Objective To identify the effects of the subthalamic nuclei(STN)high frequency stimulation(HFS)on striatal and nigral dopaminergic metabolism in rats.Methods The effects of subthalamie nuclei high frequency stimulation(STN-HFS)on striatal dopaminergie metabolism was investigated in free moving rats.Reverse transcriptase polymerase chain reaction(RT-PCR)and Western blot of striatal and nigral tyrosine hydroxyiase(TH)were performed.Results Our data suggest that STN- HFS elevated TH protein(0.99?0.14 vs 0.33?0.08,P

Methylacetoacetyl-CoA thiolase deficiency (T2 deficiency) is a rare congenital and metabolic disease affecting the ketone body and isoleucine metabolism. The typical symptoms are refractory metabolic acidosis, in which large amounts of 2-methyl-3-hydroxybutyry1 carnitine, 2-methyl-3-hydroxybutyrate and tiglylglycine are often detected in the blood and urine. We herein describe an atypical case of T2 deficiency with a high level of 3-hydroxybutyrate and a low level of 2-methyl-3-hydroxybutyrate in the urine. Such a case was diagnosed by urinary organic analysis in combination with gene mutation evaluation. Organic acids in the urine were measured using a gas chromatography mass spectrometer and all exons were sequenced via deep sequencing. Molecular biology analysis confirmed the presence of a homozygous mutation in the acetyl-CoA acetyltransferase 1 (ACAT1) gene. The patient received a special diet of deeply hydrolyzed protein milk powder and raw corn starch. She was followed about 6 months. There were no ketoacidotic episodes and hypoglycemia even when she had fever. In conclusion, patients with atypical features of T2 deficiency should also be investigated early. Gas chromatography mass spectrometry and next-generation full exome sequencing may be helpful in diagnosis.

The purpose of this study was to observe the ultrastructure of human umbilical cord mesenchymal stem cells (hUCMSC). hUCMSC from full-term newborn umbilical cord were isolated and cultured by collagenase digestion, and then subcultured, amplification, and cell morphology was observed by microscopy. The immunophenotype and trilineage differentiation potential of hUCMSCs at passage 3 were analyzed. Transmission electron microscopy and scanning electron microscopy were used to observe the ultrastructure of hUCMSC. The results indicated that appearance of hUCMSC was spindle-shaped and polygonal, and nuclei were observed. hUCMSC expressed immunophenotype CD44, CD73, CD105, did not express CD34, CD45, CD31 and human leukocyte antigen HLA-DR. hUCMSC were capable of adipogenic, osteogenic, and cartilage differentiation; the short and thick microvilli processes were seen at the surface of hUCMSC by scanning electron microscope. Two different cell morphologies of hUCMSC were seen under transmission electron microscope, the one was a quiescent period in which a large and round or oval nucleus only one nucleolus were seen, cytoplasmic organelles were less; the other was in a relatively active period in which one or two nuclei in the same one cell were observed, the organelles were rich, structure was clear, expansion of the mitochondria was visible. It is concluded that the cells successfully isolated and cultured from umbilical cord, which possess biological characteristics of MSC and display two different states of ultrastructure.
Cells, Cultured ; Humans ; Mesenchymal Stromal Cells ; cytology ; ultrastructure ; Microscopy, Electron, Scanning ; Microscopy, Electron, Transmission ; Umbilical Cord ; cytology

