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Biomedical subjects

R Ohi

Publications and source records attributed to R Ohi.

At least 55 records · Page 3Linked to original sources
PubMed1997

Clinical significance of 99mTc-DTPA galactosyl human serum albumin scintigram in follow-up after Kasai operation.

The scintigram using 99mTechnetium-DTPA galactosyl human serum albumin (99mTc-GSA) which binds to asialoglycoprotein receptors on hepatocytes is a good index of hepatocyte function in various liver diseases in adult patients. In 43 patients (4 months to 30 years old) who had undergone Kasai procedure, we performed 53 series of 99mTc-GSA scintigrams and checked the laboratory data of blood draw and the clinical status. The indices for blood clearance and liver accumulation were evaluated on the basis of the dynamic data after 99mTc-GSA injection. HH15 as an index of the blood clearance, and LHL15 as an index of the accumulation of the hepatocytes were calculated and the HH15/LHL15 ratio (H/L15) was examined. 99mTc-GSA scintigram correlated with liver function and clinical status. Our results revealed that 1) The deterioration of the liver functions and clinical status correlates proportionally with H/L15, 2) The results of 99m Technetium-GSA scintigram correlate with several liver function tests, especially direct bilirubin, albumin and choline esterase, 3) This scintigram is an useful index of clinical status and hepatic function as well as the change of the hepatic parenchymal reserve in BA patients, especially for the evaluation of liver transplantation.

Adolescent↗
PubMed1997

Assessment of nutritional status of postoperative patients with biliary atresia.

Some patients of biliary atresia (BA) suffer from chronic hepatic dysfunction and/or persistent jaundice. The adverse effects of chronic liver disease on nutrition and growth should be considered on BA patients. We studied 45 BA patients ranging in age from 0.5 to 38 years and divided them into 2 groups. Group A contains the patients whose total bilirubin > or = 2 mg/100 ml, and Group B contains the patients whose total bilirubin < 2 mg/100 ml. We measured height, weight, triceps skin fold (TSF), midarm circumference (MAC) and midarm muscle area (MAMA). Visceral protein kinetics was evaluated on the basis of serum albumin and prealbumin levels. Caloric and protein intake was calculated by collecting intake data for 3 days. The results of this study were; 1) The mean TSF in Group A (47th percentile) was not significantly different from that in Group B (53th percentile). 2) The mean MAMA was significantly lower (p < 0.01) in Group A (16.4th percentile) than in Group B (36.7th percentile) 3) The prealbumin level was significantly lower (p < 0.001) in Group A (mean 9.9 mg/100 ml) than in Group B (mean 18.8 mg/100 ml). The authors conclude that the evaluation of MAMA and prealbumin were very useful to characterize the low metabolic status of protein in the damaged liver. And repeated nutritional assessment was necessary to evaluate liver function and provide adequate nutrition in BA patients.

Adolescent↗
PubMed1997

The outcome of surgery for biliary atresia and the current status of long-term survivors.

Between 1953 and 1995, 300 patients with biliary atresia underwent surgery at Tohoku University Hospital. The 10-year survival of patients who were operated on in or before 1965 was 9%. But the survival rate went up to 61% in patients operated on between 1976 and 1985. Eighty-five patients including 2 who developed liver failure after Kasai operation and underwent liver transplantation have survived more than 10 years. Eleven of them (13%) have recurrent or persistent jaundice. Of the 30 patients who have survived more than 20 years (10 males and 20 females, age range; 20 to 41 years), 20 underwent hepatic portoenterostomy, 8 underwent hepaticoenterostomy and the remaining 2 underwent hepatic portocholecystostomy. None of these patients has undergone liver transplantation. Twenty-two patients have led near-normal lives. The remaining 8 patients have experienced some troubles due to cholangitis, portal hypertension, intrahepatic gallstones and so on. Two of them are considered as candidates for liver transplantation. While the majority of long-term survivors of biliary atresia have good quality of life, close long-term follow-up is essential even in patients with biliary atresia aged 20 years or more.

Adolescent↗
PubMed1997

Abnormal distribution of nerve fibers in the liver of biliary atresia.

