Lower Airway Involvement Research Articles

An unusual case of Juvenile-onset Recurrent Respiratory Papillomatosis with lower airway involvement in a 15-month-old boy

An unusual case of Juvenile-onset Recurrent Respiratory Papillomatosis with lower airway involvement in a 15-month-old boy

Elexacaftor-Tezacaftor-Ivacaftor Therapy for Chronic Sinus Disease in Cystic Fibrosis

Cystic fibrosis (CF) is a multiorgan genetic disease with progressive upper and lower airway involvement. The effects of CF transmembrane conductance regulator (CFTR) modifier therapies on CF-related upper airway disease, specifically chronic rhinosinusitis (CRS), are not characterized. To determine the outcome of elexacaftor-tezacaftor-ivacaftor (ETI) on CRS as measured by changes in sinus computed tomography (CT) metrics and on clinical parameters in individuals with CF. This prospective longitudinal cohort study was conducted at the CF center of a tertiary care hospital between October 1, 2019, and July 31, 2021. A total of 64 participants with CF were included in the analysis. Sinus CT was obtained within 1 month of initiation of ETI therapy (baseline), and within 1 month of 1 year of ETI therapy. Images were independently analyzed by pulmonology, radiology, and otolaryngology physicians, using the Lund-Mackay and Sheikh-Lind scoring systems. Percent predicted forced expiratory volume in 1 second (ppFEV1), body mass index (BMI), and microbiologic data collected at initiation of ETI therapy and 3-month intervals for 1 year were also measured. The study hypothesis was that ETI therapy will improve CRS as measured by changes in sinus CT at initiation and 1 year after ETI therapy and clinical parameters in individuals with CF. Among the 64 participants (39 [60.9%] female; median age, 18.5 [IQR, 16.0-28.5] years; 64 [100%] White), improvement in CRS was noted by improvements in sinus CT scans using both sinus CT scoring systems after 1 year of ETI therapy. The reduction in the median total score using the Lund-Mackay sinus CT scoring system (from 5.8 [IQR, 5.0-7.0] to 3.3 [IQR, 2.6-4.2]) and the Sheikh-Lind scoring system (from 3.8 [IQR, 3.0-5.0] to 2.2 [IQR, 2.0-2.5]) was noted. Increases in ppFEV1 and BMI were also observed by 3 months of ETI therapy with persistent improvement through 1 year of treatment. Similarly, after 1 year of ETI therapy, participants with CF had reductions in positivity for Pseudomonas aeruginosa and Staphylococcus aureus in oropharyngeal cultures. This cohort study found that use of ETI therapy was associated with improved CRS outcomes in participants with CF as quantified by improved sinus CT scans measured by 2 radiographic scoring systems and was also associated with improved clinical outcomes. Despite improvement in CT scan scores, most people with CF continue to have scores that indicate severe sinus disease.

Linking digital surveillance and in-depth virology to study clinical patterns of viral respiratory infections in vulnerable patient populations

SummaryTo improve the identification and management of viral respiratory infections, we established a clinical and virologic surveillance program for pediatric patients fulfilling pre-defined case criteria of influenza-like illness and viral respiratory infections. The program resulted in a cohort comprising 6,073 patients (56% male, median age 1.6 years, range 0–18.8 years), where every patient was assessed with a validated disease severity score at the point-of-care using the ViVI ScoreApp. We used machine learning and agnostic feature selection to identify characteristic clinical patterns. We tested all patients for human adenoviruses, 571 (9%) were positive. Adenovirus infections were particularly common and mild in children ≥1 month of age but rare and potentially severe in neonates: with lower airway involvement, disseminated disease, and a 50% mortality rate (n = 2/4). In one fatal case, we discovered a novel virus: HAdV-80. Standardized surveillance leveraging digital technology helps to identify characteristic clinical patterns, risk factors, and emerging pathogens.

Localized Granulomatous with Polyangiitis (GPA): Varied Clinical Presentations and Update on Treatment.