The study was to investigate the role of homocysteine (Hcy) which was released by hippocampal glial cells and its relationship with NMDA receptor and AMPA receptor in depression induced by chronic unpredictable mild stress (CUMS), and explore the mechanism of changes of Glu/Glu receptor in glial cells and neurons. CUMS-induced depression model was established. The body weight of rats was weighed on the 1st, 7th, 14th, and 21st days during the experiment. The behavioral performances were observed by means of sucrose consumption test, open field test and tail suspension test. Intrahippocampal microinjection of Hcy, NMDA receptor antagonist MK-801 and AMPA receptor antagonist NBQX was performed under stereotaxic guide cannula. The concentration of Glu and the expression of its receptors' subunits were detected respectively by high performance liquid chromatography (HPLC) and Western blot. The Hcy content and the levels of phosphorylation of NMDA receptor and AMPA receptor in hippocampus were separately determined by enzyme linked immunosorbent assay (ELISA). The results showed that CUMS significantly induced the depression-like behaviors in rats, and the content of Glu and Hcy, the expression of NMDA receptors' subunits NR1/NR2B and the level of phosphorylation of NMDA receptor (p-NMDAR) in hippocampus increased significantly, while the expression of AMPA receptors' subunits GluR2/3 and the level of phosphorylation of AMPA receptor (p-AMPAR) decreased significantly. Microinjection of Hcy into hippocampus resulted in similar animal depression-like behaviors and increased Glu content compared to the CON/SAL group, the expression of NR1/NR2B/GluR2/3 and the level of p-NMDAR increased significantly, but the level of p-AMPAR reduced observably. Intrahippocampal injections of MK-801 effectively improved the depression-like behaviors induced by CUMS and Hcy, and attenuated the elevation of Glu content induced by Hcy in hippocampus, whereas NBQX could not improve the depression-like behaviors, but also decreased the Glu content induced by Hcy remarkably. These results suggest that CUMS may contribute to the production and release of Hcy via hippocampal astrocytes. Through the increase of expression of NR1/NR2B/GluR2/3 and level of p-NMDAR, and the decrease of level of p-AMPAR, Hcy results in elevation of Glu level, which leads to depression-like behaviors in the end. In a word, the Hcy released by astrocytes plays an important role in stress-induced elevation of Glu content and variation of NMDA/AMPA receptors in hippocampus.
Animals ; Behavior, Animal ; Depression ; metabolism ; Dizocilpine Maleate ; pharmacology ; Glutamic Acid ; metabolism ; Hippocampus ; metabolism ; Homocysteine ; pharmacology ; Rats ; Rats, Sprague-Dawley ; Receptors, AMPA ; metabolism ; Receptors, N-Methyl-D-Aspartate ; metabolism ; Signal Transduction ; Stress, Psychological

BACKGROUNDThe major neuropathological symptoms of Parkinson's disease (PD) consist of a loss of pigmented dopaminergic neurons in the substantia nigra and the presence of Lewy bodies. This study was to investigate the effects of bilateral subthalamic nucleus (STN) stimulation on resting-state cerebral glucose metabolism in advanced PD, and investigate the mechanism of deep brain stimulation (DBS).

METHODSSeven consecutive advanced PD patients (4 men and 3 women, mean age 64 +/- 4 years, mean H-Y disability rating 4.4 +/- 0.65) receiving bilateral STN DBS underwent 18F-fluorodeoxyglucose (18F-FDG)/positron-emission tomography (PET) examinations at rest both preoperatively and one month postoperatively, with STN stimulation still on. The unified PD rating scale was used to evaluate the clinical state under each condition. Statistical parametric mapping (SPM) was used to investigate the regional cerebral metabolic rates of glucose (rCMRGlu) during STN stimulation, and to compare these values to rCMRGlu preoperation.

RESULTSSTN stimulation clearly improved clinical symptoms in all patients. A significant increase in rCMRGlu was found in the bilateral lentiform nucleus, brainstem (midbrain and pons), bilateral premotor area (BA6), parietal-occipital cortex, and anterior cingulated cortex, and a marked decrease in rCMRGlu was noted in the left limbic lobe and bilateral inferior frontal cortex (P < 0.05).

CONCLUSIONBilateral STN stimulation may activate the projection axon from the STN, improving clinical symptoms in advanced PD patients by improving both ascending and descending pathways from the basal ganglia and increasing the metabolism of higher-order motor control in the frontal cortex.

Brain ; metabolism ; Electric Stimulation ; Female ; Glucose ; metabolism ; Humans ; Male ; Middle Aged ; Parkinson Disease ; metabolism ; Subthalamic Nucleus ; physiology

OBJECTIVETo analyze the characteristics of cervical minimal deviation adenocarcinoma (MDA) and the methods of diagnosis and treatment.

METHODSA retrospective study was carried out to evaluate the clinical and pathological data of 15 patients with MDA treated from 1992 to 2007.

