Structural Characterization of Variant Forms of Arylsulfatase A that Associate with Alcoholism

Several electrophoretic forms of human platelet arylsulfatase A (ASA), including variant type III_a and normal type IV_a, have been identified by nondenaturing polyacrylamide gel electrophoresis. An alcoholic population that we have analyzed is enriched in variant type III_a compared with nonalcoholic psychiatric and normal controls. Individuals with the III_a enzyme possess greatly reduced levels of ASA activity. To understand further the structural basis for the differences and their potential biological consequences, the nature of the ASA variant expressed by fibroblasts from different individuals was explored. The electrophoretic patterns of fibroblast ASA from the III_a and IV_a individuals differ in degree of phosphorylation. Furthermore, fibroblast ASA from III_a individuals lacks an N -linked glycan found in ASA from IV_a individuals. In addition, differences in peptide and/or posttranslational modification unrelated to the N -linked carbohydrate or phosphorylation exist between the fibroblast ASA from III_a and IV_a individuals. The finding that both fibroblasts and platelets exhibit related electrophoretic isoform patterns characteristic of the donor's ASA type allows for the use of fibroblasts to study the impact of ethanol on the metabolism of cells possessing different ASA types.     

Some practical perspectives for educating gifted children

This article identifies common characteristics of educationally related programs that form a common basis for understanding and working with gifted programs. Special approaches and programs for educational enrichment as well as specific activities that have been successful are discussed.     

CD4-specific monoclonal antibody can prolong cardiac allograft survival without T-cell depletion

Current immunosuppressive regimens for clinical transplantation are immunologically non-specific, are associated with acute and chronic toxic side-effects [1] and are unable to prevent chronic graft loss in a significant proportion of patients. Additionally, new and increasingly powerful drugs are being introduced to induce non-specific immunosuppression, and therefore this is likely to be followed by an increase in related complications such as the induction of cancers. Hence, there is a need for an alternative approach. It has been shown that long-term survival of murine cardiac grafts can be induced by the monoclonal antibody YIS 191 that depletes CD4 ⁺T cells in vivo [2]. In this study, we have investigated the ability of a non-depleting antibody to produce better graft survival.     

Inhibition of mediator release in systemic mastocytosis is associated with reversal of bone changes

A 59-year-old male presented with systemic mastocytosis with extensive skeletal involvement resulting in vertebral compression fractures and bone pain. Histomorphometric analysis of bone revealed increased mast cells, elevated static parameters of bone resorption, and low bone formation. Serum calcium, phosphorus, and alkaline phosphatase were normal; however, serum 1,25-dihydroxyvitamin D3 and osteocalcin levels were low. Histamine levels in plasma and urine were elevated. Following therapy with ketotifen, the patient had resolution of bone pain along with decreased flushing and pruritus. Elevated plasma and urine histamine levels normalized, as did 1,25-dihydroxyvitamin D3 and osteocalcin levels. Indices of low bone formation improved on therapy. Eroded surfaces improved but remained elevated. This case is the first demonstration that bone symptoms and histomorphometric change in systemic mastocytosis are reversed with inhibition of mast cell degranulation. The role of mast cells and their products in bone metabolism is poorly understood, but the therapy of bone disease in systemic mastocytosis should include inhibition of the release of mast cell products along with the use of histamine antagonist.     

Subarachnoid injection as a complication of retrobulbar anesthesia

We studied eight cases of apparent subarachnoid injection of local anesthetic through the optic nerve sheath and subsequent spread to the parabrainstem cisterns and the contralateral optic nerve sheath. The patients all experienced bilateral decrease in vision and ophthalmoplegia, and variable degrees of central nervous system symptoms and respiratory depression. The complications occurred with a variety of local anesthetic agents and with sharp disposable needles in six of the eight cases. These cases demonstrated the spectrum of signs and symptoms that may follow optic nerve sheath injection of local anesthetic agents.     

Separation of internal derangements of the temporomandibular joint using sound analysis

This clinical-arthrographic investigation compares power spectral analysis of temporomandibular joint sounds with different arthrographic characteristics of intracapsular dysfunction. The quantitative comparison of specific parameters of the sound power-spectrum waveform may provide a sensitive and accurate noninvasive tool for diagnosis and treatment.