Epidermolysis bullosa (EB) is a heterogenous group of genodermatoses characterized by the formation of blisters and erosions on skin and mucous membrans from birth on. The cause are mutations in the genes of structural proteins of the junction between epidermis and dermis. This book deals with the treatment of this skin disease itself and its many extracutaneous complications. There is no previous book which has been focused on the therapy and it will be based on evidence-based data derived from the worlds largest cohort of inherited EB-patients, the American EB Registry. An important chapter will discuss gene therapy in hereditary EB which has been recently successfully performed within a localized skin site on a single EB patient as a proof-of-principle test. Given its unique collective contents, the monograph will provide the primary source for clinical informations of this oftentimes severe multiorgan disease."

Epidermolysis bullosa (EB) is a heterogenous group of genodermatoses characterized by the formation of blisters and erosions on skin and mucous membrans from birth on. The cause are mutations in the genes of structural proteins of the junction between epidermis and dermis. This book deals with the treatment of this skin disease itself and its many extracutaneous complications. There is no previous book which has been focused on the therapy and it will be based on evidence-based data derived from the world?'s largest cohort of inherited EB-patients, the American EB Registry. An important chapter will discuss gene therapy in hereditary EB which has been recently successfully performed within a localized skin site on a single EB patient as a proof-of-principle test. Given its unique collective contents, the monograph will provide the primary source for clinical informations of this oftentimes severe multiorgan disease.

The twelve essays included here explore the relationship between place and prose - between San Francisco the city and San Francisco the territory of fiction. From the Gold Rush times of Mark Twain and Bret Harte, through the Prohibition Era of Dashiell Hammett to the Beat days of Jack Kerouac and the present works of writers like Maxine Hong Kingston, Amy Tan, and Arturo Islas, San Francisco has been blessed with great writers who have given life to the land in their fiction. These essays engage the history and geography, ethnic, gender, and class conflicts, and stylistic range of the fiction. They demonstrate how authors as various as Jack London, Gertrude Atherton, Frank Norris, William Saroyan, James D. Houston, Joan Didion, and Wallace Stegner have re-created and revised our understanding of this region.

Who are the women who walk the beat in Dublin's red-light districts? How did they get there? Why do they stay? What happens when they try to leave? What are their lives really like? The Beat: Life on the Streets in a fascinating, disturbing account of the lives of sixteen women and their struggle for surival in Dublin's underworld. Haunted by the drug-related death of his lover Seema, herself a 'working girl', David Fine decided to confront his grief head-on - journeying to the heart of an invisible Ireland to find out what it means to be a prostitute. Working as a taxi driver, Fine got to know the women on the streets, unveiling every aspect of their harrowing lives. Their stories command attention and compassion on every page of this revealing book. Fine describes how these women - alternately raging or gentle, brutal or loving, vicious or simply wounded - destroy themselves, how their personalites crash and collapse, driven by the drugs coursing through their veins. Here are Dublin's 'working girls' in their own words. Imelda is fierce, and fiercely loves her two daughters. Sorcha is so strung out on heroin she eats her own clothes. Una will do anything to avoid sex. Teresa was gang-raped at the age of eleven. Despite it all, these women continue to live and love and dream of a better world. The Beat gives voice to the voiceless - Fine's admiration for their courage shining through. LIke Jim Carrol in The Basketball Diaries and Scorsese in Taxi Driver, he sees human dignity and beauty in life's darkest corners.

This volume traces the history of Sheffield from a Medieval town that Chaucer knew through the great Victorian age of industry to the late twentieth-century and a new era of post-industrial revolution. This account of Sheffield's past considers the impact of the vast steel-rolling mills, compares the lives of mill owner and mill worker and looks at life in the countless back-to-backs once strewn across this richly varied city. Using a wealth of photographs, maps and prints the author brings the story up to date and considers what the future may hold for this great city. An important feature of this book is a guided walking tour of the city centre that takes the reader through the modern city and points out features of the past that are still there to see.