Objective:To explore the risk factors for lymphoproliferative disorders (PTLD) in children with thalassemia major (TM) after allogeneic hematopoietic stem cell transplantation (allo-HSCT).Methods:This was a retrospective case-control study.A total of 482 children with TM who underwent allo-HSCT at Shenzhen Children′s Hospital between January 2020 and December 2022 were selected and classified into the PTLD and non-PTLD groups according to the occurrence of PTLD.The risk factors for PTLD after allo-HSCT in children with TM were analyzed, and the diagnostic efficiency of relevant risk factors for PTLD was analyzed by receiver operating characteristic (ROC) curve.Results:A total of 25 out of 482 patients (5.2%, 25/482) developed PTLD about 114 (54-271) days after allo-HSCT.Among them, 12 cases (12/25, 48.0%) occurred within 100 days, and 22 cases (22/25, 88.0%) occurred within 1 year after allo-HSCT.Univariate analysis showed that there were significant differences in gender composition, type of transplant donor, number of natural killer cells and B lymphocytes in peripheral blood at 30 days after allo-HSCT, positive rate of plasma Epstein-Barr virus deoxyribonucleic acid (EBV-DNA) and incidence rate of acute graft-versus-host disease (aGVHD) between the 2 groups (all P<0.05).Multivariate Logistic regression analysis showed that female ( OR=3.196, 95% CI: 1.144-8.929), positive plasma EBV-DNA ( OR=17.523, 95% CI: 5.449-56.344) and aGVHD ( OR=3.156, 95% CI: 1.161-8.575) were independent risk factors for PTLD after allo-HSCT in TM children (all P<0.05).The ROC curve analysis showed that positive plasma EBV-DNA had an excellent accuracy in predicting the occurrence of PTLD after allo-HSCT (sensitivity was 0.796, specificity was 0.800, area under the curve was 0.803).If combined with aGVHD and gender, the area under the curve for the prediction of PTLD increased to 0.831. Conclusions:Female, positive plasma EBV-DNA and aGVHD are independent risk factors for PTLD after allo-HSCT in children with TM.It provides useful early warnings for the prediction and prevention of PTLD.

Objective To analyze the etiological characteristics and the variation of pathogens spectrum in hospitalized children with a-cute respiratory tract infection(ARTI)before and after COVID-19 under"the level B of management for class B"of infectious diseases(Level B for Class B)in Shenzhen,in order to provide reference for the clinical diagnosis,treatment and prevention of ARTI.Meth-ods The ARTI cases from January 8,2022 to July 30,2022 were selected as before"Level B for Class B",and the cases from Janu-ary 8,2023 to July 30,2023 were selected as after"Level B for Class B".The pharyngeal swab samples submitted for analyzing 11 common pathogens,such as COVID-19,influenza virus(Ⅳ),respiratory syncytial virus(RSV)and mycoplasma pneumoniae(MP)in the children with ARTI admitted to Shenzhen Children's Hospital.Results SARS-CoV-2 were detected as positive in 347 cases,a-mong which 225 cases were before"Level B for Class B"including 29 cases combined with other pathogens(12.89％,29/225)and human parainfluenza viruses(HPIV)was the most common(31.03％,9/29).After"Level B for Class B",SARS-CoV-2 were detec-ted as positive in 122 cases,including 28 cases combined with other pathogens(22.95％,28/122),and RSV was the most common(28.57％,8/28).There was a statistical difference between the positive rate of SARS-CoV-2 combined with other pathogens before and after"Level B for Class B"(X2=5.834,P=0.016).After"Level B for Class B",the total pathogen detection rate(positive for at least one pathogen)was 60.82％(2 864/4 709)in the spring(January 8,2023 to April 30,2023),and influenza virus A(IVA)(22.64％,1 066/4 709),rhinovirus(HRV)(19.86％,935/4 709)and RSV(13.29％,626/4 709)were the main pathogens,and there were 301 cases(6.39％,301/4 709)of mixed infections.In the summer(May 1,2023 to July 30,2023),the total detection rate of pathogens was 70.26％(4 012/5 710),among which RSV(21.63％,1 235/5 710),MP(13.91％,794/5 710),HPIV(10.05％,574/5 710)were the main pathogens,and there were 710 cases(12.43％,710/5 710)of mixed infections,all of which were significantly higher than the same period before"Level B for Class B".The difference was statistically significant(P＜0.05).Conclusion After"Level B for Class B"for COVID-19,the detection rate of 11 common pathogens increased significantly and the pathogen spectrum of ARTI changed significantly.

