 32249511
DOI: 10.1002/art.41270 -
Monaldi Archives For Chest Disease =... May 2017The post-pneumonectomy syndrome is a rare complication consisting of rotation and herniation of the mediastinal structures, the remaining lung and the respective... (Review)
Review
The post-pneumonectomy syndrome is a rare complication consisting of rotation and herniation of the mediastinal structures, the remaining lung and the respective bronchi, into the contralateral hemithorax. This situation may produce symptomatic airway obstruction and varies in its presentation and severity. We describe one case of right and one of left pneumonectomy syndrome as well as one case of post-lobectomy syndrome. We review the literature on the pathophysiology, the clinical, radiological and bronchoscopic characteristics of this rare entity and discuss all available therapeutic alternatives.
Topics: Adult; Aged; Airway Obstruction; Bronchomalacia; Bronchoscopy; Cough; Dyspnea; Fatal Outcome; Female; Humans; Lung Neoplasms; Male; Noninvasive Ventilation; Pneumonectomy; Postoperative Complications; Silicones; Stents; Treatment Outcome
PubMed: 28635203
DOI: 10.4081/monaldi.2017.810 -
Journal of Cystic Fibrosis : Official... Mar 2021We report the first case of biodegradable airway stent insertion for a patient with bronchomalacia and cystic fibrosis (CF). This female infant with antenatally...
We report the first case of biodegradable airway stent insertion for a patient with bronchomalacia and cystic fibrosis (CF). This female infant with antenatally diagnosed cystic fibrosis developed respiratory distress by three weeks of age. On lower airway examination she was found to have severe left main stem bronchomalacia causing left upper lobe hyperinflation and subsequent right upper lobe collapse. By eight weeks of age she developed life-threatening respiratory failure requiring high pressure invasive ventilation. A biodegradable bronchial stent was inserted to the left main bronchus at thirteen weeks of age with successful extubation from invasive respiratory support four days later. A second biodegradable stent was inserted nine weeks later, due to persistent bronchomalacia following stent degradation and redevelopment of oxygen requirement. She was discharged home off all respiratory support eight weeks later and has remained well, requiring no further surgical intervention for bronchomalacia to date, now age three years.
Topics: Biocompatible Materials; Bronchomalacia; Cystic Fibrosis; Female; Humans; Infant, Newborn; Stents
PubMed: 33168477
DOI: 10.1016/j.jcf.2020.10.004 -
Journal of Veterinary Internal Medicine May 2021The origin of cough in dogs with heart murmurs is controversial, because the cough could be primary cardiac (eg, pulmonary edema, bronchi compression by left-sided...
BACKGROUND
The origin of cough in dogs with heart murmurs is controversial, because the cough could be primary cardiac (eg, pulmonary edema, bronchi compression by left-sided cardiomegaly) or respiratory (eg, bronchomalacia, other bronchial or bronchiolar disease, interstitial lung disease) in origin.
HYPOTHESIS/OBJECTIVES
To study the association between left atrium (LA) dilatation and cardiomegaly and bronchial narrowing in coughing dogs with heart murmurs using computed tomography (CT).
ANIMALS
Twenty-one client-owned coughing dogs with heart murmurs and 14 historical control dogs.
METHODS
Dogs with cough and murmur were prospectively recruited over 4 months. Cervical and thoracic radiography, echocardiography, and thoracic CT were performed in enrolled dogs. Control dogs, with no disease on thoracic CT and no records of heart murmur and coughing, were gathered from the institution's computerized database. Degree of bronchial narrowing was assessed using the bronchial-to-aorta (Ao) ratio, measured by 3 radiologists blinded to the clinical findings. After identifying bronchi that were significantly narrowed in dogs with murmur compared to controls, the relationship between degree of narrowing and LA/Ao ratio (measured echocardiographically) and vertebral heart scale (VHS) measured radiographically was studied in dogs with murmur using mixed-effects regression.
RESULT
Significant narrowing was identified for all left-sided bronchi and the right principal, middle, and caudal bronchi in the coughing dogs, compared with controls. Increasing LA size and VHS were significantly inversely associated with diameter for all left-sided and right-sided bronchi indicated above.
