Nesidioblastosis – Definition, Symptoms, Causes, Treatment

What is Nesidioblastosis?

It is a medical term that means the proliferation of pancreatic islet cell. This often occurs only during the normal development of the neonate. Yet, it can also exist pathologically in infants that will lead to both hypoglycemia and hyperinsulinism. The name was conceived by the person named George F. Laidlaw, who is a renowned scientist, which happened upon the year 1938.  He culled it from the Greek words “nesidia” and “blastos” which means islets and germ, respectively. He described it as a neoformation of the islets found in the Langerhans which is located in the pancreatic duct. He first referred this condition to babies who were born with the pancreatic abnormalities particularly involving the beta cell pancreatic, which are known to produce insulin and is located in the islet of Langerhans of the person’s pancreas. It is an inborn fault of the metabolism part. There is a discordance of the blood insulin and glycemia.

A histology of the pancreas showing proliferation of cells (shown in arrows)

Others would describe as a medical term that is controversial for the term called hyperinsulemic hypoglycemia episode. It is ascribed to the excess function of the person’s pancreatic beta cells that has an appearance of abnormal microscopic image. In addition to that, this kind of condition is known to be an inherited autosomal recessive disease condition which has, as experts would describe, a characteristic of abnormal formation of the islets found in the person’s pancreas and always having persistent hyperinsulinemia hypoglycemia of the infant person. Others would famously call it as noninsulinoma pancreatogenous hypoglycemic syndrome, congenital hyperinsulinism, islet cell dysmaturation syndrome and nesidiodysplasia. Yet despite of the fact that it most often it occurs reportedly in infants, the adults can’t be spared by this disease condition as they can be also inflicted with nesidioblastosis.

Nesidioblastosis Symptoms

Nesidioblastosis is a dangerous disease condition that affects the person’s pancreas as well as his or her glucose levels. Those who are inflicted with nesidioblastosis will commonly report the following symptoms that they manifest such as:

• Bloating of the abdominal region
• Flushing
• Diarrhea that is watery in consistency
• Headache
• Shakiness
• Blurring of vision
• Confusion
• Palpitation of the heart
• Macrosomic babies
• Neonatal hypoglycemia that is prolonged

When worse comes to worse, it can also lead to either of the following complications:

• Damage of the brain
• Seizure episodes
• Coma
• Death

Nesidioblastosis Causes

The cause of nesidioblastosis is due to the following factors such as:

• Excess of Insulin release
• Diabetes
• Defect in the pancreas
• Abnormal recognition of the glucose
• Chromosome 11p15.5
• Acute maternal glucose loading

The cause of nesidioblastosis, which is considered to be a serious kind of medical metabolic disease or disorder, which may be linked to the baby or the adult person as having excess in the release of insulin from the person’s islet of Langerhans which is found in the person’s pancreas.  It may also be associated to obesity and type 2 diabetes.

As mentioned earlier, the person who has nesidioblastosis have an abnormal functioning of the defective beta cells of the pancreas. Other researchers believed that there is a presence of abnormal recognition of glucose because of the defect of the beta cells found in the person’s pancreas. In addition to that, there has been a research that tells that this kind of disease condition is due to genes particularly chromosome 11p15.5.  The combination of the genes for insulin like growth factor as well as the insulin itself is another category that leads to the development of hypoglycemia and hyperinsulinism. Also, when there is a presence of acute maternal glucose loading it may lead to the stimulation of insulin secretion of the fetus which will make them at high risk for having neonatal hyperinsulinism hypoglycemia.

Nesidioblastosis Treatment

Nesidioblastosis can be treated by either:

• Medical treatment

It is the first thing that is done when a person is diagnosed to have this kind of disease condition. The aim of the medications is to treat the hyperglycemia and hypoglycemia episodes as well as it is also used for the reduction of the symptoms that are seen in persons with nesidioblastosis. The following are the common medications prescribed by physicians:

• Sandostatin (in either depot IM or Injection)
• Octreotide Acetate ( either in IM or Injection)
• Diazoxide
• Proglycem

• Surgical treatment

There may be a need for resectioning the pancreas which may be surgically done by a certified surgeon. Other surgeon will suggest doing distal pancreatectomy surgical procedure. Still others will suggest performing subtotal pancreatectomy but there is a risk in this procedure as it may lead to future development of diabetes mellitus or diabetes. Surgical procedure is done when the hypoglycemia episodes is not controlled by medications alone.  The surgical procedure done will be different per individual. It will greatly depend on the progression and the affectation of the disease condition as well as the keen eye of the surgeon.

Clinically speaking, the person who has nesidioblastosis is very difficult to diagnose. There is a need to recognize the disease promptly so as aggressive treatment will be given and be able to avoid any possible and irreversible damage of the central nervous system. With regards to any of the treatment provided to the patient, it is far most important that even after treatment one must be able to monitor the glucose level of the person and be able to at least keep it to a normal level so as to prevent the reoccurrence of such disease condition. This kind of keen monitoring requires skills but the person’s loved ones or the person, himself or herself, can be taught how to do it so as to learn independency. Aside from that, it makes him or her control of at least his or her condition. Also, it is necessary that the person needs to watch not only his or her glucose level but also the foods he or she consumes. He or she should be given the right information with regards to his or her disease condition.