Hereditary haemorragic teleangectasia and pregnancy: case report.

We report an uncommon case of a 38-years-old pregnant woman affected by HHT (Hereditary haemorrhagic telangiectasia; Osler-Weber-Rendu syndrome) who underwent to a caesarean section (CS) without any complication. The patient at 36th weeks+1 day pregnancy referred to the Emergency Obstetric Unit due to a intercostals pain on left side. On third day after admission the woman started travailing and physicians decided to perform the CS. Considering that no AVMs was found at MRI, a continuous spinal anaesthesia was planned. On postpartum day 4 the patient was discharged. This represents the only case published in the literature. Women with HHT, especially those with arteriovenous malformations (AVM), are at high risk in pregnancy due to physiological haemodynamic changes pregnancy associated. Early screening of patients with HHT for the presence of spinal cord or cerebral AVMs is recommended to optimise perioperative anaesthetic management and to avoid severe complications.

A Psychosocial Adjustment Measure for Persons With Upper Limb Amputation.

BACKGROUND: Measurement of psychosocial adjustment after upper limb amputation (ULA) could be helpful in identifying persons who may benefit from interventions, such as psychotherapy and/or support groups. However, available measures of psychosocial adjustment after limb loss are currently designed for prosthetic users only. OBJECTIVE: To create a measure of psychosocial adjustment for persons with ULA that could be completed by individuals regardless of whether a prosthesis is use. METHODOLOGY: We modified items from an existing Trinity Amputation and Prosthesis Experience Survey (TAPES) measure and generated new items pertinent to persons who did not use a prosthesis. Item content was refined through cognitive interviewing and pilot testing. A telephone survey of 727 persons with major ULA (63.6% male, mean age of 54.4) was conducted after pilot-testing. After exploratory and confirmatory factor analyses (EFA and CFA), Rasch analyses were used to evaluate response categories, item fit and differential item functioning (DIF). Item-person maps, score distributions, and person and item reliability were examined. Test-retest reliability was evaluated in a 50-person subsample. FINDINGS: EFA and CFA indicated a two-factor solution. Rasch analyses resulted in a 7-item Adjustment to Limitation subscale (CFI=0.96, TLI=0.95, RMSEA=0.128) and a 9-item Work and Independence subscale (CFI=0.935, TLI=0.913, RMSEA=0.193). Cronbach alpha and ICC were 0.82 and 0.63 for the Adjustment to Limitation subscale and 0.90 and 0.80 for the Work and Independence subscale, respectively. CONCLUSIONS: This study developed the Psychosocial Adjustment to Amputation measure, which contains two subscales: 1) Adjustment to Limitation and 2) Work and Independence. The measure has sound structural validity, good person and item reliability, and moderate to good test-retest reliability.

Intracranial hypotension and PRES: case report.

We report a case of a woman presenting, 7 days after epidural analgesia for a caesarean section, to the emergency room for a worsening of the headache and tonico-clonic seizures. MRI showed alterations suggestive of the presence of intracranial hypotension (IH) as well as evidence of posterior reversible encephalopathy syndrome (PRES). She was treated with a blood patch which leads to the prompt regression of the clinical symptoms and follow-up MRI, after 15 days, showed complete resolution of radiological alterations. The possible pathogenetic relationship between IH, secondary to the inadvertent dural puncture, and PRES is discussed. We suggest that venous stagnation and hydrostatic edema, secondary to intracranial hypotension, probably played a crucial role in the pathogenesis of PRES.

[Therapy of dyslipidemia in post-infarction: state of the art]. / Terapia delle dislipidemie nel post-infarto: stato dell'arte.

Between the risks factors involved in the atherogenesis LDL-cholesterol is determinant because highly associated to cardiovascular events. The primary target for the prevention of coronary diseases is a reduction of LDL-cholesterol because that reduces the cardiovascular mortality and the total mortality. The NCEP ATP III 2004 guide-lines propose as therapeutic target for the high-risk patients the reduction of plasma levels of LDL-cholesterol under 100 mg/dl and according to new trials under 70 mg/dl. The dyslipidaemia treatments are based on two approaches, i.e., the therapeutic lifestyle change and the pharmacological therapy. The available drugs are statins, fibrates, anion exchange resins, nicotinic acid. In the acute coronary syndrome patients is desirable to start immediately a therapy with statins since the hospital phase and direct the treatment to aggressive therapy. Unfortunately, the statin doses used in the most secondary prevention trials allow to get LDL-cholesterol under 100 mg/dl in the only half high-risk patients. The innovative therapeutic approach to hypercholesterolemia today is based on a double inhibition of cholesterol synthesis and absorption combining a statin with ezetimibe.

