ABSTRACT
El síndrome de Swyer-James-Mac Leod es una entidad poco frecuente adquirida en la infancia, generalmente tras una infección pulmonar moderada a grave de tipo bronquiolitis o neumonía, generalmente virales. Ocasionalmente se tiene el antecedente de infecciones repetidas de este tipo. Consiste en el desarrollo de enfisema hipoplásico pulmonar unilateral, que puede a veces relacionarse con bronquiectasias ipsilaterales o bilaterales, obstrucción fija al flujo aéreo y puede también asociarse a reducción del flujo sanguíneo del pulmón hipoplásico, de manera focal o difusa, con o sin tortuosidad de la vascularización proximal y a veces con una compensación del pulmón contralateral, en forma de sobredistensión e hiperflujo vascular relativo. Presentamos el caso de un varón de 79 años de edad con antecedentes de infecciones tipo bronquiolitis virales repetidas en la infancia, obstrucción fija grave al flujo aéreo y hemoptisis masiva secundaria a una infección por Pseudomonas aeruginosa sensible a la terapia antibiótica habitual.
Swyer-James-Mac Leod syndrome is a rare condition acquired in childhood, usually after a moderate to severe lung infection such as bronchiolitis or pneumonia, usually viral. Occasionally there is a history of repeated infections of this type. It consists of the development of unilateral pulmonary hypoplastic emphysema, which can sometimes be related to ipsilateral or bilateral bronchiectasis, fixed airflow obstruction, and may also be associated with reduced blood flow in the hypoplastic lung, with or without tortuosity of the proximal vascular supply and sometimes with compensation from the contralateral lung, in the form of overdistension and relative vascular hyperflow. We present the case of a 79-year-old man with a history of recurrent viral bronchiolitis-type infections in childhood, severe fixed airflow obstruction, and massive hemoptysis secondary to a Pseudomonas aeruginosa infection sensitive to usual antibiotic therapy.
Subject(s)
Humans , Male , Aged , Pulmonary Emphysema/complications , Lung, Hyperlucent/complications , Hemoptysis/etiology , Pulmonary Emphysema/therapy , Pulmonary Emphysema/diagnostic imaging , Radiography, Thoracic , Lung, Hyperlucent/therapy , Lung, Hyperlucent/diagnostic imaging , Computed Tomography AngiographyABSTRACT
El síndrome de Swyer-James-MacLeod es un cuadro pulmonar muy infrecuente que se caracteriza radiológicamente por hiperclaridad pulmonar lobar o multilobar, producido por la disminución de la vascularización pulmonar, por lo general, por un proceso infeccioso previo. En ocasiones, es un hallazgo casual en el estudio por infecciones respiratorias o crisis asmáticas repetidas. Se presenta el caso de un paciente de 6 años de edad que, en el contexto de probable neumonía persistente, se realizó un amplio estudio que permitió el diagnóstico del síndrome de Swyer-James-MacLeod. Se desarrolló un abordaje terapéutico multidisciplinar con el que, hasta la fecha, la evolución ha sido satisfactoria, con tratamiento médico y fisioterapia respiratoria.
Swyer-James-MacLeod syndrome is a rare pulmonary disorder that is characterized radiologically by pulmonary lobar or multilobar hyperlucency produced by decreased pulmonary vascularization usually due to a previous infectious process. It is frequently a casual finding in recurrent pulmonary infections or in the study of asthma symptoms. We present the case of a 6-year-old patient who was diagnosed with Swyer-James-MacLeod syndrome after a persistent pneumoniae. A multidisciplinary approach was conducted, with a satisfactory progress up to date, combining medical treatment with respiratory physiotherapy.
Subject(s)
Humans , Male , Child , Lung, Hyperlucent/diagnostic imaging , Pneumonia , Bronchial Spasm , Lung, Hyperlucent/drug therapy , Lung, Hyperlucent/therapyABSTRACT
Swyer-James-MacLeod syndrome is a rare pulmonary disorder that is characterized radiologically by pulmonary lobar or multilobar hyperlucency produced by decreased pulmonary vascularization usually due to a previous infectious process. It is frequently a casual finding in recurrent pulmonary infections or in the study of asthma symptoms. We present the case of a 6-year-old patient who was diagnosed with Swyer-James-MacLeod syndrome after a persistent pneumoniae. A multidisciplinary approach was conducted, with a satisfactory progress up to date, combining medical treatment with respiratory physiotherapy.
