Annals of Pediatric Endocrinology & Metabolism最新文献

Purpose: Acute leukemia is the most common form of malignancy in children; however, there is an absence of data regarding the long-term ovarian reserve of female survivors. The purpose of this investigation was to examine potential discrepancies in anti-Mullerian hormone (AMH) levels among female survivors of pediatric acute leukemia.

Methods: A cross-sectional study was conducted on female survivors aged 2 to 18 years who completed gonadotoxic chemotherapy sessions at least 1 year prior to evaluation. Based on Tanner staging, the participants were divided into pre-pubertal and pubertal groups. Serum AMH, follicle stimulating hormone (FSH), luteinizing hormone (LH), and estradiol were assessed.

Results: Three of 36 participants demonstrated low AMH levels in comparison to the adjusted age-reference range, whereas all participants had normal FSH and LH levels. However, the correlation between cumulative dose of cyclophosphamide and AMH level was not statistically significant.

Conclusion: Measurement of AMH level may not be beneficial in individuals who have received a cumulative dose of cyclophosphamide less than 5,400 mg/m2.

Purpose: 46,XX disorders of sex development (DSD) involve atypical genitalia accompanied by a normal female karyotype. This study was performed to investigate the clinical characteristics and long-term outcomes of patients with 46,XX DSD.

Methods: The study included 34 patients with 46,XX DSD who presented with ambiguous genitalia or delayed puberty. Patients with congenital adrenal hyperplasia were excluded. Clinical phenotypes and overall outcomes were analyzed retrospectively.

Results: Age at presentation ranged from birth to 40 years (median, 0.6 years), and the follow-up period ranged from 0.3 to 29.7 years (median, 8.8 years). Twenty patients were assigned female (58.8%). Etiologies included disorders of gonadal development (n=22), exogenous androgen exposure during pregnancy (n=5), association with syndromic disorders or genital anomalies (n=2), and unclassified causes (n=5). Ovotestis was the most frequent gonadal pathology (41.7%). Müllerian duct remnants were usually underdeveloped (52.9%) or absent (23.5%). Spontaneous puberty occurred in 17 of the 21 patients of pubertal age, while 9 patients required sex hormone replacement therapy. Gonadal complications were observed in 4 patients (gonadal tumors [n=3], and spontaneous gonadal rupture [n=1]), and gender dysphoria occurred in 1 patient who was assigned male.

Conclusion: This study described the wide phenotypic spectrum and pubertal outcome of patients with 46,XX DSD. Long-term multidisciplinary monitoring for pubertal development, fertility, gender identity, and gonadal complications is recommended.

Type 2 diabetes mellitus (T2DM) management demands innovative strategies that address its complex nature. Telerehabilitation and conventional rehabilitation, which utilize wearable devices, represent promising avenues in this regard. This narrative review synthesizes the current literature to comprehensively compare these modalities in terms of accessibility, monitoring mechanisms, patient adherence, cost-effectiveness, and social support. Telerehabilitation offers unparalleled convenience, real-time monitoring, and personalized feedback through wearables, thereby fostering greater patient engagement and adherence compared to conventional rehabilitation. However, conventional rehabilitation provides face-to-face interactions, immediate feedback, and a more personalized touch, albeit with logistical challenges and higher costs. This review emphasizes the significance of patient preferences, technological access, and healthcare infrastructure in selecting the appropriate approach. It also calls for further research into long-term outcomes, cost-effectiveness, and the optimal integration of wearable technology in diabetes management programs. Ultimately, both telerehabilitation and conventional rehabilitation demonstrate considerable potential in empowering individuals with T2DM, underlining the imperative for tailored and patient-centric interventions in diabetes care. The review also stresses the significance of integrating patient preferences and their level of comfort with technology when deciding on treatment approaches. It also takes into account the diverse socioeconomic contexts and healthcare infrastructures globally, which can affect the viability and efficacy of both telerehabilitation and conventional rehabilitation. Moreover, the integration of wearable technology in diabetes management programs holds promise for enhancing self-management capabilities and promoting healthier lifestyles. However, it is essential to tackle prospective discrepancies in access to these technologies and ensure fair distribution. Looking forward, ongoing research efforts should focus on justifying long-term outcomes, optimizing cost-effectiveness, and refining implementation strategies to maximize the benefits of both modalities.

