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Biomedical subjects

Hisami Ando

Publications and source records attributed to Hisami Ando.

At least 19 recordsLinked to original sources

Intrahepatic cystic lesions after hepatic portoenterostomy for biliary atresia with bile lake and dilated bile ducts.

BACKGROUND: Intrahepatic cystic lesions develop after hepatic portoenterostomy in some patients with biliary atresia and have been regarded as an indication of poor prognosis; however, there is confusion about the terminology and concepts of the lesions. We investigated whether the cystic lesions constituted a single entity. PATIENTS AND METHODS: From 1980 to 2005, we encountered 80 patients with biliary atresia. Abdominal ultrasonography disclosed intrahepatic cystic lesions in 20 (25%) patients after hepatic portoenterostomy. The cystic lesions were analyzed morphologically with radiologic imaging studies and histologically in 13 patients who underwent liver transplantation. RESULTS: Fifteen patients had solitary simple cystic lesions, and 5 patients had multiple continuous oval or beaded lesions. Solitary lesions had a fibrotic cyst wall and lacked epithelia. Continuous lesions had a cyst wall covered with biliary epithelia. Thirteen patients with solitary cysts died or required liver transplantation. In 2 patients with continuous lesions, surgical reboring of the porta hepatis could eliminate cystic lesions and jaundice. CONCLUSIONS: Intrahepatic cystic lesions include 2 different conditions. Solitary cysts are retention pseudocysts, which should be referred to as a bile lake, and are associated with poor prognosis. Continuous beaded cysts are dilated bile ducts, which may be reversed. This distinction is important when considering the treatment strategy.

Bile Duct Diseases↗

Reciprocal expression of CCAAT/enhancer binding proteins alpha and beta in hepatoblastomas and its prognostic significance.

Hepatoblastoma is one of the common pediatric solid tumors with frequent mutation of the beta-catenin gene which might be an early event of its carcinogenesis. However, the detailed molecular mechanism is still unknown. We studied the expression levels of CCAAT/enhancer binding protein alpha (C/EBPalpha) and C/EBPbeta, which regulate differentiation and growth of embryonic hepatocytes, to establish whether or not they were involved in affecting the clinical behavior of hepatoblastoma. The quantitative real-time reverse transcriptase-PCR revealed that expression of C/EBPalpha mRNA was significantly up-regulated in tumors 223% (p=0.013) as compared with that in adjacent normal livers, while expression of C/EBPbeta was down-regulated to 27% (p=0.002). Of interest, the immunohistochemical analysis showed that expression of C/EBPalpha was higher and that of C/EBPbeta lower in the poorly differentiated tumor cells than in the well-differentiated cells within the same tumor. Furthermore, high expression of C/EBPalpha (p=0.047) as well as low expression of C/EBPbeta (p=0.025) was significantly associated with poor prognosis of the patients. Cox hazard model suggested that expression of C/EBPalpha and that of C/EBPbeta were independent indicators to predict the prognosis from age but not from histology. Thus, expression of C/EBP proteins may play an important role in the genesis and clinical behavior of hepatoblastoma probably by inducing different stages of arrest of differentiation.

CCAAT-Enhancer-Binding Protein-alpha↗

Endoscopic resection of intrahepatic septal stenosis: minimally invasive approach to manage hepatolithiasis after choledochal cyst excision.

Intrahepatic calculi frequently form after excision of a choledochal cyst. Biliary stenosis is one of the major causative factors. When stenoses exist in the peripheral intrahepatic duct, hepatectomy is necessary. A new endoscopic management was performed in a 6-year-old girl, who had intrahepatic calculi, 4 years after cyst excision. A septal stenosis in the left lateral anterior branch of the bile duct interfered with evacuation of the intrahepatic stones. The stenosis was grasped with a 3-prong grasper, applied through a choloangioscope, and resected when current was applied to an electrocauery. The stenotic bile duct was widened, and the stones were easily removed. Intrahepatic calculi have not recurred for 2 years. The present technique is another modality for the correction of biliary stenoses.

