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Wilson disease (WD) is a rare inherited copper metabolism disorder characterized by progressive pathological deposition of copper, primarily in the liver and brain. This longitudinal retrospective study conducted using the French national claims (Système National des Données de Santé [SNDS]) database assessed WD prevalence in France, described patients' characterizations and healthcare resource use and associated costs. Patients with WD were identified from SNDS using the International Classification of Diseases, 10th Revision code E83.0 for copper metabolism disorder or a long-term disease (affection de longue durée [ALD]) associated with this code between 2010 and 2019. Patients were categorized into hepatic, neurological, and psychiatric sub-cohorts. We identified 2287 patients with WD yielding a crude prevalence of 1 case per 33 898 individuals in 2019. The mean age at inclusion was 39.9 (standard deviation [SD] 22.8) years, 11 years more than that of the incident cohort (28.6, SD 20.3) identified via ALD, and 1180 (51.6%) patients were male. The crude mortality was 3.2% (in total 370 patients died). Overall, 1011 (44.2%), 754 (33.0%), and 414 (18.1%) patients experienced hepatic, neurological, and psychiatric manifestations, respectively. In total, 922 (40.3%) patients were reimbursed for WD-specific treatment, the most common being D-penicillamine (74.8%), and 201 (8.8%) underwent liver transplantation. The average annual hospitalization cost per patient was 4273.7€ (SD 11916.0). At least one sick leave was reported for 533 (23.3%) patients, with an annual average cost of 788.7€ (SD 1328.6). Our findings provide an updated understanding of the prevalence of WD, and indicate a considerable level of morbidity in this population, as well as a high level of direct/indirect costs associated with WD., (© 2024 The Author(s). Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM.)

Objective: To compare morbidity and aesthetic outcomes of full-thickness skin grafts (FTSGs) and split-thickness skin grafts (STSGs) in the reconstruction of the forearm free flap donor site., Study Design: Case series, retrospective chart review., Setting: Institutional microvascular database., Subjects and Methods: Subjects who underwent forearm free flaps and FTSGs for donor site reconstruction from April 2016 to November 2017 were included. FTSGs were obtained from the donor forearm with a proximal S-shaped incision, thereby avoiding additional wound creation. Morbidity outcomes were compared to 68 consecutive patients with STSG reconstruction from January 2009 to May 2010. Complications, including tendon exposure, subjective functional impairment, complete graft loss, partial graft loss, infection, paresthesias, and hematoma/seroma, were evaluated, as were aesthetic outcomes., Results: Sixty-eight patients underwent FTSG reconstruction. No significant differences between FTSGs and STSGs were demonstrated in terms of graft loss ≥40% (4% vs 4%, P = 1.000), partial graft loss (<40%) (29% vs 40%, P = .207), tendon exposure (9% vs 12%, P = .573), infection (15% vs 13%, P = .805), paresthesias (12% vs 7%, P = .382), subjective functional impairment (0% vs 2%, P = .316), or hematoma/seroma (2% vs 0%, P = .316). Aesthetic outcomes were better in the FTSG group compared to the STSG group, as determined by both patients ( P = .004) and surgeon ( P < .001)., Conclusions: Our results advocate for the consideration of FTSGs in the reconstruction of the forearm free flap donor site given superior aesthetic results without additional donor site morbidity or additional wound creation when compared to STSGs.