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Hepatocellular adenoma is an uncommon, benign liver tumor usually occurring in patients using estrogen or anabolic androgens and in those with a genetic disease, including glycogen storage disease. Hepatocellular adenomas can sometimes induce pain. However, it is usually asymptomatic. Moreover, few studies have reported cases of hepatocellular adenomas presenting with iron deficiency anemia. Herein, we report a pediatric case of a large hepatocellular adenoma, presenting with iron therapy- refractory iron deficiency anemia. A 14-year-old boy was diagnosed with hepatocellular adenoma during an anemia work-up. Improvement in iron deficiency anemia was observed after tumor resection.

Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder characterized by heterogenous lesions infiltrated with CD1a+/CD207+ cells. Although LCH has a relatively good prognosis, the prognosis for patients with LCH refractory to standard chemotherapy is poor. Neurodegenerative LCH (ND-LCH) is a central nervous system complication of LCH that is characterized by progressive radiological and clinical abnormalities. Symptomatic ND-LCH is difficult to treat and therefore has a poor prognosis. A two-year-old boy presented with a scalp mass. Biopsy confirmed LCH. Whole-body imaging revealed LCH involvement in multiple bones of the skull, facial bones, and lungs. Prednisolone and vinblastine chemotherapy was initiated. One-year post-treatment, most of the lesions in the bones and lung nodules disappeared, and chemotherapy was discontinued. New neurodegenerative lesions appeared 4 months after chemotherapy was discontinued. Second-line chemotherapy using cytarabine, vincristine, and prednisolone was initiated. However, neurological manifestations of ND-LCH worsened post second-line treatment, and the treatment was switched to cytarabine and cladribine. Despite third-line chemotherapy, the lesions progressed, and neurological deficits worsened. After identifying BRAF V600E mutation in the tumor tissue using next-generation sequencing, cytotoxic chemotherapy was discontinued and vemurafenib treatment was initiated. One-year post-vemurafenib therapy, ND-LCH manifestations regressed, and the patient experienced neurological improvement.

Purpose We aimed to determine the current application and survival trends of hematopoietic stem cell transplantation (HSCT) among Korean children and adolescents with cancer. Materials and Methods Data of patients aged < 20 years with KCD-10 (Korean Classifications of Diseases, 10th revision) C codes and specific designation codes were collected from the National Health Insurance Service database. Thirty claim codes for HSCT were included, and data from 2009 to 2019 were analyzed. Results The operational definition of pediatric cancer yielded an annual average of 2,000, with annual cases decreasing. In 2019, 221 HSCTs were performed, a decrease from the ten-year average of 276. Allografts outnumbered autografts with a ratio of 1.5:1. The source of allograft was bone marrow in 15% of patients in 2009; however, it substantially decreased to 3.3% in 2019. Furthermore, 70.5% of allogeneic HSCT used peripheral blood stem cell (PBSC) grafts, which increased to 89.3% by 2015. Cord blood utilization markedly decreased to 2.7% in 2018. The 5-year overall survival (OS) rate of all patients was 85.1%. Overall mortality decreased among patients who underwent recent HSCT, and they exhibited a higher 5-year OS rate. Conclusion In Korea, the number of pediatric patients with cancer is declining; however, the ratio of transplants to all patients remains constant. Patients who recently underwent transplantation showed better survival rates, possibly due to HSCT optimization. Korea showed a substantially greater PBSC utilization in pediatric HSCT. An in-depth examination encompassing donor relations and cause of death with a prospective registry is required in future studies. [ABSTRACT FROM AUTHOR]

Sedation plays a crucial role in successful pediatric imaging, and chloral hydrate is commonly used for this purpose. However, the challenges associated with chloral hydrate administration, such as its unpleasant taste and potential induction of vomiting, remain a concern. Sweet oral solutions have emerged as potential solutions for reducing distress and providing analgesia. This study compared the efficacy of dextrose combined with chloral hydrate with that of conventional sedation methods. This prospective, double-blind, randomized controlled clinical study enrolled 160 pediatric outpatients scheduled for echocardiography. Chloral hydrate syrup (100 mg/mL) was supplemented with a dextrose solution (dextrose group) or distilled water (control group) in a 1:10 volume ratio. The sedation achievement time, Skeie scale score, revised Face, Legs, Activity, Cry, and Consolability (FLACC) score, and side effects (nausea, vomiting, hypoxia, and respiratory depression) were assessed. No significant difference in average time to achieve sedation was observed between the dextrose and control groups (24.4±17.8 vs. 24.7±17.1 min, p=0.92). Both groups demonstrated similar levels of sedation according to the Skeie scale and mean revised FLACC score. Although the occurrence rates of nausea and vomiting had no significant differences, the dextrose group had no cases of vomiting in children aged >24 months compared to the control group, which had three cases (30%). In conclusion, the addition of dextrose to chloral hydrate did not significantly affect sedation time, anxiety, pain reduction, or occurrence of gastrointestinal complications during sedation. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to evaluate the utility of acanthosis nigricans (AN) severity as an index for predicting insulin resistance in obese children. Methods: The subjects comprised 74 obese pediatric patients who attended the Department of Pediatrics at Chosun University Hospital between January 2013 and March 2016. Waist circumference; body mass index; blood pressure; fasting glucose and fasting insulin levels; lipid profile; aspartate transaminase, alanine transaminase, glycated hemoglobin, C-peptide, and uric acid levels; and homeostatic model assessment insulin resistance (HOMA-IR) and quantitative insulin check sensitivity index (QUICKI) scores were compared between subjects with AN and those without AN. Receiver operating characteristic curves were used to investigate the utility of the AN score in predicting insulin resistance. HOMA-IR and QUICKI were compared according to AN severity. Results: The With AN group had higher fasting insulin levels (24.1±21.0 mU/L vs. 9.8±3.6 mU/L, p<0.001) and HOMA-IR score (5.74±4.71 vs. 2.14±0.86, p<0.001) than the Without AN group. The AN score used to predict insulin resistance was 3 points or more (sensitivity 56.8%, specificity 83.9%). HOMA-IR scores increased with AN severity, from the Without AN group (mean, 2.15; 95% confidence interval [CI], 1.72-2.57) to the Mild AN (mean, 4.15; 95% CI, 3.04-5.25) and Severe AN groups (mean, 7.22; 95% CI, 5.08-9.35; p<0.001). Conclusion: Insulin resistance worsens with increasing AN severity, and patients with Severe AN (AN score =3) are at increased risk of insulin resistance. [ABSTRACT FROM AUTHOR]

Neurocutaneous melanosis (NCM) is a rare nonheritable congenital phakomatosis. NCM is characterized by large or multiple (>3) congenital nevi that are associated with benign and/or malignant melanocytic tumors of the leptomeninges. Approximately 10% of symptomatic patients with NCM have associated Dandy Walker complex (DWC), which seems to have an extremely poor prognosis. We report the case of a 7 year-old patient with multiple congenital NCM associated with DWC, who experienced a transient improvement in neurological symptoms including mental status and hemiparesis after intravenous immunoglobulin (IVIG) therapy. We suggest that the administration of IVIG may play a role in the treatment of symptomatic NCM. [ABSTRACT FROM AUTHOR]