[Protein C deficiency as the cause of a deep venous thrombosis in a patient under treatment with oral contraceptives].
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Biomedical subjects
Publications and source records attributed to E Pedrol.
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Mitochondrial diseases are polymorphic entities which may affect many organs and systems. Skeletal muscle involvement is frequent in the context of systemic mitochondrial disease, but adult-onset pure mitochondrial myopathy appears to be rare. We report 3 patients with progressive skeletal mitochondrial myopathy starting in adult age. In all cases, the proximal myopathy was the only clinical feature. Mitochondrial pathology was confirmed by evidence of ragged-red fibres in muscle histochemistry, an abnormal mitochondrial morphology in electron microscopy and by exclusion of other underlying diseases. No deletions of mitochondrial DNA were found. We emphasize the need to look for a mitochondrial disorder in some non-specific myopathies starting in adult life.
Two patients who developed pain and tenderness in their calf muscles, without other organ system involvement, are described. A gastrocnemius biopsy disclosed necrotizing muscular arteritis. Five other reported cases are reviewed and we conclude that there is a limited form of polyarteritis nodosa localized to calf muscles, with a differentiated and more benign clinical course, a differential diagnosis restricted to local pathological processes and prompt response to treatment with corticosteroids alone.
The IgM myeloma is a rare type of multiple myeloma (MM) with some features which differentiate it from other immunologic types of myeloma and from Waldenström macroglobulinemia (WM). Two patients with IgM myeloma in whom the only clinical feature of the disease was the development of bone lesions and general deterioration are reported. In none of the cases an M component was detected in proteinogram. A bone marrow proliferation of plasma cells was discovered in both, which it was accompanied with dura mater infiltration in one of them. The type of secreted immunoglobulin was IgM lambda in the first case and kappa in the second. Both patients had a poor clinical course after the diagnosis and died due to infective complications. Emphasis is made on the need to differentiate this condition from WM due to their different prognosis and therapy.
The infection of muscle is an infrequent condition. We report on a patient with a juvenile form of dermatomyositis who developed infectious myositis caused by Streptococcus pyogenes. The inflammatory myopathy probably favoured the colonization of muscle during a bacteremia related to the skin lesions. The main forms of streptococcal myositis, which can currently be differentiated by means of imaging techniques, are discussed in addition to its treatment and prognosis.
A randomized, placebo-controlled trial was performed to assess the effect of magaldrate (800 mg every 4 h) in reducing the rate of upper gastrointestinal tract bleeding among 100 consecutive patients with severe diseases admitted to a general hospital ward. Upper gastrointestinal tract bleeding occurred in 11 of 48 placebo-treated patients and in only 1 of 52 magaldrate-treated patients (p less than 0.01). Endoscopic examination of these patients showed gastric ulcer (two cases), multiple gastric mucosa ulcerations (nine), and no lesions (one). In three patients who received placebo the hemorrhage was clinically relevant and required transfusion of two or more blood units. Patients with two or more risk factors showed a higher rate of gastrointestinal hemorrhage (p less than 0.05). Respiratory failure and treatment with a high dose of corticosteroids were associated with the highest incidence of bleeding (p less than 0.05 for both). The only adverse reaction associated with magaldrate was a mild and self-limiting diarrhea in two cases. We conclude that patients seriously ill admitted to a general hospital ward should be treated with a prophylactic agent against stress-induced ulcer bleeding. Magaldrate is an effective and safe antacid to prevent gastrointestinal tract bleeding in such patients.
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We here present the clinical cases of two homosexual patients, carriers of human immunodeficiency virus (HIV), who later presented a syphilis infection and who after receiving the usually recommended treatment, suffered a relapse of the infection six months afterwards, with neurologic involvement in one case. The clinical characteristics are discussed as well as the diagnostic and therapeutic problems which syphilis infection presents in HIV infected patients. Serological results are comparable to those of the general population, although face positives have been occasionally reported as well as some abnormally elevated titers. It is possible that neurosyphilis might be more frequent and of earlier appearance in HIV infected patients. Therefore, it might be necessary to carry out a spinal fluid exam, in these type of patients, in order to rule out the existence of an early and/or asymptomatic neurologic affectation and give the appropriate antibiotic treatment.
