Usefulness of tissue Doppler on early detection of cardiac disease in Fabry patients and potential role of enzyme replacement therapy (ERT) for avoiding progression of disease.

Aims: Cardiac involvement, including progressive cardiomyopathy, is common in Fabry disease and is a leading cause of premature mortality. We sought to determine if tissue Doppler imaging (TDI) could identify Fabry disease patients at risk for the development of cardiomyopathy and if enzyme replacement therapy (ERT) with agalsidase alfa might slow or prevent the progression of cardiac involvement.

Methods And Results: Fabry disease patients were enrolled in this prospective, observational study.

Chronic aortic dissection as a cause of fever of unknown origin.

Chronic aortic dissection presenting as a prolonged febrile syndrome is an uncommon condition. We believe that only 22 cases have been previously reported. We present a case of a patient with an aortic dissection whose diagnosis was delayed because persistent fever, malaise, and night sweats dominated his clinical picture.

Fever of unknown origin: classic and associated with human immunodeficiency virus infection. a comparative study.

Fever of unknown origin (FUO) associated with HIV infection is different from classic FUO. Relevant etiologies, procedures and time to diagnosis were analyzed. Patients admitted with FUO from 1991 to 1996 were prospectively followed.

Relationship of basal and postprandial intraduodenal bile acid concentrations and plasma cholecystokinin levels with abdominal pain in patients with chronic pancreatitis.

Abdominal pain in patients with chronic pancreatitis has been related to an increase in plasma cholecystokinin (CCK) levels. The aim of the study was to disclose the relation of the altered response with the low intraduodenal bile acids levels found in these patients. Twenty patients with chronic pancreatitis were classified into groups I (n = 11) and II (n = 9) according to the presence or absence of pain.

Bilirubinate granules: main pathologic bile component in patients with idiopathic acute pancreatitis.

Objective: The aim of this study was to evaluate the main pathologic component of bile obtained by biliary drainage in patients with acute idiopathic pancreatitis and therapeutic implications.

Method: Eighteen patients diagnosed with idiopathic acute pancreatitis underwent biliary drainage. Microscopic evaluation of bile was performed and pathologic components were classified in cholesterol microcrystals, bilirubinate granules, and calcium microspherolites.

Systemic polyarteritis nodosa as the initial manifestation of a gastric adenocarcinoma.

We report a case of systemic polyarteritis nodosa (PAN) leading to the discovery of an as yet asymptomatic, surgically curable gastric adenocarcinoma. PAN is rarely associated with malignancies and in such cases these are more often malignant haematological diseases than solid neoplasms. The immunopathological findings, the temporal relationship between both conditions, and the spontaneous resolution of vasculitis after tumour removal suggest a paraneoplastic origin of the systemic angitis.

Two cases of laryngeal leishmaniasis in patients infected with HIV.

A high incidence of visceral leishmaniasis has been documented in HIV-infected patients in endemic areas. In these patients, atypical locations and a chronic course of the disease are more frequent. Two AIDS patients with laryngeal leishmaniasis are reported.

[Terminal ileitis due to cryptosporidium and cytomegalovirus in a patient with AIDS].

Terminal patients with infection by the Human Immunodeficiency Virus (HIV), gastrointestinal affection is frequently observed, caused by many etiological agents. However, the affection of the terminal ileon is and generally associated to cytomegalovirus (CMV), intracellular Mycobacterium avium (IMA) and Mycobacterium tuberculosis. We present a patient diagnosed of AIDS with clinical signs of chronic diarrhea secondary to terminal ileitis by Cryptosporidium and CMV.

Evidence of hepatitis C virus antibodies in the cryoprecipitate of patients with mixed cryoglobulinemia.

Objective: To describe the clinical features of 8 patients with mixed cryoglobulinemia and hepatitis C virus (HCV) infection.

Methods: A clinical study of the patients was performed. Anti-HCV antibodies were determined by ELISA and confirmed by immunoblot (RIBA) in the sera and in the cryoprecipitate.

Thrombotic thrombocytopenic purpura associated with human immunodeficiency virus infection: demonstration of p24 antigen in endothelial cells.

We report a case of thrombotic thrombocytopenic purpura (TTP) in a woman infected with human immunodeficiency virus (HIV) in whose endothelial cells we detected HIV p24 antigen. The patient had an excellent response to conventional therapy for TTP and remained in complete remission 12 months after diagnosis. We also present a review of the literature on the association of TTP with HIV infection and speculate on its nature.

Still's disease in a 72-year-old man.

Adult onset Still's disease is an uncommon clinical entity, usually found in young adults. Occasionally it can be seen in patients over 55 years old. Our clinical report illustrates that Still's disease may occur even in a 72-year-old man.

Osteoarticular infection in intravenous drug abusers: influence of HIV infection and differences with non drug abusers.

Objectives: To determine (a) the influence of HIV in developing osteoarticular infections in intravenous drug abusers (IVDAs) and (b) the differences between the clinical features of osteoarticular infections in IVDAs and a control group of non-IVDAs.

Methods: A comparative study of the clinical features of osteoarticular infections in all HIV positive and HIV negative IVDAs admitted to the departments of rheumatology and internal medicine during a 10 year period was carried out. The joint infections of all IVDAs, irrespective of HIV status, were compared with those of a control group of non-IVDAs lacking risk factors for HIV infection.

Pyrexia of unknown origin: changing spectrum of diseases in two consecutive series.

Comparison was made of the aetiology and methods of diagnosis in two series of patients meeting the classic criteria of pyrexia of unknown origin during 1968-1981 and during 1982-1989 seen in the Department of Internal Medicine at La Paz University Hospital, Madrid, Spain. There was a statistically significant decrease in the percentage of infections and an increase in neoplasms and connective tissue disorders in the second series. The percentage of patients diagnosed by laparatomy was similar in both series but the diagnosis yield at laparotomy was greater in the second period.

[Dermatomyositis, Sjögren's syndrome and vasculitis: an infrequent overlapping connective disease].

A dermatomyositis case with Sjögren's syndrome, Leucocytoclastic vasculitis, and sclerodermiform features is presented. This is a peculiar over lapping connectivepathy in which clinical features of three multisystemic diseases are together. The association of dermatomyositis with Sjögren's syndrome is very rare.

Temporal arteritis: a form of systemic panarteritis.

The case is reported of a patient with giant cell arteritis affecting several organs. The triggering cause of death was a brainstem infarction due to basilar artery thrombosis. The necropsy showed the systemic character of the disease affecting the coronary, bronchial, and ovarian arteries.