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06 March 2023, Volume 38 Issue 3 Previous Issue   

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Expert consensus on the prevention and treatment of iron deficiency and iron deficiency anemia in children Child Health Care Branch of Chinese Preventive Medicine Association, Early Child Development Branch of China Maternal and Child Health Association, Health Care Branch of Futang Research Center of Pediatric Development, et al 2023, 38(3): 161-167.  DOI: 10.19538/j.ek2023030601 Abstract ( )   Iron deficiency （ID） and iron deficiency anemia （IDA） affect the growth and development of the cerebral cognitive function of children. The prevalence of ID and IDA in Chinese children has decreased significantly compared with previous years， but the prevalence in children in some areas is still high due to the vast territory， the uneven development of different areas and the different living habits of various ethnic groups. The standardized prevention and treatment of child ID and IDA is of great significance for improving children's growth and development and our population quality. Therefore， the expert group of the Child Health Care Branch of Chinese Preventive Medicine Association， Early Child Development Branch of China Maternal and Child Health Association，Health Care Branch of Futang Research Center of Pediatric Development， Chinese Journal of Practical Pediatrics referred to the national documents and the clinical guidelines and expert consensus of domestic and foreign， consulted evidence-based literature， and formulated the Expert Consensus on the Prevention and Treatment of Iron Deficiency and Iron Deficiency Anemia in Children  after much discussion， which met the Chinese clinical needs.

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Expert consensus on integrated traditional Chinese and western medicine of diagnosis and treatment with combined allergic rhinitis and asthma syndrome in children（2023） Professional Committee of Child Allergology, China Maternal and Child Health Association, Editorial Committee of Chinese Journal of Practical Pediatrics 2023, 38(3): 168-176.  DOI: 10.19538/j.ek2023030602 Abstract ( )   Combined allergic rhinitis and asthma syndrome （CARAS） in children refers to a syndrome including both allergic rhinitis and asthma， which involves upper and lower airway inflammation. In traditional Chinese medicine， there was a record of “lung opening at nose”， discussed the “whole” relationship between “rhinorrhea” and “asthma”， and accumulated rich experience in “same treatment for different disease”. This consensus is formulated to improve the awareness of physicians in pediatrics， otolaryngology， traditional Chinese medicine， general practice medicine and other related professions on the importance of joint management of CARAS. The consensus-making team is composed of experts from pediatrics， otorhinolaryngology and traditional Chinese medicine. Our principle is simultaneous diagnose and treatment for both upper and lower airway， and the integration of traditional Chinese and western medicine. This paper introduces the concept， pathogenesis， clinical manifestation and stage， drug and non-drug treatment and management of CARAS in children and puts forward the future research direction.

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Expert consensus on clinical early warning and early decision of severe pneumonia in children Professional Committee of Child Allergology, China Maternal and Child Health Association, Editorial Committee of Chinese Journal of Practical Pediatrics 2023, 38(3): 177-182.  DOI: 10.19538/j.ek2023030603 Abstract ( )   Severe pneumonia is the main cause of death in children under 5 years of age worldwide. Early identification of risk factors of severe pneumonia and intervention treatment can greatly reduce the mortality of severe pneumonia. It will make sense to pediatricians of primary clinics for establishing a clinically feasible evaluation system for severe pneumonia and standardizing the diagnosis and treatment path of children's pneumonia. This expert consensus based on clinical practice， combined with feasible laboratories， image indicators， and the application of pediatric early warning scoring models. It is intended to help pediatricians of primary clinics to judge and treat the severe pneumonia early， transport dangerous patients so as to achieve a higher cure success rate.

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Interpretation of the 2022 Extracorporeal Life Support Organization Guidelines for the Provision and Evaluation of Nutritional Support in the Neonatal and Pediatric ECMO Patients ZHOU Hong-qin, CHEN Xiu-ping, HU Xuan, et al 2023, 38(3): 183-186.  DOI: 10.19538/j.ek2023030604 Abstract ( )   Children supported by extracorporeal membrane oxygenation are in a state of high nutritional risk. Once mismanaged， it will seriously affect the prognosis. It is critical to accurately estimate energy requirements and select the best nutritional supply route. In 2022， the Extracorporeal Life Support Organization promulgated the guidelines for“Provision and Evaluation of Nutritional Support in the Neonatal and Pediatric ECMO Patients” ， putting forward 7 suggestions on nutritional screening and evaluation， metabolism and nutritional needs， time to start enteral and parenteral nutrition support， identification of feeding and its management strategy， and consensus-based nutrition calculation method. The purpose of this article is to interpret the recommendations of the guideline， in order to provide guidance for clinicians to formulate nutritional strategies for these children.

