Purpose: Although surgical management of Hirschsprung disease (HD) is effective in most patients, some patients experience long-term postoperative complications, and require redo pull-through (PT). The present study evaluated clinical outcomes of redo PT in HD patients at a single center. Methods: Patients with HD who underwent redo PT procedures between 2003 and 2019 were retrospectively reviewed. Results: Thirteen patients were included. Five (38.5%) had undergone initial PT surgery at our center and 8 (61.5%) at other centers. Redo PT procedures were transanal endorectal PT in 12 patients (92.3%) and the posterior sagittal approach in 1 patient (7.7%). Indications for redo PT included pathologic misdiagnosis in 8 patients (61.5%); stricture in 2 (15.4%); and rectal stenosis, obstructing Duhamel pouch and remnant septum in 1 each (7.7%). At a median follow-up of 68 months (range, 3–227 months) after redo PT, 8 patients (61.5%) had normal bowel function, 2 (15.4%) had incontinence, and 1 (7.7%) had constipation. Conclusion Redo PT procedures could be an effective approach for improving obstructive symptoms in HD patients with anatomic or pathologic reasons following primary PT. Careful selection of patients and discreet indications for redo PT are crucial.

Purpose: Hiatal hernia in children is a rare condition, and there is limited knowledge available about the disease itself. There is currently no agreement or consensus on the treatment of hiatal hernia in the pediatric population due to lack of evidence. In this study, we were to assess our experience with hiatal hernia, including the characteristics of our patients, surgical outcomes, and factors that influence the outcomes. Methods: We retrospectively reviewed the medical records of 49 patients below the age of 18 years who underwent hiatal hernia repair at the Asan Medical Center between 2006 and 2021. We analyzed and compared the general characteristics and surgical outcomes based on the presence of recurrence and coexisting congenital diseases. Results: Hiatal hernia progression was found to be associated with various medical conditions; however, no significant differences in patient characteristics or surgical outcomes between those with and without comorbidities were observed.There were no significant differences in patient characteristics or outcomes between the initial and redo operations. Fundoplication was performed in 19 patients (36.7%) during the initial operation and in 7 patients (87.5%) during repeat hiatal hernia repair. Conclusion The presence of an underlying disease didn’t influence the treatment and progression of hiatal hernia. Furthermore, there were no significant differences in the clinical course between patients with recurrent hiatal hernia and those experiencing it for the first time. Additionally, the impact of fundoplication on the recurrence of hiatal hernia in pediatric patients was found to be minimal.

Purpose: Autologous adipose tissue-derived stem cells (ASCs) have been proposed for patients with refractory Crohn disease, but research is lacking in pediatric patients. This Phase IV study evaluated the efficacy and safety of ASCs in children with refractory Crohn’s fistulae. Methods: Patients with a refractory Crohn’s fistula who did not have conventional therapy for more than 3 months or with a recurrent complex Crohn’s fistula were included. All patients were at least 14 years old. Patients with infection, poor condition, or active Crohn disease with a disease activity index of 450 and above were excluded. Five patients were treated with ASCs from 2014 to 2015 in Asan Medical Center. ASC administration was adjusted according to fistula size (1 mL per cm 2 ). We evaluated the efficacy and safety 8 weeks after injection and followed patients for 6 months. Results: Fistulae were healed in 4 patients by 8 weeks after ASC injection. Of these 4 patients, 1 had complete fistula closure and sustainability after 6 months. The other 3 with healing effects had less than 50% fistula closure by 6 months.None of these 4 patients have persistent fistulae. One patient had no healing effect, and seton ligation was performed 8 months after ASC injection. There were no adverse effects related to ASC administration. Conclusion ASC therapy is a simple and well-tolerated therapeutic option for children with refractory Crohn’s complex fistulae. Complete closure was well-sustained. However, more data from a larger number of patients are needed.

