Efficacy of rituximab-containing regimens used as first-line and rescue therapy for giant cell hepatitis with autoimmune hemolytic anemia a retrospective study.

Objective: To evaluate the efficacy of rituximab (RTX)-containing therapy as first-line as well as rescue treatment for giant cell hepatitis with autoimmune hemolytic anemia (GCH-AHA).

Methods: This retrospective study recruited patients diagnosed with GCH-AHA and treated with conventional immunosuppressor regimens consisting of prednisone or RTX-containing regimes consisting of RTX and prednisone, with or without another immunosuppressor. The primary outcomes were the complete remission (CR) rate and time-period required for CR.

[Effect of freeze-thaw cycles on aggregate breakdown of typical black soil during transportation].

During the snowmelt period, the external erosive forces are dominated by freeze-thaw cycles and snowmelt runoff. These forces may affect soil structure and aggregate stability, thereby influencing snowmelt erosion. The process of snowmelt runoff can lead to the breakdown of aggregates during their transportation.

Early onset and liver failure indicating poor prognosis of infant liver failure syndrome type 1.

Background: Infantile liver failure syndrome type 1 (ILFS1, OMIM #615,438), caused by leucyl-tRNA synthase 1 (LARS1, OMIM *151,350) deficiency, is a rare autosomal-recessive disorder. The clinical manifestations, molecular-genetic features, and prognosis of LARS1 disease remain largely elusive.

Methods: Three new instances of ILFS1 with confirmed variants in LARS1, encoding LARS1, were identified.

Case report: Novel variants associated with idiopathic non-cirrhotic portal hypertension in a Han Chinese child.

deficiency has primarily been associated with lethal hepatic failure with or without hypotonia, nystagmus, and psychomotor retardation, features typical of mitochondrial disease. A study in 3 Turkish children identified homozygosity for a variant in as associated with idiopathic non-cirrhotic portal hypertension (INCPH). However, no further instances of INCPH associated with variants have been reported.

Spatial distribution characteristics of sediment connectivity and soil erosion in small watershed in brown soil hilly region of low mountains in western Liaoning Province, China.

Understanding the spatial distribution characteristics of soil erosion and sediment connectivity is important for formulating integrated watershed management measures. Based on the InVEST sediment delivery ratio model, we used the connectivity index and soil loss model to measure the spatial distribution characteristics of sediment connectivity and soil erosion in Erdaoling watershed of brown soil hilly region of low mountains in western Liaoning. By exploring slope, aspect and land use characteristics under different sediment connectivity levels and soil erosion levels, we analyzed the relationships among topography, land use, sediment connectivity, and soil erosion.

Neonatal Dubin-Johnson Syndrome and its Differentiation from Biliary Atresia.

Background And Aims: The aim was to determine if liver biochemistry indices can be used as biomarkers to help differentiate patients with neonatal Dubin-Johnson syndrome (nDJS) from those with biliary atresia (BA).

Methods: Patients with genetically-confirmed nDJS or cholangiographically confirmed BA were retrospectively enrolled and randomly assigned to discovery or verification cohorts. Their liver chemistries, measured during the neonatal period, were compared.

Lupus podocytopathy and antiphospholipid syndrome in a child with SLE: A case report and literature review.

Lupus podocytopathy is a glomerular lesion in systemic lupus erythematosus (SLE) characterized by diffuse podocyte foot process effacement (FPE) without immune complex (IC) deposition or with only mesangial IC deposition. It is rarely seen in children with SLE. A 13-year-old girl met the 2019 European League Against Rheumatism (EULAR)/ American College of Rheumatology (ACR) Classification Criteria for SLE based on positive ANA; facial rash; thrombocytopenia; proteinuria; and positive antiphospholipid (aPL) antibodies, including lupus anticoagulant (LAC), anti-β2 glycoprotein-I antibody (anti-β2GPI), and anti-cardiolipin antibody (aCL).

Medial Patellofemoral Ligament Reconstruction Using Suture Tape for Patellofemoral Joint Instability.

Objective: To describe a surgical technique using suture tape for reconstruction of the medial patellofemoral ligament (MPFL). This technique restores the stability of the reconstructed ligament and has excellent postoperative outcomes.

