HEMOPHILIA. — Gr., ociuu, blood, and 02,Eiv, to love.
Definition.—Ilinmophilia is an in herited or acquired disorder of the vas cular system characterized by an abnor mal liability to severe and sometimes uncontrollable haemorrhages.
Symptoms.—The condition is gener ally discovered by accident, a slight wound, the extraction of a tooth, the application of a leech, vaccination, etc., being followed by profuse and sometimes dangerous bleeding. Epistaxis is of fre quent occurrence. In women this is es pecially the case, because the hternophilic process mainly manifests itself through the mucous membranes, and menor rhagia, metrorrhagia, post - partum hemorrhage, etc., are often suffered from.
Pregnancy and labor do not present the danger for an Inemophilic that might be supposed. Of 130 eases, the death of the mother occurred only in 3 and abortion in only 1(1 cases. R. Kolster (Wratsch, No. 28, '95).
Case of a girl, aged 11 months, in whom from the third week of life there had been continuous and spontaneous halnorrhages from the nose, mouth, and rectum, and into the substance of the skin, without, however, impairing in any manifest way the child's development. There was a history on the father's side of hemophilia, but not ou the maternal side. The patient was the only living one of their children; there had been two other girls who had died in infancy, but had not shown any signs of hemo philia. Comby (Bull. et Diem. de la Soc. des Hop.. June 25, '97).
Patients subject to hamlophilia con stantly exhibit mental peculiarities of definite form. The most important and the most common mental peculiarity is an inability (it is more than unwilling ness) to tell the truth about their condi tion. even when they have had repeated and alarming experience of their defect. Frequently they will persist in obstinate denial of their liability to bleed, even when the hmmorrhage is going on, and resisting all efforts to check it. C. T. Dent (Brit. Med. Jour., Apr. 23, '98).
In men arthritic symptoms are fre quently observed, especially during cold and damp weather, the knees being most prone to pseudorheumatic manifesta tions which are sometimes accompanied by fever. The joint-symptoms are often the precursor of an approaching luemor rhage.
lifemopliilia does not always manifest itself by external luemorrhage. The arthritic complications of haemophilia are generally the result of hannorrhage into the joint-cavities, but there is no antagonism between hasmophilia and articular rheumatism. Frederick S. Eve (Lancet, Nov. 16, 'SO).
Three stages described of joint-troubles in lia‘mophilia: (1) the stage of hrem arthrosis; (2) stage of inflammation; (3) stage of retrogressive changes with deformity. The painless, sudden onset in pale young men marks the first stage. Themorrhages in the skin would com plete the diagnosis. The second stage is strikingly similar to the white swelling of tuberculous arthritis. The author has three times made a mistaken diagnosis, two of the three cases having suffered death in consequence, from hremorrhage after operation; the third recovered.
Koenig (LEncephale, June 25, '92).
Etiology.—According to Henry, hem ophilia is the most hereditary of all diseases. Although a fixed law, such as Nasse's—namely, that transmission of the disease through females that are themselves not Imnophilic—cannot be accepted as universal, 30 per cent. of the cases studied by holster, for instance, were found to be governed by this pecul iar form of heredity.
The most remarkable family of bleed ers of which we have any account is the one living in Telma, Switzerland. It springs from a couple of the same name, presumably relatives. who lived nearly three hundred years ago, and during this period there have been bleeders among the male descendants. The females, as a rule, remain exempt, the disease being transmitted through them to their male children. Anton Hoessli (Zeit. f. klin. Med., B. 15, II. 3, 'SS).