Granulomatosis with polyangiitis is a primary systemic vasculitis commonly described with the typical triad of upper airway, lung, and kidney involvement. Upper and lower airway involvement is characteristic in patients with granulomatosis with polyangiitis and can sometimes represent the initial or in some instances the sole manifestation. The objective of this review is to summarize the various clinical manifestations of localized disease in GPA and their treatment. Sinonasal disease is seen in up to 90% of patients. Otologic and ocular involvement is also commonly seen. Laryngeal and tracheal disease although less common is associated with significant morbidity and can be therapeutically challenging. Clinicians need to be aware of these localized GPA manifestations as they may be presenting disease features in the absence of other systemic findings. Treatment of localized GPA involves both immunosuppressive and surgical interventions for specific manifestations. Collaboration between specialists including rheumatologists, otolaryngologists, and ophthalmologists is often crucial to ensure optimal outcomes for patients. This is a narrative review that provides a comprehensive overview of localized granulomatosis with polyangiitis and current treatment options.

Investigation of the frequency and clinical manifestations of Wegener vasculitis among other vasculitis patients in rheumatology ward of Vali-asr hospital during the past 10 years

Background and objective: Vasculitis is a rare disease and its prevalence varies according to age, race, time and geographical location. Due to the fact that no study has been conducted in Iran to investigate the frequency, clinical and laboratory symptoms of vasculitis in a considerable population of vasculitis patients, so the need for this study is felt. The aim of this study was to evaluate the frequency and clinical signs of granulomatosis with polyangitis (GP) patients who admited in rheumatology ward of Valiasr hospital in the last 10 years.Methods and material: In this study, all patients with a possible diagnosis of vasculitis in the rheumatology ward of Valiasr Hospital during the past 10 years were evaluated. Then the GP patients were evaluated for clinical, laboratory, radiological, pathological findins, outcome, mortality, and major complications. Results: During the last 10 years, 223 vasculitis patients have been admitted to Valiasr hospital. Of these, 87 % were primary vasculitis and 13% were secondary vasculitis. The highest frequncy was related to GP (21.52 %) followed by Behcet's disease. Women and men were included 52.08 % and 47.91 % of GP patients, respectively. Upper and lower airway involvement and totally ear, nose and throat (ENT) involvement were the most common clinical symptoms in GP patients.Conclusion: According to the finding of this study, GP vasculitis was the most common type of vasculitis that ENT involvements were the most common clinical symptoms in this patients. However, more studies are required to confirm these data in Iran.

Laryngeal, Tracheal, and Bronchial Disease in the Mucopolysaccharidoses: Endoscopic Study.

Mucopolysaccharidoses (MPS) are genetically determined diseases, leading to a deficiency of enzymes in the glycosaminoglycan (GAG) degradation pathway. The accumulation of GAG occurs in connective tissue in various organs and systems of the body, including the larynx, trachea, and bronchi. Respiratory symptoms are common and severe in these patients, and respiratory disease is a frequent cause of death. A cross-sectional study with flexible bronchoscopy was conducted in 30 MPS patients (6 MPS I, 8 MPS II, 2 MPS III, 3 MPS IV-A, and 11 MPS VI). Only four patients (13.33%) had a normal airway; nine (30%) had mild to moderate disease, 12 (40%) moderate to severe, and five patients (16.67%) had severe disease. Of particular interest, neuronopathic MPS II had the largest proportion of tracheostomized patients who died due to respiratory complications; in MPS IV-A, all patients had significant tracheobronchial deformity with associated tracheomalacia, despite lacking laryngeal involvement. Laryngotracheobronchial disease (LTBD) was associated to longer disease history and was significantly more severe in older patients. Longer use of enzyme replacement therapy did not prevent the progression of LTBD, although the age of therapy introduction may be a crucial factor in lower airway involvement.

Longitudinal progression of aspirin-exacerbated respiratory disease: analysis of a national insurance claims database.

Aspirin-exacerbated respiratory disease (AERD) is a recalcitrant inflammatory disorder defined by asthma, nasal polyposis, and sensitivity to cyclooxygenase-1 inhibitors. The timeline and course of disease progression is unclear. The Truven MarketScan Database, a large American health insurance claims repository, was queried to identify patients meeting criteria for AERD from 2009 to 2015. Included patients had associated International Classification of Diseases, 9th edition (ICD-9) codes consistent with all 3 components of AERD: asthma, nasal polyposis, and drug allergy. Patterns of disease onset and time to progression were analyzed. A total of 5628 patients were identified for study inclusion. Of the 3 components of AERD, 3303 patients (59%) were initially diagnosed with asthma, 1408 (25%) were initially diagnosed with nasal polyps, and 917 (16%) were first diagnosed with drug sensitivity. The most common (36%) sequence of diagnoses was asthma, followed by nasal polyps, followed by drug allergy. The median interval between diagnosis of upper or lower airway involvement (ie, nasal polyps and/or asthma) to recognition of drug sensitivity was 259 days (quartiles Q1 to Q3: 92 to 603 days). In patients with both asthma and nasal polyps diagnoses, the risk of developing drug sensitivity during the study time period was 6%. Upper and lower airway disease is often initially recognized in patients with AERD, whereas drug sensitivity presents month to years later. This delay may be due to the pathophysiology of AERD and disease progression or due to practice patterns in diagnostic testing and coding. Further work is warranted to identify these patients at early stages in their disease progression.

