[Letter: Hypoglycemia during beta blocking treatment].
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Biomedical subjects
Publications and source records attributed to O Bletry.
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The authors have collected 19 cases of ankylosing spondylitis with an alteration of intracardiac conduction. The lesions are usually situated high in the bundle of His, as shown by successive electrocardiograms and endocavitary studies. Progression by regressive acute episodes and the response to anti-inflammatory agents suggests that the disorder of conduction is inflammatory in origin. Syncopal attacks are rare (1 case out of 19) and a pacemaker is rarely indicated. Almost half the patients had aortic insufficiency, one patient had tricuspid stenosis and two patients had heart failure in the absence of any valvular lesion. The associated ankylosing spondylitis is characterised by the severity of the inflammatory signs (average sedimentation rate 50 mm in the first hour) and by the extent of peripheral articular involvement and extra-rheumatological manifestations. Almost one in two patients had iritis, with the same proportion applying to a past history of Reiters syndrome.
The authors report ten cases of acute recurrent pericarditis observed over a period of 5 years and emphasize the frequency of these relapses involving 15 to 20% of cases of acute pericarditis. Usually, no cause is found and, althrough an immunological mechanism has been suggested, there is no common test which may confirm or disprove this theory. The risk of ultimate constrictive pericarditis is minimal and this risk is thus not taken into consideration in deciding on treatment. On the other hand, the undesirable effects of corticosteroids which favour relapses, seem to be demonstrated. The use of corticosteroids in cases of acute pericarditis should thus be exceptional and one should prefer non-steroid anti-inflammatory drugs. Immunosuppressors and pericardectomy should be reserved for the rare forms where the disease progresses and relapses continue after cortisone has been stopped.
Allergic granulomatous angeitis or Churg and Strauss disease, seems to form part of the disease of necrotising angeitis. Clinically, it may be considered when periarteritis nodosa is associated with asthma and marked eosinophilia. The diagnosis depends on the pathological findings including the venous and pulmonary lesions, giant cells and the presence of extra-vascular granulomas. The recognition of this disease does not, however, permit one to give a precise prognosis. In the absence of progress concerning the pathogenesis, treatment will be the same as that given in other forms of necrotising angeitis.
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Pleuro-pulmonary manifestations are frequent in systemic lupus erythematosus (SLE), being found in 40 to 70% of patients with this disease. However, these manifestations can be attributed to SLE only when other pathologies, and notably infections, have been excluded. The truly SLE-related pleuro-pulmonary manifestations can be divided into five types: pleurisy, interstitial pneumonia, lupus pneumonia and two new entities: diffuse pulmonary haemorrhage and pulmonary arterial hypertension. The most frequent manifestation, pleurisy, only requires symptomatic treatment combined with non-steroidal anti-inflammatory agents. Corticosteroids are seldom necessary, but they must be used in lupus pneumonia or in diffuse interstitial pneumonia, owing to the severity and potentially poor prognosis of these two manifestations. Pulmonary haemorrhage is a serious and probably underestimated manifestation; it is diagnosed by bronchoalveolar lavage which also enables other causes, in particular infections, to be excluded. As soon as the aetiological diagnosis is made, high-dose corticosteroid therapy, usually combined with immunosuppressants, is mandatory. Pulmonary arterial hypertension is a classical, but hitherto unrecognized manifestation of SLE which benefits from new exploratory techniques, such as doppler-ultrasonography. At present, its diagnosis rests on data supplied by cardiac catheterization which is generally performed too late, making it irreversible and resistant to all treatments. Some of these pleuro-pulmonary manifestations are probably underestimated and they require new methods of investigation, such as bronchoalveolar lavage or doppler-ultrasonography, resulting in earlier diagnosis and treatment at an accessible stage.
The authors report five cases of malignant fibrous histiocytoma of the soft tissues, some of them with atypical presentation (necrotizing tumor, hypereosinophilic syndrome, dermatomyositis). This is the first case of polymyositis associated with malignant fibrous histiocytoma. The tumor occurs between the fifth and the seventh decade, principally as a mass on the proximal lower limb. Local recurrence and metastasis occurs in 50% of patients. Prognosis is dependent on both the surgical possibilities and the characteristics of the tumor (site, size, histologic grade). Surgery alone is inadequate. Adjuvant chemotherapy improves the 5-year survival.
