An ICET- a survey on hypoparathyroidism in patients with thalassaemia major and intermedia

A preliminary report

Vincenzo De Sanctis, Ashraf T. Soliman, Duran Canatan, Heba Elsedfy, Mehran Karimi, Shahina Daar, Hala Rimawi, Soteroula Christou, Nicos Skordis, Ploutarchos Tzoulis, Praveen Sobti, Shruti Kakkar, Yurdanur Kilinc, Doaa Khater, Saif A. Alyaarubi, Valeriya Kaleva, Su Han Lum, Mohamed A. Yassin, Forough Saki, Maha Obiedat & 11 others Salvatore Anastasi, Maria Concetta Galati, Giuseppe Raiola, Saveria Campisi, Nada Soliman, Mohamed Elshinawy, Soad Al Jaouni, Salvatore Di Maio, Yasser Wali, Ihab Zaki Elhakim, Christos Kattamis

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Hypoparathyroidism (HPT) is a rare disease with leading symptoms of hypocalcemia, associated with high serum phosphorus levels and absent or inappropriately low levels of parathyroid hormone (PTH). In patients with thalassemias it is mainly attributed to transfusional iron overload, and suboptimal iron chelation therapy. The main objectives of this survey were to provide data on the prevalence, demographic and clinical features of HPT in thalassemia major (TM) and intermedia (TI) patients living in different countries, and to assess its impact in clinical medical practice. A questionnaire was sent to all Thalassemia Centres participating to the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescence 436 V. De Sanctis, A.T. Soliman, D. Canatan, et al.Medicine (ICET-A) Network.Seventeen centers, treating a total of 3023 TM and 739 TI patients, participated to the study. HPT was reported in 206 (6.8%) TM patients and 33 (4.4%) TI patients. In general, ages ranged from 10.5 to 57 years for the TM group and from 20 to 54 years for the TI group. Of the 206 TM patients and 33 TI patients with HPT, 117 (48.9%) had a serum ferritin level >2.500 ng/ml (54.3% TM and 15.1% TI patients) at the last observation. Hypocalcemia varied in its clinical presentation from an asymptomatic biochemical abnormality to a life-threatening condition, requiring hospitalization. Calcium and vitamin D metabolites are currently the cornerstone of therapy in HPT. In TM patients, HPT was preceded or followed by other endocrine and non-endocrine complications. Growth retardation and hypogonadism were the most common complications (53.3% and 67.4%, respectively). Although endocrine complications were more common in patients with TM, non-transfused or infrequently transfused patients with TI suffered a similar spectrum of complications but at a lower rate than their regularly transfused counterparts. In conclusion, although a large international registry would help to better define the prevalence, comorbidities and best treatment of HPT, through the result of this survey we hope to give a clearer understanding of the burden of this disease and its unmet needs. HPT requires lifelong therapy with vitamin D or metabolites and is often associated with complications and comorbidities.Therefore, it is important for endocrinologists and other physicians, who care for these patients, to be aware of recent advances of this disorder.

Original languageEnglish
Pages (from-to)435-444
Number of pages10
JournalActa Biomedica
Volume88
Issue number4
DOIs
Publication statusPublished - Jan 1 2017

Fingerprint

Hypoparathyroidism
beta-Thalassemia
Thalassemia
Hypocalcemia
Vitamin D
Comorbidity
Surveys and Questionnaires
Chelation Therapy
Iron Overload
Hypogonadism
Ferritins
Rare Diseases
Parathyroid Hormone
Serum
Phosphorus
Registries
Patient Care
Hospitalization
Therapeutics
Iron

Keywords

  • Clinical manifestations
  • Co-morbidities
  • Hypoparathyroidism
  • Iron overload
  • Survey
  • Thalassemia intermedia
  • Thalassemia major
  • Treatment

ASJC Scopus subject areas

  • Medicine(all)

Cite this

De Sanctis, V., Soliman, A. T., Canatan, D., Elsedfy, H., Karimi, M., Daar, S., ... Kattamis, C. (2017). An ICET- a survey on hypoparathyroidism in patients with thalassaemia major and intermedia: A preliminary report. Acta Biomedica, 88(4), 435-444. https://doi.org/10.23750/abm.v88i4.6837

An ICET- a survey on hypoparathyroidism in patients with thalassaemia major and intermedia : A preliminary report. / De Sanctis, Vincenzo; Soliman, Ashraf T.; Canatan, Duran; Elsedfy, Heba; Karimi, Mehran; Daar, Shahina; Rimawi, Hala; Christou, Soteroula; Skordis, Nicos; Tzoulis, Ploutarchos; Sobti, Praveen; Kakkar, Shruti; Kilinc, Yurdanur; Khater, Doaa; Alyaarubi, Saif A.; Kaleva, Valeriya; Lum, Su Han; Yassin, Mohamed A.; Saki, Forough; Obiedat, Maha; Anastasi, Salvatore; Galati, Maria Concetta; Raiola, Giuseppe; Campisi, Saveria; Soliman, Nada; Elshinawy, Mohamed; Al Jaouni, Soad; Di Maio, Salvatore; Wali, Yasser; Elhakim, Ihab Zaki; Kattamis, Christos.

In: Acta Biomedica, Vol. 88, No. 4, 01.01.2017, p. 435-444.

