Urinary free cortisol (UFC), 17-hydroxycorticosteroids (17-OHCS) in urine and dexamethasone suppression test (DST) were examined in patients with dementia of Alzheimer type (DAT) and multi-infarct dementia (MID), and nondemented elderly. Eight of 19 patients (42.1%) were DST nonsuppressor. UFC was significantly elevated in patients with dementia, compared with that in nondemented elderly. There was no difference in UFC levels between DAT and MID. The UFC level correlated with post-DST plasma cortisol level at 1600 in demented patients. The mean level of 17-OHCS in demented patients was increased, although the difference was not significant statistically. In demented patients, UFC levels, not 17-OHCS levels in urine were correlated with Mini-Mental State Examination scores. These results suggest that a hypothalamo-pituitary-adrenal axis function is activated in demented patients and that this activation relates generally to dementation itself, not to an etiology of dementia. Measurement of UFC might be a biological marker of dementia and may have a value in diagnosis of dementia.

17-Hydroxycorticosteroids↗

PubMedDate not supplied

Peribiliary cysts in liver cirrhosis: US, CT, and MR findings.

OBJECTIVE: We report the imaging features of hepatic peribiliary cysts (PC) and the course of their development on CT. MATERIALS AND METHODS: Both CT and US findings were correlated with histopathologic findings in two patients with PC at autopsy. Five patients with cysts around the portal vein on CT but without histologic correlation were also included in this study. In six patients the course of PC development was observed. RESULTS: In the two autopsy cases cystic dilatation of the peribiliary glands was confirmed. The cases showed continuous small cysts along the portal veins reflecting the periportal collar seen on CT and the abnormal MR signal intensity of the periportal area. Ultrasound (US) showed round or tubular anechoic lesions around the large portal tracts mimicking dilatation of the bile ducts. Clustered cysts and echogenic dots or bands around PC were also seen. In three of six cases the PCs gradually enlarged and increased in number. CONCLUSION: In patients with liver cirrhosis PCs can be demonstrated by CT, US, and MRI in the vicinity of the hepatic hilum. Peribiliary cysts enlarge gradually and must be differentiated from dilatation of bile ducts or edema of portal tracts.

Aged↗

PubMedDate not supplied

Ultrastructure of the testis in an XX-male with normal plasma testosterone.

Hormonal and ultrastructural examinations of the testis were performed in an XX-male. Plasma levels of testosterone and prolactin were within the normal range, but FSH and LH were higher than the normal range. The response of testosterone to the administration of HCG is within the normal range. In the interstitium marked hyperplasia of Leydig cells was observed. The presence of paracrystalline inclusions and the absence of Reinke's crystals were representative changes in Leydig cells. Seminiferous tubules were filled with Sertoli cells and spermatogenesis was completely abolished. Intermediate filaments were abundant and annulate lamellae were absent in Sertoli cells.

Adult↗

PubMedDate not supplied

'Metaplastic lesions' in intrahepatic bile ducts in hepatolithiasis: a histochemical and immunohistochemical study.

Pathology of the intrahepatic bile ducts bearing calculi was examined with an emphasis on metaplasia in 22 cases of hepatolithiasis and in seven cases of normal livers. Normal livers contained few glandular elements within the bile duct walls and no metaplastic lesions or endocrine cells. In hepatolithiasis, a number of mucous glands resembling pyloric glands (pseudopyloric gland metaplasia) were seen within duct walls in all cases. The epithelial cells of the glands were positive for class III mucin with paradoxical concanavalin A staining which is known to be specific for pyloric glands. These cells were also positive for neutral, sialo- and sulfomucin to a variable extent. Intestinal metaplasia, including goblet cell and Paneth cell metaplasia, was found within duct walls and in covering epithelia in five (23%) cases. Endocrine cells, including argyrophil, argentaffin and gut hormone-containing cells were present in these metaplastic lesions in 13 (59%) cases. The occurrence of endocrine cells was closely associated with intestinal metaplasia, although there were a few endocrine cells in metaplastic pseudopyloric glands. These findings suggest that metaplastic lesions similar to the well-known metaplastic lesions in the gallbladder occur in the intrahepatic bile duct walls in hepatolithiasis. The appearance of metaplastic lesions and endocrine cells may be causally related to chronic inflammatory processes associated with hepatolithiasis.

Bile Ducts, Intrahepatic↗

PubMedDate not supplied

Intrahepatic peribiliary glands of humans. I. Anatomy, development and presumed functions.

