你的新陈代谢如何工作？

新陈代谢是你的身体用来改变你吃进入燃料的食物的化学过程。

营养(food) consists of proteins, carbohydrates, and fats. These substances are broken down by enzymes in your digestive system, and then carried to the cells where they can be used as fuel. Your body either uses these substances immediately, or stores them in the liver, body fat, and muscle tissues for later use.

当代谢过程失败并导致身体具有太多或太少的必需物质时，发生代谢紊乱。

我们的老板dies are very sensitive to errors in metabolism. The body must have amino acids and many types of proteins to perform all of its functions. For example, the brain needs calcium, potassium, and sodium to generate electrical impulses, and lipids (fats and oils) to maintain a healthy nervous system.

Metabolic disorders can take many forms. This includes:

  • 一种缺失的酶或维生素，这是一个重要的化学反应所必需的
  • 妨碍代谢过程的异常化学反应
  • a disease in the liver, pancreas, endocrine glands, or other organs involved in metabolism
  • nutritional deficiencies

如果某些器官 - 例如，胰腺或肝脏 - 例如，您可以开发代谢障碍 - 停止正常运作。这些类型的疾病可能是遗传学的结果，某种激素或酶的缺乏，消耗过多的某些食物，或许多其他因素。

There are hundreds of genetic metabolic disorders caused by mutations of single genes. These mutations can be passed down through generations of families. According to the 国家健康研究院（NIH） ，某些种族或族裔群体更容易通过特定的天生疾病的突变基因。其中最常见的是：

Diabetes is the most common metabolic disease. There are two types of diabetes:

  • 类型1, the cause of which is unknown, although there can be a genetic factor.
  • 类型2., which can be acquired, or potentially caused by genetic factors as well.

根据这一点American Diabetes Association，3030万儿童和成人，或约9.4％的美国人口患有糖尿病。

In1型糖尿病，T细胞攻击和杀死胰腺中的β细胞，产生胰岛素的细胞。随着时间的推移，缺乏胰岛素可能导致：

  • 神经和肾脏损伤
  • eyesight impairment
  • increased risk of heart and vascular disease

Hundreds of inborn errors in metabolism (IEM) have been identified, and most are extremely rare. However, it’s estimated that IEM collectively affects 1 in every 1,000 infants. Many of these disorders can only be treated by limiting dietary intake of the substance or substances the body cannot process.

The more common types of nutritional and metabolic disorders include:

Gaucher的疾病

This conditioncauses an inability to break down a particular kind of fat, which accumulates in the liver, spleen, and bone marrow. This inability can result in pain, bone damage, and even death. It’s treated with enzyme replacement therapy.

Glucose galactose malabsorption

This is a defect in the transport of glucose and galactose across the stomach lining which leads to severe diarrhea and dehydration. Symptoms are controlled by removing lactose, sucrose, and glucose from the diet.

遗传性血液转换症

Inthis condition，多余的铁沉积在几个器官中，并可能导致：

  • 肝硬化
  • 肝癌
  • diabetes
  • 心脏病

It’s treated by removing blood from the body (phlebotomy) on a regular basis.

枫糖浆尿脓（MSUD）

MSUD.破坏某些氨基酸的代谢，导致神经元的快速变性。如果未治疗，它会在出生后的前几个月内导致死亡。治疗涉及限制支链氨基酸的膳食摄入量。

Phenylketonuria (PKU)

PKU.导致无法生产酶，苯丙氨酸羟化酶，导致器官损伤，精神发育迟滞和不寻常的姿势。它通过限制某些形式的蛋白质的膳食摄入来治疗。

代谢紊乱是高度复杂和罕见的。即便如此，他们也是正在进行的研究的主题，也是帮助科学家更好地了解更常见问题的潜在原因，例如乳糖，蔗糖和葡萄糖不耐受，以及某些蛋白质的过多。

If you have a metabolic disorder, you can work with your doctor to find a treatment plan that’s right for you.