bluuebunny/crossref_metadata_2025_split huggingface dataset:
NS15P TRANSCRIPTIONAL REPRESSION IN A MOUSE MODEL OF EPILEPTOGENESIS
Authors: Wang Y. Y. , Sang Q. , Smith P. D. , Murphy M. A. , Cook M. , Tan S. S. 
Published: April 2007

Purpose  To identify differentially expressed genes between normal and epileptic hippocampi with a two‐step transcriptome analysis using a mouse model of temporal lobe epilepsy.Method  C57/Bl6 adult male mice were subjected to a rapid electrical amygdala kindling model. Large‐scale transcriptome profiling of the hippocampi was performed using LongSAGE. Comparison was performed using Fisher’s Exact test with functional categories assigned using GO annotation. Differential gene expression was independently confirmed using targeted real‐time quantitative PCR at various time‐points after kindling. Further confirmation was performed using immunoperoxidase staining and Western blots on selected genes.Results  LongSAGE analysis revealed over 50 differentially expressed transcripts. A custom‐made microfluidics low‐density array card was made from this and other possible causative genes identified from the literature for real‐time PCR. qRT‐PCR on this card identified 38 differentially expressed genes between epileptic and normal mice hippocampi. There was clear evidence of transcriptional repression in the first 24 hours after kindling on qRT‐PCR and proteomic studies. GO annotation revealed down‐regulation in the majority of intracellular components, biological processes and molecular functions particularly those involved with metal binding activities, catalytic activities and protein binding.Conclusion  There are significant differences in the gene expression between epileptic and normal hippocampi with marked transcriptional repression following epileptogenesis. This is especially evident in metabolic and morphologic genes located within membranes, parts of the cytoskeleton and the mitochondria.
NS04 PAEDIATRIC POSTERIOR FOSSA TUMOURS IN THE CANTERBURY REGION – A 25 YEAR RETROSPECTIVE REVIEW
Authors: Lightfoot N. J. , MacFarlane M. R. , Corbett R. P. , Sullivan M. J. 
Published: April 2007

Purpose  A Neurosurgical Unit was established at Christchurch Hospital in 1981 and since this time our centre has been involved in the surgical treatment of paediatric posterior fossa tumours. The purpose of this presentation is to highlight our experience with this group and to compare this with data reported elsewhere in the literature.Methodology  The Neurosurgical department maintains an extensive database of admissions and operative experience. A search was performed to identify patients with posterior fossa tumours who were less than 18 years of age at presentation. The selected patients’ clinical notes were reviewed and demographic, clinical and outcome data collected and analysed.Results  Over this 25 year period we treated 46 children in whom the radiological diagnosis of a posterior fossa tumour had been made. This represents 1.43/ 100000 children/year. Medulloblastoma accounted for 32.6%, astrocytoma 42.5% and ependymoma 15.2%. All patients underwent neurosurgery with 61% of patients undergoing either sub‐total or macroscopic resection with a 30 day mortality of 2.1%. Postoperative management with radiotherapy and/or chemotherapy was subsequently undertaken according to international guidelines, and from 2004 including those of the Childrens’ Oncology Group. The 5 year survival was 73.3% for medulloblastoma; 84.2% for astrocytoma; 85.7% for ependymoma.Conclusion  Over the last 25 years the management of posterior fossa tumours in Christchurch has evolved has evolved in line with international trends. Our outcome data is comparable to that reported elsewhere with low operative mortality and morbidity and with exceptional clinical outcomes.
NS05 PENETRATING HEAD INJURY FROM NAILGUNS: THE NEW ZEALAND EXPERIENCE. A CASE SERIES
Authors: Monteith S. J. , Winder M. J. , Lightfoot N. , Mee E. 
Published: April 2007