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Improvement of the diagnosis and treatment of rare respiratory diseases in children XU Bao-ping, WANG Hao 2023, 38(3): 187-189.  DOI: 10.19538/j.ek2023030605 Abstract ( )   Respiratory rare diseases are an important disease in children， which seriously affects the physical and mental health of children. However， due to its low incidence rate and nonspecific clinical manifestations， it is easy to cause misdiagnosis and delayed diagnosis. Early diagnosis and treatment and standardized management of rare respiratory diseases in children are of great significance to improving their prognosis and even survival.This article discusses the diagnosis， treatment and management of children with rare respiratory diseases， as well as clinical and basic research， in order to improve the understanding and management level of pediatric medical workers of such diseases.

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Neuroendocrine cell hyperplasia of infancy LIU Xiu-yun 2023, 38(3): 190-193.  DOI: 10.19538/j.ek2023030606 Abstract ( )   Neuroendocrine cell hyperplasia of infancy （NEHI） is an pulmonary dysmaturation disorder of interstitial lung disease in infants. The etiology of NEHI is unknown. The clinical features of NEHI are tachypnea， retraction， crackles and hypoxemia. The diagnosis mainly depends on immunohistochemistry of bombesin. The number of neuroendocrine cells （NECs） increases in the distal airway. NECs exist in at least 75% of the airways; NECs account for 10% in each airway; there is lack of the features diagnosed as the other airway and interstitial diseases. High resolution computed tomography （HRCT） of lungs shows central ground glass opacities， mainly involving right middle lobe and left lingula， and air trapping in other areas. There is no specific treatment for NEHI， mainly  oxygen therapy and basic nutritional support treatment. NEHI is a benign process， no death has been reported， and it is relieved with age.

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X-linked lymphoproliferative disease type 1 HE Jian-xin 2023, 38(3): 194-198.  DOI: 10.19538/j.ek2023030607 Abstract ( )   X-linked lymphoproliferative disease type 1 is a X-linked recessive inherited disorder associated with EB virus infection. there is severe clinical manifestation with poor prognosis.Its mechanisms，clinical features，differential diagnosis and treatments will be discussed in this article.

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Congenital pulmonary alveolar proteinosis ZHANG Dong-wei, LU Gen 2023, 38(3): 199-204.  DOI: 10.19538/j.ek2023030608 Abstract ( )   In recent years， due to deeper and deeper understanding of the pathogenesis of pulmonary alveolar proteinosis， its classification has become more and more detailed， but the clinical manifestations， diagnosis， treatment and prognosis of different types of pulmonary alveolar proteinosis （PAP）are significantly different. Congenital PAP is caused by impaired pulmonary surfactant production， which is a group of diseases caused by mutations in genes encoding pulmonary surfactant or proteins involved in pulmonary surfactant production and lung development， accounting for approximately 2% of PAP. The genes involved include SFTPB， SFTPC， ABCA3， and NKX2-1. This article summ-arizes the pathogenesis， clinical manifestations， diagnostic methods， treatment strategies and prognosis of congenital PAP.

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Cystic fibrosis in Chinese patients TIAN Xin-lun 2023, 38(3): 204-209.  DOI: 10.19538/j.ek2023030609 Abstract ( )   The reported number of cystic fibrosis （CF） patients in China has increased significantly in recent years. The clinical symptoms of Chinese CF， unlike in Western world， are not typical， in which bronchiectasis may be the only manifestation and pancreatic exocrine insufficiency is rare. The common genotypes in Chinese cannot be detected by using the gene screening package which are routinely used in Western World. As a result， the disease is often misdiagnosed or can’t， be diagnosed. The treatment experiences of CF anr based on Caucasian population， and clinicians are generally in lack of treatment experiences for Chinese CF patients，and there is no specific treatment. This article reviews the progress in epidemiology， clinical manifestations， diagnosis and treatment of CF in China in order to improve clinicians' understanding of the disease and achieve early diagnosis and treatment， and reduce mortality.

