1/6. microscopic polyangiitis presenting as idiopathic pulmonary fibrosis: is anti-neutrophilic cytoplasmic antibody testing indicated?We report a 55-year old woman with microscopic polyangiitis who presented with idiopathic pulmonary fibrosis and 1 year later developed hematuria and proteinuria. She had a high serum level of perinuclear anti-neutrophilic cytoplasmic antibodies. Renal angiogram was normal. The diagnosis of microscopic polyangiitis was confirmed by renal biopsy, which showed pauci-immune crescentic glomerulonephritis. The patient received immunosuppressive therapy and improved markedly. Consideration of small vessel vasculitis is important in the differential diagnosis of idiopathic pulmonary fibrosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/6. An aged patient with Henoch-Schonlein purpura nephritis: a case report and review of the literature.A 63-year-old patient with Henoch-Schonlein purpura (HSP) nephritis showed progressive proteinuria. purpura was observed in the extremities as well as in the trunk without any preceding upper respiratory tract infections or drugs, and it disappeared after corticosteroid therapy although mild proteinuria continued throughout the therapy. Renal biopsy specimens showed mesangial IgA deposits in the glomeruli, but no IgA deposits were observed in the dermal vessels or in the intrarenal extraglomerular capillaries. Progressive proteinuria was improved with 37.5 mg/day of captopril. Severity of HSP nephritis in middle-aged to aged patients in the literature was reviewed. It was suggested that careful observation is required in aged patients with HSP nephritis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/6. fabry disease--a diagnostic and therapeutic problem.The authors present a patient with Fabry syndrome that remained undiagnosed for several years. Fabry syndrome is a genetic disease related to changes on the x chromosome. Its complex clinical presentation and diverse symptomatology is caused by deficient activity of lysosomal hydrolase alpha-galactosidase enzyme. Defect in the basic alpha-galactosidase molecule implies genetic change, which can be a predisposing factor for the development of atypical and typical forms of this genetic disease. In the presented case, clinical manifestation and hemizygous symptomatology were the evidence of metabolic and genetic irregularity, typical clinical presentation of fabry disease. Many authors report generalized vasculopathy as a basic characteristic of fabry disease and a causative factor of multiorgan changes. Some authors indicate that persons with diagnosed asymmetric hypertrophy of the left ventricle have decreased alpha-galactosidase. Cardiac complications, coronary disease, and acute myocardial ischemia are often present in cases of fabry disease, frequently causing death in such patients. Characteristic central nervous system symptoms with skin-burning sensation and paresthesia were also present in our case. Cerebrovascular complications were caused by changes on small blood vessels. Clinical signs of renal failure were nonspecific, and the diagnosis was based on extrarenal symptoms. Initial renal manifestations were insignificant as asymptomatic proteinuria and microhematuria, due to which our patient was referred to further examination. The level of alpha-galactosidase was significantly decreased. The severity and progression of this disease depends on the level of alpha-galactosidase enzyme in serum and its catabolic effect. More recent studies have showed that deficient enzyme can be synthetic zed and, accordingly our patient has been successfully enrolled in the replacement therapy program.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/6. Increased plasma levels of the novel vasoconstrictor peptide endothelin in severe pre-eclampsia.plasma endothelin levels were studied in 29 pregnancies. Levels were found to be increased in 9 pregnancies complicated by pregnancy-induced hypertensive disease and/or pre-eclampsia as compared to levels in 14 normotensive pregnancies with gestational age varying between 24-40 weeks with normal Doppler flow velocity waveforms of the uterine arcuate arteries, and 6 normotensive pregnancies with abnormal Doppler flow velocity waveforms at 24 weeks' gestation. Two patients with severe pre-eclampsia showed a rise in plasma endothelin levels, suggesting a correlation between plasma endothelin levels and the disease process. Endothelin is produced by endothelial cells of blood vessels; it is the most potent vasoconstrictor known to date. For this reason it has been suggested that endothelin might be important in the control of systemic blood pressure and local blood flow, both disturbed in pre-eclampsia. The conclusion of this study is that the venous plasma level of endothelin would seem to be a marker for severe disease, however, without any predictive value.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/6. Treatment of massive hemorrhage after renal biopsy with percutaneous arterial obliteration.Severe perirenal hemorrhage is a major, although rare, postbiopsy complication. We report a case of a 48-year-old man with a postbiopsy massive bleeding from a ramification of the renal artery supplying the lower pole of the left kidney. Treatment consisted of complete obliteration of the bleeding vessel by helding a percutaneous catether in wedge position during a renal arteriographyc study. Our case shows that percutaneous arterial obliteration is a successful procedure in treating postbiopsy renal hemorrhage, representing an effective alternative to surgical therapy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/6. Polyneuropathy with endoneurial immune complex deposition as the first manifestation of systemic lupus erythematosus.A 72-year-old male presented with progressive sensorimotor polyneuropathy. Later weight loss, proteinuria and deteriorating renal function were noted. The electrophysiological examinations revealed extensive, symmetrical demyelinating and axonal polyneuropathy. frozen sections obtained from sural nerve biopsy sample showed the presence of immune complexes and complements in the walls of the epi- and endoneurial blood vessels, and perineurium suggestive of systemic lupus erythematous (SLE). IgG and Clq deposits were also present along the basement membranes of schwann cells. The electron microscopy confirmed the presence of immune complex deposition. diagnosis of SLE was proven by positive serology (anti-nuclear antibodies, anti-Sm, anti-RNP, anti-double-stranded dna) and renal biopsy showing membranous lupus nephritis with extensive immune complex deposition in the tubular basement membranes. Despite combined immunosuppressive treatment for 10 months, the patient died of complications of generalized immune complex vasculitis. The manifestation of SLE in elderly patients, especially in males, is very rare. Moreover, the polyneuropathy is an unusual initial symptom of SLE. Immune complex deposition in Schwann cell basement membrane probably plays an important role in the pathomechanism of sensorimotor polyneuropathy in SLE.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |