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Biomedical subjects

L Durand

Publications and source records attributed to L Durand.

At least 19 recordsLinked to original sources

[Aggressive T cytotoxic CD8+ epidermotropic cutaneous lymphoma: a case in a patient with Steinert's myotonic dystrophy].

INTRODUCTION: Primary cutaneous "aggressive" CD8-positive epidermotropic cytotoxic T-cell lymphoma is a rare subset of cutaneous cytotoxic T/NK lymphomas that clearly differs from mycosis fungoides, whether CD4+ or CD8+, by the presence of rapidly evolving tumoral cutaneous lesions, foci of keratinocytes necrosis, a cytotoxic T phenotype and a poor prognosis. CASE REPORT: A 33-year-old man with Steinert's myotonic dystrophy was referred for evaluation of rapidly worsening cutaneous tumors along with marked deterioration of general status. Clinical, histological and immunohistological data led to the diagnosis of primary cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma. CHOP chemotherapy was effective despite cardiac toxicity in the setting of Steinert's dystrophy, but the patient relapsed and died of pulmonary sepsis after chemotherapy was resumed. DISCUSSION: The treatment of primary cutaneous epidermotropic CD8+ cytotoxic T-cell lymphoma is not codified. CHOP chemotherapy is usually the first-line therapy but relapses are frequent with median survival of no more than 34 months. In our patient, an additional difficulty was the cardiac toxicity of cytostatic drugs linked to the myopathy which prevented the use of high dosages, requiring a change of therapeutic regimen.

Adult↗

[Capillary malformations associated with cerebral cavernous malformation].

INTRODUCTION: Capillary malformations such as benign hereditary telangiectasia are a familial affection, of dominant autosomal transmission, characterized by the progressive development of cutaneous telangiectasia during childhood. The association with cutaneous vascular, arteriovenous or lymphatic malformations is exceptional and has only recently been described. CASE REPORT: A 5 year-old girl presented with widespread erythematous, predominantly telangiectasic, congenital and acquired macules when she was one year-old. Her history was marked by right temporal cerebral hemorrhage at the age of 4, revealing a right temporal cavernoma-like vascular malformation. The familial history of telangiectasic macules and clinical and histological examination led to the diagnosis of benign hereditary telangiectasia. DISCUSSION: This case report raises doubt on the exclusively cutaneous nature of benign hereditary telangiectasic-type capillary malformations. Moreover, it illustrates the possibility of a particular clinical form of this affection, associating classical telangiectasia and post-wine stain-type macules. The recent localization of the locus implied in this affection in 5q14 in the same chromosomic space as the CMC1 locus incriminated in familial capillary malformations, suggests that these two affections are in fact phenotype variations of a single and same clinical entity.

Central Nervous System Vascular Malformations↗

[Simvastatin-induced lichen planus pemphigoides].

INTRODUCTION: Simvastatin is a competitive inhibitor of the 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase which is effective in the treatment of various hyperlipidemia. We report a case of lichen planus pemphigoides induced by simvastatin treatment. CASE REPORT: A 63-year-old man was treated for two months with simvastatin for hypercholesterolemia. One month later he developed a pruriginous and bullous lichenoid eruption. Histological and direct immunofluorescent features were consistent with the diagnosis of lichen planus pemphigoides. The Western blot analysis revealed antibodies directed against BP 180 kDa antigens. All the lesions progressively disappeared after treatment was discontinued. DISCUSSION: Lichen planus pemphigoides may be due to the intake of drugs such as cinnarizine, captopril, ramipril and furosemide. Simvastatin may induce various drug eruptions such as pruritus, eczematous rash, cheilitis, angio-oedema and urticaria, porphyria cutanea tarda, lupus-like syndrome, dermatomyositis and lichenoid eruption. With the increasing use of HMG-CoA reductase inhibitors, an association between simvastatin and lichen planus pemphigoides should be kept in mind.

Blotting, Western↗

Collagenous gastritis: a report of six cases.

