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Objective:To investigate the gastrointestinal characteristics of children with glycogen storage disease (GSD) type Ⅰ.Methods:From June to December 2020, clinical data of children aged 0-18 years with GSD type Ⅰ diagnosed by genetic testing from all provinces and cities in China, including Beijing, Shanghai, Guangdong, Guangxi, Hunan, Sichuan, Yunnan, Guizhou, Henan, Hebei, Zhejiang, Jiangsu, Shaanxi, Anhui and Heilongjiang, were collected.A cross-sectional questionnaire survey was used for data analysis.Results:A total of 52 questionnaires were obtained, and 43 eligible patients aged 1-18 years were recruited, involving 30 males (69.8%) and 13 females (30.2%). Among them, 9 patients were GSD type Ⅰa and 34 patients were type Ⅰb.Seven patients (16.3%) had siblings who were also diagnosed as GSD type Ⅰb.The gastrointestinal manifestations included recurrent diarrhea in 26 patients (60.5%), perianal lesions (erythema, ulcer, abscess) in 25 patients (58.1%), abdominal pain/distension in 24 patients (55.8%), nausea/vomiting in 22 patients (51.1%), mucus/bloody stool in 14 patients (32.6%). Thirty-three patients (76.7%) had recurrent stomatitis and oral ulcer, and 38 patients (88.0%) had at least two gastrointestinal symptoms.White blood cell (WBC) count was <4.0×10 9/L in 24 patients (55.8%), and absolute neutrophils count was <1.5×10 9/L in 19 patients (44.2%), which was <0.5×10 9/L in 10 patients (23.3%). WBC count and absolute neutrophils count both decreased in children with GSD type Ⅰb.Platelets were >300×10 9/L in 30 patients (69.8%). Eighteen patients with GSD type Ⅰb underwent gastroscopy and colonoscopy, and 16 patients were diagnosed with GSD-related inflammatory bowel disease.Thirty-nine patients (90.7%) were fed with raw corn starch, 3 patients (6.9%) with maltodextrin and 19 patients (44.2%) with special enteral formula.Twenty patients with type Ⅰb GSD needed repeated antibiotic treatment due to neutropenia and neutrophil dysfunction.Fifteen patients were treated with granulocyte colony-stimulating factor (G-CSF). Among them, 11 patients were diagnosed as GSD-related bowel disease. Conclusions:Children with GSD type Ⅰ commonly have gastrointestinal symptoms, especially those with GSD type Ⅰb.The incidence of GSD-related inflammatory bowel disease is high in those children.G-CSF treatment cannot prevent the development of GSD-associated inflammatory bowel disease and its pathogenesis needs further research.Diet therapy is the first-line treatment of GSD type Ⅰ.Multidisciplinary management is helpful to reduce the complications and improve the quality of life in children with GSD type Ⅰ.
