hrp0095p1-416 | Adrenals and HPA Axis | ESPE2022

The assessment of the usefulness of morning cortisol and DHEAS concentration levels in the diagnosis of central adrenal insufficiency in pediatric patients - preliminary results

Szczudlik Ewa , Wójcik Małgorzata , Stępniewska Anna , Januś Dominika , B. Starzyk Jerzy

The diagnosis of central adrenal insufficiency (CAI) is challenging. The most popular diagnostic tool in pediatric population remains low-dose (1 μg) cosyntropin (LDC) test. Nevertheless, there is still a need for the CAI markers that might be used on an outpatient setting as a first-line screening. In adult patients morning cortisol and dehydroepiandrosterone sulphate (DHEAS) has been postulated as a possible markers. The aim of the study was to evaluate the usefulness of ...

hrp0098p1-57 | GH and IGFs 1 | ESPE2024

Long-term treatment with recombinant human growth hormone in pediatric patients influences but does not destroy stem cells circulating in peripheral blood

Wedrychowicz Anna , Sielatycka Katarzyna , Kucia Magda , Kubiś Ewa , Roztoczynska Dorota , B. Starzyk Jerzy

Objective: Very small embryonic/epiblast‐like stem cells (VSELs), found in human bone marrow and other adult tissues, also in circulating blood are small, non-hematopoietic cells expressing markers of pluripotent embryonic and primordial germ cells. VSELs are responsible for postnatal tissue and organ rejuvenation. It was reported that VSELs decreased after prolonged GH or IGF-1 administration in experimental animals and after prolonged IGF-1 administrati...

hrp0098p2-194 | Growth and Syndromes | ESPE2024

Effectiveness of therapy with recombinant human growth hormone (rhGH) in patients with HLHS and short stature

Wędrychowicz Andrzej , Furtak Aleksandra , Ossowska Magdalena , Komorkiewicz Karolina , Roztoczynska Dorota , B. Starzyk Jerzy

Hypoplastic left heart syndrome (HLHS) is one of the most severe congenital heart defects. Despite medical progress, HLHS patients remain at risk of many complications of this cardiac defect and cardiac surgery, including growth failure. Some of them can be treated with recombinant human growth hormone (rhGH) fulfilling criteria of GH–deficiency (GHD) or having a history of intrauterine growth restriction or small for gestational age (SGA). We assessed the effectiveness ...

hrp0098p3-307 | Late Breaking | ESPE2024

Disorders of the Melanocortin Pathway: From Genetic Inheritance to Clinical Manifestations Based on a Family Case Study

Stepniewska Anna , Szczudlik Ewa , Preizner-Rzucidło Ewelina , Wójcik MAłgorzata , B. Starzyk Jerzy

It is estimated that single-gene mutations are responsible for 3-10% of childhood obesity cases. The most common of these is a mutation in the MC4R gene, occurring in about 1-6% of children and 1% of adults with early-onset severe obesity. We report on of 7-year-old male twins with severe obesity (86% and 75% excess body mass relative to height), starting from the second year of life, along with hyperphagia. The boys were born from a twin pregnancy, PIII, CC, at 37 weeks of ge...

hrp0098p3-145 | GH and IGFs | ESPE2024

Severe growth deficiency in a patient with extreme short bowel syndrome and growth hormone deficiency - is there an effective treatment option ?

Goncerz Dawid , Wójcik Małgorzata , Pabisek-Miernik Julita , Płachno Kamila , Wierdak Anna , Szczudlik Ewa , Stępniewska Anna , B. Starzyk Jerzy

Malnutrition, including that caused by malabsorption, is the most common cause of growth failure in children. On the other hand, in children who receive total parenteral nutrition due to extremely severe forms of malabsorption disorders, which involves almost continuous intravenous infusion of nutrients (including glucose), a persistent, functional reduction in growth hormone secretion occurs. A female patient with extreme short bowel syndrome, fed almost entirely parenterally...