Vasopressin Secretion in the DIDMOAD (Wolfram) Syndrome

SUMMARY The diabetes insipidus which accompanies the DIDMOAD (Wolfram)Syndrome is thought to be hypothalamic in origin, though noformal study of vasopressin secretion in the syndrome has beenpublished, and some data in the literature suggest arenal tubulardefect. We have studied vasopressin secretion in seven patientswith the Wolfram /DIDMOAD syndrome during three dymatic stimuli:an Osmotic Stimulus (hypertonic Saline infusion), hypoglycaemia(insulin tolerance test) and a baroregulatory stimulus (trimetaphaninfusuion). Hypertonic saline infusion demonstrated three patientsto have complete and four to have partial hypothalamic diabetesinsipidus; administration of (per nasal) desmopressin excludednephrogenic diabetes insipidus in all seven patients. Insulinhypoglycaemia failed to stimulate vasopressin release, but trimetaphan-inducedhypotension produced significant though subnormal rises in plasmavasopressin in three patients with partial diabetes insipidus,though it produced a negligible rise and no rise in plasma vasopressinin two patients with complete diabetes insipidus. The data suggest a much greater frequency of hypothalamic diabetesinsipidus in the Wolfram/DIDMOAD syndrome than is reported,but did not identify nephrogenic diabetes insipidus. The absenceof vasopressin responses to non-osmotic stimuli in patientswith complete diabetes insipidus suggests global lack of vasopressinsecreting neurones, rather than an isolated osmoreceptor defector selective vasopressin secreting neuronal loss, as the lesionproducing diabetes insipidus in the DIDMOND syndr     

Isolated basal keratinocytes express pemphigoid gestationis antigen

Basal keratinocytes were isolated from epidermal cell suspensions prepared by trypsinization of normal human skin. Cells were identified as basal cells by their adherence to collagen and confirmed as basal cells by the presence of pemphigoid antigen. Using an indirect immunofluorescence assay, cells were found to express pemphigoid gestationis-related antigen. Sera from patients with pemphigoid gestationis reacted in one of two immunofluorescence patterns: either polar, in a pattern similar to that observed with bullous pemphigoid serum, or with uniform staining around the cell periphery. Pemphigoid gestationis-related antigen is expressed by isolated basal keratinocytes and is resistant to trypsinization. The heterogeneity of immunofluorescence patterns may correspond to the heterogeneity of antigen recognition by different patients with pemphigoid gestationis.     

A comparison of the effects of IGF-I and insulin on glucose metabolism, fat metabolism and the cardiovascular system in normal human volunteers

Abstract. The metabolic and cardiovascular effects of recombinant human IGF-I were compared to insulin in six normal subjects. Subjects were studied twice and intravenously received an infusion of [6,6-²H₂]glucose (0–480 min) and in random order either IGF-I 20μg kg^-1 h^-1 (43.7 pmol kg^-1 min^-1) or insulin 0.5 mU kg^-1 min^-1 (3.4 pmol kg^-1 min^-1) with an euglycaemic clamp. One subject was withdrawn following a serious adverse event. During the IGF-I infusion glucose appearance rate (Ra) decreased from 1.79 ± 0.13 at baseline (150–180 min) to 0.35 ± 0.26 mg kg^-1 min^-1 ( P < 0.01) at 360min, and glucose utilization rate (Rd) increased from 1.79 ± 0.28 to 4.17 ± 0.84 mg kg^-1 min^-1 ( P < 0.01). There was no change in free fatty acids (FFA) and an increase (percentage change from pre-infusion mean) in cardiac output + 37.3%± 9% ( P < 0.01), heart rate + 13%± 2% ( P < 0.01) and stroke volume + 21%± 7% ( P < 0.05). During the insulin infusion glucose Ra decreased from 1.89 ± 0.13 to 0.34 ± 0.33 mg kg^-1 min^-1 ( P < 0.01) and FFA from 0.546 mmoll^-1 to 0.198 mmoll^-1 ( P < 0.01), glucose Rd increased from l.89 ± 0.18 to 5.41 ± l.47mg kg^-1 min^-1 ( P < 0.01) and there were no significant changes in the cardiovascular variables.