El síndrome de Swyer-James-MacLeod es un cuadro pulmonar muy infrecuente que se caracteriza radiológicamente por hiperclaridad pulmonar lobar o multilobar, producido por la disminución de la vascularización pulmonar, por lo general, por un proceso infeccioso previo. En ocasiones, es un hallazgo casual en el estudio por infecciones respiratorias o crisis asmáticas repetidas. Se presenta el caso de un paciente de 6 años de edad que, en el contexto de probable neumonía persistente, se realizó un amplio estudio que permitió el diagnóstico del síndrome de Swyer-James-MacLeod. Se desarrolló un abordaje terapéutico multidisciplinar con el que, hasta la fecha, la evolución ha sido satisfactoria, con tratamiento médico y fisioterapia respiratoria.
Subject(s)
Lung, Hyperlucent/diagnosis , Lung/pathology , Pneumonia/etiology , Child , Humans , Lung, Hyperlucent/therapy , Male , Physical Therapy Modalities , Pneumonia/therapyABSTRACT
RATIONALE: Swyer-James-Macleod syndrome (SJMS) is a rare lung disorder characterized by unilateral hyperlucent lungs which arises as a complication of bronchiolitis obliterans. It is typically diagnosed during childhood, although some patients may only be diagnosed in adulthood, often as an incidental finding, but others due to recurrent chest infections. PATIENT CONCERNS: Three patients were referred to our institution with complaints of dyspnea on exertion, chronic productive cough, and recurrent pulmonary infections. Two of them had a history of lower respiratory tract infections during childhood. DIAGNOSIS: A computed tomography scan was performed and showed unilateral reduced density of the lung and bronchiectasis in the 3 patients. Based on the clinical presentation and radiologic features, the diagnosis of SJMS was established. INTERVENTIONS: Patients started inhaled corticosteroids and long acting beta agonist, vaccines to prevent respiratory infections were administered and airway clearance techniques were taught. OUTCOMES: Two patients became asymptomatic with inhaled therapy and no recurrent chest infections were observed over a 3-year follow-up period, being discharged from our institution to the general practitioner. The 3rd patient had some improvement in the frequency of pulmonary infections and dyspnea, without improvement in respiratory function tests, maintaining vigilance at our center. LESSONS: These cases highlight the importance of being aware of this condition and its frequent association with bronchiectasis, which may adversely affect the prognosis, to manage patients appropriately and prevent recurrent pulmonary infections.
Subject(s)
Lung, Hyperlucent/diagnosis , Adult , Aged , Humans , Lung, Hyperlucent/etiology , Lung, Hyperlucent/therapy , Male , Middle Aged , Tomography, X-Ray ComputedABSTRACT
Swyer-James-Macleod syndrome is a rare radiologic entity with radiolucent lung fields which could include the entire lung, a lobe or a subsegment. The pathogenesis is unclear but is thought to be related to childhood pulmonary viral and non-viral infections with subsequent vascular and alveolar distortion. It usually presents incidentally in asymptomatic patients on imaging, but could present with recurrent pulmonary infections, shortness of breath, cough, hemoptysis or bronchiectasis. Treatment is usually observation if asymptomatic, but could potentially include inhaled steroids, bronchodilators, lobectomy segmentectomy or pneumonectomy depending on the clinical condition and severity of the symptoms. We present a case of SJMS found in a asymptomatic patient along with a brief literature review.
Subject(s)
Bronchiectasis , Lung, Hyperlucent , Cough/etiology , Hemoptysis/etiology , Humans , Lung, Hyperlucent/complications , Lung, Hyperlucent/diagnosis , Lung, Hyperlucent/therapy , PneumonectomySubject(s)
Cough/diagnosis , Lung, Hyperlucent , Lung/diagnostic imaging , Diagnosis, Differential , Disease Management , Female , Humans , Lung, Hyperlucent/diagnostic imaging , Lung, Hyperlucent/physiopathology , Lung, Hyperlucent/therapy , Medical History Taking , Military Personnel , Physical Examination , Respiratory Function Tests , Tomography, X-Ray Computed/methods , Young AdultABSTRACT
A síndrome de Swyer James-Mc Load é uma entidade rara descrita pela primeira vez em 1953 sendo caracterizada por hipoplasia ou agenesia das artérias pulmonares, resultando em hiperlucência pulmonar unilateral. Embora a patogênese ainda não tenha sido completamente elucidada, acredita-se que seja uma complicação pós-infecciosa de bronquiolites ocorridas na infância. O objetivo deste estudo estudo foi analisar a melhor conduta terapêutica na síndrome de Swyer James-Mc Load e o impacto na qualidade de vida das pessoas acometidas. Relata-se dois casos desta síndrome diagnosticados em pacientes adultos, sendo que uma delas apresenta asma persistente grave e a outra é ex-tabagista e já realizou cirurgia para ressecção de um nódulo pulmonar no pulmão contralateral ao acometido. A terapêutica conservadora foi capaz de estabilizar a condição clínica dos pacientes, mantendo uma boa qualidade de vida. No entanto, pacientes que não se estabilizam com tal terapêutica provavelmente devem ser incluídos à intervenção invasiva, visto que fornece ao paciente melhora na qualidade e expectativa de vida. Uma breve revisão da literatura pertinente foi realizada para melhor compreensão do assunto...
