Biomedical subjects

J Yanagihara

Publications and source records attributed to J Yanagihara.

At least 37 records · Page 2Linked to original sources

PubMed1992

Motility and sensation of the rectosigmoid and the rectum in patients with anorectal malformations.

Motility of the rectosigmoid and rectum and reservoir function of the rectum after surgery for anorectal malformations were investigated in 32 patients (17 with high type, 6 with intermediate type, and 9 with low type anomaly) aged 5 to 16 years. All 32 patients were examined manometrically as well as with a newly devised myoelectrical method. Manometry showed that the values of maximum anal pressure and anorectal pressure difference in the high type were significantly lower than those in the low type. However, the incidence of contractile activity of the rectosigmoid was not significantly different between these two groups. The threshold sensation pressure and the maximum tolerable pressure in the high type were significantly higher than those in the low type, and the rectal compliance in the high type was significantly lower than that in the low type. Electromyography was recorded at 8 cm and 5 cm from the anal verge. Two types of slow waves were observed, a faster rhythm and a slower rhythm. Their frequency was similar in the three groups. However, the numbers of spike bursts in the high type and intermediate type were significantly higher than those in the low type. These results indicate that in addition to an inadequate anal resting pressure a loss of optimal rectal sensation or rectal reservoir function might be associated with fecal incontinence in the high type and that increased spike bursts might play some role in rectal motility.

Adolescent↗

PubMed1992

Prediction of affected MEN2A gene carriers by DNA linkage analysis for early total thyroidectomy: a progress in clinical screening program for children with hereditary cancer syndrome.

The gene predisposing to multiple endocrine neoplasia type 2A (MEN 2A) has been assigned to chromosome 10, and affected gene carriers can be identified before the development of associated malignancy in some informative families. We applied these advances in gene mapping to clinical screening for possible pediatric surgery. A family with MEN 2A, consisting of 88 members and their spouses, was studied to test the reliability of the provocation of plasma calcitonin with pentagastrin and the possibility of DNA diagnosis of mutated gene carriers with DNA probes closely linked to the MEN2A gene including RBP3 and FNRB genes. Nineteen of the 88 were diagnosed as MEN 2A carriers. Twelve of them were treated surgically and the others died of medullay thyroid carcinoma (MTC) or pheochromocytoma. A strikingly sensitive response of calcitonin was observed in all those with MTC. The genotypes cosegregating with the abnormal allele at MEN2A in this family could be deduced from clinically established affected members. The early detection of gene carriers allows us to concentrate our screening efforts on children at high risk and to release non gene carriers from repeated unnecessary testing. MEN2A is one of the first cancer syndromes for which DNA screening permits early detection of members at high risk.

Adolescent↗

PubMed1992

Postoperative management of children with biliary atresia and heart failure.

The postoperative course in two children with extrahepatic biliary atresia and cardiovascular disease was reviewed and the correlation between biliary drainage and cardiac function was analyzed. Both patients obtained satisfactory biliary drainage after Kasai's hepatic portoenterostomy. One patient developed heart failure postoperatively due to severe viral myocarditis. This child's total serum bilirubin concentration remained elevated for eight months, despite adequate bilirubin excretion, until her cardiac function returned to normal. Another patient died of cardiac failure due to congenital heart disease 83 days after Kasai's operation, but his postoperative biliary drainage was satisfactory as long as cardiac function remained compensated. In both cases, fluid intake was restricted severely (30 to 70 ml/kg body weight/day), as titrated by echocardiographic assessment of cardiac function, but biliary excretion was satisfactory as long as the cardiac fractional shortening ratio was greater than 30% and the ejection fraction was greater than 55%. This suggests that cardiac decompensation affects postoperative biliary excretion in patients with biliary atresia; however, with careful medical management satisfactory biliary drainage can be achieved even in patients with severe heart diseases.

Biliary Atresia↗

PubMed1992

Histological investigation of the myenteric plexus of the entire gut in an infant with hypogenesis of the intestine.