[Hereditary hemorragic telangiectasia: the state of art]. / Teleangectasia emorragica ereditaria: stato dell'arte.

Hereditary Hemorrhagic Telangiectasia (HHT) or Rendu-Osler-Weber syndrome, is a multisystemic vascular dysplasia. The disease is transmitted as a dominant autosomic character. The Hereditary Hemorrhagic Telangiectasia is differentiated in two subclasses: (1) HHT1, which is caused by mutation of ENG gene. This gene is localized on long arm of chromosome 9: (2) HHT2, which is caused by mutation of ALK-1 gene. This gene is located on long arm of chromosome 12. These two genes codify for two receptorial proteins: the endoglin and the activin-like protein 1; these proteins belong to receptorial superfamily of TGF-beta, which is involved in vascular remodelling and angiogenesis. Clinically, the consequences of these mutations are represented by the formation of cutaneous and/or mucous telangiectases and artero-venous fistulas. In both cases histological alteration is the same: extremely ectatic venules with numerous layers of myocytes around them. Arterioles communicate with venules directly without a capillary filter. Essentially, telangiectases manifest themselves with hemorrhages, while more common consequences of fistulas are secondary to formation of shunts with a possible thromboembolism; that is particularly serious in case of pulmonary artero-venous malformations. In 2000. Shovlin published 4 diagnostic criteria (criteria of Curaçao): (1) spontaneous and recurrent epistaxis; (2) multiple telangiectases; (3) visceral artero-venous malformations; (4) familiarity for HHT. Actually there is no possibility for a genetic therapy of HHT. Therefore, the therapeutic efforts are turned to control of symptoms and to the prevention of complications.

Postoperative analgesia after cesarean section by continued administration of levobupivacaine with the On-Q Painbuster system over the fascia vs ketorolac + morphine i.v.

OBJECTIVE: This study aimed to detect if continuous local infusion of levobupivacaine with the On-Q Painbuster system provided postoperative analgesia of similar quality to morphine + ketorolac i.v. in patients undergoing cesarean section. MATERIALS AND METHODS: Using a randomized prospective double-blind study, 20 women undergoing cesarean section with a standardized spinal technique were randomly assigned into two groups to receive either 10 mg morphine + 120 mg ketorolac + saline solution up to 96 ml with an elastomeric pump i.v. (group A) or local infusion of levobupivacaine 0.2% with the On-Q PAINBUSTER system (group B). Both groups were administered ketorolac i.v. in bolus in case of pain. RESULTS: The two groups differed in their VAS scores with group A experiencing significantly less pain than group B; the consumption of analgesics was significantly lower in group A than in group B. CONCLUSIONS: The i.v. system with morphine and ketorolac is more effective than levobupivacaine subcutaneous infusion in reducing postoperative pain associated with cesarean section.

New aldosteron receptor inhibitors in heart failure.

This report summarizes the new therapeutic approaches in post-ischaemic heart failure given the important sanitary and socioeconomic impact resulting from the clinical management of this syndrome. Ventricular remodeling, causing changes to left ventricular anatomy and function, is the major cause of heart dysfunction. The physio-pathological role of the renin-angiotensin-aldosterone system has emerged from experimental studies and has been confirmed by the efficacy of ACE-inhibition. Although treatment with spironolactone (a non selective aldosterone antagonist) significantly reduces both the hospitalization and the mortality of NYHA III patients, this compound has important side effects. In this review we examine the efficacy of eplerenone, a new aldosterone receptor antagonist, with a more selective activity with respect to spironolactone. Several studies versus placebo have demonstrated that this molecule, alone or in combination with anti-hypertensive drugs, is very effective in left ventricular hypertrophy and in post-ischaemic heart failure treatment. In particular, we will consider the EPHESUS as the most important study both for the size of patient group and for the experimental plan. Finally, as regards the culture and experience of single physicians, a simple flow chart of the therapy of post-ischaemic heart failure is proposed.

Further evidence against the implication of active cytomegalovirus infection in vascular atherosclerotic diseases.

