47,XXY克氏综合征91例临床分析

总结１９７４～１９９１年间９１例４７，ＸＸＹ克氏综合征的临床体征，发现腋毛和胡须减少或消失，下体长，以及小而软的睾丸是本病的重要体征。患者的阴茎虽短，但仅１／３患者性生活不正常。对本组病例和国外文献报告的病例作一比较并列举体征上的差异。     

Diagnostic features of the fat embolism syndrome.

A series of 444 patients with multiple blunt injuries were analysed with respect to the incidence of symptoms and signs generally described in connection with the fat embolism syndrome. During hospital treatment, a diagnosis of fat embolism was established for 47 patients and suspected in 23 patients. In retrospect, the latter patients had a morbid picture similar to that of the former. Hypoxia, anemia, disturbances of consciousness, and hyperpyrexia were constant signs. A high incidnece of petechiae and snowstorm infiltrations of the lung was also found. These six signs showed a marked tendency to combine; and placed the fat embolism patients in a distinctive group. The most susceptible patients were young adults with diaphyseal fractures of the lower extremities. Hypoxia was the most important early sign and the most important factor in terms of treatment. We conclude that the fact embolism syndrome is a clinical entity and that its diagnosis can be made on the basis of the symptom complex.     

Experience of application of lymphotropic methods in the treatment of lower extremity veins]

Experience of treatment of 368 patients with disease of veins of lower extremities was summarized. In patients with an acute thrombophlebitis and chronic venous insufficiency of lower extremities significant disorders of microhemo- and lymphatic circulation, manifesting as venous or lymphatic stasis, were noted. Conduction of lymphotropic therapy have promoted microcirculation improvement, elimination of interstitial oedema and signs of inflammation, permitting to shorten the stationary treatment period.     

Neutrophil Response to Replantation of Large Human Extremities

Abstract Background: Polymorphonuclear leukocytes (PMN) play a pivotal pathogenic role in ischemia/reperfusion injury of various tissues. The aim of the present study was to investigate the effect of replantation of large extremities on the function of circulating PMN in human patients. Patients and Methods: PMN were isolated from whole blood up to 90 min after vessel repair and reperfusion. PMN superoxide anion production was measured by a cytochrome C reduction assay. Ten patients with amputations of the leg (n = 2), lower leg (n = 5), upper arm (n = 2), forearm (n = 1), and three subtotal amputations of the lower leg with severe vessel damage were enrolled. Results: In four of six reamputated patients, total time of limb ischemia exceeded 5 h and PMN superoxide production was substantially increased at 60 min after reperfusion. With successful replantation, time of ischemia was < 5 h and PMN superoxide production did not further increase during reperfusion. Conclusion: The neutrophil response to replantation of large extremities is associated with the time of ischemia which may be involved in multiorgan dysfunction syndrome observed in some of these patients.     

Compression syndromes in chronic venous insufficiency of lower extremities

Results of examination and surgical treatment of 77 patients with severe forms of chronic venous and lymphovenous insufficiency of lower extremities were analyzed. The compression syndrome concept and its role in pathogenesis of severe forms of chronic venous and lymphovenous insufficiency of lower extremities were determined. There was elaborated the tactics of complex treatment of patients, application of which had permitted to achieve the trophic ulcers healing in 98% operated patients, previously considered noncurable.     

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目的 下肢静脉曲张及下肢深静脉功能不全病人的左髂总静脉（LCIV）病变的发病情况及其之间的关系。以指导临床治疗。方法 连续为73例（100条患肢）下肢静脉曲张病人行术前患肢深静脉逆行造影及LCIV造影。结果 （1）LCIV总异常率为47.9%,在有左侧下肢静脉曲张的病人中有31例(59.6%),在只有右侧下肢静脉曲张的病人中,仅4例（19.4%),两组间的LCIV异常的比率差异有显著意义（P=0.0017)。LCIV异常包括髂腔静脉交界处压迹,不同程度的狭窄,增宽,充盈缺损和侧支形成。（2）下肢深静脉功能不全67条肢体,占67.0%,在左LCIV造影异常的病斧正中,左侧下肢深列脉Ⅲ或Ⅳ级逆流者14例,在左LCIV造影正常的病例中,左侧下肢深静脉有Ⅲ或Ⅳ级逆流者仅6例,二者间差异也有显著意义（P=0.0205)。结论 Cockett综合征可能是左下肢静脉曲张的原因之一。同时也是左下肢深静脉功能不全的原因之一。     

Post-transplant distal-limb bone-marrow oedema: MR imaging and therapeutic considerations.

