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Cases of tuberous sclerosis cases the reasons outlined in the diagnosis and clinical diagnostic laboratory tests do not treat
Tuberous sclerosis (tuberono sclerosis), also known as Bournervill disease, as a stain of fault constitutive Liu Bing (phakomatosis) showed addition of peripheral nerve, musculoskeletal system, many organs outside the hamartoma (hamartorm) Comprehensive gen


etic Zheng, showing autosomal dominant inheritance, disease gene discovery and chromosome 9 linkage of ABO blood group epitopes, but also that in chromosome 16. Incidence rate of about 1 / 2 million to 1 / 4 million. The sebaceous gland tumor, mental retardation and epilepsy as the three main performance. 50% of patients with renal angiomyolipoma, renal cysts, the incidence of 30%. In addition to clinical manifestations of the skin, central nervous system and other organ involvement (such as heart, lungs, blood vessels, etc.) the performance, the renal manifestations were: back pain, hematuria, retroperitoneal hemorrhage, hypertension and renal failure, about 25% of patients of renal malignancy. 50% of life in patients <20 years old, of which the cause of death accounted for a considerable part of the kidney, including kidney tumor metastasis, retroperitoneal hemorrhage, renal failure and so on. Treatment is symptomatic treatment.
Tuberous sclerosis is characterized by seizures, mental retardation, and sebaceous gland tumors, the incidence rate is about 1:100,00 Symptoms: Seizures are a prominent symptom of this disease, more than 2 years of age at onset, and often infantile spasms seizures, myoclonus, seizures or inability to type of attack, attack with age, gradually transformed into the form of limited or grand mal seizures Board. Sometimes seizures can be natural to stop for some time, but shortly after that attack, anti-epileptic drugs more difficult to control. About 60% lower than the normal children sick child intelligence, degree of mental retardation can be mild to heavy, the performance became clear with age. Children with mental illness can also change the behavior if there is no reason why, negativism and so on. Prominent sebaceous tumors based disease symptoms, the majority of sick children aged 2 to 6 appear gradually increased, adolescent obvious. Sebaceous gland tumor in the vicinity of the nasolabial fold up to the cheek, forehead, chin and other parts of extension, and occasionally seen in the eyelids. Reddish brown skin color or the same size from the needle to the size of a pea, not hurt. The organizational structure of sebaceous tumors is neither a "adenoma", not "sebaceous", but by the excessive proliferation of connective tissue formed. Many sick children can be seen in the infant skin depigmentation spots, oval or spindle-shaped, 1 ~ 3cm size. Color gray, more common in the abdomen, back and lower limbs. Some patients may also see a shark skin, skin plaques, a little bulge in the skin, touch the rough, and skin with the same or slightly deeper.Poor prognosis of this disease. Generally symptomatic treatment, control of seizures with antiepileptic drugs, refractory cases of stubborn, the cortex has a limited current graph proved lesions Board, may consider surgical removal of lesions, cerebrospinal fluid circulation disorders caused by brain tumor, the possible surgery.
What are the performance of tuberous sclerosis and how to diagnose?
The disease often violated because of many organs and tissues, and almost any organ or tissue can be involved, so the clinical manifestations of the lesion due to the different and complex and varied, but the seizures, facial sebaceous gland tumor and the most common mental disorders, there disease patients may be one of only three, there are completely asymptomatic and pathological examination in the finder.
1. Dermatological symptoms the most common skin lesions, often diagnosed as the main basis for about 90% of patients had sebaceous gland tumor, usually found in the 2 to 5 years old, located in the cheeks and lower jaw, forehead, eyelids, nose are visible , symmetric distribution, the light red or reddish-brown hard waxy papules, may fade by the size of the tip to the beans can be large, sebaceous gland tumor has existed at birth, occasionally, to the rapid growth of puberty due to more pronounced, 85% of the patients depigmentation spots can be seen, for leaf shape, oval or irregular-shaped white, trunk and upper and lower extremities may occur, see more obvious in the UV, 20% of patients can see a green granular skin spot, common in waist and lower back skin, the local thickening of the rough, slightly higher than the skin, is grayish brown in diameter from a few millimeters to 5 ~ 6cm, (toe) Subungual fibroma occurred in adolescence, grow from a ditch, and sometimes The only skin lesions based disease, in addition, milk, coffee, pigment spots, skin tumors and other fibers can be seen.
2. Neurological symptoms and seizures and mental retardation are characteristic of this disease, seizures early in the disease, skin lesions or intracranial calcification had emerged a few years before, epilepsy, seizures can be expressed in any form, at first may appear to be baby spasms, later transformed into generalized seizures or partial seizures, some patients may only seizures and no other clinical manifestations, 60% to 70% of patients with varying degrees of mental deterioration, often appears 2 to 3 years old, even earlier, almost all of those who have mental retardation seizures, normal intelligence is about 70% of seizures, epilepsy are more likely to occur as early as age, intelligence loss, showed only a very small number of patients without seizures decreased intelligence, there showed personality and behavior disorders, emotional disorders and mental abnormalities, even there is paralysis of limbs, ataxia, involuntary movements and other symptoms, a small number of patients due to subependymal nodules occurred obstruction of cerebrospinal fluid circulation pathway hydrocephalus and cranial high-pressure performance.
3. Other manifestations of the disease is often associated with tumors of other organs such as bone cancer, cystic fibrosis, lung cancer, cardiac rhabdomyoma, fibroma or papilloma mouth, etc.; retinal crystals tumor characteristic is one of the manifestations of this disease , usually located in the posterior pole of the eye, which was slightly yellow and white or greyish yellow flash, round or oval, the surface is slightly irregular bulge, gear-shaped edges, the size of half to two times the optic disc and increased with age large and growing trend, lens tumors usually do not cause symptoms, only occasionally lead to blindness, the other still shows a small eye, exophthalmos, glaucoma, lens opacities, cataracts, vitreous hemorrhage, retinitis pigmentosa, retinal hemorrhage and primary optic nerve atrophy and other ocular manifestations, in addition to increased intracranial pressure can occur because of optic disc edema and secondary optic atrophy.
According to family history of autosomal dominant inheritance, typical of sebaceous gland tumors, more than 3 depigmentation spots, seizures, including infantile spasms, smart decline to do clinical diagnosis, skull X-ray at 50% to 70% of patients can be found intracranial calcification in the parietal region, which is due to calcium in the subependymal nodules due to the deposition, usually absent at birth, to childhood began to emerge, such as the CT examination revealed intracranial calcifications and subependymal nodules can be diagnosed with kidney or other organs if the tumor, EEG examination often peak rhythm disturbance, but also help diagnosis.


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