Child↗

Extrahepatic portal vein morphology in children with extrahepatic portal hypertension assessed by 3-dimensional computed tomographic portography: a new etiology of extrahepatic portal hypertension.

BACKGROUND/PURPOSE: The cause of extrahepatic portal hypertension in children has not been clarified. Our aim was to determine the morphological features of the extrahepatic portal vein in children with extrahepatic portal hypertension by 3-dimensional computed tomographic portography and to clarify the etiology of this disorder. MATERIALS AND METHODS: Six patients ranging in age from 10 to 18 years (median age, 12.8 years) who had portal hypertension presented with hematemesis. They underwent intravenous computed tomographic portography using a helical computed tomography scanner and 3-dimensional image reconstruction. RESULTS: The extrahepatic portal vein was visualized in all patients by 3-dimensional computed tomographic portography. None of the patients showed extrahepatic portal vein obstruction or cavernous transformation. All patients had a tortuous eta-shaped extrahepatic portal vein, and a line could be drawn through the flexures of the portal vein to the hepatic hilum. CONCLUSION: In children, extrahepatic portal hypertension is not caused by extrahepatic portal vein obstruction and may be of embryological origin.

Adolescent↗

Hepatolithiasis after hepatic portoenterostomy for biliary atresia.

BACKGROUND: Hepatolithiasis after hepatic portoenterostomy for biliary atresia has been paid little attention, with only 22 reported cases. PATIENTS AND METHODS: Fifteen patients underwent living-related liver transplantation for biliary atresia after hepatic portoenterostomy in our hospital between 1998 and 2004. The resected livers were examined for the existence and location of hepatolithiasis, composition of the calculi, and bacterial infection of bile. The relation between a history of cholangitis and the presence of hepatolithiasis was analyzed. RESULTS: Intrahepatic calculi were found in 8 (53%) of 15 patients. The calculi consisted of almost 100% calcium bilirubinate. Calculi were found in bile lakes in 8 patients. Bacteria were present in the bile in 8 (53%) of the 15 patients. Of the 8 patients, 7 (88%) had a history of ascending cholangitis. CONCLUSIONS: Hepatolithiasis occurs after hepatic portoenterostomy for biliary atresia more frequently than previously thought. Bile stasis and possibly bile infection are the main causes of calculi formation.

Adolescent↗

Living donor liver transplantation for congenital absence of the portal vein in a child with cardiac failure.

Congenital absence of the portal vein (CAPV) requires liver transplantation when encephalopathy develops. However, transplantation has technical difficulties because no collateral circulation exists except for the portosystemic shunt. Ligating the shunt will cause disastrous mesenteric venous congestion. We report a 19-month-old female infant with CAPV, who had portosystemic encephalopathy and cardiac failure, and underwent living donor liver transplantation with a partial clamp technique using a vein graft. This is the first case of successful liver transplantation for CAPV with cardiac failure.

Cardiovascular Abnormalities↗

Two cases of prenatally diagnosed congenital lobar emphysema caused by lobar bronchial atresia.

Bronchial atresia is a rare congenital anomaly, and neonatal presentation is extremely rare. Bronchial atresia often affects segmental or subsegmental bronchi, and bronchial atresia of a lobar bronchus is also rare. We report 2 cases of prenatally diagnosed congenital lobar emphysema caused by bronchial atresia at the left upper lobar bronchus. Three-dimensional computed tomography bronchoscopy was effective to demonstrate accurate anatomical changes of the bronchus.

Bronchi↗

Bile infection contributes to intrahepatic calculi formation after excision of choledochal cysts.

