Literature DB >> 10714760

Multiple endocrine abnormalities in a child with Blackfan-Diamond anemia and hemochromatosis. Significant improvement of growth velocity and predicted adult height following growth hormone treatment despite liver damage.

R Lanes1, A Muller, A Palacios.   

Abstract

We evaluated a short, prepubertal 13.9 year-old boy with Blackfan-Diamond anemia and significant liver iron stores due to multiple blood transfusions and found him to have several endocrine abnormalities, including hypothyroidism, hypoparathyroidism, primary and secondary hypogonadism and IGF-I insufficiency. Growth velocity was poor despite treatment with levothyroxine, calcitriol, calcium and aggressive therapy with chelating agents. After 25 months of treatment with rhGH his growth velocity, height for age and PAH increased significantly, suggesting a degree of sensitivity to GH despite his liver damage.

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Year:  2000        PMID: 10714760     DOI: 10.1515/jpem.2000.13.3.325

Source DB:  PubMed          Journal:  J Pediatr Endocrinol Metab        ISSN: 0334-018X            Impact factor:   1.634


  6 in total

1.  Endocrine Dysfunction in Diamond-Blackfan Anemia (DBA): A Report from the DBA Registry (DBAR).

Authors:  Amit Lahoti; Yael T Harris; Phyllis W Speiser; Evangelia Atsidaftos; Jeffrey M Lipton; Adrianna Vlachos
Journal:  Pediatr Blood Cancer       Date:  2015-10-23       Impact factor: 3.167

Review 2.  Endocrine manifestations related to inherited metabolic diseases in adults.

Authors:  Marie-Christine Vantyghem; Dries Dobbelaere; Karine Mention; Jean-Louis Wemeau; Jean-Marie Saudubray; Claire Douillard
Journal:  Orphanet J Rare Dis       Date:  2012-01-28       Impact factor: 4.123

3.  Endocrine Dysfunctions in Patients with Inherited Metabolic Diseases.

Authors:  Şahin Erdöl; Halil Sağlam
Journal:  J Clin Res Pediatr Endocrinol       Date:  2016-04-18

Review 4.  How I manage children with Diamond-Blackfan anaemia.

Authors:  Marije Bartels; Marc Bierings
Journal:  Br J Haematol       Date:  2018-12-04       Impact factor: 6.998

5.  A Rare Case of Hypoparathyroidism and Myxedema Coma in a Patient With Diamond-Blackfan Anemia.

Authors:  Raffaele A Ruggiero; Qasim Z Iqbal; Ali Akram; Jared Dendy; Julie Zaidan
Journal:  Cureus       Date:  2021-11-27

6.  Massive iron overload and acute-on-chronic liver failure in a patient with Diamond-Blackfan anaemia: a case report.

Authors:  Guilherme Rossi Assis-Mendonça; Marlone Cunha-Silva; Mariana Franson Fernandes; Luiza Dias Torres; Monica Pinheiro de Almeida Verissimo; Marcelo Trevisan Neves Okano; Daniel Ferraz Mazo; Cristina Alba Lalli; Tiago Sevá-Pereira; Rafael Fantelli Stelini; Larissa Bastos Eloy da Costa
Journal:  BMC Gastroenterol       Date:  2020-10-12       Impact factor: 3.067

  6 in total

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