deviation score
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Author(s):  
Riddhi V. Patel ◽  
Anurag T. Bajpai ◽  
Hemangkumar V. Mendpara ◽  
Chetankumar C. Dave ◽  
Sajili S. Mehta ◽  
...  

Abstract Objectives Lack of systematic evaluation of short stature results in unnecessary work-up on one hand while missing pathology on the other. We have developed a mobile application that guides work-up based on age, auxology (height, BMI, and corrected standard deviation score), and skeletal maturation with an aim of reducing the diagnostic errors. Aim of this study is to develop and validate a mobile application for point of care evaluation of short stature. Methods The application was developed (n=400) and validated (n=412) on children and adolescents (2–18 years of age) presenting to our Pediatric Endocrinology Clinic with short stature. Height standard deviation score thresholds determining the need for workup were derived from Receiver Operating Characteristics (ROC) curve. Student’s t-test and ROC curves were used to identify the most appropriate parameter differentiating constitutional delay of growth and puberty (CDGP) from pathological and nutritional from endocrine causes. The validation of the application involved comparing the application predicted and clinical diagnosis at each step of the algorithm. Results The mobile application diagnosis was concordant with clinical diagnosis in 408 (99.0%) with discordance in four (two with CDGP labeled as growth hormone deficiency [GHD] and two with GHD labeled as CDGP). Conclusions Mobile application guided short stature assessment has a high concordance with the clinical diagnosis and is expected to help point of care short stature evaluation.


Author(s):  
Ashraf Soliman ◽  
Alan D. Rogol ◽  
Sohair Elsiddig ◽  
Ahmed Khalil ◽  
Nada Alaaraj ◽  
...  

Abstract Objectives Some idiopathic short stature (ISS) patients may have varying degrees of insulin-like growth factor 1 (IGFI) deficiency. Others with growth hormone deficiency (GHD) (peak GH < 7 ng/dL after provocation) have normal IGFI levels. Do children with ISS or those with GHD with variable pretreatment IGFI standard deviation score (IGFISDS) have different IGFI and growth responses to recombinant human growth hormone (rhGH) therapy? Methods We studied the effect of GH therapy (0.035–0.06 mg/kg/day) on linear growth and weight gain per day (WGPD) in children with ISS (n=13) and those with GHD (n=10) who have low pretreatment IGFISDS (IGF SDS < −1.5) and compared them with age-matched prepubertal children with ISS (n=10) and GHD (n=17) who had normal pretreatment IGFISDS. An untreated group of children with ISS (n=12) served as a control group. Results At presentation, the height standard deviation score (HtSDS) of children with ISS who had low pretreatment IGFISDS was significantly lower compared to the normal IGFI group. The age, body mass index (BMI), BMISDS, peak GH response to clonidine provocation and bone age did not differ between the two study groups. After 1 year of treatment with rhGH (0.035–0.06 mg/kg/day) IGFISDS increased significantly in both groups (p<0.05). Both had significantly increased HtSDS (catch-up growth). The increase in the HtSDS and WGPD were significantly greater in the lower pretreatment IGFISDS group. The IGFSDS, BMISDS, HtSDS and difference between HtSDS and mid-parental HtSDS were significantly greater in the rhGH treated groups vs. the not treated group. In the GHD groups (normal and low IGFISDS), after 1 year of GH therapy (0.03–0.05 mg/kg/day), the HtSDS increased significantly in both, (p<0.01). The WGPD and increment in BMI were significantly greater in children who had low pretreatment IGFISDS. There was a significant increase in the IGFSDS in the two treated groups (p<0.05), however, the WGPD was greater in the pretreatment low IGFISDS. Conclusions IGFI deficiency represents a low anabolic state. Correction of IGFI level (through rhGH and/or improved nutrition) in short children (ISS and GHD) was associated with increased linear growth and WGPD denoting significant effect on bone growth and muscle protein accretion.


2021 ◽  
Vol 1 (2) ◽  
pp. 56-65
Author(s):  
Ila Susani Pelo

This research aims to determine whether students’ reading comprehension can be improved by using the Folklore Story. The participants were 20 eleventh grade students. The researcher had carried out research during the pandemic, the number of students was limited by the school. The research employed true experimental and need pre-test and post-test to both experimental class and control class. The population of this research is students in the eleventh grade of Man 1 Palu. The students were selected randomly. They are 20 students of class XI MIA 1 as the experiment class and XI MIA 4 as a control class. The research analysed the data statistically data collection. The result of data analysis shows that the experimental class (86) is higher than the mean score of the control class (69). The deviation score of experimental class (8.75) is higher than of the control class (5.37). So, the result of t-counted (1.644) which is higher than t-table (0.081). in analyzing the data, the researcher used 0.05 level of significance and the degree of freedom (df=10+10-2) it means that the hypothesis of the research was accepted. Therefore, the Folklore Story can improve students’ reading comprehension.


