scholarly journals Appropriate Age for Height Control Treatment in Patients With Marfan Syndrome

2021 ◽  
Vol 12 ◽  
Author(s):  
Sung Eun Kim ◽  
Dong-Yun Lee ◽  
Min-Sun Kim ◽  
Sung Yoon Cho ◽  
Dong-Kyu Jin ◽  
...  

ObjectiveThis study aimed to determine the most appropriate age for height control treatment in patients with Marfan syndrome (MFS).Materials and MethodsThis retrospective study included patients with MFS who underwent height control treatment with estradiol valerate. The estrogen dose was increased according to the height change. The cut-off age for the maximum difference between the expected height and actual final height was evaluated.ResultsSeventeen patients were included in this study. The difference between the height predicted by the growth curve and the final height (gcHtD) and that predicted by the bone age and the final height (baHtD) was the largest in the 10.5 years age group (p=0.0045 and p=0.0237, respectively). The gcHtD was 10.6 (10.2, 13.5) cm for patients aged ≤10.5 years, whereas it was 0.6 (−3.65, 5.85) cm for patients aged >10.5 years. The baHtD was 10.1 (7.31, 11.42) cm for patients aged ≤10.5 years, while it was 3.83 (0.84, 6.4) cm for patients aged >10.5 years. When height change was observed for a minimum of 6 months after completion of estrogen treatment, the average growth was 0.6 (0.2, 2.1) cm.ConclusionInitiating height control treatment before the age of 10.5 years is effective in female patients with MFS.

1986 ◽  
Vol 113 (4_Suppl) ◽  
pp. S157-S163 ◽  
Author(s):  
K.W. KASTRUP ◽  
_ _

Abstract Early therapy with a low dose of estrogen (estradiol-17β) was given to 33 girls with Turner's syndrome (T.s.) for a period of 4 years. The dose (0.25-2 mg/day) was adjusted every 3 months to maintain plasma estradiol in the normal concentration range for bone age. Growth velocity was compared with that of untreated girls with T.s. All girls were above age 10 years. Bone age was below 10 years in 11 girls (group I) and above 10 years in 22 girls (group II). Growth velocity in the first year of treatment in group I 7.5 ± 1.3 cm (SD) with mean SD score (SDS) of +4.3 and in group II 4.9 ± 1.3 with mean SDS of +3.5. Growth velocity decreased in the following years to 1.6 ± 1.0 cm, SDS -1.44 in group I and 0.9 ± 0.6cm, SDS -2.34 in group II during the fourth year. Withdrawal bleeding occurred in 16 girls of group II after the mean of 23 (range 15-33) months and in 3 girls of group I after 15 to 51 months of treatment. The treatment did not cause an inappropriate acceleration of pubertal development. Breast development appeared in most girls by 3 months of treatment. Pubic hair appeared by 12 months of treatment in group I; it was present in most girls in group II at start of treatment. Final height is known for 12 girls of group II; it was 144.2 ± 4.5 cm. The final height as predicted at the start of therapy was 142.2 ± 5.3 cm. Bone age advanced in the first year of treatment by 2 years. Early treatment with small doses of estrogens induces a growth spurt and normalizes the events of puberty. This will presumably decrease the psychological risks associated with abnormally delayed development.


2020 ◽  
Vol 98 (Supplement_3) ◽  
pp. 15-15
Author(s):  
Bradley J Heins ◽  
Siane C Luzzi ◽  
Robert D Gardner

Abstract The objective of this study was to evaluate the taste preference of calves fed Chlorella sp. microalgae produced from dairy lagoon wastewater. The study was conducted at the University of Minnesota West Central Research and Outreach Center, Morris, MN, dairy during May 2019. Six Holstein and crossbred dairy heifer calves were fed 0 (control), 30, and 60 g of Chlorella sp. daily in a sequential elimination study. For the 7-d experiment, day 1 to 2 were for diet adaptation and day 3 to 4 were for data collection. During the final 3 days, the primarily consumed treatment was removed to determine the second preferred treatment. The microalgae used in this study was isolated from the dairy wastewater lagoon. The microalgae biomass was produced using outdoor hanging bag bioreactors with Chlorella sp. to recycle the dairy wastewater. The biomass was sterilized and kept frozen at -4°C until fed to calves. Calves were housed individually in hutches with outdoor access under solar panels, with free-choice water. Kendall’s coefficient of concordance was calculated to rank the consumption of the treatments from most to least preferred using JMP 14.3 statistical software. Pairwise comparisons and Tukey adjustment were applied to evaluate the difference between the treatments for total intake. Calves consumed more (P < 0.05) dry matter from control grain (3.4 kg/d) compared to 30 g microalgae grain (2.42 kg/d) and 60 g microalgae grain (1.56 kg/d) during the first 2-d period. During the second 2-d (d 3 and 4) segment, dry matter intake was reduced (P < 0.05) for the 60 g microalgae treatment compared to the control and 30 g microalgae treatment. Five of six calves in this study always ranked the control treatment first (P < 0.05) when given a choice, and ranked the 30 g microalgae second choice. Results indicated that microalgae maybe added to calf starter grains without any adverse effects; however, calves preferred calf starter grains without microalgae.


