The James Lind Library: explaining and illustrating the evolution of fair tests of medical treatments

The James Lind Library (www.jameslindlibrary.org) has been established to improve public and professional general knowledge about fair tests of treatments in healthcare and their history. Its foundation was laid ten years ago at the Royal College of Physicians of Edinburgh, and its administrative centre is in the College's Sibbald Library, one of the most important collections of historic medical manuscripts, papers and books in the world. The James Lind Library is a website that introduces visitors to the principles of fair tests of treatments, with a series of short, illustrated essays, which are currently available in English, Arabic, Chinese, French, Portuguese, Russian and Spanish. A 100-page book-- Testing Treatments--is now available free through the website, both in English and in Arabic and Spanish translations. To illustrate the evolution of ideas related to fair tests of treatments from 2000 BC to the present, the James Lind Library contains key passages and images from manuscripts, books and journal articles, many of them accompanied by commentaries, biographies, portraits and other relevant documents and images, including audio and video files. New material is being added to the website continuously, as relevant new records are identified and as methods for testing treatments evolve. A multinational, multilingual editorial team oversees the development of the website, which currently receives tens of thousands of visitors every month.

Expanding the spectrum of mutations in GH1 and GHRHR: genetic screening in a large cohort of patients with congenital isolated growth hormone deficiency

CONTEXT: It is estimated that 3-30% of cases with isolated GH deficiency (IGHD) have a genetic etiology, with a number of mutations being reported in GH1 and GHRHR. The aim of our study was to genetically characterize a cohort of patients with congenital IGHD and analyze their characteristics. PATIENTS AND METHODS: A total of 224 patients (190 pedigrees) with IGHD and a eutopic posterior pituitary were screened for mutations in GH1 and GHRHR. To explore the possibility of an association of GH1 abnormalities with multiple pituitary hormone deficiencies, we have screened 62 patients with either multiple pituitary hormone deficiencies (42 pedigrees), or IGHD with an ectopic posterior pituitary (21 pedigrees). RESULTS: Mutations in GH1 and GHRHR were identified in 41 patients from 21 pedigrees (11.1%), with a higher prevalence in familial cases (38.6%). These included previously described and novel mutations in GH1 (C182X, G120V, R178H, IVS3+4nt, a>t) and GHRHR (W273S, R94L, R162W). Autosomal dominant, type II IGHD was the commonest form (52.4%), followed by type IB (42.8%) and type IA (4.8%). Patients with type II IGHD had highly variable phenotypes. There was no difference in the endocrinology or magnetic resonance imaging appearance between patients with and without mutations, although those with mutations presented with more significant growth failure (height, -4.7 +/- 1.6 SDS vs. -3.4 +/- 1.7 SDS) (P = 0.001). There was no apparent difference between patients with mutations in GH1 and GHRHR. CONCLUSIONS: IGHD patients with severe growth failure and a positive family history should be screened for genetic mutations; the evolving endocrinopathy observed in some of these patients suggests the need for long-term follow-up.

«Von Menschen und Göttern verlassene Leichname». Totenkult im 'Musée des Monuments Français' (1791-1816)?

Das 'Musée des Monuments Français' ging 1793 aus der Revolution hervor. Das ehemalige Kloster, in dem enteignete Kirchengüter gelagert wurden, mutierte unter Alexandre Lenoir zu einer chronologisch geordneten Ruhmesstätte der französischen Geschichte. Grabmäler und Monumente erinnern an verschiedene Personen dieser Geschichte – von Héloïse über viele Könige bis hin zu Descartes. Allerdings war das 'Musée des Monuments Français' weniger ein Ort des royalen Totenkults als vielmehr ein Museum, in dem auch die königlichen Grabmäler als Exponate innerhalb der Präsentation der französischen Geschichte fungierten. Daher führte es unter Zeitgenossen zu einer Polarisierung in Anhänger und Gegner: So waren zum Beispiel Michelet und Wilhelm von Humboldt vom 'Musée des Monuments Français' begeistert, während Chateaubriand es strikt ablehnte. Im 'Musée des Monuments Français’ waren die Grabstätten und Skulpturen permanente Zeugnisse der Niederlage des Königtums. In der Restauration wurde das Museum Ende 1816 geschlossen, die königlichen Grabmäler wurden zurück in die Kirche von Saint Denis überführt.