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Biomedical subjects

A Yamataka

Publications and source records attributed to A Yamataka.

At least 19 recordsLinked to original sources

Long term prognosis of children born to lupus patients.

OBJECTIVE: To determine the long term prognosis of children of patients with systemic lupus erythematosus (SLE). METHODS: Children of patients with SLE were invited to attend our clinic for physical examination and laboratory tests. A total of 195 children (aged 4 months to 26 years; male = 82, female = 113) were examined in 1991, 1995, 1997, and 1998. RESULTS: Two cases were diagnosed as SLE at the first visit and were excluded from the second visit. A significantly higher percentage (52/195 (27%)) of patients were positive for antinuclear antibodies (ANA) at a cut off serum dilution of 1/40 compared with controls (4/57 (7%)). ANA were detected more frequently in female subjects than in men (p<0.05). Forty four subjects were examined on more than two occasions. Nine of the 10 patients who were positive for ANA at the second visit were girls aged 4-8 years. The incidence of anti-DNA and antiphospholipid antibodies in children of patients with SLE was similar to that in the controls. CONCLUSIONS: The finding that children, especially girls, born to maternal lupus patients had a high positive rate for ANA suggests that a genetic factor is involved in SLE pathogenesis. Longitudinal observation of these patients may provide important clinical information and clues to the pathogenesis of SLE.

Adolescent↗

Transplantation of infantile bladder in rats: an alternative procedure for bladder augmentation.

BACKGROUND: Our purpose was to evaluate whether bladder transplantation (BTx) can be used for bladder augmentation (BA). METHODS: Bladders from infantile Brown-Norway rats (less than 21 days old) were excised and each transplanted into a pouch created in the distal omentum of a 6-week-old Lewis rat (fully allogeneic BTx). No immunosuppressant was used in group I (n=12). Intramuscular FK506 was used daily from the day of BTx in group II (n=16; 0.2 mg/kg), group III (n=22; 0.6 mg/kg), and group IV (n=16; 1.2 mg/kg) until harvesting 3, 4, 5, or 6 weeks after BTx. FK506 was used for only 2 weeks in group V (n=12; 0.6 mg/kg/day) and group VI (n=12; 1.2 mg/kg/day). Syngeneic bladder transplants acted as controls (n=16). Hematoxylin and eosin staining was used to examine all grafts. In six rats from group III, BA was performed by anastomosing the graft to the recipient bladder 10 days after BTx. RESULTS: Each successfully transplanted graft appeared macroscopically as a thin-walled cyst. Rejection was seen in all grafts from groups I, II, V, and VI, and was minimal or absent in groups III and IV. On medium to long-term follow-up the only side effect of FK506 observed was reduced weight gain. Graft survival in the control group was 100%. BA was successful in all six cases, and the mucosa was normal throughout each augmented bladder. CONCLUSIONS: This is the first report of the successful transplantation of infantile tissue without vascular anastomosis. Because of the efficient, safe immunosuppression possible with FK506, our BTx technique could find clinical application for creating viable vesical tissue that could be used for BA.

Animals↗

Are stable postoperative biliary atresia patients really stable?

Transforming growth factor-beta 1 (TGF beta-1) is an important mediator of liver-cell proliferation and replication that is implicated in hepatic fibrosis (HF). Hepatic stellate cells (HSC) are activated by TGF beta-1 and are the main precursor cells involved in fibrogenesis. The correlation between serum TGF beta-1, activated HSC in liver-biopsy specimens, and liver biochemistry was investigated to determine the value of TGF beta-1 as an indicator of clinical status in postoperative biliary atresia (BA) patients. Thirty-two postoperative BA patients (mean age 11.2 +/- 2.8 years) and 13 normal controls (mean age 10.3 +/- 3.7 years) were studied. Based on average liver function test (LFT) results over a 3-month period immediately prior to this study, the BA patients were divided into group I (anicteric, normal LFT; n = 10); group II (anicteric, elevated liver transaminases; n = 12), and group III (jaundiced end-stage liver fibrosis awaiting liver transplantation; n = 10). Serum TGF beta-1 was determined using ELISA. Liver-biopsy specimens were examined with antibody against TGF beta-1 and alpha-smooth muscle actin (SMA) antibody for detection of activated HSC. Serum TGF beta-1 was significantly higher in groups I (11.4 +/- 3.7 ng/ml; P < 0.01) and II (23.3 +/- 11.3 ng/ml; P < 0.001) than in group III (3.0 +/- 1.5 ng/ml) and controls (4.5 +/- 2.5 ng/ml) despite normal LFT in group I. The 3 subjects with the highest serum TGF beta-1 in group II had bile lakes. Biopsies from groups I and II were strongly positive for TGF beta-1 in hepatocytes and Kupffer cells and for activated HSC detected by SMA compared with group III and controls. Because serum TGF beta-1 and activated HSC are only present during active fibrosis, we conclude that there is progressive fibrogenesis even in seemingly normal postoperative BA patients. In particular, bile lakes should be regarded as a key sign of progressive HF, the presence of which should be regarded with extreme caution. We suggest that serum TGF beta-1 could be used as an accurate indicator of progressive fibrogenesis in postoperative BA patients.

