IgE-mediated anaphylactic reaction to purified and recombinant factor VIII in a patient with severe haemophilia A

We report a 51-year-old patient with severe haemophilia A developing a severe life-threatening anaphylactic reaction to recombinant factor VIII (rFVIII). Anaphylactic reactions are a rare but well-known side effect of FVIII products. The nature of these reactions could not be clarified as previous studies failed to demonstrate a specific IgE response. Here, we could prove a grade 3 anaphlyactic reaction as an IgE-mediated response to rFVIII for the first time by Western blotting.     

Adipose tissue and liver lipid metabolism in obese children: role of the body mass index and the presence of acanthosis nigricans.

AIMS: The aims of our study were to determine if insulin resistance is associated with increased plasma levels of non-esterified fatty acids (NEFA), glycerol, 3-hydroxybutyrate and triglycerides in obese children. We also studied whether the presence of acanthosis nigricans (AN) led to further alterations in the above parameters. METHODS: A total of 101 children were studied on their first visit to the paediatric endocrine clinic. Seventy-four were obese, 30 of them with AN. The remaining 27 were non-obese healthy children (control group). NEFAs, glycerol, triglycerides, 3-hydroxybutyrate, insulin, leptin, adiponectin and glucose were determined in blood samples obtained after overnight fasting. The insulin resistance index (IRI) was calculated following the homeostasis model assessment (HOMA). Data from the three groups were compared using appropriate statistical tests. RESULTS: No differences in age, sex ratio and pubertal stage were observed among the three groups. The group of children with the highest body mass index (BMI) showed higher plasma levels of insulin and leptin, higher IRI and lower plasma levels of adiponectin. As insulin and IRI increased, NEFA and 3-hydroxybutyrate decreased and triglycerides increased. When obese children were categorized by BMI, the presence of AN further exacerbated these differences. CONCLUSIONS: In obese children, insulin resistance is associated with plasma lipid alterations suggestive of both decreased adipose tissue lipolysis and hepatic beta-oxidation and increased hepatic synthesis of triglycerides. Such a metabolic condition may facilitate fat storage and hinder weight loss.     

Efficacy of conservative treatment modalities used in epistaxis

Objective  To assess the efficacy of conservative modalities in the management of epistaxis. Study design  Prospective case studies. Setting  Rural medical college hospital. Patients  Indoor and outdoor patients with epistaxis. lntervention  Conservative(non surgical) management techniques in epistaxis. Results  Chemical cauterization was successful in 72.07% cases, anterior nasal packing in 84.5% and posterior nasal packing in 95.6% cases. Conclusion  Out of 300 cases of epistaxis included in the study (except for one case that required internal maxillary artery ligation). all cases were treated successfully with conservative treatment alongwith treating the underlying pathology causing epistaxis.     

CT19PTRICUSPID VALVE REPAIR IN ‘TORRENTIAL TRICUSPID REGURGITATION’ FOLLOWING PERMANENT PACEMAKER LEAD EXTRACTION – A CASE REPORT

Introduction Traumatic tricuspid regurgitation secondary to blunt chest trauma has been reported in literature. We report our experience with a case of ‘Torrential Tricuspid Regurgitation’ following permanent pacemaker lead extraction which was successfully treated with tricuspid valve repair and annuloplasty. Report A 67 year old woman was treated for Sick sinus syndrome with permanent pacemaker implant. She had three generator changes for end of life and repositioning.Erosion of generator, led cardiologist to plan lead and generator extraction with the surgical backup. During lead extraction a small piece of papillary muscle was avulsed. The patient remained hemodynamically stable in the theatre. However in ward she developed right sided cardiac failure not responding to conservative therapy. A transthoracic echo (TTE) revealed torrential tricuspid regurgitation with a freely mobile posterior leaflet with attached chordae and avulsed papillary muscle.During surgery the tricuspid valve was successfully repaired and transesophageal (TOE) images showed trivial to mild tricuspid regurgitation at the end of repair procedure. Additional procedure also included ligation of both atrial appendages and implantation of epicardial leads and pacemaker. Patient made good recovery from operation. Conclusion To the best of our knowledge this is first report of repair of tricuspid valve in ‘Torrential Tricuspid Regurgitation’ following pacemaker lead extraction. We share our experience with tricuspid valve repair technique and annuloplasty.     

PR35THE INCIDENCE OF NON‐MELANOMA SKIN CANCER IN NEW ZEALAND

Background It is estimated that skin cancers cost $33 million per annum to the New Zealand healthcare system. Basal cell carcinoma and squamous cell carcinoma are the commonest types of non melanoma skin cancers (NMSCs). Anecdotal evidence indicates that there has been a doubling in the incidence of NMSCs in New Zealand over the last decade. Because of the high incidence mandatory reporting of NMSCs to the National Cancer Registry is not required. This lack of accurate data has led to poor health care policies and strategies including funding and workforce planning. Aims The aims of this study are to (1) present the latest statistics on NMSCs in New Zealand, including the incidence across different regions over the last decade, patient demographics, anatomic distribution of NMSCs, incidence and sites of metastasis, and disease‐specific survival; to (2) the histopathology of NMSCs, including surgical margins, histologic grade, and perineural, lymphatic, and vascular invasion; and (3) the relative role of different faculties treating NMSCs. Method This project has been approved by the multi‐centre ethics committee. A retrospective review was conducted from patients’ histology records from public and private pathology laboratories within defined catchment areas. Criterion for analysis is a confirmed diagnosis of NMSC treated surgically. A Microsoft Access database is created that will facilitate subsequent data retrieval and analysis. Results and Conclusion It is hoped that this up‐to‐date data will form the framework for the development of sound and sustainable healthcare policies of management of NMSCs including management strategies and workforce planning, and research direction on this common disease.     

Follow up of patients with colorectal polyps: are the BSG guidelines being adhered to?

OBJECTIVE: The publication of the BSG guidelines in 2002 provided a framework for the follow up of patients with colorectal polyps. The aim of the present study was to determine whether they had, or were being correctly adhered to in a moderately sized District General Hospital. METHOD: A total of 598 patients were on the waiting list for colonoscopy at Airedale General Hospital (AGH) in February 2005. Of these, 203 were being followed up as a result of the previous finding of a polyp. RESULTS: Only 14.8% of patients had been or were being followed up according to the BSG guidelines. The majority of the 85.2% of patients who did not comply with follow up did so as a result of over investigation. Seventy-eight per cent of the low-risk group and 55% of the intermediate-risk group had been colonoscoped, or were waiting to have colonoscopy, too soon or too frequently according to the BSG guidelines. Twenty-four patients with hyperplastic polyps were being followed up incorrectly, as were 17 patients discovered to have a polyp pathology on flexible sigmoidoscopy. It was established that 131 extra colonoscopies had been, or were planned to be performed unnecessarily. CONCLUSION: These data have major implications with regard to patient safety, service provision and cost to the NHS.