The intrahepatic biliary tree is regarded as an excretory duct of two secretory units: hepatocytes and intrahepatic peribiliary glands. This review describes the anatomy, development and presumed functions of the latter. These glands are preferentially located around the intrahepatic large bile ducts, and are histologically divided into intramural and extramural structures. The former consist of simple tubular glands with much mucin, and are sparsely and irregularly distributed within the ductal wall. The latter are characterized by the presence of excretory units that consist of seromucinous acini and a conducting system in the periductal tissue. Pancreatic exocrine acini are occasionally admixed with extramural glands. These peribiliary glands appear in the late fetal period and complete their development about 15 years after birth. Extramural and intramural glands secrete neutral and acid mucin into the ductal lumen. Extramural glands contain several enzymes for digestion of protein and lipids. Neural and vascular supply of these glands may be related to the regulation of their secretion. Specific and non-specific immune responses within this glandular system may also be essential in the sterility of bile.

Bile Ducts, Intrahepatic↗

PubMedDate not supplied

Intrahepatic peribiliary glands of humans. II. Pathological spectrum.

The pathological spectrum of intrahepatic peribiliary glands is reviewed here. Several categories of histopathological changes such as necro-inflammation, cystic dilatation, hyperplasia and neoplasia have been identified in this glandular system. Necro-inflammation is associated with biliary tract diseases and chronic advanced liver diseases and may also appear in the livers of subjects with extrahepatic diseases such as sepsis. Cystic changes of microscopic sizes are not uncommon in autopsy livers of chronic advanced liver diseases, portal hypertensive diseases and also polycystic liver of adult type. Grossly recognizable cysts are, however, infrequent and occasionally cause compression of the adjoining bile ducts. Hyperplasia of these glands, which occurs consistently in hepatolithiasis and more variably in other conditions (e.g. biliary tract infection and submassive hepatic necrosis), may be associated with hypersecretion of seromucinous substances. Hyperplasia of peribiliary glands may then lead to mucin-related biliary diseases. In addition, these glands, particularly the hyperplastic ones, could be a precursor of cholangiocarcinoma. The pathological spectrum of the intrahepatic peribiliary glands is being expanded, although a clinical pathological correlation remains uncharted. Furthermore, age-related variations and non-specific reactive changes of these glands remain unexplored.

Bile Duct Diseases↗

PubMedDate not supplied

Striatonigral degeneration combined with olivopontocerebellar atrophy with subcortical dementia and hallucinatory state.

We present an autopsied case of striatonigral degeneration (SND) combined with olivopontocerebellar atrophy (OPCA) with subcortical dementia and hallucinatory state. A Japanese woman without a remarkable family history showed hand tremor at the age of 35 years, followed by bradykinesia, muscle rigidity, orthostatic hypotension, neurogenic bladder and pyramidal signs. No obvious cerebellar symptoms were found. Various antiparkinsonian drugs were administered, but were not markedly effective for the parkinsonism. She developed a mild dementia characterized by mild memory disturbance with preservation of orientation, slowing of thought processes, emotional lability toward sadness, impaired ability to manipulate acquired knowledge and poor calculating, and by the absence of aphasia, apraxia and agnosia. The features in this patient were consistent with those seen in subcortical dementia. She also had auditory hallucinations. MRI revealed hypointense T2 signals in the putamina and substantia nigra. T1-weighted MRI demonstrated atrophy of both the pons and cerebellum in addition to atrophy of the putamina and substantia nigra. EEG showed slowing of background activity. She died of cardiac failure at the age of 47. Autopsy disclosed brain stem tegmental atrophy, SND, OPCA and many glial cytoplasmic inclusions in the central nervous system, but well-preserved cerebrum. We discuss the relationship between the psychiatric symptoms and pathologic findings of brain stem tegmentum.

Autopsy↗

PubMedDate not supplied

Sinusoidal capillarization of human hepatocellular carcinoma: possible promotion by fibroblast growth factor.

Expression of acidic and basic fibroblast growth factors (aFGF and bFGF) and von Willebrand factor (vWF) was immunohistochemically investigated in 55 nodules of human hepatocellular carcinoma (HCC), 15 nodules of adenomatous hyperplasia (AH) of the liver and 10 cirrhotic livers (LC). AH, a putative preneoplastic lesion in the cirrhotic liver, was subdivided into ordinary and atypical types: the former was characterized by little cellular and structural atypia and the latter had some atypia equivocal as to benignity and malignancy. The positive rates of FGF (aFGF and/or bFGF) were as follows: 0% in LC, 20% in AH (ordinary and atypical) and 42% in HCC, whereas the positivity of vWF was 10% in LC, 20% in ordinary AH, 30% in atypical AH and 40% in HCC. There was no correlation between the expression of FGF or vWF and the size of HCC. No correlation was also found between the positivity of FGF and that of vWF in HCC and atypical AH. While vWF was not constantly expressed in the vicinity of FGF-positive HCC cells, capillarized sinusoids were significantly more numerous in FGF-positive cases than in FGF-negative cases (p < 0.01). These data indicate that FGF may be pathogenetically linked to the multistep development of HCC in relation to sinusoidal capillarization.

