Ocular findings in familial adenomatous polyposis

Background: Retinal pigment epithelium (RPE)lesions are predictive congenital phenotypic markersfor familial adenomatous polyposis (FAP). Thisprospective screening study aims at assessing theincidence and significance of these lesions in FAPpatients and their family members.Methods: Sixty-two members from three familiesincluding five patients with the diagnosis of FAP havebeen ophthalmologically surveyed. All RPE lesions weredocumented with fundus photography and fluoresceinangiography was performed in 13 subjects.Sigmoidoscopy and/or radiological examination wereperformed annually in 9 family members with typicalRPE lesions during 4 years to allow early diagnosis ofFAP.Results: Typical RPE lesions were present infive FAP patients and 15 family members.Telangiectatic dilatations in the retinal peripherywith small dot-like hemorrhages were detected in 6subjects from 3 families These lesions wereparticularly evident on fluorescein angiography.Annual colon analysis showed polyps in 3 out of 9subjects who were positive for RPE lesions.Conclusion: RPE lesions are valuable as aclinical marker in predicting FAP. The co-existingperipheral vascular alterations which have not beenreported before, are probably related to FAP.     

Alteration of Human Placental Vascular Tone by Antiarrhythmic Medications In Vitro

Antiarrhythmic and Placental Vessels. Introduction : Antiarrhythmic medications are commonly used during pregnancy for treatment of maternal or fetal arrhythmias, but little is known about their effect on human placental vascular tone and, consequently, placental blood flow. The objective of this study was to evaluate the tone responses caused by antiarrhythmic medications in human placental vessels from normal term pregnancies in vitro.
Methods and Results : Isolated human placental arteries and veins from uncomplicated term pregnancies incubated in Krebs'-bicarbonate under 5% oxygen/5% carbon dioxide/balance nitrogen (PO₂ 35 to 38 torr) were exposed to cumulative doses of quinidine, procainamide, lidocaine, flecainide, propranolol, amiodarone, verapamil, digoxin, and adenosine after submaximal contraction with 5-hydroxytryptamine. The study was conducted both in the presence and absence of endothelium. The addition of the tested medications caused a significant, dose-dependent relaxation of human placental arteries and veins except for adenosine, which induced a sustained, dose-dependent contraction of human placental vessels regardless of the presence or absence of tone. Removal of the endothelium did not alter these responses.
Conclusions : Based on these results, the medications tested should have no decremental effect on placental blood flow, with the possible exception of adenosine, which causes significant. dosedependent contraction of human placental vessels in vitro. Should similar contraction be present in vivo, it may have an adverse effect on the fetus when administering adenosine to pregnant women at term or during labor.     

Experimental laparoscopic aortobifemoral bypass

The goal of the present study is to develop a technique for laparoscopic aortobifemoral bypass.Piglets weighing between 60 and 78 kg were anesthetized with halothane. The lateral retroperitoneal approach was preferred to the more familiar anterior transperitoneal approach and was successfully completed in 19 piglets. The piglets were placed in the right lateral decubitus position. The first port (2 cm) was inserted halfway between the tip of the 12th rib and the iliac crest. Four other trocars were placed in the retroperitoneum after balloon inflation had allowed creation of a space which permitted visualization of the aorta from the left renal artery down to the aorto-iliac junction. After evacuation of the retropneumoperitoneum, the cavity was maintained using an abdominal lift device and a retractor.Using this approach, we performed four aortobifemoral bypasses (end-to-end aortic anastomosis) after conventional intravenous heparinization (100 IU/kg) in less than 4 h. Blood loss did not exceed 250 ml and the hematocrit remained stable. Postmortem evaluation of the grafts revealed they were positioned as in a conventional bypass, their limbs having followed in the created retroperitoneal tunnels along the path of the native arteries. No mortality occurred before sacrifice of the animals. We believe that this first performed series of totally retroperitoneal laparoscopic aortobifemoral bypasses in the porcine model is useful in preparation for human application due to the anatomical similarities in the periaortic region.     

Bilateral Ulnar Agenesis: Case Report and Review of the Literature

Bilateral ulnar agenesis is a rare abnormality. A total of 36 cases are analyzed: 35 of these are documented in the literature and 1 stillborn male is presented in this study. Most patients had one of the three conditions: Al-Awadi/Raas-Rothschild syndrome, syndrome of ulnar aplasia with split hand/split foot deformity, or the Brachmann—de Lange syndrome. Fifty percent of all cases with bilateral ulnar agenesis were associated with lower limb defects and these cases, for the most part, also belonged to the aforementioned syndromes. Nonskeletal, internal organ malformations were identified in 34% of all patients. Nine patients presented with isolated bilateral ulnar agenesis. The Al-Awadi/Raas-Rothschild syndrome and the split hand/split foot deformity are heritable disorders. There was no evidence for genetic etiology in most of the other cases. Bilateral ulnar agenesis in our fetus was part of the Brachmann—de Lange syndrome with associated cardiac defect, diaphragmatic hernia, and umbilical artery agenesis.     