Endobronchial brachytherapy—A novel approach for the management of airway amyloidosis

PurposeAirway amyloidosis is an uncommon condition and may have variable clinical course. There is no proven systemic therapy, and the management mainly relies on local invasive treatments. Several reports have found external beam radiotherapy to produce durable responses. The optimal dose and fractionation has not been determined. Endobronchial brachytherapy (EBB) has never been reported to affect this disease. MethodsThe study includes a retrospective chart review of all patients with airway amyloidosis treated with EBB at the Davidoff Cancer Center. Data include symptoms, radiotherapy method and dose, treatment outcomes, and toxicities. ResultsThree patients were included. Patients were either symptomatic with extensive airway involvement or have undergone multiple local procedures with rapid recurrence of the amyloid deposits. Two patients had upper and 1 patient had lower airway involvement. Two were treated with external beam radiotherapy and EBB and 1 was treated with EBB only. All patients achieved rapid improvement of symptoms with visible improvement in followup bronchoscopies. EBB doses ranged from 7.5 to 10 Gy. Of the seven lesions treated with EBB, only one lesion recurred 6 months from treatment. No major toxicities were reported. ConclusionsEBB is effective and safe for the management of airway amyloidosis and can offer long-term control. This is the first report of EBB for this indication. EBB should be further explored as means of minimizing the irradiated lung volume.

Cholinergic Urticaria with Anaphylaxis: An Underrecognized Clinical Entity

Cholinergic urticaria is a form of physical urticaria triggered by high ambient temperature, strenuous physical activity, and strong emotion. These same triggers may cause multisystem reactions that can be life-threatening. A study of patients with cholinergic urticaria with anaphylaxis was undertaken to describe the demographic and clinical features of this form of anaphylaxis. To describe a cohort of patients with anaphylaxis triggered by high ambient temperature, exertion, and stress. Patients from an academic allergy practice in a university teaching hospital were identified by retrospective chart review. A total of 19 patients with recurrent episodes of anaphylaxis due to cholinergic triggers were identified. The female:male ratio was 15:4 (79% females). The mean age of onset was 27.5 years. Patients experienced a mean of 9.41 episodes per year. All 19 patients (100%) reported anaphylaxis triggered by high ambient temperature, 89.5% reported anaphylaxis triggered by strenuous exertion, and 78.9% reported anaphylaxis triggered by stress. Cutaneous involvement was present in 94.7%; 78.9% had upper airway obstructive symptoms, 78.9% had lower airway involvement, 57.9% had gastrointestinal involvement, and 78.9% had cardiovascular manifestations. Anaphylaxis severity scores were grade 1 (mild) in 11.1%, grade 2 (moderate) in 44.4%, and grade 3 (severe) in 44.4%. Baseline tryptase levels were normal in all but 1 patient. Anaphylaxis due to cholinergic triggers is underreported, with only several case reports in the literature. Reactions are multisystem with cutaneous, upper and lower airway, and cardiovascular involvement in most patients. Manifestations may be life-threatening, and reactions are often severe.

Late-Onset Pompe Disease With Left-Sided Bronchomalacia

Pompe disease is a rare autosomal recessive disorder caused by α-glucosidase deficiency. Lower airway involvement and management are rare in patients with late-onset Pompe disease. We describe the case of a 16-y-old girl with late-onset Pompe disease who presented with obvious progressive deterioration in respiratory function. Pulmonary hypertension was also apparent on echocardiography. She had been on enzyme replacement therapy and nighttime CPAP ventilation for several years. Flexible bronchoscopy was used for diagnosis and subsequent implantation of a bronchial airway stent. Following implantation of the stent, the patient's pulmonary function stabilized, and her pulmonary hypertension resolved. The patient continued on enzyme replacement therapy and nighttime CPAP ventilation. This case highlights that lower airway involvement may occur with late-onset Pompe disease and that flexible bronchoscopy can be an effective tool for both diagnosis and management of lower airway collapse in late-onset Pompe disease.

