Síndrome incompleto de Carney / Incomplete Carney's triad

El síndrome de Carney es una enfermedad rara, con diferentes formas de presentación, que afecta principalmente al sexo femenino y es asintomática en la mayoría de los casos. Se caracteriza por la aparición de tumores, generalmente benignos, principalmente en el pulmón, la glándula suprarrenal y el estómago; sin embargo, se pueden afectar otros órganos como el esófago. Su tratamiento es principalmente quirúrgico. Se presenta el caso de una paciente con síndrome incompleto de Carney, manejada quirúrgicamente, con excelente resultado posoperatorio. Se hace una revisión de la literatura científica actual.

Carney's triad is a rare condition with multimodal presentations, which affects mainly females and remains asymptomatic in most cases. It is characterized by the appearance of tumors, usually benign, in lung, adrenal, and stomach, but it can also affect other organs like the esophagus. Treatment of these lesions is primarily surgical. The objective of this paper is to review the current literature and to report a case of an incomplete Carney's triad managed surgically with excellent postoperative results.

Peritoneal free autologous fat graft for the control of pulmonary air leaks in emphysematous rat lungs.

BACKGROUND: Persistent pulmonary air leak is the most frequent complication after lung resection, resulting in an increase in postoperative morbidity and mortality. We evaluated the viability, integration, and efficacy of a free peritoneal fat graft as a method for controlling air leak in normal and emphysematous rat lungs. METHODS: Sixty Wistar rats were divided into two groups: elastase-produced lung emphysema (n=30) and control (normal) lungs (n=30). Pulmonary air leak was produced by puncture of the right lower lobe, and aerostasis was attempted by means of intrapulmonary injection of autologous free peritoneal fat graft. Rats in each group (n=6) were randomly allocated to subgroups and were sacrificed at 7, 14, 21, 30, and 60 days. Then, lungs were removed for histology, morphometry, vessel identification and counting, and immunohistochemistry for caspase 3, vascular endothelial growth factor, and factor VIII. RESULTS: Tissue integration of the free fat grafts was found in all animals in both groups. Vessels stained with India ink inside the fat grafts were present at all assessment periods in both groups. Vascular endothelial growth factor expression was significantly higher in all periods in the emphysema group compared with normal lungs (p<0.001). There was a significant increase in caspase 3 expression in the emphysema group at 7, 21, 30, and 60 days (p<0.001). Factor VIII showed a significant increase (p<0.001) at 30 and 60 days in emphysematous lungs. CONCLUSIONS: The use of free peritoneal fat graft was able to control the air leaks in normal and emphysematous rat lungs, with persisting graft viability for as long as 60 days after implantation.

Effect of positive end-expiratory pressure after porcine unilateral left lung transplant.

OBJECTIVES: To evaluate the effects of 2 different levels of positive end-expiratory pressure on pigs who had unilateral lung transplants. MATERIALS AND METHODS: A left lung transplant was performed in 12 pigs. The animals were randomized into 2 groups based on positive end-expiratory pressure: group 1 (5 cm H(2)O) and group 2 (10 cm H(2)O). Hemodynamics, gas exchange, and respiratory mechanics were measured before and after surgery. Cytokines, oxidative stress, and histologic scores were assessed in the lung tissue of each pig. RESULTS: Pigs in group 2 exhibited a significantly higher mean heart rate (P = .006), static compliance (P = .001), lower mean arterial pressure (P = .003), and airway resistance (P = .001) than did pigs in group 1. There were no postoperative differences between the groups in concentrations of thiobarbituric acid reactive substances, superoxide dismutase, and interleukin 8. At the end of the observation period, pigs in group 2 had higher levels of thiobarbituric acid reactive substances (P = .001) and interleukin 8 (P = .05), and pigs in group 1 had higher levels of superoxide dismutase (P = .05) than they did at baseline. CONCLUSIONS: After unilateral lung transplant, higher positive end-expiratory pressure was associated with improved respiratory mechanics, a negative effect on hemodynamics, a stronger inflammatory response, and increased production of reactive oxygen species, but no effect on gas exchange.

