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Deadlands: Reloaded Core Rulebook
This electronic book is copyright Pinnacle Entertainment Group. Redistribution by print or by file is strictly prohibited. This pdf may be printed for personal use. The Weird West Reloaded Shane Lacy Hensley and BD Flory Savage Worlds by Shane Lacy Hensley Credits & Acknowledgements Additional Material: Simon Lucas, Paul “Wiggy” Wade-Williams, Dave Blewer, Piotr Korys Editing: Simon Lucas, Dave Blewer, Piotr Korys, Jens Rushing Cover, Layout, and Graphic Design: Aaron Acevedo, Travis Anderson, Thomas Denmark Typesetting: Simon Lucas Cartography: John Worsley Special Thanks: To Clint Black, Dave Blewer, Kirsty Crabb, Rob “Tex” Elliott, Sean Fish, John Goff, John & Christy Hopler, Aaron Isaac, Jay, Amy, and Hayden Kyle, Piotr Korys, Rob Lusk, Randy Mosiondz, Cindi Rice, Dirk Ringersma, John Frank Rosenblum, Dave Ross, Jens Rushing, Zeke Sparkes, Teller, Paul “Wiggy” Wade-Williams, Frank Uchmanowicz, and all those who helped us make the original Deadlands a premiere property. Fan Dedication: To Nick Zachariasen, Eric Avedissian, Sean Fish, and all the other Deadlands fans who have kept us honest for the last 10 years. Personal Dedication: To mom, dad, Michelle, Caden, and Ronan. Thank you for all the love and support. You are my world. B.D.’s Dedication: To my parents, for everything. Sorry this took so long. Interior Artwork: Aaron Acevedo, Travis Anderson, Chris Appel, Tom Baxa, Melissa A. Benson, Theodor Black, Peter Bradley, Brom, Heather Burton, Paul Carrick, Jim Crabtree, Thomas Denmark, Cris Dornaus, Jason Engle, Edward Fetterman, -
Otoplasty-Plastic Surgery of the Ears (Pdf)
Vinod K. Anand, MD, FACS Nose and Sinus Clinic Plastic Surgery of the Ears (Otoplasty) This brochure will familiarize you with some basic facts about cosmetic surgery of the ear. It will give you enough general background to make you an "educated consumer." Your facial plastic surgeon will explain how this procedure applies to an individual's condition. A SOLUTION FOR A VERY COMMON PROBLEM The most common cosmetic problem that people have with their ears is that they pro- trude. Otoplasty is the name given to the operation designed to "pin back" the ears and to change their shape and contour. While otoplasty can be performed at any age after four or five years, it often is recom- mended in the preschool years to alleviate possible teasing at school by other children. DECIDING ON AN OPERATION Anyone interested in cosmetic surgery of the ear for himself or a child should consult a competent facial plastic surgeon. During the initial visit, the surgeon makes a thorough evaluation of the ears to determine whether surgery is indicated. The surgeon will then discuss any questions and concerns related to the surgery. In addition to the skill of the surgeon, the patient's realistic expectations about the results of the surgery and his general emotional state are important considerations. Mental attitude is as important as the ability to heal in evaluating candidates for facial plastic surgery. Once surgery is agreed upon, pre-operative photographs are taken to help the surgeon plan the operation. These photographs usually are compared with similar ones taken sometime after surgery and serve as a permanent before-and-after record of the results. -
Current Management and Outcome of Tracheobronchial Malacia and Stenosis Presenting to the Paediatric Intensive Care Unit
Intensive Care Med 52001) 27: 722±729 DOI 10.1007/s001340000822 NEONATAL AND PEDIATRIC INTENSIVE CARE David P.Inwald Current management and outcome Derek Roebuck Martin J.Elliott of tracheobronchial malacia and stenosis Quen Mok presenting to the paediatric intensive care unit Abstract Objective: To identify fac- but was not related to any other fac- Received: 10 July 2000 Final Revision received: 14 Oktober 2000 tors associated with mortality and tor. Patients with stenosis required a Accepted: 24 October 2000 prolonged ventilatory requirements significantly longer period of venti- Published online: 16 February 2001 in patients admitted to our paediat- latory support 5median length of Springer-Verlag 2001 ric intensive care unit 5PICU) with ventilation 59 days) than patients tracheobronchial malacia and with malacia 539 days). stenosis diagnosed by dynamic con- Conclusions: Length