ANESTHETIC MANAGEMENT FOR OPERATIVE DELIVERY IN A FEMALE PATIENT WITH GLYCOGEN STORAGE DISEASE TYPE V (MCARDLE'S DISEASE)


Cite item

Full Text

Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription or Fee Access

Abstract

Background. McArdle’s disease (glycogen storage disease type V) is metabolic myopathy caused by homozygous or compound heterozygous mutations in the PYGM gene. It is characterized by skeletal muscle glycogen phosphorylase deficiency. The absence of this enzyme worsens glycogenolysis. In clinical practice, there is hypodiagnosis of this condition, which does not exclude the rarity of its definition. Description. The paper presents a clinical case of anesthetic management during operative delivery in a female patient with McArdle’s disease. Patients with McArdle’s disease who undergo surgery and anesthesia have a potential risk for perioperative complications, such as hypoglycemia, rhabdomyolysis, myoglobinuria, acute renal injury, postoperative fatigue, and, possibly, malignant hyperthermia. Conclusion. Regional anesthesia is safe and effective for operative delivery in female patients with McArdle’s disease.

Full Text

Restricted Access

About the authors

Pavel V. Troshin

Academician V.I. Kulakov National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of the Russian Federation

Email: chtpv28@gmail.com
doctor of the Department of Anaesthesiology and Resuscitation, assistant of the Department of Anesthesiology and Resuscitation 117997, Russia, Moscow, Ac. Oparina str. 4

Aleksey V. Pyregov

Academician V.I. Kulakov National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of the Russian Federation

Email: a_pyregov@oparina4.ru
MD, associate professor, head of the Department of Anesthesiology and Resuscitation 117997, Russia, Moscow, Ac. Oparina str. 4

Sergey V. Pismensky

Academician V.I. Kulakov National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of the Russian Federation

Email: smsu@mail.ru
doctor of the Department of Anaesthesiology and Resuscitation, assistant of the Department of Anesthesiology and Resuscitation 117997, Russia, Moscow, Ac. Oparina str. 4

Konstantin A. Kalachin

Academician V.I. Kulakov National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of the Russian Federation

Email: konstantin.kalachin@yandex.ru
doctor of the Department of Anaesthesiology and Resuscitation, assistant of the Department of Anesthesiology and Resuscitation 117997, Russia, Moscow, Ac. Oparina str. 4

Ekaterina Yu Sazonova

Federal State Budgetary Educational Institution of Higher Education «I.P.Pavlov Ryazan State Medical University» of the Ministry of Healthcare of the Russian Federation

Email: sazonova.katerina2017@ya.ru
6th year student 117997, Russia, Moscow, Ac. Oparina str. 4

Andrey I. Belyaev

Academician V.I. Kulakov National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of the Russian Federation

Email: andruwandruw@gmail.com
medical brother of the Department of Operative Gynecology 117997, Russia, Moscow, Ac. Oparina str. 4

References

  1. Vissing J., HallerR.G. The effect of oral sucrose on exercise tolerance in patients with McArdle’s disease. N. Engl. J. Med. 2003; 349(26): 2503-9.
  2. McArdle B. Myopathy due to a defect in muscle glycogen breakdown. Clin. Sci. 1951; 10(1): 13-35.
  3. DiMauro S., Tsujino S. Nonlysosomal glycogenoses. In: Engel A.G., Franzini-Armstrong C., eds. Myology. NewYork: McGraw-Hill; 1994: 1554-76.
  4. Bartram C., Edwards R.H.T., Beynon R.J. McArdle’s disease-muscle glycogen phosphorylase deficiency. Biochim. Biophys. Acta 1995; 1272(1): 1-13.
  5. Santalla A., Nogales-Gadea G., Encinar A.B., Vieitez I., Gonzalez-Quintana A., Serrano-Lorenzo P. et al. Genotypic and phenotypic features of all Spanish patients with McArdle disease: a 2016 update. BMC Genomics. 2017; 18 (Suppl. 8): 819.
  6. Lucia A., Nogales-Gadea G., Perez M., Martin M.A., Andreu A.L., Arenas J. McArdle disease: what do neurologists need to know? Nat. Clin. Pract. Neurol. 2008; 4(10): 568-77.
  7. Quinlivan R., Buckley J., James M., Twist A., Ball S., Duno M. et al. McArdle disease: a clinical review. J. Neurol. Neurosurg. Psychiatry. 2010; 81(11): 1182-8.
  8. De Castro M., Johnston J., Biesecker L. Determining the prevalence of McArdle disease from gene frequency by analysis of next-generation sequencing data. Genet. Med. 2015; 17(12): 1002-6.
  9. Brady S., Godfrey R., Scalco R.S., Quinlivan R.M. Emotionally-intense situations can result in rhabdomyolysis in McArdle disease. BMJ Case Rep. 2014; 2014. pii: bcr2013203272.
  10. Cochrane P., Alderman B. Normal pregnancy and successful delivery in myophosphorylase deficiency (McArdle’s disease). J. Neurol. Neurosurg. Psychiatry. 1973; 36(2): 225-7.
  11. Findlay S., Liu D., Rijhsinghani A. Acute compartment syndrome: clinical course and laboratory findings in pregnant patients with McArdle’s disease. Pain Med. 2014; 15(3): 481-2.
  12. Bollig G., Mohr S., Raeder J. McArdle’s disease and anaesthesia: case reports. Review of potential problems and association with malignant hyperthermia. Acta Anaesthesiol. Scand. 2005; 49(8): 1077-83.
  13. Weglinski M.R., Wedel D.J., Engel A.G. Malignant hyperthermia testing in patients with persistently increased serum creatine kinase levels. Anesth. Analg. 1997; 84(5): 1038-41.
  14. Isaacs H., Badenhorst M.E., Du Sautoy C. Myophosphorylase B deficiency and malignant hyperthermia. Muscle Nerve. 1989; 12(3): 203-5.

Supplementary files

Supplementary Files
Action
1. JATS XML
Download

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies