[Eosinophilic gastrointestinal disease - a rare condition of unknown cause]. / Eosinofil gastrointestinal sjukdom ­ tillstånd med skiftande symtom.

Eosinophilic gastrointestinal disease is a rare condition of unknown cause with rising incidence in adults and characterized by chronic inflammation in the gastrointestinal tract with accumulation of eosinophils in the gastrointestinal wall, without other apparent cause (e.g., infections, IBD). There are three types of eosinophilic gastrointestinal disease, classified according to the location of the eosinophilic infiltration: eosinophilic esophagitis, eosinophilic gastroenteritis, and eosinophilic colitis. A combination of clinical suspicion, histologic evidence of eosinophilic infiltration, and exclusion of other causes of tissue eosinophilia leads to the diagnosis. Elimination diet, corticosteroid therapy, and steroid-sparing agents are currently used to treat eosinophilic gastrointestinal disease.

Thirty-Day Results of the Novel CGuard-Covered Stent in Patients Undergoing Carotid Artery Stenting.

OBJECTIVE: The aim of this study was to provide early-term evaluation, safety, and efficacy of the novel CGuard (Inspire MD, Tel Aviv, Israel) micromesh self-expanding stent with embolic protection system (EPS) during carotid artery stenting (CAS) procedure. MATERIALS AND METHODS: All patients who underwent CAS with CGuard carotid stent system from January 2018 to September 2020 in a tertiary center were prospectively evaluated. Primary endpoints included technical success and perioperative neurological events (0-48 hours). Secondary endpoint was the rate of neurologic, cardiac events, and death (major adverse event or MAE) at 30 days. Patency of CGuard, peak systolic velocity (PSV), and end diastolic velocity (EDV) were evaluated at 30 days with duplex ultrasound. RESULTS: A total of 103 patients underwent CAS procedure. Forty patients were symptomatic, and 63 patients were asymptomatic with stenosis greater than 70%. Technical success was 100%. Local anesthesia was applied in 94 patients (93%). Embolic protection devices were used in 6 patients (5.8%). Access site complications were 1.9%. Mean internal carotid artery stenosis diameter reduced from 79.47% to 14.68%. Two patients had transient ischemic attack (1.9%) and 1 patient experienced a cerebral hyperperfusion syndrome (CHS) perioperatively. External carotid artery (ECA) occlusion occurred in 1.9%. Four patients experienced jaw claudication (3.8%) postoperatively. Mean time of operation was 41 minutes. Mean duration of hospitalization was 3.1 days. The 30-day rate of MAE was 0%. CGuard patency was 100%, mean internal carotid PSV was reduced from 251.57 to 77.29 cm/s, and mean internal carotid EDV was reduced from 154.62 to 24.63 cm/s at 30 days. CONCLUSION: Our study shows that CGuard stent with EPS is an effective and safe device for treatment of carotid artery stenosis with acceptable low perioperative neurologic events, even with low embolic protection device usage. Larger multicenter and randomized studies are necessary to confirm its long-term efficacy.

Whipple's disease: the great masquerader-a high level of suspicion is the key to diagnosis.

BACKGROUND: Whipple's disease is a chronic infectious disease that primarily affects the small intestine, but several organs can simultaneously be involved. The disease is caused by a gram-positive bacterium called Tropheryma whipplei. The disease is difficult to suspect because it is rare with unspecific and long-term symptoms; it can be lethal if not properly treated. CASE PRESENTATION: We here present three patients who presented with a plethora of symptoms, mainly long-standing seronegative arthritis and gastrointestinal symptoms in the form of diarrhea with blood, weight loss, fever, and lymphadenopathy. They were after extensive investigations diagnosed with Whipple's disease, in two of them as long as 8 years after the first occurrence of joint manifestations. The diagnosis was made by PCR targeting the T. whipplei 16S rRNA gene from small bowel specimen in all three patients, and, besides from histopathologic findings from the duodenum and distal ileum in one and mesenteric lymph nodes in another patient. CONCLUSIONS: This report aims to raise awareness of a very rare disease that presents with a combination of symptoms mimicking other and significantly more common diseases.

