Objective: To establish the reference values of blood routine and serum iron in children aged 2~14 years in Tibet. Methods: Child Care Department, Maternity and Child Health Hospital of the People's Hospital of Tibet Autonomous Region was chosen in Tibet city of Lhasa (our hospital) as study site for preschool children. Three primary schools in Jiangzi County and Rerong Township, Lhunze County were chosen as study site for schoolage children data. In this study, the parents of Tibetan children were Tibetans, and they lived for generations in Tibetan areas; the parents of Han children were Han nationality, and they had worked and lived in Tibet for a long time. The peripheral blood samples were collected from the children during the physical examination, and the blood routine and serum iron were tested in the clinical laboratory of our hospital. The test values were extracted from the relevant instruments and analyzed. LMS program software (version: 1.35) was used to fit the percentile curve of each index according to age. Results: From Jan.1 to Dec.1 2016, 3 137 Tibetan and 744 Han healthy children aged 2 to 14 years were enrolled in this study. A total of 3 253 blood samples has been collected from preschool children and 628 blood samples from school aged children. There were no significant differences in gender between Tibetan and Han children at each age stage (P>0.05). Compared with the plain area, the reference ranges of RBC, Hb and PLT shifted right on the whole, both transferrin saturation(UIBC) and total iron binding capacity(TIBC) had a wider reference range; the reference range of WBC shifted left, and the reference ranges of neutrophil count (NEUT#), lymphocyte count (LYMPH#), monocyte count (MONO#) and the percentage of eosinophils (EO%) were wider, while that of basophil (BASO%) was narrower. As for absolute values, the reference range of NEUT# shifted left, and the reference ranges of LYMPH#, MONO#, eosinophil count (EO#) and BASO# were narrower. As age was increased, Hb, HCT, MPV, MCV, MCH, and RDWSD showed an upward trend, PLT, LYMPH, BASO and LYMPH# showed a downward trend, while NEUT，NEUT# and PCT rose at first and then fell. There were significant differences in all 27 indexes except TIBC, MCH and PCT, between the Tibetan and Han children. WBC, RBC, Fe, Hb, HCT, PLT and MCV were higher in Han children than Tibetan children, MCHC, RDWCV, PDW, UIBC, MPV, RDWSD were just the opposite. Conclusion The reference values of blood routine and serum iron of Tibetan children changed with age mainly in the same way as Han children. The range of RBC, Hb and PLT, were wider and had a whole right shift, while the range of WBC was wider and had a whole left shift. The estimation of blood routine and serum iron in Tibetan children should be based on the local normal value.
Objective: An investigation about esophageal gastroduodenoscopy (EGD) was performed, and the association between the symptoms or history and significant findings was analyzed.Methods: This cross-sectional study was conducted through a questionnaire formulated by ourselves. Then we chi-square statistical test and multivariate Logistical regression were used to analyze the correlation between indications and significant findings. Results: The study was conducted from June 10, 2016 to October 7, 2016. And 1 298 cases (male 766) were enrolled. The age was from 0.1 to 17.6 (average age: 8.0±0.01) years. Patients with abdominal pain accounted for 78.0%, then anorexia 31.3%, having breath 24.9%, having evidence for Helicobacter pylori infection 18%, nausea 17%, vomiting 16.7%, having a family history of Helicobacter pylori infection 13.4%. Others were all below 10%. There were 49.9 % having a significant lesion through EGD. Evidence of infection of helicobacter pylori (OR=5.10, 95% CI: 3.58 ~7.28), vomiting (OR=1.48, 95% CI: 1.08~2.04)and suspicious foreign body injection 3.25（95%CI: 1.31~8.02）were related with significant findings. While abdominal pain was related with significant findings (OR=0.73, 95% CI: 0.54~0.98), further investigation was made for abdominal pain. And it was found that the degree value of abdominal pain beyond 5 was related with significant findings (OR=1.37, 95% CI: 1.07~1.74). Conclusion78% of EGD patients are with the complain of abdominal pain. Evidence of infection of helicobacter pylori, vomiting and suspicious foreign body injection are positively related with significant findings. And the degree value of abdominal pain beyond 5 is positively related with significant findings.