BACKGROUND: In recent years a previously unrecognized pain syndrome of the distal lower limbs after organ transplantation has been noted. A relationship to cyclosporin A was suspected, but no clear aetiology and pathogenesis have been established. METHODS: During the last 30 months we diagnosed the pain syndrome in 10 patients after renal transplantation. We prospectively followed and evaluated the patients during their clinical courses and through pathological laboratory findings and magnetic resonance imaging (MRI). RESULTS: In all patients symptoms developed within 6 months of transplantation after otherwise uncomplicated clinical courses without graft rejection episodes. Impressive bone-marrow oedema on MRI as well as elevated serum alkaline phosphatase was seen in all patients, and often exceeded the duration of clinical symptoms. All patients were instructed to avoid stress to the extremities through immobility, and steroid doses were tapered down. Within 14 weeks, eight patients were free of symptoms. Two patients have not experienced remission after 3 and 4 months respectively. None of the patients developed signs of osteonecrosis. CONCLUSION: Post-transplant distal limb bone-marrow oedema presents with distinct clinical findings and signs of bone-marrow oedema on MRI. Proven standard treatment does not exist. In our experience the elevation of the extremities, the strict avoidance of physical strain, and a stepwise withdrawal of steroids facilitates progressive disappearance of symptoms. Long-term damage to the affected ostial structures has not been seen, in contrast to avascular femoral-head necrosis.     

Surgical treatment of postop large size abdominal hernia in patients with obesity]

There were 140 patients with morbid obesity operated on for postoperative abdominal hernia. In 2 (1.4%) patients an acute cardiopulmonary insufficiency occurred, and in another 2 (1.4%) an acute thrombophlebitis of the lower extremities veins. Two patients died. The hernia recurrence have occurred in 3 (2.1%) patients operated according to Mayo method in terms from 1 to 5 years later.     

The severity of varicose vein disease of the lower extremities in various degree of the connective tissue dysplasia syndrome

There were examined 80 patients with varicose disease of the lower extremities veins (VDLEV) using elaborated phenotypic cart, the Bayton scale, ECG, echocardiography and ultrasonic triplex phleboscanning. There were defined 24 markers of the connective tissue dysplasia, mostly characteristic for VDLEV. Correlation was established between the phenotypic signs number, the severity of clinical course and the rate of occurrence of the venous diseases complications. Moderate and severe clinical course of VDLEV is associated with various phenotypic markers of the connective tissue dysplasia.     

Caudal regression: a review of seven cases, including the mermaid syndrome

Caudal regression consists of sacral agenesis combined with variable lower extremity deformities. The patients have contractures of the lower extremities, hip dislocations, neurologic impairment, and spine instability. Treatment consists of soft tissue releases, osteotomies, or orthotics. The goal of orthopaedic intervention is proper seating and standing, which may be achieved without amputation. In our patients, the lower extremity deformities were corrected surgically, but long-term function is unknown. We report the problems and treatment of seven patients with caudal regression syndrome, including the only known survivor with sirenomelia (mermaid syndrome), who underwent separation of the lower extremities.     

Long-term physical outcome of patients who suffered crush syndrome after the 1995 Hanshin-Awaji earthquake: prognostic indicators in retrospect.

BACKGROUND: The 372 cases of crush syndrome that followed the 1995 Hanshin-Awaji earthquake have provided a unique opportunity to investigate the long-term physical outcomes and to establish indications for specific treatments in such patients. The objectives of this study were to identify independent predictors of physical outcome in patients suffering from crush syndrome and to clarify the influence of fasciotomy on outcomes. METHODS: Sensory and motor functions were examined 2 years after the earthquake in 42 patients with a total of 58 compressed lower extremities. The influences of time to rescue, fasciotomy, and radical debridement on lower leg muscle strength were evaluated by stepwise regression analysis. Correlation between the time to fasciotomy and lower leg muscle strength was also analyzed. RESULTS: Severe disabilities related to the lower leg compartment were present in 47% (8/17) of patients who underwent fasciotomy and in 16% (4/25) of patients who did not. The anterior compartment was damaged more severely than the posterior compartment. Severe sensory and motor disturbances occurred at a higher rate in relation to anterior and posterior compartments that were treated by fasciotomy than in relation to those that were not. Stepwise regression analysis showed fasciotomy/debridement score to be an independent predictor of long-term lower leg muscle strength (R = 0.67, p < 0.0001) and showed time to rescue to be an independent predictor when debrided compartments were not included in the analysis (R = 0.36, p = 0.009). In all debrided anterior compartments, muscle contractility was completely abolished. There was a significant negative correlation between time to fasciotomy and lower leg muscle strength. CONCLUSION: Secondary compartment syndrome affects physical outcome in crush syndrome patients. We obtained no evidence that fasciotomy improves outcome. Delayed rescue, delayed fasciotomy, and radical debridement may worsen the physical prognosis. Indications for fasciotomy in crush syndrome during the acute phase need further deliberation.     

