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


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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.

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作者简介

Pavel 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 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 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 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 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 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

参考

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