We tested for antibodies to hepatitis B virus (HBV), hepatitis C virus (HCV), and human T lymphotropic virus type-I (HTLV-I) in 629 normal inhabitants of an adult T cell leukemia (ATL) endemic area and in patients with ATL, HTLV-I associated myelopathy (HAM), and hepatocellular carcinoma (HCC) from the same district. The prevalence of serological positivity for each virus was 28.0, 6.4, and 32.6%, respectively, among the 629 inhabitants. There was a positive association between the presence of anti-HCV and serological HTLV-I positive or negative status of these subjects (9.3% vs 5.0%). Conversely, there was no correlation between HBV and HTLV-I serologic prevalence. Only inhabitants positive for anti-HCV showed significantly high serum aminotransferase levels. The levels were not affected by superimposed HTLV-I infection among anti-HCV positives. Fifty three percent of HCC patients were positive for anti-HCV; 35% of whom were simultaneously positive for antibody to HTLV-I. On the other hand, only 2 ATL patients (4.2%) and 2 HAM patients (7.7%) had anti-HCV. These findings suggest that high serum aminotransferase levels are mainly caused by HCV infection and persons with HCV and HTLV-I double infections are at a high risk for the development of HCC but not ATL or HAM.
IgA1(κ)-transferrin complex was ascertained in a case of multiple myeloma with hypersiderinemia. On gel filtration, this complex disclosed an orange-colored protein with two peaks between the 19S and 7S fractions. On polyacrylamide gel electrophoresis and Western blotting analysis, the complex migrated at molecular weights of 430 and 700 kD in the non-reducing condition. A small amount of free transferrin was detected simultaneously. The complex was further separated into monoclonal IgA1(κ) and transferrin at pH 4.5; these recombined at pH 7.2 after incubation (37°C, 2 h). We also showed that the pepsin-digested F(ab')2 fragment of IgA1(κ) combined with the transferrin. From these results it can be suggested that monoclonal IgA1(κ) has an antibody activity for the transferrin. Clinically, this complex disturbed the iron transport to erythroid cells in the bone marrow, resulting in hypersiderinemia, anemia, and iron deposits in the liver.
Sex and age differences of ventricular gradient (G) were analyzed and normal ranges were determined in normal Japanese subjects (120 men and 74 women), whose resting heart rate was within the range of 65 to 75 beats/min. Women showed a larger Z component in ÂT and a smaller Y and larger Z component in G than men. The common trend in age differences was a decrease in Y component of G in both men and women. The ranges obtained in the present study can be applied in automatic VCG analysis, in particular for the differentiation of primary and secondary ST-T changes.
Two-color immunofluorescence using multiparameter flow cytometry was employed to examine the antigenic characteristics of peripheral blood lymphocytes in whole blood of healthy Caucasians and Japanese. The CD4/CD8 ratio in Japanese was significantly decreased compared with that in Caucasians, because of the increased number of CD8+ cells. Although the proportions of suppressor-inducer T cells (CD4+, Leu-8+) and helper-inducer T cells (naive T cells) (CD4+, CD45RA-) were low in Japanese subjects, there were no differences in the absolute numbers of suppressor-inducer T cells and helper-inducer T cells (naive T cells) in circulation. The level of activated T cells in Japanese was similar to that in Caucasians. NK cells, CD57+, CD8+ cells and CD57+, CD3+ cells were high in Japanese. Regarding B cell subsets, CD5+ B cells and activated B cells remained unchanged. However, there were slight differences in Leu-8+ B cells and Fc ε R+ B cells (CD20+, CD23+) between the two groups. Thus, a differing influence of racial and environmental background between healthy Caucasians and healthy Japanese on human lymphocyte subsets is present in the lymphocyte immunophenotype.
Immunohistochemical studies were performed on muscle biopsy specimens of polymyalgia rheumatica (PMR) to evaluate the extent of muscle and peripheral nerve involvement. The routine histochemistry revealed a mild variation of fiber size, type 2 fiber atrophy and type 2A or 2B fiber deficiency. In 63% of the PMR cases small angular fibers, pyknotic nuclear clumps or target-targetoid fibers were observed, suggesting neurogenic changes, although abnormalities were mild in degree. In immunocytochemical studies, neither major histocompatibility complex (MHC) class 1 nor class 2 products were expressed on the muscle surface membrane of PMR. But regarding intramuscular vessels, MHC class 2 products were distinctly visualized. On serial sections, combined deposits of IgG and Clq in perimysial arteries were seen in 38% of PMR. These results suggest that arteries of small caliber might be involved in immunopathological processes, causing muscle and peripheral nerve damage.
