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

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Valeria Calcaterra ◽  
Annachiara Malvezzi ◽  
Rossana Toglia ◽  
Angela Berardinelli ◽  
Elena Bozzola ◽  
...  

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.

Author(s):  
L. I. Senkivska ◽  
M. L. Aryayev

Anotation. Regional clinical and auxological studies of growth hormone deficiency (GHD) in children contribute to a realistic assessment of the severity of the problem and prevent underdiagnostics of the disease. The aim of the study is to analyze the features of various forms of GHD in children of the Odessa region on the basis of the clinicians’ integrated assessment of history, physical examination, auxology, stature, growth velocity, bone age (BA), 2 growth hormone (GH) stimulation tests, insulin-like growth factor, magnetic resonance imaging in 92 children with GHD in the Odessa region. Parametric variables were assessed using criteria t-test and хі-square (χ2). The p value <0,05 was considered statistically significant. Decrease in SDS height from - 2,1 tо - 6,6 was found. Isolated GHD (IGHD) was diagnosed in 65,2% of diseases, multiple pituitary hormone deficiency (MPHD) in 34,8% cases. IGHD was more often idiopathic and MPHD was more often arose on an organic basis. Chronological age (CA), BA and height of children with MPHD were lower at the start of therapy compared with IGHD. Complete GH deficiency was more common in children with MPHD (62,7%), while partial GH deficiency was more common in children with IGHD (81,7%). Very low values of the coefficient CA/BA (less than 0,55) were significantly more often detected in children with MPHD compared with IGHD. The regional clinical and auxological characteristics of GHD in children in the Odessa region can be considered in further study of population aspects and patterns of growth.


1982 ◽  
Vol 99 (2) ◽  
pp. 166-173 ◽  
Author(s):  
M. Zachmann ◽  
D. Tassinari ◽  
W. Sorgo ◽  
G. U. Exner ◽  
B. Kempken ◽  
...  

Abstract. Twentythree boys with delayed adolescence (age 15.7 ± 2.0, bone age 12.4 ± 2.1 years) were studied. Their cortisol response to insulin was normal. After oral metyrapone (500 mg/m2 by mouth) one to three consecutive 12 h urine samples were collected for analysis of THS. Thirtyseven tests with 37 first, 21 second, and 11 third samples were carried out. The results could be divided into two main groups: 25 tests (group A) were subnormal in the first sample, 12 of them with a very weak (40 ± 8 μg/m2/12 h) and 13 with an insufficient (191 ± 16 μg/m2/12 h) THS response. Values in the second and third sample were higher, indicating a dealyed response. In 12 tests (group B), the results were normal (1016 ± 143 μg/m2/12 h) in the first and lower in the second and third samples. In three patients with repeated tests, there was improvement with increasing bone age. The THS-responses to metyrapone did not correlate with those of growth hormone, gonadotrophins, and TSH to stimuli. It is concluded that the THS-response to a single dose of metyrapone may be temporarily insufficient or delayed in delayed adolescence. We interpret this finding as showing transiently reduced or slow hypothalamic responsiveness.


2015 ◽  
Vol 10 (1) ◽  
pp. 38
Author(s):  
Carlos TORI TORI ◽  
Carlos ROE B.

We present a case of Klinefelter’s syndrome and short stature due to partial growth hormone deficiency. His height was below the third percentile for age and his bone age lagged behind four years. Cases like this are generally due to the presence of a an isochromosome Xq or to an isolated partial or total deficiency of growth hormone, or to partial or panhypopituitarism. We wish de emphasize the rare association between Klinefelter syndrome and growth hormone deficiency.


PEDIATRICS ◽  
1998 ◽  
Vol 102 (Supplement_3) ◽  
pp. 527-530
Author(s):  
Margaret H. MacGillivray ◽  
Sandra L. Blethen ◽  
John G. Buchlis ◽  
Richard R. Clopper ◽  
David E. Sandberg ◽  
...  

