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1.
Chinese Journal of Immunology ; (12): 629-635, 2024.
Article in Chinese | WPRIM | ID: wpr-1024775

ABSTRACT

Allergic rhinitis(AR)is a type Ⅰ allergic reaction,which mediated by immunoglobulin E immediately when an individual with a special constitution is exposed to a same allergen for second time,whose pathogenesis has not been clarified yet,and closely related to genes,immune cells and cytokines.Pathogenesis of AR become more deeper and more accurate due to rapid develop-ment of molecular biology and second-generation gene sequencing technology.Current studies have found that miR-155 and transcrip-tion factor GATA3 have important regulatory effects on occurrence and development of AR,then affect dominant differentiation of CD4+T lymphocytes and proliferation of ILC2.This article discusses and reviews pathogenesis of AR,which mainly focuses on miR-155/GATA3 pathway and effects of related upstream genes and downstream regulatory substances.

2.
Article in English | WPRIM | ID: wpr-1043751

ABSTRACT

Objective@#: Kinesin family member C1 (KIFC1), a non-essential kinesin-like motor protein, has been found to serve a crucial role in supernumerary centrosome clustering and the progression of several human cancer types. However, the role of KIFC1 in glioma has been rarely reported. Thus, the present study aimed to investigate the role of KIFC1 in glioma progression. @*Methods@#: Online bioinformatics analysis was performed to determine the association between KIFC1 expression and clinical outcomes in glioma. Immunohistochemical staining was conducted to analyze the expression levels of KIFC1 in glioma and normal brain tissues. Furthermore, KIFC1 expression was knocked in the glioma cell lines, U251 and U87MG, and the functional roles of KIFC1 in cell proliferation, invasion and migration were analyzed using cell multiplication, wound healing and Transwell invasion assays, respectively. The autophagic flux and expression levels matrix metalloproteinase-2 (MMP2) were also determined using imaging flow cytometry, western blotting and a gelation zymography assay. @*Results@#: The results revealed that KIFC1 expression levels were significantly upregulated in glioma tissues compared with normal brain tissues, and the expression levels were positively associated with tumor grade. Patients with glioma with low KIFC1 expression levels had a more favorable prognosis compared with patients with high KIFC1 expression levels. In vitro, KIFC1 knockdown not only inhibited the proliferation, migration and invasion of glioma cells, but also increased the autophagic flux and downregulated the expression levels of MMP2. @*Conclusion@#: Upregulation of KIFC1 expression may promote glioma progression and KIFC1 may serve as a potential prognostic biomarker and possible therapeutic target for glioma.

3.
Article in Chinese | WPRIM | ID: wpr-1025022

ABSTRACT

Objective To observe the characteristics of C57BL/6N-Tg(1.28HBV)/Vst transgenic hepatitis B virus(HBV-Tg)model mice and analyze their transcriptomic characteristics.Methods Twenty male HBV-Tg mice were divided into an experimental group and a wild-type(control)group(n = 10 mice per group).The virological characteristics of the model mice were evaluated according to serum levels of HBV DNA,HBsAg,and HBeAg,and expression levels of HBsAg and HBcAg in liver tissue.Serum levels of alanine transaminase(ALT)and aspartate transaminase(AST),hematoxylin and eosin(HE)and Sirius red staining,and hydroxyproline(Hyp)in liver tissue were detected to evaluate the degree of liver inflammation and fibrosis.Liver tissue samples were randomly selected from three mice in each group for RNA extraction for high-throughput transcriptome sequencing.Significantly differentially expressed genes were identified using R software.Functional enrichment of differential genes was determined by Gene Ontology(GO)and Kyoto Encyclopedia of Genes and Genomes(KEGG)analyses,and genes with significant differences were verified by real-time fluorescence quantitative polymerase chain reaction(RT-qPCR).Results ALT and AST levels were increased in the model group compared with the normal group,with the result for ALT being more significant(P<0.05).HE staining of liver tissue showed enlargement of the liver nucleus and swelling of some hepatocytes in the model group,while Sirius red staining showed a small amount of collagen deposition in the sink area and interlobule in the HBV transgenic group,in the shape of thin lines.A total of 1352 differential genes were obtained by screening(|logFC|>2 and P.adj<0.05),including 703 up-regulated and 649 down-regulated genes.KEGG analysis suggested that differential genes were mainly enriched in the peroxisome proliferator-activated receptor(PPAR)signaling pathway,retinol metabolism,fatty acid degradation,and other pathways(P.adj<0.05).The main significantly up-regulated genes included Cyp4a10,Cyp4a14,Acot1,Acot3,and Ehhadh,and the significantly down-regulated genes included Scn5a、Apol10b、Igddc4、Cxcl1、9530077C05Rik.The trend was consistent after RT-qPCR detection(P<0.05).Conclusions HBV-Tg mice have a tendency to develop spontaneous fibrosis.Transcriptomic analysis showed that chronic hepatitis B mainly involves PPAR signaling,retinol metabolism,fatty acid degradation,drug metabolism,and other pathways.

