Acute thrombosis of the superior mesenteric artery in a 39-year-old woman with protein-S deficiency: a case report

<p>Abstract</p> <p>Introduction</p> <p>Acute thromboembolic occlusion of the superior mesenteric artery is a condition with an unfavorable prognosis. Treatment of this condition is focused on early diagnosis, surgical or intravascular restoration of blood flow to the ischemic intestine, surgical resection of the necrotic bowel and supportive intensive care. In this report, we describe a case of a 39-year-old woman who developed a small bowel infarct because of an acute thrombotic occlusion of the superior mesenteric artery, also involving the splenic artery.</p> <p>Case presentation</p> <p>A 39-year-old Caucasian woman presented with acute abdominal pain and signs of intestinal occlusion. The patient was given an abdominal computed tomography scan and ultrasonography in association with Doppler ultrasonography, highlighting a thrombosis of the celiac trunk, of the superior mesenteric artery, and of the splenic artery. She immediately underwent an explorative laparotomy, and revascularization was performed by thromboendarterectomy with a Fogarty catheter. In the following postoperative days, she was given a scheduled second and third look, evidencing necrotic jejunal and ileal handles. During all the surgical procedures, we performed intraoperative Doppler ultrasound of the superior mesenteric artery and celiac trunk to control the arterial flow without evidence of a new thrombosis.</p> <p>Conclusion</p> <p>Acute mesenteric ischemia is a rare abdominal emergency that is characterized by a high mortality rate. Generally, acute mesenteric ischemia is due to an impaired blood supply to the intestine caused by thromboembolic phenomena. These phenomena may be associated with a variety of congenital prothrombotic disorders. A prompt diagnosis is a prerequisite for successful treatment. The treatment of choice remains laparotomy and thromboendarterectomy, although some prefer an endovascular approach. A second-look laparotomy could be required to evaluate viable intestinal handles. Some authors support a laparoscopic second-look. The possibility of evaluating the arteriotomy, during a repeated laparotomy with a Doppler ultrasound, is crucial to show a new thrombosis. Although the prognosis of acute mesenteric ischemia due to an acute arterial mesenteric thrombosis remains poor, a prompt diagnosis, aggressive surgical treatment and supportive intensive care unit could improve the outcome for patients with this condition.</p

Near total parathyroidectomy for the treatment of renal hyperparathyroidism

Background: Different surgical strategies are used to treat medical refractory renal hyperparathyroidism. Our preferred choice in patients with moderate secondary hyperparathyroidism (SHPT) and in patients with low compliance with medical treatment is to leave a very small parathyroid remnant in situ: we name this operation "near total parathyroidectomy" (ntPTX). We report here our results with this technique. Methods: Retrospective study [2001-2015] of all patients submitted to ntPTX in a single centre. Results: Forty-seven patients were submitted to ntPTX (32 males) aged 47.3 years. Follow-up time is 8.5 years. Thirty-five patients (74%) are alive, 12 are dead. One patient in this series had a functioning renal transplant at time of ntPTX (tertiary hyperparathyroidism), and other 27 subsequently received a renal transplantation (RTx) after ntPTX (still functioning at last follow-up or at death in 19). Amongst the 35 current survivors, the renal graft is functioning in 16 (45.7%). Parathyroid hormone (PTH) at follow-up was 116.1±135.5 pg/mL and calcium 8.6±0.9 mg/dL. Among patients with a functioning RTx PTH was 83 pg/mL and calcium 8.7 mg/dL. There was no persistent disease, and 3 patients (6.4%) had a relapse of hyperparathyroidism at follow-up. Conclusions: ntPTX is associated to very satisfying rates of normal parathyroid function and of relapse of hyperparathyroidism (6.4%) at long term, either in case of RTx or of maintenance hemodialysis: the concept of "small amount" remnant represents a valuable choice for patients undergoing PTX with a realistic chance of receiving a RTx

