Response to treatment with rosiglitazone in familial partial lipodystrophy due to a mutation in the LMNA gene

Background Familial partial lipodystrophy (FPLD) is a monogenic form of diabetes characterised by a dominantly inherited disorder of adipose tissue associated with the loss of subcutaneous fat from the limbs and trunk, with excess fat deposited around the face and neck. The lipodystrophy causes severe insulin resistance, resulting in acanthosis nigricans, diabetes, dyslipidaemia, and increased risk of cardiovascular disease. Preliminary results from animals and man suggest that increasing subcutaneous fat by treatment with thiazolidinediones should improve insulin resistance and the associated features of this syndrome. Case report We report a 24-year-old patient with FPLD caused by a mutation in the LMNA gene (R482W) treated with 12 months of rosiglitazone. Subcutaneous fat increased following rosiglitazone treatment as demonstrated by a 29% generalised increase in skin-fold thickness. Leptin levels increased from 5.8 to 11.2 ng/ml. Compared with treatment on Metformin, there was an increase in insulin sensitivity (HOMA S% 17.2–31.6) but no change in glycaemic control. The lipid profile worsened during the follow-up period. Conclusion This initial case suggests that, for modification of cardiovascular risk factors, there are no clear advantages in treating patients with FPLD with rosiglitazone despite increases in subcutaneous adipose tissue. Larger series will be needed to identify moderate beneficial effects and treatment may be more effective in patients with generalised forms of lipodystrophy.     

Peri-ventricular grey stimulation versus motor cortex stimulation for post stroke neuropathic pain.

Central post stroke pain is often difficult to manage satisfactorily with conventional treatment modalities for pain. In the last decade functional neurosurgery has offered hope with motor cortex stimulation achieving significant alleviation of pain in some patients. Unfortunately this has led to the neglect of chronic stimulation of deep grey matter as another modality of treating this condition. In this article we present our experience with motor cortex stimulation and that with deep grey matter stimulation in patients with post stroke pain. We argue that both modalities have a significant role and that what is required are better methods of identifying particular patients who are more likely to respond to one or the other.     

Differentiation of Achromobacter-like strains from human blood by DNA restriction endonuclease digest and ribosomal RNA gene probe patterns

Variation amongst Achromobacter-like strains was examined by DNA restriction endonuclease digestion and rDNA gene patterns generated using a non-radioactive probe. Chromosomal DNA was extracted from 12 cultures representing Achromobacter groups B, E and F, all from human blood cultures. DNA fingerprinting using EcoRI, Hae III or HindIII sub-divided the strains in a similar manner to that obtained by their protein patterns. The HaeIII patterns, with their small number of bands, were the easiest to interpret. The EcoRI patterns included a species-species triplet of bands but minor band patterns allowed further differentiation. The Achromobacter group F strains comprised a separate taxon and were distinct from the group B and E strains by all techniques examined. The study demonstrates that, in addition to total DNA digest analysis, rDNA gene restriction patterns provide a simple but discriminatory electrophoretic method for distinguishing within Achromobacter groups B and E.     

Human selenium status and glutathione peroxidase activity in north-west England

Nutritional selenium deficiency disease, due to low soil selenium content, is well recognized in British livestock. The adequacy of the selenium status of 25 healthy human volunteers, 50 blood donors, 94 general medical patients and 106 allergy clinic patients was investigated in 1987/8 by measuring serum selenium concentration and platelet glutathione peroxidase activity. Mean serum selenium concentration for the entire study population was 92 micrograms/l. Significant linear correlations between serum selenium concentration and peroxidase activity indicate that a substantial proportion of both healthy volunteers and medical patients are of low selenium nutritional status. Twenty-five per cent of healthy volunteers and 50 per cent of medical patients had serum selenium values below those required for full expression of selenium-dependent enzyme activity. Deficiency of the antioxidant activities of selenium and selenium-dependent enzymes may be relevant to geographical differences in morbidity from a wide range of human disease states.     

Comparative study of four wound dressings on epithelization of partial-thickness wounds in pigs

Sixteen shallow wounds were inflicted in each of five Yorkshire white female piglets, 18-20 kg body weight, by a 2-cm diameter, fast-rotating abrasive disc. The injury is similar to a second-degree burn. The wounds were dressed with one of four dressings: Duoderm (Squibb), Op-Site (Smith & Nephew) [corrected], and collagen sponge, covered with either occlusive or semiocclusive polyurethane film (Datascope Corp.). The last two dressings were moistened with saline before application. The rate of epithelization by planimetric quantitation after 3 to 5 days was the same regardless of the dressing used, although the epithelium layer was thicker in wounds treated with Duoderm. Wounds dressed by either of the collagen sponge materials showed a better appearance when visually scored. Wounds dressed with Duoderm or Op-Site were often macerated; Duoderm's paste-like material remained on the wound and was difficult to remove without inflicting discomfort to the patient. Duoderm, and also Op-Site, adhered much more strongly to the intact skin than either type of collagen sponge dressing.