Intrahepatic calculi complicate choledochal cysts in 7-8% of patients. Although congenital stenoses and dilatation of the intrahepatic bile ducts are considered responsible for calculi formation, intrahepatic calculi are usually formed after cyst excision. In this study, bile specimens from patients with choledochal cysts were cultured for bacteria. Results were retrospectively analyzed among the following groups: the primary excision group, consisting of 97 patients undergoing cyst excision as a primary treatment (mean age 5.0 years), the internal drainage group, consisting of 13 patients who had previous cyst-enterostomy at cyst excision (mean age 20.2 years); and the hepatolithiasis group, consisting of 12 patients with postoperative hepatolithiasis (mean age 24.2 years). Bacteria were present in the bile of 10 patients (76.9%) in the internal drainage group and in all patients (100%) in the hepatolithiasis group, but present in only 17 patients (17.5%) in the primary excision group (p<0.01). Polymicrobial infection with Gram-negative enterobacteria such as Escherichia coli and Klebsiella species was predominant in the internal drainage and hepatolithiasis groups, while nonenteric bacteria were found in the primary excision group. Bile infection through bilioenterostomy may play an important role in intrahepatic calculus formation after excision of a choledochal cyst.

Adolescent↗

Isolation and culture of biliary epithelial cells from bile duct remnants of patients with biliary atresia.

Biliary atresia(BA), the most common cause of obstructive jaundice in infancy, has been considered to be a result of progressive destruction of the bile ducts through a necroinflammatory process. Many immunohistochemical studies of BA remnant have been done, but it has not been shown that biliary epithelial cells (BECs) can be cultured from BA remnant. For this study, we obtained bile duct remnants from three patients with biliary atresia (one male, two females) who received Kasai's operation from 2002 to 2003. The successive cultivation rate of BECs from explants was 100% (3/3 patients). Culture of BECs on collagen gel was possible up to at least four passages. Under a phase-contrast microscope, primary and passed cultured cells on collagen gel showed a cobblestone-like spread in 2 weeks. The BECs had immunoreactivity to anti-human cytokeratin 7 antibody. In this study, we proved that BECs in the remnants of BA could be cultured, and defined the maturation of biliary epithelial cells of BA by immunocytochemistry with anti-human cytokeratin 7 antibody. In conclusion, BECs in BA remnant are "alive", and their proliferation activity can be maintained.

Bile Ducts↗

Expression profiling and differential screening between hepatoblastomas and the corresponding normal livers: identification of high expression of the PLK1 oncogene as a poor-prognostic indicator of hepatoblastomas.

Hepatoblastoma is one of the most common malignant liver tumors in young children. Recent evidences have suggested that the abnormalities in Wnt signaling pathway, as seen in frequent mutation of the beta-catenin gene, may play a role in the genesis of hepatoblastoma. However, the precise mechanism to cause the tumor has been elusive. To identify novel hepatoblastoma-related genes for unveiling the molecular mechanism of the tumorigenesis, a large-scale cloning of cDNAs and differential screening of their expression between hepatoblastomas and the corresponding normal livers were performed. We constructed four full-length-enriched cDNA libraries using an oligo-capping method from the primary tissues which included two hepatoblastomas with high levels of alpha-fetoprotein (AFP), a hepatoblastoma without production of AFP, and a normal liver tissue corresponded to the tumor. Among the 10,431 cDNAs randomly picked up and successfully sequenced, 847 (8.1%) were the genes with unknown function. Of interest, the expression profile among the two subsets of hepatoblastoma and a normal liver was extremely different. A semiquantitative RT-PCR analysis showed that 86 out of 1188 genes tested were differentially expressed between hepatoblastomas and the corresponding normal livers, but that only 11 of those were expressed at high levels in the tumors. Notably, PLK1 oncogene was expressed at very high levels in hepatoblastomas as compared to the normal infant's livers. Quantitative real-time RT-PCR analysis for the PLK1 mRNA levels in 74 primary hepatoblastomas and 29 corresponding nontumorous livers indicated that the patients with hepatoblastoma with high expression of PLK1 represented significantly poorer outcome than those with its low expression (5-year survival rate: 55.9 vs 87.0%, respectively, p=0.042), suggesting that the level of PLK1 expression is a novel marker to predict the prognosis of hepatoblastoma. Thus, the differentially expressed genes we have identified may become a useful tool to develop new diagnostic as well as therapeutic strategies of hepatoblastoma.

Cell Cycle Proteins↗

Treatment strategies for perianal rhabdomyosarcoma: report of two cases.

