Background: There has been no report on tracheotomy and home mechanical ventilation therapy for infants with severe bronchopulmonary dysplasia(BPD) in China.
Objective: To describe the experience of two cases of severe BPD treated with tracheotomy and home mechanical ventilation therapeutic.
Design: Case report.
Methods: Clinical characteristics,multidisciplinary evaluation before tracheotomy,and transitional and postdischarge care of two infants with very severe BPD and home mechanical ventilation were reported.
Results: Two premature infants were admitted for prolonged dependency of invasive mechanical ventilation (case 1: gestational age of 27+5weeks,7 months and 19 days after birth; case 2:gestational age of 24+6weeks,6 months and 24 days after birth).Evaluations on airway,lung parenchyma,and pulmonary vascular disease were performed,showing severe tracheobronchomalacia and severe heterogeneous lung parenchymal lesions.After multidisciplinary consultation and discussion,tracheotomy and long-term home mechanical ventilation support was indicated as the most suitable management strategy.The indication and risk of tracheotomy and long-term mechanical ventilation were discussed thoroughly with the parents.Tracheotomy was then performed.Both infants were discharged on home ventilators after careful assessment of coordination of postdsicharge care,readiness for home care,training of caregivers,and necessary equipment.
Conclusion: Tracheotomy and long-term home mechanical ventilation treatment provided a possible treatment choice for ventilator-dependent severe BPD infants in China,but such treatment required significant efforts from the multidisciplinary medical team and family, and difficulties still remained such as cost, high risk,and postdischarge care.
Background: There are few studies on the efficacy and safety of perampanel in children with drug-resistant epilepsy, and the efficacy and safety are not clear.
Objective: To investigate the efficacy and safety of perampanel in the treatment of children with drug-resistant epilepsy.
Design: Cohort study.
Methods: Children aged 0-18 years who were diagnosed with drug-resistant epilepsy and treated with perampanel in Peking University First Hospital from January to December 2020 were included in the cohort study, and the end point was at least 6 months' treatment. Efficacy was the dependent variable, and the gender, etiology, epileptic syndrome, baseline frequency of seizures, age at onset, age at time of dosing, course of disease at dosing, dosage of drugs, number of treatments were independent variables. The univariate and multivariate logistic regression analysis were used to analyze the related factors of efficacy. The adverse reactions of perampanel in the treatment of children with drug-resistant epilepsy were also analyzed.
Main outcome measures: The frequency of seizures decreased ≥50% from baseline at the last follow-up (within 1 month) .
Results: A total of 50 patients were included (34 males and 16 females)，and all the patients were followed up at least 6 months. The epileptic syndromes included infantile spasms in 6 cases, Lennox-Gastaut syndrome in 2 cases, epileptic encephalopathy with electrical status epilepticus during sleep in 3 cases, and Rasmussen encephalitis in 2 cases. Specific causes of epilepsy were found in 66.0% (33/50) of the patients, including structural causes in 19 cases, genetic causes in 9 cases, immunological causes in 4 cases, and metabolic causes in 1 case. The baseline frequency of seizures ranged form once per 2 weeks to hundreds of times per day. Eighteen percent (9/50) of the patients had seizure less than once a day, 36.0% (18/50) of the patients with seizures of 1-10 times per day, 32.0% (16/50) with seizures of 1-10 times per day, 14.0% (7/50) with seizures of more than 100 times per day. Three types of treatments (range from 1 to 6 types) were used in combination with perampanel, and three types of treatments (range from 0-10 types) were previously used. The age at time of dosing was 64.5 months (4 months to 18 years), and course of disease at dosing was 22.0 months (2 months to 17.3 years). The duration of perampanel at the last follow-up was 8.0 (6.0-14.0) months. At the last follow-up, the maximum daily dose of perampanel was 0.175 (0.06-0.5) mg·kg-1·d-1 and 4 (0.5-2) mg·d-1.The rate of efficacy was 46.0%(23/50). At the last follow-up, 41 patients were treated with perampanel, and the retention rate was 82.0%. Nine patients stopped treatments because of poor efficacy. No correlation was found between the efficacy and gender, etiology, epileptic syndrome, baseline frequency of seizures, age at onset, age at time of dosing, course of disease at dosing, dosage of drugs, or number of treatments. Six patients (12.0%) were observed with irritability and drowsiness during the treatment, and no other serious adverse reactions were observed.