A10: Younger Age and Severity of Renal Presentation Distinguishes Microscopic Polyangiitis From Granulomatosis With Polyangiitis in Children: An ARChiVe Study

Background/Purpose:Comparisons of pediatric ANCA‐associated vasculitis subtypes (AAV) are limited by the paucity of reported cases, standardized definitions, and overlapping classification criteria. Published work from ARChiVe (A Registry for Childhood Vasculitis) demonstrated modifications of validated classification algorithms applied to pediatric patients with AAV can classify each patient to mutually exclusive diagnostic categories. We compared presenting features of children with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) classified according to this methodology.Methods:A pediatric modification of the European Medicines Agency (EMA) algorithm for classifying AAV and polyarteritis nodosa (incorporating the EULAR/PRINTO/PRES pediatric classification criteria for GPA) was applied to patients in ARChiVe censored to April 2012. We compared characteristics of patients classified as having MPA and GPA. STATA (Statcorp, 2013) was used to calculate frequencies, percentages, and chi‐squared with fisher's exact for categorical variables and means, standard deviations, and t‐tests for continuous variables.Results:One hundred fifty‐two of 227 children in ARChiVe met criteria for diagnosis of MPA (n = 22) or GPA (n = 130). Characteristics and presenting features are shown in Table . Children with MPA were younger at diagnosis (mean diff. 2.7y, p = <0.01). Renal involvement was predominant in both groups. Renal biopsies in 40% of both groups were consistent with pauci‐immune, necrotizing glomerulonephritis. Children with MPA had higher rates of nephrosis, renal failure requiring dialysis, and abnormal creatinine clearance (Table ). Upper and lower airway involvement was more prevalent among those with GPA largely in accordance with surrogate GPA features used to differentiate GPA and MPA in the EMA algorithm. The majority of patients presented with constitutional symptoms, however, other organ systems were less frequently involved. Most patients received combination therapy corticosteroids and cytoxan (64% MPA, 81% GPA) with additional plasmapheresis (29% MPA, 21% GPA), rituximab (14% MPA, 3% GPA) or methotrexate (7% MPA, 1% GPA). The remainder of children received combination corticosteroids and methotrexate or rituximab, without cytoxan (12% MPA, 11% GPA). A larger proportion of patients with MPA received antihypertensive agents and/or ACE inhibitors (64% vs 35%, p = 0.01). Characteristics & Presenting Clinical Features of children with microscopic polyangiitis or granulomatosis with polyangiitis in the ARChiVe cohort (n = 152) Algorithm‐derived diagnosis Characteristic/Feature MPA (n = 22) GPA (n = 130) p‐value Female, n(%) 15 (68) 83 (64) 0.81 Caucasian, n (%) 11 (50) 85 (66) 0.231 Age at diagnosis, yrs, mean (sd) 11.6 (5) 14.2 (3) <0.01* Median (range) 12.8 (9–15) 14.9 (4–19) Symptom duration prior to diagnosis, mos, mean (sd) 4.4 (9) 5.2 (10) 0.72 Median (range) 3.4 (0–35.8) 4.8 (0–66.8) MD‐assigned diagnosis (clinical diagnosis) MPA or isolated MPA 8 (36) 18 (14) 0.027* WG or limited WG 7 (32) 107 (82) <0.01* ANCA 3 (14) 1 (1) 0.01* Unclassified 4 (18) 4 (3) 0.02* General Features Fatigue 19 (86) 113 (87) 1.00 Fever 10 (46) 71 (55) 0.49 Weight loss 10 (46) 60 (46) 1.00 Renal 18 (82) 103 (79) 1.00 Hypertension 8 (36) 28 (22) 0.17 Hematuria and proteinuria with red blood cell casts 16 (73) 97 (75) 0.80 Nephrotic range proteinuria with edema 6 (27) 14 (11) 0.04* Renal failure requiring dialysis 7 (32) 17 (13) 0.05* Creatinine clearance >25% lower limit of normal 8 (89) 18 (36) <0.01* Biopsy‐proven glomerulonephritis 6 (27) 28 (22) 0.58 Pulmonary 9 (41) 105 (81) <0.01* Chronic cough 6 (27) 79 (61) <0.01* Shortness of breath 4 (18) 63 (49) 0.01* Hemoptysis/alveolar hemorrhage 3 (14) 54 (42) 0.02* Fixed pulmonary infiltrates ± cavitations, nodules 4 (18) 99 (76) <0.01* Head, Ear, Nose, & Throat 4 (18) 97 (75) <0.01* Bloody nasal discharge ± crusting 0 (0) 68 (52) <0.01* Chronic sinusitis, otitis, or mastoiditis 0 (0) 63 (48) <0.01* Subglottic involvement 0 (0) 16 (12) 0.13 Cranial bone ± cartilage destruction 0 (0) 10 (8) 0.36 Acute hearing loss 0 (0) 15 (12) 0.13 Red ± painful eye conditions 2 (9) 34 (26) Gastrointestinal/Abdominal 15 (68) 51 (39) 0.01* Chronic nausea 9 (41) 17 (13) <0.01* Skin 10 (45) 71 (55) 0.49 Musculoskeletal 80 (62) 14 (64) 1.00 Nervous System 35 (27) 6 (27) 1.00 Cardiovascular 9 (7) 1 (5) 1.00 ANCA Serology pANCA or anti‐MPO 14 (70) 35 (29) <0.01 cANCA or anti‐PR3 6 (30) 86 (71) <0.01Conclusion:Children with AAV had predominantly renal and constitutional manifestations. Younger age and a more severe renal disease phenotype may characterize MPA with patients requiring additional treatment for the consequences of kidney disease. The wide variations in time to diagnosis continue to suggest that pediatric AAV is poorly recognized. Ongoing biomarker‐driven studies may complement systems for subclassifying patients with AAV.

