Objectives:To observe the supporting effect of blenderized diet on the nulritional statusin the old patients. Methods:Twenty four patients were given blenderized diet made in our department as nutrition support.The nutritional assessments were preformed before and after the support. Results:The serum levels of albumin and phosphorus and the total lymphocyte count were increased after the support( P

BACKGROUND: Homogenate meals, as a replacement of normal diet, can be used in either hospitals or families. The homogenate meal as nutritious supplements should contain multiple components when it is applied in senior patients with chronic diseases because of the longterm application and complex complications.OBJECTIVE: To investigate the impact of longterm tube feed of finished product of homogenate meal on the nutritious supplements of senior patients.DESIGN: A cross-sectional study based on diagnosis.SETTING, PARTICIPANTS and METHODS: Totally 24 senior patients with chronic diseases who had been used self-made finished products of homogenate meal for more than 1 year were selected for nutritious supplement from Guangzhou General Hospital of Guangzhou Military Area Command of Chinese PLA. The changes of nutritious index at before and 4, 8 and 12 months after supplement were observed.MAIN OUTCOME MEASURES: Anthropometric measurement index and laboratorial index of 24 senior patients with chronic diseases before and after intervention were compared.RESULTS: Serous albumin at before nutritious supplements, and 4, 8 and 12 months after supplements were[ (36.4 ± 4.03), (36.60 ± 4.42),(38.20 ± 3.77), and(40.40 ± 3. 33) ] g/L respectively. Phosphorus and peripheral lymphocytes were significantly heightened while blood sugar began to decrease since 4 months after intervention, which all had significant differences compared with that of before intervention( t =2. 196 -2. 356,P <0.05; t=2.958-3.431. P <0.01) .Anthropometric measurement index also improved compared with that of before intervention.CONCLUSION: Longterm application of finished product of homogenate meal can improve the nutritious status of senior patients with chronic diseases.

Aim To explore the effects of hydrocortisone on intracellular calcium in microglial cells.Methods The intracellular calcium was measured by instantaneous scanning with confocal laser microscope(CLM) in BV-2 cells, and fluo3-AM was used to dye the intracellular calcium.Results Both hydrocortisone and nicotine could obviously increase intracellular calcium in BV-2 cells(P<0.05).It was indicated by instantaneous scanning with CLM that hydrocortisone induced the rising of intracellular calcium immediately, and reached the peak about at the fifteenth second, and sustained for 10 seconds, then declined to baseline at 200th second.The effect of hydrocortisone on intracellular calcium exhibited a highly consistency with nicotine.Antagonist of glucocorticoid receptors RU486 could not abolish the rising of intracellular calcium induced by hydrocortisone(P>0.05);but the blocker of α7 nicotinic acetylcholine receptor(α7nAChR) methyllycaconitine could suppress the rising of intracellular calcium induced by hydrocortisone(P<0.05).Conclusion Hydrocortisone enhances intracellular calcium via α7nAChR in microglial cells, which not only demonstrates the non-genomic effect of glucocorticoid, but also suggests that glucocorticoid could serve as endogenous ligand of α7nAChR.

