Liver transplantation in Wilson’s disease: Single center experience from Saudi Arabia

Table 1 Baseline characteristics of 16 Wilson’s disease patients’ pre-transplant details

Case	Age (yr)	Gender	Nationality	Ascites	Jaundice	HE	Neuro	24-h Copper	Ceruloplasmin	Penicillamine	KF ring	CTP	MELD
1	14	F	Saudi Arabia	Yes	Yes	No	Yes	-	100	Yes	Yes	C	19
2	14	F	Saudi Arabia	Yes	Yes	Yes	No	-	155	-	No	C	31
3	3	M	Saudi Arabia	No	Yes	No	No	-	325	Yes	No	B	12
4	12	M	Saudi Arabia	No	Yes	No	No	14.1	395	Yes	No	B	11
5	8	M	Saudi Arabia	Yes	Yes	No	No	-	250	-	No	B	11
6	4	M	Saudi Arabia	Yes	Yes	Yes	No	4.5	< 71	Yes	No	C	18
7	12	F	Saudi Arabia	No	Yes	No	No	10.02	54	Yes	No	B	10
8	38	M	Saudi Arabia	Yes	Yes	No	No	14	71	-	No	B	19
9	13	M	Saudi Arabia	Yes	Yes	No	No	10.2	227	Yes	No	B	13
10	6	M	Syrian Arab	Yes	Yes	No	No	-	< 72	-	No	B	13
11	25	F	Saudi Arabia	Yes	Yes	Yes	Yes	6.84	< 71	Yes	Yes	A	12
12	23	F	Saudi Arabia	Yes	Yes	Yes	Yes	0.89	128	-	Yes	C	34
13	18	M	Saudi Arabia	Yes	Yes	No	No	37	346	Yes	No	C	27
14	7	F	Saudi Arabia	No	Yes	No	No	17.06	360	Yes	No	A	-
15	22	F	Saudi Arabia	Yes	Yes	Yes	Yes	1.86	< 71	Yes	Yes	A	14
16	5	M	Yemeni	No	Yes	No	No	0.88	373	-	No	B	-

Age: Age at the time of diagnosis of Wilson’s disease; M: Male; F: Female; HE: Hepatic encephalopathy; KF ring: Kayser-Fleischer ring; CTP: Child-Turcotte-Pugh score; MELD: Model for end-stage liver disease; Penicillamine: These patients had received treatment with D-penicillamine prior to liver transplantation.

Table 2 Post-liver transplant details of 16 Wilson’s disease patients

Case	Gender	Age	Year of TX	Indication for TX	Neurological WD	TX center	Donor	Rejection	Graft survival	Pt survival	Death or lost FU	HCC
1	F	14	1991	Chronic	Yes	United States	Cad	No	20 yr	20 yr	-	No
2	F	14	1994	Chronic	No	KFSHRC	Cad	Chronic	15 yr	17 yr	-	No
3	M	11	1995	Chronic	No	United States	Cad	No	6 yr	6 yr	Lost FU	No
4	M	12	1995	Chronic	No	KFSHRC	Cad	No	16 yr	16 yr	-	No
5	M	13	1995	Chronic	No	United States	Cad	Acute	16 yr	16 yr	-	Yes
6	M	13	1995	Chronic	No	KFSHRC	Cad	No	16 yr	16 yr	-	No
7	F	12	1996	Chronic	No	United Kingdom	Cad	No	15 yr	15 yr	-	Yes
8	M	40	1999	Chronic	No	United States	Cad	No	12 yr	12 yr	-	No
9	M	22	2003	Chronic	No	United States	Cad	No	8 yr	8 yr	-	No
10	M	23	2004	Chronic	No	KFSHRC	LR	No	6 d	6 d	Died	No
11	F	40	2004	Chronic	Yes	KFSHRC	Cad	No	7 yr	7 yr	-	No
12	F	23	2004	Chronic	Yes	KFSHRC	Cad	Acute	7 yr	7 yr	-	No
13	M	18	2006	Chronic	No	KFSHRC	Cad	Acute	5 yr	5 yr	-	No
14	F	9	2006	Chronic	No	KFSHRC	LR	No	5 yr	5 yr	-	No
15	F	25	2006	Chronic	Yes	KFSHRC	Cad	Acute	5 yr	5 yr	-	No
16	M	8	2007	Chronic	No	KFSHRC	LR	No	4 yr	4 yr	-	No

Age: Age at the time of transplantation; M: Male; F: Female; WD: Wilson’s disease; HCC: Hepatocellular carcinoma; TX: Transplantation; FU: Follow-up; KFSHRC: King Faisal Specialist Hospital and Research Center.

Table 3 Neurological and psychiatric manifestations of Wilson’s disease before and after liver transplantation

Characteristics	Case 1	Case 11	Case 12	Case 15
Neurological symptoms before transplantation	Akinesia dysarthria abnormal movement	Tremor dysarthria abnormal movement	Tremor nystagmus	Abnormal movement
Psychiatric abnormalities before transplantation	Negative	Negative	Depression restlessness	Delusion of persecution depression restlessness hallucination
MRI brain before transplantation	No available data	High signal intensities involving the thalami, mid brain, and pons	Bilateral and symmetrical abnormal hyper-intensity at level of basal ganglia	High signal intensity in the basal ganglia and substantia nigra with mild cerebral atrophy
KF ring before transplantation	Positive	Positive	Positive	Positive
Neurological symptoms after transplantation	Negative	Negative	Negative	Negative
Psychiatric abnormalities after transplantation	Negative	Negative	Negative	Negative
MRI brain after transplantation	No available data	Normal MRI of the brain	No available data	No available data
KF ring after transplantation	Negative	Negative	Negative	Negative

KF ring: Kayser-Fleischer ring; MRI: Magnetic resonance imaging.