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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

Cranial MRI shows ventricular dilatation and cortical atrophy
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Figure 0004: Cranial MRI shows ventricular dilatation and cortical atrophy

Mentions: He was non-responsive to intradermal histamine and pilocarpine iontophoresis. His whole blood count, blood chemistry, serum and urine amino acids, humoral and cellular immunity tests were all non-diagnostic. His motor and sensory peripheric nerve conduction velocities were also normal. Cranial magnetic resonance imaging showed cortical and supratentorial atrophy with dilatation of the third and fourth ventricules [Figure 4]. He was diagnosed with HSAN type IV based on the clinical and laboratory findings.


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)

Cranial MRI shows ventricular dilatation and cortical atrophy
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 0004: Cranial MRI shows ventricular dilatation and cortical atrophy
Mentions: He was non-responsive to intradermal histamine and pilocarpine iontophoresis. His whole blood count, blood chemistry, serum and urine amino acids, humoral and cellular immunity tests were all non-diagnostic. His motor and sensory peripheric nerve conduction velocities were also normal. Cranial magnetic resonance imaging showed cortical and supratentorial atrophy with dilatation of the third and fourth ventricules [Figure 4]. He was diagnosed with HSAN type IV based on the clinical and laboratory findings.

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