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1.
Both cyproterone acetate (CPA) and the gonadotrophin-releasing hormone
agonist (GnRHa) have been shown to be effective for the treatment of
hirsutism. We wished to compare the effectiveness of CPA in two standard
doses with GnRHa and add-back therapy and to compare the length of
remission after these treatments. A total of 60 hirsute hyperandrogenic
women was assigned to the following treatment groups: CPA 2 mg with 35
microg of ethinylestradiol for 21 days each month (Diane group), CPA 50 mg,
days 5-15, and ethinylestradiol 50 microg, days 5-25, each month (CPA
group) or Decapeptyl 3.75 mg i.m. every 28 days with the addition of
conjugated oestrogen 0.625 mg, days 1-21, and medroxyprogesterone acetate
10 mg, days 12-21 (GnRHa group). Hirsutism was graded by the
Ferriman-Gallwey-Lorenzo (FGL) index and anagen hair shaft diameters and
serum luteinizing hormone (LH) and testosterone were assessed before and
every 3 months during and after treatment. All women were treated for 1
year with 1 year follow-up. At baseline hirsutism and endocrine patterns
were similar in all groups. After one year of treatment, hirsutism
decreased in all groups but the changes were greater (P <0.05) in the
CPA and GnRHa groups than in the Diane group. Serum LH and testosterone
were lowest in the GnRHa group. After withdrawal, hirsutism increased
rapidly in the Diane and CPA groups and after 6 months, FGL scores and hair
shaft diameters were similar to pretreatment values. In the GnRHa group,
hirsutism increased more gradually and after 1 year of withdrawal, FGL
scores and hair diameters were significantly (P <0.05) less than
pretreatment values. Serum LH and testosterone increased rapidly in all
three groups reaching pretreatment values by 6 months. These data suggest
equal efficacy of the GnRHa and the high dose CPA regimen for the treatment
of hirsutism in hyperandrogenic women. GnRHa with add-back treatment
appears to result in a longer remission of hirsutism in comparison with
CPA.
相似文献
2.
J M Escribà Jordana J Canela Soler M R Sala Farré A Orcau Palau 《Gaceta sanitaria / S.E.S.P.A.S》1992,6(33):257-262
In order to assess what different health care workers (mainly physicians and nursing staff) know about the reporting of communicable diseases, with special emphasis on diseases that must be urgently reported, four groups were interviewed about this health information system (91 persons, who took part in different Public Health Seminars during the periods 1989-90 and 1990-91). The total mean score was 16.6 +/- 5.2, in a quantitative scale ranging from 0 to 27 points. Cholera was the communicable disease with the highest percentage of correct answers concerning the knowledge of its reporting (100% in two groups), while typhus was the disease with the lowest percentage (23%). The diversity of the results within the study groups suggests that it is necessary to insist on a continuous training on epidemiological surveillance and communicable diseases within Public Health activities in Catalonia. 相似文献
3.
Carmina E.; Gentzschein E.; Stanczyk F.Z.; Lobo R.A. 《Human reproduction (Oxford, England)》1995,10(2):299-303
Serum C19 conjugates, specifically 3-androstanediol glucuronide(3G), reflect peripheral androgen action through the actionof 5-reductase activity. The origin of 5-reduced C19 conjugateshas been controversial and it has been suggested that they arederived primarily from adrenal androgens. We examined concentrationsof 3G, 3-androstanediol sulphate (3S), androsterone glucuronide(AoG) and androsterone sulphate (AoS) in 40 hirsute hyperandrogenicwomen. These patients were divided into four groups based uponindividual, combined or normal concentrations of the adrenalandrogens dehydroepiandrosterone (DHEAS) and 11-hydroxy-androstenedione.Testosterone, unbound testosterone and androstenedione weresimilar in these groups. Serum 3G was equally high in all groupsand was correlated significantly with hirsutism, while the otherconjugates were not. Androsterone glucuronide was raised inall groups but was higher in patients with raised DHEAS. Serum3S was raised in all groups and was higher where both adrenalandrogens were raised. Serum AoS was highly correlated withDHEAS. Serum 3G was correlated with unbound testosterone andandrostenedione but not with the adrenal androgens. The glucuronideconjugates were correlated with one another as were the sulphateconjugates but glucuronides and sulphates were not correlated.These data confirm ovarian and adrenal dependency of C19 conjugates.Serum 3G appears to reflect hirsutism most accurately and isleast dependent on adrenal androgens in patients with mixedhyperandrogenism. 相似文献
4.
