Angiomiolipoma renal: un caso de difícil diagnóstico. Visitas. Management of renal angiomyolipoma: A report of 14 cases and review of the litera-ture. Angiomiolipoma epitelioide: una variante rara del angiomiolipoma renal. Epithelioid angiomyolipoma: A rare variant of renal angiomyolipoma. Juan Carlos. Num. Pages DOI: / Full text access. Angiomiolipoma renal: Revisión con imágenes ecográficas. Visits.
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Lymphangioleiomyomatosis LAM is a rare disease characterized by abnormal proliferation of immature smooth muscle cells and cystic lung destruction, which determines the prognosis of the disease. The kidney angiomyolipomas are usually very common in this disease and are usually asymptomatic unless complications arise.
In the absence of a curative treatment, recent publications show promising results in molecular therapy to prevent functional decline and to control the size of the angiomyolipomas. These therapies include mTOR complex inhibitors, especially sirolimus.
We report a case of a patient diagnosed with LAM who underwent lung transplantation with reduction of renal angiomyolipoma size after treatment with the mTOR inhibitor everolimus. Entre estas terapias destacan los inhibidores del complejo mTOR, sobre todo sirolimus.
Lymphangioleiomyomatosis LAM is a rare disease that almost exclusively affects women of childbearing age and is characterized by the abnormal proliferation of immature smooth muscle cells and cystic destruction of the lungs. It is a disease with a genetic base involving the TSC1 and 2 tuberous sclerosis complex genes.
These genes, through the mTOR mammalian target of rapamycin enzyme complex, are in charge of controlling cell survival and proliferation. Pulmonary affectation is usually responsible for the symptoms, while the extrapulmonary affectation, fundamentally renal angiomyolipomas, is usually asymptomatic.
Currently, there is no curative treatment. In patients with poor clinical evolution and progression towards respiratory failure, lung transplantation LTx may be an alternative. Recent publications have shown hopeful results in molecular therapy, 1—6 among these mTOR complex inhibitors, sirolimus and angipmiolipoma.
However, these drugs are not free from potential adverse effects, and their application has not been generalized outside clinical assays. In this publication, we present a clinical case of a patient diagnosed with LAM who underwent LTx with reduction of the size of the angiomiolipoam angiomyolipoma after treatment with the mTOR inhibitor everolimus.
A year-old woman was referred to us as a candidate for LTx due to persistent right pneumothorax in spite of endothoracic drainage. The patient was an ex-smoker whose accumulated consumption was not very significant. She had had right pneumothorax during the year prior to the consultation and early relapse after the endothoracic drain had been withdrawn. Initially, she did not present respiratory failure and spirometry detected a severe obstruction with air trapping and hyperinflation.
Radiological studies demonstrated bilateral interstitial pattern with presence of multiple small-sized, thin-walled cysts that affected the entire pulmonary parenchyma, with a preserved architecture.
With the clinical suspicion for LAM, the patient presented left pneumothorax and collapsed lung. Mechanical pleurodesis was carried out and open lung biopsy confirmed the diagnosis. Given the deterioration of the lung function and the presence of a new angioiolipoma pneumothorax with persistent loss, the patient was referred to our Lung Transplantation Unit.
Angiomiolipoma renal: un caso de difícil diagnóstico | Anales de Pediatría
During the evaluation, an abdominal CT detected a mass with heterogeneous density in the left kidney compatible with a renal angiomyolipoma Fig. She was included on the preferential national waiting list for LTx and, days later, underwent right single-lung transplantation with no immediate complications.
Pre-transplantation abdominal CT with a large heterogeneous mass in the left kidney. The initial immunosuppression was triple therapy with calcineurin inhibitors cyclosporineantiproliferative agents mofetil mycophenolate and steroids.
Six months later, the immunosuppression regimen was modified by substituting mofetil mycophenolate with everolimus with the two-fold objective of controlling the mild post-transplantation renal dysfunction and assessing the response of the angiomyolipoma to the pharmacological treatment. During follow-up, the renal and pulmonary function remained stable, at stage 0 of bronchiolitis obliterans syndrome. After 6 months of treatment with everolimus, a new abdominal CT demonstrated, after reconstruction, a significant reduction in the size of the renal angiomyolipoma Fig.
Reduction of the size of the left renal angiomyolipoma on a follow-up abdominal CT after transplantation and 6 months of treatment with everolimus.
