The clinical impact of macrophage polarity after Kasai portoenterostomy in biliary atresia

IntroductionBiliary atresia (BA) is a cholestatic hepatopathy caused by fibrosing destruction of intrahepatic and extrahepatic bile ducts, and its etiology has not been clearly revealed. In BA, liver fibrosis progression is often observed even after Kasai portoenterostomy (KPE), and more than half of cases require liver transplantation in their lifetime in Japan. Macrophages play an important role in liver fibrosis progression and are classically divided into proinflammatory (M1) and fibrotic macrophages (M2), whose phenotypic transformation is called “macrophage polarity.” The polarity has been reported to reflect the tissue microenvironment. In this study, we examined the relationship between macrophage polarity and the post-KPE clinical course.Materials and methodsThirty BA patients who underwent KPE in our institution from 2000 to 2020 were recruited. Multiple immunostainings for CD68, CD163, CK19, and α-SMA were carried out on liver biopsy specimens obtained at KPE. ROC curves were calculated based on each clinical event, and the correlation with the clinical data was analyzed.Results and discussionThe M2 ratio, defined as the proportion of M2 macrophages (CD163-positive cells), was correlated inversely with the occurrence of postoperative cholangitis (AUC: 0.7602). The patients were classified into M2 high (n = 19) and non-high (n = 11) groups based on an M2 ratio value obtained from the Youden index ( = 0.918). As a result, pathological evaluations (Metavir score, αSMA area fraction, and CK19 area fraction) were not significantly different between these groups. In mild liver fibrosis cases (Metavir score = 0–2), the M2 non-high group had a significantly lower native liver survival rate than the high group (p = 0.02). Moreover, 4 out of 8 cases in the M2 non-high group underwent early liver transplantation within 2 years after KPE.ConclusionsNon-M2 macrophages, including M1 macrophages, may be correlated with postoperative cholangitis, and the M2 non-high group in mild liver fibrosis cases had a significantly lower native liver survival rate than the high group, requiring early liver transplantation in this study. Preventing advanced liver fibrosis is a key factor in improving native liver survival for BA patients, and liver macrophages may play important roles in liver homeostasis and the promotion of inflammation and fibrosis

Prenatally Diagnosed Cystic Neuroblastoma: A Report of Two Cases

We report two cases of prenatally diagnosed cystic neuroblastoma (PDCN). In the first case, prenatal ultrasonography (US) at 33 weeks' gestation showed a 30 × 20 mm cyst at the upper pole of the right kidney. The size and content of the mass demonstrated no change during pregnancy. Postnatal US showed no change in the cystic mass 4 weeks after birth compared to the prenatal findings. The infant underwent total resection of the tumour at 28 days of age. In the second case, a left cystic mass measuring 50 × 40 mm was detected in a fetus in the 37th week of pregnancy. Postnatal US showed a cystic mass in the left adrenal gland. The US findings showed no change 18 days after birth and the infant underwent total resection of the tumour at 19 days of age. In both cases, pathological examination revealed a neuroblastoma and all of the biological prognostic factors were favourable. Surgical intervention was necessary for a final histological and biological diagnosis to be made. We recommend that prenatally suspected neuroblastomas should normally undergo surgical intervention, unless tumour size decreases within about 1 month after birth

Laparoscopic intraperitoneal onlay mesh for pediatric incisional hernia—a case report

Abstract Background The incidence of incisional hernia in pediatric patients is low in comparison with that reported in adults. In the pediatric population, primary closure has generally been favored. However, synthetic or biomedical mesh offers advantages in the repair of larger defects when primary closure is difficult. The use of laparoscopic intraperitoneal onlay mesh (IPOM) in the adult population has been well documented. In the pediatric population, a few laparoscopic approaches with direct suturing have been proposed; however, there are no reports of laparoscopic repair with the use of IPOM. Case presentation The patient was a 1-year-old girl with epigastric incisional hernia after an operation to correct a complete arteriovenous septal defect. The fascial defect (size 30 × 35 mm) was large; thus, direct suturing was considered to be associated with a high risk of thoracic deformation and recurrence. Laparoscopic IPOM was performed. The fascial defect was detected precisely through the laparoscopy, and non-absorbable mesh was placed through a 12-mm trocar. Minimal incisions were required for the trocars, and extensive dissection of the abdominal wall structure was not needed. This procedure allowed for the integrity and functional status of the abdominal wall to be maintained. Conclusion Laparoscopic IPOM is a minimally invasive and cosmetically acceptable method that can be applied to the treatment of large incisional hernias in children

Hemorrhagic shock due to rupture of a nephroblastoma in an infant: A case report

Introduction: Nephroblastoma is the most common type of malignant renal tumor in children, but hemorrhage in ruptured nephroblastoma is rare. Case presentation: A three-month-old boy was admitted to our hospital with a large right renal tumor and hemorrhagic shock. Laparotomy showed that the tumor had invaded the right lobe of the liver; therefore, surgeons decided to perform only hemostasis and a tumor biopsy. The following day, intratumoral bleeding persisted, necessitating continuous blood infusion, resulting in abdominal compartment syndrome. Therefore, tumor resection was performed. Intra-tumoral hemorrhage continued during the operation, resulting in bradycardia and chest compressions. Histopathological examination confirmed a diagnosis of nephroblastoma. The postoperative course was uneventful. Conclusion: This report underlines the need to choose treatment based on both extratumoral and intratumoral bleeding, keeping in mind the risks of each treatment option

A pedigree of a five-generation multinodular goitre family.

<p>Individuals are numbered above each symbol. Individuals affected with MNG are indicated by filled symbols and age of onset is described under the patient symbol. The arrow shows a proband of this family study. The bars above each symbol indicate individuals included in the linkage analysis.</p

Novel variants identified in the proband in the chromosome 19 linkage region.

<p>ref.: reference sequence.</p

Up-regulation of NFE2L2 target genes in the thyroid tissues of the patient.

<p>Results of the quantitative PCR of <i>NFE2L2</i> and its target genes <i>GSTA4</i>, <i>GCLC</i> and <i>NQO1</i> in the normal and goitre region of thyroid tissues obtained from the proband. The expression levels relative to control RNA from an adult thyroid are shown. The significance with the t-test is indicated with asterisks (*p<0.05 and **p<0.01).</p