Adult height predictions for Constitutional Growth Delay, growth hormone treatment for idiopathic short stature and the FDA: are they related?
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Adolescent↗
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Results for “Puberty, Delayed”
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Polyostotic fibrous dysplasia with auditory impairment and delayed menarche in a Nigerian.
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Adult↗
Hormonal changes in thalassaemia major.
Patients with severe thalassaemia major suffer endocrine and other abnormalities before their eventual death from iron overload due to repeated blood transfusions. The endocrine status of 31 thalassaemic patients aged 2-5 to 23 years was investigated. Exact data were available on the rate and duration of blood transfusion in all of them and in many the liver iron concentration was also known. Although the patients were euthyroid, the mean serum thyroxine level was significantly lower, and the mean thyrotrophic hormone level significantly higher, compared with the values found in normal children. Forty oral glucose tolerance tests with simultaneous insulin levels were performed in 19 children, of whom 5 developed symptomatic diabetes and one had impaired tolerance. Previous tests on all 6 patients were available and some showed raised insulin levels possibly due to insulin resistance. 2 patients had clinical hypoparathyroidism and are described. The parathyroid hormone levels determined by radioimmunoassay in 25 patients were below the mean for the age group in all and outside the reference range in 16. Nonfasting plasma calcium levels were not reduced. Puberty was delayed in some patients. Concentrations of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) measured in urine from 7 girls and 5 boys showed considerable variation. In the boys there was an overall tendency for FSH and LH excretion to be low with regard to age, but with respect to puberty rating FSH exretions were normal or low and LH normal or raised. The girls showed a tendency for LH but not FSH excretion to be raised in relation to puberty rating. The severity of the endocrine changes was related to the degree of iron loading and is discussed in relation to previous work in which the iron loading has rarely been accurately indicated nor parathyroid status assessed.
Adolescent↗
An approach to verifying delayed menarche in Japanese female athletes. Analysis by wavelet interpolation method.
AIM: The theory of delayed menarche in female athletes, despite some evidence for such a delay, has not yet been verified. We examined a means to verify this hypothesis by comparing ages at menarche and at peak height velocity (PHV) derived from the wavelet interpolation method (WIM) for female athletes and non-athletes (control group). METHODS: We identified age at maximum peak velocity as the index of the physical maturation rate by WIM. We then conducted a study involving 144 female athletes in their 1st year at University in the Tokai area, all of whom had competed in a national high school sports competition (athlete group). Past school records of these subjects' heights from the 1st grade of elementary to the 3rd year of senior high school (1984-1995) were collected, and ages at menarche were ascertained from questionnaires. A control group of 78 non-athletes was similarly examined. RESULTS: This difference (interval) between age at menarche and age at PHV was 1.62 years (SD=1.25) in the athlete group and 1.08 years (SD=0.74) in the control group. The difference between the 2 groups was statistically significant (P<0.01). This finding provides evidence that menarche in female athletes is delayed in relation to physical maturation rate. CONCLUSIONS: This result alone cannot establish whether athletic training is the only cause of this delay; however, an approach to verifying the hypothesis of delayed menarche in female athletes has been established by this finding.
Adolescent↗
Delayed sexual maturation in sickle cell anaemia patients--observations in one practice.
Pubic hair and breast appearance ratings, testicular volumes and age at menarche were determined in 24 sickle cell anaemia patients aged from 8.5 to 27 years between February and September 1994 in the Wesley Guild Hospital, Ilesa, Nigeria. Only three of the six females aged 15 or more years had experienced menarche and this was at the mean (SD) age of 18.8 (4.5) years. Public hair appearance and breast development had commenced in only six and seven, respectively, of the 12 females, and in every case the ratings were significantly low for their ages. Male testicular volume was also low for their age in the six of 12 males who were 13 years of age, and above, and pubic hair had appeared in only two of them, aged 20 and 27 years. The height of all the patients was delayed. It is important to recognize that significant delay in sexual development may accompany sickle cell anaemia and to take appropriate steps in managing and counselling the patients.
Adolescent↗
Development of secondary sex characteristics in multitransfused thalassemic children.
Secondary sex characteristics were evaluated in thalassemic children (41, boys and 30 girls), who were on regular transfusion therapy and were above 11 years of age. The results of this study indicate that the development of secondary sex characteristics in thalassemic children is markedly delayed as compared to their non-thalassemic siblings and to the expected development criteria. Delay in development of secondary sex characteristics appears to be secondary to chronic hypoxia and iron overload.
