Abstract:

Although thrombotic thrombocytopenic purpura (TTP) is rarely seen in pediatric patients, failure to recognize this condition often leads to severe consequences and poor outcomes. Classic features of TTP include thrombocytopenia, microangiopathic hemolytic anemia, acute kidney injury, fever, and central nervous system involvement. However, patients suffering from this condition may not present with all of the symptoms simultaneously. Therefore, it is of utmost importance for healthcare providers to have a high index of suspicion. Laboratory investigations may reveal the presence of schistocytes on peripheral blood smear, negative Coombs test, high lactate dehydrogenase levels and severely low platelet counts. The etiology of TTP is mainly due to insufficient cleavage of the large multimers of von Willebrand factor (vWF) secondary to decreased activity of ADAMTS13 (a disintegrin and metalloprotease with Thrombospondin type 1 repeats, member 13). TTP can be broadly classified into familial TTP (Upshaw Schulman syndrome) and non-familial TTP. Familial TTP is due to a congenital deficiency of ADAMTS13. Its mainstay of therapy is initiation of plasmapheresis during the acute phase, followed by regular fresh frozen plasma (FFP) infusions. Alternatively, non-familial TTP is due to a decrease in ADAMTS13 activity secondary to the presence of anti-ADAMTS13 antibodies. Once again, the primary treatment is plasmapheresis; however, recent anecdotal data also supports the use of rituximab in select cases.

Abstract:OBJECTIVE: To analyze the frequency distribution of single nucleotide polymorphisms (SNPs) of four asthma-related gene loci (ACE I/D; ADRB2 Arg16Gly; TNF-α G-308A; MS4A2 Glu237Gly) in 198 asthmatic children, and to investigate its association with genetic susceptibility to childhood asthma and some clinical phenotypes of asthma. METHODS: Polymerase chain reaction product electrophoresis identification and real-time quantitative PCR detecting system were used to determine the frequency distributions of the SNPs of the four asthma-related gene loci in 198 asthmatic children and 110 healthy controls. The serum total IgE (TIgE) levels and blood eosinophil proportion (%EOS) of the asthmatic children were measured. Different genotypes at the four asthma-related gene loci were compared in terms of TIgE and %EOS. RESULTS: The genotype DD of angiotensin-converting enzyme (ACE) had a significantly higher frequency in the asthmatic children than in the healthy controls (χ2= 30.667, P0.05). CONCLUSIONS: Genotype DD of ACE is related to genetic susceptibility to childhood asthma and may be the risk factor for childhood asthma.

Abstract:OBJECTIVE: To investigate changes in the fraction of exhaled nitric oxide (FeNO) in childhood asthma, and to evaluate the clinical value of continuous monitoring of FeNO. METHODS: Twenty children with mild to moderate asthma were enrolled from the special outpatient clinic for asthma. Follow-up was performed at 8, 16, 24, 32 and 40 weeks after treatment. At each follow-up, asthmatic symptoms were recorded, pulmonary function was evaluated and FeNO was measured. RESULTS: The mean FeNO decreased rapidly 8 weeks after treatment and slowly afterwards. It decreased significantly 8,16,24 and 40 weeks after treatment (P<0.01). It increased significantly during acute attacks of asthma and decreased rapidly during periods of remission. There was significant negative correlation between FeNO and forced expiratory volume in one second (r =-0.193，P<0.05). The receiver operating characteristic curve of FeNO showed that FeNO had a sensitivity of 87.9% and a specificity of 80% for uncontrolled asthma when FeNO was 35.5 ppb, and that it had a sensitivity of 97% and a specificity of 27.1% when FeNO was 20.5 ppb. CONCLUSIONS: monitoring of FeNO can be used to evaluate the control level of airway inflammation in childhood asthma. When FeNO is less than 20.5 ppb, airway inflammation may be well controlled. When FeNO is more than 35.5 ppb, airway inflammation may be out of control. A sharp increase in FeNO suggests the possibility of acute asthma attack in children.

