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PubMed1999

Growth of long-term survivors of liver transplantation.

OBJECTIVE: To assess growth in survivors of liver transplantation. STUDY DESIGN: Growth was studied in 105 children up to seven years after liver transplantation. RESULTS: At transplantation, mean height standard deviation score (zH) was -1.22 but 19% of patients were severely growth retarded (height below 0.4th centile). Growth and pubertal retardation were seen in the first six months after liver transplantation. Significant catch up in growth and puberty continued for more than five years. At five years, mean zH was -0.95 and at seven years -0.84. The mean zH of patients at final height was -0.55. zH at six months was predicted by zH and bilirubin at the time of transplantation and prednisolone dose at six months. At four years, zH was predicted by zH at the time of transplantation and the cumulative prednisolone dose. There was no association between zH and age at transplantation, sex, or diagnosis, although those with biliary atresia and those undergoing transplantation under 2 years of age showed more initial growth delay and subsequent catch up. Average age at menarche was 14.2 years. CONCLUSIONS: The mean height of the group to have reached final height after liver transplantation was on the 27th centile. Those transplanted earlier in childhood are likely to achieve more normal final heights. High steroid dose, poor liver function, and retransplantation are associated with poorer height outcomes. Persisting severe short stature is largely confined to children with severely retarded growth at the time of transplantation. Transient delay in puberty and menarche occur early after transplantation, although appropriate pubertal progress is resumed after two to three years.

Adolescent↗
PubMed1995

Immunization of prepubertal beef heifers against gonadotropin-releasing hormone: immune, estrus, ovarian, and growth responses.

To develop an effective immunization protocol against human serum albumin-Cys-Gly-GnRH (HSA-GnRH) conjugate to delay the onset of puberty in heifers, 58 heifers (8 mo of age; mean +/- SE BW = 203 +/- 1 kg) were randomly assigned to each of six treatments: 1) controls, .1 mg of HSA, with diethylaminoethyl (DEAE)-dextran as adjuvant, on d 0 and 28; 2) .1 mg of HSA-GnRH, with DEAE-dextran, on d 0; 3) as 2) and booster on d 28; 4) as 3) but boosters also on d 84, 140, 196, and 252; 5) as 2) but half the conjugate given with DEAE-dextran adjuvant and half with non-ulcerative Freund's adjuvant (NUFA), injected in two separate sites; and 6) as 2) but the conjugate given with DEAE-dextran and NUFA, emulsified and injected in two sites. The duration of the experiment was 342 d. Mean plasma GnRH antibody titers (samples every 2 wk) for heifers in Treatments 2 to 6 were 9.4 +/- 1.16, 20.6 +/- 2.21, 43.9 +/- 2.86, 27.9 +/- 2.67, and 44.5 +/- 3.75% binding at a plasma dilution of 1:640. The mean number of times estrus was observed in heifers was less (P < .05; pooled SEM = .53) in Treatments 4 (.2) and 6 (2.4) than in Treatments 1, 2, 3, and 5 (7.8, 7.0, 7.0, and 6.6, respectively). The mean interval to the onset of puberty (the first increase in plasma progesterone > or = .5 ng/mL for > or = 10 d with samples at 3- to 4-d intervals) was greater (P < .05; pooled SEM = 11.6) for heifers in treatments 4 (339 d) and 6 (276 d) than for heifers in Treatments 1, 2, 3, and 5 (164, 159, 165, and 170 d, respectively). Mean ADG of heifers was reduced (P < .05) in treatments 2, 3, 4, and 6 (.71, .72, .68, and .69 kg, respectively) compared with controls (.77). In summary, the multiple booster immunization treatment induced and maintained sufficient anti-GnRH titer to delay puberty for 175 d; a single immunization against GnRH with DEAE and NUFA increased antibody titers enough to delay puberty for 112 d. However, GnRH immunization treatments reduced ADG of heifers in Treatments 2, 3, 4, and 6.