RESULTSThe average age of the 15 patients was 42.3 years. The main symptoms were increased discharge and irregular vaginal bleeding. Preoperative Pap smears showed adenocarcinoma in 3 cases (27.3%). The diagnosis of MDA was confirmed in 8 cases by cervical punch biopsies (53.3%) and 2 cases by conization. Several cysts were noted in sections of the endocervix. Microscopic examination showed glands irregular in size and shape. However, the deviation of tumor cells was minimal. Immunohistochemistry revealed positive expression of CEA and alpha-SMA. The mean follow-up time was 51.0 months. The overall 5-year survival rate was 85.7%. Four cases experienced recurrence in the vagina and pelvis at 2 years after operation. Three cases died of the disease relapse with an average survival time of 36.3 months.

CONCLUSIONCervical minimal deviation adenocarcinoma is rare, with minimal deviation of cell shape from the normal cervical cells and difficult in diagnosis. A deep biopsy or conization is necessary when punch biopsy is not sufficient for diagnosis. Immunohistochemistry is helpful to make an accurate diagnosis. Surgery is the first choice for cervical minimal deviation adenocarcinoma. Radiotherapy and/or chemotherapy should be given if needed. The prognosis can be improved if a proper treatment plan is carried out.

Actins ; metabolism ; Adenocarcinoma ; diagnosis ; pathology ; therapy ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Carcinoembryonic Antigen ; metabolism ; Cervix Uteri ; pathology ; Chemotherapy, Adjuvant ; Cisplatin ; administration & dosage ; Conization ; Epirubicin ; administration & dosage ; Female ; Fluorouracil ; administration & dosage ; Follow-Up Studies ; Humans ; Hysterectomy ; methods ; Middle Aged ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Papanicolaou Test ; Radiotherapy, Adjuvant ; Retrospective Studies ; Survival Rate ; Uterine Cervical Neoplasms ; diagnosis ; pathology ; therapy ; Vaginal Smears

This study was aimed to investigate the curative effect and safety of human umbilical cord mesenchymal stem cells (hUCMSC) to treat acute graft-versus-host disease (aGVHD) of children after hematopoietic stem cell transplantation (HSCT). HUCMSC were isolated and cultured by collagenase digestion and passage culture. The 3rd to the 5th passage of hUCMSC were used for clinical treatment. Five cases of children acute leukemia achieved complete remission after chemotherapy. Two cases received HLA 3/6 loci matched haploidentical bone marrow HSCT. One case received HLA-matched sibling bone marrow and peripheral blood HSCT. One case received unrelated HLA 4/6 loci matched umbilical cord blood HSCT. One case received unrelated HLA 5/6 loci matched umbilical cord blood HSCT. The children received immunosuppressive therapy after III-IV aGVHD occurring. They received 0.5×10(6)/kg hUCMSC infusion when conventional therapy was ineffective. The results showed that 5 cases of children acute leukemia achieved hematopoietic reconstitution and developed the III-IV grade aGVHD. The five cases of children were infused with hUCMSC. The rash subsided, the liver function was normalized and the gastrointestinal symptoms were improved. The infusion-related adverse reaction did not happen. At present, the 5 children are in remission. It is concluded that allogeneic HSCT is an effective therapeutic method for children with acute leukemia. HUCMSC infusion can be safely and effectively used for the treatment of refractory aGVHD.
Child ; Child, Preschool ; Cord Blood Stem Cell Transplantation ; Female ; Graft vs Host Disease ; therapy ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Male ; Mesenchymal Stem Cell Transplantation ; Treatment Outcome

This study was purposed to culture murine compact bone-derived mesenchymal stem cell (MSC) and analyze the immunological and trilineage differentiation potential. Tibia and femur were extracted. Bone marrow cells were flushed out and compact bone fragments were digested with collagenase. The digested cells were cultured in 6-well plates. The immunophenotype, immunosuppressive function and trilineage differentiation potential were analysed by flow cytometry, mixed lympocyte reaction and Oil red O, von Kossa and alcian blue straining, respectively. The results indicated that the pure compact bone MSC could be isolated with in 3 weeks. The resulting MSC had trilineage differentiation potential and immunosuppressive effect on mixed lymphocyte reaction. The count per minute (CPM) value in control group of BALB/c T cells cocultured with irradiated C57BL/6 T cells was (2.56 ± 0.31) × 10(4), while CPM values of mixed lymphocyte cocultured with C57BL/6 compact bone MSC at ratios of 100:1 and 10:1 were (0.47 ± 0.12) × 10(4) and (0.28 ± 0.09) × 10(4). The CPM value of control group was higher than those of MSC cocultured group (P < 0.001). Compact bone-MSC had an immunosuppressive effect on mixed lymphocyte reaction in a dose dependent manner. It is concluded that murine compact bone has rich MSC and the primary MSC is contaminated with less hematopoietic cells. Murine compact bone-MSC have immunosuppressive effect on mixed lymphocyte reaction and trilineage differentiation potential. Compact bone-MSC have promising experimental study value.
Animals ; Bone Marrow Cells ; cytology ; immunology ; Bone and Bones ; cytology ; Cells, Cultured ; Female ; Immunophenotyping ; Lymphocyte Culture Test, Mixed ; Mesenchymal Stromal Cells ; cytology ; immunology ; Mice ; Mice, Inbred BALB C ; Mice, Inbred C57BL