Introduction  Penetrating head injury from nailguns has become increasingly recognised due to their frequent use in the construction industry and home. We report a New Zealand case series of 12 penetrating nailgun injuries, the largest of its type, detailing presentation, management, risk factors and outcomes.Methods  The Auckland Neurosurgical Unit database was reviewed to identify cases of cranio‐cerebral nailgun trauma. In addition we surveyed the clinical directors of the remaining three Neurosurgical units in New Zealand for cases that had presented to their institution. From the identified cases we performed a chart review to identify demographics; past medical and psychiatric history; details surrounding the event; surgical management and clinical outcomes.Results  All 12 cases were male, with a mean age of 38 years. All but one case involved one nail of 3–12 cm in length. The other case involved a staggering 24 nails of 5cm length and represents the largest number of intra‐cranial nails in a surviving patient. GCS at presentation ranged from 3 to 15. All four patients who had a GCS less than 11 at presentation died. Two thirds of cases demonstrated a psychiatric history and three quarters of cases were attempted suicide. Operative intervention ranged from non‐operative, to a small craniotomy or craniectomy depending on the case.Conclusion  This case series would suggest a minimal surgical approach and individual case assessment regarding the need for antibiotic prophylaxis and anti‐epileptic drugs. A surprisingly good outcome can occur in many of these patients.
NS09 DETECTION AND FOLLOW‐UP OF ANEURYSMS BY CTA
Authors: Dorsch N. W. C. , Young N. , Kang A. , Ly S. 
Published: April 2007

Purpose  To analyse usefulness of CTA in detecting aneurysms in subjects with a family history, and in follow‐up of patients with known aneurysms.Methodology  From 1997 to 2004, 286 adults with a family history of cerebral aneurysms were investigated by a spiral CT angiogram of the Circle of Willis. Their age range was 20–76, and 192 were female. Over a similar period 119 patients with 138 known aneurysms, described as Group 1, and another 84 with a past history of SAH and aneurysm treatment (Group 2), were followed by serial CTAs for up to 97 months, median 36 months.Results  Of those with a family history, 193 had one CTA and the other 93 averaged 2.8 studies. Aneurysms were found in 15.4%, with 56 detected in 44 patients. There were multiple aneurysms in 12 of the 44 (27%); 80% of the aneurysms were under 7mm in diameter and 93% less than 13mm; 39% were on the MCA – all findings similar to other literature.In the 119 group 1 patients there were initially 120 aneurysms. During follow‐up 18 more developed, a rate of 5% per year. Of these 138, 99 remained stable, 29 enlarged (21%), and nine became smaller. One aneurysm ruptured, a rupture rate of 0.28% per year.In group 2 patients, 75 aneurysms were found on the initial CTA in the 84 patients. Subsequent studies showed 19 new aneurysms for a total of 94 – annual development rate 7.5%. Four aneurysms ruptured in the study period, equivalent to 1.7% annually.Conclusions  CTA is useful for relatives of aneurysm patients, for subjects with unruptured aneurysms, and for patients with past aneurysmal SAH. As others have shown, in this last group new aneurysms develop more often, they grow faster, they rupture more often, and they rupture at smaller size.
PS11 PANCREATITIS – INDICATIONS FOR SURGERY IN CHILDREN
Authors: Stringer M. D. 
Published: April 2007

Purpose  With modern imaging techniques, multidisciplinary management, and advances in supportive care, what are the indications for surgery in children with pancreatitis and have outcomes improved?Methods  During a 12 year period, 62 children [31 girls, median age 12.0 (2.3 –16.9) years] with pancreatitis were managed in a single centre. All were investigated using a combination of ultrasound, ERCP, MRCP, and CT. Management comprised supportive care and treatment of any primary pathology. Severe acute pancreatitis (pancreatic necrosis, pseudocyst/abscess, and/or organ failure) was managed with broad spectrum intravenous antibiotics, H2 antagonists, prophylactic antifungal agents, and early parenteral nutrition followed by naso‐jejunal feeding.Results  51 children had acute pancreatitis, 14 (27%) of whom had severe disease. The leading causes were trauma (12), pancreaticobiliary malunion (6), gallstones (5), drug‐induced (4) and viral (4). In 10 patients no cause was found. Twenty four (47%) of these 51 children required surgery (20) and/or interventional procedures (4) to treat primary pathology (13) or pseudocysts (11) with resolution of pancreatitis in all. Eleven children had chronic pancreatitis: 6 required surgery (5) or an interventional procedure (1).All 62 patients survived. Only two experienced surgical complications.Conclusions  In this series, using modern investigative techniques, only 20% of children with acute pancreatitis had no identifiable etiology. Half of the total series of children with pancreatitis required surgery either to treat primary pathology or to treat complications of the disease. Selective surgical intervention in children with pancreatitis is both beneficial and safe.
PS12 SONIC HEDGEGHOG PATHWAY – A POSIBLE THERAPEUTIC TARGET FOR HEPATOBLASTOMA
Authors: Arsic D. , Sullivan M. J. , Beasley S. W. 
Published: April 2007

