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Purpose@#The appropriate evaluation of height and accurate estimation of bone age are crucial for proper assessment of the growth status of a child. We developed a bone age estimation program using a deep learning algorithm and established a model to predict the final adult height of Korean children. @*Materials and Methods@#A total of 1678 radiographs from 866 children, for which the interpretation results were consistent between two pediatric endocrinologists, were used to train and validate the deep learning model. The bone age estimation algorithm was based on the convolutional neural network of the deep learning system. The test set simulation was performed by a deep learning program and two raters using 150 radiographs and final height data for 100 adults. @*Results@#There was a statistically significant correlation between bone age interpreted by the artificial intelligence (AI) program and the reference bone age in the test set simulation (r=0.99, p<0.001). In the test set simulation, the AI program showed a mean absolute error (MAE) of 0.59 years and a root mean squared error (RMSE) of 0.55 years, compared with reference bone age, and showed similar accuracy to that of an experienced pediatric endocrinologist (rater 1). Prediction of final adult height by the AI program showed an MAE of 4.62 cm, compared with the actual final adult height. @*Conclusion@#We developed a bone age estimation program based on a deep learning algorithm. The AI-derived program demonstrated high accuracy in estimating bone age and predicting the final adult height of Korean children and adolescents.
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Background/Aims@#There are limited studies on the management of hepatic hemangiomas (HHs). We investigated the proportion and predictors of surgical resection and analyzed HH growth rates in addition to associated factors. @*Methods@#A retrospective case-control study of patients treated in 2 centers was conducted. Thirty-six patients who underwent surgical resection were assigned to the case group. Patients who did not undergo surgical treatment were randomly sigselected at a 1:10 ratio and assigned to the control group (n = 360). Baseline characteristics, clinical course and surgical outcomes were analyzed. @*Results@#The proportion of surgically treated HH patients was 0.3% (36 per 11,049). The longest diameter at diagnosis (mean ± standard deviation) was 7.7 ± 5.2 cm in the case group and 2.4 ± 1.8 cm in the control group (p 10 cm (OR 10.50, 95% CI 1.06–103.77, p = 0.04). The subgroup analysis showed substantial growth in 41.3% with an overall mean annual growth rate of 0.14 cm. @*Conclusions@#Approximately one in 300 patients with an HH underwent surgical treatment. Multiple HHs and a growth rate of more than 4.8%/year were indications for surgical treatment. Nearly half of the HHs showed growing pattern in our study.
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Background@#Toe ganglion cysts are often symptomatic and recurrent. Communicating lesions between ganglion cysts and the interphalangeal joint (IPJ) or tendon sheath make it difficult to prevent a recurrence. Temporary restriction of the joint and tendon motion can facilitate surgical site healing. This study analyzed the clinical results of temporary pin fixation of the IPJ after toe ganglion cyst excision. @*Methods@#Sixteen patients with symptomatic toe ganglion cysts underwent surgical treatment. Excision alone was initially performed on 10 patients. Six patients underwent temporary pin fixation of the IPJ after ganglion cyst excision. Repeat excision with pin fixation was performed for recurrence in two patients after excision only. Clinical evaluations and postoperative complications were analyzed. @*Results@#Fourteen of 16 toe ganglion cysts were located near the IPJ. Two cysts not adjacent to the joint completely healed after excision alone. Seven of 14 cysts near the joint recurred after initial excision alone and required repeated reoperation. Eight cysts did not recur after excision with pin fixation, including 2 that recurred after excision alone. @*Conclusions@#Temporary IPJ pin fixation after excision for ganglion cysts can be effective for preventing the recurrence of ganglion cysts adjacent to toe IPJ.