BACKGROUNDPolycystic ovary syndrome (PCOS) is considered to be the most common endocrine disorder in women of reproductive age. The involvement of an abnormal menstrual cycle in the etiology of PCOS remains unclear. We aimed to analyze the characteristics of abnormal menstrual cycles and their association with PCOS in community and hospital patient populations.

METHODSWomen with PCOS identified from 2111 permanent female residents in the community of Beijing and 506 outpatients obtained from the reproductive clinic of Peking University Third Hospital were recruited for this study, comprising the PCOS community group and the PCOS hospital group, respectively. Each group was further divided into four subgroups according to the length of menstrual cycles: <21 days; 21-34 days; 35-60 days; and >60 days. Women in each group were interviewed using a questionnaire to assess factors including age, age of menarche, menstrual cycle history, related family history, and modified Ferriman-Gallwey (mF-G) score. All women received transvaginal ultrasound scan and had fasting blood samples taken for endocrine evaluation. A two-tailed P value of <0.05 was considered significant.

RESULTSIn the community population, the prevalence of abnormal menstrual cycle was 27.19% (574/2111). The prevalence of PCOS in the community was 6.11% (129/2111) according to Rotterdam criteria. In the community group, the most common menstrual cycle length was 35-60 days, whereas for the hospital group, it was >60 days. In both the community and hospital groups, the most common phenotype of PCOS was that of Oligo/amenorrhea+PCO+ hyperandrogenism (HA) (O+P+H) (P=0.000). With increasing cycle length of 35-60 days to >60 days, the percentages of Oligo/amenorrhea+PCO (O+P) and O+P+H were found to significantly decrease in the community group and significantly increase in the hospital group (P=0.000 for each). In the hospital group, as the menstrual cycle length increased from 35-60 days to >60 days, the rate of spontaneous abortion increased significantly (P=0.000), meanwhile the rate of poorly-secreted endometrium and abnormal endometrial hyperplasia increased significantly (P=0.000).

CONCLUSIONSThe prevalence of PCOS in the Beijing community of women was 6.11%. Oligo/amenorrhea was the most common type of abnormal menstrual cycle and may be an indicator for PCOS and endometrial lesions. Gynecologists should seek relevant medical information from women in the community to promptly diagnose PCOS and then follow up patients for potential development of subsequent complications.

Adult ; Endometrial Hyperplasia ; diagnosis ; physiopathology ; Female ; Humans ; Menstrual Cycle ; physiology ; Middle Aged ; Polycystic Ovary Syndrome ; diagnosis ; physiopathology

Floating-Harbor syndrome (FHS) is an autosomal dominant genetic disease caused by SRCAP mutation. This article reports the clinical features of a boy with FHS. The boy, aged 11 years and 7 months, attended the hospital due to short stature for more than 8 years and had the clinical manifestations of unusual facial features (triangularly shaped face, thin lips and long eyelashes), skeletal dysplasia (curvature finger), expressive language disorder, and retardation of bone age. Genetic detection revealed a novel heterozygous mutation, c.7330 C>T(p.R2444X), in the SRCAP gene. The boy was diagnosed with FHS based on these clinical manifestations and gene detection results. FHS is rare in clinical practice, which may lead to missed diagnosis and misdiagnosis, and gene detection may help with the clinical diagnosis of FHS in children.
Abnormalities, Multiple ; Adenosine Triphosphatases ; Child ; Craniofacial Abnormalities ; Growth Disorders ; Heart Septal Defects, Ventricular ; Humans ; Male