Localized Castleman's disease associated with systemic AA amyloidosis. Regression of amyloid deposits after tumor removal.
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Biomedical subjects
Publications and source records attributed to A Aessopos.
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Hypocalcemic cardiomyopathy in primary or secondary hypoparathyroidism is usually refractory to conventional treatment of cardiac failure. We report the case of a thalassemic patient with severe cardiac failure that might have been attributed to several factors, such as hemosiderosis, hypomagnesemia, and hypocalcemia, refractory to conventional cardiac therapy. Cardiac echocardiography showed impaired biventricular performance, and laboratory analyses revealed hypoparathyroidism due to hemosiderosis. When concomitant treatment of heart failure and calcium supplementation was initiated, correction of hypocalcemia resulted in clinical and laboratory improvement, providing strong evidence in support of our hypothesis about hypocalcemic myocardiopathy.
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Although massive presacral bleeding during rectal mobilization is uncommon, it can rapidly destabilize a patient. So, effective hemostasis is critical in severe presacral hemorrhage due to the fatal course of this complication. Among the reported methods are packing, thumbtacks, inflatable devices, muscle tamponade, muscle fragment welding and application of endoscopic staplers. Local hemostatic agents in conjunction with other methods such as diathermy, cyanoacrylate tissue adhesives and application of bone wax are among other alternatives which may help to treat this serious complication. The aim of this study is to describe the anatomic and physiologic basis of our mode of treatment, which is new in the literature approach, treating two patients with presacral bleeding during low anterior resection for rectal cancer. The technique is the early clamping of the infrarenal aorta and suture ligation of the bleeding points from the presacral plexus.
Primary duodenal diverticula are usually asymptomatic. About 115 perforations have been reported, but none with right colon necrosis. We report a 45-year-old woman, with a five days history of high fever along with epigastric and periumbilical pain. Physical examination revealed right upper and lower quadrant tenderness with peritoneal signs. White blood cell count was 11 500/mm (3) while biochemical and hepatic biology tests were normal. Abdominal radiographs showed no pathologic findings. Ultrasound disclosed fluid in the lower pelvis. Computerized tomography revealed fluid collection in the right hepatorenal space. Intraoperative findings included purulent fluid in the lower pelvis, segmental necrotic changes of the right colon, and a perforated diverticulum on the antimesenteric border of the third part of the duodenum. Surgery consisted of right hemicolectomy and ileo-transverse anastomosis, diverticulectomy, and decompressive lateral duodenostomy at the second duodenal portion. The patient had an uneventful postoperative course. A contrast study from the duodenostomy tube on the 6 (th) postoperative day showed no leakage or obstruction. Duodenostomy tube was removed on the 14 (th) postoperative day. Histology confirmed the diagnosis of a primary duodenal diverticulum.
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In patients with thalassemia major (TM) who are non-compliant with long-term desferrioxamine (DFO) chelation, survival is limited mainly because of cardiac complications of transfusional hemosiderosis. Combined chelation therapy with DFO and deferiprone has maximized the efficacy of the therapy and reduced cardiological complications. The aim of this report is to present the results of this combination in a desperate case of heart failure.
Although the indications for transfusions in sickle cell syndromes are well listed, and chronic transfusion has become practicable since the recent advances in chelation therapy have essentially eliminated the risk of secondary iron overload, multi-transfused, non-compliant to long-term chelation therapy patients confront the complication of iron overload and secondary hemosiderosis. In thalassemia major patients, combined therapy with desferrioxamine and deferiprone has maximized tissue iron removal and may reduce the overall occurrence of hemosiderotic heart failure. Despite this, safety and contradictions of chelating agents are still controversial. The aim of this report is to present the results of this combination in a long-term transfused sickle beta-thalassemic patient suffering from severe heart failure and liver dysfunction.