Background and Objectives  It has been shown that the hedgehog (Hh) signalling pathway that is turned off once organogenesis is complete in the embryo is re‐activated in a number of different cancers. We analyzed re‐activation of the Hh pathway in hepatoblastoma to investigate whether this pathway can be used as a therapeutic target.Methods  Surgically resected hepatoblastoma specimens and hepatoblastoma cell lines were analyzed by immunoblot and PCR, looking into different components of the pathway.Results  We found that pharmacological pathway blockade leads to decreased cell proliferation in hepatoblastoma cells, demonstrating an ongoing requirement of Hh signalling in this tumour.Conclusion  The Hh pathway may be a useful therapeutic target for hepatoblastoma and other tumours in which the Hh pathway (or a component of the pathway) are over‐expressed or re‐activated at an inappropriate time.
PS13 DEVELOPMENT OF THE URINARY BLADDER IN ETU‐EXPOSED FETAL RATS IS AFFECTED BY DOWNREGULATION OF SHH AND ITS TARGET GENESCAGES
Authors: Quan Q. B. , Sullivan M. J. , Beasley S. W. , Mandhan P. 
Published: April 2007

Objective  The cloaca develops into the urinary bladder and distal part of the hindgut. Abnormalities of cloacal development result in a variety of anorectal and urogenital malformations. This study evaluated the formation of the urinary bladder in ETU exposed fetal rats in relation to shh and its target genes.Methodology  Embryos of pregnant SD females administered 1% ETU (125 mg/kg) on the tenth day of gestation (gD10) were collected from gD12‐16 and gD21. Cloacal development was viewed with serial histology followed by qualitative and quantitative gene expression studies of shh and target genes to elucidate the expression pattern of these genes during each time point.Results  ETU exposed fetuses have incomplete separation of the cloaca leading to a variety of anorectal and urinary abnormalities eg rectourethral fistula. Shh and downstream genes are expressed during urinary bladder development in fetal rats. Relative quantification showed that expression of shh, BMP4 and Hox genes are reduced in ETU‐exposed fetal rats during the process of cloacal separation and development of the urinary bladder.Conclusion  This study demonstrates that ETU‐exposure in fetal rats produces abnormal cloacal development, including urinary fistulae. Shh cascade is active during the process of cloacal separation and urinary bladder development and down regulation of shh, BMP4 and Hox genes during this process may contribute to the abnormal development of urinary bladder and anorectum.
PS02 ATRESIA OF THE JEJUNUM AND ILEUM: THE DISTINCTION
Authors: Tongsin A. T. 
Published: April 2007

Purpose  Atresia of the jejunum and ileum is one of the major causes of neonatal intestinal obstruction. Most affected newborn infants present with bilious emesis and abdominal distention. Traditionally, jejunal and ileal atresia have been grouped together as jejunoileal atresia. From our observation and experience, we found many differences regarding clinical features and outcome between these two groups. The purpose of this study is to elucidate the difference between jejunal and ileal atresia.Methodology  A retrospective analysis of patients diagnosed with jejunal or ileal atresia during 1991–2005 was carried out.Results  There were 68 patients with jejunal atresia and 56 with ileal atresia. The mean birth weight and gestational age of patients with jejunal atresia were significantly lower than those with ileal atresia. Antenatal perforation occurred more frequently in ileal atresia. Postoperative course was more prolonged and mortality was higher in jejunal atresia. Prolonged ileus and anastomotic dysfunction requiring long‐term parenteral nutrition were the major causes of complications leading to death.Conclusion  There were many differences between patients with jejunal atresia and those with ileal atresia. The more compliant jejunal wall allows massive dilatation upon obstruction with subsequent loss of peristaltic activity, thus poorer outcome in comparison with ileal obstruction. We suggest that atresia of the jejunum and ileum be considered distinctly.
PS03 IS THE PREDICTED OUTCOME OF DIAPHRAGMATIC HERNIA DIAGNOSED IN UTERO, DETERMINED BY POSTNATAL CARE?
Authors: Russell G. A. B. , Anderson N. , Kang A. 
Published: April 2007

