21. CIPA congenital insensitivity to pain with Anhidrosis (lack of sweating) is a rare People with congenital insensitivity to pain go through life in danger of http://helium.vancouver.wsu.edu/~woodc/CIPA.html

CONGENITAL INSENSITIVITY TO PAIN WITH ANHIDROSIS Congenital Insensitivity to Pain with Anhidrosis (lack of sweating) is a rare autosomal-recessive disorder usually manifested in childhood by a history of unrecognized trauma, indifference to pain stimuli or self-mutilation. This disorder is a rare sensory and autonomic neuropathy which is defined as the absence of normal subjective and objective responses to noxious stimuli in patients shown to have normal central and peripheral nervous systems. Clinical appearance of patients with congenital insensitivity to pain. INTRODUCTION HOW IS THE BRAIN INVOLVED? NOCICEPTION PATHWAY MAIN FEATURES OF DISORDER ... REFERENCES INTRODUCTION Patients diagnosed with congenital insensitivity to pain with anhidrosis (CIPA) suffer from abnormal pain and temperature perception, absence of sweating (anhidrosis), mental retardation, fever during hot weather and loss of unmyelinated fibers with a decrease in size of small myelinated fibers. Body temperature is disturbed as a result of the inability to sweat and insensitivity to pain may lead to bone fracture, multiple scars, osteomyelitis, and joint deformities, which may lead to limb amputation, painless injuries of arms, legs and oral structures. This disorder results from a defect in neural crest differentiation and the system responsible for pain and temperature sensation, the first-order afferent system, is lost. Nociceptive neurons in the dorsal root ganglia derive from the neural crest and they can only survive if they are stimulated by the nerve growth factor (NGF) through the TrkA receptor. Mutations in the TrkA gene have shown a correlation with the defective development of the nociceptive neurons.

22. Pain Insensitivity, Congenital Topic - Unified Search Environment pain insensitivity, congenital Topic Tree pain insensitivity, congenitalMSH/MH/D000699 MSH/EN/D000699 MSH/EN/D000699 http://www.use.hcn.com.au/portals/shared/subject.`Pain Insensitivity, Congenital

Pain Insensitivity, Congenital Topic Tree Definition: A syndrome characterized by indifference to PAIN despite the ability to distinguish noxious from nonnoxious stimuli. Absent corneal reflexes and MENTAL RETARDATION may be associated. Familial forms with autosomal recessive and autosomal dominant patterns of inheritance have been described. (Adams et al., Principles of Neurology, 6th ed, p1343) Synonyms and Source Vocabularies: Pain Insensitivity, Congenital Analgesia, Congenital Pain Indifference, Congenital Congenital Pain Indifferences Neuromuscular Diseases Chronic Fatigue Syndrome Muscular Atrophy, Spinal Myopathy Peripheral Nervous System Diseases Acrodynia Pain Insensitivity, Congenital Diabetic Neuropathies Guillain-Barre Syndrome ... Muscular Disorders, Atrophic

23. Entrez PubMed An 8year-old boy with congenital insensitivity to pain underwent open reductionof a fractured femu http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1

24. Congenital Insensitivity To Pain a CHORUS notecard document about congenital insensitivity to pain. http://chorus.rad.mcw.edu/doc/00546.html

CHORUS Collaborative Hypertext of Radiology Musculoskeletal system About CHORUS Search Feedback congenital insensitivity to pain autosomal recessive neuropathic joints micro- and macrofractures epiphyseal separation osteomyelitis (mandible, fingers, toes) DDx: congenital insensitivity to pain with anhidrosis (aut. rec.) hereditary sensory radicular neuropathy (aut. rec.) congenital sensory neuropathy (aut. dom.) familial dysautonomia (aut. rec.) Lesch-Nyhan syndrome (X rec.) Charles E. Kahn, Jr., MD - 2 February 1995 Last updated 26 May 2004 Related CHORUS documents: neuropathic (Charcot) joint Riley-Day syndrome bulging disc hepatoma ... chronic granulomatous disease of childhood Search for related articles: AJR American Journal of Roentgenology PubMed : index to biomedical literature ... Medical College of Wisconsin

25. Congenital Insensitivity To Pain congenital insensitivity to pain with anhidrosis (aut. rec.) hereditary sensoryradicular neuropathy (aut. rec.) congenital sensory neuropathy (aut. dom. http://chorus.rad.mcw.edu/to-go/00546.html

congenital insensitivity to pain autosomal recessive neuropathic joints micro- and macrofractures epiphyseal separation osteomyelitis (mandible, fingers, toes) DDx: congenital insensitivity to pain with anhidrosis (aut. rec.) hereditary sensory radicular neuropathy (aut. rec.) congenital sensory neuropathy (aut. dom.) familial dysautonomia (aut. rec.) Lesch-Nyhan syndrome (X rec.) Home Musculoskeletal system

