45 results match your criteria putterman m

Multimodal imaging of Hurler syndrome-related keratopathy treated with deep anterior lamellar keratoplasty.

BMC Ophthalmol 2020 Oct 31;20(1):433. Epub 2020 Oct 31.

GRC32, Transplantation et Thérapies Innovantes de la Cornée, Sorbonne université, Centre Hospitalier National d'Ophtalmologie des 15-20, 28 rue de Charenton, 75571 Cedex 12, Paris, France.

Background: Hurler syndrome-associated keratopathy is an exceedingly rare corneal disorder that requires corneal transplantation in advanced stages. Precise assessment of the corneal condition is necessary for deciding which type of keratoplasty (i.e. Read More

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October 2020

Effects of Hydrophobic Tail Length Variation on Surfactant-Mediated Protein Stabilization.

Mol Pharm 2020 11 15;17(11):4302-4311. Epub 2020 Oct 15.

Department of Chemistry, University of Minnesota, Minneapolis, Minnesota 55455-0431, United States.

Recently, protein therapeutics have gained significant attention as a result of their enhanced selectivity and diminished side effects compared to traditional small-molecule drugs. Despite their advantages, protein formulations typically suffer from stability issues because of aggregation and denaturation during production and storage, often resulting in detrimental immune responses. Surfactants can be used to stabilize and protect proteins in solution by preventing protein adsorption onto interfaces or by forming protective structures in solution. Read More

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November 2020

Intravoxel incoherent motion (IVIM) 3 T MRI for orbital lesion characterization.

Eur Radiol 2021 Jan 1;31(1):14-23. Epub 2020 Aug 1.

Université de Paris, PARCC, INSERM, F-75015, Paris, France.

Objectives: To determine the diagnostic accuracy of MRI intravoxel incoherent motion (IVIM) when characterizing orbital lesions, which is challenging due to a wide range of locations and histologic types.

Methods: This IRB-approved prospective single-center study enrolled participants presenting with an orbital lesion undergoing a 3-T MRI prior to surgery from December 2015 to July 2019. An IVIM sequence with 15 b values ranging from 0 to 2000 s/mm was performed. Read More

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January 2021

Sostdc1 is expressed in all major compartments of developing and adult mammalian eyes.

Graefes Arch Clin Exp Ophthalmol 2019 Nov 16;257(11):2401-2427. Epub 2019 Sep 16.

Centre de Recherches des Cordeliers, UMR_S INSERM 1138, Equipe 17, Université Paris Descartes, 15 rue de l'école de médecine, 75006, Paris, France.

Purpose: This study was conducted in order to study Sostdc1 expression in rat and human developing and adult eyes.

Methods: Using the yeast signal sequence trap screening method, we identified the Sostdc1 cDNA encoding a protein secreted by the adult rat retinal pigment epithelium. We determined by in situ hybridization, RT-PCR, immunohistochemistry, and western blot analysis Sostdc1 gene and protein expression in developing and postnatal rat ocular tissue sections. Read More

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November 2019

Dirofilariasis: a parasitic cause of subcutaneous nodule.

Clin Microbiol Infect 2019 Oct 20;25(10):1231-1232. Epub 2019 Jun 20.

Sorbonne Université, Inserm, Centre de Recherche Saint-Antoine, CRSA, AP-HP, Hôpital Saint-Antoine, Paris, France. Electronic address:

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October 2019

Evolutionary Routes in Metastatic Uveal Melanomas Depend on Alterations.

Clin Cancer Res 2019 09 21;25(18):5513-5524. Epub 2019 Jun 21.

Institut Curie, PSL Research University, INSERM U830, Paris, France.

Purpose: Uveal melanomas (UM) are genetically simple tumors carrying few copy number alterations (CNA) and a low mutation burden, except in rare -deficient, hypermutated cases. The genomics of uveal melanoma metastatic progression has not been described. We assessed the genetic heterogeneity of primary and metastatic -proficient and -deficient uveal melanomas. Read More

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September 2019

Development of Low Frequency (20-100 kHz) Clinically Viable Ultrasound Applicator for Chronic Wound Treatment.

IEEE Trans Ultrason Ferroelectr Freq Control 2019 03 14;66(3):572-580. Epub 2018 May 14.