4.
Article in Chinese | WPRIM | ID: wpr-990394

ABSTRACT

Objective:To evaluate and summarize the best evidence related to the management of cancer-related fatigue in children with leukemia and provide an evidence-based basis for clinical practice.Methods:The search for the relevant guidelines and evidence synthesis from UpToDate, JBI, Cochrane Library, Registered Nurses′ Association of Ontario, Guidelines International Network, National Institute for Health and Clinical Excellence, National Guideline Clearinghouse, YiMaiTong Guideline Network, National Comprehensive Cancer Network, PubMed, CINAHL, EMbase, China National Knowledge Infrastructure, Wanfang, China Biology Medicine from January 1st, 2012 to May 1st, 2022. Two researchers evaluated the quality of the literature independently according to the unified standard and extracted the best evidence.Results:A total of 13 literatures were extracted, including three clinical practice guidelines, two evidence summaries and eight systematic reviews. Finally, 22 pieces of evidence were summarized, involving six aspects which were environmental management, cancer-related fatigue assessment, exercise management, adventure therapy, sleep management and psychosocial interventions.Conclusions:Best evidence for the management of cancer-related fatigue in children with leukemia, which should be used in conjunction with the environment, the child's age, physical ability, medical condition and psychological acceptability to develop an individualized symptom management plan to improve the quality of care and the child′s quality of life.

5.
Article in Chinese | WPRIM | ID: wpr-991910

ABSTRACT

Reasonable nutrition management is important in the long-term care of cancer survivors. It can contribute to reducing the risk of metabolic complications, improving patients' understanding, beliefs, and behavior as regards healthy lifestyles, promoting lifestyle and dietary changes, and positively impacting disease treatment and long-term prognosis. To improve the long-term management of Chinese cancer survivors, reduce medical costs, and maximize clinical benefits and patient outcomes, the Cancer Nutrition Branch of the Chinese Nutrition Society has developed this consensus based on the current cancer patient management and nutritional therapy in China, evidence-based medicine from domestic and foreign publications, as well as expert opinions and experiences in clinical and nutritional fields. Following the Oxford Centre for Evidence-based Medicine (OCEBM) grading system, this consensus provides recommendations for nutritional risk screening, assessment (diagnosis), enteral and parenteral nutrition support, and medical nutrition therapy for cancer survivors, aiming to inform and support the standardized nutritional management of cancer survivors.

6.
Article in Chinese | WPRIM | ID: wpr-991911

ABSTRACT

Objective:To establish the Chinese version of (strength, assistance with walking, rise from a chair, climb stairs and falls, SARC-F) scale using the standardized methods and to validate the reliability and validity for sarcopenia screening among elderly population.Methods:Following the recommended procedure by World Health Organization and European Union Geriatric Medicine Society Sarcopenia Special Interest Group, the translation process included forward translation, expert panel, back-translation, pre-testing and cognitive interviewing to generate the final version. In the pilot study, the test-retest reliability, inter-rater reliability, and internal consistency of the Chinese version of SARC-F scale were assessed. In the diagnostic test for clinical validation, the participants were consecutively recruited from communities and hospitals in Beijing and Tianjin between December 2021 and October 2022. The scale administration, anthropometry, and body composition measurement were conducted by trained investigators. Participants with the SARC-F score ≥ 4 were considered at risk of sarcopenia. Diagnostic tests and receiver operating characteristic curve analysis were performed against the definitions of sarcopenia according to European Working Group on Sarcopenia in Older People (EWGSOP2) and Asian Working Group for Sarcopenia (AWGS2019), and the sensitivity, specificity, positive predictive value, negative predictive value and the area under curve were displayed.Results:The Chinese version of SARC-F scale was approved by the author that the translation has expressed the original meaning correctly. The Chinese version of SARC-F had good test-retest reliability (ICC = 0.914), inter-rater reliability ( r = 0.726), and internal consistency ( α = 0.729). There were altogether 1 882 participants included in the clinical validation. According to the diagnostic criteria of EWGSOP2 and AWGS2019, the Chinese version of SARC-F scale had low sensitivity (13.6% and 16.0%) and positive predictive value (44.6% and 35.4%), high specificity (95.1% and 94.7%) and negative predictive value (79.0% and 86.2%), and moderate AUC of 0.619 and 0.616 (all P < 0.001) for sarcopenia screening. Conclusions:The Chinese version of SARC-F scale was of good reliability and validity. The application of SARC-F in the primary healthcare settings would contribute to the early diagnosis of sarcopenia.

