PURPOSE: This study aimed to assess the long-term clinical outcome of laparoscopic Nissen fundoplication (LNF) in children according to their neurologic status. METHODS: The study retrospectively analyzed the data of 82 children (62 neurologically impaired and 20 neurologically normal children with primary gastroesophageal reflux disease) who had undergone LNF between 2003 and 2012. The main outcome measures were the occurrence of recurrence that required reoperation and post-procedure complications such as infections, pneumonia, and gastrointestinal complications including ileus, dysphagia, and delayed gastric emptying. RESULTS: The median age at the time of the LNF was 25 months (range, 1-192 months), and the median of body weight was 10.0 kg (range, 2.8-37.0 kg). The average weight gain was 1.55±1.68 kg at 6 months, 3.32±2.30 kg at 1 year, and 5.63±4.22 kg at 2 years after surgery. Six (9.7%) of the 62 neurologically impaired patients and two (10.0%) of neurologically normal lost their body weight or had no weight changes. Eight (12.9%) of the 62 neurologically impaired children had required redo surgery because of gastroesophageal reflux disease recurrences, while 2 (10.0%) of the 20 neurologically normal children had experienced recurrences. In the neurologically impaired children, the postoperative complications included pneumonia (n=1), wound infection (n=1), urinary tract infection (n=1), dysphagia (n=1), delayed gastric emptying (n=1), and ileus (n=2). All of these complications were not found in the neurologically normal group, except for only one case of infectious colitis. However, there was no statistically significant difference between the two groups in postoperative complications. CONCLUSION: The outcomes of laparoscopic fundoplication were similar in the neurologically impaired children and in the neurologically normal children.
Body Weight ; Child* ; Colitis ; Deglutition Disorders ; Fundoplication* ; Gastric Emptying ; Gastroesophageal Reflux ; Humans ; Ileus ; Laparoscopy ; Neurologic Manifestations ; Outcome Assessment (Health Care) ; Pneumonia ; Postoperative Complications ; Recurrence ; Reoperation ; Retrospective Studies ; Urinary Tract Infections ; Weight Gain ; Wound Infection

PURPOSE: The single stage transanal pull-through (SSPT) for Hirschsprung’s disease is becoming the most popular procedure. This single center study compared the result of single stage operation with two-stage operation for Hirschsprung’s disease in neonates. METHODS: We retrospectively reviewed medical records of all patients who were diagnosed as Hirschsprung’s disease and underwent SSPT or two-stage operation operation in Asan Medical Center between January 2003 and July 2014. RESULTS: There were 17 SSPT and 28 two-stage operation. The mean age of SSPT group was 14.2±7.1 days, and the mean age of two-stage operation group was 15.4±8.6 days for stomy formation, and 188.6±36.3 days for Duhamel operation. The operation time of SSPT was shorter than Duhamel operation (145.0±37.0 minutes vs. 193.0±36.0 minutes, p<0.001). The mean follow-up period of SSPT and two-stage operation was 35.5±34.9 months (range, 2-132 months) and 56.6±35.5 months (range, 1-121 months), respectively. Defecation problem rate such as fecal soiling or fecal impaction showed no significant difference between the two groups (p=0.719). Two SSPT patients required botulinum toxin injection due to rectal stenosis. Three patients of SSPT group underwent re-do endorectal pull-through due to remnant aganglionic or hypoganglionic bowel. CONCLUSION: The SSPT showed shorter hospital days. However, few patients experienced rectal stenosis, but were manageable with botulinum toxin injection. The SSPT requires experienced-pathologist, as well as surgeon, because intra-operation pathology reading is critical for appropriate SSPT. SSPT is a feasible and reasonable option to treat Hirschsprung’s disease.
Botulinum Toxins ; Chungcheongnam-do ; Constriction, Pathologic ; Defecation ; Fecal Impaction ; Follow-Up Studies ; Hirschsprung Disease ; Humans ; Infant, Newborn* ; Medical Records ; Pathology ; Retrospective Studies ; Soil

Congenital fiber type disproportion (CFTD) has been related with mutations in ACTA1, SEPN1, RYR1 and tropomyosin 3 (TPM3) genes. Particularly, TPM3 mutation was identified as one of the most frequent cause of CFTD and was also detected in cap myopathy and nemaline myopathy. Herein we report patients of autosomal dominant TPM3 missense mutations with CFTD in a Korean family over twogenerations. Two of our patients, who developed mild muscle weakness in infancy, presented with altered mentality and respiratory distress despite relatively mild limb weakness.
Extremities ; Humans ; Muscle Weakness ; Muscular Diseases ; Mutation, Missense ; Myopathies, Nemaline ; Myopathies, Structural, Congenital ; Respiratory Insufficiency ; Ryanodine Receptor Calcium Release Channel ; Tropomyosin