[Trousseau's syndrome: a combination of cancer and hypercoagulability]. / Trousseaus syndrom ­ en kombination av cancer och hyperkoagulabilitet.

Trousseau's syndrome (TS) is a combination of cancer and hypercoagulability, considered to be a chronic disseminated intravasal coagulation which leads to venous thromboembolism and arterial thrombosis in cancer patients. In TS, thrombosis results from an activation of hemostasis by different mechanisms. Cardioembolic stroke is the most common type that occurs in patients with malignancy, partly due to cancer-related hypercoagulability. The classical presentation of TS comprises multiple bilateral ischemic brain lesions in both supra- and infratentorial regions of the brain. TS occurs most commonly in patients with lung, pancreas, breast and colon cancer, usually mucin-producing cancer. TS manifests most commonly within 30 days of occurrence of cancer symptoms or longer in local and metastatic cancer, respectively. Standard treatment of TS is low molecular weight heparin, whereas in recent years non-vitamin K oral anticoagulants have been tested and not shown better effect so far, but further studies are required.

[Argon plasma coagulation effective treatment of solitary rectal ulcer syndrome]. / Argonplasmakoagulation effektiv vid solitärt rektalt ulkussyndrom.

Solitary rectal ulcer syndrome (SRUS) is a rare disorder likely to be caused by a traumatic and ischemic mucosal injury related to latent or overt rectal prolapse. Mucosal damage can vary between erythema and up to intractable ulceration. The typical symptoms of SRUS may resemble those of inflammatory bowel disease (IBD)-related proctitis or rectal malignancy with mucus- and blood-mixed defecation with urgency and sometimes transient incontinence. The purpose of this paper is to raise awareness of this rare, quality-of-life impacting and difficult-to-treat disease and the not so well-known argon plasma coagulation (APC) treatment that can provide very good and long lasting symptom relief.

[Whipple's disease: very rare, fatal without treatment but easily cured]. / Whipples sjukdom: sällsynt, dödlig utan behandling, men lätt botad.

Whipple's disease is a chronic infectious disease that primarily affects the small intestine, but several organs can be involved simultaneously. The disease is caused by a gram-positive bacterium called Tropheryma whipplei. The disease is difficult to suspect because it is rare, and produces unspecific and long-term symptoms. Whipple's disease can lead to death if not treated. We here present a case of a man who presented with gastrointestinal symptoms in the form of diarrhea with blood, weight loss, fever, and lymphadenopathy and who was finally diagnosed with Whipple's disease 4 years after the occurrence of manifestations from the joints. The diagnosis was made both with 16S rRNA against Tropheryma whipplei and histopathologically from biopsy taken from the duodenum and distal ileum. The purpose of this case report is to raise awareness of a very rare disease that presented with a combination of symptoms similar to other and significantly more common diseases.

A case of fatal cutaneous caput medusae hemorrhage.

Alcoholic liver cirrhosis leads to portal venous hypertension, which can result in a caput medusae formation. Life-threatening hemorrhage from a ruptured caput medusae vein is a rare complication. It is crucial to stop the bleeding promptly. A transjugular intrahepatic portosystemic shunt is considered potentially lifesaving.

A case of extreme weight loss due to mesenteric ischemia and antiphospholipid syndrome.

Mesenteric ischemia and antiphospholipid syndrome is a rare combination but should be suspected as a differential diagnosis. This may be presented as diffuse abdominal pain typically after food intake, diarrhea, and weight loss. Early recognition is warranted, and nutrition, stenting, and anticoagulant treatments are indicated.

Endovascular Repair for a Ruptured AAA due to a Combined Type IIIb and Ia Endoleak.