Objective: To evaluate the efficacy and safety of high-flow nasal Cannulae (HFNC) oxygen therapy in premature infants. Methods: RCT studies were identified by a systematic search in Medline, the Cochrane Library, Web of Science, Embase, Chinese Knowledge Network, VIP, CBM and Wanfang database, from its establishment to March 31, 2017. The primary outcome was hospital mortality and the incidence of mechanical ventilation (tracheal intubation). The secondary outcome included BPD, air leakage, pulmonary infection, nasal trauma,and application of pulmonary surfactant. Results: Eight articles were included in the metaanalysis, and 1 604 children were included, including 799 cases in HFNC group, 805 cases in NCPAP group. 8 articles were reported in the random sequence of the computer, 7 mentioned the use of the allocation of hidden, 8 papers were lost, data integrity was good, the Results: of the reports were not selective, other bias was not clear. There was no significant difference in mortality and intubation rate between the two groups. The tracheal intubation rate of NCPAP was lower than HFNC group in children with mean weight ≥1 500 g（OR=1.58,95％CI：1.11～2.26，P=0.01）and gestational age more than 28 weeks（OR=1.58,95％CI：1.13～2.22，P=0.008）.There was no significant difference in the incidences of BPD, air leakage and pulmonary infection and the rate of pulmonary surfactant application between the two groups.The incidence of nasal trauma HFNC group was lower than that of NCPAP （OR=-0.14,95％CI：-0.19～-0.09，P＜0.0001）.ConclusionThe efficacy and safety of HFNC in premature infants should be further confirmed, but it has obvious advantages in reducing the incidence of nasal injury.
Objective: To investigate the carriage rate, serotype distribution and antibiotic resistance profiles of Streptococcus pneumoniae (S.pneumoniae) among hospitalized children with respiratory tract infections in grade two hospital in Zhongjiang County, Sichuan and to determine the epidemiological characters of these S.pneumoniae isolates. Methods: Nasopharyngeal swabs of hospitalized children with respiratory tract infections aged from 1 month to 14 years were collected from January to December 2015 at the Zhongjiang County Hospital, and S.pneumoniae was cultured. The serotypes were detected by capsule swelling experiment to assess the coverage rate of 13 valent pneumococcal conjugate vaccine (PCV13). The sensitivity against 16 antimicrobial agents were detected by E-test or disk diffusion method for approximately half of S.pneumoniae isolates randomly selected based on the serotypes. Results: The nasopharyngeal carriage rate of S.pneumoniae was 18.4% (199/1082). The common serotypes were 19F (14.6%), 19A (10.6%), 34 (10.1%), 6A (9.0%), 23F (7.5%), 6B (7.5%) and 23A(7.0%). The coverage rate of PCV13 was 54.8%. The proportion of non-PCV13 types was up to 45.2%. All of the isolates were sensitive to penicillin using the parenteral breakpoints, but the sensitive rate decreased to 9.1% for the oral penicillin breakpoints. The sensitivity rate of amoxycillin/clavulanate was 97%. All of the isolates were sensitive to ceftriaxone, vancomycin, levofloxacin and linezolid. However, all of the isolates were resistant to erythromycin and azithromycin.ConclusionColonization of S.pneumoniae was frequent in the nasopharynx of children with respiratory tract infections in Zhongjiang. About half of the S.pneumoniae isolates were identified as the PCV13 types. Non-PCV13 serotypes were common in the county, in which type 34 and 23A were frequent, which should be monitored for long time and in wider range. The isolates showed a high resistance to oral penicillin and erythromycin, but sensitive to parenteral penicillin, ceftriaxone and vancomycin.
