This article analyzes the status quo of sales supervision on medical devices through some aspects, including the relevant regulation system, the standards of sales admittance, the supervision team and the approval of business license. According to the exiting problems, some improving countermeasures are proposed for reference.
Materials Management, Hospital ; organization & administration

This paper focuses on issues needed to be clear towards the product quality supervision of medical devices in use. The life circle of medical devices, the supervision regarding its boundary, target, emphasis, basis and standards have been analyzed in turn. A clear and creative idea is also provided for theoretical exploration of the supervision on medical devices in use.
Materials Management, Hospital ; Quality Assurance, Health Care

Actins ; metabolism ; Adult ; Breast Neoplasms ; metabolism ; pathology ; surgery ; Calcium-Binding Proteins ; metabolism ; Cell Differentiation ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Fibroadenoma ; metabolism ; pathology ; surgery ; Hamartoma ; pathology ; Humans ; Hyperplasia ; Leiomyoma ; pathology ; Microfilament Proteins ; metabolism ; Muscle, Smooth ; pathology ; Phyllodes Tumor ; pathology

Adenocarcinoma ; metabolism ; pathology ; surgery ; Cardia ; Esophageal Neoplasms ; metabolism ; pathology ; surgery ; Esophagogastric Junction ; metabolism ; pathology ; surgery ; Humans ; Neoplasm Staging ; Receptor, ErbB-2 ; metabolism ; Sirtuin 1 ; metabolism ; Stomach Neoplasms ; metabolism ; pathology ; surgery ; Survival Rate

The influencing factors on cord blood storage after collection and mononuclear cell separation as well as cryopreservation were studied. The mononuclear cell are separated from blood after blood collection, then cryopreserved and washed after thawed. Results showed that the cord blood kept at 4 degrees C or room temperature less than 24 hours after blood collection, mononuclear cell separated by hydroxyethylstarch and 2 centrifugations, mononuclear cell cryopreserved with 50% DMSO and autoplasma from cord blood as protectives and washing the cells after thawing. In conclusion, the optimal project in this study can effectively preserve cord blood mononuclear cells.
Blood Preservation ; Cell Separation ; methods ; Cryopreservation ; Fetal Blood ; cytology ; Humans ; Leukocytes, Mononuclear ; physiology

OBJECTIVESyncope is a common problem in children and adolescents. Such an event may have multiple possible causes, ranging from benign conditions to life-threatening diseases. Syncope is a major challenge for the practicing physicians. It is very important to know the etiologic and clinical characteristics of syncope in children. This study aimed to improve diagnostic efficacy of syncope in children by analyzing the etiology and clinical characteristics of syncope.

METHODSThe investigators retrospectively analyzed the causes of syncope and diagnostic workup of 154 consecutive children seen in Department of Pediatrics, Peking University First Hospital because of a syncopal event.

RESULTSAutonomic-mediated reflex syncope (AMS) was the most common cause of syncope (65.6%), whereas cardiac disorders were found in 10 cases (6.5%) comprising the second cause of syncope in children. Other causes included psychologic problems and neurological and metabolic disorders. Although many causes were studied, 25 cases (16.2%) were found to have uncertain etiologies yet. The children with AMS were commonly seen in pubertal girls, and they had clear inducement of syncope and prodromes. The children with cardiac syncope often had history of cardiac diseases, and they were often younger than those with AMS. Lack of prodromes of syncope, exercise-related syncope, syncope spells seen in any body position, frequent syncope spells and sudden death in family were clues of cardiac syncope. Neurological disorders should be considered if there are any of the followings: syncope with seizure activity, syncope spells seen in any position, and a postictal phase of disorientation or neurologic abnormal signs. A metabolic cause was entertained when the child had a history of metabolic diseases, prolonged anger, or violent vomiting and diarrhea. Children with psychiatric disorders were adolescent girls with prolonged syncope spells, and had more frequent syncopal episodes. Most children with syncope were evaluated by many of diagnostic tests, but most of those tests were not goal-directed approach. Since persons with cardiac syncope were at increased risk for death from any cause, electrocardiography was recommended in almost all children with syncope. Neurologic testing including electroencephalography, computed tomography, etc. were rarely helpful unless neurologic signs and symptoms are present. Holter electrocardiography and echocardiography were most useful in children with suspected cardiac syncope. There was little benefit of screening cardiac enzyme in children with syncope. Routine blood tests (blood electrolytes and blood glucose, etc) rarely yield diagnostically useful information unless the children had the history of metabolic diseases. Head-up tilt testing was most useful in children with recurrent syncope in whom heart disease was not suspected. The children with frequent syncope, long lasting syncopal episode and clear psychiatric inducement of syncope should be evaluated by psychiatric testing.