We report a case of a ruptured abdominal aortic aneurysm (AAA) caused by a combined type IIIb and Ia endoleak. Also, we propose the mechanism that resulted in this combined endoleak. Specifically, a 71-year old-man, with a previous history of endovascular aneurysm repair (EVAR) for an AAA, was diagnosed with a contained rupture. CT scan depicted a type Ia endoleak and a migrated Talent endograft. A proximal aortic cuff sealed the endoleak, but intraoperative angiography revealed that a type IIIb endoleak coexisted due to fabric tear close to the Talent bifurcation. A second aortic cuff could not seal the fabric tear; so, in-lay parallel limbs were sequentially deployed as a "kissing endograft" technique inside the cuff. Simultaneous treatment of combined type IIIb and Ia endoleaks has not yet been described. Maybe the type IIIb endoleak is the primary entity causing sac enlargement, neck recontouring, proximal migration, and ultimately type Ia endoleak, which leads to huge enlargement and rupture. Placement of an aortic cuff to seal the proximal endoleak/migration and kissing endografts limbs for the fabric tear seems a safe option in such patients.

Endovascular Repair of Blunt Thoracic Aortic Trauma: Is Postimplant Hypertension an Incidental Finding?

BACKGROUND: Blunt thoracic aortic injury (BTAI) is the second most common cause of death in trauma patients. Nowadays, thoracic endovascular aortic repair (TEVAR) has become the treatment of choice because of lower rates of mortality, paraplegia, and stroke. However, concerns have been raised whether graft implantation is related to the development of hypertension in the postoperative period. The aim of this study was to report short- and long-term outcomes of patients undergoing TEVAR for BTAIs at a tertiary hospital and to investigate postimplant hypertension. METHODS: Between January 2005 and January 2016, 23 patients with blunt thoracic aortic trauma underwent TEVAR. Median age was 44 years (range, 18-73). Among them, 14 (60.9%) patients were diagnosed with aortic rupture, whereas 9 (39.1%) with pseudoaneurysm. Α single thoracic stent graft was deployed in 21 patients, and the rest 2 patients received 2 stent grafts. RESULTS: Complete exclusion of the injury was feasible in all subjects (100% primary success). The left subclavian artery (SCA) was intentionally covered in 6 patients (26%). Intraoperative complications included one nonfatal stroke managed conservatively and one external iliac artery rupture treated with iliofemoral bypass. One patient (4.3%) died on the first postoperative day in the intensive care unit (ICU) because of hemorrhagic shock. The overall 30-day mortality and morbidity were 4.3% and 8.7%, respectively. New-onset postimplantation arterial hypertension was observed in 8 (34.8%) previously nonhypertensive patients. Younger age (P = 0.027) and SCA coverage (P = 0.01) were identified as potential risk factors for the development of postimplant hypertension, whereas the presence of concomitant injuries (P = 0.3) and intraoperative complications (P = 0.1) were not. After a median follow-up of 100 months (range, 18-120), 6 of them still remain on antihypertensive therapy, whereas the other 2 did not require permanent treatment. CONCLUSIONS: TEVAR is a safe approach in the treatment of BTAI associated with low short- and long-term morbidity and mortality rates. Lower age and SCA coverage may contribute to the development of postimplant hypertension. Further larger cohort studies are warranted to elucidate the underlying mechanisms of postimplant hypertension.

Prognostic Factors in Patients Treated with Drug-Coated Balloon Angioplasty for Symptomatic Peripheral Artery Disease.

PURPOSE: Aim of this study is to report real-life experience on the treatment of peripheral artery disease (PAD) with a specific drug-coated balloon (DCB), and to evaluate potential prognostic factors for outcomes. MATERIALS AND METHODS: This is a retrospective study reporting outcomes in patients with PAD who were treated with the Lutonix DCB during a four-year period. Major outcomes included: all-cause mortality, amputation, clinical improvement, wound healing and target lesion revascularization (TLR). Mean follow-up was 24.2±2.3 months. RESULTS: Overall, 149 patients (mean age: 68.6±8.3 years; 113 males) were treated, either for intermittent claudication (IC) (n=86) or critical limb ischemia (CLI) (n=63). More than half the target lesions (n=206 in total) were located in the superficial femoral artery and 18.0% were below-the-knee lesions. CLI patients presented more frequently with infrapopliteal (P=0.002) or multilevel disease (P=0.0004). Overall, all-cause mortality during follow-up was 10.7%, amputation-free survival was 81.2% and TLR-free survival was 96.6%. CLI patients showed higher all-cause mortality (P=0.007) and total amputation (P=0.0001) rates as well as lower clinical improvement (P=0.0002), compared to IC patients. Coronary artery disease (CAD), gangrene and infrapopliteal disease were found to be predictors for death whereas CLI and gangrene were found to be predictors for amputation, during follow-up. CONCLUSION: PAD treatment with Lutonix DCBs seems to be an efficient and safe endovascular strategy yielding promising results. However, CAD, gangrene, CLI and infrapopliteal lesions were found to be independent predictors for adverse outcomes. Larger series are needed to identify additional prognostic factors.

