6.24 mmol/L) and sixty healthy persons in the health center of our hospital were investigated as hyperhpidemia group (Hyperlipidemias) and control group (Controls) respectively.Hyperlipidemias were given simvastatin 20 mg?d~(-1) for twelve weeks (Statins).Blood samples of ulnar vein were extracted from Statins at the end of twelve weeks as well as Controls and Hyperhpidemias at the beginning of the experiment. Blood serum,plasma and mononuclearcell were extracted and stored at a refrigerator of-80℃.The level of plasma angiotensinⅡwas detected by the method of radioimmunity.While the expression of RANTES mRNA and protein on mononuclearcell were assessed by real time reverse transcription polymerse chain reaction and Western blot respectively.Results①The plasma angiotensinⅡof Hyperlipidemias was higher than that of Controls [(92.13?22.03) vs (50.85?12.12),P

OBJECTIVEAlthough primary ciliary dyskinesia (PCD) is a group of inherited diseases, accurate diagnosis and appropriate clinical care to prevent and treat the complications could maintain patients' quality of life and normal life span. The diagnosis of PCD may often be delayed because it is frequently misdiagnosed as bronchitis, sinusitis and otitis. This study aimed to analyze and summarize the clinical features of PCD and explore diagnostic and differential diagnostic procedures in children.

METHODSPatients were all chosen from the inpatient department of Beijing Children's Hospital, Capital Medical University between 1990 - 2006. The tunica mucosa bronchiorum and/or nasal mucous membrane were gained through bronchoscope in children suspected to have PCD. The ciliary ultrastructures were analyzed through the electron microscope. The clinical features and procedures of the diagnosis and differential diagnosis in children with PCD were analyzed.

RESULTSThere were totally 26 children diagnosed as PCD with 10 (38.5%) Kartagener syndrome. All Kartagener syndrome children had mirror image dextrocardia with normal cardiac structure and situs inversus viscerum. The bronchoscopy performed in eight of 10 Kartagener syndrome children showed bronchus transposition. Twenty-six children came from twenty-five families. Although the siblings of four probands also had the symptoms of chronic cough with sputum, running nose and recurrent respiratory infections, only a boy and his sister were diagnosed as Kartagener syndrome simultaneously. Their parents and the other family members were healthy. Of the 26 patients, 11 were boys and 15 were girls. The median age at diagnosis was 8.7 years. The age of onset was between the second day after delivery and fifteen years old, median age was 3 years. The course of disease before diagnosis was eleven days to twelve years (median 3.5 years). All the children had the symptom of cough, 24 of which had productive cough. Seven cases were found to have clubbing fingers. Dynein arm defect was found in 10 children, 6 of them had total absence of dynein arms and 4 had decreased dynein arm numbers. Microtube derangements were found in 8 children. One Kartagener syndrome child had a normal cilia structure. Bronchiectasis, consolidation and increased lung markings were found in 8, 6 and 7 patients separately on the radiographic study. Twenty patients had sinusitis. Nine of sixteen children had decreased PEF, FEV1 and/or FEF 25 - 75 on the pulmonary function test. Fifteen culture samples obtained from 6 children's sputum and/or bronchoalveolar lavage fluid were positive for 8 strains of Pseudomonas aeruginosa, 5 strains of Streptococcus pneumoniae and 2 strains of Candida albicans. In 1 subject more than one organism were found in the same sample. Hearing lost and gastroesophageal reflux were detected in 3 of 4 and 3 of 5 examined children respectively.

CONCLUSIONSThe onset of PCD can occur from neonate to adolescence and usually has a chronic course. The common symptom of pediatric PCD was productive cough and significant growth retardation. The most common ultrastructural abnormalities associated with PCD were the total absence of dynein arms, decreased dynein arm numbers and microtube derangement. Some patients have normal ciliary structures. Bronchiectasis, consolidation and sinusitis were usually seen on the radiography. Pseudomonas aeruginosa and Streptococcus pneumoniae were the two common bacterial organisms obtained from sputum and/or bronchoalveolar lavage fluid of PCD children. Some patients have mixed infections. PCD children have high percentages of hearing lost and gastroesophageal reflux.

