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Bone disease in thalassemia

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In thalassaemic patients, osteopenia and osteoporosis represent prominent causes of morbidity in young adults of both genders with thalassemia major and intermedia. Delayed diagnosis and inadequate treatment has led to significant osteoporosis,
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  Supplemento 1, Vol. 12 - n. 3 - Settembre-Dicembre 2014     P   e   r    i   o    d    i   c   o   q   u   a    d   r    i   m   e   s    t   r   a    l   e  -    P   o   s    t   e    I    t   a    l    i   a   n   e    S .   p .    A .  -    S   p   e    d    i   z    i   o   n   e    i   n   a    b    b   o   n   a   m   e   n    t   o   p   o   s    t   a    l   e  -    D .    L .    3    5    3    /    2    0    0    3    (   c   o   n   v .    i   n    L .    2    7    /    0    2    /    2    0    0    4   n .    4    6    )  -    A   r    t .    1 ,   c   o   m   m   a    1    D    C    B    M    i    l   a   n   o Indexed inEMBASE/Compendex/Geobase/SCOPUS Ten years of research on growth and endocrine complications in thalassaemia Contribution papers of the International Network of Clinicians for Endocrinopathiesin Thalassemia and Adolescence Medicine (ICET-A) Leptin and ghrelin serum concentrations in thalassemia major and intermedia patients and normal subjects Hamdollah Karamifar, Maryam Bahmanyar, Vincenzo De Sanctis, Mehran Karimi Prevalence of diabetic ketoacidosis (DKA)in thalassemia major patients in Iran due to secondary iron overload Mehran Karimi, Zohreh Karamizadeh, Saba Lahsaeizadeh, Hamta Jafari, Yunes Mavali, Vincenzo De Sanctis Credibility of HbA1c in diagnosis and management of disturbances of glucose and diabetes in transfused patients with thalassemia Christos Kattamis, Polyxeni Delaporta, Maria Dracopoulou, George Paleologos, George P. Chrousos, Ioannis Papassotiriou, Antonios Kattamis Reversible heart failure in a thalassaemic patient secondary to hypocalcemia and hypoparathyroidism  Vincenzo De Sanctis, Monica Sprocati, Maria Rita Govoni, Giuseppe Raiola Bone disease in thalassemia  Ashraf T. Soliman MRI Assessment of iron overload in thalassemia: an overview Kavita Saggar, Praveen Sobti O RGANO  U FFICIALE ISSN 2035-0678  3   3  Abbonamento annuale (3 numeri) Euro 30,00.Pagamento: conto corrente postale n. 1010097192 intestato a:Edizioni Scripta Manent s.n.c., via Bassini 41, 20133 Milano È vietata la riproduzione totale o parziale, con qualsiasi mezzo, di articoli, illustrazioni e fotografiesenza l’autorizzazione scritta dell’Editore.L’Editore non risponde dell’opinione espressa dagli Autori degli articoli. Ai sensi della legge 675/96 è possibile in qualsiasi momento opporsi all’invio della rivista comunicandoper iscritto la propria decisione a: Edizioni Scripta Manent s.n.c. Via Bassini, 41 - 20133 Milano DIRETTORE SCIENTIFICO Vincenzo De Sanctis (Ferrara) COMITATO DI REDAZIONESilvano Bertelloni (Pisa) Giampaolo De Luca (Amantea, Cosenza) Bernadette Fiscina (New York, USA) Giuseppe Raiola (Catanzaro) Tito Livio Schwarzenberg (Roma) COMITATO EDITORIALEAntonietta Cervo (Pagani, Salerno) Salvatore Chiavetta (Palermo) Michele De Simone (L’Aquila) Teresa De Toni (Genova) Piernicola Garofalo (Palermo) Maria Rita Govoni (Ferrara) Domenico Lombardi (Lucca) Carlo Pintor (Cagliari) Luigi Ranieri (Catanzaro) Leopoldo Ruggiero (Lecce) Giuseppe Saggese (Pisa) INTERNATIONALEDITORIAL BOARDMagdy Omar Abdou (Alexandria, Egypt) Mujgan Alikasifoglu (Istanbul, Turkey) Mike Angastiniotis (Nicosia, Cyprus) German Castellano Barca (Torrelavega, Spain) Elsaid Bedair (Doha, Qatar) Monica Borile (El Bolson, Argentina) Roberto Curi Hallal (Rio de Janeiro, Brasil)  Yardena Danziger (Petah-Tiqva, Israel) Oya Ercan (Istanbul, Turkey) Helena Fonseca (Lisbon, Portugal) Daniel Hardoff (Haifa, Israel) Christos Kattamis (Athens, Greece) Nogah Kerem (Haifa, Israel) Karaman Pagava (Tbilisi, Georgia) Praveen C. Sobti (Ludhiana - Punjab, India) Ashraf Soliman (Doha, Qatar) Joan-Carles Suris (Lausanne, Switzerland) SEGRETARIA DI REDAZIONELuana Tisci (Ferrara) STAFF EDITORIALE Direttore ResponsabilePietro CazzolaDirezione Generale Armando MazzùDirezione Marketing Antonio Di MaioConsulenza GraficaPiero MerliniImpaginazioneStefania Cacciaglia Scripta Manent s.n.c. Via Bassini, 41 - 20133 MilanoTel. 