Search

Leuprolide acetate, a gonadotropin-releasing hormone agonist, is used in the treatment of central precocious puberty. Besides local reactions, systemic hypersensitivity reactions have been reported in the literature during GnRH analog therapy. A case which has developed angioedema after 7th and 8th doses of the treatment while receiving leuprolide acetate treatment for central precocious puberty is presented in this article. Clinicians should be aware of allergic reactions that occur with GnRH analog therapy and injections must be administered in the hospital setting after appropriate precautions have been taken. [ABSTRACT FROM AUTHOR]

Background: Gonadotropin-releasing hormone (GnRH) analogues have been widely used in the treatment of patients with central precocious puberty (CPP) for many years. In previous studies regarding the effects of GnRH analogues therapy on body mass index (BMI), conflicting results have been obtained. In this study, it was aimed to evaluate the effects of GnRH analogues therapy on BMI in girls diagnosed with CPP. Materials and Methods: In the study, a total of 145 female patients, who were treated and followed up due to CPP between September 2016 and June 2021, were included. In the retrospective review of medical records of the patients, age at admission, height, weight, BMI and standard deviation scores (SDS), bone age, Tanner stage, serum follicle-stimulating hormone (FSH) levels, luteinizing hormone (LH) levels, estradiol (E2) levels and the peak LH level in the GnRH stimulation test in the beginning and after the first year of treatment were evaluated. Results: The mean age of 145 patients with central precocious puberty in the beginning of treatment was 7.27±0.97 years, and the mean bone age was 9.12±1.10 years. At the beginning of treatment, 118 (81.38%) of the patients were at a normal weight or underweight, and 27 (18.62%) patients were overweight or obese. In terms of stages of puberty, 109 (75.17%) of the patients were Tanner stage 2, 30 (20.69%) patients were Tanner stage 3, and 6 (4.14%) patients were Tanner stage 4. While the mean BMI-SDS of all patients was 0.11±0.99 before the treatment, it increased significantly (p<0.001) by the end of the first year of treatment and was found as 0.35 ± 0.95. While the mean BMI-SDS of patients who were at a normal weight or underweight was -0.21± 0.78 before the treatment, it was found as 0.09±0.84 after the treatment (p < 0.001). The initial BMI-SDS of overweight or obese patients was 1.53±0.40, and it was found as 1.48±0.49 after the treatment (p=0.412). Conclusions: cious puberty increased the BMI-SDS in patients who were at a normal weight or underweight, but it did not cause any change in patients who were overweight or obese. [ABSTRACT FROM AUTHOR]

Introduction: Gain-of-function mutations in the KISS1 and KISS1R genes and paternally inherited loss-of-function mutations in the Delta-like noncanonical Notch ligand 1 (DLK1) and imprinted Makorin ring finger protein 3 (MKRN3) genes are the most common genetic causes of familial central precocious puberty (CPP) cases. This report presents a girl with a family history of early puberty and a pathogenic variant in the MKRN3 gene. Case Report: The girl who was seven years and four months old with breast and pubic hair development was referred to pediatric endocrinology clinic. Medical history was usual and her parents were unrelated. Her father, paternal uncle, and cousins had a history of CPP. At the time of the first visit; weight was 34 kg (2.06 SD), height 127 cm (0.78 SD), BMI was 1.95 SDS, with a target height of 148.1 cm (-2.01 SD, mother height -1.52 SD, father height -3.44 SD), breast Tanner stage III, pubic hair Tanner stage II. In laboratory examinations, basal LH and estradiol tests were high, thyroid function tests, and routine biochemical studies were normal. Bone age, according to Greulich-Pyle was 10.5 years. On pelvic ultrasound, the long axis of the uterus was increased. LH-RH stimulation test was indicative of CPP. Considering the family medical history of the father's side, we suspected a genetic cause for the CPP and MKRN3 gene. MKRN3 gene analysis showed a previously identified c.482dupC heterozygous variant in the patient and her father. Treatment with Gonadotropin-releasing hormone analog was continued until the bone age reached 12.5 years. Our patient is now 12.5 years old and has regular menstruation, and with the help of the treatment, her height has exceeded the target height. Conclusion: In evaluating CPP cases, family history and genetic analysis are important in terms of early diagnosis and treatment with genetic counseling of the next generations. [ABSTRACT FROM AUTHOR]

