Objective:To construct an indicator system for entrustable professional activities (EPAs) in the general practice enhancement stage of pediatric specialist physician training.Methods:A draft indicator system for EPAs in the general practice enhancement stage of pediatric physician training was developed through core EPAs working group discussion, literature review, nominal group discussion, and expert consultation. Subsequently, the indicator system was preliminarily implemented and revised.Results:The core EPAs working group consisted of nine specialist physician trainers. In the initial brainstorming stage, a "potential list" of 30 activities was established. After literature review and collation, the draft indicator system included eight EPAs. Through nominal group discussion, the connotation of the draft was enriched, and the importance of the EPAs was ranked and modified. Finally, through expert consultation, the EPAs for the general practice enhancement stage of pediatric specialist physician training were determined. These included basic operations for the treatment of critically ill children, identification and management of critical illnesses, referral of critically ill children, perioperative management, in-hospital consultation, medical and teaching management and system improvement, doctor-patient communication and dispute handling, and response to public health events. During the preliminary implementation stage, a total of nine specialist physicians who participated in the training were evaluated. Based on the problems found in the pre-evaluation, the indicators of EPAs were revised, and a corresponding curriculum training system was developed.Conclusions:Through multiple rounds of nominal group discussion and expert consultation, the indicator system for EPAs in the general practice enhancement stage of pediatric specialist physician training was formulated. The system was preliminarily implemented and revised, and a curriculum system was constructed.

Objective:To construct an indicator system for entrustable professional activities (EPAs) in the general practice enhancement stage of pediatric specialist physician training.Methods:A draft indicator system for EPAs in the general practice enhancement stage of pediatric physician training was developed through core EPAs working group discussion, literature review, nominal group discussion, and expert consultation. Subsequently, the indicator system was preliminarily implemented and revised.Results:The core EPAs working group consisted of nine specialist physician trainers. In the initial brainstorming stage, a "potential list" of 30 activities was established. After literature review and collation, the draft indicator system included eight EPAs. Through nominal group discussion, the connotation of the draft was enriched, and the importance of the EPAs was ranked and modified. Finally, through expert consultation, the EPAs for the general practice enhancement stage of pediatric specialist physician training were determined. These included basic operations for the treatment of critically ill children, identification and management of critical illnesses, referral of critically ill children, perioperative management, in-hospital consultation, medical and teaching management and system improvement, doctor-patient communication and dispute handling, and response to public health events. During the preliminary implementation stage, a total of nine specialist physicians who participated in the training were evaluated. Based on the problems found in the pre-evaluation, the indicators of EPAs were revised, and a corresponding curriculum training system was developed.Conclusions:Through multiple rounds of nominal group discussion and expert consultation, the indicator system for EPAs in the general practice enhancement stage of pediatric specialist physician training was formulated. The system was preliminarily implemented and revised, and a curriculum system was constructed.

Objective:To summarize the characteristics of autosomal recessive axonal neuropathy with neuromyotonia (ARAN-NM) caused by HINT1 gene mutation. Methods:Retrospective case summary.Clinical data of 2 Tibetan siblings diagnosed with ARAN-NM in the Department of Pediatrics of Peking University First Hospital in August 2023 were retrospectively analyzed.A review of literature reporting relevant Chinese patients was conducted.Results:The proband and her elder brother were aged 13 and 19, respectively.Both developed abnormal gait at the age of 11, followed by varus, claudication, and weak thumb strength.The proband also had neuromyotonia.Physical examinations showed that the proband and her elder brother had decreased muscle strength of the extremities, mainly in the thumbs and distal ends of lower limbs.The distal muscles of the proband′s lower extremities and the muscles of both hands of the proband′s elder brother were atrophied.Both feet showed talipes equinovarus in the proband and her elder brother.The proband′s electromyography (EMG) showed peripheral nerve injuries (motor and sensory axonal involvement, especially in distal ends) and myotonic potentials.The trio-whole exon sequencing detected homozygous pathogenic variation in HINT1 gene in both the proband and her elder brother, who were diagnosed as ARAN-NM based on c. 169A>G (p.K57E). After the Carbamazepine treatment, the proband′s neuromyotonia, numbness and weakness were relieved.Both the proband and her elder brother underwent orthopaedic surgery and rehabilitation.Their foot deformities and gait were significantly improved.Two Chinese literatures (2 patients) and four English literatures (8 patients) were retrieved.Including the proband and her elder brother in this study, there were 12 ARAN-NM patients, 10 of whom had clinical data.The ages of onset and diagnosis were 2-16 (1 case unknown) and 13-33 years old, respectively.Myasthenia was present in 9 patients, especially in distal ends.Eight patients were complicated with neuromyotonia, nine patients with muscle atrophy, seven patients with foot deformity, and two patients with sensory disturbance.Creatine kinase(CK) was elevated in all 9 patients tested or CK.EMG showed neurogenic injuries in all patients and neuromyotonia discharge in six patients.Three patients were treated with Carbamazepine, and some symptoms were relieved.Missense/nonsense mutations were found in the 12 patients, and the high-frequency variation was c. 112T>C (p.C38R). Conclusions:ARAN-NM is a rare autosomal recessive neuromuscular disease caused by HINT1 gene mutation.There is no ethnic difference in clinical manifestations, mainly distal limb weakness with neuromyotonia.Carbamazepine can alleviate some symptoms, and orthopaedic surgery can improve foot deformity and gait.