0270608091 - 0270608060 / Fax 0270606917E-mail: scriman@tin.itRegistrazione Tribunale di Milano n. 404 del 23/06/2003Stampa: EURGRAF sasCesano Boscone (MI) Supplemento 1, Vol. 12 - n. 3 - Settembre-Dicembre 2014 Sommario Leptin and ghrelin serum concentrationsin thalassemia major and intermedia patientsand normal subjects  pag. ? Hamdollah Karamifar, Maryam Bahmanyar, Vincenzo De Sanctis, Mehran Karimi Prevalence of diabetic ketoacidosis (DKA)in thalassemia major patientsin Iran due to secondary iron overload  pag. ? Mehran Karimi, Zohreh Karamizadeh, Saba Lahsaeizadeh, Hamta Jafari, Yunes Mavali, Vincenzo De Sanctis Credibility of HbA1c in diagnosis and managementof disturbances of glucose and diabetesin transfused patients with thalassemia  pag. ? Christos Kattamis, Polyxeni Delaporta, Maria Dracopoulou, George Paleologos, George P. Chrousos,Ioannis Papassotiriou, Antonios Kattamis Reversible heart failure in a thalassaemic patient secondary to hypocalcemia and hypoparathyroidism  pag. ?  Vincenzo De Sanctis, Monica Sprocati, Maria Rita Govoni, Giuseppe Raiola Bone disease in thalassemia  pag. ?  Ashraf T. Soliman MRI Assessment of iron overload in thalassemia: an overview  pag. ? Kavita Saggar, Praveen Sobti O RGANO U FFICIALE  1 ICET-A (International Network of Clinicians for Endocrinopathies in Thalassemia and AdolescenceMedicine) an opportunity for improving thalassemia care The prevalence of endocrine complications in thalassemic patients are still considerably high and this neces- sitates national and international efforts for prevention, early detection and proper treatment of these disorders. Summary of results for thalassemic patients > 6 years of age 2011 The prevalence of endocrine complications differed among centers because of the following: 1. using questionnaire type of surveys, 2. different transfusion status and compliance to chelation therapy (different degrees of iron overload), 3.different degrees of hepatic dysfunction and nutritional status including  supplementation of folic acid, vitamin D and zinc and 4.using small samples (number of patients) and uncontrolled cohorts. In addition, different methods of diagnosing endocrine abnormali-ties have been used which make comparison of data difficult. Some sur-veys use only clinical data while others use biochemical data for the dia- gnosis of endocrine abnormalities e.g. (short stature versus growth hor- Editoriale Number 81 Age (years)17 1 +/- 10.5 years Sex 24 F + 57 M Short stature HtSDS < -262% Low IGF- I (IGF- I < - 2 SD)85% GHD (Peak GH < 7 ng/ml) (n = 42)45% Impaired Glucose tolerance (OGTT)22,20% Diabetes mellitus (FBG > 7.2 or 2h BG > 11.1)6,20% Hypothyroidism (low free T4 and/or high TSH)5% Hypocalcemia (Ca < 2 mmol/L)7,50% Hyperphosphatemia ( > 1.9 mmol/L)8,60% Hypoparathyroidism 7,50%  Vitamin D Deficiency (25 OHD < 20 ng/ml)52% Impaired liver function (elevated ALT)22,20% Cardiomyopathy (clinical and echocardiography)31% Hypogonadotropic hypogonadism 46% Osteoporosis/Osteopenia 25% Serum Ferritin Concentration ug/L2500 +/- 1550   mone deficiency (GHD), pubertal delay/failure, primary and secondary amenorrhea versus hypogonadotropic hypo- gonadism). Moreover the use of different biochemical and hormone assay methods for interpretation of endocrine abnor- malities produce significant difference in interpreting results e.g.: diagnosing glycemic abnormalities using fasting blood glucose (FBG) versus oral glucose tolerance test (OGTT) versus continuous glucose monitoring system (CGMS), and using different GH stimulation tests with different assays and cut-points . Moreover, the time of doing the test in relation to blood transfusion (before versus after blood transfusion) may  significantly change the results of these endocrine tests (e.g testing IGF-1 level before versus after blood transfusion. Despite all these facts, surveys from different countries (both clinical and biochemical) are very important to eva- luate the extent of growth and endocrine affection in these patients and plan for prevention, early diagnosis and proper treatment.The practical objectives of ICET-A are to encourage and guide endocrinological follow up of multi-transfused patients in developing countries, to promote and support collaborative research in this field, to encourage and guideendocrinological follow up of multi-transfused patients, and to educate and train more endocrinologists and other  pediatricians/physicians to prevent and improve management of the growth and endocrine complications in these patients.  