58 research outputs found
Profile of mecasermin for the long-term treatment of growth failure in children and adolescents with severe primary IGF-1 deficiency
No full textDanilo Fintini, Claudia Brufani, Marco CappaEndocrinology Unit, “Bambino Gesù” Children’s Hospital-IRCCS, Rome, ItalyAbstract: Growth hormone insensitivity syndrome (GHI) or insulin-like growth factor-1 (IGF-1) deficiency (IGFD) is characterized by deficit of IGF-1 production due to alteration of response of growth hormone (GH) receptor to GH. This syndrome is due to mutation of GH receptor or IGF-1 gene and patients affected showed no response to GH therapy. The only treatment is recombinant IGF-1 (mecasermin), which has been available since 1986, but approved in the United States by the US Food and Drug Administration only in 2005 and in Europe by the European Medicines Agency in 2007. To date, few studies are available on long-term treatment with mecasermin in IGFD patients and some of them have a very small number of subjects. In this review we discuss briefly clinical features of severe primary IGFD, laboratory findings, and indications for treatment. Results of long-term therapy with rhIGF1 (mecasermin) in patients affected by severe primary IGFD and possible side effects are explained.Keywords: mecasermin, therapy, Laron syndrome, IGF-
Neophobia, sensory experience and child’s schemata contribute to food choices
No full textAbstract Purpose The aim of the present review is to analyze dynamic interactions between nutrigenomics, environmental cues, and parental influence, which can all lead to children’s neophobic reactions and its persistence in time. Methods We reviewed studies available on electronic databases, conducted on children aged from birth to 18 years. We also considered official websites of Italian Institutions, providing advice on healthy eating during infancy. Results Modern day societies are faced with an eating paradox, which has severe and ever-growing implications for health. In face of a wider availability of healthy foods, individuals instead often choose processed foods high in fat, salt and sugar content. Economic reasons surely influence consumers’ access to foods. However, there is mounting evidence that food choices depend on the interplay between social learning and genetic predispositions (e.g., individual eating traits and food schemata). Neophobia, the behavioral avoidance of new foods, represents an interesting trait, which can significantly influence children’s food refusal. Early sensory experiences and negative cognitive schemata, in the context of primary caregiver–child interactions, importantly contribute to the priming of children’s food rejection. Conclusions As neophobia strongly affects consumption of healthy foods, it will be relevant to rule definitively out its role in the genesis of maladaptive food choices and weight status in longitudinal studies tracking to adulthood and, in meanwhile, implement early in life effective social learning strategies, to reduce long-term effects of neophobia on dietary patterns and weight status. Level of evidence Level II, controlled trials without randomization
Levothyroxine therapy in preventing nodular recurrence after hemithyroidectomy: a retrospective study
No full textTo determine the effect of levothyroxine (L-T4) therapy on the recurrence rate of nodular disease in patients previously treated with lobectomy for benign nodular goiter
Linear growth and puberty in childhood obesity: what is new?
No full textPediatric obesity is a growing and alarming global health problem and represents an important determinant of morbidity. Since nutrition plays an important role in regulating growth and development, the excess weight gain related to overnutrition can affect growth patterns, bone maturation and pubertal development. The purpose of this review is to summarize the current knowledge about the effect of primary obesity on linear growth and pubertal development in children and adolescents. Evidences about regulatory hormones and adipokines that may be involved in the physiology of childhood growth in the context of obesity were also discussed. The most recent literature confirms previous studies indicating that linear growth is accelerated (mainly due to longer trunks rather than longer legs) and bone age is advanced in prepubertal children with obesity, while there is a reduction of pubertal height gain and attainment of normal adult height. Conflicting results are reported on the timing of puberty, specifically in boys. Indeed, previous studies suggested earlier onset of puberty in obese girls and overweight boys, and a delayed puberty in obese boys. Conversely, the most recent studies show more consistently an earlier onset and completion of pubertal development also in boys with obesity. Considering the false belief of health associated with transient taller stature in children and the adverse outcomes related to early puberty, interventions on diet and physical activity are urgently needed to tackle the epidemics of childhood obesity in public health and clinical setting
Ectopic ACTH syndrome due to occult bronchial carcinoid
No full textwe describe the case of an ectopic ACTH syndrome due to occult bronchial carcinoi
Corrigendum to: "Bone density and body composition in small for gestational age children with adequate catch up growth: A preliminary retrospective case control study" [Bone-2021 Jul 14;153:116114. doi:10.1016/j.bone.2021.116114. Online ahead of print]
No full textPredictive factors for recurrence after thyroid lobectomy for unilateral non-toxic goiter in an endemic area: results of a multivariate analysis
No full textBACKGROUND: The aim of the study was to identify the factors that are predictive
of recurrence after thyroid lobectomy for unilateral non-toxic thyroid goiter in
an endemic region through a multivariate analysis.