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Purpose@#We aimed to investigate the prevalences of obesity, abdominal obesity, and non-alcoholic fatty liver disease (NAFLD) among children and adolescents during the coronavirus disease 2019 (COVID-19) outbreak. @*Materials and Methods@#This population-based study investigated the prevalences of obesity, abdominal obesity, and NAFLD among 1428 children and adolescents between 2018–2019 and 2020. We assessed the prevalences of obesity, abdominal obesity, and NAFLD according to body mass index, age, sex, and residential district. Logistic regression analyses were performed to determine the relationships among obesity, abdominal obesity, and NAFLD. @*Results@#In the obese group, the prevalence of abdominal obesity increased from 75.55% to 92.68%, and that of NAFLD increased from 40.68% to 57.82%. In age-specific analysis, the prevalence of abdominal obesity increased from 8.25% to 14.11% among participants aged 10–12 years and from 11.70% to 19.88% among children aged 13–15 years. In residential district-specific analysis, the prevalence of both abdominal obesity and NAFLD increased from 6.96% to 15.74% in rural areas. In logistic regression analysis, the odds ratio of abdominal obesity for NAFLD was 11.82. @*Conclusion@#Our results demonstrated that the prevalences of abdominal obesity and NAFLD increased among obese Korean children and adolescents and in rural areas during the COVID-19 outbreak. Additionally, the prevalence of abdominal obesity increased among young children. These findings suggest the importance of closely monitoring abdominal obesity and NAFLD among children during COVID-19, focusing particularly on obese young children and individuals in rural areas.
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Thyroid hormone plays a vital role in regulating human metabolism. They affect the functions of major organs, such as the brain, liver, skeletal muscle, and heart. Hypothyroidism can lead to dilated cardiomyopathy and decreased heart function. In this report, we describe a case of a teenage boy who developed dilated cardiomyopathy due to hypothyroidism and was considered to undergo heart transplantation. Levothyroxine monotherapy was initiated but produced no improvement. Thereafter, a combination therapy of liothyronine and levothyroxine was administered, and heart function was gradually restored; he recovered completely after 6 months. Cardiac myocytes respond more specifically to liothyronine than to levothyroxine. Therefore, we suggest that liothyronine and levothyroxine combination therapy should be considered rather than levothyroxine monotherapy for hypothyroidism accompanied by heart disease.
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Background/Aims@#Neoadjuvant chemotherapy is increasingly utilized in patients with borderline or locally advanced pancreatic cancer (LAPC). However, the pathologic evaluation of tumor regression is not routinely performed or well established. We aimed to evaluate the prognostic value of three tumor regression grading systems frequently used in LAPC and to determine the correlation between pathologic and clinical response. @*Methods@#We included a total of 38 patients with LAPC who were treated with neoadjuvant chemotherapy and subsequent resection. Pathologic tumor regression was graded based on the College of American Pathologists (CAP), Evans, and MD Anderson grading systems. @*Results@#One out of 38 patients (2.6%) achieved a pathologic complete response. Unlike other grading systems (Evans, p=0.063; MD Anderson, p=0.110), the CAP grading system was a significant prognostic factor for overall survival (p=0.043). Pathologic N stage (p=0.023), margin status (p=0.044), and radiologic response (p=0.016) correlated with overall survival. In the multivariate analysis, CAP 3 was an independent predictor of shorter overall survival (p=0.026). The CAP grading system correlated with the radiologic response (p=0.007) but not the carbohydrate antigen 19-9 level (p=0.333). @*Conclusions@#The four-tier CAP pathologic tumor regression grading system predicted the clinical outcome in LAPC patients who underwent resection after neoadjuvant chemotherapy. Therefore, a more comprehensive pathologic evaluation is warranted in these patients.
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Objective@#CT plays a central role in determining the resectability of pancreatic cancer, which directs the use of neoadjuvant therapy. This study aimed to assess the diagnostic accuracy of CT in predicting circumferential resection margin (CRM) involvement in patients with resectable or borderline resectable pancreatic head cancer. @*Materials and Methods@#Seventy-seven patients who were scheduled for upfront surgery for resectable or borderline resectable pancreatic head cancer were prospectively enrolled, and 75 patients (38 male and 37 female; mean age ± standard deviation, 68 ± 11 years) were finally analyzed. The CRM status was evaluated separately for the superior mesenteric artery (SMA) and posterior and superior mesenteric vein/portal vein (SMV/PV) margins. Three independent radiologists reviewed the preoperative CT images and evaluated the resection margin status. The reference standard for CRM status was pathologic examination of pancreaticoduodenectomy specimens in an axial plane perpendicular to the axis of the second portion of the duodenum. The diagnostic accuracy of CT was assessed for overall CRM involvement, defined as involvement of the SMA or posterior margins (per-patient analysis), and involvement of each of the three resection margins (per-margin analysis). The data were pooled using a crossed random effects model. @*Results@#Forty patients had pathologically confirmed overall CRM involvement in pancreatic cancer, while CRM involvement was not seen in 35 patients. For overall CRM involvement, the pooled sensitivity and specificity were 15% (95% confidence interval: 7%–49%) and 99% (96%–100%), respectively. For each of the resection margins, the pooled sensitivity and specificity were 14% (9%–54%) and 99% (38%–100%) for the SMA margin, 12% (8%–46%) and 99% (97%–100%) for the posterior margin; and 37% (29%–53%) and 96% (31%–100%) for the SMV/PV margin, respectively. @*Conclusion@#CT showed very high specificity but low sensitivity in predicting pathological CRM involvement in pancreatic cancer.
