Puberte Gecikmesi Nedeniyle Başvuran Yapisal Büyüme Puberte Gecikmesi Ve İzole Hipogonadotropik Hipogonadizm Tanili Erkek Çocuklarinin Uzun Dönem Boy Ve Puberte Gelişimi Durumlarinin Değerlendirilmesi

Abstract

Dogan O, Evaluation of Long-Term Height and Puberty Development in Boys with Constitutional Growth Delay in Puberty and Isolated Hypogonadotropic Hypogonadism Presenting Due to Delayed Puberty, Hacettepe University Faculty of Medicine, Department of Pediatrics, Thesis of Pediatrics, 2023. Delayed puberty is defined as the absence of physical signs of puberty up to a certain age (2 to 2.5 standard deviations above the mean age) in the population and affects approximately 2% of adolescents. The most common cause of delayed puberty is constitutional delay in growth and puberty(CDGP) which is fallowed by primary hypogonadism, functional hypogonadotropic hypogonadism (FHH), and secondary hypogonadotropic hypogonadism (HH), respectively. The aim of the present study is to compare the age of pubertal onset, pubertal prognosis and final adult heights of ptaients with the diagnosis of CDGP and HH, during their longterm follow up. The hospital files of 1654 male patients older than 14 years of age who were evaluated for delayed puberty between 01.01.2002 and 01.04.2022 in Hacettepe University İhsan Doğramacı Children's Hospital Pediatric Endocrinology and Hacettepe University Faculty of Medicine Endocrinology Department outpatient clinics were reviewed retrospectively. Among the years 2002-2014, 58; Between 2014-2022, 133 patients were included in the study. The mean age at first evaluation was 14.59±0.92 years; The mean height at the first evaluation was 154±9.45 cm (-1.56±1 SDS). A history of delayed puberty was present in the family of 57 patients (29.8%); Cryptorchidism in 28 (14.7%) patients; hypospadias 3 (1.6%) patients; and micropenis in 20 (10.5%) patients. The mean height of the patients was 157.1±9.2 cm in the 6th month, 160.9±9.1 cm in the 1st year, with an average growth rate of 6.88±3.46 cm/year in the first 6 months and 7±2.5 cm/year in the first 1 year. In the whole follow-up, the growth rate was 5±2 cm/year. A total of 167 patients had reached final adult height (FAH). When the FAH was evaluated according to the target height, 147 (88%) had a height consistent with target height; 13 of them (7.8%) were short; 7 (4.2%) were found taller. The mean final height of the patients was 172.1±7.2 cm (-0.53±0.97 SDS). distribution of the patients according to the final diagnosis revealed that 149 (78.01%) of the patients had CDGP; 12 (6.28%) had partial HH; 30 (15.7%) had complete HH. While 97 (50.8%) of the patients were followed without treatment; 52 (27.2%) of them had testosterone induction; 37 (22%) received testosterone replacement. Of the 191 patients evaluated in the study, 80 (41.9%) were of normal weight, 56 (29.3%) were underweight, 44 (23%) were overweight, and 11 (5.8%) were obese. A statistically significant positive strong correlation was found between BMI SDS and initial height SDS and final height SDS. The final height was 179±7.9 cm in patients with BMI SDS >2, while the final height was 171.6±6.9 cm in the remaining group. A strong positive correlation was revealed between target height SDS and final height SDS. Patients with HH had higher height and weight compared to the CDGP. The frequency of cryptorchidism and penile anomaly was significantly higher in HH patients than in CDGP patients. FSH, LH, testosterone, prolactin, ACTH, cortisol, IGF 1, IGFBP-3, TSH and free T4 levels did not differ between the CDGP and HH groups. When the HH and CDGP groups were compared in terms of the development of puberty findings, 29 (69%) patients from the HH group; on the other hand, all of the CDGP group started puberty in the follow-up; The difference between 12 (28.6%) patients in the HH group and 110 (73.8%) patients in the CDGP group was found to be statistically significant. While the mean height of HH patients at 6th months and first year was higher than CDGP patients, the growth rate at 6th months and first year was lower than that of CDGP patients. When compared according to final height, the mean FAH of HH patients was higher than that of CDGP patients. Initial evaluation delta height SDS and final delta height SDS were statistically significantly higher in the CDGP group than in the HH group. Final height status according to target height was evaluated for 128 (85.9%) patients with CDGP and 39 (92.8%) patients with HH. It was determined that 115 (89.9%) patients from the CDGP group and 32 (82%) patients from the HH group had a FAH consistent with the target height. The mean FAH of patients with CDGP who received testosterone induction therapy was 172.9±7 cm, and 170.6±6.45 cm for those who did not receive treatment. The growth rate at 6 months, 1 year, and throughout the follow-up period was similar in patients who received and did not receive treatment (Received 5,06±1,90; not receive treatment 5,14±2,1, p=0,819). Statistically significant positive correlation was seen between target height SDS and final height SDS in both CDGP group and HH group. In both patient groups, it was revealed that there was a statistically significant positive and strong correlation between the height SDS target height SDS difference in the first evaluation and the final height SDS target height SDS difference. As a result, patients with HH and CDGP, which are two groups that are difficult to distinguish from each other, have similar features in terms of height prognosis. It was determined that both groups reached their target heights to a great extent. It was observed that the patients in the HH group had higher initial, target and final heights than the group with CDGP, but the growth rate during follow-up period was lower. As predicted, puberty initiation and progression findings in the HH group were significantly less and more severe than in the CDGP group. In the CDGP group, induction of puberty by testosterone had no effect on height and onset of puberty. It was found that patients with CDGP had reached a FAH consistent with their genetic potential after a delayed but rapid growth.