METHODS: Two hundred sixty-eight consecutive patients who underwent thyroid
lobectomy and who were evaluated by the same endocrinologist were included.
Univariate and multivariate analysis analyzed the relationship between sex, age,
preoperative thyroid-stimulating hormone, duration of disease, duration of
levothyroxine (LT4) preoperative therapy, cytologic results, histologic results,
resected thyroid weight, numbers and diameters of thyroid nodules, morphologic
alterations of the remnant lobe, follow-up length, postoperative LT4 therapy,
ultrasonographic evidence of recurrence, and reoperation.
RESULTS: The incidence of recurrence was 33.9% (91/268 patients) after a mean
follow-up time of 79.9 months (range, 12-251 months), female sex ( P = .016),
multiple nodules ( P = .017), and lack of postoperative LT4 therapy ( P = .0009)
were predictive factors of recurrence. Reoperation was performed in 20 patients
(7.4%); factors that were predictive of reoperation were the presence of multiple
nodules ( P = .008), resected thyroid weight ( P = .00006), and lack of
postoperative hormonal therapy ( P = .0005).
CONCLUSIONS: Thyroid lobectomy for unilateral non-toxic goiter, when combined
with suppressive or substitutive thyroxin therapy, resulted in a low rate of
recurrence and reoperation in an endemic area
Prader-Willi Syndrome in Adults: An Update On Nutritional Treatment and Pharmacological Approach
No full textPrader-Willi syndrome (PWS) is a rare and complex genetic disorder with multiple effects on the metabolic, endocrine, and neurological systems, as well as behavioral and intellectual difficulties. Despite advances in understanding the genetic basis of obesity in PWS, there are conflicting data on its management. Therefore, the present manuscript aims to provide an update on the nutritional treatment and pharmacological approach in adult patients with PWS
Obesity management in Prader–Willi syndrome: current perspectives
No full textAntonino Crinò,1 Danilo Fintini,2 Sarah Bocchini,1 Graziano Grugni3 1Autoimmune Endocrine Diseases Unit, 2Endocrinology Unit, Bambino Gesù Children’s Hospital, Research Institute, Palidoro, Rome; 3Division of Auxology, Italian Auxological Institute, Research Institute, Piancavallo, Verbania, Italy Abstract: Prader–Willi syndrome (PWS) is a complex multisystem disorder due to the absent expression of the paternally active genes in the PWS critical region on chromosome 15 (15q11.2-q13). The syndrome is considered the most common genetic cause of obesity, occurring in 1:10,000–1:30,000 live births. Its main characteristics include neonatal hypotonia, poor feeding, and lack of appetite in infancy, followed by weight gain, lack of satiety, and uncontrolled appetite, frequently after the age of 2–3 years. The clinical picture includes short stature, multiple endocrine abnormalities (hypogonadism, growth hormone/insulin-like growth factor-I axis dysfunction, hypothyroidism, central adrenal insufficiency), dysmorphic features, scoliosis, osteoporosis, mental retardation, and behavioral and psychiatric problems. Subjects with PWS will become severely obese unless their food intake is strictly controlled. Constant and obsessive food seeking behavior can make life very difficult for both the family and caretakers. Prevention of obesity is mandatory in these patients from the first years of life, because once obesity develops it is difficult to maintain the control of food intake. In fact, PWS subjects die prematurely from complications conventionally related to obesity, including diabetes mellitus, metabolic syndrome, sleep apnea, respiratory insufficiency, and cardiovascular disease. The mechanisms underlying hyperphagia in PWS are not completely known, and to date no drugs have proven their efficacy in controlling appetite. Consequently, dietary restriction, physical activity, and behavior management are fundamental in the prevention and management of obesity in PWS. In spite of all available therapeutic tools, however, successful weight loss and maintenance are hardly accomplished. In this context, clinical trials with new drugs have been initiated in order to find new possibilities of a therapy for obesity in these patients. The preliminary results of these studies seem to be encouraging. On the other hand, until well-proven medical treatments are available, bariatric surgery can be taken into consideration, especially in PWS patients with life-threatening comorbidities. Keywords: Prader–Willi syndrome, severe obesity, hyperphagia, genetic obesity, food managemen
Relationship between nonalcoholic steatohepatitis, PNPLA3 I148M genotype and bone mineral density in adolescents
No full textIt is uncertain whether non-alcoholic steatohepatitis (NASH) is a risk factor for low bone mineral density (BMD). Our aim was to investigate: a) associations between NASH and BMD values and b) associations between PNPLA3 I148M genotypes and BMD, in children with histologically-proven NAFLD
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