Intraperitoneal transplantation of isolated hepatocytes of the pig: the implantable bioartificial liver]

To elucidate the histopathological features of pancreatic ischemia, we examined postmortem pancreases in which cholesterol emboli were present. Cholesterol emboli were detected in 17 pancreases (6 of 36 cases of aortic aneurysm and 11 of 223 control cases). Two of the 17 pancreases had well-demarcated patchy lesions composed of degenerating acinar cells showing deeply eosinophilic cytoplasm and pyknotic nuclei, indicating fresh ischemia. In the marginal zone of the larger lesions and in the small lesions, the intralobular ductules had avoided the ischemic changes. Five of the 17 pancreases had patchy fibrotic foci containing small ductules with slightly retraction features. These ductules are considered to be the remnant intralobular ductules that have avoided the previous ischemic damage. We conclude that these patchy fibrotic foci are the healed ischemic lesions. The current findings suggest that the healed ischemic lesions can be differentiated from common pancreatic fibrosis. The existence of remnant intralobular ductules and the patchy retraction features may be useful histological markers for the determination of healed ischemic lesions.     

Custom endoprostheses for limb salvage: a historical perspective and imaging evaluation

BACKGROUND & AIMS: Apart from the high-risk groups, the pathology of chronic hepatitis C in children is not well known. The aim of this study was to investigate the morphology of chronic hepatitis C in children without any underlying systemic disease and to evaluate its relationship to clinicovirological factors. METHODS: Liver biopsy specimens from 80 children positive for antibody to hepatitis C virus were evaluated using a semiquantitative scoring system. RESULTS: Chronic hepatitis was mild in most cases but had high-grade activity in 17 children (21.2%). A significant association was found between the grade of focal necrosis and alanine transaminase levels (P < 0.003). Fibrosis was absent in 22 cases (27.5%), mild in 44 (55%), and moderate in 13 (16.2%). Only 1 patient had cirrhosis. A significant relationship was detected between fibrosis scores and (1) duration of disease (P < 0.03); (2) portal inflammation (P < 0. 002); and (3) interface hepatitis (P < 0.003). CONCLUSIONS: In otherwise healthy children, chronic hepatitis C is a morphologically mild disease in most cases. Fibrosis increases with the duration of disease, suggesting that end-stage disease may develop in young adulthood. Alanine transaminase levels correlate with intralobular focal necrosis but not with other lesions. In this respect, liver biopsy retains its importance in the management of chronic hepatitis C in children.     

Characterization of Borrelia spp. isolated from the tick, Ixodes tanuki and small rodents in Japan

Rat pancreatic periacinar fibroblastoid cells (PFCs) appear to be involved in intralobular fibrosis and acinar cell regeneration. We isolated pancreatic acini of the rat, cultured the fibroblastoid cells, and characterized the cells morphologically and immunohistochemically. Isolated acini were seeded on culture dishes, and spindle-shaped cells migrated and proliferated. On Electronmicroscopic examination, microfilament bundles were seen, and the intracellular localization of vimentin, alpha-smooth muscle actin, and non-muscle myosin was identified immunohistochemically. These findings strongly suggest that the cells were myofibroblast-like. The PFCs were also demonstrated, immunohistochemically, to contain prolyl hydroxylase, type-I procollagen, type-III procollagen, type-IV collagen, fibronectin, and laminin. Stimulation by transforming growth factor beta 1 (TGF beta 1) increased intracellular immunoreactive prolyl hydroxylase and collagen synthesis in the PFCs. These findings indicate that PFCs proliferate in culture as myofibroblast-like cells and synthesize extracellular matrix components. It is possible that PFCs are involved in intralobular fibrosis in response to stimulation with TGF beta 1.     

Drug-induced linear IgA disease: target antigens are heterogeneous

BACKGROUND: Colonic strictures represent an advanced stage of fibrosing colonopathy in patients with cystic fibrosis. AIMS: To clarify whether ultrasonography can identify patients with an early stage of fibrosing colonopathy and to determine clinical factors that influence bowel wall thickening. PATIENTS: Ninety patients with cystic fibrosis, median age 10 years, and 46 healthy controls, median age 13 years, were investigated. METHODS: Bowel wall thickness was measured by ultrasound in a prospective study. RESULTS: In cystic fibrosis, wall thickness of both small intestine and colon was significantly (p < 0.0001) higher than in controls; 81% of patients with cystic fibrosis had a maximum colon wall thickness at any site of 2 mm or more, a value that was never reached by controls. The maximum colon wall thickness was 6.5 mm. Bowel wall thickness was unchanged at re-examination after one year. There was no progression even with high dose pancreatic supplements. There was no association between bowel wall thickness and clinical features such as previous meconium ileus, intestinal resection, distal intestinal obstruction syndrome, abdominal pain, or pancreatic enzyme dose. CONCLUSIONS: There is genuine intestinal involvement in cystic fibrosis; in a few cases this could lead to fibrosing colonopathy.     