Airway malacia in children with achondroplasia

This study was undertaken to assess the frequency of airway malacia in infants and young children with achondroplasia, a population well known to be at risk for a variety of respiratory problems. We also wished to evaluate what, if any, contribution airway malacia makes to the complex respiratory issues that may be present in those with achondroplasia. Retrospective chart review of all infants and young children with achondroplasia who were assessed through the Midwest Regional Bone Dysplasia Clinics from 1985 through 2012 (n = 236) was completed. Records of comprehensive clinical examinations, polysomnographic assessments, and airway visualization were reviewed and abstracted using a data collection form. Analyses were completed comparing the group with and those without evidence for airway malacia. Thirteen of 236 patients (5.5%) were found to have airway malacia. Most of those affected had lower airway involvement (9/13). The presence of airway malacia was correlated with an increased occurrence of obstructive sleep apnea as well as need for oxygen supplementation, airway surgeries and tracheostomy placement. Although estimates of the frequency of airway malacia in the general population are limited, its frequency in children with achondroplasia appears to be much higher than any published general population estimate. The presence of airway malacia appears to confound other breathing abnormalities in this population and results in the need for more invasive airway treatments.

Common and Uncommon Manifestations of Wegener Granulomatosis at Chest CT: Radiologic-Pathologic Correlation

Wegener granulomatosis is an uncommon necrotizing vasculitis that classically manifests as a clinical triad consisting of upper and lower airway involvement and glomerulonephritis. Other less frequently involved organ systems include the central and peripheral nervous system and large joints. The diagnosis is based on a combination of clinical and laboratory findings. Because thoracic involvement often predominates, chest radiographic findings are often the first to suggest the diagnosis. However, chest computed tomography (CT) has superior sensitivity and specificity for evaluation of the airways, lung parenchyma, and mediastinum, particularly with the use of multiplanar reformatted and three-dimensional images. Common pulmonary radiologic findings include waxing and waning nodules, masses, ground-glass opacities, and consolidation. Airway involvement is usually characterized by circumferential tracheobronchial thickening, which can be smooth or nodular. Pleural effusions are the most common manifestation of pleural disease and can result from primary involvement or be secondary to renal failure. Mediastinal lymphadenopathy is a nonspecific finding and is usually reactive. Uncommon thoracic radiologic manifestations include involvement of the heart and great vessels. CT is the imaging modality of choice for diagnosis, surveillance, and follow-up in patients with Wegener granulomatosis.

