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Biomedical subjects

K Nishiya

Publications and source records attributed to K Nishiya.

At least 55 records · Page 3Linked to original sources

A case of Gitelman's syndrome with chondrocalcinosis.

A 45-year-old Japanese woman, treated for Bartter's syndrome for 14 years, presented with complaints of numbness in her extremities and polyarthralgia. She was diagnosed to have Gitelman's syndrome with chondrocalcinosis, which were effectively treated with spironolactone and magnesium supplementation. Gitelman's syndrome is a primary renal tubular disorder characterized by hypomagnesemia and hypocalciuria with normal calcemia. The persistent hypomagnesemia is one of the causes of chondrocalcinosis, and many cases of Bartter's syndrome with hypomagnesemia are associated with chondrocalcinosis attributed to a tubular magnesium defect. We summarize the reported cases with Bartter's syndrome and chondrocalcinosis, referring to the possibility of Gitelman's syndrome.

Bartter Syndrome↗

[A case of pityriasis rubra pilaris associated with unclassified connective tissue disease and sepsis in clinical course].

The patient was a 23-year-old woman with HTLV-I carrier and was diagnosed as pityriasis rubra pilaris soon after her birth. In November 1990 at her age of 16, she began to have fever and polyarthralgia which were not improved despite the administration of antibiotics. Her laboratory findings showed the positive antinuclear antibody and anti-RNP antibody suggesting one of collagen diseases. A tentative diagnosis as unclassified connective tissue disease (UCTD) was made since her symptoms and laboratory findings were not satisfied with any criteria for rheumatic disorders. The steroid therapy was started in February 1991 and showed a good response. On April 9th, 1996, however, she was admitted to our hospital because of recurrence of high fever and chills. This time, she had sepsis because of the evidence that Enterococcus faecalis was detected in blood culture. Although her condition was improved by antibiotics on June 4th 1996, high fever re-appeared and followed by convulsion and disseminated intravascular coagulation. After the doses of prednisolone per day was increased to 40 mg with antibiotics and anticoagulant, her condition gradually improved. This patient was a rare case of pityriasis rubra pilaris associated with UCTD and sepsis in clinical course.

Adult↗

[Peripheral neuropathy and dysautonomia in a patient with primary Sjögren's syndrome].

A 46 year-old female presented with dry eyes and a dry mouth which she had been experiencing for about 15 years. She also began to notice dizziness (orthostatic hypotension) during the last 5 years. The symptoms gradually increased whereupon she began to have polyarthralgia, facial flashing, hyper and hyposweating in some areas on the face and trunk. Her sialography showed a damaged parotid gland. Minor salivary gland biopsy revealed chronic sialoadenitis. The Sirmer test was low, and the Rosebengal test indicated keratoconjunctivitis sicca. Her serological tests showed hypocomplementemia and were positive for antinuclear antibody and SS-A antibody. The diagnosis of primary Sjögren's syndrome (SjS) was made based on these findings. Prednisolone, at a dose of 15 mg per day, was given orally. As a result of this therapy, arthralgia disappeared immediately, although it had no effect on the neuropathy found in this patient. This is a rare case of SjS associated with peripheral neuropathy and severe dysautonomia.

Autonomic Nervous System Diseases↗

Anti-neutrophil cytoplasmic antibody in patients with systemic lupus erythematosus is unrelated to clinical features.

To investigate the incidence, the specificity and clinical significance of positivity for serum anti-neutrophil cytoplasmic antibody (ANCA) in 31 patients with systemic lupus erythematosus (SLE), the indirect immunofluorescence (IIF) technique and enzyme-linked immunosorbent assay (ELISA) were used to measure ANCA. Purified myeloperoxidase (MPO), lactoferrin (LF), cathepsin-G (CTG) and elastase (HLE) served as ANCA antigens for ELISA. Thirteen (42%) of the 31 SLE patients showed positivity for perinuclear, but not cytoplasmic, ANCA by IIF. Five of 31 sera were positive for MPO, 10 for LF, 1 for CTG and 0 for HLE by ELISA. Patients positive for ANCA had a higher score of SLE disease activity index (SLEDAI) than those without ANCA. There was no correlation between ANCA positivity, clinical manifestations, or organic involvement. While the ANCA in patients with SLE reflected disease activity, it was unrelated to organic involvement.

Adolescent↗

Co-existence of non-Hodgkin's lymphoma in the leukemic phase and polyarthritis simulating rheumatoid arthritis.

