Hereditary angioedema

Although the condition is rare, patients with hereditary angioedema often present because of abdominal pain or airway compromise. A 27-year-old woman presented to the emergency department in acute abdominal distress. Identification of the disease in this patient allowed for proper management and avoidance of invasive procedures. Pathophysiology, clinical manifestations, diagnosis, and therapy of hereditary angioedema are discussed.     

Eosinophilic gastroenteritis presenting in an adolescent with isolated colonic involvement.

Eosinophilic gastroenteritis most commonly involves the stomach and proximal small intestine with eosinophilic inflammation of either the mucosa, submucosa or serosa. The patient reported here had isolated eosinophilic colitis. The initial presentation with iron deficiency anaemia owing to occult gastrointestinal blood loss emphasises the need to evaluate the entire gastrointestinal tract in patients with eosinophilic gastroenteritis.     

Risk factors for post-ERCP pancreatitis: a prospective, multicenter study

BACKGROUND: Post-ERCP pancreatitis is poorly understood. The goal of this study was to comprehensively evaluate potential procedure- and patient-related risk factors for post-ERCP pancreatitis over a wide spectrum of centers. METHODS: Consecutive ERCP procedures were prospectively studied at 11 centers (6 private, 5 university). Complications were assessed at 30 days by using established consensus criteria. RESULTS: Pancreatitis occurred after 131 (6.7%) of 1963 consecutive ERCP procedures (mild 70, moderate 55, severe 6). By univariate analysis, 23 of 32 investigated variables were significant. Multivariate risk factors with adjusted odds ratios (OR) were prior ERCP-induced pancreatitis (OR 5.4), suspected sphincter of Oddi dysfunction (OR 2.6), female gender (OR 2.5), normal serum bilirubin (OR 1.9), absence of chronic pancreatitis (OR 1.9), biliary sphincter balloon dilation (OR 4.5), difficult cannulation (OR 3.4), pancreatic sphincterotomy (OR 3.1), and 1 or more injections of contrast into the pancreatic duct (OR 2.7). Small bile duct diameter, sphincter of Oddi manometry, biliary sphincterotomy, and lower ERCP case volume were not multivariate risk factors for pancreatitis, although endoscopists performing on average more than 2 ERCPs per week had significantly greater success at bile duct cannulation (96.5% versus 91.5%, p = 0.0001). Combinations of patient characteristics including female gender, normal serum bilirubin, recurrent abdominal pain, and previous post-ERCP pancreatitis placed patients at increasingly higher risk of pancreatitis, regardless of whether ERCP was diagnostic, manometric, or therapeutic. CONCLUSIONS: Patient-related factors are as important as procedure-related factors in determining risk for post-ERCP pancreatitis. These data emphasize the importance of careful patient selection as well as choice of technique in the avoidance of post-ERCP pancreatitis.     

Identification of circulating antigen-specific CD4+ T lymphocytes with a CCR5+, cytotoxic phenotype in an HIV-1 long-term nonprogressor and in CMV infection

Antigen-specific CD4+ effector T cells primarily provide help for B-cell antibody responses and CD8+ cytotoxic T-lymphocyte (CTL) responses. We have found an expanded population of HIV-1 p24-specific, T-cell receptor V beta 17+, CD4+ T lymphocytes, defined by in vitro proliferative and interferon-gamma responses to a 15-mer Gag peptide, in the peripheral blood of an individual with long-term nonprogressive HIV-1 infection. Ex vivo, these cells were CCR5+ and CCR7-, consistent with an effector/memory function. Surprisingly, these cells highly expressed several proteins characteristic of cytotoxic lymphocytes, including TIA-1 (T-cell intracellular antigen 1; GMP-17/NKG7), granzymes A and B, CD161 (NKRP-1), and CD244 (C1.7/2B4). Following in vitro peptide stimulation, these cells produced interleukin 2 (IL-2) and intracellular CD40L, suggesting possible helper function, in addition to induction of perforin and cytotoxicity. A subset of cytomegalovirus (CMV)-specific CD4+ T cells in healthy adults similarly expressed these CTL markers and CCR5, ex vivo. Furthermore, this distinct subset of CD4+ T cells was significantly elevated in healthy CMV-seropositive adults, compared with CMV-seronegative individuals. These results suggest that CCR5+ CD4+ CTL may be a major effector mechanism of the immune response to viral infections in humans. Moreover, expression of CCR5 may render them particularly susceptible to cytopathic effects during progressive HIV-1 infection.     