Biomarkers↗

PubMedDate not supplied

Stromal distribution of CA 19-9 as a predictor of lymph node metastases in gallbladder cancer without serosal invasion.

Gallbladder cancer without serosal invasion is often diagnosed during the pathologic examination of a gallbladder removed for presumed benign disease. The role of reoperative procedures in these patients is not well established, making it important to define prognostic factors that predict cancer spread. To determine if the pattern of immunohistochemical staining with a monoclonal antibody against CA 19-9 could predict lymph node spread of cancer, 23 patients with gallbladder cancer without serosal invasion were reviewed. CA 19-9 reaction was present in pathology specimens from all 23 patients. Twelve samples (52.2%) showed CA 19-9 in the stroma adjacent to cancer cells, while the remaining 11 did not show any stromal staining against CA 19-9. Lymph node development was found in only 2 (18.2%) of these 11 patients in the nonstromal staining group and 9 (75%) of the 12 patients in the stromal staining group. As these differences were statistically significant, we conclude that immunohistochemical localization of CA 19-9 in gallbladder cancer may be useful in predicting the presence or absence of lymph node involvement, and in developing a rational approach for a reoperative procedure.

Aged↗

PubMedDate not supplied

An autopsy case of hypertrophic cardiomyopathy showing dilated cardiomyopathy-like features by serial ventriculography.

An autopsy case of hypertrophic obstructive cardiomyopathy (HOCM), in which the clinical features of dilated cardiomyopathy (DCM) developed after 10 years is described. Three serial left ventriculograms (LVG) and bi-ventriculograms (BVG) were performed during the 10-year follow-up. At the time of the first observation, the ECG revealed a giant negative T wave (GNT) in V5 (-1.5mV) and high voltage (SV1 + RV5 = 8.1mV). The LVG and BVG showed concentric left ventricular wall hypertrophy and a pressure gradient in the left ventricular out-flow tract. However, follow-up studies disclosed resolution of the GNT with decreased high voltage. All wall thicknesses and the ejection fraction were significantly decreased and the wall motion was reduced serially. These findings were consistent with the clinical profile of DCM. At autopsy, the heart weighed 610 g and showed dilated change of the left ventricular cavity. Significant myocardial fibrosis, bizarre myocardial hypertrophy with disorganization (BMHD) and severe narrowing of the intramural small arteries due to intimal thickening were found in the left ventricular wall, especially in the anterior and interventricular septal wall. This autopsy case suggests that some cases of HCM, with serial disappearance of the deep negative T wave and high voltage, deteriorate to the clinical profile of DCM.

Adult↗

PubMedDate not supplied

Association of mixed connective tissue disease, Sjögren's syndrome and autoimmune hepatitis: report of a case.

A 51-year-old female was admitted to the hospital due to liver dysfunction. She had suffered from arthralgia, Raynaud's phenomenon, dry mouth and dry eyes. Laboratory data showed positive LE cells and anti-RNP. The diagnosis of mixed connective tissue disease (MCTD) was made according to the criteria of the international symposium on MCTD and Antinuclear Antibodies (August 1986, Tokyo). Liver biopsy showed chronic active hepatitis. The results of sialography, lip biopsy and rose-bengal test were compatible with Sjögren's syndrome (SjS). This is the report of a rare case with MCTD associated with SjS and autoimmune hepatitis.

Antibodies, Antinuclear↗

PubMedDate not supplied

[Chemotherapy in hemodialysis patient with metastatic testicular cancer; pharmacokinetics of etoposide and cisplatin].

The pharmacokinetics of intravenously administrated cisplatin and etoposide were studied in a patient with seminoma (stage IIIA) receiving hemodialysis for chronic renal failure. The treatment schedule was as follows: 7 mg/m2 of cisplatin at day 1, 3, 5; 14 mg/m2 of cisplatin at day 2, 4; 70 mg/m2 of etoposide at day 1-5; hemodialysis at day 2, 4. After the treatment myelosuppression was very strong. So the patient were received another treatment of smaller doses of cisplatin and etoposide in three courses. The other schedule was as follows: 14 mg/m2 of cisplatin at day 1, 3, 5; 35 mg/m2 of etoposide at day 1-5; hemodialysis at day 1, 3, 5. The area under the blood concentration-time curve (AUC) of free-cisplatin was 6.82 micrograms.hr/ml in first course, 4.07 micrograms.hr/ml in second course. The peak concentration of peripheral blood free-cisplatin was 0.58 microgram/ml in first course, 0.43 microgram/ml in second course. The AUC of etoposide was 241.9 micrograms.hr/ml in first course, 216.9 micrograms.hr/ml in second course. After treatment CR was observed and there was no recurrence for five years. In conclusion, it was considered that cisplatin and etoposide could be given to the patient receiving hemodialysis for chronic renal failure and smaller doses should be given to prevent side effects.

Adult↗