Clinical and gene mutation features of cystic fibrosis: an analysis of 8 cases / 中国当代儿科杂志

OBJECTIVES@#To study the clinical features and gene mutation sites of children with cystic fibrosis (CF), in order to improve the understanding of CF to reduce misdiagnosis and missed diagnosis.@*METHODS@#A retrospective analysis was performed on the medical records of 8 children with CF who were diagnosed in Hebei Children's Hospital from 2018 to 2021.@*RESULTS@#Among the 8 children with CF, there were 5 boys and 3 girls, with an age of 3-48 months (median 8 months) at diagnosis, and the age of onset ranged from 0 to 24 months (median 2.5 months). Clinical manifestations included recurrent respiratory infection in 7 children, sinusitis in 3 children, bronchiectasis in 4 children, diarrhea in 8 children, fatty diarrhea in 3 children, suspected pancreatic insufficiency in 6 children, pancreatic cystic fibrosis in 1 child, malnutrition in 5 children, and pseudo-Bartter syndrome in 4 children. The most common respiratory pathogens were Pseudomonas aeruginosa (4 children). A total of 16 mutation sites were identified by high-throughput sequencing, multiplex ligation-dependent probe amplification, and Sanger sequencing, including 5 frameshift mutations, 4 nonsense mutations, 4 missense mutations, 2 exon deletions, and 1 splice mutation. CFTR mutations were found in all 8 children. p.G970D was the most common mutation (3 children), and F508del mutation was observed in one child. Four novel mutations were noted: deletion exon15, c.3796_3797dupGA(p.I1267Kfs*12), c.2328dupA(p.V777Sfs*2), and c.2950G>A(p.D984N).@*CONCLUSIONS@#p.G970D is the most common mutation type in children with CF. CF should be considered for children who have recurrent respiratory infection or test positive for Pseudomonas aeruginosa, with or without digestive manifestations or pseudo-Bartter syndrome.

Epidemiological features of severe acute respiratory syndrome coronavirus 2 infection in children in Shijiazhuang, China: an analysis of 133 cases / 中国当代儿科杂志

OBJECTIVE@#To study the epidemiological features of children with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in Shijiazhuang, China.@*METHODS@#Based on the information officially announced on the official website of the Health Commission of Hebei Province, epidemiological data were collected from 133 children, aged 0-18 years, who were diagnosed with SARS-CoV-2 infection in Shijiazhuang from January 2 to January 30, 2021. A statistical analysis was performed for general status, regional distribution, presence or absence of clusters, and results of SARS-CoV-2 nucleic acid tests.@*RESULTS@#Among the 133 children with SARS-CoV-2 infection, there were 65 boys and 68 girls, with a male/female ratio of 0.96:1. The youngest age of onset was 3 months and 7 days, and the mean age of onset was (9±5) years. Of all the 133 children, 90(67.7%) were the first confirmed case of SARS-CoV-2 infection among their family members. Of all the children, 108(81.2%) came from the Gaocheng District in Shijiazhuang, among whom 38(28.6%) were from Xiaoguozhuang Village where the first patient with a confirmed diagnosis lived. SARS-CoV-2 nucleic acid test at week 2 after the outbreak showed positive results in 88 children (66.2%), and only 5 children had clinical symptoms before positive SARS-CoV-2 results were obtained. Of all the 133 children, 19(14.3%) were found positive in the first SARS-CoV-2 nucleic acid test after the outbreak, and 70(52.6%) had positive results for ≥4 times. There were 98 school students with infection, among whom 74(75.5%) were the first confirmed case in their family, and among 35 non-school students, 16(45.7%) were the first confirmed case in their family (@*CONCLUSIONS@#Among the children confirmed with SARS-CoV-2 infection in Shijiazhuang, there is a high proportion of children who are the first confirmed case in their family, and the children are mainly distributed in the rural areas of Gaocheng. Most of these children are students, so the prevention and control of cluster infection in schools should be taken seriously. There are often no symptoms before SARS-CoV-2 nucleic acid test, with a low positive rate of the first nucleic acid test, which increases the difficulty of early discovery of the epidemic.

