Objective To investigate the relationship between that vascular endothelial growth factor (VEGF) expression and microvessel density (MVD) distribution were in the small intestine mucous membrane and that urea and creatinine were eliminated from intestine inrenal failure rat used Shenfushu and atropine treatment.Methods Sprague-Dawely (SD) rats with 5/6 kidney resection were randomly divided into Shenfushu treatment group,Shenfushu + atropine treatment group,pathological control group,and sham operation group for the normal controls.Treatment groups were drenched for eight weeks.Before and after treatment,the concentration of blood urea nitrogen (BUN),serum creatinine (Scr),the fecal urea and fecal creatinine were detected; the expression levels of VEGF and MVD in the small intestinal were detected with small intestinal biopsy immunohistochemical method.The relationship between that expression of VEGF and MVD,and that concentration of BUN,Scr,the fecal urea,and fecal creatinine was analyzed.Results VEGF in the Shenfushu treatment group,Shenfushu + atropine treatment group,and pathological control group was (20.72 ± 1.8) pu,(24.32 ± 1.54) pu,and (28.69 ± 1.82) pu,respectively; MVD was (274.27 ± 10.62)/mm2,(332.71 ± 10.96)/mm2,(436.42 ± 13.70)/mm2,respectively.Expressions of small intestinal VEGF and MVD in two treatment groups were increased significantly than the control group.The concentrations of BUN and Scr were significantly decreased with a negative correlation with the expressions of VEGF and MVD.The concentrations of fecal urea nitrogen and fecal creatinine were significantly increased with a positive correlation with the expressions of VEGF and MVD (P ＜0.05).The concentrations of BUN and serum Cr in the Shenfushu + atropine group were significantly changed more than Shenfushu group (P ＜ 0.05).Conclusions Shenshuaikang and atropine could enhance the expression of VEGF in the small intestinal mucosa,increase microvascular density,promote the elimination of urea nitrogen and creatinine from the intestinal tract to decrease the concentrations of BUN and Scr.Those effects were more obvious with two drugs in combination.

Objective To explore the clinical manifestations, electroencephalographic characteristics and therapeutic effect of drugs in children with Jeavons syndrome. Methods The clinical and electroencephalographic characteristics and thera-peutic effect of drugs were analyzed in 4 children with Jeavons syndrome. Results Among the four children there were 3 female and 1 male. The age at the onset of the disease was from 1 to 6 years. The typical clinical manifestations of this disease were brief, fast and repeated eyelid myoclonia (EM) with or without absence seizure. The typical electroencephalography (EEG) in two patients showed 3-6 Hz generalized spike and waves and polyspikes burst, and the eye closure and intermittent photic stimu-lation helped to induce discharges and clinical events. The typictal EEG in the other two patients showed 3.0-3.5 Hz generalizedδslow wave rhythm burst. The drugs of choice for treatment was sodium valproate monotherapy in two cases, levetiracetam in one case, sodium valproate combined with levetiracetam in one case. During the follow-up, seizures were controlled in one case, decreased in frequency in two cases and were still frequent in one case. Conclusions Jeavons syndrome is one of the idiopathic and generalized epileptic syndromes and characterized by EM with or without absence seizure. Video EEG monitoring plays an important role in the diagnosis of this disease. Sodium valproate and levetiracetam were effective for this disease.

Objective To construct a geometric skull model by using three-dimensional reconstruction, computer tomography scanning and rapid prototyping technology and evaluate its significance in treatment of complex oral and maxillofacial deformities. Methods A cranial and a goldenhar syndrome patient with complex oral and maxillofacial deformities bone received continuous volumetric scanning of skull and the data acquisition was done by an electron computed tomography, by which reconstruction was performed and the obtained images saved as STL files. Then the data were input into rapid prototyping machine to make three-dimensional geometric model. Direct measurement, designation and surgery simulation could be done on this three-dimensional model. Then the mandibular mirror physical model was manufactured using rapid prototyping according to the normal side. Results A computer-aided model according to CT data could represent the three-dimensional anatomic structures and their relationships precisely. The replica exhibited dimensional errors ranging 0.02 mm to 0.53 mm. Which provided strong basis for accurate understanding of disease status and reasonable surgical plans and helped improve the curative effect of surgery. Conclusion Rapid prototyping can help surgeons in many ways for therapy of the complex oral and maxillofacial deformities.