The possible contribution of cytomegalovirus (CMV) to pathogenetic events associated with atherosclerotic lesion establishment and progression is still controversial. We evaluated the possibility that active ongoing CMV infection could be correlated to evolution of unstable atheromatous lesion, by analyzing patients suffering from unstable angina (n=61), acute myocardial infarction (n=43), stable angina (n=26) and peripheral arteriopathy (n=22) as compared to healthy subjects (n=30). Particularly, we assessed: past exposure to CMV by evaluating anti-CMV IgG antibodies; ongoing CMV infection by evaluating anti-CMV IgM antibodies and circulating interleukin (IL)-8 in serum; and CMV DNAemia in peripheral blood mononuclear cells (PBMC). Mean IgG values were significantly increased in patients from all groups, as compared to healthy subjects. CMV-specific IgM, as well as CMV DNAemia, were undetectable in both controls and patients. Circulating IL-8, significantly elevated in a group of individuals experiencing active CMV infection, was not significantly higher in cardiovascular disease patients, as compared to control subjects. These findings confirm previous evidence from the increased exposure to CMV infection in patients with atheromatous lesions. However, they provide further evidence against a direct implication of active systemic CMV infection in the pathogenesis of cardiovascular diseases, particularly those involving plaque instability.

Are elevated levels of soluble ICAM-1 a marker of chronic graft disease in heart transplant recipients?

Positivity for circulating intercellular adhesion molecule-1 (ICAM-1) in heart transplant recipients has been claimed to predict the development of coronary artery disease and risk of graft failure. Soluble ICAM-1 were evaluated in 32 heart transplant recipients. Five of these patients, who had undergone transplantation several years before, were positive for soluble ICAM-1 but did not present any clinical sign of graft rejection. Furthermore, although heart graft coronary disease was diagnosed in 15 of the 32 patients, they did not show significantly higher titres of soluble ICAM-1 compared to the remaining patients. These findings suggest that major caution is necessary when considering ICAM-1 positivity as a marker of graft disease.

Increased thrombin generation and circulating levels of tumour necrosis factor-alpha in patients with chronic Helicobacter pylori-positive gastritis.

BACKGROUND: Conflicting data have been reported concerning the relationship between Helicobacter pylori infection and coronary heart disease. AIM: To evaluate clotting system activation and plasma levels of tumour necrosis factor-alpha, a procoagulant cytokine, in patients with H. pylori-positive and -negative gastritis. METHODS: Three groups of patients were identified: 38 with H. pylori-positive gastritis, 18 with H. pylori-negative gastritis, and 40 H. pylori-negative controls with normal gastric mucosa. Plasma levels of prothrombin fragment 1 + 2 (F1 + 2) and tumour necrosis factor-alpha were assayed. Patients were also controlled after 2 and 6 months following standard H. pylori eradication treatment. RESULTS: At baseline, fragment 1 + 2 and tumour necrosis factor-alpha levels in H. pylori-positive patients were significantly higher than those in H. pylori-negative patients with gastritis (P < 0.05 and P < 0.01, respectively). After H. pylori eradication, fragment 1 + 2 and tumour necrosis factor-alpha levels showed a significant decrease at 2 months (P = 0.03 and P = 0.02, respectively) and a further reduction at 6 months, reaching levels observed in H. pylori-negative patients and controls. CONCLUSIONS: The increase thrombin generation rate and the correlation of plasma fragment 1 + 2 and tumour necrosis factor-alpha levels in H. pylori-positive patients suggest a role for inflammation in mediating the relationship between H. pylori infection and activation of the clotting system.

Met-enkephalin levels during PTCA-induced myocardial ischemia.

Met-enkephalin (Met-enk) has been demonstrated to modulate myocardial-ischemia mechanisms via the opioid receptors, but no studies are now available on Met-enk levels in the coronary circulation. In this experience Met-enk levels were evaluated in aortic root and in coronary sinus at baseline (T0), during PTCA induced transient ischemia (T1) and during reperfusion (T2). No significant differences were found at any time. Thus, it appears that there is no Met-enk extraction from the coronary circulation during provoked myocardial ischemia and no Met-enk release from the ischemic heart.

Prevalence of human herpesvirus (HHV)-8 infection among carriers of cardiovascular disease.

