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Biomedical subjects

P Fietta

Publications and source records attributed to P Fietta.

14 recordsLinked to original sources

Pachydermoperiostosis and psoriatic onychopathy: an unusual association.

A 33-year-old man, suffering from cutaneous psoriasis since the age of 16, in the last 6 years experienced slow and painless enlargement at his fingertips and later at his big toes, which resulted in digital clubbing. Since the age of 31, the patient also presented psoriatic nail changes involving all his fingernails, without joint pain or inflammation. The patient's family history was negative for psoriasis, however, his brother, a 29-year-old healthy man, also presented digital clubbing. The diagnosis of pachydermoperiostosis coexistent with ungual and cutaneous manifestations of psoriasis vulgaris was made. The differential diagnosis with psoriatic onycho-pachydermo-periostitis, as well as other clinical conditions that involve the distal interphalangeal joints is discussed.

Adult↗

[The hemophagocytic syndrome (macrophage activation syndrome)].

The hemophagocytic syndrome (HPS) is an uncommon, often misdiagnosed life-threatening disorder of immune regulation, characterized by a widespread proliferation and multisystemic infiltration of non-malignant histiocytes that undergo uncontrolled hemophagocytosis in bone marrow and/or reticulo-endothelial system. The HPS immune dysfunction consists in a low or absent cytotoxic T and natural killercell activity and in hyperactivation of T lymphocytes and macrophages, with consequent proinflammatory cytokine storm. Clinically, HPS is characterized by high fever, lymphadenopathies, hepato-splenomegaly, liver dysfunction, (pan)cytopenia, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, as well as coagulopathy and neurological manifestations in many cases. A hereditary/primary variant (familial hemophagocytic lymphohistiocytosis) and an acquired/secondary form (reactive HPS) are known. This latter may occur in several disorders, including infections, immunodeficiency states, malignancies, lymphoproliferative and autoimmune diseases. Without treatment, HPS fatally has an unfavourable prognosis. Recently, outcome improvements have been reported, due to better therapeutic strategies. The clinical and pathological features of this syndrome are reviewed.

Histiocytosis, Non-Langerhans-Cell↗

[Adult onset Still's disease].

Adult onset Still's disease (AOSD), the adult variant of the systemic form of the juvenile rheumatoid arthritis, is an uncommon disorder of unknown origin. Although the pathogenesis has not yet been clarified, an immunologically mediated inflammation occurs in active AOSD. High spiking fever, evanescent maculo-papular skin rash, arthralgias/arthritis, neutrophilic leukocytosis, negative rheumatoid factor and antinuclear antibodies, as well as a marked hyperferritinemia are the major features of AOSD. Sore throat, lymphadenopathies, hepato-splenomegaly, abdominal pain, polyserositis, respiratory distress syndrome, multiple organ dysfunction and disseminated intravascular coagulation may also occur. The clinical course of AOSD is extremely variable and unpredictable and can be divided into three main patterns: a self-limited or monocyclic pattern, a polycyclic or intermittent course, with one or more flares of the disease and complete remission among the episodes, and a chronic course, characterized by persistently active disease, usually due to a chronic, destructive arthritis. Since there are not pathognomonic laboratory parameters or histological findings, the diagnosis of AOSD requires the exclusion of infectious, malignant and autoimmune disorders. Some sets of criteria for classification have been proposed, but so far not validated. The prognosis of AOSD is usually considered relatively benign, although a destructive arthritis may cause severe disability and the multisystemic life-threatening complications of the disease may determine a fatal outcome. Treatment usually consists in nonsteroidal anti-inflammatory drugs and corticosteroids, but a more aggressive approach with disease modifying antirheumatic or immunosuppressive drugs may be required.

Adult↗

[Mondor's diseases. Spectrum of the clinical and pathological features].

Mondor's disease is an uncommon disorders, occurring mostly in middle-aged women, and characterized by superficial thrombophlebitis classically involving the thoraco-epigastric veins and/or their confluents. Rare cases have been reported in atypical sites (upper arms, abdomen, groin and penis). The most common clinical features include lateral chest wall tension and pain, as well as the presence of a tender subcutaneous linear or winding cord-like structure, corresponding to the affected vessel, often with skin redness, edema or retraction. The etiology is unknown. Mondor's disease may be primary or secondary to local trauma, surgical procedures, bandaging, tight clothes and infections; it may also be associated with breast cancer. This condition is usually a benign and self-limited process, requiring only symptomatic treatment. Surgery is indicated when it is associated with malignancies or severe local pain and retraction. The clinical and pathological spectrum of this disease are reviewed.

Humans↗

Remitting seronegative symmetrical synovitis with pitting oedema in a patient with myelodysplastic syndrome and relapsing polychondritis.

Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) may be the inaugural manifestation of different rheumatic diseases of the elderly, malignancies and myelodysplastic syndromes (MDS). Relapsing polychondritis (RP) is a rare systemic disorder characterised by an inflammatory process involving predominantly cartilaginous structures, the cardiovascular system and organs of special sense. We report on a 72-year-old man with RS3PE and MDS, refractory anaemia subtype, diagnosed at the same time as RS3PE. Several months later the patient presented a clinical and pathological picture compatible with RP. Although the association between RP and MDS is well known, no previous cases of RS3PE preceding RP have been reported. This case confirms that RS3PE may herald many diseases, among others autoimmune disorders such as RP.

Aged↗

Coexistent Marfan's syndrome and ankylosing spondylitis: a case report.

We report on a 46-year-old man with a 4-year history of predominantly nocturnal pain at the thoracic and lumbar spine as well as accompanying morning stiffness and episodes of alternating buttock pain. At physical examination the patient presented with the typical traits for Marfan's syndrome (MFS), along with limitation of both chest expansion and movement in all planes of the lumbar spine. Pelvic and lumbar spine radiographs showed findings consistent with ankylosing spondylitis (AS). Laboratory tests were consistent with an inflammatory state and HLA typing was positive for the B27 antigen. Transthoracic echocardiography showed prolapse of the posterior mitral leaflet and mild aortic insufficiency. We diagnosed co-existent MFS and AS. The association of these two pathologies is particularly interesting, owing to the co-existence of hypermobility of peripheral joints due to MFS ligamentous hyperlaxity, and the reduction of both axial skeleton motility and chest expansion related to AS. As both of these diseases may damage the cardiovascular system over time, follow-up with echocardiography monitoring is indispensable.

Echocardiography↗

Antioxidant activity of bull semen in relation with aging.

This paper reports on the findings of an antioxidant activity in whole semen from bull and its components, washed spermatozoa and seminal fluid. The antioxidant activity has been evaluated as the ability of semen or its components to inhibit the spontaneous autooxidation of epinephrine a pH 10, 2, which involves the production of superoxide radicals (0(2)). This preliminary study provides further evidence on the role of free radicals, which are well known to be dangerous to cellular life, and points to the need of a better understanding of the role of antioxidant activities as a cellular protective mechanism.

Aging↗

[Study of the effects of free radicals produced by ultraviolet rays on viscosity of human synovial fluid. Methodological details].

After having pointed out a method measurement of the synovial Fluid (S.F.) viscosity by means of a white blood cell diluting pipette, obtaining results similar to those of Ostwald's viscometer, the AA have studied the osteoarthrotic S.F. viscosity after Ultra Violet Illumination. The production of Free Radicals induced by U.V., determines a fall of osteoarthrotic S.F. viscosity towards the values found in arthritic S.F..So, the AA, indirectly, confirme the capacity of Free Radicals to induce a S.F. viscosity modification like that provoked by the phlogistic process.

Arthritis, Rheumatoid↗