Histiocytic Necrotizing Lymphadenitis ; Humans

Objective:To study the predictive values of the general movements (GMs) assessment combined total bilirubin for motor development outcomes in infants with severe neonatal jaundice.Methods:From June of 2014 to June of 2019, infants with severe neonatal jaundice in Chenzhou First People′s Hospital were enrolled in the study. Inclusion criteria included , the serum total bilirubin was measured at the time of admission, corrected gestational age of 37 to 48 weeks. General assessment were carried out when the infant was stable. The patients were regularly followed-up until the age of 12months to evaluate the predictive values.Results:A total of 204 patients with severe neonatal jaundice were enrolled in the study, with mean serum total bilirubin value (485.4±109.6)μmol/L. They were divided into two groups according to the outcome of motor development. The total bilirubin value, the proportion of abnormal GMs and dangerous total bilirubin level in the abnormal group were significantly higher than those in the normal group (all P<0.05). 13 cases (6.4%) were normal in the torsion stage of GMs; 191 cases (93.6%) were abnormal, including 164 cases (85.9%) of poor repertoire (PR) and 27 cases (14.1%) of cramped-synchronized (CS). Abnormal GMs and total bilirubin were the risk factors of abnormal motor development ( OR=4.651, 1.017, P<0.05). The predictive values of abnormal GMs for abnormal motor development outcomes were as following: sensitivity 100%, specificity 8.4%, negative predictive value (NPV) 100%. The predictive values of CS for cerebral palsy were as following: sensitivity 63.2%, specificity 97.8%, NPV 96.0%. Receiver operating characteristic (ROC) curve showed that the area under the curve predicted by GMs and total bilirubin was 0.765 and 0.757, respectively. The area under the curve of motor dysplasia predicted by combining the two was 0.854. Conclusions:The evaluation of general movement assessment combined total bilirubin has certain clinical predictive value for the outcomes of motor development in infants with severe neonatal jaundice.

OBJECTIVETo investigate the preventive effects of garlic oil (GO) on benzene-induced hematotoxicity in mice.

METHODSSpecific pathogen-free male Kunming mice were randomly divided into 5 groups, i.e., control group, model group, and low-, middle-, and high-dose GO groups (n = 20 in each group). Mice in GO groups were orally given GO at 20, 40, or 80 mg/kg BW, while mice in the other two groups received an equal volume of corn oil. Two hours later, mice in model group and GO groups were orally given benzene (20%, v/v, dissolved in corn oil, 10 ml/kg BW) for 21 days consecutively. On the 22nd day, blood was collected from the orbital sinus, to determine the counts of red blood cells (RBC), white blood cells (WBC), and platelets (PLT) and hemoglobin level using an automatic blood cell counter. The mice were sacrificed thereafter. The spleen was excised and weighed for calculation of the spleen index (spleen weight/body weight×100%).

RESULTSThe counts of WBC, RBC, and PLT and Hb level in the model group were reduced by 40%, 18%, 28%, and 23.6%, respectively, as compared with those in the control group (P < 0.01). Compared with those in the model group, WBC and PLT counts in the high-dose GO group increased by 95% and 66%, respectively (P < 0.01), wherein lymphocytes and monocytes increased by 142% and 100%, respectively (P < 0.01); the RBC count and Hb level in the low-dose GO group increased by 15% and 16%, respectively (P < 0.05). GO significantly suppressed benzene-induced decreases in spleen weight and spleen index.

CONCLUSIONGO is capable of suppressing benzene-induced hematotoxicity in mice. One possible mechanism may be promotion of hematopoiesis in the spleen.

Allyl Compounds ; pharmacology ; Animals ; Benzene ; poisoning ; Blood Cell Count ; Disease Models, Animal ; Garlic ; chemistry ; Male ; Mice ; Plant Oils ; pharmacology ; Sulfides ; pharmacology

OBJECTIVETo identify the epidemiological and clinical features of unexpected sudden cardiac deaths (SUD) in Yunnan.

