Nutritional support in glycogen storage diseases: evolution of treatment and unresolved contradictions

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Abstract

Hepatic forms of glycogen storage diseases (GSD) are a group of diseases in which abnormal accumulation or cleavage of glycogen leads to potentially life-threatening hypoglycemic conditions and metabolic disorders. The use of specialized diet therapy with the addition of raw corn starch has significantly improved the results of GSD treatment, turning a pathology previously considered fatal into a condition in which people can feel satisfactory with proper care. Despite many years of research, there is no consensus on the optimal dietary treatment of GSD. This article describes the evolution of dietary therapy of hepatic forms of GSD (types 0, I, III, VI, IX and XI), presents historical and modern approaches to the prevention of hypoglycemia and related complications.

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About the authors

Andrej N. Surkov

Petrovsky Russian Scientific Center of Surgery; Pirogov Russian National Research Medical University

Author for correspondence.
Email: surkov@gastrockb.ru
ORCID iD: 0000-0002-3697-4283
SPIN-code: 4363-0200

MD, PhD

Russian Federation, 2 Abrikosovsky lane, 119991, Moscow; 1, st. Ostrovityanova, 117997 Moscow

Aleksandr A. Baranov

Petrovsky Russian Scientific Center of Surgery; Sechenov First Moscow State Medical University

Email: baranov@pediatr-russia.ru
ORCID iD: 0000-0003-3987-8112
SPIN-code: 3570-1806

MD, PhD, Professor, Academician of the RAS

Russian Federation, 2 Abrikosovsky lane, 119991, Moscow; 8 bldn 2, Trubetskaya str., 119992, Moscow

Anna L. Arakelyan

Petrovsky Russian Scientific Center of Surgery; Sechenov First Moscow State Medical University

Email: a.silonyan@mail.ru
ORCID iD: 0000-0002-6837-9753
SPIN-code: 4218-7060

MD

Russian Federation, 2 Abrikosovsky lane, 119991, Moscow; 8 bldn 2, Trubetskaya str., 119992, Moscow

Evgenij E. Bessonov

Petrovsky Russian Scientific Center of Surgery; Sechenov First Moscow State Medical University

Email: bessonov@gastrockb.ru
ORCID iD: 0000-0001-5549-857X
SPIN-code: 2463-1374

MD

Russian Federation, 2 Abrikosovsky lane, 119991, Moscow; 8 bldn 2, Trubetskaya str., 119992, Moscow

Natal'ya V. Zhurkova

Petrovsky Russian Scientific Center of Surgery; Sechenov First Moscow State Medical University

Email: n1972z@yandex.ru
ORCID iD: 0000-0001-6614-6115
SPIN-code: 4768-6310

MD, PhD

Russian Federation, 2 Abrikosovsky lane, 119991, Moscow; 8 bldn 2, Trubetskaya str., 119992, Moscow

Diana A. Labash

Pirogov Russian National Research Medical University

Email: desyunya@mail.ru
ORCID iD: 0009-0009-8508-5245

MD

Russian Federation, 1, st. Ostrovityanova, 117997 Moscow

Marika I. Ivardava

Petrovsky Russian Scientific Center of Surgery; Pirogov Russian National Research Medical University

Email: makussa@mail.ru
ORCID iD: 0000-0002-4669-9510
SPIN-code: 4865-4688

MD, PhD

Russian Federation, 2 Abrikosovsky lane, 119991, Moscow; 1, st. Ostrovityanova, 117997 Moscow

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