经股静脉行肝内门-体静脉分流插管术的回归方程及临床意义

目的　为临床经股静脉肝内门 体静脉分流插管术提供解剖学依据。方法　在 4 5具成人尸体上观测了双侧股静脉穿刺点至肝中静脉的长度 ,与胸骨颈静脉切迹到耻骨联合上缘的距离作相关回归分析。结果　从左侧股静脉穿刺点至肝中静脉的长度为 (3 9 83± 3 87)cm ,直线回归方程为 ^y =3 0 9± 0 71x;P <0 0 2 5 ;从右侧股静脉穿刺点至肝中静脉的长度为(3 8 4 9± 3 60 )cm ,直线回归方程为 ^y =3 0 3± 0 67x ;P <0 0 1;左、右侧股静脉与髂外、髂总静脉的夹角分别为 163 2 2°± 5 5 7°和 166 0 0°± 5 10° ,左、右侧髂外、髂总静脉与下腔静脉的夹角分别为 14 6 4 4°± 9 0 7°和 15 8 0 0°± 5 2 3°。结论　经右侧股静脉插管较左侧更为有利 ,可根据方程计算出从股静脉穿刺点到肝中静脉的长度     

The three fetal shunts: A story of wrong eponyms

The fetal circulatory system bypasses the lungs and liver with three shunts. The foramen ovale allows the transfer of the blood from the right to the left atrium, and the ductus arteriosus permits the transfer of the blood from the pulmonary artery to the aorta. The ductus venosus is the continuation of the umbilical vein, allowing a large part of the oxygenated blood from the placenta to join the supradiaphragmatic inferior vena cava, bypassing the fetal liver and directly connecting the right atrium. These structures are named after the physicians who are thought to have discovered them. The foramen ovale and the ductus arteriosus are called the “foramen Botalli” and the “ductus Botalli,” after Leonardo Botallo (1530–c. 1587). The ductus venosus is styled “ductus Arantii” after Giulio Cesare Arantius (1530–1589). However, these eponyms have been incorrectly applied as these structures were, in fact, discovered by others earlier. Indeed, the foramen ovale and the ductus arteriosus were described by Galen of Pergamon centuries earlier (c. 129–210 AD). He understood that these structures were peculiar to the fetal heart and that they undergo closure after birth. The ductus venosus was first described by Andreas Vesalius (1514–1564) 3 years before Arantius. Therefore, the current anatomical nomenclature of the fetal cardiac shunts is historically inappropriate.     

Clinical outcomes of transjugular intrahepatic portosystemic shunt for portal hypertension: Korean multicenter real-practice data

Background/Aims

This retrospective study assessed the clinical outcome of a transjugular intrahepatic portosystemic shunt (TIPS) procedure for managing portal hypertension in Koreans with liver cirrhosis.

Methods

Between January 2003 and July 2013, 230 patients received a TIPS in 13 university-based hospitals.

Results

Of the 229 (99.6%) patients who successfully underwent TIPS placement, 142 received a TIPS for variceal bleeding, 84 for refractory ascites, and 3 for other indications. The follow-up period was 24.9±30.2 months (mean±SD), 74.7% of the stents were covered, and the primary patency rate at the 1-year follow-up was 78.7%. Hemorrhage occurred in 30 (21.1%) patients during follow-up; of these, 28 (93.3%) cases of rebleeding were associated with stent dysfunction. Fifty-four (23.6%) patients developed new hepatic encephalopathy, and most of these patients were successfully managed conservatively. The cumulative survival rates at 1, 6, 12, and 24 months were 87.5%, 75.0%, 66.8%, and 57.5%, respectively. A high Model for End-Stage Liver Disease (MELD) score was significantly associated with the risk of death within the first month after receiving a TIPS (P=0.018). Old age (P<0.001), indication for a TIPS (ascites vs. bleeding, P=0.005), low serum albumin (P<0.001), and high MELD score (P=0.006) were associated with overall mortality.

Conclusions

A high MELD score was found to be significantly associated with early and overall mortality rate in TIPS patients. Determining the appropriate indication is warranted to improve survival in these patients.     