Method: This is a retrospective analysis.

Glycogen storage disease type VI can progress to cirrhosis: ten Chinese patients with GSD VI and a literature review.

Objectives The aim of our study is to systematically describe the genotypic and phenotypic spectrum of Glycogen storage disease type VI (GSD VI), especially in Chinses population.  Methods We retrospectively analyzed ten Chinese children diagnosed as having GSD VI confirmed by next generation sequencing in Children's Hospital of Fudan University and Jinshan Hospital of Fudan University. We described the genotypic and phenotypic spectrum of GSD VI through the clinical and genetic data we collected.

ABCB11 deficiency presenting as transient neonatal cholestasis: Correlation with genotypes and BSEP expression.

Background & Aims: ABCB11 deficiency presenting in infancy is believed generally to manifest as persistent/progressive cholestasis. We describe a group of patients with biallelic ABCB11 variants whose disorder manifested as transient neonatal cholestasis (TNC).

Methods: Neonatal intrahepatic cholestasis in 68 children (31 males) with biallelic predictedly pathogenic variants (PPV) in ABCB11 was classified as transient (TNC group, n = 23, 11 males), intermittent (benign recurrent intrahepatic cholestasis [BRIC] group, n = 3, 1 male) or persistent/ progressive (progressive familial intrahepatic cholestasis [PFIC] group, n = 42, 19 males).

TJP2 hepatobiliary disorders: Novel variants and clinical diversity.

To assess the spectrum of pediatric clinical phenotypes in TJP2 disease, we reviewed records of our seven patients in whom intrahepatic cholestasis was associated with biallelic TJP2 variants (13; 12 novel) and correlated clinical manifestations with mutation type. The effect of a splicing variant was analyzed with a minigene assay. The effects of three missense variants were analyzed with protein expression in vitro.

A mutation in PIK3CD gene causing pediatric systemic lupus erythematosus: A case report.

Rationale: Gain of function (GOF) mutations in PIK3CD gene encoding PI3K p110δ were recently associated with a novel combined immune deficiency characterized by recurrent sinopulmonary infections, CD4 lymphopenia, reduced class-switched memory B cells, lymphadenopathy, cytomegalovirus and/or epstein-Barr virus (EBV) viremia, and EBV-related lymphoma. A subset of affected patients also had elevated serum IgM.

Patient Concerns: We report a patient who was diagnosed with systemic lupus erythematosus (SLE) at a young age and was recently found to carry heterozygous mutations in PIK3CD.

Novel methionyl-tRNA synthetase gene variants/phenotypes in interstitial lung and liver disease: A case report and review of literature.

Interstitial lung and liver disease (ILLD) is caused by biallelic mutations in the methionyl-tRNA synthetase () gene. To date, no genetic changes other than missense variants were reported in the literature. Here, we report a five-month old female infant with typical ILLD (failure to thrive, developmental delay, jaundice, diffuse interstitial lung disease, hepatomegaly with severe steatosis, anemia, and thrombocytosis) showing novel phenotypes such as kidney stones, acetabular dysplasia, prolonged fever, and extreme leukocytosis.

A novel homozygous mutation in the glycerol-3-phosphate dehydrogenase 1 gene in a Chinese patient with transient infantile hypertriglyceridemia: a case report.

Background: Transient infantile hypertriglyceridemia (HTGTI) is an autosomal recessive disorder caused by mutations in the glycerol-3-phosphate dehydrogenase 1 (GPD1) gene.

Case Presentation: We report a case of HTGTI in a Chinese female infant. She presented with hepatomegaly, hypertriglyceridemia, moderately elevated transaminases, and hepatic steatosis at 3.

Defects in myosin VB are associated with a spectrum of previously undiagnosed low γ-glutamyltransferase cholestasis.

Unlabelled: Hereditary cholestasis in childhood and infancy with normal serum gamma-glutamyltransferase (GGT) activity is linked to several genes. Many patients, however, remain genetically undiagnosed. Defects in myosin VB (MYO5B; encoded by MYO5B) cause microvillus inclusion disease (MVID; MIM251850) with recurrent watery diarrhea.

Relationship between seedling and mature vegetation on the hilly-gullied Loess Plateau.

