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Venous thromboembolism associated with hip and knee arthroplasty: current prophylactic practices and outcomes.

Joint registry and hospital data bases for 5,024 total hip and total knee arthroplasties done between 1986 and 1988 at the Mayo Clinic were used to study prophylactic measures and frequency of symptomatic deep venous thrombosis and pulmonary embolism. In virtually all patients, graduated compression stockings were used, with or without another type of prophylaxis. Only 44 of 3,115 patients who underwent hip arthroplasty (1.4%) and 32 of 1,909 patients who underwent knee arthroplasty (1.7%) had definite or probable deep venous thrombosis or pulmonary embolism. Death definitely or possibly attributable to pulmonary embolism occurred in 11 patients who underwent hip arthroplasty (0.35%) and 1 patient who underwent knee arthroplasty (0.05%). Although patients with a history of deep venous thrombosis or pulmonary embolism were more likely to receive warfarin than were patients without such a history, the relative risk of symptomatic deep venous thrombosis or pulmonary embolism in patients who underwent hip arthroplasty and received warfarin postoperatively was approximately half that in patients who received other types of prophylaxis. The risk of death from pulmonary embolism was similarly diminished in the group that received warfarin. The lower rates of these complications in the patients who received warfarin support the prophylactic use of this agent after total hip arthroplasty.

Aspirin↗

Thromboprophylaxis in neurosurgical patients.

Neurosurgical patients constitute one of the highest risk groups for postoperative thromboembolic complications. Physical methods of thromboprophylaxis have been successful in reducing the incidence of postoperative deep vein thrombosis (DVT) but the residual incidence remains considerable. Many neurosurgeons are reluctant to use perioperative anticoagulant prophylaxis, despite its proven success in reducing DVT rates, because of the potentially serious consequences of even a small intracranial bleed. Recent studies have indicated that a combination of graduated compression stockings (GCS) and low-molecular-weight heparin (LMWH), started in the postoperative period, significantly reduces the incidence of DVT compared with GCS alone. Postoperative regimens avoid the risk of surgical hemorrhage and appear to offer increased protection for this group of patients.

Anticoagulants↗

Post-thrombotic syndrome after primary event of deep venous thrombosis 10 to 20 years ago.

AIMS: We investigated the impact of the extent of primary deep venous thrombosis (DVT) and recurrent thrombotic events in accordance to other presumed prognostic factors for long-term clinical outcome after first DVT. PATIENTS AND METHODS: All consecutive in-patients, who were treated following first acute DVT between January 1, 1978 and December 31, 1988 at the Department of Angiology were identified by admission lists. Localisation, extent of primary DVT, etiology and concomitant pulmonary embolism (PE) at the time of initial presentation and occurrence of post-thrombotic syndrome (PTS) at follow-up visits were assessed by chart review. The duration and quality of the compression therapy, as well as the accuracy of the oral anticoagulant (OAC) treatment were documented. Recurrence of thrombosis embolism and/or PE with respect to the intensity of OAC was analyzed. Patients were invited to participate in clinical reinvestigation. Patients' history and clinical stage of PTS were re-evaluated and patients were asked for compliance in wearing compression stockings. A survey concerning restriction in quality of life was conducted. Hemodynamic measurements by strain-gauge plethysmography (SGP) were performed. RESULTS: One hundred and sixty-one patients were eligible for the study. Out of these 132 patients, 82% suffered from the PTS, defined as signs of chronic venous insufficiency (CVI) secondary to DVT of the lower limbs: 74 patients (46%) presented with clinical stage I after Widmer, 47 patients (29%) with clinical stage II and 11 patients (7%) with clinical stage III. No sign of PTS was seen in 29 patients (18%). The mean follow-up period of 6.6 years was statistically not different between the three severity groups of PTS. The severity of clinical symptoms was significantly associated with the recurrence of ipsilateral thrombosis (n = 26/16%). Highest risk for developing severe PTS was seen after four-level DVT and deep vein thrombosis of the lower leg. Patients having had a non-sufficient OAC (Hepatoquick > 25% in more than 50% of measurements) exhibited worse progradient clinical stages. Besides the high rate of bleeding complications after thrombolytic therapy, this strategy did not show more efficiency in prevention of development of severe PTS than heparin therapy alone. CONCLUSION: Our results show that primary four-level DVT, calf vein thrombosis, recurrence of ipsilateral DVT and a non-sufficient oral anticoagulation are of prognostic significance for developing clinically relevant PTS within 10 to 20 years after first DVT.

Adolescent↗

Frequency of venous thromboembolism in low to moderate risk long distance air travellers: the New Zealand Air Traveller's Thrombosis (NZATT) study.

BACKGROUND: The frequency and role of risk factors for venous thromboembolism related to air travel is uncertain. We aimed to establish the frequency of this disorder in a group of long distance air travellers and to investigate the role of potential risk factors. METHODS: We designed a prospective study into which we recruited individuals aged between 18 and 70 years, travelling for 4 h or more by aircraft. D-dimer measurement was done before and after travel. Participants with a negative D-dimer (<500 ng/L) before travel were included in the study. Those who became D-dimer positive or developed high clinical probability symptoms during the 3 months after travel were investigated with bilateral compression ultrasonography and CT pulmonary angiography. Suspected clinical and thrombophilic risk factors, and use of prophylactic measures, were assessed. FINDINGS: 1000 individuals were recruited, with 878 meeting inclusion criteria and completing the study. All participants travelled at least 10 h, with a mean total duration of air travel of 39 h (SD 12.5). 112 patients underwent radiological assessment on return. Frequency of venous thromboembolism associated with travel was 1.0% (9/878, 95% CI 0.5-1.9), which included four cases of pulmonary embolism and five of deep venous thrombosis. Six patients with venous thromboembolism had pre-existing clinical risk factors, two had a recognised thrombophilic risk factor, two travelled exclusively in business class, five used aspirin, and four wore compression stockings. INTERPRETATION: Our results suggest an association between multiple long distance air flights and venous thromboembolism, even in individuals at low to moderate risk. The role of traditional risk factors and prophylactic measures in air travel-related venous thromboembolism needs further investigation.

Adolescent↗

Need for long-term anticoagulant treatment in symptomatic calf-vein thrombosis.

The need for oral anticoagulation in patients with calf-vein thrombosis was examined in a randomised study of 51 patients, of whom 23 received warfarin for 3 months and 28 did not. Both groups received an initial course of heparin and all wore compression stockings. Progress was monitored by the use of serial isotope tests and physical examination. Phlebography was repeated if recurrence was suspected. During the first 3 months, 8 patients in the non-warfarin group (29%) had recurrences compared with none in the warfarin group (p less than 0.01). 5 patients had recurrence with proximal extension and 1 patient had a pulmonary embolus. After 1 year, 22 out of 23 patients in the warfarin group had not had a recurrence, compared with 19 out of 28 (p less than 0.02). The findings indicate that oral anticoagulants should be given to all patients with thrombi that produce symptoms. Treatment for 3 months seems to be sufficient.

Administration, Oral↗

[Current indications of cava filters].

Permanent vena cava filters are useful in venous thromboembolism in some rare medical situations (i.e., contra-indication or failure of anticoagulant therapy). Other indications are controversial. Indeed filters increase the risk of recurrence of deep vein thrombosis and also probably the incidence of post-thrombotic syndrome. So filter placement remains a crucial decision, according to each individual case. The operator's choice is of prime importance. When a permanent filter is inserted the use of graded compression stockings and long-term anticoagulant therapy deserves to be considered. In case of transient risk, temporary filters might be an interesting therapeutic approach but their assessment is insufficient in order for them to be currently recommended.

Contraindications↗

The post-thrombotic syndrome: current knowledge, controversies, and directions for future research.

