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      Growth Hormone Deficiency in a Patient with Becker Muscular Dystrophy: A Pediatric Case Report

      case-report

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          Abstract

          Objective. To describe a biochemical growth hormone (GH) deficiency and to evaluate therapeutic result in a six-year-old male with Becker muscular dystrophy (BMD). Methods. GH peak was evaluated after response to arginine and insulin. Bone age was evaluated according to Greulich and Pyle method. Results. The GH-supplementary therapy was very effective in terms of growth gain. Conclusion. The possibility of a growth hormone deficiency and treatment with GH in patients with BMD cannot be excluded, especially considering the good therapeutic response.

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          Reference ranges for two automated chemiluminescent assays for serum insulin-like growth factor I (IGF-I) and IGF-binding protein 3 (IGFBP-3).

          Martin Elmlinger, Werner Kühnel, Matthias M. Weber … (2004)
          Assays for insulin-like growth factor I (IGF-I) and IGF-binding protein 3 (IGFBP-3) have become essential tools in the diagnostic work-up of disorders of the somatotropic axis in children and adults. The aim of this study was to evaluate the automated IMMULITE IGF-I and IGFBP-3 assays and to establish reference limits--central 95% intervals, median, 0.1 and other centiles as clinically relevant--as a function of age from 797 females and 787 males, from the first week of life through the ninth decade. Pubertal children were classified by sex and by sexual maturation (Tanner stage). IGF-I and IGFBP-3 levels were also assayed in 20 pediatric patients each with growth hormone deficiency (GHD) and Turner syndrome (UTS), before and during 12 months of recombinant growth hormone (rhGH) therapy, as well as in 11 adult patients with GHD and seven with acromegaly before therapy. Both the IGF-I and IGFBP-3 assays were accurate, specific and sufficiently sensitive to measure IGF-I and IGFBP-3 in serum with good linearity and recovery. In the IGF-I assay, potential interference from IGFBPs was eliminated by blocking with excess IGF-II. Circulating IGF-I and IGFBP-3 concentrations, and their ratio IGF-I/IGFBP-3, were age-dependent, showing low levels immediately after birth, a typical pubertal peak for girls and boys, and a pronounced decline after puberty, reaching a plateau in early adulthood. In adults IGF-I and IGFBP-3 levels decreased smoothly but steadily with age. Children with GHD and UTS had low circulating IGF-I and IGFBP-3 levels which increased to normal reference limits under therapy with rhGH. Adult GHD patients showed IGF-I levels below the age-related median; untreated acromegalic patients mostly had IGF-I and IGFBP-3 levels above the age-related 97.5th centile. In conclusion, the automated IMMULITE IGF-I and IGFBP-3 assays are reliable tools in the diagnosis of pathologies of the GH/IGF axis and in the follow-up of their therapies.
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            Prevalence and incidence of Becker muscular dystrophy.

            K Bushby, Robert Gardner, M Thambyayah (1991)
            We measured the prevalence and incidence of Becker muscular dystrophy in the Northern Health Region of England, UK. Patients were identified from the records of the Regional Neurological Centre and Muscular Dystrophy Group laboratories, Newcastle upon Tyne, and by writing to local doctors. We used cDNA probes and/or dystrophin immunolabelling of muscle-biopsy samples to prove the diagnosis of all cases. Results were compared with the known prevalence and incidence of Duchenne muscular dystrophy. 73 patients alive and resident in the Northern Health Region were identified, giving a prevalence rate of 2.38/100,000. This compares with a prevalence of Duchenne muscular dystrophy of 2.48/100,000. The cumulative birth incidence of Becker muscular dystrophy (at least 1 in 18 450 male live births) was about one third that of Duchenne muscular dystrophy (1 in 5618 male live births), suggesting that the disorder is more common than previously thought.
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              The clinical, genetic and dystrophin characteristics of Becker muscular dystrophy. I. Natural history.

              Robert Gardner, K Bushby (1993)
              We have investigated 67 patients with proven Becker muscular dystrophy (BMD) using a standard protocol including a detailed history and a functional and clinical examination. Our aim was to define the natural history of the disease in a large cohort of patients in the light of the diagnostic methods now available. In all patients with or without an X-linked family history, the diagnosis was confirmed by the identification of a deletion or other abnormality in the dystrophin gene, and abnormal dystrophin on immunoblotting and immunocytochemistry of muscle biopsy samples. In graphs of functional and muscle score against age, two groups of patients emerged. In the larger group the disease was milder and patients remained ambulant into their forties or beyond. A smaller group had more severe disease with a slightly earlier onset, much earlier loss of ambulation, more frequent abnormal electrocardiographic findings and much lower reproductive fitness. The relationship of these clinical findings to the genetic and protein abnormalities found in the patients is explored in the accompanying paper.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Case Report Endocrinol
                Journal ID (iso-abbrev): Case Report Endocrinol
                Journal ID (publisher-id): CRIM.ENDOCRINOLOGY
                Title: Case Reports in Endocrinology
                Publisher: Hindawi Publishing Corporation
                ISSN (Print): 2090-6501
                ISSN (Electronic): 2090-651X
                Publication date (Print): 2013
                Publication date (Electronic): 14 January 2013
                Volume: 2013
                Electronic Location Identifier: 684249
                Affiliations
                1Department of Internal Medicine, University of Pavia, 27100 Pavia, Italy
                2Department of Pediatrics, IRCCS Policlinico S. Matteo Foundation, 27100 Pavia, Italy
                3IRCCS C. Mondino Foundation and University of Pavia, 27100 Pavia, Italy
                4Pediatric and Infectious Disease Unit, Department of Pediatrics, Bambino Gesù Children's Hospital, 00165 Rome, Italy
                Author notes

                Academic Editors: G. Aimaretti, M. A. Boyanov, and H. Ikeda

                Article
                DOI: 10.1155/2013/684249
                PMC ID: 3556853
                PubMed ID: 23365767
                SO-VID: eedf957f-ced4-44d9-b821-d59d6e3c5211
                Copyright © Copyright © 2013 Valeria Calcaterra et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 10 October 2012
                Date accepted : 6 November 2012
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Endocrinology & Diabetes
                Data availability:
                ScienceOpen disciplines: Endocrinology & Diabetes

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