Aging↗
PubMed1996

Spontaneous growth in idiopathic short stature. European Study Group.

Documenting the spontaneous growth pattern of children with idiopathic short stature (ISS) should be helpful in evaluating the effects of growth promoting treatments. Growth curves for children with ISS were constructed, based on 229 untreated children (145 boys and 84 girls) from nine European countries. The children were subdivided according to target range and onset of puberty, and the growth of these subgroups was evaluated from standard deviation scores (SDS). At birth, children with ISS were already shorter than normal (means; boys -0.8 SDS, girls -1.3 SDS). Height slowly decreased from -1.7 SDS at the age of 2 years to -2.7 SDS at the age of 16 years in boys and 13 years in girls. Final height was -1.5 SDS in boys and -1.6 SDS in girls (mean (SD): boys 164.8 (6.1) cm, girls 152.7 (5.3) cm)), which was 5-6 cm below their target height. The onset of puberty was delayed (boys 13.8 (1.3) years, girls 12.9 (1.1) years). Subclassification resulted in similar growth curves. These specific growth data may be more suitable for evaluating the effects of growth promoting treatments than population based references.

Adolescent↗
PubMed2001

Persistence of delayed adrenarche in boys with thalassemia.

5 years after a previous study, we followed up a group of thalassemic patients, determining DHEA-S levels in peripubertal age, with the aim of evaluating whether adrenarche maturation occurred in boys and advanced in girls. Furthermore, we evaluated the degree of bone mineral density (BMD SDS(BA)) and analyzed growth parameters calculating standard deviation score with respect to bone age (BA) of height (Ht SDS(BA)), sitting height (SH SDSBA), and subischial leg length (SLL SDSBA), body mass index (BMI) and the difference between the values of the previous and the present study (deltaBMI), thyroid function and serum markers of bone metabolism. Our results showed persistent lack of adrenarche (DHEA-S 25+/-9.5 microg/dl) in all 6 boys and the absence of pubertal signs at chronological age (CA) of 12.4+/-1.4 yr and BA of 11.1+/-1.1 yr. Only one boy, 6 months later, showed a testicular volume of 4 ml (Tanner stage G2) with an increase of DHEA-S value (181 microg/dl) at BA 12.8 yr. Body disproportion and severe degree of osteopenia (BMD SDSBA -2.41+/-0.5) were observed in all boys, even though Ht SDSBA (0.14+/-0.8) and markers of bone metabolism were within the normal range. No change in nutritional status was observed (deltaBMI 0.09+/-0.4 kg/m2). In contrast, all the thalassemic girls had DHEA-S values (172.7+/-97.7 microg/dl) within the normal range at BA 12.7 +/-0.6 yr that was similar to CA. Furthermore, the appearance of Tanner stage B2 occurred in each of them at BA, near to CA, of 10.4+/-0.9 yr, and menarche was observed in three of them at mean BA, near to CA, of 11.4+/-0.9 yr. Ht SDSBA was below normal range (-1.11+/-0.8), but SLL SDSBA and SH SDS(BA) values were reduced homogeneously, so that proportional body growth was observed. A significant change in nutritional status was observed (deltaBMI 2.69+/-0.9 kg/m2). Bone density value (BMD SDS(BA) -0.25+/-0.4) was in the normal range. There were no statistically significant differences between boys and girls for ferritin serum levels, blood consumption and desferrioxamine dosage. In conclusion, lack of change in nutritional status, measurable in the form of deltaBMI, but not BMI alone, considered an important physiological regulator of adrenarche, regardless of individual adrenal androgen secretion, could have a key role in the lack of adrenarche persisting in thalassemic boys during peripubertal age. Further follow up is necessary, in particular when boys reach puberty, because delayed adrenarche represents the most intriguing aspect in these patients.

Adolescent↗
PubMed1991

Etiologies of late puberty.