OBJECTIVE: To investigate the field planting of Bacillus bifidus and Bacillus acid lactic on mucosa membrane of gaster, jejunal, ileum, ascending colon, and descending colon in rats with renal failure, and to observe their decomposition of creatinine (Cr), urea nitrogen (UN),and uric acid (UA). METHODS: Forty SD rats were randomly divided into 4 groups. Group A was normal control group,and 10 of them were sham-operated. Thirty of them were operated with 5/6 nephrectomy. Group B was pathological control group. Group C were fed Bacillus bifidus and Group D were fed lactobacillus. After 1 week all rats were sacrificed as samples of blood, digestive juice and gastrointestinal mucosa were taken.Bacteria on the gastrointestinal mucosa were counted. The concentration of UN, Cr,and UA of blood and digestive juice was determined. RESULTS: The number of bacteria on the gastrointestinal mucosa of Group B was less than that of Group A (P<0.05), but that of Group C and D was more than that of Group A and B. The bacteria number on the gastric mucosa was least and that on the descending colon was most. There was significant difference in the bacteria number and concentration of Cr, UN,and UA in various sites of the gastrointestinal tract (P<0.05). The concentrations of Cr, UN, and UA in the digestive juice of various sites and serum in Group C and D were lower than those in Group B (P<0.05). Bacteria planting number in the digestive tract has obvious negative correlation with the concentration of Cr,UN, and UA in the blood and digestive tract. CONCLUSION Field planting of lactobacillns and Bacillus bifidus, and the concentration of low molecule urotoxin are different in various sites of the gastrointestinal tract. It can decrease the concentration of BUN,Cr,and UA in rats with renal failure by feeding lactobacillus and Bacillus bifidus.
Animals ; Bifidobacterium ; growth & development ; metabolism ; Blood Urea Nitrogen ; Creatinine ; metabolism ; Gastrointestinal Tract ; microbiology ; Kidney Failure, Chronic ; etiology ; metabolism ; microbiology ; Lactobacillus ; growth & development ; metabolism ; Male ; Nephrectomy ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Uric Acid ; metabolism

The fruit fly, Drosophila melanogaster, is able to discriminate visual landmarks and form visual long-term memory in a flight simulator. Studies focused on the molecular mechanism of long-term memory have shown that memory formation requires mRNA transcription and protein synthesis. However, little is known about the molecular mechanisms underlying the visual learning paradigm. The present study demonstrated that both spaced training procedure (STP) and consecutive training procedure (CTP) would induce long-term memory at 12 hour after training, and STP caused significantly higher 12-h memory scores compared with CTP. Label-free quantification of liquid chromatography-tandem mass spectrometry (LC-MS/MS) and microarray were utilized to analyze proteomic and transcriptomic differences between the STP and CTP groups. Proteomic analysis revealed 30 up-regulated and 27 down-regulated proteins; Transcriptomic analysis revealed 145 up-regulated and 129 down-regulated genes. Among them, five candidate genes were verified by quantitative PCR, which revealed results similar to microarray. These results provide insight into the molecular components influencing visual long-term memory and facilitate further studies on the roles of identified genes in memory formation.
Animals ; Conditioning (Psychology) ; physiology ; Drosophila Proteins ; genetics ; metabolism ; Drosophila melanogaster ; genetics ; metabolism ; physiology ; Flight, Animal ; physiology ; Gene Expression Profiling ; methods ; Memory ; physiology ; Oligonucleotide Array Sequence Analysis ; Proteomics ; methods ; Time Factors ; Vision, Ocular ; physiology

OBJECTIVETo explore the clinical and gene mutation characteristics of children with peroneal myoatrophy FGD4 mutations.