7.
Article in Chinese | WPRIM | ID: wpr-930710

ABSTRACT

Objective:To explore the inner experience and influencing factors of caregivers′ decision-making process in children with hematological tumors treated by Chimeric antigen receptor T cells (CAR-T) cell clinical trial, so as to provide reference for children and caregivers treated with CAR-T cells to carry out targeted health education and psychological support.Methods:Using the phenomenological method of qualitative study, the caregivers of 11 children were interviewed by semi-structured interview, the data were analyzed by Colaizzi analysis method, and the text data were managed, explored and searched by NVivo10.0 software.Results:The caregivers of children with hematological tumors had three topics: complex psychological experience in the initial stage of CAR-T treatment decision-making process, consultation approach and experience in the decision-making process, and overall decision-making quality evaluation.Conclusions:Pay attention to and understand the psychological experience of caregivers in CAR-T cell clinical trials, provide diversified decision-making counseling for caregivers, and ensure the right of caregivers to participate in decision-making, so as to improve caregivers′ treatment and nursing compliance.

8.
Article in Chinese | WPRIM | ID: wpr-991895

ABSTRACT

Chronic intestinal failure represents a long-lasting condition of intestinal dysfunction that contributes to inadequate absorption of macronutrients and/or water and electrolytes. It is heterogeneous in clinical manifestation, intervention and prognosis, and usually requires multi-disciplinary management. This review elucidates the state-of-the-art consensus on diagnosis and definition in chronic intestinal failure and exemplifies relevant nutritional management principles with interventions in short bowel syndrome, in hopes of improving the identification and management of chronic intestinal failure in domestic practice.

9.
Article in Chinese | WPRIM | ID: wpr-908081

ABSTRACT

Objective:To summarizes the diet management of one child with GSD I and severe hyperlipidemia.Methods:Key points of diet management include: making an individual diet plan, correcting parents' dietary misunderstanding, adjusting dietary and keeping following up regularly and keeping a food diary.Results:Following up for 11 months, the children basically formed a stable diet pattern, the blood glucose level was basically maintained between 4~6 mmol/L, the indicators of hyperlipidemia, hyperlactic acid and liver function were significantly improved compared with the previous period, and the height increase was guaranteed, while the weight gain was effectively controlled.Conclusions:It shows that individualized dietary guidance has a significant effect on the maintenance of blood glucose level, improvement of growth and development status and metabolic control in children with GSD I.

