scholarly journals SAT-101 The Booster Effect of Aromatase Inhibitor to Overcome Waning Effect of Recombinant Human Growth Hormone

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Dae Hyun Kim ◽  
Ju-hee Choi
Keyword(s):  
Growth Rate ◽  
Aromatase Inhibitor ◽  
Short Stature ◽  
Growth Velocity ◽  
Adult Height ◽  
Combined Treatment ◽  
Bone Age ◽  
Final Adult Height ◽  
Rhgh Treatment ◽  
Booster Effect

Abstract Aromatase inhibitor (AI) is a drug that blocks the conversion of androgens to estrogens, originally approved by FDA as a treatment of breast cancer in postmenopausal women. In several studies, it has been proposed that AI appears effective to improve the final adult height especially combined with rhGH therapy, by delaying growth plate closure. Patients treated with rhGH showed highest growth rate during the first year of treatment, with an average increase of 8–10 cm per year (often called “catch-up” growth), however, followed by a progressive decrease in growth rate over the next several years, which is called “waning effect” of rhGH. In this report, we would like to introduce 2 cases of short pubertal boys showing booster effect of AI when co-treated with rhGH, to overcome waning effect of rhGH and improve the final adult height. Case 1. A boy at the age of 8 years and 1 month visited out clinic with short stature. His height was 118cm (4%), weight was 19.2kg (0%). His MPH was calculated as 173cm. Bone age was 6.9 years by the TW3 method. Initial laboratory finding showed no other abnormal findings including normal IGF-1 levels, so we diagnosed the patient as idiopathic short stature (ISS), and started rhGH treatment. During the rhGH treatment of 4 years and 9 months, the patient obtained additional 30.7cm growth to 148.7cm (23%). However, the patient showed decreased growth velocity of 2.2 cm in the last 6 months, which was thought to be “waning effect” of rhGH. The patient and his parents wanted to improve his height using AI, and after taking agreement about the drug, we added AI. During the combined treatment of 1 year and 10 months, his height was improved to 164.9cm (32%) with a gain of 16.2cm, and no adverse reaction was observed. Case 2. A 8 years and 2 months-old boy visited out clinic with short stature. His height was 116.3cm (0%) and weight was 22.6kg (14%). There have been no unusual findings in past history and family history. His MPH was calculated as 165.9cm. Bone age was 8.2 years by the TW3 method. He was also diagnosed as ISS, and started rhGH treatment. During 5 years and 1 month of rhGH treatment, the patient obtained additional 33.3cm growth to 149.6cm (17%). However, the patient showed progressive decreased growth velocity of 3.3 cm in the last 6 months, so we added AI treatment, and after the combined treatment of 1 year and 5 months, his height was 161.6cm (23%). After that, his parents wanted to quit AI, so we maintained only rhGH treatment for 7 months, and his final height was checked as 167cm. In our cases, we suggest that the additional use of AI to patients with progressive decreased growth rate during previous years of GH treatment can improve growth velocity again, and increase the final adult height with successful bone age suppression, which has not been reported before. Therefore, AI could be expected as effective booster drug to overcome waning effect of rhGH.

scholarly journals Final Height in Pubertal Short Stature Boys Treated with GH Combination with Letrozole: A retrospective study

2021 ◽  
Author(s):  
Yaping Ma ◽  
Ruofan Jia ◽  
Bingyang Xia ◽  
Bin Tang ◽  
Zhuangjian Xu
Keyword(s):  
Retrospective Study ◽  
Short Stature ◽  
Adult Height ◽  
Final Height ◽  
Standard Deviation Score ◽  
Bone Age ◽  
Growth Potential ◽  
Target Height ◽  
Final Adult Height ◽  
Epiphyseal Fusion

Abstract BackgroundThe growth potential of pubertal short stature boys is limited by the effect of estrogen on epiphyseal fusion. This study aims to identify the efficacy and safety of growth hormone (GH) combination with letrozole on final adult height (FAH) in pubertal short stature boys. MethodsThis is a retrospective study. Among pubertal short stature boys who treated with GH and letrozole were be followed up in our hospital, 20 cases reached FAH. ResultsBaseline chronological age were 12.12±1.14yr, bone age were 13.00±0.93yr. The treatment duration was 1.94±0.67yr. The height standard deviation score for bone age was increased from -1.46±0.51 to -0.12±0.57 (p<0.000). The predicted FAH before treatment, predicted FAH after treatment, FAH, and genetic target height were 161.02 ±4.12 cm, 172.11±4.20 cm, 172.67±2.72cm and 167.67±3.56 cm, respectively. There was significant differences between predicted FAH before treatment and after treatment (p<0.000), as well as predicted FAH before treatment and genetic target height (p<0.000).The predicted FAH after treatment was higher than that of genetic target height (p<0.001), as well as FAH and genetic target height (p<0.000). ConclusionsThe GH combination with letrozole can enhance the FAH in pubertal short stature boys. No significant side effects were observed.

