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Hemophilia

disease, blood, tendency, life, joints, child and sometimes

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HEMOPHILIA is a congenital tendency to bleeding which manifests itself shortly after birth and lasts the life of the patient. The hmmorrhage oc curs either spontaneously or upon slight provocation, and can only be arrested with great difficulty. The subjects of the disease also exhibit a carious tendency to obstinate swellings of the joints, which are often spoken of as " rheumatism." A temporary disposition to haemorrhages, such as is sometimes left after certain diseases, does not constitute hemo philia. 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.

Causation.—Hamophilia, if not invariably hereditary, shows a singular tendency to hereditary transmission. The proclivity manifests itself more frequently in the male than in the female offspring ; but the females, if themselves exempt from this peculiarity, are still capable of transmitting the disease to their children. It is, indeed, a curious fact that the trans mission of the tendency to the child is seen more commonly in cases where the patient, whether male or female, although sprung from a family of bleeders, is individually free from the hmmorrhagic disposition. It is rare to find a father transmit the disease to his child if he is himself a sufferer. In the majority of cases the unfortunate inheritance is derived from the mother, who has probably escaped.

In a family subject to this tendency all the male children may prove bleeders. Sometimes, however, one or more escape. Dr. Wickham Legg is of opinion that when transmission is only partial the first-born are more exempt than the others. The disease is found in all countries and all con ditions of life. The Hebrew race is said to be peculiarly liable to it.

Morbid cases of 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 recognisable 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 ancemia. Petechim in the skin, and bruise-like patches from subcuta

neous extravasation, may be found ; and sometimes large collections of blood have been met with. Sir W. Jenner has reported the case of a boy, aged thirteen years, in whom an enormous extravasation of blood was dis covered beneath the fascia of the right thigh. The swelling of the joints appears.to be due to extravasation of blood into the articulations. In a case reported by M. Poncet, on opening the knee-joint, which had been obstinately swollen and painful during life, all the tissues of the articula tion were found to be stained with blood. At the circumference the tis sues'were chocolate-coloured ; the articular surfaces were red and impreg Dated with blood ; and the cartilages were the seat of advanced lesions such as have been described by Charcot as characteristic of chronic rheu matism. Microscopic examination revealed in the substance of the tissues yellow granules, irregular or rounded, and of variable size, pigment gran ules, and fat granules. Other joints in the same subject showed similar lesions.

,Symptoms.—There is nothing in the look of the child at birth to indi cate any peculiarity of constitution. Nor in after years, unless the indi vidual be actually suffering from loss of blood or disease of the joints, is there anything in his appearance to distinguish him from another without the same tendency to bleed. 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 lmmorrhagic 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 con stitutional peculiarity can be recognised. Sometimes, however, evidence of the disease is postponed until later. Bleeding may not be noticed until the second crop of teeth begins to make its appearance at about the sixth year. It has even been known to come on for the first time at a later period ; but is rarely delayed till after puberty.

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