Objective:To study the clinical application and complications of umbilical arterial catheterization (UAC) in premature infants.Methods:From January 2021 to December 2022, premature infants with UAC successfully inserted in NICU of our hospital were enrolled. According to birth weight (BW), the infants were assigned into three groups: <1 000 g, 1 000~1 499 g and ≥1 500 g. The perinatal data, UAC usage, UAC-related complications and risk factors of UAC-related complications were retrospectively analyzed.Results:A total of 39 premature infants received UAC, with gestational age 29.3(27.3, 30.4) weeks and BW 1 100 (900, 1 310) g. The insertion length (IL) of UAC was calculated using the average value of two formulas: a, IL (cm) =4×BW (kg) +7; and b, IL(cm) =3×BW (kg)+9. The accuracy of tube end position was determined using chest/abdomen radiography. 30(76.9%) cases had accurate position, 6(15.4%) had higher position and 3(7.7%) had lower position. The proportion of appropriately positioned tube end in <1 000 g, 1 000~1 499 g and ≥1 500 g groups were 80.0%, 76.5% and 71.4%, respectively, without statistically significant differences ( P>0.05) .No significant differences existed among the three groups in UAC duration and UAC routinely removal rate ( P>0.05). 9 cases (23.1%) of UAC were removed for specific reasons, including 4 cases of arterial spasm, 2 cases of withdrawal of treatment, 1 case of tube end displacement, 1 case of abdominal distension and 1 case of death. 21 cases received 1 U/ml heparin (0.9%NaCl solution) 0.5~1 ml/h arterial infusion. 23.8% (5/21) had hypernatremia and the level of sodium became normal after reducing the concentration of NaCl solution. Arterial vasospasm occurred in 4 patients with skin color changes of one side of the lower extremities. After UAC removal, the skin color returned to normal. Conclusions:UAC is helpful and safe for preterm infants, however, its complications should be alerted to.

Objective:To investigate the efficacy and safety of recombinant tissue plasminogen activator (rt-PA) in patients with wake-up ischemic stroke (WUIS) of anterior circulation non-major arteries.Methods:Sixty-seven patients with WUIS of anterior circulation non-major arteries (time from falling asleep/last use of bathroom at night to intravenous thrombolysis≤9 h and >4.5 h) admitted to Department of Neurology, Beijing Shijingshan Hospital and Department of Neurosurgery, Beijing Muyangliu Hospital from January 1 st, 2017 to December 31 st, 2021 were chosen; these 35 patients accepted routine antiplatelet therapy after intravenous thrombolytic therapy were chosen as control group, and the other 32 patients accepted rt-PA arterial thrombolysis after intravenous thrombolytic therapy were chosen as study group. Vascular recanalization after arterial thrombolysis was observed in study group (cerebral infarction thrombolysis grading 2 and 3: good vascular recanalization). Neurological deficit improvement during treatment, clinical prognoses (modified Rankin scale scores of 0-2: good prognosis) and hemorrhagic transformation incidence 3 months after treatment were compared between the 2 groups. Results:The vascular thrombolytic recanalization rate of study group was 81.25% (26/32). Patients in study group had significantly decreased National Institute of Health stroke scale scores compared with those in control group 1, 7, and 14 d after thrombolytic therapy ( P<0.05). The good prognosis rate of study group (62.50%, 20/32) was significantly higher than that in control group (37.14%, 13/35, P<0.05). No significant difference in hemorrhagic transformation rate was noted between the 2 groups (15.6% [5/32] vs. 5.71% [2/35], P>0.05). Conclusion:Patients with WUIS of anterior circulation non-major arteries (time from falling asleep/last use of bathroom at night to intravenous thrombolysis≤9 h and >4.5 h) benefit from arterial thrombolysis with rt-PA, and risk of secondary intracerebral hemorrhage is not obviously increased.