Adolescent↗

Beneficial effect of a traditional herbal medicine (inchin-ko-to) in postoperative biliary atresia patients.

Inchin-ko-to (ICKT) prevents Fas-mediated liver injury. This study evaluates the effect of ICKT on conventional markers of liver function (LF) and liver fibrosis in 18 postoperative biliary atresia (BA) patients aged 3 to 23 years with elevated glutamic oxaloacetic transaminase (GOT), glutamic pyruvic transaminase (GPT), gamma-glutamyl transpeptidase (gammaGTP) but normal serum total bilirubin (T-Bil) levels. ICKT (0.15 g/kg per day) was administered orally for 1 year. Serum GOT, GPT, gammaGTP, total bile acids (TBA), and T-Bil as markers of LF and hyaluronic acid (HA), prolyl hydroxylase (PH), procollagen III peptide (PIIIP), and type IV collagen as markers of liver fibrosis were measured before and after treatment in each patient and compared statistically. All patients tolerated ICKT well, and there were no side effects. The percentage of subjects who improved after ICKT was 45% for serum GOT, 72% for GPT, 72% for gammaGTP, 72% for TBA, 67% for HA, 40% for PH, 50% for PIIIP, and 23% for type IV collagen. Changes in the mean values of all serum markers were statistically significant (P < 0.01). It is concluded that long-term administration of ICKT in postoperative BA patients improves liver status as assessed by markers of LF and fibrosis.

Adolescent↗

A case of biliary atresia with duplication of the common bile duct.

The authors report a case of biliary atresia (BA) with duplication of the common bile duct. A 1-month-old girl was referred for investigation of persistent jaundice. BA was suspected and confirmed with intraoperative cholangiography. A portoenterostomy was performed at 50 days of age. During dissection of the fibrous remnant of the common bile duct, a separate fibrous bile duct remnant running in parallel on the left, was identified. Histologically, there was bile duct proliferation at the porta hepatis of the left bile duct remnant. A diagnosis of BA with duplicated common bile duct was made. Postoperative course was uneventful, and at a follow-up assessment at 7 months, she was jaundice free, and her progress has been unremarkable.

Biliary Atresia↗

Transplantation of newborn esophagus: an experimental study.

PURPOSE: The aim of this study was to see if allogeneic transplantation (Tx) of newborn esophagus can create viable esophageal tissue that may be used for treating long gap esophageal atresia. METHODS: Specimens of thoracic esophagus from newborn Brown-Norway rats, each were transplanted into a pouch created in the distal omentum of 5-week-old Lewis rats. In group I no immunosuppressant was used. FK-506 was used in group II (0.2 mg/kg), group III (0.6 mg/kg), and group IV (1.2 mg/kg) until a predetermined day of graft harvesting (1, 2, 3, 4, 5, 6, and 8 weeks after Tx). FK-506 was used for only 2 weeks in group V (0.6 mg/kg), and group VI (1.2mg/kg), and transplanted esophageal grafts were harvested 1, 2, 3, and 4 weeks after cessation of 2 weeks course FK-506. Syngeneic esophagus transplants were used as controls. All grafts were examined by H&E staining to assess graft viability and degree of rejection. RESULTS: Each successfully transplanted esophagus appeared macroscopically as a tube like mass. Each graft could be mobilized to the thoracic cavity, because of the long omental pedicle. Graft survival in the control group was 100%. Rejection was observed in all grafts from groups I, II, V, and VI. In contrast, grafts from groups III and IV showed only minimal or no rejection. There was no evidence of side effects of FK-506 in rats in groups III and IV, except significantly slower weight gain compared with controls (P <.05). CONCLUSIONS: FK-506 successfully prevented rejection, although immunologic tolerance was not achieved. These observations suggest that the authors' procedure has the potential to produce viable esophageal tissue that could be a new option for treating long gap esophageal atresia.