Constitutional Delay of Growth: Expected versus Final Adult Height

PEDIATRICS ◽  
1991 ◽  
Vol 87 (1) ◽  
pp. 82-87 ◽  
Author(s):  
Stephen LaFranchi ◽  
Cheryl E. Hanna ◽  
Scott H. Mandel
Keyword(s):  
Adult Height ◽  
Hormone Secretion ◽  
Growth Hormone Secretion ◽  
Human Growth ◽  
Normal Growth ◽  
Bone Age ◽  
Final Adult Height ◽  
Constitutional Delay ◽  
Height Predictions

Constitutional delay of growth and puberty is believed to represent a variation of normal growth, and it is expected that children with this condition will grow for a longer duration than average and reach a height that is normal for their genetic potential. The records of children with constitutional delay of growth and puberty who were initially seen in the Pediatric Endocrine Clinic at the Oregon Health Sciences University between 1975 and 1983 were retrospectively reviewed. Criteria for study included a height more than 2 SD below the mean, a significantly delayed bone age, and a normal growth velocity on follow-up. Forty-two subjects were located and final adult height measurements were obtained. At contact, the 29 male subjects (mean age = 23.9 years) were 169.5 ± 4.5 cm tall (mean ± SD), and the 13 female subjects (mean age = 20.5 years) were 156 ± 3.8 cm tall. Adult height predictions during follow-up, using either the Bayley-Pinneau or Roche-Wainer-Thissen method, were close to final adult heights. The males were 1.2 SD and the females 1.3 SD below the 50th percentile as adults. This finding was not fully explained by genetic short stature; the males fell 5.1 cm and the females 5.3 cm below target heights based on midparental heights. It is concluded that this discrepancy is most likely explained by a selection bias of the shortest children referred to and observed in a subspecialty clinic, although a defect in human growth hormone secretion or function in children at the far end of the spectrum of constitutional delay of growth and puberty cannot be excluded.

scholarly journals Idiopathic short stature due to novel heterozygous mutation of the aggrecan gene

2015 ◽  
Vol 28 (7-8) ◽  
Author(s):  
Jose Bernardo Quintos ◽  
Michael H. Guo ◽  
Andrew Dauber
Keyword(s):  
Short Stature ◽  
Adult Height ◽  
Frameshift Mutation ◽  
Index Case ◽  
Bone Age ◽  
Heterozygous Mutation ◽  
Maternal Grandfather ◽  
Whole Exome ◽  
Heterozygous Variant ◽  
History Of

AbstractRecently, whole exome sequencing identified heterozygous defects in the aggrecan (We report a novel frameshift mutation inWe present a 5 1/2-year-old male with a family history of short stature in three generations. The maternal grandfather stands 144.5 cm (Ht SDS –4.7), mother 147.7 cm (Ht SDS –2.6), and index case 99.2 cm (Ht SDS –2.7). Our prepubertal patient has significant bone age advancement (bone age 8 years at chronologic age 5 1/2 years) resulting in a poor predicted adult height of 142 cm (Ht SDS –5.1). DNA sequencing identified a novel heterozygous variant inMutations in the

THE FUNCTIONAL STATE OF THE HYPOTHALAMO-PITUITARY AXIS AFTER HIGH-DOSE OESTROGEN THERAPY IN EXCESSIVELY TALL GIRLS

1979 ◽  
Vol 91 (1) ◽  
pp. 19-29 ◽  
Author(s):  
J. P. Hanker ◽  
G. Schellong ◽  
H. P. G. Schneider
Keyword(s):  
Functional State ◽  
Adult Height ◽  
Bone Age ◽  
Steroid Treatment ◽  
High Dose ◽  
Functional Disturbance ◽  
Final Adult Height ◽  
Tall Girls ◽  
Lh Rh