Author(s):  
K. A. Singh ◽  
N. Harne ◽  
H. Shah

Abstract Background Little literature exists regarding aggressive treatment of the extrusion in the early stage of the disease and the outcome at skeletal maturity. The purpose of the study was to evaluate the outcome of the disease with onset younger than 7 years, treated in the early stage of the disease, with aggressive management of significant extrusion (immediate containment with fixed abduction brace in children less than 5 years and varus derotation osteotomy in older children), and reached skeletal maturity. Methods All children with the age of onset younger than 7 years of disease during active Perthes disease were prospectively followed. Children with early stages of the disease (modified Elizabethtown classification) and reached skeletal maturity were included (68 children). The extrusion of the femur head was calculated by Reimer’s migration index on both sides. A migration difference 12 % or above was considered as “significant extrusion”. Children without significant extrusion were treated non-operatively; children with significant extrusion were treated with varus derotation osteotomy. The final radiological outcome was assessed by the Stulberg classification and sphericity deviation score (SDS). The independent “t” test and Chi-square test were done to compare the difference between the two groups. Results The mean age at the onset and the final follow-up was 5.7 years and 15.3 years. The frequency of significant extrusion was 57%. At the final follow-up, an excellent clinical outcome and radiological outcomes (in 88% hips) were noted. There was no significant difference in the Stulberg groups and SDS (sphericity deviation score) in both groups. Conclusion The outcome of the children who had the age of onset of the disease less than 7 years was good with early and aggressive management of the extrusion. The reversal of extrusion is associated with a similar result of non-operative children in this age group. Level of evidence III.


Med ◽  
2020 ◽  
Author(s):  
Eivind Heggernes Ask ◽  
Astrid Tschan-Plessl ◽  
Thea Johanne Gjerdingen ◽  
Michelle Lu Sætersmoen ◽  
Hanna Julie Hoel ◽  
...  

2020 ◽  
Vol 26 (11) ◽  
pp. 1255-1268
Author(s):  
Hanting Liang ◽  
Hui Miao ◽  
Hui Pan ◽  
Hongbo Yang ◽  
Fengying Gong ◽  
...  

Objective: There are numerous reasons for short stature, including mutations in osteochondral development genes. ACAN, one such osteochondral development gene in which heterozygous mutations can cause short stature, has attracted attention from researchers in recent years. Therefore, we analyzed six cases of short stature with heterozygous ACAN mutations and performed a literature review. Methods: Clinical information and blood samples from 6 probands and their family members were collected after consent forms were signed. Gene mutations in the probands were detected by whole-exome sequencing. Then, we searched the literature, performed statistical analyses, and summarized the characteristics of all reported cases. Results: We identified six novel mutations in ACAN: c.1411C>T, c.1817C>A, c.1762C>T, c.2266G>C, c.7469G>A, and c.1733-1G>A. In the literature, more than 200 affected individuals have been diagnosed genetically with a similar condition (height standard deviation score [SDS] −3.14 ± 1.15). Among affected individuals receiving growth-promoting treatment, their height before and after treatment was SDS −2.92 ± 1.07 versus SDS −2.14 ± 1.23 ( P<.001). As of July 1, 2019, a total of 57 heterozygous ACAN mutations causing nonsyndromic short stature had been reported, including the six novel mutations found in our study. Approximately half of these mutations can lead to protein truncation. Conclusion: This study used clinical and genetic means to examine the relationship between the ACAN gene and short stature. To some extent, clear diagnosis is difficult, since most of these affected individuals’ characteristics are not prominent. Growth-promoting therapies may be beneficial for increasing the height of affected patients. Abbreviations: AI = aromatase inhibitor; ECM = extracellular matrix; GnRHa = gonadotropin-releasing hormone analogue; IQR = interquartile range; MIM = Mendelian Inheritance in Man; PGHD = partial growth hormone deficiency; rhGH = recombinant human growth hormone; SDS = standard deviation score; SGA = small for gestational age; SGHD = severe growth hormone deficiency


Author(s):  
Louise Jayne Apperley ◽  
Renuka Ramakrishnan ◽  
Poonam Dharmaraj ◽  
Urmi Das ◽  
Mohammed Didi ◽  
...  

Background: Noonan syndrome is an autosomal dominant condition with an incidence of 1:1000 to 1:2500. The disorder is associated with distinct dysmorphic features, cardiac anomalies, developmental delay and delayed puberty. Short stature is a recognised feature of Noonan syndrome. Objectives: The aim of this study is to assess the effect of growth hormone treatment in patients with Noonan syndrome. Methods: Retrospective data was collected from patients with Noonan syndrome treated with growth hormone. The results were analysed with variables expressed as mean values and standard deviation scores. Results: Twelve Noonan syndrome patients (M: F = 10:2) treated with growth hormone were identified. The mean age of starting growth hormone was 8 years, with baseline height standard deviation score of -2.96 (range: -1.64 to -5.54). The height standard deviation score significantly improved to -2.50 (P = 0.0035) and then -2.22 (P = 0.0025), following one and two years of treatment, respectively. The average height velocity for the patients prior to starting treatment was 5.16cm/year (range: 2.4 - 8.2 cm/year), which significantly improved to 7.76cm/year (ranging from 4.1 to 12.8 cm/year) after one year of growth hormone treatment (P = 0.020) and to 6.51cm/year at the end of two years. Conclusions: Our study has shown that growth hormone treatment significantly improves the height standard deviation score of patients with Noonan syndrome over a two-year course of growth hormone therapy without any side effects. Further research is required to analyse the long-term effect of growth hormone therapy in patients with Noonan syndrome, including the impact on final adult height.


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