Salaried physicians' intent to retain hospital membership: the effects of position and work attitudes

This study examined the anticipation of salaried hospital physicians in Israel to retain hospital membership for a long term until their retirement. Examined are attitudinal and position factors, as well as hospital standing personal and situational factors that relate to this anticipation. The data collected from 195 full-time salaried physicians in Israeli hospitals indicated that about one-third of the physicians anticipate to retain hospital membership until retirement. It was found that factors reflecting the physician's standing within the hospital were the main predictors of this anticipation, whereas work-related attitudes had little or no effect on it. The implications of these finding to hospital-physician integration strategies in the United States are discussed.     

Perinatal lethal hypophosphatasia; Clinical,radiologic and morphologic findings

Clinical, radiographic and morphologic analysis of nineteen cases of perinatal (lethal) hypophosphatasia was performed. Three families each had two affected offspring. All of the patients had lethal short limb dwarfism with very soft calvaria. Other clinical findings included polyhydramnios, blue sclerae and spurs in the mid-portion of the forearms and lower legs. Considerable variability was found in the skeletal radiographs. In addition to the well known radiographic features such as generalized decrease in the size of ossified bones with some bones not ossified at all, other changes observed included: 1) marked variability in the amount of bone ossification; 2) variability between patients as to which bones were most severely affected; 3) unusually dense, round, flattened, butterfly shaped; and saggitally clefted vertebral bodies; 4) variability in femoral shape including chromosome like, campomelic like, and shortening with or without metaphyseal cupping or irregularities; 5) osteochondral projections (Bowdler spurs) of the midshaft of the fibula and ulna. Recognition of the marked clinical and radiographic variability in this autosomal recessive lethal skeletal dysplasia is important for accurate genetic counseling and prenatal diagnosis.     

Pseudodiastrophic dysplasia: a distinct newborn skeletal dysplasia

Pseudodiastrophic dysplasia is a distinct disorder that differs from diastrophic dysplasia on the basis of clinical, radiographic, and chondro-osseous histopathologic findings. In addition to the rhizomelic shortening of the limbs and severe clubfoot deformity, which suggest the diagnosis of diastrophic dysplasia, distinguishing features are elbow and proximal interphalangeal joint dislocations, platyspondyly, and scoliosis, which are observed in infancy. This disorder has been reported previously in three infants, all of whom died in the first year of life. Two of these were sisters, suggesting autosomal recessive inheritance. We report four new patients with this distinct skeletal dysplasia, including two children now older than 4 years of age. In both of these patients neonatal contractures have improved with physical therapy and scoliosis has progressed significantly.     

Infective endocarditis in the billowing mitral leaflet syndrome.

Ten patients with the billowing mitral leaflet syndrome complicated by infective endocarditis are reported. Two patients had a non-ejection systolic click and 8 had both a non-ejection systolic click and a late systolic murmur. These auscultatory features were difficult to detect in 4 instances in that they were intermittent, soft, or brought out only with postural change. Seven patients were unaware of their cardiac lesions. A low grade pyrexia was present in all 10 patients. Four patients presented with clinical features caused by reversible neurological lesions. Blood cultures were positive in all patients, with Staphylococcus albus the infecting organism in 6. Antibiotic therapy was successful with significant mitral regurgitation supervening in only one instance. The importance of the billowing leaflet as a potential site of infective endocarditis is emphasized. It seems that antibiotic prophylaxis is indicated at times of increased risk of infection in subjects with a non-ejection systolic click or a late systolic murmur.     

Multiple vertebral segmentation defects: Analysis of 26 new patients and review of the literature

To further delineate and classify those forms of short trunk dwarfism characterized by multiple vertebral segmentation defects, we analyzed 26 new patients and reviewed 115 described in the literature. Three distinct entities were recognized based on radio-graphic and clinical findings. Jarcho-Levin syndrome is the lethal autosomal recessive form, characterized by a symmetric crablike chest. Spondylocostal dysostosis is the benign autosomal dominant condition. Spondylothoracic dysostosis shows considerable clinical and radiographic overlap with spondylocostal dysostosis. Malformations observed in association with multiple vertebral segmentation defects are more common in the sporadic patients. Analysis of the 26 new individuals revealed that the body segment in which these nonvertebral malformations occur corresponds to the site of the vertebral segmentation defects. © 1996 Wiley-Liss, Inc.