Background: Gaucher disease (GD) is a rare autosomal recessive inherited disease caused by the deficiency of glucocerebrosidase and characterized by a broad spectrum of clinical manifestations, including hepatosplenomegaly, bone infiltration, and cytopenia. Moreover, it is even involved in the central nervous system. GD is classified into three phenotypes on the ground of neurologic involvement: type 1 (GD1), the commonly adult-onset, non-neuropathic variant; type 2 (GD2), the acute neuropathic form; and type 3 (GD3), the severe chronic neuro-visceral form. Recently, several studies have shown a promising outcome of ambroxol chaperone therapy for the treatment of GD, but its therapeutic role in GD1-associated liver cirrhosis and portal hypertension was not verified. Case presentation: A 36-year-old male patient was admitted for esophageal varices lasting for one year with a 34-year history of liver and spleen enlargement. The patient was diagnosed with GD1 with cirrhosis and portal hypertension based on the identification of Gaucher cells and advanced fibrosis in the liver biopsy tissue and two known pathogenic mutations on the glucocerebrosidase (GBA) gene. The patient received 660 mg/d of ambroxol for up to two years. At his six-month follow-up, the patient exhibited a remarkable increase in GBA activity (+35.5%) and decrease in liver stiffness (-19.5%) and portal vein diameter (-41.2%) as examined by ultrasound elastography and computer tomography, respectively. At two-year follow-up, the liver stiffness was further reduced (-55.5%) in comparison with untreated patients. Conclusion: This case report suggests that long-term treatment with high dose ambroxol may play a role in the reduction of hepatic fibrosis in GD1.

Background Liver injury related to Graves’ Disease (GD) includes hepatotoxicity of thyroid hormone excess, drug-induced liver injury, and changes resulting from concomitant liver disease. Methimazole (MMI) has been shown to induce several patterns of liver injury. However, the diagnosis and treatment of autoimmune hepatitis (AIH) overlapping with either GD or chronic hepatitis B are challenging. Case presentation A 35-year-old man from China presented with a two-year history of GD and a 10-day history of progressive jaundice. He had taken MMI for two months and discontinuing treatment due to liver toxicity 1 year ago and for another 6 days 20 days prior to hospitalization. The patient was diagnosed with GD overlapping with chronic hepatitis B and MMI-induced liver injury with early stage of acute-on-chronic liver failure on admission. However, the elevated aminotransferase and bilirubin levels could not be controlled after correction of liver failure and effective control of HBV replication and hyperthyroidism by daily oral entecavir and one-time oral administration of 131-iodine. The patient underwent liver biopsy on the 43rd day of hospitalization, showing HBsAg expression on the membrane of hepatocytes and typical histopathological characteristics of AIH. He was finally diagnosed with GD overlapping with chronic hepatitis B and MMI-induced liver injury and AIH. The elevated aminotransferase and bilirubin completely returned to normal by 3-month glucocorticoid therapy and continuous entecavir treatment and there was no recurrence during a 6-month follow-up, suggesting that AIH in this patient is different from classical AIH or GD-associated AIH. Conclusions GD together with AIH is a complex and difficult subject. It needs to be clarified whether MMI or HBV can act as a trigger for AIH in this patient.

Polyvinylcaprolactam (PVCap) is an economic kinetic inhibitor for hydrate formation in pipelines during oil and gas transportation. However, its application is limited because of the low inhibition performance under certain conditions. In this work, a modified PVCap on its chain end is proposed.2-amino-3-propionic acid mercapto-terminated polyvinyl caprolactam (PVCap-NH2-COOH) was synthesized and its performance as a KHI for methane hydrate formation was evaluated under different conditions. Results showed that the performance of PVCap-NH2-COOH as a KHI was better than that of PVCap at the same concentrations. Gas hydrate samples with 1 wt.% PVCap-NH2-COOH were measured using Raman spectroscopy, XRD, and cryo-SEM. PVCap-NH2-COOH had a selective action on a specific crystal surface of the hydrates and could prevent methane molecules from entering large cages. Its inhibition ability increased with the decrease in the occupancy rate of large cages. The morphology of the gas hydrate crystal changed from porous in a pure water system to a chaotic but compact structure state in the system with PVCap-NH2-COOH.

Recently, aquamarine was discovered in the Cuonadong Be-W-Sn Polymetallic Deposit, Longzi County, Tibet. Longzi aquamarine is being extracted and is expected to be available over the next decade. This study provides a full set of data through standard gemmological properties, including scenes, color characteristics and advanced spectroscopic and chemical analyses, including micro ultraviolet–visible–near-infrared (UV–Vis–NIR), Raman and laser ablation inductively coupled plasma mass spectrometry (LA-ICP-MS). The main inclusions in Longzi aquamarine are gas–liquid inclusions and a great number of quartz inclusions. The content of type I H2O is greater than that of type II H2O because of the low-alkali metal content, and “tetrahedral” substitutions and “octahedral” substitutions exist at the same time.