Objective::To build a reference fetal growth chart for the Chinese population based on fetal ultrasound measurements.Methods::This was a multicenter, population-based retrospective cohort study. Longitudinal ultrasound measurement data were collected from 24 hospitals in 18 provinces of China from 1 st September through 31 st October of 2019. The estimated fetal weight (EFW) was calculated based on head circumference, abdominal circumference, and femur length using Hadlock formula 3. Fetal growth curves were estimated using a two-level linear regression model with cubic splines. All participants were divided into two groups: the northern group ( n = 5829) and the southern group ( n = 3246) based on the geographical division of China and male fetus group ( n = 4775) and female fetus group ( n = 4300) based on fetal gender. The EFW was compared by fetal gender and geographical group. All statistical models were adjusted for maternal sociodemographic characteristics. Results::A total of 9075 participants with 31,700 ultrasound measurement records were included in this study. Male fetuses demonstrated significantly larger EFW compared to female ones starting at 16 weeks of gestation and extending to delivery (global test P < 0.01). The overall geographic difference in EFW was significant (global test P = 0.03), and week-specific comparisons showed that the northern group had a greater EFW starting at 15 weeks of gestation and extending to 29 weeks of gestation, although this difference did not extend to the time of delivery. The Z-score of EFW confirmed that our Chinese fetal growth charts differed from previously published standards. Conclusion::This study provides EFW and ultrasound biometric reference measurements for Chinese fetuses and reveals differences from other fetal growth charts. The chart is worth promoting in more regions of China but should be tested prudently before use.

Objective::To build a reference fetal growth chart for the Chinese population based on fetal ultrasound measurements.Methods::This was a multicenter, population-based retrospective cohort study. Longitudinal ultrasound measurement data were collected from 24 hospitals in 18 provinces of China from 1 st September through 31 st October of 2019. The estimated fetal weight (EFW) was calculated based on head circumference, abdominal circumference, and femur length using Hadlock formula 3. Fetal growth curves were estimated using a two-level linear regression model with cubic splines. All participants were divided into two groups: the northern group ( n = 5829) and the southern group ( n = 3246) based on the geographical division of China and male fetus group ( n = 4775) and female fetus group ( n = 4300) based on fetal gender. The EFW was compared by fetal gender and geographical group. All statistical models were adjusted for maternal sociodemographic characteristics. Results::A total of 9075 participants with 31,700 ultrasound measurement records were included in this study. Male fetuses demonstrated significantly larger EFW compared to female ones starting at 16 weeks of gestation and extending to delivery (global test P < 0.01). The overall geographic difference in EFW was significant (global test P = 0.03), and week-specific comparisons showed that the northern group had a greater EFW starting at 15 weeks of gestation and extending to 29 weeks of gestation, although this difference did not extend to the time of delivery. The Z-score of EFW confirmed that our Chinese fetal growth charts differed from previously published standards. Conclusion::This study provides EFW and ultrasound biometric reference measurements for Chinese fetuses and reveals differences from other fetal growth charts. The chart is worth promoting in more regions of China but should be tested prudently before use.

Objective:To investigate the incidence of hypothyroxinemia in very low birth weight infant (VLBWI) and its effect on early postnatal feeding and weight gain.Methods:This retrospective study analyzed 164 cases of VLBWIs admitted to the Neonatal Intensive Care Unit of Peking University First Hospital from January 2017 to December 2018. According to the gestational age, these VLBWIs were divided into <30 weeks group ( n=85) or ≥30 weeks group ( n=79), and the basic data and thyroid function were compared. According to the levels of serum tetraiodothyronine and free tetraiodothyronine at the first thyroid function test, the subjects were further assigned into normal thyroxine group and hypothyroxinemia group. The risk factors of hypothyroxinemia identified at the first detection were analyzed by single and multiple-facter analysis. The results of the second detection of thyroxine were also analyzed. On the basis of the first detection and receiving treatment or not, the <30 weeks and ≥30 weeks groups were divided into normal thyroxine, hypothyroxinemia treated and hypothyroxinemia untreated subgroups, and differences in the tolerance of early feeding and weight gain were compared between different groups. Two independent samples/paired t-test, rank sum test, Chi-square test and logistic regression were used for statistical analysis. Results:Out of the 164 VLBWIs with the gestational age of (29.7±2.0) weeks and birth weight of (1 210±210) g, 27 cases (16.5%) were extremely low birth weight infants. The age at their first detection was (10.7±3.1) d and the incidence of hypothyroxinemia was 45.1% (74/164), including 71 mild and three severe cases, with a higher incidence in the ≥30 weeks group comparing to the <30 weeks group [55.7%(44/79) vs 35.5%(30/85), χ 2= 6.883, P=0.009]. All the three severe cases were in the ≥30 weeks group. The gestational age ( OR=1.413, 95% CI:1.044-1.912, P=0.025) and male infant ( OR=2.082, 95% CI: 1.047-4.143, P=0.037) were the risk factors of hypothyroxinemia. At the second detection, the incidence of hypothyroxinemia in VLBWIs with normal thyroid function at their first test was 47.6% (39/82), which is higher in the ≥30 weeks group than in the <30 weeks group [64.5%(20/31) vs 37.3%(19/51), χ 2= 5.745, P=0.017]. Among the infants with hypothyroxinemia at the first detection, those untreated had a significantly higher incidence of hypothyroxinemia at the second detection than those treated [81.3%(26/32) vs 38.7%(12/31), χ 2= 11.905, P=0.001]. The incidence of abdominal distension within 21 days, feeding volume on day 7, 14, and 21, and neonatal weight gain within 7, 14, and 21 days were similar between normal thyroxine, hypothyroxinemia treated and hypothyroxinemia untreated subgroups within the ≥30 weeks or the <30 weeks groups (all P>0.05). Conclusions:VLBWI is at high risk of hypothyroxinemia. Two times of postnatal thyroid function tests can help to detect the delayed hypothyroxinemia. Thyroxine level and receiving treatment or not may have no significant effect on the early postnatal feeding and weight gain.

