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Biomedical subjects

I Koni

Publications and source records attributed to I Koni.

At least 37 records · Page 2Linked to original sources

Decreased expression of 20-kD homologous restriction factor (HRF20, CD59) on T lymphocytes in Epstein-Barr virus (EBV)-induced infectious mononucleosis.

HRF20 (CD59) is one of the membrane-associated complement regulatory proteins. The characteristic function of CD59 is to prevent membrane attack complex (MAC) formation on the cell surface and to protect the cell from complement-mediated cell lysis. We examined the expression of CD59 antigen on T cell subpopulations in patients with acute infectious mononucleosis (IM) and analysed the relationship between the amount of CD59 expression and activation-induced cell death of mature T cells with apoptosis. Decreased expression of CD59 on CD8+ T cells, especially on CD45RO+ and HLA-DR+ activated T cells, was marked in acute IM patients. In contrast, activated CD4+ T cells from IM patients expressed as much CD59 antigen as CD4+ T cells from healthy volunteers. After incubation-induced cell death, viable CD8+ T cells showed normal amounts of CD59 antigen on their surface. CD59dim CD8+ T cells were more susceptible to apoptosis than CD59bright CD8+ T cells. These findings suggest that decreased expression of CD59 on CD8+ T cells may discriminate the susceptibility of activated CD8+ T cells to activation-induced cell death in IM.

Acute Disease↗

Angiotensin-converting enzyme inhibitors are associated with the need for increased recombinant human erythropoietin maintenance doses in hemodialysis patients. Risks of Cardiac Disease in Dialysis Patients Study Group.

The influence of angiotensin-converting enzyme inhibitors (ACEIs) on recombinant human erythropoietin (rhEPO) maintenance doses in hemodialysis patients was studied. One hundred and eight chronic hemodialysis patients (55 males and 53 females, mean age 61.2+/-12.6 years) were investigated. The rhEPO maintenance doses in the ACEI-treated group (n = 49) were 101.7+/-51.7 U/kg/week and in the nontreated group (n = 59) 79.2+/-37.8 U/kg/week (p < 0.05). No difference was observed in hematocrit between the ACEI-treated and nontreated groups. In stepwise regression analysis, the parameters associated with increased rhEPO maintenance doses were female gender, ACEI administration, low total iron binding capacity, and low serum free carnitine levels. In conclusion, ACEI administration might reduce the response to rhEPO. In hemodialysis patients who need high-dose rhEPO to maintain the target hematocrit in the absence of iron deficiency, hyperparathyroidism, infection, malignancy, malnutrition, and aluminum toxicity, ACEI administration should be considered.

Adult↗

Absence of CD69 expression on peripheral eosinophils in episodic angioedema and eosinophilia.

A 45-year-old woman with episodic angioedema and eosinophilia is presented. CD69, which is one of the surface antigens of activated eosinophils, was not expressed on the peripheral eosinophils in this patient, in contrast to hypereosinophilic syndrome. This suggests that CD69, which is not dependent on eosinophil density, may be another useful activation marker of eosinophils to distinguish episodic angioedema and eosinophilia from hypereosinophilic syndrome.

Angioedema↗

Severe gastrointestinal complications of dialysis-related amyloidosis in two patients on long-term hemodialysis.

Two patients undergoing long-term hemodialysis developed severe gastrointestinal complications due to dialysis-related amyloidosis (DRA). Case 1, a 68-year-old male on hemodialysis for 18 years developed marked gastric dilatation and severe paralytic ileus. Five years later he died of peritonitis. Autopsy showed extensive amyloid deposits in the muscle layers and blood vessels throughout the gastrointestinal tract. Case 2, a 56-year-old male on hemodialysis for 19 years, developed panperitonitis due to perforation of the sigmoid colon. The resected colon showed massive amyloid deposits in the muscle layers. In both cases, immunological studies revealed positive staining for antihuman beta2-microglobulin antibody. In long-term hemodialysis patients with gastrointestinal symptoms, gastrointestinal complications of DRA should be considered.

Aged↗

Correlation between serum carnitine levels and erythrocyte osmotic fragility in hemodialysis patients.