Adolescent↗
Delayed female sexual maturation. How to approach differential diagnosis.
Delayed sexual development is present when an adolescent girl fails to experience, at an age that is beyond the average for her peers, those pubertal events that are indexes of gonadal function. Adolescent girls whose sexual development is delayed can be divided into three groups: (1) those who have not menstruated but have well-developed secondary sexual characteristics, indicating that they have a functioning hypothalamic-pituitary-ovarian mechanism, (2) those with poorly developed secondary sexual characteristics whose hypothalamus and pituitary are functional but who have ovarian failure, as indicated by high levels of follicle-stimulating hormone (FSH) and luteinizing hormone (LH) (hypergonadotropic hypogonadism), and (3) those with poorly developed secondary sexual characteristics whose normal ovaries are not functioning because of a hypothalamic or pituitary disorder, as indicated by low or low normal serum FSH/LH levels (hypogonadotropic hypogonadism). There are, in addition, individuals with a female habitus and external female genitalia who were reared as females but have an XY karyotype and come to the physician with a complaint of delayed female sexual maturation.
Adolescent↗
Secondary sexual development of 'Cape Coloured' girls following kwashiorkor.
This report describes the secondary sexual development of 45 'Cape Coloured' female ex-kwashiorkor patients and 43 female controls. All patients were originally seen between five months and four years four months of age, treated and then followed up for 15 years after discharge. Age at menarche was available on 42 ex-patients and 33 controls, and age at peak height velocity (PHV) was available for 30 ex-patients and 15 controls. Maximum likelihood estimates of the mean age at entry to each pubertal stage were made, age at menarche was obtained directly from the subject records and age at PHV was obtained by fitting a non-linear growth function to the data for each subject. All subjects passed through the sequence of pubertal events in the normal order, i.e., no reversals were observed. Ex-patients were generally delayed in relation to controls but there were no significant differences for ages at entry to any of the pubertal stages. The subjects were combined for comparison to equivalent data on British girls. The South African girls were significantly delayed in the development of pubic hair and menarche but showed no significant differences for age at entry or duration of breast development and PHV. It is suggested that lack of delay in breast development may have selective advantages to females living in situations of chronic malnutrition.
Female↗
Anterior pituitary gland assessment in sickle cell anaemia patients with delayed menarche.
Pituitary gland dysfunction and its contribution to menarcheal delay in sickle cell anaemia patients was investigated. Ten SS patients mean age 17.5 years who had not achieved menarche were recruited and 10 each of AS and AA controls, mean ages 17.4 and 17.7 years were used as controls to study the effect of the heterozygous state. Dynamic studies with LHRH and TRH were performed for 60 minutes and LH, FSH, PRL and TSH assays were done. Median basal values were significantly lower in the SS patients compared with the AS and AA controls for LH, FSH and PRL. LH: 3.0; 7.1; 7.7 U/L, FSH: 2.1: 4.3: 5.1 U/L. PRL: 94.5; 590; 390 U/L, respectively. The median basal TSH values did not show any significant difference between the SS subjects (7.3 U/L) and the AS and AA controls (5.4 U/L) and 5.6 U/L, respectively. The readily releasable pool also showed the same pattern for LH, FSH and PRL as the basal values while the SS subjects had higher median TSH releasable pool values that were significantly different from those of the AA controls. From the prolactin responses three subjects demonstrated maturational delay in menarcheal achievement while seven demonstrated isolated gonadotrophin deficiency. It is concluded that SS patients with delayed menarche have a hypothalamopituitary axis dysfunction that gives rise to delay in menarcheal achievement and metabolic adaptations to stress of illness. The heterozygous state did not delay menarcheal onset.
Adolescent↗
Asthma, growth and inhaled corticosteroids.