The authors report a case of insufficiency fracture of the sacrum in an 81-year old woman. The fracture was related to disjunction of the pubic symphysis consecutive to curettage of the pubic bones for septic osteitis developed after vulvectomy for cancer. The diagnosis was suspected on the results of bone scintigraphy and was confirmed by computerized tomography with frontal sections and contrast inversion, showing the characteristic H-shaped fracture.
We present the case of a young woman with a protein-losing enteropathy occurring in the context of systemic lupus erythematosus. This rare complication has limited gastro-intestinal manifestations and must be systematically looked for when hypoalbuminemia occurs in the absence of a lupus nephritis. High dose corticosteroids therapy (> or = 1 mg/kg/day of prednisone) usually leads to recovery, and should be the first treatment attempted. If this treatment is ineffective, bolus injections of methylprednisolone (1 g/day for three days) may be recommended. Immunosuppressive therapy should be given only if the above treatments are ineffective, or in case of cortico-dependency.
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Much that has been written in the last few years concerning temporal arteritis has allowed us to better clarify the clinical picture, and for example frequency of hepatic involvement. The atypical forms of the disease such as those that involves large arteries, or those in which sedimentation rate is normal, are better known. It is in such atypical cases, particularly in a young patient, that an external carotid arteriography is most useful. Though the pathophysiology of the disease is not well understood, serologic and histologic arguments exist in favor of an autoimmune etiology, in which the antigen would be the alestin, found in the internal elastic lamina of the artery. Temporal arteritis is theorically well classified amongst the giant cell granulomatous arterities but there are some atypical forms difficult to distinguish from Takayasu's arteritis or even periarteritis nodosa.
Polyarteritis nodosa (PAN), with a fatal outcome, developed during the course of a therapeutically controlled Graves'disease. A review of the published literature confirms that two mechanisms may be involved in the onset of PAN in hyperthyroid patients: an immune response, which is probable in the case reported, or a toxic effect related to the treatment of the hyperthyroidism. This case also emphasizes the therapeutical difficulties encountered, in spite of the corticoid and cyclophosphamide associated treatment with plasmapheresis.
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Five patients with dermatomyositis developed calcinosis universalis, the calcinosis lesions being diffuse in three cases and localized to a subcutaneous site in the other two. The diffuse lesions developed in young subjects with severe dermatomyositis, and increased progressively during recovery from the muscle disease. Treatment was ineffective. Subcutaneous calcifications can be detected at an early stage by technetium pyrophosphate scintigraphy, while the fluid nature of the swellings due to a local inflammatory reaction is proved by ultrasonography. Good results are obtained after excision of those calcified masses requiring removal because of their size, site, or superinfection.
Sacro-iliac tuberculosis was detected in seven African immigrants following attacks of sacrolumbar pain with fever, over a period of five years. Two of the patients had a previous history of tuberculosis, and in six out of the seven cases the osteo-articular affection was part of a multifocal tuberculous disease. The value of bone scintigraphy is discussed. Surgical treatment was performed together with medical therapy in three cases.
Six patients with scleroderma (Barnett's type I and II) associated with hepatopathy are reported. Primary biliary cirrhosis was confirmed in four cases and was probably present in one case, while cirrhosis of unknown origin was present in the sixth patient. The outcome was fatal in two cases. Salivary secretions were reduced in all six cases, and a diagnosis of Gougerot-Sjögren's disease was confirmed in four patients. The frequency with which this triple association has been reported suggests that its incidence has been underestimated.
A prospective study was conducted to evaluate cardiac effects of scleroderma by means of phonomyography (30 patients) and ultrasound cardiography (18 patients). Clinically silent effusions can be detected by ultrasound cardiography, and 50 p. cent of the patients were found to have pericarditis through this investigative technique. Valvular lesions (mitral prolapsus) were present in a more important number than simply a coincidence. Measurement of myocardial relaxation appears to give the best indication of the specific myocardial lesion in this disease, and even may be of some prognostic value.