Objective To study the role of sequential cranial ultrasound (cUS) in the early prognosis of neurodevelopmental outcome in the very low birth weight and extremely low birth weight preterm infants.Method Clinical and examination data of premature infants with birth weight less than 1 500 g,who were admitted to the neonatal intensive care unit of our hospital within 24 hours after birth from January 2012 to December 2016 were analyzed retrospectively.Early cranial ultrasound refers to the cUS scans during the period of 0 to 14 days after birth.Classification was applied to the whole set of early cUS scans based on the most severe lesion observed.Index 1 was periventricular-intraventricular hemorrhage (PVH-IVH),which was divided into none and grade 1 to 4 according to the severity.Index 2 was paraventricular white matter echo,which was divided into normal,slightly enhanced and significantly enhanced.The cUS scan repeated at term-equivalent age (corrected gestational age 37 ～ 44 weeks) was referred to as term cUS.Term cUS was evaluated according to paraventricular leukomalacia (PVL) and enlargement of ventricle.Data were analyzed using IBM SPSS Statistics version 16.0.Result A total of 200 premature infants were collected.The gestational age was (30.1 ± 1.9) weeks,and the birth weight was (1 203 ± 186) g.The median time of cUS scans during hospitalization was 3 times.The age at which the Gesell Development Diagnosis Scale was completed was (11.8 ±4.0) months,the corrected age was (9.7 ±4.0) months.The development quotient (DQ) of gross motor was lower than that of the other four items,and the difference was statistically signi icant.Analysis of data suggested that PVH-IVH grade 3 or grade 4,significantly enhanced paraventricular white matter echo found in early cUS,and PVL or enlargement of ventricle found in term cUS were all associated to lower gross motor DQ (P ＜ 0.05).PVL was also significantly correlated with lower DQ of adaptability,fine motor,language and personal-social (P ＜ 0.05).Conclusion Compared to adaptability,fine motor,language and personal-social,gross motor has the lowest DQ among very low or extremely low birth weight infants.PVH-IVH grade 3 or worse,significantly enhanced paraventricular white matter echo found in early cUS,and PVL or enlargement of ventricle found in term cUS are high risk factors for adverse outcome of gross motor.Among them,PVL found in term cUS suggests poor neurodevelopmental outcome.

Objective To compare the short-term outcomes of less invasive surfactant administration (LISA) with traditional intubate-surfactant-extubate (INSURE) method for respiratory distress syndrome (RDS) in premature infants.Method From January 2017 to December 2018,premature infants (gestational age ≤32 weeks) diagnosed with RDS who needed pulmonary surfactant (PS) administration were prospectively enrolled and randomly assigned into LISA group and INSURE group.The duration of oxygen supply,side effects during PS administration,the outcome and severe adverse events,bronchopulmonary dysplasia (BPD),necrotizing enterocolitis (NEC),3 ～ 4° periventricular intraventricular hemorrhage (PIVH),periventricular leukomalacia (PVL) and death were compared.Result A total of 67 cases were enrolled including 34 in LISA group and 33 in INSURE group.No significant differences existed between the two group on mechanical ventilation rates,oxygen therapy duration and the incidences of severe adverse events.However,the duration of noninvasive ventilation in LISA group was significantly longer than INSURE group [(10.5 ± 8.7) d vs.(7.1 ± 2.6) d,P ＜ 0.05].The LISA group had significantly higher PS reflux rate than INSURE group [41.2％ (14/34)vs.18.2％ (6/33),P ＜0.05].Conclusion LISA has similar clinical effects and severe adverse events as INSURE,but with higher rates of PS reflux and longer duration of noninvasive ventilation.