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Biomedical subjects

J L Pennaforte

Publications and source records attributed to J L Pennaforte.

At least 19 recordsLinked to original sources
PubMed1999

No quantitative relationship between CR1 and Lutheran expression on erythrocytes: In(Lu) gene product is not a common regulator of CR1 expression on erythrocytes.

The density of CR1, the C3b/C4b receptor (CD35), on erythrocytes (E) (CR1/E) is genetically determined. However, the broad distribution of CR1/E within a given genotype suggests that other genetic elements might contribute to the regulation of CR1/E. In some pathological conditions, including systemic lupus erythematosus (SLE), AIDS and hemolytic anemia, CR1 deficiency parallels the severity of the disease. When compared to healthy individuals, an accelerated decrease in CR1/E in these patients has been demonstrated, but other mechanisms interfering with CR1 density regulation during erythropoiesis might also contribute. In exceptional circumstances, CR1/E can be dramatically decreased in healthy individuals by the effect of a regulatory gene, In(Lu), that switches off various surface molecules on E, the structure genes of which are located on four different chromosomes, suggesting a transcription regulatory role for In(Lu) gene products. The hypothesis that products of this gene could physiologically regulate the surface density of all these molecules has been tested by determining Lub density on E (Lub/E) using quantitative flow cytometry. Lub antigenic sites were then compared to CR1/E among healthy individuals of the different CR1 density phenotypes, SLE patients with and without CR1 deficiency, and an exceptional SLE patient totally lacking CR1/E and reticulocytes. No quantitative relationship was found between CR1 and Lub expression in either normal or pathological conditions. These data establish that In(Lu) products are not involved in normal or pathological CR1 density regulation.

Antibodies, Monoclonal↗
PubMed1998

Effect of chlorambucil on bone mineral density in the course of chronic lymphoid leukemia.

The purpose of this work was to study the effects of chronic lymphoid leukemia (CLL) and its treatments on bone mineral density (BMD). Lumbar and femoral BMD was measured by X-ray absorptiometry in 50 (32 M, 18 F, median age 65, range age: 47-87 yr) CLL patients. In order to gauge the respective effects of CLL and corticoids on bone mass, 31 CLL patients under treatment were compared with 31 controls on cortisone. Nineteen untreated patients with CLL were compared with controls devoid of osteopenia risk factor. There was no significant difference regarding lumbar and femoral BMD between the untreated patients with CLL and the healthy controls. An increase in lumbar and femoral BMD was noted in the treated CLL group compared with the controls on cortisone (lum BMD: 1.018 vs. 0.861 g/cm2, p=6.10(-4); fem BMD: 0.773 vs. 0.699 g/cm2, p=0.037). This increase was observed only in patients who had received chlorambucil (lum BMD: 1.066 vs. 0.861 g/cm2, p=0.10(-4); fem BMD: 0.806 vs. 0.699 g/cm2, p=4.10(-3)), whereas there was no difference between the CLL patients treated without chlorambucil and the controls on cortisone. Multiple linear regression analysis confirmed the marked effect of chlorambucil (r=0.3715, p<10(-3)) on BMD increase in the course of CLL.

Absorptiometry, Photon↗
PubMed1998

[Renal manifestations of Kimura disease. Apropos of a case].

A nephrotic syndrome with focal sclerosis associated with Kimura's disease is reported in a young asiatic man. The nephrotic syndrome started three years prior to the usual skin tumors and lymph nodes involvement. Epidemiological, clinical and nosological characteristics of Kimura's disease are discussed. Renal manifestations are the only visceral localisations of the disease. Thirteen Kimura's disease associated with documented nephrotic syndromes have been reported in german-english literature. They display a wide variety of histologic patterns. Their clinical course do not differ from the primary form of nephrotic syndrome with identical histology.

Adult↗
PubMed1997

[Influence of seasons on risk of flare-up of systemic lupus: retrospective study of 66 patients].

PURPOSE: To establish the possible connection between visceral, arthro-cutaneous and biological spreading of systemic lupus (SL) and hours of sunlight. MATERIAL AND METHODS: Retrospective study of 66 SL patients, consisting of 52 visceral and 14 arthro-cutaneous cases taking into account the chronological pattern of each new aggravation, based on 480 clinical records. RESULTS: Increased frequency in visceral aggravation was observed in the post-summer period (August-January) (n = 57), as compared with the pre-summer period (February-July) (n = 25) (RR = 1.75, P = 0.006). This post-summer visceral aggravation was correlated with cutaneous affection (RR = 4.18) and absence of previous corticotherapy (RR = 3.97). Visceral and arthro-cutaneous aggravations taken together revealed a more disturbed immune balance pattern in the post-summer period (anti-dsDNA: 30 versus 25.1 IU/L [P = 0.07]; C3: 0.83 vs 0.921 IU/L [P = 0.05]; C4: 0.146 vs 0.183 [P = 0.05]), providing evidence of greater severity. Moderate thrombopenia (50-120 10(9)/L) accompanying visceral SL with antiphospholipids (n = 33) was more frequent during the post-summer period, even in the absence of aggravation (P = 0.03). The quarterly distribution of visceral aggravations was correlated with average hours of sunlight in the preceding quarter (P = 0.01). CONCLUSION: There is a post-summer increase in the frequency and severity of visceral SL spreading correlated to cutaneous exacerbation and sunlight.

Adult↗
PubMed1997

Rheumatic manifestations of scurvy. A report of two cases.