26. Congenital Insensitivity To Pain congenital insensitivity to pain (hereditary sensory and autonomic neuropathy HSANtype V) is a rare disorder of pain perception in which pain sensation http://www.general-anaesthesia.com/congenital-insensitivity/

Congenital insensitivity to pain in four related Saudi families by Karkashan EM, Joharji HS, Al-Harbi NN. Department of Dermatology and Venereology, Asir Central Hospital, and Department of Pediatrics, King Khalid University, Abha, Saudi Arabia. Pediatr Dermatol . 2002 Jul-Aug;19(4):333-5 ABSTRACT C ongenital insensitivity to pain (hereditary sensory and autonomic neuropathy [HSAN] type V) is a rare disorder of pain perception in which pain sensation is absent from birth, with no other neurologic deficits. We report five Saudi patients (three male and two female) age 10 months to 23 years who lacked pain sensation from birth but have normal appreciation of other sensory modalities. They are from four related families who are descended from one grandfather. The patients had sustained many painless injuries resulting in fractures and disfigurement, but otherwise are completely normal. Life without pain? CIPA mechanisms Familial indifference to pain Congenital indifference to pain ... The Good Drug Guide The Responsible Parent's Guide To Healthy Mood Boosters For All The Family

27. Anaesthesia For People With Congenital Insensitivity To Pain BACKGROUND congenital insensitivity to pain with anhidrosis (CIPA, or hereditarysensory and autonomic neuropathy type IV) is a rare, autosomal recessive http://www.general-anaesthesia.com/congenital-insensitivity/anaesthesia.html

The anaesthetic management of patients with congenital insensitivity to pain with anhidrosis by Rozentsveig V, Katz A, Weksler N, Schwartz A, Schilly M, Klein M, Gurman GM. Division of Anesthesiology and Critical Care Medicine, Soroka University Medical Center, Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel. Paediatr Anaesth . 2004 Apr;14(4):344-8. ABSTRACT B 'Pain genes' CIPA mechanisms Life without pain? 'The secularisation of pain' ... The Good Drug Guide The Responsible Parent's Guide To Healthy Mood Boosters For All The Family

28. Revista De La Sociedad Española Del Dolor - Rosemberg S, Marie SK, Kliemann S. congenital insensitivity to pain with Yagev R, Levy J, Shorer Z, Lifshitz T. congenital insensitivity to pain with http://scielo.isciii.es/scielo.php?pid=S1134-80462004000100007&script=sci_arttex

29. Congenital Insensitivity To Pain congenital insensitivity to pain with anhidrosis (CIPA) effect of TRKA (NTRK1)missense mutations on autophosphorylation of the receptor tyrosine kinase http://opioids.com/pain/congenital-anaesthesia.html

Congenital insensitivity to pain with anhidrosis (CIPA): effect of TRKA (NTRK1) missense mutations on autophosphorylation of the receptor tyrosine kinase for nerve growth factor by Mardy S, Miura Y, Endo F, Matsuda I, Indo Y. Department of Pediatrics, Kumamoto University School of Medicine, Honjo 1-1-1, Kumamoto 860-8556, Japan. Hum Mol Genet . 2001 Feb 1; 10(3): 179-88 ABSTRACT H Pain NMDA Fentanyl Morphine ... When Is It Best To Take Crack Cocaine?

30. Genetics Of Congenital Insensitivity To Pain With Anhidrosis (CIPA) Patient medical question and doctor answer from The Neurology and NeurosurgeryForum. Health topic area and articles about neurology Topics , Keywords http://www.medhelp.org/forums/neuro/messages/30436a.html

Questions in The Neurology Forum are being answered by doctors from The Cleveland Clinic , consistently ranked one of the best hospitals in America. Genetics of Congenital Insensitivity to Pain with Anhidrosis (CIPA) Forum: The Neurology and Neurosurgery Forum Topic: Neurology Subject: Genetics of Congenital Insensitivity to Pain with Anhidrosis (CIPA) From To Post Clark My first child has a disease very close to CIPA. While waiting for further diagnosis, I am now expecting my second child. I would apprecaite it very much if anyone could help me by answering the following questions: 1/How much is the chance of my second child getting the same disease? 2/ Is it possible to have a test to find out the condition of my second baby? CCF Neuro[P] MD, RPS Clark Dear Clark: I am sorry to hear about your child. Hereditary sensory and autonomic neuropathy type IV is a rare autosomal recessive disorder characterized by congenital insensitivity to pain, anhidrosis, defective temperature control, and mild mental retardation. There is a selective loss of unmyelinated axons and small myelinated fibers. The gene locus for this entity maps to chromosome 1q21-22. Mutations in the trkA gene encoding the tyrosine kinase receptor for nerve growth factor have been described in some patients. I know you know this already, but I thought I'd put it in for others. As an autosomal recessive gene, the chances of your next child having the disease is 25%. As much as I know, there is no prenatal test for this entity. Because the gene is not known for sure, we can't test for what we do not know.

