Reporte de caso: hemoptisis masiva como manifestación clínica de un síndrome de Swyer-James-Mac Leod / Case report: massive hemoptysis as a clinical manifestation of Swyer-James-Mac Leod syndrome

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.

Enfisema pulmonar congênito: relato de caso / Congenital pulmonary enphysema: case report

Introdução: O enfisema pulmonar congênito (EPC) é uma doença rara, possuindo uma incidência de 1:20-30 mil nascimentos, é mais comum no sexo masculino do que no feminino, em uma razão de 3:1 e sua etiologia permanece desconhecida. Um terço dos casos são sintomáticos ao nascer e praticamente todos são diagnosticados nos primeiros seis meses de vida. Relato de Caso: Recém-nascido (RN) do sexo masculino, evoluiu com desconforto respiratório precoce, sendo encaminhado ao centro de terapia intensiva. Após uso de continuous positive airway pressure (CPAP) e cateter nasal de oxigênio de 12 horas, resultou em bom padrão respiratório e boa saturação. Após realização de tomografia computadorizada, foi diagnosticado o EPC. Conclusão: O EPC é uma patologia rara e deve ser suspeitado em RN com desconforto respiratório, atribuindo-se importância aos vários diagnósticos diferenciais possíveis. Apesar da etiologia incerta, é de fácil diagnóstico e possui opções de manejo clínico e cirúrgico.

Introduction: Congenital pulmonary emphysema (EPC) is a rare disease, with an incidence of 1:20-30 thousand births, it is more common in males than in females, in a ratio of 3:1 and its etiology remains unknown. One third of the cases are symptomatic at birth and practically all are diagnosed in the first six months of life. Case Report: Newborn (NB) male, developed early respiratory distress, being referred to the intensive care unit. After using continuous positive airway pressure (CPAP) and a 12-hour oxygen nasal catheter, it resulted in a good breathing pattern and good saturation. After performing computed tomography, EPC was diagnosed. Conclusion: EPC is a rare pathology and should be suspected in newborns with respiratory distress, with importance being given to the various possible differential diagnoses. Despite its uncertain etiology, it is easy to diagnose and has options for clinical and surgical management.

Pulmonary Emphysema Impairs Male Reproductive Physiology Due To Testosterone and Oxidative Stress Imbalance in Mesocricetus auratus.

This study evaluated whether pulmonary emphysema affects sperm quality, male reproductive organs, and testosterone levels in adult male hamsters. Mesocricetus auratus males (130-150 g) were subdivided into a control group (C group) and an emphysema group (E group). The C group received an intratracheal instillation of saline solution (0.3 mL/100 g of body weight), and the E group received papain (40 mg/100 g of body weight). After 60 days, the biometric, pulmonary, and reproductive parameters of each group were evaluated. The E group developed pulmonary emphysema, which decreased body weight and sperm quality compared to the C group. In oxidative stress-related assays, lipid peroxidation was increased in the testis and epididymis (caput and cauda) in the E group compared with the C group. However, only the caput epididymis showed a reduction in glutathione levels. Pulmonary emphysema also affected the testicle by inducing an increase in abnormal seminiferous tubules, accompanied by a decrease in seminiferous epithelium height. Spermatogenesis kinetics were also modified by pulmonary emphysema. The number of Leydig and Sertoli cells decreased in the E group, accompanied by an increase in the nuclear volume of Leydig cells. Testosterone concentration was increased in the E group. Similarly, pulmonary emphysema altered epididymal components in all regions. In conclusion, pulmonary emphysema affected the reproductive system in this experimental model, as shown by testicular and epididymal morphophysiology changes, hormonal alteration, and oxidative stress imbalance, inducing the loss of correct function in the male reproductive system.

Uncovering the mechanisms of exertional dyspnoea in combined pulmonary fibrosis and emphysema.

