Nesidioblastosis
Encyclopedia
Nesidioblastosis is beta cell hyperplasia, consisting of the proliferation of islet cells from pancreatic ducts. It is a cause of hypoglycemia and hyperinsulinemia in a number of infants and newborns. Recently, this condition has been recognized in adolescents and adults and occurs in 5% of patients with hyperinsulinism. Prolonged hypoglycemia leads to mental retardation in most of these children. Near-total (95%-98%) pancreatectomy appears to offer the best results.
The problem with the term ‘nesidioblastosis’ is that nesidioblastosis is a normal phase of fetal islet development rather than a pathologic entity.
Nesidioblastosis is a controversial medical term for hyperinsulinemic hypoglycemia
attributed to excessive function of pancreatic
beta cell
s with an abnormal microscopic
appearance. The term was coined in the first half of the 20th century. The abnormal histologic
aspects of the tissue included the presence of islet cell enlargement, islet cell dysplasia
, beta cells budding from ductal epithelium, and islets in apposition to ducts.
By the 1970s, nesidioblastosis was primarily used to describe the pancreatic dysfunction associated with persistent congenital hyperinsulinism
and in most cases from the 1970s until the 1980s, it was used as a synonym for what is now referred to as congenital hyperinsulinism. Most congenital hyperinsulinism is caused by different mechanisms than excessive proliferation of beta cells in a fetal pattern and the term fell into disfavor after it was recognized in the late 1980s that the characteristic tissue features were sometimes seen in pancreatic tissue from normal infants and even adults, and is not consistently associated with hyperinsulinemic hypoglycemia.
However, the term has been resurrected in recent years to describe a form of acquired hyperinsulinism with beta cell hyperplasia found in adults, especially after gastrointestinal surgery.
The problem with the term ‘nesidioblastosis’ is that nesidioblastosis is a normal phase of fetal islet development rather than a pathologic entity.
Nesidioblastosis is a controversial medical term for hyperinsulinemic hypoglycemia
Hyperinsulinemic hypoglycemia
Hyperinsulinemic hypoglycemia describes the condition and effects of low blood glucose caused by excessive insulin. Hypoglycemia due to excess insulin is the most common type of serious hypoglycemia. It can be due to endogenous or injected insulin.-Causes:...
attributed to excessive function of pancreatic
Pancreas
The pancreas is a gland organ in the digestive and endocrine system of vertebrates. It is both an endocrine gland producing several important hormones, including insulin, glucagon, and somatostatin, as well as a digestive organ, secreting pancreatic juice containing digestive enzymes that assist...
beta cell
Beta cell
Beta cells are a type of cell in the pancreas located in the so-called islets of Langerhans. They make up 65-80% of the cells in the islets.-Function:...
s with an abnormal microscopic
Microscope
A microscope is an instrument used to see objects that are too small for the naked eye. The science of investigating small objects using such an instrument is called microscopy...
appearance. The term was coined in the first half of the 20th century. The abnormal histologic
Histology
Histology is the study of the microscopic anatomy of cells and tissues of plants and animals. It is performed by examining cells and tissues commonly by sectioning and staining; followed by examination under a light microscope or electron microscope...
aspects of the tissue included the presence of islet cell enlargement, islet cell dysplasia
Dysplasia
Dysplasia , is a term used in pathology to refer to an abnormality of development. This generally consists of an expansion of immature cells, with a corresponding decrease in the number and location of mature cells. Dysplasia is often indicative of an early neoplastic process...
, beta cells budding from ductal epithelium, and islets in apposition to ducts.
By the 1970s, nesidioblastosis was primarily used to describe the pancreatic dysfunction associated with persistent congenital hyperinsulinism
Congenital hyperinsulinism
Congenital hyperinsulinism is a medical term referring to a variety of congenital disorders in which hypoglycemia is caused by excessive insulin secretion. Congenital forms of hyperinsulinemic hypoglycemia can be transient or persistent, mild or severe. These conditions are present at birth and...
and in most cases from the 1970s until the 1980s, it was used as a synonym for what is now referred to as congenital hyperinsulinism. Most congenital hyperinsulinism is caused by different mechanisms than excessive proliferation of beta cells in a fetal pattern and the term fell into disfavor after it was recognized in the late 1980s that the characteristic tissue features were sometimes seen in pancreatic tissue from normal infants and even adults, and is not consistently associated with hyperinsulinemic hypoglycemia.
However, the term has been resurrected in recent years to describe a form of acquired hyperinsulinism with beta cell hyperplasia found in adults, especially after gastrointestinal surgery.