Management of Puberty for Optimal Auxological Results in β-Thalassaemia Major

AbstractShort stature is present in a significant percentage of patients affected by β-thalassaemia major. Growth failure of patients with thalassaemia is multifactorial. The most important contribution is attributed to the toxic effect desferrioxamine and to endocrine disorders, due to iron overload. The commonest endocrine complication is hypogonadism. The growth pat- tern of patients with thalassaemia is characterized by normal growth during childhood, a deceleration of growth velocity around age 9-10 years, and a reduced pubertal growth spurt. In addition, reduced growth of the trunk is often present. Short stature and short trunk are more evident at pubertal age. Hypogonadism is usually considered responsible for the pubertal growth failure, as well as the aggravation of body disproportion at pubertal age. However, data suggest that pubertal height gain and final height are reduced in both patients with spontaneous puberty and patients with induced puberty. It is concluded that several aspects of peripubertal growth in patients with thalassaemia remain to be clarified.

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Age of onset of a normally timed pubertal growth spurt affects the final height of children

Pediatric Research ◽

10.1038/pr.2015.104 ◽

2015 ◽

Vol 78 (3) ◽

pp. 351-355 ◽

Cited By ~ 16

Author(s):

Yehuda Limony ◽

Sławomir Kozieł ◽

Michael Friger

Keyword(s):

Age Of Onset ◽

Final Height ◽

Growth Spurt ◽

Pubertal Growth Spurt ◽

Pubertal Growth

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Patterns of growth from birth to maturity in infants and children with congenital adrenal hyperplasia

Acta Endocrinologica ◽

10.1530/acta.0.112s295 ◽

1986 ◽

Vol 113 (4_Suppl) ◽

pp. S295-S304 ◽

Cited By ~ 16

Author(s):

George W. Clayton

Keyword(s):

Congenital Adrenal Hyperplasia ◽

Growth Failure ◽

Normal Growth ◽

Growth Patterns ◽

Height Velocity ◽

Growth Spurt ◽

First Year ◽

Adrenal Hyperplasia ◽

Pubertal Growth Spurt ◽

First Year Of Life

Abstract Patterns of growth of 30 children with Congenital Adrenal Hyperplasia of the 21-hydroxylase type were studied from infancy to maturity. These children were compliant as to therapy. Intramuscular (I.M.) Cortisone (40mg/M2) given every three days resulted in growth failure after the first year of life. A marked increase in height velocity occurred when oral Cortisone (20-25mg/M2) was given at approximate] 3 years. Growth was relatively normal during childhood in both boys and girls but there was a marked increase in mean weight in both sexes. Pubertal growth spurt occurred normally in males but was delayed in females. Weight decreased in both sexes at puberty but not to normal. Better methods of monitoring this condition should result in therapeutic regimes which allow normal growth patterns as well as normal mature height.

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Slow Prepubertal Linear Growth but Early Pubertal Growth Spurt in Patients With Shunted Hydrocephalus

PEDIATRICS ◽

10.1542/peds.95.6.917 ◽

1995 ◽

Vol 95 (6) ◽

pp. 917-923

Author(s):

Tuija Löppönen ◽

Anna-Liisa Saukkonen ◽

Willy Serlo ◽

Peter Lanning ◽

Mikael Knip

Keyword(s):

Linear Growth ◽

Final Height ◽

Relative Weight ◽

Bone Age ◽

Growth Spurt ◽

Healthy Children ◽

Growth Data ◽

Pubertal Growth Spurt ◽

Anthropometric Measures ◽

Pubertal Growth

Objective. To evaluate growth and to compare anthropometric measures and the degree of physical maturation in children with shunted hydrocephalus with those in healthy children. Methods. One hundred fourteen patients (62 male) and 73 healthy subjects (38 male) 5 to 20 years of age were analyzed for growth data and current auxology, stage of puberty, and bone age. Results. Boys with hydrocephalus were shorter than control boys during their first 8 years of age, and no catch-up growth was observed until puberty. Girls with hydrocephalus were of the same size at birth as the control girls, but their linear growth retarded during the first years of life, leading to reduced relative height between the age of 5 to 8 years. The pubertal growth spurt occurred earlier in boys with hydrocephalus (age at midgrowth spurt, 12.1 vs 13.3 years), and a similar trend was seen in girls (10.0 vs 10.7 years). The final height was again reduced, especially in boys. Patients with hydrocephalus were more obese than control subjects, girls more often than boys. Relative bone age was retarded in prepubertal (-0.42 vs 0.32 SD) and accelerated in pubertal patients (0.54 vs -0.19 SD). Conclusions. Children with hydrocephalus experience slow linear growth in prepuberty, but they have an earlier adolescent growth spurt. Together these factors result in a reduced final height. An increase in relative weight emerges in the preadolescent period, and this phenomenon is accentuated after puberty, leading to an increased prevalence of obesity.

