Womens And Childrens Hospital

Adelaide, Australia

Womens And Childrens Hospital

Adelaide, Australia
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STUDY DESIGN.: Systematic Review OBJECTIVE.: To determine the indications and outcomes for surgical treatment of thoracolumbar kyphosis in patients with mucopolysaccharidoses (MPS) in order to define future studies. SUMMARY OF BACKGROUND DATA.: Improvements in the medical treatment of MPS have increased lifespan and improved quality of life for many patients, but with no effect on thoracolumbar kyphosis. A greater number of these challenging patients may now be considered spinal surgical candidates. METHODS.: In accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, a PubMed database search was conducted using relevant keywords to identify articles describing MPS patients with thoracolumbar kyphosis treated surgically. Information was collected regarding demographics, indications for surgery, surgical details and outcomes. RESULTS.: 16 articles describing 58 patients were included for review. The median age at surgery was 7 years (range 2.4 – 65 years) and median follow-up was 3.7 years (range 7 weeks – 10.3 years). The most common reported surgical indication was progression of deformity in 39/58 patients (67%). Pre-operative neurological compromise was reported in 7/58 (12%) patients and resolved completely post-operatively in all paediatric patients. Three paediatric patients had died prior to final follow-up. Post-operative paraplegia was reported in 2/51 patients who were neurologically intact pre-operatively. Additional significant peri-operative medical complications were reported in 9 patients. CONCLUSION.: Thoracolumbar spinal surgery is most commonly performed in MPS I. Pre-operative neurological compromise associated with thoracolumbar kyphosis was reported only in MPS IV and VI, where it was associated with factors other than the degree of kyphosis. Peri-operative complications are common, may be catastrophic and relate to the complexity of the MPS condition. The indications for thoracolumbar kyphosis surgery in other conditions of short stature cannot be extrapolated to MPS patients.Level of Evidence: 4 Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

Louise Hull M.,University of Adelaide | Nisenblat V.,Womens and Childrens Hospital
Reproductive BioMedicine Online | Year: 2013

microRNA (miRNA) have emerged as important epigenetic modulators of gene expression in diverse pathological and physiological processes. In the endometrium, miRNA appear to have a role in the dynamic changes associated with the menstrual cycle, in implantation and in the pathophysiology associated with reproductive disorders such as recurrent miscarriage and endometriosis. This review explores the role of miRNA in endometrial physiology and endometrial disorders of reproduction and also raises the prospect that circulating miRNA may modulate endometrial function or reflect disordered endometrial activity. The clinical potential to use miRNA in diagnostic tests of endometrial function or in the treatment of endometrial disorders will also be discussed. © 2013, Reproductive Healthcare Ltd. Published by Elsevier Ltd. All rights reserved.

Hetzel B.S.,Womens and Childrens Hospital
Asia Pacific Journal of Clinical Nutrition | Year: 2012

Iodine deficiency is the most common preventable cause of brain damage with more than 2 billion people from 130 countries at risk. The global problem of iodine deficiency has been redefined by a readily transmitted population concept, with an easy acronym - the concept of the iodine deficiency disorders (IDD) - referring to all the effects of iodine deficiency in a population, that can be totally prevented by correction of iodine deficiency with special emphasis on brain damage and not just to goitre and cretinism (1983). This was followed by the creation of the International Council for Control of Iodine Deficiency Disorders (ICCIDD) supported by WHO and UNICEF with 700 multidisciplinary professionals from more than 100 countries, committed to providing technical assistance to national programs for the elimination of IDD (1986). The WHO policy of Universal Salt Iodization (USI) has been widely adopted which requires iodization of all food for human and animal consumption by the use of iodized salt (25-40 mg I/kilo). Simple practical methods for monitoring - by the measurement of salt iodine and urine iodine were developed and promoted on a large scale with the technical assistance of the ICCIDD.

Khong T.Y.,Womens and Childrens Hospital
Pathology | Year: 2010

The generation of a pathology test result must be based on criteria that are proven to be acceptably reproducible and clinically relevant to be evidence-based. This review de-constructs the umbilical cord coiling index to illustrate how it can stray from being evidence-based. Publications related to umbilical cord coiling were retrieved and analysed with regard to how the umbilical coiling index was calculated, abnormal coiling was defined and reference ranges were constructed. Errors and other influences that can occur with the measurement of the length of the umbilical cord or of the number of coils can compromise the generation of the coiling index. Definitions of abnormal coiling are not consistent in the literature. Reference ranges defining hypocoiling or hypercoiling have not taken those potential errors or the possible effect of gestational age into account. Even the way numerical test results in anatomical pathology are generated, as illustrated by the umbilical coiling index, warrants a critical analysis into its evidence base to ensure that they are reproducible or free from errors. © 2010 The Royal College of Pathologists of Australasia.

