Persistent Muellerian duct syndrome (PMDS) is a rare form of male pseudohermaphrodism without the feature of ambiguous genitalia. We present a case of PMDS with transverse testicular ectopia (TTE).
Abnormalities, Multiple ; Adult ; Disorders of Sex Development ; pathology ; surgery ; Hernia, Inguinal ; surgery ; Humans ; Male ; Mullerian Ducts ; abnormalities ; surgery ; Testicular Hydrocele ; surgery ; Testis ; abnormalities ; surgery

This study was aimed to investigate the therapeutic efficacy of rabbit anti-thymocyte globulin (r-ATG) combined with cyclosporine A (CsA) and to analyse the efficacy-related factors in children with aplastic anemia (AA). Twenty five AA children treated with r-ATG [3.5 mg/(kg·d)×5 days] combined with CsA were analyzed retrospectively. The lymphocyte subgroups, CD4(+)/CD8 ratio and expression of CD55, CD59 on surface of neutrophils and erythrocytes in peripheral blood were detected by direct immunofluorescence method and flow cytometry; the responsive time, effective rate, adverse effects and infections after immunosuppressive therapy (IST) were analyzed; the distribution of T-lymphocyte subgroups in IST-effective and IST-uneffective groups was compared, and therapeutic efficacy-related factors were evaluated. The results showed that the response to treatments was found in 21 out of 25 cases, the total responsive rate was 84.0%; the response time was 3 - 6 months, average of 4 months; the effective rates in month 3, 6, 9, 12 after treatment were 56.0%, 72.0%, 80.0% and 84.0% respectively. The AA children with age ≥ 5 years old, course of disease < 6 months and absolute neutrophil value ≥ 1.5 ×10(9)/L on 30 days after IST had good curative effect; the effective rate in AA children with age ≥ 5 years old, course of disease < 6 months, high or reverse ratio of CD4(+)/CD8(+) and absolute neutrophil value ≥ 1.5×10(9)/L after IST was higher than that in AA children with age < 5 years old, course of disease ≥ 6 months, normal ratio of CD4(+)/CD8(+) and absolute neutrophil value after IST < 1.5×10(9)/L (94.4% vs 57.1%, 90.4% vs 50.0%, 94.1% vs 62.5%, 94.1% vs 62.5%) (P < 0.05). The high effective rate was observed in AA children with decrease of CD55 and CD59 expression, but there was no significant difference (P > 0.05) as compared with normal expression of CD55, CD59. It is concluded that the treatment using r-ATG (3.5 mg/kg·d × 5 d) combined with CsA is a safe and effective for children with AA. Age, course of disease and absolute neutrophil value on 30 days after IST are the main factors affecting curative affect.
Adolescent ; Anemia, Aplastic ; drug therapy ; Antilymphocyte Serum ; administration & dosage ; therapeutic use ; Child ; Child, Preschool ; Cyclosporine ; administration & dosage ; therapeutic use ; Drug Therapy, Combination ; Female ; Humans ; Lymphocyte Count ; Lymphocyte Subsets ; Male ; Retrospective Studies ; Treatment Outcome

Effects of dietary supplementation with fructooligosaccharides on the excretion of nitrogen and phosphorus in Miichthys miiuy fries were investigated. Nine hundred Miichthys miiuy fries were divided into 3 groups, each with triplicates. The basal diet and the basal diet supplemented with carnitine groups were considered as the negative and positive controls respectively. Results showed that the nitrogen concentration in excreted feces decreased significantly in fries fed the diet supplementation with 1000 x 10(-6) fructooligosaccharides and 200 x 10(-6) carnitine (P<0.05). The ammonic-nitrogen concentration decreased significantly in the carnitine group only (P<0.05), indicating the decreasing tendency caused by the supplementation with fructooligosaccharides. Supplementation with both did not have significant effects on the concentration of phosphorus in feces of Miichthys miiuy fries.
Administration, Oral ; Animals ; Dietary Supplements ; Feces ; Fishes ; metabolism ; Nitrogen ; metabolism ; Oligosaccharides ; administration & dosage ; Phosphorus ; metabolism

OBJECTIVETo investigate the effects of phytoestrogens (daidzein and genistein) on the testosterone production of rat Leydig cells and the possible mechanisms.

