Different Faces of Minipuberty in Preterm Twin Girls: A Case Report and Review of the Literature.

Minipuberty (MP) consists of a postnatal activation of the hypothalamic-pituitary-gonadal (HPG) axis, which occurs physiologically during the first months of life. In preterm infants, MP might lead to stronger hormonal stimulation, but specific literature is still scarce. We present the case of a pair of monochorionic diamniotic twin girls, born at 31 weeks of gestation and adequate for gestational age (AGA). At one month old, one of the twins presented with severe edema in the vulva and swelling of the major and minor labia. Laboratory evaluations highlighted increased LH, FSH and estradiol serum concentration. Pelvic ultrasonography and MRI showed a pubertal pattern. Brain imaging was unremarkable. During the one-year follow-up, a decreasing trend of hormonal levels was detected, together with the spontaneous regression of clinical and sonographic pubertal signs. The same hormonal workup was also performed on the other twin, who displayed mildly elevated gonadotropins and estradiol, without evidence of pubertal clinical signs. This case suggests that the amplitude of postnatal HPG activation might be exacerbated in preterm infants, with evidence of puberty changes in clinical, laboratory and sonography data. The spontaneous resolution, together with the exclusion of other causes of precocious puberty, is suggestive for MP of infancy.

Minipuberty in born small for gestational age infants: A case control prospective pilot study.

PURPOSE: To evaluate minipuberty (MP) in small for gestational age (SGA) infants, both preterm and full-term, during the first year of life. METHODS: 33 SGA healthy newborns (group A), 21 of which full-term (subgroup A1) and 12 preterm (A2) were enrolled. Control group (B) consisted of 27 AGA, 17 full-term (subgroup B1) and 10 preterm (B2) infants. Growth parameters, FSH, LH, and Estradiol (E2) or Testosterone (T) serum levels were monitored at 3, 6, and 12 months. RESULTS: The gonadotropin surge reached greater increase of LH in males at 3 months and FSH in females at 3, 6 and 12 months (p < 0.001). In male infants: T at 3 months was higher in subgroup A2 vs A1(p = 0.001), and correlated negatively with gestational age (GA, p < 0.005), length and weight at birth (p < 0.05); LH was higher in subgroup B2 vs B1 at 6 months (p = 0.003), and in group A vs B at 12 months (p = 0.03). Females displayed higher E2 at 6 months in B2 vs B1 (p < 0.05), negatively correlated with GA and weight gain (p < 0.05); LH at 6 months was increased in A2 vs A1 (p = 0.03). Overall, preterm males displayed higher T at 3 months (p = 0.001), LH at 3, 6 and 12 months (p < 0.05), and LH/FSH ratio at 6 months (p = 0.001). Preterm females exhibited increased LH/FSH ratio at 3 and 6 months (p < 0.05). CONCLUSIONS: Irrespectively of GA, MP occurred with a typical sexual dimorphism and exhibited sex-specific correlations between hormones and perinatal parameters. SGA condition and prematurity seemed to enhance and protract MP over time in both sexes.

Metabolic Outcomes, Bone Health, and Risk of Polycystic Ovary Syndrome in Girls with Idiopathic Central Precocious Puberty Treated with Gonadotropin-Releasing Hormone Analogues.

BACKGROUND/AIMS: Gonadotropin-releasing hormone analogues (GnRHa) represent the gold standard treatment for central precocious puberty (CPP). We aimed to assess the effects of GnRHa treatment on metabolic outcomes, bone status, and polycystic ovary syndrome (PCOS) prevalence in young girls with idiopathic CPP (ICPP). METHODS: We enrolled 94 ICPP girls who were at least 2 years after menarche and had already attained adult height at the time of the study: 56 previously treated with depot triptorelin (3.4 ± 0.6 years) and 38 untreated. Auxological parameters, lipid profile, homeostatic model assessment of insulin resistance (HOMA-IR), bone state, and prevalence of PCOS were assessed. RESULTS: The 2 groups were similar for body mass index (BMI) and waist circumference. HOMA-IR, dehydroepi-androsterone sulfate, and Δ4-androstenedione were higher in the treated than in the untreated subjects (p < 0.001). Significant differences were found for amplitude-dependent speed of sound (p < 0.03) and bone transmission time z-scores (p < 0.01). The prevalence of PCOS was higher in the treated than in the untreated subjects (p < 0.04). CONCLUSION: GnRHa therapy is associated with hyperandrogenism and an increase in insulin resistance and PCOS prevalence, but not with increased BMI or lipid profile alterations. Long-term evaluations at the time of expected peak bone mass achievement are needed to understand the persistent or transient nature of subtle bone abnormalities.

Hyperthyroidism in childhood: peculiarities of the different clinical pictures.

BACKGROUND: clinical and biochemical picture of hyperthyroidism in children may significantly change according to its etiologies. OBJECTIVES: to report the most recent views about epidemiology, pathophysiology, clinical and biochemical course, diagnostic procedures and management of hyperthyroid syndrome in childhood, according to its different etiologies. DESIGN: Graves' disease and Hashimoto's thyroiditis are responsible for 84% and 12%, respectively, of all the cases of hyperthyroidism in childhood. Hyperfunctioning thyroid nodules (<3% of cases), TSH-secreting adenomas (~1%) and McCune-Albright syndrome (~1%) are distinctly less common. The main hormonal and immunological features of all these conditions were summarizes in Table1, whereas their anamnestic and clinical peculiarities and the diagnostic procedures which may be useful in the differential diagnosis were reported in Table 2. CONCLUSIONS: 1) hyperthyroid syndrome in childhood may present with very different clinical pictures, which are specifically related to the respective etiologies; 2) diagnostic procedures and therapeutic management are not the same in the various conditions.

In children with Hashimoto's thyroiditis the evolution over time of thyroid status may differ according to the different presentation patterns.

AIM: to report the salient literature news concerning the relationships between thyroid function presenting patterns and subsequent biochemical evolution of Hashimoto's thyroiditis (HT) in pediatric age. DESIGN: the most recent reports from pediatric literature concerning biochemical thyroid function patterns at HT presentation and their spontaneous changes over time were analyzed and shortly commented. RESULTS: from the analysis of pediatric literature on this theme, it emerges that HT in children may present with a very heterogeneous biochemical picture ranging from overt hypothyroidism to overt hyperthyroidism. The presenting biochemical pattern may also condition its subsequent evolution over time. CONCLUSIONS: a) the most common biochemical pattern at HT diagnosis in children is euthyroidism, followed by overt hypothyroidism, subclinical hypothyroidism (SH) and hyperthyroidism; b) the association with HT negatively affects the evolution over time of SH; c) in the cases with either Turner syndrome or Down syndrome the evolution over time of SH is more severe than in those without these chromosomopathies.