The post-thrombotic syndrome (PTS) is a long-term complication of deep venous thrombosis (DVT) that is characterized by chronic, persistent pain, swelling and other signs in the affected limb. PTS is common, burdensome and costly. It is likely to increase in prevalence, since despite widespread use of and improvements in the efficacy of thromboprophylaxis, the incidence of DVT has not decreased over time. About 20-50% of patients develop PTS within 1-2 years of symptomatic DVT, and severe PTS, which can include venous ulcers, occurs in 5-10% of cases. Although there is no gold standard for the diagnosis of PTS, the presence of typical clinical features in a patient with previous DVT provides strong supporting evidence. Objective evidence of venous valvular incompetence helps to confirm the diagnosis in symptomatic patients. Preventing ipsilateral recurrence of DVT, by ensuring an adequate duration and intensity of anticoagulation for the initial DVT and by prescribing situational thromboprophylaxis after discontinuation of oral anticoagulants, is likely to reduce the risk of developing PTS. There is no proven role for thrombolysis of the initial DVT to prevent PTS. Daily use of graduated compression stockings after DVT may reduce the risk of PTS, and may prevent worsening of established PTS. Pending the results of ongoing studies, stockings are recommended in patients with persistent symptoms or swelling after DVT. Future research should focus on standardizing criteria for PTS diagnosis, identification of DVT patients at high risk for PTS, and rigorously evaluating the effectiveness of stockings, thrombolysis, and venoactive drugs in preventing or treating PTS.

Disease Management↗

Idiopathic edema.

Idiopathic edema is a syndrome of real or perceived excessive weight gain. This article reviews what is known about the possible causes, evaluation, and treatment. Although the cause is unknown but often thought to be due to secondary hyperaldosteronism, primary abnormalities of the hypothalamus, thyroid, dopaminergic release or renal dopaminergic metabolism, vascular basement membrane, or capillary sphincter control could perhaps contribute in some patients. The diagnosis requires careful attention to possible abnormalities of the liver, heart, kidneys, gastrointestinal tract, thyroid, and pancreas. The history must include an evaluation for risks of bulimia and purging; diuretic and laxative screening should be performed. Specific records of daily weights, urinary outputs, and menstral cycle dates are useful. Treatment may include dietary counseling to provide weight control and a constant carbohydrate intake, treatments for depression, compression stockings, spironolactone, amiloride, angiotensin II inhibitors, or sympathomimetic agents, depending on the severity and timing of the patient's symptoms. Unfortunately, idiopathic edema may be a multifactorial disorder that has not been completely delineated. Further research into possible causative mechanisms is required before a more useful algorithm for evaluation and treatment is available.

Capillary Permeability↗

The management and outcome of acute venous thromboembolism: a prospective registry including 4011 patients.

OBJECTIVE: To obtain a realistic overview of management and clinical outcomes of patients with venous thromboembolism (VTE) in Spain on the basis of data from a national multicenter registry. METHODS: A prospective registry was initiated in Spain in March 2001. Data were collected from patients with objectively confirmed deep vein thrombosis (DVT) and/or pulmonary embolism (PE) and entered into the online registry by physicians who were responsible for the management of these patients. RESULTS: As of August 2002, 4011 patients with confirmed VTE were included in the registry: 60% with DVT, 23% with PE, and 17% with both DVT and PE. Diagnostic methods for VTE included compression ultrasonography (86%), venography (10%), V/Q lung scans (42%), computed tomography scan (28%), and pulmonary angiography (0.9%). D-dimer testing was performed in 61% of cases and was positive in 92% of patients with confirmed VTE. The majority of DVT (95%) were located in the lower extremities (82% proximal and 4% bilateral), while 4.8% were located in the upper extremities or neck veins. Most patients (90.5%) were admitted to hospital. In the acute phase, treatment consisted of low molecular weight heparin (LMWH) in 88%, unfractionated heparin (UFH) in 11%, and fibrinolysis in 0.8%. Cava filters were inserted in 2% of patients, mainly because of active bleeding (13%), increased hemorrhagic risk (38%), or recurrent VTE (29%). Absolute bed rest was recommended to 63% of patients. Secondary prevention of VTE included oral anticoagulants (75%) and LMWH (24.5%). Therapeutic compression stockings were prescribed to 53% of patients at the time of hospital discharge. Regarding the main clinical outcomes during an average (+/-SD) follow-up period of 156 +/- 95 days, 19% had adverse events: 12.5% of patients died, 5.5% had clinically confirmed VTE recurrence, and 9.8% suffered bleeding complications (44% with major bleeding). CONCLUSIONS: This prospective observational multicenter registry provides a large database reflecting the actual day-to-day clinical practice regarding VTE management in a European country. The most important findings were the increasing use of spiral computed tomography for PE diagnosis, the unexpectedly high proportion of patients admitted to hospital despite the use of LMWH in almost 90% of cases in the acute phase, and the utilization of LMWH for secondary prevention in almost 25% of cases. On the other hand, this large-scale prospective registry permits on-line consultation of high-risk situations to assess how difficult cases were treated and what their outcomes were. This will provide a most useful tool for the practicing physician responsible for the management of VTE patients.

