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C Frances

Publications and source records attributed to C Frances.

At least 37 records · Page 2Linked to original sources
PubMed1998

[Cutaneous signs of Rickettsia and related microorganisms].

INTRODUCTION: Rickettsiosis are emerging, life-threatening infectious diseases that are world-wide distributed and have various manifestations. Most of the time, skin involvement is the prominent feature of clinical manifestations. Clinicians must therefore be well aware of these various clinical pictures. CURRENT KNOWLEDGE AND KEY POINTS: Knowledge about rickettsial infections has recently improved due to either the identification of new vectors and reservoirs, the description of new systemic and cutaneous signs and symptoms, or the amelioration of diagnosis methods. Cutaneous manifestations mainly depend on the type of microorganism and on the host characteristics. In some cases, they are so typical that their only identification would lead to the diagnosis. FUTURE PROSPECTS AND PROJECTS: As growing population movements may lead to an increase in the disease incidence, detailed knowledge of clinical, particularly cutaneous, manifestations of rickettsiosis is required in order to improve both diagnosis and treatment. When identified, cutaneous manifestations of rickettsiosis allow the clinician to confirm rapidly the diagnosis, using polymerase chain reaction or immunofluorescence and to institute prompt and specific treatment, thus reducing unfavorable disease outcome.

Diagnosis, Differential↗
PubMed1998

Left ventricular pseudoaneurysm.

Left ventricular (LV) pseudoaneurysms form when cardiac rupture is contained by adherent pericardium or scar tissue. Although LV pseudoaneurysms are not common, the diagnosis is difficult and they are prone to rupture. We evaluated the clinical presentation, diagnostic accuracy of imaging modalities, results of therapy and prognosis of 290 patients with LV pseudoaneurysms. Most cases of LV pseudoaneurysm were related to myocardial infarction (particularly inferior wall myocardial infarction) and cardiac surgery. Congestive heart failure, chest pain and dyspnea were the most frequently reported symptoms, but >10% of patients were asymptomatic. Physical examination revealed a murmur in 70% of patients. Almost all patients had electrocardiographic abnormalities, but these were usually nonspecific ST segment changes; only 20% of patients had ST segment elevation. Although radiographic findings were also usually nonspecific, the appearance of a mass was present in more than one half of patients and may be an important clue to the correct diagnosis. Left ventricular angiography was the most definitive test and can be useful in planning surgery since concomitant coronary angiography can be performed. Regardless of treatment, patients with LV pseudoaneurysms had a high mortality rate, especially those who did not undergo surgery. Because the symptoms, signs, electrocardiographic abnormalities and radiographic findings seen in patients with LV pseudoaneurysms can be indistinguishable from those in patients with coronary disease alone, a high clinical index of suspicion is needed to avoid missing the diagnosis.

Aneurysm, False↗
PubMed1998

Apoptosis and expression of soluble Fas mRNA in systemic lupus erythematosus.

Our objective was to measure the relative proportions of messenger RNAs coding for soluble and membrane-bound Fas in peripheral blood mononuclear cells (PBMCs) and to quantify lymphocyte apoptosis in vitro in systemic lupus erythematosus (SLE). A RT-PCR (soluble/membrane Fas mRNA) ratio was calculated in 16 SLE, 11 RA and 16 healthy subjects' PBMCs. Apoptosis was quantitated in vitro using nick-end labeling and flow-cytometry analysis immediately and after overnight T cell activation. The mean soluble/membrane Fas ratio was 1.219+/-0.424 in the SLE group, significantly higher than in healthy subjects, 0.516+/-0.180 (P = 0.0001). There was no significant difference between inactive and active SLE groups whereas Fas ratio was higher in SLE patients with nephritis (1.460+/-0.365) compared to those without nephritis (1.031+/-0.380) (P=0.039). Mean soluble/ membrane Fas ratio in RA patients (0.975 +/- 0.37) was higher than in healthy subjects (P = 0.0003) and lower than lupus nephritis patients (P=0.015). A significantly higher proportion of mononuclear cells engaged apoptosis after T cell activation in active lupus patients, compared to control subjects. In comparison with healthy subjects, Fas alternative splicing was skewed toward the soluble form of Fas in SLE and RA. Apoptosis after T cell activation in vitro was increased in active SLE patients.

Adolescent↗
PubMed1998

A new method of developing expert consensus practice guidelines.

