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Biomedical subjects

F García Bragado

Publications and source records attributed to F García Bragado.

At least 19 recordsLinked to original sources

Postencephalitic chronic granulomatous disease.

This report details the evolution of a case of herpes simplex encephalitis to chronic granuloma in a 13-year old female who, at the age of 8, suffered herpes simplex virus type 1 encephalitis. Eight months later, she developed an intracranial hypertension syndrome with the onset of a new lesion in the necrosed zone of her right temporal lobe, with no viral presence in the cerebrospinal fluid. The histologic characteristics were those of chronic granuloma with multinucleated giant cells and calcifications. Initially treated for neurosarcoidosis, the patient remained steroid-dependent for 4 years and the steroids could only be withdrawn by treatment with Infliximab. This type of evolutive pattern has probably been completely overlooked until now; it can only be accurately diagnosed by biopsy. It was initially mistaken for neurosarcoidosis.

Adolescent↗

[Citrobacter freundii endocarditis].

Infection by Citrobacter appears in man only in certain circumstances, since it usually acts as contaminant or colonizer. Bacteraemia by this bacillus can affect immunodeficient people, elderly people or those patients who have undergone invasive hospital processes. Although incidence of bacteraemia is low (0.3-0.9%), the death rate is very high, about 48%. This bacillus is seldom the cause of endocarditis. That is why we describe a case of endocarditis by Citrobacter freundii, in an aged person with previous valvulopathy.

Aged↗

Limb-girdle muscular dystrophy in Guipúzcoa (Basque Country, Spain).

The concept of limb-girdle muscular dystrophy (LGMD) is changing rapidly due to the advances in molecular genetics. Recently, seven different gene loci have been described, demonstrating that limb-girdle muscular dystrophy is a heterogeneous syndrome, which includes different diseases with a similar phenotype. In isolated populations which have little genetic exchange with neighbouring populations, an accumulation of cases may be found. We carried out an epidemiological study in Guipúzcoa, a small mountainous Basque province in northern Spain, and found the highest prevalence rate of LGMD described so far: 69 per million. Genetic studies demonstrated that 38 cases corresponded to the LGMD2A type, due to calpain-3 gene mutations. Only one patient with alpha-sarcoglycanopathy was found, and in 12 patients the genetic defect was not identified. Moreover, the particular calpain-3 mutation predominant in Basque chromosomes (exon 22, 2362AG-->TCATCT), has only been rarely found in the rest of the world. This observation strongly suggests a founder effect in the indigenous population of Guipúzcoa. The clinical characteristics of the patients with calpain-3 gene mutations were quite homogeneous and different from the other groups (sarcoglycanopathy and unknown gene defect), allowing for a precise clinical diagnostic. The disease onset was between the ages of 8 and 15 years, in most cases in the pelvic girdle, and the patients became wheelchair-bound between 11 and 28 years after onset. No pseudohypertrophy of calves or contractures were observed. No clear correlations were found between the nature and site of the mutation and the resulting phenotype.

Adult↗

Prognostic value of high serum levels of CA-125 in malignant secretory peritoneal mesotheliomas affecting young women. A case report with differential diagnosis and review of the literature.

AIMS: Very few cases of diffuse, malignant, peritoneal mesothelioma have been reported in young women. Distinction between peritoneal mesothelioma and serous epithelial tumours, including papillary serous carcinomas and borderline serous tumours, can be difficult. Differential diagnosis based on clinical appearance and imaging techniques is broad and inconclusive, thus the diagnosis must be confirmed by histological examination. Because the vast majority of tumours involving the peritoneal and serosal surfaces are due to primary or metastatic serous epithelial tumours, there is a tendency on part of pathologists to disregard the possibility of mesothelioma when examining a biopsy or excision specimen. This is especially likely to occur when mesothelioma is associated with highly elevated serum levels of CA-125, which is the typical tumoral marker of epithelial serous tumours from the ovary. The association between peritoneal mesothelioma and high serum levels of CA-125 has been reported in the literature only in two cases. CASE DETAILS: In order to avoid a misdiagnosis of this neoplasm we describe a new case of peritoneal mesothelioma in an 18-year-old woman with high serum levels of CA-125. CONCLUSIONS: Besides its clinicopathological characteristics and its histological, immunohistochemical and ultrastructural features, we describe its biological behaviour, which seems to be worst when CA-125 levels are high.

Adolescent↗

Pulmonary hemorrhage and antiglomerular basement membrane antibody-mediated glomerulonephritis after exposure to smoked cocaine (crack): a case report and review of the literature.

A case of Goodpasture's syndrome with a negative immunofluorescence examination of the lung biopsy in a 32-year-old man is described. The patient was a 40 cigarettes per day smoker, who had been smoking cocaine (crack) up to 3 weeks before hospital admission. He developed a diffuse alveolar hemorrhage with extremely acute respiratory distress, followed by renal failure with anuria. Transjugular renal biopsy, immunofluorescence and serum antiglomerular basement membrane antibody titer studies confirmed the diagnosis of Goodpasture's syndrome without linear immunoglobulin G deposits as determined by immunofluorescence examination of the alveolar basement membranes. The case illustrates the potentially complex interrelations between an autoimmune disease and exposure to substances with possible antigenic properties, besides the imperative necessity for an early, accurate diagnosis and treatment for the potential for threatening life. Moreover, the association of Goodpasture's syndrome with crack has not been previously reported.

