TABLE 3.

Review of the literature: Wilson's disease patients with copper deficiency

References	Sex	Age at diagnosis of WD (years)	Clinical form of WD	Age at diagnosis of CD (years)	WD treatment when the CD is diagnosed	Symptoms	Resolution
Van Den Hamer and Hogenrad (1989) 6	Male	ND	ND	56	Zinc sulphate (1200 mg/d)	Anemia	Total resolution after parenteral copper administration
						Neutropenia
						Cu = 0.78 μmol/l
						UCE = ND
Narayan et al (2006) 7	Male	9	Neurologic	13	Zinc sulphate (280 mg/d) + D‐Penicillamine (750 mg/d)	Anemia	ND
						Cu = 2.5 μmol/L
						UCE = ND
						CNS demyelination
Foubert‐Samier et al (2009) 8	Male	15	Neurologic	43	Zinc acetate (400 mg/d) + Trientine 2HCl (900 mg/d)	Anemia	Improvement in cytopenia without improvement in peripheral neuropathy after withdrawn of zinc acetate
						Neutropenia
						Cu = 0.5 μmol/L
						UCE = 1.7 μmol/L
						Axonal sensory‐motor peripheral neuropathy
Horvath et al (2010) 9	Male	25	Neurologic	41	Zinc sulphate (1100 mg/d)	Anemia	Improvement in cytopenia without improvement in peripheral neuropathy after withdrawn of zinc acetate
						Leucopenia
						Cu = 0.5 μmol/L
						UCE = 0.54 μmol/L
						Axonal sensory‐motor peripheral neuropathy
Benbir et al (2010) 10	Male	16	Neurologic	21	Zinc acetate (100 mg/d) + D‐Penicillamine (1200 mg/d)	Cu = 0.34 μmol/L	Total resolution after withdrawn of WD therapeutics
						UCE = ND
						Partial seizures
						(No blood count available)
Cortese et al (2011) 11	Female	27	Neurologic	51	Zinc sulphate (1200 mg/d)	Anemia	Improvement in cytopenia without improvement in peripheral neuropathy
						Neutropenia
						Cu = 0.78 μmol/L
						UCE = 0.31 μmol/L
						Sensory‐motor peripheral neuropathy
Da Silva Jr et al (2011) 12	Female	29	Neurologic	44	Zinc acetate (450 mg/d)	Macrocytosis without anemia	Stabilization of clinical status
						Leukopenia
						Thrombocytopenia
						Cu = 0.47 μmol/L
						UCE = 0.11 μmol/L
						Myeloneuropathy
Lozano Herrero et al (2012) 13	Female	18	Hepatic	56	Zinc acetate (150 mg/d)	Anemia	Minimal improvement
						Neutropenia
						Cu = 0.47 μmol/L
						UCE = indétectable
						Subacute combined degeneration
Teodoro et al (2013) 14	Male	20	Neurologic	36	Zinc sulphate (330 mg/d) + Trientine 2HCl (500 mg/d)	Anemia	Partial regression
						Cu = 2.09 μmol/L
						UCE = 0.63 μmol/L
						Posterior dorsal cord myelopathy
Dziezyc et al (2014) 15	Female	19	Presymptomatic	37	Zinc sulphate (180 mg/d)	Neutropenia	Clinical and biological improvement
						Cu <0.78 μmol/L
						UCE = 0.17 μmol/L
						Posterior dorsal cord	without total resolution
						myelopathy
	Female	16	Hepatic	41	Zinc sulphate (180 mg/d)	Neutropenia	Total resolution after decreasing
						Cu <0.78 μmol/L
						UCE = 0.09 μmol/L	the treatment
	Female	12	Presymptomatic	18	Zinc sulphate (180 mg/d)	Pancytopenia	Total resolution after withdrawn of the treatment
						Cu = 1.10 μmol/L
						UCE = 0.18 μmol/L
Rau et al (2014) 19	Male	14	ND	16	Zinc sulphate	Cu = 4.87 μmol/L	Total resolution after withdrawn of the treatment
						UCE = 0.50 μmol/L
						Anemia
						Neutropenia
Mohamed et al (2018) 20	Female	13	Neurologic	26	Zinc sulphate (600 mg/d)	Pancytopenia	Total resolution after withdrawn of the treatment
						Cu <0.1 μmol/L
						UCE = ND μmol/L
Cai et al (2019) 21	Female	7	Hepatic	11	Zinc gluconate (240 mg/d)	Anemia	Total resolution after withdrawn of the treatment
						Neutropenia
						Cu = 0.88 μmol/L
						UCE = 0.47 μmol/L
						Abnormal gait
Wu et al (2020) 16	Female	17	Neurologic	18	Zinc sulphate (225 mg/d)	Cu = ND	Total resolution after copper supplementation
						UCE = significantly diminished
						Subacute combined degeneration of the spinal cord
						(No blood count available)
Ueda et al (2022) 22	Female	20	Neurologic	57	Zinc acetate (150 mg/d) + D‐penicillamine (1000 mg/d)	Anemia	Improvement in cytopenia with partial improvement in myeloneuropathy after withdrawn of zinc acetate and D‐penicillamine
						Cu = 1.73 μmol/L
						UCE = 1.16 μmol/L
						Posterior dorsal cord myelopathy

Abbreviations: CD, copper deficiency; CNS, central nervous system; ND, no data; WD, Wilson's disease.