A 52-year-old woman with a meningioma was transferred to our hospital due to abdominal mass and hypoglycemic symptoms. Abdominal computed tomography confirmed thickening of the gallbladder wall, with a 10 x 7 cm mass occupying the right inferior and medial segment of the liver. Her fasting glucose was low on hypoglycemic attack, with a serum insulin level of 173 mIU/ml (normal; 5~20 mIU/ml). Other radiologic tests, including portal venous sampling, did not localize the insulin producing lesion in the pancreas. The preoperative diagnosis was one of a malignant gallbladder tumor infiltrating the liver, with the possibility of nesidioblastosis; therefore, extended cholecystectomy and distal pancreatectomy were performed as the frozen biopsy of the pancreas confirmed an increase of cells in the islet of Langerhans. In permanent specimens, the pancreas showed normal histology and the gallbladder lesion showed a neuroendocrine carcinoma combined with an adenocarcinoma, with positive immunostaining for CD56, chromogranin A and synaptophysin. The special immunostaining for insulin was positive. The patient was in a glucose intolerant state with administration of small dose of oral hypoglycemic agents after operation. Metastases of the lesion to the skull and spine were observed two months after surgery. We report non-islet cell insulin releasing neuroendocrine carcinoma, combined with an adenocarcinoma of the gallbladder, presenting with hypoglycemic symptoms
Adenocarcinoma ; Biopsy ; Carcinoma, Neuroendocrine* ; Cholecystectomy ; Chromogranin A ; Diagnosis ; Fasting ; Female ; Gallbladder* ; Glucose ; Humans ; Hypoglycemia ; Hypoglycemic Agents ; Insulin ; Liver ; Meningioma ; Middle Aged ; Neoplasm Metastasis ; Nesidioblastosis ; Pancreas ; Pancreatectomy ; Skull ; Spine ; Synaptophysin

PURPOSE: To evaluate the outcomes of a hybrid prophylactic strategy to prevent cytomegalovirus (CMV) disease in pediatric liver transplantation (LT) patients. METHODS: CMV DNAemia was regularly monitored by quantitative nucleic acid amplification test (QNAT) and was quantified in all children. CMV infection and disease were defined according to the International Consensus Guidelines. The hybrid strategy against CMV infection consisted of universal 3-week prophylaxis and preemptive treatment of intravenous ganciclovir regardless of the recipient's serostatus. RESULTS: A total of 143 children who underwent living donor LT were managed using the hybrid strategy. The overall incidence of CMV infection by QNAT was 48.3% (n=69/143). The highest CMV DNAemia positivity was observed in 49.2% (n=60/122) of children in the D+/R+ group, followed by 46.7% (n=7/15) in the D+/R− group. CMV disease was noted in 26.1% (n=18/69) patients. Forty-three (62.3%) children had undergone preemptive therapy consisting of intravenous ganciclovir. No symptomatic patients developed tissue-invasive disease, resulting in no CMV-associated mortality. CONCLUSION: The incidence of CMV infection was high in pediatric LT patients despite the hybrid strategy. However, tissue-invasive disease in pediatric LT did not occur.
Child ; Consensus ; Cytomegalovirus Infections* ; Cytomegalovirus* ; Ganciclovir ; Humans ; Incidence ; Liver Transplantation* ; Liver* ; Living Donors ; Mortality ; Nucleic Acid Amplification Techniques

PURPOSE: Barium enema is one of the diagnostic modalities for Hirschsprung'sdisease. The present study aimed to investigate the diagnostic accuracy of barium enema for Hirschsprung's disease, especially total colonic aganglionosis (TCA). METHODS: We retrospectively reviewed the medical records of all the patients who were diagnosed as having TCA and underwent a barium enema in Asan Medical Center Children's Hospital between January 1998 and December 2016. All the tests were performed and reviewed by pediatric radiologists. RESULTS: Among the total 19 patients with TCA who underwent barium enema, 9 patients (47.4%) had accurate radiographic results. Eight of the 13 neonate patients (61.5%) showed typical TCA radiological findings. However, only one of the 6 patients aged >4 weeks (16.7%) had accurate radiological diagnosis. CONCLUSION: Barium enema showed low accuracy for TCA, and its diagnostic performance was better in neonatal period than in those aged >4 weeks.
Barium ; Chungcheongnam-do ; Diagnosis ; Enema ; Hirschsprung Disease ; Humans ; Infant, Newborn ; Medical Records ; Retrospective Studies

