Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation.

Lymphangioleiomyomatosis (LAM) is a rare, slowly progressive lethal lung disease primary afflicting young women. LAM is characterized by proliferation of abnormal smooth muscle cells that target the lungs, causing cystic destruction and eventual respiratory failure leading to death. Recent ten year mortality due to end stage LAM has been reported to be approximately 10%-20%, but may vary. The decline in lung function in LAM is gradual, occurring at a rate of about 3% to 15% per year but can vary from patient to patient. But recently therapy with mammalian target of rapamycin (mTOR) inhibitors such as sirolimus has shown promising results in the stabilization of lung function and reduction of chylous effusions in LAM. Lung transplantation is a viable option for patients who continue to have decline in lung function despite mTOR therapy. Unique issues that may occur post-transplant in a recipient with LAM include development of chylous effusion and a risk of recurrence. We describe a case of LAM recurrence in a bilateral lung transplant recipient who developed histological findings of LAM nine years after transplantation.

A non-randomized study of safety and efficacy of heparin for DVT prophylaxis in intracerebral haemorrhage.

OBJECTIVE: To evaluate the safety and efficacy of subcutaneous heparin for deep venous thrombosis (DVT) prophylaxis in patients with intracerebral haemorrhage (ICH) during acute phase in comparison to elastic stockings. METHODS: The diagnosis of ICH was based on imaging (CT or MRI) and DVT was based on Doppler ultrasound. RESULTS: A total of 458 patients were identified over a period of 5 years (1997-2001). Median age was 59 years (range:12-99 years). Two hundred (44%) patients received heparin (heparin group) in addition to elastic stockings and 258 (56%) patients were only given elastic stockings (stockings group). These two groups were not randomized. Heparin was administered subcutaneously (SQ) in doses of 2500-5000 international units twice daily. Two groups were matched for age (p = 0.5), sex (p = 0.28), presence of diabetes mellitus (p = 0.14), cigarette smoking (p = 0.045) and presence of hydrocephalus or midline shift on CT/MRI (p= 0.87). One patient developed DVT in control group while none developed DVT in heparin group (p = 0.18). One patient had worsening of ICH on repeat CT scan in Heparin group. This worsening was non fatal. Systemic haemorrhagic complications (non fatal) were seen in 7 (14%) patients receiving heparin. Twenty five patients (12%) in heparin group and 52 (20%) in control group died (p = 0.02). CONCLUSION: Subcutaneous heparin in doses of 2500-5000 units twice daily during acute phase in patients with ICH may be safe for DVT prophylaxis. It was not superior to elastic stockings in a non-randomized comparison to prevent DVT.