Preface

Chapter 8 **The Insulin-Like Growth Factor System in the Human**

Ebe D'Adamo, M. Loredana Marcovecchio, Tommaso de Giorgis, Valentina Chiavaroli, Cosimo Giannini, Francesco Chiarelli and

Carla Basualto-Alarcón, Rodrigo Maass, Enrique Jaimovich and

Emrah Yerlikaya and Fulya Akin

**Cardiovascular Diseases 237**

Chapter 9 **Pediatric Nonalcoholic Fatty Liver Disease 221**

Chapter 10 **Anabolic/Androgenic Steroids in Skeletal Muscle and**

**Pathology 197**

**VI** Contents

Angelika Mohn

Manuel Estrada

The endocrine system plays a vital role in whether or not you develop a wide range of dis‐ eases, not only including strictly endocrine-diseases.

This book includes selected contemporary topics representing exciting recent developments in the field of endocrine and endocrine-related diseases. It is the result of a collaboration among eminent scientists and clinicians coming from eight different countries, including Ar‐ gentina, Brazil, Chile, Italy, Romania, Spain, Turkey and United States.

Basic and advanced recent trends and research studies in a number of endocrine and endo‐ crine-related disorders are covered by the different chapters of the book. We will read about hereditary neuroendocrine tumors, with emphasis on their still poorly defined natural histo‐ ry and clinical course; pituitary tumors, with a comprehensive overview of the recent ad‐ vances in their pathogenesis; thyroid dysfunctions, including those related to aging and iodine deficiency; diabetes, with its hepatic complications, and double diabetes, a new defi‐ nition that summarizes the coexistence of features of both type 1 and type 2 diabetes in the same individual, with a comprehensive discussion of the various aspects of this disorder and a focus on the search for a treatment paradigm in children and adolescents. Moreover, we will read about the testosterone as a new player in metabolic disorders and as a potential drug to counteract skeletal muscle diseases; the glucagon-like peptide type 1 and its multi‐ ple actions for which it can be considered an anti-diabetogenic agent and may have impor‐ tant implications in pathophysiological states such as obesity; the-insulin-like-growth-factor role in different human pathologies, including cancer; and the pediatric nonalcoholic fatty liver disease.

I hope that, at the end, the reader will have a critic and advanced overview of selected hot pathologies related to the endocrine field.

> **Dr. Monica Fedele** Institute of Experimental Endocrinology and Oncology, National Research Council, Naples, Italy

**Chapter 1**

**Hereditary Neuroendocrine Tumor Syndromes**

Neuroendocrine tumours (NETs) are rare and heterogeneous neoplasms with variable bio‐ logical behaviour. The estimated incidence of NETs is about 1-5 cases/100,000/year. The most recent data show a progressive increase of the incidence in the last years and a high increase of their prevalence and survival [1]. NETs can be sporadic or can arise in complex hereditary endocrine disorders such as Multiple Endocrine Neoplasias (MENs), Familial Paragangliomatosis (FPGLs), Neurofibromatosis type 1 (NF1), von Hippel-Lindau Disease (VHL), Tuberous Sclerosis (TSC) [1]. It has been estimated that hereditary NETs occurrence varies with site of origin of the tumour, ranging 5 to 30% of cases [1]. Due to the recent ad‐ vances in the knowledge of biology and genetics of NETs, these rates seems to be an under‐ estimation and novel mutations of well known oncogenes or tumour suppressor genes as well as new genes and molecular pathways responsible for unknown syndromes are expect‐

Patients with hereditary NET syndromes inherit the susceptibility to develop multiple endo‐ crine neoplasias which can be associated with non-endocrine tumours and/or non-tumour lesions. They are characterized by germline mutations usually inherited as an autosomal

Compared to the sporadic forms, hereditary NETs generally present an earlier age at onset, multiple tumour localizations, higher secretory activity. Diagnosis is made around sixth dec‐ ade of life in sporadic NETs while it is anticipated of about three decades in hereditary tu‐ mours [3]. The identification of hereditary NET syndromes is relevant to achieve a precocious diagnosis and this may be important to prevent severe complications and unfav‐ ourable outcome. For this reason, the genetic screening is nowadays a well established pro‐ cedure in many tumor types allowing to reclassify as carrier of specific hereditary NET

and reproduction in any medium, provided the original work is properly cited.

© 2013 Faggiano et al.; licensee InTech. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

© 2013 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution,

dominant disease according to the Knudson's "two-hits hypothesis" [2].

Antongiulio Faggiano, Valeria Ramundo,

Additional information is available at the end of the chapter

Luisa Circelli and Annamaria Colao

http://dx.doi.org/10.5772/53841

**1. Introduction**

ed to be characterized.