2002 ◽  
pp. 319-323 ◽  
Author(s):  
Y Rakover ◽  
A Silbergeld ◽  
I Lavi ◽  
R Masalha ◽  
IB Shlomo

OBJECTIVES: In the majority of children with short stature, the etiology is unknown. Mutations of the GH receptor (GHR) have been reported in a few children with apparent idiopathic short stature (ISS). These patients had low IGF-I, IGF-binding protein-3 (IGFBP-3) and GH-binding protein (GHBP), but a normal or exaggerated GH response to provocative stimuli, suggestive of partial GH insensitivity (GHI). We attempted to identify children with partial GHI syndrome, based on their response to GH provocative stimuli and other parameters of the GH-IGF-I axis. SUBJECTS AND METHODS: One hundred and sixty-four pre-pubertal children (97 boys, 67 girls) aged 7.2 (0.5-16.75) years were studied. All had short stature with height <3rd centile. The weight, bone age (BA) and body mass index (BMI) of the subjects, as well as the parents' heights and mid parental height (MPH) were assessed. Basal blood samples were taken for IGF-I, IGFBP-3 and GHBP. All subjects underwent a GH provocative test with either clonidine, arginine or insulin. The subjects were divided into three groups: (A) patients with peak GH concentration <18 mIU/l in two different provocative tests (GH deficiency - GHD, n=33); (B) patients with peak GH between 18.2 and 39.8 mIU/l (normal response, n=78); (C) patients with peak GH >40 mIU/l (exaggerated GH response, n=53). RESULTS: No significant differences were found in age, height (standard deviation score (SDS)), parental height (SDS) and the difference between chronological age and bone age (DeltaBA) between the groups. Patients with GHD were heavier (P=0.039) and had significantly higher BMI (SDS) (P=0.001) than the other groups. MPH (SDS) was lower in the group of exaggerated responders (P=0.04) compared with the other groups. No significant differences were found between the groups for the biochemical parameters when expressed nominally or in SDS, except for IGFBP-3 (SDS), which was lower in the GHD group (P=0.005). The GHBP levels were not lower in the group of exaggerated GH response to provocative stimuli. Height (SDS) correlated negatively with basal GH values in pooled data of all the subjects (r=-0.358, P<0.0001), in normal responders (r=-0.45, P<0.0001) and in the exaggerated responders (r=-0.341, P<0.0001), but not in the GHD group. CONCLUSION: Exaggerated GH response to provocative tests alone does not appear to be useful in identifying children with GHI.


Author(s):  
Lucia Schena ◽  
Cristina Meazza ◽  
Sara Pagani ◽  
Valeria Paganelli ◽  
Elena Bozzola ◽  
...  

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.


2017 ◽  
Vol 49 (10) ◽  
pp. 778-785 ◽  
Author(s):  
Ludmila Pedrosa ◽  
Joice de Oliveira ◽  
Paula Thomé ◽  
Cristiane Kochi ◽  
Durval Damiani ◽  
...  

AbstractAromatase inhibitors (AIs) have been used to recover height loss due to their capacity to delay growth plate closure. Long-term studies describing final heights are needed to determine the efficacy and safety profiles of these drugs for the treatment of impaired growth. This study aims to identify the therapeutic efficiency of AIs in improve growth and to describe potential adverse effects during treatment. Retrospective data analysis of 96 adolescents, among which 22 patients already attained near-final height, were followed at outpatient clinics of two referral centers. Patients were all in puberty and present idiopathic decrease in predicted adult height. Patients were treated with Anastrozole (ANZ: 1 mg/day) or Letrozole (LTZ: 2.5 mg/day) with/without recombinant human growth hormone (0.05 mg/kg/day) for 1.0 to 3.5 years (2.1±1.2 years). Height gain, body mass index, lipid, liver enzyme, gonadotropins and testosterone levels were described before and at the end of treatment. Predicted adult height (PAH) and NF height were compared with the TH. The height SDS (adjusted to bone age) significantly increased (p<0.05) in all groups [0.8±0.7 (ANZ), 0.7±0.7 (ANZ+GH), 0.3±0.5 (LTZ), and 1.2±0.8 (LTZ+GH)]; the latter group exhibited the highest increment of PAH and growth recovery to the TH (p<0.004). No significant side effects were observed. AI treatment, especially when used in association with GH was able to improve growth and the attainment of familial target height.


2019 ◽  
Vol 32 (1) ◽  
pp. 85-88 ◽  
Author(s):  
Xinrui Tan ◽  
Xiaochuan Wu ◽  
Jie Chen ◽  
Yan Wu ◽  
Shijun Li ◽  
...  

