Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024530 (malaria)
44,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The death of a 23-year-old female patient is reported. She was being treated for porphyria cutanea tarda (p.c.t.) and was mistakenly given a single dose of 1250 mg chloroquine (instead of the intended dose of 125 mg). The course differed from the typical course of chloroquine toxification, which involves cardiac arrhythmias as the main complication (reported in adults only after much higher doses); in spite of intensive care, this patient died 13 days after the ingestion of chloroquine, in a coma caused by irreversible, acute decompensation of the liver. Forensic autopsy and histological and toxicological investigations confirmed the clinical findings. Starting from the chloroquine's pharmacological mechanism of effect in p.c.t., the severe hepatotoxic effect of this drug in the present case is explained. The authors give a serious warning against exceeding the dose of chloroquine now usually recommended for the treatment of the p.c.t., which is 125-250 mg twice weekly. The presence of chronic porphyria hepatica, at least in its clinically manifest form, (= p.c.t.), should be considered a contraindication for chloroquine in prophylaxis and treatment of other illnesses (e.g. malaria and rheumatism).
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PMID:[Death following administration of 1,250 mg chloroquine in porphyria cutanea tarda]. 357 36

Therapeutic erythrocytapheresis (TEA) has been used in different diseases such as polycythemia vera (PV), secondary erythrocytosis or hemochromatosis as a process of the less cumbersome but more expensive phlebotomy. TEA is preferred in emergency conditions such as thrombocytosis or in conditions such as porphyria cutanea tarda (PCT) or erythropoietic porphyria when plasma exchange (PEX) is often combined with TEA to reduce extracellular levels of uroporphyrin which contribute to plasma hyperviscosity. TEA is often combined with drug therapy that varies from etoposide in PV to EPO and desferoxamine which are used to mobilize and reduce iron stores in hemochromatosis. Benefits from this combination may be more long lasting than expected. Nonetheless for TEA, there is no standard protocol and, clinical experience with this therapy remains highly anecdotal. Therapeutic red cell-exchange (TREX) has been used with much interest over the years, starting with the management of hemolytic disease of the newborn and later used to correct severe anemia in thalassemia patients thereby preventing iron overload. It has also been used for the management of complications of sickle cell disease such as priapism, chest syndrome, stroke, retinal, bone, splenic and hepatic infarction or in preparation for surgery by reducing HbS to less than 30%. Automated apheresis has also favored the use of TREX in conditions such as paroxysmal nocturnal hemoglobinuria and aniline poisoning, arsenic poisoning, Na chlorate intoxications and CO intoxications, hemoglobinopathies, autoimmune hemolytic anemia, reactions due to ABO incompatibility, in preparation for ABO incompatible bone marrow transplantation or for preventing anti-D immunization after the transfusion of D(+) cells to D(-) recipients. Another field of application has been in the emergency management of intraerythrocytic parasite infections such as malaria and babesiosis. Application of TREX may be wide but its real use remains limited. In our personal experience, in 16 years, only 167 TREX procedures have been carried out in a total of 13,747 therapeutic procedures. This represents only 1.21% of the total.
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PMID:Clinical application of therapeutic erythrocytapheresis (TEA). 1083 21