A López de Munain A M Cobo J J Poza D Navarrete L Martorell F Palau J I Emparanza M Baiget 《Journal of medical genetics》1995,32(9):689-691
To analyse the influence of the sex of the transmitting grandparents on the occurrence of the congenital form of myotonic dystrophy (CDM), we have studied complete three generation pedigrees of 49 CDM cases, analysing: (1) the sex distribution in the grandparents' generation, and (2) the intergenerational amplification of the CTG repeat, measured in its absolute and relative values, between grandparents and the mothers of CDM patients and between the latter and their CDM children. The mean relative intergenerational increase in the 32 grandparent-mother pairs was significantly greater than in the 56 mother-CDM pairs (Mann-Whitney U test, p < 0.001). The mean expansion of the grandfathers (103 CTG repeats) was also significantly different from that seen in the grandmothers' group (154 CTG repeats) (Mann-Whitney U test, p < 0.01). This excess of non-manifesting males between the CDM grandparents' generation with a smaller CTG length than the grandmothers could suggest that the premutation has to be transmitted by a male to reach the degree of instability responsible for subsequent intergenerational CTG expansions without size constraints characteristic of the CDM range. 相似文献
5.
Friedreich's ataxia and frataxin: molecular genetics, evolution and pathogenesis (Review) 总被引:2,自引:0,他引:2
Palau F 《International journal of molecular medicine》2001,7(6):581-589
Friedreich's ataxia is an autosomal recessive neuro-degenerative disorder involving both central and peripheral nervous system. Patients also show a systemic clinical picture presenting heart disease and diabetes mellitus or glucose intolerance. The disease is caused by mutations in the FRDA gene mapped on chromosome 9q13. The product of the gene is frataxin, an 18 kDa soluble mitochondrial protein with 210 amino acids. Crystal structure suggests a new, not previously reported, protein fold. The most frequent mutation is the expansion of a GAA trinucleotide repeat located within the first intron of the gene, and represents 98% of the mutations. Point mutations are described in compound heterozygous subjects with one expanded allele. A two-step model of GAA normal alleles towards premutation alleles, which might generate further full expanded mutations in the population with Indo-European ancestry, has been postulated. Clinical phenotype is variable and an inverse correlation with the GAA expansion size has been observed. Analysis of the GAA triplet is a strong molecular tool for clinical diagnosis, genetic counselling and prenatal diagnosis. Friedreich's ataxia patho-genesis is not solved yet. Substantial data from organism models, such the S. cerevisae yeast and more recently conditioned knock-outs in mouse, and studies in heart biopsies and fibroblast cultures from patients suggest an important role of mitochondrial iron in the development of the disease. Iron is accumulated in the mitochondrial matrix of both the yeast frataxin deficient mutant and the patient fibroblasts. It has been postulated that iron-induced oxygen radical affects the oxidative phosphorylation in frataxin deficiency states favouring the disease pathology. A second hypothesis postulates a direct role of frataxin in the mitochondrial energy activation and oxidative phosphorylation. Iron chelator drugs and antioxidant drugs have been postulated for Friedreich's treatment. No results from clinical trials are available yet, but idebenone, a short-chain quinone, seems to reduce the size of hypertrophic cardiomyopathy and levels of oxidative stress molecules in patients. 相似文献
6.