Rickets in adult cystic fibrosis with myopathy, pancreatic insufficiency and proximal renal tubular dysfunction

Rickets is reported in a 19 year old white man with cystic fibrosis in whom pancreatic and hepatic involvement was advanced. There was evidence of secondary hyperparathyroidism with proximal renal tubular acidosis, aminoaciduria, phosphaturia and hypophosphatemia. Treatment with oral pancreatic and parenteral vitamin D supplements led to full recovery of the rachitic syndrome and the proximal renal tubular dysfunction.     

Periacinar collagenization in patients with chronic alcoholism

To examine the development of pancreatic fibrosis in alcoholics, the fibrosis types grouped according to Martin's classification were examined by immunohistochemistry using an antibody against alpha-smooth muscle actin (alpha-SMA). The initial stage of periacinar collagenization was also investigated by electron microscopy. The total incidence of pancreatic fibrosis at autopsy of the 29 alcoholics was significantly higher than that of the 40 non-alcoholics. Intralobular sclerosis was observed to be the most frequent type of fibrosis regardless of alcohol intake. No differences in the enhancement of alpha-SMA expression in each type of fibrosis were found between the alcoholics and non-alcoholics. Electron microscopically, myofibroblasts were found around acini in the early stage of periacinar collagenization, and were accompanied by numerous fine filaments (8-15 nm in diameter). The various changes in zymogen granules (ZG), lysosomes and lipid droplets were augmented in the acinar cells of alcoholics. Medium-density materials were also found in dilated rough endoplasmic reticulum (RER). The contents of ZG and RER occasionally leaked out. In conclusion, pancreatic fibrosis was increased in alcoholics; myofibroblasts may play an important role in the initial stage of periacinar collagenization; and the intracellular transport blockage of protein as represented by abnormalities of ZG, ER and lysosomes may contribute to the development of periacinar collagenization.     

PET evidence for an amodal verbal working memory system

To assess the influence of digestive juice on the pancreatic stump when pancreaticogastrostomy was performed after pancreatoduodenectomy, the pancreatic stump was anastomosed to the intact stomach (group I), the stomach after partial gastrectomy (group II), or the jejunum (group III) in rabbits, and the nature of the digestive juice at the anastomotic site as well as the histologic changes of the pancreatic tissue were investigated. The digestive juice was highly acidic in group I, slightly acidic in group II, and almost neutral in group III. Histological examination of the pancreatic stump revealed extensive coagulative necrosis and delayed replacement with granulation tissue in group I, while there was less prominent liquefactive necrosis and early replacement with granulation tissue in groups II and III. Intraperitoneal abscess formation around the anastomotic site and atrophic fibrosis of the pancreas (similar to the changes after pancreatic duct ligation) occurred in 27.8% and 46.2% of group I rabbits, respectively, but no such changes were detected in groups II and III (both P < 0.05). These results indicate that the highly acidic gastric juice had a widespread corrosive effect on the anastomosed pancreatic tissue, and that partial gastrectomy may be necessary to prevent anastomotic leakage and pancreatic duct obstruction after pancreaticogastrostomy.     

Severe colitis in mucoviscidosis

We report the association of severe indeterminate colitis with cystic fibrosis in a 21 year old woman, with mild pulmonary involvement, and without digestive or pancreatic symptoms or pancreatic enzyme preparation. Ten cases of inflammatory bowel disease associated with a cystic fibrosis have been reported. Most fit with the diagnostic criteria of Crohn's disease. Although this case was compatible with this diagnosis, we have retained the diagnosis of "severe indeterminate colitis" because of the lack of specific histological features of Crohn's disease. The association between inflammatory bowel disease and cystic fibrosis is probably not fortuitous, although the pathophysiological link between the two diseases is unknown.     

