2010 ? 08 ? 15 ?

Xianxie to tell the next

best answers no other mystery 1:

fibrosis, neurofibromatosis, also known as molluscum, rubber like disease neuroma, is a hereditary systemic neuroectodermal strange disease, is autosomal dominant, but penetrance vary, about 25% ~ 50% of the patients had positive history of relatives. The properties of the pigmented skin disease, and neurofibromatosis.

60% of the patients mental decline, the rest were normal. Multiple neurofibromas in the male patients, mostly in puberty onset. also known as skin color, skin pigment spots coffee spots, often at birth that is, a small amount appeared not long after birth, often multiple, prevalent in the face and trunk, irregularly scattered, light brown to dark brown, size and shape may vary from diagnosis value of neurofibromatosis usually issued after the coffee spot, occurs in adolescence, the primary fat in the trunk, from soybeans to Putao or pigeon size, touch the soft skin of the hemispherical shape of a convex or a pedicle tumor, the tumor can fingertips pressed into the top of the skin, when the pressure is removed, restitution. The patients, about 5% to 10% of dental damage, in the palate, buccal mucosa, tongue and lips appeared papillomas or unilateral macroglossia. 30% ~ 46% of patients had neurological symptoms, can cause epilepsy and other diseases.

neurofibromatosis is a rare genetic disease is characterized by skin pigmentation spots skin color and neurofibromatosis. About 25-50% of patients had positive history of relatives. Patients can not long after birth the skin pigmentation spots appears, showing the milk brown, gradually increased or expanded. Sometimes the lesions appear late in the disease development period began, physiological changes, such as development, pregnancy, menopause, infectious disease, mental stimulation and so can aggravate the disease. slow course of the disease, but malignant transformation may occur 20-50 years of age. The disease is seen all over the world, no gender, age and ethnic differences. Generally do not need therapy, seriously affect the beauty and functionality unless there is malignancy or when, before considering surgery.

[symptoms of physical disease]

1. Leather skin color pigment spots: almost all patients have skin the color of skin pigment spots, pale brown to dark brown, or brown (milk coffee spot), the number of size ranges, axillary freckle-like pigmentation can;

2. Multiple skin nodules: a few to several dozens or even hundreds, the first distribution in the trunk, often convex hemispherical or conical skin was like, Princess, or purple color, size range, such as hernia-like touch of the soft;

3. Estuary damage control: performance of papilloma or unilateral macroglossia ;

4. visceral injury: the brain tumors, vascular malformations, or skeletal deformities, can cause mental deterioration, seizures and other neurological symptoms. Tumor invasion of bone, can cause scoliosis, grams of channel involvement can cause gastrointestinal bleeding or obstruction, may also cause endocrine strange

[diagnosis is based on]

1. Milk coffee spot across 6 (each with a diameter> 1.5cm) and / or axillary freckles appear;

2. multiple skin nodules, soft texture, such as hernia-like;

3. Oral damage and damage to internal organs;

4. pathology: tumor boundaries clear, but no capsule, the nerve sheath cells and nerve membrane fibers, pudding staining.

[treatment principles]

1. Symptomatic treatment: such as epilepsy, analgesics;

2. Surgical treatment: lesions severely hampered for beauty, little impact on function or cancer, or malignant pain and doubt and so on. Need to be held neurosurgery, the removal of pain disease.

[Clinical evaluation]

1. Cure: surgery, the tumor was excised, beauty on the right about, or pressure or epilepsy, organ function dissipated;

2. improvement: After surgery, beauty on the part of the right about, the partial restoration of organ function under pressure, seizures, symptoms improved significantly.

3. Unhealed: surgery and antiepileptic treatment were ineffective.

the disease is genetic disease, 50% of their children may be the disease, it should consider sterilization. at the same time should focus on self-monitoring. such as the invention of mass short-term increases rapidly, there may be malignant. Real-time conditions to go to hospital for treatment. when severe complications such as intracranial tumor, gastrointestinal bleeding and intestinal obstruction caused by involvement or retroperitoneal neurofibroma causing great stress and other organs are needed surgery, and doctors must be mutual.

intelligence in children with neurofibromatosis affect you?

neurofibromatosis is the nervous system of multiple tumors, skin and other organs of coffee milk spots characterized by neural tumors skin syndrome.

Neurofibromatosis is a chronic disease held, followed by aging symptoms gradually increased. Invention of the skin at birth can have some bumps on the skin surface is not, coffee milk, color, circumscribed, pigmented spots of varying sizes. More common on the trunk or limbs, rarely seen in the face. Followed by age, coffee, milk spots gradually increased. Nerve fibers also appeared in the skin tumor, of varying sizes, ranging from the number of how many, often not accompanied by pain, more common on the trunk, limbs and scalp. Since tumor growth in different parts of the nerve fibers, the symptoms can be diverse, often solitary in pediatric intracranial tumors. part of the sick child can be expressed as seizures, mental retardation, language and motor retardation. also may have other system involvement.

Benbingfasheng early embryonic development may be related to changes appear strange, not easy to prevent. So when children have more skin, coffee milk spots, specific physical examination should be held to the hospital and neurological examination, when required to do X-ray examination, while focusing on whether the other system involvement.

primary treatment of this disease is held for the treatment of symptoms. when the central nervous system and peripheral nervous system tumors have symptoms, surgical resection.

what neurofibromatosis clinical manifestations, and its operation principle is what?

nerve fibers from nerve sheath tumor cells and fibroblasts constitute the primary components of the benign tumor, sub-single- hair and multiple two. Maxillofacial facial nerve fibers from the facial nerve and the tumor often trigeminal nerve, slow-growing, common in young people. Yan facial neurofibroma surface skin brown spot sizes, or gray color was xuan dot-like or flaky lesions. Palpation, the skin there are multiple tumor nodules, hard, nerve distribution along the skin, showing the number of beads or plexiform. from sensory nerve tumor, may have significant tenderness. Tumor in the nerve distribution in the region, sometimes abnormal proliferation of cartilage, skin loose or folded droop, cover the eye, and facial deformity occurring functional stagnation. The tumor is soft, rich intratumoral blood supply, generally less than her inability to compression. Invading into adjacent bone, can cause deformities. Neurofibromatosis first face may be associated with the nature of the occipital bone defect first. Neurofibromatosis a genetic tendency for autosomal dominant inheritance, its diagnosis, as long as the brown or brown skin patches more than 1.5cm, 5 to 6 above can be diagnosed with polyneuropathy fibroma.

neurofibromatosis general surgical excision. Small and limited to a resection of neurofibromas; huge part of the tumor can be removed to correct deformity, improve function of stagnation. Because tumors are often associated with skin and basal adhesion, and very rich blood supply, with sinusoids, more bleeding during surgery, and tumor crisp texture, with the general approach is not easy to stop the bleeding, it should be fully prepared before surgery blood, and select the hypothermic anesthesia . Tumor resection surgery sharp surrounding tissue, this method faster than the blunt dissection and bleeding less. Application of electric knife surgery bilateral carotid artery ligation, and the application of transcatheter arterial embolization (TAE) will also help stop the bleeding.