Near total parathyroidectomy for the treatment of renal hyperparathyroidism

Background: Different surgical strategies are used to treat medical refractory renal hyperparathyroidism. Our preferred choice in patients with moderate secondary hyperparathyroidism (SHPT) and in patients with low compliance with medical treatment is to leave a very small parathyroid remnant in situ: we name this operation "near total parathyroidectomy" (ntPTX). We report here our results with this technique. Methods: Retrospective study [2001-2015] of all patients submitted to ntPTX in a single centre. Results: Forty-seven patients were submitted to ntPTX (32 males) aged 47.3 years. Follow-up time is 8.5 years. Thirty-five patients (74%) are alive, 12 are dead. One patient in this series had a functioning renal transplant at time of ntPTX (tertiary hyperparathyroidism), and other 27 subsequently received a renal transplantation (RTx) after ntPTX (still functioning at last follow-up or at death in 19). Amongst the 35 current survivors, the renal graft is functioning in 16 (45.7%). Parathyroid hormone (PTH) at follow-up was 116.1±135.5 pg/mL and calcium 8.6±0.9 mg/dL. Among patients with a functioning RTx PTH was 83 pg/mL and calcium 8.7 mg/dL. There was no persistent disease, and 3 patients (6.4%) had a relapse of hyperparathyroidism at follow-up. Conclusions: ntPTX is associated to very satisfying rates of normal parathyroid function and of relapse of hyperparathyroidism (6.4%) at long term, either in case of RTx or of maintenance hemodialysis: the concept of "small amount" remnant represents a valuable choice for patients undergoing PTX with a realistic chance of receiving a RTx

Diagnosis and follow-up of idiopathic retroperitoneal fibrosis. role of 18F-FDG-PET/CT and biochemical parameters in patients with renal involvment

Idiopathic retroperitoneal fibrosis (IRF) is a rare disease characterized by fibro-inflammatory reaction surrounding ureters and other inner organs with possible secondary renal involvement. Symptoms are aspecific and recurrent phases of activity are generally associated with elevation of inflammatory indices. 18F-FDG-PET is nowadays an important tool for the detection of this disease, allowing differentiation between metabolically active tissue and fibrotic one. The purpose of this study was to investigate the role of 18F-FDG-PET in the management of IRF and to evaluate possible correlations between biochemical parameters and PET/CT findings of disease activity. We enrolled seven consecutive patients with IRF (in five histology proved the disease) observed from 2003 to 2012 (5 M:2 F, mean age 53.8 years, range 44-86 years). All patients presented with fever as first symptom; two had obstructive renal failure requiring hemodialysis; one underwent monolateral nephrectomy for parenchyma infiltration; six presented ureteral involvement; three underwent ureteral stent placement. For each patient, during a mean total follow-up of 26.5 months we evaluated serum creatinine, BUN, Hb, RBCs, WBCs, PLT, CRP, ESR. Periodic 18F-FDG-PET/CT scans (every 5.9 months-mean) were performed in all patients. Statistical evaluation was performed using "stepwise regression" analysis. Steroids and immunosuppressive agents induced a progressive normalization of PET/CT scans in all patients at the end of follow-up. Stepwise regression analysis showed that BUN, serum creatinine and CRP only if considered together, significantly correlated with SUV max (p value = 0.000003057). 18F-FDG-PET is a useful tool for clinical decision making in patient with IRF, allowing to evaluate the efficacy of the pharmacological treatment and to detect early recurrences, to modify the therapeutic approach. Acute phase reactants are not reliable alone for the management and the follow-up as they are often not concordant with metabolic assessment of the disease. In patients with ureteral involvement, CRP together with BUN and serum creatinine has a significant correlation with PET/CT results, and can help physicians in therapeutic approach, better than a single parameter

Spontaneous Coronary Artery Dissection in Clinical Practice: Pathophysiology and Therapeutic Approaches