Animals↗

Pyloric stenosis: new histopathologic perspective using confocal laser scanning.

BACKGROUND/PURPOSE: Idiopathic hypertrophic pyloric stenosis (IHPS) is a common infantile disorder characterized by enlargement of the pylorus and gastric outlet obstruction. Its complete etiology is still not fully understood, but recent research has focussed on abnormalities of nerve distribution. The authors used confocal laser scanning microscopy to perform 3-dimensional studies of pylorus biopsy specimens taken from cases of IHPS and present their findings. METHODS: Pylorus biopsy specimens obtained at pyloromyotomy from 6 infants with IHPS were studied using confocal microscopy and compared with 6 control pylorus biopsy specimens from patients without gastrointestinal disease. Biopsy specimens were pretreated to enhance nerve expression by using protein gene product 9.5 (PGP9.5) polyclonal antibody to identify enteric nerve system fibers. Double staining immunofluorescence was used to detect alpha smooth muscle actin (SMA), a smooth muscle marker. RESULTS: Control pylorus biopsy specimens showed many thin PGP9.5-positive nerve fibers in the circular and longitudinal muscle layers that communicated with each other to create a 3-dimensional meshlike network. Muscle cells stained by alpha SMA antibody were thin. In contrast, muscle cells from IHPS patients were fat and round. The PGP9.5 staining nerve fibers from IHPS patients formed numerous, thick, and contorted bundles that did not communicate. CONCLUSIONS: By using confocal laser microscopy the authors were able to identify abnormally thick contorted nerve bundles in the pyloric muscle layers of infants with IHPS. These anormal nerve bundles have not been described previously because of the limitations of 2-dimensional microscopy. The authors suspect that the etiology of IHPS may be related to these abnormal fibers.

Biopsy, Needle↗

Intestinal neuronal dysplasia-like pathology in Ncx/Hox11L.1 gene-deficient mice.

BACKGROUND/PURPOSE: Ncx/Hox11L.1-deficient (Ncx-/-) mice specifically created by the authors had mega-ileo-ceco-colon (mega-ICC) with a caliber change in the proximal colon. The authors studied the nerve distribution in the bowel of these Ncx-/- mice to determine the cause of their bowel dysmotility. METHODS: Four-week-old Ncx-/- mice (n = 10; 5 with mega-ICC, 5 without mega-ICC) were killed and the bowel harvested. Half of each specimen was snap frozen for AchE and NADPH-diaphorase histochemistry, and the other half were fixed with 10% formalin for H&E staining and immunohistochemistry using PGP9.5 antibody (a marker for neurons), C-kit antibody (a marker for intestinal pacemaker cells), and stem cell factor antibody (a marker for C-kit ligand). Age-matched wild-type normal mice (n = 5) served as controls. RESULTS: In the ileum, cecum, and proximal colon from all Ncx-/- mice (irrespective of the association of mega-ICC), typical findings of human intestinal neuronal dysplasia (IND) ie, obvious hyperganglionosis in neuronal plexuses on PGP9.5 immunohistochemistry, ectopic ganglia in the mucosal and muscular layers on AchE histochemistry, and ghostlike ganglia on NADPH-diaphorase histochemistry were found. Likewise, in normal caliber distal colon from these mice, the distribution of ganglion cells, C-kit, and stem cell factor was normal. In control specimens, there was no ectopic ganglia or hyperganglionosis. CONCLUSIONS: These findings suggest that the Ncx/Hox11L.1 gene is required for the proper innervation of the enteric nervous system in mice, and our deficient strain may be useful as a model for studying IND in humans.

Animals↗

Serum concentration of adhesion molecules in postoperative biliary atresia patients: relationship to disease activity and cirrhosis.

