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Cardiac arrest after anesthetic management in a patient with hereditary sensory autonomic neuropathy type IV.

Ergül Y, Ekici B, Keskin S - Saudi J Anaesth (2011)

Bottom Line: Recurrent hyperpyrexia and anhydrosis are seen in patients as a result of a lack of sweat gland innervation.Self-mutilation and insensitivity to pain result in orthopedic complications and patients undergone recurrent surgical interventions with anesthesia.However, these patients are prone to perioperative complications such as hyperthermia, hypothermia, and cardiac complications like bradycardia and hypotension.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Neurology of Cerrahpaşa Medical Faculty, Istanbul University, Istanbul, Turkey.

ABSTRACT
Hereditary sensory autonomic neuropathy type IV is a rare disorder with an autosomal recessive transmission and characterized by self-mutilation due to a lack in pain and heat sensation. Recurrent hyperpyrexia and anhydrosis are seen in patients as a result of a lack of sweat gland innervation. Self-mutilation and insensitivity to pain result in orthopedic complications and patients undergone recurrent surgical interventions with anesthesia. However, these patients are prone to perioperative complications such as hyperthermia, hypothermia, and cardiac complications like bradycardia and hypotension. We report a 5-year-old boy with hereditary sensory autonomic neuropathy type IV, developing hyperpyrexia and cardiac arrest after anesthesia.

No MeSH data available.


Related in: MedlinePlus

Partial autoamputation in the right toe
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Figure 0003: Partial autoamputation in the right toe

Mentions: On initial evaluation, he was found to have a dry skin and tissue defects in the tongue, gum, and fingers due to self-mutilation [Figure 1]. His X-rays revealed the absence of the left caput femoris due to osteomyelitis and abscess [Figure 2] as well as autoamputations in several fingers and toes [Figure 3]. He was non-responsive to pain with absent pupillary reaction to light. He had several fever attacks (39° C) without any laboratory signs of infection and/or inflammation. He also showed bradycardia which did not respond to atropine treatment.


Cardiac arrest after anesthetic management in a patient with hereditary sensory autonomic neuropathy type IV.

Ergül Y, Ekici B, Keskin S - Saudi J Anaesth (2011)

Partial autoamputation in the right toe
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3101766&req=5

Figure 0003: Partial autoamputation in the right toe
Mentions: On initial evaluation, he was found to have a dry skin and tissue defects in the tongue, gum, and fingers due to self-mutilation [Figure 1]. His X-rays revealed the absence of the left caput femoris due to osteomyelitis and abscess [Figure 2] as well as autoamputations in several fingers and toes [Figure 3]. He was non-responsive to pain with absent pupillary reaction to light. He had several fever attacks (39° C) without any laboratory signs of infection and/or inflammation. He also showed bradycardia which did not respond to atropine treatment.

Bottom Line: Recurrent hyperpyrexia and anhydrosis are seen in patients as a result of a lack of sweat gland innervation.Self-mutilation and insensitivity to pain result in orthopedic complications and patients undergone recurrent surgical interventions with anesthesia.However, these patients are prone to perioperative complications such as hyperthermia, hypothermia, and cardiac complications like bradycardia and hypotension.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Neurology of Cerrahpaşa Medical Faculty, Istanbul University, Istanbul, Turkey.

ABSTRACT
Hereditary sensory autonomic neuropathy type IV is a rare disorder with an autosomal recessive transmission and characterized by self-mutilation due to a lack in pain and heat sensation. Recurrent hyperpyrexia and anhydrosis are seen in patients as a result of a lack of sweat gland innervation. Self-mutilation and insensitivity to pain result in orthopedic complications and patients undergone recurrent surgical interventions with anesthesia. However, these patients are prone to perioperative complications such as hyperthermia, hypothermia, and cardiac complications like bradycardia and hypotension. We report a 5-year-old boy with hereditary sensory autonomic neuropathy type IV, developing hyperpyrexia and cardiac arrest after anesthesia.

No MeSH data available.


Related in: MedlinePlus