When is injury potentially reversible in a lung ischemia-reperfusion model?

OBJECTIVE: To verify the impact of ischemic time on lung cell viability in an experimental model of lung ischemia-reperfusion (IR) injury and its repercussion on lung performance after reperfusion. METHODS: Twenty-four animals were subjected to selective clamping of the left pulmonary artery and divided into four groups (n = 6) according to ischemic time: 15 (IR15), 30 (IR30), 45 (IR45), and 60 min (IR60). All animals were observed for 120 min after reperfusion. The hemodynamics, arterial blood gases measurements, and histologic changes were analyzed. Immunofluorescence assays for caspase 3 and annexin V were performed. Lipid peroxidation was assessed by thiobarbituric acid-reactive substances, and caspase 3 activity was assessed by colorimetric extract. RESULTS: The partial pressure of arterial oxygen significantly decreased at the end of the observation period in the IR30, IR45, and IR60 groups (P < 0.05). The final mean arterial pressure significantly decreased in the IR60 group (P < 0.05). We observed a significant increase in caspase 3 activity and caspase 3-positive cells by immunofluorescence in the IR45 group compared with the other groups (P < 0.05). Additionally, there was an increase in necrotic cells assessed by annexin V in the IR60 group. The histologic score did not show differences among the different groups. CONCLUSIONS: The degree of cell damage had a negative impact on lung performance. Sixty minutes of lung ischemia and posterior reperfusion resulted in an increased number of necrotic cells, suggesting that these cells may not be able to reverse the effects of the IR injury because of the lack of viable cells.

Ischemic preconditioning by selective occlusion of the pulmonary artery in rats.

OBJECTIVE: To evaluate the effect of lung ischemic preconditioning (IPC) on normothermic ischemia/reperfusion (I/R) injury in a rat model, quantifying the production of reactive oxygen species. METHODS: Forty-seven male Wistar rats were randomized into four groups: control, sham, I/R and IPC. Control group animals were anesthetized and killed by decapitation, after which pneumonectomy was performed and the left lungs were stored in liquid nitrogen. Sham, IPC and I/R group rats were anesthetized, tracheostomized, ventilated, anticoagulated and submitted to left thoracotomy with dissection of the left pulmonary artery for clamping. Sham group rats underwent dissection of the left pulmonary artery, I/R group rats underwent 30 min of total hilar clamping, and IPC group rats underwent 5-min clamping of the left pulmonary artery followed by 30 min of total hilar clamping. Lungs were reperfused for 90 min and ventilated with the same parameters, with additional positive end-expiratory pressure of 1 cmH2O. Hemodynamic and blood gas values were obtained prior to thoracotomy, prior to total hilar clamping, after 30 min of reperfusion and after 90 min of reperfusion. Lipid peroxidation was determined by measuring levels of thiobarbituric acid reactive substances. RESULTS: There were no significant differences among the groups in terms of the levels of thiobarbituric acid reactive substances. Nor were there any significant differences among the sham, I/R and IPC groups in terms of arterial oxygen tension, arterial carbon dioxide tension or hemodynamic values. CONCLUSIONS: In an in situ I/R rat model, 5-min IPC of the left pulmonary artery does not attenuate I/R injury.

The effects of positive end-expiratory pressure in alveolar recruitment during mechanical ventilation in pigs / Os efeitos da pressão positiva expiratória final no recrutamento alveolar durante a ventilação mecânica em porcos