BACKGROUND/PURPOSE: Biliary atresia (BA) is associated with progressive liver fibrosis, which may be mediated by immunologic abnormalities involving adhesion molecules. This study investigates the relationship between serum intercellular adhesion molecule-1 (sICAM-1), serum vascular cell adhesion molecule-1 (sVCAM-1), and the clinical and histologic severity of BA. METHODS: Serum ICAM-1 and VCAM-1 levels were measured by enzyme-linked immunosorbent assay in 35 patients with BA and 20 healthy controls. Standard liver function tests (LFTs), and frozen section liver biopsy specimens were used to determine liver status. On the basis of LFT results, the BA patients were classified into group I (n = 10; normal LFTs), group II (n = 15; elevated LFTs, anicteric), and group III (n = 10; elevated LFTs, icteric). Eight subjects in group II, and all subjects in group III had portal hypertension (PH). RESULTS: sICAM-1 levels were significantly elevated in group III (1760.0 +/- 717.5 ng/mL) compared with group II (555.1 +/- 199.4 ng/mL), group I (272.1 +/- 59.9 ng/mL) and controls (256.3 +/- 71.6 ng/mL). Although sVCAM-1 levels were significantly elevated in group III (1932.9 +/- 282.6 ng/mL) compared with group II (1054.3 +/- 297.0 ng/mL), group I (605.4 +/- 112.4 ng/mL), and controls (616.0 +/- 112.0 ng/mL; P <.001), there was no statistically significant difference between groups I, II, or controls. sVCAM-1 levels were elevated significantly in BA subjects in group II with PH (1253.0 +/- 245.1 ng/mL) compared with those who did not have PH (827.3 +/- 151.7 ng/mL; P <.01). PH did not affect sICAM-1 levels. There was strong expression of ICAM-1 and VCAM-1 in proliferating bile ductules, endothelial cells, and liver cells in group III compared with group II and controls. CONCLUSIONS: In BA, sICAM-1 and sVCAM-1 levels could be useful as markers of end-stage liver disease, with sVCAM-1 being more specific for PH. Induction of ICAM-1 and VCAM-1 may be an important factor in the development of cirrhosis.

Adolescent↗

Ectopic distal location of the papilla of vater in congenital biliary dilatation: Implications for pathogenesis.

BACKGROUND/PURPOSE: This study investigates the relationship between the location of the papilla of Vater and the length of the common channel in patients with congenital biliary dilatation (CBD). METHODS: Cholangiograms from 121 CBD patients and 13 normal controls were the subjects for this study. A length index defined as the length of the common channel divided by the height of the second lumbar vertebra was used for standardization. RESULTS: In the controls, the papilla of Vater was located in the middle of the descending or second part of the duodenum in all cases. In 39 (32.2%) of the 121 CBD patients papilla of Vater was located in the descending duodenum (group I), and in 82 (67.8%) it was distal to the descending duodenum (group II). The average length index of the common channel in group II was significantly longer than in group I (1.123 +/- 0.374 v 0.660 +/- 0.246; P <.001). Findings for the common bile duct were similar. CONCLUSIONS: There is a significantly higher incidence of ectopic distal location of the papilla of Vater in CBD patients than in controls. The more distal the location of the papilla of Vater, the longer the common bile duct and the common channel.

Adolescent↗

Laparoscopic muscle electrostimulation during laparoscopy-assisted anorectal pull-through for high imperforate anus.

PURPOSE: Our technique for laparoscopic muscle electrostimulation during laparoscopy-assisted anorectal pull-through (LAARPT) for high imperforate anus (HIA) in 3 patients is described. METHODS: The distal rectum and rectourethral fistula is dissected laparoscopically. A muscle stimulator is passed through one of the trocars and used to identify the center of contraction of the levator ani. The same muscle stimulator is used to identify the center of the external sphincter muscle transcutaneously. An intravenous cannulation device (SURFLO Flash IV catheter, TERUMO, CO, Yamanashi, Japan) is inserted through this proposed anus and observed piercing the center of the levator ani. A guide wire is passed through the SURFLO, and a series of dilators are passed along it to create a canal for the colonic pull-through. An anoplasty then was performed. RESULTS: Our technique was successful in all patients. Laparoscopic electrostimulation produced good levator ani contraction in patients I and II and weak contraction in patient III. Patients I and II have symmetrical anal contraction during rectal examination, but patient III has poor contraction. Stool frequency is decreasing in all. CONCLUSION: Direct laparoscopic observation of levator ani contraction allows intraoperative assessment of functional contractility and assists in the accurate placement of the colonic pull-through.