Complex formation of the nonstructural protein 3 of hepatitis C virus with the p53 tumor suppressor

BACKGROUND: Length of survival of females with cystic fibrosis is worse than it is in males. Results of current research have shown an important correlation among dietary intake, nutritional status, lung function, and survival. The purpose of this study was to explore gender differences in dietary intake and pancreatic enzyme replacement therapy in males and females with cystic fibrosis. METHODS: The study was a cross-sectional measurement of clinical characteristics, energy, and fat intakes in males and females attending the cystic fibrosis outpatients clinics of the John Hunter Hospital, Newcastle, Australia. Twenty-nine subjects, (17 females and 12 males), completed 4-day weighed food records to measure total energy intake and the contribution of macronutrients and to document use of pancreatic enzyme replacement therapy. Energy intake was assessed as the percentage of the recommended energy intake for age and sex. RESULTS: Females with cystic fibrosis had significantly lower energy and fat intakes than males, whereas the females used significantly more pancreatic enzyme replacement therapy. There were no significant differences in clinical characteristics between groups. CONCLUSION: The results support the possibility that gender differences in the energy and fat intakes of older patients may contribute to differential median survival time of males and females with cystic fibrosis.     

Factors affecting image acquisition during scanning laser polarimetry

Prognosis of systemic sclerosis (scleroderma, Ssc) is largely depending on involvement of internal organs. Abnormalities of the gastrointestinal tract are found most frequently (85%), especially decreased motility of the oesophagus, which has little impact on the longterm clinical course of Ssc. Pulmonary manifestations can be demonstrated in 40-90% of patients; one must distinguish between pulmonary hypertension or fibrotic lung disease. The heart is affected in 50% of cases. Patchy or diffuse myocardial fibrosis, as well as pericarditis and pericardial effusions can induce symptoms of arrhythmia or congestive heart failure. Renal involvement is associated with increased mortality and occurs in 45% of Ssc, producing proteinuria, hypertension, scleroderma renal crisis and renal failure. In conclusion, involvement of the lungs, heart and kidneys are determining factors for the longterm course of systemic sclerosis.     

Longitudinal analysis of multiple indicators of health decline among spousal caregivers

CONCLUSION: In view of the frequent absence of symptoms related to pancreatic lesions, screening tests for VHL should always include assessment of the pancreas and, considering the frequency of polycystic manifestations, VHL should always be borne in mind in the differential diagnosis of multiple pancreatic cysts, especially when occurring in young patients and in the absence of a positive history of pancreatic disease. BACKGROUND: Von Hippel-Lindau disease (VHL) is a hereditary disease transmitted with an autosomal dominant character and characterized by hemangioblastomas of the central nervous system and retina, renal tumors and cysts, and pheochromocytoma. Pancreatic manifestations of VHL are reported in the literature with incidences ranging from 16 to 29% of cases and consist mainly in cystadenomas of the serous type and in multiple cystic lesions, often with complete replacement of the gland. METHODS AND RESULTS: We report five cases of VHL with a polycystic pancreas as the main or only manifestation, all devoid of symptoms related to involvement of the pancreas, who were referred to our Pancreatic Surgery center with diagnoses of multiple pancreatic pseudocysts of undefined origin.     

Relationships between lymphography and the histologic nodal and splenic types in Hodgkin''s disease. Considerations on clinical evolution

Trying to establish the eventual interrelations of the initial histologic nodal type and the splenic one, the general lymphographic picture, the histologic nodal type and spleen involvement, lymphographic and histologic examinations were carried out in 151 patients with Hodgkin's disease. Lympographies were performed in 139 cases, and splenectomy (followed by splenic, hepatic and abdominal lymph node biopsies) in 32. Lymphocyte depletion was found in 72.7% of the patients with lymph node obstruction diagnosed lymphographically. Splenic involvement was more frequent in cases with pathologic lymphographic picture and histologic aspects of lymphocyte predominance or nodular sclerosis. In patients with initial nodal histologic types of nodular sclerosis or lymphocyte depletion, the splenic histopathologic types were the same, but they got more severe in cases with lymphocyte predominance or mixed cellularity. Splenic biopsy might be unconclusive after protracted cytostatic treatment or splenic X-ray therapy. In the authors' opinion, early routine splenectomy is rather more advisable than differentiated splenectomy.     