Infectious agents, such as herpesviruses, have been hypothesized to be involved in development of atheromatous plaque. The study aim was to evaluate the possibility that HHV-8 infection could be an additional risk factor for the establishment of cardiovascular disease. HHV-8 seroprevalence was determined by immunofluorescence in a population of cardiovascular disease patients (n=50) as compared to an age- and sex-matched group of control subjects (n=47); HHV-8 genome was detected in DNA extracted from circulating PBMC and from atheromatous lesions by PCR with primers specific for the minor virus capsid gene (ORF 26). The seroprevalence of HHV-8 was significantly increased in the patients as compared to the control population, while the presence of HHV-8 genome was observed in PBMC from 2 patients and 1 control. Virus-specific DNA was found in 2 out of 4 atheromatous plaques. The higher seroprevalence in patients suffering from vascular diseases as compared to age-and sex-matched controls suggests that HHV-8 infection could be an additional risk factor for the establishment of cardiovascular disease, although the data on the persistence of viral DNA in PBMC or in the arterial lesions are too exiguous to definitively support this hypothesis. More extensive studies are needed to define the exact role of HHV-8 infection in the establishment and progression of atheromatous lesions.

Left ventricular filling in young patients affected by insulin-dependent diabetes mellitus: a stress Doppler echocardiographic study.

BACKGROUND: The purpose of this study was to analyze diastolic function in two different populations of young patients affected by insulin dependent diabetes mellitus (IDDM), with and without systemic diabetic complications, and to compare the results obtained at rest with those obtained during isometric exercise. METHODS: Forty-five IDDM diabetic patients were studied. On the basis of presence or absence of systemic diabetic complications (nephropathy and retinopathy) patients were divided into two groups. Group I (20 patients) without and Group II (25 patients) with diabetic complications. Diastolic function parameters were measured by Doppler echocardiography at rest and during isometric exercise. The two groups were similar regarding age, metabolic control and insulin dosage but there were significant differences in diastolic and systolic blood pressure and heart rate between the two groups, the patients with complications showing higher values (p < 0.001, p < 0.005, p < 0.01 respectively). RESULTS: Group II already presented at rest alterations of diastolic function parameters respect to Group I with a marked increase of: peak velocity of late left ventricular filling (peakA cm/sec), (60.1 +/- 13.4 versus 48.4 +/- 8.9, p < 0.01); late left ventricular filling integral (A area), (6.5 +/- 1.4 versus 5.0 +/- 0.8, p < 0.05); late left ventricular filling integral over total filling (A area/total area), (0.31 +/- 0.06 versus 0.26 +/- 0.06, p < 0.01). There was a decrease of E/A ratio in Group II versus Group I (1.5 +/- 0.32 versus 1.9 +/- 0.5, p < 0.05). During isometric exercise these changes became even more pronounced in patients with complications and in this group there was a marked decrease of flow integral of early left ventricular filling over total filling (E area/total area), (0.57 +/- 0.09 versus 0.68 +/- 0.07, p < 0.01). CONCLUSIONS: In conclusion, young IDDM patients with complications show an impairment of diastolic function more pronounced than those without. These changes are more evident during isometric exercise. Stress Doppler echocardiography is a reliable tool to detect early diastolic dysfunction in diabetic patients.

Left ventricular filling in young patients affected by insulin-dependent diabetes mellitus: a stress Doppler echocardiography study.

BACKGROUND: The purpose of this study was to analyze diastolic function in two different populations of young patients affected by insulin dependent diabetes mellitus (IDDM), with and without systemic diabetic complications, and to compare the results obtained at rest with those obtained during isometric exercise. METHODS: Forty-five IDDM diabetic patients were studied. On the basis of presence or absence of systemic diabetic complications (nephropathy and retinopathy) patients were divided into two groups. Group I (20 patients) without and Group II (25 patients) with diabetic complications. Diastolic function parameters were measured by Doppler echocardiography at rest and during isometric exercise. The two groups were similar regarding age, metabolic control and insulin dosage but there were significant differences in diastolic and systolic blood pressure and heart rate between the two groups, the patients with complications showing higher values (p < 0.001, p < 0.005, p < 0.01 respectively). RESULTS: Group II already presented at rest alterations of diastolic function parameters respect to Group I with a marked increase of: peak velocity of late left ventricular filling (peakA cm/sec), (60.1 +/- 13.4 versus 48.4 +/- 8.9, p < 0.01); late left ventricular filling integral (A area), (6.5 +/- 1.4 versus 5.0 +/- 0.8, p < 0.05); late left ventricular filling integral over total filling (A area/total area), (0.31 +/- 0.06 versus 0.26 +/- 0.06, p < 0.01). There was a decrease of E/A ratio in Group II versus Group I (1.5 +/- 0.32 versus 1.9 +/- 0.5, p < 0.05). During isometric exercise these changes became even more pronounced in patients with complications and in this group there was a marked decrease of flow integral of early left ventricular filling over total filling (E area/total area), (0.57 +/- 0.09 versus 0.68 +/- 0.07, p < 0.01). CONCLUSIONS: In conclusion, young IDDM patients with complications show an impairment of diastolic function more pronounced than those without. These changes are more evident during isometric exercise. Stress Doppler echocardiography is a reliable tool to detect early diastolic dysfunction in diabetic patients.

