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Biomedical subjects

L Barile

Publications and source records attributed to L Barile.

At least 19 recordsLinked to original sources

The impact of gender on clinical manifestations of primary antiphospholipid syndrome.

The objective of the study was to determine the clinical differences at diagnosis and during follow-up between male and female patients with primary antiphospholipid syndrome (PAPS). We analysed 68 patients, 30 males and 38 females diagnosed and followed between 1990 and 2003. Patients with antiphospholipid syndrome associated with systemic lupus erythematosus at onset and during follow-up were excluded. The mean age at diagnosis was 31.4 +/- 11 years in males and 35.7 +/- 11 years in females (NS). The follow-up after diagnosis was 8.7 +/- 3.1 years in males and 9.2 +/- 2.9 years in females (NS). We did not find significant differences between the two groups with respect to venous and arterial thrombosis. However, in female patients, stroke was more prevalent than in male patients (12/38 versus 3/30, P = 0.03). In contrast, we found a significant prevalence of severe gastrointestinal complications in male compared to female patients (7/30 versus 1/38, P = 0.009). One male patient died due to catastrophic antiphospholipid syndrome. This study suggests that clinical course in patients with PAPS may be different with significant prevalence of central nervous system involvement in females and gastrointestinal involvement in males. Factors such as accelerated atherosclerosis, hormones, related to gender could be the explanation of these findings.

Adult↗

Potential role of mycophenolate mofetil in the management of neuroblastoma patients.

In human neuroblastoma cell lines (LAN5, SHEP and IMR32), mycophenolic acid (MPA) at concentrations (10(-7)-10(-6) M) readily attainable during immunosuppressive therapy with mycophenolate mofetil (Cellcept), induces guanine nucleotide depletion leading to cell cycle arrest and apoptosis through a p53 mediated pathway (up-regulation of p53, p21 and bax and down-regulation of bcl-2 and survivin). MPA-induced apoptosis is also associated to a marked decrease of p27 protein. In the same cell lines MPA, at lower concentrations (50 nM), corresponding to the plasma levels of the active free drug during Cellcept therapy, induces differentiation toward the neuronal phenotype by causing a partial chronic guanine nucleotide depletion. MPA-induced differentiation is not associated to p27 accumulation as occurs using retinoic acid. At a fixed concentration of MPA a higher percentage of apoptotic or differentiated cells is obtained when non dialysed serum substitutes for the dialysed one, due to the higher hypoxanthine concentration in the former (about 10 microM) leading to competition on HPRT-mediated salvage of guanine. At hypoxanthine or oxypurinol concentrations higher than 1 microM (up to 100 microM) no further enhancement of MPA effects was obtained, in agreement with the recently described safety of the allopurinol-mycophenolate mofetil combination in the treatment of hyperuricemia of kidney transplant recipients. The apoptotic effects of MPA do not appear to be significantly increased by the UDP-glucuronosyltransferase inhibitor niflumic acid.

Allopurinol↗

Cyclic nucleotides and neuroblastoma differentiation.

We have shown that intracellular cGMP levels increase during retinoic acid- and mycophenolic acid-induced neuroblastoma differentiation and that a 6 days treatment with 1 mM dbcGMP lead LAN5 cell to elaborate a network of neuritic processes suggesting an involvement of cGMP in neuroblastoma differentiation. We have also investigated the effects of some specific inhibitors of phosphodiesterases (PDE1, PDE3, PDE4 and PDE5) on human neuroblastoma (LAN5 and SHEP) growth and differentiation. After six days of incubation in the presence of each specific inhibitor at 10 x IC50 levels a cytostatic and differentiating effect was only observed with the PDE5 inhibitors Zaprinast and MY-5445. The cytostatic effect of these compounds increased increasing their concentrations far above their IC50 levels for PDE5, suggesting that these compounds could act by interfering with other molecular events than direct cGMP-PDE inhibition. No appreciable effect was observed using Dipyridamole, another specific PDE5 inhibitor.

Cell Differentiation↗

Clinical spectrum of males with primary antiphospholipid syndrome and systemic lupus erythematosus: a comparative study of 73 patients.