Abstract:OBJECTIVE: To study the clinical value of the expression of neutrophil surface CD64 in the diagnosis of community acquired pneumonia in children. METHODS: Ninety-eight children with community acquired pneumonia were recruited into the study and were classified into three groups according to pathogene: bacterial pneumonia (n=48), viral pneumonia (n=29) and Mycoplasmal pneumonia (n=21). Twenty healthy children were enrolled as controls. The bacterial infection group was subdivided into mild infection (n=36) and severe infection groups (n=12). The levels of peripheral blood neutrophil CD64 were measured using flow cytometry. Dynamic changes of C-reactive protein were also detected for each patient. RESULTS: The CD64 index and CRP levels in the bacterial pneumonia group were significantly higher than in the other three groups (P<0.05). The CD64 index in the severe bacterial infection group was significantly higher than in the mild group (P<0.05). After antibiotic treatment, expression of CD64 in the severe bacterial infection group decreased significantly (P<0.05). The CD64 index was positively correlated with CRP value (r=0.545, P<0.01). ROC curve analysis showed that the threshold of CD64 and CRP was 2.8 and 8 mg/L respectively. Specificity of CD64 index (90%) was much higher than CRP (74%). CONCLUSIONS: The determination of peripheral blood neutrophil CD64 contributes to the early diagnosis of pulmonary bacterial infection and the evaluation of anti-infection effect.

Abstract:OBJECTIVE: To study the clinical characteristics of Mycoplasma pneumoniae (MP)-associated ischemic stroke in children. METHODS: The case of a girl with MP-associated ischemic stroke was reported, including clinical manifestations and laboratory and imaging examinations, and related literature was reviewed. RESULTS: The girl, who was suffering from a respiratory tract infection was found to have hemiplegia and aphasia which were expressed in ischemic stroke. IgM antibody to MP in serum (1∶320) and lavage fluid was positive. Pulmonary imaging showed unilateral consolidation and pleural exudate. Cerebral neuroimaging examination showed occlusion of the bilateral middle cerebral artery, mainly on the left side. The neurological symptoms and signs were recovered after comprehensive therapy with medication (azithromycin, hormone and heparin) and rehabilitation training. CONCLUSIONS: Ischemic stroke is rare but severe manifestation of central nervous system damage in children suffering from MP infection. Cerebral imaging and etiological examinations contribute to the diagnosis. Early use of macrolide antibiotics, anticoagulant and hormone may improve the prognosis.

Abstract:OBJECTIVE: To study the clinical features and antimicrobial resistance of community-acquired pneumonia caused by Klebsiella pneumoniae in infants. METHODS: The clinical data of 65 infants with community-acquired pneumonia caused by Klebsiella pneumoniae between 2007 and 2011 were retrospectively studied. RESULTS: Of the 65 infants, 37 cases (57%) were aged ≤3 months, 17 cases (26%) over 4 months, 7 cases (11%) over 7 months and 4 cases (6%) between 13 and 24 months. There were no significant differences in clinical manifestations and chest X-ray features between the infants with community-acquired pneumonia caused by Klebsiella pneumoniae and those with other bacterial pneumonia. Forty strains (62%) of ESBLs-producing Klebsiella pneumoniae were detected. Klebsiella pneumoniae was 100% sensitive to imipenem, meropenem and amikacin but resistant to penicillins and cephalosporins. The resistance rates of ESBLs-producing strains to penicillins, cephalosporins, amoxicillin/clavulanic acid, ampicillin/sulbactam, compound sulfamethoxazole, gentamycin, ciprofloxacin and aztreonam were significantly higher than for non-ESBLs-producing strains. ESBLs-producing strains also showed multiple-drug resistance. CONCLUSIONS: Community-acquired pneumonia caused by Klebsiella pneumoniae is common in infants aged ≤3 months. ESBLs-producing strains are prevalent in community-acquired pneumonia caused by Klebsiella pneumoniae and demonstrate both high rates of drug resistance and multiple-drug resistance.