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Background@#Discontinuing growth hormone (GH) treatment during the transition to adulthood has been associated with adverse health outcomes in patients with childhood-onset growth hormone deficiency (CO-GHD). This study investigated the metabolic changes associated with interrupting GH treatment in adolescents with CO-GHD during the transition period. @*Methods@#This study included 187 patients with CO-GHD who were confirmed to have adult GHD and were treated at six academic centers in Korea. Data on clinical parameters, including anthropometric measurements, metabolic profiles, and bone mineral density (BMD) at the end of childhood GH treatment, were collected at the time of re-evaluation for GHD and 1 year after treatment resumption. @*Results@#Most patients (n=182, 97.3%) had organic GHD. The median age at treatment discontinuation and re-evaluation was 15.6 and 18.7 years, respectively. The median duration of treatment interruption was 2.8 years. During treatment discontinuation, body mass index Z-scores and total cholesterol, low-density lipoprotein, and non-high-density lipoprotein (HDL) cholesterol levels increased, whereas fasting glucose levels decreased. One year after GH treatment resumption, fasting glucose levels, HDL cholesterol levels, and femoral neck BMD increased significantly. Longer GH interruption (>2 years, 60.4%) resulted in worse lipid profiles at re-evaluation. The duration of interruption was positively correlated with fasting glucose and non-HDL cholesterol levels after adjusting for covariates. @*Conclusion@#GH treatment interruption during the transition period resulted in worse metabolic parameters, and a longer interruption period was correlated with poorer outcomes. GH treatment should be resumed early in patients with CO-GHD during the transition period.
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Background/Aims@#Several prediction models for evaluating the prognosis of nonmetastatic resected pancreatic ductal adenocarcinoma (PDAC) have been developed, and their performances were reported to be superior to that of the 8th edition of the American Joint Committee on Cancer (AJCC) staging system. We developed a prediction model to evaluate the prognosis of resected PDAC and externally validated it with data from a nationwide Korean database. @*Methods@#Data from the Surveillance, Epidemiology and End Results (SEER) database were utilized for model development, and data from the Korea Tumor Registry System-Biliary Pancreas (KOTUS-BP) database were used for external validation. Potential candidate variables for model development were age, sex, histologic differentiation, tumor location, adjuvant chemotherapy, and the AJCC 8th staging system T and N stages. For external validation, the concordance index (C-index) and time-dependent area under the receiver operating characteristic curve (AUC) were evaluated. @*Results@#Between 2004 and 2016, data from 9,624 patients were utilized for model development, and data from 3,282 patients were used for external validation. In the multivariate Cox proportional hazard model, age, sex, tumor location, T and N stages, histologic differentiation, and adjuvant chemotherapy were independent prognostic factors for resected PDAC. After an exhaustive search and 10-fold cross validation, the best model was finally developed, which included all prognostic variables. The C-index, 1-year, 2-year, 3-year, and 5-year time-dependent AUCs were 0.628, 0.650, 0.665, 0.675, and 0.686, respectively. @*Conclusions@#The survival prediction model for resected PDAC could provide quantitative survival probabilities with reliable performance. External validation studies with other nationwide databases are needed to evaluate the performance of this model.