The relationship between serum carnitine levels and erythrocyte osmotic fragility was investigated in 26 chronic hemodialysis patients (10 males and 16 females, mean age: 57.3 +/- 13.5 years). Serum total-carnitine (TC), free-carnitine (FC) and acyl-carnitine (AC) levels were determined by a spectrophotometric method. Erythrocyte osmotic fragility was measured with a coil planet centrifuge. Serum TC levels were 39.9 +/- 13.4 mumol/l (mean +/- SD), FC levels were 21.8 +/- 7.8 mumol/l and AC levels were 18.0 +/- 9.6 mumol/l. The mean hemolysis end point (HEP) was 67.4 +/- 5.4 mOsM, the hemolysis maximum point (HMP) was 86.3 +/- 5.4 mOsM and the hemolysis start point (HSP) was 101.2 +/- 4.4 mOsM. Each hemolysis point in hemodialysis patients was elevated in comparison with the normal range. There were no significant differences in hemolysis points between a recombinant human erythropoietin (rhEPO)-treated group and nontreated group. HEP correlated with serum TC (r = -0.56, p < 0.01) and AC levels (r = -0.58, p < 0.01). HMP correlated with serum TC (r = -0.42, p < 0.05) and FC levels (r = -0.41, p < 0.05). Dose requirement of rhEPO maintaining target hematocrit correlated with serum TC (r = 0.54, p < 0.05) and FC levels (r = 0.50, p < 0.05). These data support that low serum carnitine levels accelerate erythrocyte osmotic fragility. Carnitine may contribute to the metabolism of erythrocyte membrane and have an impact on the efficacy of rhEPO in correcting renal anemia.

Adult↗

Increased plasma levels of immunoreactive endothelin and von Willebrand factor in NIDDM patients.

OBJECTIVES: To elucidate the significance of plasma levels of endothelin (ET) and von Willebrand factor (vWF) as possible markers for endothelial dysfunction in non-insulin-dependent diabetes mellitus (NIDDM). RESEARCH DESIGN AND METHODS: Plasma levels of ET and vWF were determined in 22 NIDDM patients with or without retinopathy and 10 normal control subjects. RESULTS: The plasma levels of immunoreactive endothelin (irET) and vWF in NIDDM patients were 0.78 +/- 0.06 pmol/l and 218.3 +/- 18.4%, respectively, which represented significant (P < 0.05, P < 0.01, respectively) differences from the values in the control group (0.50 +/- 0.06 pmol/l and 139.1 +/- 11.1%, respectively, n = 10). However, when the diabetic patients were divided into two groups according to the presence or absence of diabetic retinopathy, the plasma levels of irET and vWF in the NIDDM patients with retinopathy were significantly higher (1.01 +/- 0.07 pmol/l and 283.0 +/- 21.4%, respectively, n = 12) compared with the control group and NIDDM patients without retinopathy (0.59 +/- 0.06 pmol/l and 164.3 +/- 17.0%, respectively). Plasma levels of irET showed a significant (P < 0.01) positive correlation with the levels of vWF. CONCLUSIONS: These data strongly suggest that increased plasma irET reflects the endothelial cell damage in NIDDM.

Adult↗

[EBER-1 expression in salivary glands of Sjögren's syndrome].

EB virus-encoded small nuclear RNAs (EBERs) which present in large numbers during persistent infection, are known to stably bind to SSB/La antigen, leading to speculation that EBV may be involved in the pathogenesis of primary Sjögren's syndrome (1 degree SjS). Although studied by many investigators serologically, immunohistochemically and molecular biologically, this remains controversial. In situ hybridization study for EBER-1 showed positive hybridization in ductal epithelial cells in nine of eighteen salivary glands (SGs). Hybridization was more intense in ducts surrounded by infiltrating mononuclear cells (MNCs). Positive hybridization was also seen in infiltrating MNCs in eight 1 degree SjS SGs. These results indicate that EBV plays some pathogenic roles in some cases of 1 degree SjS through induction of SSB/La antibody and impairment of lymphocytes.

Autoantigens↗

Goodpasture's-like syndrome and effect of extracorporeal membrane oxygenator support.