The patient, a 64-year-old male, complained of morning stiffness, polyarthralgia and bilateral knee joint swelling with leukocytosis (24,200/microliter) in peripheral blood. The leukocyte differentiation revealed 54% medium-size immature lymphocytes. The majority of lymphocytes showed the B-cell characteristics of IgGk monoclonality, and CD19+ and CD20+ in cell surface phenotype, suggesting a B-cell malignancy, non-Hodgkin lymphoma in the leukemic phase. Arthropathy associated with lymphoid malignancy was suspected. However, the infiltrated leukocytes in the synovial fluid of the left knee joint were dominantly neutrophils and CD3+ T-cells, and compatible with the findings in rheumatoid arthritis (RA). The association of B-cell malignancy and RA is not frequently reported. We discuss the common underlying immunological abnormalities in both B-cell malignancy and RA.

Anti-Infective Agents↗

[A case of malignant rheumatoid arthritis associated with myelodysplastic syndrome].

The patient (57 year-old female) complained of high fever and polyarthralgia in autumn of 1994, when a diagnosis of refractory anemia, a type of myelodysplastic syndrome (MDS) was made by severe anemia (RBC: 154 x 10(4)/microliters), leucopenia (2,200/microliters) and erythroid hyperplasia, ring-nucleated myelocyte, micromegakaryocyte and less than 5% of blasts in bone marrow smear. In her further clinical course, symptoms such as episcleritis, mononeuritis multiplex, skin ulcers on bilateral lower legs and circulating disturbance on left lower leg with high titer of rheumatoid factor were appeared in six months, suggesting malignant rheumatoid arthritis (MRA). She also had rectal cancer notified by anal bleeding. In spite of the administration of large doses of glucocorticoid and disease modifying anti-rheumatic drugs, circulating disturbance on left lower leg which might be caused by vasculitis was worsened to show tissue necrosis. Finally, she died of disseminated intravascular coagulation. The association of MDS with rheumatic disorders such as rheumatoid arthritis, Behçet disease and Sjögren's syndrome is not rarely reported. However, this is the first case of MDS associated with MRA.

Arthritis, Rheumatoid↗

[A case of amyopathic dermatomyositis associated with interstitial pneumonitis].

A patient (47-year old female) who had erythema similar to Gottron's sign on bilateral finger joints since two years ago, started to have polyarthralgia on bilateral knee and shoulder on the spring of 1993. Polyarthralgia was extended to both wrist and hand joints on Oct. of 1995. On the middle of Dec. 1995, she began to have exertional dyspnea and was referred and admitted in our hospital on 18th, Dec., 1995. Chest X-ray and CT scan showed the shadow for active interstitial pneumonitis on bilateral lower lung fields. Blood gas analysis indicated hypoxia (PaO2: 62.8 mmHg) and low % DLCO (64.7%). Skin eruption of face (heliotrope-like erythema) and hands (Gottron's sign) and skin biopsy (right hand) findings were compatible with that in dermatomyositis. The analysis in blood biochemistry showed no elevation for muscle enzymes. The diagnosis for amyopathic dermatomyositis (ADM) was made according to the criteria proposed by Euwer & Sontheimer (1993). The steroid pulse therapy and 50 mg per day of cyclophosphamide were immediately administered. The dyspnea and dermatitis were improved within two weeks after therapy. She is presently in remission until Jan. 1997 with the maintenance dosis of 10 mg per day of oral prednisolone.

Anti-Inflammatory Agents↗

Lobular form idiopathic glomerulonephritis with massive subendothelial and paramesangial immune deposits, a three-year follow-up case.

We report a patient with unusual glomerulonephritis. A 24-year-old Japanese female was hospitalized in October 1995 because of nephrotic syndrome. Lobular form glomerulonephritis with mesangial proliferation associated with massive wide-spread accumulation of slightly eosinophilic, periodic acid Schiff-positive amorphous materials in the luminal side of the capillary walls and paramesangial area was observed in the renal biopsy specimen. Immunofluorescent study revealed massive strong staining for IgM and C4 along the capillary walls and in the mesangium. Deposits of IgA, IgG, C3 and fibrinogen were also observed. Electron microscopy showed normal thickness of the capillary basement membrane and a large amount of subendothelial and paramesangial electron dense, finely granular deposits without fibrils or tubular structures. There were no clinical or laboratory findings of systemic diseases, such as systemic lupus erythematosus and cryoglobulinemia. Therefore, we believed that this case involved an unusual idiopathic glomerular disease with massive subendothelial and paramesangial immune deposits. Glomerulonephritis in this patient appeared to be resistant to treatment with corticosteroids and that this glomerulopathy may be a progressive disease as shown during the 3-year observation. Furthermore, our patient had idiopathic hyperprolactinemia and subclinical hypothyroidism. However, the relationship between glomerulonephritis and endocrinopathy in our patient is unknown.

