Long-term safety and effectiveness of a somatropin biosimilar (Omnitrope) in children requiring growth hormone therapy: analysis of final data of Italian patients enrolled in the PATRO children study.

Purpose: Omnitrope (a somatropin biosimilar), used to treat growth disturbances, is considered to have a good safety profile in children. Here, we present the analysis of final data of the Italian cohort of the PAtients TReated with Omnitrope (PATRO) Children study.

Methods: This multicenter, open-label, longitudinal, post-marketing surveillance study enrolled eligible children during 2010-2018.

Endocrine disorders in Rett syndrome: a systematic review of the literature.

Background: Rett syndrome (RTT) is an X-linked progressive neurodevelopmental disorder that involves mainly girls and is the second most frequent cause of genetic intellectual disability. RTT leads to neurological regression between 6 and 18 months of life and could be associated with a variable neurological impairment. However, RTT affects not only neurological function but also wide aspects of non-neurological organs.

Associations of bioelectrical impedance and anthropometric variables among populations and within the full spectrum of malnutrition.

Objectives: The aim of this study was to evaluate body composition variability assessed by bioimpedance in relation to nutritional status assessed by anthropometry in children and adolescents living in countries characterized by contrasting nutritional conditions.

Methods: The sample was comprised of 8614 children (4245 males; 4369 females), aged 3 to 19 years, from Nepal (477 children), Uganda (488 children and adolescents), UK (297 children and adolescents) and US (7352 children and adolescents). Height-for-age (HAZ) and body mass index-for-age (BAZ) z-scores were calculated according to WHO growth references.

Endocrinological features and epileptic encephalopathy in COX deficiency due to SCO1 mutations: case series and review of literature.

Context: Cytochrome C oxidase (COX) is the fourth component of the respiratory chain and is located within the internal membrane of mitochondria. COX deficiency causes an inherited mitochondrial disease with significant genetic and phenotypic heterogeneity. Four clinical subtypes have been identified, each with distinct phenotypes and genetic variants.

L-DOPA Test in the Diagnosis of Childhood Short Stature: Evaluation of Growth Hormone Peaks Over Time.

Introduction: In childhood, growth hormone (GH) deficiency (GHD) diagnosis is based on auxological assessment and biochemical provocative tests, whose reliability remains disputed. Recently, several papers have been published on standardising the duration of some tests. The aim of our study was to analyse the possible length reduction of the L-DOPA provocative test.