Anal Canal↗

Urinary trypsin inhibitor and biliary atresia--providing protection for the liver?

Urinary Tyrosine Inhibitor (UTI) is produced in the liver and excreted in urine hepatic inflammation, infection or malignancy. We assess the possible implications of UTI in biliary atresia (BA). Liver function was used to divide 34 postoperative BA patients into 2 groups: Group 1 (n=25), anicteric (total bilirubin [T-Bil] <2.0 mg/dl); and Group 2 (n = 9), icteric (total bilirubin >2.0 mg/dl) with abnormal liver function test results, and repeated episodes of cholangitis. 26 age-matched subjects with no history of liver disease acted as controls

Adolescent↗

Magnetic resonance cholangiopancreatography in assessing the cause of acute pancreatitis in children.

Magnetic resonance cholangiopancreatography (MRCP) is a new noninvasive method of obtaining images of the pancreaticobiliary tract. Recent advances in MR technology and image quality have made it easy to diagnose structural abnormalities of the pancreaticobiliary tract (SAPBT) in children. To examine the usefulness of MRCP in assessing the cause of acute pancreatitis in children, we performed MRCP in 16 patients with acute pancreatitis. The study population was divided into two groups according to the cause of acute pancreatitis as follows: group 1 consisted of seven patients sonographically diagnosed with choledochal cysts; and group 2 consisted of nine patients with no obvious cause of acute pancreatitis. Non-breath-hold MRCP using the half-Fourier, single-shot, fast spin-echo imaging method was performed within 7 days after the onset of pancreatitis. Abnormal union of the pancreaticobiliary junction was detected in six of seven group 1 patients and in one of nine group 2 patients. Pancreatic divisum was detected in one patient of group 1, but could not be confirmed in one patient of group 2. Dilatation of the main pancreatic duct was detected in one patient of group 1 and in three patients of group 2. Our results suggest that MRCP is a useful, noninvasive method of identifying and ruling out SAPBT as a cause of acute pancreatitis in children with early-stage pancreatitis.

Acute Disease↗

Bladder transplantation in rats using FK-506.

PURPOSE: We created viable bladder tissue by transplantation with immunosuppression. MATERIALS AND METHODS: For bladder transplantation the bladder of newborn Brown-Norway rats was excised and each was transplanted into a pouch created in the distal omentum of a 5-week-old Lewis rat. In 15 group 1 rats no immunosuppressive agent was used. In 20 group 2 rats 0.6 mg./kg. FK-506 daily were given intramuscularly until a predetermined day of harvest. Recipient rats were sacrificed on day 3, 5, 7 or 14 after bladder transplantation, and the bladder grafts were harvested and formalin fixed. Hematoxylin and eosin staining was done to examine bladder graft survival and the degree of rejection, and immunohistochemical testing was performed for assessing the vesical nervous system. In 5 rats in the control group bladder augmentation was performed by anastomosing the bladder graft to the native bladder. Each augmented bladder was harvested 21 days later for histopathological assessment. RESULTS: Overall bladder graft survival was 96.4%. Each successfully transplanted bladder graft appeared macroscopically as a thin walled cyst. In group 1 all bladder grafts showed rejection with cellular infiltration. In group 2 there was mild rejection in 5 rats and no evidence of rejection in the remaining 15. All group 2 bladder grafts had intact nerve distribution. Bladder augmentation was successful in all 5 cases and the mucosa was normal throughout each augmented bladder. CONCLUSION: Because FK-506 successfully prevents rejection, our technique would appear to have the potential for creating viable bladder tissue that may be used for bladder augmentation in cases of vesical exstrophy or neurogenic bladder.

Animals↗

alpha-Glutathione-s-transferase as a new sensitive marker of hepatocellular damage in biliary atresia.