Objective:To establish the biology reference interval (RI) of peripheral blood procalcitonin (PCT) for children between 3 days and 6 years old in China.Methods:Totally 3 353 reference individuals with apparent health or no specific diseases were recruited in 18 hospitals throughout the country during October 2020 to May 2021. Reference individuals were divided into four groups: 3-28 days, 29 days - 1 year, 1-3 years and 4-6 years. Vein blood or capillary blood were collected by percutaneous puncture from every reference individual. The PCT level in serum and the capillary whole blood were assayed by Roche Cobas e601 and Norman NRM411-S7 immunoanalyzer. Outliers were deleted and 95th percentiles of every group were provided as RIs. Man-Whitney U test or Kruskal-Wallis test were used performed to assess the difference among different gender, age or method groups. Results:The difference of PCT distribution between male and female is not statistically significant, but the difference between serum and capillary whole blood is statistically significant. The differences between age groups are significant too. For Roche e601, serum PCT RI of 3-28 days group is <0.23 μg/L, 29 days - 6 years are <0.11 μg/L. For NRM411, Serum PCT RI of 3-28 days group is <0.21 μg/L, 29 days - 1 year: <0.09 μg/L, 1 - 6 years: <0.10 μg/L. For whole blood PCT, RI of 3-28 days group is <0.26 μg/L, 29 days - 6 years is <0.15 μg/L.Conclusions:Serum and capillary whole blood PCT have different RIs, however, capillary whole blood PCT testing is valuable in pediatric application. Children in 3-28 days show higher PCT levels than other age group. To establish the RIs and understand the differences among different groups are essential for the interpretation and clinical application of peripheral blood PCT testing results.

Objective:To identify gene variants and investigate clinical features of nonmuscle myosin heavy chain 9-related disease (MYH9-RD).Methods:In this retrospective study, the data of patients with MYH9-RD admitted to Shenzhen Children′s Hospital from July 2017 to September 2020 were extracted. The gene variants, clinical features and laboratory tests results were summarized.Results:Among the 6 children, 4 were males and 2 were females, aged 4.0 (0.5-7.6) years. Main clinical manifestations included thrombocytopenia (6 cases), epistaxis (3 cases), petechias (2 cases), traumatic hematoma (1 case), and abnormal liver enzymes (1 case). One patient had no family history, and the other 5 cases were pedigrees. Two pedigrees (2 cases) had long-term microscopic hematuria, one pedigree (2 cases) had history of early cataract, and three pedigrees (5 cases) had chronic mild elevation of liver enzymes. Four MYH9 gene variants were found in 12 patients, including c.2104C>T(p.R702C) in exon 17, c.4270G>A(p.D1424N) in exon 31, c.5521G>A (p.E1841K) in exon 39, and c.5797C>T (p.R1933X) in exon 41. According to the family pedigrees analysis, except for the case of variant in exon 17 which was spontaneous mutation with no family history, the other variants were from their father or mother. The complete blood count results showed a decreased platelet number in these patients, and the counting results of the automated hematology analyzer were significantly lower than that of manual counting method ((33.4±17.2) × 10? vs. (60.4±21.0) × 10 9/L, t=-5.83, P<0.05). The examination of the peripheral blood smear revealed the presence of thrombocytopenia with giant platelets and granulocyte inclusion bodies. The MYH9 gene variant (R702C) located at the N-terminus head domain of non-muscle myosin heavy chain ⅡA (NMMHC-ⅡA), which has ATPase activity, led to severe reduction of platelet number (<20×10 9/L) and obscure granulocyte inclusion bodies. However, higher platelet numbers (40×10 9-80×10 9/L) and obvious granulocyte inclusion bodies were observed in patients with tail-position mutations at C-terminus. Conclusions:The clinical phenotypes of MYH9-RD were variable. The mutations in certain regions of MYH9 gene were related to platelet count and granulocyte inclusion bodies. MYH9-RD should be considered in individuals with unknown etiology and persistent thrombocytopenia which is non-responsive to conventional treatment, regardless of family history. Complete blood count and blood smear morphology examinations are the first steps to screen and diagnose the disease. The laboratory should pay attention to the morphological review rules and standardized reports.