Research output: Contribution to journalArticle

De Sanctis, V, Soliman, AT, Canatan, D, Elsedfy, H, Karimi, M, Daar, S, Rimawi, H, Christou, S, Skordis, N, Tzoulis, P, Sobti, P, Kakkar, S, Kilinc, Y, Khater, D, Alyaarubi, SA, Kaleva, V, Lum, SH, Yassin, MA, Saki, F, Obiedat, M, Anastasi, S, Galati, MC, Raiola, G, Campisi, S, Soliman, N, Elshinawy, M, Al Jaouni, S, Di Maio, S, Wali, Y, Elhakim, IZ & Kattamis, C 2017, 'An ICET- a survey on hypoparathyroidism in patients with thalassaemia major and intermedia: A preliminary report', Acta Biomedica, vol. 88, no. 4, pp. 435-444. https://doi.org/10.23750/abm.v88i4.6837
De Sanctis, Vincenzo ; Soliman, Ashraf T. ; Canatan, Duran ; Elsedfy, Heba ; Karimi, Mehran ; Daar, Shahina ; Rimawi, Hala ; Christou, Soteroula ; Skordis, Nicos ; Tzoulis, Ploutarchos ; Sobti, Praveen ; Kakkar, Shruti ; Kilinc, Yurdanur ; Khater, Doaa ; Alyaarubi, Saif A. ; Kaleva, Valeriya ; Lum, Su Han ; Yassin, Mohamed A. ; Saki, Forough ; Obiedat, Maha ; Anastasi, Salvatore ; Galati, Maria Concetta ; Raiola, Giuseppe ; Campisi, Saveria ; Soliman, Nada ; Elshinawy, Mohamed ; Al Jaouni, Soad ; Di Maio, Salvatore ; Wali, Yasser ; Elhakim, Ihab Zaki ; Kattamis, Christos. / An ICET- a survey on hypoparathyroidism in patients with thalassaemia major and intermedia : A preliminary report. In: Acta Biomedica. 2017 ; Vol. 88, No. 4. pp. 435-444.
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T1 - An ICET- a survey on hypoparathyroidism in patients with thalassaemia major and intermedia

T2 - A preliminary report

AU - De Sanctis, Vincenzo

AU - Soliman, Ashraf T.

AU - Canatan, Duran

AU - Elsedfy, Heba

AU - Karimi, Mehran

AU - Daar, Shahina

AU - Rimawi, Hala

AU - Christou, Soteroula

AU - Skordis, Nicos

AU - Tzoulis, Ploutarchos

AU - Sobti, Praveen

AU - Kakkar, Shruti

AU - Kilinc, Yurdanur

AU - Khater, Doaa

AU - Alyaarubi, Saif A.

AU - Kaleva, Valeriya

AU - Lum, Su Han

AU - Yassin, Mohamed A.

AU - Saki, Forough

AU - Obiedat, Maha

AU - Anastasi, Salvatore

AU - Galati, Maria Concetta

AU - Raiola, Giuseppe

AU - Campisi, Saveria

AU - Soliman, Nada

AU - Elshinawy, Mohamed

AU - Al Jaouni, Soad

AU - Di Maio, Salvatore

AU - Wali, Yasser

AU - Elhakim, Ihab Zaki

AU - Kattamis, Christos

PY - 2017/1/1

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N2 - Hypoparathyroidism (HPT) is a rare disease with leading symptoms of hypocalcemia, associated with high serum phosphorus levels and absent or inappropriately low levels of parathyroid hormone (PTH). In patients with thalassemias it is mainly attributed to transfusional iron overload, and suboptimal iron chelation therapy. The main objectives of this survey were to provide data on the prevalence, demographic and clinical features of HPT in thalassemia major (TM) and intermedia (TI) patients living in different countries, and to assess its impact in clinical medical practice. A questionnaire was sent to all Thalassemia Centres participating to the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescence 436 V. De Sanctis, A.T. Soliman, D. Canatan, et al.Medicine (ICET-A) Network.Seventeen centers, treating a total of 3023 TM and 739 TI patients, participated to the study. HPT was reported in 206 (6.8%) TM patients and 33 (4.4%) TI patients. In general, ages ranged from 10.5 to 57 years for the TM group and from 20 to 54 years for the TI group. Of the 206 TM patients and 33 TI patients with HPT, 117 (48.9%) had a serum ferritin level >2.500 ng/ml (54.3% TM and 15.1% TI patients) at the last observation. Hypocalcemia varied in its clinical presentation from an asymptomatic biochemical abnormality to a life-threatening condition, requiring hospitalization. Calcium and vitamin D metabolites are currently the cornerstone of therapy in HPT. In TM patients, HPT was preceded or followed by other endocrine and non-endocrine complications. Growth retardation and hypogonadism were the most common complications (53.3% and 67.4%, respectively). Although endocrine complications were more common in patients with TM, non-transfused or infrequently transfused patients with TI suffered a similar spectrum of complications but at a lower rate than their regularly transfused counterparts. In conclusion, although a large international registry would help to better define the prevalence, comorbidities and best treatment of HPT, through the result of this survey we hope to give a clearer understanding of the burden of this disease and its unmet needs. HPT requires lifelong therapy with vitamin D or metabolites and is often associated with complications and comorbidities.Therefore, it is important for endocrinologists and other physicians, who care for these patients, to be aware of recent advances of this disorder.

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