A male infant with pseudo-Hirschsprung's disease was treated for 2 years and 10 months, then died of severe enterocolitis. At autopsy examination of the entire gut was possible, and a definite histological examination could be performed which threw light on the pathogenesis. The ganglion cells of the esophagus, stomach, small intestine, colon and rectum were examined, diameters of their nuclei were measured, and their nuclear volumes were calculated. S-100 and anti-neurofilament stainings were also performed in an immunohistological investigation of the glial cells and nerve fibers. Both the diameter of the volume of the nuclei of the ganglion cells in the small intestine and colon were significantly smaller than normal. Even in the esophagus the nuclear volume was smaller than normal. The glial cells and nerve fibers gradually decreased in the caudal direction. Thus, histomorphometry and immunohistochemistry both showed that the neuroblasts in the intestine were immature and the migration of ganglion cells was disturbed in this case. The final diagnosis was hypogenesis of the intestine.

Cell Count↗

PubMed1992

Thermography in a child with varicocele.

A 12-year-old boy with a left-sided varicocele (Grade 3) was assessed preoperatively and followed postoperatively by computer-assisted infrared thermography. The temperature of the left scrotum was 35.4 degrees C before surgery which was 4 degrees C higher than that of the right scrotum. After ligation of the left internal spermatic vein and removal of the varicocele, scrotal thermograms 39 days and 12 months after operation were normal. Therefore, it seems that thermography is very valuable in the evaluation of testicular function in children with varicocele, in whom semen analysis is impossible.

Child↗

PubMed1992

Congenital choledochal dilatation with emphasis on pathophysiology of the biliary tract.

Of 37 patients with congenital choledochal dilatation, aged 8 days to 12 years, who had undergone excision with Roux-en-Y hepaticojejunostomy, 26 patients could be analyzed for morphologic abnormalities and pathophysiology of the biliary tract. Of the 26 patients with congenital choledochal dilatation, 25 (96.2%) had an abnormal choledochopancreaticoductal junction. Of the 12 patients with cystic-type choledochal dilatation, 10 had the C-P type of abnormal choledochopancreaticoductal junction, and of the 13 patients with fusiform-type choledochal dilatation, nine had the P-C type. The amylase levels in the choledochal cyst and the gallbladder were elevated regardless of the form of choledochal dilatation. An adenocarcinoma in a cystic choledochal dilatation was found in one child. Therefore, longstanding inflammation of the biliary tract caused by the reflux of pancreatic juice might be one of the factors in carcinogenesis in the biliary tract. This free reflux of pancreatic juice was demonstrated not only by amylase levels in the biliary tract but also by intraoperative biliary manometry. This reflux might be explained by the lack of sphincter function at the junction of the common bile and pancreatic ducts.

Amylases↗

PubMed1990

The natural history of multiple endocrine neoplasia type 2A--a clinical analysis.

The records of 18 patients with multiple endocrine neoplasia (MEN) type 2A in a family consisting of 87 members in 5 generations, were reviewed with respect to age at onset and length of survival in a study of the natural history of this syndrome. Of these 18 patients, 8 were alive following surgical treatment, while 10 had died without treatment. The clinical onset of medullary thyroid carcinoma was noticed as a nodule in the neck at between 17 and 34 years of age (mean 24 years); the onset of pheochromocytoma was characterized by palpitation, hypertensive attacks or recurrent headache at between 17 and 42 years of age (mean 27 years); and death occurred in 10 patients at between 23 and 67 years of age (mean 40.6 years). This information on the age at onset of individual clinical manifestations, and on the length of survival of gene carriers, may be useful for clinicians who are asked to assess the risk of the development of this syndrome in a genetically predisposed asymptomatic individual, because screening tests are often refused by members of Japanese families with MEN type 2A.

Adolescent↗

PubMed1990

Bile duct patterns in the hilar region of the liver in two cases of biliary atresia.

Bile duct patterns in the hilar region of the liver were demonstrated in two autopsy cases of biliary atresia by three-dimensional graphic reconstructions. In one case, jaundice had completely disappeared after successful hepatic portojejunostomy. However, at the age of 4 months, this patient died of cardiac failure caused by endocardial cushion defect. The histological reconstruction of this case showed that there were large patent bile ducts, 150 microns to 400 microns in diameter, and plenty of bile ductules surrounding the large ducts to form networks. The other patient died of hepatic failure at the age of 9 months. In this case, only a few large ducts ended in bile lakes that did not connect with the portojejunostomy, and only a few ductules were found around the large ducts. These results indicate that the existence of networks of bile ductules and the patency of large bile ducts at the porta hepatis contribute to good postoperative bile excretion.

Bile↗

PubMed1990

Cancer arising in a choledochal cyst in a 12-year-old girl.

