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06 August 2022, Volume 37 Issue 8 Previous Issue    Next Issue

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Expert consensus on Chinese pediatric health examination Society of Pediatrics of Chinese Medical Doctor Association, Expert Consensus Group on Chinese Pediatric Health Examination, Editorial Committee of Chinese Journal of Practical Pediatrics 2022, 37(8): 561-566.  DOI: 10.19538/j.ek2022080601 Abstract ( )   Physical examination is one of the basic and effective ways to implement early disease prevention and health management. Regular health examinations can effectively monitor children's growth and development level and health status at all stages， so that health risk problems in children's growth can be found， intervened， diagnosed， and treated early.The Pediatric Society of Chinese Medical Doctor Association， the Expert Consensus Group on Chinese Pediatric Health Examination，and the Editorial Board of the Chinese Journal of Practical Pediatrics jointly wrote this expert consensus，which is aimed to further guide and standardize children's health evaluation and health risk screening，improve children's health levels and reduce the disease burden of families and society in China.

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Expert consensus on expectorant and relieving cough in children Group of Respiratory Diseases, the Society of Pediatrics, Chinese Medical Doctor Association, Collaborative Group of Chronic Cough, the Subspecialty Group of Respiratory Diseases, the Society of Pediatrics, Chinese Medical Association, Editorial Committee of Chinese Journal of Practical Pediatrics 2022, 37(8): 567-574.  DOI: 10.19538/j.ek2022080602 Abstract ( )   Cough and expectoration are the most common symptoms of respiratory diseases in children. The main etiology of acute cough is respiratory tract infection. Common etiologies of chronic wet cough include upper airway cough syndrome，asthma with infection，and protracted bacterial bronchitis. Hypersecretion of mucus in the airway and dysfunction of mucociliary clearance system are the main mechanisms of cough sputum production in children. Clinical expectorant and cough suppressant drugs include mucolytics，mucokinetics，mucoregulators， expectorants，and traditional Chinese medicine and Chinese patent medicine. Different types of drugs can have multiple effects. The treatment for cough in children can be determined according to the specific etiology， severity and symptom improvement of cough.

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Etiology and classification of genetic skeletal disorders WU Wei, LIANG Yan 2022, 37(8): 575-579.  DOI: 10.19538/j.ek2022080603 Abstract ( )   Genetic skeletal disorders（GSD） are genetic diseases caused by the alteration of genetic materials. Genetic skeletal disorders are relatively rare， but often lead to high rate of deformity and disability，which affects physical and mental health of adolescents. In recent years， with the development molecular biolagical detection techniques，many underlying pathogenic genes of GSD have been discovered. This article focuses on the etiology and classification of genetic skeletal disorders， hoping to provide help for the clinical diagnosis， treatment and scientific research of GSD in China.

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Clinical applications of body proportion in children ZHU Gao-hui, ZHU Min 2022, 37(8): 579-583.  DOI: 10.19538/j.ek2022080604 Abstract ( )   The evaluations of body proportion are meaningful in the physical growth of children and adolescents. It will help pediatricians to find the deviation of physical growth from healthy children in time to understand the development pattern of body proportion correctly and measure and evaluate the body proportion comprehensively，which is of great significance to the diagnosis and etiological analysis of growth disorder in children.

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Progress of classification，diagnosis and treatment of metaphyseal dysplasia FAN Xin, PENG Yan, LI Chuan, et al 2022, 37(8): 583-588.  DOI: 10.19538/j.ek2022080605 Abstract ( )   Metaphyseal dysplasia is a group of rare genetic skeletal dysplasia，which is characterized by abnromal metaphyseal morphology，with or without other skeletal malformations and associated abnormalities. According to the latest classification of genetic bone diseases，ten types of epiphyseal changes are classified as metaphyseal dysplasia. These diseases have big phenotypic heterogeneity and overlap with the phenotypes of a variety of genetic bone diseases. Clinically，it needs to be diagnosed based on typical imaging features，clinical phenotypes and  genetic detection. At present， there is no specific treatment for this kind of disease. Targeted therapeutic drugs based on pathogenesis-related signaling pathways are being studied. The effectiveness and safety of growth hormone for the improvement of patients' height still need to be further studied.