The current doses of recombinant growth hormone (rGH) are two to three times those used in the pituitary growth hormone era. These rGH doses (0.025 to 0.043 mg/kg/d) are similar to or moderately greater than the physiologic requirements. Growth velocity and height gains have been shown to be greater with 0.05 mg/kg/d of rGH than with 0.025 mg/kg/d. Larger doses of GH and early initiation of treatment result in greater heights at the onset of puberty and greater adult heights. Earlier onset of puberty and more rapid maturation, as indicated by bone age, were not observed in children who were given 0.18 to 0.3 mg/kg/wk of rGH. The frequency of adverse events is very low, but diligent surveillance of all children who are treated with rGH is essential.


PEDIATRICS ◽  
1969 ◽  
Vol 43 (6) ◽  
pp. 989-1004
Author(s):  
R. Youlton ◽  
S. L. Kaplan ◽  
M. M. Grumbach

The growth hormone (GH) response to insulin-induced hypoglycemia and to arginine infusion has been evaluated in 60 children with growth retardation. These children have been classified into three groups: Group 1-9 children had peak serum growth hormone values of 7 mµg/ml or greater to both stimuli, a normal growth hormone response. Group 2-18 children had peak GH values of ≤ 3 mµg/ml to both stimuli, an abnormal response indicating growth hormone deficiency. Group 3-6 children had a blunted GH response (&gt; 3 &lt; 7 mµg/ml) to both stimuli; 8 showed a normal rise in serum GH following arginine infusion (&gt; 7 mµg/ml) but exhibited no rise, or a minimal one, following insulin administration; 9 children had minimal increase in serum GH concentration following arginine infusion but showed a normal GH response to insulin administration (&gt; 7mµg/ml). Children included in Group 3 represent a heterogenous population. In some patients with a blunted response to both stimuli, evidence of partial or less severe form of GH deficiency was found, whereas in 17 of 18 children exhibiting a disparate response the impaired growth was not attributable to growth hormone deficiency. The blood glucose at all sampling periods was significantly lower following insulin administration in patients in Group 2 than that observed for children in Group 1 and 3. The blood glucose was significantly lower at 90 and 120 minutes following arginine infusion in Group 2 compared to values for patients in Group 1 and 3. Changes in serum insulin in response to the infusion of arginine did not provide a useful index of discrimination among these groups. Administration of diethylstilbestrol, 10 mg/day times 2 days, prior to testing can modify the GH response to both hypoglycemia and arginine; it is a useful ancillary procedure in children with blunted or disparate responses. These studies suggest that two types of stimulation tests are necessary to establish the diagnosis of isolated GH deficiency with a high degree of probability.


1989 ◽  
Vol 120 (6) ◽  
pp. 795-799 ◽  
Author(s):  
Jean Edmond Toublanc ◽  
Claire Couprie ◽  
Philippe Garnier ◽  
Jean-Claude Job

Abstract. The final height of patients treated with growth hormone for isolated growth hormone deficiency has, up to now, been subnormal, with a mean below −2 sd in the series reported, an insufficient height at the onset of puberty and a more or less accelerated bone maturation during puberty being two important factors of the poor results. A long-acting analogue of gonadoliberin, Trp6-GnRH, has been given to GH-treated patients with isolated growth hormone deficiency at the time they reached pubertal stage 2, in combination with unchanged doses of GH, for one year in 11 and for two years in 7 of them. It resulted in an increase in the height age/bone age ratio and a reduction of the height insufficiency for bone age. The increase was slight but significant after one year, and fair after two years, in spite of a reduced annual growth rate. Post-analogue follow-up in 5 patients with continued GH treatment showed a good development of growth and of puberty. It is concluded that combination of the long-acting Trp6-GnRH analogue and GH for 1–2 years in patients with isolated growth hormone deficiency whose puberty starts with a very insufficient height may be an appropriate way to improve their growth parameters. Studies with increased doses of GH or increased frequency of injections could help to optimize the results. Several years of follow-up are needed for demonstrating the results on final height.


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