Anesthetic management for operative delivery in a female patient with glycogen storage disease type V (McArdle’s disease)
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.Troshin P.V., Pismensky S.V., Kalachin K.A., Sazonova E.Yu., Pyregov A.V., Belyaev A.I.
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.
Keywords
anesthesia
glycogen storage disease
McArdle’s disease
delivery
rhabdomyolysis
acute renal injury
malignant hyperthermia
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Received 02.02.2019
Accepted 22.02.2019
About the Authors
Troshin, Pavel V., doctor of the Department of Anaesthesiology and Resuscitation, assistant of the Department of Anesthesiology and Resuscitation, National Medical Research Center for Obstetrics, Gynecology and Perinatology named after Academician V.I. Kulakov Ministry of Health of Russia.Tel.: +7 (495)4387777. E-mail: chtpv28@gmail.com 117997, Russia, Moscow, Ac. Oparina str. 4.
Pyregov, Aleksey V., MD, associate professor, head of the Department of Anesthesiology and Resuscitation, National Medical Research Center for Obstetrics,
Gynecology and Perinatology named after Academician V.I. Kulakov Ministry of Health of Russia. Tel.: +7(495)4387777. E-mail: a_pyregov@oparina4.ru
117997, Russia, Moscow, Ac. Oparina str. 4.
Pismensky, Sergey V., doctor of the Department of Anaesthesiology and Resuscitation, assistant of the Department of Anesthesiology and Resuscitation,
National Medical Research Center for Obstetrics, Gynecology and Perinatology named after Academician V.I. Kulakov Ministry of Health of Russia.
Tel.: +7 (495)4387777. E-mail: smsu@mail.ru 117997, Russia, Moscow, Ac. Oparina str. 4.
Kalachin, Konstantin A., doctor of the Department of Anaesthesiology and Resuscitation, assistant of the Department of Anesthesiology and Resuscitation,
National Medical Research Center for Obstetrics, Gynecology and Perinatology named after Academician V.I. Kulakov Ministry of Health of Russia.
Tel.: +7(495)4387777. E-mail: konstantin.kalachin@yandex.ru 117997, Russia, Moscow, Ac. Oparina str. 4.
Sazonova Ekaterina Yu, 6th year student, Federal State Budgetary Educational Institution of Higher Education «I.P.Pavlov Ryazan State Medical University»
of the Ministry of Healthcare of the Russian Federation; Tel.: +7491297180. E-mail: sazonova.katerina2017@ya.ru
390026, Russia, Ryazan, ul. Vysokovoltnaya, d 9.
Belyaev, Andrey I., medical brother of the Department of Operative Gynecology, National Medical Research Center for Obstetrics, Gynecology and Perinatology named
after Academician V.I. Kulakov Ministry of Health of Russia. Tel.: +7(999)8086255. E-mail: andruwandruw@gmail.com 117997, Russia, Moscow, Ac. Oparina str. 4.
For citation: Troshin P.V., Pyregov A.V., Pismensky S.V., Kalachin K.A., Sazonova E.Yu., Belyaev A.I. Anesthetic management for operative delivery in a female patient with glycogen storage disease type V (McArdle’s disease).
Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2019; (8): 170-73 (in Russian).
http://dx.doi.org/10.18565/aig.2019.8.170-173
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