Hemophilia

tendency, bleeding, existence, affection, repeated, disposition and haemorrhage

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In addition to the articular affection, pains may be complained of in the limbs about the joints, although unaccompanied by swelling. These may be so severe as to interfere with exercise. The subjects of hmno philia also suffer much from cold, and the haemorrhage may be determined by exposure to weather.

It might, perhaps, be expected that the existence of the constitutional tendency would influence unfavourably the course of the exanthemata and other intercurrent diseases to which childhood is liable ; but this does not appear to be the case. Measles, scarlet fever, and whooping-cough are said to run their normal course in such subjects without manifesting ex ceptionally unfavourable symptoms ; and although the patients are prone to chest affections, such as pleurisy and pneumonia, these diseases are not attended with special dangers. There is no peculiar liability to phthisis ; but sloughing and gangrene are said to be not uncommon accidents in the course of wounds and traumatic injuries generally.

Diagnosis.—In pronounced cases the detection of the limorrhagic tendency is a matter of little difficulty. The history of repeated bleedings, the habitual appearance of bruises upon slight injury, and the affection of the joints, furnish sufficient evidence of the existence of this constitutional peculiarity. In cases where the tendency is present in a less degree the diagnosis is not so easy. Repeated epistaxis is often seen in children whose health in other respects is perfectly satisfactory ; and the occurrence of spontaneous haemorrhage from this source is therefore of no value in establishing the existence of lifemophilia. Again, profuse and even fatal bleeding from the stomach and bowels may be met with in new-born in fants. The cause of haemorrhage in the newly-born is often obscure ; and in the absence of any evident reason for its occurrence some observers have attributed it to a special la.xmorrhagic tendency existing in the in fant. This may be so ; but the cases differ from haemophilia in the fact that where life is preserved no special proneness to bleeding is manifested in after years (see page 655). So, also, in lawmorrliagic purpura profuse bleeding may occur from all the mucous surfaces and into the tissues ; but the disposition to bleed is hei-e, also, a temporary infirmity which passes off and is completely recovered from.

In all cases of true hemophilia careful inquiry will discover the exis tence 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 hmmorrhagic tendency ; for there is nothing in the character of the joint symptoms to distinguish the swelling from that pro duced by other causes.

Prognosis.—Hremophilia is a disease which is accompanied by serious danger to life. The exhaustion produced by repeated hemorrhages is so great that comparatively few of the patients reach adult years. Out of one hundred and fifty-two boys, the subjects of the hniorrhagic disposition, Grandidier found that only nineteen attained the age of twenty-one, and that more than half of the number died before completing their seventh year. Death usually occurs from hemorrhage, but some kinds of bleeding appear to be more unfavourable than others. Thus after ex traction of a tooth is found to be especially dangerous ; obstinate epis taxis is also to be viewed with grave apprehension ; indeed, to these two varieties of bleeding a large proportion of the deaths may be attributed.

Children are said rarely to die from a first bleeding, and one profuse gush which causes fainting is thought to be more favourable than a slower .and persistent oozing. Still, in any case we should speak very cautiously of the future, whether immediate or remote ; for if the tendency be pro nounced, the boy's chances of growing into manhood are not promising.

Treatment.---In cases of haemophilia great care should be taken to pro tect the child from all forms of injury. Vaccination has been seldom fol lowed by dangerous bleeding ; but the operation should be performed, as Dr. Wickham Legg suggests, rather by scarification than by puncture. Surgical operations, even of the simplest kind, should be undertaken only as a last resource, and the extraction of a tooth should be expressly for bidden.

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