PURPOSE: Barium enema is one of the diagnostic modalities for Hirschsprung'sdisease. The present study aimed to investigate the diagnostic accuracy of barium enema for Hirschsprung's disease, especially total colonic aganglionosis (TCA). METHODS: We retrospectively reviewed the medical records of all the patients who were diagnosed as having TCA and underwent a barium enema in Asan Medical Center Children's Hospital between January 1998 and December 2016. All the tests were performed and reviewed by pediatric radiologists. RESULTS: Among the total 19 patients with TCA who underwent barium enema, 9 patients (47.4%) had accurate radiographic results. Eight of the 13 neonate patients (61.5%) showed typical TCA radiological findings. However, only one of the 6 patients aged >4 weeks (16.7%) had accurate radiological diagnosis. CONCLUSION Barium enema showed low accuracy for TCA, and its diagnostic performance was better in neonatal period than in those aged >4 weeks.

Purpose: We analyzed the timing of inguinal hernia repair in premature infants in the neonatal intensive care unit (NICU) considering recurrence, incarceration, and other complications. Methods: In this multicenter retrospective review, premature infants (<37 weeks) in the NICU diagnosed with inguinal hernia between 2017 and 2021 were segregated into 2 groups based on the timing of inguinal hernia repair. Results: Of 149 patients, 109 (73.2%) underwent inguinal hernia repair in the NICU and 40 (26.8%) after discharge. Preoperative incarceration did not differ, but complications with recurrence and postoperative respiratory insufficiency were higher in the NICU group (11.0% vs. 0%, P = 0.029; 22.0% vs. 5.0%, P = 0.01). Multivariate analysis showed that the significant factors affecting recurrence were preoperative ventilator dependence and body weight of <3,000 g at the time of surgery (odds ratio [OR], 16.89; 95% confidence interval [CI], 3.45–82.69; P < 0.01 and OR, 9.97; 95% CI, 1.03–95.92; P = 0.04). Conclusion Our results suggest that when premature infants are diagnosed with inguinal hernia in the NICU, inguinal hernia repair after discharge may decrease the odds of recurrence and postoperative respiratory insufficiency. In patients who have difficulty delaying surgery, it is thought that surgery should be performed carefully in a ventilator preoperatively or weighed <3,000 g at the time of surgery.

Purpose: Sirolimus has emerged as a safe and effective treatment for complicated lymphatic malformations (LMs). We aim to prove the effectiveness and safety of sirolimus as a therapeutic option for patients with complicated LMs. Methods: Fifty-eight patients with complicated LMs treated with sirolimus for at least 6 months at multicenter between July 2018 and January 2023 were enrolled. All patients were administered oral sirolimus starting at 0.8 mg/m 2 every 12 hours, with target serum concentration levels of 8–15 ng/mL. Evaluation for clinical symptoms and LMs volume on MRI were reviewed to assess treatment response and toxicities. Evaluation of disease response was divided into 3 values:complete response, partial response (significant, moderate, and modest), and progressive disease. Results: The median age at the initiation of sirolimus treatment was 6.0 years (range, 1 month–26.7 years). The median duration of treatment was 2.0 years (range, 6 months–4.4 years). The most common lesions were head and neck (25 of 58, 43.1%). Forty-six patients (79.3%) demonstrated a reduction in LMs volume on MRI or improvement of clinical symptoms including 2 complete responses. The young age group and the patients who underwent few prior therapies showed better responses. None of the patients had toxicities attributable to sirolimus with a Common Terminology Criteria for Adverse Events grade of ≥3. Conclusion Oral sirolimus treatment brought a successful outcome without severe adverse effects. It could be the firstline therapy, especially for the young age group of complicated LMs, and an additional option for refractory lesions that did not respond to conventional treatment.