Adult↗

Differential changes of corticotropin releasing hormone (CRH) concentrations in plasma and synovial fluids of patients with rheumatoid arthritis (RA).

Corticotropin releasing hormone (CRH) and ACTH concentrations in plasma and CRH and IL-6 concentrations in synovial fluid in patients with rheumatoid arthritis (RA) were examined to clarify the relationship between cytokines and the hypothalamic-pituitary-adrenal axis (HPA axis). Concentrations of serum amyloid A protein (SAA), one of the acute phase proteins, were also measured as an indicator of inflammation. CRH and IL-6 concentrations in synovial fluid were higher in RA patients than in control patients (osteoarthritis, OA). Plasma ACTH and CRH levels were significantly lower in RA patients than in OA patients. This suggests that CRH secretion in synovial fluid is regulated differently from plasma CRH secretion, as CRH levels in synovial fluid and plasma showed opposite changes in RA patients. SAA levels were positively correlated with the levels of CRH or IL-6 in synovial fluid, whereas there was no correlation between CRH and IL-6 levels. The results suggest that CRH and IL-6 play important independent roles in producing SAA in synovial fluid.

Adrenocorticotropic Hormone↗

Positivity for antinuclear antibody in patients with advanced rheumatoid arthritis.

Some patients with rheumatoid arthritis (RA) as well as those with other collagen diseases are positive for antinuclear antibody (ANA). We investigated the frequency of positivity for ANA in 104 patients with RA and evaluated the clinical features and laboratory data in the ANA-positive and -negative groups. The presence of ANA in sera was studied by indirect immunofluorescence using HEp-2 cells as the antigen substrate. Sera with a positive fluorescence at a dilution of 1:20 were considered to be positive for ANA. Of the 104 patients, 39 (37.5%) were positive for ANA. The staining pattern in the positive cases varied, but most were speckled (64.1%) and homogeneous (48.7%). A small number showed a nucleolar (20.5%) or a centromere (10.3%) pattern. None showed a shaggy pattern. The ANA titer was lower in RA patients compared with those with other collagen-related diseases such as systemic lupus erythematosus or progressive systematic sclerosis. None of the patients positive for ANA with either a nucleolar or centromere staining pattern had progressive systemic sclerosis or the CREST syndrome. One patient each had Raynaud's phenomenon and pulmonary fibrosis. There was no correlation between ANA positivity and indicators of joint inflammation. The prevalence of ANA positivity in patients with advanced or prolonged disease was higher than those with early stages or short durations. There was no correlation with drug therapy.

Adult↗

[Adherent reaction among activated platelets, polymorphonuclear cells and vascular endothelial cells under thrombin stimulation or shear stress].

Platelet activation in addition to blood coagulation abnormality is regarded as a primary factor of thrombosis by atherosclerotic obstruction. Therefore, the platelet activation on atherosclerosis is considered to correlate with shear stress generated between blood cells and endothelial cells in blood flow. P-selectin on the surface of the platelet membrane is measured by flowcytometry as a marker of platelet activation. Herein, we examined the phenomena of platelet activation and adherent platelets with leukocytes (ad-P.L) on stored platelet concentration (PC) and chronic rheumatoid arthritis (RA), moreover, of the adherent platelets or polymorphonuclear cells (PMN) with endothelial cells (EC) in shear stress by using an apparatus we devised. The rate of platelet activation and ad-P.L increased in PC with storage, and the sensitivity of platelets to thrombin decreased. The rate of platelet activation and ad-P.L increased in RA in vivo. Many adherent platelets with EC were found at a low shear rate on normal EC but at a high shear rate on denatured EC without any specific adherent property. Adherent PMN with EC had several hundred times more denatured EC than normal EC and the relationship with shear rate disappeared on denatured EC. The platelet activation and relationship between platelets or leukocytes and EC as to the cause of thrombosis are important subjects for future studies.

Arteriosclerosis↗

[Increased urinary iron excretion rate in patients with non-insulin dependent diabetes mellitus].

Urinary iron excretion rate(u-FeER, microgram/min) in urine at night time from 58 patients with non-insulin dependent diabetes mellitus and 8 controls was measured by atomic absorption. The patients were divided into three groups according to urinary albumin excretion rate(u-AER), namely, Group I(n = 44): less than 20 micrograms/min of u-AER; Group II(n = 9): 20 < or = u-AER < 200 micrograms/min; Group III(n = 5): more than 200 micrograms/min of u-AER. The u-FeER in group III(56.3 +/- 14.8 ng/min, mean +/- SEM) was significantly higher than that in controls (4.0 +/- 1.6ng/min), group I(8.3 +/- 1.6 ng/min) and group II(18.5 +/- 6.5ng/min). The increase of u-FeER in group III indicates that urinary iron may at least partly play a role in development of diabetic nephropathy.

