The rheumatic diseases in adolescence.
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Biomedical subjects
Publications and source records attributed to V Hanson.
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Evidence is presented here that demonstrates the presence of NP185 (AP3) in neuronal cells, specifically within syn-aptic terminals of the central nervous system and in the peripheral nervous system, particularly in the neuro-muscular junction of adult chicken muscle. Biochemical results obtained in our laboratories indicate that NP185 is associated with brain synaptic vesicles, with clathrin-coated vesicles, and with the synaptosomal plasma membrane. Also, NP185 binds to tubulin and clathrin light chains and the binding is regulated by phosphorylation (Su et al., 1991). Based on these properties and the data reported here, we advance the postulate that NP185 fulfills multiple functions in synaptic terminals. One function is that of a plasma membrane docking or channel protein, another of a signaling molecule for brain vesicles to reach the synaptic terminal region, and a third is that of a recycling molecule by binding to protein components on the lipid bilayer of the synaptic plasma membrane during the process of endocytosis. In support of these premises, a thorough study of NP185 using the developing chick brain, adult mouse brain, and chicken straited muscle was begun by temporally and spatially mapping the expression and localization of NP185 in evolving and mature nerve endings. To achieve these objectives, monoclonal antibodies to NP185 were used for immunocytochemistry in tissue sections of chicken and mouse cerebella. The distribution of NP185 was compared with those of other cytoskeletal and cytoplasmic proteins of axons and synapses, namely synaptophysin, vimentin, neurofilament NF68, and the intermediate filaments of glial cells (GFAP). The data indicate that expression of NP185 temporally coincides with synaptogenesis, and that the distribution of this protein is specific for synaptic terminal buttons of the CNS and the PNS.
Solitary plasmacytoma rarely involves the larynx and trachea. This tumor caused severe laryngeal and tracheal obstruction in an adolescent female with systemic lupus erythematosus of nine years' duration. Her systemic disease had been in remission at the time she developed symptoms of laryngeal and tracheal obstruction by plasmacytoma invasion. A complete survey failed to reveal any other focus of tumor. Because steroid therapy failed to clear the plasma cell infiltrate, she was treated with irradiation after the tumor was surgically debulked endoscopically. Postirradiation biopsy showed no evidence of tumor, but the patient continued to experience moderate airway obstruction from fibrosis in the irradiated trachea. The patient has been followed, and is free of disease nine years after establishing the diagnosis.
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Thirty-nine patients with JRA were treated with sodium meclofenamate (Meclomen) during a 4-wk open-labeled, non-controlled trial. Increasing doses started at 3 mg/kg/d qid, up to 7.5 mg/kg/d, not to exceed 300 mg/d. Seven patients dropped out due to adverse side effects, and 1 from inefficacy. Efficacy analysis showed statistically significant decreases in several disease indices, in particular the duration of morning stiffness. Twenty-one patients who completed the 4-wk study entered an extended open-labeled study. Nine patients completed at least 9 months of sodium meclofenamate therapy. At 9 months, these 19 showed mean decreases in all rheumatologic disease indices measured. The drug has recently been approved by the Food and Drug Administration for use in adults.
Thirty-three patients with JRA were treated with proquazone during a 4-wk open-labeled, non-controlled trial. Increasing doses started at 400 mg/m2/d up to 800 mg/m2/d, not to exceed 960 mg/d. Oral administration was 4 times/d. One patient did not complete the study because an erythematous rash developed. Efficacy analysis showed significant decreases in the total number of active joints, the number and severity of joints with tenderness and LOM, DMS, and travel time (in all cases p less than .05). The drug appears to be similar in efficacy and safety to other NSAID that have been studied in children.