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Early and progressive insulin resistance in young, non-obese cancer survivors treated with hematopoietic stem cell transplantation.

It is unclear whether there is a causative relationship between the development of metabolic syndrome (MS) and increased risk of early cardiovascular morbidity in patients receiving hematopoietic stem cell transplantation (HSCT) during childhood. Early identification of risk factors associated with insulin resistance, MS, and abnormal glucose tolerance during childhood or adolescence in these patients could represent a useful tool for preventing cardiovascular disorders.

Glucose tolerance affects pubertal growth and final height of children with cystic fibrosis

There are few data about the impact of cystic fibrosis‐related diabetes (CFRD) on growth. We analyzed 17 children with cystic fibrosis (CF) presenting with newly diagnosed CFRD during puberty, in comparison with a matched control group of 52 CF children with normal glucose tolerance (NGT). Anthropometric evaluation showed that body mass index at CFRD diagnosis was significantly reduced in children with CFRD, in comparison with children with NGT (CFRD: −0.48 ± 1.08 vs. NGT: 0.2 ± 0.99; P = 0.01), and the same difference remained evident at the end of follow up (CFRD: −0.49 ± 0.95 vs. NGT: 0.13 ± 0.89; P = 0.04). Height standard deviation score (SDS) at baseline was slightly but not significantly lower in CFRD children (CFRD: −0.71 ± 0.83 vs. NGT: −0.25 ± 1.08; P = 0.08), while final height SDS was significantly reduced (CFRD: −1.61 ± 1.12 vs. NGT: −0.61 ± 1.15; P = 0.003). Mean final height SDS of the whole group was lower than mean target height SDS (final height SDS: −0.86 ± 1.2 vs. target height SDS: −0.3 ± 0.85; P < 0.001). Target adjusted final height was lower in CFRD children, although the difference between CFRD and NGT children did not reach statistical significance (CFRD: −0.8 ± 1.03 vs. NGT: −0.47 ± 0.9; P = 0.09). Pubertal growth and final height are negatively affected by CFRD. Intensive insulin treatment does not appear to be effective in normalizing growth, even when treatment is started early in the course of the disease, before the onset of clinical deterioration. Pediatr Pulmonol. 2015; 50:144–149.

Long-term first line medical treatment in a 4-year-old girl with Xq26.3 microduplication-negative somatotropinoma. Case report and literature review.

Abstract Growth hormone (GH) secreting adenoma represents a therapeutic challenge in childhood. Because of its rarity no treatment guidelines are available and pediatric management often results from recommendations issued for adults. We report a case of a 4-year-old girl with somatotropinoma successfully treated with only medical treatment. She presented with tall stature and history of growth acceleration. Imaging and laboratory confirmed the diagnosis of GH secreting macroadenoma. She started medical treatment with a somatostatin analogue and a dopamine agonist. During an 8-year follow-up period, a good clinical control of the disease and a shrinkage of the adenoma have been demonstrated. At the last observation she achieved normal near-adult height and pubertal development. According to our experience and limited literature evidences, first line treatment with somatostatin analogues can be attempted in patients with somatotropinoma. This approach seems to be able to control the clinical course of the disease, allowing to postpone transphenoidal surgery to adult age or to avoid it.

L’iposurrenalismo non autoimmune nel bambino

SommarioL’iposurrenalismo in età pediatrica è una condizione complessa e risulta fondamentale il suo riconoscimento perché, se non adeguatamente trattata, può risultare fatale. Tale quadro può derivare da una patologia primitivamente surrenalica (deficit di glucocorticoidi e di mineralcorticoidi) o da una disfunzione dell’asse ipotalamo-ipofisi-surrene (deficit isolato di glucocorticoidi). Le cause principali nel bambino sono i disturbi della steroidogenesi, la forma autoimmunitaria, l’adrenoleucodistrofia, altre forme più rare di iposurrenalismo e la sospensione incongrua di terapie steroidee utilizzate per lungo tempo. La diagnosi si fonda sul sospetto clinico e sugli esami ematici ormonali basali e/o dopo stimolo. Il trattamento sostitutivo è basato sulla somministrazione di idrocortisone per via orale; talora possono risultare necessari l’utilizzo di mineralcorticoidi e la supplementazione di sodio. Il medico e i genitori devono essere allertati alla necessità di aumentare la dose di corticosteroide nei periodi di stress e l’eventuale somministrazione i.m. o e.v. in caso di impossibilità di assunzione orale.

Timing della pubertà: variabilità e secular trend

SommarioLa variabilità interindividuale del timing della pubertà è ampia e risulta influenzata da fattori genetici e ambientali quali nutrizione, eventi stressanti e contaminanti. Tra la metà dell’Ottocento e la metà del Novecento si è osservato nei Paesi industrializzati un trend secolare di anticipo puberale, che pare essersi arrestato negli ultimi decenni, mentre risulta ancora in atto nei Paesi poveri. Alcune evidenze sarebbero a favore di un progressivo anticipo del telarca a fronte di una stabilità del menarca.