Page:Encyclopædia Britannica, Ninth Edition, v. 18.djvu/397

 PATHOLOGY 375 itself for some time after birth. It is highly probable that the placental structure and function suffer under many less special con ditions of ill-health and mal-iiutrition of the mother. The placenta is, in fact, a great formative effort, and the formative power cannot always be adequate. There are in particular two conditions in the mother favourable to rickets in the child, in each of which an absence of structural and functional perfection in the new-formed organ of intra-uterine nutrition is a priori probable. The one is the extreme youth or immaturity of the mother, assigned by Schb nlein as the chief cause of rickets ; the other is child-bearing up to a comparatively late period, the latest of a succession of pregnancies being often found to be those which yield the rickety members of a family. But amongst the poor there must be many other causes of general ill - health in the mother operating from time to time. Whatever makes the mother s milk poor cannot but have told at an earlier stage upon the placental structure and function ; and that earlier stage is a vastly more critical time for the endowments of the child, for all its formative, nutritive, and functional tendencies. Mollities Ostcomalacia. A sort of counterpart to rickets occurs in the ossium. disease known as osteomalacia or mollities ossium ; and, curiously enough, this is a disease (as distinguished from senile softening) almost exclusively of women during mature life, apt to occur in the gravid state, and especially if there have been repeated pregnancies. It is mostly a disease of poor and hard -worked women, just as rickets is a disease of the children of poor and hard-worked women ; it is not very common, although it is said to be endemic in some localities. The bones become soft or friable, owing to the encroach ment of the medullary cavity upon the compact substance and the further absorption of spongy bone ; the encroachment may be so extensive that only a thin shell of bone or parchment-like mem brane remains. This enormous medullary space is filled with marrow, but not the marrow of adult life. The marrow is of the fcetal kind, red, and often containing areas of blood, abounding in nucleated marrow-cells, and with a decreasing number of fat- cells. Ultimately the marrow becomes gelatinous. The process consists essentially of a reduction of the bone to red marrow, as in the first formation of the medullary cavities of long bones ; the earthy salts are removed, and all the cells of the tissue acquire an embryonic character. Although there are some facts to show that this process takes place sometimes in the young, especially in young animals under confinement, yet its characteristic occurrence is in women during one of their later pregnancies. It is generally admitted that there is some intimate connexion between the out break of mollities ossium and the gravid state. We have found reason to conclude that there is an equally intimate connexion between rickets and the gravid state, only that the rickets is in the child. If, in rickets, the child is deprived of something maternal which it should have received, then in osteomalacia the mother parts with something for the child which she ought to have kept. In both cases the organism of the mother is overtaxed ; but in the more general case, where the child becomes rickety, the tax has not been met. In the rarer case, the welfare of the child in utero takes precedence of the welfare of the parent ; one may conceive that the formative effort for the placenta had been so great that the organism in genei al was impoverished. As a matter of fact, the bones of the mother are robbed of their earthy matter, and the commencement, at least, of that diversion of substance is somehow connected with the gravid state. It is noteworthy, in this con nexion, that a fractured bone in a pregnant woman repairs badly, owing to the deficient production of bony callus. Having once begun, the disease progresses, and the patient dies bedridden ; only in rare instances do the bones become hard again. The loss of osseous matter in mollities is accompanied by a return to em bryonic characters and function on the part of all the cells that now form the very extensive marrow ; the hffiinatoblastic function is conspicuous in the process, and there are also numerous rnyelo- plaxes. Both the unmaking of bone in the parent and the diversion of embryonic tissue from bone-making in the child would appear to be correlated with the hrcmatoblastic function of the cells. In both diseases phosphates are discharged in excess in the urine, and in neither is there any advantage from the excessive formation of blood. In osteomalacia the embryonic state of the marrow changes after a time to a more gelatinous state ; sometimes a wall forms round the red pulpy fluid, producing a cyst of the bone with brownish contents, and in these cases the disease is said not to progress farther. Cretin- Cretinism. A much more profound error or defect of all the ^m. developmental powers of the body than that of rickets is found in cretinism. Certain aspects of this subject have already been treated of in the articles CRETINISM and INSANITY ; and another aspect of it is referred to in the section of this article dealing with the thyroid gland (see p. 385). It remains to mention here a few of the anatomical and external characters of the disease. With the low mental development there usually go a large tongue, a broad and flat nose, loose and thick skin, and stunted limbs. The error of growth in the bones, which is only a part of a very extensive range of erroneous development, is somewhat different