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 <9 g/dL (p<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

scholarly journals Long-Term Growth Hormone Therapy Does Not Advance Skeletal Maturation in Children and Adolescents

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A679-A679
Author(s):  
Benjamin Udoka Nwosu ◽  
Sadichchha Parajuli ◽  
Gabrielle Jasmin ◽  
Austin F Lee
Keyword(s):  
Growth Hormone ◽  
Short Stature ◽  
Adult Height ◽  
Recombinant Human Growth Hormone ◽  
Bone Age ◽  
Skeletal Maturation ◽  
Z Score ◽  
Rhgh Therapy ◽  
Catch Up

Abstract Context: There is no consensus on the effect of recombinant human growth hormone (rhGH) therapy on skeletal maturation in children despite the current practice of annual monitoring of skeletal maturation with bone age in children on rhGH therapy. Aims: To investigate the effects of long-term rhGH therapy on skeletal age in children and explore the accuracy of bone age predicted adult height (BAPAH) at different ages based on 13 years of longitudinal data. Methods: A retrospective longitudinal study of 71 subjects aged 2-18 years, mean 9.9 ± 3.8y, treated with rhGH for non-syndromic short stature for a duration of 2-14y, mean, 5.5 ± 2.6y. Subjects with syndromic short stature and systemic illnesses such as renal failure were excluded. Results: Bone age minus chronological age (BA-CA) did not differ significantly between baseline and the end of rhGH therapy (-1.05 ± 1.42 vs -0.69 ± 1.63, p=0.09). Piece-wise regression however showed a quantifiable catch-up phenomenon in BA of 1.6 months per year of rhGH therapy in the first 6.5y, 95%CI 0.023 - 0.229, p=0.017, that plateaued thereafter, β=0.015, 95% CI -0.191-0.221, p=0.88. There was no relationship between BAPAH z score – height z score and the duration of rhGH therapy, p=0.68. BAPAH overestimated final adult height in younger subjects but became more precise in older subjects (p<0.0001). Conclusion: Long-term rhGH therapy demonstrated an initial catch-up phenomenon in skeletal maturation in the first 6.5y that plateaued thereafter with no overall significant advancement in bone age. These findings are reassuring and do not support the practice of yearly monitoring of skeletal maturation with bone age in children on rhGH therapy, especially in younger subjects where BAPAH is imprecise.

Pubertal development and adult height in patients with congenital hypothyroidism detected by neonatal screening in southern Brazil

2020 ◽  
Vol 33 (11) ◽  
pp. 1449-1455
Author(s):  
Suzana Nesi-França ◽  
Rodrigo B. Silveira ◽  
Juliana Cristina R. Rojas Ramos ◽  
Adriane A. Cardoso-Demartini ◽  
Monica N. Lima Cat ◽  
...  
Keyword(s):  
Congenital Hypothyroidism ◽  
Neonatal Screening ◽  
Adult Height ◽  
Screening Program ◽  
Pubertal Development ◽  
Z Score ◽  
Normal Range ◽  
Adequate Treatment ◽  
Increased Risk ◽  
Puberty Onset

AbstractObjectivesAdequate treatment of congenital hypothyroidism (CH) is required for normal growth and sexual development. To evaluate pubertal development in patients with permanent CH detected by a statewide Neonatal Screening Program of Paraná and, secondly, to evaluate adult height (AH) in a subgroup of patients.MethodsClinical, laboratory, and auxological data obtained from medical records of 174 patients (123 girls).ResultsMedian chronological age (CA) at treatment initiation was 24 days, and mean initial levothyroxine dose was 11.7 ± 1.9 μg/kg/day; mean CA at puberty onset was 11.5 ± 1.3 years (boys) and 9.7 ± 1.2 years (girls); mean CA in girls who underwent menarche (n=81) was 12.1 ± 1.1 years. Thyroid-stimulating hormone (TSH) values above the normal range were observed in 36.4% of the boys and 32.7% of the girls on puberty onset, and in 44.6% around menarche. Among 15 boys and 66 girls who had reached the AH, the median height z-score value was significantly greater than the target height (TH) z-score value in boys (p=0.01) and in girls (p<0.001). Boys with normal TSH values at puberty onset had greater mean AH z-score compared with boys with TSH values above the normal range (p=0.04).ConclusionsIn this group, pubertal development in girls with CH was not different from that reported in healthy girls in the general Brazilian population. Boys with higher TSH at puberty onset may have an increased risk of not reaching their potential height compared with those with normal TSH during this period. In a subgroup who attained AH, the median AH z-score was greater than the median TH z-score.

