Rendu-Osler-Weber disease and intracranial hemorrhage during pregnancy. A case report

A 24-year-old woman with Rendu-Osler-Weber disease presented an intracranial hematoma during pregnancy. The main localizations of the Rendu-Osler-Weber disease, an inborn disease, are discussed. Neurological complications are rare. Vascular malformations are more fragile during pregnancy. The obstetrical attitude depends on the organ involved and the severity of the complications.     

Angiographic findings in hereditary hemorrhagic teleangiectasia: a case study of Rendu-Osler-Weber disease

A case of Rendu-Osler-Weber disease in 48 year old male was described. Selective arteriography of superior mesenteric artery revealed its widening (including intestine branches), numerous anastomoses in the form of microhaemangioma and fast venous return with broad superior mesenteric and portal vein.     

Thrombocytosis and coronary disease. Report of a case

We report the case of a young male with an inferior myocardial infarction who was treated with fibrinolytic agents and displayed a good evolution. The only cardiovascular risk factor that this patient had was an idiopathic thrombocytosis with abnormal platelet hyperreactivity tests. The angiogram showed an eccentric lesion of 60% in the left main coronary artery. A week later, after treatment based on anticoagulants and antithrombotic agents the angiogram was normal, and the lesion had disappeared. The association between these conditions is discussed the therapeutic approach is also discussed. A review of the literature is conducted.     

Coronary aneurysm in Behcet''s disease. Report of a case

Polymyositis, dermatomyositis, and inclusion body myositis, although immunopathologically distinct, share 3 dominant histological features: inflammation, fibrosis, and loss of muscle fibers. Progress in molecular immunology and immunogenetics has enhanced our understanding of these cellular processes. Based on the T-cell receptor gene rearrangement, the autoinvasive CD8+ T cells in polymyositis and inclusion body myositis, but not dermatomyositis, are specifically selected and clonally expanded in situ by heretofore unknown muscle-specific autoantigens. The messenger RNA of cytokines is variably expressed, except for a persistent up-regulation of interleukin 1beta in inclusion body myositis and transforming growth factor beta in dermatomyositis. In inclusion body myositis, the interleukin 1, secreted by the chronically activated endomysial inflammatory cells, may participate in the formation of amyloid because it up-regulates beta-amyloid precursor protein (beta-APP) gene expression and beta-APP promoter and colocalizes with beta-APP within the vacuolated muscle fibers. In dermatomyositis, transforming growth factor beta is overexpressed in the perimysial connective tissue but is down-regulated after successful immunotherapy and reduction of inflammation and fibrosis. The degenerating muscle fibers express several antiapoptotic molecules, such as Bcl-2, and resist apoptosis-mediated cell death. In myositis, several of the identified molecules and adhesion receptors play a role in the process of inflammation, fibrosis, and muscle fiber loss, and could be targets for the design of semispecific therapeutic interventions.     

Retroperitoneal Castleman's disease. Presentation of a clinical case

Castelman disease (CD) is a rare lymphadenopathy that usually presents as a solitary, slow growing mass; its etiology is still unknown. The authors described a case of CD localized in the retro-pancreatic space. A 33-year old patient, female, underwent abdominal ultrasonography because of upper right abdominal pain. The ultrasound showed gallbladder stones and a mass of 4 cm in diameter behind the pancreatic head, modifying the shape of the inferior vena cava. The patient underwent surgical excision and histological examination showed a hyaline type of CD. In the 70% of the cases, the disease is located in the mediastinum, only in 14% of the cases in the retroperitoneal space. The case we report is a hyaline type of CD that is the most frequent histological type, constitutes 90% of the cases, occurs usually in the young population and after surgical excision has a very good prognosis. Different is the clinical behaviour of the plasma cell type of CD that has an aggressive and often fatal clinical outcome. The patient we treated is still free of any symptoms one year after the operation.     