An expected lack of pubertal development can be due to an already diagnosed disease. The diagnosis of an unexpected lack of puberty is a difficult task. We have analyzed the etiologies of late puberty in 106 adolescents: 68 boys aged over 15 years and 38 phenotypic girls aged over 13 years. According to their clinical and biological (gonadotropin) data, they were classified in 3 groups. In the first group, hypergonadotropism was observed only in 19 females; pure gonadal dysgenesis was found in 2 cases with 46,XY, in 2 with 46,Xdel(Xq) karyotypes and 9 cases were 46,XX constitutions; 2 sisters had also blepharophimosis; in 3 cases the ovarian failure was due to autoimmune disease, and 1 case, genetically male, had 17 alpha-hydroxylase deficiency. The second group had gonadotropin insufficiency and consisted of 68 adolescents, 57 males and 11 females, with low gonadotropin levels: 33 were anosmic; in the boys, cryptorchism was present in 68% and micropenis in 31%; 38 had a familial history of hypogonadism, the transmission of which was matrilineal in 16, patrilineal in 13 and recessive autosomal in 7. The third group had low or low-normal gonadotropin levels: 22 cases of constitutional delay of puberty (11 cases in both sexes), demonstrated by further normal puberty during the follow-up. No clinical marker and familial history in 50% were noted.

Adolescent↗
PubMed1988

Precocious and delayed sexual development in children.

A review is presented of tests used to diagnose either isosexual precocity or delayed pubertal development in children. The importance of auxological measurements is emphasised. Attention is drawn to the limitations of measuring basal or stimulated levels of LH, FSH and the sex steroids for the diagnosis of these conditions. The value of gonadotropin profiles is discussed for either diagnosis or for assessing the response to GnRH therapy in patients with either isosexual precocity or isolated gonadotropin deficiency. Examples are given of new therapeutic agents and procedures that are used to treat these two groups of patients. These include GnRH agonists for treatment of children with isosexual precocity either alone, or in combination with inhibitors of aromatase or C17-20 lyase enzyme activity in the biosynthesis of the sex steroids and pulsatile GnRH for the treatment of adolescents with gonadotropin deficiency.

Adult↗
PubMed1992

Effects of wheat bran and energy restriction on onset of puberty, cell proliferation and development of mammary tissue in female rats.

Delayed onset of puberty and mammary development is assumed to reduce the risk of mammary cancer. An animal experiment was performed to investigate the influence of dietary fiber, which is known to affect hormonal balance, on these characteristics. Forty-five immature female rats were randomized into three groups, which were fed ad libitum a low-fiber diet (less than 0.5% dietary fiber based on white wheat flour), a high-fiber diet (9.2% dietary fiber based on wheat bran), or an energy-restricted low-fiber diet providing 90% of the energy of the ad libitum low-fiber diet. Energy intake in the second and third groups was similar. Wheat bran slightly delayed onset of puberty, whereas restricted energy intake delayed onset of puberty by about six days. At 48-58 days of age, 14 rats of the low-fiber group, 10 of the high-fiber group and 7 of the restricted group were in cycle. Development of mammary tissue was rudimentary in rats of the energy-restricted low-fiber group, stronger in the high-fiber group and strongest in the ad libitum low-fiber group. Cell proliferation in mammary tissue was similar for both groups fed ad libitum, but significantly lower in the restricted group. Peroxidase activity, a biomarker for estrogenicity, was lower in the high-fiber group than in the two low-fiber groups. It is concluded that wheat bran and, even more effectively, an imposed restricted energy intake delays onset of puberty and mammary development. This shortens the time for mammary cells to be initiated to tumor cells and hence reduces the risk of mammary cancer development.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1990

[Constitutional slow growth with delayed adolescence, presentation of 3 clinical cases].

