tratamento quer do quilotórax congénito quer. do secundário a quilotórax pós- cirúrgico entre Janeiro de interpessoais no manejo terapêutico. Epublication , Nov ;15(11). URL: vd01pt Ask a question to the author. Ask a question to the author. You must be. El presente estudio analizó la efectividad del manejo conservador, incluyendo pleurodesis, del quilotórax como complicación de la resección.
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Lymphangioleiomyomatosis LAM is a rare disease that is currently considered a low-grade neoplasm with metastatic potential and variable progression. Mammalian target of rapamycin mTOR inhibitors, such as sirolimus and everolimus, have recently become a treatment option for LAM patients, especially those with extrapulmonary manifestations.
We describe four cases of LAM patients with different extrapulmonary manifestations who were treated with sirolimus. After treatment with sirolimus for 12 months, one patient presented resolution of severe chylothorax; one had a significant reduction in renal angiomyolipoma maneuo and one showed significant regression of retroperitoneal lymphangioleiomyomas and abdominal lymph node enlargement.
After treatment with sirolimus for 6 months, the remaining patient had a significant reduction in the volume of a massive retroperitoneal lymphangioleiomyoma. Our findings confirm that mTOR inhibitors are beneficial for patients with LAM, especially those with extrapulmonary manifestations, such as renal angiomyolipoma, lymphangioleiomyomas, and chylous effusions. However, certain aspects, such as the optimal dose, duration of treatment, and long-term adverse effects, have yet to be sufficiently clarified for mTOR inhibitors to be incorporated into LAM management protocols.
Lymphangioleiomyomatosis LAM is a rare disease of unknown etiology. It can occur in isolation or in association with tuberous sclerosis complex TSC. Major clinical manifestations of LAM include progressive dyspnea on exertion, dry cough, recurrent spontaneous pneumothorax, chylothorax, and hemoptysis.
A CT scan of the chest typically shows the presence of diffuse, well-defined, thin-walled lung cysts. Although pulmonary function test results can be normal, variable and progressive lung function decline can occur over time. A definitive diagnosis of LAM is based on the following 3 Determination of serum levels of VEGF-D, which is a marker of lymphangiogenesis, has been increasingly used in order to aid in the diagnosis of LAM and to quilototax disease progression.
The progression of LAM is highly variable, ranging from asymptomatic patients to patients with progressive respiratory failure requiring lung transplantation. Various maneji have been used in an attempt to control LAM. Matrix metalloproteinase inhibitors, such as doxycycline, and hormonal blockade have yielded controversial results and are not recommended for the treatment of LAM.
The objective of the present study was to describe a case series of patients with LAM in Brazil treated with sirolimus, which had positive effects, particularly on extrapulmonary manifestations.
Of a total of 10 patients with LAM treated with sirolimus ,anejo our center during the study period, we describe four cases of patients in whom the drug was primarily used because of extrapulmonary involvement.
A year-old smoker with a smoking history of 15 pack-years was admitted with a two-year history of dyspnea, which had worsened one week prior. She reported no other diseases. The patient reported that she had undergone oophorectomy nine years prior because of a benign tumor and right nephrectomy six months prior because of renal angiomyolipoma.
She had no relevant exposure history. A chest X-ray showed a large right pleural effusion Figure 1. The patient underwent thoracentesis, and a diagnosis of chylothorax was established. A pulmonary function test performed after chest tube drainage showed an FVC of 3. A chest CT scan showed diffuse, well-defined lung cysts. Her dyspnea worsened, and she quiootorax with difficult-to-manage chylothorax, with a high output from the chest tube despite treatment with total parenteral nutrition.
Resolution of the chylothorax and functional stability were observed after 12 months of treatment Figure 1 and Table 1. A year-old patient diagnosed with LAM and TSC presented with a two-year history of recurrent pneumothorax and pleurodesis. She reported no smoking and had no relevant exposure history. Because of the neurological qiilotorax associated with TSC, the patient had frequent convulsive seizures and a cognitive deficit.
Manejo toracoscópico de quilotórax após esofagectomia
She also had large bilateral renal angiomyolipomas and had previously undergone a right partial nephrectomy because of quilotodax. She had been using goserelin and doxycycline sinceand the renal tumor had progressively increased in the last two years.
After 12 months of treatment, there was a significant reduction in the size of the angiomyolipoma from approximately 20 cm, with confluent masses, to approximately 3 cm; Figure 2.
Because of her cognitive deficit, the patient did not undergo pulmonary function testing. A year-old nonsmoker with a three-year history of dyspnea on exertion presented with acute worsening of dyspnea.
Although a large chylothorax was identified init was not investigated at the time. One year later, the patient was admitted for investigation. Chest and abdominal CT scans showed diffuse cysts in the lung parenchyma, as maneio as large retroperitoneal lymphangioleiomyomas and abdominal lymph node enlargement.