31. MeSH-D Terms Associated To MeSH-C Term Pain Insensitivity, Congenital MeSHD terms associated to MeSH-C term pain insensitivity, congenital, G2D Home association of the corresponding term to pain insensitivity, congenital. http://www.bork.embl-heidelberg.de/g2d/c2d.pl?Pain_Insensitivity,_Congenital:unk

32. Arch Pediatr Adolesc Med -- Abstract: Congenital Insensitivity To Pain With Anhy congenital insensitivity to pain with anhydrosis. Report of a family and reviewof literature with reference to immune deficiency. PA Vardy, LW Greenberg, http://archpedi.ama-assn.org/cgi/content/abstract/133/11/1153

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress Vol. 133 No. 11, November 1979 Featured Link E-mail Alerts ARTICLE Article Options Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager Articles in PubMed by Vardy PA de Leon GF Contact me when this article is cited Congenital insensitivity to pain with anhydrosis. Report of a family and review of literature with reference to immune deficiency P. A. Vardy, L. W. Greenberg, C. Kachel and G. F. de Leon Congenital indifference to pain with anhydrosis (CIPA) is a well-defined entity among a group of sensory deficiency syndromes. Children with this genetic disease are insensitive to pain and temperature and do not sweat and suffer from psychomotor retardation. Self-inflicted trauma may be severe and lead to insoluble orthopedic problems. To date, 11 cases have

33. To Web Page - Known Indo, Y., Molecular Basis of congenital insensitivity to pain with Anhidrosis (CIPA)Mutations and Polymorphisms in TRKA (NTRK1) Gene Encoding the http://marketing.appliedbiosystems.com/mk/submit/iscience_v3_rd?_JS=T&rd=s

34. Congenital Insensitivity To Pain (hereditary Sensory And Autonomic Neuropathy) H Journal of Indian Society of Pedodontics and Preventive Dentistry. 2002 Jun;20(2) 513. http://medind.nic.in/imvw/imvw689.html

Extracted from IndMED Kumar RK ; Kiran NDS ; Reddy VVS Dept. of Pedodontics and Preventive Dentistry, Collage of Dental sciences, Davangere, Karnataka Congenital Insensitivity to pain (hereditary sensory and autonomic neuropathy) HSAN : a report of two cases Journal of Indian Society of Pedodontics and Preventive Dentistry. 2002 Jun; 20(2): 51-3 ABSTRACT: Pain is one of the protective phenomenon possessed by the body. Pain arouses and demands our immediate attention. There are instances in which there is a congenital insensitivity to pain. Two cases of congenital insensitivity to pain are reported. KEYWORDS: Hereditary sensory and Autonomic Neuropathies/DI; Pain insensitivity, Congenital/DI; Hereditary sensory and Autonomic Neuropathies/CL; Case Report; Human; Child; Male; Female References: 11 Record Identifier: NI208071

35. Journal Of Bone And Joint Surgery: Congenital Insensitivity To Pain: Orthopaedic Full text of the article, congenital insensitivity to pain Orthopaedicmanifestations from Journal of Bone and Joint Surgery, a publication in the field http://www.findarticles.com/p/articles/mi_qa3767/is_200203/ai_n9081169

@import url(/css/us/style1.css); @import url(/css/us/searchResult1.css); @import url(/css/us/articles.css); @import url(/css/us/artHome1.css); Advanced Search Home Help IN free articles only all articles this publication Automotive Sports 10,000,000 articles - not found on any other search engine. FindArticles Journal of Bone and Joint Surgery Mar 2002 Content provided in partnership with 10,000,000 articles Not found on any other search engine. Featured Titles for AAACN Viewpoint ABNF Journal, The AIDS Treatment News AMAA Journal ... View all titles in this topic Hot New Articles by Topic Automotive Sports Top Articles Ever by Topic Automotive Sports Congenital insensitivity to pain: Orthopaedic manifestations Journal of Bone and Joint Surgery Mar 2002 by Bar-On, E Weigl, D Parvari, R Katz, K ... Et al Save a personal copy of this article and quickly find it again with Furl.net. It's free! Save it. ORTHOPAEDIC MANIFESTATIONS We reviewed 13 patients with congenital insensitivity to pain. A quantitative sweat test was carried out in five and an intradermal histamine test in ten. DNA examination showed specific mutations in four patients. There were three clinical presentations: type A, in which multiple infections occurred (five patients); type B, with fractures, growth disturbances and avascular necrosis (three patients); and type C, with Charcot arthropathies and joint dislocations, as well as fractures and infections (five patients, four with mental retardation). Patient education, shoeware and periods of non-weight-bearing are important in the prevention and early treatment of decubitus ulcers. The differentiation between fractures and infections should be based on aspiration and cultures to prevent unnecessary surgery. Established infections should be treated by wide surgical debridement. Deformities can be managed by corrective osteotomies, and shortening by shoe raises or epiphysiodesis. Joint dislocations are best treated conservatively.

