Validation on the “first retrospective cohort”.

<p>(A) Percentage of MODY2 patients positive to each item of the 7-iF. (B) Percentage of MODY2 patients positive to 7, 6 or less than 6 items of the 7-iF.</p

P.I. and city of each participating ISPED centers.

<p>A. Fabrizio Barbetti (Roma), B. Corrado Mammì (Reggio Calabria), C. Maurizio Delvecchio (San Giovanni Rotondo), D₁. Nadia Tinto (Napoli), D₂. Enza Mozzillo (Napoli), E. Luigi Pianese (Ascoli Piceno), F. Sonia Toni (Firenze), G. Bruno Pasquino (Bolzano), H. Valeria Calcaterra (Pavia), I. Ivana Rabbone (Torino), J. Barbara Felappi (Brescia), K. Francesca Cardella (Palermo), L. Valentino Cherubini (Ancona), M. Franco Mammì (Locri), N. Anna Paola Frongia (Cagliari), O. Francesco Gallo (Brindisi), P. Stefano Tumini (Chieti), Q. Sonia Lucchesi (Livorno), R. Carla Maria Monciotti (Padova), S. Susanna Coccioli (Francavilla Fontana), T. Amedeo Vergerio († deceased) (Feltre), U. Stefano Zucchini (Bologna), V. Francesco Cadario (Novara), Riccardo Lera (Alessandria), X. Andrea Scaramuzza (Milano).</p

Validation of the 7-item flowchart.

<p>Phase I: The electronic records of the patients were queried (Items 1–3). Phase II, each selected patient was clinically re-evaluated (Items 4–7). IFG, impaired fasting glucose; IGT, impaired glucose tolerance. ^aFirst drop out; 18 patients were unreachable or refused to undergo clinical re-evaluation. ^bSecond drop out; 4 patients refused the genetic test.</p

The real-life management of glucose homeostasis abnormalities in pediatric onco-hematological diseases: data from a national survey

Glycemic abnormalities are a frequent finding in pediatric oncological patients, both during treatment and after its discontinuation. Moreover, impaired glucose tolerance (IGT), impaired fasting glycemia (IFG) and diabetes mellitus (DM) are not rarely diagnosed in non-oncological hematological diseases. To explore the current pediatric Italian approach to the diagnosis and the management of the glycemic alterations in this clinical setting and, thus, to identify and enforce current clinical needs, we submitted an online 23-items survey to all the Italian Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers, and surveys were descriptively analyzed. Thirty-nine AIEOP centers were involved in the study. In 2021, among 75278 children and adolescents affected by an oncological or a hematological disease, 1.2 and 0.65% developed DM, while IGT or IFG were widespread in 2.3 and 2.8%, respectively. The main causes of DM were the use of corticosteroids in patients with cancer and the iron overload in patients with thalassemia. Venous fasting plasma glycemia was the most used tool to detect glycemic abnormalities. The performance of oral glucose tolerance test (OGTT) was extremely limited, except when IFG occurred. Despite the diagnosis of DM, ∼45% of patients with cancer and 30% of patients with one hematological disease did not receive an appropriate treatment. In the other cases, insulin was the drug of first choice. Emerging technologies for diabetes care (glucose sensors and insulin pumps) are not largely used yet. The results of our study support the standardization of the care of the glycemic abnormalities during or after onco-hematologic diseases in the pediatric age. Despite the scarce data in pediatric literature, proper guidelines are needed.</p