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Biomedical subjects

A Aessopos

Publications and source records attributed to A Aessopos.

At least 37 records · Page 2Linked to original sources
PubMed2001

Erythropoietic response to hypoxaemia in diffuse idiopathic pulmonary fibrosis, as opposed to chronic obstructive pulmonary disease.

The aim of this study was to assess the erythropoietic response to hypoxaemia in patients with diffuse idiopathic pulmonary fibrosis (DIPF), and to speculate on the underlying mechanisms. Patients on an established chronic respiratory failure due to DIPF or chronic obstructive pulmonary disease (COPD) were studied. The erythropoietic response to hypoxaemia in both conditions was assessed. We studied 18 patients with DIPF and 29 patients with COPD in respiratory failure in a stable stage, free from acute infection and congestive heart failure. Blood gases, erythrocytic parameters, as well the serum levels of iron, ferritin and erythropoietin were determined. All the DIPF patients studied, apart from two, had normal or subnormal haematocrit values. The patients with COPD had an inconsistant response to hypoxaemia; 12 had normal or subnormal haematocrit values and the remaining 17 were erythraemic. The mean value of erythropoietin (EPO) in both DIPF and COPD patients was significantly higher than normal. In conclusion, patients with DIPF exhibit a lack of erythropoietic response to hypoxaemia, despite the augmented erythropoietin levels. This may reflect a defective bone marrow erythropoietic response in DIPF patients. It is suggested that the pathophysiology of DIPF underlies this mechanism.

Adult↗
PubMed2001

Multilocalized pyomyositis in a previously healthy subject.

A case of pyomyositis is presented. This case is unique in the literature as at least 29 abscesses were detected, affecting the vast majority of big muscle groups. We outline the origin of this disease entity which selectively affects striated muscles. We also discuss its natural history and management strategy.

Aged↗
PubMed2000

Neutrophil elastase in patients with homozygous beta-thalassemia and pseudoxanthoma elasticum-like syndrome.

In this study we investigated the possible role of neutrophil (PMN) elastase and its natural inhibitor, alpha1-proteinase inhibitor (alpha1-PI) in the pathogenesis of the pseudoxanthoma elasticum (PXE)-like syndrome which is found in patients with homozygous beta-thalassemia. We studied 30 beta-thalassemia homozygotes with the PXE-like syndrome [PXE(+) group], 20 beta-thalassemia homozygotes without this syndrome [PXE(-) group] and 15 healthy controls. Plasma PMN elastase concentration in the PXE(+) and in the PXE(-) group was 136.4 +/- 89 and 163.8 +/- 126 microg/L, respectively (P > 0.05). In the control group, the concentration was 42.9 +/- 16.8 microg/L (P < 0.01 for the comparison with both patients' groups). The plasma alpha1-PI concentration in the PXE(+) and in the PXE(-) group was 2.28 +/- 0.75 and 2.6 +/- 0.96 g/L, respectively (P > 0.05). Using logistic regression, we studied the prognostic value for PXE of the following independent variables: number of transfusions, chelation therapy, mean hemoglobin concentration, PMN elastase concentration, alpha1-PI concentration, chronic transaminase elevation, and positivity for anti-HCV. None of the above variables was found to have significant prognostic value for the PXE. Plasma PMN elastase concentration is elevated in all beta-thalassemia homozygotes; its role in the pathogenesis of the PXE-like syndrome in beta-thalassemia can not be established, but our findings suggest that neutrophils of beta-thalassemia patients are activated, since PMN elastase is a marker of neutrophil activation.

Adolescent↗
PubMed2000

Doppler-determined peak systolic tricuspid pressure gradient in persons with normal pulmonary function and tricuspid regurgitation.

The Doppler-estimated peak systolic tricuspid pressure gradient is the most reliable noninvasive method for the evaluation of pulmonary artery systolic pressure in patients with tricuspid regurgitation. Our goal was to evaluate the range of this gradient in healthy persons and determine a normal upper limit. We studied 53 healthy persons (34 women, 19 men; aged 14 to 55 years, mean 38.9 +/- 12.7 years) who did not smoke and who had an adequate Doppler signal of tricuspid regurgitation. The presence of pulmonary or cardiac disorders was excluded by a review of the subject's medical history in addition to physical examination, spirometry, arterial blood gasses determination, electrocardiography, chest x-ray examination, and rest echocardiography. Tricuspid gradient ranged from 12.6 to 29. 3 mm Hg (mean 19.3 +/- 4.0); 35.8% of patients had values higher than 20 mm Hg. In conclusion, a tricuspid gradient of 30 mm Hg may be considered as the upper normal limit. The different approaches for estimating mean right atrial pressure are also discussed.

