Current Concepts in Tall Stature and Overgrowth Syndromes

2001 ◽  
Vol 14 (s2) ◽  
Author(s):  
S.L.S. Drop ◽  
N. Greggio ◽  
M. Cappa ◽  
S. Bernasconi
Keyword(s):  
Sex Steroids ◽  
Bone Age ◽  
Prediction Equation ◽  
Favourable Effect ◽  
Tall Stature ◽  
Growth Data ◽  
Therapeutic Modalities ◽  
Height Prediction ◽  
High Doses ◽  
Overgrowth Syndromes

AbstractIn this overview an update is given on the pathogenesis, classification and differential diagnosis of overgrowth syndromes. In addition, height prognosis and therapeutic modalities available for managing mainly constitutional tall stature are discussed. Constitutional tall stature comprises normal variants in which one or both parents are tall. Primary disorders may have a prenatal onset and may be of chromosomal or genetic origin. Secondary overgrowth syndromes are most often the result of hormonal disturbances. Height prediction plays a key role in the management of tall children. Prediction equation models have been developed based on the growth data of healthy tall children. There is general agreement that a favourable effect on reducing ultimate height is obtained using high doses of sex steroids (girls 100-300 μg ethinyl- oestradiol; boys testosterone (T) ester depot preparations 250-1000 mg/month), the height reduction being greater when the treatment is started at a lower chronological and/or bone age. An alternative is the induction of puberty with low doses of sex steroids (girls 5-50 μg ethinyloestradiol; boys T esters 25-50 mg/m

Growth (including short stature and tall stature)

2020 ◽  
pp. 1-48
Author(s):  
Gary Butler ◽  
Jeremy Kirk
Keyword(s):  
Short Stature ◽  
Sex Steroids ◽  
Genetic Disorders ◽  
Bone Age ◽  
Birth Size ◽  
Tall Stature ◽  
Prader Willi Syndrome ◽  
Chronic Disorder ◽  
Growth Problem ◽  
Underlying Disorder

• Growth occurs in three separate phases, all of which are under different nutritional and/or hormonal controls: ◦ infantile (mainly nutritional) ◦ childhood (hormonal, mainly growth hormone (GH)) ◦ pubertal (hormonal; GH and sex steroids acting synergistically). • Height: ◦ should be measured supine up until 2 years of age, and standing after that, and plotted on appropriate charts ◦ is a normally distributed variable, with extremes (0.4th/2nd centiles and 99.6th/98th centiles) arbitrarily defined as short and tall stature respectively. • Two major sets of genes determine height and rate of development; the first is assessed using mid-parental and target height, and the second using bone age. • Short stature: ◦ Failure to achieve an acceptable height can be due to a primary growth problem, or secondary to an underlying disorder. ◦ Causes include familial, genetic disorders (syndromic), small birth size, chronic illness, psychological, environmental, and endocrine. ◦ Generally, short stature due to a hormonal issue is associated with (relative) overweight, and that due to an underlying chronic disorder with (relative) underweight. ◦ GH therapy is licensed for short stature in children in the following situations: GH deficiency, Turner syndrome, chronic renal insufficiency, children born small for gestational age, Prader–Willi syndrome, and SHOX deficiency. • Tall stature: ◦ Although in theory this should present as frequently as short stature, in practice this is not the case. ◦ The commonest cause of tall stature is constitutional, although other forms include: ■ syndromic: e.g. Klinefelter, Marfan, and Sotos syndromes ■ hormonal: GH, sex steroid excess. ◦ Therapy (sex steroids, GH blockade, epiphyseal stapling) is less effective than in short stature.

scholarly journals Treatment of tall stature in boys with somatostatin analogue 201–995: effect on final height

2006 ◽  
Vol 154 (2) ◽  
pp. 253-257 ◽  
Author(s):  
C Noordam ◽  
S van Daalen ◽  
BJ Otten
Keyword(s):  
Adult Height ◽  
Final Height ◽  
Bone Age ◽  
Somatostatin Analogue ◽  
Tall Stature ◽  
Short Term ◽  
Greulich And Pyle ◽  
Long Term Treatment ◽  
Height Prediction ◽  
Final Height Prediction