The Swyer-James Mc Load syndrome is a rare entity first described in 1953 and is characterized by hypoplasia or agenesis of the pulmonary arteries, resulting in unilateral lung hyperlucency. Although the pathogenesis has not yet been fully elucidated, it is believed to be a post-infectious complication of bronchiolitis occurring in childhood. The objective of this article was analyzing the best treatment for SSJM and its impact on affected people's lives. We report two cases of this syndrome diagnosed in adult patients, one of whom has severe persistent asthma and the other is a former smoker and has undergone surgery for resection of a pulmonary nodule in the contralateral lung to the affected. We concluded that conservative therapy is able to stabilize the clinical condition of patients maintaining a good quality of life. However, patients who do not stabilize with such therapy are likely to be included in invasive intervention, since it improves the quality of life and life expectancy of the patient. A brief review of the literature was conducted to better understand the subject...
Subject(s)
Humans , Female , Adult , Middle Aged , Asthma , Solitary Pulmonary Nodule/diagnosis , Lung, Hyperlucent/diagnosis , Lung, Hyperlucent/physiopathology , Lung, Hyperlucent/therapy , SyndromeABSTRACT
A 21-year-old man with a history of bronchial asthma during childhood presented with left recurrent pneumothorax. Chest x-ray showed collapse of the left lung. Computed tomography showed hyperlucency and some bullae in the left upper lobe. Thoracoscopic bullectomy and pleurodesis were performed. Pneumothorax recurred twice, for which thoracoscopic bullectomy and pleurodesis were performed. During the third operation, pleurodesis of almost the entire left upper lobe was performed. Since this third operation, the patient has been free from recurrences of pneumothorax for three years. The lung perfusion scintigram performed during the absence of pneumothorax showed a lack of accumulation in the left upper lobe. The lung ventilation scintigram performed during the absence of pneumothorax showed low accumulation and delay of washout in the left upper lobe. These findings are compatible with Swyer-James syndrome. To prevent the recurrence of pneumothorax related to Swyer-James syndrome, some kind of procedure, such as extensive pleurodesis, is necessary.
Subject(s)
Lung, Hyperlucent/complications , Pneumothorax/etiology , Humans , Lung, Hyperlucent/diagnosis , Lung, Hyperlucent/therapy , Male , Perfusion Imaging , Pleurodesis , Pneumothorax/diagnosis , Pneumothorax/therapy , Predictive Value of Tests , Recurrence , Reoperation , Risk Factors , Thoracoscopy , Time Factors , Tomography, X-Ray Computed , Treatment Outcome , Young AdultSubject(s)
Chest Tubes , Choristoma/diagnosis , Diagnostic Errors , Drainage/instrumentation , Kidney , Lung, Hyperlucent/diagnosis , Pneumothorax/diagnosis , Pneumothorax/therapy , Choristoma/complications , Humans , Lung, Hyperlucent/complications , Lung, Hyperlucent/therapy , Male , Predictive Value of Tests , Thoracostomy , Tomography, X-Ray Computed , Unnecessary Procedures , Young AdultABSTRACT
Swyer-James-MacLeod syndrome is characterised by unilateral hyperlucency on chest radiograph with small or normal-sized lung on the affected side and compensatory hyperinflation of opposite lung. Hyperinflation of the affected lung is a very rarely reported entity. An adult female patient, who presented with exertional breathlessness and diagnosed to have hypoplastic left pulmonary artery with hyperlucent, hyperinflated and herniated left lung is described.
