What is the longest disease name in the world?

Congenital interstitial cell hyperplasia of Cajal with abnormal development of intestinal neurons is just two more words, which is the longest known disease name in medical field.

Interstitial cells (ICC) of Cajal are gastrointestinal pacing cells, which are located between nerve plexuses that regulate intestinal muscle activity. Only ICC expresses c-kit in gastrointestinal tract, so ICC can be identified by immunohistochemical method. The function of ICC is closely related to its anatomical distribution. ICC(IC-MY) and ICC(IC-SM (IC-SM) distributed between the stomach and the longitudinal muscle of small intestine are pacing cells, which can generate and spread slow waves.

ICC disperses in the circular and longitudinal muscles, or ICC-IM gathers in some areas of the circular muscles, which has been proved to mediate enteric nervous system's signal transmission. If the interstitial cells of Cajal between submucosa and neuromuscular plexus proliferate abnormally, their functional nerve cells may be overactive, which can stimulate the nerve plexus to cause the proliferation of ganglion cells, leading to the increase of giant ganglion and acetylcholine ester activity, which is manifested as the symptoms of abnormal development of intestinal neurons.

Check:

1, X-ray examination, abdominal plain film and barium enema X-ray examination can understand the situation of intestinal obstruction.

2, intestinal transmission function test, gastrointestinal specific markers can be used to observe the transit time of gastrointestinal tract.

3. Pathological diagnosis was made by histopathological examination, gastrointestinal biopsy and immunohistochemical staining.

According to the gastrointestinal symptoms such as difficulty in defecation, constipation or diarrhea, abdominal distension and abdominal pain after birth, combined with intestinal transmission function examination, endoscopic examination and histopathological examination, the diagnosis can be made clearly.

If the patient has gastrointestinal symptoms such as abdominal distension and constipation, drugs or a small amount of enema can be used to relieve discomfort, and surgery is needed if necessary.

Because of the excessive proliferation of interstitial cells of Cajal in gastrointestinal tract, there may be a risk of developing into gastrointestinal stromal tumors, so patients should be followed up regularly, and early detection and treatment should be carried out.

The above content refers to congenital interstitial cell proliferation of Cajal with intestinal neuron dysplasia.