Phenotypic analysis of circulating and intrahepatic dendritic cell subsets in patients with chronic liver diseases

BACKGROUND/AIMS: Dendritic cells (DCs) are the most potent professional antigen-presenting cells. Although two subsets of circulating DCs, lineage(-)CD11c(+)CD4(low) (CD11c(+)DCs) and lineage (-)CD11c(-)CD4(+)CD123(+) (CD123(+)DCs) are identified in humans, the role of each DC subset in the immunopathogenesis of liver diseases is unknown.METHODS: We examined the numbers and activation status of each DC subset in the circulation and in the inflamed livers in patients with chronic liver diseases by flow cytometry and immunohistochemistry.RESULTS: The numbers of circulating CD11c(+)DCs were inversely correlated with serum alanine aminotransferase (ALT) levels in patients with chronic viral hepatitis, and that the expression of costimulatory molecules on circulating CD11c(+)DCs in patients with chronic viral hepatitis was significantly up-regulated in patients with high serum levels of ALT. Both DCs are also identified in the livers by flow cytometry, and the expression of costimulatory molecule CD40 on those DCs was significantly higher in liver DCs than that in circulating DCs. Moreover, the ratios of CD11c(+)DCs/CD123(+)DCs were higher in liver DCs (mean+/-SD, 7.2+/-6.0) than those of circulating DCs (4.0+/-4.6). Immunohistochemically, CD11c(+) or CD123(+) cells and CD83(+) activated DCs were observed mostly in portal areas with mononuclear cell infiltration in various liver diseases. These overall data suggest that DCs, especially CD11c(+)DCs, could be associated with the necroinflammatory response in the liver of chronic viral liver diseases.CONCLUSIONS: DCs, especially CD11c(+)DCs, may be involved in the immunopathogenesis of chronic liver diseases.     

Problems in serum albumin measurement and clinical significance of albumin microheterogeneity in cirrhotics

OBJECTIVES: To clarify problems with the determination of serum albumin levels, the definition of hypoalbuminemia, and the implications of microheterogeneity of albumin, serum albumin was measured by using dye-binding methods and the authentic method (immunoassay) in patients with liver cirrhosis and healthy subjects. METHODS: We enrolled 103 patients with liver cirrhosis and 36 healthy subjects. Serum albumin levels were analyzed by immunoassay and the bromcresol green and bromcresol purple methods. Oxidized albumin and glycoalbumin were determined by high-performance liquid chromatography. RESULTS: In cirrhotic patients, serum albumin levels measured by the bromcresol green method was about 0.2 g/dL higher than that by immunoassay. Serum albumin levels measured by the bromcresol purple method also was higher in cirrhotic patients than those measured by immunoassay and varied widely. In addition, extensive variation was found across serum albumin levels determined by the bromcresol green method at individual institutions (five university hospitals) and those determined by immunoassay at a contract laboratory. The percentages of oxidized albumin and glycoalbumin within total serum albumin increased with progression of liver disease. Further, an increase in oxidized albumin led to an increase in the albumin level as measured by the bromcresol purple method. CONCLUSION: These results show that adequate assessment of the pathophysiology and prognosis of patients with liver cirrhosis and the efficacy of treatment is not possible with dye-binding methods for determination of serum albumin. Further, the conventional definition of hypoalbuminemia as a serum albumin level of 3.5 g/dL or lower should be reconsidered, and the clinical implications of qualitative changes in albumin should be investigated in consideration of the microheterogeneity of albumin, such as oxidized albumin and glycoalbumin.     

A New Instrument for Measurement of Gastrointestinal Mucosal Color

Abstract: We designed and produced a new instrument that measures the color of the gastrointestinal mucosa during conventional endoscopic examination. The instrument consists fundamentally of an optical fiber sensor and a light source. One end of the optical fiber sensor is connected to the light source and a commercially available spectrophotometric colorimeter, while the other end is passed through the biopsy channel of a commercially available endoscope and placed in contact with the gastrointestinal mucosa. The color of the mucosa is then measured. To evaluate the accuracy of color measurement, the instrument was used to measure the color of 108 color chips belonging to the color range of the gastrointestinal mucosa or surrounding regions, selected from the Munsell Book of Color. The instrument correctly determined the relative hue, chroma and value of all 108 color chips tested. It also had the advantages of consistently measuring the color chips under standardized conditions and of promptly displaying the color readings during endoscopic examination. This instrument was also used to measure the color of the mucosa at different sites of the upper gastrointestinal tract in 20 patients. There were no noteworthy problems in using the instrument clinically. Mucosal color (hue, value and chroma) of the upper gastrointestinal tract was demonstrated to have specific characteristics according to the region measured. The instrument provided highly accurate, objective information that facilitated the distinction of slight differences in mucosal color, an important factor in the endoscopic diagnosis of gastrointestinal diseases.     