Acute Disease↗

Modifications of plasma levels of tissue factor pathway inhibitor and endothelin-1 induced by a reverse Trendelenburg position: influence of elastic compression--preliminary results.

PURPOSE: The purpose of this study was to assess the effects of the passive 45-degree reverse Trendelenburg position and graduated compression stockings (GCS) on plasma tissue factor pathway inhibitor (TFPI) and endothelin-1 levels in a group of volunteers. METHODS: Ten healthy subjects lay on an examining table for 30 minutes while baseline measurements were made. The table was then tilted to a 45-degree upright position for 60 minutes, and measurements were repeated. On a different day, subjects were tilted again, but on this occasion they wore thigh-length GCS. Blood was drawn before and 60 minutes after tilting during both sessions, and plasma TFPI and endothelin-1 were obtained. Cross-sectional areas of the calf medial gastrocnemius vein, before and after tilting, were measured by a duplex scanner. RESULTS: Upright tilting induced a significant dilation of the medial gastrocnemius veins that was partially corrected by the use of elastic stockings. Similarly, endothelin-1 levels significantly increased after tilting. The use of GCS did not modify these differences. On the other hand, although TFPI levels were not affected by tilting without stockings, they were significantly elevated after tilting when GCS were used. CONCLUSIONS: Upright passive tilting induces significant dilation of the deep calf veins and is associated with a significant increase in plasma levels of endothelin-1, whereas TFPI levels remain unchanged. The use of elastic stockings reduces the degree of calf distention but does not prevent an increase in endothelin-1. TFPI levels are significantly increased after tilting when GCS are used. This might represent a previously unknown mechanism of action of elastic stockings with interesting potential for deep vein thrombosis prophylaxis. More studies are warranted in a larger series to confirm these results.

Adult↗

Autogenous aortoiliac/femoral reconstruction from superficial femoral-popliteal veins: feasibility and durability.

PURPOSE: Autogenous aortoiliac/femoral reconstruction with superficial femoral-popliteal veins (SFPVs) has been successfully used to treat prosthetic infection and failure, but outcome data are scant. In this prospective, observational study, we hypothesized that (1) replacement of the aortoiliac/femoral system with SFPVs could be performed with perioperative morbidity and mortality rates equal to those of conventional methods; (2) long-term patency rates would be excellent; (3) limb salvage rates would be correspondingly high; (4) aneurysmal degeneration would not occur, and (5) venous morbidity would be minimal. METHODS: Since 1990, 41 patients have undergone complete or partial aortoiliac/femoral reconstruction with 63 SFPVs with a mean (+/-SD) follow-up time of 32 +/- 21 months. With the exception of two patients lost to follow-up, all have been observed at 6-month intervals with clinical examination and noninvasive tests. RESULTS: There were no immediate operative deaths, but three patients (7.3%) died of multisystem organ failure after 1 month. Forty-nine percent of patients had significant perioperative complications including amputation (5%), compartment syndrome (12.3%), and pulmonary embolism (2.4%). Most patients (85%) had multilevel occlusive disease, and the mean SVS/ISCVS runoff score for the group was 4.9 +/- 2.6 (1 = normal, 10 = no runoff). Fifty-eight percent of all distal anastomoses were end-to-end, and in 68% of limbs the profunda femoris artery or superficial femoral artery was the sole runoff vessel. At 5 years, the cumulative secondary patency rate was 100%; primary patency rate, 83%; limb retention rate, 86%; and survival rate, 69%. Four patients had permanent limb edema controlled by compression stockings but none have had venous ulceration. There has been no aneurysmal dilation of SFPV grafts, and mean diameter shown by serial duplex imaging at 6 months (10.8 +/- 1.1 mm) was not significantly different from that at 60 months (7.8 +/- 1.1 mm). CONCLUSIONS: Aortoiliac/femoral reconstruction with SFPVs is a successful and durable option for infection and other complex aortic problems.