METHODSChoosing the old SUD cases from Xiangyun, Heqing, Nanjian and Dayao counties and using the standardized verbal autopsy Form, we interviewed the family members of the cases, witnesses and doctors as well as reviewing their medical files to get relative information.

RESULTSWe identified 116 SUDs in 21 villages from 1984 to 2004. The village-specific annually standardized incidence rates were ranged from 0.2/1000 to 8.9/1000 (median = 0.8/1000). 66% and 29% of the SUDs occurred in July and August respectively. The incidence rates of SUD were higher (1.6/1000, chi(2) = 16, P < 0.01) in 10 - 39 year-olds, and higher in females than in males (RR = 1.6, 95% CI: 1.1 - 2.3). Seventy percent of SUD occurred in families having clustering nature and 60% of the additional cases in the family were occurred within 24 hours (median = 20 hours) after the first SUD identified in the family. SUD occurred in 23 families followed the first affected family in a village during the same season. In these 23 families, 61% of the first SUD occurred within 8 days after the first SUD in the first affected family. 68% and 66% of the SUDs did not have any complaints or signs during the last 3 weeks or from 3 weeks to 2 days prior to the onset of the disease. 63% of the SUDs had cardiac symptoms within the last 2 days prior to the onset with major symptoms as dizziness, nausea, faintness, unconsciousness, weakness and palpitation. The median duration from acute onset to death was 2 hours.

CONCLUSIONSThe extreme time-space clustering of SUD in families and in villages suggested that the risk factors occurred in specific time and location. Familial clustered SUD cases had common exposure pattern. Sudden onset of acute cardiac symptoms often followed by sudden death. Epidemiological study on new cases was necessary to identify risk factors and to develop hypothesis for causation. In July 2005, we instituted a special SUD surveillance system for all the affected counties together with 10 counties which had no reported cases.

Adolescent ; Adult ; Age Factors ; Child ; China ; epidemiology ; Death, Sudden, Cardiac ; epidemiology ; Female ; Humans ; Incidence ; Interviews as Topic ; Male ; Retrospective Studies ; Risk Factors ; Sex Factors ; Space-Time Clustering ; Young Adult

OBJECTIVETo analyze the mutation rate and clinical characteristics of CALR, MPL W515K and JAK2 V617F genes in patients with primary thrombocythemia (PT).

METHODSFifty-six patients with PT were selected as the research objects in our hospital. The CALR and MPL W515K gene mutations were determined by genomic DNA-PCR direct sequencing of the PCR products, and the JAK2 V617F gene mutation was detected by allele specific PCR method.

RESULTSAmong the 56 patients with PT there were 14 cases of CALR gene mutation with the incidence rate of 25%, including 6 cases of type I, 5 cases of type II and 3 cases of type III. The sex, age, platelet(Plt) count, white blood cell (WBC) count and hemoglobin (Hb) level in the type I case of CALR gene mutation all were not significantly different from that in type II and III(all P>0.05); the WBC level in type III group significantly increased in comparison of type II group (P<0.05), while the sex, age, Hb and Plt levels showed no significant difference between the type III and type II groups (P>0.05). There were 3 cases of MPL W515K gene mutation with the incidence rate of 5.36%; 21 cases of JAK2 V617F gene mutation with the incidence rate of 37.50%. There were 13 cases of CALR gene mutation in negative patients with MPL W515K and JAK2 V617F (18 cases) with 72.22% incidence rate (13/18), and there was no cases of 1 or 2 gene mutations coexisted. The levels of Hb and WBC in peripheral blood of patients with CALR mutation were significantly lower than those of JAK2 V617F mutation (both P<0.05). In 56 cases, there were 3 cases of abnormal karyotype, with the incidence rate of 5.36%. The mutation rate of CALR gene in abnormal karyotypes (66.67%) was significantly higher than that of normal karyotypes (20.75%) (P<0.01).

CONCLUSIONThe incidence of JAK2 V617F gene mutation increases in the patients with primary thrombocythemia; CALR mutation rate is higher in the patients with negative MPL W515K and JAK2 V617F gene mutation, which may closely correlate with abnormal karyotype; the levels of peripheral Hb and WBC in PT the patients with CALR gene mutation are significantly lower than those in patients with JAK2 V617F mutation.