Perspectives in the treatment of dyslipidemias in the prevention of coronary heart disease.

In this review the indications for the available treatments for dyslipidemias in the prevention of coronary heart disease (CHD) are considered, and their efficacy according to the latest studies is analyzed. As data sources the authors used the main multicenter studies performed in the last twenty years to evaluate primary and secondary prevention of CHD by correcting dyslipidemias as well as the results of meta-analyses of these studies. All treatments considered were found effective in preventing CHD morbidity and mortality to some extent. In particular, the combination of diet with niacin or hydroxymethylglutaryl coenzyme A (HMG CoA) reductase inhibitors seems to give the best results. These drugs induce a marked reduction of total and low-density lipoprotein (LDL) cholesterol and an increase of high-density lipoprotein (HDL) cholesterol concentrations. The use of diet, niacin, and HMG CoA reductase inhibitors reduces total as well as specific mortality. Treatment of dyslipidemia to prevent CHD depends on the pattern and severity of dyslipidemia, the presence of overt CHD, and the patient's response to diet. Pharmacologic treatment should be started only after dietary modifications have been tried and must be combined with diet. Drug side effects must also be considered, for they may affect patient compliance. High levels of total and LDL and low levels of HDL cholesterol are major risk factors for coronary atherosclerosis. Correcting lipid abnormalities can reduce the risk of development or progression of CHD. Diet and drugs are the main instruments available to normalize lipid levels. The choice of drug to combine with diet must be based on its specific effects on lipid metabolism, side effects, and efficacy in reducing CHD.

Angiotensin-converting-enzyme inhibitor administration must be monitored for serum amylase and lipase in order to prevent an acute pancreatitis: a case report.

Some clinical cases published in literature show that angiotensin-converting enzyme (ACE)-inhibitor administration may cause acute pancreatitis. In this work, the authors report a case of a patient affected by hypertension. Upon admission, the authors started antihypertensive therapy using captopril, which caused an important amylase and lipase rise within 13 days. When the ACE-inhibitor therapy was stopped, a rapid decrease of the serum enzyme was observed within 3 days. The high levels of serum amylase and lipase were linked to neutrophilia but were not associated with relevant symptomatic findings or features of pancreatopathy. The absence of the usual conditions that may cause pancreatitis, such as biliary stasis, hypercalcemia, or alcohol abuse, and the rapid decrease of serum enzyme levels after drug suspension suggested an ACE-inhibitor-induced pancreatitis. This is the first clinical report of an ACE-inhibitor-induced pancreatitis in which captopril administration was found after hospitalization. The drug suspension probably prevented other complications. This case report suggests that, when ACE-inhibitor administration is started, serum amylase and lipase should be monitored in order to prevent acute pancreatitis without waiting for clinical evidence of a pancreatopathy.

Alteration of left ventricular filling evaluated by Doppler echocardiography as a potential marker of acute rejection in orthotopic heart transplant.

To evaluate the changes in left ventricular (LV) filling associated with acute cardiac rejection, serial Doppler echocardiographic (ED) examination were performed on the same day as endomyocardial biopsy (EMB) in 40 patients who underwent orthotopic transplantation. The diameters and wall thickness of the left ventricle were measured. The indexes of LV filling in the following parameters were measured by pulsed Doppler: isovolumic relaxation time (IRT), peak early mitral flow velocity (V max E), and pressure half-time (PHT). The patients were classified into three groups on the basis of EMB: Group I (19 patients without rejection), Group II (11 patients with mild or moderate rejection), and Group III (10 patients with severe rejection). In Group III rejection was associated with a significant increase of posterior wall thickness (P < 0.05), with a decrease of IRT (P < 0.05), and an increase of V max E velocity (P < 0.01) in comparison with Group I. In Group II, Doppler indexes were not statistically significant in comparison with Groups I and II. In conclusion, in transplant patients, a diagnosis of acute rejection can be suspected in severe rejection by use of echocardiography when the diagnosis is based on a multiparametric evaluation of different ED indexes (m-mode and Doppler indexes). Doppler echocardiography is a method with an excellent specificity but insufficient sensitivity; this is due to the influence of recipient atrial contraction timing on Doppler indexes of LV filling.

