scholarly journals Clinical Pearls in Anesthesia for Xeroderma Pigmentosum: A Case Report

2015 ◽  
Vol 9 (1) ◽  
pp. 36-38
Author(s):  
S.B. Shah ◽  
U. Hariharan ◽  
B.K. Naithani ◽  
A.K. Bhargava
Keyword(s):  
Case Report ◽  
High Frequency ◽  
Xeroderma Pigmentosum ◽  
Protective Clothing ◽  
Autosomal Recessive ◽  
Perioperative Management ◽  
Ultra Violet ◽  
Neurological Complications ◽  
Operation Theatre ◽  
Prolonged Effect

Xeroderma Pigmentosum (XP) is a rare autosomal recessive (AR) disease characterized by hypersensitivity of the skin to ultra violet (UV) radiation, resulting in a high frequency of UV induced skin tumors and progressive neurological complications at an early age. Through the following case report we emphasize that perioperative management of xeroderma patients entails meticulous evaluation for neurological abnormalities, shielding the skin from OT (operation theatre) lights by using protective clothing, sunscreen and UV blocking film as well as avoidance of genotoxic drugs like volatile anaesthetics and paracetamol. One must be prepared for the possibility of difficult mask ventilation (we used a mask one size larger), difficult intubation and prolonged effect of muscle relaxants (as in our case) due to skin atrophy, neoplasia, joint contracture and neuronal dysfunction.

scholarly journals Molecular mechanism in a rare autosomal recessive case of xeroderma pigmentosum - a case report

2014 ◽  
Vol 03 (03) ◽  
pp. 178-183
Author(s):  
Vijaya Ramanathan ◽  
Anand Ramanathan
Keyword(s):  
Case Report ◽  
Xeroderma Pigmentosum ◽  
Chromosomal Instability ◽  
Autosomal Recessive ◽  
Excision Repair ◽  
Sister Chromatid Exchanges ◽  
Ocular Manifestations ◽  
Nucleotide Excision ◽  
Chromosomal Breaks ◽  
Chromatid Exchanges

AbstractChromosomal instability syndromes are a special group of disorders of cytogenetic interest which comprises of several rare, autosomal recessive conditions. Following exposure to sunlight, excessive chromosomal instability, breakage, defective nucleotide excision repair in DNA, defective apoptosis and increased susceptibility to neoplasia occurs. Xeroderma pigmentosum (XP) is characterised by the presence of chromosomal breakages, associated with increased frequency of sister chromatid exchanges. This is a case report of a 6 year old, male child having XP with dermal and ocular manifestations. Chromosomal breaks in chromosomal spread are seen. If it occurs in families, consanguinous marriages should be avoided; appropriate genetic counselling suggested and simple sun guarding techniques with appropriate protection from UV exposure can reduce the morbidity in these patients.

scholarly journals Xeroderma Pigmentosum: Important Oral Manifestations Case Report

2021 ◽  
Vol 3 (1) ◽  
pp. 01-04
Author(s):  
Guadalupe Tostado
Keyword(s):  
Case Report ◽  
Xeroderma Pigmentosum ◽  
Skin Disorder ◽  
Autosomal Recessive ◽  
Oral Manifestations ◽  
Professional Management ◽  
Dental Professional ◽  
Autosomal Recessive Manner

The Xeroderma pigmentosum [XP] is a rare inherited skin disorder and transmitted in an autosomal recessive manner. The aim of the present article is to report a XP case with oral manifestations and to discuss the role of the dental professional management of this entity. A 5 year old male presented lentigos all over the skin and predominated in photoexposed areas. Oral hygiene was good and dental affection was important. No lesions were observed in the lips, tongue and the rest of the bucal mucosa but multiples teeth with cavities were observed. Antibiotic treatment was started and surgical treatment. Besides dermatological, ophtamological and neurological management, XP patients require constant dental care and follow –up in order to control the occurrence of new lesions on the lips or inside oral cavity.

scholarly journals COVID‐19 complicating perioperative management of LVAD implantation: A case report and systematic review

2021 ◽  
Author(s):  
Deborah de Sá Pereira Belfort ◽  
Bruno Biselli ◽  
Mônica Samuel Ávila ◽  
Maria Tereza Sampaio de Sousa Lira ◽  
Filomena Regina Barbosa Gomes Galas ◽  
...  
Keyword(s):  
Systematic Review ◽  
Case Report ◽  
Perioperative Management

scholarly journals Anesthetic management for cesarean delivery in a woman with congenital atlantoaxial dislocation and Chiari type I anomaly: a case report and literature review

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yuyan Nie ◽  
Weimin Zhou ◽  
Shaoqiang Huang
Keyword(s):  
Case Report ◽  
Cesarean Delivery ◽  
Anesthetic Management ◽  
Neurological Complications ◽  
Atlantoaxial Dislocation ◽  
Epidural Needle ◽  
Team Approach ◽  
Anesthetic Techniques ◽  
Type I ◽  
Combined Spinal Epidural Anesthesia

Abstract Background The preferable choice of anesthesia for the patients with congenital atlantoaxial dislocation (CAAD) and type I Arnold Chiari malformations (ACM-I) has been a very confusing issue in clinical practice. We describe the successful administration of combined spinal-epidural anesthesia for a woman with CAAD and ACM-1 accompanied by syringomyelia. Case presentation Our case report presents the successful management of a challenging obstetric patient with CAAD and ACM-1 accompanied by syringomyelia. She had high risks of difficult airway and aspiration. The injection of bolus drugs through the spinal or epidural needle may worsen the previous neurological complications. The patient was well evaluated with a multidisciplinary technique before surgery and the anesthesia was provided by a skilled anesthesiologist with slow spinal injection. Conclusions An interdisciplinary team approach is needed to weigh risks and benefits for patients with CAAD and ACM-1 undergoing cesarean delivery. Therefore, an individual anesthetic plan should be made basing on the available anesthetic equipments and physicians’ clinical experience on anesthetic techniques.

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

2013 ◽  
Vol 7 (1) ◽  
Author(s):  
Salaheddine Fjouji ◽  
Mustapha Bensghir ◽  
Bahija Yafat ◽  
Najib Bouhabba ◽  
Elhoucine Boutayeb ◽  
...  
Keyword(s):  
Case Report ◽  
Xeroderma Pigmentosum
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