Ashraf Soliman 1  and Vincenzo de Sanctis 2 1 Department of Pediatrics, Alexandria University Children’s Hospital, Egypt 2 Pediatric and Adolescent Outpatient Clinic, Private Accredited Quisisana Hospital, Italy 2  Abstract Endocrine dysfunctions related to iron overload, such as delayed puberty, short stature and hypogonadism, lead to major  problems in thalassaemic patients. Leptin, a polypeptide with 167 amino acids produced by white adipose tissue, is a hormone which reduces appetite andincreases energy consumption by affecting the central nervous system. Ghrelin, a peptide hormone produced by the stom-ach, stimulates growth hormone release via growth hormone secretagogue receptor. To evaluate leptin and ghrelin serum levels in thalassemia, 50 normal subjects, 50   -thalassaemia major patients and 50thalassaemia intermedia patients were randomly selected. Mean leptin concentration was 2.6 ± 1.2    g/L in patients with   -thalassaemia major, and 2.8 ± 2.4    g/L in patients with   -thalassaemia intermedia. These values appeared to be significantly lower than controls (9.2 ± 2.9    g/L) (p < 0.001).Mean ghrelin concentrations were 1042.1 ± 275.9 pg/mL and 989.3 ± 275.5 pg/mL in   -thalassaemia major and inter-mediate groups, respectively. This value in thalassaemia major appeared to be significantly higher compared to the con-trol group (876.9 ± 384.3 pg/mL) (p < 0.01).There was a positive correlation between serum leptin concentration and body mass index (BMI) in thalassaemia major and intermedia. Leptin levels were significantly lower in thalassaemia major patients with short stature compared to con-trols, but no correlation was found between ghrelin levels and short stature in any of the three groups. These results suggestthat one of the endocrinopathies affecting thalassaemic patients is adipose tissue dysfunction and it may be that low leptinlevels play a role in the endocrine dysfunction in these patients. These findings need to be confirmed in further studies. Key words: leptin, ghrelin, thalassemia.. Leptin and ghrelin serum concentrationsin thalassemia major and intermedia patientsand normal subjects Hamdollah Karamifar 1 , Maryam Bahmanyar 2 , Vincenzo De Sanctis 3 , Mehran Karimi 4 1 Pediatric Endocrine Department – Shiraz University of Medical Sciences – Shiraz (Iran) 2 Pediatric Department – Shiraz University of Medical Sciences – Shiraz (Iran) 3 Department of Reproduction and Growth – Pediatric and Thalassaemia Unit – Ferrara (Italy) 4 Hematology Research Center – Shiraz University of Medical Sciences – Shiraz (Iran) Direttore Scientifico Vincenzo De Sanctis (Ferrara) Comitato di Redazione Vincenzo Caruso (Catania) , Paolo Cianciulli (Roma), Maria Concetta Galati (Catanzaro) , Maria Rita Gamberini (Ferrara) , Aurelio Maggio (Palermo) Comitato Editoriale Maria Domenica Cappellini (Milano) , Marcello Capra (Palermo) , Gemino Fiorelli (Milano) , Alfio La Ferla (Catania) , Turi Lombardo (Catania) ,Carmelo Magnano (Catania) , Roberto Malizia (Palermo) , Giuseppe Masera (Monza) , Lorella Pitrolo (Palermo) , Luciano Prossomariti (Napoli) ,Michele Rizzo (Caltanisetta) , Calogero Vullo (Ferrara) Segretaria di Redazione Gianna Vaccari (Ferrara) International Editorial Board  A. Aisopos (Athens, Greece) , M. Angastiniotis (Nicosia, Cyprus) , Y. Aydinok (Izmir, Turkey) , D. Canatan  (Antalya, Turkey) , S. Fattoum (Tunis, Tunisia) , C. Kattamis (Athens, Greece) , D. Malyali (Istanbul, Turkey) , P. Sobti (Ludhiana, India) , T. Spanos (Athens, Greece)    C   o   m   i   t   a   t   o   E   d   i   t   o   r   i   a   l   e Agosto 2010
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