Background: An optimal treatment for tall stature in boys in terms of efficacy and safety is not available. Treatment with somatostatin analogue 201–995 (SMS) has been tried with positive short-term results. Methods: We evaluated the effect of SMS treatment on reducing adult height. Over 2 years, 16 boys presenting to our university hospital with tall stature (constitutional tall stature (n = 13), Marfan syndrome (n = 2) and tethered spinal cord (n = 1)) with a predicted final height above 197 cm were included in the study and prospectively followed until final height was reached. As one boy was lost to follow-up we have reported on 15 boys. Treatment with SMS as a single subcutaneous dose was started and continued until final height was reached. In eight boys androgens were given to induce puberty after the start of SMS and five boys were on treatment with androgens prior to SMS treatment. Effect on reduction of final height prediction, calculated with the index of potential height based on the bone age of Greulich and Pyle, was the main outcome measure. Standard anthropometric assessments were performed a year before and every 3 months during treatment. Bone age was assessed by the method of Greulich and Pyle at the start and after 6 and 12 months. Results: Mean reduction in final height prediction (predicted adult height minus achieved adult height) was −0.1 cm (range −6.4 to +5.7). In three boys, asymptomatic microlithiasis of the gall bladder was diagnosed. Conclusions: We have concluded that, in spite of encouraging short-term results, long-term treatment with SMS does not reduce final height in a manner sufficient to justify SMS treatment in tall stature.

scholarly journals Epiphysiodesis for the treatment of tall stature and leg length discrepancy

2021 ◽  
Author(s):  
Madeleine Willegger ◽  
Markus Schreiner ◽  
Alexander Kolb ◽  
Reinhard Windhager ◽  
Catharina Chiari
Keyword(s):  
Surgical Planning ◽  
Multidisciplinary Approach ◽  
Treatment Options ◽  
Bone Age ◽  
Leg Length Discrepancy ◽  
Tall Stature ◽  
Complication Rates ◽  
Leg Length ◽  
Growth Prediction ◽  
Length Discrepancy

SummaryPainful orthopedic conditions associated with extreme tall stature and leg length discrepancy (LLD) include back pain and adopting bad posture. After failure of conservative treatment options, blocking of the growth plates (epiphysiodesis) around the knee emerged as gold standard in patients with tall stature and LLD in the growing skeleton. Surgical planning includes growth prediction and evaluation of bone age. Since growth prediction is associated with a certain potential error, adequate planning and timing of epiphysiodesis are the key for success of the treatment. LLD corrections up to 5 cm can be achieved, and predicted extreme tall stature can be limited. Percutaneous epiphysiodesis techniques are minimally invasive, safe and efficient methods with low complication rates. In general, a multidisciplinary approach should be pursued when treating children and adolescents with tall stature.

Special Considerations in the Use of Glucocorticoids in Children

1995 ◽  
Vol 16 (7) ◽  
pp. 266-272
Author(s):  
Joseph D. Spahn ◽  
Alan K. Kamada
Keyword(s):  
Adverse Effects ◽  
Autoimmune Diseases ◽  
Early Recognition ◽  
Therapeutic Effects ◽  
Therapeutic Modalities ◽  
High Doses

GCs are used commonly for the treatment of various inflammatory and autoimmune diseases. Although potent and generally effective, they are not without risks for producing serious adverse effects, especially when used in high doses for prolonged periods of time. Thus, the clinician must balance the therapeutic effects of GCs with their risks for adverse effects; using the lowest possible effective GC doses as well as maximizing other therapeutic modalities are means by which this goal can be achieved. Early recognition and appropriate management are other methods to minimize GC-induced adverse effects. Maximization of therapy, early recognition, and appropriate management of adverse effects can minimize the potential severe complications of GC therapy.

Obesity

2020 ◽  
pp. 225-252
Author(s):  
Gary Butler ◽  
Jeremy Kirk
Keyword(s):  
Cushing Syndrome ◽  
Bone Age ◽  
Melanocortin Receptor ◽  
Hormone Deficiency ◽  
Endocrine Disorders ◽  
Tall Stature ◽  
Genetic Syndromes ◽  
Monogenic Disorders ◽  
Dysmorphic Features ◽  
Significant Impairment

