[A case of myelofibrosis with multiple tumorous formation at the terminal stage].
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Biomedical subjects
Publications and source records attributed to H Wakui.
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1. On Western blot analysis, serum IgG from a healthy human subject reacted with a stress-induced protein, having an apparent molecular mass of 70 kDa, from PC12 cells. 2. This serum IgG also reacted with hsp70 (70-kDa heat-shock protein) purified from the bovine brain. 3. In 5 out of 34 healthy human subjects, IgG antibodies against hsp70 were detected. 4. These antibodies were directed against the stress-inducible 72-kDa protein, but did not cross-react with the constitutive 73-kDa protein.
We examined characteristics of contractile proteins of rat cultured mesangial cells. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis revealed that actomyosin was a major protein component in the extract of mesangial cells. By Western blot, tropomyosin, caldesmon, alpha-actinin, and vinculin were recognized in mesangial cells. The molecular masses of alpha-actinin and vinculin were the same as aortic smooth muscle. Mesangial cells contained five tropomyosin isoforms: TM-1, -2, -3, -4, and -5. TM-1 was present as a major isoform in mesangial cells, and it had an immunological cross reactivity with beta-tropomyosin of aortic smooth muscle. On the other hand, the molecular mass of caldesmon was similar to dermal fibroblasts, and differed from aortic smooth muscle. Immunofluorescent studies showed that the staining patterns of tropomyosin and caldesmon between cultured mesangial cells and cultured vascular smooth muscle cells were somewhat different. From these results, we conclude that mesangial cells abound in contractile proteins, and that the compositions of these proteins are similar to those of aortic smooth muscle with minor differences. Thus, this study appears to biochemically support a hypothesis that mesangial cells are derived from vascular smooth muscle cells, but with a minor modification in their evolution.
A 62-year-old man was admitted because of nausea and vomiting. Severe hyponatremia with renal sodium loss was found. Endocrinological studies revealed that the patient had isolated adrenocorticotropin (ACTH) deficiency and secondary adrenocortical insufficiency. Furthermore, an inappropriate secretion of antidiuretic hormone (ADH) in relation to the low plasma osmolality was observed at an early stage of hyponatremia. Hydrocortisone therapy effectively corrected his hyponatremia. Following the correction of hyponatremia, the value of free water clearance increased and the level of the plasma ADH decreased. Thus, the present case indicates that ACTH deficiency can cause the syndrome of inappropriate secretion of ADH.
The ddY mice are known to develop spontaneous glomerulonephritis resembling human IgA nephritis after 40 weeks of age. A sharp rise of circulating polyclonal IgG and IgA is also observed at this stage. Since these overproduced immunoglobulins seem to be related to the development of murine glomerulopathy, antigen-antibody interactions between renal tissue proteins and serum immunoglobulins were analyzed by Western blotting in ddY mice before and after 40 weeks of age. Serum IgG at 50 weeks reacted with an 18-kDa renal tissue protein which was identified as histone H3, as well as with histone H1. Renal histones were extracted along with IgG from the murine kidney at 50 weeks in a high salt soluble fraction. Serial studies of anti-histone antibodies by enzyme-linked immunosorbent assay showed that IgG class antibodies markedly increased after 40 weeks of age. IgA class antibodies mildly increased after 56 weeks of age. Anti-DNA antibodies were not detected. These results demonstrate that ddY mice also develop mainly IgG class and partly IgA class anti-histone autoantibody after 40 weeks of age, and that histone-anti-histone complexes may contribute to the development of murine glomerulopathy. Although anti-histone antibodies have been reported in lupus mice, ddY mice differ from these mice in that no anti-DNA antibodies develop.
Ordinary methods for purification of complex-forming glycoprotein (protein HC) or alpha 1-microglobulin, a protein closely related to protein HC, require either large volumes of urine or special collection of urine specimens from patients with tubular proteinuria. Here we describe a fast, efficient procedure for isolating protein HC in high yield from urines from healthy and diseased subjects, with use of Cibacron blue, hydroxylapatite, and gel chromatography. Using this procedure, we also obtained a considerable amount of polymeric protein HC from the urine of a patient with chronic renal failure. The pattern of charge heterogeneity and immunoreactivity with anti-protein HC differed between the polymeric and monomeric forms of protein HC. We also observed a variability in charge heterogeneity of protein HC among patients with renal disorders. These results demonstrate that this purification method is useful for further studies to elucidate the biochemical properties of protein HC and its clinical significance in renal disorders.
Ethylene oxide gas (EO gas) adsorbed onto sterilized objects was quantitated in an effort to take a safety measure against residual EO gas following its use in sterilization. We measured residual amounts of EO gas adsorbed onto laboratory wares, small medical tools and appliances just after post-sterilization airation to examine whether they were rendered entirely free from EO gas by the process. The results led us to arrive at the following conclusions: 1. EO gas was recognized to remain on sterilized objects even after allowing them to stand for about 16 hours following airation. 2. The amount of residual EO gas was smaller on metallic products and larger on rubber products (notably rubber gloves). An exceedingly high concentration of EO gas was noted to remain on a tooth-brush, among other plastic materials. These facts seem to indicate that the amount of residual EO gas is closely related to the texture and shape of materials to be sterilized. 3. As regards the potential toxicity of residual EO gas. However, their sensitivity was proved to be questionable, judgment on a result being subject to considerable individual variations. 4. Nevertheless, it is incontestable that these devices can at least provide a rough estimate of unavailing since monitoring by direct measurement of residual EO gas is virtually infeasible from a practical point of view.