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

BACKGROUND: Despite recent improvements in survival outcome after ABO incompatible (ABOi) adult living donor liver transplantation (ALDLT), concerns about the incidence of biliary stricture (BS) still exist. However, reports on the actual incidence of BS have been scarce. METHODS: From November 2008 to August 2011, 77 cases of ABOi ALDLTs have been performed. We compared patient and graft survival and BS-free survival rates (BSFSR) between these ABOi ALDLTs and 734 ABO compatible (ABOc) ALDLTs performed during the same period. We also analyzed characteristics of BS in ABOi ALDLT. RESULTS: There was one mortality (1.3%) and one re-transplantation (due to small-for-size graft syndrome) among 77 cases of ABOi ALDLTs. Overall, 1-, 2-, and 3-year patient survival rates were 94.8%, comparable to ABOc ALDLTs (93.7%, 90.1%, 90.1%, P=0.20). BS occurred in 11 (13.8%) ABOi ALDLT patients. There were no significant differences in 1-, 2-, and 3-year BSFSR between ABOi and ABOc ALDLT patients (87.5% vs. 88.1%, 83.4% vs. 87.5%, and 83.4% vs. 86.4%, P=0.55). Among 10 patients with BS, four patients showed diffuse multiple intrahepatic strictures, which were linked to the death of two patients. CONCLUSIONS: The survival outcome of ABOi ALDLT is comparable to ABOc ALDLT. The incidence of BS of ABOi ALDLT was not superior to that of ABOc ALDLT. However, ABO incompatibility is related to the development of diffuse multiple intrahepatic BSs (rarely seen in ABOc ALDLT) and can cause graft failure and patient death.
Adult ; Blood Group Incompatibility ; Constriction, Pathologic ; Graft Survival ; Humans ; Incidence ; Liver ; Liver Transplantation ; Living Donors ; Survival Rate ; Transplants

BACKGROUND: Since the time various strategies have been introduced to overcome the ABO-blood barrier including local infusion therapy (LIT), plasmapheresis and rituximab, the graft and patient survival outcome of ABO-incompatible (ABOi) adult living donor liver transplantation (ALDLT) has remarkably improved. But, the need for LIT under rituximab prophylaxis should be reevaluated because of high incidence of the LIT-related complications. The aim of this study was to verify the safety and efficacy of the protocol without local infusion therapy in ABOi ALDLT. METHODS: From November 2008 to December 2010, 43 cases of ABO-incompatible adult living donor liver transplantation were performed. In all cases, the spleen was preserved. From the 1st to 20th case, LIT was employed (group I, n=20). From the 21th case onwards, LIT was eliminated from the protocol (group II, n=23). The 3-month and 1-year patient and graft survival rates were compared between the two groups. The clinical parameters including recipient, donor and graft-related factors were also compared. The graft function was assessed in each group based on the serial changes in serum AST/ALT, total bilirubin and prothrombin time. RESULTS: There was 1 case of in-hospital mortality (2.3%) among the 43 cases. Overall 3-month and 1-year patient and graft survival rate was 97.7% and 92.1% during a mean period of 11.4 +/- 0.4 (0.9~28.9) months. There was no significant difference in the 3-month and 1-year patient and graft survival rates (95.0 vs. 100% and 90.0 vs. 92.9%, P=0.60) between groups. LIT-related complications occurred in 4 patients (20.0%). One case of antibody-mediated rejection occurred in group II. Both groups showed no difference in graft function at postoperative 3rd month. CONCLUSIONS: ABOi ALDLT without splenectomy and LIT resulted in promising outcomes. Therefore, LIT can be safely eliminated from the protocol.
Adult ; Antibodies, Monoclonal, Murine-Derived ; Bilirubin ; Graft Survival ; Hospital Mortality ; Humans ; Incidence ; Liver ; Liver Transplantation ; Living Donors ; Plasmapheresis ; Prothrombin ; Rejection (Psychology) ; Rituximab ; Spleen ; Splenectomy ; Tissue Donors ; Transplants

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) transmission among non-close contacts is not infrequent. We evaluated the proportion and circumstances of individuals to whom SARS-CoV-2 was transmitted without close contact with the index patient in a nosocomial outbreak in a tertiary care hospital in Korea. From March 2020 to March 2021, there were 36 secondary cases from 14 SARS-CoV-2 infected individuals. Of the 36 secondary cases, 26 (72%) had been classified as close contact and the remaining 10 (28%) were classified as non-close contact. Of the 10 non-close contact, 4 had short conversations with both individuals masked, 4 shared a space without any conversation with both masked, and the remaining 2 entered the space after the index had left. At least one quarter of SARSCoV-2 transmissions occurred among non-close contacts. The definition of close contact for SARS-CoV-2 exposure based on the mode of droplet transmission should be revised to reflect the airborne nature of SARS-CoV-2 transmission.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) transmission among non-close contacts is not infrequent. We evaluated the proportion and circumstances of individuals to whom SARS-CoV-2 was transmitted without close contact with the index patient in a nosocomial outbreak in a tertiary care hospital in Korea. From March 2020 to March 2021, there were 36 secondary cases from 14 SARS-CoV-2 infected individuals. Of the 36 secondary cases, 26 (72%) had been classified as close contact and the remaining 10 (28%) were classified as non-close contact. Of the 10 non-close contact, 4 had short conversations with both individuals masked, 4 shared a space without any conversation with both masked, and the remaining 2 entered the space after the index had left. At least one quarter of SARSCoV-2 transmissions occurred among non-close contacts. The definition of close contact for SARS-CoV-2 exposure based on the mode of droplet transmission should be revised to reflect the airborne nature of SARS-CoV-2 transmission.