The link between allergic rhinitis and asthma: the united airways disease

Rhinitis and asthma are often associated and the two disorders interact at various levels. Rhinitis typically precedes the development of asthma and can contribute to unsatisfactory asthma control. The presence and type of asthma is influenced by sensitization, and the duration and severity of allergic rhinitis. Nasal symptoms, airflow and markers of inflammation directly correlate with lower airway involvement. Local tissue factors, such as microbial stimuli and systemic inflammatory mechanisms, play a role in the clinical expression of the allergic airway syndrome. There is increasing evidence that suggests a major involvement of airway epithelial cells in the pathogenesis of both asthma and allergic rhinitis. Even in patients with rhinitis who do not have asthma, subclinical changes in the lower airways and inflammatory mediators can be detected. The pathogenic role of paranasal sinus infections in respiratory allergy has been better elucidated but there remains a need for further research. Treatment of established rhinitis may affect asthma control and could have some impact on airway obstruction, but a direct effect of rhinitis therapy on lower airway inflammation remains to be clearly established.

Wegener's granulomatosis: clinical and laboratory results of a university hospital study of 20 patients from Turkey

The aim of this study was to evaluate the clinical and laboratory features, the treatment approaches, and the long-term outcome of patients with Wegener's granulomatosis (WG) who were followed up in our hospital. The hospital files of the patients with the diagnosis of WG who were followed up between the years 1985 and 2003 in Hacettepe University Hospital were retrospectively evaluated. Male/female ratio was 12:8. The mean age was 39 years (range 20-65 years). Constitutional symptoms and upper and lower airway involvement were seen in 95% of all patients. Renal and musculoskeletal symptoms were seen in 90 and 80% of the patients, respectively. Five patients were treated with oral monotherapy (three with methylprednisolone and two with cyclophosphamide). Three patients were given a combination of orally administered cyclophosphamide and methylprednisolone. Ten patients were treated with pulse cyclophosphamide and methylprednisolone combination together with oral alternate-day methylprednisolone therapy. The remaining two resistant patients were treated with pulse cyclophosphamide, methylprednisolone, and intravenous immunoglobulin combination together with oral alternate-day methylprednisolone. Four patients died because of the disease activity. Intravenous pulse therapies with oral, alternate-day methylprednisolone were well tolerated. Sixteen patients experienced long-term remission after immunosuppressive treatment. Eleven patients have been asymptomatic for more than 12 months. In five patients, residual symptoms persisted: constitutional symptoms and renal and respiratory tract symptoms in varying combinations. The demographic and laboratory findings in this trial were similar with those of the previous results. Alternate-day glucocorticoids plus cyctotoxic drugs may be beneficial in patients with WG.

Influenza A aviária (H5N1): a gripe do frango

Este estudo tem como objetivo rever a literatura sobre o vírus influenza A aviária (H5N1). O levantamento bibliográfico foi realizado nos bancos de dados eletrônicos Medline, MD Consult, HighWire, Medscape e Literatura Latinoamericana y del Caribe en Ciencias de la Salud (LILACS, Literatura Latinoamericana e do Caribe em Ciências da Saúde), e por pesquisa direta, referentes aos últimos dez anos. Foram selecionados 32 artigos originais abordando os surtos recentes de infecção por um subtipo de vírus influenza A aviária, o H5N1, em criações de aves domésticas na Ásia, que resultaram em importantes prejuízos econômicos e repercussões em saúde pública, além de casos de infecção humana de alta letalidade. A maioria dos casos está associada com a exposição direta a aves infectadas ou superfícies contaminadas com excrementos dessas aves, porém foi confirmada a transmissão entre humanos. O período de incubação foi de dois a quatro dias. As manifestações clínicas variaram de infecção assintomática e doença leve do trato respiratório superior a pneumonia grave e falência múltipla de órgãos. A radiografia de tórax pode apresentar infiltrado intersticial bilateral, colapso lobar, consolidação focal e broncograma aéreo sem derrame pleural. A presença de linfopenia indica pior prognóstico. O tratamento de suporte parece ser o único tratamento aceitável. Os fatores de risco para mau prognóstico incluem idade avançada, demora na hospitalização, envolvimento do trato respiratório inferior, baixa contagem de leucócitos totais e linfopenia à admissão. Controlar os surtos em aves domésticas e o contato entre seres humanos e tais aves deve ser a prioridade no manejo da doença em nível de saúde pública, e medidas e conhecimentos acerca da doença devem ser amplamente divulgados.