Adult↗

Jejunal stromal tumor with skeinoid fibers or myenteric plexoma: a case report.

Small intestinal stromal tumors with 'skeinoid fibers' are uncommon stromal tumors with an associated controversial histogenesis. Although their microscopic appearance is suggestive of a smooth muscle nature, they lack specific smooth muscle features, as evident by electron microscopy and immunohistochemistry. They also appear to lack features of neurogenic origin because they fall to react with neural/neuroendocrine markers such as S-100 protein, neuron-specific enolase and chromogranin. It is interesting, nonetheless, to note that the ultrastructural examination of these tumors may show structures reminiscent of neural differentiation, such as cytoplasmic projections, containing occasional membrane-bound, dense-core, neurosecretory-type granules, which mimick the long cytoplasmic processes seen in tumors of neural origin. Moreover, the association of these tumors with Von Recklinghausen's neurofibromatosis, as well as the presence of 'skeinoid fibers' in proven neurogenic spindle cell neoplasms such as gastrointestinal autonomic nerve tumors and schwannomas, suggests that these tumors might also be neurogenic in origin and enhances the diagnostic value of 'skeinoid fibers' as a possible ultrastructural marker of neural differentiation. Thus, light microscopic evaluation is clearly insufficient to accurately diagnose these tumors and to determine their histogenesis, electron microscopic and immunohistochemical studies being necessary. In this article the histogenesis of small intestinal stromal tumors with 'skeinoid fibers', regarding a jejunal neoplasm in a 63-year-old patient, is reviewed. The light microscopic, immunohistochemical and ultrastructural features are described and compared with findings usually seen in all those stromal tumors which may raise a differential diagnosis, such as smooth muscle stromal tumors, gastrointestinal autonomic nerve tumors, schwannomas, paragangliomas and fibrosarcomas.

Actins↗

[HELLP syndrome: case report and review of the literature].

The case of a 32-years-old primipara in her thirty-second week of pregnancy who presented acute epigastric pain, citopenia, elevation in hepatic enzymes and analytical signs of hemolysis is presented. Ultrasonography demonstrated multiple hyperechogenic nodular images in the liver leading to a wide differential diagnosis. Liver biopsy findings, together with the mentioned clinico-biological alterations were indicative of the HELLP syndrome. Following caesarean, the patient presented rapid and total, symptomatic and analytical, improvement. Based on this case, a review of this entity in the literature and its prognostic implications and possible therapeutic approaches are reported.

Adult↗

[Diffuse pulmonary hemorrhage: review of nine cases].

Nine cases of diffuse pulmonary hemorrhage (DPH) diagnosed in our hospital during the past eight years are reviewed. We assess the clinical, diagnostic, etiologic and evolutive characteristics of all these cases and, thus, of such entity in our environment. The nine patients had anemia, hemoptysis and transient pulmonary infiltrations. Renal affectation was observed in seven patients. Based on clinical and laboratory data, supplementary explorations and immunological and histological studies, the following etiologic diagnosis were established: idiopathic extracapillary glomerulonephritis (three cases), idiopathic pulmonary hemosiderosis (two cases), Wegener's granulomatosis (one case), unclassifiable systemic necrosant vasculitis or overlapping syndrome (two cases). In one patient, it was not possible to establish the etiology. Global mortality was 44.4% (four patients); two of them died during the course of the initial massive hemoptysis (not controlled, as in the other patients, with steroids) and two other patients died due to late complications. In this series, none of the patients developed a disease associated to antibodies against the basal membrane. The application of a severe diagnostic systematic has been a key factor for the management of these patients.

Adolescent↗

[Atraumatic rupture of the spleen. A proposal of new attitudes. Apropos a case in brucellosis].

A case of spontaneous spleen rupture during acute brucellosis, followed by clinical and ultrasound examinations until the natural resolution of the subcapsular hematoma, is presented. The causes of this complication are reviewed and a new classification is proposed, creating 3 groups of nontraumatic ruptures: spontaneous, with basal splenic disease and associated to other pathology. We highlight the several states of spleen ruptures--some with spontaneous resolution--and the utility of the radiological techniques to carry out a follow-up on them, which can in some cases help avoid surgical intervention.

Adult↗

[Pulmonary hemorrhage and glomerulonephritis associated with essential mixed cryoglobulinemia].

A 49-year-old male with essential mixed cryoglobulinemia IgM kappa-IgG of 4-years duration was treated with corticosteroids and immunosuppressive drugs during the repeated episodes. Finally, he developed a new episode with severe renal involvement and pulmonary hemorrhage without other associated etiological factors. The optical and immunofluorescent histological findings in lung and kidney are described. This is the second case of pulmonary hemorrhage and cryoglobulinemia associated with essential mixed cryoglobulinemia reported in the literature. A relation between essential mixed cryoglobulinemia and glomerulonephritis and pulmonary hemorrhage is suggested.

Cryoglobulinemia↗