Subject(s)
Lung, Hyperlucent/diagnosis , Diagnosis, Differential , Diagnostic Imaging , Female , Humans , Lung, Hyperlucent/therapy , Young AdultSubject(s)
Diagnostic Errors , Lung, Hyperlucent/diagnosis , Anti-Bacterial Agents/therapeutic use , Asthma/diagnosis , Bronchodilator Agents/therapeutic use , Child , Diagnosis, Differential , Drug Therapy, Combination , Humans , Lung, Hyperlucent/therapy , Male , Physical Therapy Modalities , Treatment OutcomeSubject(s)
Hernia, Hiatal/complications , Lung, Hyperlucent/etiology , Pneumothorax/diagnosis , Pneumothorax/therapy , Respiratory Distress Syndrome/etiology , Drainage , Hernia, Hiatal/diagnosis , Hernia, Hiatal/therapy , Humans , Intubation, Gastrointestinal , Lung, Hyperlucent/diagnosis , Lung, Hyperlucent/therapy , Male , Middle Aged , Pneumothorax/complications , Respiratory Distress Syndrome/diagnosis , Respiratory Distress Syndrome/therapyABSTRACT
This case report describes a patient with Swyer-James-MacLeod Syndrome (SJMS) in an adult male diagnosed on the basis of findings on X-ray chest, high resolution CT (HRCT) of chest and radionuclide perfusion lung scan. This rare syndrome is considered to be an acquired disease due to repeated pneumonias in early childhood.
Subject(s)
Lung, Hyperlucent/diagnosis , Lung/diagnostic imaging , Adult , Anti-Bacterial Agents/administration & dosage , Bronchodilator Agents/administration & dosage , Humans , Influenza Vaccines , Lung, Hyperlucent/etiology , Lung, Hyperlucent/therapy , Male , Pneumococcal Vaccines , Pneumonia/complications , Prognosis , Radiographic Image Enhancement , Radiography, Thoracic , Radionuclide Imaging , Recurrence , Time Factors , Tomography, X-Ray ComputedABSTRACT
No disponible
Subject(s)
Female , Adult , Humans , Lung, Hyperlucent/diagnosis , Lung, Hyperlucent/therapy , Bronchitis/complications , Bronchiolitis Obliterans/complications , Radiography, Thoracic , Diagnosis, Differential , Ventilation-Perfusion Ratio , Ventilation-Perfusion Ratio/physiology , Spirometry/methods , Respiratory Tract Diseases/complications , Bronchography/methods , Angiography/methods , Angiography/trendsABSTRACT
As with other neurodegenerative disorders, research into the group of diseases known under the umbrella term of "neuroacanthocytosis" has greatly benefited from the identification of causative genes. The distinct and unifying aspect of these disorders is the presence of thorny deformations of circulating erythrocytes. This may be due to abnormal properties of red cell membranes, which could lead to insights into mechanisms of neurodegeneration. Research approaches in this field, in addition to examining functions and protein interactions of the affected proteins with particular respect to neurons, have also drawn upon the expertise of hematologists and red cell membrane biologists. In this article, recent developments in the field are presented.
Subject(s)
Chorea/physiopathology , Lung, Hyperlucent/physiopathology , Neurodegenerative Diseases/physiopathology , Animals , Chorea/genetics , Chorea/history , Chorea/pathology , Chorea/therapy , Disease Models, Animal , History, 20th Century , History, 21st Century , Humans , Lung, Hyperlucent/genetics , Lung, Hyperlucent/pathology , Lung, Hyperlucent/therapy , Magnetic Resonance Imaging/methods , Neurodegenerative Diseases/genetics , Neurodegenerative Diseases/history , Neurodegenerative Diseases/therapy , Vesicular Transport Proteins/geneticsSubject(s)
Lung, Hyperlucent/diagnostic imaging , Bronchoscopy , Cardiac Catheterization , Child , Diagnosis, Differential , Female , Follow-Up Studies , Humans , Lung Diseases/diagnostic imaging , Lung, Hyperlucent/diagnosis , Lung, Hyperlucent/therapy , Radiography, Thoracic , Respiratory Function Tests , Severity of Illness Index , Taiwan , Tomography, X-Ray ComputedABSTRACT
Os autores relataram dois casos de hipertransparência pulmonar unilateral,ou síndorme de Swyer-James-MacLeod,envolvendo o hemitórax direito e caracterizados por diminuição do murmúrio vesicular no lado afetado,hipoplasia da artéria pulmonar e akterações da árvore brônquica. Feita uma discussão das prováveis causas dessa entidade clínica e identificadas as outras doenças que podem simular a síndrome.O tratamento proposto nesses casos é conservador,reservando-se a cirurgia aos casos complicados por infecções intercurrentes ou hemorragia