We investigated changes in the pattern of hepatic innervation in liver specimens from 15 infants with biliary atresia and 4 age-matched controls by immunohistochemical methods. In the control, nerve fibers identified by immunoreactivity for neural cell adhesion molecule (NCAM) and S100 protein were present around the branches of hepatic arteries, portal veins and bile ducts in the portal areas and the hepatic lobules. In biliary atresia, NCAM and S100 positive nerve fibers were increased in the vicinity of the hepatic arteries and the portal veins in the enlarged portal areas, while no nerve fibers were observed around bile ducts and periportal ductules which became NCAM positive. No innervation in the lobules was seen in any cases regardless of the histological alteration. These findings may suggest that the abnormal innervation in the liver with biliary atresia does not occur as a result of structural changes in liver architecture caused by portal fibrosis and inflammation, but is associated with immaturity or malformation of hepatic innervation in the patients.

Bile Ducts↗
PubMed1997

Japanese Biliary Atresia Registry, 1989 to 1994.

The Japanese Biliary Atresia Society founded in 1980 for the aim of investigations of all aspects of biliary atresia (BA), started a nationwide registry of BA patients in 1989. A total of 626 cases were registered from 1989 to 1994. The male to female ratio was 0.58. Corrective surgery was performed in 603 patients. Regarding the type of obstruction, 63 cases were Type I, atresia of the common bile duct, 9 were Type II, atresia of the hepatic ducts, and 543 were Type III, atresia of the porta hepatis. As initial corrective procedures, original Roux-en Y, Suruga II and Roux-en Y with intestinal valve were mainly employed. Jaundice cleared in 346 patients (57%) and decreased in 131, while it persisted in 120. The 5-year-follow-up showed that 34 patients, 49% of the patients who were followed up, were alive without jaundice, while 28 (41%) are dead. Thirty five, 33% of the patients who were entered to the Registry, were lost to follow-up.

Biliary Atresia↗
PubMed1996

Current status of 21 patients who have survived more than 20 years since undergoing surgery for biliary atresia.

Between 1952 and 1993, 289 patients with biliary atresia underwent surgery at the authors' institution. Twenty-two of them survived more than 20 years; one has since died of hepatic failure (at age 28 years). Of the 21 current survivors (age range, 20 to 39 years), 13 underwent hepatic portoenterostomy; the others had hepaticoenterostomy. None of these patients has undergone liver transplantation. Sixteen patients have led near-normal lives. This includes three married women, one of whom has given birth to a healthy baby boy. Of the six patients who had portal hypertension, three underwent both splenectomy and proximal splenorenal shunting in or before 1985. None of these patients has required additional treatment for portal hypertension. The quality of life of one patient has been severely affected by an unrelated condition (Turner's syndrome). A 22-year-old man was diagnosed as having intrahepatic stones 3 years ago. In another 22-year-old man, hepatic dysfunction developed after frequent episodes of cholangitis. He is now being considered for liver transplantation. The majority of the long-term survivors have good quality of life. However, a few continue to suffer from complications including recurrent cholangitis. Close long-term postoperative follow-up is required for patients with biliary atresia.

Adult↗
PubMed1996

The structure of the transitional and aganglionic zones of Auerbach's plexus in patients with Hirschsprung's disease: a computer-assisted three-dimensional reconstruction study.

Intestines resected from two patients with Hirschsprung's disease, both aged 6 months, were submitted to serial sectioning and three-dimensional reconstruction to visualize the overall structure of Auerbach's plexus and correlate its changes with functional abnormalities. Reconstruction was made possible by using a graphics computer system. In normal intestines taken from an autopsy case, both large and small, the plexus was shown extending along the intermuscular septum as a regular network. But in the large intestine, the density of the network was apparently higher and the individual bundles thicker than in the small intestines. This neural network was absent in the aganglionic zone in Hirschsprung's disease, as expected, where only hypertrophic extrinsic nerves were running in the septum. The "transitional zone" of Hirschsprung's disease was clearly definable as an area extending over a certain length, where the network meshes grow more irregular and the bundles taper more, toward the aganglionic zone. This finding was considered to be of profound significance in the design of a surgical strategy to prevent postoperative bowel dysfunction. In determining the range of resection, one must consider not only the presence or absence of nerve cells but also the grade of plexus hypoplasia.

Biopsy↗
PubMed1996

[Long-term outcome of surgery for biliary atresia].