The role of transjugular intrahepatic portosystemic shunt in patients with portal hypertension: Advantages and pitfalls

Transjugular intrahepatic portosystemic shunt (TIPS) is an effective interventional procedure to relieve portal hypertension, which is a main mechanism for the development of complications of liver cirrhosis (LC), such as variceal hemorrhage, ascites, and hepatorenal syndrome. However, the high incidence of adverse events after TIPS implementation limits its application in clinical practice. Esophageal variceal hemorrhage is one of the major indications for TIPS. Recently, preemptively performed TIPS has been recommended, as several studies have shown that TIPS significantly reduced mortality as well as rebleeding or failure to control bleeding in patients who are at high risk of treatment failure for bleeding control with endoscopic variceal ligation and vasoactive drugs. Meanwhile, recurrent ascites is another indication for TIPS with a proven survival benefit. TIPS may also be considered as an effective treatment for other LC complications, usually as an alternative therapy. Although there are concerns about the development of hepatic encephalopathy and hepatic dysfunction after TIPS implementation, careful patient selection using prognostic scores can lead to excellent outcomes. Assessments of cardiac and renal function prior to TIPS may also be considered to improve patient prognosis.     

经颈静脉肝内门体静脉分流术对脾切除术后门静脉高压患者治疗的有效性与安全性

目的:分析经颈静脉肝内门体静脉分流术(transjugular intrahepatic portosystemic shunt,TIPS)对行脾切除术后的门静脉高压治疗的有效性与安全性.方法:选取2005年5月至2010年5月于空军总医院放射介入科接受TIPS治疗的68名行脾切除术的门静脉高压患者为病例组,以同期接受TIPS治疗的未行脾切除术的门静脉高压患者68例为对照组,分析比较两组患者的手术成功率、治疗前后肝功能、血小板及门静脉压力的变化情况,并记录两组患者不良事件的发生情况.结果:病例组TIPS成功率为97.06％(66/68),对照组TIPS成功率为100％(68/68),两组比较差异无统计学意义(x2=0.04,P=0.15);两组患者手术前、后肝功能及血小板计数比较差异无统计学意义;病例组与对照组术后PLT计数分别降至45.4±8.6,59.4±15.8,差异有统计学意义(P＜0.05);病例组术后ALB降至29.8±6.3,差异有统计学意义(P＜0.05);两组患者手术前后门静脉压力比较无统计学差异,术后病例组与对照组分别降至27.3±5.4,28.5±4.3,差异有统计学意义(P＜0.05);随访观察12～60个月,病例组分流道失效率病例组高于对照组(22.73％ vs.8.82％,P=0.04),再出血率及肝性脑病发生率比较,差异无统计学意义.结论:已行脾断流术的患者接受TIPS治疗,仍可获得满意的临床疗效,但术后发生分流道失效的风险较高,因此临床应用时应注意前瞻性预防,以获得较满意的效益安全比.     

Non‐invasive diagnosis and metabolic consequences of congenital portosystemic shunts in C57BL/6 J mice

This study demonstrates the suitability of magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) for the imaging of congenital portosystemic shunts (PSS) in mice, a vascular abnormality in which mesenteric blood bypasses the liver and is instead drained directly to the systemic circulation. The non‐invasive diagnosis performed in tandem with other experimental assessments permits further characterization of liver, whole‐body and brain metabolic defects associated with PSS. Magnetic resonance measurements were performed in a 26‐cm, horizontal‐bore, 14.1‐T magnet. MRA was obtained with a three‐dimensional gradient echo sequence (GRE; in‐plane resolution, 234 × 250 × 234 μm³) using a birdcage coil. Two‐dimensional GRE MRI with high spatial resolution (in‐plane resolution, 100 × 130 μm²; slices, 30 × 0.3 mm) was performed using a surface coil. Brain‐ (dorsal hippocampus) and liver‐localized ¹H magnetic resonance spectroscopy (MRS) was also performed with the surface coil. Whole‐body metabolic status was evaluated with an oral glucose tolerance test (OGTT). Both MRA and anatomical MRI allowed the identification of hepatic vessels and the diagnosis of PSS in mice. The incidence of PSS was about 10%. Hepatic lipid content was higher in PSS than in control mice (5.1 ± 2.8% versus 1.8 ± 0.6%, p = 0.02). PSS mice had higher brain glutamine concentration than controls (7.3 ± 1.0 μmol/g versus 2.7 ± 0.6 μmol/g, p < 0.0001) and, conversely, lower myo‐inositol (4.2 ± 0.6 μmol/g versus 6.0 ± 0.4 μmol/g, p < 0.0001), taurine (9.7 ± 1.2 μmol/g versus 11.0 ± 0.4 μmol/g, p < 0.01) and total choline (0.9 ± 0.1 μmol/g versus 1.2 ± 0.1 μmol/g, p < 0.001) concentrations. Fasting blood glucose and plasma insulin were lower in PSS than in control mice (4.7 ± 0.5mM versus 8.8 ± 0.6mM, p < 0.0001; and 0.04 ± 0.03 μg/L versus 0.3 ± 0.2 μg/L, p = 0.02, respectively). Glucose clearance during OGTT was delayed and less efficient in PSS mice than in controls. Thus, given the non‐negligible incidence of PSS in inbred mice, the undiagnosed presence of PSS will, importantly, have an impact on experimental outcomes, notably in studies addressing brain, liver or whole‐body metabolism.     

