ABSTRACT
Objective To evaluate the use of intercostal trocars (ICS) and transthoracic trocars in laparoscopic resection of liver segments 7 and 8.Method From November 2015 to June 2017,20 patients who underwent laparoscopic S7 or 8 segmentectomy for liver tumors in the Department of Hepatobiliary Surgery,the First Affiliated Hospital,Guangxi Medical University were analyzed retrospectively.Results Ports were inserted at the 8th or 9th ICS,respectively,in addition to the conventional abdominal ports.The mean operation time was 225.0 min (110.0 ～ 486.0 min).Anatomical resection was completed in 1 patient,and non-anatomical resection in 19 patients.The conversion rate was 0％.Pringle's maneuver was used in 9 patients.The mean blood loss was 85.0 (25.0 ～410.0) ml,and the mean length of hospital stay was 7.0 (5.0 ～ 12.0) days.The complication rate was 10.0％.Pathologic findings revealed that 17,2,1 patient(s) had HCC,hemangioma,and inflammatory nodule,respectively.The mean tumor size and tumor free margin were 33.8 (15.0 ～ 74.0) mm;and 15.0 (1.0 ～ 30.0) mm,respectively.There was no HCC recurrence on follow-up,expect for one patient who developed tumor recurrence at 20 months after laparoscopic liver resection.Conclusions In selected patients,laparoscopic liver resection using intercostal trocars was useful and safe for tumors located in liver segments 7 and 8.The long-term oncologic outcomes need to be further evaluated.
ABSTRACT
Objective To investigate the clinical and molecular genetic characteristics of hypermethioninemia caused by methionine adenosyltransferase deficiency. Methods The clinical data and related gene analysis of hypermethioninemia caused by methionine adenosyltransferase deficiency in 3 children were retrospectively analyzed. The core pedigree analysis was carried out. Results Three children (2 boys and 1 girl) aged from 5 months to 3 years, were from 3 unrelated families. All of them had no family history. One case was found in neonatal screening. One case was onset with pathological jaundice at 1 month old. Another case was found due to tremor and growth retardation at 2 years old. Blood amino acid ester acyl carnitine spectrum analysis showed that all of them had significantly elevated levels of methionine at 134.50-790.67 μmol/L. All children had MAT1A mutation in methionine adenosyltransferase gene. One case was heterozygous mutations with third exon c.274T>C and seventh exon c.895C>T mutation; one case had sixth exon c.757G>A homozygous mutation; and another case had seventh exon c.791G>A homozygous mutation. The core pedigree analysis showed that the mutations were from theirs parents respectively. Conclusions For children with neurologic impairment, methionine metabolic disorders should be considered. Blood amino acids and gene analysis are important methods for confirmation of the diagnosis. Neonatal screening is an effective way to detect this disease.