Long-Acting Growth Hormone Therapy in Pediatric Growth Hormone Deficiency: A Consensus Statement.

Context: Several long-acting growth hormone (LAGH) therapies have recently become available, but guidance on their usage in children with growth hormone (GH) deficiency is limited.

Methods: International experts in pediatric endocrinology were invited to join a consensus group based on their expertise in treating children with daily GH and LAGH. The group comprised 11 experts from 10 countries across the world.

Inflammatory markers in prepubertal children and their associations with abdominal fat.

Objective: To evaluate the association between inflammatory markers and abdominal fat assessed by ultrasound in prepubertal children with and without excess weight.

Methods: A cross-sectional study involving 241 prepubertal children, 156 with obesity, 37 with overweight, and 48 with normal weight, aged five to ten years, who were followed at a research unit on Childhood Obesity from a teaching hospital belonging to a public health system. The concentration of interleukin-6, tumor necrosis factor-α and C-reactive protein were assessed and regression analyses, considering outcome variables such as abdominal wall and intra-abdominal fat thickness measured by ultrasound, were performed.

Vitamin D-Dependent Rickets Type 3: A Case Report and Systematic Review.

Although vitamin D deficiency resulting from insufficient sunlight exposure or inadequate dietary vitamin D intake is the most common cause of rickets, mutations in genes involved in vitamin D metabolism can cause genetic forms of rickets termed Vitamin D-Dependent Rickets (VDDR). In 2018, Roizen et al. described a new type of VDDR, named VDDR3, caused by a recurrent missense mutation in the CYP3A4 gene that leads to accelerated inactivation of vitamin D metabolites.

Achondroplasia in Latin America: practical recommendations for the multidisciplinary care of pediatric patients.

Background: Achondroplasia is the most common bone dysplasia associated with disproportionate short stature, and other comorbidities, such as foramen magnum stenosis, thoracolumbar kyphosis, lumbar hyperlordosis, genu varum and spinal compression. Additionally, patients affected with this condition have higher frequency of sleep disorders, ear infections, hearing loss and slowed development milestones. Considering these clinical features, we aimed to summarize the regional experts' recommendations for the multidisciplinary management of patients with achondroplasia in Latin America, a vast geographic territory with multicultural characteristics and with socio-economical differences of developing countries.

Daily physical activity, cardiorespiratory fitness, nutritional status, endothelial function, and autonomic modulation in school-age adolescents: A principal component analysis.

Purpose: This study evaluated the association between cardiorespiratory fitness, abdominal obesity, blood pressure, endothelial function, and autonomic modulation in school-age adolescents exhibiting different levels of habitual physical activity and nutritional status, through a multivariate statistical approach.

Methods: 101 adolescents aged 15-18 years (54 females) underwent assessments of daily physical activity, body mass index, cardiorespiratory fitness, reactive hyperemia, and heart rate variability. Based on BMI adjusted for age and sex (z-BMI), 21 adolescents were classified as 'overweight' (9 girls), and 9 as 'obese' (4 girls).

Growth hormone deficiency and the transition from pediatric to adult care.

Objective: To discuss the approach to patients diagnosed with growth hormone deficiency (GHD) in childhood during the transition period from puberty to adulthood, focusing on the following: (1) physiology; (2) effects of recombinant human GH (rhGH) interruption/reinstitution after adult height achievement; (3) re-evaluation of somatrotropic axis; (4) management of rhGH reinstitution, when necessary.

Source Of Data: Narrative review of the literature published at PubMed/MEDLINE until September 2020 including original and review articles, systematic reviews and meta-analyses.

Synthesis Of Data: Growth hormone is crucial for the attainment of normal growth and for adequate somatic development, which does not end concomitantly with linear growth.

Diagnosis, Genetics, and Therapy of Short Stature in Children: A Growth Hormone Research Society International Perspective.

The Growth Hormone Research Society (GRS) convened a Workshop in March 2019 to evaluate the diagnosis and therapy of short stature in children. Forty-six international experts participated at the invitation of GRS including clinicians, basic scientists, and representatives from regulatory agencies and the pharmaceutical industry. Following plenary presentations addressing the current diagnosis and therapy of short stature in children, breakout groups discussed questions produced in advance by the planning committee and reconvened to share the group reports.

Growth hormone therapy in children; research and practice - A review.

Short stature remains the most common reason for referral to a pediatric Endocrinologist and its management remains a challenge. One of the main controversies is the diagnosis of idiopathic short stature and the role of new technologies for genetic investigation of children with inadequate growth. Complexities in management of children with short stature includes selection of who should receive interventions such as recombinant human growth hormone, and how should this agent dose be adjusted during treatment.

Physical fitness and activity, metabolic profile, adipokines and endothelial function in children.

Objectives: The prevalence of obesity is increasing. The aim of this study was to investigate if there is endothelial dysfunction in children with normal or excess weight, and whether the metabolic profile, adipokines, and endothelial dysfunction would be more strongly associated with physical fitness or with physical activity levels.

Method: Cross-sectional study involving children aged 5-12 years.

Evaluation of sublingual microcirculation in children with dengue shock.