OBJECTIVE: To evaluate the right and left ventricular systolic and diastolic function in middle aged patients with sickle beta thalassaemia. METHODS: Forty three patients with sickle beta thalassaemia were recruited for echocardiographic study while 55 controls, matched for age and sex, served as the control group. Parameters measured included: dimensions and wall thickness of left (LV) and right (RV) ventricle and left atrium, LV mass, and cardiac index. LV and RV contractility variables--ejection fraction, circumferential fibre shortening velocity, end systolic stress, end systolic stress/volume index ratio, mitral and tricuspid annulus systolic excursion, and Tei index--were also calculated. The study also evaluated parameters of RV and LV diastolic function including early and late atrioventricular flow velocities (E and A wave respectively), E/A ratio, deceleration time (DT), isovolumic relaxation time (IVRT) as well as pulmonary and hepatic veins systolic to diastolic (S/D) ratio. RESULTS: Chamber enlargement, greater LV mass index, cardiac index, and RV wall thickness were found in the anaemic group compared with controls. The LV and RV contractility variables of the patients were similar to controls. Conversely the LV and RV Tei index was significantly greater in the patient group. Diastolic dysfunction was present in the anaemic patients resulting from the increased LV and RV A-wave, the longer LVIVRT, RVIVRT, and RVDT, as well as the higher hepatic and pulmonary veins S/D ratio. CONCLUSIONS: The results show that in middle aged patients with sickle beta thalassaemia the diastolic function is abnormal in both ventricles but still more in RV, whereas the systolic function remains unchanged.
OBJECTIVE: The aim of this study was to investigate the endothelial status in young women with polycystic ovary syndrome (PCOS), using a simple and easily reproducible hemodynamic method combined with a biological marker and to evaluate the effect of metformin treatment on these parameters. DESIGN: Descriptive clinical trial. METHODS: Forty young women, 20 with PCOS and 20 normal women of similar age and body mass index were studied. Metformin (1700 mg daily) was administered for 6 months to the PCOS group. The endothelium status and the metabolic and hormonal profile were studied in both groups, as well as after metformin, by flow-mediated dilatation (FMD) on the brachial artery and by measurements of plasma endothelin-1 (ET-1) levels. RESULTS: FMD was impaired in the PCOS group when compared with controls (3.24+/-0.71% vs 8.81+/-1.07% respectively, P<0.0001), but this difference normalized after metformin treatment (PCOS(post-metformin) vs controls: 8.17+/-1.26 vs 8.81+/-1.07%, P = 0.70) since the values significantly improved after metformin treatment (PCOS(pre-metformin) vs PCOS(post-metformin): 3.24+/-0.71 vs 8.17+/-1.26%, P=0.003). ET-1 levels were significantly higher in the PCOS women compared with the control group (7.23+/-0.50 vs 4.99+/-0.69 fmol/l, P=0.01), they improved significantly after metformin treatment (PCOS(pre-metformin) vs PCOS(post-metformin): 7.23+/-0.50 vs 3.57+/-0.60 fmol/l, P<0.0001) and their difference compared with the control group was reversed (PCOS(post-metformin) vs controls: 3.57+/-0.60 vs 4.99+/-0.69 fmol/l, P=0.13). Metformin administration improved hyperandrogenemia. However, in this study, mathematical methods used to assess insulin resistance failed to show any detected alteration after treatment with metformin. CONCLUSIONS: PCOS women were found to exhibit endothelial dysfunction compared with controls, which was reversed 6 months after metformin administration.
This study was performed to determine whether phenotypically healthy sisters of women with polycystic ovary syndrome (PCOS) have evidence of insulin resistance. We studied 54 women: 17 with PCOS, 17 sisters of these probands and 20 control women with similar age, body mass index (BMI) and waist-to-hip ratio (WHR). The PCOS sisters had neither clinical nor laboratory evidence of hyperandrogenism. However, estimated insulin resistance indices indicated decreased insulin sensitivity in PCOS sisters compared with the controls. No difference of insulin resistance indices was detected between the PCOS and their sisters. This finding provides additional evidence that there is a hereditary trait regarding insulin resistance in the PCOS families.
Multiple sclerosis (MS) has been reported in association with haematological abnormalities, including monoclonal gammopathies. We present a 54-year-old male patient with a 30-year history of MS who was admitted to our hospital for investigation of anaemia and increased erythrocyte sedimentation rate. A monoclonal IgM protein was detected by serum protein immunofixation, while bone marrow biopsy indicated a 70% infiltration by small lymphoplasmacytoid cells, in the context of a lymphoplasmacytoid immunocytoma, findings compatible to the diagnosis of Waldenström's macroglobulinemia (WM). To our knowledge, this is the first report of WM in a patient with MS. Further to the coexistence of the two diseases observed in the case presented here, there is additional evidence suggesting that the association of MS with plasma cell dyscrasias may not be coincidental.
We describe a case of protein-losing enteropathy in association with cryoglobulinaemia. No underlying disorder could be definitively diagnosed, but several clinical and laboratory findings suggested an immune mediated or autoimmune disorder. We propose that the mechanism of the protein-losing enteropathy in our case was immune complex formation, complement activation and endothelial damage.
