Successful pregnancy complicated by persistent pneumothorax in a patient with lymphangioleiomyomatosis (LAM) on sirolimus.
نویسندگان
چکیده
We report a successful pregnancy in a patient with longstanding LAM on treatment with sirolimus. During temporary discontinuation fo sirolimus in early pregnancy, lung function declined but recovered after resumption of sirolimus. Pregnancy was complicated by a persistent pneumothorax which was treated surgically postnatally. The child has had a normal development despite exposure to low dose sirolimus intermittently during early embryonal and mid-fetal life.
منابع مشابه
Successful management of bilateral refractory chylothorax after double lung transplantation for lymphangioleiomyomatosis
Lymphangioleiomyomatosis (LAM) is a rare disease that leads to airways and lymphatic channels obstruction due to abnormal smooth muscle proliferation. It presents with dyspnea, pneumothorax or chylothorax. Lung transplantation (LT) has emerged as a valuable therapeutic option with limited reports. We report a case of LAM that underwent double LT and complicated by refractory bilateral chylothor...
متن کاملLong-term stable lung function and second uncomplicated pregnancy on sirolimus in lymphangioleiomyomatosis (LAM).
We present a patient with lymphangioleiomyomatosis (LAM) on long-term sirolimus (now 79 months) who has had a second successful pregnancy. The second pregnancy on uninterrupted low-dose sirolimus (plasma levels 3-5 mg/L) was uncomplicated both with respect to mother and child suggesting that low-dose sirolimus might be safe in selected pregnant patients with stable LAM. The long-term time cours...
متن کاملEffects of sirolimus on Lung function in patients with Lymphangioleiomyomatosis
Background: Lymphangioleiomyomatosis (LAM) is a progressive lungs disease that affects women at reproductive years. Sirolimus inhibits mammalian target of rapamycin (mTOR) and its administration in past studies was hopeful in treatment of patients with LAM. The aim of this study was to evaluate sirolimus therapy on lung function in LAM patients. Methods: We conducted a trial to evaluate the ef...
متن کاملPulmonary lymphangioleiomyomatosis presenting as spontaneous pneumothorax treated with sirolimus - A case report
Spontaneous pneumothorax is a very common medical emergency. Patients are often treated without treating the underlying cause. Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease. Until recently, diagnosis of LAM was a challenge with nearly 100% mortality in 10 years, but better understanding of the disease through research and better radiological techniques and newer drugs such as sir...
متن کاملChyloperitoneum, chylothorax and lower extremity lymphedema in woman with sporadic lymphangioleiomyomatosis successfully treated with sirolimus: a case report.
Lymphangioleiomyomatosis (LAM) is a rare disease characterized by diffuse thin-walled cysts throughout the lungs on computed tomography and diffuse proliferation of abnormal smooth muscle-like cells (LAM cells) on lung biopsy. LAM affects women almost exclusively, predominantly in their reproductive age. The most typical presenting symptoms include dyspnea, spontaneous pneumothorax, cough and c...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG
دوره 28 2 شماره
صفحات -
تاریخ انتشار 2011