An adenocarcinoma was found in a choledochal cyst in a 12-year-old girl. This is the youngest patient so far reported with an adenocarcinoma in a choledochal cyst. The cyst consisted of dilatations of the intrahepatic bile ducts and the extrahepatic bile duct. Most of the cyst wall was resected. However, resection of the distal part of the cyst wall in the infrapancreatic region was impossible because of invasion of the portal vein and the inferior vena cava. This patient is now being treated with chemotherapy. We believe that early diagnosis is mandatory, and that total excision of a choledochal cyst is advisably to prevent the development of carcinoma.

Adenocarcinoma↗

PubMed1990

Accessory scrotum with lipoma in an infant.

We report on a 2-year-old male infant with accessory scrotum with lipoma presenting a perineal mass. Preoperatively no abnormality in the urinary tract and the anorectum was demonstrated. The tumour was resected and perineoplasty was performed. The resected specimen histologically revealed lipoma with tissue suggestive of scrotum. The patient recovered well and was discharged with no clinical complaints. This rare congenital anomaly is described in this paper. The slight continuity between the left scrotum and an accessory scrotal tissue in this case may suggest that this rare anomaly was derived from some abnormality in the migration of precursor tissue of scrotum.

Child, Preschool↗

PubMed1989

Rectoanal pressure studies and postoperative continence in imperforate anus.

Functional results after surgical correction of anorectal malformations were assessed on a clinical basis using the Kelly score and by manometric study. In all, 65 patients, aged 5-28 years, were interviewed personally, and 51 of these 65 had manometric studies to evaluate postoperative continence. The manometric study was also performed on 45 normal children as control group. Continent patients characteristically had a marked high-pressure zone, as did the normal subjects. On the other hand, in the patients with fair or poor results, the anorectal pressure profile had no marked high-pressure zone in the anal canal. The presence of normal anal pressure at rest as well as adequate anorectal pressure difference was found to correlate well with continence. In the patients with perineoplasty, the anorectal reflex correlated well with continence, but not in patients treated by abdominoperineal rectoplasty.

Adolescent↗

PubMed1989

[Treatment of lymphangiomas arising around cervico-facial region: surgery, bleomycin therapy and OK-432 therapy].

To determine a favorable treatment for lymphangiomas arising around cervico-facial region, a 10-year (1979, 1-1988, 9) experience with 58 these lesions were reviewed. Therapeutic records were divided into 3 groups according to the treatment: surgical Excision (n = 16), intralesional Bleomycin (BLM) therapy (n = 11), and intralesional OK-432 Picibanil therapy (n = 31). Complete disappearance of the lesions without serious complications was noted in 3 of 16 patients treated by surgery, in 3 of 11 patients treated by BLM therapy, and in 21 of 31 patients treated by OK-432 therapy. Serious complications such as facial nerve palsy (3), ugly scars due to repeated excision (5) and short of tongue (1) were noted in surgically treated group. In BLM and OK-432 therapy groups, only a fever of 2 to 3 days' duration and local inflammatory reaction of 3 to 5 days were noted. The results suggest that OK-432 therapy is effective for these lesions.

Biological Products↗

PubMed1989

Results of a screening program for multiple endocrine neoplasia type 2A: a clinical study of a Japanese family.

A Japanese family of 87 members in five generations with multiple endocrine neoplasia type 2A (MEN 2A) is described regarding the utility of screening tests for early detection of medullary thyroid carcinoma and the potential for DNA diagnosis of MEN 2A gene carriers. The screening programs for family members in this series include measurements of plasma calcitonin concentrations after intravenous injection of pentagastrin (0.5 micrograms/kg/5 sec) and 24-hour urinary excretion of catecholamines. While 18 MEN 2A patients had been previously diagnosed, these screening programs revealed five additional patients with MEN 2A (aged 16, 19, 35, 37, and 57). Prediction of MEN 2A gene carriers by DNA analysis has been attempted but is not yet possible in this family.

Adolescent↗

PubMed1988

Voluntary anal continence after surgery for anorectal malformations.