Adolescent ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Infant ; Kartagener Syndrome ; diagnosis ; Male

OBJECTIVETo study the clinicopathological, immunohistochemical and electron microscopic characteristics of pediatric rhabdomyosarcomas (RMS).

METHODSOne hundred and forty-five cases of pediatric rhabdomyosarcomas were studied by routine histological, immunohistochemical and electron microscopic studies.

RESULTSThere were 97 male and 48 female patients with ages ranging from 4 months to 13 years and a mean of 4.2 years. The follow-up period of 100 patients was from 1 year to 20 years with a mean of 5 years after diagnosis. All cases were subtyped into the following histological categories: embryonal RMS, botryoid RMS, spindle cell RMS, alveolar RMS and solid RMS. Histopathological subtypes, tumor site and tumor stage correlated significantly with the patients' 5 years survival. The best prognosis was observed in spindle cell and botryoid RMS. Embryonal RMS carried an intermediate prognosis. Patients with alveolar RMS and solid RMS had the worst prognosis. Tumors involving bladder, head and neck carried a favorable clinical outcome. Patients with tumors involving trunk extremities retroperitoneum and pelvis did poorly. Immunohistochemically, all cases were positive for Vimentin. The positive staining rates for desmin, SMA and myoglobin were 78%, 75% and 37%, respectively. All tumors were negative for NSE, CD99 and LCA. Electron microscopy study showed features of myofilament and sarcomere in 10 of 15 cases.

CONCLUSIONSRMS is the most common soft tissue sarcoma of childhood. Immunohistochemistry and electron microscopy are helpful in diagnosis and classification of RMS.

Adolescent ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Head and Neck Neoplasms ; pathology ; ultrastructure ; Humans ; Immunohistochemistry ; Infant ; Male ; Retrospective Studies ; Rhabdomyosarcoma ; classification ; pathology ; ultrastructure ; Soft Tissue Neoplasms ; classification ; pathology ; ultrastructure ; Urogenital Neoplasms ; metabolism ; pathology ; ultrastructure

Objective To investigate the flexibility of both the covered stents specially designed for use in intracranial vasculature and the delivering system in passing through the bone tube and the physiological curves of the cranial internal carotid artery(CICA)to reach the targeted area,the performance (adherence)of the covered stents in occluding vascular wall diseases and the impact on the vascular branches of the covered segment.Methods The covered stents specially designed for use in intracranial vaseulature were used to treat 13 patients with CICA diseases using endovascular techniques.There were 4 huge pseudoaneurysms,4 giant aneurysms,3 small wide-necked aneurysms,1 giant pseudoaneurysm with concurrent internal carotid cavernous fistula(CCF),and 1 CCF.Prior to the detachment of the covered stents,balloon occlusion test(BOT)of the internal carotid artery on the diseased side and whole-brain digital subtraction angiography(DSA)were performed in all the patients.Three to 16 months following procedure,DSA and clinical follow-ups were performed.Results Thirteen patients all tolerated the BOT well with the DSA demonstrating well-opened anterior and posterior communicating arteries.The covered stents and the delivering systems all successfully passed CICA to reach the targeted diseased area,with the diseased segments of the internal carotid artery including C3—C4 in 4 cases,C4—C5 in 4 and C6—C7 in 5.Immediately following the detachment of the covered stents,DSA demonstrated that 7 aneurysms were completely occluded,4 aneurysms had slight endoleak,and 1 CCF had markedly-decreased blood flow through the fistula.In the patient with concurrent pseudoaneurysm and CCF,the pseudoaneurysm disappeared and the blood flow through the fistula was markedly-reduced immediately following the stenting procedure.Apart from one patient with aneurysmal subarachnoid hemorrhage who died due to extensive vascular spasm on the 9th day following the stenting procedure,all the other 12 patients had unobstructed stented vessels on the follow-up DSA images,with 2 demonstrating slight stenosis.In the 6 patients with post-procedure endoleak,DSA showed that the endoleak in 4 patients had disappeared,one endoleak disappeared following the second stenting,and one CCF remained low-flow fistula.There was no sequela related to the occlusion of branches in the covered arterial segment.Conclusion The covered stents specially designed for use in the intracranial vasculature and the delivering system are both flexible enough to pass the tortuous CICA to reach the intracranial diseased artery,and are effective in managing CICA diseases.Further follow-up is still needed to determine the long-term effect of the covered stents,and the adherence of the covered stents needs further investigation.

