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Monatsschrift Kinderheilkunde

, Volume 155, Issue 3, pp 281–286 | Cite as

Hereditäre sensorische und autonome Neuropathie Typ IV

2 Fallberichte
  • B. KohlEmail author
  • W. Hülsemann
  • R. Habenicht
  • M. J. Hilz
  • B. Stemper
  • B. Püst
Originalien
  • 120 Downloads

Zusammenfassung

Wir berichten über 2 Schulkinder mit hereditärer sensorischer Neuropathie Typ IV, die im englischsprachigen Raum auch mit dem Akronym CIPA für „congenital insensitivity to pain and anhidrosis“ bezeichnet wird. Der englischsprachige Ausdruck nennt bereits die wesentlichen Symptome dieser sehr seltenen Erkrankung: kongenitale Unempfindlichkeit gegenüber Schmerz und die Unfähigkeit, zu schwitzen. Neben der klinischen Symptomatik und diagnostischen Maßnahmen wird der Krankheitsverlauf der beiden Patienten unter besonderer Berücksichtigung handchirurgisch relevanter Komplikationen der Erkrankung dargestellt.

Schlüsselwörter

HSAN Typ IV CIPA Kind Handchirurgisch relevante Komplikationen 

Hereditary sensory neuropathy type IV

Two case reports

Abstract

We report on two schoolboys suffering from hereditary sensory neuropathy type IV (HSAN IV). HSAN IV is also referred to in English-speaking countries as CIPA, which stands for congenital insensitivity to pain and anhidrosis. Thus, this term names the cardinal symptoms of this extremely rare disease. In our article, in addition to symptoms and diagnostic procedures we illustrate the clinical course in both our patients, focusing on complications requiring reconstructive surgery of the hands and feet.

Keywords

HSAN Type IV CIPA Childhood Hand surgery 

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Copyright information

© Springer Medizin Verlag 2005

Authors and Affiliations

  • B. Kohl
    • 1
    • 4
    Email author
  • W. Hülsemann
    • 2
  • R. Habenicht
    • 2
  • M. J. Hilz
    • 3
  • B. Stemper
    • 3
  • B. Püst
    • 1
  1. 1.Neuropädiatrische AbteilungKatholisches Kinderkrankenhaus Wilhelmstift Hamburg
  2. 2.Handchirurgische AbteilungKatholisches Kinderkrankenhaus Wilhelmstift Hamburg
  3. 3.Klinik für NeurologieUniversität Erlangen-NürnbergErlangen
  4. 4.Neuropädiatrische AbteilungKatholisches Kinderkrankenhaus WilhelmstiftHamburg

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