Aim:Gonadotropin-releasing hormone agonists (GnRHa) are widely used in the treatment of central precocious puberty (CPP). There is concern that GnRHa treatment, whose positive effects on the adult height are known, may cause weight gain and body mass index (BMI) increase. The aim of this study was to assess the effect of the GnRHa treatment on BMI and height in female patients with CPP.Materials and Methods:Ninety-two patients diagnosed with idiopathic CPP and 22 patients diagnosed with organic CPP, who received GnRHa treatment were included in the study. Data taken on the treatment start date, 6th month, 1st and 2nd year for height, weight, BMI and bone age were obtained retrospectively from the file records.Results:BMI standard deviation score (SDS) increased during the treatment period in all the patients. In the second year of GnRHa treatment, BMI SDS was higher in the organic CPP, compared to the idiopathic CPP (0.66±0.84 and 1.35±0.72, p=0.007). In both groups, at the beginning of GnRHa treatment, the BMI SDS increase was higher in those patients with normal weight compared to those who were overweight/obese. In both groups, the prevalence of obesity was higher than the reference population at the beginning of treatment. An increase was determined in the height SDS and predicted adult height in both groups according to bone age.Conclusion:In patients with CPP, the prevalence of obesity was higher in the first application compared to the reference population. In CPP, BMI SDS increased with GnRHa treatment. The weight of the patients at the beginning of the treatment affected the weight and BMI change with GnRHa treatment. Those patients with organic CPP were more prone to weight gain and BMI increase.

Aim: To determine the clinical, anthropometric, and laboratory parameters that could be used for differentiating central precocious puberty from premature thelarche in girls who had breast development between the ages of 3 and 8 years. Material and Methods: The study included 344 girls (196 girls with idiopathic central precocious puberty, 148 girls with premature thelarche) who underwent gonadotropin- releasing hormone stimulation tests for breast development. Age at diagnosis, bone age, anthropometric measurements, basal/stimulated hormone levels were recorded. Univariate regression analysis was performed to determine the parameters that could be used for differentiating precocious puberty from premature thelarche. Significant parameters in univariate analyses were grouped according to the thresholds determined using receiver operating characteristic curves and reevaluated through multivariate analysis. Results: The bone age, height-standard deviation score, body mass index- standard deviation score, and growth velocity-standard deviation score at diagnosis were found to be higher; pubertal stages were found to be more advanced; uterus and ovary volumes were found to be larger; and the basal/peak luteinizing hormone, follicle-stimulating hormone, luteinizing hormone/follicle-stimulating hormone levels were found to be higher in the subjects with precocious puberty. There was no difference between estradiol levels between the two groups. The best threshÖz olds to differentiate the two groups were found as 0.65 IU/L (78% sensitivity, 100% specificity), 1.9 IU/L (100% sensitivity, 72% specificity), 0.25 (67% sensitivity, 100% specificity) and 1.1 (69% sensitivity, 71% speci- ficity), respectively, for basal luteinizing hormone, follicle-stimulating hormone, luteinizing hormone/follicle-stimulating hormone ratio, and the growth velocity-standard deviation score. Conclusion: In girls presenting with early breast development, a basal luteinizing hormone level of ≥.65 IU/L and a luteinizing hormone/follicle- stimulating hormone ratio of ≥0.25 are sensitive ways to demonstrate activation of the hypothalamo-pituitary-gonadal axis. Among these, the variable that gives the best sensitivity and specificity is the measurement of basal luteinizing hormone levels (≥0.65 IU/L), which can be used as a screening test in the diagnosis of central precocious puberty. [ABSTRACT FROM AUTHOR]

Anaphylaxis is an acute, potentially life-threatening allergic multisystemic hypersensitivity reaction. Although the anaphylaxis incidence is not clearly known, serious reactions are seen at a rate of approximately 1-3 per 10,000. Drugs are among the most common causes of anaphylactic reactions. Triptorelin acetate treatment aims to alleviate the clinical symptoms of early pubertal development, the psychological consequences and the effects on growth. Triptorelin acetate is major long-acting gonadotropin releasing hormone (GnRH) analogue. Our case diagnosed with central precocious puberty was being followed up by the pediatric endocrinology department when anaphylaxis occurred following an injection at the 18th month of treatment. Anaphylaxis with Triptorelin acetate has been reported in only two cases in the literature. Although the risk of anaphylaxis with this drug is low, the possibility should be considered and appropriate measures taken. [ABSTRACT FROM AUTHOR]