Facilitating data entry, eliminating redundant effort and providing decision support are some of the factors upon which the successful uptake of Electronic Healthcare Record (EHCR) technology is dependent. The European Standardization Committee (CEN), on the other hand, has proposed a standard EHCR architecture, which allows patient record contents to be highly diverse, customized to individual user needs; this makes their processing a challenging task and poses a demand for specially designed mechanisms. We describe the requirements for a macro-directive mechanism, pertaining to CEN-compatible EHCR software that can automate updating and processing of patient records, thus enhancing the functionality of the software. We have implemented the above-mentioned mechanism in an EHCR application that has been customized for use in the care process of patients suffering from beta-Thalassemia. The application is being used during the last two years in the Thalassemia units of four Greek hospitals, as part of their every day practice. We report on the experience we have acquired so far.
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PURPOSE: To determine the mechanism of myocardial ischemia in patients with sickle beta-thalassemia, we performed a scintigraphic evaluation of myocardial perfusion during exercise. SUBJECTS AND METHODS: We studied 30 patients with sickle beta-thalassemia, (mean [+/-SD] age, 37 +/- 10 years) who had no electrocardiographic (ECG), radiographic, or echo-Doppler signs of pulmonary hypertension, left ventricular hypertrophy, or impaired contractility. All patients had a hemoglobin level greater than 7 g/dL. Treadmill exercise test was performed according to the Bruce protocol. Myocardial perfusion was assessed by single-photon emission computed tomography, using Tetrofosmin Tc-99 m Myoview as radiotracer, at peak exercise and again 4 hours later. RESULTS: Eight patients (27%) developed stress-induced scintigraphic perfusion abnormalities that were reversible in all but 1 patient. Subsequent coronary angiograms were normal in all 8 patients. ST segment depression was seen during exercise in 5 of the 7 patients who had reversible perfusion defects. Except for a significantly greater white blood cell count, these 5 patients did not differ from the rest of patients by sex, age, hemoglobin level, percentage hemoglobin F, beta-thalassemia genotype, or risk factors for coronary artery disease. Three of the 5 patients with perfusion and ECG abnormalities (and another with only perfusion defects) developed a stress-induced sickling crisis. CONCLUSION: Physical stress may induce myocardial ischemia in sickle beta-thalassemia patients with normal coronary arteries and elicit painful crises. The sickling process, activated by exercise, could be the common underlying mechanism.
Cardiac complications in 110 patients (mean age, 32.5 +/- 11.4 years) with thalassemia intermedia (TI) were studied. Sixty-seven (60.9%) of them had not been transfused or were minimally transfused (group A). The rest had started transfusions after the age of 5 years (mean, 15.1 +/- 10.1 years), initially on demand and later more frequently (group B). Overall mean hemoglobin and ferritin levels were 9.1 +/- 1.1 g/dL and 1657 +/- 1477 ng/mL, respectively. Seventy-six healthy controls were also studied. The investigation included thorough history taking, clinical examination, electrocardiography, chest radiograph, and full resting echocardiography. Of 110 patients, 6 (5.4%) had congestive heart failure (CHF), and 9 (8.1%) had a history of acute pericarditis. Echocardiography showed pericardial thickening, with or without effusion, in 34.5% of the patients. Valvular involvement included leaflet thickening (48.1%), endocardial calcification (20.9%), and left-sided valve regurgitation (aortic, 15.4%; mitral, 47.2%). All patients had normal left ventricular contractility (fractional shortening, 0.43 +/- 0.05), and high cardiac output (CO; 9.34 +/- 2.28 L/min). Pulmonary hypertension (PHT), defined as Doppler peak systolic tricuspid gradient greater than 30 mm Hg, developed in 65 patients (59.1%). PHT correlated positively with age and CO and did not differ significantly between groups. Cardiac catheterization in the 6 patients with CHF revealed severe PHT, increased pulmonary resistance (PVR), and normal capillary wedge pressure. It was concluded that in patients with TI, the heart is primarily affected by PHT, which is the leading cause of CHF. High CO resulting from chronic tissue hypoxia and increased PVR are the main contributing factors. Doppler tricuspid gradient measurement should be considered, in addition to other factors, when determining the value of transfusion therapy for patients with TI. (Blood. 2001;97:3411-3416)