The objective of this study was to compare the clinical findings, laboratory data, functional outcome and chronic damage in male patients with primary antiphospholipid syndrome (PAPS) and systemic lupus erythematosus (SLE). We studied 29 male patients with PAPS and 44 with SLE. Clinical findings, laboratory data, lupus damage index (SLICC/ACR DI), and functional outcome in PAPS, were analysed in each group. The mean age at diagnosis was 29.8 +/- 10.4 years in patients with PAPS and 26 +/- 10.1 years in SLE patients. The duration of disease was 4.5 +/- 2.6 versus 5.2 +/- 3.8 years in patients with PAPS and SLE, respectively (P = NS). In patients with PAPS the most frequent clinical manifestations were venous thrombosis, thrombocytopenia, and pulmonary thromboembolism. Patients with SLE had joint, skin and renal involvement more frequently than those with PAPS (P = 0.0001). All PAPS patients had anticardiolipin antibodies (aCL), and 14 patients (48%) had lupus anticoagulant (LA). All SLE patients had antinuclear antibodies (ANAs). Anti-dsDNA antibodies were positive in 39% of SLE patients. Five patients died: one with 'catastrophic' APS and four with SLE. SLICC/ACR-DI score in SLE patients was 1.9 (SD = 1). In PAPS patients poor functional outcome was due to myocardial infarction, pulmonary thromboembolism, stroke and mesenteric thrombosis. Lupus nephritis was the principal organ damage in SLE. In conclusion, in male patients with PAPS and SLE, the clinical manifestations were significantly different. Arterial thrombosis was the major cause of functional impairment and permanent organ damage in PAPS. Renal involvement was the major cause of chronic damage in SLE.

Adolescent↗

Increased carotid artery intima-media thickness may be associated with stroke in primary antiphospholipid syndrome.

OBJECTIVE: To investigate the prevalence and clinical significance of carotid artery intima-media thickness (IMT) in patients with primary antiphospholipid syndrome (APS). METHODS: 28 patients with primary APS with at least a five year follow up, and 28 healthy subjects, matched by age and sex, were included in the study. Colour Doppler with high resolution B mode carotid ultrasonography and spectral analysis were performed in patients and controls. Information on cardiovascular risk factors and the clinical course were collected. RESULTS: The mean (SD) age of patients and controls (12 male, 16 female in each group) was 40 (8.5) years; the mean (SD) disease duration 7.7 (3) years. Carotid artery IMT was found in 23/28 patients (2.6 (1.14) mm) and 7/28 controls (1.2 (0.44)) (p=0.0001). A decrease in the lumen diameter was also found in 11/28 patients with primary APS without carotid atherosclerotic plaque, and 2/28 controls (p=0.004). Hyperlipidaemia, diabetes, smoking, obesity, and hypertension were not associated with carotid artery IMT. Patients with carotid artery IMT had arterial vascular disease more often than patients without: 9/23 v 0/5 (p<0.009). These patients had stroke (seven patients), myocardial infarction (one), and mesenteric thrombosis (one). Subjects with IMT had a threefold higher risk for stroke than those without IMT (95% CI 0.78 to 14.3). CONCLUSIONS: Patients with primary APS have a high prevalence of carotid artery IMT and a decreased lumen diameter. IMT in primary APS may be associated with stroke. Patients with primary APS with IMT must be considered as carriers of atherosclerosis.

Adult↗

Does being a woman make a difference in professional practice? A qualitative view to the practice of rheumatology.

OBJECTIVE: To explore to what extent female rheumatologists perceived that gender had an influence on their professional practice or on their choice of specialty. METHODS: We collected data from 29 female rheumatologists and used qualitative and quantitative methods to describe the findings. RESULTS: Three groups of respondents were identified (cluster analysis). Most of these rheumatologists perceived that gender influenced the way they practiced, although the specific weights of the topics were different in the 3 groups. Gender also influenced these physicians' choice of specialty field. CONCLUSION: The differences in practice style may have a potential effect on health systems as feminization of the medical profession increases.

Female↗

Clinical significance of serum and urine prolactin levels in lupus glomerulonephritis.

UNLABELLED: Prolactin (PRL) has been involved in the pathogenesis of systemic lupus erythematosus (SLE) and hyperprolactinemia has been connected with systemic activity. However, the clinical significance of PRL has not been investigated in lupus glomerulonephritis (GN). METHODS: We studied SLE patients (ACR criteria) with biopsy-proven renal disease. Renal histology was classified according to World Health Organization (WHO) criteria. Renal function tests, albuminuria, complement levels (nephelometry), anti-DNA antibodies (C. luciliae) and serum and urine PRL concentrations (RIA) were determined at baseline and at 4-month intervals for one year. Renal activity was defined as mild, moderate or severe according to serum creatinine, creatinine clearance, albuminuria, red blood cells (RBC), and casts. RESULTS: There were 26 patients with mean age 28.5 y and mean disease duration 47.9 months. Twenty patients had diffuse proliferative glomerulonephritis (GN), four had focal GN and two had membranous GN with proliferative changes. Renal activity was mild in ten patients, moderate in ten and severe in six. Mean serum (24.7+/-5.3) and urine (0.90+/-0.36) PRL levels were higher in patients with severe renal activity (P < 0.05 compared with mild group). PRL levels decreased after treatment, but this trend was not uniform during the follow-up period. CONCLUSION: Hyperprolactinemia was prevalent in SLE patients and high levels of PRL in the serum and urine could be related to severe renal disease.

