Monique Fabrè

Assistance Publique – Hôpitaux de Paris

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Catherine Guettier

Assistance Publique – Hôpitaux de Paris

Sophie Branchereau

Bicêtre Hospital

Emmanuel Jacquemin

Inserm

Olivier Bernard

Université de Poitiers

Laurence Brugières

Université Paris-Saclay

Frédéric Gauthier

Bicêtre Hospital

Dominique Debray

Centre de Recherche Saint-Antoine

Jessica Zucman‐Rossi

Centre de Recherche des Cordeliers

Thierry Lazure

Bicêtre Hospital

Danièle Pariente

Bicêtre Hospital

Cooperative Institutions

Inserm

748

Assistance Publique – Hôpitaux de Paris

543

Université Paris Cité

487

Sorbonne Université

294

Centre National de la Recherche Scientifique

290

Bicêtre Hospital

264

European Foundation for the Study of Chronic Liver Failure

215

Canon (Japan)

215

Université Paris-Saclay

180

Sorbonne Paris Cité

177

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Complications of Congenital Portosystemic Shunts in Children: Therapeutic Options and Outcomes

Journal of Pediatric Gastroenterology and Nutrition (2010)

Stéphanie Franchi‐Abella Sophie Branchereau Virginie Lambert Monique Fabrè Clarisa Steimberg

ABSTRACT Background and Objective: Congenital portosystemic shunts are rare vascular malformations that lead to severe complications. Their management is controversial. The aim of this study was to propose a clear definition of the risks and management of congenital portosystemic shunts in children according to our experience and a review of the literature. Patients and Methods: Twenty‐two children with a complicated congenital portosystemic shunt were studied in our institution. When necessary, management included portal pressure measurement and portal vein angiography during an occlusion test and closure of the shunt by surgical and/or endovascular methods. Results: Five neonates with intrahepatic shunts presented with cholestasis that resolved spontaneously, and 17 older children presented with liver tumors (13) and/or hepatopulmonary syndrome (2), pulmonary artery hypertension (3), portosystemic encephalopathy (3), heart failure (1), and glomerulonephritis (1). The portosystemic shunt was extrahepatic (11) or intrahepatic (6). Portosystemic shunts were closed by endovascular methods in 5 children and surgically in 10, 4 of whom had portal pressure during occlusion above 35 mmHg and extremely hypoplastic or undetectable portal veins requiring banding of the fistula before closure. Shunt closure resulted in restoration of intrahepatic portal flow in all, with complete or partial regression of benign liver masses, and regression or stabilization of pulmonary, cardiac, neurological, and renal complications. Conclusions: Congenital portosystemic shunt carries risks of severe complications in children. Closure of a shunt persisting after age 2 years should be considered preventively. Intrahepatic portal flux restoration can be expected, even when intrahepatic portal veins are extremely hypoplastic or undetectable.

Portosystemic shunt

Hepatic Encephalopathy

Alagille Syndrome

Hepatopulmonary syndrome

Congenital hepatic fibrosis

10.1097/mpg.0b013e3181d9cb92

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Citations (238)

Table of Contents

Gastroenterology (2009)

Lloyd P. Gerson Geoffrey C. Nguyen C. Daniel Prather M. A. Benninga Emeran A. Mayer

Table (database)

10.1053/s0016-5085(09)01517-0

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Citations (0)

Transcription factors involved in pancreas development are expressed in paediatric solid pseudopapillary tumours

Histopathology (2008)

Louise Galmiche Sabine Sarnacki Virginie Verkarre Brigitte Boizet Bertrand Duvillié

Solid pseudopapillary tumours (SPT) are rare pancreatic tumours, especially in children. The origin of this benign tumour remains unknown. Mutations of beta-catenin, a gene essential for pancreatic development, are constantly found, leading to delocalization of immunohistochemical signals from the cytoplasm to the nuclei of tumour cells. The aim was to report clinical and histological data of eight children with SPT and explore the immunohistochemical expression of pancreatic duodenal homeobox (PDX) 1 and Sox9, known to be crucial for pancreatic development and linked to the beta-catenin cascade.Eight children with features suggestive of SPT underwent surgical resection. Tumours displayed typical histological appearances. One was incompletely resected and recurred. Immunolabelling revealed nuclear location of beta-catenin in all cases and strong cytoplasmic but no nuclear expression of PDX1 or Sox9 in all but one case.The clinical behaviour of SPT in the paediatric population is similar to its adult counterpart. Complete surgical resection is essential. PDX1 and Sox9 proteins are exclusively expressed in the cytoplasmic compartment in SPT, suggesting overexpression of the corresponding genes linked to beta-catenin mutations. These findings favour the hypothesis that SPT originates from transformation of normally quiescent pancreatic stem cells.

PDX1

10.1111/j.1365-2559.2008.03108.x

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Citations (16)

Erdheim-Chester Disease in Childhood

Journal of Pediatric Hematology/Oncology (2009)

Tú-Anh Tran Monique Fabrè Danièle Pariente Irina Craiu Julien Haroche

Erdheim-Chester disease is a rare, non-Langerhans systemic histiocytosis characterized by bilateral sclerosis of the metaphyseal regions of the long bones and infiltration in other organs. The histopathologic hallmark is defined by a mononuclear infiltrate of foamy histiocytes and rare pathognomonic Touton giant cells with extensive fibrosis. This condition is exceptional in children. We report here a case of Erdheim-Chester disease in a 10-year-old girl with retroperitoneal infiltration and bone involvement, for whom the diagnosis was only established after a 3-year course with multiple biopsies. It is also the first pediatric case successfully treated with interferon-α suggesting that interferon-α can be a safe and efficient first-line therapy for this disease in children.

