Results of Life Quality Evaluation in Children With Hepatic Variant of Glycogen Disease

Сурков, Андрей Николаевич; Chernikov, V.V.; Баранов, А. А.; Намазова-баранова, Л. С.; Потапов, А. С.; Винярская, И В

doi:10.15690/pf.v10i4.759

Cited by 8 publications

(1 citation statement)

References 4 publications

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“…Фиброз как процесс избыточного роста в печени соединительной ткани, развивающийся в ответ на повреждение паренхимы органа при многих хронических заболеваниях печени, на ранних стадиях заболевания носит динамический характер и вполне устраним: ликвидация причинно-значимого агента способно привести к регрессу фиброзирования. Выраженный ФП поддается обратному развитию значительно труднее [3].…”

Section: различия фп у детей и взрослыхunclassified

Liver fi brosis formation in children: the basics of preventive pediatric hepatology

Харитонова

Grigorуev

2023

jour

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Liver fibrosis is a natural outcome of almost any liver disease with a steady increase in incidence throughout the world. Considering the pathogenesis of liver fibrosis, the doctor- researcher is faced with the fact that the balance of regeneration processes in relation to the process of chronic inflammation is disturbed. The extracellular matrix accumulates in the liver tissue. Although this is a genetically determined process, but modifying factors play an important role in the progression of the disease. Liver fibrosis in its dynamic development leads to liver cirrhosis, hepatocellular carcinoma. Current data indicate the possibility of reversibility of liver fibrosis at any stage. Understanding the molecular mechanisms of the development of the pathological process is a key area of work for scientists involved in the development of antifibrotic therapy. The article discusses modern views on the prevention of the disease and the prospects for influencing the processes of liver fibrosis with an emphasis on childhood.

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Section: различия фп у детей и взрослыхunclassified

Liver fi brosis formation in children: the basics of preventive pediatric hepatology

Харитонова

Grigorуev

2023

jour

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Glycogen storage disease type Ib: modern understanding of the pathogenesis of neutropenia and prospects for its treatment with empagliflozin

Surkov,

Baranov,

Namazova-Baranova

et al. 2023

Pediatr. farmakol.

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Glycogen storage disease type Ib (GSD Ib) — is a disease from the group of hereditary metabolic diseases caused by insufficiency of the glucose-6-phosphate transporter (G6PT, SLC37A4), which leads to a violation of both glycogenolysis and gluconeogenesis and, as a consequence, to excessive accumulation of glycogen and fat in the liver, kidneys and intestinal mucosa. The main clinical manifestations and laboratory data include growth retardation, hepatomegaly, hypoglycemia, lactic acidosis, hyperuricemia and hyperlipidemia. Complications of this disease are hepatocellular adenoma with a possible risk of malignancy, nephropathy and osteoporosis. A specific sign of GSD Ib is neutropenia with impaired neutrophil function, which creates prerequisites for recurrent infections and the development of inflammatory bowel disease. Until the present, enzyme replacement therapy of GSD Ib has not been developed, therefore, the main methods of treatment are a specialized diet with the addition of raw corn starch (for relief of hypoglycemia) and the use of granulocyte colony stimulating factor (for relief of neutropenia). However, the recent establishment of the role of 1,5-anhydroglucitol in the pathogenesis of neutrophil dysfunction in GSD Ib has led to a reprofiling of indications for the use of empagliflozin, a type 2 renal sodium—glucose cotransporter inhibitor (SGLT2). In the modern literature, it is reported about a minor, but very successful experience of its use in patients with GSD Ib (outside the framework of official indications for use) and a beneficial effect on neutrophil dysfunction and its clinical consequences. Oddly enough, this hypoglycemic drug improved not only metabolic, but also glycemic control in patients with GSD Ib, despite the fact that the pathology is based on chronic hypoglycemia. More and more evidence points to the role of empagliflozin in the regulation of cellular homeostasis (for example, fatty acid metabolism, glucose, cholesterol, apoptosis and cell proliferation, in particular in the liver) by influencing the activity of sirtuin 1 (SIRT1), AMP-activated protein kinase (AMPK) and signal molecules such as -serine/threonine protein kinase (Akt) and a mechanical target of rapamycin (mTOR), which leads to an improvement in the structure and function of mitochondria, stimulation of autophagy, reducing oxidative stress and suppressing inflammation. Modulation of these pathways shifts oxidative metabolism from carbohydrates to lipids and leads to a key decrease in insulin levels, resistance to it, glucose and lipotoxicity. This review presents current data on the pathogenesis of neutropenia and the possibility of using empagliflozin for its relief in patients with GSD Ib.

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

Surkov,

Baranov,

Arakelyan

et al. 2023

Annals RAMS

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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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Results of Life Quality Evaluation in Children With Hepatic Variant of Glycogen Disease

Cited by 8 publications

References 4 publications

Liver fi brosis formation in children: the basics of preventive pediatric hepatology

Liver fi brosis formation in children: the basics of preventive pediatric hepatology

Glycogen storage disease type Ib: modern understanding of the pathogenesis of neutropenia and prospects for its treatment with empagliflozin

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

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