A cohort study of children and young people with progressive neuromuscular disorders: clinical and demographic profiles and changing patterns of referral for palliative care.

BACKGROUND: Progressive neuromuscular disease in children is life limiting and these children and young people would benefit from palliative care services, but data are limited on the number and demography of these children. AIM: To describe the clinical and demographic profile of children referred to a Children's hospice in the UK with progressive neuromuscular disease. SETTING/PARTICIPANTS: All children and young people with progressive neuromuscular disorders referred to Martin House Children's Hospice between 1987 and 2010. DESIGN: Retrospective cohort study. RESULTS: 300 children with progressive neuromuscular disease were referred to the hospice. Seventy percent (210) of these children had Duchenne Muscular Dystrophy, 22% (67) had Spinal Muscular Atrophy (34 with Type I) and 8% had other neuromuscular diseases. Numbers of referrals have not significantly increased over the last 15 years, although an increasing number come from a South Asian background (from 4% to 32%) and a higher number of children have conditions other than Duchenne Muscular Dystrophy. A total of 55.3% (166) of all referrals came from areas of the highest deprivation. Survival patterns varied by diagnostic group, but ethnicity and deprivation were not associated with survival in these children. CONCLUSIONS: The profile of children with progressive neuromuscular conditions who were referred for palliative care has changed over the last 20 years, with a different spectrum of underlying diagnoses and a greater number from a South Asian background. The higher than expected proportion of children living in areas of high deprivation has been consistent over time.

Parotid duct transposition in dogs: a retrospective review of 92 eyes from 1999 to 2009.

OBJECTIVE: The aim of this study was to perform a retrospective review of parotid duct transposition (PDT) in the dog to determine the rate and nature of complications, the success and failure rates and to evaluate owner satisfaction. METHODS: Medical records of 56 dogs (92 eyes) that underwent PDT and subsequent follow-up by a veterinary ophthalmologist were reviewed. Forty owners (40 dogs/66 eyes) were contacted by telephone and 37 owners (37 dogs/60 eyes) also completed a visual analog scale questionnaire. Statistical evaluation included Wilcoxon Signed Rank tests, one-way analysis of variance and Kaplan-Meier survival analysis with Wilcoxon and Log-rank tests. RESULTS: The mean follow-up was 38.7 months (range 1-109 months). The surgical success rate was 92% (85/92 eyes). Total failures (8%, 7/92 eyes) were because of severe saliva intolerance (n = 5 eyes) and PDT failure (n = 2 eyes). The complication rate was 50% (46/92 eyes) of which 61% (28/46 eyes) were managed medically and 39% (18/46 eyes) required further surgery. Ninety percent (36/40) of owners indicated that they would proceed with surgery again. Statistically significant improvements in owner perception of ocular comfort, number of daily topical treatments, ocular wetness, and postoperative vision were identified. CONCLUSIONS: This study shows that PDT is a successful procedure based on clinical findings and in terms of owner perception. It has also demonstrated that PDT improves ocular comfort and vision in medically refractive cases of keratoconjunctivitis sicca, and that a low level of on-going management is required in 33% of cases.

Efficacy of lamellar resection, cryotherapy, and adjunctive grafting for the treatment of canine limbal melanoma.

OBJECTIVE: To evaluate the efficacy and complication rate of partial lamellar resection followed by cryotherapy for the management of canine limbal melanoma. ANIMALS STUDIED: Fourteen dogs with unilateral canine limbal melanoma which were managed surgically by partial lamellar resection, cryotherapy (1,1,1,2-tetrafluoroethane) and an adjunctive graft procedure. METHODS: The clinical records of dogs treated between June 1998 and June 2008 were reviewed. The signalment, approximate size and location of the melanoma, variation in surgical technique, recurrence rate, short-term (< three months) and long-term (> three months) complications were assessed. Follow-up information was collected by patient re-examination or telephone interview. RESULTS: The mean age at diagnosis was 6.3 years, with a range from 3.2 to 12 years. Seven breeds were affected, including five cross-breed dogs and four Golden Retrievers. The tumour involved the dorsal arc (from the dorsomedial to the ventrolateral quadrant) in 12 eyes and the ventral arc in two eyes. The size of the tumour ranged from 30 to 180 degrees of the limbal circumference. A double freeze-thaw cycle of cryotherapy was performed in 7/14 eyes and a triple freeze-thaw cycle in 7/14 eyes. An adjunctive conjunctival graft was performed in 13/14 eyes (free graft n = 3, posterior nictitans conjunctiva/cartilage n = 4, advancement graft n = 5, small intestinal submucosa/advancement graft n = 1) and a frozen homologous graft in 1/14 eyes. The duration of follow-up ranged from 6 months to 8.5 years with a median of 2.1 years. Recurrence was not clinically detected in any of the 14 eyes. Early complications occurred in 8/14 eyes and included anterior uveitis (7/14), corneal ulceration (5/14), marked corneal granulation tissue at the graft margin (2/14), dyscoria (2/14), corneal lipidosis (1/14) and corneal oedema (1/14). Intra-operative globe perforation had occurred in 5/7 eyes with anterior uveitis and 2/2 eyes with transient dyscoria. Late complications occurred in 3/14 eyes and included corneal lipidosis which was either mild (< 2mm in diameter, 1/14) or marked (>1cm in diameter, 2/14). Marked lipidosis only occurred following the treatment of extensive limbal melanomas which involved approximately 50% of the limbal circumference. CONCLUSIONS: Surgical management comprising partial lamellar resection, cryotherapy and adjunctive graft placement is technically straightforward, minimally invasive, well tolerated and highly effective. Marked corneal lipidosis is most likely to occur as a post-operative complication when the limbal melanoma is extensive.

A survey of clinician and patient attitudes towards the use of progesterone for women at risk of preterm birth.

BACKGROUND AND AIMS: To assess the current use of vaginal progesterone in women at increased risk of preterm birth among practitioners within Australia and New Zealand, and the willingness of both clinicians and women to participate in a randomised controlled trial to further evaluate the role of progesterone in preterm birth. METHODS: A survey of fellows and members of the Royal Australian and New Zealand College of Obstetricians and Gynaecologists, and women who had a spontaneous preterm birth at less than 34 weeks gestation, at the Women's and Children's Hospital was conducted. RESULTS: A total of 1430 surveys were distributed to members and fellows, of which 738 (52%) were returned. Of these, 490 were from currently practising obstetricians (34% of total college membership). Twelve of the 490 (2%) respondents indicated that they currently use progesterone in women with a previous spontaneous preterm birth at less than 34 weeks gestation. Of the respondents, 317 (65%) indicated a willingness to participate in a multicentred randomised controlled trial assessing the use of progesterone in women with a previous spontaneous preterm birth at less than 34 weeks gestation. A total of 207 eligible women identified from the hospital database were sent a questionnaire, with responses obtained from 119 women (57%). Overall, women were satisfied with their preterm birth experience. Fifty-two women (44%) indicated a willingness to consider participation in a randomised trial of vaginal progesterone. CONCLUSIONS: Progesterone is not widely used in Australia and New Zealand for women considered at increased risk of preterm birth. Conducting a randomised trial of vaginal progesterone is feasible.