Abstract:OBJECTIVE: To investigate the epidemiological features of influenza virus B (IVB) in the winter and the clinical features of pediatric pneumonia caused by IVB only. METHODS: A retrospective study was performed on the clinical data of children with respiratory infection who received pathogen testing and therapy at Soochow University Affiliated Children′s Hospital during the winters of 2008, 2009, 2010 and 2011. RESULTS: The positive rates of influenza viruses A and B in the winters of 2008, 2009, and 2010 were 0.89%, 5.49%, and 6.24% respectively; the positive rate of influenza viruses A and B in the winter of 2011 was 8.72%, significantly higher than those in 2008-2010. The positive rates of IVB in the winters of 2008, 2009, and 2010 were 0%, 0%, and 0.21% respectively; the positive rate of IVB in the winter of 2011 was 5.36%, which was significantly higher than in the years 2008 to 2010. Pneumonia caused by IVB was confirmed in 94 children during the winter of 2011, including 27 cases of pneumonia caused by IVB only. Most of children with pneumonia caused by IVB only were aged over 6 months. The common symptoms in the 27 children caused by IVB only were fever (85%), runny nose (89%), and cough (100%). Wheezing (26%) and dyspnea (7%) were also seen in some cases. Among the 27 children, 19% showed abnormal white blood cell count, 30% showed increased C-reactive protein, 70% showed decreased prealbumin, and none showed visible organ dysfunction. No specific imaging findings were seen in the children with pneumonia caused by IVB only. Howerer, many abnormal humoral and cellular immunological parameters were found in the majority of these children. The average length of hospital stay was approximately one week, there were no critical patients and the prognosis was good. CONCLUSIONS: Influenza viruses were at a peak level in inpatient children in the winter of 2011. IVB infection rate was gradually increasing. In children with pneumonia caused by IVB only, there are few critical patients, the symptoms are nonspecific and the prognosis is good.

Abstract:OBJECTIVE: To investigate the association of non-bacterial respiratory pathogens with asthmatic diseases in children, and the clinical significance of total serum IgE levels and peripheral eosinophil count in infection with non-bacterial respiratory pathogens. METHODS: Indirect immunofluorescence assay was used to detect IgM antibodies against nine types of non-bacterial respiratory pathogens in the sera of 490 children with asthmatic diseases between September 2010 and September 2011. Pathogens were analyzed and total serum IgE levels and peripheral eosinophil count were measured in IgM-positive cases. RESULTS: Of the 490 children with asthmatic diseases, 47.6% (233 cases) were positive with IgM antibodies against non-bacterial respiratory pathogens, the most common being Mycoplasma pneumoniae (MP) (25.3%), followed by adenovirus (ADV) (8.9%) and influenza B virus (Flu B) (8.8%). Thirty-six cases suffered from co-infection of two or more non-bacterial pathogens, mainly comprising MP and other pathogens (94%). There were significant differences in the total detection rate of IgM antibodies among all age groups (0-30 days: 50.0%; 1-6 months: 67.3%; 0.5-1 year: 33.1%; 1-3 years: 57.3%; 3-8.9 years: 61.7%). The positive rate of IgM antibodies against respiratory pathogens was highest in children with bronchial asthma, followed by children with asthmatic bronchitis, and it was lowest in children with bronchiolitis. IgM-positive children had significantly decreased blood eosinophils and significantly increased total serum IgE levels. CONCLUSIONS: The main non-bacterial respiratory pathogens include MP, ADV and Flu B in children with asthmatic diseases, and co-infection of MP and other non-bacterial pathogens is common. Infants aged 1 to 6 months have a higher infection rate than other age groups. Monitoring the changes in total serum IgE levels and peripheral eosinophil count has great significance for the clinical diagnosis and treatment of asthmatic diseases in children.

Abstract:OBJECTIVE: To study risk factors for periventricular-intraventricular hemorrhage (PVH-IVH) in premature infants treated with mechanical ventilation. METHODS: A total of 205 premature infants who were admitted to the neonatal intensive care unit (NICU) and treated with mechanical ventilation between January 2009 and December 2011 were enrolled. They were classified into PVH-IVH and non-PVH-IVH groups according to the results of head ultrasonography performed at 3 to 7 days after birth. Single factor and multivariate logistic regression analysis were used to identify risk factors for PVH-IVH. RESULTS: Single factor analysis indicated 9 factors associated with the development of PVH-IVH, including a gestational age of<32 weeks, a birth weight of<1500 g, intrauterine distress, severe asphyxia, vaginal delivery, maternal perinatal infection, premature rupture of membranes (PROM) at ≥8 hours, mechanical ventilation duration of ≥7 days and ventilator-associated pneumonia (VAP) (P<0.05). Multivariate logistic regression analysis showed that a birth weight of<1500 g (OR=2.665), intrauterine distress (OR=2.177), severe asphyxia (OR=5.653), maternal perinatal infection (OR=4.365) and VAP (OR=2.299) were independent risk factors for the development of PVH-IVH (P<0.05). CONCLUSIONS: Very low birth weight, intrauterine distress, severe asphyxia, maternal perinatal infection and VAP are closely associated with an increased risk of PVH-IVH in premature infants treated with mechanical ventilation. These clinical risk factors should be given more attention in the prevention of PVH-IVH.

