Pubertal status of children with congenital heart disease.

BACKGROUND: CHD influences many aspects of life in affected individuals. Puberty, a major aspect of development, is a concern for patients and families. OBJECTIVES: We investigated pubertal status in children and adolescents with CHD. METHODS: Patients with CHD aged 6-18 were enrolled. Cardiac diagnoses were confirmed using history, examination, and paraclinical tools including echocardiography. An endocrinologist determined pubertal stages, and the second Tanner stages for pubarche (P2), thelarche (B2), and gonadarche (G2) were considered as the pubertal onset. A study with a large sample size on pubertal onset in a normal population was used for comparison. RESULTS: Totally, 451 patients (228 girls and 223 boys) at a median (10th-90th percentile) age of 10.79 (8.02-14.28) years for the girls and 10.72 (8.05-14.03) years for the boys were enrolled. The median (10th-90th percentile) ages at B2 and P2 in the girls with CHD were 10.77 (9.55-12.68) and 10.53 (9.39-12.28) years, respectively, which were higher than the median ages of 9.74 (8.23-11.94) and 10.49 (8.86-12.17) years in the normal girls.The median (10th-90th percentile) ages at G2 and P2 in the boys with CHD were 11.04 (8.85-13.23) and 11.88 (9.78-13.46) years, correspondingly, which were higher than the median ages of 9.01 (6.00-11.84) and 10.34 (6.84-13.10) years in the normal boys. CONCLUSIONS: Pubertal onset could be delayed in children with CHD when compared with the normal population.

FSH may be a useful tool to allow early diagnosis of Turner syndrome.

BACKGROUND: Ultrasensitive assays to measure pre-pubertal gonadotropins levels could help identify patients with Turner syndrome (TS) in mid-childhood, but studies in this field are scarce. The aim of this study was to analyze gonadotropins levels in girls with TS throughout childhood. METHODS: Retrospective longitudinal study conducted with 15 girls with TS diagnosed with < 5 years whose FSH and LH measures were available since then. Hormones were evaluated in newborn/mini-puberty (< 0.5 years), early childhood (0.5-5 years), mid-childhood (5-10 years) and late childhood/adolescence (> 10 years). In newborn/mini-puberty and late childhood/adolescence pre-pubertal or pubertal gonadotropins were considered normal; in early childhood and mid-childhood concentrations above the pre-pubertal range were considered abnormal. RESULTS: Abnormally high FSH alone was found in four of five patients in newborn/mini-puberty, 13 of 15 during early childhood and nine of 15 during mid-childhood. In the group of 12 patients in late childhood/adolescence, the three girls with spontaneous puberty had only normal levels; the remaining showed only post-menopausal concentrations. In mid-childhood one patient exhibited only pre-pubertal FSH. Conversely, most LH measurements in early and mid-childhood were normal. CONCLUSION: Karyotyping of girls with short stature and high FSH levels would allow early diagnosis of Turner syndrome in a significant number of patients, particularly when resources for chromosome study of all girls with growth deficiency are limited.

Pubertal timing in girls and depression: a systematic review.

BACKGROUND: Because the incidence of depression increases after puberty, it is possible that pubertal timing in girls influences the onset of depression. Our objective was to assess the effect of early and late puberty in girls on the incidence of depression. METHODS: We systematically searched relevant databases for controlled studies that assessed the impact of pubertal timing in girls on the incidence of depression or depressive symptoms. The last search was completed in August 2013. Two authors selected the studies, extracted the data, and assessed the quality of the evidence. Meta-analyses of the adjusted and unadjusted results were calculated using random effects. RESULTS: Four cohort studies were included (n=8055 participants). Early puberty significantly increased the risk of new cases of depression in the unadjusted meta-analysis (RR=1.33; CI 95%: 1.02, 1.73) but not in the adjusted estimate of two of the included studies (RR=1.48; CI 95%: 0.69, 2.28). For late puberty, no significant associations were found (unadjusted RR=1.28; CI 95%: 0.87, 1.88). Two studies assessed the effect of early puberty on depressive symptoms and found positive associations. The quality of the available evidence was rated as very low. LIMITATIONS: The polled results had wide confidence intervals, and the available evidence was of very low quality. CONCLUSIONS: The available evidence supports little confidence regarding the impact of pubertal timing on the onset of depression in girls but suggests that early puberty in girls may increase the risk of depression. Further higher quality studies are needed to clarify the association between pubertal timing and the incidence of depression in girls and women.