To improve the quality of medical care while reducing costs, it is necessary to standardize best practice habits at the most crucial clinical decision points. Because many pertinent questions encountered in everyday practice are not well answered by the available research, expert consensus is a valuable bridge between clinical research and clinical practice. Previous methods of developing expert consensus have been limited by their relative lack of quantification, specificity, representativeness, and implementation. This article describes a new method of developing, documenting, and disseminating expert consensus guidelines that meets these concerns. This method has already been applied to four disorders in psychiatry and could be equally useful for other medical conditions. Leading clinical researchers studying a given disorder complete a survey soliciting their opinions on its most important disease management questions that are not covered well by definitive research. The survey response rates among the experts for the four different psychiatric disorders have each exceeded 85%. The views of the clinical researchers are validated by surveying separately a large group of practicing clinicians to ensure that the guideline recommendations are widely generalizable. All of the suggestions made in the guideline are derived from, and referenced to, the experts' survey responses using criteria that were established a priori for defining first-, second-, and third-line choices. Analysis of survey results suggests that this method of quantifying expert responses achieves a high level of reliability and reproducibility. This survey method is probably the best available means for standardizing practice for decisions points not well covered by research.

Bias↗
PubMed1998

Ribavirin in hepatitis C related cryoglobulinemia.

OBJECTIVE: An open, uncontrolled trial of ribavirin, an oral guanosine nucleoside analog for treatment of hepatitis C, in patients with hepatitis C virus (HCV) associated cryoglobulinemia intolerant to interferon. METHODS: Five patients with cryoglobulinemia related to HCV infection unresponsive to interferon therapy received oral ribavirin (100 to 1200 mg daily) for 10 to 36 months. RESULTS: Patients treated with ribavirin had prompt decrease in serum aminotransferase levels and marked improvement of manifestations of cryoglobulinemia within a few weeks. Ribavirin did not eradicate HCV RNA from the sera, but a decrease in viral load was observed in 3 patients, from 232 to 86 x 10(5) copies HCV/ml. Relapse occurred within 3 months once therapy was discontinued. The drug was well tolerated, but mild dose related hemolysis was common. CONCLUSION: Ribavirin monotherapy may be effective in patients with symptomatic cryoglobulinemia related to HCV infection, but this effect is not sustained when ribavirin therapy is discontinued.

Aged↗
PubMed1998

[Neutrophilic eccrine hidradenitis associated with relapse of acute myeloblastic leukemia].

BACKGROUND: Neutrophilic eccrine hidradentitis is a recently described clinical entity. Most reported cases have occurred in patients given chemotherapy for acute myelogenous leukemia, suggesting a drug induced mechanism. Some authors have considered however that neutrophilic eccrine hidradenitis belongs to the group of neutrophilic dermatoses. CASE REPORT: We observed neutrophilic eccrine hidradentitis in a 48-year-old man which developed when he suffered a relapse of acute leukemia. He had not been given chemotherapy in the preceding months. DISCUSSION: This case favors the hypothesis that neutrophilic eccrine hidradenitis is associated with myeloid hemotology disorders and not a complications secondary to treatment.

Hidradenitis↗
PubMed1997

African tick-bite fever. An imported spotless rickettsiosis.

OBJECTIVES: To characterize the clinical presentation and course of African tick-bite fever, a recently rediscovered rickettsiosis caused by Rickettsia africae (a new species within the spotted fever group of rickettsiae), to establish its relationship with Amblyomma tick species, and to discuss its role in the etiology of fever in patients who are returning from the tropics. PATIENTS: Seven patients who returned from Zimbabwe of the Republic of South Africa and presented with fever. METHODS: Cases were recognized clinically by the presence of multiple taches noire and were diagnosed as having a rickettsial infection by identification of the organisms in circulating endothelial cells. The causative role of R africae was further demonstrated using cross-absorption and immunoblotting of patients' serum samples and isolation of the agent from blood and skin biopsy specimens. Isolates were characterized using the restriction fragment length polymorphism-polymerase chain reaction and sequence analysis of the gene that encodes for the 190-kd Rickettsia-specific antigen. RESULTS: All 7 patients presented with fever and multiple taches noire. Further physical examination of patients revealed lymphadenopathy, lymphangitis, and edema, but there were virtually no signs of a rash. These findings are characteristic of R africae-infected patients and are distinct from those observed in patients with Rickettsia conorii-induced Mediterranean spotted fever. All 7 patients were infected with R africae as demonstrated by immunoblotting or isolation of the agent, and all were cured. CONCLUSIONS: With increasing international travel, a need for the recognition of rickettsial diseases by physicians is becoming more important. Tick-bite fever, a disease caused by R africae and transmitted by Amblyomma ticks, is characterized by multiple taches noire, lymphadenopathy, lymphangitis, and edema, but no rash or a discrete rash. It is a frequent but benign disease that physicians should consider when presented with febrile patients returning from southern Africa.

Adult↗
PubMed1997

A peculiar form of amicrobial pustulosis of the folds associated with systemic lupus erythematosus and other auto-immune diseases.