Amputation, Surgical↗

Epidemiology, risk factors and prophylaxis of venous thrombo-embolism in obstetrics and gynaecology.

Venous thrombo-embolism remains a major cause of mortality and morbidity following gynaecological surgery and in association with pregnancy and delivery. Specific risk factors can be identified pre-operatively and before or during pregnancy and delivery. Clinicians and units should develop guidelines for risk assessment and the implementation of specific thromboprophylactic measures in patients considered to have significant risk. The main prophylactic techniques are unfractionated and low-molecular-weight heparins and physical methods such as graduated elastic compression stockings. It should be noted that there are particular concerns with regard to the use of pharmacological thromboprophylaxis with both heparin and warfarin in pregnancy. Unfractionated heparin is associated with osteoporotic problems, allergy and heparin-induced thrombocytopenia which can cause significant thrombotic problems. Warfarin is associated with teratogenesis and the risk of bleeding in mother and fetus. Clearly, where antenatal thromboprophylaxis is to be used, the risk of the anticoagulants employed must be weighed against the potential benefits. Such assessment might be best done prior to pregnancy in order that the patient can enter pregnancy with a clear view of the potential hazards and benefits. Low-molecular-weight heparins are being increasingly used in pregnancy but it is unclear to what extent they are safer than unfractionated heparins. However, they do appear to have substantially less risk of heparin-induced thrombocytopenia and possibly less risk of heparin-induced osteoporosis. Increasingly, thrombophilia is recognized as underlying many thrombotic problems, particularly in young women, and when the events occur in association with pregnancy. In view of the complexity in the management of such patients, it is important that they be referred to a unit with specific expertise in the management of thrombophilia.

Cesarean Section↗

Treatment of venous thrombo-embolism.

Clinically suspected deep vein thrombosis (DVT) or pulmonary thromboembolism (PE) should be initially treated with heparin, and an objective diagnosis obtained. In pregnancy, heparin is usually continued until delivery, following which warfarin is substituted. In the absence of pregnancy, warfarin is substituted and usually continued for 3 months after a first thrombo-embolic event. Low molecular weight heparins are increasingly preferred to unfractionated heparin in non-pregnant patients with acute DVT, because of efficacy when given by daily subcutaneous injection without routine monitoring of coagulation assays, greater efficacy, and lower risks of major bleeding and of mortality. Unfractionated heparin requires monitoring by the APTT (target ratio 1.5-2.5), and warfarin requires monitoring by the International Normalized Ratio (INR) of the prothrombin time (target ratio 2.0-3.0). Graduated elastic compression stockings reduce post-thrombotic leg symptoms after DVT. Secondary prevention is important in future high risk situations.

Anticoagulants↗

Surgical thrombectomy versus conservative treatment for deep venous thrombosis; functional comparison of long-term results.

