Hepatolenticular degeneration?

summary

Hepatolenticular degeneration (HLD) is an inherited disorder of copper metabolism characterized by various abnormalities of copper deposition in the lenticular nucleus, liver, kidney and cornea. The clinical manifestations were progressive extrapyramidal symptoms, liver cirrhosis, mental symptoms, renal function damage and corneal pigment ring. Hepatolenticular degeneration is rare in China. Hepatolenticular degeneration? Let's talk about it

Hepatolenticular degeneration?

The first is in the kidney. The degree of renal function damage is different. In general, there will be renal diabetes, a variety of amino acid urine, phosphate urine, uric acid urine, hypercalciuria, proteinuria and other diseases. In general, penicillamine can be used to improve renal function, but there may be some side effects, such as osteoporosis, bone and cartilage degeneration.

Hepatolenticular degeneration can also be manifested as liver. General patients will begin to appear fatigue, anorexia, jaundice, spider nevus and other symptoms, but often end up with portal hypertension, ascites, variceal bleeding and liver failure. A few patients showed asymptomatic hepatomegaly, splenomegaly, or continuous elevation of transaminase without any symptoms.

Hepatolenticular degeneration may also have other manifestations, such as cardiac arrhythmia, cardiomyopathy and autonomic nerve dysfunction; Most patients had skin pigmentation; The pancreas was damaged; The nail arc was blue and the copper content increased; Young women have amenorrhea, male growth retardation, breast development and other manifestations.

matters needing attention

In our normal diet, do not eat food with high copper content, do not use copper tableware, and do not eat food that excites the nervous system. Eat more calcium rich food. Regular physical examination.