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Lili Yu,1,2,* Wancheng Chen,2,* Qingshuang Tang,2,* Kai-Yuan Ji1 1The Eighth Affiliated Hospital, Sun Yat-sen University, Shenzhen, Guangdong 518033, People’s Republic of China; 2Cancer Research Institute, Guangdong Provincial Key Laboratory of Cancer Immunotherapy, School of Basic Medical Sciences, Southern Medical University, Guangzhou, Guangdong 510515, People’s Republic of China*These authors contributed equally to this workCorrespondence: Kai-Yuan JiThe Eighth Affiliated Hospital, Sun Yat-sen University, Shenzhen, Guangdong 518033, People’s Republic of ChinaTel +8675515625055871Email 369027938@qq.comPurpose: Micheliolide (MCL) is an effector compound of the flower which has been traditionally used to treat inflammation and cancer patients in oriental medicine. MCL has killing effects on several cancer and immune cells by modulating apoptosis, cell cycle, and metabolism. However, the detail of the mechanisms of anti-cancer activity remains to be elucidated and the effect on liver cancer cells is unknown.Methods: Cell proliferation was determined by CCK8 and clone formation assay. The xenograft liver cancer model formed by injecting Huh7 cells into NUDE mice was used to evaluate the effects of MCL on liver cancer cells in vivo. We evaluated the stemness of cells with spheroid formation assay and flow cytometry assay. The apoptosis was determined by Annexin V assay. F-actin staining and ROS were performed to detect the impairment of the F-actin cytoskeleton and mitochondria.Results: Here, we first show that MCL inhibits liver cancer cells both in vivo and in vitro by triggering apoptosis which was reduced by anti-oxidant, but not cell-cycle arrest. In addition, MCL induces mitochondrial ROS and caspase-3 activation. Also, we found that the aggregation of mitochondria and the perturbation of F-actin fibers in the MCL-treated liver cancer cells coincidently occurred before the induction of apoptosis and mitochondrial ROS.Conclusion: These results suggest that F-actin perturbation is involved in impaired mitochondria and apoptosis. Therefore, MCL can be a potent therapeutic reagent for liver cancer, primarily targeting the actin cytoskeleton.Keywords: Micheliolide, liver cancer, apoptosis, ROS, actin cytoskeleton

Leydig cell tumors can cause precocious puberty in prepubertal males. Radical orchiectomy is the standard therapy for Leydig cell tumors, with a good overall prognosis. Here, we present a rare case of secondary central precocious puberty caused by a Leydig cell tumor in a 5 year-old boy. He had an isosexual precocity and advanced bone age. Laboratory investigations showed high testosterone and low gonadotropin levels, presenting as precocious pseudo-puberty. Seven months after surgical removal of the tumor, the patient was diagnosed with central precocious puberty by GnRH stimulation test. He was initiated on GnRH agonist therapy, which has been administered every 13 weeks to date. Post-surgery surveillance of Leydig cell tumors should include evaluation for progression to secondary central precocious puberty as well as recurrence of Leydig cell tumor or metachronous tumors in prepubertal children.

Hereditary spherocytosis is the most common hereditary red blood cell membrane disorder. It results from a deficiency in certain proteins that are part of the red blood cell membrane cytoskeleton. We report a case of adrenal myelolipoma in a 23-year-old patient with hereditary spherocytosis. She was diagnosed with hereditary spherocytosis at 7 months of age. As she grew older, hemoglobin level was low, so splenectomy was recommended, but continuous follow up was not possible due to the circumstances of the patient, so the splenectomy was delayed. Adrenal myelolipoma was discovered incidentally at the age of 23 with abdominal pain and cholecystitis with gallstones at the time. Myelolipoma is a benign tumor of the mesenchymal origin; its etiology remains unclear. Myelolipoma is composed of adipose and hematopoietic tissues and mainly arises from adrenal tissues. The mass is often detected during routine radiologic examinations because myelolipoma is usually asymptomatic and not generally associated with hematologic diseases. The prevalence of myelolipoma appears to be increasing due to the increased use of imaging modalities. The association of myelolipoma with hereditary spherocytosis has rarely been reported in the literature. To our best knowledge, this is the first report of adrenal myelolipoma associated with hereditary spherocytosis in the Korean population.

Background : : In Korea, in the case of patients with significant bleeding symptoms due to unknown causes, there are very few studies that evaluate bleeding disorders, including von Willebrand disease (VWD). VWD should be considered as an important causative factor in patients with iron deficiency anemia (IDA) and unexplained menorrhagia. This study aimed to understand the clinical characteristics of VWD and the significance of evaluation for VWD in premenopausal women in Korea with menorrhagia and IDA.Methods : : Premenopausal women who were diagnosed with IDA and menorrhagia from January 2009 to March 2020 were included. IDA was diagnosed by either low ferritin or transferrin saturation with microcytic anemia. Menorrhagia was evaluated based on the medical records obtained from a gynecologist. VWD diagnosis was defined as von Willebrand factor antigen

Background : : von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. However, the number of patients who register to the Korea Hemophilia Foundation (KHF) is much lower than the expected prevalence rate and only few hospitals perform tests for diagnosis autonomously. Thus, we surveyed practical realities of VWD in Yeungnam region. Methods : : Patients with VWD (N=267) who were diagnosed at eleven university hospitals from March 1995 to March 2018 were enrolled in this study. We evaluated the medical records from each hospital retrospectively. Results : : Two hundred and twenty-eight children and 39 adults met the diagnostic criteria for VWD. Seventy-eight (57.4%) patients had the blood type O. Fifty-eight patients were definite type 1 (21.7%), 151 were possible type 1 (56.6%), and the others were type 2. Abnormal laboratory findings were the most common factor for the diagnosis in children. VWF mutations were detected in 17 patients. Patients with a family history showed age of diagnosis of 9 y, which is higher than in those with no family history (6 yr), and also showed a higher rate of significant bleeding (32.1% vs. 14.2%). VWF:RCo and VWF:Ag tests were performed in-hospital at only 1 of 11 hospitals. Twelve of 267 patients were enrolled at the KHF (4.5%). Conclusion : : A high rate of out-sourcing studies may result in inaccurate diagnosis. The registration rate to the KHF is still lower than the prevalence rate. A comprehensive nationwide registration system is necessary in order to identify the actual prevalence rate and promote the diagnosis of VWD in Korea.