Abstract:OBJECTIVE: To observe catch-up growth in height within two years of birth in infants of different sexes, gestational ages, and birth weights with intrauterine growth retardation (IUGR). METHODS: Follow-up was performed on 294 IUGR infants and 300 healthy full-term infants at 4, 6, 9, 12, 15, 18, 21 and 24 months after birth to measure the height, calculate the height increase and compare the two groups with respect to height increase. RESULTS: The success rates of catch-up growth in height were 72.2% in male infants and 71.5% in female infants (P=0.90), and were 77.4% in preterm small-for-gestational age (SGA) infants and 68.6% in full-term SGA infants (P=0.11). Success rates of catch-up growth in height in infants with birth weights between 1500-2499 g was higher than in those with birth weights of <1500 g and ≥2500 g (P<0.01). The male infants showed significant catch-up growth at 4, 6, 18, 21 and 24 months after birth, while significant catch-up growth was found in female infants at 4, 6, 9, 12 and 21 months after birth. Of the male infants, preterm SGA infants showed significantly greater height increase than the full-term SGA infants at 6 and 9 months after birth. Of the female infants, preterm SGA infants showed significantly greater height increase than the full-term SGA infants at 4 and 18 months after birth. For both male and female infants, height increase at 4 months after birth was significantly greater in those with birth weights of<1500 g than in those with birth weights of ≥2500 g. For male infants, height increases at 4, 6, 18, 21 and 24 months after birth were significantly greater in those with birth weights of 1500-2499 g than in those with birth weights of ≥2500 g. For female infants, height increases at 4, 6, 9, 12 and 21 months after birth were significantly greater in those with birth weights of 1500-2499 g than in those with birth weights of ≥2500 g. CONCLUSIONS: The catch-up growth in height within two years of birth in infants with IUGR occurs mainly in the first year after birth in female infants, but can be seen in the first six months and the second year after birth in male infants. Preterm SGA infants better catch-up growth than full-term SGA infants, and infants with birth weights of below 1500 g and between 1500-2499 g show better catch-up growth than those with birth weights of ≥2500 g.

Abstract:OBJECTIVE: To study the clinical features and treatment outcomes of head and neck rhabdomysarcoma (RMS) in children. METHODS: The clinical data and results of follow-up visits for 39 children with head and neck RMS were retrospectively reviewed. The children (23 males and 16 females) with a median age of 6 years old (ranged 3 months to 14 years) were admitted to the Beijing Tongren Hospital between November, 2004 and November, 2010. RESULTS: The 39 children mainly presented with exophthalmos and eyelid swelling (56%, 22/39), rhinostegnosis and nasal bleeding (28%, 11/39) and check mass (15%, 6/39). Common primary sites were the eyelid and orbit (56%, 22/39), followed by the nasopharynx and ethmoid antrum (28%, 11/39). Thirty-seven of the 39 patients showed a definite pathologic type and the embryo type was the most common (89%, 33/37). Follow-up visits were carried out for 35 children, with a median follow-up time of 38 months (10-80 months). Of the 35 children, 4 cases received surgery alone, 1 case received chemotherapy alone, 12 cases received surgery plus chemotherapy, 2 cases received surgery plus radiochemotherapy, 13 cases received surgery, chemotherapy and radiochemotherapy (8 cases received 125I particles implants), 2 cases received surgery, chemotherapy, radiochemotherapy and autologous peripheral blood stem cells transplantation (APBSCT), and 1 case received chemotherapy and APBSCT. Seven cases relapsed and 5 cases died of brain metastasis. The total survival rate was 86% (30/35), the complete remission rate was 66% (23/35), and the partial remission rate was 20%. In the 8 cases receiving 125I particles implants, 6 survived without tumor. CONCLUSIONS: Exophthalmos and eyelid swelling are the main presentations in children with head and neck RMS. Common primary sites of this disease are the eye and nasopharynx. The most common pathologic type is embryo type. Comprehensive treatment, including chemotherapy, surgery, 125I particles implants and APBSCT therapy, can improve outcome.