Objective: To investigate the clinical characteristics and prognosis of chronic inflammatory demyelinating polyneuropathy (CIDP) in children. Methods: Clinical data were collected and analyzed from patients with CIDP who visited the Department of Neurology, Children's Hospital of Fudan University between January 1,2008 and January 31,2017. PubMed databases were retrieved from January 1991 to February 2017 with CIDP cases in children. According to the peak time of the disease(4-8 weeks for subacute onset group, >8weeks for chronic onset group), the retrieved literature cases and the cases in the study were aggregated and divided into two groups, They were compared in clinical manifestation, laboratory examination, treatment and prognosis. Results: Firstly, there were 15 CIDP cases who met the criteria of inclusion and exclusion, including 6 males and 9 females; 4 cases between 1-3 years old, 4 cases between 4-7 years old, 7 cases between 8-15 years old; the age of disease onset was 1-11 years old;7 patients had a history of prodromal infection; development to the peak of the disease required 4 weeks to 13 months, with an average (12.93±13.57) weeks. At the beginning of the disease all cases showed dyskinesia;4 cases with sensory disorder; the peak of the disease MRS score: 3 points in 8 cases, 4 points in 6 cases and 5 points in 1 case; the course of disease was monophase in 3 cases, progressive in 5 cases and recurrent in 7 cases;all cases had nerve electrophysiological changes; in all cases dissociation of protein from cell in CSF occurred. 14 cases were treated with glucocorticoid, 1 case was treated with intravenous immunoglobin; 1 case was discharged from hospital voluntarily and 14 cases were hospitalized between 7-17 days with the clinical symptoms improved;during the follow-up period, 7 cases were relapsed.Secondly, 87 cases were retrieved from PubMed, together with our 15 cases，102 cases were analyzed. There were 38 patients with subacute-onset disease and 64 patients with chronic-onset disease. Patients exhibited symptoms in less than 2 months with more often sensory abnormalities (57.1% vs 23.5%，P=0.002), and cranial nerve abnormalities (25.7% vs 7.8%，P=0.023). There was no significant difference between the two groups in gender, age of disease onset and movement disorders. Patients with subacute-onset relapsed more than patients with chronic-onset(62.2% vs 34.0%，P=0.010).There were more monophasic evolution in chronic-onset disease than in subacute-onset disease (50.8% vs 27.8%,P=0.026).Conclusion: Children with subacute onset of CIDP are slightly more than adults, and their sensory abnormalities and cranial nerve abnormalities are more frequent than children with chronic onset of CIDP, and they are prone to relapse during disease development. Dyskinesia is common in children with CIDP, and cranial neuropathy and respiratory failure are unusual. Nerve electrophysiological changes and dissociation of protein from cell in CSF are common in CIDP, and early diagnosis and initial treatment are important.
Objective: To summarize and review the clinical data of two children with lysinuric protein intolerance so as to improve its knowledge. Methods: Clinical data of two cases with lysinuric protein intolerance were summarized, including clinical manifestations, laboratory findings, renal pathological changes and family investigation. This study used next generation sequencing to screen all exons of genome in proband and her parents. Significant variants detected by next generation sequencing were confirmed by conventional Sanger sequencing and segregation analysis was performed using parental DNA and her brother samples.Results: The proband, a 10-year-old girl, presented with recurrent vomiting and episodes of diarrhea, aversion to protein-rich food and failure to thrive after weaning. She often had nasal hemorrhage since the age of 5 years. Peripheral blood cell count suggested white blood cell, red blood cell and platelet count were all under normal value. She had mild proteinuria and persistent microscopic hematuria at the age of 9.7 years. At the same time, laboratory tests showed that serum ferritin, lactate dehydrogenase and ammonia increased, and orotic acid increased in urine, but lysine, arginine and citrulline were not changed significantly in serum and urine. The pathology of renal biopsy suggested lupus nephritis. The proband's younger brother, 6.5-year-old, presented with recurrent vomiting, aversion to protein-rich food and failure to thrive after weaning. He had no episodes of diarrhea. laboratory tests also showed that serum ferritin, lactate dehydrogenase and ammonia increased, and orotic acid increased in urine, but lysine, arginine and citrulline were not changed significantly in serum and urine. Whole exon sequencing was performed in core family, including proband and her parents. Homozygous c.625+1G>A mutation in SLC7A7 gene was detected in proband, which was from her parents. The mutation was confirmed by Sanger sequencing in core family. The same mutation was found in proband's younger brother by Sanger sequencing. The proband was diagnosed as LPI complicted with SLE. The proband's younger brother was diagnosed as LPI.Conclusion: Due to the heterogeneity of LPI and lack of understanding of LPI for clinicians, it is easy to cause misdiagnosis or miss diagnosis. The SLC7A7 gene sequencing is the basis for diagnosis. LPI patients with systemic lupus erythematosus (SLE) is very rare. LPI patients complicated with SLE need glucocorticoid or immunosuppressive therapy. Mutations in SLC7A7 gene can cause SLE. whether SLC7A7 is one of the genes causing a single gene type SLE needs further study.
