Multinodular pleural involvement in aggressive systemic mast cell disease.
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Biomedical subjects
Publications and source records attributed to J Ducla-Soares.
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Spontaneous bacterial peritonitis (SBP) is a frequent cause of decompensated alcoholic cirrhosis. The authors describe the first two cases caused by infection with Streptococcus bovis. They suggest that this microorganism may be present in the intestinal flora of these patients more frequently than assumed. Furthermore, the course of SBP due to S. bovis may be particularly severe.
To assess the correlation between the severity of clinical symptoms and sensorymotor and autonomic function in familial amyloidotic polyneuropathy of the Portuguese type (FAP-PT), clinical grade (CG), electromyographic score (ES), sympathetic (SS) and parasympathetic score (PS) were compared in 47 patients. SS and PS were both abnormal in 72% of patients with ES = 0% and in 73% of patients with CG = 0. When SS and PS were compared with either CG and ES, PS progressed more rapidly and stabilized earlier than SS. As a whole, SS and PS were greater as CG and ES were higher. This pattern of autonomic involvement should be taken into account when assessing the course of the disease and evaluating new forms of therapy.
BACKGROUND: Serial cerebral angiograms, computed tomography, and magnetic resonance imaging are among the proposed methods for monitoring disease activity and response to therapy in isolated angiitis of the central nervous system. Cerebrospinal fluid has not proved to be useful in monitoring clinical course. CASE DESCRIPTION: We describe a 45-year-old man with histological diagnosis of isolated angiitis of the central nervous system that was treated with prednisone plus azathioprine and monitored for 2 years. Samples of the cerebrospinal fluid were obtained for cytological and routine chemical examination, as well as albumin and immunoglobulin content. Before treatment, cerebrospinal fluid showed marked plasmatic transudation of albumin and intrathecal synthesis of immunoglobulins. During the first year of immunosuppression no events were noticed, and the previously abnormal aspects of the cerebrospinal fluid showed improvement. During the weaning of azathioprine, a new stroke occurred in conjunction with a marked deterioration of cerebrospinal fluid parameters. Immunosuppression was resumed at previous levels, and during the following year no further events occurred. Once again, abnormal cerebrospinal fluid values improved significantly. CONCLUSIONS: We report a case of isolated angiitis of the central nervous system in which the serial cerebrospinal fluid examinations (albumin and immunoglobulin content) showed a close correlation with clinical course. This method may be useful in monitoring response to therapy.
Herein we describe what is, to our knowledge, the first reported case of a composite tumor of the main bile duct with epiploon metastases. Glucagon, pancreatic polypeptide, and somatostatin-immunoreactive cells were demonstrated in these metastases, but not serotonin, gastrin, or insulin-immunoreactive cells. The clinical significance of the neuroendocrine cells in the present case is discussed.
Plasma adrenaline and noradrenaline levels were measured while supine and following head-up tilt to 45 degrees, in both normal controls and in patients with familial amyloidotic polyneuropathy of the Portuguese type. In nine patients systolic blood pressure fell by less than 15 mmHg, while in seven patients it fell by more than 15 mmHg. Plasma noradrenaline rose during tilt in the majority of patients, as in the controls. There was no correlation between levels of catecholamines and fall in blood pressure on head-up tilt. The data excludes widespread sympatho-neural failure as a cause for postural hypotension in familial amyloidotic polyneuropathy of the Portuguese type. The results are compatible with either segmental/patchy sympathetic denervation or dysfunction of the receptor/effector mechanisms in target organs such as the heart and blood vessels.
A 68-year-old man presented with a 6-month history of fatigue, rhinorrhoea, pruritic skin lesions, left pleural effusion, ascites, oedema and weight loss of 10 kg. Investigations revealed hepatosplenomegaly, retroperitoneal lymphadenopathy, anaemia, leucocytosis with eosinophilia, hypoprothrombinaemia, hypocholesterolaemia and elevation of both gamma glutamyltransferase and alkaline phosphatase. Biopsies of a skin lesion, bone marrow and liver revealed mast cell infiltration, allowing the diagnosis of systemic mastocytosis (SM). Hydroxyzine plus ranitidine were given without success. Hydroxyzine treatment was stopped, and ketotifen was initiated; substantial symptomatic improvement was observed within 8 d. This case report indicates the effectiveness of ketotifen in the symptomatic treatment of SM.
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