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71.
Wraith JE 《Journal of inherited metabolic disease》2001,24(2):245-250
Mucopolysaccharidosis type I is due to a deficiency of the lysosomal enzyme -L-iduronidase (EC 3.2.1.76) and is associated with a defect in the catabolism of the glycosaminoglycans heparan and dermatan sulphate. The clinical picture produced by this abnormal storage is diverse and ranges from a disorder that is fatal in the early months of life, due to cardiomyopathy, to a condition compatible with a normal life-span. It has become usual to describe the phenotypes associated with this spectrum of disorders after their eponymous names, Hurler (MPS IH, severe), Scheie (MPS IS, mild) and Hurler/Scheie (MPS IH/S, intermediate). Severely affected patients have progressive learning difficulties, facial and skeletal deformities, cardiac disease, corneal clouding, respiratory compromise and joint stiffness. Patients with MPS IH typically die in the first decade of life. MPS IH/S usually have normal intelligence and die in their twenties of cardiorespiratory disease. Patients with MPS IS may have joint stiffness, aortic valve disease and corneal clouding, but are often able to live a normal life-span. Enzyme replacement therapy has been developed as a potential therapy for some patients with MPS I. This process has been helped by the study of a naturally occurring canine model of the disease, which produces a phenotype similar to MPS IH/S in the human. This review details the progress that has been made in this area and also highlights some potential problems with the introduction of therapy. 相似文献
72.
Resistance of Copenhagen rats to chemical induction of glutathione S- transferase 7-7-positive liver foci 总被引:2,自引:0,他引:2
Copenhagen (Cop) rats are completely resistant to the chemical induction of
mammary adenocarcinomas, but their susceptibility to hepatocarcinogenesis
is virtually unknown. Rat liver is a well- characterized and easily
manipulated tissue in which to study carcinogenesis. Therefore, if Cop rats
are resistant to hepatocarcinogenesis, studies into resistance mechanisms
may be feasible. Male Cop and F344 rats, 7-8 weeks old, were initiated
using either N-nitrosodiethylamine (DEN) (200 mg/kg, i.p.) or a two-thirds
partial hepatectomy (PH) followed by N-methyl-N-nitrosourea (MNU) (60
mg/kg, i.p.). The rats were then promoted using a modified resistant
hepatocyte (RH) protocol (a combination of four doses of 2-
acetylaminofluorene (2-AAF) and a single dose of CCl4 that provides a
selective mitotic stimulus for initiated cells). Six weeks after initiation
the rats were killed and liver sections were stained for glutathione
S-transferase 7-7 (GST 7-7), a marker for putative preneoplastic
hepatocytes. Cop rats were found to be highly resistant, having a
approximately 9- and approximately 27-fold smaller percentage of liver area
occupied by GST 7-7-positive foci than susceptible F344 rats following
initiation by DEN and MNU respectively. Furthermore, gross liver nodules
did not form in any of the Cop rats, whereas all F344 rat livers contained
nodules. Hepatic necrosis caused by DEN during initiation, and CCl4 during
promotion is necessary to stimulate compensatory hepatocyte division. We
demonstrated that these agents do indeed increase serum transaminase levels
and produce histologic evidence of necrosis in Cop rats. In order for liver
foci to grow rapidly in the RH protocol, the surrounding normal hepatocytes
must be mito-inhibited by 2-AAF. We found that the degree of
mito-inhibition of normal hepatocytes by 2-AAF is the same in Cop and F344
rats. These results show that the Cop rat is highly resistant to the
chemical induction of putative preneoplastic liver foci and nodules.
相似文献
73.
74.
PG GIBSON JE STUART J WLODARCZYK LG OLSON MJ HENSLEY 《Journal of paediatrics and child health》1996,32(2):143-147
Objective : Chronic middle ear disease is common in Aboriginal children, and may be linked to nasal inflammation and Eustachian tube dysfunction. The pattern of nasal inflammation is unknown. The study reported here was performed to define the role of allergy and infection in causing nasal inflammation in Aboriginal children with chronic middle ear disease.
Methodology : Thirty-one Aboriginal children aged between 3 and 7 years underwent clinical assessment, audiometry and allergy skin tests. Nasal swabs for bacterial culture and cytology were performed during the winter and again in spring to identify any seasonal variation. A randomized trial of nasal beclomethasone for 8 weeks was conducted in children with abnormal tympanometry to identify the effect of therapy upon nasal cytology.
Results : Twenty-six of the 31 children had abnormal tympanograms. Average hearing levels were reduced in nine children. Pathogenic organisms were isolated from most children: Streptococcus pneumoniae (82%), Haemophilus influenzae (79%), Moraxella catarrhalis (39%) and Staphylococcus aureus (29%). Eight of the 31 children (26%) were atopic. Nasal cytology disclosed a marked neutrophil infiltrate (80% of cells) during the winter, which fell significantly in spring to 52% of cells. Only two subjects had nasal eosinophilia of >10%. There was no effect of beclomethasone on nasal cytology.
