Hypotonic, Acidic Oxidizing Solution Containing Hypochlorous Acid (HClO) as a Potential Treatment of Hailey-Hailey Disease.

Hailey-Hailey disease (HHD) is a rare, chronic and recurrent blistering disorder, characterized by erosions occurring primarily in intertriginous regions and histologically by suprabasal acantholysis. Mutation of the Golgi Ca2+-ATPase ATP2C1 has been identified as having a causative role in Hailey-Hailey disease. HHD-derived keratinocytes have increased oxidative-stress that is associated with impaired proliferation and differentiation. Additionally, HHD is characterized by skin lesions that do not heal and by recurrent skin infections, indicating that HHD keratinocytes might not respond well to challenges such as wounding or infection. Hypochlorous acid has been demonstrated in vitro and in vivo to possess properties that rescue both oxidative stress and altered wound repair process. Thus, we investigated the potential effects of a stabilized form of hypochlorous acid (APR-TD012) in an in vitro model of HHD. We found that treatment of ATP2C1-defective keratinocytes with APR-TD012 contributed to upregulation of Nrf2 (nuclear factor (erythroid-derived 2)-like 2). Additionally, APR TD012-treatment restored the defective proliferative capability of siATP2C1-treated keratinocytes. We also found that the APR-TD012 treatment might support wound healing process, due to its ability to modulate the expression of wound healing associated cytokines. These observations suggested that the APR-TD012 might be a potential therapeutic agent for HHD-lesions.

First-ever stroke incidence and 30-day case fatality in the Sicilian Aeolian archipelago, Italy.

BACKGROUND AND PURPOSE: Not many data on stroke epidemiology come from studies on islands. This is the first report on a Mediterranean archipelago population. METHODS: Using recommended criteria, from July 1, 1999, to June 30, 2002, information was collected on first-ever stroke and 30-day case fatality in Aeolian island residents (13,431). RESULTS: The overall crude incidence rate was 154 of 100,000 (95% CI, 118 to 197; 128 in men and 180 in women) or 180, 154, and 87, if adjusted to the Italian, European, and world populations, respectively. The 30-day case fatality rate was 24.2% (95% CI, 14.22 to 36.75). CONCLUSIONS: Besides genetic or dietary factors, our results may reflect local, limited possibilities of diagnosis and management for stroke patients.

Prevalence and characteristics of epilepsy in the Aeolian islands.

PURPOSE: To estimate the prevalence and define the clinical characteristics of epileptic disorders in the 13,431 residents of the Sicilian Aeolian archipelago, on June 1, 1999. METHODS: All established or suspected cases were identified by the neurologists of our working group from available medical information sources. Possible epilepsy cases were then evaluated by the epileptologists by using a standardized questionnaire. The patients were further reviewed by the whole research team to confirm the clinical diagnosis. For a more detailed syndromic definition, some patients underwent EEG or neuroradiologic investigations or both. RESULTS: The crude point prevalence rate of active epilepsy was 3.13 (95% confidence interval, 2.2-4.2). The prevalence rate age-adjusted to the 2001 Italian population was 3.01. Females had a slightly higher prevalence rate than did males. The highest age-specific prevalence was found in patients aged 5 to 14 years (5.05) and in those aged 65 to 74 years (5.41). Partial seizures with or without secondary generalization were more common (61.7%) than were generalized seizures. Eighty-three percent of cases had symptomatic or cryptogenic localization-related epilepsies, and 8.5% had idiopathic (generalized or partial) epilepsies. Epilepsy was unclassified in 8.5% of cases. CONCLUSIONS: The prevalence of active epilepsy in the Aeolian islands is lower than that in other developed areas, including northern Italy, but is similar to that in Sicily. Partial seizures were the most common type, and localization-related symptomatic epilepsies were the largest syndromic category.