36. Congenital Insensitivity Of Pain With Anhidrosis.,Gupta B: Indian Journal Of Ped Indian Journal of Pediatrics is an open access http://www.ijppediatricsindia.org/article.asp?issn=0019-5456;year=2003;volume=70

37. An Infant With Primary Tooth Loss And Palmar Hyperkeratosis: A Novel Mutation In congenital insensitivity to pain with anhidrosis (CIPA) is an autosomal recessivedisorder that was first described almost 40 years ago.1 CIPA is http://pediatrics.aappublications.org/cgi/content/full/112/3/e237

QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This Article Abstract Full Text (PDF) P ... Citation Map Services E-mail this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Download to citation manager PubMed PubMed Citation Articles by Bonkowsky, J. L. Articles by Swoboda, K. J. Related Collections PEDIATRICS Vol. 112 No. 3 September 2003, pp. e237-e241 ELECTRONIC ARTICLE An Infant With Primary Tooth Loss and Palmar Hyperkeratosis: A Novel Mutation in the Gene Causing Congenital Insensitivity to Pain With Anhidrosis Joshua L. Bonkowsky, MD, PhD Justine Johnson John C. Carey, MD A. Gordon Smith, MD and Kathryn J. Swoboda, MD Departments of Pediatrics Neurology, University of Utah School of Medicine, Salt Lake City, Utah ABSTRACT TOP ABSTRACT METHODS RESULTS DISCUSSION REFERENCES Patients with congenital insensitivity to pain and anhidrosis (CIPA), caused by mutations in the gene, can be difficult to diagnose because of their variable presentation, the lack

38. An Infant With Primary Tooth Loss And Palmar Hyperkeratosis: A Novel Mutation In Patients with congenital insensitivity to pain and anhidrosis (CIPA), caused bymutations in the NTRK1 gene, can be difficult to diagnose because of their http://pediatrics.aappublications.org/cgi/content/abstract/112/3/e237

QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This Article Full Text Full Text (PDF) P ... Citation Map Services E-mail this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Download to citation manager PubMed PubMed Citation Articles by Bonkowsky, J. L. Articles by Swoboda, K. J. Related Collections PEDIATRICS Vol. 112 No. 3 September 2003, pp. e237-e241 ELECTRONIC ARTICLE An Infant With Primary Tooth Loss and Palmar Hyperkeratosis: A Novel Mutation in the Gene Causing Congenital Insensitivity to Pain With Anhidrosis Joshua L. Bonkowsky, MD, PhD Justine Johnson John C. Carey, MD A. Gordon Smith, MD and Kathryn J. Swoboda, MD Departments of Pediatrics Neurology, University of Utah School of Medicine, Salt Lake City, Utah Patients with congenital insensitivity to pain and anhidrosis (CIPA), caused by mutations in the gene, can be difficult to diagnose because of their variable presentation, the lack of simple diagnostic tests, and the paucity of cases reported

39. Insensitivity To Pain, Congenital Complete online version of The Encyclopaedia of Medical Imaging including textand images from The Encyclopaedia of Medical Imaging s eight book volumes http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/INSENSITIVITY TO

financial services our commitment our company Search Medcyclopaedia for: Search marked text (mark text before you click) Browse entry words starting with: A B C D ... amershamhealth.com Insensitivity to pain, congenital, absent or markedly reduced sensitivity to pain which means that children repeatedly injure themselves but do not present clinically as they do not feel pain in relation to the injury. They may also have multiple ulcerations of the lips and tongue due to biting. Inheritance is autosomal recessive. Clinically, children can present with palpable healing fractures, deformity, ulceration or evidence of osteomyelitis. Radiologically, the appearances are those of multiple fractures which heal without osteoporosis. There may also be evidence of osteomyelitis. HC The Encyclopaedia of Medical Imaging Volume VII Corporate Products Financial Services Our Commitment ... Terms of Conditions

40. Congenital Insensitivity To Pain Complete online version of The Encyclopaedia of Medical Imaging including textand images from The Encyclopaedia of Medical Imaging s eight book volumes http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/CONGENITAL INSEN

financial services our commitment our company Search Medcyclopaedia for: Search marked text (mark text before you click) Browse entry words starting with: A B C D ... amershamhealth.com Congenital insensitivity to pain, see arthropathy neuropathic insensitivity to pain congenital HC The Encyclopaedia of Medical Imaging Volume VII Corporate Products Financial Services Our Commitment ... Terms of Conditions

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