Direct intrahepatic portocaval shunt through transhepatic puncture via retrohepatic inferior vena cava: applied anatomical study

Background  DIPS is to create a portosystemic shunt directly between the portal vein and the retrohepatic inferior vena cava (RIVC) without passing through the hepatic vein. It has been recommended that the DIPS could be applied when routine TIPS is unsuccessful or the patient has anatomical variations of the hepatic vein. The aim of this study was to identify the safe area of the RIVC where the DIPS can be safely established. Materials and methods  The lengths of the safe and unsafe areas of the RIVC were measured. The tributaries of the RIVC were examined. The diameter of these tributaries was measured and their incidence and relation to the safe area of the RIVC were observed. The puncture distances of DIPS and TIPS were measured and compared. Results  The liver together with the RIVC was collected from 31 adult cadavers (age 32–65 years; M/F 25/6). 1. The safe and unsafe areas of the RIVC: the total length of the RIVC was 70.1 ± 13.0 mm (33.1–92.0 mm), whereas the length of the safe area of the RIVC was 54.3 ± 12.3 mm (20.2–71.1 mm), which was about over 70% of the total length. The length of the unsafe area at the upper end was 5.9 ± 1.8 mm (3.0–10.2 mm), and at the lower end was 8.9 ± 2.9 mm (3.1–20.0 mm). 2. The tributaries of the RIVC: In about 90% of the cadavers (90.3%; 28 out of 31), the LHV and MHV had the common trunk. The other three cadavers (9.7%; 3 out of 31) had independent RHV, MHV and LHV. There were 217 of small hepatic veins draining into the lower segment of the RIVC. Over 70% of the small hepatic veins were smaller than 5 mm in diameter and distributed on the anterior and left wall of the lower RIVC. 3. Puncture distances of the DIPS and TIPS: The distances from the bifurcation of the portal vein to the RIVC, to the right and to middle hepatic veins were 31.2 ± 7.9 mm (15.0–47.2 mm), 38.6 ± 8.1 mm (17.2–59.0 mm), and 46.6 ± 8.2 mm (34.0–68.1 mm), respectively. Thus, the puncture distances via the RIVC, RHV and LHV were significantly different (P < 0.001). The puncture distance of the DIPS was shortest. Conclusion  Anatomically, DIPS is a feasible interventional procedure to make a intrahepatic shunt between IVC and portal vein directly, and has its anatomical advantages compared to TIPS.     

Transesophageal echocardiography guided patent ductus arteriosus occlusion in adults with severe pulmonary hypertension through a parasternal approach