Amicrobial pustulosis of the folds is a new entity which was initially described in association with systemic lupus erythematosus (SLE). We report four cases of this rare pustulosis occurring in the course of various auto-immune disorders. Dermatological features were similar in all patients. The primary lesions were small pustules which coalesced and formed erosive areas underlined with pustular lesions. They were localized in the folds. Three of the four patients had also isolated pustules on the scalp. Spongiform pustules were histologically present. A primary bacterial or fungal infection was ruled out by cultures on appropriate media. Serum calcium level was normal. Associated auto-immune diseases were: systemic lupus erythematosus, incomplete systemic lupus erythematosus, 'idiopathic' thrombocytopenic purpura and myasthenia gravis without thymoma (one of each). The relationship between this peculiar form of pustulosis and autoimmunity remains to be elucidated. The pustulosis did not respond to antibiotics but disappeared with systemic or topical corticosteroids.

Adolescent↗
PubMed1997

[Degos disease].

Explore the source record for details and available documents.

Atrophy↗
PubMed1996

Bedside measurement of factor VIII:C activity in individuals with hemophilia A.

Factor VIII replacement therapy for patients with hemophilia A is conventionally monitored using a plasma-based factor VIII:C assay (a modified activated partial thromboplastin time [APTT] test). The plasma factor VIII assay requires the preparation of plasma from citrated whole blood and measurement of the clotting times of mixtures of patient plasma, factor VIII-deficient substrate, and APTT reagent. Results are not routinely available in less than 1.5 hr, reducing the clinical value of the laboratory data regarding the ability to immediately adjust patient therapy. Results from the whole blood factor VIII assay, performed on a portable coagulation analyzer and using test tubes prefilled with the necessary APTT and factor VIII-deficient reagents, are available within 5-7 min. This immediate determination of the factor VIII:C level from citrated whole blood provides the opportunity to greatly reduce turnaround time and improve the efficacy of factor VIII replacement therapy. Based on clotting time, factor VIII:C activity is read from a standard curve. A clinical evaluation of this whole blood test was performed in two hemophilia centers. A high degree of correlation was seen (r=0.813, n=220) between the whole blood values obtained and conventional laboratory results. This level of correlation was superior to that obtained when comparing two different plasma-based systems(r=0.753, n=23). Factor VIII:C activity levels measured using the whole blood assay system were similar,irrespective of the test operator (laboratory technologist, nurse clinician, or patient). This study indicates that the whole blood factor VIII assay provides results comparable to those of conventional plasma-based assays, but in a more rapid and efficient manner. It provides an opportunity to reduce unnecessary patient consumption of replacement preparations, hence reducing the cost of hemophilia A maintenance and prophylaxis regimens, and to reduce overall patient exposure to human blood products.

Factor VIII↗
PubMed1996

Risks of ovulation-induction therapy in systemic lupus erythematosus.

We report on four women with systemic lupus erythematosus who developed two types of complications after ovulation-induction therapy for primary or secondary infertility. Primary infertility was associated with endometriosis in one patient. Three had previously known systemic lupus erythematosus. All had inactive disease at onset of ovulation-induction therapy. Three patients developed symptoms consistent with moderate lupus flare a few weeks after the onset of ovulation-induction therapy. One patient developed inferior vena cava and unilateral left renal vein thrombosis. No patient became pregnant. A high oestrogen level induced by ovulation-induction therapy may explain the occurrence of lupus flare in patients with prior inactive lupus. All our patients had prior asymptomatic antiphospholipid antibodies. One patient developed a major thrombotic event. The presence of antiphospholipid antibodies increases the thrombotic risk related to ovulation-induction therapy. We conclude that ovulation-induction therapy should be restricted to patients with long-standing inactive systemic lupus erythematosus. A preventive increase of the corticosteroid dosage should be proposed in addition to heparin or antiaggregant therapy for those with prior asymptomatic antiphospholipid antibodies, or with heparin therapy for those with prior antiphospholipid antibody-related events.

Adult↗
PubMed1996

Specific mucosal erosions in hypereosinophilic syndrome. Evidence for eosinophil protein deposition.

BACKGROUND: Mucosal erosions can be a presenting feature of the hypereosinophilic syndrome. The aim of this study was to analyze in situ the presence of eosinophil proteins and the state of eosinophil activation. Biopsy specimens of mucosal lesions and normal skin were taken from two men with oral and genital erosions typical of hypereosinophilic syndrome. Tissue sections were immunohistochemically labeled with anti-major basic protein, anti-eosinophil-derived neurotoxin, and anti-eosinophil peroxidase antibodies. The same specimens were also subjected to electron microscope examination. OBSERVATIONS: Eroded specimens displayed areas of eosinophil spongiosis within which extracellular deposits of eosinophil peroxidase, major basic protein, and eosinophil-derived neurotoxin were present. In normal skin, only a few eosinophils were present within the capillary lumen, and no extracellular deposits of these proteins were seen. Under the electron microscope, the cytoplasmic membranes of eosinophils located around the erosion were disrupted. Remnants of necrotic keratinocytes were found near these lysed eosinophils. CONCLUSION: As with other involved organs in hypereosinophilic syndrome, mucosal erosions seem to be the consequence of eosinophil protein release.

Humans↗