The prevailing view is that exertional dyspnoea in patients with combined idiopathic pulmonary fibrosis (IPF) and emphysema (CPFE) can be largely explained by severe hypoxaemia. However, there is little evidence to support these assumptions.We prospectively contrasted the sensory and physiological responses to exercise in 42 CPFE and 16 IPF patients matched by the severity of exertional hypoxaemia. Emphysema and pulmonary fibrosis were quantified using computed tomography. Inspiratory constraints were assessed in a constant work rate test: capillary blood gases were obtained in a subset of patients.CPFE patients had lower exercise capacity despite less extensive fibrosis compared to IPF (p=0.004 and 0.02, respectively). Exertional dyspnoea was the key limiting symptom in 24 CPFE patients who showed significantly lower transfer factor, arterial carbon dioxide tension and ventilatory efficiency (higher minute ventilation (V'E)/carbon dioxide output (V'CO2 ) ratio) compared to those with less dyspnoea. However, there were no between-group differences in the likelihood of pulmonary hypertension by echocardiography (p=0.44). High dead space/tidal volume ratio, low capillary carbon dioxide tension emphysema severity (including admixed emphysema) and traction bronchiectasis were related to a high V'E/V'CO2 ratio in the more dyspnoeic group. V'E/V'CO2 nadir >50 (OR 9.43, 95% CI 5.28-13.6; p=0.0001) and total emphysema extent >15% (2.25, 1.28-3.54; p=0.01) predicted a high dyspnoea burden associated with severely reduced exercise capacity in CPFEContrary to current understanding, hypoxaemia per se is not the main determinant of exertional dyspnoea in CPFE. Poor ventilatory efficiency due to increased "wasted" ventilation in emphysematous areas and hyperventilation holds a key mechanistic role that deserves therapeutic attention.

Emphysema induced by elastase alters the mRNA relative levels from DNA repair genes in acute lung injury in response to sepsis induced by lipopolysaccharide administration in Wistar rats.

Purpose/Aim of the study: Patients suffering from chronic obstructive pulmonary disease (COPD) in association with acute respiratory distress syndrome (ARDS) present oxidative stress in lung cells, with production of free radicals and DNA lesions in pulmonary and adjacent cells. Once the DNA molecule is damaged, a set of enzymatic mechanisms are trigged to preserve genetic code integrity and cellular homeostasis. These enzymatic mechanisms include the base and the nucleotide excision repair pathways, as well as telomere regulation. Thus, the aim of this work was to evaluate the mRNA levels from APEX1, ERCC2, TP53, and TRF2 genes in lung tissue from Wistar rats affected by acute lung injury in response to sepsis and emphysema. MATERIALS AND METHODS: Adult male Wistar rats were randomized into 4 groups (n = 6, for each group): control, emphysema, sepsis, and emphysema with sepsis. Pulmonary emphysema was induced by intratracheal instillation of elastase (12 IU/animal) and sepsis induced by intraperitoneal Escherichia coli lipopolysaccharide (LPS) injection (10 mg/kg). Lungs were removed, and samples were withdrawn for histological analysis and total RNA extraction, cDNA synthesis, and mRNA level evaluation by real time quantitative polymerase chain reaction. RESULTS: Data show acute lung injury by LPS and emphysema by elastase and that APEX1, ERCC2, TP53, and TRF2 mRNA levels are increased significantly (p < 0.01) in emphysema with sepsis group. CONCLUSION: Our results suggest that alteration in mRNA levels from DNA repair and genomic stability could be part of cell response to acute lung injury in response to emphysema and sepsis.

Effects of the association of diabetes and pulmonary emphysema on cardiac structure and function in rats.

Chronic obstructive pulmonary disease is often associated with chronic comorbid conditions of cardiovascular disease, diabetes mellitus and hypertension. This study aimed to investigate the effects of the association of diabetes and pulmonary emphysema on cardiac structure and function in rats. Wistar rats were divided into control non-diabetic instilled with saline (CS) or elastase (CE), diabetic instilled with saline (DS) or elastase (DE), DE treated with insulin (DEI) groups and echocardiographic measurements, morphometric analyses of the heart and lungs, and survival analysis conducted 50 days after instillation. Diabetes mellitus was induced [alloxan, 42 mg/kg, intravenously (iv)] 10 days before the induction of emphysema (elastase, 0.25 IU/100 g). Rats were treated with NPH insulin (4 IU before elastase plus 2 IU/day, 50 days). Both CE and DE exhibited similar increases in mean alveolar diameter, which are positively correlated with increases in right ventricular (RV) wall thickness (P = 0.0022), cavity area (P = 0.0001) and cardiomyocyte thickness (P = 0.0001). Diabetic saline group demonstrated a reduction in left ventricular (LV) wall, interventricular (IV) septum, cardiomyocyte thickness and an increase in cavity area, associated with a reduction in LV fractional shortening (P < 0.05), and an increase in LViv relaxation time (P < 0.05). Survival rate decreased from 80% in DS group to 40% in DE group. In conclusion, alloxan diabetes did not affect RV hypertrophy secondary to chronic emphysema, even in the presence of insulin. Diabetes per se induced left ventricular dysfunction, which was less evident in the presence of RV hypertrophy. Survival rate was substantially reduced as a consequence, at least in part, of the coexistence of RV hypertrophy and diabetic cardiomyopathy.

