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Hereditary spastic paraplegias (HSPs) are genetically heterogeneous Mendelian disorders characterized by spastic gait with stiffness and weakness in the legs and an associated plethora of neurological or extraneurological signs in “complicated” forms. Major advances have been made during the past two decades in our understanding of their molecular bases with the identification of a large number of gene loci and the cloning of a set of them. The combined genetic and clinical information obtained has permitted a new, molecularly-driven classification and an improved diagnosis of these conditions. This represents a prerequisite for better counseling in families and more appropriate therapeutic options. However, further heterogeneity is expected and new insight into the possible mechanisms anticipated.
The child is neither an adult miniature nor an immature human being: at each age, it expresses specific abilities that optimize adaptation to its environment and development of new acquisitions. Diseases in children cover all specialties encountered in adulthood, and neurology involves a particularly large area, ranging from the brain to the striated muscle, the generation and functioning of which require half the genes of the whole genome and a majority of mitochondrial ones. Human being nervous system is sensitive to prenatal aggression, is particularly immature at birth and development may be affected by a whole range of age-dependent disorders distinct from those that occur in adults. Ev...
A young woman encounters strange events in her Mexican hometown in this novel by an author who “immerses us...in her wickedly funny and imaginative world” (Latina). Leaving Tabasco tells of the coming of age of Delmira Ulloa, raised in an all-female home in Agustini, in the Mexican province of Tabasco. In Agustini it is not unusual to see your grandmother float above the bed when she sleeps, or to purchase torrential rains at a traveling fair, or to watch your family’s elderly serving woman develop stigmata, then disappear completely, to be canonized as a local saint. But as Delmira becomes a woman, she will set out on a search for her missing father, and must make a choice that could ...
Russia, 1905. Behind the gates of the Karenin Palace, Sergei, son of Anna Karenina, meets Tolstoy in his dreams and finds reminders of his mother everywhere: the almost-living portrait that the Tsar intends to acquire and the opium-infused manuscripts she wrote just before her death, one of which opens a trapdoor to a wild feminist fairytale. Across the city, Clementine, an anarchist seamstress, and Father Gapón, the charismatic leader of the proletariat, tip the country ever closer to revolution. Boullosa lifts the voices of coachmen, sailors, maids, and seamstresses in this playful, polyphonic, and subversive revision of the Russian revolution, told through the lens of Tolstoy’s most beloved work.
Dendrimer-Based Nanotherapeutics delivers a comprehensive resource on the use of dendrimer-based drug delivery. Advances in the application of nanotechnology in medicine have given rise to multifunctional smart nanocarriers that can be engineered with tunable physicochemical characteristics to deliver one or more therapeutic agent(s) safely and selectively to cancer cells, including intracellular organelle-specific targeting. This book compiles the contribution of dendrimers in the field of nanotechnology to aid researchers in exploring dendrimers in the field of drug delivery and related applications. This book covers the history of the area to the most recent research. The starting chapter...
A dark, thought-provoking adventure that “artfully evokes the blood-soaked reality of 17th-century pirates” (Entertainment Weekly). This “wryly humorous, satiric, and often macabre novel” (Library Journal) follows Jean Smeeks, a Flemish thirteen-year-old who signs up as an indentured servant with the French West Indies Company, but instead winds up a slave on the notorious island of Tortuga. Over time, he learns the arts of herbal medicine and surgery—a skill that allows him to join a band of Caribbean pirates. Contrasting Jean’s romantic pull toward the “Brethren of the Coast”—an all-male society pursuing socialist, anti-colonialist ideals—with the brutal reality of their lawless existence, They’re Cows, We’re Pigs is a “unique and memorable” novel whose “pirate world leaves you as a good book should: thinking” (The Boston Herald).
Rosenberg’s Molecular and Genetic Basis of Neurologic and Psychiatric Disease, Fifth Edition provides a comprehensive introduction and reference to the foundations and key practical aspects relevant to the majority of neurologic and psychiatric disease. A favorite of over three generations of students, clinicians and scholars, this new edition retains and expands the informative, concise and critical tone of the first edition. This is an essential reference for general medical practitioners, neurologists, psychiatrists, geneticists, and related professionals, and for the neuroscience and neurology research community. The content covers all aspects essential to the practice of neurogenetics...
The development of paediatric medicines can be challenging since this is a different patient population with specific needs. A medicine designed for use in paediatric patients must consider the following aspects: patient population variability; the need for dose flexibility; route of administration; patient compliance; excipient tolerability. For example, the toxicity of excipients may differ in children compared to adults and children have different taste preferences. Globally, about 75% of drugs do not carry regulatory approval for use in children; worldwide, many medications prescribed for the treatment of paediatric diseases are used off-label, and less than 20% of package inserts have sufficient information for treating children. This book provides an update on both state-of-the-art methodology and operational challenges in paediatric formulation design and development. It aims at re-evaluating what is needed for more progress in the design and development of age-appropriate treatments for paediatric diseases, focusing on: formulation development; drug delivery design; efficacy, safety, and tolerability of drugs and excipients.
An enchanting, audacious retelling of the Cleopatra story from a Mexican novelist who is “a luminous writer” and “a masterful spinner of the fantastic” (The Miami Herald). In Cleopatra Dismounts, Carmen Boullosa has written a remarkable imaginary life of one of history’s most legendary women. Dying in Marc Antony’s arms, Cleopatra bewails the end of her political career throughout ancient Egypt, Greece, and the Mediterranean. But is this weak woman the true Cleopatra? Through the intervention of Cleopatra’s scribe and informer Diomedes, Boullosa creates two deliriously wild other lives for the young monarch—a girl escaping the intrigues of royal society to disguise herself an...
This concise sourcebook of the electrochemical, engineering and economic principles involved in the development and commercialization of fuel cells offers a thorough review of applications and techno-economic assessment of fuel cell technologies, plus in-depth discussion of conventional and novel approaches for generating energy. Parts I and II explain basic and applied electrochemistry relevant to an understanding of fuel cells. Part III covers engineering and technology aspects. The book is useful for undergraduate and graduate students and scientists interested in fuel cells. Unlike any other current book on fuel cells, each chapter includes problems based on the discussions in the text.