10.
Article in Chinese | WPRIM | ID: wpr-910466

ABSTRACT

Objective:To investigate the optimal treatment modalities and prognostic factors of adenoid cystic carcinoma of the head and neck salivary gland.Methods:From January 2016 to December 2018, clinical data of 166 patients with adenoid cystic carcinoma of the head and neck salivary gland who received postoperative radiotherapy at Department of Radiation Oncology of the Ninth People′s Hospital of Shanghai Jiao Tong University School of Medicine were retrospectively analyzed. The survival analysis was performed by Kaplan-Meier method. Univariate prognostic analysis was conducted by log-rank test. Multivariate prognostic analysis was carried out by Cox proportional hazard regression model.Results:Among 166 enrolled patients, 70 cases were male and 96 female with an average age of 53 years (18 to 71 years). The median follow-up time was 31.2 months (8.6-63.1 months). Sixty-six patients underwent radical surgery (extended resection across the anatomical areas outside the tumor bed, pursuing negative margins of various resections), and the remaining 100 patients underwent conservative surgery (only extended resection of lesions, not pursuing negative nerve resection margins). The median dose of postoperative radiotherapy was 66 Gy (54-70 Gy). Seventy-three patients were treated with TP regime due to positive or close margins and 9 cases of cervical lymph node metastasis. The 3-and 5-year overall survival (OS), locoregional recurrence-free survival (LRRFS), distant metastasis-free survival (DMFS), progression-free survival (PFS) rates were 95% and 95%, 93% and 93%, 74% and 66%, 73% and 65%, respectively. Seven patients experienced primary lesion recurrence, 5 cases of regional lymph node recurrence and 38 cases of distant metastasis. T 3-T 4 stage, lymph nodes (+ ), stage IV, solid pathologic subtype, Ki-67≥10% and perineural invasion were associated with worse PFS and DMFS. Multivariate analysis demonstrated that only solid pathologic subtype was the independent prognostic factor of OS, LRRFS, PFS and DMFS. Conclusions:The local control rate of salivary gland adenoid cystic carcinoma can be improved by postoperative intensity-modulated radiotherapy (IMRT) with a recommended dose of ≥66 Gy. Solid pathologic subtype is the most important adverse prognostic factor.

11.
Article in Chinese | WPRIM | ID: wpr-883051

ABSTRACT

The paper reviewed the application of child life in the care of children with acute leukemia in recent years, including related overviews, application effects, shortcomings and prospects in the process. The purpose is to provide references for further improving the application of child life in children with acute leukemia.

12.
Article in Chinese | WPRIM | ID: wpr-868572

ABSTRACT

Objective To assess the efficacy and safety of post operative adjuvant concurrent chemoradiotherapy for patients with high-risk salivary gland tumors (SGT).Methods Fifty-two patients with moderate or high malignant pathological stage complicated with locally advanced stage Ⅲ/ⅣA±positive margin/close margin admitted to Shanghai Ninth People's Hospital from 2016 to 2018 were enrolled in this study.Among them,35 patients were male and 17 female with a median age of 55.5 years old (range:21-73 years old).All 52 patients were treated with intensity-modulated radiotherapy and concurrent chemotherapy.Patients with adeno carcinoma of the salivary gland receives concurrent chemotherapy with TP regimen.Patients with lympho epithelial cancer and squamous cell carcinoma were treated with cisplatin regimen.Results Forty-seven patients (90%) completed two cycles of concurrent chemotherapy,and five patients (10%) completed one cycle of concurrent chemotherapy.The median follow-up time was 15.7 months (3.2-34.8 months).The 2-year disease-free survival (DFS) and overall survival (OS) rates were 74% and 98%.Three patients experienced regional lymph recurrence and 6 cases had distant metastasis.Grade Ⅲ oral mucositis was observed in 30 patients.Grade Ⅲ dermatitis occurred in 5 cases.Only one patient experienced Grade Ⅳ neutropenia,and 2 patients developed Grade Ⅲ neutropenia.DFS was positively correlated with the cycle of postoperative adjuvant concurrent chemotherapy (P=0.006).Conclusions Patients with high-risk SGT can obtain higher 2-year DFS and OS rates and tolerable adverse events after postoperative concurrent chemoradiotherapy.Nevertheless,the long-term outcomes remain to be validated by randomized controlled clinical trials.

13.
Article in Chinese | WPRIM | ID: wpr-733448

ABSTRACT

Objective To understand the negative emotion in primary caregivers of early diagnosed children with acute leukemia. Methods By purposive sampling, 17 cases were selected and investigated by semi-structuredinterviews. Colaizzi principles were used to analyze the data. Results Three themes were extracted:complex emotional response, too many concerns and weak support system. Conclusions The primary caregivers of children with acute leukemia at the early stage of diagnosis have negative emotions such as anxiety and fear, and suffer from psychological and economicpressures.Nurses should provide disease-related nursing skills training and personalized psychological care,and call for the establishment of the social support system and online communication platformto really meet their needs and alleviate the burden of care in order to better meet the follow-up treatment and care of children.

