The duration of this tendency, and its perpetuation in a family, is remarkable. The Appleton-Swain family of Reading, Mass., has shown examples for two centuries. Osler has been advised of instances already occurring in the seventh generation. Kolster has investigated hemophilia in women, and reports a case of bleeding in the daughter of a hemophilic woman. He also analyzes 50 genealogic trees of hemophilic families, and remarks that Nasse's law of transmission does not hold true. In 14 cases the transmission was direct from the father to the child, and in 11cases it was direct from the mother to the infant.
The hemorrhagic symptoms of bleeders may be divided into external bleedings, either spontaneous or traumatic; interstitial bleedings, petechiae, and ecchymoses; and the joint-affections.
The external bleedings are seldom spontaneous, and generally follow cuts, bruises, scratches, and often result seriously. Aminor operation on a hemophile may end in death; so slight an operation as drawing a tooth has been followed by the most disastrous consequences.
Armstrong, Blagden, and Roberts, have seen fatal hemorrhage after the extraction of teeth. MacCormac observed five bleeders at St.
Thomas Hospital, London, and remarks that one of these persons bled twelve days after a tooth-extraction. Buchanan and Clay cite similar instances. Cousins mentions an individual of hemorrhagic diathesis who succumbed to extensive extravasation of blood at the base of the brain, following a slight fall during an epileptic convulsion. Dunlape reports a case of hemorrhagic diathesis, following suppression of the catamenia, attended by vicarious hemorrhage from the gums, which terminated fatally.
Erichsenf cites an instance of extravasation of blood into the calf of the leg of an individual of hemophilic tendencies. Acavity was opened, which extended from above the knee to the heel; the clots were removed, and cautery applied to check the bleeding. There was extension of the blood-cavity to the thigh, with edema and incipient gangrene, necessitating amputation of the thigh, with a fatal termination.
Mackenzie reports an instance of hemophilic purpura of the retina, followed by death. Harkin gives an account of vicarious bleeding from the under lip in a woman of thirty-eight. The hemorrhage occurred at every meal and lasted ten minutes. There is no evidence that the woman was of hemophilic descent.
Of 334 cases of bleeding in hemophilia collected by Grandidier, 169 were from the nose, 43 from the mouth, 15 from the stomach, 36 from the bowels, 16 from the urethra, 17 from the lungs, and a few from the skin of the head, eyelids, scrotum, navel, tongue, finger-tips, vulva, and external ear. Osler remarks that Professor Agnew knew of a case of a bleeder who had always bled from cuts and bruises above the neck, never from those below. The joint-affections closely resemble acute rheumatism. Bleeders do not necessarily die of their early bleedings, some living to old age. Oliver Appleton, the first reported American bleeder, died at an advanced age, owing to hemorrhage from a bed-sore and from the urethra. Fortunately the functions of menstruation and parturition are not seriously interfered with in hemophilia.
Menstruation is never so excessive as to be fatal. Grandidier states that of 152 boy subjects 81 died before the termination of the seventh year. Hemophilia is rarely fatal in the first year.
Of the hemorrhagic diseases of the new-born three are worthy of note. In syphilis haemorrhagica neonatorum the child may be born healthy, or just after birth there may appear extensive cutaneous extravasations with bleeding from the mucous surfaces and from the navel; the child may become deeply jaundiced. Postmortem examination shows extensive extravasations into the internal viscera, and also organic syphilitic lesions.
Winckel's disease, or epidemic hemoglobinuria, is a very fatal affection, sometimes epidemic in lying-in institutions; it develops about the fourth day after birth. The principal symptom is hematogenous icterus with cyanosis,--the urine contains blood and blood-coloring matter. Some cases have shown in a marked degree acute fatty degeneration of the internal organs--Buhl's disease.
Apart from the common visceral hemorrhages, the results of injuries at birth, bleeding from one or more of the surfaces is a not uncommon event in the new-born, particularly in hospital-practice. According to Osler Townsend reports 45 cases in 6700 deliveries, the hemorrhage being both general and from the navel alone. Bleeding also occurs from the bowels, stomach, and mouth, generally beginning in the first week, but in rare instances it is delayed to the second or third. Out of 50 cases collected by Townsend 31 died and 19 recovered. The nature of the disease is unknown, and postmortem examination reveals no pathologic changes, although the general and not local nature of the affection, its self-limited character, the presence of fever, and the greater prevalence of the disease in hospitals, suggest an infectious origin (Townsend). Kent a speaks of a new-born infant dying of spontaneous hemorrhage from about the hips.
Infantile scurvy, or Barlow's disease, has lately attracted marked attention, and is interesting for the numerous extravasations and spontaneous hemorrhages which are associated with it. A most interesting collection of specimens taken from the victims of Barlow's disease were shown in London in 1895.