Adult↗

[A case of rheumatoid arthritis associated with pernicious anemia and bronchiolitis obliterans organizing pneumonia].

A case of rheumateid arthritis (RA) with pernicious anemia (PA) and wandering multiple patchy densities in bilateral lung fields is reported. A 72-year-old woman was hospitalized in February 1994, because of cough. She had already advanced RA (Class IV, Stage IV). She showed macrocytic and hyperchromic anemia as follows ; red-cell count (RBC), 176 x 10(4)/microliters; hemoglobin (Hb),7.2 g/dl; hematocrit (Ht), 21.0% ; MCV, 119.3 fl; and MCH, 40.9 pg. Chest roentgenogram revealed multiple patchy densities in bilateral lung fields and there was no response to the administration of antibiotic agents. From these clinical pictures bronchiolitis obliterans organizing pneumonia (BOOP) was highly suspected. After steroid injection into the joint space, the abnormal lung shadows disappeared. Anemia had been recovering spontaneously, but recurred in July. The results of blood examination were as follows ; RBC, 162 x 10(4)/microliters; Hb, 6.7ng/dl; Ht, 19.1%; MCV, 117.9 fl; and MCH, 41.4 pg. Anti-intrinsic factor antibody was positive. The level of serum vitamin B12 was low, 76 pg/ml. Sternal bone marrow aspiration showed magaloblastic changes with hypersegmentation of granulocytes. PA was diagnosed and improvement was noted after the intramuscular administration of vitamin B12. Subjective symptoms based on RA did not change during the clinical course. It is suggested that the pathogenesis about the combination of RA, BOOP and PA is related to common immunological abnormalities in our patient. A case of RA with PA and BOOP has not been reported previously, thus this case is considered clinically valuable.

Aged↗

Impaired interleukin-8-dependent chemotaxis by synovial fluid polymorphonuclear leukocytes in rheumatoid arthritis.

The accumulation of polymorphonuclear leukocytes (PMN) in synovial fluid is a common feature of rheumatoid arthritis (RA). We studied the chemotactic response of PMN obtained from the synovial fluid and from the peripheral blood of patients with RA using a modified Boyden's method, in which interleukin-8 (IL-8) or N-formyl-methionyl-leucyl-phenylalanine (FMLP) was used as a chemotactic agent. The IL-8-induced response of peripheral blood PMN from 15 patients with RA did not differ from that of 15 healthy controls. A decreased chemotactic response to IL-8 was, however, observed in PMN from the synovial fluid of 12 patients with RA compared with peripheral blood cells of the same individual. This defective chemotactic ability of PMN was inversely correlated with the number of infiltrating cells in the synovial fluid. We also obtained similar results with FMLP. These results indicate that the chemotactic ability of PMN may be reduced after migrating to the synovial fluid.

Adult↗

Stimulation of human synovial cell DNA synthesis by iron.

OBJECTIVE: To determine the effect of iron and cytokines on proliferation of synovial cells (SC), we isolated SC obtained at the time of total knee replacement from 11 patients with rheumatoid arthritis (RA) and 2 with osteoarthritis (OA) following enzymatic treatment of synovial tissue. METHODS: SC were cultured in the presence of ferric citrate or sodium citrate at concentrations of 0, 0.01, 0.1, and 1 mM for 72 h. In vitro synthesis of DNA by SC was measured by intracellular 3H-thymidine uptake. RESULTS: Synthesis of DNA by SC was significantly enhanced by ferric citrate but not sodium citrate. The maximum synthesis was observed following stimulation with 0.1 mM ferric citrate and was obtained after 3 days of culture, thereafter declining until Day 7. This stimulatory effect of ferric citrate was observed in all specimens of SC tested. In our examination of the concomitant addition of each of 8 different recombinant human cytokines with 0.1 mM ferric citrate on SC DNA synthesis, 4 of them, i.e., interleukin-1 beta (IL-1 beta), IL-7, tumor necrosis factor alpha (TNF alpha) and interferon gamma (IFN-gamma) each enhanced the synthesis of SC DNA at concentrations ranging from 1 to 100 u/ml, while IL-1 beta, IL-7, TNF alpha or IFN-gamma together showed an additive effect. No significant effect was shown by IL-2, IL-6, and IL-8, or granulocyte colony stimulating factor (G-CSF). CONCLUSION: Iron stimulated in vitro SC DNA synthesis and had an additive effect on the activity of human cytokines for SC proliferation. Iron may play a role in the proliferation of synovial cells in patients with RA synovitis.

Adult↗