Manejo nutricional e digestibilidade no quilotórax canino
A diagnosis of LAM was established, and initial pulmonary function test results were as follows: A year-old manej presented with diffuse abdominal pain.
The patient had no respiratory symptoms, relevant exposure history, or associated diseases.
During the investigation, a large, heterogeneous retroperitoneal mass of approximately 18 cm in size was identified. A biopsy was performed, maneno the mass maneejo diagnosed as quilohorax lymphangioleiomyoma. A chest CT scan showed diffuse cysts in the lung parenchyma, whereas pulmonary function test results, including DLCO, were normal Quipotorax 1.
After 6 months of treatment with the drug, there was a significant reduction in qhilotorax volume Figure 4. On the basis of the various mechanisms involved in the pathophysiology of LAM, several drugs have been used in an attempt to control the disease. Although doxycycline blocks metalloproteinases, it is currently not recommended, because a recent randomized study showed that it had no effect on lung function. Inhibitors of mTOR inhibit T-lymphocyte activation and proliferation in response to antigenic and cytokine stimulation, binding to the FKBP protein to form an immunosuppressive complex.
This complex binds to mTOR, an important regulatory kinase, inhibiting its activity and ultimately blocking cell proliferation. Recent evidence has shown that mTOR inhibitors are promising options for patients with LAM and can be used in cases of large renal angiomyolipomas and lymphangioleiomyomas, as well as in the control of chylothorax and chylous ascites; in addition, mTOR inhibitors have beneficial effects on lung function, particularly in patients with a rapid decline in respiratory capacity and mild to moderate airflow limitation, 2022 as observed in our case series.
Although the use of sirolimus in patients with LAM is promising, certain issues have yet to be clarified, including the need for determining serum levels of the drug as is done in kidney or lung transplant recipients, for exampletreatment duration, and long-term treatment safety.
Recent studies have shown that it is possible to use sirolimus at lower doses in quilotoraz to reduce adverse effects without compromising treatment efficacy. A study by Yao et al. Inhibitors of mTOR have auilotorax an important treatment option for selected cases of patients with LAM, especially those with significant extrapulmonary manifestations, such as renal angiomyolipomas, lymphangioleiomyomas, and chylous effusions. Manejoo, certain issues such manrjo the optimal dose and duration of treatment have yet to be investigated in prospective studies for mTOR inhibitors to be permanently incorporated into LAM management protocols.
De um total de 10 pacientes com LAM em uso de sirolimo em nosso centro durante o estudo, descrevemos quatro casos de quilotogax nos quais o medicamento foi utilizado fundamentalmente em virtude do acometimento extrapulmonar.
A paciente relatou que fora submetida a ooforectomia havia nove anos em virtude mmanejo um tumor benigno e a nefrectomia direita havia 6 meses em virtude de angiomiolipoma renal.
Um estudo realizado por Yao et al. National Center for Biotechnology InformationU. Journal List J Bras Pneumol v. Bruno Guedes Baldi 2. Glaucia Itamaro Heiden 1. Ronaldo Adib Kairalla 3. Carlos Roberto Ribeiro Carvalho 4. Author information Article notes Copyright and License information Disclaimer. Received Jan 13; Accepted Apr 8. This is an Open Access article distributed under the terms of the Creative Commons Attribution Quiloorax License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided maneejo original work is properly cited.
This article has been cited by other articles in PMC. Introduction Lymphangioleiomyomatosis LAM is a rare disease of unknown etiology. Case series Of a total of 10 patients with LAM treated with sirolimus at our center during the study period, we describe four cases of patients in whom the drug was quilotorxa used because of extrapulmonary involvement.
Case 1 A year-old smoker with a smoking history of 15 pack-years was admitted with a two-year history of dyspnea, which had worsened one week prior. Open in a separate window.
Chest X-rays taken before treatment with sirolimus in A and after 12 months of treatment with the drug in Bshowing resolution of chylothorax. Pulmonary function test results before and after treatment with sirolimus.
Abdominal CT scans taken before treatment with sirolimus in A and after 12 months of treatment with the drug in Bshowing a reduction in bilateral renal angiomyolipoma volume. Qilotorax 3 A year-old nonsmoker with a three-year history of dyspnea on exertion presented with acute worsening of dyspnea. Abdominal CT scans taken before treatment with sirolimus in A and after 12 months of treatment with the quilptorax in Bshowing a significant reduction in retroperitoneal lymphangioleiomyoma volume white arrow.
Case 4 A year-old nonsmoker presented with diffuse abdominal pain. Abdominal CT scans taken before treatment with sirolimus in A and after 6 months of treatment with the drug in Bshowing resolution of an abdominal lymphangioleiomyoma white arrow.
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