Epithelial anomalies in chronic pancreatitis as a risk factor of pancreatic cancer

BACKGROUND/AIMS: The relationship between chronic pancreatitis and the development of pancreatic cancer is still a matter of dispute. Our aim was to determine the frequency of hyperplastic, metaplastic and dysplastic epithelial anomalies in the course of chronic pancreatitis and the potential steps in their development to malignancy. METHODOLOGY: The study was based on biopsy material of 70 patients with clinically diagnosed advanced chronic pancreatitis, who underwent partial or total pancreatectomy, as well as other operations. The patients were assigned to 2 groups: Group I (n = 41) with calcifying chronic pancreatitis; Group II (n = 29) with other forms of the disease. Histological sections were stained with hematoxylin-eosin, Mallory-azan, Gomori's silver method, and glycosaminoglycans (PAS and Alcian blue staining). Special interest was focused on the type and incidence of epithelial ductal and acinar cell anomalies, and on the degree of parenchymal scarring. RESULTS: Hyperplasia of the ductal epithelium was present in 31.4%, focal squamous metaplasia in 21.4%, mucous metaplasia in 11.1%, cellular dysplasia in 8.6%, dysplastic acinar cell nodules in 21.4%, and "tubular complexes" in 30.0% of all cases. The differences in the frequency of these changes, except for ductal epithelial hyperplasia, were not statistically significant in two comparable groups. Advanced pancreatic fibrosis was associated with epithelial anomalies in 65.7% of all cases. CONCLUSIONS: From the morphological point of view, the adequate prerequisites for the consideration of advanced forms of chronic pancreatitis, independent of type, as a risk factor of pancreatic cancer exist, necessitating the surgical removal of pathological lesions.     

Pituitary abscess: a series of six cases

This study examined the influence of zopiclone, a third generation hypnotic, on the transition from slow wave sleep to paradoxical sleep (PS) which is increased at the expense of PS by barbiturates and benzodiazepines. The compound decreased sleep latency and increased the latency of the intermediate stage (IS) and PS at 2.5, 5 and 7.5 mg/kg IP. The amount of the IS was decreased because of the decrease in phase number up to 6 h at all doses. PS amount was decreased during 2 h at 2.5 mg/kg and during 4 h at 5 and 7.5 mg/kg also because of the decrease in phase number. The IS never substituted for PS. The IS spindle characteristics were not modified and the theta rhythm frequency slightly decreased at 5 mg/kg (IS) and 7.5 mg/kg (PS).     

Histological assessment of chronic pancreatitis at necropsy

AIMS: To evaluate the histological criteria used to diagnose chronic pancreatitis; and to assess interobserver variation among general pathologists. METHODS: Forty five cases of chronic pancreatitis diagnosed in necropsy were reviewed to determine whether the diagnosis was acceptable retrospectively. These cases were diagnosed initially as chronic pancreatitis in the final necropsy report complied by general pathologists. In reviewing these cases, special attention was paid to irregular fibrosis and destruction of the lobular architecture. RESULTS: The 45 cases were re-assigned to seven different diagnostic categories: chronic pancreatitis, 21 (47%) cases; interstitial fibrosis with or without chronic inflammation, 11 (24%) cases; repair stage of acute pancreatitis, four (9%) cases; severe fatty infiltration, three (7%) cases; chronic inflammation without interstitial fibrosis, two (4%) cases; haemochromatosis, one (2%) case; and undetermined, three (7%) cases. CONCLUSIONS: The histological spectrum of chronic pancreatitis was very wide and it was often misdiagnosed. Acinar atrophy, acinar dilation and intralobular fibrosis were diagnostic of chronic pancreatitis. Differential diagnoses include the repair stage of acute pancreatitis, severe fatty infiltration and haemochromatosis. Recognition of these findings may help to reduce overdiagnosis of chronic pancreatitis.     

