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101.
DL Domingo MI Trujillo SE Council MA Merideth LB Gordon T Wu WJ Introne WA Gahl TC Hart 《Oral diseases》2009,15(3):187-195
Objective: Hutchinson-Gilford progeria syndrome (HGPS) is a rare early-onset accelerated senescence syndrome. In HGPS, a recently identified de novo dominant mutation of the lamin A gene ( LMNA ) produces abnormal lamin A, resulting in compromised nuclear membrane integrity. Clinical features include sclerotic skin, cardiovascular and bone abnormalities, and marked growth retardation. Craniofacial features include 'bird-like' facies, alopecia, craniofacial disproportion, and dental crowding. Our prospective study describes dental, oral soft tissue, and craniofacial bone features in HGPS.
Methods: Fifteen patients with confirmed p.G608G LMNA mutation (1–17 years, seven males, eight females) received comprehensive oral evaluations. Anomalies of oral soft tissue, gnathic bones, and dentition were identified.
Results: Radiographic findings included hypodontia ( n = 7), dysmorphic teeth ( n = 5), steep mandibular angles ( n = 11), and thin basal bone ( n = 11). Soft tissue findings included ogival palatal arch ( n = 8), median sagittal palatal fissure ( n = 7), and ankyloglossia ( n = 7). Calculated dental ages (9 months to 11 years 2 months) were significantly lower than chronological ages (1 year 6 months to 17 years 8 months) ( P = 0.002). Eleven children manifested a shorter mandibular body, anterior/posterior cranial base and ramus, but a larger gonial angle, compared to age/gender/race norms.
Conclusion: Novel oral-craniofacial phenotypes and quantification of previously reported features are presented. Our findings expand the HGPS phenotype and provide additional insight into the complex pathogenesis of HGPS. 相似文献
Methods: Fifteen patients with confirmed p.G608G LMNA mutation (1–17 years, seven males, eight females) received comprehensive oral evaluations. Anomalies of oral soft tissue, gnathic bones, and dentition were identified.
Results: Radiographic findings included hypodontia ( n = 7), dysmorphic teeth ( n = 5), steep mandibular angles ( n = 11), and thin basal bone ( n = 11). Soft tissue findings included ogival palatal arch ( n = 8), median sagittal palatal fissure ( n = 7), and ankyloglossia ( n = 7). Calculated dental ages (9 months to 11 years 2 months) were significantly lower than chronological ages (1 year 6 months to 17 years 8 months) ( P = 0.002). Eleven children manifested a shorter mandibular body, anterior/posterior cranial base and ramus, but a larger gonial angle, compared to age/gender/race norms.
Conclusion: Novel oral-craniofacial phenotypes and quantification of previously reported features are presented. Our findings expand the HGPS phenotype and provide additional insight into the complex pathogenesis of HGPS. 相似文献
102.
Oddo M Levine JM Mackenzie L Frangos S Feihl F Kasner SE Katsnelson M Pukenas B Macmurtrie E Maloney-Wilensky E Kofke WA Leroux PD 《中华神经外科疾病研究杂志》2011,(5):452-452
Background:Brain hypoxia(BH)can aggravate outcome after severe traumatic brain injury(TBI).Whether BH or reduced brain oxygen(Pbto2)is an independent outcome predictor or a marker of disease severity is not fully elucidated.Objective To analyze the relationship between Pbto2,intracranial pressure(ICP),and cerebral perfusion pressure(CPP)and to examine whether BH correlates with worse outcome independently of ICP and CPP.Methods We studied103patients monitored 相似文献
103.
Objective
To evaluate the therapeutic effects of the embedding needle therapy in the preventative treatment of chronic migraine.Methods
A total of 36 patients with chronic migraine were divided into an embedding needle therapy group and a flunarizine group according to random number table, 18 cases in each group. In the embedding needle therapy group, the thumb-tack needles were used at Sīzhúkōng (丝竹空TE 23), Hànyàn (颔厌GB 4), Wáng? (完骨GB 12) and Cuánzhú (攒竹BL 2) and the needles were embedded for 24 h. The embedding needle therapy was given three times a week. In the flunarizine group, flunarizine was prescribed, 10 mg a day. Separately, at the end of the 4-week treatment and in 4 weeks of the follow-up, the number of days of headache attack per month (days/month) and the headache duration (hours/month) in the cases with moderate to severe headache were observed in the two groups.Results
The number of days of headache attack in the cases with moderate to severe headache was reduced after treatment as compared with the number before treatment in the two groups. The headache duration was reduced obviously after treatment in the cases of moderate to severe headache in the two groups. In the 4-week follow-up, all the indexes were still reduced as compared with the indexes before treatment, indicating the significant differences in the two groups (both P < 0.05). In the embedding needle therapy group, at the end of the 4-week treatment and in 4 weeks of the follow-up, the number of days of headache attack per month was (8.1 ± 2.4) days and (7.2 ± 1.6) days separately, which were significant difference as compared with (9.7 ± 2.3) days and (8.9 ± 1.4) days in the flunarizine group (both P < 0.05). Regarding the headache duration, at the end of the 4-week treatment and in 4 weeks of the follow-up, it was (105.6 ± 10.5) h/month and (92.1 ± 7.2) h/month respectively in the embedding needle therapy group, indicating the significant differences as compared with (135.4 ± 8.9) h and (116.1 ± 8.2) h in the flunarizine group respectively (both P < 0.05).Conclusion
The 4-week treatment with the embedding needle therapy obviously reduces the number of days of headache attack and the headache duration, presents the preventative effects in the patients with chronic migraine. 相似文献104.