CONCLUSION AND CLINICAL IMPORTANCE
Results indicate an association between LA enlargement and cardiomegaly and bronchial narrowing and support heart size-associated exacerbation of cough in dogs with murmurs.
Topics: Animals; Bronchi; Cough; Dog Diseases; Dogs; Heart Murmurs; Tomography, X-Ray Computed
PubMed: 33811698
DOI: 10.1111/jvim.16115 -
Animals : An Open Access Journal From... Nov 2022Airway collapse (AC) in dogs includes a tracheal collapse, mainstem and lobar bronchial collapse, and bronchomalacia (i.e., segmental/subsegmental bronchial collapse)....
Ventilator-Assisted Inspiratory and Expiratory Breath-Hold Thoracic Computed Tomographic Scans Can Detect Dynamic and Static Airway Collapse in Dogs with Limited Agreement with Tracheobronchoscopy.
Airway collapse (AC) in dogs includes a tracheal collapse, mainstem and lobar bronchial collapse, and bronchomalacia (i.e., segmental/subsegmental bronchial collapse). The clinical presentation of AC may overlap with non-collapsible airway disease (NCAD) or another non-lower airway respiratory disease (NLARD). This study determined whether paired inspiratory (I)/expiratory (E)-breath-hold computed tomography (I/E-BH CT) can detect a static and dynamic AC in dogs with spontaneous respiratory disease and it compared the CT-derived metrics of the AC to the tracheobronchoscopy metrics. The CT-acquired I and E diameter and cross-sectional area (CSA) for the trachea, mainstem and lobar bronchi in dogs with an AC ( = 16), NCAD (16), and NLARD (19) served for a dynamic percent of the airway narrowing (%AN) calculation. A scoring system assessed the bronchomalacia. The circularity was calculated for each airway. The results were compared to the tracheobronchoscopy collapse grading. In the dogs with an AC, the %AN was larger for the trachea, right mainstem bronchus and right middle lobar bronchus when they were compared to the dogs with NCAD and NLARD. Flattening was only identified for the trachea of the AC dogs. The agreement between the CT and tracheobronchoscopy scores was 20% from trachea to the lobar bronchi and 47% for the segmental/subsegmental bronchi. Paired I/E-BH CT can detect static and dynamic AC with limited agreement with the tracheobronchoscopy metrics. Independent scoring systems that are tailored to the clinical manifestations of functional impairments are needed.
PubMed: 36428319
DOI: 10.3390/ani12223091 -
Indian Pediatrics Oct 2018To describe clinical presentation, bronchoscopy findings and associated anomalies in cases of congenital tracheomalacia in infants (age <1y).
OBJECTIVE
To describe clinical presentation, bronchoscopy findings and associated anomalies in cases of congenital tracheomalacia in infants (age <1y).
METHODS
Hospital record review of 88 infants (mean age 8 mo, 57 males) diagnosed as having tracheomalacia by flexible bronchoscopy between 2012 and 2015.
RESULTS
The predominant features were wheeze (57.9%), stridor (42.1%), cough (38.6%), pneumonia (29.5%) and collapse (12.5%). On bronchoscopy, malacia was observed in lower half of trachea in 51 (57.9%) infants. Synchronized airway lesions observed were laryngomalacia (30.7%) and bronchomalacia (3.4%). 15 (17%) infants had associated congenital heart disease and 21 (23.8%) required care in intensive care unit.
CONCLUSION
Wheeze,stridor and cough are the main symptoms in tracheomalacia. Laryngomalacia and congenital heart diseases are the most common other anomalies associated in these infants.
Topics: Bronchoscopy; Female; Heart Defects, Congenital; Humans; Infant; Male; Retrospective Studies; Trachea; Tracheomalacia
PubMed: 30426955
DOI: No ID Found -
Journal of Smooth Muscle Research =... 2021Pompe disease is a lysosomal storage disease caused by mutations within the GAA gene, which encodes acid α-glucosidase (GAA)-an enzyme necessary for lysosomal glycogen...
