Biomedical subjects
Publications and source records attributed to R M Pereira.
Cardiac involvement in dermatomyositis has been well described; myocarditis and cardiac arrhythmias are the most frequent manifestations. An 8-year-old girl is presented, who developed pericardial tamponade in the course of the disease. It is the first time this association has been reported.
Although vasculitidis may involve several organs and tissues, ureteral involvement is rare. The present report describes a case of a female child with ureteral stenosis in whom a diagnosis of necrotizing vasculitis was made.
Antiganglioside antibodies (AGA) were determined in sera and cerebrospinal fluids (CSF) from 50 systemic lupus erythematosus (SLE) patients, and age-matched normal controls. The SLE patients were subdivided according to the type of clinical manifestation into two groups: neuropsychiatric SLE and active SLE without neuropsychiatric manifestation. The presence of these antibodies showed a significant correlation between IgG AGA in the CSF and IgM AGA in the serum and neuropsychiatric SLE. Fifteen patients had this antibody in the CSF without detectable levels in the serum. No correlation was seen between anticardiolipin antibodies in the serum of CSF and neuropsychiatric SLE. The present work suggests that antibodies against gangliosides may be a marker for neuropsychiatric SLE and that intrathecal antibody production can result in the development of this manifestation.
Traumatic arthritis resulting from self-aggression is rarely encountered in children. Differentiation from child abuse and common causes of childhood arthritis is difficult and rests upon a high level of suspicion. We describe a 10-year-old girl with hand deformities associated with joint pain and swelling managed as juvenile rheumatoid arthritis for 3 years. Reevaluation revealed both physical and radiographic evidence of recurrent trauma. Psychiatric assessment confirmed the diagnosis of autoaggression leading to self-mutilation and psychosocial rehabilitation was essential in successful management.
Fourteen children with adrenal cortical carcinoma were evaluated by ultrasonography. The neoplasms, 2.5 cm-19 cm in maximum diameter, were all well circumscribed. The four smaller lesions were either homogeneously hypoechoic (2 patients) or generally hyperechoic (2 patients). In the 10 patients with larger tumors, a complex predominantly echogenic pattern was demonstrated, eight of which contained radiating linear echoes, the "scar sign". This finding, although not specific, when present in a large adrenal mass, is suggestive of a cortical carcinoma. Associated findings of vascular invasion or retroperitoneal adenopathy supported the diagnosis of a malignant adrenal neoplasm in 3 patients.
Four children with benign intraperitoneal lipomatous tumors (mesenteric lipoma, mesenteric lipoblastoma, omental lipoblastoma and mesenteric mesenchymoma) are reported. The ultrasonographic findings of these unusual pediatric masses are described and correlated with their conventional radiologic and pathologic appearance. The pre-operative diagnosis of these entities can be suggested by these features.
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A high incidence of anticardiolipin antibodies were detected in 7 of 20 patients (35%) with Behçet's Syndrome. Three patients had IgG-ab, three had IgM-ab and one had both IgG and IgM antibodies. IgG-ACA was detected mainly in patients with ocular disease (30%) and one of them also has cerebral vascular disease. A lower incidence of ACA was found in the patients taking steroids compared with the ones taking other drugs. This work draws attention to the more severe disease present in patients with ACA and also the possibility of such tests become negative in patients taking immunosuppressive drugs.
Sonography was utilized to evaluate 12 patients with hypertrophy of the septum of Bertin. To confirm the diagnosis, angiography was used in 7 patients and radionuclide imaging in 2. Three sonographic characteristics were found: (1) an isoechogenic, ellipsoid mass with an echogenic linear rim of renal sinus fat; (2) contiguity of the mass with a normal-placed septum of Bertin; and (3) the occurrence of the mass effect always at the level of the emergence of the renal vein. The sonographic findings could be divided into two definite types. Type I showed moderate hypertrophy with a mass effect ranging from 1.1 by 2.3 cm to 2.0 by 3.2 cm in diameter, usually presenting with a smooth contour and discrete mass effect on the intravenous pyelogram (IVP). Type II showed severe hypertrophy with a mass effect ranging from 3.2 by 4.0 cm to 3.5 by 4.5 cm in diameter, usually presenting with a lobulated contour and irregular stretching of the calyceal system on IVP. The differential diagnosis is discussed. In conclusion, we found that sonography associated with excretory urography is an accurate method for the diagnosis of hypertrophy of the septum of Bertin.