Objective:To analyze the clinical and endoscopic findings of non-variceal upper gastrointestinal bleeding (NVUGIB) in children and to evaluate the efficacy of endoscopic treatment.Methods:A total of 56 children with NVUGIB admitted to Children′s Hospital Affiliated to Nanjing Medical University from May 2013 to April 2018 were enrolled.After admission, they were treated with endoscopic hemostasis, and they were divided into hematemesis group(8 cases), melena group(31 cases), and hematemesis + melena group(17 cases). In every group, hemoglobin level, blood transfusion rate, helicobacter pylori infection rate, source of hemorrhage, microscopic grading, hemostasis and results were statistically analyzed which they were compared with domestic and foreign studies.Results:In terms of hemoglobin level, hematemesis group was (92.00±25.66) g/L, melena group was (70.29±19.08) g/L, hematemesis + melena group (65.12±12.62) g/L.The differences among the 3 groups were statistically significant ( F=363.301, P<0.01). For blood transfusion rate, 25.00%(2/8 cases) was in hematemesis group, 74.19%(23/31 cases) was in melena group, and 94.12%(16/17 cases) was in hematemesis + melena group.There were significant differences among the 3 groups ( χ2=13.286, P=0.002). Totally, 50 cases (89.28%) were infected with Helicobacter pylori, and there were no significant differences among the 3 groups ( χ2=2.315, P=0.314). About bleeding source, 45 cases (80.35%) suffered from duodenal bulbar ulcer, 8 cases(14.28%) experienced gastric ulcer, 3/56 cases (5.35%) had gastric duodenal compound ulcer, and there were 25 cases(44.64%) with severe digestive tract bleeding.Forrest grade Ⅰa 2/56 cases [3.57%, 2/2 cases of rebleeding (100%)], Ⅰb 10/56 cases [17.85%, 2/10 cases of rebleeding (20%)], Ⅱa 3/56 cases (5.35%), Ⅱb 4/56 cases (7.14%), Ⅱc 2/56 cases (3.57%), and Ⅲ 35/56 cases (62.5%). Forty-eight cases (85.71%) accepted injection hemostasis, 2 cases (3.57%) obtained titanium clip hemostasis, 2 cases (3.57%) had injection + titanium clip hemostasis, 2 cases (3.57%) performed injection + titanium clip + electricity hemostasis, and 2 cases (3.57%) were given injection and surgical hemostasis.Totally, 52 cases (92.85%) achieved successful endoscopic hemostasis, 2 cases (3.57%) had successful second hemostasis, and 2 cases performed surgical operation (3.57%). Conclusions:In children with NVUGIB who need endoscopic hemostasis, duodenal ulcer is the main resource, Helicobacter pylori is the main cause.Children with melena are more likely to have severe bleeding and higher transfusion rate.Endoscopy is the preferred method for diagnosis and treatment.For Forrest Ⅰa and Ⅰb, the conbination of hemostasis under endoscope is more effective.

Objective Small cell neuroendocrine carcinoma of the ovary is a kind of ovarian cancer with a very low incidence.Its clinical manifestations are not obvious.The diagnosis should be based on the pathology and neuroendocrine indicators, and its primary nature should be determined.The main treatment is operation combined with platinum based chemotherapy.The survival period is related to clinical stage and treatment plan.The patient was hospitalized for 2 days because of the aggravation of abdominal distention and pain for half a year.The diagnosis of adnexal mass was confirmed by pathology.After three cycles of neoadjuvant chemotherapy (etoposide+ cisplatin), the patients underwent abdominal " total hysterectomy+ greater omentum resection+ appendectomy+ right pelvic wall peritoneal biopsy+ mesenteric biopsy" . After the operation, the patients received three cycles of EP chemotherapy, and they have been followed up for 15 months.

A 2-year- and 2-month-old girl developed recurrent eczema-like rashes 7 days after birth, followed by the occurrence of poikiloderma and hair loss. Cholestasis occurred at the age of 1 month and 10 days, which was improved but serum transaminase levels were elevated after 4 months. The patient usually presented with slight sweating, heat intolerance, and delayed gross motor development. Skin examination showed generalized mottled hypo- and hyper-pigmented patches, especially in the exposed areas, and sparse hair and eyebrows. Her parents had no similar clinical manifestations. Whole-exome sequencing showed a mutation c.1883G>A (p.Ser628Asn) in the FAM111B gene in the child, which was not found in her parents. According to the typical skin lesions, abnormal liver function and genetic testing results, this patient was diagnosed with hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis, and the mutation c.1883G>A in the FAM111B gene may be the cause of the patient′s clinical manifestations. The patient received hepatoprotective therapy, sun screen intervention, rehabilitation training, etc. After 10-month follow-up, the patient still presented with skin lesions and elevated transaminases, but without other discomforts.

OBJECTIVE: To explore the clinical features and genetic basis of a patient with glycogen storage disease type VI (GSD-VI). METHODS: Clinical data of the patient was collected. Genomic DNA was extracted from peripheral blood samples of the proband and his parents. Genetic variants were detected by using whole exome sequencing. Candidate variants were verified by Sanger sequencing followed by bioinformatics analysis. RESULTS: The proband presented fasting hypoglycemia, hepatomegaly, growth retardation, transaminitis, metabolic acidosis and hyperlactatemia. Liver biopsy indicated GSD. Novel compound heterozygous PYGL gene variants (c.2089A>G/c.158_160delACT) were detected in the proband. Compound heterozygosity was confirmed by Sanger sequencing of the patient's genomic DNA. Provean and MutationTaster predicted the two variants as deleterious and the variant sites are highly conserved. CONCLUSION The compound heterozygous variants (c.2089A>G/c.158_160delACT) of PYGL gene probably underlay the GSD in the patient. The two novel variants have expanded the spectrum of PYGL gene variants and provided the basis for genetic counseling of the family.
Child ; Family ; Genetic Testing ; Glycogen Storage Disease Type VI/genetics* ; Humans ; Mutation ; Whole Exome Sequencing

