Leber's Hereditary Optic Neuropathy and Hypertrophic Cardiomyopathy

Leber’s hereditary optic neuropathy (LHON) is a mitochondrial condition that gradually affects the central vision. In the current case report, we present 2 relatives with LHON due to a pathogenic variant within ND1 with a clinical phenotype resembling hypertrophic cardiomyopathy, including a short PQ-interval and hypertrophy on electrocardiogram as well as severe hypertrophy of the left ventricle on echocardiography. These findings highlight the importance of offering routine cardiac investigation to patients with LHON and their relatives carrying the ND1 variant to hopefully improve correct diagnosis and clinical management of LHON patients

Methane Steam Reforming over an Ni-YSZ Solid Oxide Fuel Cell Anode in Stack Configuration

The kinetics of catalytic steam reforming of methane over an Ni-YSZ anode of a solid oxide fuel cell (SOFC) have been investigated with the cell placed in a stack configuration. In order to decrease the degree of conversion, a single cell stack with reduced area was used. Measurements were performed in the temperature range 600–800°C and the partial pressures of all reactants and products were varied. The obtained rates could be well fitted with a power law expression (r ∝PCH40.7). A simple model is presented which is capable of predicting the methane conversion in a stack configuration from intrinsic kinetics of the anode support material. The predictions are compared with the stack measurements presented here, and good agreement is observed