Marleen?J?NahuisEmail author Nienke?S?Weiss Fulco?van der Veen Ben?Willem?J?Mol Peter?G?Hompes Jur?Oosterhuis Nils?B?Lambalk Jesper?MJ?Smeenk Carolien?AM?Koks Ron?JT?van Golde Joop?SE?Laven Ben?J?Cohlen Kathrin?Fleischer Angelique?J?Goverde Marie?H?Gerards Nicole?F?Klijn Lizka?CM?Nekrui Ilse?AJ?van Rooij Diederik?A?Hoozemans Madelon?van Wely 《BMC women's health》2013,13(1):42
Background
Clomiphene citrate (CC) is first line treatment in women with World Health Organization (WHO) type II anovulation and polycystic ovary syndrome (PCOS). Whereas 60% to 85% of these women will ovulate on CC, only about one half will have conceived after six cycles. If women do not conceive, treatment can be continued with gonadotropins or intra-uterine insemination (IUI). At present, it is unclear for how many cycles ovulation induction with CC should be repeated, and when to switch to ovulation induction with gonadotropins and/or IUI.Methods/Design
We started a multicenter randomised controlled trial in the Netherlands comparing six cycles of CC plus intercourse or six cycles of gonadotrophins plus intercourse or six cycles of CC plus IUI or six cycles of gonadotrophins plus IUI.Women with WHO type II anovulation who ovulate but did not conceive after six ovulatory cycles of CC with a maximum of 150 mg daily for five days will be included.Our primary outcome is birth of a healthy child resulting from a pregnancy that was established in the first eight months after randomisation. Secondary outcomes are clinical pregnancy, miscarriage, multiple pregnancy and treatment costs. The analysis will be performed according to the intention to treat principle. Two comparisons will be made, one in which CC is compared to gonadotrophins and one in which the addition of IUI is compared to ovulation induction only. Assuming a live birth rate of 40% after CC, 55% after addition of IUI and 55% after ovulation induction with gonadotrophins, with an alpha of 5% and a power of 80%, we need to recruit 200 women per arm (800 women in total).An independent Data and Safety Monitoring Committee has criticized the data of the first 150 women and concluded that a sample size re-estimation should be performed after including 320 patients (i.e. 80 per arm).Discussion
The trial will provide evidence on the most effective, safest and most cost effective treatment in women with WHO type II anovulation who do not conceive after six ovulatory cycles with CC with a maximum of 150 mg daily for five days. This evidence could imply the need for changing our guidelines, which may cause a shift in large practice variation to evidence based primary treatment for these women.Trial registration number
Netherlands Trial register NTR1449105.
106.
107.
108.
Predicting serious bacterial infection in young children with fever without apparent source 总被引:1,自引:0,他引:1
SE Bleeker KGM Moons G Derksen-Lubsen DE Grobbee HA Moll 《Acta paediatrica (Oslo, Norway : 1992)》2001,90(11):1226-1231
The aim of this study was to design a clinical rule to predict the presence of a serious bacterial infection in children with fever without apparent source. Information was collected from the records of children aged 1-36 mo who attended the paediatric emergency department because of fever without source (temperature > or = 38 degrees C and no apparent source found after evaluation by a general practitioner or history by a paediatrician). Serious bacterial infection included bacterial meningitis, sepsis, bacteraemia, pneumonia, urinary tract infection, bacterial gastroenteritis, osteomyelitis and ethmoiditis. Using multivariate logistic regression and the area under the receiver operating characteristic curve (ROC area), the diagnostic value of predictors for serious bacterial infection was judged, resulting in a risk stratification. Twenty-five percent of the 231 patients enrolled in the study (mean age 1.1 y) had a serious bacterial infection. Independent predictors from history and examination included duration of fever, poor micturition, vomiting, age, temperature < 36.7 degrees C or > or = 40 degrees C at examination, chest-wall retractions and poor peripheral circulation (ROC area: 0.75). Independent predictors from laboratory tests were white blood cell count, serum C-reactive protein and the presence of >70 white blood cells in urinalysis (ROC area: 0.83). The risk stratification for serious bacterial infection ranged from 6% to 92%. CONCLUSION: The probability of a serious bacterial infection in the individual patient with fever without source can be estimated more precisely by using a limited number of symptoms, signs and laboratory tests. 相似文献
109.
110.
Detection of circulating crosslinked fibrin derivatives by a heat extraction-SDS gradient gel electrophoretic technique 总被引:2,自引:1,他引:2
A technique has been developed to identify and quantitate unique plasmic degradation products of crosslinked fibrin in plasma. In this method, fibrin derivatives are extracted by heat precipitation and dissolved with disulfide bond reduction, after which the crosslinked gamma-gamma chain remnants are identified by SDS-polyacrylamide gradient gel electrophoresis and quantitated by densitometric analysis. A heterogenous group of gamma-gamma chains with molecular weights between 100,000 and 76,000 daltons was identified in lysates of crosslinked fibrin during plasmic degradation in vitro. Three stages of crosslinked fibrin degradation have been arbitrarily defined based primarily on the extent of degradation of these gamma-gamma polypeptide chains. As little as 20 microgram of crosslinked fibrin digests added to 1 ml of normal plasma could be detected by the heat-extraction--gel- electrophoresis technique, identifying the gamma-gamma derivatives with molecular weights of 96,000, 86,000, 82,000, and 76,000 daltons. Plasmic derivatives of gamma-gamma chains were not found in normal plasma, but they were identified in the plasma of patients with disseminated intravascular coagulation and deep-vein thrombosis, both before and in increased quantity during successful thrombolytic therapy. 相似文献