Literature DB >> 23429452

Congenital Insensitivity to Pain with Anhidrosis (HSAN Type IV), Extremely Rare Syndrome that Can Be Easily Missed by Bone and Joint Surgeons: A Case Report.

Nadeem Ali1, Sudesh Sharma, Sonali Sharma, Younis Kamal, Sushil Sharma.   

Abstract

BACKGROUND: Congenital insensitivity to pain with anhidrosis is an extremely rare disorder in which injuries can often be missed by patient, parents and even by orthopedic surgeon. Pain and tenderness, on which a trauma team so much depends to make a clinical diagnosis and to decide whether to go for radiological evaluation can be misleading in this rare syndrome. So complete clinical examination still forms the corner stone to avoid misdiagnosis and pick up the rare disorders. CASE
PRESENTATION: We present a 5 year old girl child, who was brought to us as a case of one and a half month old neglected trauma left leg and was diagnosed to be suffering from congenital insensitivity to pain with anhidrosis (HSAN Type IV).
CONCLUSION: Congenital insensitivity to pain with anhidrosis is extremely rare entity, in which patients are subjected to repeated injuries which are often neglected. There is no specific treatment but patient training and parent education are key to avoid further neglect and damage.

Entities:  

Keywords:  Anhidrosis; Congenital Insensitivity to Pain with Anhidrosis; Hereditary sensory autonomic neuropathy; Hyperpyrexia; Osteoacrolysis

Year:  2012        PMID: 23429452      PMCID: PMC3533163     

Source DB:  PubMed          Journal:  Iran J Pediatr        ISSN: 2008-2142            Impact factor:   0.364


  11 in total

1.  Congenital insensitivity of pain with anhidrosis.

Authors:  Bhaskar Gupta
Journal:  Indian J Pediatr       Date:  2003-01       Impact factor: 1.967

Review 2.  Congenital insensitivity to pain: an update.

Authors:  Elna M Nagasako; Anne Louise Oaklander; Robert H Dworkin
Journal:  Pain       Date:  2003-02       Impact factor: 6.961

3.  Congenital insensitivity to pain with anhidrosis and progressing acro-osteolysis: a case report with 7-year follow-up.

Authors:  Jun Hu; Ai-bin Zhang; Zhen Lin; Jiang-nan Zhou
Journal:  Chin Med J (Engl)       Date:  2006-12-20       Impact factor: 2.628

4.  [Orthopedic aspects of congenital insensitivity to pain].

Authors:  C Bronfen; H Bensahel; J G Teule
Journal:  Chir Pediatr       Date:  1985

5.  Humoral immunodeficiency in congenital insensitivity to pain with anhidrosis.

Authors:  Sara Sebnem Kilic; Rifatcan Ozturk; Bartu Sarisozen; Annelies Rotthier; Jonathan Baets; Vincent Timmerman
Journal:  Neurogenetics       Date:  2008-12-17       Impact factor: 2.660

6.  Neuropathic osteoarthropathy in a patient with congenital insensitivity to pain.

Authors:  Maryam Mobini; Ali Javadzadeh; Jafar Forghanizadeh
Journal:  Arch Iran Med       Date:  2009-11       Impact factor: 1.354

7.  Traumatic lesions from congenital insensitivity to pain with anhidrosis in a pediatric patient: dental management.

Authors:  Beatriz Gonçalves Neves; Rosemere Teixeira Roza; Gloria Fernanda Castro
Journal:  Dent Traumatol       Date:  2009-10       Impact factor: 3.333

Review 8.  Congenital insensitivity to pain with anhidrosis (hereditary sensory and autonomic neuropathy type IV)

Authors:  S Rosemberg; S K Marie; S Kliemann
Journal:  Pediatr Neurol       Date:  1994-07       Impact factor: 3.372

9.  Congenital insensitivity to pain with anhidrosis: a case report.

Authors:  J S Kim; Y J Woo; G M Kim; C J Kim; J S Ma; T J Hwang; M C Lee
Journal:  J Korean Med Sci       Date:  1999-08       Impact factor: 2.153

10.  Congenital Insensitivity to Pain and Anhydrosis (CIPA) Syndrome; A Report of 4 Cases.

Authors:  Khadije Daneshjou; Hanieh Jafarieh; Seyed-Reza Raaeskarami
Journal:  Iran J Pediatr       Date:  2012-09       Impact factor: 0.364

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  5 in total

1.  Congenital Insensitivity to Pain with Anhidrosis in Twin Sisters with Sensorineural Deafness.

Authors:  Savitha Mysore Ramaraj; Divya Durga
Journal:  Indian J Pediatr       Date:  2015-03-18       Impact factor: 1.967

2.  Multidisciplinary assessment of congenital insensitivity to pain syndrome.

Authors:  Vugar Nabiyev; Ateş Kara; M Cemalettin Aksoy
Journal:  Childs Nerv Syst       Date:  2016-03-21       Impact factor: 1.475

3.  Hereditary and sensory autonomic neuropathies.

Authors:  Mojdeh Khaledi; Nima Rezaei
Journal:  Iran J Pediatr       Date:  2012-12       Impact factor: 0.364

4.  Guided growth in the correction of knee deformity in patients with congenital insensitivity to pain.

Authors:  Soroush Baghdadi; Sadegh Saberi; Taghi Baghdadi
Journal:  J Orthop Surg Res       Date:  2021-03-11       Impact factor: 2.359

5.  Anesthetic management of children with congenital insensitivity to pain with anhidrosis.

Authors:  Yongsheng Qiu; Liyuan Zhao; Dongdong Yao; Yingping Jia
Journal:  Pediatr Investig       Date:  2019-10-28
  5 in total

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