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A prospective study is performed in 108 patients presenting hemoptysis who were attended in the emergency department. Final diagnosis was achieved in 89 cases (82.4%), being lung infections of tuberculous origin or not, neoplasias, and chronic obstructive lung emphysema the main observed etiologies. A low percentage of severe hemoptysis is found (5.6%). Diagnosis was obtained in 79.6% of patients by clinical history, physical exam, chest x-ray and ORL exam. Only 13.6% of patients who were not diagnosed in the Emergency department were later diagnosed by follow up and performance of complementary tests. Similarly, the lack of an initial etiological diagnosis had no repercussion, by itself, in a worse prognosis. Therefore, it is recommended to perform a single strict evolution control in patients with hemoptysis of unknown origin who present risk factors of lung neoplasia (male sex, age greater than 40 years, smoking greater than 45 packs/year).
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During a 40-month period 78 episodes of encephalic toxoplasmosis (ET) were diagnosed in 57 patients among 394 with acquired immunodeficiency syndrome (AIDS) (based on CDC criteria) in the Hospital Clinic from Barcelona. 38 patients were parenteral drug abusers (66.7%), 17 were homosexual males (29.8%) and 2 were heterosexual females (3.5%). ET was the first opportunistic infection in 28 patients (49.1%). The most common symptoms at the time of diagnosis were focal neurological signs (62.8%) and fever (52.6%). Usually, computed tomography showed single or multiple hypodense lesions (70.3%) with ring-like (46.1%) or nodular (28.3%) uptake. Serology (indirect immunofluorescence) was diagnostic in only 24 cases (42.1%). The acute episodes were treated with the combination pyrimethamine/sulfadiazine (P/S) during 3-6 weeks, and folinic acid was associated. If the patient had previous allergy to sulfonamides or allergy developed during the acute phase, clindamycin (C) was given instead of S. The patients surviving the acute episode (49, 86, 0%) underwent, if they consented, maintenance therapy with P/S two days per week (15 patients) or with C if sulfonamide allergy was present (10 patients). No relapse was observed in those receiving P/S, but 40% of those treated with P/C relapsed. It was concluded that ET is a common opportunistic infection in the AIDS patients, that the treatment of the acute phase with P/S or with P/C is satisfactory, and that maintenance therapy with P/S two days per week may be effective to prevent relapses.
Two patients are reported with secondary amyloidosis in whom renal biopsy disclosed massive amyloid deposition with exclusively extravascular localization. In one case, autopsy showed selective amyloid deposition in the vessel walls of most examined organs. In both patients, the clinical presentation consisted of subacute renal failure without proteinuria. A possible reduction of renal perfusion, secondary to a depleted blood volume in one case and to pharmacological inhibition of prostaglandin synthesis in the other, could enhance renal dysfunction in both patients, who, on the other hand, previously had mild renal failure. The clinical presentation of renal amyloidosis basically depends on the distribution and severity of amyloid deposits. Vascular localization represents an uncommon pattern of renal amyloidosis, generally associated with chronic renal failure with minimal or no proteinuria.
Fifty-five episodes of central nervous system (CNS) toxoplasmosis developing in 43 of the 329 AIDS cases seen at our institution were diagnosed during a 34-month period and were prospectively studied. Acute episodes were treated with a pyrimethamine/sulfadiazine (P/S) combination for a mean of 21 days. Because of a previously known major allergy to sulfonamides, three episodes were treated with clindamycin instead of sulphadiazine. In those patients who accepted maintenance therapy, a combination of P/S or pyrimethamine and clindamycin (P/C) was administered 2 days per week. Thirty-six patients (83.7%) survived the first episode. Four of these 36 were lost to further study. Six of the 12 (50%) who decided not to undergo maintenance therapy relapsed (mean follow-up: 12 months). Fourteen patients were given P/S and none relapsed while they were on maintenance therapy (mean follow-up: 10.3 months). Six patients received an intermittent maintenance treatment with P/C and one relapsed 2 months after starting the maintenance therapy (mean follow-up: 13.7 months). We conclude that an intermittent (2 days per week) maintenance treatment for CNS toxoplasmosis with P/S was effective in preventing relapses, although prospective randomized studies remain to be done.
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Two cases of colitis due to Salmonella enteriditis which later developed a toxic megacolon with intestinal perforation are presented and the probable pathogenesis is discussed. This exceptional clinical course which has not been previously described forces to perform a differential diagnosis with chronic intestinal inflammatory disease which must be based on microbiologic, serologic and/or histologic criteria. Moreover, the authors warn about the unsuitability of using anticholigernic drugs in gastroenteritis since they could be related to the ethiopathogenical basis of the disease's unfavorable course in the described patients.
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