Combined pulmonary fibrosis and emphysema: an increasingly recognized condition.

Combined pulmonary fibrosis and emphysema (CPFE) has been increasingly recognized in the literature. Patients with CPFE are usually heavy smokers or former smokers with concomitant lower lobe fibrosis and upper lobe emphysema on chest HRCT scans. They commonly present with severe breathlessness and low DLCO, despite spirometry showing relatively preserved lung volumes. Moderate to severe pulmonary arterial hypertension is common in such patients, who are also at an increased risk of developing lung cancer. Unfortunately, there is currently no effective treatment for CPFE. In this review, we discuss the current knowledge of the pathogenesis, clinical characteristics, and prognostic factors of CPFE. Given that most of the published data on CPFE are based on retrospective analysis, more studies are needed in order to address the role of emphysema and its subtypes; the progression of fibrosis/emphysema and its correlation with inflammation; treatment options; and prognosis.

Combined pulmonary fibrosis and emphysema: an increasingly recognized condition / Combinação de fibrose pulmonar e enfisema: uma doença cada vez mais reconhecida

Combined pulmonary fibrosis and emphysema (CPFE) has been increasingly recognized in the literature. Patients with CPFE are usually heavy smokers or former smokers with concomitant lower lobe fibrosis and upper lobe emphysema on chest HRCT scans. They commonly present with severe breathlessness and low DLCO, despite spirometry showing relatively preserved lung volumes. Moderate to severe pulmonary arterial hypertension is common in such patients, who are also at an increased risk of developing lung cancer. Unfortunately, there is currently no effective treatment for CPFE. In this review, we discuss the current knowledge of the pathogenesis, clinical characteristics, and prognostic factors of CPFE. Given that most of the published data on CPFE are based on retrospective analysis, more studies are needed in order to address the role of emphysema and its subtypes; the progression of fibrosis/emphysema and its correlation with inflammation; treatment options; and prognosis.

A combinação de fibrose pulmonar e enfisema (CFPE) é cada vez mais reconhecida na literatura. Os pacientes são geralmente fumantes pesados ou ex-fumantes nos quais a TCAR de tórax revela enfisema nos lobos superiores e, concomitantemente, fibrose nos lobos inferiores. Esses pacientes comumente apresentam dispneia grave e baixa DLCO, não obstante os volumes pulmonares relativamente preservados em exames espirométricos. Hipertensão arterial pulmonar de moderada a grave e aumento da incidência de câncer de pulmão também são comuns nesses pacientes. Infelizmente, ainda não existe um tratamento eficaz para a CFPE. O objetivo desta revisão é discutir o que se sabe atualmente a respeito da patogênese, das características clínicas e dos fatores prognósticos da CFPE. Como a maioria dos dados publicados baseia-se em análise retrospectiva, são necessários mais estudos sobre o papel do enfisema e seus subtipos, a progressão da fibrose/enfisema e sua correlação com a inflamação, as opções de tratamento e o prognóstico em pacientes com CFPE.

Lung injury-dependent oxidative status and chymotrypsin-like activity of skeletal muscles in hamsters with experimental emphysema.