Immunoproliferative disease of the small intestine. Report of a case

We report one case of immunoproliferative small intestinal disease with two rare characteristics. Firstly, the detection of monoclonal IgA-Kappa in serum and in the intestinal infiltrate and secondly, the advanced age of the patient at diagnosis. We checked up on Spanish literature and found an important number of patients that were diagnosed at such an age. We suggest that this disease may appear in elderly people in developed countries.     

Retroperitoneal ganglioneuroma. Report of a case

OBJECTIVE: To report an additional case of retroperitoneal ganglioneuroma. METHODS/RESULTS: A case of retroperitoneal ganglioneuroma that had been incidentally discovered in a 27-year-old male during abdominal US evaluation is described. The diagnosis was based on the histopathological findings after US-guided biopsy. The clinical features and the findings of the complementary tests, which included radiological assessment, intravenous urography, CT and cavography are presented, as well as the pathology findings. Treatment was by complete surgical excision of the tumor. CONCLUSION: Treatment of ganglioneuroma is by surgery, since the diagnosis is generally based on the histopathological analysis of the surgical specimen. For those cases with a preoperative diagnosis, some authors advocate surgery for patients with clinical evidence of neuroblastoma or another pathology arising from this disease. Although infrequent, ganglioneuroma coexisting with neuroblastoma can occur. For this reason, complete excision of the tumor is preferred.     

Spinal cord compression as initial manifestation of Hodgkin's disease. Report of a case

A case of Hodgkin's disease in which the first clinical manifestation was a radiculo-spinal compression is reported. The authors comment about the possible mechanisms to explain this radiculo-spinal compression in this granulomatosis.     

Fahr's disease and hypocalcemic syndromes. Presentation of a clinical case]

A clinical case of a man with calcification of basal ganglions (CBG) is presented. The first manifestations of this syndrome were loss of consciousness and convulsions. The potential causes of his hypocalcemia, Fahr's syndrome, myocardiopathy of hypoparathyroidism and hypocalcemia are reviewed and a study oriented to autoimmune polyendocrinopathy is performed.     

Renal endometriosis. Report of a case

In the light of a personal case and a review of the literature, the authors recall the specific features of renal endometriosis. This rare lesion, with a favourable prognosis, is difficult to diagnose and is rarely diagnosed preoperatively. Medical treatment by LHRH agonists is discussed and has been used exceptionally.     

Sertoli cell tumor of the testis: clinical pathologic varieties. Report of a case

Tumours derived from sex cords and primitive gonadal stroma account for 4% of total testicular tumours. The low frequency of Sertoli's cells tumour (SCT) and the uneven study and follow-up of patients makes analysis of this tumoral entity difficult. This paper contributes one case report of a Sertoli's giant cell tumour calcified in a 13-year old patient, and reviews the clinical aspects, clinico-pathological varieties believed to require assessment in patients with this type of disease. This type of tumour is considered benign in its biological behaviour, although some malignant forms have also been described. SCT is actually an heterogeneous tumoral pathogenic entity with regard to pathogenic and prognostic aspects. Our final conclusions show that the clinico-pathological variety, age, size and associated clinical manifestations appear to be related to the prognosis.     

Thoracic epidural angiolipoma. Report of a case

Angiolipomas of the spine are rare (40 cases in the literature). When they are intraspinal, these masses are epidural in more than 90% of the cases. Angiolipomas are benign tumors containing vascular and mature adipose elements. The clinical symptomatology is non specific, but computed tomography and mostly MRI provide a precise diagnosis. With one additional case and a review of the literature, we define the main characteristics of these tumors.     

Essential iris atrophy. Report of a case

The clinicopathologic findings in an eye from a 40-year-old man with essential iris atrophy are reported. Noteworthy is the proliferation of corneal endothelium and a laying down of a new Descemet (cuticular) membrane over the trabecular meshwork and in areas of anterior peripheral synechiae formation over the pseudoangle. The histologic characteristics favor a vascular cause for the condition, but the evidence is circumstantial.