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Progress of PedsQLTM in cerebral palsy children

International Journal of Pediatrics.2010;37(5):527-530. doi:10.3760/cma.j.issn.1673-4408.2010.05.028

More recently, the Pediatric Quality of Life Inventory Measurement Model (PedsQLTM)is wildly applicated in the research of pediatric quality of life as a pediatric population health measure. Considerable emphasis has been placed on the measurement of the quality of life of children with cerebral palsy, and many scholars carried on the investigations using the PedsQLTM abroad, while there was no related literature reported presently in China. This paper summarizes the basic structure and scoring method of PedsQLTM, and synthetically sums up the determinations of reliability, validity, and sensitivity of PedsQLTM in cerebral palsy children, and gives an overview of the effects on assessment of clinical efficacy and making a medical decision. The PedsQLTM has some superiority in measuring the quality of life in cerebral palsy children.

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The reseach advancement of manufacture and identfication of animal models of cerebral palsy

Jielu TAN

International Journal of Pediatrics.2010;37(5):494-496. doi:10.3760/cma.j.issn.1673-4408.2010.05.017

Cerebral palsy(CP)is one of the main diseases which induce the invalids of children nervous system.The pathogenesis of CP iS fairly complex,which correlates with many factors.At present,there is no one kind of animal odel of CP that can be received by the public.To solve this problem,this review summarizes the most up-to-date three models which relate to the pathogenesis,ischemia and hypoxia model,infection model and hyperbilirubin model,and compares their different validities,eventually brings forward the common effective criterions to identiticare different models.Among these three models,the ischemia and hypoxia model is the most popular application and research objection,the two others are sprouting on their way waiting further develpoment.

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Current situation, clinical characteristics and management of human swine influenza A H1N1) in children

Zhaoyuan QIN ; Lizhi CHEN ; Ling ZHANG

International Journal of Pediatrics.2010;37(5):462-465. doi:10.3760/cma.j.issn.1673-4408.2010.05.007

Human swine influenza A (H1N1) is a highly transmissible infectious disease, which has spreaded globally and represented a continuous pandemic threat. The novel virus has predominantly affected the children and young adults. Clinical manifestations generally appear mild, but there are still many patients with severe complications leading to hospitalization. According to the current reports, the mortality in the early stages of the pandemic appears no more than seasonal influenza A . Children (especially less than 5years) are considered to be at higher risk of infection and complications. Pediatric patients with a underlying significant chronic disease such as chronic respiratory disease,cardiovascular disease and immunodeficiency disease, are at a higher risk of death. The neuraminidase inhibitors Oseltamivir and Zanamivir are effective for prophylaxis and treatment. Effective vaccines are regarded to be crucial for the control of influenza pandemics. This review focuses on the epidemiological situation, clinical characteristics and management of human swine influenza A (H1N1), so as to provide practical advice for clinicians.

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CFI gene and atypical hemolytic uremic syndrome

Jiamei TIAN

International Journal of Pediatrics.2010;37(5):534-537. doi:10.3760/cma.j.issn.1673-4408.2010.05.030

Mutations in CFI, mapped to 4q25 and encoding complement factor Ⅰ, are responsible for autosomal recessive atypical hemolytic uremic syndrome aHUS). Complement factor Ⅰ, a serine proteinase, plays a key role in inhibition of the amplification loop in alternative pathway. Most of mutations in the CFI gene identified in patients with aHUS are point mutations without large deletion ones. The patients with aHUS with CFI mutations, from 2 months to 32 years of age at disease onset, have a poor outcome. Approximate 70% of the patients have low levels of complement 3, and 69.6% of the cases progress to end-stage renal disease within 3 years. The risk of post-transplantation recurrences in the CFI-mutated patients is high.Therefore,detection of the CFI gene in the patients suffering from aHUS will be beneficial to making therapeutic decisions and predicting prognoses.

