Respiratory support is the treatment of respiratory diseases.Currently, the commonly used methods of respiratory support include nasal catheter oxygen or mask oxygen, noninvasive and invasive ventilation, etc.High flow nasal cannula oxygen (HFNC) is a new kind of therapy, which was commonly used in preterm infants as the instead of continuous positive airway pressure (CPAP). However, in recent years, HFNC is more commonly used in children′s intensive care unit and general wards.In this paper, the efficacy and safety of HFNC in pediatric are reviewed.

Objective To summarize the clinical features of the allergic bronchopulmonary aspergillosis (ABPA) in children,in order to improve the understanding for ABPA and make early diagnosis and treatment of the disease.Methods A retrospective study was performed on ABPA patients diagnosed in Department of Respiratory Medicine,Beijing Children's Hospital Affiliated to Capital Medical University from March 2010 to December 2013.The clinical features,laboratory results,image characteristics and the prognosis information were reviewed.Results Eight ABPA cases met the diagnostic criterion.All patients were school-age children (7 years and 2 months to 13 years and 8 months old).Cough (8 cases),productive sputum (8 cases),wheezing (5 cases),fever (4 cases) and hemoptysis (3 cases) were the main clinical features.Six of the 8 patients showed eosinophilia.IgE level was elevated in 7 patients (1.000-5.000 IU/L).All 8 patients were allergic to aspergillus fumigatus,while only 2 cases were positive in sputum culture for aspergillus fumigatus.CT scans showed pulmonary opacities in all 8 cases,while 7 patients had typical central bronchiectasis.Seven patients were treated with the regimen,which included glucocorticosteroid,antifungal agents (voriconazole or itraconazole) and regular bronchoscope.The symptoms of all treated patients relieved,the total serum IgE level and eosinophil cell count decreased spontaneously after the therapy.Conclusions ABPA is rare in children and the clinical features are non-specific.If the patient has elevated total IgE level in serum and eosinophilia,especially in patients with underling diseases,ABPA should be suspected.The positive result of specific antibodies to aspergillus fumigatus and central bronchiectasis on the radiology may give the suggestive diagnosis.ABPA patients generally have good response to the therapy of glucocorticosteroid and antifungal agents.

Objective To identify the association between human glucocorticoid receptor gene G166T polymorphism and essential hypertension(EH).Methods DNA samples from 71 normotensive cases and 48 EH cases were analyzed by a polymerase chain reaction(PCR) for restriction fragment length polymorphism(RFLP) to determine the intron 4 variant of human glucocorticoid receptor gene.Results No association was observed between EH group and control group.However,in females,a little association was observed between the EH group and control group.Frequencies of allele G were 0.64 in EH group and 0.46 in control group.Conclusion The G allele may be a predisposing gene marker,HGR gene intron 4 polymorphism contributes to the development of EH in females.

Studying classic cases is one of the applicable methods for student's self-learning,especially at clinical rotation period.Seven-year program pediatric students (2004 grade) from pediatric medical collage,Capital Medical University of China shared classic cases from New England Journal of Medicine (NEJM),reported clinical cases they took part in and summarized their writing of case report.Through these measures,students felt the significance of studying classic cases and noticed that case study played an important role in training of clinical reasoning,multidisciplinary approach,as well as self-learning ability for medical students.

Objective: To investigate the effect of moxibustion on telomerase activity and genes expression in tissues of senescent rats. Methods: Subacute aging rats model were established by injection with D-gal solution. Points Shenshu (BL 23) were treated with moxibustion in treatment group, contrasting with a model group and a normal group. Enzyme-Linked Immunosorbent Assays(ELISA) was used for the level oftelomerase activity in liver tissues, and In Situ Hybridization(ISH) was used for the condition of expression of telomerase genes in liver tissues. Results: The level of telomerase activity in the aging model group was obviously lower than that in normal control group (P＜0.01), the level in moxibustion group was obviously higher than that in model group(P＜0.05). In comparison with normal rats, the positive-expressed areas and photodensity of telomerase genes in aging model group were all significantly lower (P＜0.01,P＜0.01), and the positive-expressed areas in moxibustion group was significantly higher than that in model group (P＜0.01). Conclusions: Moxibustion could regulate telomerase activity of senile rats, hence delaying aging.

Objective:To investigate the etiology of pleural effusion in hospitalized children in Beijing Children′s Hospital.Methods:Clinical information of children with pleural effusion admitted to Beijing Children′s Hospital Affiliated to Capital Medical University from January 2016 to December 2018 was retrospectively analyzed.According to the etiology, the children were divided into infection group (parapneumonic pleural effusion, tuberculous pleurisy and empyema) and non infection group.According to the age, the children were further divided into ≤ 3 years old, >3-7 years old and > 7 years old groups.Classification of statistics was performed, and the etiology of pleural effusion were retrospectively analyzed.Results:Among the 1 165 children with pleural effusion, 746 cases(64.0%) were infected with pleural effusion, 697 cases (697/746, 93.4%) of who were parapneumonic effusion.In patients with parapneumonic effusion, 457 cases (61.3%) had Mycoplasma pneumonia (MP) infection.Infectious pleural effusion was more common in children >7 years old(339/479 cases, 70.8%), while non-infectious pleural effusion was prevalent in children under 3 years old(188/324 cases, 58.0%). The difference was statistically significant ( χ2=96.33, P<0.05). Among the patients with non-infectious pleural effusion, 239 cases (239/419 cases, 57.0%) had multi-system diseases and 97 cases (97/419 cases, 23.2%) had malignant pleural effusion.All the 18 deaths were non-infectious pleural effusion. Conclusions:The leading reason for pleural effusion in children is infection.The most prevalent symptom is parapneumonic effusion, which is mainly caused by MP.