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Objective:To discover the epidemiological characteristics and clinical manifestations of Nontyphoidal Salmonella(NTS) infection in children suffering from diarrhea in Guangzhou, and to provide references for the prevention and treatment of salmonella infection. Methods:A total of 570 diarrhea children and 296 non-diarrhea controls were collected with stratified sampling from three districts of Guangzhou Women and Children′s Medical Center from January 2019 to December 2019. Through bacterial culture, corresponding colonies were selected and Salmonella diagnostic serum was applied for preliminary serum diagnosis, and then systemic biochemical method was adopted for diagnosis. A structured questionnaire was conducted to record the demographic information and clinical symptoms from each subject. Results:The detection rate of NTS was 6.67% (38/570 cases, 95% CI: 4.90%-9.02%) in children with diarrhea, and 1.01% (3/296 cases, 95% CI: 0.34%-2.93%) in children without diarrhea. There were statistically differences in NTS detection rate between children with diarrhea and the control ( χ2=13.805, P<0.05, OR=6.976, 95% CI: 2.135-22.796). There were no significant differences in NTS detection rate between male and female children with diarrhea ( χ2=0.395, P>0.05, OR=1.254, 95% CI: 0.619-2.541). The detection rate of NTS was 5.30% (22/416 cases, 95% CI: 3.52%-7.88%) in children younger than 2 years old, and was 10.40% (16/154 cases, 95% CI: 6.50%-15.21%) in children over 2 years old. There were statistically differences ( χ2=4.700, P<0.05, OR=2.076, 95% CI: 1.060-4.068) between the younger and the older groups.The detection rate of NTS was 5.40% (25/460 cases, 95% CI: 3.70%-7.89%) for children with diarrhea in outpatient department and 11.80% (13/110 cases, 95% CI: 7.04 %-19.18%) for children with diarrhea in inpatient department, with statistically significant differences ( χ2=5.813, P<0.05, OR=2.332, 95% CI: 1.152-4.721). The detection rate of NTS diarrhea in children was 4.60% (10/217 cases, 95% CI: 2.52%-8.28%) in spring, 8.50% (12/141 cases, 95% CI: 4.93%-14.29%) in summer, 9.60% (15/144 cases, 95% CI: 6.41%-16.48%) in autumn and 1.50% (1/168 cases, 95% CI: 0.11%-3.30%) in winter, respectively, with statistically differences among the four seasons( χ2=9.404, P<0.05). There were significant differences in most common clinical symptoms of fever, vomiting, abdominal pain, bloody stool and pasty stool between NTS positive and negative children(all P<0.05). Salmonella enteritis is the main type, and Salmonella typhimurium is the second type. Conclusions:NTS is one of the most important bacterial pathogens and leads to diarrhea in children in Guangzhou city, without differences in gender.Children over 2 years old are more likely to suffer from NTS infection.High incidence is in autumn.The most common clinical symptoms include fever, vomiting, abdominal pain, bloody stool and pasty stool. Salmonella enteritis is the main type, and Salmonella typhimurium is the second type.Laboratory tests can provide references for the diagnosis and treatment of NTS-related diarrhea.
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【Objective】 To investigate the genotype of anti-HCV reactive blood donors by one ELISA assay and provide scientific basis for the reentry of anti-HCV false positive blood donors. 【Methods】 The data of 453 blood donors reactive to antibody to HCV(anti-HCV) with one ELISA assay(third generation) were extracted via the blood donor information system of Shaoguan Central Blood Station from January 1, 2014 to December 31, 2018. The subjects were recalled to the station for the serological retest, using a 4th generation ELISA reagent, and PCR qualitative test. The PCR reactive samples were sent to the genetic testing laboratory for HCV genotyping, in order to guide diagnosis and treatment in the future. Meanwhile, those PCR negative blood donors returned to be eligible again based on the Guidelines for the Return of Reactive Blood Donors for Blood Screening. 【Results】 70.2% (318/453) of the previous anti-HCV-reactive blood donors, using a third-generation ELISA assay responded to the HCV genotyping, of which 83.0%(264/318) were negative, and 17%(54/318) positive. The profile of HCV subtypes in positive donors was HCV2a>1b>3a=6a. A little bit high false positive rate was presented by the third, and former, generation reagent than the four generation(0.41% vs 0.06%), which was confirmed by HCV RNA qualitative and HCV genotyping tests.After two rounds of reentry testing, 98 eligible blood donors returned to the blood donor team, with the return rate at 21.63% (98 / 453). 【Conclusion】 NAT or (and) HCV genotyping for anti-HCV reactive blood donors screened out by the third, and former, generation, should be carried out to permanently shield the true positive donors and reenter the negative ones.