Quantitation of vancomycin and its crystalline degradation product (CDP-1) in human serum by high performance liquid chromatography

Mantle cell lymphoma (MCL) patients represent a difficult problem, sometimes to establish the diagnosis but mostly because of their refractoriness to standard lymphoma treatments. Which treatments to apply and to whom is not yet defined. In this study, we attempted to analyze the clinical features, to identify the major prognostic factors, and to evaluate the outcome of 121 MCL patients treated in our institution between 1979 and 1997. Clinical data, treatment modalities, and International Prognostic Index (IPI) score were evaluated. Median age was 63 years. Patients usually presented with advanced stage disease (87%), disseminated lymph nodes (57%), bone marrow involvement (79%), but with a good performance status (PS) (81%). Lymphocytosis >4000/microl and/or peripheral blood involvement was present in 36% of cases, and gastrointestinal disease in 18%. The t(11;14)(q13;q32) and/or bcl-1 rearrangement was detected in 47/57 studied cases. Median overall survival (OS) was 3.12 years and a longer survival was significantly associated with younger age (<70 years), good PS (<2), localized disease (stage I-II), fewer than two extra-nodal sites, absence of spleen or peripheral blood involvement, normal serum LDH and beta2-microglobulin levels, and hemoglobin level greater than 12 g/dl. However, the IPI failed to identify patients with longer OS and in a multiparametric analysis, only older age, hemoglobin less than 12 g/dl, poor PS, and blood involvement were associated with a poorer outcome. Treatment modalities had no impact on survival with 75% of patients relapsing or progressing. Our data showed that the poor outcome of MCL patients is mainly related to adverse patient characteristics, a highly disseminated tumor, and some unknown parameters associated with the refractoriness to standard therapy.     

Renal disease in an adult patient with type I glycogen storage disease

A 26-year-old Chinese male patient with type I glycogen storage disease presented with chronic renal disease, proteinuria, and urolithiasis. On renal biopsy, focal glomerular sclerosis, increased mesangial matrix and cellularity, interstitial fibrosis, tubular atrophy, and prominent arteriosclerosis were observed. Immunofluorescence microscopy revealed Ig A deposits predominantly in the glomerular mesangium. The possible mechanisms of renal involvement in glycogen storage disease are briefly discussed.     

Renal glomerular fibrosis in a cat

Renal glomerular fibrosis was observed in a 1-year-old spayed female Japanese domestic cat that showed clinically advanced renal failure. In the glomeruli, increased homogeneous materials were stained strongly with aniline blue by Masson's trichrome and positive for anti-type III collagen antibody by immunohistochemical staining, causing mesangial sclerosis and capillary collapse. By electron microscopy, randomly arranged fibrils were observed in the expanded subendothelial and mesangial areas, and the fibrils showed periodicity characteristic of collagen fibers in longitudinal sections. These findings of glomerular lesions closely resemble those of human "collagenofibrotic glomerulonephropathy," which has recently been described as a new type of glomerulonephropathy.     

CT findings in chondroradionecrosis of the larynx

PURPOSE: Our goal was to describe the CT findings before and after radiation therapy in a series of patients with laryngeal chondroradionecrosis. METHODS: The CT studies obtained before and after radiation therapy in nine patients with the diagnosis of laryngeal chondroradionecrosis were reviewed retrospectively. RESULTS: CT scans revealed abnormalities in all patients. A variable degree of laryngeal soft-tissue swelling was seen in eight of the patients. In four patients, cartilaginous abnormalities were visible initially, and appeared in three of four other patients who had further follow-up CT studies. Six patients had involvement of the thyroid cartilage; collapse of the thyroid cartilage was seen in two cases and gas bubbles were visible adjacent to the thyroid cartilage in three cases. Four patients with involvement of the thyroid cartilage eventually underwent total laryngectomy, and one died suddenly in severe respiratory distress. In all three patients with arytenoidal involvement, anterior dislocation of this cartilage was seen; in two of these patients, the adjacent part of the cricoid cartilage showed some sclerosis. Two patients with arytenoidal necrosis (both with cricoidal sclerosis) kept a functional larynx. In one case, cricoidal sclerosis was seen in association with lysis of the thyroid cartilage. CONCLUSION: The CT appearance of laryngeal chondroradionecrosis is nonspecific, but the diagnosis can be strongly suggested in cases of sloughing of the arytenoid cartilage, fragmentation and collapse of the thyroid cartilage, and/or in the presence of gas bubbles around the cartilage.     

Oxalosis. An unusual cause of myelophthisis in childhood

In a child with renal failure and oliguria due to hyperoxaluria myelophthisis developed as a result of extensive bone-marrow replacement with calcium oxalate crystals and an accompanying fibrous proliferations. The histopathology associated with this metabolic disorder was demonstrated in posterior iliac crest bone-marrow trephine biopsies, renal biopsies, and nephrectomy specimens. Crystals were demonstrated in biopsy specimens of transplanted kidneys within six weeks following renal transplantation.