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J F Emile

Publications and source records attributed to J F Emile.

70 records · Page 4Linked to original sources
PubMed1995

Expression of GM-CSF receptor by Langerhans' cell histiocytosis cells.

Langerhans' cell histiocytosis (LCH) is characterized by the proliferation of large mononucleated cells containing Birbeck granules and expressing CD1a. Recent studies have demonstrated that LCH is a clonal proliferation; however, its aetiology is still unknown. Growth and differentiation of bone-marrow-derived cells are controlled by cytokines. The proliferation, differentiation and activation of normal Langerhans cells are controlled by granulocyte/macrophage colony-stimulating factor (GM-CSF) in vitro. Therefore, GM-CSF could be implicated in the pathogenesis of LCH. Indeed, LCH cells contain GM-CSF, and children with disseminated LCH have an elevated GM-CSF serum level. As a cytokine only acts on cells expressing a specific receptor, we investigated the presence of GM-CSF receptor on LCH cells. Fourteen frozen tissue samples from children with LCH were studied by in situ immunohistochemistry with two mouse monoclonal antibodies specific for the alpha chain of the GM-CSF receptor (CDw116). LCH cells of all the samples were positively stained with both antibodies. This study suggests that GM-CSF may be a growth factor for LCH cells.

Animals↗
PubMed1995

Langerhans' cell histiocytosis. Definitive diagnosis with the use of monoclonal antibody O10 on routinely paraffin-embedded samples.

The histological and immunohistochemical findings of 34 biopsy specimens from patients with Langerhans' cell histiocytosis (LCH) are reported, with special emphasis on the findings with CD1a mouse monoclonal antibody (MAb) O10 using paraffin-embedded material. Eighteen patients were treated in an adult hospital (mean age, 26.3 years), and the 16 others were children (mean age, 3 years) from a pediatric center. Specimens included 17 bone, 14 skin, two lung, and one lymph node. Tissue was fixed in formalin or Bouin's, and most bone samples were decalcified in nitric acid. Frozen sections were available for 16 cases and electron microscopy for one. Light microscopy was suggestive of LCH in all cases, characterized by large mononucleated cells with abundant eosinophilic cytoplasm and "coffee bean" nucleus. In 33 of the 34 paraffin-embedded LCH samples, mononucleate cells were stained by MAb O10. As controls, we investigated seven tumors expressing S-100 protein (three nevi, two melanomas, two neurofibromas): all were negative with MAb O10. Five non-Langerhans' cell histiocytoses (three juvenile xanthogranulomas and two Rosai-Dorfman lymphadenopathies) were also negative with MAb O10. The results show that in most cases a definitive diagnosis of LCH can be assessed on paraffin-embedded tissue specimens with the help of immunohistochemistry using MAb O10.

Adult↗
PubMed1994

Langerhans' cell histiocytosis cells are activated Langerhans' cells.

Langerhans' cell histiocytosis (LCH) is characterized by the presence of large mononucleated cells, associated with inflammatory cells. The Langerhans' cell (LC) lineage of the mononucleated cells is suggested by the presence of Birbeck granules and the expression of CD1a. We investigated the presence of 14 markers expressed by normal LCs in vitro. Nine skin and one lymph node frozen biopsies of LCH children were analysed by in situ immunohistochemistry. The data were compared with six skin and five lymph node frozen biopsies. LCH cells of the ten samples were positive for all 14 LC markers. We observed three different groups of markers, according to the respective staining of normal LCs and LCH cells. Group 1 included DR, DQ, CD1a, CD1c, and ICAM-3. Markers of group 1 were present on the majority of both normal LCs and LCH cells. Group 2 included CD1b, CD4, LFA-1, LFA-3, CD32, and CD68. Markers of group 2 were detected on the majority of LCH cells, but only on a fraction of normal LCs. Group 3 included CD11b, CD24, and B7/BB1. Markers of this group were detected on LCH cells, but not on normal LCs. This in situ immunohistochemical study confirms that LCH cells belong to the LC lineage. The different clinical LCH syndromes had the same immunohistochemical staining. The expression of some markers of groups 2 and 3 is known to be related to the activation of LCs in vitro. Our study suggests that LCH cells are activated LCs.

Antibodies, Monoclonal↗
PubMed1994

Detection of GM-CSF in the sera of children with Langerhans' cell histiocytosis.

GM-CSF induces proliferation and activation of Langerhans' cells in vitro. The density of Langerhans' cells in human tumours is correlated to the in situ density of GM-CSF, and intradermal injection of GM-CSF induces local accumulation of Langerhans' cells. Therefore, we investigated the presence of GM-CSF in the sera of children with Langerhans' cell histiocytosis (LCH). We detected GM-CSF in the sera of all children with disseminated and active LCH, but not in the sera of patients with localized (i.e. bone) LCH. These results suggest that GM-CSF level is related to extent and the activity of LCH.

Adolescent↗
PubMed1993

The presence of epithelioid granulomas in hepatitis C virus-related cirrhosis.

We examined surgical liver specimens from 52 patients with hepatitis C virus-related cirrhosis. All patients underwent orthotopic liver transplantation at Paul Brousse Hospital. They were found to be seropositive for antibodies to hepatitis C virus by second-generation testing (RIBA 2, Ortho Diagnostic Systems Inc, Westwood, MA). We detected multiple granulomas in five (10%) of the cirrhotic livers. These granulomas were composed of epithelioid cells, sometimes associated with multinucleated giant cells, and were surrounded by small lymphocytes and fibrosis. The epithelioid granulomas were located within the cirrhotic nodules. They were not present within the portal tracts or within the fibrosis. These granulomas were diffusely distributed in the liver. None of the patients with diffuse hepatic epithelioid granulomas had evidence of tuberculosis or brucellosis before transplantation or during the follow-up period (range, 3 to 20 months). They had no detectable cause of granulomatous hepatitis. The role of hepatitis C virus as a cause of epithelioid granulomas is discussed.