Outcome of isolated left congenital diaphragmatic hernia (CDH) has been predicted by the position of the fetal liver and lung:head ratio (LHR) on ultrasound scans between 22–28 weeks gestation (1). Variations in postnatal management will determine if the findings of such studies can be generalised.This retrospective study aimed to (1) determine if outcome, predicted by antenatal findings, was achieved in a small regional centre and (2) identify what postnatal factors could adversely alter a predicted favourable outcome.Method  All fetal cases of isolated CDH diagnosed between 1998 and 2006 were included if liver position and LHR could be measured and babies were born alive. Survival to discharge was compared to the predicted chance of survival (1). Significant physiologic disturbances, possibly due to care practices including lung overdistension, were identified.Results  Ten cases were identified. The median (range) gestation at earliest estimate of liver position and LHR was 27 (16–35) weeks. The median (range) of LHR was 1.28 (0.63–3.35) and part of the liver was in the thoracic cavity in 2/10. The overall survival was 7/10 (70%). Eight babies had a predicted survival of 70%. Three cases predicted to have a favourable outcome had significant and unexpected perioperative deterioration related to lung overdistension, and 2 died.Conclusion  Failure to achieve expected outcome in individual cases may be determined by avoidable postnatal factors.
PS05 LONG‐TERM OUTCOMES IN NEONATAL SURGERY
Authors: Stringer M. D. 
Published: April 2007

Historically, for good reason, the main measures used to assess outcome after neonatal surgery have been mortality and early postoperative morbidity. These remain crucial yardsticks of surgical care. However, as results have steadily improved more refined indicators of outcome are needed. Long‐term results and quality of life outcomes are being demanded increasingly by clinicians, parents, and health economists.For paediatric surgeons, knowledge of long‐term outcomes not only enable us to inform parents and patients better about future health expectations but also help us to anticipate potentially avoidable late complications. Outcomes inform us when to operate and when not to operate. Some neonatal surgical conditions and their treatment have almost no long‐term sequelae but most have definite consequences of variable severity.Common themes run through the analysis of long‐term outcomes. These centre around the future impact on growth, risk of malignancy, psychological issues, implications for fertility, sexuality and inheritance, and quality of life. There are many reasons why long‐term data are lacking. These include the difficulties of maintaining long‐term surveillance within healthcare systems overstretched by lack of manpower and funding. Problems of continuity are exacerbated by geographic mobility in society. Robust long‐term studies require careful data collection over many years, the co‐operation of ex‐patients, and familiarity with tools such as independent quality of life measures not readily accessible to paediatric surgeons.Only by addressing long‐term outcomes will the true impact of neonatal surgical conditions be fully understood.
PS06 LONG‐TERM OUTCOME OF BILIARY ATRESIA IN NEW ZEALAND
Authors: Yu T. , Morreau P. , Beasley S. , Brown S. , Samarakkody U. , Mandhan P. 
Published: April 2007

Objective  This is a multicenter study to assess the long‐term outcomes of children with biliary atresia (BA) who have undergone Kasai operation in New Zealand.Material and Methods  This retrospective analysis includes 97 patients who underwent a Kasai operation in the tertiary paediatric surgical centres between 1989 and 2006 in New Zealand. The demographic data, age at diagnosis and surgery, success of surgical procedure, need for liver transplantation and long‐term outcomes were studied.Results  BA was diagnosed in 58 Mäori and Pacific People, 31 Caucasian and 8 Asian children. The average age and time at presentation and surgery was 50.37(±37.3) and 61.6 (±25.7) days respectively. Eighty‐six (89%) children had recoloration of the stool and 71 (68%) became jaundice free (bilirubin <20 µmol/L) 1 year after the surgery. Ascending cholangitis was the commonest complication and occurred in 41% of children. Five and 10‐year survival rates with native liver were 47% and 23% respectively. Liver transplantation was performed in 50 patients and 38 (76%) survived. The overall actuarial 5 and 10‐year survival rates for BA patients were 45.4% and 27.8% respectively.Conclusion  BA was more common in Mäori and Pacific People children. There was a trend of less favourable results in children who had surgery late (>90 d). Long‐term prognosis was not directly related to age at diagnosis and surgery. The survival of patients with and without liver transplantation was similar to that of other series.
PS08 BILIARY ATRESIA: POLITICS AND PROGRESS – A UK PERSPECTIVE
Authors: Stringer M. D. 
Published: April 2007

