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    • Dicer1 Mutations
    • John R Priest
    • John R Priest: Influence Statistics

      John R Priest

      John R Priest

      Minneapolis, MN, USA | Minneapolis, Minnesota, USA | Minneapolis, USA | Minneapolis, Minnesota | From the Departments of Human Genetics (M.A.-R., M.K.W., W.D.F.), Pharmacology ...

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      John R Priest:Expert Impact

      Concepts for whichJohn R Priesthas direct influence:Dicer1 mutations,Pleuropulmonary blastoma,Dicer1 syndrome,Pulmonary blastoma,Pituitary blastoma,Cerebral metastasis,Somatic dicer1 mutations,Dicer1 mutation.

      John R Priest:KOL impact

      Concepts related to the work of other authors for whichfor which John R Priest has influence:Pleuropulmonary blastoma,Inflammatory pseudotumor,Dicer1 mutations,Myofibroblastic tumor,Acute lymphoblastic leukemia,Urinary bladder,Central nervous.

      KOL Resume for John R Priest

      Year
      2021

      Minneapolis, MN, USA

      2020

      Minneapolis, Minnesota, USA

      2019

      Minneapolis, USA

      From the Departments of Human Genetics (M.A.-R., M.K.W., W.D.F.), Pharmacology (D.P.), Oncology (M.R.F., W.D.F.), and Biochemistry (M.R.F.), and the Lady Davis Institute, Segal Cancer Centre, Jewish General Hospital (M.A.-R., D.P., N.S., M.K.W., M.R.F., W.D.F.), McGill University, the Department of Pathology, Montreal Children's Hospital (V.-H.N.), the Department of Radiology (K.M.), and the Cancer Research Program, Research Institute (W.D.F.), McGill University Health Centre, and the Department of Pathology, Centre Hospitalier Universitaire Sainte-Justine (D.B.-D.S.) - all in Montreal; the Department of Pediatrics, Endocrinology Unit, Sapienza University, Rome (M.S.), and Centro Diagnostico Italiano, Milan (S.Z.) - both in Italy; the Department of Pediatrics and Adolescent Medicine, Faculty of Medicine (M.K.), and the Institute for Diagnostic and Interventional Radiology, Faculty of Medicine (J.M.), Georg-August University, Göttingen, the Department of Pediatric Surgery, St. Bernward Krankenhaus Hildesheim, Hildesheim (S.G.), and the Institute of Human Genetics, Ulm University and Ulm University Medical Center, Ulm (R.W., C.L., R.S.) - all in Germany; and Minneapolis (J.R.P.).

      2018

      Department of Medicine, MinneapolisMinnesota, USA.

      Minneapolis Minnesota USA

      2017

      55454, Minneapolis, MN, USA

      2016

      Departments of Human Genetics (L.d.K., M.K.W., W.D.F.), Pathology (I.B.), Oncology (W.D.F.), McGill University; Department of Medical Genetics, Lady Davis Institute, Segal Cancer Centre, Jewish General Hospital (L.d.K., M.K.W., N.S., W.D.F.); Research Institute of the McGill University Health Centre (L.d.K., W.D.F.); McGill University and Genome Québec Innovation Centre (T.R., P.B., J.R.), Montréal, Québec, Canada; and (J.R.P.), Minneapolis, Minnesota, USA.

      2015

      Minneapolis, MN 55414

      2014

      Children's Hospitals and Clinics of Minnesota International Pleuropulmonary Blastoma Registry Minneapolis Minnesota

      International Pleuropulmonary Blastoma Registry, Minneapolis, MN

      2011

      International Pleuropulmonary Blastoma Registry, Children’s Hospitals and Clinics of Minnesota, 2545 Chicago Ave. S., Suite 412, 55404, Minneapolis, MN, USA

      2010

      The International Pleuropulmonary Blastoma Registry, St Paul, Minnesota, USA

      International Pleuropulmonary Blastoma Registry, Children's Hospitals and Clinics of Minnesota, 2525 Chicago Ave. S., MS 17‐412, Minneapolis, MN 55404

      2009

      The International Pleuropulmonary Blastoma Registry, Children’s Hospitals and Clinics of Minnesota, Minneapolis, MN 55404, USA.