Conclusion: The overall rate of efficacy of using perampanel to treat drug-resistant epilepsy was 46%. The safety and tolerability of perampanel in the treatment of children with drug-resistant epilepsy were relatively good, and no related factors with efficacy were found.
Background: Gestational hypertension is not only one of the leading causes of perinatal death of pregnant women, but also might increase the risk of cardiovascular and cerebrovascular diseases in offspring.
Objective: To investigate the effects of gestational hypertension on blood pressure of offspring in childhood.
Design: Retrospective cohort study.
Methods: The data of baseline survey conducted between 2014 and 2016 from a cohort study including children aged from 6 to 12 years in Chongqing were used. The exposure group was defined as those whose mother had gestational hypertension, and controls were randomly selected in a 1∶4 ratio from the children whose mother had normal blood pressure during pregnancy, matched with gender, age and BMI. Physical examination was performed by well-trained nurses from Children's Hospital of Chongqing Medical University. BP levels and heart rate were measured on three to five separate occasions with an OMRON arm-type electronic sphygmomanometer (HEM-7051). The average value of the closest three of the blood pressure measurements was used to represent resting systolic blood pressure (SBP) and diastolic blood pressure (DBP) level. If the first blood pressure screening met the criteria of hypertension, the second measurement was conducted in the following 2~4 weeks. Children were diagnosed with hypertension if both two BP measurements met the criteria for hypertension. Prehypertension and hypertension was defined as mean clinic-measured SBP and/or DBP ≥P90 and ＜P95, and ≥P95 respectively, based on age, gender, and height percentiles.
Main outcome measures: Odds ratio (OR) that estimating the effect of gestational hypertension on the risk of blood pressure of offspring in childhood.
Results: A total of 1,630 children (exposure group: 326; control group: 1,306) with mean age of 9.10±1.73 years were included for statistical analysis. The systolic and diastolic blood pressures of children in the exposed group were significantly higher than that of control group (SBP: 105.1±10.0 mmHg vs 103.5±9.7 mmHg; DBP: 64.2±7.6mmHg vs 63.2±7.9mmHg). The proportion of prehypertension and hypertension (SBP≥P90or DBP≥P90) in exposure group was 28.5%, which was significantly higher than that of control group (21.9%, P=0.012). The prevalence of childhood hypertension in exposure group was higher than that of control group (16.9% vs 12.9%, P=0.061). After adjusted for age, gender, household income, obesity of mother, breast-feeding, birthweight, weight, heart rate, and obesity, gestational hypertension was an independent risk factor for childhood hypertension (OR=1.66, 95%CI: 1.13~2.43).
Conclusion: Gestational hypertension of the mother is an independent risk factor for offspring's childhood hypertension.
Background: Animal models and cytological studies suggested that Rett syndrome(RTT) had neuronal development and connection deficits,but it still remains unclear how the brain white matter develops and connects in patients.
Objective: To explore the abnormalities of multiple white matter parameters using diffusion tensor imaging (DTI) in RTT children and its correlation with disease severity and developmental level.
Design: Case-control study.
Methods: Children aged 2 to 6 years old, diagnosed with typical RTT and confirmed with MECP2 mutation or deletion, were recruited as the RTT group. Routine MRI scan, Griffith Developmental Scale-Chinese Version (GDS-C) and RTT Severity Score (RSSS) were taken as evaluations. Children aged 2 to 6 years with normal Developmental Screening Test (DST) scores, undergoing MR scan because of paroxysmal dizziness or headache but without recognizable lesions or abnormalities on scans, were recruited as typically developing (TD) controls. GDS-C, RSSS and DST were performed in the Health Care Department of Children Hospital of Fudan University. MR was scanned in the same GE 750 3T scanner in the Radiology Department of our hospital. All of the GDS-C, RSSS, DST and MR scans were completed within one month. Four DTI parameters were collected and tract-based spatial statistics (TBSS) were applied to obtain DTI parameter differences and locations between two groups.
Main outcome measures: Abnormal white matter brain areas in RTT and the correlations with RSSS and developmental domains of GDS-C.