Between 1953 and 1995, 300 patients with biliary atresia underwent surgery at Tohoku University Hospital. Among them, 31 patients survived more than 20 years, while one of these patients died of hepatic failure at the age of 28 years. Of the 30 surviving patients (10 males and 20 females, age range; 20 to 41 years), 20 underwent hepatic portoenterostomy, 8 underwent hepaticoenterostomy and the remaining 2 underwent hepatic portocholecystostomy. None of these patients has undergone liver transplantation. Twenty-two patients have led near normal lives. This includes three married women, one of whom gave birth to 2 healthy babies. The remaining 8 patients have had experienced some troubles due to cholangitis, portal hypertension and intrahepatic gallstones. Two of them who have progressive liver dysfunction are being considered as candidates for liver transplantation. The quality of life of one patient has been severely affected by an unrelated (Turner's syndrome). While the majority of long-term survivors of biliary atresia have good quality of life, close long-term postoperative follow-up is required.

Adult↗
PubMed1995

Right pneumonectomy syndrome: report of two cases.

We report herein the cases of two infants who developed right pneumonectomy syndrome, both of whom were born with gross C-type esophageal atresia (EA/TEF), and a hypoplastic right lung arising from the lower esophagus, being a bronchopulmonary foregut malformation (BPFM). Appropriate and well-timed treatments for a variety of sequelae primarily caused by the mediastinal shift must be considered after right pneumonectomy in early childhood.

Bronchi↗
PubMed1995

Problems of neuroblastoma screening for 6 month olds and results of second screening for 18 month olds.

Nationwide neuroblastoma mass screening for 6-month-old infants (first screening) was introduced in Japan in 1985. About 110 neuroblastoma cases are detected annually by the first screening and treated, with a survival rate of 97%. Sensitivity of the first screening (positive cases/positive cases+false negative cases) is about 75%, and the prognosis of false-negative cases is unfavorable. A second screening at 18 months of age was started to rescue false-negative cases in Miyagi Prefecture in May 1992. Of 62 neuroblastoma cases treated in our hospital since 1985, 40 cases had received the first screening. Twenty cases were positive at first screening, 18 cases were false negative, and 2 cases were false negative and picked up by the second screening. Age distribution of false-negative cases ranged from 12 to 83 months and included 12 cases younger than 36 months old. Only 5 of 18 false-negative cases are alive without the disease. From May 1992 to November 1993, 14,282 infants had received the second screening (compliance rate: about 75%), and 2 neuroblastoma cases were detected. The first case was stage III with paraortic lymph node metastases, Shimada UH, aneuploidy and negative N-myc amplification. The second case was stage II with Shimada FH, aneuploidy, and negative N-myc amplification. Both cases are alive now without the disease after undergoing radical operation and chemotherapy. The first screening is effective for early detection of neuroblastoma cases, but the sensitivity is insufficient; the authors recommend a second screening to rescue false-negative cases.

Adrenal Gland Neoplasms↗
PubMed1995

[Familial neuroblastoma].

Neuroblastoma (NB) is a common malignant solid tumor in childhood, accounting for about 40% of the solid children's neoplasms on the Japanese registry in 1993. It is known that some cases of NB are hereditary, but their incidence is very low. We report two patients with adrenal familial NBs and review the literature on familial NB. Two hit theory was proposed to explain the occurrence of hereditary and non-hereditary NBs. This theory supports the fact that the median age at diagnosis is much younger and the incidence of multiple primaries is higher in the familial NB than in non-familial cases. As therapy for NB improves and more children survive to adulthood, there will be more opportunity to study their offspring.

Adrenal Gland Neoplasms↗
PubMed1994

The Schizosaccharomyces pombe cdc5+ gene encodes an essential protein with homology to c-Myb.

The Schizosaccharomyces pombe cdc5+ gene was identified in the first screen for cell division cycle mutants in this yeast. The cdc5+ gene was reported to be required for nuclear division but because of its modest elongation and leaky nature at the non-permissive temperature, it was not investigated further. Here, we report the characterization of the single allele of this gene, cdc5-120, in more detail. The mutant arrests with a 2N DNA content and a single interphase nucleus. Further genetic analyses suggest that cdc5+ gene function is essential in the G2 phase of the cell cycle. We have cloned and sequenced the cdc5+ gene. The deduced protein sequence predicts that Cdc5 is an 87 kDa protein and contains a region sharing significant homology with the DNA binding domain of the Myb family of transcription factors. Deletion mapping of the cdc5+ gene has shown that the N-terminal 232 amino acids of the protein, which contain the Myb-related region, are sufficient to complement the cdc5ts strain. A cdc5 null mutant was generated by homologous recombination. Haploid cells lacking cdc5+ are inviable, indicating that cdc5+ is an essential gene. A fusion protein consisting of bacterial glutathione S-transferase joined in-frame to the N-terminal 127 amino acids of the Cdc5 protein is able to bind to DNA cellulose at low salt concentrations. This evidence suggests that cdc5+ might encode a transcription factor whose activity is required for cell cycle progression and growth during G2.