Long-acting growth hormones (LAGHs) represent a significant advancement in the treatment of pediatric GH deficiency, offering an alternative to daily recombinant human GH (rhGH) therapy. Traditional rhGH treatments, although effective, require daily injections, often leading to poor adherence due to the frequency of dosing, injection pain, and difficulties with storage and travel. In contrast, LAGHs, such as somatrogon, somapacitan, and lonapegsomatropin, are designed for once-weekly administration, improving patient compliance and quality of life. LAGHs have demonstrated non-inferiority to daily rhGHs in phase 3 clinical trials, showing similar efficacy in terms of growth velocity and safety profiles. Despite these advantages, concerns remain regarding the altered pharmacodynamics of LAGHs such as the lack of pulsatile secretion and potential for antibody formation. Although the overall safety of LAGHs has been confirmed, side effects, such as lipoatrophy at the injection site, may occur, especially with PEGylated formulations. Guidelines for prescribing LAGHs are currently evolving. LAGHs are not yet approved for other conditions traditionally treated with rhGHs, such as Turner or Noonan syndrome. Pediatric endocrinologists should carefully consider which patient groups would benefit most from this therapy, particularly individuals at risk of poor adherence to daily injections such as patients undergoing multidrug therapy, patients with needle phobia or behavioral disorders, very young children, adolescents, patients with separated parents, families that travel frequently, or children involved in activities such as scouting. LAGHs present an opportunity to enhance therapeutic outcomes and adherence, but careful patient selection remains critical to maximize their potential benefits.

Exercise is known to promote physical health and reduce the risk of various diseases. During exercise, skeletal muscle actively contracts to perform movements and secretes hormone-like molecules termed myokines. The beneficial effects of exercise have been assessed with respect to myokine production, and those of irisin on bone, adipose tissue, and the brain have been well documented. Irisin, through its interactions with the integrin αV family, plays a crucial role in bone maintenance, metabolic regulation, and cognitive function. Building on the established understanding of irisin, this discussion will examine the functions and effects of other myokines as key secretory factors in exercise, emphasizing their broader roles in health promotion and the potential for new therapeutic strategies in disease prevention and treatment.

Purpose: We evaluated the effectiveness of early start of long-acting insulin during management of diabetic ketoacidosis (DKA) in pediatric patients.

Methods: Patients with DKA were randomly assigned to receive either a traditional DKA management protocol or concurrent administration of subcutaneous (SC) long-acting insulin alongside intravenous insulin during DKA treatment. The primary outcomes were duration of insulin infusion and adverse effects of the intervention, mainly hypoglycemia and hypokalemia.

Results: For this study, 100 pediatric patients with DKA were enrolled, 50 in each group (group I received the conventional DKA management and group II received conventional DKA management plus SC long-acting insulin once daily). Patients in group II showed a significant reduction in both duration and dose of insulin infusion compared to group I, with a median (interquartile range) of 68.5 hours (45.00-88.25 hours) versus 72 hours (70.25-95.5 hours) (P=0.0001) and an insulin dose of 3.48±1.00 units/kg versus 4.04±1.17 units/kg (P=0.016), respectively. Concurrent administration of SC long-acting insulin with intravenous insulin during DKA treatment was associated with a decreased risk of hypoglycemia (number of hypoglycemia events: group I, 22 events; group II, 12 events, P=0.029), with no increased risk of hypokalemia compared to the control group (number of hypokalemia events: group I, 12 events; group II, 19 events, P=0.147).

Conclusion: The current study showed that coadministration of SC long-acting insulin in addition to the usual insulin infusion during DKA management in the pediatric population can lead to a shorter duration of insulin infusion. In addition, this approach is not associated with increased risk of hypoglycemia or hypokalemia. Moreover, coadministration of long-acting insulin may be associated with a decreased incidence of hypoglycemia.

Purpose: Guidelines of the Pediatric American Thyroid Association (ATA) serve as a vital reference for managing the rare thyroid cancers in childhood. This study evaluates differentiated thyroid cancer (DTC) patients using the ATA guidelines, dynamic risk stratification (DRS), and other established risk classification systems.

Methods: Pediatric patients with DTC under observation after total thyroidectomy were included in the study. We assessed preoperative and postoperative features based on the ATA guidelines, other risk scoring systems (TNM; De Groot staging; metastasis, age, completeness of resection, invasion, and tumor size; and combined risk), and the DRS.

Results: A total of 41 patients was enrolled in the study, with a median follow-up duration of 5.14±3.94 years. Of the patients who underwent total thyroidectomy, 33 were diagnosed with papillary carcinoma and 8 with follicular thyroid carcinoma. During follow-up, cervical metastases were detected in 27 patients, and one had distant metastasis. All patients underwent total thyroidectomy, and 68% received lymph node dissection. Additionally, 16 patients received radioactive iodine therapy. Of the postoperative patients, 85.3% were classified as low risk. Based on DRS, patients were classified as having no evidence of disease (n=29, 70.7%), biochemical evidence of persistent disease (n=5, 12.2%), structural evidence of persistent disease (n=6, 14.6%), and recurrent disease (n=1, 2.5%). Notably, 98% of the patients showed no evidence of disease during their latest follow-up.

Conclusion: Persistent disease in patients classified as low risk according to the ATA guidelines resolved following radioactive iodine therapy, emphasizing the importance of risk stratification in postoperative care.