Results: From June 2014 to June 2021, we recruited 22 children as RTT group and 23 children as TD controls. The age difference was not statistically significant. The RSSS score in RTT was 5.5±1.33. The total DQ of GDS-C was 15.8±6.8 and as in domains ADQ was 30.2±10.6, BDQ was 14.6±9.1, CDQ was 15±7.7, DDQ was 9.9±6.2, EDQ was 9.2±3.9. DST score in TD group ranged from 94 to 118 （103±7）. The general FA value in RTT group was lower than that in TD group, MD and DR values in RTT group was higher than that in TD group, and no group difference was found as for DA. DTI-TBSS analysis results (FDR corrected, P<0.05)showed the fibers with lower FA values in RTT group were mainly located in the forceps minor, splenium of corpus callosum, bilateral superior longitudinal fasciculus, left inferior longitudinal fasciculus, retrolenticular part of internal capsule and uncinate fasciculus;the fibers with increased MD values in the RTT group were mainly located in the body of corpus callosum;the fibers with increased DR values in the RTT group were mainly located in the genu of corpus callosum;the fibers with increased DA values in the RTT group were mainly located in the body and splenium of corpus callosum. In RTT group, RSSS was negatively correlated with FA values in splenium of corpus callosum and left superior longitudinal fasciculus, and was positively correlated with MD value and DR value in the body of corpus callosum. FA value of retrolenticular part of internal capsule was positively correlated with the total DQ and DQs of each domain from the GDS-C. DR value of right sagittal stratum was negatively correlated with the total DQ and DQs of each domain from the GDS-C.
Conclusion: This study applied DTI combined with TBSS method in children with RTT and typical development. Results revealed that corpus callosum dysplasia, pervasive brain connectivity deficit and subcortical-cortical circuit structure and connection abnormalities could be found in RTT children.
Background:Children with ASD often encounter difficulties in processing different sensory stimuli, which affect their self-regulation ability to participate in activities of daily living.
Objective: To explore the influence of sensory processing factors on the self-regulation abilities of preschool children with ASD.
Design: Case-control study.
Methods: Preschool children who were first diagnosed of ASD and whose parents completed SPSRC were included. The SPSRC z-score of -1 was used to divide the self-regulation abilities,the sensory processing abilities and their different scales into the group of abnormal (z<-1) and normal. Univariate and multivariate logistic regression analysis were performed to analysis the influence of gender, age and 6 scales of sensory processing abilities on self-regulation function and its scales.
Main outcome measures: Sensory processing factors of APSRC scale affecting self-regulation of ASD children .
Results: The SPSRC scale data of 155 children with ASD were included in this study, including 126 males and 29 females, with an average age of (48.1±10.2) months. The overall self-regulation dimension was abnormal in 112 cases (72.3%) and normal in 43 cases. Fifty-four cases (34.8%) had abnormal physiological conditions and 101 cases were normal, 85 cases (54.8%) had abnormal emotional regulation, and 70 cases were normal state, 72 cases (46.5%) had abnormal adaptability and 83 were normal. Univariate analysis showed that among children with abnormal self-regulation abilities, the incidence of abnormal auditory, visual, tactile, gustatory/olfactory, vestibular and proprioceptive sense was higher than that of normal children, and the differences were statistically significant. Multivariate logistic regression analysis showed that auditory and tactile abnormalities were the risk factors for overall abnormal self-regulation (OR=0.319, 95%CI: 0.111~0.912;OR=0.236, 95%CI: 0.060~0.928) and abnormal emotional regulation (OR=0.314, 95%CI: 0.130~0.759;OR=0.369, 95%CI: 0.145~0.937), and tactile abnormality was the risk factor for abnormal physiological conditions (OR=0.382, 95%CI: 0.155~0.941) and adaptability (OR=0.393, 95%CI:0.170~0.909).
Conclusion: Abnormal auditory and tactile sensens were risk factors for abnormalities in self-regulation,physiological conditions and adaptability.
Background: The detection rate of polycystic thyroid disease (PCTD) by thyroid ultrasonography in healthy children is high, but the incidence of PCTD and related studies are few, and the other group of PCTD deserves attention.
Objective: Thyroid ultrasonography was performed to screen for PCTD in children who needed intervention after the assessment of growth and development .
Design: Cross-sectional survey.