CONCLUSIONSyncope in children may result from a wide variety of causes, and clinicians often use a wide range of investigation to try to achieve a diagnosis. But most of investigations have low diagnostic yield. Thorough history taking, physical examination and electrocardiography are the core of the syncope workup.

Adolescent ; Child ; Child, Preschool ; Electrocardiography ; Female ; Humans ; Male ; Retrospective Studies ; Syncope ; diagnosis ; etiology

Objective:To identify Down syndrome (DS) fetal encephalopathy related genes and signaling pathways via bioinformatics analysis, and to explore their potential mechanisms underlying the occurrence and development of DS neuropathology.Methods:Retrospective study.In December 2021, dataset GSE59630 was downloaded from the gene expression omnibus (GEO), and differentially expressed genes (DEGs) between DS and normal fetal brain tissue were identified by R software.Gene Ontology (GO), Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analysis and gene set enrichment analysis (GSEA) were performed on the genes identified.The protein-protein interaction (PPI) network was constructed based on search tool for the retrieval of interacting genes online database and Cytoscape software, and key modules and hub DEGs were identified.Real-time quantitative polymerase chain reaction technique was used to verify the expression of hub genes related to neurodegeneration in brain tissue of 3 pairs of DS and normal fetuses at the gestational age of 22-33 weeks.Results:A total of 225 DEGs were screened out from DS and normal fetal brain tissue, including 18 up-regulated genes and 207 down-regulated genes.GO functional enrichment analysis showed that DEGs were mainly enriched in neurogenesis, neuronal apoptosis, transcriptional regulation, mitochondrial energy metabolism, etc.KEGG pathway enrichment analysis revealed that DEGs were associated with a variety of neurodegenerative diseases.GSEA suggested that apoptosis and inflammatory responses play a vital part in the occurrence of DS neuropathology.Ten hub genes were identified by the PPI network established, and they were mainly related to histone acetylation and transcriptional regulation.According to the tissue verification result, the variations of RAB8A, TBP and TAF6 expression conformed to the microarray data. Conclusions:The key genes and signaling pathways identified by transcriptome analysis of fetal brain tissue facilitate the comprehensive understanding of the molecular mechanism of DS neuropathology.This study provides a novel insight into the clinical diagnosis and treatment of neurodevelopmental abnormalities and mental retardation in DS.

This paper introduces the sampling scheme and the inspection scheme paradigm followed in organizing the sampling inspection of medical devices. The sampling scheme paradigm includes the definition and operation guidelines for sampling products, the regulations for individual sampling batches and related model specifications, accessory data, validity period, storage conditions, etc., the requirements for sampling areas, places and quantities, and the requirements for samples to be sent to inspection institutions. The inspection scheme paradigm includes the provisions for inspection basis, inspection items and determination principles.
Equipment Safety ; methods ; Guidelines as Topic

OBJECTIVEMethylmalonic aciduria (MMA) is a common one of the congenital disorders of organic acids metabolism. Some of the patients with the disorder are complicated with homocysteinemia. Recently, gas chromatography-mass spectrometry (GCMS) has been used to diagnose MMA in China. However, the diagnosis of the patients with combined MMA and homocysteinemia is often delayed. In this study, the natural history, clinical features and outcome of 57 Chinese patients with combined MMA and homocysteinemia were investigated.

METHODSFrom 1996 to 2006, 96 MMA patients from 16 provinces or cities were diagnosed in our hospital by urine organic acids analysis using GCMS. Homocysteinemia was found by serum and urine total homocysteine determination using a fluorescence polarization immunoassay.