Charlton S.,Womens and Childrens Hospital
Cochrane database of systematic reviews (Online) | Year: 2010

The transversus abdominis plane (TAP) block is a peripheral nerve block which anaesthetises the abdominal wall. The increasing use of TAP block, as a form of pain relief after abdominal surgery warrants evaluation of its effectiveness as an adjunctive technique to routine care and, when compared with other analgesic techniques. To assess effects of TAP blocks (and variants) on postoperative analgesia requirements after abdominal surgery. We searched specialised registers of Cochrane Anaesthesia and Cochrane Pain, Palliative and Supportive Care Review Groups, CENTRAL, MEDLINE, EMBASE and CINAHL to June 2010. We included randomised controlled trials (RCTs) comparing TAP block or rectus sheath block with: no TAP or rectus sheath block; placebo; systemic, epidural or any other analgesia. At least two review authors assessed study eligibility and risk of bias, and extracted data. We included eight studies (358 participants), five assessing TAP blocks, three assessing rectus sheath blocks; with moderate risk of bias overall. All studies had a background of general anaesthesia in both arms in most cases.Compared with no TAP block or saline placebo, TAP block resulted in significantly less postoperative requirement for morphine at 24 hours (mean difference (MD) -21.95 mg, 95% confidence interval (CI) -37.91 to 5.96; five studies, 236 participants) and 48 hours (MD -28.50, 95% CI -38.92 to -18.08; one study of 50 participants) but not at two hours (all random-effects analyses). Pain at rest was significantly reduced in two studies, but not a third.Only one of three included studies of rectus sheath blocks found a reduction in postoperative analgesic requirements in participants receiving blocks. One study, assessing number of participants who were pain-free after their surgery, found more participants who received a rectus sheath block to be pain-free for up to 10 hours postoperatively. As with TAP blocks, rectus sheath blocks made no apparent impact on nausea and vomiting or sedation scores. No studies have compared TAP block with other analgesics such as epidural analgesia or local anaesthetic infiltration into the abdominal wound. There is only limited evidence to suggest use of perioperative TAP block reduces opioid consumption and pain scores after abdominal surgery when compared with no intervention or placebo. There is no apparent reduction in postoperative nausea and vomiting or sedation from the small numbers of studies to date. Many relevant studies are currently underway or awaiting publication.

Gould J.F.,Womens and Childrens Hospital | Smithers L.G.,Womens and Childrens Hospital | Makrides M.,Womens and Childrens Hospital
American Journal of Clinical Nutrition | Year: 2013

Background: Maternal fish consumption during pregnancy has been positively associated with cognitive and visual abilities in the offspring, leading to the hypothesis that maternal omega-3 (n23) long-chain PUFA (LCPUFA) supplementation improves children's neurologic and visual development. Objective: The objective was to evaluate the effect of maternal omega-3 LCPUFA supplementation in pregnancy on neurologic and visual development in the offspring. Design: Five electronic databases were searched. Human randomized controlled trials that supplemented the maternal diet with omega-3 LCPUFAs during pregnancy, or pregnancy and lactation, and that assessed either neurologic or visual development of the offspring were included. Trial quality was assessed, and the results of eligible trials were compared in meta-analyses. Results: Eleven RCTs involving 5272 participants were included in the review. Most trials had methodologic limitations. No differences in standardized psychometric test scores for cognitive, language, or motor development were observed between the LCPUFA-supplemented and control groups, except for cognitive scores in 2-5-y-old children, in whom supplementation resulted in higher Developmental Standard Scores (mean difference: 3.92; 95% CI: 0.77, 7.08; n = 156; P = 0.01). However, this effect was from 2 trials with a high risk of bias. Because of the variety of visual assessments and age ranges, it was not possible to combine studies with visual outcomes in a meta-analysis, although 6 of the 8 assessments in 5 trials reported no difference between the supplemented and control groups. Conclusion: The evidence does not conclusively support or refute that omega-3 LCPUFA supplementation in pregnancy improves cognitive or visual development. © 2013 American Society for Nutrition.

Goldwater P.N.,Womens and Childrens Hospital
Emerging infectious diseases | Year: 2013

Pediatric infectious disease clinicians in industrialized countries may encounter iatrogenically transmitted HIV, hepatitis B virus, and hepatitis C virus infections in refugee children from Central Asia, Southeast Asia, and sub-Saharan Africa. The consequences of political collapse and/or civil war-work migration, prostitution, intravenous drug use, defective public health resources, and poor access to good medical care-all contribute to the spread of blood-borne viruses. Inadequate infection control practices by medical establishments can lead to iatrogenic infection of children. Summaries of 4 cases in refugee children in Australia are a salient reminder of this problem.