GENERALIZED FIBROSIS ASSOCIATED WITH PULMONARY ASBESTOSIS

An autopsy case of pulmonary asbestosis with fibrosis of various organs was reported. Pulmonary carcinoma and glomerulonephritis were complicated. Asbestos bodies (ABs) and fibers (AFs) were identified in the organs with fibrosis such as the lung, liver, kidney, heart, and thyroid gland by electron microscopy and digestion method. Moreover, we identified AFs in the spleen by electron microscopy which had been resected due to idiopathic portal hypertension six years previously. It is suggested that AFs can be transported to various organs and persist for a long period of time to cause pathological changes there.     

BEHÇET SYNDROME ASSOCIATED WITH NASAL MALIGNANT LYMPHOMA – REPORT OF AN AUTOPSY CASE –

The present case deals with a patient who was suffering concurrently from malignant lymphoma of the nasal cavities and Behcet disease accompanied with colitis ulcerosa.
A possibility that Behçet syndrome might be accompanied with malignant tumor is discussed. Moreover, it may be possible to point out the presence of such a type of Behçet syndrome as a "tumoral Behcet".     

Congenital Hemangiopericytoma: Report of a Case

(Received for publication on Feb. 12, 1999; accepted on Nov. 11, 1999)     

Liver bilirubin UDP-glucuronosyltransferase activity in chronic nonhemolytic unconjugated hyperbilirubinemia of adults

Ten adult patients with chronic nonhemolytic unconjugated (indirect) hyperbilirubinemia were analyzed by determining bilirubin uridine diphosphate-glucuronosyltransferase activity according to a more physiological and sensitive method (9 control cases, 0.457±0.163 nmole/mg protein/min). There was no overlap of the enzyme activities of 2 cases with Crigler-Najjar syndrome (type II) (0.006 nmole/mg protein/min on average) and 6 cases with Gilbert’s syndrome (0.051±0.016 nmole/mg protein/min). The enzyme activities in 2 patients with post-hepatitic hyperbilirubinemia were within the normal range. A new classification of nonhemolytic unconjugated hyperbilirubinemia in adults is proposed according to the results of this enzyme activity and the recent data on the gene mutation of this enzyme.     

Rapid recovery from anorexia nervosa after a life‐threatening episode with severe thrombocytopenia: Report of three cases

We report the cases of three patients with anorexia nervosa (AN) who each recovered rapidly after experiencing a life‐threatening episode with severe thrombocytopenia. All three cases were the typical restricting‐type of AN, occurring in adolescence. They refused to be admitted to a hospital until their general condition had been severely deteriorated. Their lowest platelet counts were 2.9, 4.6, and 2.3 × 10⁴/mm³, respectively. Apparent hemorrhagic tendencies, such as purpura, gingival and nasal bleeding, and gastrointestinal bleeding were observed. The bone marrow examination showed apparent hypoplasia in two patients. No evidence of disseminated intravascular coagulation or autoantibody to platelets was detected. The platelet counts recovered rapidly by water and nutritional supplementation. The recovery from the AN itself was excellent in all three patients without specific psycho‐ therapy. © 1999. John Wiley & Sons, Inc. Int J Eat Disord 25:113–118, 1999.     

Surgical treatment for a supra sinotubular junctional saccular aneurysm associated with aortic regurgitation

We reported a patient with a saccular ascending aortic aneusysm located just above the non-coronary sinotubular junction. The aneurysm produced severe aortic regurgitation and two episodes of cardiac tamponade. By intraoperative inspection, the border between the aneurysmal wall and non-dilated portion of the normal aortic wall was distinct, and the aortic valve leaflets and aortic annulus appeared normal. Aortic valve dysfunction appeared to be caused by dilation of the noncoronary sinotubular junction and mild distortion of the noncoronary sinus because of the aneurysmal formation. We performed patch closure of the aneurysmal ostium and repaired the dilated noncoronary sinotubular junction. Postoperative echocardiography and aortograpy demonstrated a good coaptation of the aortic valve leaflets with trivial aortic regurgitation. Although a rupture site, dissection or carcinomatous pericarditis which is attributable to the two episodes of cardiac tamponade could not be found, pathologic examination of the aneurysm wall revealed intramural blood leakage between the mucoid degenerated media and notably thickened adventitia. In addition, there was thinning and interruption of the elastic fibers of the media. These findings are consistent with a leaking aneurysm which cause the slow development of cardiac tamponade.