Adolescent↗

Salmonella pyomyositis in patients with the human immunodeficiency virus.

Pyomyositis is a common disease in the tropics, mostly due to Staphylococcus aureus. We report two patients infected with the human immunodeficiency virus (HIV) who presented with fever and unilateral limb swelling and in whom pyomyositis was diagnosed in quadriceps and gluteus major, respectively. Salmonella enteritidis was isolated in both, with recurrent episodes of muscle involvement and secondary osteomyelitis in one case. Non-typhi Salmonella pyomyositis may occur in HIV + patients with a relapsing and aggressive clinical course in some cases.

Adult↗

The heart in the primary antiphospholipid syndrome.

Twenty consecutive female patients (mean age 30.1 years) with Primary Antiphospholipid Syndrome (PAPS) were studied cardiologically through noninvasive methods and compared with 20 age-and sex-matched healthy subjects. On physical examination 13/20 patients (65%) with PAPS had a valvular abnormality. In 12/20 (60%) patients with PAPS the ECG was abnormal, mainly due to sinus tachycardia in 5 (25%) and acute myocardial infarction in 3 (15%). In 7/20 patients with PAPS (35%) abnormal pulmonary findings were detected by X-ray and in 6 (30%) they were related to dilated pulmonary arch and pulmonary hypertension. In 14/20 cases (70%) with PAPS, abnormal echocardiographic findings were present; 13/20 patients (65%) had valvular complications attributable to PAPS: mitral insufficiency in six cases; mitral valve prolapse in three and aortic insufficiency in three. Two had pulmonary artery hypertension and two, tricuspid regurgitation (one attributable to PAPS). All valve diseases were regurgitant with mild to moderate hemodynamic repercussion. No stenotic lesions were detected. The mean mitral thickness in patients with mitral valve involvement was 7.0 +/- 1.6 mm, compared to 2.8 +/- 0.7 mm in patients with normal valves and 3.1 +/- 0.9 mm in the control group (p < 0.001). The mean aortic valve thickness in patients with aortic valve involvement was 3.6 +/- 0.5 mm compared to 1.5 +/- 0.3 mm in patients with normal valves and 1.4 +/- 0.4 in the control group (p < 0.001). None of the patients from the control group had valve disease (p < 0.0001). Three cases (15%) had pericardial effusion diagnosed by echo. Two patients with PAPS died during the 4.7 +/- 1.2 years of cardiological follow up, due to acute myocardial infarction and embolic cerebrovascular accident, respectively. In conclusion, cardiologic complications are common in PAPS, including left side regurgitant lesions that might be hemodynamically significant, acute myocardial infarction, pericardial effusion and pulmonary hypertension.

Adult↗

Tru-cut needle biopsy of subcutaneous fat in the diagnosis of secondary amyloidosis in rheumatoid arthritis.

In order to investigate the presence of secondary amyloidosis in patients with rheumatoid arthritis (RA), we performed an abdominal subcutaneous fat biopsy with a tru-cut needle in 50 patients. The tissue was stained with Congo red and was observed with polarized light microscopy. We found amyloid deposits in 78% of our patients. We randomly selected ten patients with a positive biopsy and a second procedure was performed. Tissues were studied with electron microscopy. We found unbranched fibrils characteristic of amyloid in all of them. We found a direct correlation with rheumatoid factor titers: the more intense the amyloid deposit, the higher the rheumatoid factor titers (p < 0.001). We did not find any correlation between amyloid deposits and clinical manifestations of disease. Amyloid deposits in RA are more frequent than previously thought, and their clinical importance remains to be determined.

Adipose Tissue↗

Transverse myelitis in systemic lupus erythematosus--the effect of IV pulse methylprednisolone and cyclophosphamide.

Transverse myelitis is a rare but serious complication of systemic lupus erythematosus (SLE). Standard treatment with medium to high doses of oral prednisone has been inadequate to control neurologic sequelae, and patients remain confined to a wheel chair or even die. We employed aggressive treatment in 7 patients with transverse myelitis complicating SLE with pulse methylprednisolone for acute episodes followed by pulse cyclophosphamide for a mean of 6 months; most of our patients are currently able to walk and have partial or total sphincter control.

Adolescent↗

Apophyseolysis and osteochondrosis of the ischial tuberosity. Criteria of differential diagnosis.

The authors describe three pathological entities affecting the apophysis of the ischial tuberosity observed in young athletes. Acute and chronic apophyseolysis and osteochondrosis are studied separately in terms of their clinical characteristics, radiographic aspects, and treatment. A rare case of bilateral apophyseolysis is also reported. Unless correctly diagnosed and treated, these lesions may in the long term affect the sporting activity of the patient. Six cases are presented illustrating each of these syndromes.

Adolescent↗