The authors report three patients with subcutaneous erythematous nodules in different phases of development, unspecific systemic symptoms, positive PPD test, and normal chest X-rays. The histopathological study of the older nodules showed a granulomatous arteritis with a few acid-fast bacilli in the vascular wall. The nodules at an early phase showed an unspecific panniculitis with some acid-fast bacilli in apparently normal cutaneous vessels. These findings suggest that the mycobacterium has a vascular tropism and may cause a primary granulomatous arteritis.
An outbreak of American Cutaneous Leishmaniasis (ACL) occurring in the Northeast region of São Paulo state in 1992 is described. After the notification of the 12 human cases, a skin test survey of the local population was carried out in a rural area of the Itupeva municipality. The survey consisted of 144 interviews and 100 clinical examinations using the Montenegro skin test (MST). A prevalence of 34% positive MST was encountered. The predominant species of sandflies captured both in domestic and nearby areas of secondary vegetation were L. intermedia, L. whitmani and L. migonei. The presence of L. longipalpis in the nearby area of secondary vegetation was also registered.
Erythromelalgia is a rare disease characterized by intense erythema, burning pain and increased temperature in the distal of the extremities. Primary forms and secondary forms have been described, most commonly with essential thrombocythemia and policythemia vera. The authors describe a fifteen year old patient with primary erythromelalgia and discuss the pathogenic, clinical and therapeutic features of this disease.
Despite the high prevalence of psychic symptoms in lupus patients, there are few systematic studies in this area. Through a multidisciplinary approach, the authors developed a prospective study to characterize and correlate psychopathological aspects with clinical and laboratory data concerning neural manifestations of the disease. Out of 23 patients studied, 12 showed psychic alterations, which were interpreted as primary manifestations of the disease. All of them presented organic mental syndromes (DSM-III-R) in which cognitive symptoms were the most prominent, followed by affective, catatonic and hallucinatory features. The neurologic findings (seizure, migraine and muscular atrophy), as well as the ophthalmologic alterations (hemorrhage and soft exudates) were frequent and concomitant with the psychic features. The laboratory findings were: LE cells 50%; anti-Sm: 16%; anti-U1 RNP: 50%; anti-Ro/SS-A: 50%; anti-nDNA: 58%; decreased CH50 or fractions (C3, C4): 67%; anti-P: 18%; antigangliosides IgG: 67%; antigangliosides IgM: 78%. The cerebrospinal fluid analysis showed: increased cellularity: 18%; elevated protein: 36%; antigangliosides IgG: 67%; antigangliosides IgM: 33%; immunocomplexes: 36%. In spite of the absence of an adequate control group and of the small number of patients, the multidisciplinary approach leads to a better characterization of the nervous system involvement in this disease.
Neuropsychiatric alterations appear in 14-75% of lupic patients. Verified in 59% of the patients, psychiatric changes are the most frequent. Psychic symptoms are primarily related to the disease and secondary to uremia, hypertension, infection, and corticosteroids. Manifestations were also seen as a reaction to this chronic disease, which are potentially severe and causing many limitations to the patients. The authors review the literature considering the multidisciplinary aspects of this disease related to its pathogenesis, clinical features, diagnosis, differential diagnosis, and treatment.
A patient with systemic lupus erythematosus presenting abdominal pain, nausea, vomiting and severe mucocutaneous vasculitis had significant elevation of serum amylase and hepatic enzymes levels during a flare-up of the disease. Clinical and laboratory alterations disappeared after therapeutic increase of corticosteroids given to the patient a fact that suggested etiologic correlation between systemic lupus erythematosus, hepatitis and pancreatitis.
Central nervous system involvement in systemic lupus erythematosus is rather frequent whereas peripheral nervous system involvement is much less common. The three patients studied by us had isolated manifestations uncommon in nature. The first one developed a sensory-motor polineuropathy with signs of axonal degeneration. It responded to the therapeutic association of corticosteroids with an immunosuppressive agent. Satisfactory recovery took place over a time span of a year. The second patient had encephalic and cerebral trunk involvement from which an irreversible dementia resulted. The third patient, who had recurrent aseptic meningitis, is asymptomatic for six months now. Patients one and two had no systemic manifestations at the time of nervous system involvement. Suspicion of systemic lupus erythematosus was made on the basis of past inspecific articular symptoms. The neurological and systemic manifestations may be sometimes simultaneous; they are usually followed by serologic changes. Isolated nervous system involvement may be seen with and without sorologic changes, and there may be found antibodies reactive with phospholipids (anticardiolipin, antigangliosides and anticerebrosides). The employment of nonsteroid immunosuppressive drugs associated with corticosteroids in small doses seems to be useful in cases of systemic lupus erythematosus with nervous system involvement.