of ventilation Dr Inwald was supported by the Medical Research Council. This work was jointly trast bronchograms. and bronchographic diagnosis did undertaken in Great Ormond Street Hos- Design: Retrospective review. not predict survival. The only factor pital for Children NHSTrust, which re- Setting: Tertiary paediatric intensive found to contribute significantly to ceived a proportion of its funding from the care unit. mortality was the presence of com- NHSExecutive; the views expressed in this Patients: Forty-eight cases admitted plex cardiac and/or syndromic pa- publication are those of the authors and not to our PICU over a 5-year period in thology. However, patients with necessarily those of the NHSexecutive. whom a diagnosis of tracheobron- stenosis required longer ventilatory chial malacia or stenosis was made support than patients with malacia. -
Management and Investigation of Neonatal Encephalopathy: 2017 Update Kathryn Martinello,1 Anthony R Hart,2 Sufin Yap,3 Subhabrata Mitra,1 Nicola J Robertson1
Review Arch Dis Child Fetal Neonatal Ed: first published as 10.1136/archdischild-2015-309639 on 6 April 2017. Downloaded from Management and investigation of neonatal encephalopathy: 2017 update Kathryn Martinello,1 Anthony R Hart,2 Sufin Yap,3 Subhabrata Mitra,1 Nicola J Robertson1 1Department of Neonatology, ABSTRACT definite aetiological diagnosis is known, and Institute for Women’s Health, This review discusses an approach to determining the hypoxic-ischaemic encephalopathy (HIE) where University College London, UK 2 cause of neonatal encephalopathy, as well as current clear diagnosis of hypoxia-ischaemia is known to Department of Neonatal and ’ Paediatric Neurology, Sheffield evidence on resuscitation and subsequent management have led to the neonate s clinical state. Children’s Hospital NHS of hypoxic-ischaemic encephalopathy (HIE). Foundation Trust, Sheffield, UK Encephalopathy in neonates can be due to varied 3 DETERMINING THE AETIOLOGY OF NE Department of Inherited aetiologies in addition to hypoxic-ischaemia. A Metabolic Diseases, Sheffield The initial stages of managing NE will be the same Children’s Hospital NHS combination of careful history, examination and the for most babies, with good resuscitation and sup- Foundation Trust, Sheffield, UK judicious use of investigations can help determine the portive management. However, as the picture cause. Over the last 7 years, infants with moderate to evolves and investigations return, clinicians should fi Correspondence to severe HIE have bene ted from the introduction of consider the aetiology of NE as this could lead to Professor Nicola J Robertson, routine therapeutic hypothermia; the number needed to specific treatments, aid with prognosis and recur- Institute for Women’s Health, treat for an additional beneficial outcome is 7 (95% CI University College London, 74 rence risk counselling, and assist with the evalu- Huntley Street, London WC1E 5 to 10). -
The Role of Larygotracheal Reconstruction in the Management of Recurrent Croup in Patients with Subglottic Stenosis
International Journal of Pediatric Otorhinolaryngology 82 (2016) 78–80 Contents lists available at ScienceDirect International Journal of Pediatric Otorhinolaryngology jo urnal homepage: www.elsevier.com/locate/ijporl The role of larygotracheal reconstruction in the management of recurrent croup in patients with subglottic stenosis a,b,c, a,b a,d,e Bianca Siegel *, Prasad Thottam , Deepak Mehta a Department of Pediatric Otolaryngology, Childrens Hospital of Pittsburgh of UPMC, Pittsburgh, PA, USA b Children’s Hospital of Michigan, Detroit, MI, USA c Wayne State University School of Medicine Department of Otolaryngology, Detroit, MI, USA d Texas Children’s Hospital, Houston, TX, USA e Baylor University School of Medicine Department of Otolaryngology, Houston, TX, USA A R T I C L E I N F O A B S T R A C T Article history: Objectives: To determine the role of laryngotracheal reconstruction for recurrent croup and evaluate Received 16 October 2015 surgical outcomes in this cohort of patients. Received in revised form 4 January 2016 Methods: Retrospective chart review at a tertiary care pediatric hospital. Accepted 6 January 2016 Results: Six patients who underwent laryngotracheal reconstruction (LTR) for recurrent croup with Available online 13 January 2016 underlying subglottic stenosis were identified through a search of our IRB-approved airway database. At the time of diagnostic bronchoscopy, all 6 patients had grade 2 subglottic stenosis. All patients were Keywords: treated for reflux and underwent esophageal biopsies at the time of diagnostic bronchoscopy; 1 patient Laryngotracheal reconstruction had eosinophilic esophagitis which was treated. All patients had a history of at least 3 episodes of croup Recurrent croup in a 1 year period requiring multiple hospital admissions. -
