Pegvisomant-primed growth hormone (GH) stimulation test is useful in identifying true GH deficient children.

OBJECTIVE: Provocative stimulation tests for growth hormone (GH) assessment have poor reproducibility and can often elicit false positive results in normal children. The aim of our study was to confirm the capability of pegvisomant as an enhancer of GH secretion in unmasking false-positive results in short children (height <-2.0 standard deviation score, SDS) undergoing GH testing. DESIGN: A prospective study was conducted between March and August 2016. Twenty short children (10 males and 10 females), aged 4.6-13.4 years, previously diagnosed as GH deficient (GHD) were included in the study. All subjects received 1 mg/kg of pegvisomant subcutaneously; three days later an insulin tolerance test (ITT) was performed. Insulin-like growth factor-I (IGF-I) was evaluated before and three days after pegvisomant administration. RESULTS: After pegvisomant priming and the ITT stimulation test, 12 out of the 20 children initially classified as GHD showed a GH peak of more than 10 ng/ml and were thus reclassified as short normal. Furthermore, a significant reduction of IGF-I was observed in the GHD group (pre IGF-I: median (IQR) 144.0 (109-248) ng/ml, post IGF-I: 98 (49-165) ng/ml; p<0.001) after pegvisomant administration. CONCLUSIONS: Pegvisomant priming before GH stimulation tests can be used to improve the reliability of the diagnostic work-up in GH deficiency.

Serum α-klotho levels are not informative for the evaluation of growth hormone secretion in short children.

BACKGROUND: α-Klotho is a transmembrane protein that can be cleaved and act as a circulating hormone (s-klotho). s-Klotho serum levels seem to reflect growth hormone (GH) secretory status. We investigated the role of s-klotho as a reliable marker of GH secretion in short children and the factors influencing its secretion. METHODS: We enrolled 40 short Egyptian children (20 GH deficiency [GHD] and 20 idiopathic short stature [ISS]). They underwent a pegvisomant-primed insulin tolerance test (ITT) and were accordingly reclassified as 16 GHD and 24 ISS. The samples obtained before and 3 days after pegvisomant administration, prior to the ITT, were used for assaying insulin-like growth factor (IGF)-I and s-klotho. RESULTS: IGF-I and s-klotho serum levels were not significantly different (p=0.059 and p=0.212, respectively) between GHD and ISS. After pegvisomant, a significant reduction in IGF-I and s-klotho levels was found in both groups. s-Klotho significantly correlated only with IGF-I levels in both groups. CONCLUSIONS: s-Klotho mainly reflects the IGF-I status and cannot be considered a reliable biomarker for GH secretion in children.

Ketogenic diet versus gluten free casein free diet in autistic children: a case-control study.

Many diet regimens were studied for patients with autism spectrum disorder (ASD) over the past few years. Ketogenic diet is gaining attention due to its proven effect on neurological conditions like epilepsy in children. Forty-five children aged 3-8 years diagnosed with ASD based on DSM-5 criteria were enrolled in this study. Patients were equally divided into 3 groups, first group received ketogenic diet as modified Atkins diet (MAD), second group received gluten free casein free (GFCF) diet and the third group received balanced nutrition and served as a control group. All patients were assessed in terms of neurological examination, anthropometric measures, as well as Childhood Autism Rating Scale (CARS), Autism Treatment Evaluation Test (ATEC) scales before and 6 months after starting diet. Both diet groups showed significant improvement in ATEC and CARS scores in comparison to control group, yet ketogenic scored better results in cognition and sociability compared to GFCF diet group. Depending on the parameters measured in our study, modified Atkins diet and gluten free casein free diet regimens may safely improve autistic manifestations and could be recommended for children with ASD. At this stage, this study is a single center study with a small number of patients and a great deal of additional wide-scale prospective studies are however needed to confirm these results. TRIAL REGISTRATION NUMBER: UMIN-CTR Study Design: trial Number UMIN000021433.