Referral and treatment pathways for pseudomyxoma peritonei of appendiceal origin within a national treatment programme.

AIM: Pseudomyxoma peritonei (PMP) is a rare neoplasm of the appendix, which if untreated disseminates throughout the abdominal cavity and generates considerable morbidity. Since 2002 in the UK, patients with PMP have been managed via two nationally commissioned centres. We evaluated referrals and treatment pathways over time at the Manchester centre. METHOD: Data from all patients referred with suspected PMP were prospectively collected (2002-2015). Definitive treatment was cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy. Disease burden was quantified using the Peritoneal Cancer Index (PCI) (score 0-39) and complete cytoreduction (CC) defined by scores of 0/1. Novel treatment algorithms were developed for patients with low grade appendiceal mucinous neoplasm (LAMN) localized to the peri-appendiceal tissue. RESULTS: In all, 817 patients with confirmed PMP were referred increasing from 11 in 2002 to 103 in 2015. Disease burden was high with a mean PCI of 31 in the first quartile (Q1), levelling off to 15, 15, 17 thereafter (P = 0.002). The proportion of CC0/1 increased from 67% in Q1 to 77% Q2 and 74% Q3/4. Where complete cytoreduction was achieved, 5- and 10-year overall survival was 77% and 66%. The proportion of patients referred with localized LAMN increased over time reaching 25% each year since 2010 (Ptrend  < 0.0001). Two-thirds of localized LAMN now undergo laparoscopically assisted risk-reducing CRS. CONCLUSION: The establishment of a national treatment centre was associated with an initial presentation of patients with advanced disease. The programme has demonstrated a clear trend over time towards earlier referral and adoption of minimally invasive techniques for localized disease.

Methylxanthines for prolonged non-specific cough in children.

BACKGROUND: Non-specific cough is defined as non-productive cough in the absence of identifiable respiratory disease or known aetiology. It is commonly seen in paediatric practice. These children are treated with a variety of therapies including a variety of asthma medications. Methylxanthines, the main medication used for paediatric asthma for many decades in Western countries, is still widely used in non-Western countries. Also, methylxanthines have other pharmacological properties and their bronchodilator effect is only modest. OBJECTIVES: To evaluate the efficacy of methylxanthines in treating children with non-specific cough. SEARCH STRATEGY: The Cochrane Register of Controlled Trials (CENTRAL), the Cochrane Airways Group Specialised Register Collaboration and Cochrane Airways Group, MEDLINE and EMBASE databases were searched by the Cochrane Airways Group. The latest searches were performed in Jan 2005. SELECTION CRITERIA: All randomised controlled trials comparing methylxanthines with a placebo medication in treating children with non-specific cough. DATA COLLECTION AND ANALYSIS: Results of searches were reviewed against pre-determined criteria for inclusion. No eligible trials were identified and thus no data were available for analysis. Four small non-randomised controlled trials were reported. MAIN RESULTS: No randomised controlled trials that examined the efficacy of methylxanthines in the management of prolonged non-specific cough in children were found. In the non randomised trials above, a significant effect was seen within 2-14 days of therapy. AUTHORS' CONCLUSIONS: There is currently an absence of reliable evidence to support the routine use of methylxanthines for symptomatic control of non-specific cough in children. If methylxanthines were to be trialled in children with prolonged non-specific cough, cohort data (thus limited) suggest a clinical response (subjective cough severity) would be seen within 2-5 days (and certainly within 14 days) of therapy. However methylxanthine use has to be balanced against the well known risk of toxicity and its low therapeutic range in children. Further research examining the efficacy of this intervention is needed.

Familial sudden death. Report of a case and review of the literature.

A 15 1/2-year-old boy died suddenly while swimming. He had a family history of sudden death involving three consecutive generations, including a brother. The patient had a history of exercise-related syncope, for which he was being treated with nadolol. Autopsy showed enlarged heart, normal coronary arteries, right ventricular septal hypertrophy, quadricuspid pulmonary valve, accessory tricuspid valve, and a moderately elongated and thickened mitral valve. Conduction system revealed that the penetrating bundle was pushed to the left side of the summit of the ventricular septum by the right ventricular septal hypertrophy; it was lobulated and showed fatty-fibrous changes. These findings extended throughout the beginning of the bundle branches. We conclude that in this patient with familial sudden death and normal QT interval, the abnormal right ventricular septal hypertrophy altered the course and produced degenerative changes in the conduction system, which may have caused sudden death.