Biliary atresia (BA) is a congenital obliterative cholangiopathy of unknown aetiology. It is a rare condition with an incidence of ∼1 in 17,000 live births in the UK. BA must be diagnosed promptly in any infant with conjugated hyperbilirubinaemia since prognosis is improved by early detection and timely surgery (Kasai portoenterostomy).In the UK, a survey of all infants with BA conducted between 1993 and 1995 demonstrated that outcome was related to centre experience. Consequently, in 1999 the management of BA in England and Wales was centralised to three supra‐regional paediatric liver units. The results of this national change in practice were carefully audited. This showed that approximately 60% of all infants undergoing Kasai portoenterostomy for BA in these centres now achieve clearance of jaundice (plasma bilirubin <20 umol/l). Infants with BA that require a liver transplant remain under the care of the same team and can be listed for transplant at the optimum time.An additional benefit of centralised management has been to facilitate clinical research as a result of concentrating a relatively rare disease into specialist centres with multidisciplinary teams. Recent advances include improved diagnostic accuracy of BA using ultrasound scanning and a better understanding of the role of adjunctive postoperative corticosteroids in improving outcome.Despite these advances, BA remains the commonest indication for liver transplantation in children. Five‐year survival after liver transplantation for BA is currently 80–90%. Current medical and surgical management has transformed a disease that was almost invariably fatal in the 1960s into one with an overall 5‐year survival of about 90%.
PS09 CONTEMPORARY SURGICAL MANAGEMENT OF SILALORRHEA
Authors: Hornibrook J. 
Published: April 2007

Peristsent sialorrhea (drooling) is often a problem in cerebral palsy, mental retardation and neurological conditions. Management modalities have included behavioural and postural modifications, pharmacotherapy, radiotherapy and surgery. Early surgical treatments were removal of glands, duct ligations and neurectomies. The newest and most conservative operation is relocation of submandibular ducts to the tongue base and removal of sublingual glands. In a personal series of 16 cases 11 were children.Purpose  To review the indications and results of submandibular duct relocations (with sublingual gland removal) in 11 children with uncontrolled sialorrhea.Methodology  Review of hospital and outpatient records, and illustration of the operative technique on a video. Anatomical variations can be encountered.Results  The median hospital stay was 2 days. There were no significant complications. Caregivers judged the efficacy with a median score of “75%”.Conclusion  Duct relocation with sublingual gland removal is a safe and reliable technique for sialorrhea, with very good control in most cases. In contrast to botulinum toxin treatment its effects are permanent.
PM01 SPINAL CORD STIMULATION FOR CHRONIC REFRACTORY ANGINA
Authors: Acland R. H. 
Published: April 2007

Spinal cord stimulation (SCS) involves the use of an epidurally placed, multiple electrode lead. Electrical stimulation between electrodes produces stimulation of the posterior spinal cord and can provide excellent pain relief and increased blood flow in a number of chronic limb pain conditions. Its efficacy has more recently been demonstrated in angina.Chronic stable angina pectoris is a major cause of disability and suffering. The aims of treatment are to prevent MI and death (increase the quantity of life) and reduce the symptoms (improve the quality of life). In the non‐acute condition, practitioners often struggle to manage angina because of a lack of understanding of modern concepts of pain. There is a widely held misconception that only revascularisation improves prognosis in chronic refractory angina‐ the term used to describe patients with stable angina that is treatment refractory. Over the last decade two pain services in New Zealand have been approached by cardiologists to provide spinal cord stimulation for such patients. This has been an intriguing experience! We have demonstrated that the placement of such leads in the lower cervical cord region provides excellent relief of angina. The technology is expensive (akin to the overall cost of CAVG surgery). We have demonstrated cost recoupment, by decreased hospitalisation, at approximately16 months post procedure. Sadly, this therapy has not been embraced. Possible reasons for this will be discussed. The concept of electrically modifying the neuronal signals versus re‐plumbing the blockage appears to be an anathema to cardiologists.