Yuanyuan Jing

Effect of clarithromycin in von Hippel-Lindau syndrome: a case report

Von Hippel-Lindau (VHL) syndrome is caused by germline mutations in the VHL gene and is accompanied by the development of both benign and malignant tumors. Clarithromycin (CAM) is a widely used anti-inflammatory drug that has also been proven effective for treating some cancers. In this study, we present a novel case of a 38-year-old female patient with VHL syndrome confirmed by computed tomography, with no relevant family history. The patient was only offered close follow-up observation after diagnosis, but was orally administered CAM twice daily when her disease progressed. After treatment for 3 months, her inner ear tumors had stopped growing and her kidney cysts had shrunk. Her pancreatic tumors treated with CAM showed no significant increase in size, and the lymph node swelling disappeared. CAM treatment was interrupted for 2 years and then stopped for 5 years when improvements were noted; the patient’s condition thereafter gradually improved and finally stabilized with no obvious side effects. We discuss this case in the context of the published literature and consider the possible mechanisms of CAM action in the treatment of VHL syndrome. To our knowledge, this is the first case report of a patient with VHL syndrome treated with CAM.

A comparison of systemic and local dexamethasone administration: From perilymph/cochlea concentration to cochlear distribution

ABSTRACT Different types of inner ear diseases can damage different cochlear subsites by different mechanisms. Steroids administered by different methods are commonly used for treating inner ear diseases. There is reason to believe that dexamethasone (Dex) may reach cochlear subsite targets via different pathways after administration by different methods: Intratympanic (IT), postaural (PA), and intraperitoneal (IP). The purpose of this study was to explore the cochlear concentration and distribution of Dex after administration by different methods. High‐performance liquid chromatography‐mass spectrometry and immunofluorescence technology were employed to measure and compare the Dex concentration in the perilymph and cochlear tissue and the cochlear distribution of Dex. IT administration resulted in higher Dex concentrations in the perilymph and cochlear tissues than those with the other administration methods. Intratympanic and postaural administration could result in higher Dex concentrations in the organ of Corti than systemic administration, but systemic administration could result in higher Dex concentrations in the stria vascularis than the other administration methods. A decreasing basal‐apical gradient of Dex uptake was present in the cochlea after IT but not IP or PA administration. These results indicate that different administration methods result in different Dex distributions, which can be attributed to features of the cochlear vascular system and intracochlear diffusion. Our results provide clinicians with an experimental basis for the use of different steroid injection routes to optimize the effects on inner ear diseases with different target organs. HighlightsIT group caused higher Dex in perilymph and cochlea than IP and PA groups.A decreasing basal‐apical gradient of Dex was only present in IT group.Local injection caused higher Dex in the organ of Corti than systemic injection.Circulation is more effective for delivering Dex to stria vascularis than diffusion.