Amino Acid Sequence↗
PubMed1994

The effect of hepatic portal dissection on the portal vein structure in biliary atresia.

Superior mesenteric portograms were performed on 30 patients with biliary atresia (BA) at the time of initial portoenterostomy in 20, and at the stoma closure operation in 10. A withered-branch-shaped irregularity of the intrahepatic portal vein (PV) and collateral vessels were seen in 2 of 11 patients with portal pressures (PP) of 200 to 300 mmH2O; in 1 of 2 patients with PPs of over 300 mmH2O at the initial operation; and in 3 jaundice-free patients with PPs of 285, 320, and 305 mmH2O, respectively, at the stoma closure operation. Collaterals were the only abnormalities seen in two additional jaundice-free patients with PPs of 370 and 183 mmH2O, respectively. No anatomic changes in the extrahepatic PV at the porta hepatis were found on the portograms of either group of patients. Thus, we conclude that portal dissection itself does not affect the PV structure anatomically, a finding which has important implications in determining whether or not portoenterostomy adversely affects potential liver transplantation.

Biliary Atresia↗
PubMed1993

Pituitary adenylate cyclase activating polypeptide (PACAP)-like immunoreactivity in ganglioneuroblastoma and neuroblastoma.

Pituitary adenylate cyclase activating polypeptide (PACAP) is a 38 amino acid peptide originally isolated from ovine hypothalamus. It has a potent stimulatory action on adenylate cyclase in the rat pituitary. The presence of PACAP was studied in the tumor tissues of ganglioneuroblastoma and neuroblastoma by radioimmunoassay and immunocytochemistry. Immunocytochemical studies showed positive immunostaining in 4 out of 7 ganglioneuroblastomas and 4 out of 6 neuroblastomas. Immunoreactive PACAP concentrations in tissues of 3 ganglioneuroblastomas ranged from 14.5 to 27.8 pmol/g wet weight (20.0 +/- 5.7 pmol/g wet weight, mean +/- S.D.) and the concentration in one neuroblastoma tissue was 111.0 pmol/g wet weight. Reverse phase high performance liquid chromatography of the tumor tissue extract of ganglioneuroblastoma showed a peak eluting in the position of PACAP1-38 and smaller broad peaks eluting later. These results indicated that high concentrations of immunoreactive PACAP were present in the tumor tissues of ganglioneuroblastoma and neuroblastoma, and suggest the possibility that this peptide plays a pathophysiological role in some ganglioneuroblastomas and neuroblastomas.

Child↗
PubMed1992

Bannayan-Zonana syndrome associated with lipomas, hemangiomas, and lymphangiomas.

Bannayan-Zonana syndrome is a rare disorder characterized by macrocephaly and multiple soft tissue and visceral hamartomas. This report presents a sporadic patient with macrocephaly, lipomas, hemangiomas, and lymphangiomas who died of cardiac and respiratory failure due to progressive cervicomediastinal arteriovenous fistulous hemangiomas at the age of 9 years.

Abnormalities, Multiple↗
PubMed1992

Late complications in long-term survivors of biliary atresia.

Ninety patients with biliary atresia surviving more than 5 years were analysed with respect to late complications occurring after the age of 4 years. Thirty-five had complications including cholangitis, portal hypertension, hypersplenism, gastrointestinal bleeding, and esophageal varices. These complications occurred at various times. The background factors of late complications were past history of cholangitis soon after the operation, advanced age at operation, re-operation, high portal pressure at initial operation, and a long interval before disappearance of jaundice after surgery. These factors are mostly related to the first operation and its postoperative course. Therefore, we stress that late complications can be prevented by intensive treatment of the patient at the time of the first operation.

Adolescent↗