introduced Shanghai Hongqiao Hospital pediatrician, pediatric liver degeneration occurred in the 9 to 25 years of age, as early as 3 years old to 50 years of age or late onset, initial symptoms in the younger age group of symptoms common to the liver, in the older age group was more common neurological symptoms, a few cases with psychotic symptoms, acute hemolysis, bone changes, kidney damage, muscle pain, skin pigmentation as the first symptoms. pediatric liver degeneration is a types of hereditary diseases of copper metabolism. which is characterized by copper deposition in liver, brain, kidney, cornea and other tissues, causing a series of clinical symptoms.
pediatric liver degeneration symptoms (a)
1. eye symptoms, corneal pigmented ring (KF ring), Department of copper deposition in Descemet result, golden brown, brown-green, gray or golden brown, early before they can require the use of slit lamp found that corneal KF ring is an important basis for diagnosis of the disease.
pediatric liver degeneration symptoms (b)
2. kidney symptoms, and renal proximal tubular ball involved, there may be tubular reabsorption barrier of renal glucosuria, amino acids in urine, proteinuria, hematuria, or Fanconi syndrome can occur.
pediatric liver degeneration symptoms (c)
3. psychotic symptoms, mainly apathy, depression, high intensity cry laughing, movement and behavioral disorders, few patients have delusions, hallucinations and personality changes, when in psychiatric symptoms or psychiatric symptoms as the first significant time and easy misdiagnosed as other mental illness.
pediatric liver degeneration symptoms (d)
4. neurological symptoms, mainly extrapyramidal symptoms, common manifestations of lack of coordination, tremors , dance, athetosic, dystonia, language vague, talks slowly, dysphagia, drooling, gait abnormalities, ataxia, etc., there may be involvement of the cerebral cortex or hypothalamus of symptoms, such as pyramidal tract signs, seizures , obesity, high blood pressure.
pediatric liver degeneration symptoms (e)
5. liver damage of all ages are visible, but the smaller age of onset, the liver damage the greater the possibility of common symptoms are fatigue, anorexia, fever, etc.; can gradually occur after liver pain, hepatomegaly, jaundice, splenomegaly, liver cirrhosis, etc., these performance easily with other liver diseases (eg hepatitis) confusion .
pediatric liver degeneration symptoms (VI)
6. other symptoms, a few patients there may be blood system, skeletal system and skin, etc. The involvement, acute hemolytic , bleeding, osteoporosis, bone (cartilage) degeneration, joint deformities.