14.
Article in Chinese | WPRIM | ID: wpr-803571

ABSTRACT

Objective@#To investigate the dynamic changes of quality of life and its influencing factors in children with acute lymphoblastic leukemia during chemotherapy.@*Methods@#A total of 134 children who were diagnosed with acute lymphoblastic leukemia were selected. The demographic questionnaire and Pediatric Quality of Life InventoryTM General Core Module were completed in children at three different time points during chemotherapy (the first month, the third month, and the sixth month of chemotherapy).@*Results@#There were significant differences in the quality of life between children with acute lymphoblastic leukemia (t=-23.117-209.225, P<0.01). Multiple factors analysis showed that the factors influencing the quality of life of children at the first month of chemotherapy were age (t=-10.413, P=0.000) , and family emotional support (t=2.231, P=0.027) ; the factors at the third month of chemotherapy included age (t=-12.439, P=0.000) , whether the child was the only child (t=2.267, P=0.025) , education level of primary caregiver (t=2.091, P=0.038) , and family emotional support (t=5.374, P=0.014) ; the factor at the sixth month of chemotherapy were age (t=-11.254, P=0.000) , and family emotional support (t=2.184, P=0.035) .@*Conclusions@#The total quality of life and the scores of all dimensions of children with acute lymphoblastic leukemia were lower and showed dynamic changes. Age and family emotional support were continuing factors influencing quality of life. Medical staff should take targeted interventions according to the characteristics of the quality of life of children at different ages, and guide and help parents and children to establish good family emotional support and improve their quality of life.

15.
Article in Chinese | WPRIM | ID: wpr-791012

ABSTRACT

Objective To explore the relationship between sarcopenia and the risks of osteoporosis and fragility fractures among community-dwelling middle and old people (≥50 years) . Methods OVID/Medline, Pubmed, EMBASE, Cochrane Library, Web of Science ( SCI ) , China National Knowledge Infrastructure ( CNKI) , and Chinese WanFang Database were searched systematically for literatures related to the relationship between sarcopenia and osteoporosis and fragility fractures from January 1987 to July 2018. The searched litera-tures were screened based on the inclusion and exclusion criteria. The quality of the literatures were evaluated by using the risk assessment tool NOS ( Newcastle-Ottawa Scale) and AHRQ ( Agency for Healthcare Research and Quality) . Meta-regression analysis was used to explore the cause of heterogeneity between studies. The sub-group analysis was used to assess the differences in the risk of osteoporosis based on the important characteristic variables, such as gender, ethnicity, age, diagnostic criteria of sarcopenia, and outcome type. Sensitivity anal-ysis and trim and fill method were conducted to test the stability of the results of this Meta-analysis. Data col-lected and summarized by Stata 12. 2 software. Results A total of 23 studies in line with quality requirements were included eventually, including 56, 544 subjects. The results of this Meta-analysis were relatively robust. Compared with non-sarcopenia, the relative risk ( RR ) for osteoporosis among subjects with sarcopenia was 1. 61 (95% CI: 1. 42~1. 82, P<0. 00001). Especially, compared with postmenopausal women RR=1. 37 (95% CI: 1. 23-1. 53, P<0. 0001) and yellow race RR=1. 53 (95% CI: 1. 34-1. 75, P<0. 0001), sar-copenia had a higher impact trend on the risk of osteoporosis in older men RR=2. 26 ( 95% CI: 1. 71-2. 98, P<0. 0001 ) and Caucasian RR = 2. 03 ( 95% CI: 1. 46-2. 81, P<0. 0001 ) . Conclusion Among community-dwelling middle and old aged people (≥50 year) , sarcopenia increases the risk of osteoporosis and fragility fracture by 61% and 59% -61% significantly, respectively. Middle and old aged people should be pre-vented and screened early for sarcopenia, which attributes to identify high risk groups of fragile fractures and re-duce the risk of adverse outcomes.