Methods: We included the cases whose complains were relatively short stature, decreased height growth rate or puberty development assessment, with normal FT3 and FT4 and negative antibodies(Tg-Ab,TR-Ab and TPO-Ab), requiring drug intervention and thyroid ultrasonography after clinical evaluation. Medical records and ultrasound results on the day of visit and other were collected. According to the ultrasound image observation and analysis indicators, the patients were divided into normal group, PCTD group (≥4 thyroid cysts, negative tests for thyroid autoantibody,no evidence for thyroid diseases and abnormal thyroid structure) and other group (solid thyroid nodules, multiple cysts with solid nodules and the number of cysts less than 4).
Main outcome measures: Detection rate of PCTD.
ResultsA total of 572 children met inclusion and exclusion criteria, aged from 3.0 to 17.0 (9.5±2.74) years, including 259 males and 313 females. There were 364 cases in normal group, 161 cases in PCTD group (28.2%), and 47 cases in follow-up group. HtSDS<-2 was found in the normal group at 3 to 5 years old and the PCTD group at 4 to 5 years old. WtSDS<-2 was found in the PCTD group at 5 years old. The standard deviation of height and weight in other age groups was in the normal range, but the HtSDS and WtSDS ranged from -2 to 0 . The detection rate of boys and girls with PCTD was 23.6% (61/259) and 31.9% (100/313), respectively . The rate of PCTD was significantly increased (＞27.0%) for 7 years or older. There were 11 cases of goiter in PCTD group (7.1%) and 14 cases of goiter in normal group (4.6%).All cysts in PCTD had regular margins and cysts were distributed diffusely in bilobal and isthmus in 28 cases, 113 cases in bilobal, 17 cases in right lobe and 3 in left lobe. 85.7% of PCTD cysts showed spotty strong echo with comet tail sign, and 74.5% of thyroid parenchyma echo was uniform. Fourteen cases in the normal group and 9 cases in the PCTD group with TSH > 5 mIU·L-1 were in subclinical hypothyroidism.
Conclusion: Pediatric PCTD is not a rare condition, especially higher in children after 7 years of age. The detection rate of PCTD in girls is higher than that in boys. The follicular cysts were mostly located in bilateral lobes, with spotty strong echo and comet tail sign. The cyst size was mostly less than 5 mm.
Background: It is unknown whether the pathogen spectrum of acute respiratory infection (ARTI) have changed significantly since the outbreak of COVID-19.
Objective: To analyze the etiological characteristics of hospitalized children with ARTI before and after COVID-19 epidemic in Shenzhen, in order to provide reference for the clinical diagnosis, treatment and prevention of ARTI.
Design: Cross-sectional survey.
Methods: Pharyngeal swab samples submitted for the first time excluding those of neonates after hospitalization for clinical diagnosis of ARTI in Shenzhen Children's Hospital from September 2, 2019 to February 28, 2021 were included. Multiple PCR and capillary electrophoresis were used to detect 13 kinds of common pathogens in nasopharyngeal secretion samples. Based on the data from March 2020 to February 2021, the pathogen profiles of ARTI in different seasons were compared, from March to May in spring, from June to August in summer, from September to November in autumn, and from December to February of the next year in winter. Data from September 2,2019 to January 24,2020 were used as pre-COVID-19 data and from September 2, 2020 to January 24, 2021 as post-COVID-19 data for comparison.
Main outcome measures: Mycoplasma, chlamydia and common virus spectrum of ARTI.
Results: Among 12,022 cases of nasopharyngeal secretion, pathogens were detected in 7,589 cases with the detection rate of 63.13%. The highest detection rate was for human rhinovirus (HRV,24.83%), followed by respiratory syncytial virus (RSV,14.12%), human parainfluenza virus (HPIV,8.33%), human metapneumovirus (HMPV,7.01%), and mycoplasma pneumoniae (MP,5.37%).There were 1,329 cases of mixed infection with two or more pathogens was detected simultaneously, accounting for 11.05% (1,329/12,022). HRV and MP were the most common combination. The highest detection rate was 66.99% (1,924/2,874) in autumn, with RSV ranked first in the list (25.89%), followed by HRV (24.01%).The lowest detection rate was 16.20% (132/815) in spring, with HRV ranked first in the list (5.03%), followed by MP (3.80%).The detection rate of pathogens in children aged 6 months to 6 years was higher than the overall average.With the increase of age, the detection rate of RSV and HPIV decreased significantly, while the detection rate of MP increased. There was no significant difference in the detection rate between male and female patients (P>0.05). Before COVID-19 epidemic, the detection rate of pathogens was 78.57%（2,592/3,299）, with HRV(36.37%), MP (15.40%), RSV(10.03%), adenovirus (9.03%) as the main pathogens. A total of 764 mixed infection cases were found (23.16%). After COVID-19 epidemic, the detection rate of pathogens during the same period was 65.89%（3,500/5,312）, with HRV (23.78%), RSV (14.74%), HMPV(12.65%) and HPIV (12.12%) as the main pathogens, and there were 459 cases (8.64%) of mixed infections.