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<p><b>OBJECTIVE</b>To analyze the efficacy of percutaneous endoscopic gastrostomy (PEG) in pediatric patients.</p><p><b>METHOD</b>From October 2011 to October 2014, children in the gastrointestinal ward of Guangzhou Women and Children's Medical Center received PEG or jejunal tube PEG(JET-PEG). The success rate, operation time were recorded. The changes of their weight, enteral nutrition calories and the incidence of pneumonia before and after the first 6 months of operation were compared. Follow-up was conducted until October 2014, the recent and long term complications, the length of indwelling time, the replacement or removal of the tube were recorded, the patients swallowing function or the primary disease's outcomes were observed.</p><p><b>RESULT</b>Of the 13 cases, 10 were male, 3 were female, their average age was 2 years (range 1.8 months-9 years). We performed PEG for 12 of the patients who had congenital craniofacial problems that led to feeding difficulties or recurrent cough and pneumonia (6/12), or neurological disorders (6/12) with inability to swallow, and in one case JET-PEG was performed, this child suffered from chronic intestinal pseudo-obstruction with vomiting and abdominal distension. The gastrostomy was successful in all the patients through one operation, the average operation time of PEG was (25 ± 3) minutes, JET-PEG was 60 minutes. One local skin infection was noted, no long-term complication occurred. In the first 6 months after operation, all the patients gained weight((5.5-30.5) kg postoperation vs. (3.0-30.0) kg preoperation), and 12 cases' enteral nutrition calories increased (from (209-502) to(272-543) kJ/(kg·d)), the incidence of pneumonia decreased in the children who had recurrent pneumonia before the operation (from (0-1.5) to (0-0.16) per month). Until October 2014, their average length of gastric tube indwelling time was 17.8 months (range 4-36 months). In 4 cases PEG tube was removed when they could eat completely independently, the other 9 needed enteral vein nutrition via PEG tube or jejunal tube, in 3 of them balloon type gastric fistula tube was applied. Two of the 13 cases who had cleft palate received stomatological operations when their weight grew to meet the standard.</p><p><b>CONCLUSION</b>PEG and JET-PEG are safe and effective method for enteric nutrition feeding in pediatrics, the technique causes minimal trauma and has rapid postoperative recovery, few complications, good aesthetic appearances and simple nursing, it can significantly improve their nutritional status and quality of life.</p>
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Child , Child, Preschool , Female , Humans , Infant , Male , Enteral Nutrition , Methods , Gastrostomy , Incidence , Nervous System Diseases , Therapeutics , Pneumonia , TherapeuticsABSTRACT
Objective To develop the clinical practice guidelines for the management of medication adherence to highly active antiretroviral therapy (HAART) in China. Methods The development methods included qualitative interview of 31 stakeholders, questionnaire survey of 423 PLHIV, adaptation of 30 clinical practice guidelines related to AIDS care, and overviews of reviews of 44 systematic reviews/Meta-analysis. Results 10 clinical practice guidelines and 10 systematic reviews/Meta-analysis were included. The clinical practice guidelines for the management of HAART were formed. Conclusions The formed clinical practice guidelines showed better applicability and higher general quality. It is recommended to use the guidelines in AIDS care.