Collagenous gastritis is an exceptional entity with eight cases documented to date characterized by the presence of a thick subepithelial collagen band associated with an inflammatory infiltrate of the gastric mucosa. The aim of our study was to describe the clinical and histologic characteristics of six new cases of collagenous gastritis. All cases showed a subepithelial collagen band that averaged 30 microm but often measured up to 120 microm. This finding was almost always accompanied by mixed chronic inflammation in the lamina propria and by surface epithelial damage of varying severity. Our study seems to delineate two subsets in patients with collagenous gastritis: 1) collagenous gastritis occurring in children and young adults presenting with severe anemia, a nodular pattern on endoscopy, and a disease limited to the gastric mucosa without evidence of colonic involvement, and 2) collagenous gastritis associated with collagenous colitis occurring in adult patients presenting with chronic watery diarrhea. These findings highlight the fact that subepithelial collagen deposition may be a generalized disease affecting the entire gastrointestinal tract.

Adult↗

[Association of Kaposi sarcoma--multiple myeloma. A new case].

INTRODUCTION: Kaposi's disease is an angiogenic multifocal cancer process that has several forms, namely Mediterranean, African, HIV-associated, and secondary to a preexisting immunodepressive state (hematological disorder, corticosteroid therapy, immunodepressive treatment). Whatever its form, Kaposi's sarcoma is probably associated with a chronic viral human herpes type 8 infection (HHV8). EXEGESIS: This virus has been implicated in the pathogenesis of multiple myeloma (17 cases recorded to date). In the present study, a further case of Kaposi's sarcoma associated with multiple myeloma has been reported. However, Epstein-Barr virus, cytomegalovirus, hepatitis B and C, HIV and HHV8 serologies were negative. Radiotherapy on the lower limbs was initiated. CONCLUSION: It is concluded that HHV8 does not appear to play a pathogenic role in cases of multiple myeloma, given the rarity of the association between Kaposi's sarcoma/multiple myeloma/HHV8.

Aged↗

[Amyloidoma of the central nervous system: CT and MR aspects].

PURPOSE: To report a case of tumor like amyloïd formation (amyloidoma) of the brain with etiologic discussion. MATERIAL: and Methods. A 46-year-old female had a 7 year history of epilepsy. CT scan and MRI revealed two enhancing lesions in the white matter of the right temporal and frontal lobes. RESULTS: Stereotactic biopsy examination showed large amyloid deposits surrounded by sparsely scattered lymphocytes, few matures plasma cells, macrophages and rare foreign body type giant cells. The congophilic amyloid was found in the interstitium as well as within the walls of blood vessels with close connection to the choroid plexus. No significant abnormalities were noted on routine laboratory studies. DISCUSSION: Unlike all other types of amyloid, AL amyloid can produce both systemic amyloidosis and solitary amyloidoma. Amyloidomas are rare and usually found in the lung, skin, lower urinary tract. The location in the brain is extremely rare, with only 16 cases reported in the literature. All this cases as well as our example in the current study to some extent involved deep white matter. The association with choroid plexus is possible. The relatively indolent course of this amyloidomas is noted. Our study showed a presence of monotypic lambda producing plasma cells. Thus this lesion could be the result of monoclonal B cell neoplasm capable of terminal differentiation and the existence of AL amyloid producing low grade B lymphomas of the brain could be discussed.

Amyloid↗

The Mi15 monoclonal antibody (anti-syndecan-1) is a reliable marker for quantifying plasma cells in paraffin-embedded bone marrow biopsy specimens.

Plasmocyte selective monoclonal antibodies (MAb) recognizing syndecan-1 have recently been described. They belong to a new cluster, CD138. Using the MAb MI15, we investigated the expression of syndecan-1 in routinely paraffin-embedded tissues. Nontumoral lymph nodes (25 cases) and bone marrow biopsy specimens (63 cases) showed strong membrane staining of plasma cells only, allowing accurate analysis of the nuclear structure. The MI15 positivity correlated with kappa and lambda light chain expression in the cytoplasm. The percentages of plasma cells calculated in bone marrow biopsy specimens after MI15 staining were, respectively, 2.1% (range, 1% to 4%) in normal bone marrows, 8.5% (range, 5 to 17) in reactive plasmocytosis, and 4.66% in monoclonal gammapathy of undetermined significance (MGUS) patients (range, 1 to 13), in the same range but slightly higher than those obtained on smears or on hematoxylin and eosin (H&E)-stained sections. In multiple myeloma (40 cases), all plasma cell types were marked, and Mi15 MAb gave additional information in 8 of 40 (20%) patients. In lymph nodes, Mi15 MAb reacted with Reed-Sternberg cells of classical Hodgkin's disease in 23 of 31 cases (74%) with variable intensity. In contrast, nodular lymphocyte predominance Hodgkin's disease (10 cases), most B cell lymphomas (88 of 107 cases) and all T cell lymphomas (30 cases) were negative. In B cell lymphomas, plasmocytomas (8 cases), plasmocytic lymphomas (2 cases), and 5 of 13 cases of immunoblastic lymphoma with plasmocytoid differentiation were stained. In lymphoplasmocytoid lymphomas (4 lymph nodes and 20 bone marrow biopsy specimens), only mature plasma cells were positive. Moreover, a wide distribution of syndecan-1 was observed in normal and tumoral epithelial tissues. Finally, Mi15 MAb appears to be a reliable marker for identifying and quantifying normal and tumoral plasma cells in paraffin-embedded bone marrow and lymph node samples.