Constitutional delay of growth and puberty is a constitutional delay of growth occurring in otherwise healthy adolescents with stature reduced for chronological age but generally appropriate for bone age and stage of pubertal development, both of which are usually delayed; psychological distress is the predominant consequence. The analysis of three cases shows that hand and wrist X-ray to study bone age is very important in patients with short stature, because we can make a better orthodontic treatment due to this bone age delay. The maturation stage investigation can be made in both hands because there are not significant differences between right and left one.

Adolescent↗
PubMed1986

Pubertal development in male hypopituitarism.

The spontaneous or therapeutically induced pubertal development of 65 male patients with idiopathic hypopituitarism was analysed. Spontaneous puberty occurred in 82% of the patients with prepubertal isolated growth hormone deficiency and in 32.5% of those with impairment in the secretion of more than one pituitary hormone. Out of this group, 36 patients could be studied longitudinally. In 15 patients, the onset of spontaneous puberty was delayed, on average, 3.2 years. It started at a bone age of 10.36 +/- 1.25 "years" and followed a pattern similar to that of normal boys. Testosterone levels at each pubertal stage were not different from those of normal boys. Mean peak height velocity reached 7.27 +/- 1.82 cm/year. In 21 patients with gonadotropin deficiency, hCG treatment was started at a chronological age of 19.04 +/- 2.17 years and a bone age of 12.94 +/- 0.80 "years". Plasma testosterone attained normal adult levels in the majority of boys, while the development of sexual characteristics showed a wide variation. Mean growth velocity during the first year of hCG therapy reached 6.11 +/- 2.47 cm/year. Partial gonadotropin deficiency was diagnosed in two boys. Although testosterone seems today to be, for practical reasons, the replacement therapy of choice, hCG treatment is an alternative for hypopituitary patients with absent gonadotropin function.

Adolescent↗
PubMed1987

Changes of skinfold thickness during puberty induced by pulsatile gonadotrophin-releasing hormone therapy.

We have analysed the changes in triceps and subscapular skinfold thickness ratios of 66 (46 male, 20 female) children with constitutional delay of growth and puberty who progressed through puberty spontaneously and 16 (7 male, 9 female) children with delayed puberty who were treated with pulsatile gonadotrophin-releasing hormone (GnRH). The pattern of change in skinfold thickness in both groups were identical and indistinguishable from standards of normal children when later chronological ages of the patients were allowed for. We observed a decrease in skinfold ratio just prior to the onset of puberty in boys, and such an assessment may be a useful early predictor of the onset of puberty. Our data provided further evidence that all aspects of human puberty are entirely GnRH-dependent.

Adolescent↗
PubMed1981

Sexual development and fertility of Jamaican female patients with homozygous sickle cell disease.

Results of a questionnaire study of 91 Jamaican patients with homozygous sickle cell (SS) disease and 59 Jamaican control subjects of similar socioeconomic status indicated a mean delay of 2.3 years in age at menarche and of 3.9 years in age at first pregnancy in Ss disease. The mean interval between first sexual exposure and pregnancy was similar in the two groups. The delay in age at first pregnancy in SS disease resulted partly from the delay in puberty, but it also resulted from a further delay in first sexual exposure, with a mean interval between menarche and first sexual exposure of 2.6 and 4.4 years in control subjects and patients, respectively. Physical and social factors that related to this delay were reflected in the higher prevalence of casual unions in patients; this finding implied lesser sexual exposure. Although the similar interval between first sexual exposure and pregnancy did not suggest a lesser fertility in patients with SS disease, the number of infants born to patients with SS disease was less at all ages compared with Jamaican standards. This finding might reflect lesser fertility subsequent to the first pregnancy.

Adolescent↗
PubMed1991

Plasma melatonin rhythm in disorders of puberty: interactions of age and pubertal stages.

