What’s new with numbers? Sociological approaches to the study of quantification

Calculation and quantification have been critical features of modern societies, closely linked to science, markets, and administration. In the past thirty years, the pace, purpose, and scope of quantification have greatly expanded, and there has been a corresponding increase in scholarship on quantification. We offer an assessment of the widely dispersed literature on quantification across four domains where quantification and quantification scholarship have particularly flourished: administration, democratic rule, economics, and personal life. In doing so, we seek to stimulate more cross-disciplinary debate and exchange. We caution against unifying accounts of quantification and highlight the importance of tracking quantification across different sites in order to appreciate its essential ambiguity and conduct more systematic investigations of interactions between different quantification regimes

Current treatments in familial dysautonomia

INTRODUCTION: Familial dysautonomia (FD) is a rare hereditary sensory and autonomic neuropathy (type III). The disease is caused by a point mutation in the IKBKAP gene that affects the splicing of the elongator-1 protein (also known as IKAP). Patients have dramatic blood pressure instability due to baroreflex failure, chronic kidney disease, and impaired swallowing leading to recurrent aspiration pneumonia, which results in chronic lung disease. Diminished pain and temperature perception results in neuropathic joints and thermal injuries. Impaired proprioception leads to gait ataxia. Optic neuropathy and corneal opacities lead to progressive visual loss. AREAS COVERED: This article reviews current therapeutic strategies for the symptomatic treatment of FD, as well as the potential of new gene modifying agents. EXPERT OPINION: Therapeutic focus on FD is centered on reducing the catecholamine surges caused by baroreflex failure. Managing neurogenic dysphagia with effective protection of the airway passages and prompt treatment of aspiration pneumonias is necessary to prevent respiratory failure. Sedative medications should be used cautiously due to risk of respiratory depression. Non-invasive ventilation during sleep effectively manages apneas and prevents hypercapnia. Clinical trials of compounds that increase levels of IKAP (ELP-1) are underway and will determine whether they can reverse or slow disease progression