ABSTRACT Sixteen excessively tall girls were treated with 0.3 mg of ethinyloestradiol daily and 10 mg of norethisterone for 5 days every 3 weeks for 7–26 months. The reduction of adult height varied from 0–12.3 cm, depending on the bone age (115/12–148/12) before treatment. The more advanced the bone age was the less final adult height was reduced. The functional state of the hypothalamo-pituitary axis was assessed by standardized LH-RH testing immediately after termination of therapy as well as 1, 4, 8 and 12 weeks thereafter. Basal levels of oestradiol and prolactin were recorded before each test. Absent LH-responses to LH-RH were observed in all girls when therapy was stopped. Four to eight weeks later the LH responses had normalized in 13 girls and 12 weeks after therapy normal LH responses were found in 14 girls. Mean basal oestradiol levels were low (20 ± 9 pg/ml) (X̄ ± sd) at the end of therapy but increased significantly (P < 0.0025) to levels similar to different stages of the menstrual cycle after 4 weeks. In contrast mean basal prolactin levels were elevated (21±9 ng/ml) (X̄ ± sd) when therapy was stopped. Within one week a significant (P < 0.01) decrease to values averaging 13 ± 4 ng/ml (sd) was seen. A further but only moderate decline occurred until the 12th week after therapy. The decrease of prolactin paralleled to same extend the increase of endogenous oestradiol. All girls experienced spontaneous menstrual bleedings within 3 to 22 weeks after termination of therapy. In all cases but one menses have been regular since. The data presented suggest that no major functional disturbance of the hypothalamo-pituitary axis has to be expected after long-term steroid treatment in excessively tall girls.

scholarly journals SAT-LB14 Anthropometry and Vertebral Abnormality in Children With Transfusion-Dependent Thalassemia in Phramongkutklao Hospital, Bangkok, Thailand

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Nawachai Lertvivatpong ◽  
Voraluck Phatarakijnirund
Keyword(s):  
Short Stature ◽  
Adult Height ◽  
Screening Program ◽  
Multivariate Logistic Regression Analysis ◽  
Growth Failure ◽  
Bone Age ◽  
Team Approach ◽  
Z Score ◽  
Lower Segment ◽  
Tertiary Center

Abstract Anthropometry and vertebral abnormality in Children with Transfusion-dependent Thalassemia in Phramongkutklao HospitalBackgrounds: Thalassemia is an untreatable inherited hematologic disorder, unless stem cell transplantation, characterized by anemia from decreased hemoglobin production. Growth failure is one of the most common endocrine dysfunction in children with transfusion-dependent thalassemia (TDT) Objective: To evaluate the prevalence and associated factors of anthropometry and vertebral abnormality in transfusion-dependent thalassemia (TDT) children in a single tertiary center. Method: A cross-sectional study was conducted in transfusion dependent thalassemia patients who had visited in pediatric hematology clinic during 1st January 2018 to 31st December 2019. Collaborators had examined by history taking, physical examinations, laboratory and radiology reviewed. Results: Eighty-one collaborators were enrolled. Mean age was 13.7 ± 6.4 years and 46 of them (56.8%) were male. Pre-transfusion Hb and serum ferritin were 8.0 ± 1.0 g/dL and 1,562 + 1,394 ng/mL, respectively. Twenty-one (25.9%) had short stature determined by predicted adult height (PAH) below target adult height (TAH), 27(33.3%) had decreased upper-lower segment ratio for and 21 (26%) had BMI z-score below -2SD for age. Delay puberty was found in 13.2% of patients. Radiological examinations revealed delayed bone age of 4.9% and osteopenia of 25.9% whereas no vertebral fracture was documented. In multivariate logistic regression analysis (backward Wald), Serum ALP (p=0.009), mean pre-transfusion hemoglobin &lt;9 g/dL (p&lt;0.001), osteopenia (p=0.05) and delay bone age (p=0.019) were associated with PAH below TAH. Duration of chelation (p=0.013) and osteopenia (p=0.015) were associated with decreased upper-lower segment ratio. Low serum calcium (p=0.009), high serum phosphate (p=0.04) and impaired fasting glucose (p=0.004) were associated with BMI z-score below -2SD for age. Conclusions: Anthropometry abnormalities, including short stature, abnormal upper-lower segment ratio and low BMI, are common in TDT children. However, no vertebral abnormality was found in this study. Routine screening program by multidisciplinary team approach should be applied in thalassemia children.Keywords: Thalassemia major, endocrinopathies, growth failure, short stature, body disproportion

GROWTH VELOCITY AND FINAL ADULT HEIGHT FOLLOWING SURGERY FOR CROHN'S DISEASE

1997 ◽  
Vol 25 (4) ◽  
pp. 451
Author(s):  
P D Han ◽  
G R Lichtenstein ◽  
W C Jeshion ◽  
J Berlin ◽  
D A Piccoli ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Growth Velocity ◽  
Adult Height ◽  
Final Adult Height
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