门静脉先天性缺如

作者报道一门静脉先天性缺如(CAPV)病例.患者女性,22岁,临床表现为易疲,肝大.肝功能检查:除GOT、GPT 轻度增高外,其余在正常范围.影像学检查(腹部平片、CT、US、血管造影)显示一极短的门静脉进入下腔静脉的肾上段,门静脉末端及其肝内分支缺如,而肠系膜上静脉、肠系膜下静脉及脾静脉联接正常.该患者同时伴有肝右叶局灶性结节增生(FNH),输尿管平滑肌瘤及脊柱侧突伴半椎体。门静脉在胚胎5—10     

膀胱缺如一例

惠者女,6岁,1980年5月20日入院。出生后即持续性漏尿,无正常排尿现象,会阴皮肤经常被尿浸蚀糜烂并出现湿疹。家族中未发现遗传病或先天性畸形。体检:发育正常,检查合作。会阴部可见大片皮肤潮红,有湿疹病变,可见尿道外口间断向外溢尿。余无异常。腰骶椎摄片:腰骶椎各椎体及附件无     

阑尾缺如1例

患者,女,79岁。因转移性右上腹疼痛16小时,其疼痛呈持续性,渐加重件恶心,未见呕吐。自觉畏寒且发烧,在家自服多酶片、解痉药,症状无缓解,以急性阑尾炎收入院治疗。既往无类似发作史。体检:体温37.8℃,右下腹肌紧张,麦氏点有明显压痛及反跳痛。结肠充气,腰大肌试验阳性。化验:白细胞12,300,中性77％。按急性阑尾炎行手术治疗。术中:见盲肠末端约3×2厘米的组织坏死,呈紫黑色。游离盲肠末端,沿结肠带,仔细寻找,并检     

阑尾缺如2例

1病例介绍 例1，张××，男54岁，右下腹痛1天，伴腹胀，并停止排气、排便，低热。无手术史。查体：T37．9℃，P90次／分，R20次／分BP115／75mmHg，痛苦貌，头颈正常，心肺无异常，心率135次／分，律不整，心音有力，腹胀，未见胃肠型，右下腹肌稍紧、压痛，肠鸣音减弱。血RBC483×10^12／L，WBC14．8×10^9／L，HB150g／L，NO．870，L0．128。X线透视：右中腹部肠管胀气，伴数个液平面。EKG：异位心律，房颤，心肌劳累，BUS：肝、胆、胰、脾、肾无异常。[第一段]     

胎儿左肺缺如合并右肺叶缺如超声表现1例

正孕妇,29岁,孕1产0。孕31周当地医院考虑左肺发育不良来就诊。超声检查:胎儿双顶径69mm,股骨长59mm,羊水量正常,胎儿左侧胸腔内未探及肺脏回声,心脏增大,完全占据左侧胸腔,心轴左偏,约为99°(图1),气管长轴切面仅见气管向右下侧延伸变细,未见左侧分支(图2),动态扫查始终未见左肺动脉,仅见右肺动脉及动脉导管结构,右肺增大,内部回声未见明显异常;心脏除合并永存     

超声诊断胎儿主动脉瓣缺如合并肺动脉瓣缺如1例北大核心CSCD

孕妇,33岁,孕2产1,身体健康,无遗传病史,孕11^＋3周来我院行孕11～14周超声筛查。超声检查：宫内孕,单活胎,头位,顶骶径4．6cm,胎儿颈项透明层厚度2.3mm。胎儿头颈部、腹部及肢体未见明显异常,胸部横断面显示心胸比率增大,彩色多普勒显示主动脉在心脏舒缩周期中往返的彩色血流信号（图1）。     

阑尾缺如1例报告

1．资料与方法 1．1一般资料 患者，男，藏族，28岁，因右下腹疼痛6小时入院。患者入院前出现持续性右下腹疼痛，阵发性加剧，伴恶心、呕吐、发热，病后大小便正常。患者既往有右下腹疼痛病史，反复发作3年，均未经治疗缓解。     

先天性胆囊缺如1例

1病例报告女,32岁。间歇性右上腹胀痛6a,外院多次超声检查诊断为胆囊结石,间断服用消炎利胆药物治疗,症状可以缓解。查体:皮肤及巩膜无黄染。腹部平坦,腹软,右上腹及剑下轻压痛。复查超声:肝内胆管不扩张,隐约可见胆囊,大小约3.2cm×1.8cm,胆囊腔小,壁毛糙,胆囊底部可见一1.4cm较强回声团块,无声影,胆总管上段内径0.57cm。印象:胆囊内强回声团块。积极术前准备,在全麻下行胆囊切除术。术中见横结肠与肝脏面正常胆囊床位置轻度粘连,分离粘连后,探查未见胆囊,胆总管及其周围结构清晰。完全解剖出肝外胆管,上至肝门暴露左右肝管,下达十二指肠上…     

先天性胼胝体缺如一例

靳××,男,28岁。患者于1982年元月自觉头疼,发烧,体温曾达38.5℃,以为感冒。10余天后突发抽搐,四肢抽动,翻白眼,口吐白沫,意识丧失历时约20分钟。其后一个月内又发作4次。头疼日益加剧,并有呕吐及双眼视物不清。平素健康,无特殊病史。     