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Changes in guidelines and recommendations for multidis-ciplinary diagnosis and treatment of achondroplasia WANG Rui-fang, YU Yong-guo 2022, 37(8): 588-595.  DOI: 10.19538/j.ek2022080606 Abstract ( )   Achondroplasia is the most common skeletal dysplasia，which is characterized by short limbs，macrocephaly，midface hypoplasia，and trident configuration of the hands. Potential complications associated with achondroplasia include longbone arches of lower extremity， middle ear dysfunction， obstructive sleep apnea，and，more rarely，cervical cord compression，hydrocephalus，and thoracolumbar kyphosis. The management of achondroplasia is multifaceted and requires the involvement of multiple disciplines throughout the life course. This article focuses on the guidelines or consensus on the treatment and care of patients with achondroplasia published in recent years at home and abroad. The aim is to provide guidance for healthcare providers to help identify patients with achondroplasia who are at high risk for severe sequelae，and to carry out intervention before complications arise.

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Diagnosis and treatment of osteoporosis in children WANG Li, SU Zhe, JIAO Yan-hua 2022, 37(8): 595-600.  DOI: 10.19538/j.ek2022080607 Abstract ( )   More and more attention has been paid to osteoporosis in children caused by primary or secondary causes. Because children are still in the growth stage， the diagnosis，treatment and follow-up strategies of osteoporosis in children cannot completely follow the adult standards. Early identification and correct diagnosis of different types of osteoporosis in children，proper treatment and monitoring of adverse drug reaction and continuous health management are of great significance to improving patients' quality of life and long-term bone health. There is still a lot to explore in the detection methods and drug therapy of osteoporosis in children.

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Application of bone turnover markers in pediatric endocrine diseases CAO Bing-yan, GONG Chun-xiu 2022, 37(8): 601-606.  DOI: 10.19538/j.ek2022080608 Abstract ( )   Bone turnover markers （BTMs）are metabolites or enzymes produced during bone remodeling，usually considered as either bone formation or bone resorption markers. In children，BTMs are closely related to gender， age and pubertal status. Higher BTMs maybe associated with osteoporosis in children at high risk for primary or secondary osteoporosis. BTMs can be helpful to monitor treatment effects of drugs that have an effect on bone metabolism. The levels of BTMs increase among the children with secondary hyperparathy-roidism of metabolic bone disease，also increase in obese and pre-diabetic children. Nowadays，BTMs are mainly used as reasearch tools，far from clinical applicaticn because of the inconsistent result. In the future，more studies are needed to explore the clinical application of these markers.

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Genetic hypoparathyroidism in children WU Wei, FU Jun-fen 2022, 37(8): 606-613.  DOI: 10.19538/j.ek2022080609 Abstract ( )   Decreased parathyroid hormone （PTH） secretion or hormone resistance can both lead to symptoms of PTH function defect. The former is called hypoparathyroidism （HP） and the latter is called pseudohypoparathyroidism （PHP），both being characterized by hypocalcaemia and commonly accompanied with hyperphosphatemia. HP is classified as acquired and genetic，and adult HP is practically acquired. Childhood HP is rare，and more commonly caused by various genetic variations. PHP is a rare genetic disease mostly caused by molecular alterations of the GNAS gene，has various types with different clinical manifestations. The diagnosis is based on clinical manifestations and genetic testing. Calcium supplementation and activated vitamin D are required for hypocalcemia in the treatment of HP and PHP，and nephrocalcinosis needs to be monitored during treatment. PHP also needs to manage other hormone resistance and symptoms of AHO，and corresponding follow-up and treatment should be conducted.

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Skeletal changes in lysosomal storage diseases ZHANG Yao, XIONG Hui 2022, 37(8): 613-618.  DOI: 10.19538/j.ek2022080610 Abstract ( )   Inherited metabolic disorders（IMD） are a large class of monogenic genetic diseases，characterized by functional defects of enzymes，coenzymes or transporters in biochemical metabolic pathways. Nearly 1500 kinds of IMD have been named. Lysosomal storage diseases are a large group of IMD characterized by multisystem damage，which is caused by the genetic variation of various enzymes，enzyme activators or lysosomal membrane proteins in lysosomes. A variety of lysosomal storage diseases represented by mucopolysaccharide storage disease can appear characteristic multiple bone metabolic disorders. Some lysosomal storage diseases characterized by bone changes are introduced briefly in this paper.