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As dyslipidemia at a young age is a risk factor for cardiovascular disease in adulthood, the screening and management of dyslipidemia in children and adolescents might be an important health issue. This review deals with issues related to the prevalence, diagnosis, screening, and treatment of pediatric dyslipidemia.Current Concepts: In Korea, the prevalence of pediatric dyslipidemia was 19.7% between 2007 and 2009. Dyslipidemia was defined according to the guidelines of the Korean Society of Pediatric Endocrinology: total cholesterol ≥200 mg/dL, low-density lipoprotein cholesterol ≥130 mg/dL, triglycerides ≥130 mg/dL, high-density lipoprotein cholesterol <40 mg/dL, or non-high-density lipoprotein cholesterol ≥145 mg/dL.Discussion and Conclusion: We recommend universal screening tests for dyslipidemia at ages 9–11 years and 17–21 years. Diet and lifestyle modifications are essential in the treatment of dyslipidemia. In children aged ≥10 years with a poor response to lifestyle modification, drug therapy is recommended. Pediatric dyslipidemia should be diagnosed and treated properly to reduce adult cardiovascular diseases and improve quality of life in this age group.
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Although doctors expend their best effort in treating their patients’ illnesses or injuries, the patients may retain some disabilities even after treatment. Accordingly, some conflicts occur between the patients’ expectations of financial compensation for their disabilities and the financial manager’s policy to efficiently distribute limited resources. The mediation of these social conflicts requires the determination of the degree of physical disability, which can be done by doctors alone. Hence, a reliable disability evaluation guideline should be established. However, currently, only a few educational programs on disability evaluation are available for doctors and there is no reliable guideline for disability evaluation.Current Concepts: There were attempts to implement the American Medical Association guideline in Korea; however, it is currently not being used efficiently because it is quite complicated and unsuited to Korean settings. Mcbride’s disability evaluation, published 60 years ago, is being used, but it is not realistic and unreasonable in Korea. To prepare a standard guideline for disability evaluation, the Korean Academy of Medical Sciences published a booklet, “Disability Evaluation Guideline: Explanation and Case Studies”, based on the American Medical Association guideline. The academy published the booklet’s revised version (2nd edition), ‘Disability evaluation guideline and utilization’, in 2016. However, Korean Academy of Medical Sciences guideline still not being used because it is too complicated to use.Discussion and Conclusion: Fair disability evaluation is a social responsibility given to doctors, and there should be a useful guideline for disability evaluation that reflects the characteristics of each medical society or association.
ABSTRACT
17α-hydroxylase/17,20-lyase deficiency, caused by mutations in the cytochrome P450 family 17 subfamily A member 1 gene (CYP17A1), is an extremely rare form of congenital adrenal hyperplasia that is characterized by diverse phenotypes resulting from specific mutations. Here, we report 2 phenotypic females with 17α-hydroxylase/17,20-lyase deficiency: one with the 46,XX karyotype presenting primary amenorrhea and sexual infantilism, and the other with the 46,XY karyotype presenting a disorder of sexual development. In both cases, the serum levels of adrenocorticotropic hormone, 11-deoxycorticosterone, and gonadotropin were elevated, whereas the levels of testosterone and dehydroepiandrosterone were reduced. Next-generation sequencing revealed one patient with compound heterozygosity for p.Trp17Ter (c.51G>A) and p.His373Leu (c.1118A>T), and the other with homozygosity for p.His373Leu (c.1118A>T). This report further describes 2 cases of 17α-hydroxylase/17,20-lyase deficiency in patients who harbored a p.His373Leu substitution, commonly found in Korean individuals, and presented diverse phenotypes.
ABSTRACT
X-linked adrenal hypoplasia congenita caused by a mutation in NR0B1/DAX-1 is a rare inherited disorder. Patients with adrenal hypoplasia congenita are usually diagnosed with primary adrenal insufficiency in infancy or early childhood and present hypogonadotropic hypogonadism during adolescence. Our patient first presented with adrenal crisis at the age of 2 months, which was managed with glucocorticoids and mineralocorticoids. At the age of 17 years, testicular volumes of 5 mL each and a stretched penile length of 4 cm were noted. A combined pituitary function test showed a peak luteinizing hormone level of 2.68 mIU/mL, testosterone 13.5 ng/dL, confirming hypogonadotropic hypogonadism. After whole-exome sequencing, a new variant of DAX-1, c.881T>C (p.Leu294Pro), was found. He was diagnosed with X-linked adrenal hypoplasia congenita and then treated with human choriogonadotropin for the induction of spermatogenesis as well as with steroid replacement therapy.