This paper details the systematic approach used to develop a viable clinical prototype of a therapeutic ultrasound applicator and discusses the rationale and deliberations that led to the design strategy. The applicator was specifically devised to treat chronic wounds and-to the best of the author's knowledge-is the first truly wearable device with a proven record of reducing healing time, directly translating to a reduction of healthcare costs. The prototype operates in the kHz (20-100) range of frequencies and uses noncavitational and nonthermal levels of ultrasound energy. Read More

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Outlier response to anti-PD1 in uveal melanoma reveals germline MBD4 mutations in hypermutated tumors.

Nat Commun 2018 05 14;9(1):1866. Epub 2018 May 14.

Institut Curie, PSL Research University, INSERM U830, DNA Repair and Uveal Melanoma (D.R.U.M.), Equipe labellisée par la Ligue Nationale contre le Cancer, Paris, 75248, France.

Metastatic uveal melanoma is a deadly disease with no proven standard of care. Here we present a metastatic uveal melanoma patient with an exceptional high sensitivity to a PD-1 inhibitor associated with outlier CpG>TpG mutation burden, MBD4 germline deleterious mutation, and somatic MBD4 inactivation in the tumor. We identify additional tumors in The Cancer Genome Atlas (TCGA) cohorts with similar hypermutator profiles in patients carrying germline deleterious MBD4 mutations and somatic loss of heterozygosity. Read More

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[Orbital rhabdomyosarcoma associated with a varicella eruption].

J Fr Ophtalmol 2017 Nov 9;40(9):e349-e351. Epub 2017 Oct 9.

Service de chirurgie plastique et reconstructrice ophtalmologique, Fondation Rothschild Paris, Paris, France.

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November 2017

Recurrent lacrimal gland choristoma of the ciliary body in an infant mimicking a medulloepithelioma.

Eur J Ophthalmol 2016 Nov 4;26(6):e145-e148. Epub 2016 Nov 4.

 Department of Pathology, Necker-Enfants Malades Hospital, Paris - France.

Purpose: Choristoma is a congenital tumor made up of ectopic normal tissue. Different histopathologic subtypes have been described. Among them, lacrimal gland choristoma is found mainly in infants and can affect the iris, the ciliary body, or the choroid and epibulbar region. Read More

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November 2016

[Aneurysmal bone cyst: A rare cause of orbital disease].

J Fr Ophtalmol 2016 Jun 8;39(6):498-505. Epub 2016 Jun 8.

Institut Curie, 26, rue d'Ulm, 75248 Paris, France; Université Paris Descartes, 15, rue de l'École de Medecine, 75270 Paris cedex 06, France.

Aneurysmal bone cyst is a rare benign bone neoplasm of unknown cause. The most commonly affected anatomical sites are the vertebral column and long bones. We report two uncommon cases of primary orbital aneurysmal bone cyst presenting as an acute orbital compartment syndrome due to subperiosteal hemorrhage. Read More

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Usefulness of colour Doppler flow imaging in the management of lacrimal gland lesions.

Eur Radiol 2017 Feb 7;27(2):779-789. Epub 2016 Jun 7.

Department of Radiology, Fondation Ophtalmologique Adolphe de Rothschild, 25 rue Manin, 75019, Paris, France.

Objective: To assess the role of colour Doppler flow imaging (CDFI) in the diagnosis and management of lacrimal fossa lesions.

Methods: Institutional ethical committee approval was obtained. Fifty-one patients with 62 lacrimal fossa lesions were retrospectively included from 2003-2015. Read More

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February 2017

The double-histidine Cu²⁺-binding motif: a highly rigid, site-specific spin probe for electron spin resonance distance measurements.

Angew Chem Int Ed Engl 2015 May 27;54(21):6330-4. Epub 2015 Mar 27.

Department of Chemistry, University of Pittsburgh, 219 Parkman Avenue, Pittsburgh, PA 15260 (USA).

The development of ESR methods that measure long-range distance distributions has advanced biophysical research. However, the spin labels commonly employed are highly flexible, which leads to ambiguity in relating ESR measurements to protein-backbone structure. Herein we present the double-histidine (dHis) Cu(2+)-binding motif as a rigid spin probe for double electron-electron resonance (DEER) distance measurements. Read More

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Cysteine-specific Cu2+ chelating tags used as paramagnetic probes in double electron electron resonance.

J Phys Chem B 2015 Feb 30;119(7):2839-43. Epub 2015 Jan 30.

Department of Chemistry, University of Pittsburgh , 219 Parkman Avenue, Pittsburgh, Pennsylvania 15260, United States.

