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Biomedical subjects

R Ohi

Publications and source records attributed to R Ohi.

At least 91 records · Page 5Linked to original sources

Surgical limitation for biliary atresia: indication for liver transplantation.

Of 245 patients undergoing corrective operations for biliary atresia, jaundice was cleared in 113. In January 1988, 84 of them were living and free of jaundice and the other 19 were alive with jaundice. A vast majority of long-term survivors showed normal growth and development, and were leading normal lives for their respective ages. Portal hypertension, a common late complication, improved spontaneously or after sclerotherapy in jaundice-free patients. Therefore, liver transplantation is not recommended for jaundice-free patients even with esophageal varices. Patients with persistent severe jaundice (serum bilirubin over 10 mg/dL) and those with moderate jaundice (serum bilirubin 5 to 10 mg/dL) and severe esophageal varices require liver transplantation. Patients with moderate jaundice having no or slight varices should be carefully followed. When varices become worse or serum bilirubin rises, liver transplantation is indicated. Patients with mild jaundice (serum bilirubin lower than 5 mg/dL) have a possibility of improvement in their condition before the age of 15 years, and are not recommended for liver transplantation. The high value of the lowest postoperative bilirubin level suggests the necessity of liver transplantation in early childhood. Liver transplantation as the primary treatment for biliary atresia may be indicated only for patients over 120 days of age with an enlarged and hard liver.

Adolescent↗

Bronchopulmonary foregut malformation in 3 infants--with special references to cases in childhood.

We encountered three infant patients with bronchopulmonary foregut malformation. Two of them were associated with oesophageal atresia, and the remaining one with jejunal atresia. Two of the patients showed total lung sequestration, and the rest showed intralobar sequestration. 37 paediatric cases of this type were reviewed from the literature. Total lung sequestration was observed in 19 of the 37 cases, showing an increase in the incidence in comparison with that reported previously. There were also 7 cases associated with oesophageal atresia.

Abnormalities, Multiple↗

Effect of VAB-6 combination chemotherapy on malignant germ cell tumours in childhood.

The VAB-6 protocol was applied to 5 children with malignant germ cell tumour. Four of them achieved complete remission after 3 inductions of this protocol and surgery. Death in one case was attributed to chemotherapy. Complete responders have been in remission now for 4 months to 2 years without maintenance chemotherapy. Adverse effects including myelosuppression, ototoxicity, cardiotoxicity and pulmonary toxicity were observed. The VAB-6 protocol is an effective chemotherapy in children with malignant germ cell tumour and requires only 3 to 4 months of treatment.

Adolescent↗

Long-term follow-up study of patients with cholangitis after successful Kasai operation in biliary atresia: selection of recipients for liver transplantation.

Since the introduction of cyclosporine A, liver transplantation has become accepted as the therapy for end-stage liver disease. However, there are no definite criteria for liver replacement in biliary atresia. We investigated (a) the survival rate after hepatic portoenterostomy (n = 131), (b) liver function tests in fatal cases after an initially successful hepatic portoenterostomy (n = 9), and (c) liver function tests in the patients with episodes of cholangitis after a successful surgical treatment (n = 8). Patients with persisting jaundice after the surgery cannot be expected to survive long, and therefore they definitely should undergo liver transplantation. When total bilirubin concentration was above 10 mg/dl in patients with cholangitis after a successful operation, conservative therapy had almost no effect. Therefore, patients with total bilirubin levels above 10 mg/dl should be considered for liver transplantation. Of the liver function tests, only total bilirubin was reliable as a marker for hepatic failure in the end stage of biliary atresia. Prolongation of thrombo test and episodes of gastrointestinal bleeding also were used in selection of patients for liver replacement.

Biliary Atresia↗

Neuropeptide Y- and somatostatin-like immunoreactivities in ganglioneuroma, ganglioneuroblastoma and neuroblastoma.

Neuropeptide Y (NPY)- and somatostatin (SS)-like immunoreactivities (LI) were investigated in tumor tissues of one ganglioneuroma (GN), 3 ganglioneuroblastomas (GNB) and one neuroblastoma (NB) by radioimmunoassay. NPY-LI was detected from all 5 tumor tissues (16.4-1247 pmol/g wet tissue). Sephadex G-50 column chromatography and reverse phase high performance liquid chromatography (HPLC) revealed that most of the NPY-LI in tumor extracts was eluted in an identical position to synthetic human NPY except one GNB (case 2). In this case, most of the NPY-LI was eluted in a higher molecular weight region than synthetic human NPY in Sephadex G-50 column chromatography and in a more hydrophobic position in HPLC. SS-LI was detected from 4 tumor extracts except one GNB (case 2) (21.3-787 pmol/g wet tissue). Sephadex G-25 column chromatography and reverse phase HPLC revealed that SS-LI in tumor extracts was eluted just after the void volume and then in the same positions as SS-28 and SS-14. These results suggest that NPY, SS-14 and SS-28 exist in tumor tissues of GN, GNB and NB, and most of the NPY-LI in one GNB was a higher molecular and more hydrophobic form of NPY-LI.