Between April 2010 and April 2014, 39 consective adult patients (> 18 years) with PDA associated severe pulmonary hypertension underwent transesophageal echocardiography guided patent ductus arteriosus occlusion through a parasternal minimally invasive approach. Among 39 patients, the procedure was successful in 32 cases (82.1%) and failed in 7 cases (17.9%). In the failed cases, 3 cases had a large residual shunt and 4 cases had persistent pulmonary hypertension. The mean minimum miameter of the successfully closed PDAs was 15.2 ± 2.1 mm (range 9 to 24), and the mean diameter of the mushroom-shaped occluder was 17.5 ± 2.5 mm (range 11 to 26). The pulmonary artery pressure decreased significantly after occlusion (P < 0.05), but there were no significant differences in the aortic pressure and blood oxygen saturation before and after occlusion (P > 0.05). Echocardiography performed on the first postoperative day showed decreased volume within the left atrium, left ventricle, and pulmonary artery in 23 cases, decreased volume within the left atrium and left ventricle in 4 cases, and no change in the volume of the atrium and ventricle in 3 cases. A minor residual shunt was observed in 6 cases. The posteroanterior chest X-ray showed improved pulmonary congestion in all cases and significantly reduced cardiothoracic ratio in 25 cases. Patients were followed-up at least for 1 year. No symptoms including palpitation, dyspnoea, or chest tightness were observed. The heart function ranged from NYHA class I to II. A minor residual shunt was observed only in one case. There were varying degrees of decrease in volume within the atrium and ventricle. In conclusion, transesophageal echocardiography guided patent ductus arteriosus occlusion through a parasternal minimally invasive approach is a feasible and effective method for the treatment of PDA in adults with severe pulmonary hypertension.     

Anatomical and experimental study of the ductus venosus

Summary The authors report an anatomical study of the ductus venosus in the stillborn fetus based on resin-corrosion casts and angiography. Study in adult material was done by dissection. In the first part of this paper the classical anatomy of the ductus venosus is described and an anomalous case of a hepatic parenchymal bridge between the left and caudate lobes of the liver is reported. In the second part of this paper study of patency and dilation of the ligamentum venosum to a diameter exceeding 10 mm in adult cadavers is presented. A brief study of portal flow demonstrated that an increase in flow of 30% can be obtained by experimental induction of patency. A patent ligamentum venosum was grafted onto the abdominal aorta in the rabbit. The transplanted segment was fully patent and competent immediately after grafting, but showed thrombosis when the animal was sacrified two months after the operation. In the opinion of the authors, it may be possible to use the patent ductus venosus in man once the problems of long term resistance and patency have been solved. The latter problems are currently under investigation in our laboratory.

---

Etude anatomique et expérimentale du canal d'Arantius ou ductus venosus

Résumé Les auteurs rapportent une étude anatomique du canal d'Arantius chez le ftus mort-né, à partir de moulages et d'angiographies, et chez l'adulte à partir de dissections. Ils rappellent l'anatomie classique du Canal d'Arantius et rapportent un cas de pont parenchymateux hépatique entre le lobe gauche et le lobe de Spiegel. Dans une deuxième partie ils réalisent des reperméabilisations et des dilatations jusquá un diamètre de plus de 10 mm sur des cadavres d'adulte. Une courte étude débitmétrique a montré la possibilité d'augmenter le débit portal de 30 %. Un ligament veineux reperméabilisé a été utilisé comme greffon sur une aorte abdominale de lapin. Perméable et étanche d'emblée, il est retrouvé thromboséá deux mois. Les auteurs pensent possible l'utilisation du ligament veineux reperméabilisé chez l'homme lorsqu'auront été résolus les problèmes de résistance et de perméabilité à long terme. Une étude est en cours.

     

遗传及先天性疾病微机咨询系统的研制及临床应用

计划生育、优生优育、控制人口数量、提高民族素质，已受到广泛重视，遗传及先天性疾病的防范及诊治殊属必要。由于此类疾病多达数千种，在普查及咨询中极为困难。为此我们将２２１２种遗传及先天性疾病输入微机，包括染色体异常、神经精神、肌肉、关节、循环、呼吸、消化、代谢、内分泌、泌尿生殖、血液、免疫、眼耳鼻喉、口腔、皮肤等系统及先天异常综合征，每种疾病包括异名、英文名称、症状体征、辅助诊断结果、病因，发病机理、遗传方式、再发风险、治疗、预防及预后。设计检索条件１６５２４条，对症状、体征、辅助诊断方法及疾病名称进行检索，可应用于疾病检索、症状体征诊断及鉴别诊断、辅助诊断方法结果查询。可广泛应用于遗传病流行学调查、遗传病诊断及鉴别诊断，门诊及书面咨询、教学、科研等，是一个遗传病的巨大资料库和数据库。微机采用ＩＢＭ２８６、３８６、４８６系列及各种兼容机，可普及到综合医院、计生站或家庭个人。     

