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Article Synopsis
  • * The study included 118 participants (87 children/adolescents and 31 adults) with PWS, assessing various health metrics, including glucose and cholesterol levels, as well as using a Hyperphagia Questionnaire to measure eating behaviors.
  • * Results showed that adults had higher asprosin levels than adolescents, and those with obesity had higher levels than normal-weight individuals, but no significant correlation was found between asprosin and metabolic health indicators;
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Background: Growth hormone deficiency (GHD) and idiopathic short stature (ISS) are the major etiologies of short stature in children. For the diagnosis of GHD and ISS, meticulous evaluations are required, including growth hormone provocation tests, which are invasive and burdensome for children. Additionally, sella magnetic resonance imaging (MRI) is necessary for assessing etiologies of GHD, which cannot evaluate hormonal secretion.

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Significance: Reference cerebral near-infrared spectroscopy (NIRS) data on the pediatric population are scarce, and in most cases, only cerebral oxygen saturation ( ) measured by continuous wave spatially resolved spectroscopy NIRS is reported. Absolute data for baseline optical and hemodynamic parameters are missing.

Aim: We aimed at collecting baseline cerebral optical parameters [absorption coefficient, ; reduced scattering coefficient, ; differential pathlength factor (DPF)] and hemodynamic parameters [oxy-hemoglobin content ( ), deoxyhemoglobin content (HHb), total hemoglobin content (tHB), ] in a large cohort of pediatric patients.

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Growth Parameters and Prevalence of Obesity in PKU Patients and Peers: Is This the Right Comparison?

Pediatr Rep

October 2024

Department of Metabolic Diseases, Clinical Genetics and Diabetology, Giovanni XXIII Children Hospital, Azienda Ospedaliero-Universitaria Consorziale, 70126 Bari, Italy.

Article Synopsis
  • The study examines long-term growth trends in children with Phenylketonuria (PKU) compared to healthy peers, focusing on their body mass index (BMI) and weight-for-length ratio.
  • Results show that PKU children's median BMI z-scores were within the normal range, similar to controls, but their weight distributions were broader, particularly peaking around 3 years old.
  • Despite comparable rates of overweight between the two groups, the higher prevalence of overweight in the general population raises concerns about the nutritional risks for PKU patients.
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Aim: Current non-invasive near-infrared spectroscopy (NIRS) tissue oximetry suffers from suboptimal reproducibility over probe repositioning, hindering clinical threshold establishment. Time Domain-NIRS (TD-NIRS) offers higher precision but lacks sufficient paediatric data, preventing effective clinical application. We aimed to establish reference ranges for cerebral and mid-upper arm (MUA) tissue haemodynamics in paediatric subjects using TD-NIRS and explore correlations with auxological variables.

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