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Biomedical subjects

P Disdier

Publications and source records attributed to P Disdier.

At least 55 records · Page 3Linked to original sources
PubMed2001

[Acute transitory intrafamilial erythroblastopenia and hereditary spherocytosis: role of parvovirus B19].

INTRODUCTION: Acute parvovirus B19 infection induces a transient inhibition of erythroid cell formation, which may induce an erythroblastopenia crisis in patients suffering from chronic hemolytic anemia. EXEGESIS: We report here an exceptional observation of acute erythroblastopenia crisis with good outcome, occurring at the same time in a mother and her son, both suffering from hereditary spherocytosis. Diagnosis of parvovirus infection is based on detection of serum parvovirus B19-specific immunoglobulin M antibodies in the mother and her son and by the positivity of parvovirus B19 DNA detected by PCR in serum in the mother. Outcome was good, with the end of the erythroblastopenia crisis obtained 7 to 10 days later, but requiring blood transfusion in the mother. CONCLUSION: Our observation is a reminder that the contagiosity of parvovirus B19 is high in household contacts and that protection of family members should rapidly be considered in hereditary spherocytosis.

Acute Disease↗
PubMed2001

[Is chronic or recurrent idiopathic pericarditis an autonomous inflammatory disease?].

PURPOSE: Chronic pericarditis or recurrent pericarditis is mostly considered to be idiopathic even when up-to-date medical investigations are undertaken. The absence of aetiology and the associated inflammatory process are features of a common disease for internists. As there are only a few published reports on this disease, therapeutic options are not easily envisaged. CURRENT KNOWLEDGE AND KEY POINTS: Idiopathic pericarditis and its evolution, characterized by recurrence or chronicity, has long been diagnosed and studied. Faced with a case of acute pericarditis, no clinical or biological data can preclude evolution towards a chronic or a recurrent form. The two major complications are tamponade and constriction. Classical treatment is aspirin and nonsteroidal anti-inflammatories. Steroids have a spectacular effect but steroid dependence is frequently observed. Colchicine treatment seems to be efficacious and can be used to stop steroid therapy. There are only a few published reports on the importance of immunosuppressive drugs such as azathioprine and cyclophosphamide. FUTURE PROSPECTS AND PROJECTS: Through our own experience and literature review, we propose to consider chronic and/or recurrent pericarditis as an autonomous inflammatory disease of the pericardium. Thus, large-scale studies concerning the treatment should improve the outcome of patients.

Adrenal Cortex Hormones↗
PubMed2001

Prelymphomatous presentation of T-cell non-Hodgkin lymphomas. A clinical and histopathological study of 11 cases.

We report a retrospective study of 11 patients suffering from T-cell non-Hodgkin lymphomas preceded by immunological disorders including two chronic granulomatous diseases, one midline granuloma, four autoimmune hematologic disorders, one hypereosinophilic syndrome, two chronic lymphadenopathies, and one chronic angioedema. In the follow-up of these 11 patients, T-cell non-Hodgkin lymphomas were diagnosed. Even if we cannot exclude a fortuitous association, we feel that these conditions could constitute a 'prelymphomatous' stage.

Journal Article↗
PubMed2001

A nucleotide insertion and frameshift cause albumin Kénitra, an extended and O-glycosylated mutant of human serum albumin with two additional disulfide bridges.

Albumin Kenitra is a new type of genetic variant of human serum albumin that has been found in two members of a family of Sephardic Jews from Kenitra (Morocco). The slow-migrating variant and the normal protein were isolated by anion-exchange chromatography and, after treatment with CNBr, the digests were analyzed by two-dimensional electrophoresis in a polyacrylamide gel. The CNBr peptides of the variant were purified by reverse-phase high performance liquid chromatography and submitted to sequence analysis. Albumin Kenitra is peculiar because it has an elongated polypeptide chain, 601 residues instead of 585, and its sequence is modified beginning from residue 575. DNA structural studies showed that the variant is caused by a single-base insertion, an adenine at nucleotide position 15 970 in the genomic sequence, which leads to a frameshift with the subsequent translation to the first termination codon of exon 15. Mass spectrometric analyses revealed that the four additional cysteine residues of the variant form two new S-S bridges and showed that albumin Kenitra is partially O-glycosylated by a monosialylated HexHexNAc structure. This oligosaccharide chain has been located to Thr596 by amino-acid sequence analysis of the tryptic fragment 592-597.

Adenine↗
PubMed2001

Cannabis arteritis revisited--ten new case reports.

