About L-2-Hydroxyglutaric Aciduria

L-2-Hydroxyglutaric Aciduria, also known as l-2-hydroxyglutaric acidemia, is related to combined d-2- and l-2-hydroxyglutaric aciduria and d-2-hydroxyglutaric aciduria 1, and has symptoms including seizures, abnormal pyramidal signs and abnormality of extrapyramidal motor function. An important gene associated with L-2-Hydroxyglutaric Aciduria is L2HGDH (L-2-Hydroxyglutarate Dehydrogenase), and among its related pathways/superpathways are Metabolism and "Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. ". Affiliated tissues include cerebellum, brain and skeletal muscle, and related phenotypes are intellectual disability, severe and intellectual disability, progressive

Major Symptoms of L-2-Hydroxyglutaric Aciduria

The main symptoms of L-2-Hydroxyglutaric aciduria (L-2 aciduria) include:

1. Metabolic acidosis: The body of patients with L-2 aciduria produces a large amount of acidic substances, resulting in a decrease in blood pH and metabolic acidosis. Poisoned.

2. Kidney damage: The accumulation of acidic substances in the body can damage kidney function and lead to abnormal kidney function.

3. Digestive system symptoms: Patients with L-2 aciduria may experience gastrointestinal discomfort, diarrhea, constipation and other symptoms.

4. Nervous system symptoms: Acidic substances may have an impact on the nervous system, causing patients to have symptoms such as difficulty concentrating, headaches, and insomnia.

5. Increased risk of cardiovascular disease: Patients with L-2 aciduria are at higher risk to the cardiovascular system, and acidic substances may increase the risk of cardiovascular disease.

6. Skin symptoms: Acidic substances may affect the skin, causing itching, inflammation, redness and swelling on the patient's skin.

7. Respiratory symptoms: Acidic substances may have an impact on the respiratory system, causing patients to have difficulty breathing, asthma and other symptoms.

8. Blood system symptoms: Acidic substances may affect the blood, causing patients to experience symptoms such as anemia and leukopenia.

9. Electrolyte disorders: Acidic substances may affect the electrolyte balance in the body, causing patients to suffer from symptoms of electrolyte disorders such as low potassium, low sodium, and low chloride.

10. Nutritional disorders: Patients with L-2 aciduria may suffer from malnutrition due to nutrient absorption disorders caused by the influence of acidic substances.

Suitable Lifestyle for People with L-2-Hydroxyglutaric Aciduria

L-2-Hydroxyglutaric aciduria (LHGD) is an inherited metabolic disorder that often results in liver damage and kidney disease. Therefore, for patients with LHGD, lifestyle adjustments are crucial for disease control and recovery. For patients with LHGD, the following are some recommended lifestyle adjustments:

1. Follow the doctor's treatment recommendations: First, patients should follow the doctor's treatment recommendations and undergo treatment under the doctor's supervision.

2. Adjust diet: Patients need to follow the doctor's dietary recommendations, avoid eating high-fat, high-cholesterol and high-salt foods, and control the intake of carbohydrates and protein in the diet.

3. Weight control: Patients need to control their weight according to the doctor's advice to reduce the burden on the liver.

4. Avoid drinking alcohol and smoking: Patients should avoid drinking alcohol and smoking to reduce damage to the liver.

5. Perform appropriate exercises: Patients can perform appropriate exercises to maintain good health, but strenuous exercise should be avoided.

6. Regular examination: Patients should have regular examinations of liver and kidney function to monitor the development of the disease and the effect of treatment. In short, lifestyle adjustments are very important for patients with LHGD. They should follow the doctor's treatment recommendations and pay attention to adjustments in diet, weight, drinking, and smoking.

Other Diseases

D-2-Hydroxyglutaric Aciduria Orotic Aciduria 3-Methylglutaconic Aciduria Glutaric Aciduria Type 1 3-Methylglutaconic Aciduria Type I Glutaric Aciduria Type 2 3-Hydroxy-3-Methylglutaric Aciduria Glutaric Aciduria Type 3 3-Methylglutaconic Aciduria Type IV CblC Type Methylmalonic Aciduria and Homocystinuria

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