Penttila I.A.,Womens and Childrens Hospital
Journal of Pediatrics | Year: 2010

Breast milk cytokines have the potential to regulate the immune response to food antigens in infants. Cytokines are present in all mammalian milks and are capable of inhibiting excess inflammation and modulating epithelial proliferation. There are a range of candidate cytokines in milk such as transforming growth factor-β (TGF-β), the major cytokine present, and interleukin-10, which play a role in immune regulation in the developing infant. This article will be a review of the current literature with regard to TGF-β in infant immune development. Our data on supplementation of formula with rTGF-β2 will be discussed in view of the current literature. Oral antigen exposure also plays an important role in priming the developing immune response. The influence of early introduction of oral β-lactoglobulin in allergy prone rat pups will also be discussed. © 2010 Mosby, Inc. All rights reserved.

Lambert P.,Womens and Childrens Hospital
The Cochrane database of systematic reviews | Year: 2014

Postoperative pain remains a significant problem following paediatric surgery. Premedication with a suitable agent may improve its management. Clonidine is an alpha-2 adrenergic agonist which has sedative, anxiolytic and analgesic properties. It may therefore be a useful premedication for reducing postoperative pain in children. To evaluate the evidence for the effectiveness of clonidine, when given as a premedication, in reducing postoperative pain in children less than 18 years of age. We also sought evidence of any clinically significant side effects. We searched the Cochrane Central Register of Controlled Trials (CENTRAL) in The Cochrane Library (Issue 12, 2012), Ovid MEDLINE (1966 to 21 December 2012) and Ovid EMBASE (1982 to 21 December 2012), as well as reference lists of other relevant articles and online trial registers. We included all randomized (or quasi-randomized), controlled trials comparing clonidine premedication to placebo, a higher dose of clonidine, or another agent when used for surgical or other invasive procedures in children under the age of 18 years and where pain or a surrogate (principally the need for supplementary analgesia) was reported. Two authors independently performed the database search, decided on the inclusion eligibility of publications, ascertained study quality and extracted data. They then resolved any differences between their results by discussion. The data were entered into RevMan 5 for analyses and presentation. Sensitivity analyses were performed, as appropriate, to exclude studies with a high risk of bias. We identified 11 trials investigating a total of 742 children in treatment arms relevant to our study question. Risks of bias in the studies were mainly low or unclear, but two studies had aspects of their methodology that had a high risk of bias. Overall, the quality of the evidence from pooled studies was low or had unclear risk of bias. Four trials compared clonidine with a placebo or no treatment, six trials compared clonidine with midazolam, and one trial compared clonidine with fentanyl. There was substantial methodological heterogeneity between trials; the dose and route of clonidine administration varied as did the patient populations, the types of surgery and the outcomes measured. It was therefore difficult to combine the outcomes of some trials for meta-analysis.When clonidine was compared to placebo, pooling studies of low or unclear risk of bias, the need for additional analgesia was reduced when clonidine premedication was given orally at 4 μg/kg (risk ratio (RR) 0.24, 95% confidence interval (CI) 0.11 to 0.51). Only one small trial (15 patients per arm) compared clonidine to midazolam for the same outcome; this also found a reduction in the need for additional postoperative analgesia (RR 0.25, 95% CI 0.09 to 0.71) when clonidine premedication was given orally at 2 or 4 μg/kg compared to oral midazolam at 0.5 mg/kg. A trial comparing oral clonidine at 4 μg/kg with intravenous fentanyl at 3 μg/kg found no statistically significant difference in the need for rescue analgesia (RR 0.89, 95% CI 0.56 to 1.42). When clonidine 4 μg/kg was compared to clonidine 2 μg/kg, there was a statistically significant difference in the number of patients requiring additional analgesia, in favour of the higher dose, as reported by a single, higher-quality trial (RR 0.38, 95% CI 0.23 to 0.65).The effect of clonidine on pain scores was hard to interpret due to differences in study methodology, the doses and route of drug administration, and the pain scale used. However, when given at a dose of 4 μg/kg, clonidine may have reduced analgesia requirements after surgery. There were no significant side effects of clonidine that were reported such as severe hypotension, bradycardia, or excessive sedation requiring intervention. However, several studies used atropine prophylactically with the aim of preventing such adverse effects. There were only 11 relevant trials studying 742 children having surgery where premedication with clonidine was compared to placebo or other drug treatment. Despite heterogeneity between trials, clonidine premedication in an adequate dosage (4 μg/kg) was likely to have a beneficial effect on postoperative pain in children. Side effects were minimal, but some of the studies used atropine prophylactically with the intention of preventing bradycardia and hypotension. Further research is required to determine under what conditions clonidine premedication is most effective in providing postoperative pain relief in children.

Womens And Childrens Hospital | Date: 2012-03-28

A novel protein profiling method of testing for Lysosomal Storage Diseases (LSD) using discovered normalized lysosomal fingerprint patterns. The fingerprint patterns reveal the health of lysosomal organelles, specific LSD, and clinical severity. Multiplexing bead technology for simultaneous screening of multiple LSD and normalizing measured enzyme activity or protein levels against other lysosomal proteins, enzymes, or enzyme activities. Compounds, reagents, and methods for identifying and quantifying multiple target enzymes and proteins.

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