Chromatography, Gel↗

[The present status and problems in the treatment of biliary atresia with special reference to surgical and long-term results].

The surgical results are not satisfactory yet in Japan and only 46% of the patients are free of jaundice 3 years ago. However, we found many presentations which reported jaundice disappearance rate with more than 80% in the last several years. The main cause of death in jaundiced patients with the failed hepatic portoenterostomy was hepatic failure. Other causes of deaths in these patients, including those died within one month after operation, were peritonitis, cardiac and/or renal failure, hemorrhagic tendency and lung edema. The main cause of death in jaundice-free patients was rupture of the esophageal varices. We examined 48 long-term survivors ranging in age from 10 to 33 years. There were 41 cases without and 7 with jaundice. Thirty-seven (77%) of 48 cases were leading their normal lives. Among them, 6 cases were enjoying their lives after overcoming the sequelae, such as portal hypertension. The morbidities of the remaining 11 (23%) long-term survivors were jaundice in 7, portal hypertension in 5, encephalopathy after splenorenal shunt in 1 and so on. The cured states of most cases without jaundice are satisfactory and eventually these former patients can achieve a favorable quality of life.

Adolescent↗

A patient with biliary atresia who died 28 years after hepatic portojejunostomy.

A patient who died of hepatic failure nearly 29 years after hepatic portojejunostomy is reported. His death may be attributed to several faults in treatment. From our experiences it can be said that biliary atresia is a curable disease, although liver-transplantation is required in some patients in whom hepato portoenterostomy is unsuccessful.

Adult↗

Persistent jaundice after hepatic porto-jejunostomy in biliary atresia: are the patients' prognoses determined within 3 months after surgery?

Eighteen patients with biliary atresia who showed persistent jaundice after hepatic porto-jejunostomy were studied clinically. It was revealed that: 1) Patients who had abnormally high preoperative values of laboratory data showed persistent jaundice; 2) high portal pressure occurred frequently in patients with persistent jaundice; 3) most patients of persistent jaundice had a history of postoperative cholangitis; 4) in contrast to jaundice-free patients, those with persistent jaundice tended to show elevation in gamma-globulin, TTT and gamma-GTP in the early postoperative period. These results suggest that for patients showing an upward tendency in these laboratory data after surgery, carefully management should be taken, even if the serum bilirubin level continue to decrease.

Adolescent↗

[Nutritional condition and absorptive capacity of 14 infants with short bowel syndrome].

We examined the absorptive capacity and the nutritional condition of 14 infants with short bowel syndrome, whose residual small intestine was 90 cm or less. Their age ranged from 1 year to 18 years. Examined items were body weight, height, serum albumin, total cholesterol, triglyceride, fat soluble vitamins, trace elements and rapid turn over protein as markers of the nutritional condition. Fecal fat, fecal bile acid, d-xylose absorption test, sugar-, amino acid-evoked potential difference in the small intestine and disaccharidase activity of the mucosa were examined as markers of the absorptive capacity. Our results showed that the body weight was below the normal range in the patients with small intestine of less than 50 cm. Most nutritional markers were within normal range, however, cholesterol and vitamin D were low in the patients with fat malabsorption, especially in patients with less than 50 cm of small intestine. Fecal bile acid was higher than the normal range in all the patients. Potential difference was in normal range or slightly lower than normal in all the patients. We concluded that infants with less than 50 cm of small intestine had malabsorption of sugar, protein and fat. Therefore, prolonged nutrient support, especially fat, is necessary.

Adolescent↗

Developmental change of interrelationship between sugar-, amino acid- or peptide-evoked potential and influx across the mucosal border in the small intestine.

In this study, the electrical resistance of guinea pig small intestine was examined during postnatal development in vitro. The interrelationship between sugar-, neutral amino acid- or peptide-current calculated from potential difference and their influxes across the mucosal border of the small intestine was also investigated during postnatal development. At all stages from one week to adult animal, the electrical resistance remained at nearly the same value. Currents of glucose, maltose and lactose corresponded with influxes at any stage and these sugars were completely carried by an active transport system from the period of lactation. Glycine might be actively transported at any stage of development. Therefore the transmural potential differences induced by these substances are adequate indices of intestinal absorptive capacities. On the other hand, glycyl-glycine transport was independent of Na+ and glycyl-glycine induced PD does not reflect the influx.