Efficacy of early treatment via fiber bronchoscope in children with Mycoplasma pneumoniae pneumonia complicated by airway mucus obstruction / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the efficacy of early treatment via fiber bronchoscope in children with Mycoplasma pneumoniae pneumonia (MPP) complicated by airway mucus obstruction.</p><p><b>METHODS</b>According to the time from admission to the treatment via fiber bronchoscope, the children with MPP who were found to have airway mucus obstruction under a fiber bronchoscope were randomly divided into early intervention group (≤3 days; n=40) and late intervention group (>3 days; n=56). The two groups were compared in terms of clinical data and imaging recovery.The children were followed for 1-3 months.</p><p><b>RESULTS</b>Of the 96 children, 38 were found to have the formation of plastic bronchial tree, among whom 10 were in the early intervention group and 28 were in the late intervention group (P=0.01). Compared with the late intervention group, the early intervention group had a shorter duration of fever, length of hospital stay, and time to the recovery of white blood cell count and C-reactive protein (P<0.05), as well as a higher atelectasis resolution rate (P<0.05). Compared with the late intervention group, the early intervention group had a higher percentage of children with a ≥ 60% absorbed area of pulmonary consolidation at discharge. After 3 months of follow-up, the early intervention group had a higher percentage of children with a ≥ 90% absorbed area of pulmonary consolidation than the late intervention group (80% vs 55%; P=0.01), and the early intervention group had a lower incidence rate of atelectasis than the late intervention group (P<0.05).</p><p><b>CONCLUSIONS</b>Early treatment via fiber bronchoscope can shorten the course of the disease and reduce complications and sequelae in MPP children with airway mucus obstruction.</p>

Risk factors for infection with extended-spectrum beta-lactamase-producing strains in children / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the risk factors for infection with extended-spectrum beta-lactamase (ESBL) producing strains in children.</p><p><b>METHODS</b>The clinical data of 242 pediatric in-patients with lower respiratory tract infections from February 2009 to January 2011 were retrospectively analyzed. The risk factors of ESBL-producing strain infections were investigated using univariate analysis and multivariate logistic regression analysis.</p><p><b>RESULTS</b>Univariate analysis showed that six factors were related with ESBL-producing strain infections: repeated sucking of phlegm (OR: 2.279, P<0.01), tracheal intubation(OR: 3.101, P<0.01), administration of the third generation cephalosporin for more than three days (OR: 3.628, P<0.01), admission to the pediatric intensive care unit (PICU) (OR: 2.378, P<0.01), indwelling of nasogastric tube (OR: 2.460, P<0.01), prophylactic use of antibiotics (OR: 1.747, P<0.05). Multivariate logistic regression showed that the application of the third-generation cephalosporin for more than three days (OR: 5.672, P<0.01), repeated sucking of phlegm (OR: 3.917, P<0.01), tracheal intubation (OR: 3.717, P<0.01), indwelling of nasogastric tube (OR: 2.961, P<0.01), and admission to PICU (OR: 3.237, P<0.01) were the independent risk factors for the infections.</p><p><b>CONCLUSIONS</b>The infections of ESBL-producing strains are caused by many factors, among which the application of the third-generation cephalosporin for more than three days, invasive operations, and admission to PICU are the independent risk factors.</p>

Interforen Role of Ligustrazine to Collagen-Ⅲ and Laminin in Pulmonary Fibrosis Rats / 实用儿科临床杂志

Objective To study the role of ligustrazine injection on collagen-Ⅲ(Col-Ⅲ) and laminin(LN) in pulmonary fibrosis rats induced by bleomycin A5.Methods Seventy Wistar rats were divided into 5 groups at random:normal group,model group,large-dose group,middle-dose group,little-dose group.The other groups were injected bleomycin A5 in intratrache except for normal group.From the second day,large-dose group,middle-dose group,little-dose group were given ligustrazine injection by intraperitoneal injection to 28th day,the dose was dividedly:250,150,40 mg/(kg?d).Col-Ⅲ and LN in serum were detected in 14th,28th days and the content of them in lung tissue homogenate in 28th day were also detected.HE staining was detected on lung tissue.Results The contents of Col-Ⅲ and LN of lung tissue and serum in pulmonary fibrosis rats were significantly upgraded compared with that of normal group(P