We observed the presence of a new autoantibody, anti-erythrocyte protein 4.1, in a patient with autoimmune hemolytic anemia (AIHA). Western blotting analysis revealed that IgG from the patient's plasma reacted with erythrocyte protein 4.1. However, among other patients with hemolytic diseases (six having AIHA and three each having either hereditary spherocytosis, elliptocytosis, or lead poisoning) as well as among control subjects, no antibody activity to protein 4.1 was observed. In addition to the anti-protein 4.1 antibody, two different kinds of anti-erythrocyte antibodies were detected by conventional serological studies in this patient. One of them was an anti-Ena-like antibody in the eluate from the patient's erythrocytes, while another was the anti-S-specific antibody in the plasma. An elution study and an absorption study using S antigen-positive erythrocytes demonstrated that the anti-protein 4.1 antibody differed from both the anti-Ena-like antibody and the anti-S antibody. Familial analysis of the patient revealed the same antibody in her brother, who did not have hemolytic anemia. These results demonstrate that anti-protein 4.1 antibody is considered to be included in the spectrum of anti-cytoskeleton autoantibodies, which have been observed in patients having increased cell lysis as well as in healthy subjects.
Eleven patients with adult idiopathic thrombocytopenic purpura (ITP) were treated with bolus methylprednisolone at doses of 1 g/day for 3 days. In nine of eleven patients the platelet count rose to more than 100 X 10(9)/l during 2-9 days. In two other patients, it rose to 81 X 10(9)/l and 43 X 10(9)/l. In six of eleven patients splenectomy was carried out without any platelet transfusion when the platelets rose to more than 100 X 10(9)/l after bolus mPSL therapy. Bolus mPSL therapy is efficient as a pre-treatment for a planned splenectomy in adult ITP.
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Two patients with malignant lymphoma complicated by hemophagocytic syndrome (HPS) are reported. Their clinical signs at onset were similar to those of orbital cellulitis. Lymphoma complicated by hemophagocytosis is called lymphoma-associated hemophagocytic syndrome (LAHS) and its prognosis is reported to be very poor. As far as we know, this is the first report in the ophthalmological field. In our patients, we suspected that the lesions occurred from the orbit or skin of this area. The first patient was a 22-year-old man and the second patient a 16-year-old girl. The diagnosis was very difficult at the onset of disease. They died within 6 months after the first ophthalmological examination. When orbital cellulitis is suspected and antibiotic therapy is ineffective, we should suspect HPS and should examine serum ferritin, which is a good marker of HPS. Early biopsy and consultation with a hematologist are very important.
Several proteins interact with elastin, an essential extracellular matrix element. We describe here an additional elastin-binding protein from the porcine kidney. This protein was extractable with an isotonic buffer, and was purified by a rapid and simple procedure employing its characteristic properties: high affinity for red A-agarose and spontaneous formation of cryoprecipitate. Immunohistochemical studies using antibody against the purified protein demonstrated its localization in the cytoplasm of proximal tubules of the normal rat kidney. On Western blotting using the antibody, an immunoreactive protein was detected in the liver as well as in the kidney. On the basis of the partial amino acid sequences of the purified protein, it was identified as argininosuccinate synthetase. Some biochemical properties of the protein were different from the data reporting a hepatic form of argininosuccinate synthetase, a key enzyme in the urea cycle in the liver. These results suggest that the elastin-binding property of a renal form of the enzyme may be related to a tissue-specific function in the kidney.
We have purified a 17-kD puromycin aminonucleoside (PAN) binding protein from porcine kidney and identified it as the reported 17-kD protein kinase C inhibitor on the basis of its partial amino acid sequence. Of 54 determined amino acid sequences of the 17-kD porcine renal protein, 51 residues were identical to those of the 17-kD bovine brain protein kinase C inhibitor. However, our purified protein did not carry the inhibitory activity on protein kinase C. Immunohistochemical studies showed a unique intrarenal distribution of the 17-kD PAN-binding protein at the apical side of epithelial cells of Henle's loops in the inner medulla and of distal convoluted tubules. Immunoblot analysis revealed that the 17-kD PAN-binding protein was extractable by an isotonic buffer without sodium deoxycholate extraction. These results suggest that this protein binds loosely to the apical membranes of epithelial cells of Henle's loops and distal tubules and has specific functions related to tubular functions of these nephron segments at the apical side. Whether this protein is a real inhibitor of protein kinase C or not remains to be investigated.
We report an Rh(D)-negative man with myelodysplastic syndrome who produced six anti-erythrocyte alloantibodies (anti-D, -C, -E, -Dia, -Jka and -S) in succession. Three of these antibodies (anti-E, -Jka and -S) were not noted until delayed hemolytic transfusion reactions occurred. Treatment with cortico-steroids was effective in preventing both further formations of antibodies and other transfusion reactions. It was very difficult to find blood compatible with the patient, but repetitive blood transfusions were required for his progressive anemia and thrombocytopenia. Several problems concerning the transfusion of blood in such a case are discussed.
A 28-year-old woman developed central pontine myelinolysis (CPM) following severe hyponatremia. Radiological examinations demonstrated a characteristic pontine lesion of CPM. Her neurological symptoms (drowsiness, emotional lability, dysarthria, dysphagia, and quadriparesis) were improved dramatically by treatment with thyrotropin-releasing hormone (TRH) and rehabilitation. However, results of repeat computed tomographic (CT) scans of the brain remained unchanged. This case therefore suggests that TRH may be beneficial for the treatment of CPM, and that CT findings appear to be a limited prognostic indicator for CPM.