1/4. Steroid-responsive pleuropericarditis and livedo reticularis in an unusual case of adult-onset primary hyperoxaluria.We present a case of a 54-year-old woman with rapidly progressive renal failure of uncertain origin, who developed pleuropericarditis and livedo reticularis 6 weeks after initiation of hemodialysis (HD). The presentation with acute renal failure, the development of serositis, and the dramatic clinical response to empiric steroid therapy initially suggested the diagnosis of a systemic inflammatory disorder or vasculitis. Renal biopsy, performed 3 days after presentation, suggested crystal deposition disease, and subsequent investigations, using both dialysate oxalate concentrations and liver biopsy, led to the diagnosis of primary hyperoxaluria (PH). We discuss this atypical adult presentation of PH and propose a role for the use of steroids in the management of the acute inflammatory symptoms of oxalosis. We also briefly discuss the current medical management of patients with PH, including transplantation.- - - - - - - - - - ranking = 1keywords = adult-onset (Clic here for more details about this article) |
2/4. Cases from the Osler Medical Service at Johns Hopkins University.PRESENTING FEATURES: An 18-year-old white man was admitted to the Osler Medical Service with the chief complaint of back pain. Two weeks prior to admission, the patient developed diffuse and aching upper back pain. Over the next couple of days, he also developed severe anterior chest pain that was somewhat pleuritic in nature but diffuse and extending bilaterally into the shoulders. One week prior to admission, he developed intermittent fevers and night sweats. The patient denied any lymphadenopathy, pharyngitis, sick contacts, shortness of breath, rash, or bleeding. He was seen by a physician and told that he had thrombocytopenia. There was no history of recent or remote unusual bleeding episodes. His medical history was unremarkable except for a childhood diagnosis of attention deficit/hyperactivity disorder. He was not taking any medications and had no history of tobacco, alcohol, or illicit drug use. He had no risk factors for human immunodeficiency virus infection. physical examination showed that he was afebrile and had normal vital signs. He was a well-appearing man who was lying still because of pain. HEENT examination was unremarkable. There was no pharyngeal erythema or exudates. His lungs were clear. His neck was supple and without lymphadenopathy. Examination of his back and chest revealed no focal tenderness. There was no hepatosplenomegaly, and his skin was without petechiae or rashes. Examination of the patient's joints showed pain on passive and active movement of his shoulders bilaterally, but no frank arthritis. There was no rash, petechiae, or echymoses. Chest radiograph and electrocardiogram were unremarkable. On admission, the laboratory examination was notable for a hematocrit level of 32.5%, with a mean corpuscular volume of 79 fL, and white blood cell count of 2.8 x 10(3)/microL. platelet count was 75 x 10(3)/microL. A white blood cell differential revealed 7% bands, 53% polys, 34% lymphs, 5% atypical lymphocytes, 2% nucleated red cells, and a few young unidentified cells. His chemistry studies were unremarkable. What is the diagnosis?- - - - - - - - - - ranking = 3.7954927488685keywords = still (Clic here for more details about this article) |
3/4. adult-onset Still's disease revealed by a pleuropericarditis.We report a case of adult-onset Still's disease (AOSD) revealed by pleuropericardial manifestations. A 40 yr old black woman was admitted for flu-like syndrome with pharyngitis, hectic fever, polymorphonuclear hyperleucocytosis and pleuropericarditis. The diagnosis of AOSD was supported by 3 major and 3 minor criteria after exclusion of infectious, haematological and connective tissue diseases. Pulmonary involvement is infrequent in AOSD, and consists of transient pulmonary infiltrates and chronic restrictive pattern. However, pleuritis, like pericarditis, is present in 25% of cases. Initial onset of pleuritis, associated with fever and hyperleucocytosis preceding articular manifestations could be responsible for a delay in diagnosis and a subsequent worsening in the prognosis of the disease. A rapid improvement is usually observed under nonsteroidal anti-inflammatory drug or corticosteroid treatment.- - - - - - - - - - ranking = 0.25keywords = adult-onset (Clic here for more details about this article) |
4/4. Human adjuvant disease. A new cause of chylous effusions.Connective-tissue disease occurring after cosmetic surgery with silicone injections or implants has been reported. This disorder has been called human adjuvant disease. One patient is described in whom a chylous effusion and systemic lupus erythematosus-like disorder developed after mammary augmentation with silicone gel-filled prostheses. This patient represents still another example of human adjuvant disease. A brief review of the literature regarding human adjuvant disease is also presented.- - - - - - - - - - ranking = 3.7954927488685keywords = still (Clic here for more details about this article) |