16.
Article in English | WPRIM | ID: wpr-914291

ABSTRACT

MicroRNAs (miRNAs) are important diagnostic markers and therapeutic targets for many diseases. However, the miRNAs that control the pathogenesis of idiopathic pulmonary fibrosis (IPF) and act as potential therapeutic targets for the disease are rarely studied. In the present study, we analyzed the function and regulatory mechanism of microRNA-708-3p (miR-708-3p) and evaluated this marker’s potential as a therapeutic target in IPF. The clinical and biological relevance of fibrogenesis for miR-708-3p was assessed in vivo and in vitro, specifically in matching plasma and tissue samples from 78 patients with IPF. The data showed that the miR-708-3p levels decreased during fibrosis and inversely correlated with IPF. The experiments showed that the decreased miR-708 promoter activity and primer-miR-708(pri-miR-708) expression were the potential causes. By computational analysis, a dual luciferase reporter system, rescue experiments and a Cignal Finder 45-Pathway system with siADAM17 and a miR-708-3p mimic, we identified that miR-708-3p directly regulates its target gene, a disintegrin and metalloproteinase 17 (ADAM17), through a binding site in the 3′ untranslated region, which depends on the GATA/STAT3 signaling pathway. Finally, an miR-708-3p agomir was designed and used to test the therapeutic effects of the miR-708-3p in an animal model. Small-animal imaging technology and other experiments showed that the dynamic image distribution of the miR-708-3p agomir was mainly concentrated in the lungs and could block fibrogenesis. In conclusion, the miR-708-3p–ADAM17 axis aggravates IPF, and miR-708-3p can serve as a potential therapeutic target for IPF.

17.
Article in Chinese | WPRIM | ID: wpr-733943

ABSTRACT

Objective To explore the relationship between sarcopenia and the risks of falls, osteoporo-sis, fractures and all-cause mortality among elderly people. Methods This was a meta-analysis of prospective cohort studies. Databases of OVID/Medline, PubMed, EMBASE, Cochrane Library, China National Knowledge Infrastructure ( CNKI) and Chinese WanFang Database were searched systematically according to the inclusion and exclusion criteria. The literatures related to the relationship between sarcopenia and falls, os-teoporosis, fractures and all-cause mortality among elderly people from January 1987 to June 2017 were identi-fied. The quality of the literature was evaluated by the risk assessment tool Newcastle-Ottawa Scale recommen-ded by the Cochrane. Meta-analysis was conducted by RevMan 5. 3 and Stata 12. 1 software. Results Totally 13 prospective cohort studies including 19 376 subjects and 3 190 outcome events were entered in meta-analysis. The relative risk ( RR) for comprehensive adverse outcome events among subjects with sarcopenia was 1. 64 times of non-sarcopenia subjects (95% CI=1. 51-1. 78, P<0. 000 01), and the RRs for fall, osteoporosis, fractures and all-cause mortality were 1. 60 (95% CI=1. 42-1. 81, P<0. 000 01), 4. 85 (95% CI=2. 18- 10. 79, P=0. 000 1), 1. 59 (95% CI=1. 40-1. 80, P<0. 000 01), 2. 08 (95% CI=1. 18-3. 69, P=0. 01) times of non-sarcopenia subjects respectively. Conclusion Sarcopenia increases the risk of falls, fractures, all-cause mortality and comprehensive adverse outcome significantly, suggesting that sarcopenia might be a pre-dictor for adverse outcomes among elderly people.

18.
Article in Chinese | WPRIM | ID: wpr-668416

ABSTRACT

Objective To evaluate the relationship between sarcopenia and the incidence of fall in elderly,with a systematic review of advanced nutritional and physical interventions studies.Methods A systematic review of randomized controlled trails (RCTs) and cohort studies was conducted.All published literature related to the sarcopenia,falls,and nutritional,physical interventions in the elderly were searched both in Chinese and English databases including Wanfang Database,CBM/SinoMed,PubMed,EMBASE,and Cochrane Library from January 1987 to January 2017 based on the inclusion and exclusion criteria.According to the standard procedure of systematic review,all selected articles were evaluated and graded by two researchers independently by using Jadad scale or Newcastle-Ottawa scale.Results A total of 168 articles were found,of which 11 (4 RCTs and 7 cohort studies) met the inclusion criteria.It was found that sarcopenia was an independent risk factor for fall among the elderly.Early prevention,accurate identification,and timely nutrition and physical interventions on the sarcopenia were the critical factors for reducing the incidence of accidental falls and fractures in the elderly,which helped to greatly reduce the health care expenditures and improve clinical outcomes and the quality of life.Conclusion Elderly should be screened early for sarcopenia,and nutrition and physical interventions should be applied to improve clinical outcomes and reduce health care costs.