Conclusion: The pathogen spectrum of ARTI had changed significantly after the outbreak of COVID-19. The detection rate of HMPV,HPIV,RSV,Bocavirus had increased significantly. However, the detection rate of MP, chlamydia, influenza A,H1N1, H3N2, influenza B, HRV, adenovirus, mixed infections decreased significantly.
Background: There are few reports on the breast milk composition of mothers of premature infants in China. The composition may be affected by genetics and dietary habits.
Objective: To explore the content and changes of macronutrients in breast milk of mothers of premature infants with different gestational weeks and different delivery modes at different stages of lactation.
Design: Cross-sectional survey.
Methods: Mothers of premature infants admitted to NICU with birth weight ＜2,500 g and gestational age＜37 weeks were included. Mothers were＞18 years of age, had no serious congenital malformations, didn't use immunosuppressants, had no breastfeeding contraindications and voluntarily provided fresh breast milk. The whole milk of bilateral breasts was collected at 4 to 5 am by bilateral electric breast pump to disposable breast milk storage bottle, and transported by cold chain. The process of collecting breast milk from mothers to machine for component analysis was completed within 4 hours. MIRIS HMA breast milk analyzer (MIRIS AB, Sweden) was used, and ultrasonic technology was used for homogenization before detection. The clinical information was obtained from the medical records. According to the gestational age of the included mothers, they were categarized into ＜28 weeks group, ~32 weeks group and ~＜37 weeks group. According to the delivery age of the included mothers, they can be categarized into the puerpera in childbearing age ≤34 years old and elderly puerpera > 35 years old. According to the time of collecting breast milk after delivery, they can be categarized into colostrum 1 ~ 7 days postpartum, transition milk ～14 days postpartum and mature milk ~42 days postpartum.
Main outcome measures: Macronutrient content in breast milk of mothers of premature infants at different lactation stages.
Results: Among 191 mothers of premature infants who met the inclusion criteria, 79 (41.4%) delivered vaginally, aged from 18 to 44 (31.1±4.5) years, and gestational age at delivery was (29.2±2.2) weeks. Among 191 infants, 102 (53.4%) were males and the average birth weight was (1,286±344) g. A total of 305 samples of breast milk were collected, including 55 colostrums, 72 transition milk and 178 mature milk. In colostrum, transition milk and mature milk, the fat increased and the protein decreased successively, and the difference was statistically significant. The multiple comparison results of colostrum, transition milk and mature milk showed that there was significant difference in carbohydrate and total energy, but there was no significant difference in carbohydrate and total energy between transition milk and mature milk. There was no significant difference in macronutrients and total energy in breast milk of premature mothers at different gestational weeks. At different gestational weeks, the protein content in transition milk at 24-28 weeks was higher than that at ~ 32 weeks and ～＜37 weeks, and the carbohydrate content in transition milk at 24-28 weeks and ~ 32 weeks was higher than that at ～＜37 weeks, and the difference was statistically significant. For different delivery methods, the fat in colostrum in vaginal delivery was lower than that of cesarean section, the protein content in transition milk and mature milk in vaginal delivery was lower than that of cesarean section, and the carbohydrate of mature milk in vaginal delivery was higher than that of cesarean section, and the difference was statistically significant. For different maternal ages, the fat and total energy in mature milk were significantly lower in mothers with childbearing age than in elderly mothers.
Conclusion: There were significant differences in the macronutrient elements and total energy in breast milk of premature infants at different stages of lactation. It can be considered to carry out breast milk composition analysis to guide the individualized breast-feeding of premature infants in the future.