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

scholarly journals The Efficacy of Anastrozole and Growth Hormone Therapy on Adult Height in Six Adolescent Males with Growth Hormone Deficiency or Idiopathic Short Stature

2017 ◽  
Vol 2017 ◽  
pp. 1-6
Author(s):  
Serife Uysal ◽  
Juanita K. Hodax ◽  
Lisa Swartz Topor ◽  
Jose Bernardo Quintos
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Short Stature ◽  
Adult Height ◽  
Bone Age ◽  
Idiopathic Short Stature ◽  
Hormone Deficiency ◽  
Chronological Age ◽  
Gh Therapy ◽  
The Mean

Background. Data on adult height outcomes of the use of Anastrozole and Growth Hormone (GH) in pubertal males with Growth hormone deficiency (GHD) and Idiopathic short stature (ISS) are limited. Objective. We examined the effect of Anastrozole and GH therapy on near adult height (NAH) with pubertal males with GHD or ISS. Methods. Retrospective review of 419 charts from 2008 to 2015. The primary outcomes are NAH compared to mid-parental target height (MPTH) and predicted adult height (PAH). Results. We identified 23 patients (5 SGA/IUGR, 1 Noonan syndrome, 6 GHD, and 11 ISS). Six patients (4 GHD; 2 ISS) achieved NAH. Prior to Anastrozole treatment, the mean chronological age was 13.9±0.2 years (range 13.7–14.4), bone age was 13.6±0.6 years (range 12.5–14), mean height SDS was -1.5±0.5 (range −0.8 to −2.3), and mean PAH was 162.6±5.9 cm (range 153.5–168.6). MPTH was 173.6 cm ± 7 (range 161.8–181.6). Patients received Anastrozole for an average of 30.5 months (range 19–36 months). At NAH, the mean chronological age was 16.7±0.8 years (range 15.9–18.1 years) and height was 170±1.8 cm (range 168.5–173.4 cm). The mean height SDS improved to +0.81±0.6 (range +0.08 to +1.92, p=0.002). Net height gain was 7.3 cm compared to pretreatment PAH (p<0.01) and overall the mean adult height remained 3.5 cm below MPTH. Conclusion. Anastrozole and GH therapy can be effective in augmenting adult height without significant side effects. However, the long-term safety and efficacy of aromatase inhibitor use in pediatrics remain limited.

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.

scholarly journals Towards a Rational and Efficient Diagnostic Approach in Children Referred for Growth Failure to the General Paediatrician

2019 ◽  
Vol 91 (4) ◽  
pp. 223-240 ◽  
Author(s):  
Jan M. Wit ◽  
Gerdine A. Kamp ◽  
Wilma Oostdijk ◽  
Keyword(s):  
Short Stature ◽  
Diagnostic Yield ◽  
Secondary Growth ◽  
Growth Failure ◽  
Bone Age ◽  
Bone Diseases ◽  
Diagnostic Approach ◽  
Hormone Deficiency ◽  
Growth Disorders ◽  
Metabolic Bone Diseases

Based on a recent Dutch national guideline, we propose a structured stepwise diagnostic approach for children with growth failure (short stature and/or growth faltering), aiming at high sensitivity for pathologic causes at acceptable specificity. The first step is a detailed clinical assessment, aiming at obtaining relevant clinical clues from the medical history (including family history), physical examination (emphasising head circumference, body proportions and dysmorphic features) and assessment of the growth curve. The second step consists of screening: a radiograph of the hand and wrist (for bone age and assessment of anatomical abnormalities suggestive for a skeletal dysplasia) and laboratory tests aiming at detecting disorders that can present as isolated short stature (anaemia, growth hormone deficiency, hypothyroidism, coeliac disease, renal failure, metabolic bone diseases, renal tubular acidosis, inflammatory bowel disease, Turner syndrome [TS]). We advise molecular array analysis rather than conventional karyotyping for short girls because this detects not only TS but also copy number variants and uniparental isodisomy, increasing diagnostic yield at a lower cost. Third, in case of diagnostic clues for primary growth disorders, further specific testing for candidate genes or a hypothesis-free approach is indicated; suspicion of a secondary growth disorder warrants adequate further targeted testing.