Diagnostic Issues in Systemic Lupus Erythematosis
266 Postgrad Med J 2001;77:266–285 Postgrad Med J: first published as 10.1136/pmj.77.906.268 on 1 April 2001. Downloaded from SELF ASSESSMENT QUESTIONS Diagnostic issues in systemic lupus erythematosis N Sofat, C Higgens Answers on p 274. A 24 year old woman was diagnosed with sys- (4) What other tests (apart from 24 hour urine temic lupus erythematosis (SLE) based on a creatinine clearance) are available to measure few months’ history of a photosensitive skin the glomerular filtration rate? rash, predominantly on her face, arthralgia The patient had a 24 hour urinary protein col- involving both hands and wrists, a positive lection, which showed a 24 hour protein measure- antinuclear antibody (ANA) test and a raised ment of 1.8 g. There was no evidence of cellular antinative double stranded DNA antibody casts on urine microscopy. Her blood results were binding level. She was treated with oral as below (normal values are in parentheses): hydroxychloroquine 400 mg daily and short x Sodium 134 mmol/l (135–145) courses of prednisolone during flare-ups. x Potassium 4.5 mmol/l (3.5–5.0) She was reviewed in clinic for her regular x Urea 7.0 mmol/l (2.5–6.7) follow up appointment when she was found to x Creatinine 173 µmol/l (70–115) be hypertensive on repeated measurements of x Haemoglobin 108 g/l (115–160) her blood pressure, an average value being x White cell count 4.5 × 109/l (4.0–11.0) 150/90 mm Hg. She was also urine dipstick x Platelets 130 × 109/l (150–400) positive for blood and protein. -
The Posterior Muscles of the Auricle: Anatomy and Surgical Applications
Central Annals of Otolaryngology and Rhinology Research Article *Corresponding author Christian Vacher, Department of Maxillofacial Surgery & Anatomy, University of Paris-Diderot, APHP, 100, The Posterior Muscles of the Boulevard Général Leclerc, 92110 Clichy, France, Tel: 0033140875671; Email: Submitted: 19 December 2014 Auricle: Anatomy and Surgical Accepted: 16 January 2015 Published: 19 January 2015 Applications Copyright © 2015 Vacher et al. Rivka Bendrihem1, Christian Vacher2* and Jacques Patrick Barbet3 OPEN ACCESS 1 Department of Dentistry, University of Paris-Descartes, France Keywords 2 Department of Maxillofacial Surgery & Anatomy, University of Paris-Diderot, France • Auricle 3 Department of Pathology and Cytology, University of Paris-Descartes, France • Anatomy • Prominent ears Abstract • Muscle Objective: Prominent ears are generally considered as primary cartilage deformities, but some authors consider that posterior auricular muscles malposition could play a role in the genesis of this malformation. Study design: Auricle dissections of 30 cadavers and histologic sections of 2 fetuses’ ears. Methods: Posterior area of the auricle has been dissected in 24 cadavers preserved with zinc chlorure and 6 fresh cadavers in order to describe the posterior muscles and fascias of the auricle. Posterior auricle muscles from 5 fresh adult cadavers have been performed and two fetal auricles (12 and 22 weeks of amenorhea) have been semi-serially sectioned in horizontal plans. Five µm-thick sections were processed for routine histology (H&E) or for immuno histochemistry using antibodies specific for the slow-twitch and fast-twich myosin heavy chains in order to determine which was the nature of these muscles. Results: The posterior auricular and the transversus auriculae muscles looked in most cases like skeletal muscles and they were made of 75% of slow muscular fibres. -
2018 Etiologies by Frequencies