Intensive chemotherapy and high-dose radiation with complete excision of the tumor are the treatment of choice for rhabdomyosarcoma (RMS). However, because there are so few reports of perianal RMS, a mainstream treatment has not been established. We report two cases of perianal RMS and review 13 cases documented in Japan since 1990. Case 1 was a 7-year-old boy first treated by tumor excision followed by chemotherapy and external irradiation. He has since been tumor-free but suffered severe perianal erosion and ulceration. Case 2 was a 17-year-old girl first treated by preoperative chemotherapy to reduce the tumor size, after which the tumor was completely excised with anatomical guidance using three-dimensional computed tomography via the posterior sagittal approach, followed by open intraoperative irradiation. She has since been tumor-free with preserved anal function. These case reports show the importance of cogitated treatments for preservation of anal function and optimal therapeutic outcome in patients with perianal RMS.

Adolescent↗

The role of midkine and pleiotrophin in liver regeneration.

BACKGROUND/PURPOSE: We studied roles of two closely related heparin-binding growth factors, midkine (MK) and plieotrophin (PTN) in the processes of liver regeneration after partial hepatectomy. METHODS: Liver regeneration was comparatively analyzed using mice deficient in the MK gene (Mdk), mice deficient in the pleiotrophin gene (Ptn) and wild-type mice. RESULTS: Migration of neutrophils and macrophages to the liver 1-5 days after partial hepatectomy was suppressed in Mdk (-/-) mice. Liver damage as assessed by serum aspartate aminotransferase was also less severe in the deficient mice. On the other hand, cell growth in the liver was generally suppressed in the deficient mice 3-14 days after partial hepatectomy. The weight of the liver 7 and 14 days after partial hepatectomy was significantly less in Mdk (-/-) mice than wild-type mice. Very similar results were obtained using Ptn (-/-) mice except that a significant difference in the repaired liver weight was not observed between Ptn (-/-) mice and wild-type mice. CONCLUSIONS: MK and PTN are involved both in the inflammatory and reparative processes after partial hepatectomy, and as a whole are beneficial for liver regeneration.

Animals↗

Three-dimensional image reconstruction of an anorectal malformation with multidetector-row helical computed tomography technology.

The presentation of the surgical anatomy of anorectal malformation by standard anatomical figures is not suitable for individual anorectoplasty. It is essential to understand the anatomy of the pelvic muscle (striated muscle complex: SMC) including the external anal sphincter and their three-dimensional (3D) configuration in each patient. Thus, we studied the SMC three-dimensionally with multidetector-row helical computed tomography (MRH-CT) preoperatively, and evaluated its usefulness. Fourteen patients with anorectal malformations before anorectoplasty (types: high n=6, intermediate n=2, low n=6) and two patients without anorectal malformations as controls (total: male n=8, female n=8) were investigated. An image of pelvic region was prepared with a slice thickness of 0.5 mm and a reconstruction pitch of 0.5 mm. A 3D reconstruction on a conventional personal computer (PC) was made with a volume rendering method, and assisted by our own software. The SMC was analyzed with three modified modes of 3D reconstruction corresponding to the surrounding tissues. A length of the parasagittal muscle, and both the sagittal and transverse width of the vertical fibers in the SMC at the connection to the parasagittal muscle were measured on a 3D image and then compared among three different types and controls. To eliminate variations in age, a length index was used to allow comparison. The 3D configuration of the SMC was different in every case. The arranged image mode, which displayed the SMC and the pelvic bones simultaneously, enabled to use conventional knowledge in cysto-urethrography. The length of the parasagittal muscle was longest in the high type but the width of the vertical fibers was smallest. Anatomical figures of the SMC including the external anal sphincter were clearly demonstrated on a PC in every anorectal malformation by our program. A 3D reconstruction image provides positional information on the SMC for the body surface and pelvic bone at the same time. Both a 3D image and positional information with MRH-CT offers the surgeon a simulated operative profile of the SMC superior to CT or magnetic resonance imaging slices alone.

Anal Canal↗

A case of retroperitoneal abscess caused by Salmonella Oranienburg.