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Bone Age at Onset of Pubertal Growth Spurt and Final Height in Normal Children

Clinical Pediatric Endocrinology ◽

10.1297/cpe.4.129 ◽

1995 ◽

Vol 4 (2) ◽

pp. 129-136 ◽

Cited By ~ 3

Author(s):

Mari Satoh ◽

Toshiaki Tanaka

Keyword(s):

Final Height ◽

Age At Onset ◽

Bone Age ◽

Growth Spurt ◽

Pubertal Growth Spurt ◽

Normal Children ◽

Pubertal Growth

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Estimation of Pubertal Growth Spurt Parameters in Children and Adolescents Living at Moderate Altitude in Colombia

Frontiers in Endocrinology ◽

10.3389/fendo.2021.718292 ◽

2021 ◽

Vol 12 ◽

Author(s):

Marco Antonio Cossio-Bolaños ◽

Ruben Vidal-Espinoza ◽

Juan Minango-Negrete ◽

Pedro R. Olivares ◽

Luis Urzua-Alul ◽

...

Keyword(s):

Children And Adolescents ◽

Final Height ◽

Peak Height ◽

Height Velocity ◽

Growth Spurt ◽

Moderate Altitude ◽

Biological Parameters ◽

Pubertal Growth Spurt ◽

Peak Height Velocity ◽

Pubertal Growth

ObjectiveKnowledge of the biological parameters of pubertal growth spurt allows verification of secular changes and exploration of the timing of puberty. The aim of the study was to estimate final height, age at peak height velocity (APHV), and peak height velocity PHV (cm/y) in children and adolescents living at moderate altitude in Colombia.MethodsA cross-sectional study was designed in 2.295 schoolchildren from Bogotá (Colombia) with an age range from 5.0 to 18.9 years. Height (cm) was assessed. Preece–Baines model 1 (1PB) was used to make inferences about mathematical and biological parameters.ResultsThe five mathematical parameters estimated in general have reflected quality in the fit to the model, reflecting a small residual error. Final height was reached in boys at 170.8 ± 0.4 cm and in girls at 157.9 ± 0.2 cm. APHV was estimated at 12.71 ± 0.1 years in boys and 10.4 ± 0.2 years in girls. Girls reached APHV 2.2 years earlier than boys. In relation to PHV (cm/y), boys reached higher growth speed in height (7.4 ± 0.4 cm/y), and in girls it was (7.0 ± 0.2 cm/y).ConclusionIt was determined that final height was reached at 170.8 ± 0.4 cm in boys and 157.9 ± 0.2 cm in girls, and APHV (years) and PHV (cm/ye) were reached relatively early and with average peak velocity similar to Asian and Western populations. A large-scale longitudinal study is needed to confirm these findings.

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Skeletal Features and Growth Patterns in 14 Patients with Haploinsufficiency of SHOX: Implications for the Development of Turner Syndrome

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/jcem.84.12.6289 ◽

1999 ◽

Vol 84 (12) ◽

pp. 4613-4621 ◽

Cited By ~ 75

Author(s):

Tomoki Kosho ◽

Koji Muroya ◽

Toshiro Nagai ◽

Masatoshi Fujimoto ◽

Susumu Yokoya ◽

...