OBJECTIVETo preliminarily investigate the long-term structural and functional injuries of mitochondria in rat brain caused by sepsis.

METHODSWistar rats were randomly assigned into sepsis and control groups. A rat model of sepsis was prepared by an intraperitoneal injection of 10 mg/kg lipopolysaccharide (LPS) of gram-negative bacteria, and the survival assay was performed. Eight rats in the sepsis group were sacrificed at 12, 24, 48, or 72 hours after LPS injection, while rats in the control group were sacrificed after an intraperitoneal injection of an equal volume of normal saline. Mitochondria were extracted from rat brain tissue. Mitochondrial membrane potential (MMP) and mitochondrial swelling level were determined by flow cytometry, and the activities of electron transport chain complexes (I-V) were measured using enzyme assay kits. Hematoxylin-eosin (HE) staining and electron microscopy were used to observe morphological changes in brain tissue and mitochondria.

RESULTSThe sepsis group had a significantly lower survival rate than the control group (P<0.01). The MMP and activities of electron transport chain complexes (I-V) in the sepsis group, which were significantly lower than those in the control group (P<0.05), were reduced to the lowest levels at 48 hours and partially recovered at 72 hours. The mitochondrial swelling level in the sepsis group, which was significantly higher than that in the control group (P<0.05), increased to the peak level at 48 hours and partially recovered at 72 hours. Hematoxylin and Eosin staining revealed substantial damages in the structure of brain tissue, and electron microscopy showed mitochondrial swelling, and vacuolization in a few mitochondria.

CONCLUSIONSIn the rat model of LPS-induced sepsis, both structural and functional injuries are found in cerebral mitochondria, and achieve the peak levels probably at around 48 hours.

Animals ; Brain ; pathology ; physiopathology ; ultrastructure ; Lipopolysaccharides ; toxicity ; Male ; Membrane Potential, Mitochondrial ; Mitochondria ; physiology ; ultrastructure ; Rats ; Rats, Wistar ; Sepsis ; chemically induced ; mortality ; physiopathology

OBJECTIVETo analyze the clinical, imaging and pathological findings of congenital intrapulmonary lymphangioma and hemangioma in 5 infants and young children.

METHODData of 3 cases with congenital intrapulmonary lymphangioma and 2 cases with haemangioma were analyzed.

RESULTAll the 5 cases had cough, difficulty in breathing, cyanosis of lips, and shortness of breath. Imaging study indicated cystic and space occupying changes of partial lung. In the two cases of hemangioma, the blood vessels passing through the hemangioma were observed on CT films. Histochemical studies showed that the cystic pockets from removed tissues were different in size and there was a line of flat endothelial tissue around these pockets. Immunochemical studies indicated D2-40 positive, factor VIII weak positive for lymphangioma cases; while in hemangioma cases, factor VIII was positive, D2-40 was negative or weakly positive, and both of cytokeratin was negative.

CONCLUSIONIt is very important to consider and identify congenital intrapulmonary lymphangioma or hemangioma when a patient has cystic pockets and space occupying change in their lung by imaging and pathological studies.

Female ; Hemangioma ; congenital ; pathology ; Humans ; Infant ; Lung Neoplasms ; congenital ; pathology ; Lymphangioma ; congenital ; pathology ; Male