• Obesity is defined as: ‘An excess of body fat frequently resulting in a significant impairment of health and longevity’. • In most cases obesity is not due to an underlying endocrine disorder, although it may produce endocrine morbidity such as type 2 diabetes. • Although there are a number of different methods to assess overweight and obesity, the most common is body mass index (BMI): weight (kg)/height (m)2. • Classification is: • primary: exogenous or ‘simple’ obesity • secondary: ■ identified genetic syndromes, e.g. Prader–Willi, Bardet–Biedl, pseudohypoparathyroidism ■ monogenic disorders, e.g. leptin deficiency, leptin/melanocortin receptor defects ■ CNS disease, e.g. hypothalamic obesity ■ endocrine disorders, e.g. hypothyroidism, Cushing syndrome, growth hormone deficiency, precocious puberty ■ immobility, e.g. cerebral palsy ■ iatrogenic. • Generally, children with obesity which is: • primary often have a family history, tall stature, advanced bone age, and no dysmorphic features • secondary often have short stature, delayed bone age, dysmorphic features, and developmental delay. • Complications of obesity are multisystem: metabolic, cardiovascular, respiratory, gastrointestinal/hepatic, orthopaedic, neurological, dermatological, gynaecological, and psychological. • Therapy is aimed at modifiable factors restoring the balance between energy intake (e.g. dietary) and expenditure (e.g. exercise), and preferably a combination of both along with counselling and behaviour modification. There is currently only limited data on the benefits of pharmacotherapy and bariatric surgery.

scholarly journals The Use of Large Doses of Adrenaline and Other Vasopressors in the Low Cardiac Output Syndrome after Cardiac Surgery—A Report of Four Cases

1974 ◽  
Vol 2 (1) ◽  
pp. 58-68 ◽  
Author(s):  
V. I. Callanan ◽  
G. A. Harrison
Keyword(s):  
Cardiac Output ◽  
Cardiac Surgery ◽  
Myocardial Metabolism ◽  
Favourable Effect ◽  
Ventricular Failure ◽  
Low Cardiac Output Syndrome ◽  
Low Cardiac Output ◽  
Blood Volume Expansion ◽  
Adrenaline Infusion ◽  
High Doses

Four cases are described of the use of prolonged and/or high doses of adrenaline in the treatment of the low cardiac output syndrome following cardiac surgery. Adrenaline was chosen because it produced a more favourable effect on central cardiovascular haemodynamics and myocardial metabolism when compared with isoprenaline, while its detrimental effects on renal function were less marked than those found when using noradrenaline. The renal insufficiency encountered was reversed in those cases without previous renal disease. Tachyphylaxis was demonstrated and weaning was achieved by gradual withdrawal of the vasopressor with concomitant blood volume expansion. Two cases with predominant right ventricular failure developed gross ascites and peripheral oedema, making control of fluid balance difficult. These problems are discussed with a summary of the relevant pharmacology of isoprenaline, noradrenaline, adrenaline and glucagon. The conclusions reached regarding the use and management of adrenaline infusion are given.

Long-term growth and development in 268 bevacizumab (BEV)-treated and 135 control pediatric/adolescent patients (pts): An integrated analysis.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 10554-10554
Author(s):  
Hermann L. Müller ◽  
Johannes H.M. Merks ◽  
Birgit Geoerger ◽  
Jacques Grill ◽  
Darren Hargrave ◽  
...  
Keyword(s):  
Negative Impact ◽  
High Grade Glioma ◽  
Bone Age ◽  
Reference Population ◽  
Integrated Analysis ◽  
Healthy Children ◽  
Growth Data ◽  
Growth Development

10554 Background: BEV has an established safety profile in adults, but long-term data in children are limited. This analysis examined the effects of BEV on growth/development in pediatric/adolescent pts. Methods: Data (height, weight, body mass index [BMI], bone age data) were pooled (5 trials): NCT00643565 (Ph2/soft tissue sarcoma); NCT01390948 (Ph2/high-grade glioma); NCT00085111 (Ph1/refractory solid tumors); NCT00667342 (Ph2/osteosarcoma); NCT00381797 (Ph2/glioma, medulloblastoma, ependymoma). Pts (<18 yrs old) received ≥1 dose of BEV + chemotherapy (CT) (n=268) or CT alone (n=135). Analyses were exploratory/descriptive. Reference growth data: WHO (<2 yrs); Centres for Disease Control (≥2 yrs). Results: Across the trials, mean number of BEV administrations per pt ranged 5.6–19.9 (dose 5–15mg/kg every 2/3 weeks). Median follow-up time, months (range): BEV+CT, 37.9 (2.4–64.2); CT, 22.9 (2.8–69.2). At baseline, median height, weight, and BMI were close to that of the reference population (mean standard deviation scores [SDS] close to 0). Over 60 months, a slight decline was observed in the mean SDS for height and weight in both arms in this cohort with different tumors/treatments (Table), but remained within normal range of healthy children. Trends were similar for BMI. No delay in growth velocity or bone age in BEV-treated pts vs CT only was observed up to 3 yrs, regardless of age/gender. A subgroup analysis of pts in the growth hormone-dependent development phase was consistent with the overall results. Conclusions: In this analysis, BEV inclusion in the treatment regimen did not have a negative impact on pediatric growth/development beyond that of CT alone. [Table: see text]

scholarly journals Adult height prediction by bone age determination in children with isolated growth hormone deficiency