Seedling is an indispensable stage in plant cycle life, and seedling survival is important during natural vegetation restoration, especially on the Loess Plateau. In 2007, we selected 4 plots of Artemisia scoparia communities (ASC) and 4 plots of Artemisia gmelinii + Artemisia giraldii communities (AGC), examined seedling richness, diversity during the rainy season, and examined mature vegetation richness, coverage, and frequency in August. The results showed that seedlings density of ASC were 29 n m(-2), 33 n m(-2), 20 n m(-2) and 31 n m(-2) in July to October respectively, and that of AGC were 14 n m(-2), 12 n m(-2), 6 n m(-2) and 9 n m(-2) respectively; A.

Anti-inflammatory role of cannabidiol and O-1602 in cerulein-induced acute pancreatitis in mice.

Objectives: The anti-inflammatory effects of O-1602 and cannabidiol (CBD), the ligands of G protein-coupled receptor 55 (GPR55), on experimental acute pancreatitis (AP) were investigated.

Methods: Acute pancreatitis was induced in C57BL mice by intraperitoneal injection of 50 μg/kg cerulein hourly, with a total of 6 times. Drugs (O-1602, 10 mg/kg, or CBD, 0.

Overexpression of Sterol Carrier Protein 2 in patients with hereditary cholesterol gallstones.

Background: Lithogenic bile is the major cause of cholesterol gallstone, but its pathogenesis is not well understood. The hypersecretion of biliary cholesterol is believed to be an important cause of lithogenic bile. Sterol Carrier Protein 2 (SCP2) participates in cholesterol trafficking and lipid metabolism in hepatocytes and may play a key role in cholesterol gallstone formation.

Alteration and role of heat shock proteins in acute pancreatitis.

Many etiological factors are involved in the pathogenesis of acute pancreatitis. The pathogenesis of acute pancreatitis has been attributed to such causes as trypsin autodigestion, pancreatic microcirculation malfunction, the calcium overload in pancreatic acinar cells, oxygen free radical injury, cytokine injury, and has been treated in detail in numerous reviews. More recently, heat shock proteins (HSP), particularly heat shock protein 60 (HSP60), have receive increasing attention as another possible factor in the pathogenesis and development of acute pancreatitis.

Germinable soil seed banks and the restoration potential of abandoned cropland on the Chinese hilly-gullied loess plateau.

Poor vegetation cover is generally considered to be a major factor causing soil erosion on the Loess Plateau in China. It has been argued that tree planting restoration is ineffective, and natural re-vegetation is an alternative ecological solution for restoring abandoned cropland and controlling soil erosion. The aims of this study were to investigate the characteristics of soil seed banks and to assess the natural restoration potential of abandoned cropland in the hilly-gullied Loess Plateau.

Down-regulation of HSP60 expression by RNAi increases lipopolysaccharide- and cerulein-induced damages on isolated rat pancreatic tissues.

The objective of this study was to investigate the function of heat shock protein 60 (HSP60) on pancreatic tissues by applying HSP60 small interfering RNA (siRNA) to reduce HSP60 expression. Rat pancreas was isolated and pancreatic tissue snips were prepared, cultured, and stimulated with low and high concentrations of cerulein (10(-11) and 10(-5) mol/L) or lipopolysaccharide (LPS, 10 and 20 μg/mL). Before the stimulation and 1 and 4 h after the stimulation, the viability and the level of trypsinogen activation peptide (TAP) in the tissue fragments were determined and the levels of tumor necrosis factor-alpha (TNF-α) and interleukin 6 (IL-6) in the culture supernatants were measured.

Ascitic fluid and serum from rats with acute pancreatitis injure rat pancreatic tissues and alter the expression of heat shock protein 60.

Acute pancreatitis (AP) is an inflammatory process in which cytokines and chemokines are involved. After onset, extrapancreatic stimuli can induce the expression of cytokines in pancreatic acinar cells, thereby amplifying this inflammatory loop. To further determine the role and mechanism of irritating agents in the pathogenesis of AP, rat pancreatic tissues were stimulated with ascitic fluid (APa) and serum (APs) from rats with AP or with lipopolysaccharide (LPS).