Hemophilia is a congenital tendency to bleeding which manifests itself shortly after birth and lasts the life of the patient. The hemorrhage occurs either spontaneously or upon slight provocation, and can only be arrested with great difficulty. The subjects of the disease also exhibit a curious tendency to obstinate swellings of the joints, which are often spoken of as rheumatism. A temporary disposition to hemorrhages, such as is sometimes left after certain diseases, does not constitute hemophilia. The true disease dates from birth, or appears shortly after it; is always seen in childhood, and persists, as a rule, to the very end of life.
[...] The child may be fair or dark, tall or short, of robust frame or of slender build. As a rule, he looks healthy, and his intellectual capacity is above the average. It is rarely before the end of the first twelve months of life that any sign is noticed of the hemorrhagic disposition. Bleeding seldom occurs at the time of separation of the umbilical cord, or during the operation of vaccination; and it is not until the infant is able to crawl or walk, and thus becomes exposed to injuries from falls or other violence, that his constitutional peculiarity can be recognized. [...]
[...] In less common cases blood is also poured out from the mucous membrane of the stomach and bowels, and may be vomited up or discharged by stool. As a rule, the younger the child the more likely is the hemorrhage to come from the nose or mouth. Renal hemorrhage is rare. Once started, the loss of blood may be continuous and copious, so as to be arrested with the greatest difficulty; or may cease for a time and then return. [...]
[...] death from hemophilia little is found to explain the nature of the disease. The body is usually blanched from loss of blood, but the organs, especially the heart and large vessels, present no appearance of disease. No change is discovered in the blood, and the vessels seldom present any alterations recognizable by the microscope. In some cases, indeed, a partial fatty degeneration of the lining membrane of the arteries has been observed; but this is probably the consequence of the anaemia. [...]
[...] In the case of traumatic bleeding the hemorrhage usually begins some hours after the infliction the injury. It often does not cease until the patient becomes faint, and even then is liable to renewal when consciousness returns. By this means the child may be reduced to a state of profound anaemia, and only slowly regains his color and strength. Trifling blows may produce copious effusions. In some cases the blood infiltrates extensively through the areola tissue of a limb, and death may even ensue from this inward bleeding. [...]
[...] In all cases of true hemophilia careful inquiry will discover the existence of a hereditary tendency, especially on the side of the mother, and also in most cases a disposition on the part of the child himself to bleed profusely upon slight provocation. The nature of the joint affection can only be discovered by establishing the existence of the hemorrhagic tendency; for there is nothing in the character of the joint symptoms to distinguish the swelling from that produced by other causes. [...]
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