Restrictive dermopathy.

Restrictive dermopathy is an inherited syndrome characterized clinically by severe growth retardation, abnormal skin, characteristic facies, and multiple congenital contractures. Distinctive radiologic features include deficient mineralization of the clavicles and the skull, overtubulation and frequent modeling defects of the long bones, and occasional abnormalities of the ribs and scapulae.

A confocal microscopic evaluation of the interface between Syntac adhesive and tooth tissue.

The aim of this study was to microscopically evaluate the effect of dentine smear layer thickness, tubular orientation and immediate stress application on a modern dentine bonding agent. Eighty mesial and distal wedge-shaped cavities were cut into dentine/cementum cervically in 40 extracted human lower third molars. The thickness of the dentine smear layer was reduced by polishing the cavity surface in half the samples. Each component of the bonding agent (Syntac: Ivoclar Vivadent) was labelled with a fluorescent dye, the unfilled resin being light cured for 30 s with the composite restoration placed in one increment and light cured for 40 s. The samples were longitudinally sectioned using a slow speed diamond saw underwater, either immediately or 24 h post placement. The sectioned surfaces were then viewed using a confocal optical microscope. The thickness of the smeared layer only effected the penetration of adhesive in dentine tubules which were separated from the pulp chamber by the cavity design. These areas were well filled with adhesive; however, areas communicating with the pulp chamber showed no penetration differences due to smear layer reduction by polishing. The interdependence of the adhesive components was illustrated by failure to achieve a well-impregnated tooth/adhesive hybrid layer when the materials were incorrectly handled. The Syntac/dentine interface was generally able to withstand the stress of sectioning immediately post placement, but showed signs of failure in the cavity line angle when the applied stresses were greatly increased.(ABSTRACT TRUNCATED AT 250 WORDS)

The radiology of juvenile rheumatoid arthritis. A review of the English language literature.

The radiologic abnormalities seen in patients with juvenile rheumatoid arthritis (JRA) include disturbances of growth, various types of joint destruction, abnormalities of bone density, periostitis, and soft tissue abnormalities. We review the English language literature, which deals with the radiologic abnormalities in general, and at specific sites. We also review briefly radiologic abnormalities seen in other organ systems. The role of the other imaging modalities in the assessment of JRA is discussed.

A bullseye display of regional perfusion of the brain.

The authors submit a method of viewing Tc-99m HMPAO SPECT images of the brain based on the principles of the myocardial bullseye or polar mapping. Commercially available software for the production of a myocardial bullseye display is suitable for the preparation of a bullseye display of regional perfusion image of the brain. This display facilitates the detection of cerebral cortical perfusion defects by displaying all SPECT oblique slices concentrically from base to vertex in a single image.

Physeal involvement in chronic recurrent multifocal osteomyelitis.

Chronic recurrent multifocal osteomyelitis is a recently recognized disease characterized by remissions and exacerbations of multiple bone lesions which radiographically and pathologically have the appearance of hematogenous osteomyelitis. No consistent etiology can be identified, and antimicrobial agents seem to have no beneficial effect. A review of the appearances on imaging modalities and the clinical and pathologic manifestations is undertaken in seven cases of CRMO. No therapeutic regimen resulted in consistent clinical or radiographic improvement. We suggest the sequelae of this process are not as benign as previously reported.

John Caffey Award. Intussusception reduction in children by rectal insufflation of air.

Air was used as the contrast medium for colonic studies in 282 patients with suspected intussusception. Intussusception was documented in 118 of these patients (42%), and reduction was achieved in 89 (75%) of those 118. Twenty-nine patients, in whom the intussusception could not be reduced with air, underwent surgery. Seven of these patients required surgical resection of devitalized bowel. In seven others, the intussusception was reduced by the surgeon with difficulty; in 12, surgical reduction was achieved easily. Three perforations occurred with the use of air. These patients were treated surgically without sequelae. This pilot study of 282 patients suggests that air is a safe substitute for hydrostatic reduction of intussusception.

Chronic recurrent multifocal osteomyelitis and psoriasis--a report of a new association and review of related disorders.

In summary, we have described two patients with CRMO and psoriasis, and have reviewed the musculoskeletal manifestations associated with pustular eruptions of the palms and soles. In view of the frequent occurrence of PPP in patients with CRMO, we suggest that the occurrence of psoriasis in our two patients is more than coincidence, and that noninfectious, inflammatory lesions of bone may be another musculoskeletal manifestation of psoriasis. This rare association, as well as the association of PPP with disorders associated with new bone formation, may shed new insights on the relatively common finding of periosteal elevation associated with psoriatic arthritis and the occasional severe juxta-articular osteolytic destructive bone lesions seen in psoriatic arthritis.

Idiopathic periosteal hyperostosis with dysproteinemia (Goldbloom's syndrome): case report and review of the literature.

Idiopathic subperiosteal new bone formation is uncommon in childhood. We report a case of diffuse periostitis in association with hypergammaglobulinemia and hypoalbuminemia in a 12-year-old boy. In 1966, 2 cases of idiopathic periosteal hyperostosis with accompanying dysproteinemia were documented. This report serves as a review of this unique and perplexing problem in clinical diagnosis.