Objective : To explore the effects of autophagy activation on propofol⁃induced apoptosis in hippocampal neurons. Methods : Primary hippocampal neurons were extracted and isolated from fetal rats and randomly divided into control group, propofol group, rapamycin group and chloroquine group. 2,3⁃Bis⁃(2⁃methoxy⁃4⁃nitro⁃5⁃sulfo phenyl) Ⅳ2H⁃tetrazolium⁃5⁃carboxanilide ( XTT) and flow cytometry were used to detect apoptosis, and Western blot was used to detect the expression of autophagy and apoptosis⁃related proteins. Then, aged rats were randomly divided into control group, propofol group, rapamycin group and chloroquine group. 100 mg/kg propofol was used for continuous anesthesia for 1 week, during which 50 mg/kg rapamycin and 10 mg/kg chloroquine were treated. Morris water maze was used to detect cognitive function, TUNEL staining was used to detect apoptosis, Golgi staining was used to observe autophagy, and Western blot was used to detect autophagy⁃related apoptosis⁃related protein expression. Results : In vitro, rapamycin obviously reversed the apoptosis caused by propofol, but chloroquine had no effect. Compared with propofol group, the expression of microtubule⁃associated protein light chain 3 Ⅱ / microtubule⁃associated protein light chain 3 I (LC3 Ⅱ/LC3 Ⅰ) and Beclin⁃1 in the rapamycin group increased, but the expression of apoptotic proteins Cleaved⁃caspase⁃3 and Bax decreased. In vivo, compared with propofol group, the water maze escape latency of the rapamycin group reduced. In addition, the number of TUNEL⁃positive cells and autophagosomes in the rapamycin group decreased. Furthermore, the expression of mammalian target of rapamycin (mTOR) in the rapamycin group decreased, and the expression of LC3 Ⅱ/LC3 Ⅰ and Beclin⁃1 increased, as well as the expression of Cleaved⁃caspase⁃3 and Bax decreased. But chloroquine had no effect on autophagy and apoptosis⁃related proteins. Conclusion Rapamycin can further activate autophagy by inhibiting the activation of mTOR signal, ameliorate the neuronal apoptosis caused by propofol, which will lead to the improvement of the cognition in rats.

Objective: To investigate the value of acoustic parameters in the voice therapy for patients with unilateral vocal cord paralysis (UVCP). Methods: From May 2015 to April 2018, 51 patients with UVCP and 59 healthy controls in Department of Otorhinolaryngology Head and Neck Surgery, Tianjin First Central Hospital, were involved in this research retrospectively. The UVCP patients were diagnosed with stroboscopic laryngoscopy. The minimum glottal area (MGA) was calculated by KIPS software when the people were pronouncing/i:/. The fundamental frequency (F0), Jitter, Shimmer and NHR were detected by CSL4500 multiple acoustic voice analyzer. Results: MGA of UVCP patients was much higher than that of healthy control (male: 433.68±64.52 vs. 294.41±51.82, t=9.23, P=0.000; female: 498.80±73.42 vs. 302.03±76.54, t=13.21, P=0.000), which meaned vocal cord insufficiency.After voice therapy, MGA reduced significantly (male: 288.48±55.09, female: 258.22±57.17, t=24.41 and 31.22, P=0.000 vs. pre-therapy). MGA of untreated patients decreased in varying degrees. Compared with the voice therapy group, the MGA decreased in a significantly lower extent (24.25±22.91 vs. 188.31±54.37, t=8.97, P=0.000). The F0, Jitter, Shimmer and NHR raised significantly in UVCP patients group (P=0.000 vs. healthy control group), and they were reduced by voice therapy (all P<0.05). Each of the four acoustic parameters was relative with MGA, r=0.551, 0.867, 0.853 and 0.875 in turn, P=0.001, 0.000, 0.000, and 0.000. Conclusion MGA and acoustic parameters can reflect the acoustic features of UVCP patients, which are useful tools in the UVCP assessment and voice therapy.