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Clinical analysis of children with anti -myelin oligodendrocyte glycoprotein antibody-associated disorders complicated with seizures ZHENG Ping, ZHANG Han-zi, SUN Jing, et al 2022, 37(8): 619-624.  DOI: 10.19538/j.ek2022080611 Abstract ( )   Objective    To explore the features of children with anti-myelin oligodendrocyte glycoprotein autibody-associated disorders（MOGAD） complicated with seizures. Methods    Clinical data were obtained from 48 children with MOGAD treated in Capital Institute of Pediatrics from January 2017 to June 2021. The features of the seizures were analyzed. The different clinical characteristics between children with seizures and those without seizures were evaluated. Results    In this case series，thirteen children （27.1%） with MOGAD suffered from seizures and 35 children （72.9%） didn’t. There were no significant differences in the age of onset or the ratio of sex between children with and without seizures （t=-1.76，P=0.09；χ2=1.33，P=0.25）. Thirteen patients had 18 episodes with seizures. A status epilepticus was found in 10/18 episodes （55.6%）. Among the 18 episodes，7 were acute disseminated encephalomyelitis，4 were cortical encephalitis，4 were undefined phenotype and 3 were isolated epilepsy. There was a higher proportion of cortical lesions in children with seizures as compared to those without seizures （χ2=7.27， P=0.01）. Less involvement of the spinal cord and cerebellum was found in children suffering from seizures（χ2=4.85，P=0.03；χ2=4.31，P=0.04）. Relapses of neurological symptoms occurred in 8 of 13 （61.5%） children with seizures， and in 8 of 35 （22.9%） children without seizures（χ2=4.76，P=0.03），and the number of relapse had significant difference between the two groups（Z=-2.43，P=0.02）. All patients were sensitive to immunotherapy， and most of them remained seizure free. Conclusion    Seizures are the clinical manifestation of MOGAD children，and half of them have status epilepticus. They are sensitive to immunotherapy and most seizures have good prognosis. Relapses mostly occur in children with MOG associated seizures.

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Efficacy and safety of cyclosporine A versus tacrolimus in children with refractory nephrotic syndrome SUN Lei, KUANG Xin-yu, KANG Yu-lin, et al 2022, 37(8): 625-631.  DOI: 10.19538/j.ek2022080612 Abstract ( )   Objective    To evaluate the efficacy and safety of cyclosporine A（CsA）and tacrolimus（TAC）in children with refractory primary nephrotic syndrome（PNS）. Methods    One hundred and eighteen children with refractory PNS， who were treated with TAC or CsA from June 2012 to June 2018，were included in the study. Patient’s clinical and pathological information were gathered for retrospective analysis，including general informatioin，laboratory tests，renal pathology，prognosis and side effects. Kaplan-Meier was used to draw survival curves and comparative analysis was made. Results    In the CsA group（66 cases），patients with frequent relapses（FRNS），steroid dependency （SDNS），or resistance to steroid therapy（SRNS）were 3，45 and 18，respectively. In the TAC group（52 cases），patients with SDNS or SRNS were 40 and 12，respectively. Fifty-six of the 66 patients（84.8%）in the CsA group and 47 of the 52 patients （90.4%） in the TAC group received percutaneous renal biopsy. There was no difference between the two groups in the long-term complete remission of NS. At the end of 6 months of therapy，complete remission was seen in 68.2%（45/66）and 86.5% （45/52）of refractory PNS patients treated with CsA and TAC，respectively（P?< 0.05）. The efficacy of CsA and TAC in FRNS and SDNS was significant difference，but there was no significantly different in SRNS. There was no difference in the effects between CsA and TAC in the minimal change diseace（MCD） group and non-MCD group. The side effects of gum hyperplasia and hypertrichosis were significantly less in TAC group as compared to CsA group（P< 0.01）. But two patients had acute pancreatitis after 16 and 18 months of treatment with TAC. The incidence rate of acute kidney injury（5/66）and chronic nephrotoxicity（2/66）in CsA group was higher than that in TAC group. Conclusion    In the long term，the efficacy of CsA and TAC in refractory PNS has no significant difference. But the treatment for FRNS and SDNS with TAC is associated with higher efficacy in comparison with CsA at 6 months of therapy，but it is necessary to be alert to the presence of acute pancreatitis. The patient with CsA treatment over 30 months should be alert to the CNI nephrotoxicity.

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Diagnosis and treatment progress in eosinophilic gasrointestinal disorders in children ZHANG Tian-he, MAO Zhi-qin 2022, 37(8): 632-636.  DOI: 10.19538/j.ek2022080613 Abstract ( )

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One case of chronic recurrent multifocal osteomyelitis in children ZHANG Zi-bo, TIAN Zhi-gang, LIU Li, et al 2022, 37(8): 637-640.  DOI: 10.19538/j.ek2022080614 Abstract ( )