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Biomedical subjects

P E Marchiori

Publications and source records attributed to P E Marchiori.

At least 73 records · Page 4Linked to original sources
PubMed1979

[Hematin treatment of acute intermittent porphyria. A case report (author's transl)].

A young woman with acute intermittent porphyria in profound relapse and severe nervous and respiratory involvement was treated by intravenous infusions of hematin, followed by improvement of symptoms. The diet with high carbohydrate and protein content and a B-adrenergic blocking agent, not showed any beneficial effect on acute attack. The infusion of hematin was followed by a lowering effect on urine porphybilinogen and delta-aminolevulinic acid and clinical improvement. The return to normal of porphyrin precursors in the urine was accompanied by almost complete clinical remission. The relationship of remission and repression of delta-aminolevulinic acid, decrease of urinary levels of porphyrin precursors, pulmonaries tests and electromyograms, were discussed.

Adolescent↗
PubMedDate not supplied

Thymectomy for myasthenia gravis: evaluation of results in 282 patients.

The results of thymectomy in 282 patients with acquired and generalised myasthenia gravis are presented. The study includes patients submitted to surgical treatment in the period between 1958 and 1990. Remission or marked improvement was obtained in 70% of the patients with follow-up extending from one to 25 years. The total post-operative mortality rate was 8% dying predominantly patients with thymoma. Median sternotomy was made in 278 patients, transcervical thymectomy in two patients and thyroidectomy with concomitant thymectomy in other two. Surgical methods, operatory phases, previous treatment with prednisone and long term evolution with final results are discussed.

Adolescent↗
PubMedDate not supplied

Juvenile myasthenia gravis and thymectomy: report of ten cases.

Ten patients from 7 to 12 years of age presented generalized acquired myasthenia gravis: six had a severe form and four a moderate one. All patients showed progressive worsening and poor response to the medical treatment; all of them were submitted to thymectomy by median sternotomy. Long-term results were beneficial for 60% of the patients who experimented a marked improvement or even complete remission. One patient improved without medication being followed for two years. Another patient has been in remission taking only a minimal dose of anticholinesterase drug for seven years. In most cases prednisone has been used as long-term maintenance in the postoperative phase. Short-term results were not favorable: one death occurred and 30% of the patients worsened.

Child↗
PubMedDate not supplied

[Progressive systemic sclerosis, B-cell malignant lymphoma of the ileum and thyroid adenocarcinoma].

A patient with progressive systemic sclerosis with rapid evolution to death is presented. The post-morten examination revealed besides the typical changes of the progressive systemic sclerosis in the skin, kidneys, heart and blood vessels a B-cell ileal malignant lymphoma with generalized metastases, and an adenocarcinoma of the thyroid gland. A possible mechanism involved in the association of these diseases might be the action of the oncogenes modifying the cellular hormostasis and the immunological tolerance.

Adenocarcinoma↗
PubMedDate not supplied

[Percutaneous synovial biopsy in the diagnostic evaluation of the patient with rheumatic disease].

Forty seven percutaneous synovial biopsies from 22 patients with rheumatoid arthritis, four of whom with the juvenile form, 13 with indetermined polyarthritis and 12 with monoarthritis, were evaluated. The histopathological examination confirmed the clinical diagnosis in 76% of cases with rheumatoide arthritis and juvenile rheumatoid arthritis, and it suggested the diagnosis of rheumatoid arthritis in 80% of cases with indetermined polyarthritis. In two cases of monoarthritis it reveled acid-fast bacilli, and a granulomatous process in one. These resuls suggest that the synovial biopsies can be useful for the establishment of diagnosis in patients suffering from indetermined poly or monoarthritis.

Adolescent↗
PubMedDate not supplied

Nervous system involvement in systemic lupus erythematosus: report of three cases.

Central nervous system involvement in systemic lupus erythematosus is rather frequent whereas peripheral nervous system involvement is much less common. The three patients studied by us had isolated manifestations uncommon in nature. The first one developed a sensory-motor polineuropathy with signs of axonal degeneration. It responded to the therapeutic association of corticosteroids with an immunosuppressive agent. Satisfactory recovery took place over a time span of a year. The second patient had encephalic and cerebral trunk involvement from which an irreversible dementia resulted. The third patient, who had recurrent aseptic meningitis, is asymptomatic for six months now. Patients one and two had no systemic manifestations at the time of nervous system involvement. Suspicion of systemic lupus erythematosus was made on the basis of past inspecific articular symptoms. The neurological and systemic manifestations may be sometimes simultaneous; they are usually followed by serologic changes. Isolated nervous system involvement may be seen with and without sorologic changes, and there may be found antibodies reactive with phospholipids (anticardiolipin, antigangliosides and anticerebrosides). The employment of nonsteroid immunosuppressive drugs associated with corticosteroids in small doses seems to be useful in cases of systemic lupus erythematosus with nervous system involvement.

Adolescent↗