PubMed Health⌕ Search

Biomedical subjects

H J Longhurst

Publications and source records attributed to H J Longhurst.

11 recordsLinked to original sources

C1-inhibitor concentrate home therapy for hereditary angioedema: a viable, effective treatment option.

Economic and political factors have led to the increased use of home therapy programmes for patients who have traditionally been treated in hospital. Many patients with hereditary angioedema (HAE) experience intermittent severe attacks that affect their quality of life and may be life-threatening. These attacks are treated with C1-inhibitor concentrate which, for most patients, is infused at the local hospital. Home therapy programmes for HAE are currently being established. This paper reviews the extent of use of these programmes and summarizes the advantages and potential disadvantages of the concept so far.

Acute Disease↗

C1 inhibitor deficiency: consensus document.

We present a consensus document on the diagnosis and management of C1 inhibitor deficiency, a syndrome characterized clinically by recurrent episodes of angio-oedema. In hereditary angio-oedema, a rare autosomal dominant condition, C1 inhibitor function is reduced due to impaired transcription or production of non-functional protein. The diagnosis is confirmed by the presence of a low serum C4 and absent or greatly reduced C1 inhibitor level or function. The condition can cause fatal laryngeal oedema and features indistinguishable from gastrointestinal tract obstruction. Attacks can be precipitated by trauma, infection and other stimulants. Treatment is graded according to response and the clinical site of swelling. Acute treatment for severe attack is by infusion of C1 inhibitor concentrate and for minor attack attenuated androgens and/or tranexamic acid. Prophylactic treatment is by attenuated androgens and/or tranexamic acid. There are a number of new products in trial, including genetically engineered C1 esterase inhibitor, kallikrein inhibitor and bradykinin B2 receptor antagonist. Individual sections provide special advice with respect to diagnosis, management (prophylaxis and emergency care), special situations (childhood, pregnancy, contraception, travel and dental care) and service specification.

Adolescent↗

Emergency treatment of acute attacks in hereditary angioedema due to C1 inhibitor deficiency: what is the evidence?

Hereditary angioedema (HAE) is caused by a deficiency in C1 esterase-inhibitor (C1-INH) and is characterised by skin swelling, abdominal pain and episodes of upper respiratory tract obstruction. Oedema of the larynx can result in rapid asphyxiation and requires emergency treatment. Three treatment options for emergency treatment of HAE are reviewed: fresh frozen plasma, solvent/detergent-treated plasma and C1-INH concentrate. It is concluded that while all three treatment options are theoretically effective in the emergency treatment of HAE, C1-INH is the treatment of choice.

Acute Disease↗

Serological markers (anti-Saccharomyces cerevisiae mannan antibodies and antineutrophil cytoplasmic antibodies) in inflammatory bowel disease: diagnostic utility and phenotypic correlation.

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.

Antibodies, Antineutrophil Cytoplasmic↗

Anti-D immunoglobulin treatment for thrombocytopenia associated with primary antibody deficiency.

AIMS: To review our experience of anti-D immunoglobulin for immune thrombocytopenia (ITP) in patients with primary antibody deficiency. METHODS/PATIENTS: A retrospective case notes review of four Rhesus positive patients with ITP and primary antibody deficiency, treated with anti-D. Patients were refractory to steroids and high dose intravenous immunoglobulin (IVIG). Two patients were previously splenectomised. RESULTS: All patients responded to anti-D immunoglobulin. Improved platelet counts were sustained for at least three months. Side effects included a fall in haemoglobin in all cases; one patient required red blood cell transfusion. Two patients had transient neutropenia (< 1 x 10(9)/litre). CONCLUSION: Anti-D immunoglobulin may be an effective treatment for antibody deficiency associated thrombocytopenia, even after splenectomy. Anti-D immunoglobulin may have considerable clinical advantages in this group of patients, where treatments resulting in further immunosuppression are relatively contraindicated.

Adult↗

The histones of Plasmodium falciparum: identification, purification and a possible role in the pathology of malaria.

A quick and simple method of purifying the histones from Plasmodium falciparum culture supernatant or from infected erythrocytes is described. The proteins were present only in preparations rich in P. falciparum nuclear material and were soluble at acid pH and in strongly anionic detergents. Four proteins, of 14-18 kDa were identified as the P. falciparum core histones. N-terminal sequence analysis of the 16 and 18 kDa proteins and of a tryptic fragment of the protein mixture revealed strong homologies with deduced cDNA or protein sequences of histones H2A, H2B, and H3 of P. falciparum and other species. Antibodies raised against the proteins cross-reacted weakly with histones of other species and, on immunofluorescence, localized the proteins to schizont nuclei. Anti-P. falciparum histone antibodies were detected in sera of semi-immune human adults but these antibodies did not react with human histones on a Western blot. The large quantities of P. falciparum histone released and the chronic nature of malarial infection, together with the unusually high avidity of histones for ligands found in renal and vascular basement membrane, raise the question of a role for histones in the pathogenesis of malarial infection. We suggest that histones or histone-antibody complexes may contribute to disease pathology.

Adult↗

A simple method for the detection of Chlamydia trachomatis infections in general practice.

Chlamydial infection is an important cause of genital tract disease in women and is often silent. Collection, storage and transportation of specimens required for culture pose problems which have made studies difficult and diagnosis impractical outside hospitals or sexually transmitted disease clinics.The direct monoclonal antibody test (MicroTrak, Syva) for detecting chlamydiae is comparable with the traditional culture method in sensitivity and specificity. The test requires only the preparation of a smear on a slide, making it convenient for use in general practice. The feasibility of using this procedure in an inner city practice was demonstrated in tests on 188 women who required pelvic examination. Of 169 women from whom valid specimens were obtained 18 (10.7%) were found to have a chlamydial infection. Only three of the infected women were asymptomatic and the organisms were associated particularly with dysuria. The value of the test in comparison with other procedures currently available for detecting chlamydiae is emphasized.

Chlamydia Infections↗