The ability of plasma to support prostacyclin like activity from human umbilical arterial rings was studied in 17 patients with Henoch-Schönlein purpura and 17 controls matched for age and sex. Plasma from 13 of the 17 patients showed a diminished or absent ability to support prostacyclin like activity in vitro. Six patients whose plasma had a low or absent ability to support prostacyclin like ...

Article history: Received 16 May 2017 Accepted 16 May 2017 Available online 17 May 2017 blood oxygen saturation (hypoxemia). Even more surprisingly, the functional changes are not accompanied by the expected histopathological abnormalities, such as inflammation, fibrosis or vascular changes. The finding of restrictive lung disease without readily visible microstructural abnormalities is unusual...

Spinal muscular atrophy (SMA) is an important cause of death in children and SMA type I, also known as Werdnig-Hoffman disease, is the most severe form of this disease. We report 2 cases of infants with SMA I in whom a distal necrosis developed, a feature not previously reported. Poor perfusion, autonomic dysfunction, and position-dependent factors may all play a role in the development of this...

Methods This monocentric observational study was conducted at the French National Reference Center for TTR-FAP. Genetically confirmed TTR-FAP patients with suspected retinal and/or choroidal vascular abnormalities underwent fluorescein and indocyanine green angiography. Sensorimotor polyneuropathy (SPN) was staged with the Polyneuropathy Disability (PND) score, vegetative neuropathy was staged ...

We examined the hypothesis that the vascular abnormalities of Bartter's syndrome are due to excess production of prostaglandin. Balance studies and vascular reactivity studies were performed before and after indomethacin (200 mg/day) in a patient with well-documented Bartter's syndrome. During indomethacin, potassium balance became positive, serum potassium rose from 2.1--3 mEq/1 in the absence...