Heterozygous deficiency of hypoxia-inducible factor-2alpha protects mice against pulmonary hypertension and right ventricular dysfunction during prolonged hypoxia

Chronic hypoxia induces pulmonary vascular remodeling, leading to pulmonary hypertension, right ventricular hypertrophy, and heart failure. Heterozygous deficiency of hypoxia-inducible factor-1alpha (HIF-1alpha), which mediates the cellular response to hypoxia by increasing expression of genes involved in erythropoiesis and angiogenesis, has been previously shown to delay hypoxia-induced pulmonary hypertension. HIF-2alpha is a homologue of HIF-1alpha and is abundantly expressed in the lung, but its role in pulmonary hypertension remains unknown. Therefore, we analyzed the pulmonary response of WT and viable heterozygous HIF-2alpha-deficient (Hif2alpha(+/-)) mice after exposure to 10% O(2) for 4 weeks. In contrast to WT mice, Hif2alpha(+/-) mice were fully protected against pulmonary hypertension and right ventricular hypertrophy, unveiling a critical role of HIF-2alpha in hypoxia-induced pulmonary vascular remodeling. Pulmonary expression levels of endothelin-1 and plasma catecholamine levels were increased threefold and 12-fold respectively in WT but not in Hif2alpha(+/-) mice after hypoxia, suggesting that HIF-2alpha-mediated upregulation of these vasoconstrictors contributes to the development of hypoxic pulmonary vascular remodeling.     

Using antibiotics responsibly: right drug, right time, right dose, right duration

Everyone prescribing antibiotics should consider both their clinical and public health responsibilities. The objective should be to provide optimal patient care while at the same time seeking to minimize selective pressure that may result in the emergence and spread of antibiotic resistance. To this end, in 2008 the European Centre for Disease Control initiated the annual European Antibiotic Awareness Day (EAAD) to take place on 18 November, when Europe-wide activities are undertaken to highlight the critical importance of prudent antibiotic prescribing. This year activities in England will focus on the optimal management of infections in secondary care, and will have two inter-related aims. The first is to improve the quality of the initial decision to prescribe an antibiotic (including making an informed choice of empirical drug and dose) in particular ensuring rapid prescribing and administration in presumed sepsis. This is deliberately combined with a second focus on the critical importance of formally reviewing antibiotic therapy at 48 h, based on the patient's clinical response and the availability of microbiology test results. This should lead to a clear decision to stop, switch to oral, switch to outpatient antibiotic therapy (OPAT) or change antibiotic, if possible to a narrower spectrum. The EAAD campaign in England will highlight the need to 'Start Smart-Then Focus'. The aim is that patients receiving antibiotics should receive the right drug at the right time at the right dose for the right duration.     

Sustained pulmonary hypertension and right ventricular hypertrophy after chronic hypoxia in mice with congenital deficiency of nitric oxide synthase 3.

Chronic hypoxia induces pulmonary hypertension and right ventricular (RV) hypertrophy. Nitric oxide (NO) has been proposed to modulate the pulmonary vascular response to hypoxia. We investigated the effects of congenital deficiency of endothelial NO synthase (NOS3) on the pulmonary vascular responses to breathing 11% oxygen for 3-6 wk. After 3 wk of hypoxia, RV systolic pressure was greater in NOS3-deficient than in wild-type mice (35+/-2 vs 28+/-1 mmHg, x+/-SE, P < 0.001). Pulmonary artery pressure (PPA) and incremental total pulmonary vascular resistance (RPI) were greater in NOS3-deficient than in wild-type mice (PPA 22+/-1 vs 19+/-1 mmHg, P < 0.05 and RPI 92+/-11 vs 55+/-5 mmHg.min.gram.ml-1, P < 0.05). Morphometry revealed that the proportion of muscularized small pulmonary vessels was almost fourfold greater in NOS3-deficient mice than in wild-type mice. After 6 wk of hypoxia, the increase of RV free wall thickness, measured by transesophageal echocardiography, and of RV weight/body weight ratio were more marked in NOS3-deficient mice than in wild-type mice (RV wall thickness 0.67+/-0.05 vs 0.48+/-0.02 mm, P < 0.01 and RV weight/body weight ratio 2.1+/-0.2 vs 1.6+/-0.1 mg. gram-1, P < 0.05). RV hypertrophy produced by chronic hypoxia was prevented by breathing 20 parts per million NO in both genotypes of mice. These results suggest that congenital NOS3 deficiency enhances hypoxic pulmonary vascular remodeling and hypertension, and RV hypertrophy, and that NO production by NOS3 is vital to counterbalance pulmonary vasoconstriction caused by chronic hypoxic stress.     

Getting the right ambulance to the right patient at the right time

This paper outlines recent developments in dispatching ambulances according to the clinical needs of the patient. Criteria Based Dispatch (CBD) uses accurate and effective interrogation of the caller, with reference to clinically approved guidelines, to ensure that the appropriate level of ambulance support is deployed. The pattern of calls in Glasgow, UK, was monitored in order to evaluate CBD in this context, and the potential benefits of adopting the system are summarised.