Pompe disease is a lysosomal storage disease caused by mutations within the GAA gene, which encodes acid α-glucosidase (GAA)-an enzyme necessary for lysosomal glycogen degradation. A lack of GAA results in an accumulation of glycogen in cardiac and skeletal muscle, as well as in motor neurons. The only FDA approved treatment for Pompe disease-an enzyme replacement therapy (ERT)-increases survival of patients, but has unmasked previously unrecognized clinical manifestations of Pompe disease. These clinical signs and symptoms include tracheo-bronchomalacia, vascular aneurysms, and gastro-intestinal discomfort. Together, these previously unrecognized pathologies indicate that GAA-deficiency impacts smooth muscle in addition to skeletal and cardiac muscle. Thus, we sought to characterize smooth muscle pathology in the airway, vascular, gastrointestinal, and genitourinary in the Gaa mouse model. Increased levels of glycogen were present in smooth muscle cells of the aorta, trachea, esophagus, stomach, and bladder of Gaa mice, compared to wild type mice. In addition, there was an increased abundance of both lysosome membrane protein (LAMP1) and autophagosome membrane protein (LC3) indicating vacuolar accumulation in several tissues. Taken together, we show that GAA deficiency results in subsequent pathology in smooth muscle cells, which may lead to life-threatening complications if not properly treated.
Topics: Animals; Disease Models, Animal; Enzyme Replacement Therapy; Glycogen; Glycogen Storage Disease Type II; Humans; Mice; Mice, Knockout; Muscle, Smooth; alpha-Glucosidases
PubMed: 33883348
DOI: 10.1540/jsmr.57.8 -
Pediatric Pulmonology May 2022To study the clinical characteristics and impact of bronchoscopy in children from developing countries, referred for cardiac surgery, through the "Save a Child's Heart"...
OBJECTIVES
To study the clinical characteristics and impact of bronchoscopy in children from developing countries, referred for cardiac surgery, through the "Save a Child's Heart" (SACH) organization.
METHODS
We performed a retrospective hospital-chart review of SACH children (0-18 years old) referred between 2006 and 2021 who underwent fiberoptic bronchoscopy. We examined demographics, congenital-heart-disease (CHD) types, bronchoscopy's indications and findings, subsequent recommendations, number of ventilation, and intensive-care-unit days. The primary outcome was percent changes in management and diagnosis, following the bronchoscopy. We included a control group matched-for-age and CHD type, who did not undergo bronchoscopy.
RESULTS
We performed 82 bronchoscopies in 68 children: 18 (26.5%) preoperatively; 46 (67.6%) postoperatively; and four (5.9%) both. The most prevalent CHDs were Tetralogy-of-Fallot (27.9%) and ventricular-septal-defect (19.1%). The main indications were persistent atelectasis (41%) and mechanical ventilation/weaning difficulties (27.9%). Bronchoscopic evaluations revealed at least one abnormality in 51/68 (75%) children. The most common findings were external airway compression (23.5%), bronchomalacia (19.1%), and mucus secretions (14.7%). Changes in management were made in 35 (51.4%) cases, with a major change made in 14/35 (40%) children. Compared to the control group, the children undergoing bronchoscopy were both ventilated longer (median 6 vs. 1.5 days, p < 0.0001) and stayed longer in the intensive care unit (median 1.5 vs. 18.5 days, p < 0.0001).
CONCLUSION
A bronchoscopy is an important tool in the diagnosis and management of the unique group of children from developing countries with CHD referred for cardiac surgery. The results of our study, reveal a more complicated clinical course in children requiring bronchoscopy compared to controls.
Topics: Adolescent; Bronchoscopy; Child; Child, Preschool; Developing Countries; Heart Defects, Congenital; Humans; Infant; Infant, Newborn; Pulmonary Atelectasis; Retrospective Studies
PubMed: 35212183
DOI: 10.1002/ppul.25869 -
Respiratory Medicine May 2021Recurrent pulmonary infections, wheezing and stridor due to swallowing dysfunction, esophageal dysmotility, gastroesophageal reflux, tracheomalacia and bronchomalacia...