The impact of the Shanley Pressure Ulcer Prevention Programme on older persons' knowledge of,and attitudes and behaviours towards,pressure ulcer prevention

Pressure ulcers (PUs) have a profound impact on individuals, with studies demonstrating that compared with similarly aged persons, those living with a PU have a significantly lower quality of life. The aim of this study was to explore the impact of the Shanley Pressure Ulcer Prevention Programme (SPUPP) on older persons'' knowledge of, and attitudes and behaviours towards, PU prevention. This was a multi‐centre, open‐label, randomised controlled trial. The population of interest was older persons living in the community who attended either a day care centre or a retirement group and were deemed to be at risk of PUs due to reduced mobility. Stratified random sampling was used to randomise based on days of attendance at day care centre/retirement group. Pretest and post‐test were applied to the intervention and control groups. The SPUPP is a multimedia programme delivered using electronic media, hard copy materials, activities, and patient diaries and addresses the key tenets of PU prevention as described by the SKIN bundle. The programme contains five separate sessions delivered over 5 weeks. The impact of the SPUPP was assessed using the patient knowledge of and attitude and behaviour towards PU prevention instrument (KPUP). A total of 64 persons, 32 in each group, took part in the study. Of these, 75% (n = 48) were female, with a mean age of 81.9 years (SD: 5.56 years). Further, 68.8% (n = 44) were either overweight or obese and 40.6% (n = 26) were usually incontinent of urine. There were no differences between the intervention and control groups in mean scores during the pretest stage. However, at post‐test, the mean scores for the intervention group were higher than the control group, 16.87 (SD: 1.88) versus 12.41 (SD: 3.21), respectively. For the post‐test stage, mean differences between the two groups in favour of the intervention group (∆ = 4.46) were statistically significant, as t = 6.76, P = .0001, and equal variances were not assumed. The SPUPP impacted positively on knowledge scores of the participants and positively influenced attitudes and behaviours towards PU prevention. Thus, this research provides information regarding the potential to enhance patient involvement in PU prevention.     

Frequencies of epstein-barr virus—inducible IgM anti-IgG B lymphocytes in normal children and children with juvenile rheumatoid arthritis

The relative frequencies of IgM antilgG autoantibody (rheumatoid factor) producing cells induced by the polyclonal B cell activator Epstein-Barr virus were measured in peripheral blood lymphocyte cultures of normal children and patients with juvenile rheumatoid arthritis. The frequencies of rheumatoid factor precursor B cells in normal children were lower than adults, but higher than neonates. The frequency increased with the age of the donor. In seronegative children with the systemic-onset or pauclarticular-onset types of juvenile rheumatoid arthritis, the number of IgM antiIgG inducible B cells was not significantly different (P>0.05) from age-matched controls. Patients with seropositive juvenile rheumatoid arthritis or seropositive adult rheumatoid arthritis had significantly higher IgM antiIgG precursor cell frequencies than age-matched normal subjects (P<0.01 and P<0.02, respectively). In contrast, the patients with seronegative polyarticular-onset juvenile rheumatoid arthritis had an average precursor frequency significantly lower than normal age-matched controls (P<0.05), analogous to results previously noted in adult seronegative rheumatoid arthritis. Thus, both children and adults with seronegative polyarticular rheumatoid arthritis had a deficiency in B cells that produce IgM antiIgG and that are induced by Epstein-Barr virus. This distinguished them from seropositive juvenile rheumatoid arthritis and rheumatoid arthritis patients, normal subjects, and patients with the pauciarticular-onset and systemic-onset types of seronegative juvenile rheumatoid arthritis.     

Novel light treatment for digital ulcers in systemic sclerosis: a feasibility study

Background

Locally acting, well-tolerated treatments for digital ulcers in patients with systemic sclerosis are needed. We aimed to investigate the safety, feasibility, and tolerability of a novel light treatment, and to tentatively assess treatment efficacy.