Purpose: Autologous adipose tissue-derived stem cells (ASCs) have been proposed for patients with refractory Crohn disease, but research is lacking in pediatric patients. This Phase IV study evaluated the efficacy and safety of ASCs in children with refractory Crohn’s fistulae. Methods: Patients with a refractory Crohn’s fistula who did not have conventional therapy for more than 3 months or with a recurrent complex Crohn’s fistula were included. All patients were at least 14 years old. Patients with infection, poor condition, or active Crohn disease with a disease activity index of 450 and above were excluded. Five patients were treated with ASCs from 2014 to 2015 in Asan Medical Center. ASC administration was adjusted according to fistula size (1 mL per cm 2 ). We evaluated the efficacy and safety 8 weeks after injection and followed patients for 6 months. Results: Fistulae were healed in 4 patients by 8 weeks after ASC injection. Of these 4 patients, 1 had complete fistula closure and sustainability after 6 months. The other 3 with healing effects had less than 50% fistula closure by 6 months.None of these 4 patients have persistent fistulae. One patient had no healing effect, and seton ligation was performed 8 months after ASC injection. There were no adverse effects related to ASC administration. Conclusion ASC therapy is a simple and well-tolerated therapeutic option for children with refractory Crohn’s complex fistulae. Complete closure was well-sustained. However, more data from a larger number of patients are needed.

Purpose: Intrahepatic biliary cysts (IBCs) after Kasai portoenterostomy (KPE) are associated with intractable recurrent cholangitis. This study aimed to investigate the feasibility of its use as well as indication for surgical management of IBCs in pediatric patients. Methods: We retrospectively reviewed the medical records and imaging studies of patients who underwent KPE for biliary atresia from 2010 to 2020. Results: An imaging study identified IBCs in 28 of 129 patients who underwent KPE with biliary atresia (21.7%). Among them, 5 patients were subjected to surgical treatment for intractable cholangitis. The median time from KPE to the development of IBCs was 1.7 years. Four out of 5 patients had IBCs confined to the left lateral lobe, and in one patient, the IBCs were in the hepatic hilum. All 5 patients experienced more than one cholangitis. Although they received intravenous antibiotic treatment and percutaneous transhepatic cholangiodrainage as treatment, they were intractable. Three patients underwent hepatectomy, and 2 underwent cystojejunostomy. There was no recurrence of cholangitis during the median follow-up period of 2.9 years. Conclusion Surgical treatment for IBCs after KPE could be considered a safe and effective surgical procedure for children if appropriate indications are applied.

Purpose: Intrahepatic biliary cysts (IBCs) after Kasai portoenterostomy (KPE) are associated with intractable recurrent cholangitis. This study aimed to investigate the feasibility of its use as well as indication for surgical management of IBCs in pediatric patients. Methods: We retrospectively reviewed the medical records and imaging studies of patients who underwent KPE for biliary atresia from 2010 to 2020. Results: An imaging study identified IBCs in 28 of 129 patients who underwent KPE with biliary atresia (21.7%). Among them, 5 patients were subjected to surgical treatment for intractable cholangitis. The median time from KPE to the development of IBCs was 1.7 years. Four out of 5 patients had IBCs confined to the left lateral lobe, and in one patient, the IBCs were in the hepatic hilum. All 5 patients experienced more than one cholangitis. Although they received intravenous antibiotic treatment and percutaneous transhepatic cholangiodrainage as treatment, they were intractable. Three patients underwent hepatectomy, and 2 underwent cystojejunostomy. There was no recurrence of cholangitis during the median follow-up period of 2.9 years. Conclusion Surgical treatment for IBCs after KPE could be considered a safe and effective surgical procedure for children if appropriate indications are applied.

Purpose: Intrahepatic biliary cysts (IBCs) after Kasai portoenterostomy (KPE) are associated with intractable recurrent cholangitis. This study aimed to investigate the feasibility of its use as well as indication for surgical management of IBCs in pediatric patients. Methods: We retrospectively reviewed the medical records and imaging studies of patients who underwent KPE for biliary atresia from 2010 to 2020. Results: An imaging study identified IBCs in 28 of 129 patients who underwent KPE with biliary atresia (21.7%). Among them, 5 patients were subjected to surgical treatment for intractable cholangitis. The median time from KPE to the development of IBCs was 1.7 years. Four out of 5 patients had IBCs confined to the left lateral lobe, and in one patient, the IBCs were in the hepatic hilum. All 5 patients experienced more than one cholangitis. Although they received intravenous antibiotic treatment and percutaneous transhepatic cholangiodrainage as treatment, they were intractable. Three patients underwent hepatectomy, and 2 underwent cystojejunostomy. There was no recurrence of cholangitis during the median follow-up period of 2.9 years. Conclusion Surgical treatment for IBCs after KPE could be considered a safe and effective surgical procedure for children if appropriate indications are applied.