Prevalence and Predictors of Growth Impairment and Short Stature in Pediatric-Onset Inflammatory Bowel Disease

Digestion ◽  
2019 ◽  
Vol 101 (6) ◽  
pp. 674-682
Author(s):  
Firas Rinawi ◽  
Amit Assa ◽  
Tal Almagor ◽  
Tomer Ziv-Baran ◽  
Raanan Shamir
Keyword(s):  
Risk Factors ◽  
Short Stature ◽  
Adult Height ◽  
Male Gender ◽  
Z Score ◽  
Growth Impairment ◽  
Bowel Diseases ◽  
Z Scores ◽  
Inflammatory Bowel ◽  
Pediatric Onset

<b><i>Background and Aims:</i></b> Growth impairment is common in children with inflammatory bowel diseases (IBD). However, the magnitude of short stature at adulthood is not well characterized. We aimed to determine the prevalence and predictors of growth impairment at diagnosis and adulthood in children with IBD. <b><i>Methods:</i></b> Height z-scores at diagnosis of IBD and at adulthood among 291 children with Crohn’s disease (CD) and 125 with ulcerative colitis (UC) were retrieved retrospectively and compared to matched controls. Growth impairment at diagnosis was defined as height z-score for age less than or equal to –1 and short stature at adulthood as less than or equal to –2. <b><i>Results:</i></b> Mean height z-score at adulthood in subjects with CD or UC was significantly different from controls although mean height did differ in males only (CD 172.3 cm ± 6.7, UC 172.7 cm ± 5.3, controls: 174.2 cm ± 7.3, <i>p</i> = 0.003 and <i>p</i> = 0.047, respectively). Diagnosis prior to final stage of puberty and male gender were risk factors for being short statured at adulthood in CD (mean difference [MD] 2.5, <i>p</i> = 0.013 and MD 6.25, <i>p</i> = 0.001, respectively) and UC (MD 4.9, <i>p</i> = 0.011 and MD 3.3, <i>p</i> = 0.034, respectively). <b><i>Conclusion:</i></b> Increased proportion of pediatric-onset IBD patients has growth impairment at adulthood. Male gender and diagnosis prior to puberty were found to impose risk for reduced adult height in both diseases.

scholarly journals A Clinical Study on the Treatment of Children’s Short Stature with Auxiliary Comprehensive Management Combined with Growth Patch

2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Haiying Feng ◽  
Weizhu Zhao ◽  
Huijun Yu ◽  
Guanfu Wang ◽  
Qunhong Wang
Keyword(s):  
Body Weight ◽  
Short Stature ◽  
Statistical Difference ◽  
Adult Height ◽  
Bone Age ◽  
Combination Group ◽  
Clinical Effects ◽  
Comprehensive Management ◽  
Before And After ◽  
Igfbp 3

Objective. To explore the clinical effect of auxiliary comprehensive management combined with growth patch in the treatment of childhood idiopathic short stature (ISS). Methods. From September 2017 to December 2019, 120 children with ISS who met the selection criteria were collected. Random number table method divided them into 2 groups: one group was given auxiliary comprehensive management and recorded as the routine group (n = 60), and the other group was given auxiliary comprehensive management and combined growth patch treatment and recorded as the combination group (n = 60). The course of treatment was 12 months. The effects of the two methods on children’s height, bone age, body weight, and insulin-like growth factor (IGF)-1 and IGF-binding protein (IGFBP)-3 levels were compared. Results. There was no statistical difference between the two groups in baseline height, genetic height, baseline bone age, baseline body weight, and body weight before and after treatment ( P > 0.05 ). After treatment, the heights of the two groups were higher than before for the same group, the height growth values and predicted adult height of the combination group were higher than those of the routine group, and the predicted adult height of the combination group was higher than the genetic height of the same group P < 0.001 . There was no statistical difference in IGF-1 and IGFBP-3 levels before treatment between the two groups ( P > 0.05 ). The levels of IGF-1 and IGFBP-3 after treatment in the two groups were higher than those in the same group before treatment, and the combination group was higher than that in the routine group ( P < 0.05 ). Conclusion. On the basis of auxiliary comprehensive management, combined with growth patch for the treatment of children with ISS, it can effectively increase the height of the children, improve the levels of serum IGF-1 and IGFBP-3, and have significant clinical effects, which is beneficial to the healthy growth of the children.

scholarly journals Anastrozole Improves Final Adult Height in Severe Hypothyroidism With Rapid Pubertal Progression

2021 ◽  
Vol 5 (5) ◽  
Author(s):  
Juanita K Hodax ◽  
Lisa Swartz Topor ◽  
Shara R Bialo ◽  
Jose Bernardo Quintos
Keyword(s):  
Adult Height ◽  
Final Height ◽  
Growth Failure ◽  
Brain Magnetic Resonance Imaging ◽  
Bone Age ◽  
Hashimoto Thyroiditis ◽  
Once Daily ◽  
Final Adult Height ◽  
Epiphyseal Fusion ◽  
Height Prediction