2018 Etiologies in Order of Frequency by Category Hereditary Syndromes and Disorders Count CHARGE Syndrome 958 Down syndrome (Trisomy 21 syndrome) 308 Usher I syndrome 252 Stickler syndrome 130 Dandy Walker syndrome 119 Cornelia de Lange 102 Goldenhar syndrome 98 Usher II syndrome 83 Wolf-Hirschhorn syndrome (Trisomy 4p) 68 Trisomy 13 (Trisomy 13-15, Patau syndrome) 60 Pierre-Robin syndrome 57 Moebius syndrome 55 Trisomy 18 (Edwards syndrome) 52 Norrie disease 38 Leber congenital amaurosis 35 Chromosome 18, Ring 18 31 Aicardi syndrome 29 Alstrom syndrome 27 Pfieffer syndrome 27 Treacher Collins syndrome 27 Waardenburg syndrome 27 Marshall syndrome 25 Refsum syndrome 21 Cri du chat syndrome (Chromosome 5p- synd) 16 Bardet-Biedl syndrome (Laurence Moon-Biedl) 15 Hurler syndrome (MPS I-H) 15 Crouzon syndrome (Craniofacial Dysotosis) 13 NF1 - Neurofibromatosis (von Recklinghausen dis) 13 Kniest Dysplasia 12 Turner syndrome 11 Usher III syndrome 10 Cockayne syndrome 9 Apert syndrome/Acrocephalosyndactyly, Type 1 8 Leigh Disease 8 Alport syndrome 6 Monosomy 10p 6 NF2 - Bilateral Acoustic Neurofibromatosis 6 Batten disease 5 Kearns-Sayre syndrome 5 Klippel-Feil sequence 5 Hereditary Syndromes and Disorders Count Prader-Willi 5 Sturge-Weber syndrome 5 Marfan syndrome 3 Hand-Schuller-Christian (Histiocytosis X) 2 Hunter Syndrome (MPS II) 2 Maroteaux-Lamy syndrome (MPS VI) 2 Morquio syndrome (MPS IV-B) 2 Optico-Cochleo-Dentate Degeneration 2 Smith-Lemli-Opitz (SLO) syndrome 2 Wildervanck syndrome 2 Herpes-Zoster (or Hunt) 1 Vogt-Koyanagi-Harada -
Genevista Microdeletion and Microduplication Syndromes
GeNeViSTA Microdeletion and Microduplication Syndromes: An Update Priya Ranganath, Prajnya Ranganath Department of Medical Genetics, Nizam’s Institute of Medical Sciences, Hyderabad, India Correspondence to: Dr Prajnya Ranganath Email: [email protected] Abstract containing dosage sensitive genes responsible for the phenotype is generally involved (Goldenberg, Microdeletion and microduplication syndromes 2018). Theoretically, for every microdeletion (MMS) also known as ‘contiguous gene syndrome there should be a reciprocal syndromes’ are a group of disorders caused microduplication syndrome, but microdeletions by sub-microscopic chromosomal deletions or are more common. Microduplications appear to duplications. Most of these conditions are typically result in a milder or no clinical phenotype. associated with developmental delay, autism, multiple congenital anomalies, and characteristic Molecular Etiopathology phenotypic features. These chromosomal abnormalities cannot be detected by conventional Copy number variation (CNV) is defined as the gain cytogenetic techniques like karyotyping and or loss of a stretch of DNA when compared with require higher resolution ‘molecular cytogenetic’ the reference human genome and may range in techniques. The advent of high throughput tests size from a kilobase to several megabases or even such as chromosomal microarray in the past one an entire chromosome. The CNVs associated with or two decades has led to a continuously growing MMS constitute only a small fraction of the total list of microdeletions and microduplication number of possible copy-number variants. There syndromes along with identification of the ‘critical are two major classes of CNVs: recurrent and region’ responsible for the main phenotypic non-recurrent. Recurrent CNVs generally result features associated with these syndromes. This from Non-Allelic Homologous Recombination review discusses the etiopathogenic mechanisms (NAHR) during meiosis. -
Genetics of Congenital Hand Anomalies
G. C. Schwabe1 S. Mundlos2 Genetics of Congenital Hand Anomalies Die Genetik angeborener Handfehlbildungen Original Article Abstract Zusammenfassung Congenital limb malformations exhibit a wide spectrum of phe- Angeborene Handfehlbildungen sind durch ein breites Spektrum notypic manifestations and may occur as an isolated malforma- an phänotypischen Manifestationen gekennzeichnet. Sie treten tion and as part of a syndrome. They are individually rare, but als isolierte Malformation oder als Teil verschiedener Syndrome due to their overall frequency and severity they are of clinical auf. Die einzelnen Formen kongenitaler Handfehlbildungen sind relevance. In recent years, increasing knowledge of the molecu- selten, besitzen aber aufgrund ihrer Häufigkeit insgesamt und lar basis of embryonic development has significantly enhanced der hohen Belastung für Betroffene erhebliche klinische Rele- our understanding of congenital limb malformations. In addi- vanz. Die fortschreitende Erkenntnis über die