Objective To explore the diagnosis and treatment of atypical severe combined immunodeficiency disease (SCID). Methods The clinical data of atypical SCID in 7 children with IL2RG,JAK3,and RAG1 mutations were reviewed and analyzed from September 2012 to June 2017. Results In 7 cases (6 males and 1 female), there were 5 infants, 1 toddler and 1 school-age child. Cases 2, 4, and 6 were classic SCID clinical phenotypes. Cases 1, 3, 5, 7 were atypical SCID clinical phenotypes. Case 6 were diagnosed with Omenn syndrome. Cases 2, 5 were classic SCID immune phenotypes, cases 1, 3, 4, 6, 7 were atypical SCID immune phenotypes, and case 1 had maternal chimera. The next generation sequencing indicated that case 1 had a compound heterozygous JAK3 mutation with c.3097-1G>A/c.946-950GCGGA>ACinsGGT.Cases 2,3,and 4 had IL2RG mutations,with c.865C>T/p.R289X,c.664C>T/R222C,52delG,respectively.Case 5 had JAK3 mutations with c.2150A>G/p.E717G and c.1915-2A>G.Sanger sequencing indicated that case 6 had a RAG1 mutation of complex heterozygosity with c.994C>T/p.R332X and c.1439G>A/p.S480N. Case 7 had homozygous RAG1 mutation with c.2095C>T/p.R699W.Conclusion Under certain conditions,gene mutation can lead to atypical clinical and/or immune phenotypic SCID.

Objective:To describe the clinical features of pediatric biotinase deficiency (BTD) manifested as spinal cord disease.Methods:The clinical data of a child with spinal cord lesions due to biotinase deficiency, diagnosed in Beijing Children′s Hospital in 2020, were collected. The cases with complete clinical data retrieved on literature reported in China National Knowledge Infrastructure, Wanfang Data knowledge Service Platform and PubMed (up to August 2021) by using search terms of biotinase deficiency, pediatric, spinal cord, myelopathy and myelitis were summarized.Results:The patient was a 3 years and 5 months old boy with the main clinical manifestations of subacute progressive limb weakness and wheezing. Physical examination showed sparse hair, rough skin, spastic paraparesis and developmental delay. Cerebrospinal lactic acid was increased (5.67 mmol/L). Cranial magnetic resonance imaging (MRI) showed diffuse T 2/fluid attenuated inversion recovery hyperintensity of the midbrain, dorsal pons, edulla, periacqueductal grey and optic tracts. Spinal cord lesions were extended from the medulla up to the level of the conus. Urineketone bodies and 3-hydroxyisurate were increased. The activity of biotinidase was 0.27 pmol/min (3 mm disc), being 7% of mean normal serum activity. Genetic studies revealed homozygous mutation in the BTD gene [c.284T>A (p.I95N)]. After biotin supplementation for 6 months, the only evident abnormality was residual spasticity of lower limbs. Fourteen English literatures and 2 Chinese literatures including 18 cases were collected. The onset age was from 2 months to 15 years (median age was 4 years). Among them, 11 cases had cranial MRI abnormalities, of which all involved brain stem, 6 cases involved optic tracts and (or) optic chiasm. All 18 cases had spinal cord MRI abnormalities with longitudinally extensive lesion, mostly involved cervical and thoracic spinal segments, and 3 cases involved all spinal segments. Twelve cases received immunotherapy, and 6 were partially improved, 6 were completely invalid. After biotin supplementation, 12 patients had neurological disability. Conclusions:BTD should be included in the differential diagnosis of subacute myelopathy, regardless of the onset age. Early diagnosis and treatment can prevent irreversible neurological damage.

Many different types of neuromuscular disorders can affect respiratory function by either a muscle itself or the nerve(s) supplying that muscle.Patients with certain inherited neuromuscular diseases,such as spinal muscular atrophy,spinal muscular atrophy with respiratory distress type 1,SEPNl-related myopathies,can present with recurrent respiratory system infections,acute or chronic respiratory failure as well as sleep disordered breathing.The respiratory complications could show up even before the manifestation of neuromuscular diseases.The chest X-ray,blood gas analysis,spirometry,sleep studies etc can be used to assess the affected respiratory muscle function,and also can be used to prove the suspicion of the neuromuscular disorders.

Objective:A case of cystic fibrosis admitted in the Respiratory Department of Beijing Children′s Hospital, Capital Medical University in June 2018 and underwent lung transplantation later was analyzed retrospectively.A 10-year-old girl had intermittent productive cough for more than 4 years with clubbed-finger.The lung high resolution CT (HRCT) showed bronchiectasis and mucus impaction, and the nasal sinus HRCT showed sinusitis.She had cystic fibrosis transmembrane conductance regulator ( CFTR) gene complex heterozygous mutation and positive sweat test.The immunoglobulin E (IgE) level and eosinophil count increased, and aspergillus fumigatus-specific IgE was positive.She was diagnosed as cystic fibrosis, allergic bronchopulmonary aspergillosis and sinusitis.Anti-infection, glucocorticoid and symptomatic treatment were given.During the following 21 months, the child had repeated respiratory difficulties and respiratory failure.Her lung function declined.Bilateral lung transplantation was performed in March 2020.She had a good recovery at more than 1 year and 3 months postoperatively.