It is known that deep venous thrombosis (DVT) of the ilio-femoro-popliteal axis is frequently associated with irreversible damage to valvular competence of the veins and consequently with varying degrees of chronic venous insufficiency. Because preservation of the valvular function of deep veins can play an important role in preventing the postphlebitic syndrome we analysed and compared the long-term functional outcome of two equally large cohorts of patients treated either surgically for restoration of venous patency and valvular function (24 patients) or medically with heparin, oral anticoagulants and compression stockings (25 patients). The study was also intended to examine the impact of duration and extent of DVT as predictive factors of late outcome. Follow-up time was 7.6 and 7.9 years respectively, operative mortality nil. Assessment of venous function was based on clinical observations as well as on measurement of haemodynamic parameters. Non-fatal pulmonary embolism after onset of treatment occurred in both cohorts with an equal frequency of 13%. Patients operated on for ilio-femoral DVT were with few exceptions totally independent of any form of adjunctive hosiery which was in sharp contrast to the conservatively managed group. If onset of DVT had occurred more than 3 days earlier and extended from the ilio-femoral axis to the popliteo-crural level, surgery usually failed and patients were no better off than in the comparable medical group. The same pattern of late outcome was found for all other clinical and haemodynamic parameters; i.e. clinical signs of venous hypertension, valvular competence as judged by sonography, patient's self-assessment and the expelled volume and refilling time measured by dynamic plethysmography after standardised leg work. The mean expelled volume was 1.1 +/- 0.5 ml/100 g/min. for the surgical group treated early for ilio-femoral DVT and 0.7 +/- 0.5 ml/100 g/min for the corresponding medical group (P = 0.05). Recovery or refilling time was 50 +/- 21 s for the surgical group and 28 +/- 26 s for the medical group (P = 0.03). Thus, the clinical and haemodynamic effect of surgical thrombectomy was significantly superior to conservative management in ilio-femoral thrombosis treated within 3 days. For extensive thrombosis treated early the advantage of surgical thrombectomy was also evident, but the difference between the two treatment groups was not significant. The advantage of surgery was however totally lost in patients operated on for extensive DVT of long duration (i.e. greater than 3 days).(ABSTRACT TRUNCATED AT 400 WORDS)

Bandages↗

Deep vein thrombosis and malignancy: a surgical oncologist's perspective.

Oncology patients are at increased risk of developing deep vein thrombosis (DVT) and its potentially fatal sequel, pulmonary embolism. This is due to multiple factors, including the presence of the malignancy itself, comorbid factors and therapy-related interventions. Issues that are peculiar to venous thrombosis in the oncology setting are discussed, based on a MEDLINE search of the English literature. These include the need to screen for malignancy in idiopathic DVT, a high index of suspicion for venous thrombosis in the cancer patient, the use of vena cava filters, and the anti-neoplastic effects of heparin. Asian patients appear to have a lower incidence of DVT compared to Caucasians. A recommended regimen for prophylaxis of DVT must take into account the varying thrombosis risk associated with different malignancies. Cancer patients not undergoing abdominal, pelvic or orthopaedic surgery (e.g. mastectomy) should use elastic compression stockings and be mobilized early, whereas low-molecular-weight heparin should be given to those undergoing more major surgery. In advanced malignancy, treatment of DVT palliates symptoms. These patients may need long-term anticoagulation with warfarin.

Anticoagulants↗

[Anticoagulation in patients with venous thromboembolism].

Deep venous thrombosis and pulmonary embolism are considered to be two variants of one disease--'venous thromboembolism'. Pathogenesis, therapy and prognosis of these both entities are very similar and therefore the term 'venous thromboembolism' has been used in recent literature. The cornerstone of therapy is anticoagulation and initially consists of heparin for at least five days. Because of pharmacokinetic advantages low molecular weight heparins are the therapy of choice. They are as efficient and save as unfractionated heparins and allow weight-adapted dosing with daily subcutaneous injections in most patients. Low molecular weight heparins do not require regularly laboratory monitoring with few exceptions, e.g. renal failure. Therefore outpatient treatment of deep venous thrombosis is possible in most patients. Although there are promising data about outpatient treatment of pulmonary embolism, this is still being studied and can not be recommended outside clinical trials. Introduction of coumarin therapy for venous thromboembolism should be started on day 1 of diagnosis, keeping the total duration of heparin therapy at no more than five days and therefore minimizing the incidence of heparin-induced thrombocytopenia. Evidence from multiple studies indicates that effective coumarin therapy in venous thromboembolism is usually reflected by an INR of 2.0 to 3.0. In patients with massive and hemodynamically relevant thromboembolism alternative therapeutic approaches such as thrombolytic therapy, thrombectomy or insertion of intravenous filters may be useful. Adequately fit compression stockings can reduce the risk of post-thrombotic syndrome after deep venous thrombosis.

