Purpose: This study aimed to examine the effects of gonadotropin-releasing hormone agonist (GnRHa) treatment on final height outcomes in girls with idiopathic central precocious puberty (CPP) from the start of treatment to their postmenarche visit. Methods: We conducted a retrospective analysis of 200 girls with idiopathic CPP who received GnRHa therapy, focusing on auxological and clinical outcomes at treatment initiation, treatment completion, and the last, postmenarche visit. Results: The mean chronological age (CA) at GnRHa treatment initiation was 8.24±0.73 years. The mean duration of GnRHa treatment was 3.12±0.81 years. The average age at menarche was 12.73±0.56 years, occurring a mean of 17.15±5.52 months after completing GnRHa therapy. The predicted adult height (PAH) standard deviation score (SDS) after menarche (0.48±0.99) was significantly greater than before treatment (-1.33±1.46) (P<0.001). Factors including greater bone age advancement (P<0.001), lower height SDS for CA at treatment initiation (P<0.001), and higher midparental height SDS (P=0.001) were positively associated with an increase in PAH SDS at the last visit. However, near-final height and the increase in PAH SDS at the last visit were not significantly different between patients who received early treatment (<8 years) and those who received later treatment (8–9 years). Conclusion GnRHa treatment improved the final height outcomes in all girls with CPP, including those treated between 8 and 9 years of age.

Purpose: This study aimed to assess prognostic factors associated with combined hepatocellular-cholangiocarcinoma (cHCC-CCA) and to predict 5-year survival based on these factors. Materials and Methods: Patients who underwent definitive hepatectomy from 2006 to 2022 at a single institution was retrospectively analyzed. Inclusion criteria involved a pathologically confirmed diagnosis of cHCC-CCA. Results: A total of 80 patients with diagnosed cHCC-CCA were included in the analysis. The median progression-free survival was 15.6 months, while distant metastasis-free survival (DMFS), hepatic progression-free survival, and overall survival (OS) were 50.8, 21.5, and 85.1 months, respectively. In 52 cases of recurrence, intrahepatic recurrence was the most common initial recurrence (34/52), with distant metastasis in 17 cases. Factors associated with poor DMFS included tumor necrosis, lymphovascular invasion (LVI), perineural invasion, and histologic compact type. Postoperative carbohydrate antigen 19-9, tumor necrosis, LVI, and close/positive margin were associated with poor OS. LVI emerged as a key factor affecting both DMFS and OS, with a 5-year OS of 93.3% for patients without LVI compared to 35.8% with LVI. Based on these factors, a nomogram predicting 3-year and 5-year DMFS and OS was developed, demonstrating high concordance with actual survival in the cohort (Harrell C-index 0.809 for OS, 0.801 for DMFS, respectively). Conclusion The prognosis of cHCC-CCA is notably poor when combined with LVI. Given the significant impact of adverse features, accurate outcome prediction is crucial. Moreover, consideration of adjuvant therapy may be warranted for patients exhibiting poor survival and increased risk of local recurrence or distant metastasis.

Purpose: We report a case of bilateral nasal and temporal four-site scleromalacia following cosmetic conjunctivectomy, successfully managed with simultaneous restoration surgery at all four sites using autologous tragal perichondrium and preserved scleral graft.Case summary: A 56-year-old woman who underwent cosmetic conjunctivectomy on both eyes 15 years earlier, presented with intermittent eye pain. Examination revealed scleromalacia with calcium plaques in all four nasal and temporal scleral areas. The most extensive avascular area was in the nasal region of the right eye, and the most prominent uveal exposure was in the nasal area of the left eye. Considering the lesion and exposure sizes of the avascular zone and uvea exposure, preserved sclera was transplanted to the temporal and nasal areas of the right eye, while autologous tragal perichondrium was transplanted to the nasal area of the left eye. Tenon's capsule vessels were fixed with pedicle flaps in all four surgical areas for vascularization. Frozen amniotic membrane was transplanted in the nasal area of the left eye, where the conjunctival defect was substantial, and in the lateral area of the left eye, where no graft was used. The conjunctival epithelium was closed using autologous conjunctival flaps in all four areas. Post-surgery, successful engraftment and vascularization of the graft materials were observed in both eyes. Conclusions Even with multiple areas of scleromalacia in both eyes, successful results can be achieved during simultaneous surgery if an appropriate graft material is selected and the surgery is performed utilizing principles of tissue restoration.