Bleeding into the muscles and joints can be the presenting manifestation of scurvy, as illustrated by two case-reports. One patient presented with hemarthrosis of the tibiotalar joint due to an insufficiency fracture and was suspected to have scurvy based on the presence of purpura and hypertrophy of the gums with loss of teeth. In the other patient, multiple hematomas in the lower limbs were found at presentation and the presence of coiled hairs suggested the diagnosis. Both patients had completely eliminated fruit and vegetables from their diet. Low levels of ascorbic acid were found in serum and urine. A full recovery was achieved in both cases under ascorbic acid supplementation.

Adult↗
PubMed1996

Avascular necrosis of the femoral head as a complication of chronic myelogenous leukaemia.

A 17-year-old male patient complaining of intense pain in his right hip was found to be suffering from chronic myelogenous leukaemia. Preliminary X-rays and bone scintigraphy did not suggest avascular necrosis of the femoral head. Magnetic resonance imaging (MRI) did, however, reveal leukaemic infiltration of the femoral neck and generalised ischeamia in the femoral head. Further, MRI carried out 4 months later disclosed typical signs of osteonecrosis, despite previous indications of an improvement under chemotherapy. Flattening of the head of the femur appeared in radiographs taken in the 9th month. In the 12th month, recurrence of pain made it necessary to perform a total hip arthroplasty. Anatomo-pathological investigation confirmed both the necrosis and the leukaemic invasion.

Adolescent↗
PubMed1996

[Pulmonary manifestations of primary Gougerot-Sjögren syndrome. Apropos of 8 cases in a series of 35 patients].

Clinical, roentgenologic, functional and broncho-alveolar lavage features of lung involvement in primary Sjögren's syndrome were assessed in a retrospective study of 35 cases. Diffuse interstitial patterns on chest radiography were present in six patients and alveolar patterns were suggestive of lymphoid interstitial pneumonitis or pseudolymphoma in two. Acute and febrile onset mimicked infectious pneumonitis in three patients when dyspnea was the most common clinical feature in others. Patients with primary Sjögren's syndrome and pulmonary disease were older (65 vs 56 years) (P = 0.025), have more frequently extra-glandular manifestations (P = 0.03), keratoconjunctivis sicca (P = 0.018) and biological perturbations (hypergammaglobulinemia (P = 0.03), antinuclear antibodies (P = 0.01) than those without lung involvement. Low diffusion capacity was present in seven patients associated twice to small airways obstruction. Bronchoalveolar lavage revealed in all cases an increased total cells count (mean: 6.96 10(5)/mm3) and a lymphocytic alveolitis (range: 11 to 66%; mean: 38%) associated with an elevated percentage of alveolar neutrophils in four patients. A low CD4/CD8 ratio was related to a pejorative issue. Treatment consisted in corticotherapy combined with oral cyclophosphamide in case of pseudolymphoma.

Aged↗
PubMed1994

[Cardiac anomalies in disseminated lupus erythematosus diagnosed by Doppler ultrasonography. Prevalence and association with antiphospholipid syndrome].

OBJECTIVES: Antiphospholipid antibodies in patients with systemic lupus erythematosus (SLE) are widely associated with thrombosis, recurrent abortions and a thrombocytopenia. The purpose of this study was first to evaluate the prevalence of cardiac abnormalities in patients with SLE and secondly to establish the relationship between the findings and the presence of an antiphospholipid syndrome. SUBJECTS AND METHODS: First of all, a total of 52 consecutive patients with SLE and 52 healthy sex-and-age-matched control subjects were evaluated in a cross sectional study. All underwent M mode and two dimensional echocardiography, color flow imaging, pulsed and continuous wave Doppler. Secondly, in the SLE group, subjects exposed to antiphospholipid syndrome (n = 20) were compared to the control subjects. RESULTS: Compared with the control group, patients with SLE had significantly more pericardial abnormalities [(p = 0.0006) RR [3.36-infinity], mitral regurgitation [(p = 0.032) RR 2.48 [1.25-5.6]], tricuspid regurgitation [(p = 0.0016) RR 2.41 [1.58-8.85]. There was no significant difference between both groups for the left ventricular mass (p = 0.07), posterior wall (p = 0.25) and interventricular septum dimension (p = 0.16), and mitral valve thickness (p = 0.66). The antiphospholipid syndrome was significantly associated with increased left ventricular mass (p = 0.0054), posterior wall (p = 0.022) and interventricular septum dimension (p = 0.026). The relative risk increased for tricuspid and pulmonary regurgitation in SLE patients who had the antiphospholipid syndrome. CONCLUSION: The prevalence of left ventricular hypertrophy and right heart valvular regurgitation is significantly raised in SLE patients who have the antiphospholipid syndrome. However, antiphospholipid syndrome does not modify the prevalence of pericardial abnormalities which seems to be due to SLE alone.

Adult↗
PubMed1994

Prevalence of valvular involvement in systemic lupus erythematosus and association with antiphospholipid syndrome: a matched echocardiographic study.

Antiphospholipid antibodies in patients with systemic lupus erythematosus (SLE) are often associated with thrombosis, recurrent abortions and thrombocytopenia. The purpose of this study was to evaluate the prevalence of cardiac valvular abnormalities in patients with SLE and to establish the relationship between the echographic findings and the presence of an antiphospholipid syndrome. A total of 52 consecutive patients with SLE and 52 healthy sex- and age-matched controls were therefore evaluated in a cross-sectional study. All underwent M-mode and two-dimensional echocardiography, color-flow imaging, pulsed and continuous-wave Doppler. In the SLE group, subjects exposed to antiphospholipid syndrome (n = 20) were compared to controls. Patients with SLE had significantly more mitral (p = 0.032; RR 2.48; 1.25-5.6) and tricuspid regurgitations (p = 0.0016; RR 2.41; 1.58-8.85). There was no significant difference between either group for mitral valve thickness (p = 0.66). The antiphospholipid syndrome was significantly associated with increased relative risk for tricuspid.

Adolescent↗