The authors report a case of retroperitoneal abscess caused by Salmonella Oranienburg in an 8-year-old girl. This was one case in an epidemic of food poisoning from Salmonella Oranienburg or Salmonella Chester transmitted by many kinds of contaminated dried squid products. This is the first reported case of a retroperitoneal abscess by Salmonella Oranienburg.

Abscess↗

Development of disposable self-regulating blood pumps and automatically-controlled portable extracorporeal membrane oxygenation systems for neonatal extracorporeal membrane oxygenation.

For safer and simpler neonatal ECMO management, the authors have been developing an innovative, automatically-controlled ECMO machine using self-regulating blood pumps. The newest model is an air-driven, tube-type blood pump. A pair of blood pumps are placed in parallel and actuated alternately with compressed air. The pump flow is automatically regulated in accordance with hemodynamic changes of the body. The need for a venous reservoir is eliminated because the circuit does not generate excessive negative pressure when there are shortages of draining blood. Therefore, the priming volume of the circuit is only 85 ml. This ECMO apparatus has three driving modes, one of which is a "delay" mode. This enables the pump flow to easily be varied from 10 to 500 ml/min under automatic control, and it can be used when the pump flow is decreased for weaning. This newest ECMO apparatus was clinically used in 3 neonates with severe lung hypoplasia and persistent fetal circulation. The ECMO duration was 139 h to 168 h, and the maximum ECMO flow 71.3 to 109.0 ml/min/kg. Thanks to the self-regulation, intensive observation of the circuit was not necessary during ECMO. Damage to blood cells was less significant, and the use of blood products was only minimal (15.7 ml/kg/day) because a large volume of blood to stabilize the pump performance was not necessary. In conclusion, the automatically-controlled ECMO apparatus worked well without complications by changing the ECMO flow automatically in accordance with hemodynamic changes. This automatic ECMO system required fewer personnel expenditures and was also favorable in terms of hematological findings.

Acute Disease↗

Attenuated nitrergic inhibitory neurotransmission to interstitial cells of Cajal in the lower esophageal sphincter with esophageal achalasia in children.

BACKGROUND: Esophageal achalasia (EA) is a rare disease in children, the etiology and pathogenesis of which remain controversial. Previous studies have suggested that a specific class of interstitial cells of Cajal (ICC) act as mediators in nitrergic inhibitory neurotransmission in the lower esophageal sphincter (LES). The aim of this investigation is to clarify the status of ICC and nitrergic inhibitory neurons in the LES of EA using immunohistochemistry. METHODS: Specimens were obtained from two patients with EA (aged 6 and 10 years) and two patients with esophageal carcinoma (aged 56 and 63 years) not involving the lower esophagus as controls. Immunohistochemistry was used to study the distribution of ICC and nitrergic inhibitory neuron. RESULTS: The LES contains the c-kit positive ICC in the muscle layers, which form close relationships with nitric oxide synthase (NOS)-containing nerve fibers in the controls. The distribution of ICC was almost the same between samples with EA and controls. However, such nerve fibers were absent in EA with a longer duration of the symptoms, but were reduced in a shorter duration. CONCLUSIONS: Decreased nitrergic inhibitory neurotransmission to ICC in LES is a possible cause of sphincter achalasia in pediatric patients with EA. The decrease in NOS-positive neurons of patients with achalasia may be gradual, which may account for the long duration of symptoms prior to treatments. Further advancement of esophageal motility damage was suspected in pediatric EA.

Child↗

Colonic atresia and Hirschsprung's disease: importance of histologic examination of the distal bowel.

Hirschsprung's disease associated with colonic atresia is rare. A boy with colonic atresia at the hepatic flexure who had a colostomy in the neonatal period suffered from severe constipation after definitive colocolostomy. Hirschsprung's disease was diagnosed with anorectal manometry and rectal mucosal biopsy, and a Duhamel-Ikeda's pull-through procedure was performed. Aganglionosis of the entire distal colon was seen, and intrauterine torsion of the dilated proximal colon followed by necrosis and absorption was suspected as the cause of colonic atresia. Colonic atresia should be generally screened for Hirschsprung's disease with a rectal biopsy. J

Biopsy↗