Human parvovirus and aplastic crisis in chronic hemolytic anemias: a study of 24 observations

From March 1984 through November 1985, 24 children and adults with aplastic crises were admitted in several Parisian hospitals. Twelve patients had known hemolytic anemia. Aplastic crisis revealed hemolytic anemia in the remaining patients. The detection of human parvovirus antigen was performed by counter-immunoelectrophoresis, and specific IgM antibodies were detected by IgM-antibody-capture-radioimmunoassay, in order to establish the incidence of human parvovirus infection in the genesis of the aplastic crisis. Twenty-one patients had acute infection with human parvovirus. In the three remaining patients, no marker of human parvovirus infection was found. The features of the human parvovirus linked aplastic crisis are described. We consider that human parvovirus infection, and unknown hemolytic anemia, must be systematically researched in any case of unexplained acute aplastic anemia.     

Usefulness in clinical practice of noninvasive measurement of colloids osmotic pressure-capillary pulmonary pressure gradient in the management of acute dyspnea

BACKGROUND: Increased pulmonary capillary pressure (PAWP) and decreased serum colloid osmotic pressure (COP) are important factors in pulmonary edema (PE) formation. The aim of this study was to evaluate the clinical usefulness of noninvasive determination of COP, PAWP and COP-PAWP gradient in patients with acute dyspnea. METHODS: Thirty four patients presenting with PE, 19 with preserved left ventricular systolic function (Ia) and 15 with depressed systolic function (Ib), and 26 patients presenting with acute pulmonary diseases (II) were enrolled. COP was estimated using Landis and Pappenheimer formula (COP = 2.1 x P + 0.16 x P2 + 0.009 x P3, P: serum total protein concentration). PAWP was measured by transthoracic Doppler echocardiography using E/Vp and 1000/(2 x IPT + Vp) Doppler indexes. RESULTS: The mean value of the gradient was 0.5 +/- 5, 1.7 +/- 3.4 and 10.4 +/- 4.7 mmHg in Ia, Ib and II respectively (P < 0.001 Ia and Ib vs II). PAWP was higher in Ia and Ib than in II (P < 0.001). However, hypoalbuminemia with COP < or = 18 mmHg was observed in 63% of patients in Ia as compared to those in Ib (7%) and II (8%). CONCLUSIONS: Severe hypoalbuminemia frequently contributes to PE formation in the presence of normal left ventricular systolic function. A COP-PAWP gradient value lower than 6 mmHg is highly predictive of the diagnosis of acute heart failure in these patients presenting with acute dyspnea.     

Dermatologic manifestations in lupus erythematosus

Various dermatologic manifestations are observed in the different lupus subsets. Lupus lesions are most frequently characterized by a dermoepidermal dermatitis. Other lesions, vascular or nonvascular, are essentially present in association with systemic lupus erythematosus. Acute, subacute and chronic lupus erythematosus are distinguishable according to their clinical aspects, pathological features and their evolution. Acute lesions are either localized to the midface or widespread. Subacute lesions may be annular or psoriasiform. Chronic lupus erythematosus includes localized or widespread discoid lupus, lupus tumidus, chilblain lupus and panniculitis. Therapy of cutaneous lupus is mainly based on antimalarials and avoidance of sun exposure. In refractory cutaneous lupus, no universal guidelines are available. Except for acrosyndromes and urticaria-like lesions, vascular lesions may be due to vasculitis or thrombosis. An accurate diagnosis is necessary, since therapeutic management is quite different in these two conditions. Nonvascular and nonlupus lesions are numerous and some of them require specific treatment, such as dapsone for bullous lupus.     

Multiple medullary and extramedullary plasmacytomas in an HIV infected female patient

INTRODUCTION: Before the HIV infection era, plasmocyte tumor rarely occurred in patients younger than 40 years of age. Less frequent than lymphomas, the incidence of these blood diseases has however substantially increased in HIV-infected patients. In these patients, in addition to onset at earlier age, their clinical presentation is quite different and extramedullary plasmocytomas in unexpected locations are more common. EXEGESIS: We report the case of a 29-year-old HIV-infected female patient in whom were diagnosed occipital, parotidal, sphenoidal, epidural, and uterine plasmocytomas for which chemotherapy and subsequent radiotherapy were successful. The increase in the incidence of plasmocyte tumors in HIV-infected patients might be facilitated by Epstein Barr Virus (EBV) co-infection, HIV-related chronic antigenic stimulation, and secretion of interleukin 6 by infected lymphocytes. CONCLUSION: Plasmocyte tumors belong to neoplasia whose incidence is increased in HIV infection. Their currently poor diagnosis should be improved by highly active antiretroviral therapies allowing enhanced chemotherapy with possibility of autograft.     

Therapeutic education for cardiac failure patients: the I-care programme

Therapeutic education is becoming increasingly important in the management of chronic diseases including cardiac failure. The I-CARE programme consists of an evaluation of the role of therapeutic education in France, creating standardised tools and setting up training sessions for therapeutic education in the context of cardiac failure. Approximately two thirds of the French centres contacted perform therapeutic education with their available means. The lack of personnel, space, and training tools represent obstacles to the development of therapeutic education. The tools developed in the programme fall into 5 areas: diagnosis education, understanding the illness, diet, physical activity/daily life, and treatment. Training sessions were organised for the teams, consisting of at least one cardiologist and nurse. The I-CARE programme should allow the expansion of therapeutic education for cardiac failure and improve the multidisciplinary management of this disease which increasingly affects often elderly subjects.     