Double electron electron resonance (DEER) is an attractive technique that is utilized for gaining insight into protein structure and dynamics via nanometer-scale distance measurements. The most commonly used paramagnetic tag in these measurements is a nitroxide spin label, R1. Here, we present the application of two types of high-affinity Cu(2+) chelating tags, based on the EDTA and cyclen metal-binding motifs as alternative X-band DEER probes, using the B1 immunoglobulin-binding domain of protein G (GB1) as a model system. Read More

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February 2015

[Hyper-IgG4 disease presenting with lid involvement].

J Fr Ophtalmol 2014 Feb 5;37(2):e19-21. Epub 2013 Nov 5.

Service de chirurgie oculopalpébrale, fondation ophtalmologique Adolphe-de-Rothschild, 25-29, rue Manin, 75019 Paris, France.

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February 2014

[Management of orbital cavernous hemangioma - evaluation of surgical approaches: report of 43 cases].

J Fr Ophtalmol 2013 Dec 10;36(10):820-9. Epub 2013 Oct 10.

Service de chirurgie reconstructive orbito-palpébrale, fondation Rothschild, 25, rue Manin, 75019 Paris, France. Electronic address:

Cavernous hemangioma is the most frequent benign orbital tumor in adults. The purpose of this study was to examine its clinical features, to define surgical indications, and to determine the roles of the various surgical approaches praticed in ophthalmology: transconjunctival (increasingly utilized), anterior transcutaneous, and lateral orbitotomy. The records of all patients treated for orbital cavernous hemangioma (OCH) since 2004 at the Fondation Rothschild (Paris, France) were retrospectively reviewed. Read More

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December 2013

Teaching neuroimages: IgG4-related orbital disease and enlargement of the trigeminal nerve branches.

Neurology 2013 Oct;81(15):e117-8

From the Departments of Neurology (R.D., O. Gout) and Ophthalmology (C.V., O. Galatoire), Fondation Ophtalmologique Adolphe de Rothschild; Department of Pathology (L.D.), Bichat Hospital, APHP; and Department of Pathology (M.P.), Necker-Enfants Malades Hospital, APHP, Paris, France.

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October 2013

High prevalence of IgG4-related lymphoplasmacytic infiltrative disorder in 25 patients with orbital inflammation: a retrospective case series.

Br J Ophthalmol 2013 Aug 12;97(8):999-1004. Epub 2013 Jun 12.

Department of Neurology, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France.

Aims: To evaluate retrospectively the prevalence of positive IgG4-immunostaining in orbital tissue of patients with idiopathic orbital inflammation and to compare the clinical, radiographic and pathologic features among patients with and without IgG4-positive cells.

Patients And Methods: 25 patients with biopsy-proven idiopathic orbital inflammation examined from January 2006 through December 2011 were included. Immunohistochemistry with IgG and IgG4 immunostaining from biopsy specimens of all patients was performed. Read More

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[Evaluation of reconstructive techniques after orbital exenteration in 56 cases].

J Fr Ophtalmol 2012 Nov 19;35(9):667-77. Epub 2012 Sep 19.

Service de chirurgie reconstructive orbito-palpébrale, fondation Rothschild, 25-29, rue Manin, 75019 Paris, France.

Purpose: To describe the management of orbital exenterations and the surgical techniques for the reconstruction of orbital exenteration cavities.

Patients And Methods: This retrospective study includes 56 patients who underwent orbital exenteration between 2000 and 2009. Patients' age at the time of exenteration, diagnoses, complications and reconstructive techniques were studied. Read More

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November 2012

Superficial punctate keratitis and conjunctival erosions associated with congenital tufting enteropathy.

Am J Ophthalmol 2010 Jul 5;150(1):116-121.e1. Epub 2010 May 5.

Department of Ophthalmology, Necker-Enfants Malades Hospital, Hôpitaux de Paris, University René Descartes-Paris V, France.

Purpose: To study the value of conjunctival biopsy in congenital tufting enteropathy diagnosis.

Design: Case-comparative study.

Methods: Between January 2000 and June 2007, all children seeking treatment with an early onset of intractable diarrhea were examined in the ophthalmology department of Necker-Enfants Malades Hospital, Assistance Publique-Hôpitaux de Paris, France. Read More

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[Surgical treatment of diffuse adult orbital lymphangioma: two case studies].

J Fr Ophtalmol 2008 Dec;31(10):1006-17

Fondation ophtalmologique Rothschild, Paris.