Adolescent↗
PubMed2000

Chelation therapy in patients with thalassemia using the orally active iron chelator deferiprone (L1).

BACKGROUND AND OBJECTIVE: Excessive hemosiderosis is the main reason for the multi-organ failure observed in multitransfused patients. Deferiprone (1,2-dimethyl-3-hydroxy-pyridine-4-one, L1) is an orally active iron chelator mainly excreted via urine. We conducted a study in order to determine the efficacy and safety of L1 in Greek thalassemic patients. DESIGN AND METHODS: A group of 11 thalassaemic patients entered the study; L1, the Cipla formulation for deferiprone, at a daily dose of 75-100 mg/kg bw t.i.d. was used. After giving informed consent all patients were subjected to clinical examination and biological tests. RESULTS: All patients tolerated the L1 well; there were no significant side effects (except for slight gastrointestinal disturbances for the first days). The net urinary iron excretion ranged from 6.96 to 26.1 mg/24h. Serum ferritin declined within 4-6 months in most of the patients. INTERPRETATION AND CONCLUSIONS: The results suggest that L1 is a rather safe drug which decreases iron overload without causing any considerable side-effects in Greek thalassemics.

Administration, Oral↗
PubMed1999

Pregnancy in patients with well-treated beta-thalassemia: outcome for mothers and newborn infants.

OBJECTIVE: Our purpose was to investigate the course and outcome of pregnancy in women with well-treated beta-thalassemia. STUDY DESIGN: Twenty-two pregnancies, including one twin pregnancy, in 19 women were studied. Pregnancy was advised when patients had received a prolonged intensive treatment with hypertransfusions and iron chelation and had echocardiographically normal resting left ventricular performance. All conceptions were spontaneous. Cardiac function, along with hematologic, endocrinologic, and hepatic parameters were initially assessed and monitored throughout pregnancy and for 2 to 9 years post partum. Babies were delivered by elective cesarean section. RESULTS: Twenty-one healthy newborn infants were delivered. A spontaneous abortion and a case of exomphalos also occurred. Gestation, delivery, and recovery were surprisingly uneventful, and no significant cardiac complications were encountered. CONCLUSION: Pregnancy can be safe for mothers and babies, provided that women with thalassemia have been started early on intensive treatment and have a normal resting cardiac performance.

Adult↗
PubMed1999

Tuberculoma of the spleen: a rare but important clinical entity.

Tuberculoma of the spleen has been an extremely rare entity during the last decades in the Western world. We describe a case in a young, HIV-negative woman who was evaluated for fever of unknown origin. The workup was initially negative, and she was treated successfully with steroids and nonsteroidal anti-inflammatory analgesics. Recurrence of the fever and an abnormal CT of the abdomen lead to an exploratory laparotomy, which revealed a subcapsular caseous material containing splenic abscess. The very few cases reported in the literature, and the increasing incidence of extrapulmonary tuberculosis due to the alarming numbers of immunodeficient patients that a surgeon may encounter are also discussed.

Adult↗
PubMed1998

Apolipoprotein E epsilon4 allele as a genetic risk factor for left ventricular failure in homozygous beta-thalassemia.

In homozygous beta-thalassemia, the organ damage is mainly attributed to excessive iron deposition through the formation of oxygen free radicals. Despite appropriate transfusion and chelation therapy and low ferritin levels, patients still develop organ failure, heart failure being the main cause of death. This study was designed to determine whether the decreased antioxidant activity of the apolipoprotein E (APOE) 4 allele could represent a genetic risk factor for the development of left ventricular failure (LVF) in beta-thalassemia homozygotes. A total of 251 Greek beta-thalassemia homozygotes were studied. Patients were divided in three groups: group A (n = 151) with no cardiac impairment, group C (n = 47) with LVF, and 53 patients with LV dilatation and normal LV systolic function constituted the group B. DNA was obtained from all patients, and the polymerase chain reaction was used to analyze the polymorphism at the APOE locus. The APOE allele frequencies were compared with those of a Greek control sample of 216 healthy blood donors. Patients with no cardiac impairment had an APOE 4 allele frequency (7.9%) not different from population controls (6.5%, P > .05), while patients with LVF had a significantly higher frequency of APOE 4 (12.8%) than the controls (P < .05, odds ratio = 2.11, 95% confidence interval 1.03 to 4.32). The APOE 4 allele may represent an important genetic risk factor for the development of organ damage in homozygous beta-thalassemia.

Adolescent↗
PubMed1998

Arterial calcifications in beta-thalassemia.