Keyword(s):

Short Stature ◽

Growth Patterns ◽

Pseudoautosomal Region ◽

Growth Spurt ◽

Pubertal Growth Spurt ◽

Cubitus Valgus ◽

Partial Monosomy ◽

Early Maturation ◽

Pubertal Growth ◽

Skeletal Lesions

We report on clinical features in 14 Japanese patients (4 males and 10 females) with partial monosomy of the short arm pseudoautosomal region involving SHOX (n = 11) or total monosomy of the pseudoautosomal region with no involvement of disease genes on the sex-differential regions (n = 3). Skeletal assessment showed that three patients had no discernible skeletal abnormalities, one patient exhibited short 4th metacarpals and borderline cubitus valgus, and the remaining 10 patients had Madelung deformity and/or mesomelia characteristic of Léri-Weill dyschondrosteosis (LWD), together with short 4th metacarpals and/or cubitus valgus. Skeletal lesions were more severe in females and became obvious with age. Growth evaluation revealed that patients without LWD grew along by the −2 sd growth curve before puberty and showed a normal or exaggerated pubertal growth spurt, whereas those with LWD grew along by the standard growth curves before puberty but exhibited an attenuated pubertal growth spurt and resultant short stature. Maturational assessment indicated a tendency of relatively early maturation in patients with LWD. There was no correlation between the clinical phenotype and the deletion size. These findings suggest that haploinsufficiency of SHOX causes not only short stature but also Turner skeletal anomalies (such as short 4th metacarpals, cubitus valgus, and LWD) and that growth pattern is primarily dependent on the presence or absence of LWD. Because skeletal lesions have occurred in a female-dominant and age-influenced fashion, it is inferred that estrogens exert a maturational effect on skeletal tissues that are susceptible to premature fusion of growth plates because of haploinsufficiency of SHOX, facilitating the development of skeletal lesions.

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Serum DHEA-S levels could be used as a comparable diagnostic test to assess the pubertal growth spurt in dentofacial orthopedics

Progress in Orthodontics ◽

10.1186/s40510-020-00317-5 ◽

2020 ◽

Vol 21 (1) ◽

Author(s):

V. Anusuya ◽

Amit Nagar ◽

Pradeep Tandon ◽

G. K. Singh ◽

Gyan Prakash Singh ◽

...

Keyword(s):

Diagnostic Test ◽

Growth Spurt ◽

Pubertal Growth Spurt ◽

Pubertal Growth ◽

Dentofacial Orthopedics

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Prepubertal urinary estrogen excretion and its relationship with pubertal timing

AJP Endocrinology and Metabolism ◽

10.1152/ajpendo.00374.2010 ◽

2010 ◽

Vol 299 (6) ◽

pp. E990-E997 ◽

Cited By ~ 22

Author(s):

Lijie Shi ◽

Thomas Remer ◽

Anette E. Buyken ◽

Michaela F. Hartmann ◽

Philipp Hoffmann ◽

...

Keyword(s):

Pubertal Timing ◽

Height Velocity ◽

Breast Development ◽

Growth Spurt ◽

Age At Menarche ◽

Healthy Children ◽

Pubertal Growth Spurt ◽

Peak Height Velocity ◽

Pubertal Growth ◽

Estrogen Production

Whether prepubertal estrogen production impacts on the timing of puberty is not clear. We aimed to investigate prepubertal 24-h estrogen excretion levels and their association with early and late pubertal markers. Daily urinary excretion rates of estrogens of 132 healthy children, who provided 24-h urine samples 1 and 2 yr before the start of the pubertal growth spurt [age at takeoff (ATO)], were quantified by stable isotope dilution/GC-MS. E-sum3 (estrone + estradiol + estriol) was used as a marker for potentially bioactive estrogen metabolites and E-sum5 (E-sum3 + 16-epiestriol + 16-ketoestradiol) for total estrogen production. Pubertal outcomes were ATO, age at peak height velocity (APHV), duration of pubertal growth acceleration (APHV-ATO), age at Tanner stage 2 for pubic hair (PH2), genital (G2, boys) and breast (B2, girls) development, and age at menarche. Prepubertal urinary estrogen excretions (E-sum3 and E-sum5) were not associated with ATO, APHV, and age at PH2 but with duration of pubertal growth acceleration ( P < 0.01) in both sexes. Girls with higher E-sum3 reached B2 0.9 yr ( P = 0.04) and menarche 0.3 yr earlier ( P = 0.04) than girls with lower E-sum3. E-sum3 was not associated with age at G2 in boys ( P = 0.6). For most pubertal variables, the associations with E-sum3 were stronger than with E-sum5. In conclusion, prepubertal estrogens may not be critical for the onset of the pubertal growth spurt but are correlated with its duration in both boys and girls. Prepubertal estrogen levels may already predict the timing of girls' menstruation and breast development but do not appear to affect sexual maturation in boys.

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