PURPOSE: To evaluate the effects of alveolar recruitment based on mean airway pressure (MAP) on pig lungs submitted to thoracotomy through blood gas exchange and hemodynamic parameters. METHODS: Twelve pigs weighting approximately 25Kg were intubated and ventilated on volume controlled ventilation (tidal volume 10ml/Kg, respiratory rate 16min, FiO2 1.0, inspiratory:expiratory ratio 1:2, PEEP 5cmH2O). The animals were then randomized into two groups: control and left lateral thoracotomy. The PEEP was increased at each 15-minute intervals to reach a MAP of 15, 20 and 25cmH2O, respectively. Hemodynamic, gas exchange and respiratory mechanic data were measured immediately before each PEEP change. RESULTS: There were no significant differences between both groups in all parameters analyzed (P=1.0). The PaO2, PaCO2, MAP, PAP and plateau pressure were significantly worse at MAP of 25cmH2O, when compared with the other values of MAP (P=0.001, P=0.039, P=0.001, P=0.016 e P=0.027, respectively). The best pulmonary performance according to the analyzed parameters was observed at MAP of 20cmH2O. CONCLUSION: PEEP adjusted to MAP of 20cmH2O resulted in best arterial oxygenation, without compromising the venous return, as opposed to MAP of 25cmH2O, which caused deterioration of gas exchange, hemodynamics and respiratory mechanic.

OBJETIVO: Avaliar os efeitos do recrutamento alveolar baseado na pressão média das vias aéreas (Pmédia) sobre os pulmões de porcos submetidos a toracotomia, através de dados gasométricos e hemodinâmicos. MÉTODOS: Doze porcos machos pesando em média 25Kg receberam indução anestésica, sendo intubados e ventilados a volume (FiO2=1.0, volume corrente=10 ml/kg/min, FR=16 cpm, relação I:E=1:2 e PEEP 5 mmHg). Os animais foram randomizados em dois grupos: controle e toracotomia lateral esquerda. Os valores de PEEP em cada grupo foram aumentados a cada 15 minutos para atingir valores de Pmédia de 15, 20 e 25cmH2O, sendo coletados dados hemodinâmicos, gasometria arterial e mecânica respiratória imediatamente antes de cada acréscimo do PEEP. RESULTADOS: Não houve diferença estatisticamente significativa entre os 2 grupos em todos os parâmetros analisados (P=1,0). Ocorreu piora significativa da PaO2, PaCO2, pressão arterial média, pressão da artéria pulmonar e pressão de platô com Pmédia de 25cmH2O comparado com os demais valores de Pmédia (P=0.001, P=0.039, P=0.001, P=0.016 e P=0.027, respectivamente). O melhor desempenho pulmonar pelos parâmetros analisados ocorreu com a Pmédia de 20cmH2O. CONCLUSÃO: As manobras de recrutamento alveolar alteram o desempenho pulmonar e função hemodinâmica independemente da cavidade torácica estar aberta ou fechada. O PEEP ideal para o recrutamento alveolar independente dos grupos estudados foi obtido a uma Pmédia de 20cmH2O em relação a todos os parâmetros analisados.

Pré-condicionamento isquêmico por oclusão seletiva da artéria pulmonar em ratos / Ischemic preconditioning by selective occlusion of the pulmonary artery in rats

OBJETIVO: Avaliar o efeito do pré-condicionamento isquêmico (PCI) em modelo de isquemia e reperfusão (I/R) pulmonar normotérmica em ratos, quantificando a produção de espécies reativas do oxigênio. MÉTODOS: Quarenta e sete ratos Wistar foram randomizados em quatro grupos: controle, sham, I/R e PCI. Após anestesia, animais do grupo controle foram sacrificados por decapitação, pneumonectomizados, e os pulmões esquerdos armazenados em nitrogênio líquido. Animais dos grupos sham, I/R e PCI foram anestesiados, traqueostomizados, ventilados, anticoagulados e submetidos a uma toracotomia esquerda com dissecção da artéria pulmonar esquerda para clampeamento. No grupo sham procedeu-se a dissecção da artéria pulmonar esquerda; no grupo I/R, clampeamento hilar total de 30 min e no grupo PCI, clampeamento da artéria pulmonar esquerda por 5 min seguido por reperfusão de 10 min e um clampeamento hilar total de 30 min. Pulmões foram reperfundidos por 90 min e ventilados com os mesmos parâmetros, acrescidos de pressão expiratória final positiva de 1 cmH2O. Foram obtidas medidas hemodinâmicas e gasométricas antes da toracotomia, antes do clampeamento hilar total, aos 30 e 90 min de reperfusão. A peroxidação lipídica foi estabelecida por meio da determinação das substâncias reativas ao ácido tiobarbitúrico. RESULTADOS: A determinação das substâncias reativas ao ácido tiobarbitúrico analisada nos grupos controle, sham, I/R, PCI não revelou diferenças significativas, o mesmo ocorrendo com a pressão parcial arterial de oxigênio, pressão parcial arterial de gás carbônico e medidas hemodinâmicas entre os grupos sham, I/R e PCI. CONCLUSÕES: O PCI de 5 min da artéria pulmonar esquerda em modelo de I/R in situ em ratos não atenua a lesão de I/R.

