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  • Electronic Resource  (4)
  • Bone age  (2)
  • Growth hormone deficiency  (2)
  • 1
    ISSN: 1433-0474
    Keywords: Schlüsselwörter CHARGE-Assoziation ; Kleinwuchs ; Wachstumshormonmangel ; Key words CHARGE association ; Growth retardation ; Growth hormone deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A patient with CHARGE-association showed severe growth retardation in early childhood. Endocrinological investigations showed extremely low serum IGF-1 levels, three pathological stimulation tests of growth hormone, and only the insulin stimulation test showed a normal increase of growth hormone. Discussion: It is hypothesized that a disturbance of the growth hormone-IGF-1 axis is present in this patient, leading to severe growth failure.
    Notes: Zusammenfassung Bei einem Patienten mit CHARGE-Assoziation fiel bereits früh ein Kleinwuchs auf. Die endokrinologischen Untersuchungen ergaben sehr niedrige IGF-1-Serumkonzentrationen sowie im Verlauf 3 pathologische Wachstumshormonstimulationstests. Lediglich ein Insulintoleranztest ergab einen im Normbereich liegenden Anstieg des Wachstumshormons. Diskussion: Es wird postuliert, daß damit eine Störung der Wachstumshormon-IGF-1-Achse vorliegt. Ein Übergang in einen kompletten Wachstumshormonmangel könnte in den folgenden Jahren noch erfolgen.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Septo-optic dysplasia ; De Morsier syndrome ; Optic nerve hypoplasia ; Growth hormone deficiency ; Diabetes insipidus ; Malformations ; Central nervous system
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Septo-optic dysplasia (SOD) is characterized by hypoplasia of the optic nerve, various types of forebrain defects and hormonal deficiencies. We have studied the clinical and endocrinological characteristics of 18 such patients retrospectively to: (1) better define the endocrine abnormalities in children with SOD; and (2) to find approaches for the interdisciplinary long-term care of children with SOD. The children were seen at the Children's Hospital of the University of Munich from 1976 to 1992 (8 boys, 10 girls; age at initial presentation: 1 day–13 years of age, mean 1.9 years). Unilateral hypoplasia of the optic nerve was found in 7 cases, bilateral hypoplasia in 11. Sonographic, CCT or MRI yielded the following results: 4 of the patients had a cavum septum pellucidum, 3 patients had hypoplasia of the cerebellum, 1 aplasia of the corpus callosum and 1 aplasia of the fornix. An empty sella with or without an ectopic pituitary was seen in 4 cases. Height standard deviation score (SDS) at time of diagnosis was −4.0 to +0.4, mean −2.92. Endocrine deficiencies were present in all 11 patients who had undergone endocrinological investigations. Seven patients suffered from isolated growth hormone (GH) deficiency or multiple hypopituitarism. One had diabetes insipidus centralis, 2 had hypogonadotropic hypogonadism, 1 had hypothyroidism and 2 adrenal insufficiency. Hypothalamic testing was performed only in a subset of patients: in 5 of 11 children tested a thyrotropin releasing hormone (TRH test), in two out of nine a gonadotropin releasing hormone (GnRH) test, and in three out of six GH releasing hormone (GHRH) test yielded abnormal results. High prolactin levels were measured in two out of five patients. Conclusion SOD is characterized by optic nerve hypoplasia and a variety of endocrine deficiencies. In addition, forebrain malformations are present in most SOD patients. Hormonal disorders are present in some SOD patients which may be of hypothalamic origin and need to be investigated systematically.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 154 (1995), S. 627-632 
    ISSN: 1432-1076
    Keywords: Key words Final height ; Constitutional growth delay ; Height ; prediction ; Bone age ; Males
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on 49 boys with constitutional growth delay (CGD) who were initially seen in our clinic at a mean chronological age of 13.3 years (range, 7.3–16.4) and a bone age of 11.1 years (range, 6.0–13.5). All were below the 5th height percentile for chronological age. A positive family history with delayed growth and puberty in one or both parents could be elicited in 75%. All 49 patients were re-examined at a mean age of 22.9 years (range, 20.4–31.2). Measured final height was 171.3 cm (range, 161.2–181.7), which was slightly, but significantly lower than mean target height of 173.0 cm. Final height expressed as standard deviation score (SDS) of a male adult population standard was –1.0 (range, –2.4 to +0.5) , also significantly lower than initial height SDS related to bone age (SDSBA) of –0.5 (range, –1.6 to +1.2). If related to target height (Tanner), final height was found to correlate positively with the initial bone age deficit and the initial height SDSBA. Observed final height was also compared with the predicted adult height by the methods of Bayley-Pinneau (BP), Tanner-Whitehouse Mark II (TW II) and Roche-Wainer-Thissen. Regression equations between all three prediction methods and final height showed an excellent correlation (P 〈 0.0001). However, only by the BP method was predicted height very close to and no different from measured final height (paired t-test). Despite this, final height in 16 of 49 patients (32.6%) differed by more than 5.0 cm from BP predicted height. An overwhelming majority of the patients (88.6%) expressed complete or reasonable satisfaction with their adult height. Conclusion In our sample of male patients with CGD, adult height came close to but did not quite reach mean target height. The BP prediction method offers a good compromise between simplicity and accuracy but must be used judiciously because individual discrepancies with attained final height may be large and unexplained.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 154 (1995), S. 627-632 
    ISSN: 1432-1076
    Keywords: Final height ; Constitutional growth delay ; Height prediction ; Bone age ; Males
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Abstract We report on 49 boys with constitutional growth delay (CGD) who were initially seen in our clinic at a mean chronological age of 13.3 years (range, 7.3–16.4) and a bone age of 11.1 years (range, 6.0–13.5). All were below the 5th height percentile for chronological age. A positive family history with delayed growth and puberty in one or both parents could be elicited in 75%. All 49 patients were re-examined at a mean age of 22.9 years (range, 20.4–31.2). Measured final height was 171.3 cm (range, 161.2–181.7), which was slightly, but significantly lower than mean target height of 173.0 cm. Final height expressed as standard deviation score (SDS) of a male adult population standard was −1.0 (range. −2.4 to +0.5), also significantly lower than initial height SDS related to bone age (SDSBA) of −0.5 (range, −1.6 to +1.2). If related to target height (Tanner), final height was found to correlate positively with the initial bone age deficit and the initial height SDSBA. Observed final height was also compared with the predicted adult height by the methods of Bayley-Pinneau (BP), Tanner-Whitehouse Mark II (TW II) and Roche-Wainer-Thissen. Regression equations between all three prediction methods and final height showed an excellent correlation (P〈0.0001). However, only by the BP method was predicted height very close to and no different from measured final height (pairedt-test). Despite this, final height in 16 of 49 patients (32.6%) differed by more than 5.0 cm from BP predicted height. An overwhelming majority of the patients *88.6%) expressed complete or reasonable satisfaction with their adult height. Conclusion In our sample of male patients with CGD, adult height came close to but did not quite reach mean target height. The BP prediction method offers a good compromise between simplicity and accuracy but must be used judiciously because individual discrepancies with attained final height may be large and unexplained.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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