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Objective To evaluate the value of diagnosis in fallopian tube patency by using four dimensional transvaginal hysterosalpingo-contrast sonography (4D-HyCoSy) and two-dimensional transvaginal hysterosalpingo-contrast sonography(2D-HyCoSy) with SonoVue in infertile women.Methods First,fifty seven infertile women(113 fallopian tubes) were examined by 4D-HyCoSy,then examined by 2D-HyCoSy,and the results of examination were respectively recorded.Results By 4D-HyCoSy,54 fallopian tubes were patency,42 were unobstructed but unsmooth flow,17 were obstructed.By 2D-HyCoSy,48 of them were patency,40 of them were unobstructed but unsmooth flow,25 of them were obstructed.Three fallopian tubes were obstructed by 4D-HyCoSy,but by 2D-HyCoSy they were patency.Eleven fallopian tubes were patency by 4D-HyCoSy,but obstructed by 2D-HyCoSy.Nine fallopian tubes were patency by 4D-HyCoSy,but fimbriated extremity of these fallopian tubes were far awary from homolateral overys.They were unuseful.Eighteen patients underwent laparoscopy,30 fallopian tubes (91.4％) were concordant with laparoscopy using combination of 4D-HyCoSy and 2D-HyCoSy,the sensitivity,specificity,positive predictive value and negative predictive value were 86.7 ％,90.5 ％,86.7 ％ and 90.5 ％.The concidence rate,sensitivity,specificity,positive predictive value and negative predictive value were 85.7％,84.6％,90.5％,84.6％ and 86.4％ by 4D-HyCoSy and 71.4％,64.7％,70.0％,64.7％ and 77.8％ by 2D-HyCoSy,respectively.Accuraty of combination of 4D-HyCoSy and 2D-HyCoSy was similar with laparoscopy.Five patients appeared moderate pelvic pain,three patients appeared mild pelvic pain,two patients appeared nausea and vomiting.None need resuscitation.Conclusions Combination of 4D-HyCoSy and 2D-HyCoSy can more accurately diagnose fallopian tube patency,it can judge if fallopian tube is valuable or not and reduce misdiagnosing.It is a simple,safe,accurate,efficient method for evaluating the fallopian tube patency.
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To discuss clinical diagnosis and treatment of cow’s milk protein allergy and cow’s milk protein-induced FPIES (food protein induced enterocolitis syndrome). Methods We retrospectively analyzed clinical data of one infant with milk protein allergy-induced FPIES. Results A 67 days old female on mixed breast and formula feeding developed recurrent diarrhea, abdominal distension, vomiting, mucousy and bloody stools, feeding dififculty, anemia, and failure to thrive since 2 weeks after birth. Laboratory studies showed anemia, increased CRP level and elevation of peripheral white blood count and eosinophil proportion. Milk-speciifc IgE was negative. She was previously hospitalized 4 times, all with admitting diagnosis of“necrotizing enterocolitis”. We treated her with milk protein elimination for 4 weeks and all symptoms were resolved. Milk protein re-challenge test was positive, consistent with clinical features of cow’s milk protein allergy-induced infant FPIES. Conclusions Cow’s milk protein allergy and cow’s milk protein-induced FPIES can present with non-speciifc and variable clinical symptoms and signs, and should be considered in the differential diagnosis.
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Objective To evaluate the changes in eutopic endometrial vascularity of endometriosis using three-dimensional power Doppler ultrasound.Methods Forty-four women with endometriosis and 51 normal controls were studied by three-dimensional power Doppler ultrasound.Eutopic endometrial volume,vascularization index (VI),flow index (FI) and vascularization flow index (VFI) were calculated using VOCAL software.Results In proliferative phase,the mean VI was (5.499 ± 5.153)％ in patients with endometriosis and ( 1.135 ± 1.333) ％ in normal controls ( P ＜0.001 ),the mean FI was (28.832 ± 4.279)dB in patients with endometriosis and (25.544 ± 3.465)dB in normal controls ( P =0.006),the VFI was (1.769 ± 1.981 )dB in patients with endometriosis and (0.321 ± 0.397)dB in normal controls( P =0.002).In secretory phase,the mean VI was (8.693 ± 5.940) ％ in patients with endometriosis and ( 1.014±0.968)％ in normal controls ( P ＜0.001); the mean FI was (30.689 ± 3.632)dB in patients with endometriosis and (24.748 ± 3.811 )dB in normal controls( P ＜0.001 ),the VFI was (2.753 ± 2.044)dB in patients with endometriosis and (0.27 ± 0.293)dB in normal controls( P =0.001 ).Both in proliferative and secretory phase,no significant difference in endometrial volume between the two groups was observed ( P =0.108 and 0.068,respectively).Conclusions Three-dimensional power ultrasound provides a useful tool to investigate eutopic endometrial vascularization of endimetriosis.
