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Abstract. citrin is a liver-type aspartate/glutamate carrier (AGc) encoded by the gene SLc25A13. Two phenotypes for human citrin deficiency have been described, namely the adult-onset citrullinemia type II (cTLN2) and the neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). However, citrin deficiency currently remains a perplexing and poorly recognized disorder. In particular, description of postNIccd clinical presentations before cTLN2 onset is rather limited. Analysis of SLC25A13 mutations, identification of dysmorphic erythrocytes, hepatobiliary scintigraphic imaging and investigation of post-NIccd clinical presentations were performed in a citrin-deficient cohort comprised of 51 cases of children diagnosed with citrin deficiency in a Chinese pediatric center. Twelve SLc25A13 mutations were detected in this cohort, including the novel V411M and G283X mutations. Among the 51 citrin-deficient subjects, 7 cases had echinocytosis, which was associated with more severe biochemical abnormalities. delayed hepatic discharge and bile duct/bowel visualization were common scintigraphic findings. Moreover, 9 of the 34 post-NIccd cases demonstrated concurrent failure to thrive and dyslipidemia, constituting a clinical phenotype different from NIccd and cTLN2. The novel mutations, echinocytosis, hepatobiliary scintigraphic features and the novel clinical phenotype in this study expanded the genotypic and phenotypic spectrum of citrin deficiency, and challenge the traditionally-assumed 'apparently healthy' period after the NIccd state for this disease entity.

Potassium ion batteries (KIBs) are the emerging and promising energy storage system for large-scale electrochemical energy storage.

A robust artificial solid electrolyte interphase (SEI) film with biomimetic ionic channels and high stability is rationally designed and fabricated by combining the ClO4−‐decorated metal‐organic framework (UiO‐66‐ClO4) and flexible lithiated Nafion binder (Li‐Nafion). The high electronegativity and lithiophilicity of ClO4− groups chemically anchored into the UiO‐66 channels endow the SEI film with an excellent single‐ion conducting pathway, high Li+ transference number, and outstanding ionic conductivity, which can effectively prohibit undesirable reactions of the Li metal with the electrolyte and regulate fast and uniform Li+ flux. With further assistance of the flexible Li‐Nafion binder, the resulting UiO‐66‐ClO4/Li‐Nafion (UCLN) composite film exhibits an excellent mechanical strength to suppress the growth of Li dendrites and maintain the integral stability of the Li metal anodes during cycling. Consequently, the UCLN coated Li metal anodes (Li@UCLN) deliver remarkable cycling stabilities even under a high current density of up to 20 mA cm−2 and large areal capacity of up to 30 mAh cm−2, as well as enhanced rate capacities and cycle lifespans in the full cells even under the harsh conditions for high‐energy density applications.