      2008

      International Pleuropulmonary Blastoma Registry, Children's Hospitals and Clinics of Minnesota, 2545 Chicago Ave. S., Minneapolis, MN 55404

      2007

      International Pleuropulmonary Blastoma Registry, Children's Hospitals and Clinics of Minnesota, 345 North Smith Avenue, Mailstop 70‐301, St. Paul, MN 55102

      2006

      Center for Care Innovation and Research, Children's Hospitals and Clinics of Minnesota, Minneapolis, MN

      and the Department of Pathology and Immunology, Barnes-Jewish and St Louis Children’s Hospitals at Washington University, St Louis, MO

      From the International Pleuropulmonary Blastoma Registry

      2004

      Children's Hospitals and Clinics, Saint Paul, MN; Barnes-Jewish and St. Louis Children's Hospital, St. Louis, MO

      2001

      Children's Hospital of St. Paul, St. Paul, Minnesota

      1997

      Department of Hematology/Oncology, Children's Health Care—St. Paul, St Paul, Minnesota, USA

      1996

      Department of Hematology/Oncology, Children's Health Care, St. Paul, Minnesota 55102, USA.

      1995

      Lauren V. Ackerman Laboratory of Surgical Pathology, Department of Pathology, Barnes and St. Louis Children's and Jewish Hospital of St. Louis, Washington University Medical Center, St. Louis, Missouri, and Children's Hospital of St. Paul, St. Paul, Minnesota, U.S.A.

      1994

      Children's Hospital, St. Paul, Minnesota

      1993

      Children's Hospital of St. Paul, St Paul, Minnesota

      1992

      Department of Pediatrics, Naval Hospital, Oakland, CA 94627.

      1990

      Department of Pediatrics, University of Minnesota St Paul, MN

      1989

      Department of Hematology/Oncology, Children's Hospital of St. Paul, St. Paul, Minnesota, USA

      Children's Hospital of St. Paul, Pediatric Hematology-Oncology, 345 Smith Avenue North, St. Paul, MN 55102, U.S.A.

      1988

      St Paul, Minnesota, USA

      1987

      University of Minnesota, USA

      1982

      University of Minnesota Health Sciences Center, Minneapolis, Minn. USA

      1981

      Department of Laboratory Medicine and Pathology and the Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota

      1980

      Box 56 Mayo Memorial Building, University of Minnesota Hospitals, Minneapolis, Minnesota 55455

      Department of Pediatrics, University of Minnesota Health Sciences Center, Minneapolis, Minnesota

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      Sample of concepts for which John R Priest is among the top experts in the world.
      Concept World rank
      diagnostic entity 1980s #1
      wt dicer1 mutations #1
      dehner rarity #1
      dehner contrast #1
      ppb dehner 1994 #1
      pleiotropic tumour #1
      wdfa #1
      malignant ­sarcoma #1
      1980s dehner #1
      medulloepithelioma cbme #1
      ppb sertoli #1
      cystic nephroma medulloepithelioma #1
      blastoma cystic #1
      intrathoracic extravasation #1
      familial pleuropulmonary #1
      neuroblastoma medulloblastoma retinoblastoma #1
      child ppb #1
      ppb malignant ­sarcoma #1
      ppb cystic nephroma #1
      disease pleuropulmonary #1
      australian ppb #1
      wt dicer1 #1
      dicer1 germline mutations #1
      erms neuroblastoma medulloblastoma #1
      ppb malignant #1
      blastoma registry #1
      bile flow evidence #1
      wdfa ppb #1
      sertolileydig ovarian tumor #1
      benign kidney tumour #1
      revision pathologic diagnoses #1
      nephroblastoma embryonal rhabdomyosarcoma #1
      cases pleuropulmonary #1
      ppb germline #1
      25–50 ppbs pleuropulmonary #1
      patient ppb #1
      detailed inquiry medical #1
      international pleuropulmonary #1
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      Prominent publications by John R Priest

      KOL-Index: 19454

      CONTEXT: Nontoxic multinodular goiter (MNG) is frequently observed in the general population, but little is known about the underlying genetic susceptibility to this disease. Familial cases of MNG have been reported, and published reports describe 5 families that also contain at least 1 individual with a Sertoli-Leydig cell tumor of the ovary (SLCT). Germline mutations in DICER1, a gene that codes for an RNase III endoribonuclease, have been identified in families affected by ...

