Search PubMed⌕ Search

Biomedical subjects

A Lorber

Publications and source records attributed to A Lorber.

84 records · Page 5Linked to original sources

Chrysotherapy. Suppression of immunoglobulin synthesis.

Forty-four subjects with classic or definite rheumatoid arthritis who were on individualized chrysotherapy were observed for changes in serum protein electrophoresis, immunoglobulins, and circulating lymphocyte counts. By paired variate analysis, significant declines from pretreatment values were recorded for the following--electrophoretic protein fractions: gamma, alpha-1, alpha-2, (P less than 0.05); immunoglobulins: IgM--53% (P less than 0.001), IgG--37% (P less than 0.01), IgA--34% (P less than 0.001). Rheumatoid factor decreased in 29 of 39 subjects, 15 becoming seronegative (P less than 0.001); circulating lymphocytes decreased by 27% (P less than 0.001). The maximal suppressive effect on IgG and IgM was not achieved until the third and fourth years of therapy by sustained weekly administration of gold sodium thiomalate (one year cumulative dosage, mean 2106 mg, range 1065-2,885; greater than or equal to 4 year cumulative dosage, mean 8747 mg, range 5,385-15,160 mg). An immunosuppressive effect is suggested by these results.

Adult↗

Cardiac manifestations in thiamine-responsive megaloblastic anemia syndrome.

Thiamine-responsive megaloblastic anemia (TRMA) syndrome is a rare autosomal recessive disorder defined by the occurrence of megaloblastic anemia, diabetes mellitus, and sensorineural deafness, responding in varying degrees to thiamine treatment. Other features of this syndrome gradually develop. We describe three TRMA patients with heart rhythm abnormalities and structural cardiac anomalies. Eight other reported TRMA patients also had cardiac anomalies. Recently, the TRMA gene, SLC19A2, was identified, encoding a functional thiamine transporter. Characterization of the metabolic defect of TRMA may shed light on the role of thiamine in common cardiac abnormalities.

Adolescent↗

Percutaneous transaortic occlusion of patent ductus arteriosus using a new versatile angiographic and delivery catheter.

We evaluated the usefulness of patent ductus arteriosus (PDA) occlusion using a new catheter that combines good angiographic properties, easy manipulation across the duct, documentation of aortic and pulmonary artery pressure, and coil delivery for percutaneous transaortic ductal occlusion. Thirty patients (13 females and 17 males) who met the clinical and echocardiographic criteria for the percutaneous closure of PDA were catheterized. The patients selected for this study had a small to moderate PDA with a conal or tubular shape, adequate aortic ampula, and normal or mildly elevated pulmonary artery pressure. The median age was 4.8 years (range, 10 months-20 years). Patients were catheterized by the transaortic approach using the new angiographic and coil delivery catheter specially designed for this procedure. A PDA was closed in every case. A single DuctOcclud pfm coil was delivered in 28 patients, and 2 patients had NitOcclud pfm coils for ductal occlusion. The screening time was measured and found to be 50% less than the measured screening time using other catheters in our laboratory. Only one catheter and one guidewire were used per procedure, with no need for exchange wires or the establishment of an arteriovenous loop. The procedure time was also reduced by 50%. A minimal residual shunt, demonstrated by color-flow Doppler mapping, was present in 2 patients. The new combined angiographic and coil delivery catheter is most suitable for transaortic PDA coil occlusion, significantly decreasing the procedure and screening time.

Adolescent↗

Neonatal advanced heart block due to cardiac tumor.

We describe a unique case of a child who, since birth, has had a heart tumor at the atrioventricular junction associated with second degree atrioventricular block. The tumor is probably a rhabdomyoma.

Echocardiography↗

Quantitation of gold lymphocyte binding during chrysotherapy.

Carbon rod atomic absorption analysis (CRA) was applied to the quantiation of gold lymphocyte content (GLC) during chrysotherapy. Sensitivity and accuracy of CRA compared favorably with 195Au isotopic scintillation counting, circumventing the limitations and hazards of the latter in clinical applications. Picogram gold quantification of limited sample volume, less than 10 ml blood, e.g., 10(4)-10(5) lymphocytes (5 microliters) containing 5-10 pg was achieved. GLC after IM administration increased significantly in 60% of patients; for the remainder, GLC was observed to be independent of plasma gold content or cumulative dosage. GLC during auranofin administration (6 mg/day p.o.) approached values for IM gold despite significantly lower plasma levels. Unique sensitivity of CRA enables analysis of GLC under therapeutic conditions that could elucidate whether gold alters functional determinants.

Arthritis, Rheumatoid↗

IV N-acetylcysteine treatment of hematologic reactions to chrysotherapy.

Twelve patients with adverse hematologic reactions to chrysotherapy received intravenous N-acetylcysteine (IV NAC) to achieve removal and/or redistribution of gold. Urine gold excretion while on IV NAC doubled that predicted from control measurements. Treatment within 20 days of the last gold injection was associated with better recovery of hematologic parameters. No untoward reactions from IV NAC were encountered. The pathogenesis of gold induced hematologic reactions is examined from both toxic as well as immunological perspectives.

Acetylcysteine↗

Effects of chrysotherapy on cell mediated immune response.

Auranofin (AF) differs significantly from gold sodium thiomalate (GSTM) in formulation, i.e., aurous gold is stabilized by dual sulfur and phosphorus ligands, hydrophobic rather than hydrophilic characteristics, and lack of ionic charge. These attributes facilitate: oral absorption of AF, plasma membrane penetration, increase in intracellular lymphocyte gold concentration; and perhaps thereby influence lymphocyte function. AF treated subjects recorded prompt and sharp declines in mitogen-induced lymphoproliferative response (LMR) greater than 80%; suppressed response to skin testing with dinitrochlorobenezene (DNCB) in 11 of 14 subjects; and blebbing of lymphocyte membranes by scanning electron microscopy. In contrast, lymphocytes from a matched group of GSTM treated subjects recorded later onset and less suppression of LMR; normal response to DNCB skin testing; and did not manifest membrane blebbing. Accordingly, the therapeutic action of AF on immune response was observed in the 16 subjects receiving 6 mg/d of an average of 45 weeks to effect primarily cell mediated rather than humoral immune response when compared with a matched group of GSTM treated patients.

Arthritis, Rheumatoid↗