Liver disease develops in one-third of patients with cystic fibrosis (CF). It is rare for liver disease to have its onset after 20 years of age. Lung disease, however, is usually more severe in adulthood. A retrospective analysis was performed on nine patients. Three patients required lung transplantation approximately a decade after liver transplant, and another underwent combined liver and lung transplants. Four additional patients with liver transplants are awaiting assessment for lung transplants. One patient is awaiting combined liver and lung transplants. With increased survival in CF, several patients may require more than single organ transplantation.

Background: Children born preterm are at increased risk for learning difficulties and special educational needs (SEN) compared with term-born peers. This will challenge the education system as increasing numbers survive. To date, teachers' knowledge in this area has not been studied. Aim: To investigate teachers' knowledge of the outcomes and educational needs of children born preterm. Methods: A qualitative study of 13 education professionals in UK primary schools. Semi-structured interviews explored: Results: Conclusions: Teachers' knowledge of the educational needs of preterm children is limited and related only to personal experience. There is a pressing need to communicate information to education professionals about the impact of preterm birth on long-term outcomes for educational management and service provision. These findings will be used to develop structured questionnaires to further explore this area.

Background: The Gatekeeper ™ Reflux Repair System (Medtronic, Shoreview, MN) is a promising endoscopic therapy that is designed to augment the functioning of the lower esophageal sphincter through the implantation of prostheses into the submucosal layer of the LES in an outpatient procedure. These prostheses, made of a biocompatible hydrogel material, expand upon contact with moisture, creating a mechanical barrier between the stomach and the esophagus, restricting the flow of gastric contents into the esophagus. Preliminary data from 68 patients in a European Multi-center study showed implantation of approximately 4 prostheses at the LES improved GERD symptoms in a majority of PPI dependent GERD patients. Aim: To evaluate the effects of the Gatekeeper Therapy in symptomatic GERD patients in a large, prospective, multi-center, randomized, sham-controlled study. Method: Recruitment is ongoing for a Multicenter U.S./European study. Inclusion criteria include: Symptomatic GERD with symptom control on PPI therapy. Baseline 24 hr with pH<=4.0 =>4% of time. Baseline GERD-HRQL >=20 off PPI. Exclusion criteria: Barrett's > 2cm, strictures, varices, grade 3 or greater esophagitis, hiatal hernia > 3cm, prior anti-reflux surgery. Procedure: Implant 4 prostheses circumferentially in LES. 144 patients will be randomized by a sealed envelope at ratio of 2:1 implant vs. sham. Blind will be broken at 6 months, with sham patients offered Gatekeeper therapy. Outcome parameters being evaluated include HRQL, esophagitits, medication use, 24 hr pH, and implant persistence. Results: Enrollment started 09/24/03 with 210 subjects enrolled and 52 subjects randomized and implanted to date (12/01/04). Summary: The study is ongoing with enrollment completion anticipated by April, 2005.

Purpose: The long-term outcomes after endoscopic GERD therapy are not fully known, since these interventions have only been recently introduced. As part of the initial cohort study of the BARD EndoCinch, thirteen patients underwent endoscopic gastric plication between March 1998 and April 1999. At 6 months post-procedure, 9 of 13 were no longer taking any acid suppressive therapy. Mean 2-year follow-up showed 5/13 were on no antisecretories and another 4 were on < 50% of their usual pre-treatment dose. At a follow-up of nearly 2 years later (mean 3.8 yrs post-treatment) these results had not changed. We present further long-term follow-up in this cohort, now a mean of 6.4 years after initial treatment. Methods: Treated subjects had mild GERD, required daily PPIs, were responsive to the antisecretories and had abnormal pH profiles. No patients had a hiatal hernia >2cm or Barrett's esophagus. They received 2 to 4 linear or circumferential plications during their procedure. Of the original 13 subjects, 12 have agreed to annual questionaires about medication use and to assess GERD symptoms off medications. Results: At a mean follow-up of 6.4 years (range 6.1 to 7.2 years), only 2/12 patients remain completely off PPIs. One additional subject uses 2 doses of PPI per week, and another takes a PPI every other day. 8 patients are back on their pre-procedure daily PPI doses, and one subject underwent a Nissen fundoplication (still symptomatic). No patient or procedural characteristics appeared predictive of long-term improvement or medication use. Conclusions: Compared with follow-up at a mean of 24 and 41 months, where 5/13 subjects continued to be off all antisecretory therapy post-procedure, only 2 remained off all PPIs at a mean of 6.4 years after treatment. While there was a durable response for nearly 40% of treated subjects for about 3.5 years, this further follow-up may reflect the natural history of gastric plication treatment. Factors such as the effect of the investigator's learning curve and the role of placebo response should be addressed by further study of endoscopic GERD treatment outcomes in larger cohort series and in randomized controlled trials.