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Progresses of diagnosis and therapy in multiple acyl-CoA dehydrogenase deficiency

Yazhi XING

International Journal of Pediatrics.2010;37(5):518-521. doi:10.3760/cma.j.issn.1673-4408.2010.05.025

Multiple acyl-CoA dehydrogenase deficiency (MADD) is a common inborn error of fatty-acid metabolism characterized by vomiting, acidosis and lipid storage myopathy, and the clinical manifestations of MADD are highly heterogeneous. MADD can be diagnosed by the elevation of multiple acyl-carnitine in blood and glutaric acid or other organic acid in urine. The neonatal-onset patients have severe symptoms and poor prognosis. However, oral riboflavin supplementation (can completely rescue) ameliorate the clinical and laboratory disorders rapidly especially to the riboflavin responsive MADD. Additionally, patients not sensitive to riboflavin should also take low lipid, low protein and high carbonhydrate diet besides riboflavin.

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Study of fetus and infant's hematological diseases due to vertical transmission of human cytomegalovirus

Saijun GAO

International Journal of Pediatrics.2010;37(5):512-514. doi:10.3760/cma.j.issn.1673-4408.2010.05.023

Human cytomegalovirus (HCMV) is one of the most important pathogens of congenital infection. HCMV of primary or secondary infection can be transferred to newborns. Haematological disorders may occur in some cases. This paper will summarize the mechanism of hematological disorder due to vertical transmission of HCMV, and also focus on diagnostic strategies, risk factors and intervention measures.

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SLC11A1 gene mechanism and its relationship with susceptibility to tuberculosis

Xirong WU

International Journal of Pediatrics.2010;37(5):499-502. doi:10.3760/cma.j.issn.1673-4408.2010.05.019

Tuberculosis is the leading cause of death due to infection from a single microbial agent. With indepth study of the pathogenesis of tuberculosis,it was found that solute carrier family 11 member I gene SLC11A1) was ignificantly associated with susceptibility to tuberculosis. SLC11A1 is located in phagocytic lysosome membrane,influencing the occurrence and development of tuberculosis by changing the intracellular environment. In this paper, the mechanism of SLC11A1 gene and its relationwith susceptibility to tuberculosis are reviewed.

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New clinical features of Fanconi anemia

Yanke YIN

International Journal of Pediatrics.2010;37(5):490-493. doi:10.3760/cma.j.issn.1673-4408.2010.05.016

Fanconi anemia performs (FA) great clinical heterogeneity. Although the identification of FA related genes extends the recognition of FA pathogenesis, few stringent genotype-phenotype connections have emerged. For the same FA genotype patients, it seems that type of mutation influences clinical phenotype more importantly, Modifier genes,environmental factor, can also result in obviously different clinical phenotype. Different from multiple genotypes and multiple clinical phenotypes, FA cellular phenotype is characteristic, and is the most important basis of diagnosis.

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The role of vascular endothelial growth factor in lung development

Juanmei WANG

International Journal of Pediatrics.2010;37(5):455-457. doi:10.3760/cma.j.issn.1673-4408.2010.05.005

Lung development is affected by many factors, and vascular endothelial growth factor is a specially growth factor which has a broad impact on endothelial cell function, it can promote the proliferation and differentiation of cells,as well as promoting angiogenesis and increasing permeability of micro vascular. Further more, it participates in pulmonary vascular development, alveolarization, surfactant synthesis and lung maturity. Thus vascular endothelial growth factor plays an important role in the establishment of normal lung morphology and function, and its abnormal expression may induce abnormal lung development.

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Progress in GJB2 gene and its diagnostic screening technique

Liangliang SONG

International Journal of Pediatrics.2010;37(5):545-547. doi:10.3760/cma.j.issn.1673-4408.2010.05.034

Deafness gene GJB2 is located in 13q11-q12,which codes for the protein connexin 26(CX26). Up to 50% of autosomal recessive nonsyndromic hearing loss are accounted for by mutations in the GJB2 gene. However, in different ethnic groups, the mutational sites of GJB2 gene are also different. For examples, 35delG is the most common mutation in Europe and USA. In Ashkenazi Jews, 167delT is most common. While among the Asians,235delC is makes up a large proportion of the GJB2 gene mutation. Because of the special status of GJB2 gene in hereditary hearing loss, its diagnostic screening technique is especially important. On the basis of newborn hearing screening, it is suggested that we should be to blend into the deaf disease gene screening at the molecular level, because they play an important role in early detection and intervention on congenital hearing loss.

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