How to treat infantile hemangioma syndrome?

summary

Angiokeratoma syndrome is also called Fabry syndrome, Anderson Fabry syndrome, sweeley klionsky syndrome, ruiter pompen syndrome, diffuse systemic angiokeratoma glycolipid deposition, hemorrhagic sarcoidosis and so on. Let's share my experience with you.

How to treat infantile hemangioma syndrome?

There is no root therapy for this symptom. Phenytoin sodium can be used to relieve joint pain and peripheral neuralgia in children over 10 years old. At present, enzyme supplement therapy is used, but the effect is short. Because the highly purified ceramide trihexoside cleaving enzyme in normal human urine or placenta can reduce the concentration of brain aminohexoside in blood, it has been reported that the injection of normal human plasma or white blood cells has certain curative effect.

Dexamethasone can help relieve acute symptoms. In some cases, not only azotemia but also other symptoms, such as paresthesia and skin damage, were relieved after renal transplantation.

This is a sex linked hereditary disease. Due to ceramide triohexose- α- The deficiency of galactosidase results in the deposition of ceramide trihexoside in various tissues of the body, mainly in vascular endothelium and kidney.

matters needing attention

Urine routine early proteinuria, containing glycolipid vacuole cells, later tubular hematuria, isotonic urine. In the late stage, there are anemia, hyperazotemia, and 40 cases in bone marrow μ M-sized cells with many vacuoles are similar to Niemann pick cells in shape. Children without skin rash should be differentiated from collagen diseases such as rheumatic fever.