A Decade Surveillance Study of Mycobacterium xenopi Disease and Antimicrobial Susceptibility Levels in a Reference Teaching Hospital of Northern Italy: HIV-Associated Versus Non-HIV-Associated Infection

Objective and Method: The aim of our survey is to investigate the epidemiology and in vitro antimicrobial susceptibility levels of 35 consecutive Mycobacterium xenopi strains responsible for confirmed disease at a University Hospital from 1993 to 2002 and to identify eventual differences in the in vitro sensitivity profile between the 17 strains isolated from patients with HIV disease and the 18 isolates cultured from non-HIV-infected individuals. Results: The involvement of lower airways accounted for 88.6% of cases; but atypical pulmonary findings, including cavitation and a prominent inflammatory reaction, recently emerged in HIV-infected patients successfully treated with HAART, which raises the possible role of immune reconstitution syndrome in the clinical pathomorphism of this opportunistic disease. When compared with non-HIV-infected patients, patients with HIV disease had a lower mean age and a tendency to suffer from late relapses. The greatest overall in vitro sensitivity rate was registered for capreomycin and protionamide (100% of strains) followed by kanamicin (96.6%), whereas susceptibility rates for the first-line compounds such as ethambutol, isoniazid, and rifampicin were slightly lower (85.7% to 91.4%). No temporal variation in the susceptibility index was seen over the study decade. Non-HIV-infected patients experienced a higher frequency of M. xenopi isolates that proved to be resistant to at least one tested compound compared with HIV-associated episodes, despite the heavy and prolonged exposure of HIV-infected patients to broad spectrum antimicrobials, which included agents effective on atypical mycobacteria. Only one HIV-positive patient developed rifampicin resistance in his third disease recurrence. Conclusion: A rapid diagnosis, a reliable differentiation between colonization and disease, and an optimal therapeutic choice for atypical mycobacterial disease (including M. xenopi one) are still serious challenges for clinicians and bacteriologists who treat immunocompromised patients, such as those with HIV disease. In the immunocompromised host, diagnostic difficulties posed by late identification and eventually concurrent opportunistic disorders add their negative effects to therapeutic problems due to the unpredictable in vitro susceptibility profile of atypical mycobacteria, such as M. xenopi.

Necrotizing Tracheobronchitis With Progressive Airflow Obstruction Associated With Paraneoplastic Pemphigus

Paraneoplastic pemphigus (PNP) is an autoimmune disease associated with leukemia and non-Hodgkin's lymphoma. A patient with stage IVB poorly differentiated lymphocytic lymphoma developed characteristic upper and lower airway involvement with profound mucocutaneous erosion and tracheobronchial epithelial desquamation. Immunofluorescence testing confirmed autoantibody deposition along the basement membrane of bronchial epithelium. Disruption of the cellular adhesion mechanisms, including desmosomes, hemidesmosomes, and possibly the integrin subunits, is presumed to have led to disruption and desquamation of the tracheobronchial epithelial barrier, severe obstruction of the airways and hypoxia, and possibly bacterial superinfection. As far as can be determined, the feature of airflow obstruction occurring in association with PNP has not been described. Physicians should be aware that these complications of PNP may rapidly lead to hypoxic respiratory failure and death.

Stevens-Johnson Syndrome and Respiratory Failure in a 9-Year-Old Boy

The etiology of respiratory failure associated with Stevens-Johnson syndrome may be multifactorial, including upper airway involvement, pneumothorax/pneumomediastinum, and direct involvement of the respiratory mucosa. Respiratory failure from direct involvement of the respiratory mucosa is relatively uncommon. We describe a 9-year-old boy who had respiratory failure associated with Mycoplasma pneumoniae-induced Stevens-Johnson syndrome. Bronchoscopic examination of the airways revealed sloughed mucosa, ulcerative lesions, and inspissated secretions indicative of lower airway involvement with Stevens-Johnson syndrome. Although the mainstay of therapy is supportive care with controlled ventilation, rigid bronchoscopy with bronchoalveolar lavage to clear the airways of the debris was an invaluable adjunct to this patient's care.

Radiologic evaluation of swallowing.

Diagnostic imaging procedures are useful to analyze the complex mechanism of swallowing. They may be employed beneficially to answer specific questions of swallow coordination, and to distinguish between various forms of airway penetration. The pathophysiology of upper and lower airway involvement may be determined and visualized.