De Castro M Cruz-Martínez A Vílchez JJ Sevilla T Pineda M Berciano J Palau F 《Journal of the peripheral nervous system : JPNS》1999,4(1):58-62
The aim of this study was to determine phenotypie characteristics of patients with early onset cerebellar ataxia (EOCA) with preserved tendon reflexes. The series comprises 25 patients, representing 10% of all ataxic patients who have been genetically studied in our laboratory since 1990. There were 11 males and 14 females. Fourteen patients were homozygous for the GAA expansion on chromosome 9q13 (group 1) and therefore a diagnosis of Friedreich's ataxia with retained reflexes (FARR) was given. The remaining 11 patients had two normal non-expanded alleles (group 2) and a working diagnosis of EOCA with retained reflexes (EOCARR) was established. Mean ages of onset were 13.7 +/- 5.9 years (3-25) for group 1 and 10.3 +/- 7.3 for group 2; the difference was not significant. Frequencies of symptoms and signs were also comparable for both groups the only significant differences being the higher frequency of nystagmus, cardiomyopathy and sensory neuropathy in group 1 patients. There was a tendency for FARR patients to have higher frequencies of hypopallesthesia in the lower limbs and skeletal deformities. In none of the cases diabetes mellitus was observed. We conclude that differentiation of FARR and EOCARR may be suspected by classical clinical and electrophysiological data and confirmed by analysis of the GAA repeat. 相似文献
7.
Pilar García-Peña Javier Lucaya G. Michael A. Hendry Philip T. McAndrew Carmina Duran 《Pediatric radiology》1998,28(4):266-270
Background. Two cases of pulmonary sequestration which regressed spontaneously are presented. Objective. To demonstrate the value of imaging studies in the diagnosis and follow-up of some forms of congenital masses of the lung
in asymptomatic patients. Material and methods. We reviewed the clinical records and imaging studies of two asymptomatic children, one newborn and the other 3 months old,
with thoracic masses which demonstrated variable degrees of spontaneous involution. Results. Abdominal ultrasound performed on the newborn with a palpable mass showed a triangular echogenic mass with a large central
feeding vessel arising from the aorta. The mass had disappeared on follow-up US exam performed 6 years later. CT was performed
in the 3-month-old patient with a persistent retrocardiac mass. A soft-tissue density mass in the left pulmonary base with
a large feeding vessel arising from the aorta was visualised on contrast-enhanced CT. Five years later, a new CT scan showed
significant shrinkage of the mass and no vessel. Conclusion. Radiological techniques such as real-time US with Doppler imaging and contrast-enhanced CT may establish the diagnosis of
pulmonary sequestration by demonstrating the mass and its systemic vessel, thereby eliminating the need for more aggressive
imaging procedures. Partial or total disappearance of these masses represents a further example of involutive pathology and
suggests that not all cases of pulmonary sequestration should be surgically treated.
Received: 12 September 1997 Accepted: 18 September 1997 相似文献
8.
9.
Minimal hepatic encephalopathy identifies patients at risk of faster cirrhosis progression 下载免费PDF全文
10.
M Perpi?á M Palau J Cortijo E Fornas C Sanz E Morcillo 《Respiration; international review of thoracic diseases》1990,57(2):81-84
Tracheal strips from actively sensitized guinea pigs exhibited an enhanced responsiveness (greater maximal effects; Emax) and sensitivity (smaller effective concentration 50%; pD2) to CaCl2 (in K(+)-depolarized tissues), KCl and histamine compared with that of strips from nonsensitized animals. A significant correlation was found between the magnitude of the contraction produced by bovine serum albumin (1 mg/ml) and the Emax and pD2 values of CaCl2, KCl and histamine in tracheal strips from sensitized guinea pigs. This indicates that specific and nonspecific challenges correlate in sensitized guinea pig trachea. 相似文献