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Diagnostics (Basel, Switzerland) Jul 2023Treatment of Wilson's disease (WD), an inherited disease characterized by copper overload, is lifelong and there is the possibility that copper deficiency (CD) may... (Review)
Review
BACKGROUND
Treatment of Wilson's disease (WD), an inherited disease characterized by copper overload, is lifelong and there is the possibility that copper deficiency (CD) may occur. We systematically reviewed the literature to describe treatment patterns, symptoms and outcomes associated with CD.
METHODS
Using preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines, the PubMed database was searched up to 6 April 2023.
RESULTS
Across 17 articles, 20 cases of CD were described, most commonly (15 cases) in WD patients treated with zinc salts (ZS), less often on combined chelator and ZS therapy (3 cases), molybdate salts plus ZS (1), or molybdate alone (1). CD symptoms occurred insidiously, including sideroblastic anemia, neutropenia, axonal sensory neuropathy, posterior cord myelopathy and increased ratio of epileptic seizures (or epilepsy). CD diagnosis was based on symptoms and severely reduced urinary copper excretion (<20 µg/24 h [<0.3 µmol/24 h] on ZS, or <100 µg/24 h [<1.6 µmol/24 h] on chelators) with low total serum copper and ceruloplasmin.
CONCLUSIONS
Awareness of CD and regular monitoring of copper metabolism is needed during WD treatment. Temporary cessation of anti-copper treatment usually reverses serum copper reductions as well as pancytopenia; however, some symptoms, especially neuropathy and myelopathy, may persist.
PubMed: 37510170
DOI: 10.3390/diagnostics13142424 -
American Journal of Hematology Aug 2023
Topics: Humans; Myelodysplastic Syndromes; Anemia, Sideroblastic; Activin Receptors, Type II; Immunoglobulin Fc Fragments
PubMed: 37222267
DOI: 10.1002/ajh.26960 -
Mediterranean Journal of Hematology and... 2023
PubMed: 38028395
DOI: 10.4084/MJHID.2023.062 -
Clinical Case Reports Dec 2023Dolutegravir, the most recent antiretroviral drug with high efficacy, good tolerability, infrequent drug-drug interactions, and a favorable safety profile has not been...
Dolutegravir, the most recent antiretroviral drug with high efficacy, good tolerability, infrequent drug-drug interactions, and a favorable safety profile has not been reported in current literature as a cause of acquired sideroblastic anemia. Here, we present a 35-year-old male patient who was diagnosed with acquired sideroblastic anemia to Dolutegravir therapy.
PubMed: 38084360
DOI: 10.1002/ccr3.8301