2020 ◽  
Vol 9 (5) ◽  
pp. 370-378
Author(s):  
Thomas Reinehr ◽  
Martin Carlsson ◽  
Dionisios Chrysis ◽  
Cecilia Camacho-Hübner
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Adult Height ◽  
Age Determination ◽  
Bone Age ◽  
Hormone Deficiency ◽  
Gh Treatment ◽  
Height Prediction ◽  
The Mean ◽  
Tw2 Method

Background The precision of adult height prediction by bone age determination in children with idiopathic growth hormone deficiency (IGHD) is unknown. Methods The near adult height (NAH) of patients with IGHD in the KIGS database was compared retrospectively to adult height prediction calculated by the Bayley–Pinneau (BP) prediction based on bone age by Greulich–Pyle (GP) in 315 children and based on the Tanner-Whitehouse 2 (TW2) method in 121 children. Multiple linear regression analyses adjusted for age at GH start, age at puberty, mean dose and years of of GH treatment, and maximum GH peak in stimulation test were calculated. Results The mean underestimation of adult height based on the BP method was at baseline 4.1 ± 0.7 cm in girls and 6.1 ± 0.6 cm in boys, at 1 year of GH treatment 2.5 ± 0.5 cm in girls and 0.9 ± 0.4 cm in boys, while at last bone age determination adult height was overestimated in mean by 0.4 ± 0.6 cm in girls and 3.8 ± 0.5 cm in boys. The mean underestimation of adult height based on the TW2 method was at baseline 5.3 ± 2.0 cm in girls and 7.9 ± 0.8 cm in boys, at 1 year of GH treatment adult height was overestimated in girls 0.1 ± 0.6 cm in girls and underestimated 4.1 ± 0.4 cm in boys, while at last bone age determination adult height was overestimated in mean by 3.1 ± 1.5 cm in girls and 3.6 ± 0.8 cm in boys. Conclusions Height prediction by BP and TW2 at onset of GH treatment underestimates adult height in prepubertal IGHD children, while in mean 6 years after onset of GH treatment these prediction methods overestimated adult height.

scholarly journals Effect of enzymatic activity of diesel oil contaminated soil on the chemical composition of oat (Avena sativa L.) and maize (Zea mays L.)

2011 ◽  
Vol 51 (No. 8) ◽  
pp. 360-367 ◽  
Author(s):  
M. Wyszkowski ◽  
J. Wyszkowska
Keyword(s):  
Alkaline Phosphatase ◽  
Enzymatic Activity ◽  
Contaminated Soil ◽  
Diesel Oil ◽  
Sodium Content ◽  
Favourable Effect ◽  
Soil Enzymatic Activity ◽  
Negative Effect ◽  
Lower Effect ◽  
High Doses

The aim of the study was to determine the effect of soil contamination with diesel oil (3, 6, 9, 12 and 24 g/kg soil) on the yield and the content of macroelements in oat and maize cultivated in soil supplemented with nitrogen and sawdust. The correlation between the content of macroelements in the crops and the soil enzymatic activity was also determined. High doses of diesel oil had a definite negative effect on the content of all macroelements in oat, excluding phosphorus. The presence of diesel oil had a favourable effect on the accumulation of most of macroelements in the above-ground parts of maize. Nitrogen application to the soil caused an increase in the content of nitrogen and in the accumulation of calcium and magnesium in the above-ground parts of both crops as well as an increase in sodium content in maize. Sawdust applied to the soil had a considerably lower effect (several to less than 20%) on the content of macroelements in plants. A correlation between the activity of urease and acid and alkaline phosphatase in the soil and the content of macroelements in plants cultivated in diesel oil contaminated soil was observed. This correlation was positive only in the case of alkaline phosphatase activity and phosphorus content in oats.

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