Abstract:OBJECTIVE: To study blood concentrations of methotrexate (MTX) in Uyghur and Han children with acute lymphoblastic leukemia (ALL), and to provide criteria for judging the incidence of adverse effects of MTX. METHODS: Twenty-eight children with ALL (15 Han children and 13 Uyghur children), who received high-dose MTX chemotherapy, were divided into >10 μmol/L and ≤10 μmol/L groups according to 24-hour blood concentration of MTX, and divided into >1.0 μmol/L and ≤1.0 μmol/L groups according to 48-hour blood concentration of MTX. Enzyme multiplied immunoassay was used to measure blood concentrations of MTX in the MTX-treated children at 24 and 48 hours after MTX administration, and the adverse effects were observed. RESULTS: There was no significant difference in the incidence of adverse effects between the >10 μmol/L and ≤10 μmol/L groups (P>0.05). The >1.0 μmol/L group showed higher incidences of gastrointestinal reactions and mucosal injuries than the ≤1.0 μmol/L group (P0.05). Compared with Uyghur children, Han children showed higher 24- and 48-hour blood concentrations of MTX (P<0.05) and higher incidence of abnormal liver function, mucosal injuries, and bone marrow suppression (P<0.05). CONCLUSIONS: The 24-hour blood concentration of MTX cannot be used to predict the incidence of adverse effects in MTX chemotherapy, but 48-hour blood concentration of MTX is helpful in this regard. There are significant differences in 24- and 48-hour blood concentrations of MTX and the incidence of adverse effects between Uyghur and the Han children with ALL who receive MTX chemotherapy. Monitoring of blood MTX concentration maybe significant for timely adjustment of MTX dosage and individualized MTX chemotherapy.

Abstract:OBJECTIVE: To investigate the mutation of glucose-6-phosphatase gene (G6PC gene) in a patient with glycogen storage disease Ⅰa. METHODS: PCR was used to amplify all five exons of G6PC gene. The PCR products were directly sequenced to detect the mutations. RESULTS: A heterozygous 743G>A mutation was found in the patient and his mother, resulting in the substitution of glycine (G) by arginine (R) in codon 222(G222R) in the putative membrane-spanning domain in human G6Pase, but not in his father and his sister. CONCLUSIONS: G222R mutation in G6PC gene was first identified in a patient with glycogen storage disease Ⅰa in mainland China.

Abstract:OBJECTIVE: To study the significance and safety of flexible bronchoscopy and balloon dilatation in the diagnosis and treatment of respiratory diseases in children. METHODS: A total of 438 children (236 males and 202 females) with respiratory diseases who were aged from 17 days to 15 years, were examined and/or treated by bronchoscopy (including bronchoscopic intervention) under local anesthesia. RESULTS: Of the 438 children, 311 were diagnosed with pulmonary infection, 68 with atelectasis, 36 with recurrent cough and asthma, 6 with hemoptysis of unknown origin, 6 with bronchial foreign body, 5 with congenital bronchopulmonary dysplasia, 2 with bronchiectasis, 1 with ciliary dyskinesia syndrome, 1 with lung tumor, and 2 with congenital immunodeficiency disease. After bronchoscopic examination, local flushing or bronchoalveolar lavage, and foreign body extraction, marked response was seen in 379 cases and response was seen in 46 cases. High-pressure balloon dilatation under bronchoscopy was performed in 5 cases with inflammatory stricture and achieved satisfying clinical effect. No severe complications were found in bronchoscopy. CONCLUSIONS: Bronchoscopy and balloon dilatation under local anesthesia is safe and effective for the diagnosis and treatment of respiratory diseases in children.