Objective: To discuss the clinical manifestation of DiGeorge syndrome complicated with hyperthyroidism in a child to raise awareness of the disease.Methods: To report a case of DGS complicated with hyperthyroidism symptoms, auxiliary examination results and peripheral blood genomic DNA chromosome microarray results, treatment and follow-up, by retrieving literatures of DGS combined with hyperthyroidism of child in CNKI and PubMed .The retrieval time was from the database was created to May 31, 2017. The relevance of clinical characteristics and cytogenetic abnormalities was summarized.Results: A 12 years old girl visited the First Affiliated Hospital of Zhejiang University for "seizures occurred 2 times in one year, thyroid disorders for four months " .The Child has hypocalcemic tetany, hyperthyroidism, microsomia, mental abnormality, anemia, chronic otitis media and other diseases. The chromosome microarray detection showed 22q11.21 microdeletion, and the deletion size was 2512 kb ,the genome location was 18919095-21431174 (hg19), and was diagnosed as DGS complicated with hyperthyroidism. She was treated with thyrozol (Bid, started at 10 mg, then changed to 5 mg after 2 days), meanwhile, calcium supplementation and other symptomatic treatment were given. When the blood calcium reached the normal range, she was discharged.The patient was advised to continue treatment by thyrozol (5mg, qd, po) , and to be followed-up for the thyroid function, serum calcium and so on. In March and June, telephone follow-up was made for the child,and her parents complained no seizures after discharged. A total of 10 articles of English literatures were retrieved in PubMed, and 17 cases of DGS combined with hyperthyroidism were reported, the conditions were different, and more systems were involved.Conclusion: DiGeorge syndrome had variable clinical manifestations and types, involving a wide range of systems .Therefore, it was difficult to diagnose clinically, prone to misdiagnosis, missed diagnosis and delayed diagnosis.The patients with primary parathyroid dysfunction should be inspected for routine chromosome microarray analysis and evaluated for the function of thyroid.
Objective: To observe the changes of polyunsaturated fatty acid metabolism in Balb/C mice model of ovalbumin(OVA)induced allergy. Methods: At 21 day old, 24 baby Balb/C mice, whoes mother had a diet of n-3PUFA deficiency, randomly divided into 3 groups, blank, OVA and PBS groups. There were 8 offsprings in each group. Weaned BALB/c offsprings in group h, m and l were sensitized with 50 μg of OVA (grade V, Sigma) in the presence of 1.3 mg of aluminum hydroxide gel (Sigma) as an adjuvant by intraperitoneal injection twice at 3 and 5 weeks old. Half of the offsprings delivered from the mice of the control group were sensitized with OVA as positive control. And remainders of the offsprings were injected with PBS as negative control.The sensitized BALB/c mice with 7 weeks of life were fed with 50 mg of OVA dissolved in 0.3 mL of sterile saline by intragastric needles at interval day for six times. One hour after the last oral administration, all BALB/c mice were sacrificed with excessive carbon dioxide and blood samples were collected by cardiac puncture.At the first day of the test, blood samples of baby mice in blank group were collected by cardiac puncture. The offsprings in group OVA were sensitized with 50 g of OVA dissolved in 0.2 mL PBS in the presence of 1.3 mg of aluminum hydroxide gel by intraperitoneal injection at 1 and 15 day. From 29 to 39 day, the offsprings in OVA group were fed wiht 50 mg of OVA dissolved in 0.3 mL of PBS by intragastric needles at interval day for six times. The offsprings in PBS group were given equivalent PBS. After the last oral administration, all BALB/c mice were sacrificed by cardiac puncture and blood, jejunum, spleen samples were collected. To establish the model of OVA-induced allergy, diarrhea was observed after OVA challenge; histological examinations of jejunum were performed by HE staining and the mast cells in jejunum were observed by toluidine blue staining; and the levels of OVAsIgE in serum, IL-4 and IFN-γ in spleen cell culture supernatants were measured by ELISA. Fatty acids were extracted from serum in each group. The linoleic acid (ALA), inolenic acid (LA), arachidonic acid (AA), eicosapentaenoic acid (EPA) and docosahexenoic acid (DHA) levels of serum were analyzed by high performance liquid chromatography.Results：①The model of OVA-induced allergy in Balb/C mice was established: compared with group PBS, after the oral OVA challenge allergic diarrhea, gathered phenomenon of inflammatory cells in jejunum villus，aggregation of mast cells in lamina propria（P＜0.05），increasing of the levels of OVA-specific IgE in serum and IL-4, IL-4/IFN-γ in spleen cells(P＜0.05) were observed in the offspring of OVA group. ②The levels of DHA, EPA and LA of mice in group PBS were statistically lower than those in blank group. Compared with PBS group, the levels of DHA decreased in mice of OVA group.Conclusion：Balb/C mice，repeatedly exposed with OVA, exerted a fatty acid metabolic disorder, mainly a reduction of DHA.