Purpose: We report a case of bilateral nasal and temporal four-site scleromalacia following cosmetic conjunctivectomy, successfully managed with simultaneous restoration surgery at all four sites using autologous tragal perichondrium and preserved scleral graft.Case summary: A 56-year-old woman who underwent cosmetic conjunctivectomy on both eyes 15 years earlier, presented with intermittent eye pain. Examination revealed scleromalacia with calcium plaques in all four nasal and temporal scleral areas. The most extensive avascular area was in the nasal region of the right eye, and the most prominent uveal exposure was in the nasal area of the left eye. Considering the lesion and exposure sizes of the avascular zone and uvea exposure, preserved sclera was transplanted to the temporal and nasal areas of the right eye, while autologous tragal perichondrium was transplanted to the nasal area of the left eye. Tenon's capsule vessels were fixed with pedicle flaps in all four surgical areas for vascularization. Frozen amniotic membrane was transplanted in the nasal area of the left eye, where the conjunctival defect was substantial, and in the lateral area of the left eye, where no graft was used. The conjunctival epithelium was closed using autologous conjunctival flaps in all four areas. Post-surgery, successful engraftment and vascularization of the graft materials were observed in both eyes. Conclusions Even with multiple areas of scleromalacia in both eyes, successful results can be achieved during simultaneous surgery if an appropriate graft material is selected and the surgery is performed utilizing principles of tissue restoration.

Purpose: We report a case of bilateral nasal and temporal four-site scleromalacia following cosmetic conjunctivectomy, successfully managed with simultaneous restoration surgery at all four sites using autologous tragal perichondrium and preserved scleral graft.Case summary: A 56-year-old woman who underwent cosmetic conjunctivectomy on both eyes 15 years earlier, presented with intermittent eye pain. Examination revealed scleromalacia with calcium plaques in all four nasal and temporal scleral areas. The most extensive avascular area was in the nasal region of the right eye, and the most prominent uveal exposure was in the nasal area of the left eye. Considering the lesion and exposure sizes of the avascular zone and uvea exposure, preserved sclera was transplanted to the temporal and nasal areas of the right eye, while autologous tragal perichondrium was transplanted to the nasal area of the left eye. Tenon's capsule vessels were fixed with pedicle flaps in all four surgical areas for vascularization. Frozen amniotic membrane was transplanted in the nasal area of the left eye, where the conjunctival defect was substantial, and in the lateral area of the left eye, where no graft was used. The conjunctival epithelium was closed using autologous conjunctival flaps in all four areas. Post-surgery, successful engraftment and vascularization of the graft materials were observed in both eyes. Conclusions Even with multiple areas of scleromalacia in both eyes, successful results can be achieved during simultaneous surgery if an appropriate graft material is selected and the surgery is performed utilizing principles of tissue restoration.

Purpose: This study aimed to assess prognostic factors associated with combined hepatocellular-cholangiocarcinoma (cHCC-CCA) and to predict 5-year survival based on these factors. Materials and Methods: Patients who underwent definitive hepatectomy from 2006 to 2022 at a single institution was retrospectively analyzed. Inclusion criteria involved a pathologically confirmed diagnosis of cHCC-CCA. Results: A total of 80 patients with diagnosed cHCC-CCA were included in the analysis. The median progression-free survival was 15.6 months, while distant metastasis-free survival (DMFS), hepatic progression-free survival, and overall survival (OS) were 50.8, 21.5, and 85.1 months, respectively. In 52 cases of recurrence, intrahepatic recurrence was the most common initial recurrence (34/52), with distant metastasis in 17 cases. Factors associated with poor DMFS included tumor necrosis, lymphovascular invasion (LVI), perineural invasion, and histologic compact type. Postoperative carbohydrate antigen 19-9, tumor necrosis, LVI, and close/positive margin were associated with poor OS. LVI emerged as a key factor affecting both DMFS and OS, with a 5-year OS of 93.3% for patients without LVI compared to 35.8% with LVI. Based on these factors, a nomogram predicting 3-year and 5-year DMFS and OS was developed, demonstrating high concordance with actual survival in the cohort (Harrell C-index 0.809 for OS, 0.801 for DMFS, respectively). Conclusion The prognosis of cHCC-CCA is notably poor when combined with LVI. Given the significant impact of adverse features, accurate outcome prediction is crucial. Moreover, consideration of adjuvant therapy may be warranted for patients exhibiting poor survival and increased risk of local recurrence or distant metastasis.

