scholarly journals Near-final height in patients with congenital adrenal hyperplasia treated with combined therapy using GH and GnRHa

2011 ◽  
Vol 55 (8) ◽  
pp. 661-664 ◽  
Author(s):  
Carlos Alberto Longui ◽  
Cristiane Kochi ◽  
Luís Eduardo Procópio Calliari ◽  
Maria Barcellos Rosa Modkovski ◽  
Marisa Soares ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Precocious Puberty ◽  
Combined Therapy ◽  
Final Height ◽  
Standard Deviation Score ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Hormonal Control ◽  
Adrenal Hyperplasia ◽  
Gh Treatment

INTRODUCTION: Intrinsic limitations of glucocorticoid therapy in patients with congenital adrenal hyperplasia (CAH) determine frequent loss in final height. The association of secondary central precocious puberty and early epiphyseal fusion is also frequent. In these conditions, GnRHa treatment alone or in combination with GH has been indicated. OBJECTIVES: This is a retrospective study, describing the estatural findings of CAH patients with significant decrease in height prediction, who were submitted to combined GH plus GnRHa therapy up to near-final height. SUBJECTS AND METHODS: We studied 13 patients, eight females and five males, eight with the classical and five with the nonclassical form of the disorder. Treatment with hydrocortisone (10-20 mg/m²/day) or prednisolone (3-6 mg/kg/day) was associated with GnRHa (3.75 mg/months) for 4.0 (1.5) years, and GH (0.05 mg/kg/day) for 3.6 (1.4) years. RESULTS: Stature standard deviation score for bone age improved significantly after GH treatment, becoming similar to target height at the end of the second year of GH treatment. CONCLUSION: We conclude that combined GH plus GnRHa therapy can be useful in a subset of CAH patients with significant reduction of predicted final height associated with poor hormonal control and central precocious puberty.

Efficacy of long-term growth hormone therapy in short non-growth hormone-deficient children

2017 ◽  
Vol 30 (2) ◽  
Author(s):  
Lucia Schena ◽  
Cristina Meazza ◽  
Sara Pagani ◽  
Valeria Paganelli ◽  
Elena Bozzola ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Final Height ◽  
Standard Deviation Score ◽  
Bone Age ◽  
Gh Treatment ◽  
Short Children ◽  
Igf I ◽  
Height Gain ◽  
The Cost

AbstractBackground:In recent years, several studies have been published showing different responses to growth hormone (GH) treatment in idiopathic short stature children. The aim of the present study was to investigate whether non-growth-hormone-deficient (non-GHD) short children could benefit from long-term GH treatment as GHD patients.Methods:We enrolled 22 prepubertal children and 22 age- and sex-matched GHD patients, with comparable height, body mass index (BMI), bone age, and insulin-like growth factor 1 (IGF-I) circulating levels. The patients were treated with recombinant human GH (rhGH) and followed until they reach adult height.Results:During GH treatment, the two groups grew in parallel, reaching the same final height-standard deviation score (SDS) and the same height gain. On the contrary, we found significantly lower IGF-I serum concentrations in non-GHD patients than in GHD ones, at the end of therapy (p=0.0055).Conclusions:In our study, the response to GH treatment in short non-GHD patients proved to be similar to that in GHD ones. However, a careful selection of short non-GHD children to be treated with GH would better justify the cost of long-term GH therapy.

scholarly journals In Boys with Abnormal Developmental Tempo, Maturation of the Skeleton and the Hypothalamic-Pituitary-Gonadal Axis Remains Synchronous

2004 ◽  
Vol 89 (1) ◽  
pp. 236-241 ◽  
Author(s):  
Armando Flor-Cisneros ◽  
Ellen W. Leschek ◽  
Deborah P. Merke ◽  
Kevin M. Barnes ◽  
Marilena Coco ◽  
...  
Keyword(s):  
Body Mass Index ◽  
Congenital Adrenal Hyperplasia ◽  
Short Stature ◽  
Precocious Puberty ◽  
Bone Age ◽  
Skeletal Maturation ◽  
Chronological Age ◽  
Adrenal Hyperplasia ◽  
Normal Bone ◽  
Primary Mechanism