A 49-year-old man was admitted because of general fatigue, cough and hematuria. During the hospital course, acute renal failure, hemoptysis and dyspnea developed. A percutaneous renal biopsy revealed a diffuse crescentic glomerulonephritis, and direct immunofluorescence showed a linear pattern of IgG along the glomerular basement membrane. Although serum anti-glomerular basement membrane (anti-GBM) antibody was not detected. Goodpasture's-like syndrome was suspected, and methylprednisolone pulse therapy and plasmapheresis were administered. Concomitantly, extracorporeal membrane oxygenation (ECMO) was instituted because of deterioration in respiratory status due to a severe pulmonary hemorrhage despite maximal ventilatory support. Temporarily, the patient improved and ECMO was discontinued. ECMO may be a useful therapeutic support for hypoxia resulting from pulmonary hemorrhage in Goodpasture's syndrome (GPS) and Goodpasture's-like syndrome.

Anti-Glomerular Basement Membrane Disease↗

[A case of renal hypouricemia associated with IgA nephropathy--with special reference to changes in serum uric acid and uric acid clearance in a clinical course exceeding 15 years].

We report a case of IgA nephropathy associated with renal hypouricemia. The patient's renal function had decreased gradually during the previous 15 years, resulting in chronic renal failure. Levels of serum uric acid and uric acid clearance were 1.0 mg/dl and 39.4 ml/min, respectively. Pyrazinamide suppression test indicated that the patient had defective tubular reabsorption of uric acid at the presecretory site. The serum uric acid level elevated linearly to 5.0 mg/dl during the 15-year period, in parallel with a change in serum creatinine level, giving a significantly positive correlation coefficient of 0.9776. The ratio of uric acid and creatinine clearances showed no significant change, although both decreased during the 15 years. These results were a different from those in patients with chronic glomerulonephritis, including IgA nephropathy, where the serum uric acid level had shown no significant correlation with serum creatinine level above 2.0 mg/dl and the ratio of uric acid clearance and creatinine clearance had exponentially elevated after the latter decreased below 30-40 ml/min. The patient presented here has defective tubular transport of uric acid at the site of urate reabsorption, and appears to show a different tubular dysfunction response from patients with chronic glomerulonephritis and decreased renal function.

Creatinine↗

Hereditary angioedema complicated with chronic renal failure: report of sibling cases.

Hereditary angioedema (HAE) is known as a deficiency state of C1 inhibitor (C1 INH), an important protease inhibitor protein involved in the complement system. As with other components of the classical pathway of the complement system, a state of its deficiency often causes clinical immunoregulatory disorders. A 45-yr-old brother and a 63-yr-old sister with HAE both developed chronic renal failure, probably due to chronic glomerulonephritis, and required regular hemodialysis. This is, to our knowledge, the first report of sibling cases of HAE associated with chronic renal failure.

Angioedema↗

[Guanidino compounds in the elderly].

To examine the metabolism of guanidino compounds in the elderly, we measured the serum concentrations of urea nitrogen, creatinine (Cr), guanidinoacetic acid (GAA) and creatine (CR) in middle-aged and elderly subjects. We also measured muscle mass in the elderly. The elderly subjects tended to have lower serum GAA concentrations than middle-aged subjects. On the contrary, CR concentrations of elderly subjects were higher than those of middle-aged subjects. Bedridden elderly subjects tended to have lower serum GAA concentrations and lighter muscle mass than ambulatory elderly subjects. On the contrary, serum CR concentrations of bedridden subjects were higher than those of ambulatory subjects. CR is an essential substance for muscle energy metabolism. These results indicate that high serum CR concentrations due to low CR metabolism in skeletal muscle might suppress glycine amidinotransferase (GAT) activity, resulting in decreased GAA production in the elderly.

Aged↗

[Acid-base disturbance in adrenal and parathyroid diseases].

Aldosterone stimulates sodium absorption in the collecting tubule, which makes the potential more lumen-negative, and secondarily stimulates proton and potassium secretion. Potassium depletion stimulates ammonia synthesis, which increases distal buffer delivery, increases luminal pH in the collecting tubule, and secondarily stimulates proton secretion. This stimulation of the distal nephron acidification by aldosterone can result in metabolic alkalosis. Inversely, aldosterone deficiency can produce characteristic metabolic acidosis with hyperkalemia (type IV renal tubular acidosis). Acute administration of parathyroidhormone (PTH) decreases renal bicarbonate reabsorption in the proximal tubule. Since the effect of PTH is overridden by other factors, such as PTH-induced base release from bone, hyperparathyroidism does not uniformly cause metabolic acidosis.

Acidosis↗