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Background@#Post-transplant immunosuppression with calcineurin inhibitors (CNIs) is associated with kidney function impairment while mammalian target of rapamycin (mTOR) inhibitors, such as everolimus, can be used for its renal-sparing effects. In this study, we compared the efficacy and safety of everolimus with low dose tacrolimus (EVR+Low TAC) and conventional dose tacrolimus (TAC) in liver transplantation recipients. @*Methods@#Medical records of recipients who received liver transplantation at Seoul National University Bundang Hospital from January 1st 2009 to December 31st 2018 were retrospectively reviewed. Cohort entry date was defined as the day everolimus was initiated and tacrolimus dosage was reduced. All patients were followed up for 1 year. Indicator of efficacy was the incidence of rejection and safety was evaluated by incidence of drug adverse events including renal function. @*Results@#Among 118 patients, there were 40 patients (33.9%) in EVR+Low TAC group. Incidence of rejection, including both biopsy proven acute rejection and clinical rejection, was similar in two groups [7.5% (n=3) vs. 6.4% (n=5), p=1.000]. Renal dysfunction was less frequent in EVR+Low TAC [17.5% (n=7) vs. 35.9% (n=28), p=0.038]. However, incidence rates of dyslipidemia, oral ulcer were more frequent in EVR+Low TAC [45.0% (n=18) vs. 21.8% (n=17), p=0.009; 15.0% (n=6) vs. 1.3% (n=1), p=0.006]. @*Conclusions@#In terms of prevention of rejection, EVR+Low TAC was as effective as TAC and had renal-sparing effect but was associated with increased risk of dyslipidemia and oral ulcer. This study demonstrates that EVR+Low TAC could be an alternative to liver transplant recipients with nephrotoxicity after administration of conventional dose tacrolimus.
ABSTRACT
As dyslipidemia at a young age is a risk factor for cardiovascular disease in adulthood, the screening and management of dyslipidemia in children and adolescents might be an important health issue. This review deals with issues related to the prevalence, diagnosis, screening, and treatment of pediatric dyslipidemia.Current Concepts: In Korea, the prevalence of pediatric dyslipidemia was 19.7% between 2007 and 2009. Dyslipidemia was defined according to the guidelines of the Korean Society of Pediatric Endocrinology: total cholesterol ≥200 mg/dL, low-density lipoprotein cholesterol ≥130 mg/dL, triglycerides ≥130 mg/dL, high-density lipoprotein cholesterol <40 mg/dL, or non-high-density lipoprotein cholesterol ≥145 mg/dL.Discussion and Conclusion: We recommend universal screening tests for dyslipidemia at ages 9–11 years and 17–21 years. Diet and lifestyle modifications are essential in the treatment of dyslipidemia. In children aged ≥10 years with a poor response to lifestyle modification, drug therapy is recommended. Pediatric dyslipidemia should be diagnosed and treated properly to reduce adult cardiovascular diseases and improve quality of life in this age group.
ABSTRACT
Although doctors expend their best effort in treating their patients’ illnesses or injuries, the patients may retain some disabilities even after treatment. Accordingly, some conflicts occur between the patients’ expectations of financial compensation for their disabilities and the financial manager’s policy to efficiently distribute limited resources. The mediation of these social conflicts requires the determination of the degree of physical disability, which can be done by doctors alone. Hence, a reliable disability evaluation guideline should be established. However, currently, only a few educational programs on disability evaluation are available for doctors and there is no reliable guideline for disability evaluation.Current Concepts: There were attempts to implement the American Medical Association guideline in Korea; however, it is currently not being used efficiently because it is quite complicated and unsuited to Korean settings. Mcbride’s disability evaluation, published 60 years ago, is being used, but it is not realistic and unreasonable in Korea. To prepare a standard guideline for disability evaluation, the Korean Academy of Medical Sciences published a booklet, “Disability Evaluation Guideline: Explanation and Case Studies”, based on the American Medical Association guideline. The academy published the booklet’s revised version (2nd edition), ‘Disability evaluation guideline and utilization’, in 2016. However, Korean Academy of Medical Sciences guideline still not being used because it is too complicated to use.Discussion and Conclusion: Fair disability evaluation is a social responsibility given to doctors, and there should be a useful guideline for disability evaluation that reflects the characteristics of each medical society or association.
