The performance of nerve fiber lump

Belong to chromosome to often show sex heredity disease and there are 2.1 namely and around type the nerve fiber lump disease, as well call the disease of Von Reckling-hausen;2s are more rare, is axis type the nerve fiber lump disease, its characteristic listens to nerve lump for the double side.
A pathologic gene is located on No.17th chromosome, 2 pathologic genes are located on No.22th chromosome.In a case, the tumor of nerve fiber egg white(neurofibromin) represses a function to appear an obstacle or loses.
In a case, have 13 have no clinical symptom but is in the normal regulations check-up disheveled hair now;The lord of 13 cases' telling is the problem of hairdressing;13 cases have a nerve section problem.
The characteristic skin pathological changes is coffee milk dye spot,(cafe-au-laitmacules) , most often distribute in the torso, the pelvis takes and the bending of elbow and knee muscle wrinkly plait, in the case of above 90% while being born or the infant expect to be obvious already it is thus clear that.Expect after child, can appear much the skin tumor of hair, present nude, the size is different, the shape is each difference;Littlely only many several of can have up to thousand.Can have the skin the bottom knot stanza rarely or skin the bottom organizes formless excessively grow(result in anomaly, increase the thick, transform of the structure-cluster form nerve lump), or it underneath of bone substance of have no fixed form excessively growth, produce the abnormal of strange kind.The abnormal of skeleton includes the bone fiber structure bad disease, under the periosteum of the bone bag is swollen, fan-shaped vertebrate, the spinal column side is convex, the imperfection companion of false joint formation and the Die bone big wing(namely Houpi of the eye socket) after hair of pulsate sex Tu eye.
Along the thoroughfare of surroundings nerve in skin can touch several nerve fibers lump, the latter from Shi Wan's cells and nerve of become the fiber cell constitute, seldom in puberty ex- appear.The nerve fiber lump can grow on the nerve root, can pass a Zhui the bore take care of in the Zhui inside take care of with Zhui to all outside have a growth, form typical"dumbbell-like in shape" tumor.The tumor growth in the Zhui tube can oppress spinal cord.The cluster-like in shape nerve lump can involve in surroundings nerve and cause the function obstacle of pathological changes distant place.The tumor of Lu nerve includes to cause to carry on sex to become blind of see the nerve gum quality lump, and can cause to faint, totally help maladjustment, the listenning to of deaf and buzzing in the ears nerve lump(ex- Shi Wan cell lump in the court).The disease usually makes progress to aggravate.
In 2 cases, the occurrence double side listens to nerve lump, about at patient about 20 years old appear clinical symptom.There can be gum quality in the household member the lump or meninges lump disease of history, some members have a teenager the type the cataract.