METHODSPrimary Leydig cells were obtained from 3-month old male SD rats using discontinuous Percoll density gradient centrifugation. The effects of phytoestrogens at various concentrations were evaluated by ELISA, with hCG as the positive control. The mRNA expression of P450 side-chain cleavage enzyme (P450scc) was analyzed by semi-quantitative RT-PCR.

RESULTSGenistein at 0.1 micromol/L obviously promoted the secretion of testosterone and upregulated the mRNA level of P450scc. At a higher concentration of 5 micromol/L, however, both daidzein and genistein significantly inhibited the testosterone production of Leydig cells (P > 0.05).

CONCLUSIONGenistein can promote the testosterone production of Leydig cells at a low concentration (0.1 micromol/L), but both daidzein and genistein can inhibit it at a higher concentration ( >5 micromol/L).

Animals ; Cells, Cultured ; Genistein ; pharmacology ; Isoflavones ; pharmacology ; Leydig Cells ; drug effects ; secretion ; Male ; Phytoestrogens ; pharmacology ; Rats ; Rats, Sprague-Dawley ; Testosterone ; biosynthesis

Positional asphyxia of restraint means that when an individual was limited in an abnormal body position, asphyxia would take place owing to the disorder of spontaneous respiratory function, and finally it lead to die. So, it belongs to a special type of the mechanical asphyxia. From the cases reported, we could found that it would take place in several conditions. Because the cases were not caused enough recognition, the study has been researched carefully only in recent years. Following the more cases reported, many experts of forensic medicine had investigated it on the mechanism of death and the standard of identification, but they could not reach to agreements. So, they have changed the directions of the researches, began to value the factors of risk and research how to avoid it. In the following text, the mechanism of death, factors of risk, preventive methods, standard of identification and prospecting of positional asphyxia of restraint were reviewed.
Alcoholic Intoxication/physiopathology* ; Animals ; Asphyxia/prevention & control* ; Cause of Death ; Diaphragm/physiopathology* ; Expert Testimony/standards* ; Forensic Medicine ; Humans ; Muscle Fatigue ; Posture/physiology* ; Respiratory Mechanics ; Restraint, Physical/adverse effects*

【Objective】The aim of this study was to investigate the effect and mechanism of cabozantinib combined with anti-PD-L1 antibody on the growth of subcutaneous transplanted malignant melanoma in mice.【Methods】Established mouse subcutaneous xenograft model using mouse melanoma cell line B16- F10，and then randomly divided into five groups：saline control group，vehicle control group，anti PD- L1 antibody group，cabozantinib group，cabozantinib in combined with anti- PD- L1 antibody group （combination group）. Tumor growth was observed and tumor volume was measured every 2 days. The research endpoint was defined as when the tumor volume reached 2 000 mm3 or the difference between the groups was statistically significant. Then the mice were sacrificed and tissue samples were taken at the endpoint of the study. Infiltrating immune cells including CD4 + ，CD8 + T lymphocytes and myelogenous suppressor cells （MDSC）were detected by flow cytometry. In addition，B16-F10 cells cultured in vitro were treated with different drugs， the apoptosis was detected by flow cytometry ，and the protein expressions of AKT ，p-AKT ，mTOR and p-mTOR were detected by western blot assay.【Results】B16- F10 melanoma xenograft model showed that anti- PD- L1 antibody group had no obvious antitumor effect ，while both cabozantinib group and combination group produced significant antitumor effect，and the combination group had more obvious antitumor effect compared to cabozantinib group（P=0.001 5）. B16- F10 cells were treated with different drugs in vitro，and the apoptosis rate of the combination group was significantly higher than that of cabozantinib group at 24 h and 48 h，respectively（24 h:P=0.003 5；48 h:P=0.002 9）. Western blot assay showed that the combination group and cabozantinib group had no significant effect on the protein expression of AKT and mTOR，but both could reduce their phosphorylation levels，and the combination group was more remarkable. 【Conclusion】Cabozantinib in combined with anti-PD-L1 antibody had synergistic anti-tumor effect，which might be achieved by promoting B16-F10 cells apoptosis and inhibiting of AKT/mTOR pathway.