The purpose of this paper was to revisit the old concept of cannabis arteritis first described in the 1960s and report 10 new cases. Ten male patients, with a median age of 23.7 years developed subacute distal ischemia of lower or upper limbs, leading to necrosis in the toes and/or fingers and sometimes to distal limb gangrene. Two of the patients also presented with venous thrombosis and three patients were suffering from a recent Raynaud's phenomenon. Biological test results did not show evidence of the classical vascular risk factors for thrombosis. Arteriographic evaluation in all cases revealed distal abnormalities in the arteries of feet, legs, forearms, and hands resembling those of Buerger's disease. A collateral circulation sometimes with opacification of the vasa nervorum was noted. In some cases, arterial proximal atherosclerotic lesions and venous thrombosis were observed. All patients were moderate tobacco smokers and regular cannabis users. Despite treatment with ilomedine and heparin in all cases, five amputations were necessary in four patients. The vasoconstrictor effect of cannabis on the vascular system has been known for a long time. It has been shown that delta-8- and delta-9-tetrahydrocanabinols may induce peripheral vasoconstrictor activity. Cannabis arteritis resembles Buerger's disease, but patients were moderate tobacco smokers and regular cannabis users. These cases show that prolonged use of cannabis could be an additive risk factor for juvenile and young adult arteritis. Cannabis arteritis is a forgotten and severe occlusive vascular disease occurring in young adults. Search for cannabis use may be an important tool for a better knowledge of arteritis in young smokers.

Adolescent↗
PubMed2001

Severe lower limbs lymphedema of late onset revealing polysplenia syndrome--a case report.

Polysplenia syndrome includes a group of congenital abnormalities involving the spleen, gastrointestinal tract, liver, and cardiovascular system. A case of severe lower limbs lymphedema occurring in a young woman with polysplenia, azygous continuation of the inferior vena cava, short pancreas, and preduodenal portal vein is reported. Lower limb lymphedema could represent a new clinical manifestation associated with polysplenia syndrome. Lymphedema could be the result of a primary anomaly of the lymphatic system or be induced by high pressure in the venous system or by compression of the lymphatic circulation by the hypertrophic azygous vessels in the posterior mediastinum.

Abnormalities, Multiple↗
PubMed2001

Pinguecula and Sjögren's syndrome: two cases.

A pinguecula is a small, raised conjunctival nodule at the temporal or nasal limbus classically associated with conjunctival microtrauma from exposure to sunlight and/or to dust. We report two cases occurring during the course of Sjögren's syndrome. To our knowledge this association has never been described.

Antibodies, Antinuclear↗
PubMed2001

[Mitochondrial diseases in adults].

PURPOSE: Mitochondrial diseases have numerous phenotypic expression, and form an heterogeneous group of genetic diseases in which the production of energy fails. Well known in childhood, these mitochondrial diseases can onset in adulthood and may remain unrecognized. We propose a recent review (Medline 1981-2001) of the literature on adult forms of mitochondriopathies, illustrated with a typical case report. CURRENT KNOWLEDGE AND KEY POINTS: Mitochondrial diseases have numerous phenotypic expression in adulthood. Principles of diagnosis are i) recognize a phenotype, ii) prove the mitochondrial abnormalities, iii) realize a genetical analysis. Main varieties of adult phenotypes are studied and separated in 1) skeletal muscular involvement, ocular myopathies above all; 2) mitochondrial cardiomyopathies; 3) neurological involvement (MERRF, MELAS, NARP, MNGIE syndromes); 4) endocrinological involvement and diabetes mellitus; 5) multisystemic diseases with a particular focus on Kearns Sayre syndrome. FUTURE PROSPECTS AND PROJECTS: Phenotypic analysis of a patient with mitochondrial disease is not simple. A "multi-tissues" involvement is the main characteristic feature. Faced with such patients, replacement therapy, genomic therapy and genetic advice are evoked.

Adult↗
PubMed2000

Lymphomatoid papulosis associated with both severe hypereosinophilic syndrome and CD30 positive large T-cell lymphoma.

BACKGROUND: Previous reports have found an association between lymphomatoid papulosis and hypereosinophilic syndrome, as well as lymphomatoid papulosis and lymphoma. In the current study the authors report what to their knowledge is the first reported case of these three diseases occurring simultaneously in the same patient. METHODS: The authors followed the clinical course of a 64-year-old man with lymphomatoid papulosis associated with severe hypereosinophilic syndrome complicated by involvement of the lungs and heart. RESULTS: After 6 years of follow-up, the patient developped a large T-cell, CD30 positive lymphoma. The bone marrow biopsy was typical of hypereosinophilic syndrome associated with fibrosis, with focal lymphomatous infiltrates comprised of large cells resembling the type A cells of lymphomatoid papulosis. Complete remission of the lymphoma was obtained with chemotherapy. CONCLUSIONS: This exceptional case report suggests a link between the three diseases. Lymphomatoid papulosis belongs to the spectrum of CD30 positive lymphoproliferative disorders and CD30 positive lymphocytes of lymphomatoid papulosis are known to have a Th2 profile with possible secretion of eosinopoietic cytokines.

Bone Marrow Cells↗