Uniparental disomy and pretreatment IGF-1 may predict elevated IGF-1 levels in Prader-Willi patients on GH treatment.


Journal

Growth hormone & IGF research : official journal of the Growth Hormone Research Society and the International IGF Research Society
ISSN: 1532-2238
Titre abrégé: Growth Horm IGF Res
Pays: Scotland
ID NLM: 9814320

Informations de publication

Date de publication:
Historique:
received: 04 05 2019
revised: 09 08 2019
accepted: 26 08 2019
pubmed: 6 9 2019
medline: 13 5 2020
entrez: 6 9 2019
Statut: ppublish

Résumé

Pediatric patients with Prader-Willi syndrome (PWS) can be treated with recombinant human GH (rhGH). These patients are highly sensitive to rhGH and the standard doses suggested by the international guidelines often result in IGF-1 above the normal range. We aimed to evaluate 1 the proper rhGH dose to optimize auxological outcomes and to avoid potential overtreatment, and 2 which patients are more sensitive to rhGH. In this multicenter real-life study, we recruited 215 patients with PWS older than 1 year, on rhGH at least for 6 months, from Italian Centers for PWS care. We collected auxological parameters, rhGH dose, IGF-1 at recruitment and (when available) at start of treatment. The rhGH dose was 4.3 (0.7/8.4) mg/m

Identifiants

pubmed: 31487604
pii: S1096-6374(19)30040-1
doi: 10.1016/j.ghir.2019.08.003
pii:
doi:

Substances chimiques

IGF1 protein, human 0
Human Growth Hormone 12629-01-5
Insulin-Like Growth Factor I 67763-96-6

Types de publication

Journal Article Multicenter Study Observational Study

Langues

eng

Sous-ensembles de citation

IM

Pagination

9-15

Informations de copyright

Copyright © 2019 Elsevier Ltd. All rights reserved.

Auteurs

Viviana Valeria Palmieri (VV)

Department of Biomedicine and Human Oncology, Pediatric Section, University A. Moro, Bari, Italy.

Antonella Lonero (A)

Pediatrics Unit "A. Perrino" Hospital, Brindisi, Italy.

Sarah Bocchini (S)

Reference Center for Prader-Willi syndrome, Bambino Gesù Hospital, Research Hospital, Roma, Italy.

Gilda Cassano (G)

Department of Pediatrics, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.

Alessio Convertino (A)

Reference Center for Prader-Willi syndrome, Bambino Gesù Hospital, Research Hospital, Roma, Italy.

Domenico Corica (D)

Department of Human Pathology of Adulthood and Childhood, University of Messina, Italy.

Antonio Crinò (A)

Reference Center for Prader-Willi syndrome, Bambino Gesù Hospital, Research Hospital, Roma, Italy.

Valentina Fattorusso (V)

Department of Translational Sciences, University Federico II, Naples, Italy.

Silvio Ferraris (S)

Department of Pediatric Endocrinology, Regina Margherita Children's Hospital, University of Torino, Torino, Italy.

Danilo Fintini (D)

Reference Center for Prader-Willi syndrome, Bambino Gesù Hospital, Research Hospital, Roma, Italy.

Adriana Franzese (A)

Department of Translational Sciences, University Federico II, Naples, Italy.

Graziano Grugni (G)

Division of Auxology, Italian Auxological Institute, Research Institute, Piancavallo, Verbania, Italy.

Lorenzo Iughetti (L)

Department of Medical and Surgical Sciences of the Mother, Children and Adults, University of Modena and Reggio Emilia, Modena, Italy.

Rosanna Lia (R)

Department of Pediatrics, Locri, Italy.

Francesca Macchi (F)

Department of Medicine and Surgery, University of Insubria, Varese, Italy.

Simona Filomena Madeo (SF)

Department of Medical and Surgical Sciences of the Mother, Children and Adults, University of Modena and Reggio Emilia, Modena, Italy.

Patrizia Matarazzo (P)

Department of Pediatric Endocrinology, Regina Margherita Children's Hospital, University of Torino, Torino, Italy.

Luana Nosetti (L)

Department of Medicine and Surgery, University of Insubria, Varese, Italy.

Sara Osimani (S)

Department of Pediatrics, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.

Roberta Pajno (R)

Department of Pediatrics, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.

Giuseppa Patti (G)

Department of Pediatrics, Giannina Gaslini Institute, Genoa, Italy.

Maria Chiara Pellegrin (MC)

Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", Trieste, Italy.

Annamaria Perri (A)

Rare Diseases Unit Pediatric Department, University of Bologna, Bologna, Italy.

Letizia Ragusa (L)

Oasi Research Institute-IRCCS, Troina, Italy.

Irene Rutigliano (I)

Department of Pediatrics, IRCCS Casa Sollievo della Sofferenza, San Giovanni Rotondo, Italy.

Michele Sacco (M)

Department of Pediatrics, IRCCS Casa Sollievo della Sofferenza, San Giovanni Rotondo, Italy.

Alessandro Salvatoni (A)

Department of Medicine and Surgery, University of Insubria, Varese, Italy.

Emanuela Scarano (E)

Rare Diseases Unit Pediatric Department, University of Bologna, Bologna, Italy.

Stefano Stagi (S)

Anna Meyer Children's University Hospital, Florence, Italy.

Gianluca Tornese (G)

Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", Trieste, Italy.

Giuliana Trifirò (G)

Pediatric Unit, ASST-Rhodense, Rho, Milan, Italy.

Malgorzata Wasniewska (M)

Department of Human Pathology of Adulthood and Childhood, University of Messina, Italy.

Rita Fischetto (R)

Metabolic Diseases, Clinical Genetics and Diabetology Unit, Giovanni XXIII Children's Hospital, Bari, Italy.

Paola Giordano (P)

Department of Biomedicine and Human Oncology, Pediatric Section, University A. Moro, Bari, Italy.

Maria Rosaria Licenziati (MR)

Obesity and Endocrine disease Unit, Department of Neurosciences, Santobono-Pausilipon Children's Hospital, Naples, Italy.

Maurizio Delvecchio (M)

Metabolic Diseases, Clinical Genetics and Diabetology Unit, Giovanni XXIII Children's Hospital, Bari, Italy. Electronic address: mdelvecchio75@gmail.com.

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