Endovascular aneurysm repair with fascia suture technique: short and mid-term results.

BACKGROUND: Percutaneous endovascular aneurysm repair (p-EVAR), has been widely spread based on the recent improvements in stent-graft technology and mainly in delivery system downsizing. Aim of this study is to investigate the safety and efficacy of fascia suture technique (FST) in p-EVAR during the short and mid-term follow-up (FU). METHODS: Between April 2011 and July 2013, 64 consecutive patients with abdominal aortic aneurysm were enrolled in a prospective single center registry. Fifty-four patients were eligible for elective p-EVAR assisted by the fascia suture technique. Patients were prospectively followed with duplex scan 24 hours and 30 days postoperatively and with CTA annually thereafter. Femoral haematoma, pseudoaneurysm and limb ischaemia were the primary outcomes. RESULTS: The study investigated 103 femoral arteries reconstructions using the FST. Intraoperatively, one patient was diagnosed with limb ischaemia treated with open repair. During the short-term, 4 (3.8%) pseudoaneurysms were diagnosed, treated with open (2) or endovascular (2) repair. During mid-term 43 patients (85 arteries, 82.6%) underwent CTA. Eighteen (17.4%) patients were lost at FU. At 12 months CTA two pseudoaneurysms (2.35%) were detected, treated with open repair. CONCLUSIONS: The FST seems safe and effective for femoral reconstruction after p-EVAR. Complications are comparable to closure devices and to conventional repair.

Active proximal sealing in the endovascular repair of abdominal aortic aneurysms: early results with a new stent-graft.

PURPOSE: To investigate the performance of a new device that uses the STRATA polytetrafluoroethylene graft material and a mechanism that provides active proximal sealing in order to prevent type Ia endoleak during endovascular aneurysm repair (EVAR). METHODS: Between April 2013 and July 2014, 21 consecutive patients (all men; median age 71 years, range 60-84 years) with abdominal aortic aneurysm (median diameter 5.9 cm, range 4.9-7.8 cm) and suitable anatomy were offered elective EVAR using the AFX endograft. These patients had an irregular, conical, tapered, or bulging proximal neck, for which this specific device was considered appropriate. Aneurysm exclusion and incidence of type Ia endoleak were the primary outcomes; secondary outcomes included mortality, morbidity, migration, and other graft-related complications. RESULTS: Primary technical success was 90%; 2 intraoperative type Ia endoleaks due to low endograft deployment were treated with additional proximal cuffs. During a median follow-up of 10 months (range 2-15 months), no type I endoleak was observed. One type II endoleak was encountered, with no associated sac enlargement. There was no stent-graft migration or any other device-related complication. One patient had a nonfatal myocardial infarction and another developed renal failure requiring transient dialysis. No deaths occurred. CONCLUSION: In this early experience, this newly available device appears to be safe and efficient in providing seal along irregularly shaped necks over the short term.

Association between seven single nucleotide polymorphisms involved in inflammation and proteolysis and abdominal aortic aneurysm.