Purpose: This study aimed to examine the effects of gonadotropin-releasing hormone agonist (GnRHa) treatment on final height outcomes in girls with idiopathic central precocious puberty (CPP) from the start of treatment to their postmenarche visit. Methods: We conducted a retrospective analysis of 200 girls with idiopathic CPP who received GnRHa therapy, focusing on auxological and clinical outcomes at treatment initiation, treatment completion, and the last, postmenarche visit. Results: The mean chronological age (CA) at GnRHa treatment initiation was 8.24±0.73 years. The mean duration of GnRHa treatment was 3.12±0.81 years. The average age at menarche was 12.73±0.56 years, occurring a mean of 17.15±5.52 months after completing GnRHa therapy. The predicted adult height (PAH) standard deviation score (SDS) after menarche (0.48±0.99) was significantly greater than before treatment (-1.33±1.46) (P<0.001). Factors including greater bone age advancement (P<0.001), lower height SDS for CA at treatment initiation (P<0.001), and higher midparental height SDS (P=0.001) were positively associated with an increase in PAH SDS at the last visit. However, near-final height and the increase in PAH SDS at the last visit were not significantly different between patients who received early treatment (<8 years) and those who received later treatment (8–9 years). Conclusion GnRHa treatment improved the final height outcomes in all girls with CPP, including those treated between 8 and 9 years of age.

Purpose: This study aimed to examine the effects of gonadotropin-releasing hormone agonist (GnRHa) treatment on final height outcomes in girls with idiopathic central precocious puberty (CPP) from the start of treatment to their postmenarche visit. Methods: We conducted a retrospective analysis of 200 girls with idiopathic CPP who received GnRHa therapy, focusing on auxological and clinical outcomes at treatment initiation, treatment completion, and the last, postmenarche visit. Results: The mean chronological age (CA) at GnRHa treatment initiation was 8.24±0.73 years. The mean duration of GnRHa treatment was 3.12±0.81 years. The average age at menarche was 12.73±0.56 years, occurring a mean of 17.15±5.52 months after completing GnRHa therapy. The predicted adult height (PAH) standard deviation score (SDS) after menarche (0.48±0.99) was significantly greater than before treatment (-1.33±1.46) (P<0.001). Factors including greater bone age advancement (P<0.001), lower height SDS for CA at treatment initiation (P<0.001), and higher midparental height SDS (P=0.001) were positively associated with an increase in PAH SDS at the last visit. However, near-final height and the increase in PAH SDS at the last visit were not significantly different between patients who received early treatment (<8 years) and those who received later treatment (8–9 years). Conclusion GnRHa treatment improved the final height outcomes in all girls with CPP, including those treated between 8 and 9 years of age.

Purpose: This study aimed to assess prognostic factors associated with combined hepatocellular-cholangiocarcinoma (cHCC-CCA) and to predict 5-year survival based on these factors. Materials and Methods: Patients who underwent definitive hepatectomy from 2006 to 2022 at a single institution was retrospectively analyzed. Inclusion criteria involved a pathologically confirmed diagnosis of cHCC-CCA. Results: A total of 80 patients with diagnosed cHCC-CCA were included in the analysis. The median progression-free survival was 15.6 months, while distant metastasis-free survival (DMFS), hepatic progression-free survival, and overall survival (OS) were 50.8, 21.5, and 85.1 months, respectively. In 52 cases of recurrence, intrahepatic recurrence was the most common initial recurrence (34/52), with distant metastasis in 17 cases. Factors associated with poor DMFS included tumor necrosis, lymphovascular invasion (LVI), perineural invasion, and histologic compact type. Postoperative carbohydrate antigen 19-9, tumor necrosis, LVI, and close/positive margin were associated with poor OS. LVI emerged as a key factor affecting both DMFS and OS, with a 5-year OS of 93.3% for patients without LVI compared to 35.8% with LVI. Based on these factors, a nomogram predicting 3-year and 5-year DMFS and OS was developed, demonstrating high concordance with actual survival in the cohort (Harrell C-index 0.809 for OS, 0.801 for DMFS, respectively). Conclusion The prognosis of cHCC-CCA is notably poor when combined with LVI. Given the significant impact of adverse features, accurate outcome prediction is crucial. Moreover, consideration of adjuvant therapy may be warranted for patients exhibiting poor survival and increased risk of local recurrence or distant metastasis.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.