The primary mechanism that initiates puberty is unknown. One possible clue is that pubertal maturation often parallels skeletal maturation. Conditions that delay skeletal maturation also tend to delay the onset of puberty, whereas conditions that accelerate skeletal maturation tend to hasten the onset of puberty. To examine this relationship, we studied boys with congenital adrenal hyperplasia (n = 13) and familial male-limited precocious puberty (n = 22), two conditions that accelerate maturational tempo, and boys with idiopathic short stature (n = 18) in which maturational tempo is sometimes delayed. In all three conditions, the onset of central puberty generally occurred at an abnormal chronological age but a normal bone age. Boys with the greatest skeletal advancement began central puberty at the earliest age, whereas boys with the greatest skeletal delay began puberty at the latest age. Furthermore, the magnitude of the skeletal advancement or delay matched the magnitude of the pubertal advancement or delay. This synchrony between skeletal maturation and hypothalamic-pituitary-gonadal axis maturation was observed among patients within each condition and also between conditions. In contrast, the maturation of the hypothalamic-pituitary-gonadal axis did not remain synchronous with other maturational processes including weight, height, or body mass index. We conclude that in boys with abnormal developmental tempo, maturation of the skeleton and the hypothalamic-pituitary-gonadal axis remains synchronous. This synchrony is consistent with the hypothesis that in boys, skeletal maturation influences hypothalamic-pituitary-gonadal axis maturation.

scholarly journals Central Precocious Puberty as a Complication of Congenital Adrenal Hyperplasia in a Boy: Case Report

2021 ◽  
Vol 44 (3) ◽  
pp. 184-187
Author(s):  
Farzana Sharmin ◽  
Suraiya Begum ◽  
Ismat Jahan ◽  
Tawhid Alam ◽  
Dhiraj Chandra Biswas
Keyword(s):  
Case Report ◽  
Child Health ◽  
Congenital Adrenal Hyperplasia ◽  
Precocious Puberty ◽  
Central Precocious Puberty ◽  
Well Being ◽  
Adrenal Hyperplasia ◽  
Peripheral Precocious Puberty

Precocious puberty has intense influence on physical and psychosocial well-being of affected children and raises a lot of concerns as well as uncertainties in family.Here,we report a case of Central precocious puberty (CPP)superimposed on peripheral precocious puberty (PPP) due to congenital adrenal hyperplasia(CAH). Bangladesh J Child Health 2020; VOL 44 (3) :184-187

Long-Term Outcomes of Patients with Central Precocious Puberty due to Hypothalamic Hamartoma after GnRHa Treatment: Anthropometric, Metabolic, and Reproductive Aspects

2017 ◽  
Vol 106 (3) ◽  
pp. 203-210 ◽  
Author(s):  
Carolina O. Ramos ◽  
Ana C. Latronico ◽  
Priscilla Cukier ◽  
Delanie B. Macedo ◽  
Danielle S. Bessa ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Final Height ◽  
Standard Deviation Score ◽  
Central Precocious Puberty ◽  
Cross Sectional Study ◽  
Hypothalamic Hamartoma ◽  
Cross Sectional ◽  
Female Patients ◽  
Tertiary Center ◽  
The Mean

Background: Hypothalamic hamartoma (HH) represents the commonest cause of organic central precocious puberty (CPP). Follow-up of these patients in adulthood is scarce. Objective: To describe the anthropometric, metabolic, and reproductive parameters of patients with CPP due to HH before and after treatment with gonadotropin-releasing hormone analog (GnRHa). Methods: We performed a retrospective and cross-sectional study in a single tertiary center including 14 patients (7 females) with CPP due to HH. Results: The mean duration of GnRHa treatment was 7.7 ± 2.4 years in boys and 7.9 ± 2.1 years in girls. GnRHa treatment was interrupted at the mean chronological age (CA) of 12.1 ± 1.1 years in boys and 10.7 ± 0.5 years in girls. At the last visit, the mean CA of the male and female patients was 21.5 ± 3.2 and 24 ± 3.9 years, respectively. Eleven of the 14 patients reached normal final height (FH) (standard deviation score -0.6 ± 0.9 for males and -0.6 ± 0.5 for females), all of them within the target height (TH) range. The remaining 3 patients had predicted height within the TH range. The mean body mass index and the percentage of body fat mass was significantly higher in females, with a higher prevalence of metabolic disorders. All patients presented normal gonadal function in adulthood, and 3 males fathered a child. Conclusion: All patients with CPP due to HH reached normal FH or near-FH. A higher prevalence of overweight/obesity and hypercholesterolemia was observed in the female patients. Finally, no reproductive disorder was identified in both sexes, indicating that HH per se has no deleterious effect on the gonadotropic axis in adulthood.

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