Calreticulin ; Humans ; Janus Kinase 2 ; Mutation ; Mutation Rate ; Receptors, Thrombopoietin ; Thrombocythemia, Essential

Objective To analyze the death status and main causes of death among children under 5 years old in Changsha from 2016 to 2021, and to provide a scientific basis for formulating preventive measures for children's health care. Methods The data of 1 761 deaths of children under 5 years old in Changsha City from 2016 to 2021 were collected, and the mortality trend, the order of causes of death and the utilization of pre-death medical care services were retrospectively analyzed. Results The 7-day neonatal mortality, 28-day neonatal mortality, 0-1-year-old neonatal mortality, and the mortality rate of children under 5 years old (U5MR) in Changsha City from 2016 to 2021 were 0.76‰, 1.28‰, 2.41‰, and 3.86‰, respectively. All the mortality rates showed a decreasing trend (P<0.05). U5MR in males was significantly higher than that in females (P<0.05), and U5MR in rural areas was significantly higher than that in urban areas (P<0.05). The top five causes of U5MR were drowning, premature delivery or low birth weight, pneumonia, other congenital anomalies, and accidental asphyxia, respectively. The death places of children under 5 years old were mainly medical and health institutions, and 81.72% of them were treated in hospitals before death. Conclusion From 2016 to 2021, the mortality rate of children under the age of 5 in Changsha City has gradually decreased. Preventing congenital malformations, reducing preterm birth or low birth weight, improving the treatment level of pneumonia, and preventing accidents such as drowning and accidental suffocation are the key to reducing the mortality rate of children under 5 years old.

OBJECTIVETo investigate the influencing factors and pathogenesis of osteopenia in the patients with hemophilia.

METHODSTwenty-three patients with hemophilia were admitted in the hospital affiliated to North China University of Science and technology from March to August 2015, including 13 severe cases, 10 mild and moderate cases. All the patients accepted the detection of serum I collagen cross-linking N terminal peptide (NTX I), osteoprotegerin (OPG), bone alkaline phosphatase (BALP), basic fibroblast growth factor (bFGF), insulin-like growth factor (IGF) and transforming growth factor-β1 (TGF-β1), the score scale of activity ability was recorded according to the criteria published by the U.S. Center for disease prevention and control in 2002, and 21 patients received the measurement of bone mineral density. According to the World Health Organization (WHO) definition, the clinical significance of bone mineral density (BMD) was assessed by measuring the Z level.

RESULTSZ level>-2 was recorded in 10 cases, Z≤-2 was recorded in 11 cases; the levels of body mass index (BMI) and human bone alkaline phosphatase (BALP) reflecting bone formation in 11 cases (Z≤-2) were lower than there in 10 cases (Z>-2) (P<0.05); the levels of BALP (r=0.489, P<0.05), IGF (r=0.538, P<0.05) and BMI (r=0.572, P<0.01) positively correlated significantly with BMD (P<0.05); the levels of bFGF (r=0.570, P<0.01) and OPG (r=0.505, P<0.05) positively correlated with NTX I, indicating bone destruction (P<0.05); the score of activity ability of severe patients was significantly lower than that of mild and moderate cases (P<0.05), BMD levels of these 2 groups were not statistically different (P>0.05).

CONCLUSIONThe BMD level does not correlate with the clinial grouping of hemophilia, the low body mass index may be a risk factor for bone lose; the mechanism of hemophilia patient's bone lose may be related with the decrease of osteogenic activity, the IGF can prevent bone lose in hemophilia, the bFGF and OPG can promote bone metabolism of the patients with hemophilia.

