Cysteamine is a chemical compound that can be biosynthesized in mammals, including humans, by the degradation of coenzyme A. The intermediate pantetheine is broken down into cysteamine and pantothenic acid. It is the biosynthetic precursor to the neurotransmitter hypotaurine.It is a stable aminothiol, i.e., an organic compound containing both an amine and a thiol functional groups. Cysteamine is a white, water-soluble solid. It is often used as salts of the ammonium derivative [HSCH2CH2NH3]+ including the hydrochloride, phosphocysteamine, and bitartrate.As a medication, cysteamine, sold under the brand name Cystagon among others, is indicated to treat cystinosis. Medical uses
Cysteamine is used to treat cystinosis. It is available by mouth (capsule and extended release capsule) and in eye drops.When applied topically it can scavenge free radicals and lighten skin thats been darkened as a result of post-inflammatory hyperpigmentation, sun exposure and Melasma. Tentative evidence suggests that it may be a more effective depigmentation agent than hydroquinone, retinoids and topical corticosteroids in individuals with chronic skin discoloration. Topical application of cysteamine cream has also demonstrated similar efficacy to intradermal tranexamic acid injections for the treatment of Melasma but with much fewer adverse effects. Adverse effects
Topical use
The most important adverse effect related to topical use might be skin irritation. However its significantly better tolerated than alternative skin lightening treatments with similar efficacy.
An orthotopic liver transplant is another option, used only when antibiotics have no effect, in combination with recurring cholangitis. With a liver transplant, cholangiocarcinoma is usually avoided in the long run.Family studies are necessary to determine if Caroli disease is due to inheritable causes. Regular follow-ups, including ultrasounds and liver biopsies, are performed. Prognosis
Mortality is indirect and caused by complications. After cholangitis occurs, patients typically die within 5–10 years. Epidemiology
Caroli disease is typically found in Asia, and diagnosed in persons under the age of 22. Cases have also been found in infants and adults. As medical imaging technology improves, diagnostic age decreases. History
Jacques Caroli, a gastroenterologist, first described a rare congenital condition in 1958 in Paris, France. He described it as "nonobstructive saccular or fusiform multifocal segmental dilatation of the intrahepatic bile ducts"; basically, he observed cavernous ectasia in the biliary tree causing a chronic, often life-threatening hepatobiliary disease. Caroli, born in France in 1902, learned and practiced medicine in Angers. After World War II, he was chief of service for 30 years at Saint-Antoine in Paris. Before dying in 1979, he was honored with the rank of commander in the Legion of Honour in 1976. References


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