Pituitary stalk interruption syndrome: a report of 9 cases / 中华神经医学杂志

Objective To explore the clinical characteristics of pituitary stalk interruption syndrome (PSIS) to raise our awareness of this disease. Methods The clinical data, including clinical manifestations, MR image changes and disorders of endocrine system, of 9 patients admitted to our hospital were collected and analyzed. Results Eight of 9 patients showed absence of pituitary stalk under MRI with height of the pituitary no more than 3 mm; only one exceptional patient with traumatic etiology showed 4.5 mm of the pituitary. Two patients were adult-onset and clearly induced by head trauma, and both of them were hospitalized due to pituitary crisis; the other 7 patients, having the disease at the age of 5 to 12, were complained of growth and development retardation at the age of 17 to 28. All the patients were totally deficient in growth hormone (GH) and pituitary gonadotropin (GnH) secretion; in addition, secondary hypothyroidism and hypocortisolism occurred in 6 of the 7 young-onset patients. No consanguinity, sign of pituitary crisis, and septooptic dysplasia were noted in those young-onset patients.Conclusion PSIS is characterized by absence of pituitary stalk and pituitary hypoplasia, by GH and GnH deficiency, and mostly combined with ACTH and TSH deficiency of different extent.

Analysis on the proportion of incidence of etiological agents in 133 cases with constitutional delayed puberty / 中国基层医药

Objective To analyze the proportion of incidence of etiological agents in 133 cases with constitutional delayed puberty.Methods Clinical data of etiological agents in 133 patients with constitutional delayed puberty were retrospectively analyzed.Results Etiological agents in 133 cases with constitutional delayed puberty were as follows:Hypo-gonadotrophic hormone group(56.39%,n=75):39 cases with unknown reason(idiopathy,3 cases were female),intrapartum asphyxia/hypoxia or hemorrhage(n=23),pituitary glands dysplasia(n=6),cephal trauma(n=3),postoperative craniopharyngioma(n=2),empty sella turciea(n=2),combined hormone deficiency(n=59).Hyper-gonadotrophic hormone group(17.29%,n=23):17 cases with chromosomal disorders(n=17,male:female=7:16),3 cases with unknown reason(idiopathy).31 cases with constitutional delayed puberty(23.31%),4 cases with functional delayed puberty(3.01%).Conclusion Many etiological agents could result in delayed puberty,different origins of delayed puberty had different therapies.Classification of etiological agents in patients with constitutional delayed puberty phyed an important role in guiding option of clnical treatment.

Optimal age of sexual maturation in Egyptian children

To establish the optimal age of sexual maturation in Egyptian children, Tanner's maturity stages were determined for a sample of children and adolescents [1550 girls, 1563 boys] ranging from 6.5 to 18.5 years. The mean age for attainment of pubic hair [stage PH2] was 10.46 [SD 1.36] years for girls and 11.86 [SD 1.45] years for boys. For axillary hair [stage A2], mean age was 11.65 [SD 1.62] years for girls and 13.55 [SD 1.52] years for boys. The mean age at menarche in girls was 12.44 years and for breast development [stage B2] was 10.71 [SD 1.30] years. Testicular volume by palpation showed that the mean age of genital stage G2 for boys was 10.56 [SD 1.40] years. The study results can aid in the assessment of sexual maturation and pubertal disorders in Egyptian adolescents

Clinical value of LHRH exciting test in differential diagnosis of constitutional delayed puberty and male hypogonadotropic hypogonadism / 中华内分泌代谢杂志

Objective To analyse the clinical significance of LHRH exciting test in the differential diagnosis of constitutional delayed puberty (CDP) and hypogonadotropic hypogonadism (HH). Methods Eighty-one cases from 1982 to 1998 were investigated and followed up. They were all at genital stage Ⅰ. After injection of 100 ?g LHRH, the blood samples (3 ml) were taken at -15, 0, 15, 30, 45, 60, 90 and 120 min. The serum LH and FSH levels were determined by radioimmunoassay. Then they were followed up every 3-24 months. After they received LHRH exciting test, they were followed up until over 18 years old. According to their puberty development status, they were divided into 3 groups, normal group (n=34),CDP group (n=16) and HH group (n=31),andthemeanage,whenthey received LHRH exciting test, was (10.2?0.9, range 9-14) years, (16.0?1.0, range 14-18) years and (17.1?1.4, range 16-22) years respectively. Results There were no significant differences in serum LH baseline level and peak time in normal, CDP and HH groups, but the serum LH peak level, LH increment (peak LH level minus baseline LH level), LH increment ratio (peak level/baseline level of LH) and the area under LH curve (AUC LH ) of normal group were significantly higher than those of CDP group and HH group (all P