The purpose of this study was to define the incidence of arterial calcifications in patients with beta-thalassemia. Beta-thalassemia patients have been shown to present a high prevalence of angioid streaks and skin lesions characteristic of pseudoxanthoma elasticum (PXE). Given the fact that vascular involvement in the form of arterial calcifications is also a common manifestation of PXE, the authors investigated radiographically the presence of arterial calcifications in beta-thalassemia patients. They studied 40 patients with beta-thalassemia over 30 years of age. Forty healthy, age- and sex-matched subjects were chosen as a control group. Radiographs of the tibias were performed in order to disclose arterial calcifications. The occurrence of PXE skin lesions and of angioid streaks (AS) was also investigated. Arterial calcifications were detected in the posterior tibial artery in 22 (55%) beta-thalassemia patients and in six (15%) controls (P < 0.01 for the comparison). PXE skin lesions and AS were found in eight (20%) and 21 (52%) patients respectively. A total of 34 patients (85%) had at least one of the three lesions, namely, arterial calcifications, angioid streaks, and/or PXE-like skin lesions. Stepwise logistic regression analysis did not reveal prognostic value in independent variables such as transfusions, chelation therapy, pseudoxanthoma elasticum skin lesions and/or angioid streaks, diabetes, hemoglobin, serum ferritin, and uric acid. It was concluded that arterial calcifications are common in older beta-thalassemia patients. This finding could be a manifestation of an acquired PXE syndrome associated with beta-thalassemia, and consequently, vascular events complicating PXE should be expected in these patients.

Adult↗
PubMed1997

Episodic pain associated with a tumor in the parietal operculum: a case report and literature review.

We report a young woman suffering brief painful episodes in her right arm which sometimes spread to the whole right side of her body. The episodes were initially rare, but over 13 months they became increasingly frequent until occurring every 20 min. Neurological examination was normal. Magnetic resonance imaging revealed a lesion in the white matter of the parietal operculum. Lesions in the parietal operculum associated with persisting thalamic pain or loss of pain sensation have been reported, but rarely with episodic pain. Since episodic painful attacks have been described in association with various suprathalamic lesions, we conclude that paroxysmal pain attacks may be another consequence of the disturbance of the normal pattern of thalamocortical connections to the second sensory cortical area by a lesion, in our case, of the subcortical area of the parietal operculum.

Adult↗
PubMed1997

Pseudoxanthoma elasticum lesions and cardiac complications as contributing factors for strokes in beta-thalassemia patients.

BACKGROUND AND PURPOSE: Pseudoxanthoma elasticum (PXE) lesions, which lead to intracranial hemorrhages and cardiac complications, predisposing to thrombotic strokes, are frequent findings in beta-thalassemia. Nevertheless, the association of these lesions with strokes in thalassemic patients has not been previously discussed. METHODS: Ten beta-thalassemic patients who developed an intracranial hemorrhage or a thrombotic stroke were reviewed. RESULTS: In the group of the four patients presenting with hemorrhage, one had PXE lesions, one had cardiac abnormalities, and one both PXE and cardiac disorders. In the group presenting with thrombotic stroke, all six patients had cardiac abnormalities and platelet count elevation due to splenectomy. Three also had PXE findings. No other predisposing factor for stroke was present. CONCLUSIONS: Cardiac complications and PXE may be risk factors for strokes in beta-thalassemia. Their frequent coexistence leads to a therapeutic dilemma in patients requiring antithrombotic therapy.

Adolescent↗
PubMed1995

Pure red cell aplasia associated with true thymic hyperplasia.

Pure red cell aplasia was diagnosed in a 35-year-old otherwise healthy woman. Chest computed tomography (CT)-imaging investigation, detected an upper mediastinal mass corresponding to the thymus gland. A thoracotomy was performed and an enlarged thymus mass was removed, rapidly followed by a full hematologic recovery. Thymic histology confirmed a significant degree of hyperplasia. We conclude that not only thymomas but also other types of thymic pathology may be associated with this type of hematologic dyscrasia.

Adult↗
PubMed1995

Pulmonary hypertension and right heart failure in patients with beta-thalassemia intermedia.