Erdheim–Chester disease

Pathognomonic

Infiltration (HVAC)

Rare disease

10.1097/mph.0b013e3181b76827

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Citations (57)

[Macronodules, dysplastic nodules and cirrhosis].

PubMed (2001)

B Cochand‐Priollet Monique Fabrè

Nodule (geology)

Source

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Citations (2)

Les anticorps anti-thrombomoduline induisent une endocytose médiée par récepteur sur des cellules endothéliales humaines

C Brisson G Archipoff M Hartmann Daniel Hanau Alain Beretz

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Citations (0)

Anti‐CD20 Monoclonal Antibody (Rituximab) Treatment for Epstein‐Barr Virus‐associated, B‐cell Lymphoproliferative Disease in Pediatric Liver Transplant Recipients

Journal of Pediatric Gastroenterology and Nutrition (2002)

Marie Odile Serinet Emmanuel Jacquemin Dalila Habès Dominique Debray Monique Fabrè

ABSTRACT Objectives Anti–B‐cell immunotherapy has been used with success in adults with posttransplant B‐cell lymphoproliferative disease (PTLD), but such treatment has rarely been reported in children. We report the outcome of anti–CD20 antibody (rituximab) therapy for Epstein‐Barr virus (EBV)–associated PTLD in six pediatric liver transplant recipients. Methods In these six patients, PTLD was diagnosed within 2 to 4 months after transplantation. The tumors were classified as monomorphic or polymorphic B‐cell infiltrate expressing CD20 antigen and EBV genome. Anti‐CD20 therapy was associated with withdrawal of tacrolimus or ciclosporine therapy in all patients. Intravenous rituximab was administered at 375 mg/m 2 once a week for 3 to 4 consecutive weeks. Results Rituximab treatment was associated with decreased EBV load, disappearance of abnormal serum immunoglobulin concentration, and disappearance of tumoral masses, which occurred 1 to 2.5 months after treatment onset. Despite rituximab therapy, one patient was diagnosed subsequently with a cerebral tumor. Five patients experienced acute liver graft rejection episodes within 10 days to 2.5 months after beginning treatment. In these patients, immunosuppression was reintroduced, but three children experienced fatal chronic rejection, whereas two experienced complete tumor remission. Three children are alive and in complete remission, with normal liver tests, 15 months to 3 years after PTLD onset. Conclusions Rituximab therapy is an interesting approach for children with early EBV‐associated PTLD after liver transplantation. It does not prevent cerebral localization, and rapid resumption of immunosuppression may be advisable to prevent lethal chronic liver graft rejection.

Immunosuppression

Post-transplant lymphoproliferative disorder

Lymphoproliferative Disorders

10.1002/j.1536-4801.2002.tb07657.x

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Citations (2)

Macrocystic pancreatic cystadenoma: the role of EUS and cyst fluid analysis in distinguishing mucinous and serous lesions

Gastrointestinal Endoscopy (2004)

Dermot O’Toole Laurent Palazzo Pascal Hammel Lamia Ben Yaghlène Anne Couvelard

Serous Cystadenoma

Mucinous cystadenoma

Cystadenoma

Carcinoembryonic antigen

Cystadenocarcinoma

10.1016/s0016-5107(04)00346-3

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Citations (151)

[Unusual site of an embryonal rhabdomyosarcoma of the mesenchymal hepatic pedicle].

PubMed (1999)

Sophie Ferlicot J Quillard Christophe Chardot Bernard Caillou Odile Oberlin

Desmin

Embryonal rhabdomyosarcoma

Immunostaining

Source

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Citations (5)

Bone Marrow Transplantation in Congenital Erythropoietic Porphyria: Sustained Efficacy but Unexpected Liver Dysfunction

Biology of Blood and Marrow Transplantation (2019)

Caroline Besnard Caroline Schmitt Louise Galmiche Dominique Debray Monique Fabrè

Congenital erythropoietic porphyria (CEP) is a rare disease characterized by erosive photosensitivity and chronic hemolysis due to a defect of the enzyme uroporphyrinogen-III-synthase (UROS). To date, hematopoietic stem cell transplantation (HSCT) is the only curative therapy for the devastating early and severe form of the disease. We describe 6 patients with CEP treated with HSCT (3 of them twice after failure of a first graft) between 1994 and 2016 in our center, including 2 of the very first living patients treated more than 20 years ago. Four patients are doing well at 6 to 25 years post-HSCT, with near-normal biochemical parameters of porphyrin metabolism without the cutaneous or hematologic features of CEP. One patient died within the first year after HSCT from severe graft-versus-host disease (GVHD), and 1 child died of unexplained acute hepatic failure at 1 year after HSCT, despite full donor chimerism. Retrospectively, it appears that all but 1 child had increased transaminase activity with onset from the early postnatal period, which was significantly more marked in the child who died of liver failure. In contrast, liver function values progressively normalized after engraftment in all other children. Liver pathology before HSCT for 3 patients revealed varying degrees of portal, centrilobular, and perisinusoidal fibrosis; clarification of hepatocytes; and cytosolic porphyrin deposits. The liver porphyrin content in biopsy specimens was >60 times the normal values. Despite difficult engraftment, the long-term efficacy of HSCT in CEP appears to be favorable and reinforces its benefits for the severe form of CEP. Hepatic involvement requires careful evaluation before and after HSCT and further investigation into its pathophysiology and care.

Liver disease

10.1016/j.bbmt.2019.12.005

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Citations (16)