OBJECTIVE: To evaluate the effect of lung ischemic preconditioning (IPC) on normothermic ischemia/reperfusion (I/R) injury in a rat model, quantifying the production of reactive oxygen species. METHODS: Forty-seven male Wistar rats were randomized into four groups: control, sham, I/R and IPC. Control group animals were anesthetized and killed by decapitation, after which pneumonectomy was performed and the left lungs were stored in liquid nitrogen. Sham, IPC and I/R group rats were anesthetized, tracheostomized, ventilated, anticoagulated and submitted to left thoracotomy with dissection of the left pulmonary artery for clamping. Sham group rats underwent dissection of the left pulmonary artery, I/R group rats underwent 30 min of total hilar clamping, and IPC group rats underwent 5-min clamping of the left pulmonary artery followed by 30 min of total hilar clamping. Lungs were reperfused for 90 min and ventilated with the same parameters, with additional positive end-expiratory pressure of 1 cmH2O. Hemodynamic and blood gas values were obtained prior to thoracotomy, prior to total hilar clamping, after 30 min of reperfusion and after 90 min of reperfusion. Lipid peroxidation was determined by measuring levels of thiobarbituric acid reactive substances. RESULTS: There were no significant differences among the groups in terms of the levels of thiobarbituric acid reactive substances. Nor were there any significant differences among the sham, I/R and IPC groups in terms of arterial oxygen tension, arterial carbon dioxide tension or hemodynamic values. CONCLUSIONS: In an in situ I/R rat model, 5-min IPC of the left pulmonary artery does not attenuate I/R injury.

The effects of positive end-expiratory pressure in alveolar recruitment during mechanical ventilation in pigs.

PURPOSE: To evaluate the effects of alveolar recruitment based on mean airway pressure (MAP) on pig lungs submitted to thoracotomy through blood gas exchange and hemodynamic parameters. METHODS: Twelve pigs weighting approximately 25 Kg were intubated and ventilated on volume controlled ventilation (tidal volume 10 ml/Kg, respiratory rate 16 min, FiO2 1.0, inspiratory:expiratory ratio 1:2, PEEP 5 cmH2O). The animals were then randomized into two groups: control and left lateral thoracotomy. The PEEP was increased at each 15-minute intervals to reach a MAP of 15, 20 and 25 cmH2O, respectively. Hemodynamic, gas exchange and respiratory mechanic data were measured immediately before each PEEP change. RESULTS: There were no significant differences between both groups in all parameters analyzed (P=1.0). The PaO2, PaCO2, MAP, PAP and plateau pressure were significantly worse at MAP of 25 cmH2O, when compared with the other values of MAP (P=0.001, P=0.039, P=0.001, P=0.016 e P=0.027, respectively). The best pulmonary performance according to the analyzed parameters was observed at MAP of 20 cmH2O. CONCLUSION: PEEP adjusted to MAP of 20 cmH2O resulted in best arterial oxygenation, without compromising the venous return, as opposed to MAP of 25 cmH2O, which caused deterioration of gas exchange, hemodynamics and respiratory mechanic.