Animals↗

Significance of serum lipoprotein-X and gammaglutamyltranspeptidase in the diagnosis of biliary atresia. A preliminary study in 27 cholestatic young infants.

As simple and nonsurgical means of differentiating biliary atresia (BA) from intrahepatic cholestasis of unknown origin (IC), liver function tests including serum lipoprotein-X (LP-X) and gamma-glutamyltranspeptidase (GGTP) were done and evaluated for their usefulness in the diagnosis of 27 cholestatic Japanese young infants. Except for LP-X and GGTP levels (P less than 0.01, P less than 0.001), there were no significant differences between the BA (n = 11) and IC (n = 13) groups. When values of mean plus 4 standard deviations were used to differentiate BA from IC (89 mg/100 ml for LP-X and 194 IU/l for GGTP), all BA patients gave positive results for either the critical LP-X of GGTP values. On the other hand, all IC patients gave negative results for both levels, although patients with a paucity of intrahepatic biliary ducts (n = 3) were also positive for either the critical LP-X or GGTP values. The combination test with serum LP-X and GGTP is recommended for helping to differentiate BA from IC in cholestatic young infants.

Bile Ducts↗

Postoperative changes of intrahepatic portal veins in biliary atresia--a 3-D reconstruction study.

Intrahepatic portal veins were reconstructed from serial histologic sections of surgical liver specimens from patients with biliary atresia to explain the pathogenesis of portal hypertension, an important late postoperative complication of this disease. It was found that abnormal intrahepatic vasculature was already notable in an evidently reduced number of portal veins in the second month of age, and this was considered responsible for causing presinusoidal block that gave rise to the elevated resistance to intrahepatic portal flow. Reconstruction also revealed that restoration of ordinary portal structure was expectable if, after successful surgery, a patient was sufficiently protected from cholangitis and there was much room for portal veins to grow without being impeded by inflammatory and/or scarring processes. In contrast, the patient whose condition was complicated by cholangitis was shown to acquire a markedly hypoplastic portal system with apparently reduced vascularity.

Bile Ducts↗

Portal hypertension after successful hepatic portoenterostomy in biliary atresia.

From 1953 through 1984, we have operated on 225 cases of biliary atresia, and 95 patients are presently surviving. Portal hypertension with esophageal varices was endoscopically confirmed in 26 of 66 patients (39%) examined, 14 with and 52 without jaundice. All these patients except two had had frequent episodes of postoperative cholangitis. Eight patients have undergone treatment for portal hypertension. The treatment for variceal bleeding in jaundice-free infants with biliary atresia should be initiated conservatively, including endoscopic sclerotherapy. The results of our experience, however, justifies the employment of shunt procedures for patients older than 6 or 7 years of age.

Adolescent↗

Reoperation in patients with biliary atresia.

Twenty-seven reoperations were done on 23 patients among 100 infants with biliary atresia who have been treated at Tohoku University Hospital between 1971 and 1981. Nineteen patients had a single reoperation and 4 patients had 2 reoperations. We present the results and the role of reoperation in biliary atresia patients in our institution. Excellent bile drainage after reoperation was obtained in 13 of 15 patients with good bile flow after the initial operation. On the contrary, good bile drainage was not obtained by reoperation in 8 of 12 cases without active bile flow after the initial operation. Cessation of bile flow after successful initial operation is an absolute indication for reoperation. Aggressive reoperations under proper indications improve the surgical results in biliary atresia patients.

Age Factors↗

Changes of intrahepatic bile duct dilatation after surgery for congenital dilatation of the bile duct.

Of 47 patients suffering from congenital dilatation of the bile duct whose intrahepatic bile ducts could be clearly demonstrated by operative cholangiogram or preoperative ERCP, 39 patients (83%) had dilatation of the intrahepatic bile ducts. Follow-up studies in infants and children ranging from 1 year to 11 years after their corrective operation for congenital dilatation of the common bile duct showed no morbidity that was attributed to the presence of the dilatation of the intrahepatic bile ducts. ECHO grams in 21 patients revealed marked decrease in size of the intrahepatic bile ducts except for two patients with huge cystic dilatation of the intrahepatic bile ducts. These findings justify the employment of the standard operative procedure for the treatment of congenital dilatation of the bile duct even if it is associated with dilatation of the intrahepatic bile ducts.

Adolescent↗