Linear growth can be divided into three phases: during infancy it is influenced mainly by nutrition; thereafter throughout childhood it comes under hormonal control; puberty constitutes the third phase. The pubertal growth spurt, which may account for up to 30 cm of linear growth, results from an increase in growth hormone pulse amplitude that is mediated by sex steroids. The rate of growth in childhood, onset of puberty and final height are determined by the impact of a range of environmental factors on genetic potential. These variables and the effect of asthma itself need to be taken into account when assessing the possible effect of inhaled corticosteroids on growth. Many asthmatic children have a slower growth rate than normal children, and a physiological delay in puberty which does not affect final adult height. Prolonged administration of oral corticosteroids retards growth. The mechanism is not fully understood, but may involve an indirect effect on growth hormone secretion following adrenal suppression. Short-term deceleration of growth has been demonstrated by knemometry in children taking inhaled corticosteroids, but there is no evidence of long-term growth suppression with conventional doses of inhaled corticosteroids. Growth should be monitored in children taking over 0.8 mg day-1 of inhaled corticosteroid, but the priority should be to treat the asthma adequately.
Administration, Inhalation↗
CHARGE syndrome from birth to adulthood: an individual reported on from 0 to 33 years.
CHARGE syndrome was independently reported by Hall [(1979): J Pediatr 95:395-398] and Hittner et al. [(1979): J Pediatr Ophthalmol Strabismus 16:122-128] and was initially considered to be a non-random association between distinct multiple congenital anomalies. It is now considered to be a recognizable syndrome with well-characterized diagnostic criteria and a genetic pathogenesis. We report on a 33-year-old adult male with CHARGE syndrome, with emphasis on the unique medical, behavioral, and psychological issues faced in adulthood. Characteristic facial and ear abnormalities were obvious in early childhood, and bilateral retinal colobomata, left choanal atresia, right congenital hip dislocation, and hypogonadism were diagnosed during the first year. Walking was delayed due to vestibular problems, speech was impaired due to moderately severe hearing loss, and use of sign communication was limited. Choanal atresia was surgically corrected in infancy, and atrial septal defect, ventricular septal defect, and patent ductus arteriosus were surgically corrected in childhood. Undescended testes were removed in adolescence, and gallstones were removed in early adulthood. Puberty was delayed until hormone replacement therapy began at 15 years. Behavioral disturbances and anxiety persisted throughout childhood, adolescence, and into adulthood, often resulting from communication challenges. At 33 years of age, he lives independently in a supervised group home, receives regular eye check-ups and is being monitored for severely reduced bone density.
Abnormalities, Multiple↗
HIV-associated immune dysfunction and delayed pubertal development in a cohort of young hemophiliacs. Hemophilia Growth and Development Study.
As part of the Hemophilia Growth and Development Study (HGDS), we investigated the relationship between HIV-associated immune dysfunction and delayed pubertal development in a cohort of 333 boys and adolescents with moderate or severe hemophilia who were between the ages of 6 and 19 years at study entry in 1989. Sixty-two percent of the cohort was infected with HIV in the late 1970s and early 1980s through exposure to contaminated clotting factor concentrates. The cohort was observed during follow-up at 6-month intervals; measurements taken at each follow-up visit included Tanner stage and CD4+ cell count. This analysis of data from the first 4 years of follow-up revealed statistically significant delays in pubertal development associated with increasing levels of immune dysfunction. Our results emphasize the importance of following pubertal development in HIV-infected adolescent boys since delays in maturation may reflect underlying disease progression.
Adolescent↗
Unresolved problems in optimal therapy of pubertal disorders in oncological and bone marrow transplanted patients.
Specialised clinics for the long-term follow-up of survivors from childhood cancer have developed over recent years. The problems encountered among patients who received multiple chemotherapy and radiotherapy can be challenging and require high expertise and close collaboration among different professionals (e.g. oncologists, endocrinologists, radiotherapists, psychologists). Endocrine disorders are often seen, particularly among those who received cranial radiotherapy or gonadotoxic chemotherapy; puberty can be affected and the spectrum of disorders may range from precocious or accelerated puberty to delayed, arrested or even absent pubertal development. Growth impairment can be multifactorial and growth hormone deficiency is an important but probably not the only factor involved. Many questions remain about the optimal management of this group of young patients. In the consensus guidelines that follow the overview an attempt is made to help optimise patients' growth and puberty by suggesting practical clinical approaches to some of the most challenging issues.
Adolescent↗
Delayed sexual maturation.