BACKGROUND: Peripheral skeletal muscle is altered in patients suffering from emphysema and chronic obstructive pulmonary disease (COPD). Oxidative stress have been demonstrated to participate on skeletal muscle loss of several states, including disuse atrophy, mechanical ventilation, and chronic diseases. No evidences have demonstrated the occurance in a severity manner. METHODS: We evaluated body weight, muscle loss, oxidative stress, and chymotrypsin-like proteolytic activity in the gastrocnemius muscle of emphysemic hamsters. The experimental animals had 2 different severities of lung damage from experimental emphysema induced by 20 mg/mL (E20) and 40 mg/mL (E40) papain. RESULTS: The severity of emphysema increased significantly in E20 (60.52 ± 2.8, p < 0.05) and E40 (52.27 ± 4.7; crossed the alveolar intercepts) groups. As compared to the control group, there was a reduction on body (171.6 ± 15.9 g) and muscle weight (251.87 ± 24.87 mg) in the E20 group (157.5 ± 10.3 mg and 230.12 ± 23.52 mg, for body and muscle weight, respectively), which was accentuated in the E40 group (137.4 ± 7.2 g and 197.87 ± 10.49 mg, for body and muscle weight, respectively). Additionally, the thiobarbituric acid reactive substances (TBARS), tert-butyl hydroperoxide-initiated chemiluminescence (CL), carbonylated proteins, and chymotrypsin-like proteolytic activity were elevated in the E40 group as compared to the E20 group (p < 0.05 for all comparisons). The severity of emphysema significantly correlated with the progressive increase in CL (r = -0.95), TBARS (r = -0.98), carbonyl proteins (r = -0.99), and chymotrypsin-like proteolytic activity (r = -0.90). Furthermore, augmentation of proteolytic activity correlated significantly with CL (r = 0.97), TBARS (r = 0.96), and carbonyl proteins (r = 0.91). CONCLUSIONS: Taken together, the results of the present study suggest that muscle atrophy observed in this model of emphysema is mediated by increased muscle chymotrypsin-like activity, with possible involvement of oxidative stress in a severity-dependent manner.

Neumotórax y bulla enfisematosa gigante rota como manifestación inicial de un carcinoma / Pneumothorax and ruptured giant emphysematous bulla as a manifestation of carcinoma

Se presenta el caso de un paciente de 64 años de edad que acudió al cuerpo de guardia con dolor torácico agudo localizado en hemitórax derecho y disnea intensa. La persistencia del neumotórax sin que se lograra la reexpansión pulmonar con la pleurotomía y el drenaje torácico obligó a una toracotomía. Se encontró una bulla enfisematosa gigante rota, y se realizó una bilobectomía superior y media. El diagnóstico histológico fue carcinoma bronquioalveolar. La coincidencia de un cáncer de pulmón en una bulla enfisematosa no es excepcional, pero la aparición y persistencia de un neumotórax como manifestación inicial de un cáncer de pulmón es poco frecuente(AU)

This is the case of a patient aged 64 seen in the emergency room due to a acute thorax pain located in right hemithorax and intense dyspnea. The persistence of pneumothorax without to achieve a pulmonary re-expansion with a pleurotomy and thoracic drainage be necessary a thoracotomy. A ruptured giant emphysematous bulla was found thus the superior and middle bilobectomy. The histological diagnosis was a bronchoalveolar carcinoma. Coincidence of a lung cancer in a emphysematous bulla isn't exceptional and the persistence of pneumothorax as initial manifestation of lung cancer is not frequent(AU)

Persistence of ventilatory defect after resolution of pulmonary interstitial emphysema in a preterm baby.

Pulmonary interstitial emphysema is a common complication of mechanical ventilation in preterm babies. We report a case of severe unilateral pulmonary interstitial emphysema in a premature newborn, treated with high-frequency oscillatory ventilation, lateral decubitus positioning and selective intubation. After complete radiological resolution of the pulmonary emphysema in the left lung, the patient was studied by electrical impedance tomography and a marked reduction of ventilation was identified in the left lung despite radiological resolution of the cysts. This finding indicates that functional abnormalities may persist for longer periods after radiologic resolution of such lesions.

[Functional respiratory evolution in two patients with emphysema and pulmonary fibrosis]. / Evolución funcional respiratoria en dos pacientes con enfisema y fibrosis pulmonar.

Combined pulmonary fibrosis and emphysema (CPFE) is a frequently under-diagnosed condition. Isolated pulmonary function tests (PFT) can give rise to misinterpretations. We have found no reports on these patients' spirometric progression. We describe two cases of CPFE, showing long-term functional evolution to have a more accurate understanding of current spirometric values. The most relevant findings are: 1) spirometry with discrete functional alterations in the presence of a marked dyspnea and the need, in one patient, for chronic oxygen therapy; and 2) functional evolution reflecting "pseudonormalisation" of the initial obstructive spirometric pattern, possibly as a result of fibrosis development. A mild obstructive defect in a patient with chronic airflow limitation and marked impairment of his/her clinical status and functional class should alert on the possibility of associated pulmonary fibrosis. A computed tomography (CT) and previous PFTs will allow a better understanding of this condition.