Electromyography (EMG) and measurement of voluntary contraction pressure of the external sphincter muscle were performed in 28 patients, aged 5 to 14 years, to assess the function of the external sphincter after surgical correction of anorectal malformations. Ten normal children, aged 5 to 15 years, served as controls. External sphincter function in patients with high-type anomalies was disturbed in the areas of tonic activity, inflation reflex, and activity during further rectal filling. In patients with low-type or intermediate-type anomalies, function was preserved and was equal to that in normal controls. Phasic activity was observed in patients with all types of anomalies and in normal subjects. Among those with high-type anomalies, the three patients with Kelly's score of less than 2 had voluntary contractions of 20 cmH2O or less. However, the mean voluntary contraction pressures were not significantly different among the three types of anomalies. Therefore, patients with high-type anomalies may acquire compensatory voluntary continence through bowel training.

Adolescent↗

PubMed1988

Reliability of anorectal manometry in the diagnosis of Hirschsprung's disease.

Anorectal manometry was performed in 79 patients, aged 2 days to 19 years, who were referred to us because of constipation or symptoms of intestinal obstruction. A definitive diagnosis was obtained in 75 of the 79 patients (95%). Thus, anorectal manometry is a reliable tool in the diagnosis of Hirschsprung's disease. In the neonatal period, however, a definite diagnosis was possible in only 17 of the 21 neonates (81%). The age at the time of the first examination was 14 +/- 2 days in those with a definite diagnosis and 5 +/- 1 days in those with an indefinite diagnosis (p less than 0.001). If intestinal obstruction can be managed by conservative therapy, examination should be repeated after two weeks of age if the diagnosis before one week of age is not definite. These results indicate that anorectal manometry is non-invasive and reliable in the diagnosis of Hirschprung's disease. However, further examinations such as barium enema or rectal biopsy should be done, even in the neonatal period.

Anal Canal↗

PubMed1988

Results of surgical correction of anorectal malformations. A 10-30 year follow-up.

Of 119 patients with surgical correction of anorectal malformations, 47 who were 10-30 years of age were interviewed personally and had manometric studies to evaluate postoperative continence. This clinical study included not only long-term anorectal function but also sexual function. Patients with low type anomalies or with intermediate type anomalies were more likely to be continent, whereas patients with high type lesions had some problems with continence. However, only two of the 16 patients (12%) with high type anomalies were classified as having poor results. This rate is perhaps lower than might be expected. Thus, incontinent patients may become continent even if they were classified as having fecal incontinence before 6 years of age. Most patients who were 15-30 years of age had normal sexual function except for two females with irregular menstruation. These results indicate that achievement of fecal continence and sexual function in patients with high type anomalies treated by abdominoperineal rectoplasty depends on careful dissection as close as possible to the rectal wall and bringing the terminal bowel down exactly within the sling of the puborectal muscle.

Adolescent↗

PubMed1987

Clinical and manometric assessment of anorectal function after Martin's operation.

Anorectal function after Martin's operation for total colonic aganglionosis was assessed in five patients by clinical and manometric studies. One year after operation the frequency of bowel movements decreased to three times a day, and the stools became semi-formed. Anorectal manometry showed decreasing anal resting pressure and anorectal pressure difference one month after the operation. However, these values became gradually normal. Rectal compliance increased as bowel function improved clinically. These results show that increasing rectal compliance and normalisation of stools by adequate absorption of intestinal fluid are important factors in the improvement of faecal continence after Martin's operation.

Child, Preschool↗

PubMed1986

Biliary manometry in choledochal cyst with abnormal choledochopancreatico ductal junction.

Intraoperative manometry of the biliary tract and measurement of amylase levels in choledochal cysts were performed in seven patients, aged 14 months to 5 years, with choledochal cysts, in an investigation of the pathophysiology of the biliary tract. An abnormal choledochopancreatico ductal junction was observed in these seven patients by preoperative endoscopic retrograde cholangiopancreaticography (ERCP) or intraoperative cholangiograms. All six patients examined showed a high amylase level in the choledochal cyst (5,450 to 46,500 Somogyi Units). The intraoperative manometry of the biliary tract showed that a remarkable high pressure zone as was found in the area of sphincter of Oddi was not found in the area of abnormal choledochopancreatico ductal junction. The pressure recordings also demonstrated that the sphincter of Oddi pressure in the patient with choledochal cyst was increased by gastrin stimulation. On the contrary, no pressure reaction to gastrin or secretin was found in the area of abnormal choledochopancreatic ductal junction. From these results it seems that free reflux of pancreatic juice into the biliary system occurs, and the reflux stream depends upon the pressure gradient between pancreatic ductal pressure and common bile duct pressure because of the lack of a sphincter function at the choledochopancreatico ductal junction.

Amylases↗