HEMOPHILIA 



429 



the mystery of the transmission of the disease in a hemophilic family, why the 

 mother herself should remain unaffected while she transmits the disease, why 

 the male members of the family only are attacked, why men from bleeder 

 families do not beget hemophilic children, whether they themselves are bleeders 

 or not, etc. Moreover, congenital hemophilia is much rarer than that form 

 in which children in the second year or adolescents manifest the signs of the 

 disease ! Are the pathogenic agents latent in such cases ? And what causes 

 them to assert their virulence ? 



In rare cases mothers with relapsing fever have given birth to children 

 with relapsing fever! This is probably analogous to the "toxic infection" 

 which Koch assumes as the cause of congenital hemophilia. 



In the last few years, Kolb, Babes, Gartner, Tizzoni and Giovannini have 

 demonstrated bacteria in patients suffering from purpura hemorrhagica as 

 well as in hemophilia of the newborn; these bacilli were apparently also 

 pathogenic in animals, and upon inoculation produced a disease characterized 

 by hemorrhages. These maladies are probably analogous to the hemorrhages 

 often seen in other infections, and we may assume these hemorrhages to be due 

 partly to local changes in the vessels caused by collections of bacteria, and 

 partly to the toxic efEect of bacterial products. These may, therefore, to some 

 extent be classed with toxic hemorrhages, but have not the siightest element 

 in common with human hemophilia. 



That with the hemophilic predisposition severe or even uncontrollable 

 hemorrhages follow trifling occasional causes such as never produce hemorrhage 

 in the healthy is a generally well-known fact, and this has led to a differentia- 

 tion between spontaneous and traumatic hemorrhage. But obviously in this 

 division the conception of " trauma " must be very elastic. The hemophilic 

 tendency to hemorrhage shows itself during the period of physiologic develop- 

 ment, particularly in the two periods of dentition, at puberty, and at the 

 climacterium. Even in tying and severing the umbilical cord we sometimes 

 produce severe, even fatal hemorrhage, and we recognize by this and other 

 signs the hemophilic predisposition of the newborn. 



Severe hemorrhages from the gums are prone to occur in the first period 

 of dentition, and we are forced to look upon the cutting of the teeth as trauma. 

 Quite slight corporeal punishment may produce cutaneous hemorrhages and 

 infiltrations of blood out of all proportion to the nature and extent of the 

 injury. Blowing the nose may cause uncontrollable epistaxis; profuse con- 

 junctival hemorrhages may be produced by touching the eye. Purgatives may 

 result in severe intestinal hemorrhages. At the menstrual period and in 

 normal labor extraordinarily severe hemorrhages have been observed. 



Especially frequent are effusions of blood into the Joints. 



From the observations of surgeons it is evident that the first hemorrhages 

 into the joints, as well as also the great majority of the later ones, are caused 

 by external conditions. This explains why children are not attacked by hemor- 

 rhages into the joints before they learn to walk; the "trauma" is absent. 

 But when they first begin to walk, when the joints are utilized and the entire 

 muscular system comes into action, trauma after trauma occurs, and arthritic 

 hemorrhages are apt to appear. We quite often observe severe hemorrhages 



