How is nodular lipofuscinosis treated?

【Treatment plan and principles】 There is no special treatment yet 1. General treatment: First, the suspected cause should be removed, such as eliminating the foci of infection and discontinuing the suspected disease-causing drugs. Appropriate use of antibiotics to control infection. You can exercise at will, but should avoid trauma to the involved parts. No special diet is needed. 2, drug treatment: (1) non-steroidal anti-inflammatory drugs (NSAIDS): ① Aspirin: mainly used for antipyretic and reduce the production of thromboxane A2. Commonly used dose 300~600mg 4~6 times a day, not more than 4g/day, taken with or after meals. 3~5 days before the maximum anti-inflammatory effect is usually achieved within 2~4 weeks. Contraindicated in patients with liver damage, hypoprothrombinemia, vitamin K deficiency, bleeding disorders and asthma. Use with caution in pregnant women. Indomethacin (anti-inflammatory pain): See the Ankylosing Spondylitis Treatment Guide for details. It can cause headache during the first few days of treatment, but this side effect can sometimes be avoided if the initial dose is halved and subsequently increased. If combined with aspirin, probenecid or methotrexate, it can increase its associated toxicity; combined with antihypertensive drugs such as hydrazidazine, captopril and b-blockers, it can decrease its antihypertensive effect; combined with tachyphylaxis and thiazide diuretics can decrease its diuretic effect. It is usually safe for pregnant women to apply this drug. (3) Other NSAIDS; see the rheumatoid arthritis treatment guidelines for details. (2) Adrenal corticosteroids (such as prednisone): can be used in acute exacerbations of the disease. The common dose is 40mg~60mg per day, which can be taken once or divided into 2~3 times, and gradually amounted to 2 weeks when the symptoms are relieved. Contraindicated in anyone with viral infection, peptic ulcer, hepatic insufficiency, skin mycobacterial infection or tuberculosis. It is usually safe for pregnant women. (3) Immunosuppressants: The more commonly used are azathioprine, hydroxychloroquine or chloroquine, reactive stop, cyclophosphamide, cyclosporine and mycophenolate. ① Azathioprine: commonly used dose, 50~100mg per day, can be taken in 1 dose or in 2 doses. If combined with angiotensin-converting enzyme inhibitors, it can cause severe leukopenia. It is toxic to liver, kidney and hematology, so regular blood tests and liver and kidney functions should be performed. It should not be taken during pregnancy. ②Chloroquine or hydroxychloroquine: the common dose of chloroquine is 0.25/day once; hydroxychloroquine is 200mg once or twice a day, and it is changed to 100-200mg daily for long-term maintenance treatment after the effect. Long-term use should be alert to retinopathy and visual field changes, and eye examination should be done every six months. ③ Cyclophosphamide: the commonly used dose is 2.5~3mg/kg/day, divided into 3~4 times orally; in severe cases, 500~1000mg/m2/time, once every 2~4 weeks intravenously. It is contraindicated in severe bone marrow suppression or in pregnant women. Blood routine and liver and kidney function should be checked regularly and attention should be paid to prevent the occurrence of hemorrhagic cystitis. ④Cyclosporin A: The usual dose is 2.5~4mg/kg/day, divided into 2~3 doses. Difficult to control hypertension is prohibited, and pregnant women should use with caution. ⑤ Reactive stop (thalidomide): The commonly used dose is 100mg~300mg per day, taken at least one hour at night or after meals, starting from a small dose if the body weight is less than 50kg. It is prohibited for pregnant women due to its fetal malformation effect. In conclusion, in the acute inflammatory phase or in the presence of high fever, etc., treatment with adrenocorticotropic hormone with NSAIDS is generally effective. In patients with systemic type, especially in severe cases, 1~2 immunosuppressive agents can be added to the above treatment and treated accordingly according to the visceral involvement, and supportive therapy can be strengthened.