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Biomedical subjects

G Gremignai

Publications and source records attributed to G Gremignai.

8 recordsLinked to original sources

Lung involvement in systemic sclerosis sine scleroderma treated by plasma exchange.

Systemic sclerosis sine scleroderma can present in some patients as pulmonary interstitial fibrosis. Until now ten cases with this particular clinical variant, all men, have been reported in the literature. The knowledge of systemic sclerosis sine scleroderma presenting as lung interstitial involvement is important in clinical practice for an early diagnosis and correct therapeutic strategy. This work reports the clinico-serological features of two further cases, one a woman, of systemic sclerosis sine scleroderma with prevalent lung involvement, and describes the effects of therapeutic plasma exchange.

Female

Concomitant systemic lupus erythematosus and ankylosing spondylitis.

The case is reported of a 42 year old white woman meeting currently used diagnostic criteria for both ankylosing spondylitis and systemic lupus erythematosus (SLE). As found in a previously described similar case of a black man, HLA typing showed antigens associated with both SLE and seronegative spondyloarthropathy. This case thus supports the hypothesis that the two diseases occur together only when this rare combination of HLA antigens is present.

Adult

Tarsal tunnel syndrome in seronegative spondyloarthropathy.

A 53-year-old man affected by ankylosing spondylitis associated with psoriasis developed bilateral tarsal tunnel syndrome caused by synovitis of the accompanying sheaths of the tendons that run through the 'fibro-osseous tunnel'. This report confirms the hypothesis that the tarsal tunnel syndrome may be a clinical manifestation of seronegative spondyloarthropathy.

Humans

Treatment of the renal involvement in mixed cryoglobulinemia with prolonged plasma exchange.

Nine patients with mixed cryoglobulinemia and severe membranoproliferative glomerulonephritis were treated with plasma exchange alone or in combination with medium to low amounts of corticosteroids, but never with cytotoxic drugs. In 5 patients renal function and/or proteinuria improved after plasma exchange, and no clinical relapse usually occurred when the procedures were reduced or discontinued. These procedures seemed of particular effect in the presence of histologically active and not irreversible lesions and rapid deterioration of renal function. While cryocrit almost invariably decreased, circulating immune complex or complement levels were unpredictably affected by plasma exchange. Cryocrit, but not immune complex or complement levels, was the serological parameter which most often closely correlated with signs of renal involvement (i.e., proteinuria and/or serum creatinine). Thus, plasma exchange might be a safe and useful tool in the treatment of an often drug-resistant and rapidly progressive renal involvement occurring in patients with mixed cryoglobulinemia.

Adult

Plasma exchange with prostaglandin I2 and ACD solution: comparative effects.

Discontinuous plasma exchange with prostaglandin I2 (5 ng/kg/min) and low dosage heparin (5-6 IU/kg/min) (treatment I), and ACD solution alone (treatment II) was studied. During both treatments the activated partial thromboplastin time remained within the normal range. After treatment I platelet count was not decreased but in vitro platelet aggregation was reduced (p less than 0.001). After treatment II platelet count was reduced and in vitro platelet aggregation unchanged. Prostaglandin I2 at this dosage caused no cardiovascular complications. The physiopathological implications of these differences are discussed.

Adult

Plasma-exchange in mixed cryoglobulinemia. Effects on renal, liver and neurologic involvement.

Prolonged plasma-exchange without addition of cytotoxic agents was employed in 16 patients with mixed cryoglobulinemia and kidney, liver or neurologic involvement. Patients with rapidly progressive renal failure or active and reversible lesions generally improved after plasma-exchange, as well as those with a recently occurring sensory-motor peripheral neuropathy. In 4 out of 6 patients with mixed cryoglobulinemia and chronic active hepatitis, plasma-exchange was followed by either normalization or significant reduction of liver enzymes and bromosulfophthalein retention. In all cases responding to plasma-exchange the beneficial effects were evident after the first 2-3 weeks of treatment, while symptoms did not generally recur when the procedures were either slowly tapered or discontinued. Although the pathogenetic mechanism(s) of action of plasma-exchange remains largely unknown, preliminary data indicate that these procedures induce quantitative as well as qualitative changes in the immune system.

Adrenal Cortex Hormones

Decrease and rapid recovery of protein C after plasma exchange.

The anticoagulant protein, protein C (PC), was measured after 40 plasma exchanges (PEs) in 26 patients treated for a variety of disorders, most of which were immunological in nature. After 27 PEs involving exchange of 50 percent of the plasma volume with albumin and saline, mean PC activity and antigen decreased in parallel to about one-half normal levels, with good correlation between the two assays. Antithrombin III and prothrombin decreased to about the same levels as PC, with no significant differences between the percentage changes for either protein. After five PEs, during which exchange of larger plasma volumes was performed (86%), the percentage change of PC was greater than after the 50 percent exchange (38 +/- 22 vs. 55 +/- 24). To study postexchange recovery, PC was also measured serially for up to 24 hours after eight PEs (50% exchange). At 24 hours postexchange, PC levels did not differ significantly from pre-exchange levels. This study demonstrates that decreases in PC are in proportion to the volume of plasma exchanged during PE. However, PC levels returned to normal within 24 hours after PE, so that any hemostatic imbalance induced by low PC should be transient.

Antithrombin III

Blood viscosity and filtration abnormalities in mixed cryoglobulinemia patients.

Blood, plasma and serum viscosity and blood filtration were investigated in 43 unselected mixed cryoglobulinemia patients. A hyperviscosity syndrome was present in only one patient, and rapidly improved after plasma exchange and cyclophosphamide therapy. A clear-cut increase in blood viscosity was infrequently observed in mixed cryoglobulinemia, although significant differences were present in the plasma and serum viscosity of patients and controls. In contrast, blood filtration was severely impaired in a high percentage of cases (51 and 72% of the values recorded at 37 degrees and 25 degrees C, respectively), and was on the average significantly higher in patients than in controls. Indirect evidence suggests that blood viscosity is at least in part related to cryoglobulins. In 19 patients studied before and after cryoglobulin removal, serum viscosity significantly decreased when the serum was deprived of cryoglobulins. In addition, the cryocrit correlated with all the hemorheological parameters with the exception of blood filtration. The hemorheological findings were compared with multisystemic features of the disease, i.e. liver, renal, lung, neurologic, vascular and funduscopic alterations. The potential clinical relevance of the hemorheological parameters was stressed by the close correlation between blood filtration parameters and serum creatinine. Furthermore, by discriminant analysis, viscosity and blood filtration changes were the serological parameters most significantly associated with the presence of renal, liver and neurological involvement. Thus, hemorheological parameters are frequently abnormal in mixed cryoglobulinemia patients, and seem to play a significant clinical role; they should therefore be included in the work-up of these patients.

Adult