Orthopedic aspects of the Marfan phenotype.

Thirty-six patients with the phenotypic features of Marfan's syndrome (arachnodactyly, abnormal ratio of arm span to height, ligamentous laxity, and tall stature) were evaluated and classified into three groups. Group 1 represented definite Marfan's syndrome and included patients with two or more major signs, with additional minor signs as described by McKusick (18 patients). Group 2 represented probable Marfan's syndrome and included patients with one major sign and multiple minor signs (nine patients). Group 3 represented the Marfan phenotype and included patients with multiple minor signs (nine patients). A high incidence of scoliosis occurred in all groups (100% in Group 1 and 89% in Groups 2 and 3 combined). Group 1 patients had longer, multiple, and more progressive spinal curves. Protrusio acetabuli (Type II and III hips) was present in 22 patients (11 in Group 1, six in Group 2, and five in Group 3), with more severe involvement noted in Group 1. Foot deformities of varying types and severity occurred in all groups but were seen most often in Group 1 patients. Patients with the features of Marfan's syndrome (even without major diagnostic criteria) have a high incidence of progressive scoliosis, protrusio acetabuli, and foot deformities.     

重症下肢深静脉血栓形成治疗的临床研究

目的 探讨综合应用下腔静脉滤器置入、手术取栓、同时处理Cockett综合征、术后抗凝及溶栓等方法 治疗重症下肢深静脉血栓(股青肿)的疗效.方法 回顾近10余年9例重症下肢深静脉血栓患者的临床资料.9例中8例行下腔静脉滤器置入、手术取栓、术后抗凝及溶栓,8例中有7例为左下肢深静脉血栓患者.均发现合并不同程度的Cockett综合征,其中2例同时处理Cockett综合征;9例中1例(左下肢深静脉血栓患者)行下肢静脉溶栓术.结果 所有患者均无肺栓塞发生,经手术的8例患者除1例(左下肢深静脉血栓患者)术后出现湿性坏疽,被迫截肢外,其余7例均取得较为满意的疗效,挽救了患肢.1例行下肢静脉溶栓的患者死亡.1个月后复查彩超,没有同时处理Cockett综合征的5例中有2例左下肢血栓患者复发血栓,但均未再次发展为股青肿.7例随访1.5～10年,均未发生严重并发症.结论 综合应用下腔静脉滤器置人、手术取栓、同时处理Cockett综合征、术后抗凝及溶栓等方法 是治疗重症下肢深静脉血栓(股青肿)的有效方法 ,Cockett综合征是左下肢DVT取栓术后复发的重要原因.     

Efficacy of percutaneous ethanol sclerotherapy for venous malformation in lower extremities: a retrospective review of 21 cases

Background

Venous malformations (VM) of lower extremities have characteristic symptoms, especially swelling, pain at rest or with dependence, or in the morning, or with exertion. Sclerotherapy has been applied and has been showed to alleviate the associated signs and symptoms. The aim of this study is to evaluate the outcomes of ethanol sclerotherapy for VM of lower extremities.

Methods

The 21 patients of intramuscular VM of the lower extremities, who received percutaneous sclerotherapy using absolute ethanol in our institute, were reviewed retrospectively. The average age at the time of the initial diagnosis was 18.6 years, and average follow-up period after last sclerotherapy is 19.4?±?13.5 months. The postinterventional changes of the associated signs and symptoms were evaluated by utilizing original VM scoring system.

Results

The average number of sclerotherapy sessions was 2.6 times per case. The average total amount of ethanol used in each patient was 41.3 ml. Sclerotherapy reduced the associated signs and symptoms in many of the patients (n?=?19/21). Preinterventional VM score as the overall baseline status was 10, and the average VM score after sclerotherapy was 4.1. There was no inverse correlation between the amount of ethanol used and the VM score. In seven cases treated more than three times, reverse correlation between the “VM score” and the number of sclerotherapy sessions was demonstrated (p?<?0.05, ρ?=?0.8214).

Conclusions

The characteristic symptoms and signs of the VM were improved by sclerotherapy. More than three sessions of ethanol sclerotherapy improved the overall status of extensive intramuscular VM in lower extremities. Level of Evidence: Level IV, therapeutic study.     

Endovascular laser coagulation in the treatment of varicose disease the the lower extremities

The experience of treatment of 55 patients, suffering varicose disease of the lower extremities, using endovascular laser coagulation in combination with crossectomy and operation according to Babcock method, was analyzed. Good and excellent results were achieved in 53 (96.4%) patients, complications have had occurred in 1 (1.8%).     

Blood flow "stealing" phenomenon in the foot as one of the causes of ischemia of the toes

An examination of 750 patients with chronic ischemia of lower extremities has revealed 12 patients whose foot arteries pulsation could be determined by pulpation. However, by means of angiography of the ischemic lower extremity, ultrasound flowmetry of the foot arterial arch with the use of compression tests these patients were found to have the syndrome of "robbing" the foot bloodflow.     

4例髂股血管外膜囊肿的临床分析

目的 探讨髂股血管外膜囊肿的临床特征、治疗及预后.方法 回顾性分析2017年3月至2019年6月济宁医学院附属医院血管外科诊治的4例髂股血管外膜囊肿患者的临床资料.男性2例,女性2例;年龄54～82岁,中位年龄61岁.分析其就诊资料和临床特点.结果 3例患者为股总静脉外膜囊肿,均表现为下肢肿胀.1例为髂股动脉外膜囊肿,...     

Postthrombotic syndrome after asymptomatic deep vein thrombosis following total knee and hip arthroplasty

Lower extremity deep vein thrombosis (DVT) is relatively common after total knee arthroplasty (TKA) and total hip arthroplasty (THA). In this study, we sought to identify whether post-thrombotic syndrome is a significant risk in patients with asymptomatic DVT after TKA and THA. Included in the study were 186 patients (203 lower extremities) who had undergone routine lower extremity venography after TKA or THA between 1989 and 1993. Patients were contacted a minimum of 7 years after surgery. Postoperative varicose veins occurred in 8% of limbs without DVT compared with 12% of limbs with DVT. Hyperpigmentation occurred in 13% of limbs without DVT and in 16% and 18% of limbs with proximal and distal DVT, respectively. Swelling occurred in 6% of limbs without DVT, in 8% of limbs with proximal DVT, and in 12% of limbs with distal DVT. None of these differences was statistically significant, and there were no differences for proximal versus distal DVT. There was no significant increase in risk for postthrombotic syndrome after asymptomatic proximal or distal DVT after TKA or THA.     

Blunt vascular trauma in the extremity: diagnosis, management, and outcome

BACKGROUND: Blunt vascular trauma in an extremity is an uncommon diagnosis. Considering the complexity of these injuries, it is worthwhile to determine how select factors affect the outcome of the limb and the patient. The objectives of this study were to review the diagnosis, management, and outcomes of patients who sustained blunt vascular injuries in the extremities and relate factors in their treatment to the outcome of the injured extremity. METHODS: A retrospective review of data on adult and pediatric patients who had a diagnosis of blunt vascular injury in an extremity and underwent some attempt at restoration of vascular flow was conducted. RESULTS: From January 1995 to December 2002, 62 patients (80.3% male; mean age, 33.2 +/- 15.8 years) sustained blunt trauma (mean Injury Severity Score, 14.6 +/- 8.4), with 93 vascular injuries in 65 extremities (16 upper and 49 lower). Hard signs of vascular injury occurred in 41 (66%) patients. An associated fracture and/or dislocation was present in 59 patients (95%). Preoperative arteriograms were obtained in 20 patients (17 occlusions, 2 embolizations, and 1 untreated). Vessel injuries were as follows: 16 upper (brachial artery, 50%) and 63 lower (tibial/peroneal/popliteal, 84%), with ligation being the most common treatment in the latter. Intravascular shunts were used to restore blood flow in 18 vessels (13 arteries and 5 veins) in 13 patients. Delays in diagnosis or treatment occurred in six patients, mostly because of errors in management/judgment. Delayed or late fasciotomies were performed in six patients, and five developed rhabdomyolysis. Six patients died. The age (p = 0.0006), Injury Severity Score (p = 0.0007), and Mangled Extremity Severity Score (p = 0.0009) were significantly different for the survivors compared with the nonsurvivors. CONCLUSION: Blunt vascular injuries in the lower extremities occur most commonly in the anteroposterior tibial arteries; injured arteries in the proximal upper and lower extremity require resection with interposition grafting, whereas those in the forearm or calf are usually ligated; the amputation rate in 65 injured extremities with blunt vascular trauma was 18.%, which is at least three times that for those who sustain penetrating injury; and delays in diagnosis and treatment are uncommon in these patients with multiple injuries.     

Arteriovenous anastomoses in the treatment of postphlebitic syndrome of the lower extremities

The authors describe an original method of putting arteriovenous end-to-end anastomosis between the distal fragment of the artery and central fragment of the vein in postphlebitic + syndrome of lower extremities. Operations were performed on 27 patients with different forms of the syndrome. Clinical improvement was obtained in 22 patients (81%), which was confirmed by findings of functional investigations. This operation was most effective in patients with recanalization or partly recanalized forms of the postphlebitic syndrome.