The genetics of isolated congenital heart disease

The genetic mechanisms underlying congenital heart disease (CHD) are complex and remain incompletely understood. The majority of patients with CHD have an isolated heart defect without other organ system involvement, but the genetic basis of isolated CHD has been even more difficult to elucidate compared to syndromic CHD. Our understanding of the genetics of isolated CHD is advancing in large part due to advances in next generation sequencing, and the list of genes associated with CHD is rapidly expanding. Variants in hundreds of genes have been identified that may cause or contribute to CHD, but a genetic cause can still only be identified in about 20–30% of patients. Identifying a genetic cause for CHD can have an impact on clinical outcomes and prognosis and thus it is important for clinicians to understand when and what to test in patients with isolated CHD. This chapter reviews some of the known genetic mechanisms that contribute to isolated inherited and sporadic CHD as well as recommendations for evaluation and genetic testing in patients with isolated CHD.     

Comparison of clinical characteristics between congenital fiber type disproportion myopathy and congenital myopathy with type 1 fiber predominance

Congenital myopathies are clinical and genetic heterogeneous disorders characterized by skeletal muscle weakness and specific structural changes in muscle fiber. Congenital myopathy with fiber type disproportion (CFTD) is an established disorder of congenital myopathy. CFTD is characterized by non-progressive childhood neuromuscular disorders with a relatively good prognosis and type 1 fiber predominance and smallness. Congenital myopathy with type 1 fiber predominance (CMT1P) is also a distinct entity of congenital myopathy characterized by non-progressive childhood neuromuscular disorders and type 1 fiber predominance without smallness. Little is known about CMT1P. Clinical characteristics, including dysmorphic features such as hip dislocation, kyphoscoliosis, contracture, and high arch palate, were analyzed along with laboratory and muscle pathologies in six patients with CMT1P and three patients with CFTD. The clinical manifestations of CFTD and CMT1P were similar. However, the frequency of dysmorphic features is less in CMT1P than in CFTD. Long term observational studies of CMT1P are needed to determine if it will change to another form of congenital myopathy or if CMT1P is a distinct clinical entity.     

A review of the anatomy and clinical significance of adrenal veins

The adrenal veins may present with a multitude of anatomical variants, which surgeons must be aware of when performing adrenalectomies. The adrenal veins originate during the formation of the prerenal inferior vena cava (IVC) and are remnants of the caudal portion of the subcardinal veins, cranial to the subcardinal sinus in the embryo. The many communications between the posterior cardinal, supracardinal, and subcardinal veins of the primordial venous system provide an explanation for the variable anatomy. Most commonly, one central vein drains each adrenal gland. The long left adrenal vein joins the inferior phrenic vein and drains into the left renal vein, while the short right adrenal vein drains immediately into the IVC. Multiple variations exist bilaterally and may pose the risk of surgical complications. Due to the potential for collaterals and accessory adrenal vessels, great caution must be taken during an adrenalectomy. Adrenal venous sampling, the gold standard in diagnosing primary hyperaldosteronism, also requires the clinician to have a thorough knowledge of the adrenal vein anatomy to avoid iatrogenic injury. The adrenal vein acts as an important conduit in portosystemic shunts, thus the nature of the anatomy and hypercoagulable states pose the risk of thrombosis. Clin. Anat. 27:1253–1263, 2014. © 2014 Wiley Periodicals, Inc.     

The phenotypic spectrum of congenital Zika syndrome

In October 2015, Zika virus (ZIKV) outbreak the Brazilian Ministry of Health (MoH). In response, the Brazilian Society of Medical Genetics established a task force (SBGM‐ZETF) to study the phenotype of infants born with microcephaly due to ZIKV congenital infection and delineate the phenotypic spectrum of this newly recognized teratogen. This study was based on the clinical evaluation and neuroimaging of 83 infants born during the period from July, 2015 to March, 2016 and registered by the SBGM‐ZETF. All 83 infants had significant findings on neuroimaging consistent with ZIKV congenital infection and 12 had confirmed ZIKV IgM in CSF. A recognizable phenotype of microcephaly, anomalies of the shape of skull and redundancy of the scalp consistent with the Fetal Brain Disruption Sequence (FBDS) was present in 70% of infants, but was most often subtle. In addition, features consistent with fetal immobility, ranging from dimples (30.1%), distal hand/finger contractures (20.5%), and feet malpositions (15.7%), to generalized arthrogryposis (9.6%), were present in these infants. Some cases had milder microcephaly or even a normal head circumference (HC), and other less distinctive findings. The detailed observation of the dysmorphic and neurologic features in these infants provides insight into the mechanisms and timings of the brain disruption and the sequence of developmental anomalies that may occur after prenatal infection by the ZIKV.

