ABSTRACT
PURPOSE: We evaluated the outcomes of liver transplantation (LT) in pediatric and adult patients with biliary atresia (BA). We focused on bowel perforation after LT (BPLT) as the most common surgical complication and analyzed its risk factors. METHODS: This was a retrospective analysis of 70 BA patients who underwent LT. The patients were divided into three groups according to the timing of LT: within the first year of age (Group A), between 1 and 12 years of age (Group B), and after 12 years of age (Group C). The outcomes of LT and the clinical presentations of BPLT were compared. The surgical variables of patients with and without BPLT were analyzed to assess the risk factors. RESULTS: The timing of LT did not affect patient survival. The incidence of BPLT was significantly higher in Group C. In Group C, BPLT progressed to severe peritonitis. No cases of BPLT-associated mortality were observed. A multivariate analysis revealed that a prolonged operative time for LT was an independent risk factor (p = 0.03). CONCLUSION: The clinical course after transplantation was complicated after adolescence. BPLT should be strongly suspected and relaparotomy should be performed in a timely manner for patients undergoing LT after adolescence.
Subject(s)
Biliary Atresia/surgery , Intestinal Perforation/epidemiology , Liver Transplantation , Postoperative Complications/epidemiology , Transition to Adult Care , Adolescent , Adult , Age Factors , Biliary Atresia/epidemiology , Child , Child, Preschool , Female , Humans , Infant , Intestinal Perforation/surgery , Male , Retrospective Studies , Risk Factors , Treatment Outcome , Young AdultABSTRACT
OBJECTIVES: Connexin43 (Cx43) is one of the proteins associated with gap junction. Connexin43 knockout mice die after birth owing to hypoplastic lungs. The purpose of this study was to analyze the hypoplastic lung of Cx43 knockout mice to clarify the role of the Cx43 during lung development. METHODS: Adult hetero Cx43 mice were mated. Newborn mice were divided into the following groups: wild, hetero, and knockout. Total RNA was extracted from the right lung, and the left lung was fixed for immunohistochemical staining. The mRNA expression of surfactant protein C, aquaporin-5, and alpha-smooth muscle actin were analyzed by reverse transcriptase polymerase chain reaction. H&E and immunohistochemical staining for those markers were performed. RESULTS: The mRNA expression of aquaporin-5, surfactant protein C, and alpha-smooth muscle actin was significantly lower in knockout mice than that in the wild and hetero mice. H&E staining in the knockout mice showed narrow airspaces and thicker interalveolar septae. Immunohistochemical staining in all markers showed the formation of alveoli to be delayed in the knockout mice. CONCLUSION: Based on these findings, Cx43 is closely related to alveolar and vascular formation during lung development.
Subject(s)
Connexin 43/physiology , Lung/growth & development , Actins/genetics , Actins/physiology , Animals , Aquaporin 5/genetics , Aquaporin 5/physiology , Connexin 43/genetics , Connexins/genetics , Connexins/physiology , Gene Expression Regulation, Developmental/genetics , Gene Expression Regulation, Developmental/physiology , Immunohistochemistry , Intercellular Signaling Peptides and Proteins , Lung/abnormalities , Lung/pathology , Mice , Mice, Inbred C57BL , Mice, Knockout , Muscle, Smooth/growth & development , Peptides/genetics , Peptides/physiology , Pulmonary Alveoli/growth & development , Pulmonary Surfactant-Associated Protein C , RNA, Messenger/metabolism , Reverse Transcriptase Polymerase Chain Reaction/statistics & numerical dataABSTRACT
BACKGROUND: The natural history of patients with antenatally diagnosed congenital cystic adenomatoid malformation of the lung (CCAM) is still fully unknown. In symptomatic patients with respiratory distress, an operation is performed during the neonatal period. However, in asymptomatic patients, the optimal timing of the operation remains controversial. During the period from 1977 to 2007, we experienced 14 CCAM patients diagnosed antenatally. Therefore, we investigated the outcome of antenatally diagnosed CCAM patients to clarify the optimal treatment for such patients. METHODS: Fourteen patients were reviewed regarding the antenatal ultrasonography findings and postnatal clinical course. They were then classified into three groups according to the clinical manifestations. Group A was associated with hydrops fetalis (n = 2), group B had respiratory symptoms just after birth (n = 6), and group C was asymptomatic at birth (n = 6). The postnatal clinical courses in three groups were reviewed. RESULTS: In group A, all two patients with hydrops fetalis died just after birth. In group B, six patients had a severe respiratory distress and underwent operation during the neonatal period. In group C, five out of six patients were asymptomatic and received elective operation during the early infant period. In the remaining one patient, the lesion spontaneously disappeared over time after birth. The mean age at the time of operation in group B and group C was 4.5 days and 4.5 months of age, respectively. In almost all patients in group C, we performed an operation within the first 6 months. During this observation period, we did not experience any complications associated with CCAM. CONCLUSION: In patients with hydrops fetalis, fetal intervention is thought to be needed. In patients with asymptomatic CCAM, an elective operation during the early infant period is recommended to prevent the risk of complications associated with CCAM before 6 months of age. In addition, we recommend the performance of a partial lung resection using an axillary skin crease incision in order to obtain a good postoperative quality of life.