Abstract Severe prolonged hypothyroidism due to Hashimoto thyroiditis may lead to rapid pubertal progression and compromised adult height after initiation of levothyroxine (LT4) therapy. There are no reports of aromatase inhibitor use to augment height in these patients. We describe a patient with severe hypothyroidism and growth failure who experienced rapid pubertal and bone age maturation on initiation of LT4 therapy. Anastrozole was added after 2 years to delay epiphyseal fusion. A boy aged 12 years and 1 month presented to the endocrine clinic with short stature and a markedly delayed bone age of 6 years. Brain magnetic resonance imaging showed a 1.5 × 1.0 × 1.2-cm enlarged lobular anterior pituitary. On examination, his height was –3.5 SD score (SDS) and weight was –2.87 SDS. Laboratory studies showed elevated thyrotropin (TSH) 850.6 μIU/mL, low free thyroxine 0.25 ng/dL, and elevated antithyroid antibodies. LT4 was initiated with normalization of TSH after 6 months. After 2 years of treatment he demonstrated catch-up growth with rapid bone age maturation, and his predicted adult height was compromised at 164.6 cm vs a midparental target height of 175.4 cm. Anastrozole 1 mg once daily was initiated. After 1.5 years of anastrozole treatment, the rate of his bone age advancement had slowed and his linear growth remained robust. The patient’s near-final height (167 cm) was 2.4 cm taller than his height prediction prior to starting anastrozole. Anastrozole slowed the rate of bone age advancement in a patient with severe hypothyroidism and rapidly progressive puberty during treatment with LT4, leading to improvement in near-final height.

scholarly journals Hydrocortisone Suspension Provides Similar Growth Outcomes as Hydrocortisone Tablets in Young Children With Congenital Adrenal Hyperplasia: A Cross Sectional Study

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A84-A84
Author(s):  
Heba Al-Rayess ◽  
O Yaw Addo ◽  
Elise Palzer ◽  
Mu’taz Jaber ◽  
Kristin Fleissner ◽  
...  
Keyword(s):  
Young Children ◽  
Adult Height ◽  
Bone Age ◽  
Age At Diagnosis ◽  
Z Score ◽  
Linear Regression Models ◽  
Target Height ◽  
Cross Sectional ◽  
Final Adult Height ◽  
Z Scores

Abstract Young children with CAH require small doses (0.1–1.25mg) and incremental adjustments of hydrocortisone (HC) to control excess androgen production and avoid the negative effects of overtreatment. A recent 6 hour pharmacokinetic/pharmacodynamic study reported that alcohol-free HC suspension provides similar cortisol exposure to tablets (1), but more data is needed to assess its clinical efficacy. We performed a chart review to determine the effect of the alcohol-free HC suspension compared to tablets on height, weight, BMI, bone age z-scores and corrected height z-scores to target height z-scores in children aged 2 yrs and 4 yrs in a cohort with classic CAH. Independent 2-sample t-tests examined cumulative and average HC dose at 2 and 4 yrs. Triple logistic modeling of longitudinal heights were used to calculate predicted near-adult height. Adjusted linear regression models assessed the effect of HC suspension compared to tablets on final adult height. Charts of 130 children (70 females, 100 salt wasting and 30 simple virilizing) were reviewed. At 2 yrs, 97 were treated with tablets and 33 with suspension (17 previously switched from tablets). At 4 yrs, 89 were treated with tablets and 41 with suspension (25 switched). No significant differences in height or BMI z-scores at both 2 and 4 yrs, before or after adjusting for age at diagnosis and sex were found. Bone age z-scores averaged 7.2 SDs lower for patients treated with HC suspension only compared to patients on HC tablets at age 4 (p&lt;0.001), and 5.93 SDs lower for patients switched from tablets to suspension compared to tablets (p&lt;0.001). The suspension group received 16% lower (p=0.055) and 25% lower (p=0.002) cumulative HC doses by the ages of 2 yrs and 4 yrs respectively. Average daily HC dose was lower by 3.44 and by 4.46 mg/m2/d over the first 2 and 4 yrs of life respectively. No significant differences were found between patients treated with tablets and suspension in the predicted final adult height, its z-score or its corrected z-score to target height after adjusting for age at diagnosis, sex and diagnosis. Our data indicates that treatment with alcohol-free HC suspension decreased androgen exposure as shown by lower bone age z-scores, generated no significant differences in SDS in observed height, BMI or predicted near-adult height, and allowed for lower average and cumulative daily HC dose compared to HC tablets in children with CAH. Reference: (1) Sarafoglou et al., J Clin Pharmacol.2015;55(4):452–7.

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