      Known for Dicer1 Mutations | Leydig Cell | Familial Multinodular | Mng Slct | Loss Heterozygosity
      KOL-Index: 13554

      OBJECTIVE: Pleuropulmonary blastoma (PPB) is a childhood cancer arising from pleuropulmonary mesenchyme. This neoplasm is a sentinel disease in a familial tumor syndrome recently found to be associated with germline mutations in DICER1. Observations of ovarian sex cord-stromal tumors (OSCST) in PPB kindreds led to further study. We sought to characterize ovarian tumors seen in probands and families with PPB and PPB-related conditions and define germline DICER1 status.

      METHODS: Patient ...

      Known for Pleuropulmonary Blastoma | Ovarian Tumors | Sex Cord | Dicer1 Mutations | Cell Tumor
      KOL-Index: 12921

      CONTEXT: Carriers of germline DICER1 mutations are predisposed to a rare cancer syndrome, the DICER1 syndrome. Thyroid abnormalities are a common finding in DICER1 syndrome with multinodular goiter frequently present in many families in which a germline DICER1 mutation is segregating. Differentiated thyroid carcinoma (DTC) is infrequently seen in such pedigrees. In addition to germline DICER1 mutations, specific somatic mutations have been identified in the DICER1 ribonuclease IIIb ...

      Known for Dicer1 Mutations | Differentiated Thyroid | Ppb Dtc | Catalytic Domain | Multinodular Goiter
      KOL-Index: 12494

      OBJECTIVES: Nasal chondromesenchymal hamartoma (NCMH) is an uncommon chondro-stromal tumor of the nasal cavity and paranasal sinuses in infancy and childhood. Pleuropulmonary blastoma (PPB) is also a rare malignancy of lung and pleura in childhood and is the sentinel disease of an important familial tumor and dysplasia syndrome. This study identified NCMH in PPB patients.

      METHODS: The International PPB Registry collects cases of PPB using central pathology review and evaluation of ...

      Known for Pleuropulmonary Blastoma | Ncmh Ppb | Nasal Chondromesenchymal | Familial Tumor | Sentinel Disease
      KOL-Index: 11750

      Individuals harboring germ-line DICER1 mutations are predisposed to a rare cancer syndrome, the DICER1 Syndrome or pleuropulmonary blastoma-familial tumor and dysplasia syndrome [online Mendelian inheritance in man (OMIM) #601200]. In addition, specific somatic mutations in the DICER1 RNase III catalytic domain have been identified in several DICER1-associated tumor types. Pituitary blastoma (PitB) was identified as a distinct entity in 2008, and is a very rare, potentially lethal early ...

      Known for Dicer1 Mutations | Pituitary Blastoma | Germ Mutation | Rnase Iiib | Pitb Identified
      KOL-Index: 11498

      BACKGROUND: Constitutional DICER1 mutations were recently reported to cause familial pleuropulmonary blastoma (PPB).

      AIM: To investigate the contribution and phenotypic spectrum of constitutional and somatic DICER1 mutations to cancer.

      METHODS AND RESULTS: The authors sequenced DICER1 in constitutional DNA from 823 unrelated patients with a variety of tumours and in 781 cancer cell lines. Constitutional DICER1 mutations were identified in 19 families including 11/14 with PPB, 2/3 with ...

      Known for Dicer1 Syndrome | Cystic Nephroma | Tumour Predisposition | Constitutional Dna | Sertoli Leydig
      KOL-Index: 11425

      Germ-line RB-1 mutations predispose to pineoblastoma (PinB), but other predisposing genetic factors are not well established. We recently identified a germ-line DICER1 mutation in a child with a PinB. This was accompanied by loss of heterozygosity (LOH) of the wild-type allele within the tumour. We set out to establish the prevalence of DICER1 mutations in an opportunistically ascertained series of PinBs. Twenty-one PinB cases were studied: Eighteen cases had not undergone previous ...