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Diffuse pulmonary lymphangiomatosis in children NONG Guang-min 2023, 38(3): 209-214.  DOI: 10.19538/j.ek2023030610 Abstract ( )   Lymphangiomatosis is a rare disease， which is characterized by abnormal proliferation of lymphatic endothelial cells. The proliferative lesions are usually diffuse and multicentric in lymphangiomatosis. It is referred to as diffuse pulmonary lymphangiomatosis when the thoracic viscera including lung， mediastinum and pleura are involved. Usually， diffuse pulmonary lymphangiomatosis has poor  prognosis. Recently， the understanding of the mechanisms of diffuse pulmonary lymphangiomatosis has been impoved by the breakthough of genetics as well as cellular?signaling pathway research. What’s more， the clinical application of targeted drug and their significant effects have brought a new hope to the treatment of diffuse pulmonary lymphangiomatosis. This article mainly introduces the pathogenesis， clinical manifestations， diagnostic methods and treatment progress of diffuse pulmonary lymphangiomatosis in children.

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Treatment for pediatric lung transplantation YUE Bing-qing, CHEN Jing-yu 2023, 38(3): 215-217.  DOI: 10.19538/j.ek2023030611 Abstract ( )   Pediatric lung transplantation is an effective treatment for children with end-stage lung diseases. The indications include end-stage pulmonary vascular disease， bronchiolitis obliterans， interstitial lung disease， cystic fibrosis and so on. In recent years， with the development and maturity of lung transplantation technology， the number of pediatric lung transplantation has also increased year by year. This article summarizes the current status of pediatric lung transplantation in the treatment of different lung diseases in children in order to provide a reference for the lung transplantation evaluation， diagnosis and treatment of children with end-stage lung diseases.

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Gene and clinical follow-up analysis of 30 patients with congenital adrenal hyperplasia by neonatal screening GONG Li-fei, YANG Nan, ZHAO Jin-qi, et al 2023, 38(3): 218-223.  DOI: 10.19538/j.ek2023030612 Abstract ( )   Objective　To analyze the clinical and gene mutation characteristics of patients with congenital adrenal hyperplasia （CAH） confirmed by neonated screening to understand the effect of early diagnosis and treatment. Methods　The clinical data of 30 patients with CAH born from August 2016 to April 2022 who were diagnosed by neonatal screening were retrospectively analyzed. Results　（1） Among the 30 cases （19 males and 11 females） there were 24 （80%） patients with salt-wasting type， 5 （16.67%） simple-virilizing type， and 1 non-classical type; 2 patients had related family history.（2） The median age at screening was 4 （3， 6.25） days， and the median age at diagnosis was 19 （13.75， 27） days. The median age at the last follow-up was 2.39 （1.10， 3.43） years， and the average dose of hydrocortisone（HC） was 10.15 （8.20， 11.37） mg/（㎡·d）. Growth and development were all within the normal range for children of the same gender and age. （3） A total of 16 mutations were found in 28 patients undergoing genetic testing. It was concluded that c.293-13C/A>G was the hot spot mutation of CAH in this study. Conclusion　Early screening， diagnosis and treatment can help to detect CAH early， thus improving growth， development and prognosis. Identifying the type of mutation facilitates genetic counseling.