BACKGROUND: Abdominal aortic aneurysm (AAA) formation involves an inflammatory and proteolytic process. Previous studies suggest that AAA is a multifactorial disease with a strong genetic background. This study evaluated the role of seven important functional single nucleotide polymorphisms (SNPs) in AAA. METHODS: This was a case-control study of two independent populations: 397 AAA patients (mean aortic diameter, 6.2 ± 1.4 cm) and 393 controls (mean diameter, 2.4 ± .2 cm) recruited from Greece (the main cohort), and 400 patients (mean diameter: 5.4 ± 1 cm) and 400 controls (mean diameter, 2.4 ± .6 cm) recruited from the United Kingdom (replication cohort). The functional SNPs analyzed were rs3025058, rs3918242, rs2276109, rs1801133, rs1799752, rs1799983, and rs16874954. These regulate the following enzymes: matrix metalloproteinases (MMPs), angiotensin-converting enzyme, endothelial nitric oxide synthase, methylenetetrahydrofolate reductase (MTHFR), and platelet-activating factor acetylhydrolase or lipoprotein-associated phospholipase A2. RESULTS: Genotype distributions (univariate analyses) did not differ significantly between cases and controls in the main or the replication cohort, with the exception of the MMP-3 rs3025058 SNP, where differences were borderline significant (odds ratio [OR], 1.42; 95% confidence interval [CI], 1.02-1.97; P = .04) in the replication cohort. Adjusted analyses for age, sex, smoking, hypertension, and hypercholesterolemia disclosed no differences in either cohort. For SNPs that had previously been associated with AAA presence, meta-analysis of currently available data together with the two study cohorts disclosed positive associations for the MMP-3 rs3025058 (OR, 1.15; 95% CI, 1.06-1.25; P = .0009) and MTHFR rs1801133 (OR, 1.07; 95% CI, 1.02-1.12; P = .0088). CONCLUSIONS: The SNPs included in this analysis were not associated with AAA presence in either study population. However, meta-analysis of the currently available data disclosed a positive association for MMP-3 rs3025058 and MTHFR rs1801133.

Comparison of the impact of open and endovascular abdominal aortic aneurysm repair on renal function.

OBJECTIVE: The impact of any intervention on renal function is a crucial determinant of outcome. Open (OR) and endovascular (EVAR) abdominal aortic aneurysm (AAA) repair can affect renal function during the short and longer term. This study aimed to directly compare the effect of those different types of aneurysm repair during a period of 2 years. METHODS: This was a nested case-control study including patients undergoing either OR or EVAR of an infrarenal AAA. Three groups were included: OR, EVAR with suprarenal endograft fixation, and EVAR with infrarenal fixation. These were matched for age (within 2 years), sex, AAA size (within 1 cm), hypertension, smoking, and proximal neck diameter (within 5 mm). The primary end point was change in estimated glomerular filtration rate (eGFR) calculated by the Chronic Kidney Disease Epidemiology Collaboration formula at baseline, 6 months, 12 months, and 2 years. RESULTS: A total of 225 patients were included [(45 ORs matched vs 90 suprarenal and 90 infrarenal fixation EVARs; 35 women (16%); age, 71 ± 8 years; AAA size, 6.4 ± 1 cm]. Groups did not differ significantly in terms of diabetes, hypercholesterolemia, or baseline eGFR (P = .89). On average, those undergoing OR lost a mean 5.39 mL/min/1.73 m(2) (P = .48) within 1 year and 5.49 units (P = .42) after 2 years. The suprarenal fixation patients lost 5.58 units (P = .002) after 1 year and 6.57 units (P = .001) after 2 years. Finally, the infrarenal fixation patients lost 0.53 unit (P = .74) after 1 year and 2.24 units (P = .22) after 2 years. CONCLUSIONS: OR and suprarenal fixation EVAR are associated with significant declines in renal function during 2 years, in contrast to infrarenal EVAR fixation. The patterns of eGFR decline in OR and suprarenal fixation EVAR are not similar, suggesting different causal mechanisms.

Severe Plasmodium vivax malaria complicated with acute respiratory distress syndrome: a case associated with focal autochthonous transmission in Greece.

In 2011, autochthonous Plasmodium vivax malaria emerged in a focal geographical area in Greece after importation by immigrants from the Indian subcontinent. We report the case of complicated P. vivax malaria in a previously healthy 42-year-old Greek female. The patient presented acute respiratory distress syndrome (ARDS), worsening jaundice, and thrombocytopenia after the administration of antimalarial treatment and despite a decreasing burden of parasitemia. She recovered fully after admission in the intensive care unit and support with mechanical ventilation. We discuss the risks potentially associated with the reappearance of P. vivax malaria in a previously malaria-free area.

