Hepatonephromegaly Glycogen

Hepatoneuromegalic glycogenic disease (HTGB), also known as “nephrogenic” hypoglycemic hepatopathy, is a rare genetic metabolic disorder in which glycogen accumulates in the liver and kidneys, which can lead to serious health problems.

Most people with HTGB have symptoms soon after birth. They may show signs of vomiting, fatigue, and sometimes consciousness to the point of coma. This disease occurs as a result of problems with the production of glycogen (the main storage carbohydrate in cells), which accumulates in the liver and kidney tubules. When glycogen stores become full, the body loses the ability to process it into glucose, leading to sudden drops in blood sugar levels.

Although certain factors can lead to the development of HTGB, the genetic defect itself is rarely specific. This usually occurs due to abnormalities in the ribosomal endoplasmic reticulum molecule 4.

**Hepatonephromegaly** Richter's disease (“hepatonephritis”), according to Yu. I. Prikhodko et al. (2019), is a systemic disease of unknown etiology, occurring with an increase in the activity of nonspecific coagulation factors in the blood, overload of various organs and tissues with blood, generalized necrosis, and the formation of