Absence of any sign of gonadal development (i.e. increase in size of the testes or presence of breast budding) before the age of 13 years in girls and 14 years in boys represents the condition of delayed sexual maturation. After this age, the patient should be thoroughly clinically examined and a basic biological work-up should be performed. Pathological conditions such as hypergonadotropic hypogonadism with a bone age usually greater than 13 years can be easily diagnosed. Hypogonadotropic hypogonadism is more difficult to differentiate from delayed adolescence of good prognosis. Means of diagnosis as well as therapy are discussed. In addition, micropenis and primary amenorrhea are frequent clinical observations.
Adolescent↗
The neuropeptide Y Y1 receptor mediates NPY-induced inhibition of the gonadotrope axis under poor metabolic conditions.
Hypothalamic neuropeptide Y (NPY) plays a central role in the control of food intake, energy balance, and modulation of neuroendocrine functions. In particular, an increase in NPY expression participates in the inhibition of the reproductive activity under poor nutritional conditions. The present study was designed to evaluate further the involvement of the Y1 subtype of NPY receptors in these effects. Food intake, body weight gain, and the onset of puberty were studied in groups of wild-type and Y1 deficient mice that were either fed ad libitum or subjected to a 30% restriction in food intake. This moderate feeding restriction induced a similar deficit in body weight gain in wild-type and in Y1 knockout mice. However, although wild-type mice experienced the expected delay of puberty, all mice in the food restriction group and lacking Y1 could go through puberty over the time of the experiment despite decreases in circulating leptin levels and increases in hypothalamic NPY expression. This observation demonstrates that the absence of Y1 impairs the perception of decreasing energy stores by the gonadotrope axis, demonstrating a physiological role for Y1 in the sensing of endogenous metabolic parameters by the hypothalamus.
Adaptation, Physiological↗
Transient impairment or delay of urinary trihydroxypregnanone (THS) response to metyrapone in boys with delayed adolescence and in patients with isolated growth hormone deficiency.
Twenty three boys with delayed adolescence (age 15.7 +/- 2.0, bone age 12.4 +/- 2.1 years) were studied. Their cortisol response to insulin was normal. After oral metyrapone (500 mg/m2 by mouth) one to three consecutive 12 h urine samples were collected for analysis of THS. Thirty seven tests with 37 first, 21 second, and 11 third samples were carried out. The results could be divided into two main groups: 25 tests (group A) were subnormal in the first sample, 12 of them with a very weak (40 +/- 8 micrograms/m2/12 h) and 13 with an insufficient (191 +/- 16 micrograms/m2/12 h) THS response. Values in the second and third sample were higher, indicating a delayed response. In 12 tests (group B), the results were normal (1016 +/- 143 micrograms/M2/12 h) in the first and lower in the second and third samples. In three patients with repeated tests, there was improvement with increasing bone age. The THS-responses to metyrapone did not correlate with those of growth hormone, gonadotrophins, and TSH to stimuli. It is concluded that the THS-response to a single dose of metyrapone may be temporarily insufficient or delayed in delayed adolescence. We interpret this finding as showing transiently reduced or slow hypothalamic responsiveness.
17-Hydroxycorticosteroids↗
Shprintzen-Goldberg marfanoid syndrome: a case followed up for 24 years.
A patient with Shprintzen-Goldberg syndrome was followed up for 24 years. The patient's parents were unrelated and unaffected. Walking and speech were delayed. Facial dysmorphism was obvious as well as pectus excavatum, scoliosis, bilateral dislocation of the radial heads, hammer toes and hallux valgus, pes planus, arachnodactyly, and camptodactyly. Skin was thin and fragile. CT scan showed ventricular enlargement. He had a mitral valve prolapse. He was operated on twice for bilateral inguinal and crural hernia and several times for his foot deformities. Puberty was delayed until 18 years. At 24 years of age he was still thin, dolichocephalic, and the facial dysmorphic features were still obvious. Pectus excavatum was still severe but the thoracolumbar scoliosis was mild. His foot deformities had improved and his joints were less hypermobile. Psychomotor development was quite normal. His main concern was the minimal subcutaneous fat and fragility of skin. Echocardiography was now normal.
Abnormalities, Multiple↗
Severe mental deficiency, proportionate dwarfism, and delayed sexual maturation. A distinct inherited syndrome.
Two 46,XY brothers were found to have a previously undescribed syndrome characterized by severe mental deficiency, proportionate dwarfism, and delayed sexual development. A recessive mode of inheritance, either autosomal or X-linked, is assumed.
Adolescent↗