Influence of thrombolytic therapy on inferior acute myocardial infarction with concomitant anterior ST segment depression.

The purposes of this study were to analyze the prognostic significance of precordial ST segment depression and to determine whether thrombolytic therapy is effective for all patients with inferior acute myocardial infarction (AMI) or whether there is a different effectiveness for patients with concomitant anterior ST segment depression persisting for 24 hours or longer. Medical charts of 176 patients were studied. On the basis of ECG the patients were subclassified into three groups according to the presence, persistence, or absence of significant ST segment depression: Group 1: anterior ST segment depression persisting for less than 24 hours (45.4%); Group 2: anterior ST segment depression persisting for more than 24 hours (17.6%); Group 3: no anterior ST segment depression (37%). Age, Killip class, peak creatine kinase, hospital deaths, left ventricular ejection fraction, regional wall motion score, postinfarction angina, and ventricular/supraventricular arrhythmia of all patients were studied. Parameters of the three groups were compared: worse results were found in group 1 and the worst in group 2. This result is independent of thrombolytic therapy. Finally, the same parameters of thrombolyzed and nonthrombolyzed groups were compared: no statistically significant difference was observed. Among thrombolyzed patients the number of those with ST depression lasting more than 24 hours is lower than in nonthrombolyzed patients. It can be assumed that thrombolytic therapy in inferior AMI determines a shifting of patients from a worse prognosis group (ST segment depression persisting for more than 24 hours) to a better prognosis group (ST segment depression persisting for less than 24 hours).

Myocardial scintigraphy with 99mTc-sestamibi in children with Kawasaki disease.

Kawasaki disease (KD) is a systemic vasculitis syndrome of early childhood. It involves particularly the coronary arteries and may cause aneurysms and thrombotic occlusions. Echocardiography is the most useful method of detecting coronary aneurysms. Nevertheless, obstructive lesions are difficult to evaluate and often need invasive coronary angiography. An important feature of this disease is the possibility of finding coronary pathology several years after the onset. This characteristic makes KD an important cause of coronary artery disease (CAD) in young adults. Thus patients with KD and previously diseased coronary arteries should be kept under long-term control. However, coronary angiography is invasive and cannot be performed repeatedly, especially in young patients. As an alternative, thallium 201 scintigraphy has been employed, but its low-energy photons are suboptimal for standard gamma-camera imaging, particularly in children aged less than three years. To verify the usefulness of a noninvasive assessment of myocardial perfusion, the authors used rest and dipyridamole 99mTc-Sestamibi scan in 15 children (ranging from one to six years of age) with Kawasaki's cardiac involvement. Coronary aneurysms have been demonstrated by echocardiography in 12 patients; 8 patients were also submitted to cardiac catheterization. The sensitivity of 99mTc-Sestamibi imaging for detection of overall coronary lesions was 88% and the specificity was 93%. These data suggest that rest/dipyridamole 99mTc-Sestamibi scintigraphy is an accurate and noninvasive method for the detection and follow-up of Kawasaki's cardiac damage even in patients aged one year.

ET-1 plasma levels during cold stress test in sclerodermic patients.

Endothelial cell injury in blood vessels of small arteries and capillaries is considered the primary event in the pathogenesis of systemic sclerosis (SSc). Because endothelin-1 (ET-1) is mainly released in the site of endothelial cell damage, thereby inducing a potent vasoconstriction, it was our intention to study ET-1 release in a group of SSc patients during a cold pressor test (CPT). Twelve SSc patients and a control group of 10 healthy subjects underwent CPT. Blood samples for ET-1 assay were collected at 90 and 180 seconds of exposure to cold stress. Heart rate and blood pressure were recorded at the same intervals. A capillaroscopic examination was performed in both groups before and after CPT. We observed significantly higher levels of plasma ET-1 in SSc patients compared with those of the control group at baseline (P=0.007) and at 90 (P=0.006) and 180 seconds (P=0.03) of CPT. During the test, the capillaroscopic examination showed a dramatic worsening of the vascular picture that was parallel to the increase in ET-1 plasma levels. This suggests that higher ET-1 plasma levels can have a part in the acute vascular reactivity of SSc patients undergoing CPT.