Conclusions : Chronic ear disease in Aboriginal children is associated with nasal inflammation, neutrophil infiltration and the presence of bacteria. These features suggest respiratory infection as the main cause of chronic nasal inflammation in Aboriginal children with middle ear disease. There is a seasonal variation in the severity of the nasal infiltrate, consistent with increased infections during winter. Despite a high prevalence of atopy, allergic nasal disease was uncommon. 相似文献
Methodology : Thirty-one Aboriginal children aged between 3 and 7 years underwent clinical assessment, audiometry and allergy skin tests. Nasal swabs for bacterial culture and cytology were performed during the winter and again in spring to identify any seasonal variation. A randomized trial of nasal beclomethasone for 8 weeks was conducted in children with abnormal tympanometry to identify the effect of therapy upon nasal cytology.
Results : Twenty-six of the 31 children had abnormal tympanograms. Average hearing levels were reduced in nine children. Pathogenic organisms were isolated from most children: Streptococcus pneumoniae (82%), Haemophilus influenzae (79%), Moraxella catarrhalis (39%) and Staphylococcus aureus (29%). Eight of the 31 children (26%) were atopic. Nasal cytology disclosed a marked neutrophil infiltrate (80% of cells) during the winter, which fell significantly in spring to 52% of cells. Only two subjects had nasal eosinophilia of >10%. There was no effect of beclomethasone on nasal cytology.
Conclusions : Chronic ear disease in Aboriginal children is associated with nasal inflammation, neutrophil infiltration and the presence of bacteria. These features suggest respiratory infection as the main cause of chronic nasal inflammation in Aboriginal children with middle ear disease. There is a seasonal variation in the severity of the nasal infiltrate, consistent with increased infections during winter. Despite a high prevalence of atopy, allergic nasal disease was uncommon. 相似文献
75.
Histidinaemia is a relatively common inherited metabolic disorder with an incidence similar to phenylketonuria. This paper reports the long term outcome of patients diagnosed by newborn screening in the north west of England. Between 1966 and 1990, 108 infants were diagnosed as having histidinaemia by a regional neonatal screening programme (incidence 1:11,083). A further five children were detected following diagnosis in a sibling. Of the 113, nine were lost to follow up. Infants diagnosed before 1981 (n = 47) were placed on a low histidine diet (225 mg/kg/d) for an average period of 21 months (SD 4.5). All patients were reviewed regularly, Griffiths developmental quotients (DQ) were assessed at 2 and 4 years, and WISC-R intelligence quotients (IQ) at 8, 12, and 18 years. IQ data were converted to standard deviation scores (IQ SDS) to account for increasing IQ norms with time. Neither DQ nor IQ correlated with plasma histidine at diagnosis or with the mean plasma histidine throughout life. Growth was normal in all patients. There was no apparent benefit from a low histidine diet in early childhood. In contrast to other studies, there was no excess of clinical symptoms. On the basis of these findings, histidinaemia is a benign metabolic disorder that does not require treatment. 相似文献
76.
A premature infant developed pericardial effusion four days after the insertion of a 25-gauge silastic percutaneous central venous catheter. The effusion contained parenteral nutrition fluid and resolved rapidly after withdrawal of the catheter. Pericardial effusion is a potential complication of percutaneous, as well as surgically placed, central venous catheters. 相似文献
77.
Walter JH Roberts RE Besley GT Wraith JE Cleary MA Holton JB MacFaul R 《Archives of disease in childhood》1999,80(4):374-376
The generalised form of epimerase deficiency galactosaemia has been described in only two children from unrelated families. Their progress is reported and three other affected children from these families are described. The initial presentation was similar to classic galactosaemia. Despite treatment all have shown poor growth and moderate learning difficulties. Three have sensorineural deafness and four have pronounced dysmorphic features. The two older female patients have normal pubertal development. 相似文献
78.
TAYLOR JE; CALNE RY; STEWART WK 《QJM : monthly journal of the Association of Physicians》1991,80(3):771-775
A patient with cystic kidney disease of adult onset and severecystic hepatomegaly is presented. The patient was severely disabledsolely by her abdominal bulk. Simultaneous liver and renal transplantationwas undertaken successfully. 相似文献
79.
Thymic cysts in mediastinal Hodgkin disease 总被引:3,自引:0,他引:3
Three cases of proved thymic cysts associated with mediastinal Hodgkin disease are presented. Two illustrate regression of lymphoma with chemotherapy but persistence of thymic cysts. The third case demonstrates a thymic cyst in untreated Hodgkin disease. These cases suggest that such cysts are probably neither coincidental with nor a consequence of therapy but are probably related to initial thymic involvement by Hodgkin disease. 相似文献
80.