Spontaneous coronary artery dissection (SCAD) is a cause of myocardial infarction without obstructive coronary artery disease (MINOCA). It is determined by a coronary artery wall layers separation, which occurs regardless of traumatic or iatrogenic injuries. Even if it is often a missed diagnosis, its incidence is growing along with the improvement of intracoronary imaging techniques that allow for better detection. The main angiographical classification distinguishes three different forms, with slightly different prognoses at long-term follow up. SCAD is a recurrent condition, severely hampering the life quality of affected patients. The predominantly young age of patients with SCAD and the high prevalence of females among them have made the topic increasingly important, especially regarding therapeutic strategies. According to the data, the most recommended treatment is conservative, based on the use of antiplatelet agents and supportive anti-ischemic therapy. However, there are conflicting opinions concerning the need for dual antiplatelet therapy and its duration. In the case of invasive treatment, the choice between percutaneous coronary intervention and coronary artery bypass graft depends on the patient’s clinical stability and the interested vessel. The purpose of the current review is to revise the pathophysiological mechanisms underlying SCAD and the current knowledge of its treatment

Telemedicine: an effective and low-cost lesson from the covid-19 pandemic for the management of heart failure patients

: The purpose of this review is to explore the benefits and controversies that telemedicine (TM), applied to patients with heart failure (HF), can provide in terms of diagnosis, therapeutic management, and prognosis improvement. During the coronavirus disease 19 (COVID-19) outbreak, TM emerged as the most effective and feasible method available to ensure continuous care for chronic diseases. Among these, HF, characterized by high mortality, morbidity, and the need for frequent visits, may benefit of the TM role. HF patients are affected by frequent exacerbations undergoing a progressive prognosis impoverishment, strongly depending on the disease's management. A precise clinical handling is always required, with a constant optimization of the therapy, a continuous control of risk factors, and a sensitive attention to any change in symptoms, clinical signs, and laboratory tests. In this context, TM has shown to improve therapy adherence and HF: patients' self-care, impacting the prognosis even if specific results are controversial. Major evidence shows that TM may allow an adequate primary prevention, reducing the impact of the main cardiovascular risk factors. TM can also be useful for the secondary prevention, early detecting a likely HF exacerbation before it becomes clinically manifest, thereby lowering the need for hospitalization. Moreover, an optimal up-titration of the therapy and an increase in treatment adherence are feasible by using TM. However, some studies did not show unambiguous results, and uncertainties still remain

Potential role of eNOS genetic variants in ischemic heart disease susceptibility and clinical presentation

Background: IHD is determined by an inadequate coronary blood supply to the myocardium, and endothelial dysfunction may represent one of the main pathophysiological mechanisms involved. Genetic predisposition to endothelial dysfunction has been associated with IHD and its clinical manifestation. However, studies are often confounding and inconclusive for several reasons, such as interethnic differences. Validation of results in larger cohorts and new populations is needed. The aim of this study is to evaluate the associations between the allelic variants of the eNOS rs1799983 single-nucleotide polymorphism, IHD susceptibility and its clinical presentation. Methods: A total of 362 consecutive patients with suspected myocardial ischemia were enrolled. Patients were divided into three groups: G1, coronary artery disease (CAD); G2, coronary microvascular dysfunction (CMD); and G3, a control group with anatomically and functionally normal coronary arteries. Analysis of three allelic variants, GT, GG and TT, of rs1799983 for the NOS3 gene, encoding for eNOS, was performed. Results: rs1799983_GT was significantly more expressed by the ischemic groups (G1 and G2) compared to G3. The TT variant was significantly more expressed by the G1 group, compared to the G2 group. Among ischemic patients, GT was significantly more expressed in patients with acute coronary syndrome (ACS) presentation, compared to other clinical presentations. In the multivariate analysis, the allelic variant GT was found to potentially represent an independent predictor of IHD and ACS presentation. Conclusion: The presence of the SNP rs1799983_GT, encoding for eNOS, is an independent risk factor for IHD and, remarkably, for ACS presentation, independently of cardiovascular risk factors. These results may be useful for the prediction of IHD development, particularly with an acute clinical manifestation. They may allow the early identification of patients at high risk of developing IHD with an ACS, promoting a genetic-based prevention strategy against IHD