We analyzed seven patients with beta-thalassemia intermedia presenting with congestive heart failure secondary to pulmonary hypertension. This condition has been recognized only recently as part of the clinical spectrum of beta-thalassemia. Our group of patients included two men and five women with the clinical picture and laboratory data typical of beta-thalassemia intermedia. The mean age was 37.7 +/- 11.4 years, mean hematocrit value was 28.5 +/- 1.8%, mean number of transfused blood units was 171 +/- 153, and mean serum ferritin levels were 4,428 +/- 2,006 ng/mL. All but one of these patients had undergone splenectomy. Common findings of the investigative procedures include the following: dilation of the main pulmonary artery and cardiac enlargement in the chest radiograph; signs of right ventricular hypertrophy in the ECG; and dilated right ventricle with good left ventricular function in the echo study. Right heart catheterization showed the pulmonary systolic pressure to range from 55 to 90 mm Hg (74.1 +/- 10.3), pulmonary diastolic pressure from 25 to 50 mm Hg (37.7 +/- 8.7), mean pressure from 35 to 60 mm Hg (49.7 +/- 7.9), and pulmonary vascular resistance from 267 to 667 dynes.s.cm-5. Pulmonary capillary wedge pressure was within the normal range of values. The pathophysiologic condition of pulmonary hypertension in these patients is most probably associated with beta-thalassemia. There are mechanisms that increase cardiac output and at the same time restrict the pulmonary vascular bed. The results of this study imply that treatment decisions should be reconsidered for such patients.

Adult↗
PubMed1995

Spleen size in beta-thalassaemia heterozygotes.

In 45 beta-thalassaemia heterozygotes and in 38 normal controls we determined by ultrasound tomography: (a) the surface of the projection of the spleen to thoracic and abdominal wall, (b) the maximum diameters of the organ, and (c) its volume. The volume of the spleen is significantly bigger in heterozygotes as opposed to normals (132.94 +/- 41.76 and 80.29 +/- 25.88, respectively). In 17.8% of heterozygotes a palpable spleen was found. The findings of this study lead to the hypothesis that in all heterozygotes the final volume of the organ is increased; however, in only 17.8% of them a palpable spleen is found.

Heterozygote↗
PubMed1994

Angioid streaks in sickle-thalassemia.

Angioid streaks have been described in a diverse group of diseases including hemoglobinopathies such as sickle cell anemia and beta-thalassemia. We investigated the prevalence of angioid streaks and pseudoxanthoma elasticum in the rare situation of patients who had compound heterozygous traits for hemoglobin S and beta-thalassemia. We examined 58 consecutive patients with sickle-thalassemia. Of these, 25 were men and 33 were women, and they ranged in age from 19 to 58 years (mean, 32.6 years). Angioid streaks were identified in six of 58 patients (10%), and of these three also displayed the cutaneous lesions of pseudoxanthoma elasticum, which were confirmed by skin biopsy. An expanded study on several relatives of the patients with angioid streaks failed to identify any similar cases. Statistical evaluation of the main hematologic and biochemical parameters in the patients with and without angioid streaks did not demonstrate any significant differences, except that the thalassemic component in all six patients with angioid streaks was beta(0) (that is, did not allow the synthesis of hemoglobin A). We conclude that angioid streaks and pseudoxanthoma elasticum skin lesions occur with an increased frequency in patients with sickle-thalassemia.

Adult↗
PubMed1994

The association of systemic lupus erythematosus and myasthenia gravis.

Two women with the rare association of systemic lupus erythematosus (SLE) and myasthenia gravis (MG) are reported. The first patient developed SLE (arthritis, severe thymectomy for MG. The second patient developed SLE (oral ulcers, arthritis, serositis, leukopenia, high titres of anti-DNA and anti-nuclear antibodies) 4 years prior to the clinical and serological onset of MG. Lymphocyte subsets and in vitro proliferative responses of peripheral blood mononuclear cells to mitogens were normal in both patients. A review of the literature revealed 26 additional patients with definite SLE coexisting with MG. Besides the theoretical interest of this association, the differential diagnosis of fatigue in patients with SLE should always include the possibility of MG.

Adult↗
PubMed1992

Pseudoxanthoma elasticum-like skin lesions and angioid streaks in beta-thalassemia.

One hundred patients with homozygous or doubly heterozygous beta-thalassemia (62 with the major form and 38 with beta-thalassemia intermedia) were examined for signs of Pseudoxanthoma elasticum (PXE). Diagnostic skin lesions were found in 16 patients with either form of the basic disease. Twenty percent of all patients had angioid streaks (AS); both PXE skin lesions and AS were found in 10% of the patients; in all, 26% had either one or both of these manifestations. A positive correlation was found between the presence of one or both types of lesion and age of the patients (P = 0.032); there were no differences as regards ferritin and hematocrit levels, number of transfused units, chelation therapy, and splenic status between patients with PXE/AS findings and those without. The pathogenesis of these connective tissue manifestations at such a high frequency in beta-thalassemia is not clear; the possibilities of it's being acquired or inherited are discussed, the former being considered to be the more economical interpretation.

Adolescent↗