No abstract available.
Asian Continental Ancestry Group* ; Humans ; Keratin-17* ; Mutation, Missense* ; Steatocystoma Multiplex*

To investigate the methylation status of CpG islands of the secreted frizzled-related protein (SFRP) gene promoter region in malignant hematopoietic cell lines, and to explore the possible relationship of CpG abnormal methylation status with pathogenic mechanism of hematologic malignancies. Methylation-specific PCR was used to detect the status of SFRP gene promoter region in nine malignant hematopoietic cell lines and peripheral blood mononuclear cells from healthy people. The results indicated that hypermethylation of 2 genes coding for SFRP1 and 2 were present in nine malignant hematopoietic cell lines, however, methylation and unmethylation of SFRP4 were both detected in CA46, HL60 and U937 cell lines, and SFRP5 in U266 as well. None of the normal mononuclear cells showed methylation of SFRP 1-5 genes. It is concluded that the hypermethylation of SFRP genes is related to the evolution of malignant hematopoiesis. Methylation of SFRP genes may serve as potential independent biomarkers for early detection of hematologic malignancies.
Cell Line, Tumor ; CpG Islands ; DNA Methylation ; Hematologic Neoplasms ; genetics ; Humans ; Intercellular Signaling Peptides and Proteins ; genetics ; Membrane Proteins ; genetics ; Promoter Regions, Genetic ; Proto-Oncogene Proteins ; genetics

This study was aimed to investigate the efficiency of nested methylation specific polymerase chain reaction (nMS-PCR) for detecting the APC gene promoter methylation and to clarify the roles of methylation in genesis and development of hematologic malignancies, as well as to screen the hematologic malignant cell lines with hypermethylation of APC gene promoter to use as an ideal cell model for exploring the relationship between gen methylation and gene expression. The genome DNA of 10 cell lines modified with bisulfide was amplified and the methylation status of APC gene promoter was detected by using nMS-PCR. The results showed that the methylation of APC gene promoter was detected in Jurkat cells, while could not be detected in CA46, U266, Molt4, K562, HL-60, CEM, AKR, U937 and Raji cell lines. In conclusion, APC gene methylation in hematological malignant cell lines can be accurately detected by nMS-PCP method, which is simple method for detecting methylation status of various hematological malignant cell lines.
DNA Methylation ; Genes, APC ; HL-60 Cells ; Humans ; K562 Cells ; Polymerase Chain Reaction ; methods ; Promoter Regions, Genetic

BACKGROUNDBilirubin is the end product of heme catabolism and has strong antioxidant properties. Serum bilirubin levels are reported to be reduced in patients with multiple sclerosis (MS) and neuromyelitis optica (NMO). The pathophysiology of optic neuritis (ON) resembles that of MS; however, the role of endogenous bilirubin in ON is unclear. The aim of this study is to measure serum bilirubin levels in patients with ON, and to investigate the correlation between ON and serum antioxidant status of bilirubin.

METHODSSerum levels of bilirubin were measured in 42 patients with ON, 50 patients with multiple sclerosis (MS), 48 patients with neuromyelitis optica (NMO) and 48 healthy control subjects.

RESULTSSerum total bilirubin (Tbil), direct bilirubin (Dbil) and indirect bilirubin (Ibil) levels in patients with ON were significantly lower than those in the healthy controls. However, no statistical significance was found between levels in the ON and MS, ON and NMO, and MS and NMO groups. In patients with ON, serum Tbil, Dbil, and Ibil levels were lower in those with recurrence or those with ON for a longer duration (≥ 1 year). Moreover, Tbil, Dbil, and Ibil concentrations were lower in patients with papillitis than in those with retrobulbar type ON, but the differences were not statistically significant.

CONCLUSIONSLow antioxidant status may exist in patients with ON. But serum levels of Tbil, Dbil, and Ibil did not correlate with clinical presentations, such as recurrence, duration of disease and subtypes of ON. Low antioxidant status already existed in MS or NMO patients before systemic symptoms appeared.

Adolescent ; Adult ; Aged ; Antioxidants ; metabolism ; Bilirubin ; blood ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Multiple Sclerosis ; blood ; Neuromyelitis Optica ; blood ; Optic Neuritis ; blood ; Young Adult