Objective: To report the sublingual microcirculation observed using Sidestream Dark Field imaging in two children with dengue shock.

Method: Two children, aged 9 and 10 years, were admitted to the pediatric intensive care unit with dengue shock and multiple organ dysfunction. Sublingual microcirculation was assessed in each patient on the first and second days of shock and was assessed a final time when the patients were no longer in shock (on the day prior to extubation) using Sidestream Dark Field technology.

Leydig cell tumour in a 46,XX child with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

A 10-year-old male was referred to our institution due to short stature and bilateral cryptorchidism and reported pubic hair development and acne since the age of 4 years. Laboratory and molecular genetic tests indicated congenital adrenal hyperplasia due to 21-hydroxylase deficiency. After treatment with prednisone, adrenal hormones normalised but testosterone remained elevated.

[Hyperinsulinemic hypoglycemia of the infancy: analysis of clinical data from a Brazilian sample].

Objective: To review the presentation of hyperinsulinemic hypoglycemia of the infancy (HHI), its treatment and histology in Brazilian pediatric endocrinology sections.

Materials And Method: The protocol analyzed data of birth, laboratory results, treatment, surgery, and pancreas histology.

Results: Twenty-five cases of HHI from six centers were analyzed: 15 male, 3/25 born by vaginal delivery.

Adverse effects of growth hormone replacement therapy in children.

Human growth hormone (hGH) replacement therapy has been widely available for clinical purposes for more than fifty years. Starting in 1958, hGH was obtained from cadaveric pituitaries, but in 1985 the association between hGH therapy and Creutzfeldt-Jakob disease was reported. In the same year, the use of recombinant hGH (rhGH) was approved.

Endocrine diseases, perspectives and care in Turner syndrome.

Turner syndrome is a frequent chromosome disorder in clinical practice. It is characterized by short stature, gonadal dysgenesia and multisystemic involvement, responsible for a high morbidity and reduced life expectancy. The aim of the present paper is to describe the endocrinopathies and major problems at different ages, and to present suggestion for follow-up care in these patients.

Update in growth hormone therapy of children.

Although recombinant human GH (rhGH) has been available since 1985, there are several questions related to its use that remain unanswered. The Entrez-PubMed search engine was used to conduct a review of publications appearing since 2007 that address growth and GH treatment. Recent publications related to the diagnosis of GH deficiency, genetics of growth, the use of rhGH in different genetic conditions, in idiopathic short stature, and in puberty, and strategies to adjust rhGH dose were reviewed.

IGF-I and IGF Binding Protein-3 Generation Tests and Response to Growth Hormone in Children with Silver-Russell Syndrome.

Objectives. To evaluate, in children with Silver-Russell Syndrome, the response to the IGF-I and IGFBP-3 generation test and compare results to the growth response after 6 months of rhGH. Methods.

Comparison between Actual and Perceived Height of Parents of Children with Short Stature and Controls.

Objectives. We investigate whether parents complaining of their children's short stature have misconception of their height. Methods.

Perinatal goiter with increased iodine uptake and hypothyroidism due to excess maternal iodine ingestion.

Background/aims: To review cases of fetal/newborn goiter due to excess maternal iodine ingestion.

Methods: We reviewed the medical records of all patients that presented with congenital goiter in 2003. We used the PubMed search engine to conduct a review of publications addressing congenital goiter and excessive iodine intake.

Vitamin D deficiency in children and its management: review of current knowledge and recommendations.

Given the recent spate of reports of vitamin D deficiency, there is a need to reexamine our understanding of natural and other sources of vitamin D, as well as mechanisms whereby vitamin D synthesis and intake can be optimized. This state-of-the-art report from the Drug and Therapeutics Committee of the Lawson Wilkins Pediatric Endocrine Society was aimed to perform this task and also reviews recommendations for sun exposure and vitamin D intake and possible caveats associated with these recommendations.

The role of recombinant human insulin-like growth factor-I in treating children with short stature.

Context: Recombinant human (rh) IGF-I is now available to treat children with short stature resulting from severe primary IGF-I deficiency. This review from the Drug and Therapeutics Committee of the Lawson Wilkins Pediatric Endocrine Society discusses different aspects of rhIGF-I therapy, particularly with regard to potential advantages and disadvantages in comparison with the traditional use of rhGH for treatment of short stature.

Evidence Acquisition: We used the Entrez-PubMed search engine to conduct a review of publications addressing IGF-I deficiency, the use of rhIGF-I, and treatment for short stature.

IGF-I, IGFBP-3 and ALS generation test in Turner syndrome.

Objectives: The pathophysiology of the short stature in girls with Turner syndrome (TS) is not well understood. The "IGF-I generation test" is used to assess the sensitivity to growth hormone. We compared the biochemical response to four days of growth hormone of TS and controls.

Short stature in children with sickle cell anemia correlates with alterations in the IGF-I axis.

Children with sickle cell anemia (SCA) frequently have short stature. We propose that alterations in the IGF-I axis are involved in their growth failure. We investigated the IGF-I axis in children with SCA and height below the 25th percentile (n = 15) and compared it with that of children with SCA and height above the 50th percentile (n = 7).