Gonadotropin-releasing hormone agonist (GnRHa) is a safe and effective therapy used to treat central precocious puberty (CPP). Although most studies have reported no significant difference in body mass index (BMI) in girls during and after GnRHa therapy, few studies have investigated changes in BMI in boys with CPP. This study evaluated the effects of GnRHa therapy on BMI in boys with CPP.

Objective Gonadotropin-releasing hormone agonist (GnRHa) treatment improves the potential for gaining height in patients with central precocious puberty (CPP). However, most studies have focused on girls because CPP in boys is relatively rare. Therefore, we aimed to determine the effect of GnRHa treatment on auxological outcomes in boys with CPP. Methods Eighty-five boys with CPP were treated with leuprolide or triptorelin acetate 3.75 mg over 2 years. Anthropometry, bone age, sexual maturity rating, and predicted adult height (PAH) were assessed every 6 months. Furthermore, 20 boys were followed up after treatment discontinuation until achievement of the final adult height (FAH). Results The mean chronological age (CA) and bone age (BA) of the patients with CPP at treatment initiation were 9.5 ± 0.5 years and 11.7 ± 0.9 years, respectively. The mean duration of treatment was 2.87 ± 0.63 years. The PAH at treatment initiation was 172.1 cm (-0.23 ± 1.05 PAH standard deviation score). The PAH at treatment discontinuation (176.2 ± 6.6 cm) was significantly higher than the pretreatment PAH. In addition, the mean final adult height in the 20 boys who were followed up after discontinuation of treatment was 173.4 ± 5.8 cm, which was significantly higher than the initial PAH (170.1 ± 4.5 cm; p = 0.006). In multivariate analysis, the height gain (the difference between the FAH and PAH at treatment initiation) significantly correlated with the target height. Conclusion Long-term GnRHa treatment significantly improved the growth potential and FAH in boys with CPP.

Maturity-onset diabetes of the young (MODY) is a rare, autosomal dominant disease characterized by non-ketogenic diabetes mellitus (DM). MODY type 4, caused by <i>PDX1</i> mutation, is a very rare subtype of MODY, especially in Korea. We report a case of a 10-year-old, nonobese girl with a family history of type 2 DM. After diagnosis, the patient’s serum glucose level was well controlled using metformin monotherapy; however, the glycated hemoglobin level increased to 9.0% approximately 2 years after treatment. No obesity or lifestyle problems were observed, and serum fasting C-peptide level was within the normal range. Furthermore, no islet-related autoantibodies were detected. A genetic screening for MODY using a next-generation sequencing panel was performed, and a likely heterozygous pathogenic <i>PDX1</i> mutation (p.Gly246ArgfsTer21) was identified. The <i>PDX1</i> variant was not detected in her mother, implying that the mutation had arisen <i>de novo</i> in the proband. She was prescribed insulin degludec in addition to metformin therapy, which improved her hyperglycemia. This report presents a novel MODY type 4 phenotype and highlights the importance of genetic screening in patients with MODY characteristics.

We report the case of a 5-year-old male patient with multiple aseptic splenic abscesses associated with Behçet's disease. The patient visited Gachon University Gil Hospital with fever, abdominal pain, and acute watery and bloody diarrhea, and reported a 2-year history of chronic abdominal pain and intermittent watery diarrhea. He was treated with antibiotics at a local clinic for fever and cervical lymph node swelling. Additionally, he had recurrent stomatitis. A colonoscopy showed multiple well-demarcated ulcerations throughout the colon, and abdominal computed tomography showed multiple splenic abscesses. Pathergy and HLA-B51 tests were positive. Investigations did not reveal any infectious organisms in the aspirate obtained via ultrasound-guided fine needle aspiration. After steroid treatment, all symptoms and multiple aseptic splenic abscesses resolved. However, oral ulcers, genital ulcers, and abdominal pain recurred after tapering the steroids. Infliximab treatment improved the patient's symptoms. However, 5 months after the treatment, the symptoms recurred. The treatment was changed to include adalimumab. Subsequently, the patient's symptoms resolved and colonoscopic findings improved. No recurrence was noted after 3 months of follow-up.

PurposeDespite the increasing incidence of pediatric Crohn disease (CD) in Korea, data on the characteristics of perianal lesions are scarce. Therefore, we aimed to investigate the characteristics of pediatric CD with accompanying perianal lesions in Korea.MethodsWe retrospectively reviewed the medical records of children (age ≤18 years) with confirmed CD at Gachon University Gil Medical Center between 2000 and 2014. Patients were classified into two groups based on the presence or absence of any perianal lesions including skin tags. Additional analysis was performed according to the presence or absence of perianal perforating lesions.ResultsAmong the 69 CD children (mean age, 15.4 years) include in the analysis, 54 (78.3%) had a perianal lesion and 29 (42.0%) had a perianal perforating lesion. The median duration of chief complaints was longer in pediatric CD with any accompanying perianal lesions (5.40 months vs. 1.89 months, p=0.02), while there was no difference between pediatric CD with and without perianal perforating lesions (5.48 months vs. 4.02 months, p=0.18). Perianal symptoms preceded gastrointestinal symptoms in 13 of 29 (44.8%) patients with perianal perforating lesions.ConclusionsCD should be suspected in children with perianal lesions, even in circumstances when gastrointestinal symptoms are absent.