Obesity as an independent predictor of outcome after endovascular abdominal aortic aneurysm repair.

BACKGROUND: Obesity is increasingly common in patients referred for the management of an abdominal aortic aneurysm (AAA). Evidence of the effect of obesity on outcomes after endovascular repair (EVAR) is not well established. We sought to compare the immediate and midterm outcomes of elective EVAR between obese and nonobese patients in a case control study. METHODS: Patients undergoing elective EVAR were divided in 2 groups: obese (defined as a body mass index [BMI] ≥30 kg/m(2)) and nonobese (mean BMI [kg/m(2)] ± SD: 33 ± 1 vs 25 ± 3). Both groups were case-matched for age, sex, smoking, and AAA diameter. One hundred fifty-nine patients were included (mean age: 69 ± 9 years; 10 women [9%]; mean BMI: 28 ± 5 kg/m(2); 53 were obese and 106 were nonobese). All aneurysms were successfully excluded. Mean follow-up was 34 ± 13 months. RESULTS: All patients who developed a complication within the perioperative period (≤30 days) were obese (P = 0.01). Thirteen patients (8.2%) died during follow-up (8 obese versus 5 nonobese; P = 0.76). Survival and non-procedure-related morbidity did not differ significantly between the obese and nonobese groups (P = 0.64 and 0.16; log-rank test). BMI was not associated with mortality or non-procedure-related morbidity on multivariate analysis (mortality-hazard ratio: 1.0 [95% confidence interval: 0.9-1.2]; P = 0.37; non-procedure-related morbidity-hazard ratio: 1.0 [95% confidence interval: 0.9-1.1], P = 0.2). CONCLUSIONS: This is the first case control study to assess the independent impact of obesity in the outcome after EVAR. No difference was documented with regards to mortality or non-aneurysm-related morbidity.

Evaluation of IScore validity in a Greek cohort of patients with type 2 diabetes.

BACKGROUND: Diabetes constitutes a risk factor for stroke that also aggravates stroke prognosis. Several prognostic models have been developed for the evaluation of neurologic status, severity, short-term functional outcome and mortality of stroke patients. IScore is a novel tool recently developed in order to predict mortality rates within 30 days and 1 year after ischemic stroke and diabetes is not included in the scoring scale of IScore. The aim of the present study was to evaluate and compare IScore validity in ischemic stroke patients with and without diabetes. METHODS: This prospective study included 312 consecutive Caucasian patients with type 2 diabetes and 222 Caucasian patients without diabetes admitted for ischemic stroke in a tertiary Greek hospital. Thirty-day and 1-year IScores were individually calculated for each patient and actual mortality was monitored at the same time intervals. IScore's predictive ability and calibration was evaluated and compared for ischemic stroke patients with and without diabetes. The performance of IScore for predicting 30 and 1-year mortality between patients with and without diabetes was assessed by determining the calibration and discrimination of the score. The area under the receiver operating characteristic curve was used to evaluate the discriminative ability of IScore for patients with and without diabetes, whereas the calibration of IScore was assessed by the Hosmer-Lemeshow goodness-of fit statistic. RESULTS: Baseline population characteristics and mortality rates did not differ significantly for both cohorts. IScore values were significantly higher for patients with diabetes at 30 days and 1 year after ischemic stroke and patients with diabetes presented more frequently with lacunar strokes. Based on ROC curves analysis IScore's predictive ability for 30 day mortality was excellent, without statistically significant difference, for both cohorts. Predictive ability for 1 year mortality was also excellent for both groups with significantly better ability for patients with diabetes especially at high score values. Calibration of the model was good for both groups of patients. CONCLUSIONS: IScore accurately predicts mortality in acute ischemic stroke Caucasian patients with and without diabetes with higher efficacy in predicting 1 year mortality in patients with diabetes especially with high scores.