Objective: To study the characteristics of clinical diagnosis and treatment for 3 children with phytosterolemia. Methods: The different clinical manifestations of 3 children with phytosterolemia were retrospectively reviewed. The case 1 and case 2, who were 7 years and 2 months old twin sisters, hospitalized for frequent epistaxis and abdominal pain. The case 3, who was 5 years and 7 months old male, came to the hospital for cutaneous xanthoma. The phytosterol levels in serum of the children were analyzed by gas chromatography-mass spectrometry, and the second generation sequencing method was used to analyze the disease-causing gene. Sanger sequencing method was used to verify the ABCG5 gene mutation and parental source. Results: (1) The case 1 and case 2 showed moderate anemia, raised reticulocytes, total bilirubin and indirect bilirubin as well as splenomegaly. The blood smear showed that there were more irregular red blood cells, such as oral red blood cells, increased large/giant platelets, and ristomycin-induced platelet aggregation test was decreased. The urine routine examination indicated that there was bleeding in the urinary system. The results of blood lipid test were almost normal. The case 3 showed mild anemia with normal shape of erythrocyte and normal size of spleen. The large/giant platelets increased. The results of platelet aggregation test, bilirubin and urine routine examination were in normal range, but the levels of total cholesterol and low-density lipoprotein cholesterol increased significantly. (2) The levels of serum phytosterol were significantly increased in all the 3 children. (3) Two heterozygous mutations were detectable in ABCG5 gene of case 1 and 2 which were complex heterozygous mutation, i.e., c.9041G＞A and c.751C＞T. The variations were from their father and mother respectively. In case 3, only one homozygous mutation was detectable in ABCG5 gene which originated from their parents. Conclusion When the child showed increased large/giant platelets, hemolytic anemia, erythrocytosis or xanthoma of skin and rised total cholesterol and low-density lipoprotein cholesterol at first visit, the possibility of phytosterolemia should be considered. The blood phytosterol content and gene detection should be carried out as early as possible in order to treat early and improve prognosis.

Objective To evaluate the creditability of warning message of white differential count (WDF) and white precursor cell (WPC) channels in Sysmex XN-3000 hematology analyzer,and verify its optimal threshold and adjust the alarm threshold.Methods A total of 61 EDTA-K2 anticoagulated blood samples without abnormal warning and 521 EDTA-K2 anticoagulated blood samples with abnormal warning were simultaneously detected in WDF and WPC channels.After the smear specimens of blood sample were automatically prepared by the instrument,microscopic examinations were performed manually.The results of microscopic examination were considered as the gold standard to determine the reliability of the warning message from the instrument and verify the reasonability of initial warning threshold value provided by the manufacture.Consequently,the threshold values were adjusted based on the requirements in practical work.Results The warning messages of atypical lymphocytes and blasts/abnormal lymphocytes in WDF channel were higher sensitive (95.8％ and 100％ respectively),but lower specific (34.7％ and 23.5％ respectively) compared with microscopic examination.The warning messages of atypical lymphocyte,blasts and abnormal lymphocytes in WPC channel were lower sensitive (81.3％,66.7％,and 76.5％ respectively) but higher specific (61.9％,55.5％ and 88.3 ％ respectively) compared with microscopic examination.According to the ROC curve analysis,the prognostic values of warning message of microscopic examination were of medium level,except the warning message for abnormal lymphocytes was poor compared with WPC channel.Combining the practical retest rules,the optimal critical threshold values of atypical lymphocytes and blasts/Abn lymph in WDF channel were adjusted as 120,and they were adjusted as 140 in WPC channel.Conclusion The high sensitive WDF channel should first be used for screening,and the detectable warning message could be retested by using high specific WPC channel to shorten the turnaround time of the test results and improve the working efficiency.The initial critical warning threshold provided by the manufacture should be verified and adjusted to the optimum critical threshold in order to ensure the accuracy of test results.