     

The placental lesions in congenital syphilis

Summary Placentas from six mothers with serological tests suggestive of recent syphilitic infection and whose babies were suspected of being or proven to be infected by Treponema pallidum are described. One placenta from this series was large, bulky and pale, while the other 5 were without remarkable gross features. In all cases, the associated histological lesions were 1) hypercellular areas in the terminal and stem villi and 2) a focal peri- and/or intravillous polymorphonuclear concentration with or without necrosis. The former change which was the most frequent was characterized by an apparent increase of villous stromal cells, ultrastructurally indentified as mesenchymal cells and Hofbauer cells. In addition, numerous fetal monocytes were found in the villous vascular lumina. The findings described here and in the literature suggest that congenital syphilis is associated with a spectrum of placental changes. We believe that these changes depend on the immunological reaction of the fetus. According to the sequence of events described in untreated patients, we distinguish two morphological phases: 1) an inductive phase without placental changes and 2) a reactive phase characterized by a predominantly lymphocytic inflammatory infiltration of the villi, followed by a reaction of mononuclear phagocytes.     

以胎儿睾丸为移植体的应用解剖

观多了50侧男性足月胎尸的睾丸血供。讨论了睾丸移植体的截取方法以及受体血管选择和手术切口的设计等。     

Vascular endothelial growth factor (VEGF) and VEGF receptor expression in biopsy samples of liver from dogs with congenital portosystemic shunts

Surgical attenuation of a congenital portosystemic shunt (CPSS) results in increased liver mass, development of intrahepatic portal vasculature and improved liver function. Vascular endothelial growth factor (VEGF) is a key regulator of angiogenesis. The aim of this study was to investigate the role of VEGF and its receptor in the hepatic response to CPSS surgery. The study included 99 dogs with CPSS treated with either partial or complete suture attenuation. Forty-four dogs with partial attenuation underwent a second surgery for complete attenuation. The expression of VEGF and VEGF receptor 2 (VEGFR2) in biopsy samples of liver was assessed by immunohistochemistry with rabbit anti-human VEGF polyclonal antibody and mouse anti-human VEGFR2 monoclonal antibody. Expression of these molecules was graded. The proportion of samples expressing VEGF was significantly greater in samples from dogs with CPSS compared with control samples (P=0.04) and the proportion of samples expressing VEGFR2 was significantly greater in control samples compared with samples from dogs with CPSS (P=0.04). VEGF labelling grade decreased significantly (P=0.038) and VEGFR2 increased significantly (P=0.046) between first and second surgery. The decrease in VEGF may reflect transient expression, preferential expression of other factors, reperfusion of existing vessels and/or increased angiogenesis before surgery in the form of arterialization and subsequent reduction due to improved portal blood flow. Partial suture attenuation was associated with a degree of 'normalization' of VEGF and VEGFR2 expression when compared with the control samples. Further investigation is needed to provide more information on the hepatic response to CPSS surgery.     

IgM rheumatoid factor in congenital syphilis: associations with clinical and laboratory findings.

IgM rheumatoid factor (RF), measured by means of an ELISA, was detected in 92% of infants with congenital syphilis. Elevated levels were found to correlate with liver and renal involvement as well as the extent of the disease (P less than 0.05). In addition, levels of circulating immune complexes were closely related to the RF concentration (P less than 0.001). Following treatment of the infants both RF levels and VDRL titres declined at a similar rate (P less than 0.001). These findings indicated a close relationship between the disease process and IgM RF levels. It is postulated that IgM RF may add to immune complex deposition and exacerbate tissue damage in congenital syphilis.