Primary IGF-1 Deficiency

Primary IGF-1 Deficiency General Information  

Primary IGF-1 Deficiency is considered a condition that develops from a lack of a growth factor, like insulin, that can also be known as somatomedin C. This kind of growth factor is a polypeptide protein hormone that can be similar to the molecular structure of insulin. This hormone is responsible for childhood growth and can have anabolic effects on adult patients. IGF-1 factor contains 79 amino acids that form a single chain with other three intramolecular disulfide bridges. Generally, it has a molecular weight of 7649 daltons. The hormone is usually secreted by the liver, as an endocrine hormone, but it can be produced by the target tissue as a paracrine or autocrine factor. In most cases, the secretion is stimulated by the patient’s growth hormone, and an imbalance of one of these hormones can cause retardation by undernutrition, growth hormone sensibility, lack of growth hormone receptors, or certain failures of the patient’s downstream. Usually, although young patients have enough growth hormone in their organism, they present a small level of IGF-1 which can lead to a condition called Primary IGF-1 Deficiency. This kind of disease can be diagnosed depending on the patient’s difference of height from other normal, healthy persons. If the disease called Primary IGF-1 Deficiency is not treated properly or is not treated at all, it can cause serious complications such as extremely short stature and an increased risk of developing other metabolic disorders such as lipid abnormalities, decreased bone density, insulin resistance and even obesity.

Primary IGF-1 Deficiency Symptoms  

There are many causes that can determine a child to have a short stature, such as genetic disorders, constitutional growth delay or poor nutrition, but there are cases in which the patient recovers from a growth experience several years later than he should normally do. There are also cases in which the short stature can be caused by certain conditions such as chromosomal defects, kidney disease, lung disorders, heart malfunction, or gastrointestinal disease. Usually, suffering from a pituitary dysfunction that can cause the levels of growth hormone to decrease can lead to growth failure. The low levels of growth hormone can cause the levels of IGF-1 factor to decrease leading to the appearance of a condition called Primary IGF-1 Deficiency. This kind of factor is usually produced by the patient’s liver and can also have growth effects. There are cases in which the patient can present short stature despite the fact the he has normal of higher levels of growth hormone, and in these cases the patient can be diagnosed with a condition called idiopathic short stature (ISS), a condition with an unknown cause. In most cases, patients who suffer from Primary IGF-1 Deficiency may not experience any signs and symptoms until the age of six years old, when the growth retardation becomes obvious. If Primary IGF-1 Deficiency is not treated properly in time or not treated at all the patient can develop certain complications such as secondary tooth eruption or delayed puberty which can affect the patient’s social life.

Primary IGF-1 Deficiency Treatment

The diagnosis of the Primary IGF-1 Deficiency is usually made by identifying the growth problems or short stature of the patient by ruling out any other causes or diseases that can lead to poor growth. It can also be done by eliminating the possibility of a lack of growth hormone by measuring the levels of IGF-1 factor in the patient’s blood. As soon as the physician sets a final diagnosis, he or she will seek for an appropriate therapy option. In most cases, the disease can be classified in certain categories such as moderate to severe forms, depending on the decreased level of IGF-1 factor according to the normal levels of it in a normal, same age, person. In the past, the disease presented no available treatment, and was left to take its own course. Nowadays, scientists have found a way to effectively improve the patient’s condition and health status by applying a treatment trial with hormone-like medication.

This treatment is generally administered to young patients but it can have same effects in adult patients. It is very important for the doctors to test firstly the patient with certain trials before applying the main hormone treatment. Usually, the most common medicine that can be a part of the treatment for Primary IGF-1 Deficiency in Increlex or Mecasermin, which is an injectable substance and is the first product that can effectively replace the missing IGF-1 factors in the patient’s organism, stimulating the growth. This kind of medicine is usually used for long term treatments for growth dysfunction in patients who suffer from the severe form of Primary IGF-1 Deficiency or who are suffering from growth hormone gene deletion. If a patient is suffering from a growth hormone gene deletion, he can develop neutralizing antibodies to this hormone. If the treatment with the Increlex is interrupted, the patient can develop certain complications that can include intracranial hypertension or hypoglycemia, which if they are not treated properly, can worsen the condition.

The medicine is a synthetic insulin factor that is similar to the IGF-1 factor that the patient’s body should normally produce. This kind of factor plays an important role in the growth of the patient’s bones and muscles. It is very important for the patient to know how to inject the medicine; it is better for him to ask a doctor or a pharmacist how to use the drug, especially if the prescription does not contain additional instructions that show the patient how to inject it. In treating the Primary IGF-1 Deficiency, Increlex is generally administered 20 minutes before or after each meal. If the patient develops over dosage, he can experience symptoms such as tremor, cold sweats, increased hunger, headache, drowsiness, restlessness, irritability, weakness, trouble concentration, dizziness, increased heart beat, and nausea, all signs of low levels of sugar in the patient’s blood. Another medicine used to treat Primary IGF-1 Deficiency is Iplex, which a synthetic growth factor. It is very important to administrate the medicines properly, because if they are not used correctly, the patient can develop serious side effects that can worsen the general heath status.