Recurrence of spontaneous bacterial peritonitis in cirrhosis: frequency and predictive factors

We investigated whether spontaneous bacterial peritonitis in cirrhosis is a recurrent process and attempted to identify possible predictors of recurrence in 75 consecutive cirrhotics who had recovered from a first episode of spontaneous bacterial peritonitis between January, 1981 and December, 1984 and who were followed closely throughout their illness (follow-up period 10 +/- 13 months; mean +/- S.D.). Thirty-eight patients (51%) developed one or more episodes of spontaneous bacterial peritonitis during follow-up, the probability of recurrence (Kaplan-Meier's method) being 43% at 6 months, 69% at 1 year and 74% at 2 years. Twenty-three variables (age, sex, etiology of cirrhosis, standard liver and renal function tests and characteristics of the first spontaneous bacterial peritonitis) were analyzed as possible predictors of recurrence of spontaneous bacterial peritonitis. In univariate analysis (curves of Kaplan-Meier compared with Mantel-Cox's method), serum bilirubin greater than 4 mg per dl, prothrombin less than or equal to 45% and protein concentration in ascitic fluid less than or equal to 1 gm per dl were significantly (p less than 0.05) associated with a high risk or recurrence of spontaneous bacterial peritonitis. In multivariate analysis (Cox multiple regression model), only ascitic fluid protein concentration (p = 0.005) and prothrombin activity (p = 0.009) were found to be independent predictors of recurrence of spontaneous bacterial peritonitis. Fifty-nine patients (79%) died during follow-up, 18 of them (31%) secondary to spontaneous bacterial peritonitis. The 1-year survival probability in the whole series of patients was 38%.(ABSTRACT TRUNCATED AT 250 WORDS)     

Sacral inflammatory pseudotumor revealed by paraneoplastic syndrome

There is still debate on whether inflammatory pseudotumor should be considered benign or malignant. This lesion has only been reported twice in bone, apart from cases complicating foreign body reaction to joint replacement arthroplasty. We report here a third case, localized at the sacrum. A 31-year-old man had inflammatory dorsalgia and polyarthralgia without synovitis but with fever, asthenia, and erythema nodosa. Biological tests and X-rays were not informative, but technetium scintigraphy revealed a high level of left sacroiliac tracer binding. Several nonsteroidal anti-inflammatory drugs and sulfasalazine treatment were given over 3 months but ineffective. Pelvic magnetic resonance imaging showed an osteolytic tumor of the sacrum. Biopsy suggested a malignant fibrosarcoma, but complete evaluation after surgical resection demonstrated an inflammatory pseudotumor. All clinical symptoms disappeared within a few days after surgery, which is suggestive of a paraneoplastic syndrome. No relapse has occurred after 4 years.     

Arterial and portal circulation and parenchymal changes in Budd-Chiari syndrome: a study in 17 explanted livers

Hepatic parenchymal changes associated with Budd-Chiari syndrome (BCS) have been tentatively explained by combined arterial and portal perfusion disturbances in addition to the complete occlusion of hepatic veins. The aim of this study was to correlate pretransplant course and vascular imaging with pathologic findings in livers explanted for BCS. Seventeen consecutive white patients who underwent transplantation for severe classic BCS were retrospectively analyzed. Pretransplant course was 1 year or less in 8 patients and more than 1 year in 9 patients. Thrombophilia was found in 16 patients (94%). Imaging showed decreased portal perfusion in 16 patients (94%) and increased arterial perfusion in 9 patients. Histology showed obstructive portal venopathy and nodular regenerative hyperplasia (NRH) aspects in all cases, large regenerative nodules resembling focal nodular hyperplasia (FNH) in 9 cases, and cirrhosis in 2 cases. Patients with increased arterial inflow had large regenerative nodules and a protracted pretransplant course. Patients with acute thrombi in portal veins had parenchymal infarcts (2 cases) and a short pretransplant course. In conclusion, patients with severe BCS have a constant impaired perfusion inflow unrelated to progression of cirrhosis but related to the outcome. An early decrease in portal perfusion is observed in the short term and is responsible for NRH or infarcts if complicated with large thrombi. An increase in arterial perfusion compensates impaired portal flow in chronic BCS. Arterial hyperemia contributes to the development of large regenerative nodules that are FNH-like. This pathologic situation offers an interesting vascular model to further understand the parenchymal response to changes in hepatic blood flow.     

Inhibition of rat liver fibrogenesis through noradrenergic antagonism

The effect of adrenergic innervation and/or circulating catecholamines on the function of liver fibrogenic cells is poorly understood. Our aim was to investigate the effects of noradrenergic antagonism on carbon tetrachloride (CCl4)-induced liver fibrosis in rats. Two weeks of CCl4 induced an approximately 5-fold increase in the area of fibrosis as compared with controls. The addition of 6-hydroxydopamine (OHDA), a toxin that destroys noradrenergic fibers, decreased fibrosis by 60%. After 6 weeks of CCl4, the area of fibrosis increased about 30-fold in CCl4-treated animals and was decreased by 36% with OHDA. At 2 weeks, OHDA abrogated the CCl4-induced increase in mRNA level of tissue inhibitor of matrix metalloproteinases-1 (TIMP-1), an inhibitor of extracellular matrix degradation, and it greatly reduced it at 6 weeks. Finally, when rats treated with CCl4 for 2 weeks also received prazosin, an antagonist of alpha1-adrenergic receptors, fibrosis was decreased by 83%. In conclusion, destruction of noradrenergic fibers or antagonism of noradrenergic signaling through alpha1 receptors inhibited the development of liver fibrosis. Because adrenoreceptor antagonists have a very sound safety profile, they appear as attractive drugs to reduce liver fibrogenesis.