Interactions of lipoproteins containing apolipoprotein (apo) A-I with or without apoA-II with human hepatoma cell line HepG2 were studied to investigate the ligand specificity for high density lipoprotein receptor on human hepatic cells and their metabolism. The two types of lipoproteins were isolated by immunoaffinity chromatography, in which apoE-containing lipoproteins were removed. Specific binding kinetics at 0°C were observed for the apoA-I-containing lipoproteins with or without apoA-II (Kd = 18 or 20 μg protein/ml, Bmax = 110 or 120 ng/mg cell protein, respectively). The binding of these lipoproteins to HepG2 cells was competitively inhibited by excess unlabeled apoA-I-containing lipoproteins or apoA-I•phospholipid complexes, but not by apoA-II•phospholipid complexes. Interactions of these lipoproteins with HepG2 cells at 37°C were further examined. These results suggested that HepG2 cells have a specific binding site for apoA-I-containing lipoproteins, and that apoA-I might be a crucial component in the binding of these lipoproteins to human hepatic cells.
The geographic distribution and clinical features of patients with HTLV-I-associated myelopathy (HAM) in Tokushima prefecture were investigated. Nine patients were found prior to December 1990. The minimal prevalence was estimated as 1.1 per 100, 000 in the general population, and 1 per 1, 309 in HTLV-I-seropositive persons. Seven patients were found in the southern district facing the Pacific Ocean, but only 1 patient each was found in the northern and western districts. The age at disease onset ranged from 15 to 53 yr (average 33 yr). The ratio of male to female patients was 1:8. Adult T cell leukemia was associated with HAM in 1 patient, and Hashimoto's disease in 2 patients. These cases have not been reported previously. The route of transmission of HTLV-I was concluded to be vertical in 4 patients and horizontal in 4 patients, but was uncertain in 1 patient. No evidence of transmission by blood transfusion was found in these patients.
The dopamine system may be involved in three situations: the nigral projection to the basal ganglia, the mesocortical projection to the anterior cingulate gyms, or the medial forebrain bundle projection to cortical and limbic sites. Because of the close association of dopamine systems with the known neurological syndromes of akinesia, we elected to treat a patient with akinesia due to rupture of anterior communicating artery (ACA) aneurysm with the dopamine agonist, bromocriptine. This case has important implications for the understanding of brain/behavior relationships as well as for the development of new therapies for patients who have sustained neurological injury.
A patient with hypophosphatemic vitamin D-resistant rickets developed secondary erythrocytosis during treatment with large doses of vitamin D2 and phosphate. Erythrocytosis was accompanied by a fall in circulating plasma volume and appeared to have developed as a consequence of nephrocalcinosis because it occurred after the appearance of nephrocalcinosis following several episodes of hypercalcemia and hyperphosphatemia. Nephrocalcinosis and erythrocytosis did not disappear even after recovery of renal function. Thus, the present observations point to the importance of preventing these irreversible complications that could cause renal failure, erythrocytosis, and thrombotic events during the management of hypophosphatemic vitamin D-resistant rickets.
A case of acute interstitial nephritis associated with uveitis is presented. An 18-year-old man developed uveitis followed by renal dysfunction. Moderate renal dysfunction (sCr 3.0 mg/dl), and increased levels of urinary β2 microglobulin (over 8, 000μg/l) were observed. Renal tissue obtained by open renal biopsy revealed severe infiltration of mononuclear cells in the tubulo-interstitial area. The majority of infiltrating cells were CD4-positive T cells demonstrated by immunoperoxidase staining, suggesting the involvement of T cell-mediated delayed hypersensitivity. Corticosteroid was effective against both uveitis and renal dysfunction. The possible pathogenesis of this syndrome is reviewed from the literature.
Co-trimoxazole (trimethoprim-sulfamethoxazole) and pyrimethamine-sulfadoxine have been recommended for treatment and prophylaxis, respectively, of Pneumocystis carinii pneumonia (PCP). However, toxic epidermal necrolysis (TEN) occurred in three of four patients with acquired immunodeficiency syndrome (AIDS) during the course of treatment or prophylaxis of PCP with these agents. On the other hand, 14 patients with PCP treated with pentamidine never developed TEN. Because the incidence of adverse skin reactions is higher among patients with AIDS than those without AIDS, and TEN is a severe, potentially fatal skin reaction, sulfonamide-containing drugs should be given cautiously to patients with AIDS.
