When hepatitis C virus (HCV) infection becomes chronic, spontaneous viral eradication is a rare event. We report two patients with chronic hepatitis C, non-responders to standard interferon alone. They were treated with Pegylated interferon plus ribavirin. At the end of therapy, HCV RNA was still detectable. Several months after stopping treatment, aminotransferase level normalized and HCV RNA became undetectable. No case of sustained viral response happening several months after therapy has been yet described. During long-term follow-up of non-responders, when a persistent normalization of aminotransferase level is observed after stopping treatment, viral clearance could be suspected.

The aim of this study was to characterize the adequacy and longevity of biological response to desmopressin (DDAVP) in a large Amish kindred of Type 2M von Willebrand disease (VWD) possessing C-to-T transition at nucleotide 4120 in exon 28 of A1 domain of von Willebrand factor (VWF) gene. Response to both intranasal (Stimate) and subcutaneous DDAVP administration was assessed. Rise in ristocetin cofactor activity (VWF:RCo) > or = 40% at 90-min post-Stimate and 1-2 h after subcutaneous DDAVP was defined as initial response; response longevity was assessed only after subcutaneous dosing by measuring VWF:RCo levels at time-points 1, 2, 4 and 6 h. Eleven patients (five males, six females; age range: 20-56 years) participated in intranasal and 9/11 (four males, five females) in subcutaneous testing. Baseline haemostatic profiles included: VWF:RCo < 15%, VWF:Ag < 40% and normal VWF multimers. Initial response was comparable by both intranasal (6/11; 54.5%) and subcutaneous (4/9; 44%) routes; sustained response (VWF:RCo > 40% for 2 h) was observed in only one in nine (11%) patients tested. Median VWF:RCo peak levels after intranasal (40%) and subcutaneous (39%) routes were equivalent. Peak VWF:Ag levels were significantly higher after subcutaneous than intranasal DDAVP (94% vs. 54%; P = 0.03). Area under the curve for VWF:RCo was significantly decreased (170 microg h mL(-1)) compared with VWF:Ag (471 microg h mL(-1)) and FVIII:C (624.60 microg h mL(-1)). This study suggests that in this population: (i) intra-individual DDAVP response is consistent with subcutaneous and intranasal administration; and (ii) extending DDAVP challenge test up to at least 6 h is required to characterize adequacy and longevity of biologic response prior to using DDAVP as a sole haemostatic intervention.

No abstract

No abstract

No abstract

No abstract

The effect of dietetic advice on hyperlipidaemia in type 2 diabetic patients is uncertain. We have investigated this modality of treatment in 50 type 2 diabetic patients (24 female), mean (±SD) age 54±4 years and diabetes duration 5±4 years. All had a random plasma total cholesterol concentration of >6.5 mmol/L (mean 7.5±0.7 mmol/L). Three months after dietetic intervention, cholesterol fell to 7.1±1.1 (p=0.049), but triglycerides, LDL‐cholesterol and HDL‐cholesterol were unchanged, as were HbA1c and body mass index (BMI). Thirteen (26%) patients reduced total cholesterol levels to <6.5 mmol/L on dietary treatment (‘diet responders’). In this group there were significant improvements in total cholesterol (6.9±0.3 versus 5.9±0.6, p=0.03) and LDL‐cholesterol (4.8±0.5 versus 4.1±0.5, p=0.003). This group had lower baseline total cholesterol levels than ‘diet non‐responders’. Multiple regression analysis revealed no association between diet response and baseline levels of HbA1c, BMI, age, sex, diabetes duration or compliance with dietetic advice. After two years of follow‐up only four of these 13 ‘diet responders’ had cholesterol levels<6.5 mmol/L without drug treatment. ‘Diet non‐responders’ at 3 months were treated with fenofibrate, which resulted in significant improvements in total cholesterol (7.6±0.9 before versus 6.8±1.1 after, p=0.012), LDL‐cholesterol (5.2±0.8 before versus 4.6±0.8 after, p=0.019) and triglycerides (3.7±2.7 before versus 2.7±1.4 after, p=0.008). HDL cholesterol rose (1.0±0.3 before versus 1.1±0.3 after, p=0.048), and HbA1c also fell from 7.5±1.9 to 6.9±1.8 (p=0.024) on fenofibrate treatment. We conclude that dietary treatment of dyslipidaemia in type 2 diabetes is effective only in a minority of patients, who are characterised by milder degrees of hypercholesterolaemia. Fenofibrate however was effective in improving dyslipidaemia, and was also associated with a reduction in HbA1c. Copyright © 2001 John Wiley & Sons, Ltd.

1991 Malignant hyperthermia susceptibility (muscle Ca release channel, RYR1) [3] intent of the conference was to stimulate new ideas that Hyperkalemic periodic paralysis (muscle Na channel, SCN4A)would ultimately lead to greater advances in ion channel [4,5] disease biology, as well as improved diagnostic and thera-1992 Paramyotonia congenita (muscle Na channel, SCN4A) [6,7] peutic modalities. An eclectic mix of scientists was as-Recessive generalized myotonia (muscle Cl channel, CLCN1) [8] sembled to provide opportunities for cross-fertilization 1993 Autosomal dominant myotonia congenita-Thomsen's disease (muscle Cl channel, CLCN1) [9]of ideas and expertise. The conference served to dissemi-Central core disease (muscle Ca release channel, RYR1) [10, 11] nate information about ion channel disease research and Hyperekplexia (glycine receptor, GLRA1) [12] provided a forum for sharing the latest experimental 1994 Liddle's syndrome (epithelial Na channel, SCNN1B, SCNN1G)approaches to studying these fascinating syndromes.[13] Autosomal recessive nephrogenic diabetes insipidus (renal The conference was organized into six main sessions, water channel, AQP2) [14] two poster sessions, and three state-of-the-art lectures. Dent's disease (X-linked nephrolithiasis; renal Cl channel, The organizational structure focused on common mecha-CLCN5) [15, 16] Episodic ataxia with myokymia (K channel, KCN4A) [17] nisms of ion channel dysfunction to facilitate the goals Hypokalemic periodic paralysis (muscle Ca channel, of the conference. By all accounts, this plan was a tre-CACNLA3) [18] mendous success. The following paragraphs briefly sum-1995 Familial persistent hyperinsulinemia (pancreatic K ATP channel/ marize the content of the conference, and several more sulfonylurea receptor, SUR1) [19] Congenital long QT syndrome-LQT2 (cardiac K channel, formal excerpts have been generously supplied by many HERG) [20] of the participants to form this Symposium issue of Kid-Congenital long QT syndrome-LQT3 (cardiac Na channel, ney International. SCN5A) [21] Autosomal recessive retinitis pigmentosa (cGMP-gated channel, CNCG1) [22] Slow-channel myasthenic syndrome (muscle acetylcholine MECHANISMS OF ION CHANNEL receptor, CHRNB1, CHRNE) [23, 24] DYSFUNCTION: RELATING Nocturnal frontal lobe epilepsy (neuronal acetylcholine receptor, CHRNA4) [25] STRUCTURE TO FUNCTION 1996 Bartter's syndrome (renal K channel, ROMK) [26] The identification and functional characterization of Congenital long QT syndrome-LQT1 (cardiac K channel, ion channel mutations have, in many cases, revealed KvLQT1) [27] Pseudohypoaldosteronism (epithelial Na channel, SCNN1A,