PURPOSE: Spontaneous neonatal gastric perforation is a rare but fatal disease with unclear etiology. In this study, we reviewed its clinical manifestations, outcomes, and discussed the etiology and prognostic factors. METHODS: There were 12 neonates with gastric perforation in our hospital from 1989 to 2015. Their medical records were reviewed retrospectively including birth record, associated disease, site and size of perforation, type of surgical management, clinical outcome. Also, the prognostic factors were analyzed. RESULTS: The median gestational age and birth weight was 32 weeks (range, 26-43 weeks; preterm birth rate, 66.7%) and 1,883 g (range, 470-4,400 g), respectively. Five patients had associated gastrointestinal anomalies including esophageal atresia and tracheoesophageal fistula (two patients), midgut volvulus, non-rotation and microcolon, and meconium plug syndrome. The median age at surgery was six days after birth (range, 2-13 days), and the median weight at surgery was 1,620 g (range, 510-3,240 g). Upper third part of stomach was the most frequently involved location of perforation. The size of perforation varied from pin point to involving the whole greater curvature. Primary repairs were done in seven cases, and in five cases, resections of necrotic portion were needed. Mortality rate was 33.3% (n=4), morbidity (re-operation) rate was 16.7% (n=2). The causes of death were sepsis (n=3), and heart failure from Ebstein anomaly (n=1). The median hospital stay was 92.5 days (range, 1-176 days). The factors mentioned as prognostic factors in previous studies showed no significant relations to the mortality and morbidity in our study. CONCLUSION: There were improvements of outcomes in patients with large size perforation. As previous studies, we assume these improvements were possible due to the improvements of critical care medicine. Given that rare incidence, a multi-center study can help us get a better understanding of this disease, and a better outcome.
Birth Certificates ; Birth Weight ; Cause of Death ; Critical Care ; Ebstein Anomaly ; Esophageal Atresia ; Gestational Age ; Heart Failure ; Humans ; Incidence ; Infant, Newborn ; Intestinal Volvulus ; Length of Stay ; Meconium ; Medical Records ; Mortality ; Parturition ; Premature Birth ; Retrospective Studies ; Sepsis ; Stomach ; Tracheoesophageal Fistula

Thelephoric acid is an antioxidant produced by the hydrolysis of polyozellin, which is isolated from Polyozellus multiplex. In the present study, the inhibitory effects of polyozellin and thelephoric acid on 9 cytochrome P450 (CYP) family members (CYP1A2, CYP2A6, CYP2B6, CYP2C8, CYP2C9, CYP2C19, CYP2D6, CYP2E1, and CYP3A4) were examined in pooled human liver microsomes (HLMs) using a cocktail probe assay. Polyozellin exhibited weak inhibitory effects on the activities of all 9 CYPs examined, whereas thelephoric acid exhibited dose- and time-dependent inhibition of all 9 CYP isoforms (IC50 values, 3.2-33.7 muM). Dixon plots of CYP inhibition indicated that thelephoric acid was a competitive inhibitor of CYP1A2 and CYP3A4. In contrast, thelephoric acid was a noncompetitive inhibitor of CYP2D6. Our findings indicate that thelephoric acid may be a novel, non-specific CYP inhibitor, suggesting that it could replace SKF-525A in inhibitory studies designed to investigate the effects of CYP enzymes on the metabolism of given compounds.
Cytochrome P-450 CYP1A2 ; Cytochrome P-450 CYP2D6 ; Cytochrome P-450 CYP2E1 ; Cytochrome P-450 Enzyme System* ; Humans ; Hydrolysis ; Metabolism ; Microsomes, Liver ; Proadifen* ; Protein Isoforms

Coronary artery involvement leading to acute coronary syndrome is a rare complication of essential thrombocythemia. A 43-year-old woman with essential thrombocythemia complained of severe acute chest pain. She had undergone percutaneous coronary intervention (PCI) with a drug-eluting stent (DES), due to unstable angina, 3 and 2 years earlier. Emergency coronary angiography revealed total occlusion of the DES with thrombus. Twenty minutes after successful primary PCI with a DES, an acute stent thrombosis developed. She was subsequently treated with coronary artery bypass graft surgery.
Acute Coronary Syndrome ; Adult ; Angina, Unstable ; Chest Pain ; Coronary Angiography ; Coronary Artery Bypass ; Coronary Vessels ; Drug-Eluting Stents ; Emergencies ; Female ; Humans ; Percutaneous Coronary Intervention ; Stents ; Thrombocythemia, Essential ; Thrombosis ; Transplants