LAM is a pathology that is grouped anigomiolipoma the rare diseases and whose clinical manifestations are fundamentally respiratory. It may appear sporadically S-LAM or associated with tuberous sclerosis TSC-LAMa dominant autosomal neurocutaneous syndrome with the formation of hamartomas in the central nervous system, skin, eyes, abdominal organs especially kidneys and the lungs. In these pathologies, the most rennal abdominal alteration is the presence renl angiomyolipomas, which are generally asymptomatic and located predominantly in the kidneys.
Both diseases have a genetic base that involve the TSC1 and 2 genes, which encode the protein products hamartin and tuberin. These genes have an important tumor suppressor role. The main role of the hamartin—tuberin complex is to inhibit the mTOR complex, a central regulatory element of cell growth through protein synthesis.
Supported by new knowledge in physiopathology, specific inhibitor agents are being assayed in the different pathogenic pathways. One of these pathways, the inhibition of mTOR, has brought about new hope for the use of drugs that impede the progression of the disease.
Among these are rapamycin sirolimus and everolimus, immunosuppressant agents used to prevent rejection in transplant recipients given its powerful inhibitory effect on lymphocytic activation. The preliminary results reflected in case studies and in a clinical assay published in are hopeful, 4—6 both in the reduction of the volume of renal tumors as well as in the declining lung function.
Nowadays, the tendency in the majority of transplant groups is to individualize immunosuppression regimes according to risk factors.
Given the high incidence of abdominal tumors in LAM and the published results for reducing their size after treatment with mTOR inhibitors 4 I-mTORit is possible that, in the future, these drugs will be part of the immunosuppression protocol recommended in patients with LAM. The growing experience with these drugs 10 is due to their usefulness in repeated acute rejection, in the loss of graft function as a reflection of chronic rejection, for the control of chronic renal dysfunction due to calcineurin inhibitors, in the control of repetitive infections due to cytomegalovirus and in the management of post-transplant neoplasms.
The anti-tumor or cell-growth control role of these drugs makes their use very hopeful in cases of renal angiomyolipoma in the context of LAM. The pharmacological advantages that everolimus provides lead us to believe that the use of this drug is growing among transplantation groups, particularly in the subgroups of patients with a greater expected benefit.
In this regard, we report the positive results in renal and lung angiomiolupoma and reduction of the size of the angiomyolipoma in our patient with LAM and lung transplantation. Please cite this article as: Bujalance-Cabrera C, et al. Previous article Next article.
December Pages This item has received. These therapies include mTOR complex inhibitors, ajgiomiolipoma sirolimus. We report a case of a patient diagnosed with LAM who underwent lung transplantation with reduction of renal angiomyolipoma size after angiomoilipoma with the mTOR inhibitor everolimus. Entre estas terapias destacan los inhibidores del complejo mTOR, sobre todo sirolimus.
Introduction Lymphangioleiomyomatosis LAM is a rare disease that almost exclusively affects women of anhiomiolipoma age and is characterized by the abnormal proliferation of immature smooth muscle cells and cystic destruction of the lungs. However, these drugs are angiomolipoma free from potential adverse effects, and their application has not been generalized outside clinical assays. In this publication, we present a clinical case of a patient diagnosed with LAM who underwent LTx with reduction of the size of the renal angiomyolipoma after treatment with the mTOR inhibitor everolimus.
Clinical Observation A year-old woman was referred to us as a candidate for LTx due to persistent right pneumothorax in spite of endothoracic drainage. She was included on the preferential national waiting list for LTx and, days later, underwent right single-lung transplantation with no immediate complications. Pre-transplantation abdominal CT with a large heterogeneous mass in the left kidney.
Reduction of the size of the left renal angiomyolipoma on a follow-up abdominal CT after transplantation and agiomiolipoma months of treatment with everolimus. Eur J Intern Med, 19pp. Chest,pp.
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Arch Bronconeumol, 47pp. The angiomolipoma of the international society for heart and lung transplantation: J Heart Lung Transplant, 30pp. International guidelines for the selection of lung transplant candidates: J Heart Lung Transplant, 25pp. The US experience with lung transplantation for pulmonary lymphangioleiomyomatosis.
J Heart Lung Transplant, 24pp. Lung transplantation for lymphangioleiomyomatosis: J Heart Lung Transplant, 28pp. Current trends in immunosuppression for lung transplantation. Semin Respir Crit Care Med, 31pp.
Expert Opin Pharmacother, 7pp. Two-year outcomes in thoracic transplant recipients after conversion to everolimus with reduced calcineurin inhibitor within a multicenter, open-label, randomized trial.