Abstract:OBJECTIVE: To investigate the effect of vitamin D on the expression of chemokine regulated on activation, normal T cells expressed and secreted (RANTES) in the lung tissue of asthmatic rats, and the role of vitamin D in the control of asthmatic airway inflammation and the synergistic action of hormones. METHODS: Forty female Wistar rats were randomly and equally divided into normal control, asthma, vitamin D intervention, budesonide intervention, and budesonide+vitamin D intervention groups. Hematoxylin and eosin staining was used to observe pathological changes in the lung tissue. Immunohistochemistry was used to measure the protein expression of RANTES in lung tissue. Enzyme-linked immunosorbent assay was used to measure the level of RANTES in bronchoalveolar lavage fluid (BALF). Real-time quantitative PCR was used to measure the mRNA expression of RANTES. RESULTS: The asthma group showed the most significant pathological changes in the lung tissue, including inflammatory cell infiltration, bronchial stenosis and distortion and smooth muscle rupture, while the intervention groups showed fewer pathological changes. Of the intervention groups, the budesonide intervention group showed fewer pathological changes than the vitamin D intervention group, and the budesonide+vitamin D intervention group showed the mildest pathological changes, which were similar to those observed in the normal control group. Protein expression of RANTES in the lung tissue and BALF was significantly higher in the asthma group than in the normal control group (P<0.05), while it was lower in the intervention groups than in the asthma group, exhibiting significant differences between each intervention group and the asthma group (P<0.05) (except the difference in protein expression of RANTES in BALF between the vitamin D intervention and asthma groups). The budesonide+vitamin D intervention group showed less protein expression of RANTES in the lung tissue and BALF than both the budesonide intervention and vitamin D intervention groups (P<0.05). The mRNA expression of RANTES was significantly higher in the asthma group than in the normal control group (P<0.05), while it was significantly lower in three intervention groups than in the asthma group (P<0.05), however no significant difference was found between the intervention groups in this regard. The budesonide+vitamin D intervention group showed the lowest level of RANTES mRNA, with no significant difference from the normal control group. CONCLUSIONS: The mRNA and protein expression of RANTES in BALF and lung tissue increases significantly in asthmatic rats. Vitamin D intervention can decrease the expression of RANTES, suggesting that vitamin D can reduce airway inflammation by regulating the expression of RANTES. Vitamin D can be used together with budesonide to further decrease the mRNA and protein expression of RANTES.

Abstract:OBJECTIVE: To study changes in the expression levels of parvalbumin (PV), glutamate decarboxylase 67 (GAD67) and K+-Cl- cotransporter 2 (KCC2) in the brain tissue of rats with schizophrenia (SZ) induced by dizocilpine (MK-801), and to investigate the mechanism involving gamma-aminobutyric acid (GABA) by which NMDA receptor blocker induces SZ in the perinatal period. METHODS: Thirty-six neonatal male Sprague-Dawley rats were randomly assigned to two batches on postnatal day 6. Each batch was divided into normal control (treated by 0.9% normal saline), SZ-development model (treated by subcutaneous injection of 0.1 mg/kg MK-801 on postnatal days 7-10; bid), and SZ-chronic medication model groups (treated by intraperitoneal injection of 0.2 mg/kg MK-801 on postnatal days 47-60; qd). On postnatal day 63, the brain tissue of the first batch of rats was obtained and then fixed with paraform for histological sections; expression levels of PV and GAD67 in the medial prefrontal cortex (mPFC) and hippocampus CA1 were measured by immunohistochemistry. Simultaneously, the second batch of rats was sacrificed and the mPFC and hippocampus were obtained and homogenized; expression levels of KCC2 in the mPFC and hippocampus were measured by Western blot. RESULTS: Expression levels of PV and GAD67 in the mPFC and hippocampus CA1 were significantly lower in the SZ-development and chronic medication model groups than in the normal control group (P<0.05). Expression levels of KCC2 in the mPFC and hippocampus were significantly lower in the SZ-development model group than in the SZ-chronic medication model and normal control groups (P<0.05).ConclusionsThe expression changes of PV and GAD67 in SZ can be simulated using the SZ development model induced by MK-801, which might affect the development of the GABA system in the PFC and hippocampus by downregulating KCC2 expression.

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