19.
Article in Chinese | WPRIM | ID: wpr-668482

ABSTRACT

Objective To investigate the biochemical disturbances,nutritional assessment and nutritional interventions in hospitalized patients with glycogen storage disease (GSD) in a general hospital in Beijing and provide examples of advanced nutritional management for these patients.Methods We retrospectively reviewed 22 consecutive hospitalized patients with GSD diagnosed from January 1,2013 to January 1,2016 in Peking Union Medical College Hospital and analyzed their medical data,nutritional assessments,and interventions.Results These 22 GSD patients included 2 cases with GSD-Ⅰa,3 cases with GSD-Ⅰb,and 17 cases with GSD-Ⅲ.They were composed of 21 children (including a 6-month old infant and a 7-month old infant) and one 18-yearold man.Biochemical abnormalities:hypoglycemia (5/5),hyperlactacidemia (4/5),hyperlipidemia (4/5),hyperuricemia (4/5) were common in GSD-Ⅰ patients,while hypoglycemia (17/17),hyperlactacidemia (5/17),hyperlipidemia (7/17),and hyperuricemia (3/17) were also present among patients with GSD-Ⅲ.Growth and development assessments:among 5 patients with GSD-Ⅰ,the body weight was below the 10th percentile in of 4 patients and the height was below the 3rd percentile in 5 patients;among 17 patients with GSD-Ⅲ,the body weight was below the 10th percentile in 4 patients and the height was below the 10th percentile in 9 patients.Nutritional management condition:all the patients received nutritional assessment,dietary modulation and nutritional support.Twenty children or adolescents were prescribed with raw corn starch (RCS) every 4-6 hours,with a mean initial dose of (1.58±0.28) g and (1.21±0.33) g for patients with GSD-Ⅰ and GSD-Ⅲ,respectively;the other 2 infant aged 6-month old and 7-month old were regularly administered amino acid-based enteral nutritional agents.In addition,all the patients with GSD-Ⅲ were recommended high protein intake of 3 g/(kg · d) and received dietary guidance and modulation.Follow-up condition:5 cases with GSD-Ⅰ and 2 cases with GSD-Ⅲ stayed in track after discharge from our hospital.The mean follow-up duration was (480.40±246.16) d and (373.00± 108.89) d for patients with GSD-Ⅰ and GSD-Ⅲ,respectively.All the patients reported alleviated symptom of hypoglycemia,and the examinations also indicated improved fasting blood glucose,uric acid,lipid profile,and growth status.Conelusions Hypoglycemia,metabolic disturbances,and growth retardation are prevalent in both GSD-Ⅰ and GSD-Ⅲ patients.Potential biochemical abnormalities should not be ignored in GSD-Ⅲ patients,especially in young patients.Comprehensive nutrition assessment,regular administration of RCS,sufficient protein intake,and continuous patient follow-up and surveillance can help to alleviate symptoms,correct metabolic disturbances,and improve growth status of patients with GSD.

20.
Article in Chinese | WPRIM | ID: wpr-509126

ABSTRACT

Objective To retrospectively analyze the treatment outcomes and failure patterns in patients with head?and?neck cancer of unknown primary head?and?neck cancer of unknown primary, and to compare the efficacy between elective mucosal irradiation and ipsilateral neck treatment. Methods The clinical data of patients with head?and?neck cancer of unknown primary who were admitted from January 2007 to December 2013 were retrospectively collected. Thirty?one patients received elective pharyngeal mucosal irradiation and 61 patients only received ipsilateral neck treatment. The SPSS 19. 0 software was used for comparison of the survival and local control between the two groups. Results In the 92 patients, the median age was 57 years;79. 3% had metastasis to level Ⅱ lymph nodes;the median follow?up time was 36. 5 months;the 3?year overall survival, mucosal control, and neck control rates were 89. 0%, 86. 6%, and 82. 4%, respectively. Primary sites were found in 15 patients, containing nasopharynx in 4 patients, oropharynx in 3 patients, oral cavity in 3 patients, throat and hypopharyngeal part in 3 patients, maxillary sinus in 1 patient, and esophagus in 1 patient. The patients undergoing elective pharyngeal mucosal irradiation had significantly higher 3?year mucosal control and neck control rates than those undergoing ipsilateral neck treatment ( 100% vs. 74. 9%, P= 0. 040;87. 5% vs. 62. 2%, P= 0. 037 ) . There was no difference in the 3?year overall survival rate between the two groups ( 83. 5% vs. 88. 7%, P= 0. 910 ) . Conclusions For patients with head?and?neck cancer of unknown primary, elective pharyngeal mucosal irradiation can reduce the incidence of primary site and increase the neck control rate. A new standard for target volume delineation should be established as soon as possible for elective prophylactic pharyngeal mucosal irradiation.

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