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1.
致密性骨发育不全系一种罕见的骨密度增高性疾患,为常染色体隐性遗传。其主要特点为侏儒、普遍性骨质硬化、骨发育不全和容易骨折等特点,类似石骨症和颅锁骨发育不全、软骨发育不全等。Maroteaux1962年首先将其列为独立的疾患,并命名为致密性骨发育不全。现国内报告仅有3例,我院发现一例,其临床X线表现均较典型报告如下: 患者女,13岁。因活动后双侧足背肿痛三个月,而来我院就诊。查体:身材较矮,身高122cm,营养中等,头大面小,鼻梁塌陷,突眼,眼距较宽,颅缝未闻合,手足指处粗短,指甲平坦,下颌角消失…  相似文献   

2.
张庄熠  高长启  汪荣灿   《放射学实践》2011,26(4):466-467
骨斑点症又称为周身性致密性骨炎、周身性脆性骨硬化症或播散性致密性骨病,是一种罕见病症。1905年由Stie ola首先描述,1915年Albers-Schonbery报道典型病例,1916年由Ledoux命名为骨斑点症[1]。  相似文献   

3.
致密性骨发育不全症(Pycnodysorstosis),系较为少见的以侏儒、骨发育不全伴全身骨硬化、骨脆性增加的常染色体隐性遗传性疾病.1962年由Marioteux 和Famy 首先提出并命名.迄今为止,国外报告已有百余例,国内报告甚少.我院收治1例,具有典型的临床及X 线表现,现报告如下:  相似文献   

4.
致密性骨发育不全(pyenodysostosis)是一种罕见的骨发育障碍,笔者遇到2例。报告如下。  相似文献   

5.
致密性骨发育不全症(Pycnodysorstosis),系较为少见的以侏儒,骨发育不全伴全身骨硬化,骨脆性增加的常染色体隐性遗传性疾病,1962年由Marioteux和Famy首先提出并命名,迄今为止,国外报告已有百余例,国内报告减少,我院收治1例,具有典型的临床及X线表现,现报告如下。  相似文献   

6.
致密性骨发育障碍,又称致密性成骨不全症(Pycnodysostos's),致密性骨质发育不全症。以身材矮小、骨密度增高和颅骨发育异常为其特征。 王×× 女 34岁 自幼发育矮小,近7—8年来间断出现额部胀痛,重时伴恶心,呕吐,对症治疗可缓解。12年前曾发生右胫骨骨折。父母非近亲婚配,二哥患同样疾病,已多次下肢骨折。查  相似文献   

7.
本症罕见,亦称播散性致密性骨斑症、斑驳状骨脆病、周围性脆性骨硬化症等。1905年由Stieda首先描述,1915年Albers——Schonberg报告典型病例,1916年由Ledoux命名为骨斑点症。国内报道较少,现将我们发现的一例报告如下。 陈某,女,29岁,行输卵管造影时发现骨盆诸  相似文献   

8.
家族性致密性骨发育不全(附二家族四例分析)曹庆选惠生才赵岗致密性骨发育不全系家族性遗传性骨病,甚为少见。我们搜集到来自两个家族的兄弟俩和姐弟俩共4例,表现典型。例1男,13岁。自幼身材矮小,因生长缓慢而就诊。体检:身高1.25m,体重25kg,头大面...  相似文献   

9.
颅骨—锁骨发育不全症,又称遗传性颅骨锁骨发育不全,骨-牙形成障碍,全身性骨发育障碍,骨盆、颅锁骨发育不全。我们发现一家族3个病例,报道如下: 例1,男,15岁。生后发现两肩内收隆起,活动范围  相似文献   

10.
颅骨锁骨发育不全的影像学诊断   总被引:1,自引:0,他引:1  
颅骨锁骨发育不全是一种少见的先天性全身骨胳发育不全性疾病[1]。1766年由Morand首先报道,1870年Cuffer又首先报道了家庭性的发病,Marie和Santon于1897年正式命名为锁颅骨发育不全症。我们收集9例,其中散发3例,家庭性发病6例,现就该病的影像学改变分析如下。1材料与方法9例中男6例,女3例,男女之比为2:1。年龄最小2岁,最大34岁。3例无明显家庭遗传史,6例来自3个家庭,即父亲和儿子,父亲和女儿,母亲和儿子。9例前囱未闭,颅缝增宽,身材较同龄人矮小,头面部比例失调,头大面小。6例眼距增宽,鼻梁塌陷。5例后囱未闭。8例锁骨…  相似文献   

