PubMed · 8251673
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S M Aronson. 1993. A loathsome custom.. https://pubmed.ncbi.nlm.nih.gov/8251673/
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Neurofibromatosis (NF) has perhaps been the most notorious of the neurocutaneous disorders. Both Quasimoto of Victor Hugo's The Hunchback of Notre Dame and John Merrick, known as the Elephant Man (who subsequently has been more properly classified as having Proteus syndrome, a quite different disorder), are 2 infamous examples that have shaped many popular misconceptions about this disease. Neurofibromatosis is now understood as a hamartomatous disorder on the basis of molecular genetic studies. Studies on NF have been pivotal to understanding the functions of oncogenes in tumorigenesis.
The spleen is an enigmatic organ with a peculiar anatomy and physiology. Though our understanding of this organ has improved vastly over the years, the spleen continues to produce problems for the surgeon, the hematologist, and the patient. The history of the spleen is full of fables and myths, but it is also full of realities. In the Talmud, the Midrash, and the writings of Hippocrates, Plato, Aristotle, Galen, and several other giants of the past, one can find a lot of Delphian and Byzantine ambiguities. At that time, splenectomy was the art of surgery for many splenic diseases. From antiquity to the Renaissance, efforts were made to study the structure, functions, and anatomy of the spleen. Vesalius questioned Galen; and Malpighi, the founder of microscopic anatomy, gave a sound account of the histology and the physiologic destiny of the spleen. Surgical inquiry gradually became a focal point, yet it was still not clear what purpose the spleen served. It has been within the past 50 years that the most significant advances in the knowledge of the spleen and splenic surgery have been made. The work of Campos Christo in 1962 about the segmental anatomy of the spleen helped surgeons perform a partial splenectomy, thereby avoiding complications of postsplenectomy infection. With the recent successes of laparoscopic splenectomy in selected cases, the future of splenic surgery will undoubtedly bring many more changes.
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