Lester W. Paul

Hereditary Multiple Diaphyseal Sclerosis (Ribbing)

In 1949 Ribbing (3) described a peculiar form of diaphyseal sclerosis which he had observed in four of six siblings and which differed in some respects from any lesions previously reported. He was unable to determine the cause or nature of the condition and gave it the name of hereditary multiple diaphyseal sclerosis. We have observed two examples, occurring in brothers, of a type of sclerosis resembling that described by Ribbing. Since this appears to be an unusual form of bone disease or dysplasia we are reporting our cases at this time. The essential finding in this disease consists in a fusiform thickening of a portion of the diaphysis of a long bone. The involved area is extremely dense and the cancellous structure largely or completely obliterated. The lesions have been quite similar in their roentgen appearances, varying only in the length of the involved area and the intensity of the sclerosis. There is a tendency toward symmetrical changes and to multiplicity of bones involved, with the tibia and...

ROENTGEN OBSERVATIONS IN FAMILIAL METAPHYSEAL DYSOSTOSIS.

Metaphyseal dysostosis is a disease with important genetic implications, characterized by selectively disturbed endochondral bone formation leading to reduced stature. As in achondroplasia and other conditions simulating it, the extremities are chiefly affected, resulting in a disproportionate dwarfing which spares the trunk. Roentgen investigation often yields appearances easily confused with other diseases affecting bone development. Calcium and phosphorus metabolism and osteoblastic activity are normal, yet radiographic similarities to resistant rickets and other metabolic abnormalities have led to mistaken diagnoses and treatment with high doses of vitamin D without benefit and occasionally with some unnecessary complications (2). It is apparent, therefore, that awareness of the disease spectrum is important for accurate roentgen interpretation. We have recently had the opportunity of studying several patients and families affected by this condition. Some unique features observed form the basis of thi...

Pulmonary Adenomatosis: Further Roentgen Observations

There continues to be disagreement as to the place of pulmonary adenomatosis in the classification of neoplasms, the correct nomenclature to be employed, the origin of the basic cell, and even the existence of the condition as a separate entity. Nevertheless, reports on the disease have been appearing with increasing frequency, and further information regarding its nature, clinical course, incidence, and related features has been accumulating. Our discussion will be concerned mainly with the clinical and roentgenologic aspects based on personal observation of 8 cases (4 reported previously and 4 new ones), together with a review of some of the pertinent information to be found in the literature. In 1945, Paul and Ritchie (7) reported 4 cases of pulmonary adenomatosis and discussed the general features of this relatively uncommon disease in so far as they were then known. At that time the condition had been of interest mainly to pathologists, since the diagnosis had not been made during life (except in 1 c...