Alkaline Phosphatase ; metabolism ; Biomarkers ; Bone Density ; Bone Diseases, Metabolic ; pathology ; Bone and Bones ; pathology ; Collagen Type I ; metabolism ; Fibroblast Growth Factor 2 ; metabolism ; Hemophilia A ; pathology ; Humans ; Osteogenesis ; Osteoprotegerin ; metabolism ; Peptides ; metabolism ; Somatomedins ; metabolism ; Transforming Growth Factor beta1 ; metabolism

OBJECTIVETo explore the expression of CC-chemokine Receptor 7 (CCR7) in adult acute leukemia patients, and to analyze the relationship of CCR7 expression with the clinical characteristics of patients.

METHODSThe expression of CCR7 in bone marrow samples from adult acute leukemia patients were detected by flow cytometry (FCM), the relationship of CCR7 expression with the clinical characteristics of patients such as sex, age, WBC count, blast cell ratio, CD56 expression, molecular biology, cell genetics, risk stratification, extramedullary infiltration was analyzed.

RESULTSThe expression rate of CCR7 in adult ALL and AML patients was 36.8% and 9.6%, respectively, and the expression level of CCR7 in ALL patients was higher than that in AML patients (P < 0.05). The extramedullary infiltration rate was 100% and 41.7 % for CCR7 positive and negative groups of ALL, respectively (P < 0.05). While the mean fluorescence intensity (MFI) in extramedullary infiltration group of ALL was higher than that in none-extramedullary infiltration group of ALL (50.00 ± 10.42 vs 18.14 ± 1.39), respectively (P < 0.05).

CONCLUSIONCCR7 is higher expressed in adult acute leukemia cells, moreover its expression rate in ALL is higher than that in AML, and the expression of CCR7 is related with extramedullary infiltration in ALL.

Adult ; Bone Marrow ; metabolism ; Flow Cytometry ; Humans ; Leukemia, Myeloid, Acute ; genetics ; metabolism ; Leukocyte Count ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; genetics ; metabolism ; Receptors, CCR7 ; genetics ; metabolism

OBJECTIVE: To construct a mouse model of Glanzmann's thrombasthenia (GT) with ITGA2B c.2659 C>T (p.Q887X) nonsense mutation by CRISPR/Cas9 technology, and then further explore the expression and function of glycoprotein αIIbβ3 on the surface of platelet membrane. METHODS: The donor oligonucleotide and gRNA vector were designed and synthesized according to the ITGA2B gene sequence. The gRNA and Cas9 mRNA were injected into fertilized eggs with donor oligonucleotide and then sent back to the oviduct of surrogate mouse. Positive F0 mice were confirmed by PCR genotyping and sequence analysis after birth. The F1 generation of heterozygous GT mice were obtained by PCR and sequencing from F0 bred with WT mice, and then homozygous GT mice and WT mice were obtained by mating with each other. The phenotype of the model was then further verified by detecting tail hemorrhage time, saphenous vein bleeding time, platelet aggregation, expression and function of αIIbβ3 on the surface of platelet. RESULTS: The bleeding time of GT mice was significantly longer than that of WT mice (P<0.01). Induced by collagen, thrombin, and adenosine diphosphate (ADP), platelet aggregation in GT mice was significantly inhibited (P<0.01, P<0.01, P<0.05). Flow cytometry analysis showed that the expression of αIIbβ3 on the platelet surface of GT mice decreased significantly compared with WT mice (P<0.01), and binding amounts of activated platelets to fibrinogen were significantly reduced after thrombin stimulation (P<0.01). The spreading area of platelet on fibrinogen in GT mice was significantly smaller than that in WT mice (P<0.05). CONCLUSION A GT mouse model with ITGA2B c.2659 C>T (p.Q887X) nonsense mutation has been established successfully by CRISPR/Cas9 technology. The aggregation function of platelet in this model is defective, which is consistent with GT performance.
Animals ; CRISPR-Cas Systems ; Codon, Nonsense ; Disease Models, Animal ; Fibrinogen/genetics* ; Humans ; Integrin alpha2/genetics* ; Mice ; Oligonucleotides ; Platelet Glycoprotein GPIIb-IIIa Complex/genetics* ; RNA, Guide ; Thrombasthenia/genetics* ; Thrombin/genetics*