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Clinical researh of hypophosphatemia in diabetic ketoacidosis children with type 1 diabetes mellitus PENG Xuan-dong, MEI Hui-fen, SU Ling, et al 2023, 38(3): 224-227.  DOI: 10.19538/j.ek2023030613 Abstract ( )   Objective　To summarize the clinical data of diabetic ketoacidosis （DKA） in children with type 1 diabetes complicated with hypophosphatemia， and to explore the incidence and prognosis of hypophosphatemia during DKA treatment. Methods　A total of 133 children with type 1 diabetes complicated with DKA admitted to the Department of Genetics and Endocrinology of Guangzhou Women and Children's Medical Center from January 2016 to June 2020 were analyzed retrospectively. The serum phosphorus levels were detected 4 to 24 hours after admission， and the related factors affecting the changes of blood phosphorus in DKA children were analyzed. Results　Among 133 children with DKA， 55 （41%） were male and 78 （59%） were female. The age ranged from 6 months to 15 years， with an average age of （6.7 ± 3.9） years. The newly diagnosed type 1 diabetes accounted for 72%. Among the children， 75 cases （56%） had hypophosphatemia during DKA， of which 41（31%） had 0.81~1.29 mmol/L， 22（16%） had 0.61～0.81mmol/L of blood， 10 （7.5%） had 0.38～0.61mmol/L phosphorus， and 2 （1.5%） had blood phosphorus < 0.38mmol/L. There were significant differences in pH， HCO3-， BE-B， blood potassium and blood chlorine between normal and low blood phosphorus groups. The acidosis and age≤5 years old were the risk factors for the severity of low blood phosphorus in children with DKA. None of the 75 children with hypophosphatemia had clinical symptoms related to hypophosphatemia and were not treated with phosphate. The 34 children with blood phosphorus<0.81mmol/L had normal blood phosphorus after 72 hours. Conclusion　Hypophosphatemia are prone to occur during DKA treatment. For DKA children with mild hypophosphatemia， routine phosphate suppleme-ntation is not necessary. Mild hypophosphatemia during DKA treatment does not cause serious clinical consequences， so it is recommended to monitor blood phosphorus only in children with severe hypophosphatemia.

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Research progress in immune function of protein tyrosine phosphatase receptor-type C and its related diseases LI Yu-lu, MAO Hua-wei 2023, 38(3): 228-234.  DOI: 10.19538/j.ek2023030614 Abstract ( )   Protein tyrosine phosphatase receptor type C（PTPRC），also known as CD45，is a transmembrane glycoprotein exclusively expressed on all nucleated cells of the hematopoietic system.PTPRC exists in multiple isoforms generated by alternative splicing of exons.The isoform expression varies depending on the developmental or functional status of the cell. PTPRC has rich physiological functions.It plays an important role in T cell and B cell antigen receptor signal transduction， participates in T and B cell development，and regulates the production of cytokines through JAK kinase.Moreover，PTPRC is involved in the pathogenesis of infectious diseases，autoimmune diseases，severe combined immunodeficiency and cancer.

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Inhalation of amikacin in the treatment of CF children with recurrent respiratory Pseudomonas aeruginosa infections：A case report ZHANG Dong-wei, FAN Hui-feng, ZHOU Gan, et al 2023, 38(3): 235-237.  DOI: 10.19538/j.ek2023030615 Abstract ( )   Cystic fibrosis （CF） is a rare disease caused by a mutation of the gene encoding the cystic fibrosis transmembrane conductance regulator （CFTR）. One case of repeated severe pulmonary infection in children with CF of Han nationality was reported in this paper. The main pathogen of the respiratory tract was Pseudomonas aeruginosa. After the child developed bronchiectasis, the main treatment options during hospitalization were intravenous antibiotics and bronchoalveolar lavage. At follow-up, the main treatment options were inhaled amikacin and oral n-acetylcysteine. After treatment, the patient has a good prognosis.

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Pseudohypoparathyroidis with heterotopic ossification of skin as first symptom: One case report XIE Liu-lu, LI Yan-hong, ZHENG Ru-jiang, et al 2023, 38(3): 238-240.  DOI: 10.19538/j.ek2023030616 Abstract ( )   To report a case of the pseudohypoparathyroidism with the initial manifestation of subcutaneous heterotopic ossification and to explore the clinical characteristics and to investigate the correlation between clinical phenotypes and genotypes.The boy was admitted due to subcutaneous hard nodules.The dermal biopsy showed the subcutaneous heterotopic ossification. GNAS1 analysis revealed a heterozygous mutation: c.140-2A>G, inherited from his mother. PHP Ia can be diagnosed according to heterotopic ossification.This case enriches the data on this rare disease.