Protection against Ischemic Heart Disease: A Joint Role for eNOS and the K_ATP Channel

Genetic susceptibility may influence ischemic heart disease (IHD) predisposition and affect coronary blood flow (CBF) regulation mechanisms. The aim of this study was to investigate the association among single nucleotide polymorphisms (SNPs) of genes encoding for proteins involved in CBF regulation and IHD. A total of 468 consecutive patients were enrolled and divided into three groups according to coronary angiography and intracoronary functional tests results: G1, patients with coronary artery disease (CAD); G2, patients with coronary microvascular dysfunction (CMD); and G3, patients with angiographic and functionally normal coronary arteries. A genetic analysis of the SNPs rs5215 of the potassium inwardly rectifying channel subfamily J member 11 (KCNJ11) gene and rs1799983 of the nitric oxide synthase 3 (NOS3) gene, respectively encoding for the Kir6.2 subunit of ATP sensitive potassium (KATP) channels and nitric oxide synthase (eNOS), was performed on peripheral whole blood samples. A significant association of rs5215_G/G of KCNJ11 and rs1799983_T/T of NOS3 genes was detected in healthy controls compared with CAD and CMD patients. Based on univariable and multivariable analyses, the co-presence of rs5215_G/G of KCNJ11 and rs1799983_T/T of NOS3 may represent an independent protective factor against IHD, regardless of cardiovascular risk factors. This study supports the hypothesis that SNP association may influence the crosstalk between eNOS and the KATP channel that provides a potential protective effect against IHD

Heart failure ‘the cancer of the heart’: the prognostic role of the HLM score

AimsThe multi-systemic effects of heart failure (HF) resemble the spread observed during cancer. We propose a new score, named HLM, analogous to the TNM classification used in oncology, to assess the prognosis of HF. HLM refers to H: heart damage, L: lung involvement, and M: systemic multiorgan involvement. The aim was to compare the HLM score to the conventional New York Heart Association (NYHA) classification, American College of Cardiology/American Heart Association (ACC/AHA) stages, and left ventricular ejection fraction (LVEF), to assess the most accurate prognostic tool for HF patients.Methods and resultsWe performed a multicentre, observational, prospective study of consecutive patients admitted for HF. Heart, lung, and other organ function parameters were collected. Each patient was classified according to the HLM score, NYHA classification, ACC/AHA stages, and LVEF assessed by transthoracic echocardiography. The follow-up period was 12 months. The primary endpoint was a composite of all-cause death and rehospitalization due to HF. A total of 1720 patients who completed the 12 month follow-up period have been enrolled in the study. 520 (30.2%) patients experienced the composite endpoint of all-cause death and rehospitalization due to HF. 540 (31.4%) patients were female. The mean age of the study population was 70.5 +/- 12.9. The mean LVEF at admission was 42.5 +/- 13%. Regarding the population distribution across the spectrum of HLM score stages, 373 (21.7%) patients were included in the HLM-1, 507 (29.5%) in the HLM-2, 587 (34.1%) in the HLM-3, and 253 (14.7%) in the HLM-4. HLM was the most accurate score to predict the primary endpoint at 12 months. The area under the receiver operating characteristic curve (AUC) was greater for the HLM score compared with the NYHA classification, ACC/AHA stages, or LVEF, regarding the composite endpoint (HLM = 0.645; NYHA = 0.580; ACC/AHA = 0.589; LVEF = 0.572). The AUC of the HLM score was significantly better compared with the LVEF (P = 0.002), ACC/AHA (P = 0.029), and NYHA (P = 0.009) AUC.ConclusionsThe HLM score has a greater prognostic power compared with the NYHA classification, ACC/AHA stages, and LVEF assessed by transthoracic echocardiography in terms of the composite endpoint of all-cause death and rehospitalization due to HF at 12 months of follow-up