Purpose: Orbital lymphangioma is a rare vascular malformation; it is a benign but severe anomaly because of its infiltrative, diffuse, and hemorrhagic nature, and its high morbidity rate. Surgical resection is a real challenge on account of the intricate architecture of the lesion. The authors report their surgical experience concerning two cases of diffuse orbital lymphangioma whose diagnosis was established in adulthood and whose surgical treatment was successful. Read More

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December 2008

Luteinizing hormone pulsatility in patients with major ovarian hyperandrogenism.

J Endocrinol Invest 2007 Sep;30(8):636-46

Endocrinology, Pitié-Salpétrière Hospital, 83 boulevard de l'Hôpital, 75013, Paris, France.

Hyperandrogenism and ovulatory dysfunction are common in women with either polycystic ovary (PCOS) or ovarian virilizing tumor. However, contrasting with the numerous studies that have extensively described gonadotropin secretory abnormalities, principally increased LH pulse amplitude and frequency, few studies have concerned gonadotropin secretion in patients with ovarian virilizing tumors; low gonadotropin levels have occasionally been reported, but never extensively studied. The goal of the present study was to further evaluate the pulsatility of LH secretion in women with ovarian virilizing tumor compared with that of PCOS patients. Read More

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September 2007

Eosinophilic angiocentric fibrosis of the sinonasal tract in a male patient with chronic bowel inflammation.

Am J Rhinol 2006 Jan-Feb;20(1):91-4

Department of Otolaryngology Head and Neck Surgery, Soroka University Medical Center, Ben-Gurion University of the Negev, Beer-Sieva, Israel.

Background: Eosinophilic angiocentric fibrosis (EAF) is an uncommon inflammatory fibrosing lesion involving the upper respiratory mucosa, occurring mainly in young to middle aged women (female/male ratio = 2:1). The etiology is unknown; however, severalfactors might play a role in the development of EAF. Among them are prior nasal trauma (in most of the reported cases nasal surgery had been performed afew years prior to diagnosis) and inflammatory or autoimmune etiology (suggested by the ratio and the fact that in many of the reported cases a history of nonspecific allergy was found). Read More

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[Adenocarcinoma in a pleomorphic adenoma of the lacrimal gland: a case study].

J Fr Ophtalmol 2005 Oct;28(8):896-901

Fondation Ophtalmologique A. de Rothschild, 75019 Paris.

Purpose: To report a patient with an adenocarcinoma in a pleomorphic adenoma of the lacrimal gland. Adenocarcinoma constitutes a distinct group of epithelial malignancies of the lacrimal gland.

Methods: The clinical presentation, workup, surgical approach, and pathological findings were reviewed. Read More

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October 2005

Corneal choristoma associated with trisomy 8 mosaic syndrome: a clinicopathologic report.

J AAPOS 2004 Apr;8(2):204-5

Department of Pediatric Ophthalmology, Fondation Ophtalmologique Adolphe de Rothschild, 25-29 Rue Main, 75940 Paris Cedex 19, France.

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[Persistence of the pupillary membrane. Apropos of 3 cases].

J Fr Ophtalmol 1999 May;22(5):522-4

Service d'Ophtalmologie, Hôpital Necker-Enfants malades, Paris.

Persistent pupillary membrane has in most cases no functional consequence. Nevertheless, a thick membrane involving visual axis sometimes requires surgical removal. We describe three cases of this surgery with pathologic examination and functional results. Read More

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N-MYC amplification, loss of heterozygosity on the short arm of chromosome 1 and DNA ploidy in retinoblastoma.

Eur J Cancer 1996 Apr;32A(4):645-9

Service de Pédiatrie, Paris, France.

Recurrent genetic alterations different from the alteration of the RB1 gene on chromosome 13q14 have been described in retinoblastoma, including structural alterations on the short arm of chromosome 1 and amplification of the N-MYC oncogene. These two genetic alterations are major prognostic factors in neuroblastoma, another embryonic neuro-ectodermal tumour. In order to assess the frequency of these alterations and their possible association with clinical parameters in retinoblastoma, we studied a series of 46 retinoblastoma tumour samples. Read More

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Head and neck pilomatrixoma.

Am J Otolaryngol 1996 Mar-Apr;17(2):133-5

Department of Otorhinolaryngology-Head & Neck Surgery, Laennec Hospital, Paris, France.

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December 1996