ABSTRACT
17α-hydroxylase/17,20-lyase deficiency, caused by mutations in the cytochrome P450 family 17 subfamily A member 1 gene (CYP17A1), is an extremely rare form of congenital adrenal hyperplasia that is characterized by diverse phenotypes resulting from specific mutations. Here, we report 2 phenotypic females with 17α-hydroxylase/17,20-lyase deficiency: one with the 46,XX karyotype presenting primary amenorrhea and sexual infantilism, and the other with the 46,XY karyotype presenting a disorder of sexual development. In both cases, the serum levels of adrenocorticotropic hormone, 11-deoxycorticosterone, and gonadotropin were elevated, whereas the levels of testosterone and dehydroepiandrosterone were reduced. Next-generation sequencing revealed one patient with compound heterozygosity for p.Trp17Ter (c.51G>A) and p.His373Leu (c.1118A>T), and the other with homozygosity for p.His373Leu (c.1118A>T). This report further describes 2 cases of 17α-hydroxylase/17,20-lyase deficiency in patients who harbored a p.His373Leu substitution, commonly found in Korean individuals, and presented diverse phenotypes.
ABSTRACT
X-linked adrenal hypoplasia congenita caused by a mutation in NR0B1/DAX-1 is a rare inherited disorder. Patients with adrenal hypoplasia congenita are usually diagnosed with primary adrenal insufficiency in infancy or early childhood and present hypogonadotropic hypogonadism during adolescence. Our patient first presented with adrenal crisis at the age of 2 months, which was managed with glucocorticoids and mineralocorticoids. At the age of 17 years, testicular volumes of 5 mL each and a stretched penile length of 4 cm were noted. A combined pituitary function test showed a peak luteinizing hormone level of 2.68 mIU/mL, testosterone 13.5 ng/dL, confirming hypogonadotropic hypogonadism. After whole-exome sequencing, a new variant of DAX-1, c.881T>C (p.Leu294Pro), was found. He was diagnosed with X-linked adrenal hypoplasia congenita and then treated with human choriogonadotropin for the induction of spermatogenesis as well as with steroid replacement therapy.
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Purpose@#The impact of conversion on perioperative and long-term oncologic outcomes is controversial. Thus, we compared these outcomes between laparoscopic (Lap), unplanned conversion (Conversion), and planned open (Open) liver resection for hepatocellular carcinoma (HCC) located in anterolateral (AL) liver segments and aimed to identify risk factors for unplanned conversion. @*Methods@#We retrospectively studied 374 patients (Lap, 299; Open, 62; Conversion, 13) who underwent liver resection for HCC located in AL segments between 2004 and 2018. @*Results@#Compared to the Lap group, the Conversion group showed greater values for operation time (p < 0.001), blood loss (p = 0.021), transfusion rate (p = 0.009), postoperative complication rate (p = 0.008), and hospital stay (p = 0.040), with a lower R0 resection rate (p < 0.001) and disease-free survival (p = 0.001). Compared with the Open group, the Conversion group had a longer operation time (p = 0.012) and greater blood loss (p = 0.024). Risk factors for unplanned conversion were large tumor size (odds ratio [OR], 1.35; 95% confidence interval [CI], 1.05–1.74; p = 0.020), multiple tumors (OR, 5.95; 95% CI, 1.45–24.39; p = 0.013), and other organ invasion (OR, 15.32; 95% CI, 1.80–130.59; p = 0.013). @*Conclusion@#In conclusion, patients who experienced unplanned conversion during LLR for HCC located in AL segments showed poor perioperative and long-term outcomes compared to those who underwent planned laparoscopic and open liver resection. Therefore, open liver resection should be considered in patients with risk factors for unplanned conversion.
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A notable secular trend in early puberty onset has been described over the past few decades. Also, the prevalence and incidence of precocious puberty is increasing not only in Korea, but also around the world. The manifestation of secondary sex characteristics before 8 years in girls and 9 years in boys is defined as precious puberty. The causes of precocious puberty can be classified as gonadotropin releasing hormone (GnRH)-dependent, also known as central precocious puberty (CPP), or GnRH-independent. Evaluation of patient with precocity requires detailed examination of the clinical manifestation, GnRH stimulation test, and imaging of the central nervous system if indicated. The standard treatment for CPP is GnRH agonist, which is beneficial for adequate pubertal development and preservation of final adult height. In this paper, we investigate the diagnosis and adequate treatment of CPP.