A concurrent effect of age and puberty has hampered assessment of the influence of melatonin on human puberty. This study examined the pineal-puberty interaction in 41 subjects (ages 5-17 years) whose puberty was asynchronous from age (constitutional delay, hypopituitarism and idiopathic precocious puberty). Melatonin nocturnal profile was determined using blood samples drawn hourly by constant withdrawal. There was no significant trend for melatonin peak or time peak in pubertal or age groups. The linear correlation between age and melatonin peak was not significant. Thus, neither age nor puberty alone can account for the decline in nocturnal melatonin concentration observed across human development.

Adolescent↗
PubMed1992

Effects of age, photoperiod and follicle-stimulating hormone on lactate production by cultured Sertoli cells from prepubertal Siberian hamsters (Phodopus sungorus).

To define a functional difference in Sertoli cells of animals exposed to different photoperiodic conditions, we isolated Sertoli cells from the testes of juvenile Siberian hamsters and cultured them in serum-free medium. In all age groups studied, Sertoli cells isolated from hamsters with delayed and normal puberty responded to follicle-stimulating hormone (FSH) with an increase in lactate production. The increase in lactate production induced by 1000 ng FSH ml-1 was significantly greater in Sertoli cells isolated from hamsters with delayed puberty than in those with normal puberty. These results suggest that Sertoli cells of Siberian hamsters exposed to short photoperiod in vivo may respond to increases in plasma FSH concentrations associated with photostimulation or spontaneous sexual maturation by an increase in secretory activity that may be critical for the initiation of spermatogenesis.

Aging↗
PubMedDate not supplied

[Auxological data and definitive height in short patients without hormonal deficiencies. A longitudinal study].

61 children (49 males and 12 females) with stature before puberty less than 3. centile were studied. They had been previously diagnosed with the various GH stimulation tests as "constitutional short" and they were measured after reaching final adult height. During longitudinal follow-up, the following auxological data were calculated: height for chronological and bone age sds (HCAsds, HBAsds), height age/bone age ratio (HA/BA), bone age sds (BA sds); all the latter were calculated both in prepuberty and in puberty (mean 1.24 years after the onset of puberty). Target height and predictable height according to Bayley and Pinneau were also calculated. Final height for males was 152-175 cm (mean 162.3) and for females 144-163 cm (mean 151.5). These values were lower than expected with both target height and Bayley and Pinneau prediction, especially if the values of Bayley and Pinneau had been calculated before puberty. In females there was a significant positive correlation with target and final height. In males final height was positively correlated with Target height, with prepuberal data of HCA sds and with pubertal data of HBA sds and HA/BA. A negative correlation was found with final height in males and BA sds during puberty. A delay in bone age, probability, represented a positive factor in increasing final height only if it was maintained during puberty. Bayley and Pinneau's method overestimated final height, in particular when it was used before puberty or with a severe bone delay. Subject with pubertal delay are 2.42 cm taller than normal pubertal children. This difference had been acquired before puberty.

Age Determination by Skeleton↗
PubMed1987

Short stature and pubertal delay in cystic fibrosis.

Survival of the patient with cystic fibrosis has been considerably extended in the last two decades due to improved therapy of pulmonary infection, nutritional management, and an organized system of centralized care. Psychological and social aspects of cystic fibrosis may be intensified during adolescence: developing independence in the face of required daily care; developing self-esteem in the face of illness-associated problems such as discolored teeth, malabsorption, short stature, and cough; and participating in group activities despite physical limitations and the disruption in peer relationships when recurrent acute illness is interposed. Pubertal delay is more common in the cystic fibrosis population than the general population, and attendant psychologic dysfunction may be particularly common in males. Comparative studies suggest that this pubertal delay is a function of malnutrition rather than intrinsic to the disease. Normal growth potential may be postulated from somatomedin studies and has been demonstrated in patients treated with long-term aggressive nutritional management. Intervention utilizing testosterone enanthate in males with cystic fibrosis and pubertal delay has resulted in improved rate of growth, progression through puberty, and self-image.

Adolescent↗