11.
One of the factors of the successful military career guidance Cadet schools students is preserving and promoting their health. Medical support of children and adolescents aged 10-17 years should include the full range of medical and preventive measures defined for this group. The state of providing outpatient care for pupils at the Cadet School in St. Petersburg was studied. These results show that full medical care in accordance with the standards can be based only on children's health clinics. It is important that the organization of medical support pupils cadet schools should be cooperate with civilian health care.  相似文献   

12.
带状疱疹是由水痘—带状疱疾病毒引起的皮肤科常见疾病。其主要的病理损害,一是受累神经的严重炎症性浸润,继而导致受侵犯神经节内神经细胞变性、坏死;二是皮肤的水泡。迅速抑制神经节和相应的感觉神经纤维的充血、水肿和坏死,防止粘连形成,达到迅速镇痛、改善皮损,缩短病程及防止后遗症的发生是治疗的关键。因而,尽早明确诊断,  相似文献   

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ESR-spectrometry was used to investigate radiation-induced paramagnetic centers in enamel of mammals: carnivores (polar bear and fox), ungulates (reindeer, European bison, moose), and man. Values at half the microwave power saturation of the radiation signal, P1/2, evaluated at room temperature, was found to range from 16 to 26 mW for animals and man. A new approach to discrimination of the radiation induced signal from the total ESR spectrum of reindeer enamel is proposed. ‘Dose-response’ dependencies of enamel of different species mammals were measured within the dose range from 0.48 up to 10.08 Gy. Estimations of ‘radiosensitivity’ enamel of carnivores and ungulates showed good agreement with radiosensitivity enamel of man by ESR method.  相似文献   

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18.
The results of an international comparison of activity measurements of a solution of 55Fe organized by the BIPM in 2005 are reported and analysed. This exercise, which follows the procedures of the CIPM mutual recognition arrangement to update older comparisons, is a renewal of the comparison organized by the BIPM that took place in 1978. A EUROMET comparison was organized in 1996 specifically to compare activity measurements of a 55Fe solution by means of liquid-scintillation techniques. Results of these three comparisons are presented and discussed in this paper.

The radionuclide solution was provided by the NPL, which also distributed the samples to the participants. The activity of the ampoules was measured by 16 laboratories using 12 methods producing 25 results. Some general considerations on uncertainty assessments pertaining to the different techniques used are drawn. The outcome of four different estimators is compared from which the presence of at least one outlier can be confirmed. Further measurements should be made to try to reduce the discrepancy between the results. To date the outcome of the present comparison does not show an improvement to that of the 1996 comparison.  相似文献   


19.
A new method of non-surgical treatment of varicocele syndrome is described: it consists in sclerotherapy of spermatic vein by trans-femoral percutaneous catheterization with balloon-catheters. In 8 cases venous thrombosis has been induced by direct electric clotting. The techniques and a 6 months follow-up are discussed. It is pointed out that this procedure should be considered as the method of choice for tubular lesions and sub-fertility prophylaxis in young people and in childhood.  相似文献   

20.
目的探讨延迟性脾破裂误漏诊原因和预防措施.方法回顾性分析总结12例延迟性脾破裂中的诊断和误漏诊的经验与教训.结果本组延迟性脾破裂的误漏诊5例(41.66%).对多发伤与脾破裂并存可能认识不足,外伤史轻微或伤员隐瞒外伤史,缺乏腹痛-缓解-突然再腹痛的典型病史,缺乏“对冲性脾破裂”力学分析和整体化诊断思路等为其误漏诊的主要原因.结论详细的外伤史和全面系统检查,重视腹以外多发伤掩盖腹内脏器伤及延迟性脾破裂可能.确立外伤-腹内脏器伤-脾破裂整体化诊断思路.不间断地辅以B超检查脾形态学变化和腹内有无积液,腹腔穿刺确定有无血腹、X线胸腹部检查观察左侧胸肋角和膈肌运动情况、必要时CT检查以尽早发现脾包膜下血肿,降低延迟性脾破裂误漏诊率.  相似文献   

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