lymphangioleiomyomatosis/edema

Tuberous sclerosis complex (TSC) is an autosomal-dominant disorder characterized by seizures, mental retardation, and various hamartomatous lesions, including renal angiomyolipoma (AML) and pulmonary lymphangioleiomyomatosis. A 22-year-old woman with TSC presented with multiple renal AMLs exceeding

METHODS A 45-year-old woman presented with marked edema of both lower extremities over 6 weeks for a nephrological work-up; she had gained 8 kg of body weight. Voiding was asymptomatic and she had a stable diuresis. The patient took oestrogens for contraception over 10 years. Blood pressure was

OBJECTIVE To evaluate the imaging and clinical features of lymphangioleiomyomas and to describe the phenomenon of diurnal variation in the size of lymphangioleiomyomas in patients with lymphangioleiomyomatosis. METHODS One hundred twenty-eight patients with lymphangioleiomyomatosis underwent chest

This is a rare case of sporadic lymphangioleiomyomatosis (S-LAM) manifesting as refractory chylothorax and chyloperitoneum. A middle-aged woman with unremarkable medical history presented with respiratory failure, abdominal distension and anasarca. She was found to have high-output chylous effusion

A 29-year-old woman was admitted to our hospital with a 7-day history of elevated temperature to 39.5 degrees C associated with headache and nausea. She had been diagnosed with tuberous sclerosis complex 10 years earlier. Her unconsciousness progressed, and she was diagnosed as having aseptic

Immediately after delivery a 17-year-old Turkish primipara developed edema, supraclavicular lymphoma and pleural effusion. CT-scans showed massive abdominal and mediastinal lymphoma. Lymphangioleiomyomatosis (LAM) was diagnosed by supraclavicular and retroperitoneal biopsy and progesterone receptors

A 29-year-old woman was referred to our hospital for the intensive examination of leg edema and hypoproteinemia. CT scan of showed multiple thin-walled cysts in both lungs, suggesting lymphangioleiomyomatosis. CT scan of the abdomen, lymphoscintigraphy showed enlarged abdominal lymph nodes. Protein

BACKGROUND Combined simvastatin and sirolimus therapy reduces tuberous sclerosis complex 2-null lesions and alveolar destruction in a mouse model of lymphangioleiomyomatosis (LAM), suggesting that therapy with both drugs may benefit patients with LAM. METHODS To determine whether simvastatin changed

OBJECTIVE Primary chylous disorders (PCDs) are rare. Rupture of dilated lymph vessels (lymphangiectasia) may result in chylous ascites, chylothorax, or leakage of chyle through chylocutanous fistulas in the lower limbs or genitalia. Chyle may reflux through incompetent lymphatics, causing

OBJECTIVE The purpose of this review was to outline the mechanisms and to review recent literature on pulmonary arterial hypertension (PAH) medications in group 5 pulmonary hypertension (PH). RESULTS The first steps in management are to understand the mechanisms and hemodynamic profile and to

Congenital disorders of the lymphatics of the lung are rare. On the basis of a literature review and our own experiences the disorders are divided into four groups and their radiographic findings are described. Pulmonary lymphangiectasia shows not typical signs. As an expression of the underlying