Anticoagulants↗

Proteus syndrome.

A 34-year-old male patient was referred with a recalcitrant leg ulcer overlying an extensive vascular malformation, which had led several times to septic soft tissue infections. During his infancy he had been diagnosed to have Klippel-Trenaunay syndrome. Clinical examination revealed asymmetric hypertrophy of the lower extremities, an extensive portwine stain on the more severely affected left limb as well as prominent venous varicosities of both legs. Hands and feet showed striking cerebriform palmoplantar hypertrophy, and macrodactily with syndactily of several fingers. All toes had been amputated in early childhood due to extreme overgrowth and currently the patient walked on his forefeet in a prominent pes equinus deformity. Further symptoms consisted in several lipomas at both arms, another portwine stain at the left hemithorax and a single café-au-lait spot at the left scapula. Angio-magnetic resonance imaging scans of both legs showed an extensive venous-lymphatic vascular malformation involving the whole subcutis and infiltrating the muscle. The chronic wound was interpreted as venous stasis ulceration. Local percutaneous sclerotherapy of the dilated veins underneath the ulcer was discussed, but considered to carry a relevant risk of skin necrosis with consecutive progression of the wound. A conventional split-skin graft led to complete wound healing. Since, the patient consequently wears custom-made compression stockings and remained free from recurrences. The syndromatic constellation of palmoplantar overgrowth, multiple lipomas, giant fingers and toes, limb overgrowth, venous-lymphatic malformation and a café-au-lait spot led to the diagnosis of Proteus syndrome. The possible aetiology, clinical manifestations, differential diagnosis and management of this rare disorder are discussed.

Adult↗

Preliminary experience with midodrine in kidney/pancreas transplant patients with orthostatic hypotension.

In an effort to ameliorate the problem of orthostatic hypotension in pancreas transplant patients, current medical management consists of maximizing the patient's hydration, altering antihypertensives, increasing sodium intake, initiation of fludrocortisone, compression stockings, and behavioral modifications. Despite these medical interventions, a subset of patients remains symptomatic. Midodrine (ProAmatine), an alpha-adrenergic agonist, was approved for the treatment of symptomatic orthostatic hypotension in the US. This preliminary report attempts to assess the safety and efficacy of midodrine use in kidney/pancreas (KP) or pancreas alone (PA) transplant recipients. A retrospective review was performed of 7 KP and 1 PA recipient experiencing symptomatic postural hypotension after maximizing other medical treatments. Blood pressure, serum creatinine (SrCr), and objective responses to postural hypotension were assessed at routine intervals. Pre-midodrine monitoring revealed a mean orthostatic change in systolic blood pressure from sitting to standing of 43 mmHg (range 20-100 mmHg). Patients received a mean starting midodrine dose of 18 mg/d, which was titrated to a maximum dose of 30 mg/d. Systolic blood pressure monitoring revealed a mean orthostatic change of 27 mmHg (range 0-81 mmHg) after initiation of treatment with midodrine and a mean follow-up of 3.2 months. All study patients reported improvement in symptoms of orthostatic hypotension. SrCr was not affected based upon comparison of pre-treatment and current SrCr values of 1.4 and 1.3 mg/dL, respectively. The most common side effect experienced was supine hypertension. These preliminary results suggest that midodrine is safe and effective in transplant recipients; however, the dosage should be titrated to symptomatic relief or a maximum dose of 30 mg. Careful monitoring for supine hypertension is necessary.

Adrenergic alpha-Agonists↗