Adult↗
PubMed1993

Immunohistochemical detection of granulocyte/macrophage colony-stimulating factor in Langerhans' cell histiocytosis.

Granulocyte/macrophage-colony stimulating factor (GM-CSF) induces in vitro activation of Langerhans' cells. The association of GM-CSF and tumour necrosis factor alpha (TNF alpha) induces the differentiation of Langerhans' cells from CD34 positive haematopoietic progenitors. Intradermal administration of recombinant GM-CSF is associated with local accumulation of Langerhans' cells. We investigated the presence of GM-CSF in tissue samples of 10 patients with Langerhans' cell histiocytosis. Four patients had skin involvement, three had bone and three had diffuse disease. Eight normal skin samples were analysed as controls. Immunohistochemistry was performed on frozen tissue samples with two specific monoclonal antibodies directed against two different epitopes of GM-CSF. We detected GM-CSF in all the histiocytosis tissue samples. The GM-CSF was detected within the cytoplasm of all the tumoral Langerhans' cells. We did not find GM-CSF in any other cell type. These results suggest that GM-CSF may be implicated in the pathogenesis of Langerhans' cell histiocytosis.

Antigens, CD↗
PubMed1993

[Myxoma of the pulmonary infundibulum as the cause of sudden death in an 18-month-old infant].

BACKGROUND: Myxoma, a benign cardiac tumor, develops in intracavitary locations. It is most frequent in the left atrium, but rare in the pulmonary infundibulum. CASE REPORT: An 18 month-old girl suddenly became cyanotic during playing and lost consciousness. Resuscitation was ineffective. Post-mortem examination showed diffuse pulmonary edema and multiple small pulmonary emboli. There was a myxoma of the septal wall of pulmonary infundibulum and histological examination showed that the infundibulum and the pulmonary valve were completely infiltrated by myxoid tissue. CONCLUSION: The death of this girl was probably due to a sudden embolism of blood clots developed in contact with the myxoma. This case emphasizes the need for pathologic investigation in all cases of sudden death.

Female↗
PubMedDate not supplied

[Combined transplantation of liver and small intestine in an adult. First case in France. Surgical aspects].

BACKGROUND: We report hare the first adult case of combined liver-small bowel transplantation performed in France. CASE REPORT: A double liver + small bowel graft was transplanted in a 21-year-old patient hospitalized for 4 years for a short bowel syndrome requiring total parenteral nutrition. The patient also had severe hepatic fibrosis. The immediate post-operative period was uneventful. Two and one-half years after the double graft, the patient is on strictly oral nutrition, no longer has a stomy and lives a normal life in his home. DISCUSSION: The advent of tacrolimus has led to long-term success of bowel grafts, developed earlier in children and now possible in adults. Combined liver-small bowel transplantation is formally indicated in patients with cirrhogenic liver disease associated with ineversible small bowel failure.

Adult↗
PubMedDate not supplied

[Combined liver and small intestine transplantation in an adult. First case in France. Medical aspects: digestive and nutritional].

OBJECTIVE: We report the digestive, hepatic, intestinal and nutritional history of the first combined liver-small bowel transplantation performed in France in an adult recipient. Currently, the clinical course has been favorable at 30 months. METHODS: In May 1997, a 21-year-old man underwent a double liver-small bowel transplantation for irreversible chronic bowel failure with severe chronic liver disease subsequent to post-surgical short-bowel syndrome. After 28 laparotomies, there were 15 cm of small bowel left for the gastric anastomosis. The patient had a terminal jejunostomy and a left colostomy, excluding a short segment of the transverse colon and the rectum. Total parenteral nutrition including lipids and been initiated in December 1992 (3500 kcal/d) and had led to severe complications. Intestinal absorption before and after the graft were studied with the balance method (input-output) and used bomb calorimetry and measurements of the steatorhhea and creatorrhea. Functional enterocyte mass was assessed from serum citrullin. RESULTS: The postoperative period after the liver-bowel graft (220 cm small bowel with terminal ileostomy and jejunostomy) was uneventful. The immunosuppression protocol included tacrolimus, corticosteroids and azathioprin. One mild episode of acute rejection occurred at day 26 and was controlled with a corticosteroid bolus. No episode of liver rejection occurred. Moderate renal failure regressed partially after reestablishing the fluid-electrolyte balance and adapting tacrolimus dosage. Total parenteral nutrition which had sustained the patient for 4 and a half years was definitely discontinued three months after transplantation. Oral nutrition was initially associated with enteral nutrition (from day 20 to day 90) and became exclusive three months after the transplantation. Intestinal absorption coefficients measured before tranplantation, at 3 months (2200 kcal/d, ileostomy flow 1600 g/d), and at 18 months (2400 kcal/d, ileostomy flow 1300 g/d) post transplantation were, respectively, 22%, 90% and 88% for overall calorie absorption, 25%, 65% and 73% for fat absorption, and 47%, 83% and 67% for nitrogen absorption. At 18 months post-graft, there was a spectacular improvement in the patient's neurological status and his liver function was normal. Endoscopy, radiography, histology, and immunohistochemistry explorations were normal. Ileo-rectal anastomosis was re-established at 23 months post-transplantation. At 30 months the patient is living in his home and on 100% oral nutrition. CONCLUSION: Clinical, nutritional and functional outcome at 30 months in this first French case of liver-small bowel transplantation in an adult recipient has been excellent.

Adult↗