title: Insulin-like Growth Factor-1 (IGF-1): Demographic, Clinical and Laboratory Data in 120 Consecutive Adult Patients with Thalassaemia Major.
abstract: BACKGROUND
  IGF-1 deficiency in TM patients in children and adolescents has been attributed to chronic anemia and hypoxia, chronic liver disease, iron overload and other associated endocrinopathies, e.g. growth hormone deficiency (GHD). Few data are available in the literature regarding adult TM patients and growth disorders. The aim of this study was to measure IGF-1 values and other clinical data in a large number of adult patients with TM to evaluate the possible relationships between them.
  
  
  METHODS
  A cohort of 120 adult patients with TM was studied for plasma levels of IGF-1. Plasma total IGF-1 was determined by chemiluminescent immunometric assay (CLIA) method. In eleven patients (3 females) the GH response during glucagon stimulation test (GST) was also evaluated.
  
  
  RESULTS
  Fifty percent of patients (33 males and 27 females) had IGF-1 levels <- 2 SDs below normative values for healthy subjects matched for age and sex. In these patients endocrine complications and elevations of aminotransferases (ALT) were more common compared to TM patients with IGF1 > -2SDs. In multivariate regression analyses, height, weight, BMI, serum ferritin, ALT, HCV serology and left ventricular ejection fraction (LVEF) were not significantly related to IGF-1, but a significant correlation was found in females between HCV-RNA positivity and IGF-1, ALT and serum ferritin. AGHD was diagnosed in 6 (4 males) out of 11 patients (54.5%) who had glucagon stimulation tests and in 5 out of 8 (62.5%) with IGF-1 <-2SD. The mean age of patients with GHD was 39.3 years (range: 25-49 years, median: 39 years) versus 35.8 years (range: 27-45 years, median: 37.5 years) in non-GHD patients. A positive correlation between GH peak after GST and IGF-1 level was found (r: 0.6409; p: < 0.05).
  
  
  CONCLUSIONS
  In 50% of TM patients the IGF-1 levels were 2SDs below average values for healthy individuals. IGF-1 deficiency was more common in TM patients with associated endocrine complications, and a significant correlation was found in HCV-RNA positive females among IGF-1, ALT, and serum ferritin. Further data in a larger group of patients are needed to confirm whether IGF-1 level <-2 SDs may be a potential criterion for additional studies in TM patients. This datum could avoid performing GH stimulation tests in the majority of them.
authors.affiliation: Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: De Sanctis
authors.affiliation: Department of Pediatrics, Division of Endocrinology, Hamad General Hospital Doha, Qatar.
authors.forename: Ashraf T
authors.initials: AT
authors.lastname: Soliman
authors.affiliation: Department of Medical Physics, St. Anna Hospital, Ferrara, Italy.
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Candini
authors.affiliation: Department of Hematology, Alamal Hospital, Hamad Medical Center, Doha, Qatar.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Yassin
authors.affiliation: Department of Paediatrics, Pugliese-Ciaccio Hospital, Catanzaro, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Raiola
authors.affiliation: Department of Haematology, Thalassaemia and Prenatal Diagnosis Regional Center, Pugliese-Ciaccio Hospital, Catanzaro, Italy.
authors.forename: Maria Concetta
authors.initials: MC
authors.lastname: Galati
authors.affiliation: Department of Primary Health Care (PHC), Doha, Qatar.
authors.forename: Rania
authors.initials: R
authors.lastname: Elalaily
authors.affiliation: Department of Pediatrics, Ain Shams University, Cairo, Egypt.
authors.forename: Heba
authors.initials: H
authors.lastname: Elsedfy
authors.affiliation: St. George's University Medical School at the University of Nicosia, Cyprus.
authors.forename: Nicos
authors.initials: N
authors.lastname: Skordis
authors.affiliation: Endocrine Unit, Villa Sofia -Cervello Hospital, Palermo, Italy.
authors.forename: Piernicola
authors.initials: P
authors.lastname: Garofalo
authors.affiliation: Department of Thalassaemia, Garibaldi Hospital, Catania, Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Anastasi
authors.affiliation: Unit for the Diagnosis and Treatment of Thalassaemia, Umberto I Hospital, Siracusa, Italy.
authors.forename: Saveria
authors.initials: S
authors.lastname: Campisi
authors.affiliation: Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Mehran
authors.initials: M
authors.lastname: Karimi
authors.affiliation: First Department of Paediatrics, University of Athens, Athens, Greece.
authors.forename: Christos
authors.initials: C
authors.lastname: Kattamis
authors.affiliation: Director of Hemoglobinopathy Diagnosis Center and President of Mediterranean Blood Diseases Foundation, Antalya, Turkey.
authors.forename: Duran
authors.initials: D
authors.lastname: Canatan
authors.affiliation: Department of Pediatric Hematology, Faculty of Medicine, University of Çukurova, Adana, Turkey.
authors.forename: Yurdanur
authors.initials: Y
authors.lastname: Kilinc
authors.affiliation: Professor of Pediatrics, Christian Medical College and Hospital, Ludhiana, India.
authors.forename: Praveen
authors.initials: P
authors.lastname: Sobti
authors.affiliation: Department of Pediatrics, NYU School of Medicine, New York, USA.
authors.forename: Bernadette
authors.initials: B
authors.lastname: Fiscina
authors.affiliation: Department of Pediatrics, Ain Shams University, Cairo, Egypt.
authors.forename: Mohamed
authors.initials: M
authors.lastname: El Kholy