INTRODUCTION
Recurrent pulmonary infections, wheezing and stridor due to swallowing dysfunction, esophageal dysmotility, gastroesophageal reflux, tracheomalacia and bronchomalacia are frequently seen complications after esophageal atresia and tracheo-esophageal fistula (EA-TEF) surgeries. This study aimed to investigate the frequency and causes of respiratory problems and to evaluate the factors that affect respiratory morbidity in patients who had undergone EA-TEF repair in a tertiary referral center.
METHODS
Preoperative and postoperative records of patients with EA, TEF + EA and isolated EA were examined retrospectively. Accompanied diseases and swallowing dysfunction symptoms were questioned. Bronchoalveolar lavage results were investigated if the patient had flexible bronchoscopy.
RESULTS
A total of 71 children with EA were included in the study, and seven patients who did not have follow-up after surgery were excluded. 46 of the 64 patients continue regular follow-up visits in our department. Male sex, primary EA repair in another center, EA type C, accompanying genetic anomalies, severe tracheomalacia, late per oral feeding (1 year after surgery), and severe GER were found to cause significantly higher incidence of coughing, recurrent wheezing, recurrent pneumonia, and bronchiectasis despite surgical and medical treatments (p = 0.048, p = 0.045, p = 0.009, p = 0.029, p = 0.025). CONCLUSİON: Even if anatomical anomalies are corrected by surgery in patients who underwent EA repair, precautions can be taken for GERD, laryngotracheomalacia, and swallowing dysfunction, and effective pulmonary rehabilitation can be initiated with early multidisciplinary approach before the development of respiratory tract symptoms.
Topics: Adolescent; Bronchomalacia; Child; Child, Preschool; Deglutition Disorders; Esophageal Atresia; Esophageal Fistula; Esophageal Motility Disorders; Female; Fistula; Gastroesophageal Reflux; Humans; Infant; Male; Postoperative Complications; Respiratory Sounds; Retrospective Studies; Tracheal Diseases; Tracheomalacia
PubMed: 33813207
DOI: 10.1016/j.rmed.2021.106376 -
Medical Science Monitor : International... Oct 2021BACKGROUND Post-tuberculosis bronchomalacia (PTBM) is one of the main conditions occurring in patients after tracheobronchial tuberculosis (TBTB), and is also associated...
BACKGROUND Post-tuberculosis bronchomalacia (PTBM) is one of the main conditions occurring in patients after tracheobronchial tuberculosis (TBTB), and is also associated with the recurrence of symptoms. The present study aimed to investigate the predictors of PTBM in patients who had been undergoing appropriate TB treatment. MATERIAL AND METHODS Clinical data of 104 patients with symptomatic airway stenosis after TBTB between January 01, 2019 and June 31, 2020 were recorded and analyzed. The association between baseline clinical characteristics, laboratory results, and PTBM was calculated with logistical regression. The time from onset of bronchoscopic intervention was examined by Kaplan-Meier estimates; differences between the 2 groups were tested by the log-rank test. RESULTS Fifty-seven patients (54.81%) had PTBM. In the multivariate logistical analysis, the left main bronchus stenosis lesion (odds ratio [OR]=3.763), neutrophil (NEUT) count (OR=1.527), and platelet (PLT) (OR=1.010) count were predictors of PTBM. During follow-up, patients with BM had a significantly longer duration from onset of bronchoscopic intervention than patients without BM (hazard ratio=2.412, P<0.0001). Further, all patients needing long-term bronchoscopic intervention therapy were subsequently identified as having PTBM. Additionally, blood PLT counts were significantly decreased to normal levels in the non-BM group (P<0.05), but not in the BM group (P>0.05). CONCLUSIONS PTBM is most likely to occur in the left main bronchus. The inflammatory and immune responses associated with NEUT and PLT may represent therapeutic targets of PTBM. Our study is the first to report that decreased blood PLT count has the potential to monitor the treatment response.
Topics: Adult; Bronchi; Bronchial Diseases; Bronchomalacia; Bronchoscopy; Constriction, Pathologic; Female; Humans; Male; Mycobacterium tuberculosis; Neutrophils; Platelet Count; Prognosis; Retrospective Studies; Risk Assessment; Risk Factors; Tomography, X-Ray Computed; Tuberculosis, Pulmonary; Young Adult
PubMed: 34620816
DOI: 10.12659/MSM.931779