Objective To analyze the difference of serum levels of anti-Mullenan hormone (AMH) in chil-dren with different ages and different types of cryptorchidism,and to explore its role in the evaluation of tes-ticular development.Methods 60 children with simple cryptorchidism were selected as case group and 52 healthy children were selected as control group.The levels of serum AMH in two groups of children were measured and the differences were compared.Results (1)The level of AMH in the case group was lower than that in control group (P < 0.05),and there was no statistical significance between two subgroups of >6 to 11 years old children with cryptorchidism and healthy children (P>0.05).(2)The level of AMH in bi-lateral cryptorchidism group was lower than that in unilateral cryptorchidism group (P<0.05),and there was no significant difference between two subgroups of >6 to 11 years old children with bilateral cryptorchidism and unilateral cryptorchidism (P>0.05).(3)The level of AMH in the high level cryptorchidism group was lower than that of the low level cryptorchidism group (P<0.05),and there was no statistical difference be-tween between two subgroups of 3~11 year old children with cryptorchidism and low level cryptorchidism (P>0.05).(4)AMH level was negatively correlated with age,and positively correlated with testicular devel-opment.Conclusion AMH can be used as an important indicator of testicular development in children with cryptorchidism.

Objective To evaluate the effect of dexmedetomidine pretreatment on expression of se-cretion protein of Clara cell secretory protein(CC16)during endotoxin-induced acute lung injury(ALI) in rats.Methods One hundred and twelve healthy Wistar rats of both sexes, aged 8-12 weeks, weighing 250-350 g, were divided into ALI group(n=56)and dexmedetomidine pretreatment group(group DEX, n= 56)using a random number table.ALI was induced by intravenously injecting lipopolysaccharide (LPS)5 mg∕kg over 1 min.Dexmedetomidine 10 μg∕kg was intravenously infused over 10 min starting from 10 min before LPS in group DEX.At 10 min before LPS injection and 0.5, 1, 2, 4, 6 and 24 h after LPS injection, 8 rats were sacrificed and lungs were removed for examination of the pathological changes(with a light microscope)and ultrastructure of Clara cells(with a transmission electron microscope)and for deter-mination of CC16 expression in bronchioles(by immunohistochemistry). Results Compared with the baseline at 10 min before LPS injection, the expression of CC16 in bronchioles was significantly down-regu-lated at 1, 2, 4, 6 and 24 h after LPS injection in group ALI and at 1, 2, 4 and 6 h after LPS injection in group DEX(P<0.01), the pulmonary small arterial hyperemia, alveolar septa edema, red blood cell exudation and inflammatory cell infiltration were found, the Clara cells in bronchioles were reduced, the secretory granules in the cytoplasm were reduced, and the mitochondria were swollen and deformed after in-jection of LPS.Compared with group ALI, the expression of CC16 in bronchioles was significantly up-regu-lated at 1, 2, 4, 6 and 24 h after LPS injection(P<0.01), the pathologic changes of lung tissues were significantly attenuated, and the number of Clara cells was increased in group DEX.Conclusion The mechanism by which dexmedetomidine pretreatment reduces endotoxin-induced ALI may be related to up-regulating CC16 expression in rats.

To study the current situation of Chinese medicine services from both perspective of supply and demand, looking to promote the healthy development of Chinese medicine community health service strategy. Questionnaire survey was conducted to practitioners of 14 community health service centers in Longhua district (80) and service objects (800). Ratios and SWOT methods were used for analysis. 75 qualified practitioners and 736 qualified service objects questionnaire recoveries showed that there are different degrees of commands from both sides, different options on therapeutic methods and lack of practitioners. It is suggested that community health service center should supply TCM service according to different time, different place and different patient on different type and levels.

Objective To study the plasma biochemical indexes among the hypertension in Xining area.Methods According to diagnosis standard of hypertension,104 males who emigrated to Xining area were divided into hypertension group and control group,height,weight,blood routine,renal function,blood lipid of the two groups were measured.Results Among the 104 subjects, 17 cases were hypertensive patients,and other 87 cases were served as control group.The body weight,blood uric acid levels in hy-pertension group were significantly higher than those in the control group(P <0.05),the other indexes had no significant difference between two groups.Conclusion The uric acid may be a risk factor for hypertension people living in plateau area,and the mecha-nism need to be further studied.