molekularen Me- tion, genetic studies have revealed the molecular basis of an in- chanismen der Embryonalentwicklung haben in den letzten Jah- creasing number of conditions with primary or secondary limb ren wesentlich dazu beigetragen, die genetischen Ursachen kon- involvement. The molecular findings have led to a regrouping of genitaler Malformationen besser zu verstehen. Der hohe Grad an malformations in genetic terms. However, the establishment of phänotypischer Variabilität kongenitaler Handfehlbildungen er- precise genotype-phenotype correlations for limb malforma- schwert jedoch eine Etablierung präziser Genotyp-Phänotyp- tions is difficult due to the high degree of phenotypic variability. Korrelationen. In diesem Übersichtsartikel präsentieren wir das We present an overview of congenital limb malformations based Spektrum kongenitaler Malformationen, basierend auf einer ent- 85 on an anatomic and genetic concept reflecting recent molecular wicklungsbiologischen, anatomischen und genetischen Klassifi- and developmental insights. -
CHARGE Syndrome
orphananesthesia Anaesthesia recommendations for CHARGE syndrome Disease name: CHARGE syndrome ICD 10: Q87.8 Synonyms: CHARGE association; Hall-Hittner syndrome Disease summary: CHARGE syndrome was initially defined as a non-random association of anomalies: - Coloboma - Heart defect - Atresia choanae (choanal atresia) - Retarded growth and development - Genital hypoplasia - Ear anomalies/deafness In 1998, an expert group defined the major (the classical 4C´s: Choanal atresia, Coloboma, Characteristic ear and Cranial nerve anomalies) and minor criteria of CHARGE syndrome [1]. In 2004, mutations in the CHD7 gene were identified as the major cause. The inheritance pattern is autosomal dominant with variable expressivity. Almost all mutations occurs de novo, but parent-to-child transmission has occasionally been reported [2]. Clinical criteria for CHARGE syndrome [1] Major criteria: • Coloboma • Choanal Atresia • Cranial nerve anomalies • Abnormalities of the inner, middle, or external ear Minor criteria: • Cardiaovascular malformations • Genital hypoplasia or delayed pubertal development • Cleft lip and/or palate • Tracheoesophageal defects • Distinctive CHARGE facies • Growth retardation • Developmental delay Occasional: • Renal anomalies: duplex system, vesicoureteric reflux • Spinal anomalies: scoliosis, osteoporosis • Hand anomalies 1 • Neck/shoulder anomalies • Immune system disorders Individuals with all four major characteristics or three major and three minor characteristics are highly likely to have CHARGE syndrome [1]. CHARGE syndrome -
CHARGE Factsheet 3 Clinical Diagnosis and Features
The Information Pack CHARGE for Practitioners Factsheet 3 CHARGE syndrome: major and minor medical diagnostic criteria plus later onset features DR JEREMY KIRK, MD, FRCP, FRCPCH, Consultant Paediatric Endocrinologist, Birmingham Children’s Hospital Original diagnostic criteria The initial association of coloboma and choanal atresia with other congenital abnormalities was first described by Hall and separately Hittner et al. in 1979 (Hall, 1979; Hittner et al., 1979). In 1981 there was further description and expansion of the condition (Pagon et al., 1981). It was at this stage that the acronym CHARGE (C–coloboma, H–heart disease, A–atresia choanae, R–retarded growth and retarded development and/or CNS anomalies, G–genital hypoplasia, and E–ear anomalies and/or deafness) was made. In order to make the diagnosis of CHARGE syndrome, historically four out of six of the features of the acronym needed to be fulfilled, although one should be either choanal atresia or a coloboma (Pagon et al., 1981). From association to syndrome been several attempts to refine the diagnostic criteria, Initially CHARGE was described as an association; namely by Blake et al. (1998) and Verloes (2005). Both a nonrandom collection of birth defects, rather than of these use major features which are very specific for a syndrome, which is a more recognisable pattern of CHARGE syndrome, along with other minor features. birth defects (often with a known genetic cause). With the identification of the gene CHD7 in 2004 (Vissers The criteria suggested by Blake et al. consist of four et al., 2004) it has now been renamed a syndrome, major ‘C’s: as CHD7 is mutated in at least 60% of patients with 1.