Early identification of patients likely to deteriorate post-hepatic portoenterostomy for biliary atresia (BA) would be beneficial. alpha-Glutathione-s-transferase (alpha-GST) is a serologic marker of reactive hepatocellular damage because of its low molecular weight, uniform hepatic distribution, high cytosol concentration, and short half-life. We evaluated whether serum alpha-GST in post-surgical BA patients correlates with liver function (LF) and investigated its potential as a medium- to long-term marker of prognosis. Postoperative BA patients (n = 30; mean age: 11.8 +/- 3.7 years) were divided into three groups based on average LF over the 3 months prior to this study. Group I (n = 8) were jaundice-free and had normal LF. Group II (n = 12) had moderate liver dysfunction, and group III (n = 10) had severe liver dysfunction. Serum alpha-GST was determined using a specific ELISA. Tissue alpha-GST was determined immunohistochemically, using liver needle-biopsy specimens. Bile lakes were found in 5 group II patients and 5 group III patients. Serum alpha-GST was significantly higher in group II (20.7 +/- 8.4 ng/ml) than in groups I (4.7 +/- 1.3 ng/ml) and III (8.0 +/- 1.2 ng/ml) (P < 0.0001) and was highest in group II subjects with bile lakes. In control liver specimens alpha-GST distribution was weak but uniform throughout normal liver lobule hepatocytes. In group II there was strong staining in centrilobular hepatocytes, and in group III alpha-GST was only found in regenerative nodules. We conclude that alpha-GST may be a more sensitive indicator of hepatocellular damage in BA because its distribution is correlated to the proportion of functioning liver tissue present. This is the first report of this relationship, which has great implications for group II subjects because a sudden shift in concentration of alpha-GST may be a better predictor of impending hepatic dysfunction than conventional LF tests.

Adolescent↗

Histology of the neobladder mucosa after sigmoidocolocystoplasty.

PURPOSE: The aim of this study was to examine the histopathology of neobladder mucosa biopsy specimens obtained routinely as part of postsigmoidocolocystoplasty (SCP) follow-up. METHODS: One hundred cases of SCP (mean age at surgery, 10.6 years) performed by the authors were examined for the presence of dysplasia or malignant changes in the mucosa of the neobladder using H&E and proliferating cell nuclear antigen (PCNA) staining. RESULTS: No dysplastic or malignant changes were identified in any case. Metaplasia was found in 5 cases and hyperplasia in 2. There were no major differences found on H&E and PCNA staining of specimens obtained after different periods of follow-up post-SCP; follow-up was short term (up to 5 years) in 44 cases, medium term (from 5 to 10 years) in 48 cases, and long term (over 10 years) in 8 cases. PCNA staining was significantly more intense in subjects who stopped regular bladder irrigations (BI) post-SCP and in subjects in whom bladder stones developed (P < .05; Welch's t test), compared with subjects who continued BI and subjects in whom bladder stones did not develop. CONCLUSIONS: After SCP, patients are advised to continue BI. Regular biopsies should be part of routine follow-up, especially in subjects with bladder stones.

Adolescent↗

Pyloromyotomy versus atropine sulfate for infantile hypertrophic pyloric stenosis.

PURPOSE: Atropine sulfate (atropine) and pyloromyotomy were compared for managing infantile hypertrophic pyloric stenosis (IHPS). METHODS: From 1996 to 1998, cases of IHPS treated surgically (pyloromyotomy; n = 20) or medically (atropine; n = 14) at separate institutions were compared retrospectively with regard to status on presentation, physical symptoms and signs, progress, and costs. Atropine was given orally, then intravenously if ineffective. Refractory cases were referred for pyloromyotomy. RESULTS: All subjects were matched for clinical and physiological status on admission. Oral atropine alone was effective in 11 cases, was converted to intravenous atropine in 2 cases, and was terminated in 1 case because of hematemesis. Two cases were referred for pyloromyotomy. All pyloromyotomies were successful. Atropine took on average, 2.6 days to take effect. The difference in time taken for normalization of pyloric muscle thickness between the 2 groups was not significant. Average time to return to full feeding was longer in the atropine group (P<.01). Costs were lower in the atropine group (P<.01). There were 2 wound infections in the pyloromyotomy group, but no adverse effects of atropine. There were no recurrences in either group. CONCLUSION: This study provides reasonable evidence to support a trial of atropine in IHPS.

Adult↗