Abstract Background Aromatase excess syndrome (AEXS) is a rare autosomal dominant disorder caused by CYP19A1 overexpression. Clinical manifestations of AEXS include pre- or peri-pubertal gynecomastia, advanced bone age and compromised adult height. Case presentation Here we report an 8-year-old boy diagnosed with AEXS by chromosomal array that revealed a 1.1 Mb novel de novo duplication at 15q21.2, with a predicted final height of 157.4 cm. We prescribed letrozole and growth hormone (GH) to maximize his linear growth. Without further bone age advancement, his height increased from 137.7 cm to 144 cm after an 8-month treatment period. Conclusions We identified a novel duplication at 15q21.2 in AEXS, and found that aromatase inhibitor (AI) plus GH might provide a better growth-promoting approach for AEXS patients.


Author(s):  
Andressa C. Neves ◽  
Camila N. Bergamini ◽  
Rafaela de O. Leonardo ◽  
Manoel P. Gonçalves ◽  
Dilcemara C. Zenatti ◽  
...  

ABSTRACT This study aimed to evaluate the effect of applying increasing doses of biofertilizer obtained by the anaerobic digestion of cassava effluent on the development of crambe plants. The experiment was conducted in a protected environment at the Federal University of Paraná (UFPR), Palotina Sector, between April and August 2015. A completely randomized design was used, and five different treatments with the following doses were applied in five replicates: 0, 40, 80, 120, and 160 kg ha-1 of K2O. The following parameters related to plant development were evaluated: final height, stem diameter, number of branches, dry shoot and root biomass, mass of the grains, and oil content. The 160 kg K2O ha-1 dose was found to have the best influence on the plant development, because all the measured parameters reached their highest values at this dose, except for oil content, which attained the highest percentage in the case of the control treatment (0 kg ha-1 of K2O). This study proved that the biofertilizer obtained by anaerobic digestion of cassava effluent can be used as an alternative to regular fertilizers in cultivating crambe.


bionature ◽  
2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Erpi Nurdin ◽  
Gaby Maulida Nurdin

Abstract. In the growth of microorganisms such as fungi, it is necessary to grow a medium that can provide nutrients and as an energy source. Other alternative carbohydrate sources include potatoes, breadfruit, sago, and cassava, which is a distinctive and easy to find food companion. This type of research is descriptive with cross sectional design that aims to determine the difference in alternative media variation from various sources of carbohydrate to Candida albicans. The samples were then made to be the treatment for the creation of alternative media from various carbohydrate sources that further carried out the growth test against Candida albicans. The results of the study are the average growth of colonies on alternative media from Potato carbide sources, namely 655 colonies, breadfruit 1380 colonies, cassava 862 colonies, 372 sago colonies, and semi-synthetic media as a control of 874 colonies. This indicates there is growth of Candida albicans on all alternative media so that it can be used as an alternative medium of fungi growth, as well as the best growth of Candida albicans found in the media Breadfruit Dextrose Agar. Keywords: alternative media, carbohydrate sources, Candida albicans


2000 ◽  
Vol 85 (9) ◽  
pp. 3262-3265
Author(s):  
Marie-Christine Lebrethon ◽  
Ashley B. Grossman ◽  
Farhad Afshar ◽  
P. Nicholas Plowman ◽  
G. Michael Besser ◽  
...  

Abstract Cushing’s disease is associated with growth failure in childhood and adolescence. Growth and final height were analyzed in 10 patients who were cured or in remission after treatment of Cushing’s disease. Seven males and 3 females, aged 6.8–17.6 yr (bone age, 3.3- 15.4 yr), had transsphenoidal surgery, which was combined with pituitary irradiation (4500 cGy in 25 fractions) in 5 patients. At presentation, 5 patients were prepubertal (males), and 5 were pubertal (2 males and 3 females). The mean height sd score was −2.15 ± 1.26 (range, −0.21 to −4.32) compared with mean target height sd score of −0.43 ± 0.58. Height velocity in 6 patients was subnormal (0.9–3.8 cm/yr). After treatment, short-term height velocity, over a mean interval of 0.57 yr, in 8 patients not receiving human GH (hGH) therapy, was variable (range, 0.8–7.6 cm/yr). GH stimulation tests (insulin tolerance test/glucagon) in 9 subjects showed peak GH levels of 0.5–20.9 mU/L. Eight were treated with hGH (14 IU/m2·wk), combined in 2 girls and 1 boy with a GnRH analog. After 1 yr of hGH, the mean height sd score had increased from −2.45 ± 1.0 at initiation of hGH to −2.07± 1.2 (P = 0.01). GH therapy was continued until final height or latest assessment. The mean final height sd score (n = 6) was −1.24 ± 1.38, and at the latest assessment the mean height sd score (n = 4) was− 1.52 ± 1.33. Combining these 2 groups, the mean height sd score was −1.36 ± 1.29. The difference between final or latest height sd score and target height sd score was 0.93 ± 1.13, i.e. less (P = 0.005) than the difference between height and target height sd score of 1.72 ± 1.26 at presentation. In conclusion, catch-up and favorable long-term growth was seen after treatment for Cushing’s disease. Posttreatment GH deficiency was frequent, and early hGH replacement may have contributed to the encouraging outcome.


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