We report a 29-year-old male with pelvic lipomatosis that presented with edema of the lower extremities. The patient visited our department because of the marked edema of the lower extremities with body weight gain. The pelvic region was generally radiolucent on plain abdominal radiogram. Excretory urogram and pelvic computed tomography scan revealed compression of the bladder by an adipose tissue mass. On lymphograms, lymph vessels were compressed from the inguinal area in the common iliac region. The edema of the lower extremities was thought to be caused by lymph vessel obstruction.
A 54-year-old man complained of burning pain, warm skin and erythema in his extremities. A diagnosis of primary erythromelalgia was made. Microneurography was used to clarify the role of skin sympathetic nerve activity in the pathophysiology of primary erythromelalgia. The patient showed normal skin sympathetic nerve activity but no vasoconstriction response. Aspirin activated the skin sympathetic nerve activity and improved vasoconstriction producing symptomatic relief. These results suggest that the lack of vasoconstriction following vasoconstrictor activity of the skin sympathetic nerves results in increased skin blood flow and burning pain.
Mixed connective tissue disease (MCTD) is characterized as a benign rheumatic disease with a favorable response to therapy. When pulmonary hypertension is a complication, however, it is often reported to be fatal. A 32-year-old female patient with MCTD who had developed rapidly progressive pulmonary hypertension and disseminated intravascular coagulopathy was admitted to our hospital and was successfully treated with corticosteroids and anticoagulants. The failure of microcirculation due to coagulopathy is considered to be one of the possible entities of pulmonary hypertension.
A 59-year-old male with acute myocarditis presenting symptoms resembling acute myocardial infarction underwent urgent coronary angiography due to difficulties in determining a diagnosis. Coronary artery spasm was demonstrated in the proximal right and left anterior descending coronary arteries. The spasm varied from time to time and lesion to lesion. But no fixed occluded lesion was observed. Predischarge coronary angiography showed no abnormal findings. Histological findings of the biopsy specimen from the left ventricle were consistent with myocarditis. The findings of this case indicate that a coronary artery spasm may aggravate the myocardial damage occurring in acute myocarditis. Urgent coronary angiography and endomyocardial biopsy are important to make the diagnosis of myocarditis in patients presenting symptoms resembling acute myocardial infarction.
We report a case of myasthenia gravis (MG) which became worse shortly after the administration of methimazole (MMI) for hyperthyroidism. The activation of immune responses was found during the worsening of MG. The findings suggests the possibility that the worsening of MG might be induced by MMI, presumably by its immunomodulatory property.
We report a case of HAM/TSP presenting with short stature, mental retardation, skin eruptions, uterine and ovarian hypogenesis and nephropathy. Skin erythema was noted since from the age of three years old and spasticity of lower extremities from elementary school age. Serum calcium level showed 4.1 mEq/l. Recombinant human PTH infusion resulted in no response of phosphate excretion. The persistent proteinuria prompted renal needle biopsy, which revealed IgA and C1q deposits in glomerular mesangium. A diagnosis of pseudohypoparathyroidism and IgA nephropathy was entertained. This patient with pseudohypoparathyroidism who has a deficient immune system was seized with the early onset of HAM/TSP and IgA nephropathy.
We report a case of IgM-associated mesangial proliferative glomerulonephritis with microscopic hematuria and renal tubular dysfunction. On electron microscopy, a giant spherical deposit was observed in the glomerular paramesangial area. Deposition of C3 in the glomerular mesangial areas was not observed by immunofluorescence. IgM-associated mesangial proliferative glomerulonephritis is clinically characterized by nephrotic syndrome, mild proteinuria and/or hematuria, tubular dysfunction or renal failure, suggesting that it is considered to be a heterogeneous disorder.
Granulocyte colony-stimulating factor (G-CSF) is a glycoprotein hormone which stimulates the proliferation and differentiation of a subset of granulocyte precursors and enhances some functional activities of mature neutrophils. We tested the effects of G-CSF on a patient with intractable infective endocarditis. The white blood cell count was increased 3-fold and the inflammatory reactions such as C-reactive protein and erythrocyte sedimentation rate were completely normalized without any side effects. This is the first report describing the use of G-CSF for infective endocarditis. Administration of G-CSF might be suitable for treating intractable infections which cannot be controlled by antibiotics alone.
We report a rare case of dry eyes and dry mouth caused by primary amyloidosis. A 66-year-old woman with keratoconjunctivitis sicca and xerostomia died of acute respiratory failure. Shirmer's test, gum test, and sialography indicated Sjögren's syndrome. Lip biopsy revealed amyloid deposition around the salivary ducts. Bence-Jones protein was noted in the urine. At autopsy, amyloid deposition was identified histochemically in many organs, mainly on the vessel walls. Primary amyloidosis should be considered as a differential diagnosis of Sjögren's syndrome.