PAGETʼS DISEASE (OSTEITIS DEFORMANS)

Abstract

The well-known association between phosphorus, carbohydrate metabolism and bone development led us to study a series of cases of Paget's disease. This series of cases, though small, shows certain significant facts which it is hoped will stimulate others to study their cases along similar lines. Hunter (1) says that compounds take part in at least four important metabolic processes. They are in some way essential to the storage or utilization of carbohydrate, they enter into the chemical changes which precede the contraction of muscle, they are concerned with the acidbase equilibrium and they are essential to the deposition of bone.'' It has been known for some time that phosphorus is related to both carbohydrate metabolism and bone formation. In osseous dystrophies such as Paget's disease it should be possible to demonstrate certain characteristics pointing to involvement of carbohydrate and phosphorus metabolism. We were particularly interested from the standpoint of constitutional inheritance showing a disturbance in carbohydrate metabolism and bone formation. On the following page is a table giving the salient points. An analysis of this clinical data shows that of twelve patients with Paget's disease, five, or 41 plus per cent, give a family history of diabetes mellitus. In two patients (Nos. 8 and 9) there was more than one member of the family afflicted with diabetes. In patient 8 both parents died of diabetes and one brother, still living, has diabetes.* A sister had had a parathyroidectomy for generalized osteoporosis. The patient had a giant cell sarcoma of the antrum, beginning, no doubt, in the ethmoid, removed twelve years previously. Patient 9 had two brothers and two sisters who died of diabetes; the latter also had goiters. In regard to heights, it will be seen that twelve, or 100 per cent, had at least one member of the family 70 inches or more in height. In eleven families one or more members was 71 inches in height. In eight families one or more members was 72 inches in height. In patient 1 all members, both male and female (total four), were 71 inches in height. In five families in which the weight was obtained one or more members weighed 200 pounds or more. Two of the twelve patients, or 16 plus per cent, had uterine leiomyomata. Interestingly enough, one of us (R. C. M.) has a series of one hundred patients with uterine leiomyomata in which the incidence of familial