from that of rickets. In the bones of the skull there is usually found synostosis, or premature union at one suture or another, not unfrequently at the sphenobasilar, giving the base of the skull an up-and-down direction. The premature union along one line or other leads to compensating expansion elsewhere, so that the skull is misshapen ; the forehead usually retreats, the top of the head is flat, and the occiput small, the type of skull being markedly brachycephalic or broad. One distinctive point in the bone-lesions of cretinism relates to the stunted limbs, which are not at all characteristic of rickets. The stunted growth depends upon a complete departure from the ordinary relation of the epiphysis to the shaft. A bone such as the thigh-bone grows normally to the length, chiefly by the activity of the cartilage of the epiphysis along the epiphysial line : the carti lage-cells multiply on the surface of the epiphysis next to the shaft ; they become grouped in long perpendicular columns ; and, as ossi fication proceeds, the new bone becomes an integral part of the shaft. Meanwhile the epiphysis itself is becoming ossified radially from the centre outwards. In the cretin the activity along the epiphysial line is somehow checked, and it has been found that a fibrous band extending inwards from the periosteum forms a kind of barrier in the position of the proliferating epiphysial line, cutting off the shaft from the epiphysis ; thus the shaft is deprived of those accretions at each end upon which its elongation mainly depends. At the same time the cartilaginous epiphysis spends its proliferative force within itself ; it expands in all directions, becoming a large knob, and part of its ossification may be effected by a sort of in verted activity of the epiphysial line, which proliferates towards the interior of the epiphysis, instead of growing towards the con tiguous shaft. No analysis of these peculiarities of bone-growth in cretins need be attempted, but some remarks are offered on p. 385 with reference to the mother s share in this congenital condition. Chlorosis. Contrasting with rickets, in which the tendency Chlor- born with the child produces symptoms of ill-health in children of osis. both sexes within the first year, and seldom later than the second, chlorosis is a congenital condition of which there are symptoms first at the age of puberty, and almost exclusively in the female sex. The congenital nature of this condition has been made prob able by the anatomical observations of Virchow, which go to show that in chlorotic subjects there is very uniformly found a narrow or inadequate aorta, much more elastic than usual, with its inner coat irregular in thickness and disposed to degenerative changes, and with its intercostal branches coming off in a more than ordi narily irregular manner. These anatomical peculiarities are natur ally part of the congenital endowment of the individual. The full force of the chlorotic state is not felt until the time of puberty, and in the male sex it is hardly felt at all. It is, indeed, associated in the most intimate way with the remarkable periodicity of ovu- lation to which the female sex is subject ; it manifests itself in the years when that function begins, and chiefly at each successive period of the function. After a few years the indications of it become feebler and tend to disappear. Want of sunlight in the daily life of the individual is the chief aggravating circumstance of the anaemia of chlorosis. The vascular system is on a small scale, to begin with, and there is too much blood in the body for the size of the vessels ; the blood is not quite normally constituted, having too few corpuscles in proportion to the plasma, and in the red disks there is too little haemoglobin or colouring matter. While the blood and blood-vessels are poor, the fat of the body, and especially the subcutaneous, is abundant. Haemophilia. This is another general state of the vascular sys tem, which is always congenital, and often runs in families, one or more of whose members are &quot; bleeders.&quot; It is a disorder of the boys of a family just as distinctively as chlorosis is a disorder of the girls. A remarkable disposition to bleed, with or without the pro vocation of an injury, is the whole disease ; neither structural change of the blood-vessels nor peculiar composition of the blood has been made out, and there is nothing remarkable in the ordinary appear ance of a bleeder. When the bleeding is spontaneous it comes from the mucous membranes, especially from the nose, but also from the mouth, bowel, and bronchial tubes ; one of the most common and fatal traumatic occasions of bleeding is the extraction of a tooth. Even slight bruises are very apt to be followed by extrava sations of blood into the tissues ; the swollen joints (knee especially) of a bleeder are probably due, in the first instance, to the escape of blood into the joint-cavity or into the synovial membrane. It is always from the very smallest vessels that the blood escapes, and from these it may escape in such quantities as to cause death within a few hours. It appears that the same extensive capillary haemor rhage may occur anywhere in the body provided the opportunity is furnished, by a slight injury or otherwise, for the blood to escape. 1 1 Literature. Of rickets : W. Jenner, Med. Times and Gaz., 1860, vol. i. ; Virchow, Cellular- Pathologic, 4th ed., 1871, chap. xx. (also in his Archiv, vol. v., 1851) ; Kassowitz, Die normale Ossification und die Erkrankungen des Knochensystems bei Rachitis und hereditarer Sy2)hiiis, Vienna, 1883; Id., in summary, in Trans. Internat. Med. Congress, vol. iv. p. 45, Lond., 1881 ; J. Guerin, Memoires sur les