METHODThe clinical data of a patient with peroneal myoatrophy with novel FGD4 gene mutations were collected, the related literature was searched from China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform, National Center for Biotechnology Information and PubMed (up to December 2014) by using search terms"muscular disorders, atrophic"peripheral nervous system diseases"genes". The clinical features and treatment of the patients with FGD4 gene mutations were studied.

RESULTThe patient was a 10-years-old boy, he was presented to our clinic due to lower extremity weakness for 3 years, worsening for one year with normal family history and birth history. When he was 6 years old, his feet turned inward as he walked, at 7 years of age, his toes pointed toward the ground, the heel could not touch the ground, the right foot was more serious. During the recent year his symptoms were worsened, manifested as clubfoot, foot drop, arched feet, crane legs, difficult in squatting, walking with swaying gait, easy to fall. He was brought to a number of domestic general hospitals' neurology clinic, he was clearly diagnosed as peroneal myoatrophy, but failed to make typing. Electromyography (EMG) showed neurogenic damage (peripheral neuropathy - motor and sensory fibers are involved). Target gene sequencing showed that the child had FGD4 genes compound heterozygous mutation: c. 338A> G and c. 1730G> A, where the former was inherited from his father, the latter inherited from his mother, it was a new mutation unreported previously. Literature search retrieved six reports (all in English literature) with FGD4 10 cases with mutations, which were expressed as peroneal myoatrophy, but were homozygous mutation.

CONCLUSIONThis study found the FGD4 4th and the 14th exons' c. 338A> G and c. 1730G> A heterozygous mutations, and this mutations may lead to peroneal myoatrophy.

Atrophy ; Child ; China ; Exons ; Heterozygote ; Humans ; Male ; Microfilament Proteins ; Muscular Diseases ; Mutation

Objective This study was aimed to investigate the effects of ω-3 polyunstaurated fatty acids (ω-3 PUFAs) on the growth of gastric cancer cells in nude mice,and to find whether the Ros homolog gene Rho-associated coiled-coil containing protein kinase 1 (RHO-ROCK1) signaling pathway is involved.Methods 16 BALB/C nude mice were injected subcutaneously with SGC7901 gastric cancer cells to establish the tumor-bearing mouse model.The mice were randomized:control group (normal saline) and intervention group (ω-3 PUFAs).The mRNA expression of Ros homolog gene family,member A (RHOA),RHOC,and ROCK1 in tumor tissue were detected by quantitative polymerase chain reaction (qPCR).Immunofluorescence and Western blot were used to detect RHOA,RHOC,and ROCK1 protein expression.Results The volume and weight of the tumors in the ω-3 PUFAs group were slightly smaller than that in the control group (P ＞ 0.05).Compared to the control group,hematoxylin and eosin staining showed multifocal tumor necrosis in the ω-3 PUFAs group,while the tumors of the control group showed abundant blood supply.qPCR and Western blot showed that the mRNA and proteins expression of RHOA and ROCK1 in the ω-3 PUFAs group was significantly lower than those in the control group (P ＜ 0.05).The immunofluorescence redults also showed that the expression of these proteins in the ω-3 PUFAs group was slightly lower than that in the control group.Conclusions These results suggested that ω-3 PUFAs may affect the growth of gastric cancer in nude mice by affecting the expression of RHOA,RHOC and ROCK1,thus inhibiting the excessive proliferation of gastric cancer cells and leading to tumor necrosis.