床上体操训练对偏瘫患者的效果观察

目的：观察偏瘫患者早期床上训练的效果。方法：对５８例脑血管意外致偏瘫患者随机分成２组：观察组３０例，护理中指导早期床上体操训练；对照组２８例，按常规护理。以上、下肢肌力和日常生活活动能力（ＡＤＬ）为评价指标，于训练开始时、训练４周、训练８周各评价１次。结果：观察组训练４周、８周时患侧上肢肌力以及ＡＤＬ水平明显增加，与对照组比较有显著差异（Ｐ＜００１）。患侧下肢肌力训练４周及８周时均增加，与对照组比较，４周时无明显差异（Ｐ＞００５），８周时有显著性差异（Ｐ＜００１）。结论：对早期偏瘫患者实施床上功能训练，不仅可以提高患者的生命质量，而且可以减少、甚至解除患者及家属的经济和精神负担。     

RELAXATION OF MESENTERIC ARTERY OF STROKE PRONE SPONTANEOUSLY HYPERTENSIVE RATS BY CALCIUM REMOVAL

1. The time courses of the relaxation, induced by removal of extracellular Ca2+, of K-depolarized mesenteric artery preparations from stroke prone spontaneously hypertensive rats (SHRSP) and Wistar-Kyoto rats (WKY) were compared. 2. The time course of the decline in extracellular Ca2+ was estimated from the time course of the relaxation and the concentration-response curve of K(+)-depolarized preparations to Ca2+. The time course of the decline in the intracellular free Ca2+ concentration was also estimated from the reported relation between Ca2+ concentration and the contraction of skinned vascular smooth muscle. 3. The time course of relaxation was exponential, the curve being made up of three components. The time course was slower in preparations from SHRSP, especially the first component of the relaxation curve. 4. The time courses of the decline in the intracellular and extracellular Ca2+ concentrations were also exponential, being made up of three components and were also slower in the preparation made from SHRSP. 5. The wall and muscle layer of the mesenteric arteries used in the present experiments were significantly thicker in the SHRSP preparations. 6. Calculation of the half relaxation time, based on the diffusion of Ca2+ across the blood vessel wall, suggested that the slower relaxation in preparations from SHRSP is due largely to the thicker muscle layer, although differences in Ca2+ sequestration by the smooth muscle cells may also be involved.     

Posterior cranial fossa dimensions in the Chiari I malformation: Relation to pathogenesis and clinical presentation

Summary Skull dimensions were measured on lateral skull radiographs in 33 adult patients with MRI-verified Chiari I malformations and in 40 controls. The posterior cranial fossa was significantly smaller and shallower in patients than in controls. In the patients, there was a positive correlation between posterior fossa size and the degree of the cerebellar ectopia, which might indicate that a posterior cranial fossa which was originally too small had been expanded by the herniation of hindbrain structures at an early stage. Pyramidal signs and cerebellar symptoms and signs, which may be due to compression of neural structures, were associated with a large degree of ectopia and a relatively large posterior cranial fossa. Syringomyelia and headache, which may be due to the valve action of the herniated cerebellar tissue, were not associated with a particularly large posterior fossa or herniation. No special clinical presentation was associated with a very small posterior cranial fossa, which may indicate that a small posterior cranial fossa per se has little or no clinical significance, although it may be the primary developmental anomaly.     

复合带瓣人工血管替换升主动脉和主动脉瓣(24例报告)

报告２４例升主动脉瘤伴主动脉瓣关闭不全行升主动脉和主动脉瓣替换及冠状动脉开口移植术（Ｂｅｎｔａｌ术２２例，底盘法２例）。１３例伴有升主动脉夹层分离，对其中８例ＤｅＢａｋｅｙＩ型者，以Ｔｅｆｌｏｎ毡条内外加固主动脉切端后吻合。１３例用人工血管周围间隙与右心耳吻合以控制升主动脉吻合之外的出血。３例以人工血管片环包主动脉吻合口控制局部广泛渗血及出血。２例术毕不能脱离体外循环死亡，手术死亡率８３％。随访平均２１８个月，２例死于蛛网膜下腔出血，１例右股动脉栓塞经手术治愈；其余病人康复良好，心功能（ＮＹＨＡ）Ｉ～ＩＩ级。     

小血管套叠粘接吻合法在血液透析者中建立动静脉内瘘的应用

慢性肾病患者常需要建立动静脉内瘘进行透析，常规间断缝合法吻合动静脉难度大、耗时长。为了缩短吻合时间，提高远期通畅率，进行了套叠粘接吻合法建立动静脉内瘘的实验研究，并将这一方法应用于透析患者建立动静脉内瘘的血管吻合。将动脉端套入静脉端，只需吻合两针，用医用胶封闭吻合口。经８个月观察，通畅率达１００％，血流量均超过３００ｍｌ／ｍｉｎ。达到了透析需要。     

Basilar arterio-venous pseudoparallelism due to persistence of embryonal venous pattern

Summary A vascular malformation, consisting of a venous vessel bridgeing the right inferior petrosal sinus and the anterior spinal veins, was found in the posterior fossa. The vessel presented a ring-like course around the right trigeminal root, and it was parallel and dorsal to the basilar artery. The malformation was associated with cutaneous and hepatic angiomas and peri-osteal lipomas. It had been clinically silent for 52 years, when it thrombosed causing death. The authors think that, within a general mesenchymopatic state, this is a result of the persistence of an embryonal cerebral venous pattern.