OBJECTIVE: To explore the clinical characteristics and genetic etiology of a patient with adolescent-onset hypomyelinated leukodystrophy with atrophy of basal ganglia and cerebellum (H-ABC). METHODS: A patient who was diagnosed with H-ABC in March 2018 at the First Affiliated Hospital of Nanjing Medical University was selected as the study subject. Clinical data was collected. Peripheral venous blood samples of the patient and his parents were collected. The patient was subjected to whole exome sequencing (WES). Candidate variant was verified by Sanger sequencing. RESULTS: The patient, a 31-year-old male, had manifested with developmental retardation, cognitive decline and abnormal gait. WES revealed that he has harbored a heterozygous c.286G>A variant of the TUBB4A gene. Sanger sequencing confirmed that neither of his parents has carried the same variant. Analysis with SIFT online software indicated the amino acid encoded by this variant is highly conserved among various species. This variant has been recorded by the Human Gene Mutation Database (HGMD) with a low population frequency. The 3D structure constructed by PyMOL software showed that the variant has a harmful effect on the structure and function of the protein. According to the guidelines formulated by the American College of Medical Genetics and Genomics (ACMG), the variant was rated as likely pathogenic. CONCLUSION The c.286G>A (p.Gly96Arg) variant of the TUBB4A gene probably underlay the hypomyelinating leukodystrophy with atrophy of basal ganglia and cerebellum in this patient. Above finding has enriched the spectrum of TUBB4A gene variants and enabled early definitive diagnosis of this disorder.
Male ; Humans ; Adolescent ; Adult ; Magnetic Resonance Imaging ; Basal Ganglia/pathology* ; Cerebellum ; Atrophy/pathology* ; Mutation ; Tubulin/genetics*

Objective To systematically review the efficacy of angiotensin receptor blocker (ARB) combined with glucocorticoid for patients with idiopathic pulmonary fibrosis (IPF).Methods Electronically searched PubMed,EmBase,the Cochrane Library,CNKI,CBM,VIP,Wanfang Database from the date of establishment to March 2017 for all randomized controlled trials (RCTs) and quasi-RCTs based on ARB combined with glucocorticoid therapy for patients with IPF.Manual search in relevant journals and search of relevant literature on other websites were also performed.The data extraction and quality assessment of included RCTs and quasi-RCTs were conducted by two reviewers independently.Then,Meta-analysis was conducted by using RevMan 5.3 software.Results A total of 12 RCTs involving 1239 patients were included,621 cases in treatment group and 618 cases in control group.The results of Meta-analysis indicated that the treatment group was superior to the control group in clinical effectiveness (OR =2.78,95％ CI:2.12-3.66,P ＜ O.001),partial pressure of oxygen (PaO2,MD =8.76,95％ CI:7.26-10.26,P ＜0.001),partial pressure of carbon dioxide (PaCO2,MD =-14.04,95％ CI:-15.03--13.05,P ＜0.001),forced vital capacity (FVC,MD =0.74,95％ CI:O.55-0.94,P ＜0.001),forced expiratory volume in one second (FEV1,MD =0.74,95％ CI:0.63-0.86,P ＜0.001),diffusion capacity for carbon monoxide (DLCO) value (MD =3.70,95％ CI:1.88-5.51,P ＜ 0.001) and vital capacity (VC,MD=5.23,95％ CI:3.38-7.08,P＜O.001).Conclusion The current evidence suggested that ARB combined with glucocorticoid could increase clinical effectiveness,improve PaO2,FVC,FEV1,DLCO and VC,and decrease PaCO2 in serum of IPF patients.

Angioplasty, Balloon, Coronary ; adverse effects ; methods ; Drug-Eluting Stents ; Humans ; Paclitaxel ; therapeutic use

Molecular pharmacognosy is a science of classification and identification, cultivation and protection, and production of active ingredients of graduated drugs at the molecular level. The proposal of molecular pharmacognosy allows the research of crude drugs to advance from the microscopic level to the genetic level. Pueraria lobata root, as a medicinal and edible plant, has high application value and economic value. There are many varieties that are easy to cause confusion, and it is not easy to distinguish and identify according to traditional identification methods. Moreover, the research of P. lobate root at the genetic level is still relatively shallow. the study received extensive attention of scholars. This article reviews recent research on molecular identification of P. lobate, transcriptome sequencing, cloning and synthesis of functional genes of P. lobate root in recent years in order to provide references for further promoting the development and utilization of P. lobate root and its active ingredients.
Pharmacognosy ; Plant Roots/genetics* ; Pueraria