The clinical data of a case with late-onset isolated sulfite oxidase deficiency(ISOD)admitted in the Department of Neurology, Children′s Hospital, Zhejiang University School of Medicine in July 2021 were retrospectively analyzed.Fifteen previously published cases of late-onset ISOD were also reviewed.The patient was a girl, who was hospitalized because of " motor regression with mental retardation for 5 days" at 1 year old.The manifestations of the patient were extrapyramidal symptoms, regression of motor development and seizures.The level of urinary sulfites in the patient was increased.Magnetic resonance imaging (MRI) features were bilateral pallidus and substantia nigra.Gene sequencing suggested a pure missense mutation of the sulfite oxidase( SUOX) gene c. 650(exon5)G>A(p.Arg217Gln). In 16 cases of late-onset ISOD, the median age at onset and diagnosis was 10.5 months and 34.0 months, respectively.The common clinical manifestations were hypotonia (13 cases), seizures (10 cases), movement disorders (9 cases), and ectopia lentis (6 cases). The most common brain MRI feature was pallidus changes (11 cases), followed by lesions of substantia nigra (5 cases), and cerebral atrophy (4 cases). Fourteen cases of late-onset ISOD showed a positive urinary sulfite test.The missense mutation of the SUOX gene was found in 9 cases.It suggested that brain MRI involvement of bilateral pallidus, high excretion of urine sulfites and the missense mutation of the SUOX gene were important diagnostic clues for late-onset ISOD.

Objective:To investigate the clinical features and treatment effect of children with central nervous system demyelinating diseases and seropositivity to myelin-oligodendrocyte glycoprotein (MOG) antibody.Methods:The clinical characteristics of 28 had seropositivity to MOG among 115 children with central nervous system demyelinating diseases and who were hospitalized at Department of Neurology, Children′s Hospital of Zhejiang University School of Medicine from March 2017 to February 2019 were retrospectively analyzed.Results:Twenty-eight patients were included in this study, including 10 males and 18 females, with the ratio of male/female of 1.00∶1.80, and the median age of 7 years and 9 months.The clinical manifestations were diverse, including encephalopathy symptoms such as hea-dache, vomiting, and drowsiness (13/28 cases), vision loss (7/28 cases), spinal symptoms (6/28 cases), cerebellar symptoms such as ataxia, slurred speech (4/28 cases), convulsions (2/28 cases), and cranial nerve symptoms (1/28 cases). Among 24 cases who underwent CSF detection, 10 patients (41.7%) had slightly increased white blood cells, 2 patients (8.3%) had elevated protein, 6 patients (25.0%) had positive MOG antibody, and CSF-restricted oligoclonal band was negative in all 24 patients.Twenty-five cases (89.3%) showed brain magnetic resonance imaging (MRI) abnormalities, including cerebral white matter (20/28 cases), cerebellum (10/28 cases), cerebral gray matter (9/28 cases), thalamus/basal ganglia (6/28 cases), brainstem (6/28 cases), optic nerve (5/28 cases), and corpus callosum (4/28 cases). Of the 28 cases, 13 patients had spinal cord involvement, involving cervical spinal cord in 10 cases, thoracic cord in 9 cases and lumbar spinal cord in 5 cases; besides, 8 cases of them had long segmental spinal cord lesions with ≥ 3 segments.Fourteen patients received the visual evoked potentials′ examination, and the subclinical visual impairment was found in 2 of them with unobstructed clinical performance.All patients underwent high-dose Methylprednisolone therapy.The clinical symptoms of 16 patients who were treated with Gamma globulin were relieved in the acute phase.Seven patients had recurrence during the follow-up period, with the recurrence rate of 25.0%.Relapsed patients re-treated with high-dose Methylprednisolone therapy combined with Gamma globulin, clinical symptoms could be alleviated.Conclusion:The main clinical phenotype of children with central nervous system demyelinating diseases and seropositivity to MOG is acute disseminated encephalomyelitis.The spinal cord lesions are mainly involving cervical and thoracic segments.The current treatments of this disease include glucocorticoid and Gamma globulin, which have significant effect, but the disease is easy to relapse.The re-use of glucocorticoid and Gamma globulin after relapse is still effective.