BACKGROUND Hailey-Hailey disease (HHD) is an inherited blistering dermatosis characterized by recurrent erosions and erythematous plaques that generally manifest in intertriginous areas. Genetically, HHD is an autosomal dominant disease, resulting from heterozygous mutations in ATP2C1, which encodes a Ca2+/Mn2+ATPase. In this study, we aimed at identifying and analyzing mutations in five patien...

BACKGROUND Home haemodialysis (HHD) and self-care satellite dialyses (SHD) have been suggested to offer significant benefits over conventional in-centre haemodialysis. However, little is known about differences between these two modalities. The purpose of the study was to analyse costs and health-related quality of life (HRQoL) of HHD and SHD. METHODS On 15 October 2004, a total of 65 patient...

Hailey-Hailey disease (HHD) and Darier's disease (DD) are caused by mutations in Ca(2+)-ATPases with the end result of desmosomal disruption and suprabasal acantholysis. Tight junctions (TJ) are located in the granular cell layer in normal skin and contribute to the epidermal barrier. Aberrations in the epidermal differentiation, such as in psoriasis, have been shown to lead to changes in the e...

Tumor xenografts in immunodeficient mice, while routinely used in cancer research, preclude studying interactions of immune and cancer cells or, if humanized by allogeneic immune cells, are of limited use for tumor-immunological questions. Here, we explore a novel way to generate cancer models with an autologous humanized immune system. We demonstrate that hematopoietic stem and progenitor cell...

Using the idea due to P. DUCHET in proving his well–known theorem on powers of chordal graphs, we shall describe some theorems of DUCHET–type for powers of graphs that have no long induced cycles. In particular, our DUCHET–type theorem for HHD–free graphs improves a recent result due to DRAGAN, NICOLAI, BRANDSTÄDT saying that odd powers of HHD–free graphs are also HHD–free.

Hypertensive heart disease (HHD) is the leading cause of mortality and morbidity in the United States. Despite the availability of medical therapy it remains a challenge to treat. Autonomic nervous system imbalance resulting in overactivity of the sympathetic nervous system and under activity of the parasympathetic nervous system is integral in the development of hypertension and ultimately the...

Vascular access infections are of concern to hemodialysis patients and nurses. Best demonstrated practices (BDPs) have not been developed for home hemodialysis (HHD) access use, but there have been generally accepted practices (GAPs) endorsed by dialysis professionals. We developed a survey to gather information about training provided and actual practices of HHD patients using the NxStage Syst...

Alterations in trinucleotide repeat length during transmission are important in the pathophysiology of Huntington's disease (HD). However, it is not well understood where, when and by what mechanism expansion occurs. We have followed the fate of CAG repeats during development in mice that can [hHD(-/+)/Msh2(+/+)] or cannot [hHD(-/+)/Msh2(-/-)] expand their repeats. Here we show that long repeat...

BACKGROUND/AIMS Survival for dialysis patients is poor. Earlier studies have shown better survival in home-hemodialysis (HHD). The aims of this study are to compare survival for matched patients with HHD and institutional hemodialysis (IHD) and to elucidate the effect on factors related to survival such as hyperphosphatemia, fluid overload and anemia. METHODS In this retrospective, observatio...

Active cancer immunotherapy, such as cancer vaccine, is based on the fundamental knowledge that tumor‑associated antigens (TAAs) are presented on MHC molecules for recognition by specific T cells. However, most TAAs are self-antigens and are also expressed on normal tissues, including the thymus. This fact raises the issue of the tolerance of the TAA‑specific T‑cell repertoire and consequently ...