      Known for Dicer1 Mutations | Germ Mutation | Wildtype Allele | Rna Helicases | Dead Box
      KOL-Index: 10931

      BACKGROUND: Pleuropulmonary blastoma (PPB) has 3 subtypes on a tumor progression pathway ranging from type I (cystic) to type II (cystic/solid) and type III (completely solid). A germline mutation in DICER1 is the genetic cause in the majority of PPB cases.

      METHODS: Patients confirmed to have PPB by central pathology review were included, and their clinical characteristics and outcomes were reported. Germline DICER1 mutations were sought with Sanger sequencing.

      RESULTS: There were 435 ...

      Known for Pleuropulmonary Blastoma | Type Iii | Ppb Cases | Germline Mutation | Central Review
      KOL-Index: 10322

      BACKGROUND: Multilocular cystic nephroma (CN) is a benign kidney tumour and is part of a family of kidney neoplasms including cystic partially differentiated nephroblastoma and Wilms tumour (WT). CN is rarely familial or bilateral, but it occurs in about 10% of families where pleuropulmonary blastoma (PPB) is present. Recently, germline mutations in DICER1 were found in familial PPB.

      OBJECTIVE: To search for DICER1 mutations in two families with familial CN; PPB was present in one ...

      Known for Dicer1 Mutations | Cystic Nephroma | Familial Ppb | Kidney Neoplasms | Pleuropulmonary Blastoma
      KOL-Index: 9452

      Key PointsDicer is a type III cytoplasmic endoribonuclease that is involved in the maturation of several classes of small non-coding RNAs, such as microRNAs.Dicer is involved in the maturation of precursor microRNAs, it loads the derived small RNAs into effector Argonaute proteins, and functions as a scaffold for several interactions in the RNA interference mechanisms.Genetic perturbation of Dicer results in defects affecting functions ranging from embryogenesis, differentiation and ...

      Known for Mutations Dicer1 | Pleuropulmonary Blastoma | Cystic Nephroma | Reduced Expression | Rare Entities

      Pleuropulmonary blastoma


      [ PUBLICATION ]
      KOL-Index: 9046

      BACKGROUND: Pleuropulmonary blastoma (PPB) is a unique dysontogenetic neoplasm of childhood that appears as a pulmonary and/or pleural-based mass and is characterized histologically by a primitive, variably mixed blastematous and sarcomatous appearance.

      METHODS: Histologic material from all cases was reviewed and the tumors subclassified as type I (purely cystic), type II (cystic and solid), or type III (purely solid). Data regarding presenting symptoms, family history, operative ...

      Known for Pleuropulmonary Blastoma | Type Type Iii | Ppb Patients | Pleural Neoplasms | Cyst Formation
      KOL-Index: 9028

      OBJECTIVE: To characterize the association of pleuropulmonary blastoma (PPB) with cystic nephroma (CN) and other renal tumors.

      STUDY DESIGN: Complete clinicopathologic review of cases from the International PPB Registry and literature.

      RESULTS: We identified 18 patients with PPB associated with 20 renal tumors (15 CN), either in themselves or family members. All patients with PPB were <5 years of age. All but one of the renal diagnoses were made before 4 years of age. Eleven children had ...

      Known for Cystic Nephroma | Renal Tumors | Pleuropulmonary Blastoma | Patients Ppb | Familial Association

      Key People For Dicer1 Mutations

      Top KOLs in the world
      #1
      John R Priest
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      #5
      Yoav H Messinger
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      #6
      Gretchen M Williams
      pleuropulmonary blastoma dicer1 mutations cystic nephroma

      Minneapolis, MN, USA | Minneapolis, Minnesota, USA | Minneapolis, USA | Minneapolis, Minnesota | From the Departments of Human Genetics (M.A.-R., M.K.W., W.D.F.), Pharmacology (D.P.), Oncology (M.R.F., W.D.F.), and Biochemistry (M.R.F.), and the Lady

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