Subject(s)
Hematinics/therapeutic use , Interleukin 1 Receptor Antagonist Protein/therapeutic use , Paraproteinemias/etiology , Schnitzler Syndrome/diagnosis , Urticaria/etiology , Drug Resistance , Humans , Male , Middle Aged , Paraproteinemias/drug therapy , Recurrence , Schnitzler Syndrome/drug therapyABSTRACT
There are estimated to be approximately 1500 people in the United Kingdom with C1 inhibitor (C1INH) deficiency. At BartsHealth National Health Service (NHS) Trust we manage 133 patients with this condition and we believe that this represents one of the largest cohorts in the United Kingdom. C1INH deficiency may be hereditary or acquired. It is characterized by unpredictable episodic swellings, which may affect any part of the body, but are potentially fatal if they involve the larynx and cause significant morbidity if they involve the viscera. The last few years have seen a revolution in the treatment options that are available for C1 inhibitor deficiency. However, this occurs at a time when there are increased spending restraints in the NHS and the commissioning structure is being overhauled. Integrated care pathways (ICP) are a tool for disseminating best practice, for facilitating clinical audit, enabling multi-disciplinary working and for reducing health-care costs. Here we present an ICP for managing C1 inhibitor deficiency.
Subject(s)
Case Management , Complement C1 Inactivator Proteins/deficiency , Disease Management , Hereditary Angioedema Types I and II/drug therapy , Medical Records, Problem-Oriented/standards , Complement C1 Inhibitor Protein , Critical Pathways , Guideline Adherence , Hereditary Angioedema Types I and II/epidemiology , Hereditary Angioedema Types I and II/genetics , Hereditary Angioedema Types I and II/physiopathology , Humans , Interdisciplinary Communication , Physician-Patient Relations , Practice Guidelines as Topic , Prevalence , United KingdomABSTRACT
Angioedema is a result of increased vascular permeability, with subsequent extravasation of intravascular fluid into the surrounding tissues. Angioedema may be mediated by histamine, bradykinin or other mediators. Histaminergic angioedema generally presents with urticaria and/or pruritus and will respond to conventional treatment with antihistamines, corticosteroids or epinephrine. Bradykinin-mediated angioedema, which includes hereditary angioedema (HAE types I, II and III), acquired C1-INH deficiency, and angiotensin-converting enzyme inhibitor-induced angioedema does not typically present with urticaria/weals and does not respond to conventional agents such as antihistamines or corticosteroids. In recent years, several agents that prevent the generation or activity of bradykinin have been developed for the treatment of HAE types I and II and are also being evaluated in other types of bradykinin-mediated angioedema. These agents have the potential to improve outcomes for patients with different forms of bradykinin-mediated angioedema.
Subject(s)
Angioedema/etiology , Bradykinin/metabolism , Angioedema/diagnosis , Angioedema/therapy , HumansABSTRACT
Occasional serum samples (<0.5%) tested by indirect immunofluorescence showed less fluorescence than did negative-control serum. A retrospective review of these patients' serum immunoglobulins revealed a high percentage of abnormalities (71%, versus 22% of controls). We suggest that this observation should be reported when seen and that the clinician should be alerted to an association with immunoglobulin abnormalities.
Subject(s)
Immunoglobulins/blood , Adolescent , Adult , Animals , Cell Line , Female , Fluorescent Antibody Technique, Indirect , Haplorhini , Humans , Male , Middle Aged , Retrospective Studies , Rodentia , Staining and LabelingABSTRACT
We have evaluated the utility of antineutrophil cytoplasmic antibodies and anti-Saccharomyces cerevisiae mannan antibodies for distinguishing Crohn's disease from ulcerative colitis and other diarrheal illnesses by evaluating sera from 396 patients. Sensitivity, specificity, and phenotypic correlations were investigated. The implications of our findings for implementing these tests in routine clinical testing are discussed.
Subject(s)
Antibodies, Antineutrophil Cytoplasmic , Inflammatory Bowel Diseases/diagnosis , Mannans/immunology , Saccharomyces cerevisiae/immunology , Biomarkers , Colitis, Ulcerative/diagnosis , Colitis, Ulcerative/immunology , Crohn Disease/diagnosis , Crohn Disease/immunology , Diagnosis, Differential , Diarrhea/classification , Diarrhea/etiology , Humans , Inflammatory Bowel Diseases/classification , Inflammatory Bowel Diseases/immunology , Inflammatory Bowel Diseases/physiopathology , Predictive Value of Tests , Sensitivity and SpecificitySubject(s)
HIV Infections/therapy , HIV/immunology , AIDS Vaccines , Anti-HIV Agents , HIV Infections/immunology , Humans , ImmunotherapyABSTRACT
Using a Humphrey field analyzer and fine matrix mapping, we measured photopic and scotopic thresholds for a blue light stimulus at 100 locations on a 9 degrees by 9 degrees matrix of 1 degree spacing centered at the fovea in 14 normal subjects. Additionally, trial lenses were used to investigate the effect of refractive error. Under photopic conditions the mean sensitivity varied by less than 1 dB over this region. Under scotopic conditions the central values were reduced in sensitivity by 15 dB compared with those at 4 degrees eccentricity. Defocus showed less than 1.2 dB loss with 1.00D of refractive error under photopic condition. The results reflect the properties of rod and cone photoreceptors and the effects of the rod mosaic near the fovea. This technique is a sensitive test of macular visual function.
