Hers Disease: Glycogen Storage Disease

Diabetes mellitus is the only existing disease most commonly known to people as its frequency is reaching its astronomical limits. Liver indeed has still many disorders that are yet to be discovered by human race. As the scientific knowledge of man kind increases new creed and kind of diseases emerge spontaneously as if to throw a new challenge for human brain to solve another new and complex puzzle. “Hers Disease” as it is called, after the famous scientists Henri-Géry Hers, who discovered it in 1959, is a by product of liver malfunction and is some what similar to diabetes.

Definition:-

Hers disease is known by various names in scientific community these are,

1) Liver Phosphorylase Deficiency

2) Hers' syndrome

3) Cori’s type VI glycogenosis

Hers disease is caused by the deficiency of an enzyme called liver phosporylase, which is vital for proper break down of glycogen. In other words it is a glycogen metabolic disorder.

When dealing with Liver Phosphorylase Deficiency or"Hers Disease" we should have a clear tailored vision of the nature of the disease, which can be generalized as,

1) Glycogen metabolic disorder

2) Autosomal recessive in nature “X” linked

3) Caused by mutation

1)     Hepatomegaly:- Hers disease is characterized by enlargement of liver, this may be due to abnormal acumination of glycogen in liver due to improper metabolism .

2)     Mild hypoglycemia: - Also termed as “sugar shock” is caused by hypo secretion of insulin and hyper secretion of glucagons in the islets of langerhans. Since the glycogen is not converted into glucose the patient tends to become weak and if the condition persists goes into deep shock and many a times into critical coma.

3)     Ketosis Ketone bodies and fatty acid accumulate in the liver due to metabolic disorder of glycogen, causing much damage to liver cells and increase the chances of renal malfunctions.

4)     Low growth: - Mild retardation of growth and some times symptoms like dwarfism is seen, many cases of hers disease confirm conditions similar to Harris syndrome.

The onset of the disease can start in the early childhood and can well go undetected. The children may lead a normal life only to realize later during adulthood that they are suffering from Liver Phosphorylase Deficiency or"Hers Disease".Male and female are equally affected by this disease and can occur in any person of any age group or race.

Since the disease is autosomal recessive in nature and is “X” linked the frequency of the diseases is low in the human race. However, Males have a greater chance of acquiring the disease when compared to females as the male patient will require single recessive mutated X^ gene on the chromosome which will be X^Y and female patent will require both the X chromosome to be infected by the recessive gene X^ which will look like X^X^.

There is however no permanent cure for the disease although artificial injected insulin may help from time to time to breakdown the acuminated glycogen into glucose. Genetic Engineering may have the answer to this disease in future, hypothetically if the research in the genetic field becomes successful then it will be possible to detect and slice out the mutated autosomal recessive gene from the human chromosome, thus eradicating not only Liver Phosphorylase Deficiency or"Hers Disease" but also other forms of diseases like diabetes mellitus and even cancer                                         

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