A study of Conger conger diet was carried out with 213 specimens from Irish inshore waters (0–200 m) collected from winter 1998 to spring 1999 and 96 from offshore waters (200–600 m) from spring to summer 2000. The primary diet in both areas was fish, with a complete species shift in diet between areas. The dominant species inshore were Merlangius merlangus and offshore Micromesistius poutassou. Decapod crustaceans and cephalopods were observed in stomachs, but contributed little to the overall diet.

A sample of 166 conger eel, Conger conger (Linnaeus, 1758) were captured between August 1998 and June 1999, from the inshore south coastal waters of Ireland using demersal trawl, longlines, lobster and shrimp pots. The range in length was 33–159cm and weight was 0.05–12.0kg. Age, determined from vertebrae and sagittal otoliths, ranged from 4 to 20 years. Vertebrae were found to be the most effective tool for ageing. A linear relationship was found between total length and otolith radius. A curvilinear model best described the relationship between total length and vertebral radius. Both otoliths and vertebrae were used in the back-calculation technique and the von Bertalanffy growth function was fitted. Population growth curves were constructed from both vertebral and otolith measurements. Growth parameters, L∞ and k were estimated for individual fish showing large ranges in values. L∞ from otoliths ranged from 170 to 320m, and k from 0.01 to 0.09. From vertebrae, L∞ ranged from 130 to 390m and k from 0.01 to 0.13. Population estimates from otoliths gave results of L∞=271cm, k=0.037, t0=−1.396 and from vertebrae gave results of L∞=214cm, k=0.059, t0=−1.960. Condition factor ranged from 0.03 to 1.73 and gonadosomatic index from 0.53 to 7.05. The greatest increase in condition occurred between summer and autumn, while the greatest increase in gonadosomatic index occurred between autumn and winter. It was not possible to determine the sex of the eels from gross gonad appearance. Histological analysis of the gonads revealed that all conger eels were female. The absence of males and of ripe or spent females in the inshore south coastal inshore waters suggests that conger eels leave this region to spawn. This study has provided the first published data on age, growth and gonad development of a population of the species in the Northeast Atlantic.

Results are presented from an extensive fyke net study of the Erne lakes undertaken in 1998 and 1999 with a sampling effort of over 56,000 net-nights. Highest catches were obtained from Upper and Lower Lough Erne in the lower reaches of the catchment where the greater part of commercial eel fishing takes place. CPUE ranged from 30 to 45 eels weighing 4.5-8.5 kg per 10 fyke nets per night. Mean length ranged from 42 to 44 cm with growth rates of 2.5-3.5 cm per year. In contrast, numbers from the upper reaches of the catchment were lower (4-15 eels, 1.3-4.2 kg) and were characterized by the preponderance of large 46-64 cm, fast growing females (4.1-4.8 cm per year). The results indicate that: the effect of stocking by overland transport of elver can be discerned using commercial gear after as little as five years; and that eel production can be greatly enhanced by stocking, thanks to the existence of extensive areas of excellent habitat which are seriously under-populated. The data provide essential baseline information for the assessment of the progress of a major enhancement program initiated in 1997. The results clearly support the initial assumption, made on the basis of small samples taken in the 1970s, that a stocking program would be effective.