Antibodies, Monoclonal↗

Hepatitis C virus genotypes and quantification of serum hepatitis C RNA in liver transplant recipients. Relationship with histologic outcome of recurrent hepatitis C.

The reasons for wide variations in the severity of recurrent hepatitis C after liver transplantation are unclear. We studied liver transplant recipients to assess the effect of hepatitis C virus (HCV) genotype and HCV RNA quantification on histologic progression of recurrent hepatitis C after transplantation. Twenty-five patients underwent transplantation for HCV cirrhosis and were followed up with virologic and histologic assessments for a mean of 51 months. HCV genotype was determined by line probe assay. HCV RNA was quantitated in serum samples by nested polymerase chain reaction. The HCV genotype 1 was detected in 17 patients and other genotypes in 8. Acute lobular hepatitis developed in 17 patients 162 days posttransplantation on average. Long-term biopsy specimens (mean, 51 months after the date of liver transplantation; range, 24-86 months) showed chronic hepatitis in 19 patients (mild, 5; moderate, 9; and severe, 5, 2 with extensive scarring). The serum alanine aminotransferase level was correlated with hepatocyte necrosis (piecemeal and lobular) but not with portal inflammation or fibrosis. Patients infected with genotype 1 had a higher Knodell score, and the 5 patients with severe hepatitis C all were infected with genotype 1. HCV RNA levels were significantly higher in patients with genotype 1 than in patients with other genotypes, as were the severity of histologic recurrence and levels of viral replication.

Adult↗

[Acute hepatitis due to ecstasy].

BACKGROUND: Ecstasy is a synthetic amphetamine which causes a wide variety of adverse effects. Hepatic toxicity was only recently demonstrated but can be quite severe. CASE REPORT: A 27-year-old male with no past medical or surgical history developed jaundice without fever. He was a regular user of ecstasy and had recently increased the number of doses consumed. No evidence of a viral, alcoholic, metabolic or autoimmune mechanism was found which could explain the hepatitis. Complete cure was obtained by discontinuing ecstasy. DISCUSSION: Few cases of ecstasy hepatic toxicity have been reported. Ecstasy was undoubtedly the causal agent in this case since other known causes of acute hepatitis were excluded, confirming the hepatotoxicity of ecstasy reported in the literature. The liver disease has been reported to range form acute regressive hepatitis to fatal liver failure. Iterative exposure can lead to fibrosis. The pathophysiological mechanism of this toxic effect is not well elucidated. Ischemia alone cannot explain all the clinical forms described, particularly cases without hyperpyrexia. Ecstasy must be added to the list of potential causes of acute hepatitis. Exposure must always be searched for in cases of acute hepatitis in young subjects.

Acute Disease↗

Identification of five new genes, closely related to the interleukin-1beta converting enzyme gene, that do not encode functional proteases.

Interleukin-1beta converting enzyme (ICE) was the first identified member of a growing family of cysteine proteases that now includes ten mammalian homologs. Within this large family, two functional proteins, denoted TX and TY share 60% amino-acid identity with ICE in the mature protein and, together with ICE, constitute the ICE subfamily. The present study describes the identification of five new gene sequences, denoted S1-S5, closely related to ICE and TX and belonging to this subfamily. Sequences were identified using genomic Southern-blot analysis of human DNA with probes corresponding to ICE and TX exon 6. Using PCR amplification and cloning, the complete exon-6 sequence of these new genes was identified; three exhibit around 90% identity with Ice within exon 6, whereas the two others share about 70% identity with Ice. Examination of open reading frames and of amino acids essential for ICE activity indicate that none of these genes encodes for a functional protease. In conclusion, extensive analysis of the genes closely related to Ice shows that the Ice subfamily is constituted of eight members. Three of them encode for functional proteases (ICE, TX and TY) whereas the remaining members probably correspond to pseudogenes.