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Objective To discuss the significance and value of speckle tracking imaging (STI) technique for accurate evaluating different types myocardial systole. Methods Anterior interventricular branches of 11 healthy crossbreed dogs were separated and ligatured to establish acute myocardial infarction models. The short axle radial strain (RS), circumferential strain (CS) of ventricular sinister and long axle longitudinal (LS) of apex of experiment dogs were measured with STI technique before and after myocardial infarction. Results Before myocardial infarction, no statistical difference of RS, CS was detected among sections of left ventricle (P>0.05), and RS was greater than LS (P<0.05). When detector was at the apex of base, LS of middle, apex sections of left ventricle myocardium increased gradually (LS of base section was least and apex section was most) (P<0.05). RS, CS and LS of antetheca, forepart of interventricular septum of middle of left ventricle and antetheca, posterior and forepart septum of apex all became lower after myocardial infarction (P<0.05). Conclusion Strains caused by all types of myocardium action can be accurately evaluated with STI.
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Objective:To detect the expression of vascular endothelial growth factor receptor-3(VEGFR-3)in earlystage tongue cancer and the relationship with occult metastasis thereof.Methods:The expression of VEGFR-3 was examined in paraffin-embedded specimens from 62 patients with early-stage tongue cancer by immunohistochemical staining.The relationship was analyzed between the expression of VEGFR-3 and size,occult metastasis and differentiation of early-stage tongue cancer.Results:The positive expression of VEGFR-3 mainly located in the lymphatic endothelial cells of peritumoral areas and negative expression in tumor area.The higher expression of VEGFR-3 was found in patients with occult metastasis.The expression of VEGFR-3 was significantly higher in patients of Ⅱ-Ⅲ grade than that of patients in I grade carcinoma(t=4.77,P ＜ 0.05).The expression of VEGFR-3 was higher in patients with occult metastasis than that in patients with negative lymph node(t=6.33,P ＜ 0.05).But there were no significant correlation between the expression of VEGFR-3 and the age,gender and size of tumor in patients.Conclusion:The value of VEGFR-3 can be a useful indicator to evaluate the occult metastasis of early-stage tongue cancer.
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Objective To determine whether regulatory T cells(Tr)are increased in patients with tuberculosis and whether they are associated with its immunopathology.Meantime,to investigate the possibility of tuberculosis(TB)as a model for studying Tr functions.Methods The lymphocyte subsets were isolated from peripheral blood mononuclear cells by sorting with flow cytometry.Total cellular RNA was extracted and RT-PCR was performed to detect the Foxp3 mRNA in purified CD3+CIM+T cells,CD3+CD8+T cells and non-CD3+CD4+CD8+T cells.Using FACS analysis.we further investigated the distribution of Foxp3+ population in CD4+ CD25+T cells.Finally,we compared the percentage of CD4+CD25highFoxp3+T cells present in 51 active patients with tuberculosis and 40 uninfected healthy control subjects by FACS.The detection of Tr infiltration of Foxp3+ cells were performed with immunohistochemistry(IHC)method on tuberculosis pathological sections.Results Foxp3 was specific expressed in CD3+CD4+T cells,either in tuberculosis patients or healthy control subjects.Foxp3+ T cells took about 85%fraction of CD4+ CD25highpopulation.We used CD4+CD25high Foxp3+as a detective markers for Tr in the FACS analysis.The results showed that patients with active TB had a 4.4 fold higher percentage within the CD4+T cells in peripheral blood compared to healthy control group(modian,1.01%vs 0.23%,P<0.01).Much higher frequency of Tr were found along with T cells infiltration at the tuberculosis pathological tissues.A few individuals that we can followed indicated the expanded Tr was declined after curative treatment with operation.Conclusion Tr cells are increased in tuberculosis patients and closely correlate with its immunopathology.Tuberculosis should be a valuable model for Tr functional study.