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S R Fernandes

Publications and source records attributed to S R Fernandes.

8 recordsLinked to original sources
PubMed2001

Ehlers-Danlos syndrome type IV and multiple aortic aneurysms--a case report.

Beside atherosclerosis, aortic aneurysms can be part of the clinical spectrum of many systemic diseases, including infectious, inflammatory, genetic and, less often, congenital disorders. A 48-year-old white man presented with multiple large aneurysms of the aorta and its main branches. Medical history was unremarkable except for the presence of a softened abdominal mass since he was 28 years old. On the physical examination, an arterial murmur was heard over the left carotid artery and a palpable mass was noted in the whole right side of the abdomen. No skin or joint abnormalities were noted. Aortography, computed tomography, and magnetic resonance angiography showed multiple large aneurysms of the descending thoracic and abdominal aorta. Aneurysms of the innominate, left subclavian, and carotid arteries were also seen. This case resembles those previously reported, in which multiple aortic aneurysms were associated with abnormalities of the type III procollagen gene (COL3A1). Although the classic stigmas of the Ehlers-Danlos syndrome type IV were lacking, this genetic disease may be the cause of the multiple aneurysms in this patient.

Aneurysm↗
PubMed2000

Weismann-Netter-Stuhl syndrome: first Brazilian case reports.

Weismann-Netter-Stuhl syndrome was first described in 1954 and is defined by an anterior curvature of the bones of the lower limbs, usually bilateral and symmetrical. Since its initial description, 82 cases were reported, including only 14 pediatric patients. The authors report two cases of this syndrome. One patient was an adult who presented with almost all the characteristic features of the disease. The second case was a 12-year-old girl who also presented with severe bone deformities of the upper limbs. Weismann-Netter-Stuhl syndrome is probably more common than previously reported and must be included in the differential diagnosis of rickets/osteomalacia, congenital syphilis and some cases of Paget's disease.

Bone Diseases, Developmental↗
PubMed2000

Sarcoidosis and systemic vasculitis.

BACKGROUND: Systemic vasculitis is an unusual complication of sarcoidosis. Over a 10-year period, the authors have provided care for six patients who had features of both sarcoidosis and vasculitis. Vasculitis could not be attributed to other causes. OBJECTIVES: To report six patients (five children) who had sarcoidosis and systemic vasculitis and compare our experience with previous literature. To better delineate the clinical spectrum of sarcoid vasculitis and its response to therapy. METHODS: Retrospective analysis and a Medline literature review of sarcoid and concurrent vasculitis from 1966. RESULTS: Our six patients had systemic illnesses that included fever, peripheral adenopathy, hilar adenopathy, rash, pulmonary parenchymal disease, musculoskeletal symptoms, and scleritis or iridocyclitis. Biopsies revealed features compatible with the diagnosis of sarcoidosis or necrotizing sarcoid granulomata in either skin, lymph node, lung, synovium, bone, bone marrow, liver, trachea, or sclera. Arteriography showed features of large vessel vasculitis in three patients, all of whom were African American, whereas patients with small vessel vasculitis were white. Prior reports of sarcoid and vasculitis included 14 adults, of whom half had predominantly small vessel disease, and half had medium- or large-sized vessel disease. Eight previously reported children included seven with primarily large vessel sarcoid vasculitis. Racial background was noted in 15 reported cases and included whites (6), African Americans (5), and Asians (4). Among the authors' six patients, four improved when treated with prednisone alone. However, relapses occurred when the drug was tapered or withdrawn. CONCLUSIONS: Sarcoidosis may be complicated by systemic vasculitis that can affect small- to large-caliber vessels. Sarcoid vasculitis can mimic hypersensitivity vasculitis, polyarteritis nodosa, microscopic polyangiitis, or Takayasu's arteritis. African American and Asian patients are disproportionately represented among cases with large vessel involvement. Corticosteroid and cytotoxic therapy is palliative for all forms of sarcoid vasculitis. However, relapses and morbidity from disease and treatment is common.

Adolescent↗
PubMed1999

Polyarteritis nodosa and cytomegalovirus: diagnosis by polymerase chain reaction.

We investigated the occurrence of an active cytomegalovirus (CMV) infection in patients with polyarteritis nodosa (PAN). Eleven patients with PAN were screened for the presence of CMV-DNA in their blood using the polymerase chain reaction (PCR). Serum anti-CMV IgG and anti-CMV IgM antibodies were determined by enzyme-linked immunosorbent assays (ELISA). The ELISA for IgM was negative in all cases whereas that for IgG was positive in eight cases. Only one patient was positive for CMV-DNA by PCR. He presented with myalgia, polyarthralgia, fever and weight loss, suggesting PAN activity. CMV infection was uncommon in our series of patients with PAN, despite disease activity and immunosuppressor therapy. The finding of a transient CMV infection in one case at the beginning of PAN activity suggests that CMV may be involved in the pathogenesis of PAN.

Adolescent↗
PubMed1998

Uncommon features of polyarteritis nodosa: psychosis and angio-oedema.

Psychosis and swelling of the face and hands are rarely observed in adult polyarteritis nodosa (PAN). We describe a 21-year-old woman who presented with fever, livedo reticularis, tender subcutaneous nodules and arthritis. These manifestations did not respond to prednisone, but remitted when the drug was tapered. She had had psychosis since the age of 16 years. During the flares of the disease she presented with facial, periorbital and hand swelling. This finding is rarely observed in adult PAN. Arteriography showed multiple small aneurysms, of the mesenteric vessels consistent with a diagnosis of PAN. Our report discusses the diagnosis of PAN and emphasises the uncommon presentation of this case.

Adult↗
PubMed1994

99mTc labeling white blood cells with a simple technique: clinical application.

Several radionuclides and different methods have been employed as cellular labels to study inflammatory sites in man. Here we present the results obtained with white blood cells (WBC) labeled with 99mTc using a simple and low cost new technique (SnTec). WBC were incubated with 12 micrograms/ml of stannous chloride for 10 min at room temperature. Then 99mTc was added. After 10 min, the 99mTc-labeled WBC were washed and injected into the patient. Comparison studies with 99mTc-labeled WBC using the HMPAO technique were carried out in patients with suspected osteomyelitis. Since the results are similar with both methods, we suggest the use of SnTec to label WBC, in cases of inflammatory diseases.

Adolescent↗