Biomedical subjects
Publications and source records attributed to R A Frayha.
Three of 59 patients with systemic lupus erythematosus developed a total of eight Salmonella typhimurium bacilluria over five years of study. Lupus was the commonest disease entity associated with this urinary infection.
A child is reported with Behcet's disease who presented with skin and joint manifestations, oral ulcers, brainstem syndrome and neurogenic muscular atrophy. The neurogenic muscular atrophy was confirmed by electrophysiologic, histologic and histochemical studies. Electron microscopy of muscle showed a vasculopathy. The possible etiology of the muscle lesions is discussed. The relevant literature on muscle and peripheral nerve involvement in Behcet's disease is reviewed. It is proposed that neuromyopathy be added to the neurological manifestations of Behcet's disease. To our knowledge, this is the first case of neurogenic atrophy reported in Behcet's disease.
A young female with mixed connective tissue disease (MCTD) presented with the unusual occurrence of abdominal pseudolymphoma. Although the pseudolymphoma could be related to concomitant sicca complex, some data suggest that MCTD, as such, could carry a high incidence of malignancy.
A child with clinicopathological features of eosinophilic fasciitis terminated in progressive systemic sclerosis. The clinical and laboratory similarities and disparities between these two conditions are presented.
The case of a 23-year-old woman with systemic lupus erythematosus and homonymous hemianopia is presented. A calcarine cortical infarct occurred as a result of a postpartal exacerbation of her disease and was demonstrated by CT scanning of the brain. The neuro-ophthalmic manifestations of SLE are reviewed briefly. The authors propose to include collagen vascular disease in the differential diagnosis of homonymous hemianopia in the young age group.
Vesical bleeding and sicca complex proved to be due to unrecognized amyloidosis in a patient with chronic rheumatoid arthritis. The features of amyloidosis of the lower genitourinary tract are outlined.
Symptomatic hypocalcaemia was the dominant feature of acute colchicine poisoning in a young female with periodic polyserositis. Experimental data suggest that colchicine-induced hypocalcaemia is secondary to colchicine inhibition of bone resorption.
A patient with papular mucinosis (scleromyxedema) developed an erosive seronegative rheumatoid-like arthropathy, carpal tunnel syndrome, sicca complex, and marked increase in TG(OKT8) suppressor/cytotoxic circulating T-cells akin to that reported in scleroderma. Sclerodactyly, acrolysis and stiff digits were striking but other features of scleroderma, i.e., Raynaud's and esophageal hypoperistalsis, were absent. The diagnosis of papular mucinosis, a pseudoscleroderma syndrome, should be considered in a patient with atypical arthritis, median neuropathy, myopathy, and/or sclerodactyly and a papular lichenoid dermatopathy. Skin mucin stain and the demonstration of the distinct serum paraprotein (PM-spike) are confirmatory. We stress the salient diagnostic clinical features of the leonine-like facies of papular mucinosis.
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During an outbreak of trichinosis, two young men--one with established trichinosis and the other with suspected infection--were found to have clinical, radiologic and histologic stigmata of a systemic necrotizing vasculopathy equivalent to classic polyarteritis nodosa. The parasitosis manifested as a pentad of fever, myalgias, facial edema, eosinophilia and hyperimmunoglobulinemia E. Features of the arteritis included mononeuritis multiplex, pain in the abdomen and joints, weight loss, hypertension, leukocytosis, thrombocytosis, microhematuria and raised alkaline phosphatase levels. A sustained remission was achieved by the administration of thiabendazole, prednisone and cyclophosphamide. Pathogenetic links between the two diseases are presented: (1) deposition of circulating immune complexes in the vessel wall; (2) adjuvant activity with cross reaction between parasitic antigen and human vessel wall; (3) immunoglobulin E (IgE) aggregates and soluble antigen IgE complexes precipitation in vessel wall; and (4) hypereosinophilia-induced tissue damage. A causal relationship of trichinosis to polyarteritis nodosa is persuasive, and we suggest that cases of hepatitis B surface antigen (HBsAg) negative polyarteritis nodosa, especially those in which myalgias and eosinophilia are prominent, may be related to trichinosis and that, conversely, patients with trichinosis and multiorgan disease should be studied for polyarteritis nodosa.
The myopathology of Behçet's disease is described in 7 patients using conventional light microscopy, histochemistry, high-resolution light microscopy and electron microscopy. Two patients had clinical evidence of peripheral neuropathy: 5 patients had no clinical evidence of neuromuscular involvement. Histochemical profile of muscle in all patients was normal. Abnormalities in muscle histology using conventional light microscopy were found in only 1 patient. High-resolution light microscopy revealed abnormalities in 3 patients whereas electron microscopy revealed abnormalities in muscle of all 7 patients. The myopathology of Behçet's disease includes thickening of capillary basement membrane, excessive pleating of sarcolemma, subsarcolemmal aggregates of mitochondria and glycogen, disorganization and breakdown of myofibrils, central nucleation and the presence of a variety of cytoplasmic inclusions. No virus particles were present.
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Ten patients with the diagnosis of systemic sclerosis developed cranial nerve involvement. A trigeminal sensory neuropathy evolved insidiously in all patients and in five of these it was a presenting complaint. The glossopharyngeal nerve was involved in one patient. Taste was impaired in one patient and a unilateral loss of taste with fasciculations of the tongue were noted in another. Tinnitus was a complaint in three patients, two of whom had bilateral impairment of hearing. Facial weakness was noted in five patients. In three, this weakness was bilateral, while in the others the weakness was unilateral, and a past history of acute onset was obtained. The microangiopathy of systemic sclerosis is felt to be primarily responsible for these neurological deficits. The deposition of fibrous tissue may be a secondary phenomenon and contribute to the process by compression of nerves.
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Hematological abnormalities in scleroderma indicate a specific complication of the disease itself, or an associated illness. Among 180 patients with scleroderma, anemia was detected in 25% and was attributed to chronic inflammatory disease (usually an overlap syndrome), bleeding mucosal telangiectases as part of the CREST syndrome, intestinal malabsorption, and microangiopathic hemolysis. Leukocytosis, present in 14%, was correlated with active myopathy and/or advanced visceral involvement while leukopenia was suggestive of a crossover with systemic lupus erythematosus. Thrombocytopenia was often a manifestation of microangiopathy, and thrombocytosis was associated with an arteritis or a tumor syndrome.