Anestesia em paciente com Xeroderma Pigmentoso: relato de caso

Oliveira, Carlos Rogério Degrandi; Elias, L; Barros, Ana Cláudia de Melo; Conceição, Diogo Brüggemann da

doi:10.1590/s0034-70942003000100006

Cited by 26 publications

(13 citation statements)

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“…Xeroderma pigmentosum is inherited as an autosomal recessive trait and the main characteristics of this disease include sunlight hypersensitivity, tremendous risk of skin cancer and neurologic disorders (1,(3)(4)(5). First described by Kaposi in 1870, XP begins in childhood and progresses with premalignant and malignant lesions often leading to death in early adulthood.7 Classical types of XP have a defect in nucleotide excision repair (NER)3.…”

Section: Discussionmentioning

confidence: 99%

“…Xeroderma pigmentosum (XP) is a rare genetic disorder with a recessive autosomal inheritance (1)(2)(3)(4)(5), which is characterized by hypersensitivity of the skin to ultraviolet (UV)radiation and results in premature development of neoplasias and progressive neurological complications (2,3) The underlying mechanism is deletion and reconstruction of DNA. These patients are at excessively high risk of skin cancer on sun-exposed areas compared to normal individuals (4,5). The aim of this report was to evaluate specific anesthetic and airway considerations in these patients.…”

Section: Introductionmentioning

confidence: 99%

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Airway Management in a Pediatric Patient with Xeroderma Pigmentosum: A Case Report

Seyedhejazi

Dehdilani

Mehdi

et al. 2015

ABCmed

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Airway Management in a Pediatric Patient with Xeroderma Pigmentosum: A Case Report

Seyedhejazi

Dehdilani

Mehdi

et al. 2015

ABCmed

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“…Sevoflurane is often chosen for such patients based on its favorable properties for pediatric anesthesia, its ease of use, its malleability, and its clinical safety reported during its use even at high concentrations in inhalational anesthesia using a mask [4]. In our institution, sevoflurane has been used since 2006 with approximately 30 operations per day.…”

Section: Discussionmentioning

confidence: 99%

Postoperative neurological aggravation after anesthesia with sevoflurane in a patient with xeroderma pigmentosum: a case report

et al. 2013

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IntroductionXeroderma pigmentosum is a rare autosomal recessive disease that causes changes in skin pigmentation, precancerous lesions and neurological abnormalities. It is a defect in the nucleotide excision repair mechanism. It has been reported that volatile anesthetics has a possible genotoxic side effect and deranged nucleotide excision repair in cells obtained from a patient with xeroderma pigmentosum.We report an unusual case of postoperative neurological aggravation in a patient with xeroderma pigmentosum anesthetized with sevoflurane.Case presentationA 24-year-old African woman, who has had xeroderma pigmentosum since childhood, was admitted to our hospital for a femoral neck fracture. A preoperative physical examination revealed that she had a resting tremor with ataxia. She had cutaneous lesions such as keratosis and hyperpigmentation on her face and both hands. There was no major alteration of cognitive function, muscular strength was maintained and her osteotendinous reflexes were preserved. Surgical fixation was performed under general anesthesia after the failure of spinal anesthesia. All parameters were stable during surgery. When she woke up four hours later, the patient presented with confusion and psychomotor agitation, sharpened reflexes and the Babinski reflex was present. Her postoperative test results and a magnetic resonance imaging scan were unremarkable. It was suggested that sevoflurane had had a probable deleterious effect on the neurological status of this patient.ConclusionThe anesthetizing of a patient with xeroderma pigmentosum is associated with a risk of worsening neurological disorders. At present, there are no clear recommendations to avoid the use of volatile agents in the anesthetic management of patients with xeroderma pigmentosum. More clinical and experimental research is needed to confirm the sensitivity of patients with xeroderma pigmentosum to sevoflurane and other halogenated anesthetics.

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“…fasiyal ve orofarengeal anomalileri nedeni ile maske ventilasyonlarında ve entübasyonlarında sorun yaşanabilmektedir. 2 Zor hava yolu nedeni ile kör nazal entübasyon uygulanarak anestezi verilen bir olgu bildirilmiştir. 3 Ağız açıklığı 2,5 cm ölçülen hastamızın mallampati skoru 4 olup, fasiyal kontraktürleri de mevcut olduğundan zor hava yolu ekipmanı hazırlanmıştı (Resim 2).…”

Section: Olgu Sunumuunclassified