Amino Acid Sequence↗

Diagnostic and therapeutic aspects of endoscopic retrograde cholangiography in papillomatosis of the bile ducts: analysis of five cases.

BACKGROUND: Biliary papillomatosis is an uncommon disease, the definitive diagnosis and treatment of which generally require surgery. In the present study, we described the diagnostic and therapeutic aspects of endoscopic retrograde cholangiography in five such patients. METHODS: Since 1982, five patients with biliary papillomatosis have been admitted to our unit. All these patients underwent endoscopic retrograde cholangiography. RESULTS: Endoscopic examination of the ampulla of Vater showed a dilated papillary orifice with mucin discharge in all five patients. Biliary papillomatosis was diagnosed from the following features: multiple filling defects in the lumen of the biliary system in four patients and several supple stenoses in one patient. Histologic examination of biliary specimens taken after endoscopic sphincterotomy showed typical lesions of papillomatosis in the five patients. Palliative endoscopic treatment was performed in three patients. CONCLUSION: This case series reports the typical endoscopic retrograde cholangiography and endoscopic biopsy findings in biliary papillomatosis. For inoperable patients, endoscopic palliative evacuation of mucus and debris and/or stent placement are of benefit.

Adenocarcinoma↗

Evidence for an interleukin-1beta converting enzyme (ICE)-like activity distinct from ICE and responsible for ICE and CPP32 cleavage during apoptosis.

IL-1beta converting enzyme (ICE) and ICE-related proteases (IRPs) have been suggested to play a central role in apoptosis. We report the use of peptidic ICE inhibitors to reassess the role of this enzyme in the apoptosis induced by Fas or TNFalpha receptor ligation in Jurkat cells, U937 cells or monocytes. Our results show that inhibition of IL-1beta processing can be dissociated from inhibition of apoptosis. Indeed, two out of three com-pounds active on ICE are not inhibitory for apoptosis. This shows that ICE is not required for progression in the apoptotic pathway, but that one or several IRPs are necessary. In addition, Western blot analysis of cell lysates shows that both ICE and CPP32 precursors disappear rapidly after apoptosis induction, while ICH-1L precursor remains intact. Concomitant appearance of cleavage products can be visualized for CPP32, but not for ICE, suggesting that the former is proteolytically activated. In addition, this precursor cleavage can be blocked by an ICE inhibitor active on apoptosis. Altogether, our data support the hypothesis that one or several IRPs are necessary for apoptosis and are responsible for ICE and CPP32 cleavage during this process.

Journal Article↗

Solid dermoids of the limbus and the cornea.

PURPOSE: To better define the spectrum of limbal and corneal dermoid characteristics and to obtain data on the visual outcome following surgery. METHODS: We reviewed the ocular records of 12 consecutive patients with solid dermoids which underwent surgery in our institution from 1985 to 1993. According to tumor size and location, limbal dermoids can be managed with a simple excision associated with a conjunctival flap or corneoscleral lamellar keratoplasty. RESULTS: Surgical techniques, cosmetic and visual outcomes are documented. Limbal dermoids were more noticeable cosmetically, and they often induced oblique astigmatism. In 6 cases, they could easily be shaved off the cornea and adjacent sclera to improve the appearance of the eye; visual acuity was unchanged. In 3 cases, unfortunately a degree of refractive amblyopia remained after late surgery and visual acuity was inferior to 20/200. In 2 large-diameter limbal dermoids, early corneolamellar keratoplasty allowed to improve best corrected visual acuity. In the corneal dermoid, amblyopia was the indication for early surgical removal. CONCLUSION: We believe that early surgery with simple local resection (combined with a conjunctival flap in order to cover the bare area) may be preferred to lamellar keratoplasty. Nevertheless, corneoscleral lamellar grafts may be required in advanced cases, especially when the whole thickness of the corneal stroma is involved or when the tumor deeply extends around the limbus.

Child↗