RESULTSFifty-seven of the 96 MMA patients (59.4%, 32 males and 25 females) were found to have combined MMA and homocysteinemia. They had markedly increased urine methylmalonic acid, total serum homocysteine (81.5 to 226.5 micromol/L vs. normal range 4.5 to 12.4 micromol/L) and urine homocysteine (79.1 to 414.5 micromol/L vs. normal range 1.0 to 25.0 micromol/L). Thirteen (22.8%) of them presented with symptoms resembled hypoxic-ischemic encephalopathy in the neonatal period. Fourteen (24.6%) patients had the onset at the age of one month to 1 year with mental retardation, vomiting and epilepsy. Nine (15.8%) showed developmental delay, seizures, poor appetite or anemia from the age of 1 to 3 years. Eighteen (31.6%) had psycho-motor degeneration at the age of 6 to 15 years. Among them, 7 patients experienced multiple organ dysfunctions with liver dysfunction, hematuria, renal failure and peripheral neuropathy. Three (5.3%) patients developed progressive mental degeneration, motor disorders and anorexia at the ages of 16, 24 and 34 years. Eleven (19.3%) patients ultimately died; 5 (8.8%) of them were diagnosed postmortem. Forty-six (80.7%) patients were treated with vitamin B12, folic acid, L-carnitine and betaine supplementation and 11 (19.3%) of them recovered completely.

CONCLUSIONSCombined MMA with homocysteinemia is a common form of MMA in China. The clinical spectrum of the patients varies from severe neonatal-onset forms with high mortality to milder forms with adult-onset. Serum or urine total homocysteine analysis is important for the deferential diagnosis of the patients with MMA.

Adolescent ; Adult ; Amino Acid Metabolism, Inborn Errors ; complications ; Anemia ; complications ; metabolism ; Carnitine ; metabolism ; Child ; Child, Preschool ; China ; epidemiology ; Female ; Gas Chromatography-Mass Spectrometry ; Homocysteine ; blood ; Humans ; Male ; Metabolic Diseases ; blood ; complications ; metabolism ; Methylmalonic Acid ; urine ; Urologic Diseases ; complications ; metabolism ; Vitamin B 12 ; pharmacology ; Vitamin B Complex ; pharmacology ; Young Adult

OBJECTIVEPelizaeus-Merzbacher disease (PMD) is a rare X-linked recessive leukoencephalopathy. Few reports of PMD patients without genetic confirmation have been published in the mainland of China. The clinical and genetic features of a family with PMD were analyzed, which may contribute to definite diagnosis, genetic counseling and prenatal diagnosis of this rare hereditary disease in China.

METHODSClinical data of the proband and other family members as well as 14 DNA samples were collected. Clinical features including symptoms, signs and cranial MRI were analyzed. Multiplex ligation-dependent probe amplification (MLPA) assays were performed to detect PLP1 duplication, which helps identify the type of PLP1 mutation in this family and the genotype-phenotype correlations.

RESULTS(1) The proband and the other 3 male patients in the family presented with nystagmus, motor retardation followed by regression. The cranial MRI of proband showed evidence of poor myelination with diffused high signal in white matter region on T2-weighed image and reduced amount of white matter in volume, which is consistent with the typical features of cranial MRI in PMD. (2) PLP1duplication was identified in the proband. Combined with the clinical features of the proband and other patients in this family, the diagnosis of classic form of PMD was confirmed. Another 3 females with normal phenotype in the family were proved to be carriers of PLP1duplication.

CONCLUSIONS(1) The Classic form of PMD in this pedigree is resulted from the PLP1 duplication, which is consistent with the previously reported genotype-phenotype correlations; (2) The results serve as an evidence for reliable genetic counseling and prenatal diagnosis for this family. (3) MLPA, which is a newly developed method, is a rapid and reliable technique to detect the whole gene duplication of PLP1.

Adult ; DNA Probes ; Genes ; Genetic Association Studies ; Humans ; Infant ; Male ; Mutation ; Myelin Proteolipid Protein ; genetics ; Pedigree ; Pelizaeus-Merzbacher Disease ; genetics ; Phenotype