BACKGROUND:Colonisation with Burkholderia cepacia complex in patients with cystic fibrosis (CF) has been associated with adverse outcomes. The aim of the present study was to determine the actuarial survival of CF patients colonized with B. cepacia and to evaluate the efficacy of the Royal Prince Alfred Hospital segregation policy. A secondary aim was to characterize the specific genomovars and strains of B. cepacia isolated in an Australian clinic. METHODS:Retrospective review of spirometric and microbiological data on all patients colonized with B. cepacia. Each B. cepacia-colonized subject was matched with three case-control subjects. Phenotype and genomovar typing, random amplified polymorphic DNA strain type and B. cepacia epidemic strain marker analyses were performed. The effect of B. cepacia colonization on transplant-free survival was estimated by Cox's proportional hazards regression using the entire clinic population. RESULTS:Fifteen patients were colonized with B. cepacia, of whom six (40%) had died from CF-related disease by August 1998, compared with 30 of 173 (17.3%) of the entire clinic population. Cepacia status had a significant adverse effect on survival, with a hazard ratio of 2.16 (95% confidence interval 1.0-4.69; P = 0.05). The outcome was variable in subgroups of B. cepacia. DISCUSSION:Colonization with B. cepacia had a significant adverse effect on survival within the study population. Genomovar and strain typing contributed usefully in accessing the effectiveness of the hospital's segregation policy in preventing cross-colonization.

The aim of the study was to measure the effect of a short course of recombinant human deoxyribonuclease I (rhDNase) on ciliary and cough clearance in a group of cystic fibrosis patients, using a radioaerosol and gamma camera technique. Patients were initially randomized to receive either rhDNase (2.5 mg qd) or placebo. Following the measurement of baseline clearance, patients were given a 7-day course of either rhDNase or placebo. The patient then returned on the seventh day for follow-up clearance measurements. This was followed by a 2-week washout period before the whole process was repeated with the alternative inhalation solution. On each of the study days, mucociliary clearance was initially measured for a period of 60 min (IC). This was followed by cough clearance (CC) measurements for 30 min, during which patients were requested to cough a total of 120 times. Post-cough clearance (PCC) was then measured for a further 60 min. Thirteen patients completed the study. Patients' age ranged between 18-38 years, and they had baseline values of FEV(1) of 27-103% of predicted values. Following completion of the course of rhDNase, there was a mean percent increase from baseline of 7.5% for FEV(1) and 5.4% for FVC% (P = 0. 03). There was a small, nonsignificant increase in IC (6.2 +/- 3.6%) on the rhDNase arm compared with the placebo arm (-2.3 +/- 2.9%), P = 0.1. No changes were seen in either CC (1.0 +/- 3.2% [rhDNase] vs. 1.9 +/- 2.4% [placebo], P = 0.9) or PCC (-0.7 +/- 1.5% [rhDNase] vs. 0.9 +/- 1.7% [placebo], P = 0.3). Patients who achieved a 10% or greater improvement in FEV(1) (n = 5) in response to rhDNase did not show any greater change in clearance than nonresponders. In conclusion, we were unable to demonstrate any improvements in either ciliary or cough clearance in response to a short course of rhDNase. The mechanism of action of this drug in vivo remains uncertain.

Data on catches of O+ elvers from nine countries in Europe and of juvenile eels ascending the St. Lawrence River in Canada are presented and compared with previous records. Consideration of a parallel decline in recruitment in Canada supports the theory that oceanic conditions are responsible for the observed fall in catches. A substantial increase in the Loire, to 95 tons in 1994, may indicate an overall improvement in stocks.

Abstract– A total of 8,903 eels were collected in 503 fyke‐net samples during the months March to December from 1981 to 1994 in a small bay in Lough Derg, a mesotrophic lake. Variations in catch between months and between years are described. In addition to expected decreases in catch in the colder months, a regular reduction was observed in June. Low catches were observed from 1985 to 1987, coinciding with low water temperatures. A very low catch in August 1994 may have been a chance variation but could have been a first indication of the effects of poor recruitment or of increased commercial fishing effort. The results support the initial hypothesis that a long‐term study is required to determine the extent of variation in an eel population.