Clinical utility of anti-signal recognition particle antibody in the differential diagnosis of myopathies.

OBJECTIVE: Auto-antibodies to signal recognition particle (SRP) are known to be specific to PM among rheumatic disorders, but the specificity in myopathic diseases remains unclear. The clinical utility of anti-SRP antibody in the differential diagnosis of myopathies has not been studied. The aim of the present study was to elucidate whether detection of anti-SRP antibody can discriminate of PM from muscular dystrophy (MD). METHODS: We report a patient with a childhood onset myopathy, in whom it was clinically difficult to make a differential diagnosis of PM or MD for 21 yrs, despite repeated muscle biopsies. Myositis-specific auto-antibodies to RNA-associated antigens were screened in this particular case as well as in 105 serum samples from various types of MD and 84 from PM patients using RNA immunoprecipitation. The MD and PM serum samples were obtained from different institutions. The presence of anti-SRP antibody was confirmed by RNA immunoprecipitation combined with immunodepletion of SRP from the antigen. RESULTS: Anti-SRP antibody was positive in the present patient, supporting the diagnosis of PM. Anti-SRP antibody was detected in seven (8.3%) patients with PM, but in none of the patients with MD. Myositis-specific auto-antibodies were not detected in any of the patients with MD. CONCLUSION: Anti-SRP antibody is useful for discriminating PM from MD among patients with myopathies.

Increased urinary leukotriene E4 concentration in patients with eosinophilic pneumonia.

Although eosinophils produce cysteinyl leukotrienes (CysLTs) in large quantities, information on the relationship between CysLTs and eosinophilic pneumonia (EP) is lacking. Inflammatory mediator concentrations in urine were quantified to clarify the relationship between CysLT concentrations and EP severity. Leukotriene (LT)E(4), eosinophil-derived neurotoxin (EDN), 9alpha,11beta-prostaglandin F2 and LTB(4) glucuronide concentrations were quantified in the urine of: EP patients during acute exacerbation and clinical remission; asthmatic patients during acute exacerbation and under stable conditions; and healthy control subjects. The urinary LTE(4) and EDN concentrations of EP patients during acute exacerbation were significantly higher than those of asthmatic patients and healthy subjects, and decreased immediately during clinical remission. The urinary LTE(4) concentration was associated with the urinary EDN concentration of EP patients during acute exacerbation. The urinary LTE(4) concentration significantly correlated with the diffusing capacity of the lung for carbon monoxide in EP patients during acute exacerbation. The increased urinary concentrations of leukotriene and eosinophil-derived neurotoxin were associated with acute exacerbation in eosinophilic pneumonia patients. The increased leukotriene concentration significantly correlated with diffusing capacity of the lung for carbon monoxide, suggesting that the monitoring of leukotriene concentration may aid in the management of eosinophilic pneumonia patients.

[Successful therapy of cyclosporin A in a case with idiopathic interstitial pneumonia].

Seventy-one-year-old woman was visited to our hospital because of dry cough and dyspnea on effort. Fine crackle was audible on both lower lung fields. Joints and skin were normal. Laboratory examination revealed elevation of serum LDH and CRP level. Both anti-nuclear antibody and Jo-1 antibody were negative. Blood gas analysis showed hypoxia after exercise. Chest X-ray film showed reticular shadow in both lower lung fields. Chest CT finding showed patchy area of ground glass attenuation, air-space consolidation, and reticular shadow. Scintigram showed diffuse uptake of Gallium-67 in both lung. Transbronchial biopsy specimen revealed alveolar wall thickness, lymphocyte infiltration and swelling of type II pneumocytes. Bronchoalveolar lavage fluid analysis revealed elevation of CD4/CD8 ratio. She was given a diagnosis of idiopathic interstitial pneumonia. Combination therapy of cyclosporin A and steroid was performed. After therapy, her chest CT findings and her data of pulmonary function test were improved. Then therapy of cyclosporin A was continued and dose of prednisolone was gradually decreased. After that, she was suffered from respiratory tract infection. After administration of antibiotics and cyclosporin A, she was getting well without acute exacerbation of interstitial pneumonia. Since then, she was treated with cyclosporin A only and her pulmonary function test data were gradually improved more. It suggests that cyclosporin A may be useful for the treatment of idiopathic interstitial pneumonia.

[A case of idiopathic subglottic stenosis successfully treated with endoscopic laser treatment].

A 24-year-old woman presented with an abnormal sound in her neck. She had no history of previous surgery, endotracheal intubation or neck trauma. She had been aware of this abnormal sound since she was 12 years old, but paid no attention to it. She had been recommended to visit a hospital for a neck examination. Stridor was heared in the neck ["coming from the throat"?]. Peak flow was markedly suppressed in pulmonary function tests, and the flow volume loop indicated upper airway stenosis. Bronchoscopy revealed subglottic tracheal stenosis, and magnetic resonance imaging showed that the cricoid cartilage was normal. Nd-YAG laser treatment was performed in Mitsui Memorial Hospital and her symptoms were improved. Biopsy of the stenotic area of the trachea showed dense fibrous tissue proliferation in the submucosa. Neither granuloma nor vasculitis was found in the biopsy specimen. No systemic disease capable of inducing tracheal stenosis was found on systemic examination. From these clinical and pathological findings, we diagnosed idiopathic subglottic stenosis.

[Successful treatment of steroid-resistant bronchiolitis obliterans-organizing pneumonia with orally administered cyclosporin and pirfenidone].

A 27-year-old man with fever and dyspnea was admitted to our hospital. Chest computed tomography and a lung biopsy were performed, and bronchiolitis obliterans-organizing pneumonia (BOOP) was diagnosed. The patient was treated with corticosteroid, and a marked improvement was noted. However, when the dosage was tapered, BOOP recurred. Although the dosage was increased again, the corticosteroid alone was no longer effective against BOOP. While continuing with corticosteroid therapy, we also put the patient on a daily regimen of cyclosporin and pirfenidone, a recently developed anti-fibrotic agent. Both drugs were administered orally, and were so effective that we gradually decreased the dosage of corticosteroid. Several journals have reported that cyclosporin may be effective in the treatment of interstitial pneumonitis associated with collagen disease. We concluded that cyclosporin may also be useful in the treatment of refractory BOOP.

[A case of eosinophilic gastroenteritis complicated with ileus and ascites collection].

A 30-year-old woman was admitted to our hospital because of ileus and ascites. Laboratory data on admission demonstrated marked eosinophilia (42.5% of WBC) but negative CRP-value. Abdominal CT showed marked ascites and diffuse thickening of intestinal walls. Ascites examination revealed eosinophilic ascites. The level of IL-5 both in the serum and in the ascites were also high. No evidence of eosinophilic infiltration was noted both gastric and colonic mucosal biopsy specimens. Oral prednisolone treatment (50 mg/day) was effective for her. We diagnosed her as a case of sub-serosal type eosinophilic gastroenteritis. It is essential to obtain eosinophilic ascites for correct diagnosis of the disease. And it is possible that serum and ascites IL-5 value would be reliable indicator of the activity of this disease.

Lipoprotein, lecithin:cholesterol acyl transferase and acetyl CoA carboxylase in stroke-prone spontaneously hypertensive rats fed a diet high in eicosapentaenoic acid.

To investigate the effect of dietary eicosapentaenoic acid (EPA) on plasma lipoprotein levels, lecithin:cholesterol acyltransferase (LCAT) activity and liver acetyl CoA carboxylase activity, highly concentrated EPA (78%) purified from sardine oil was fed to stroke-prone spontaneously hypertensive rats (SHRSP) for 30 days. Significantly (P < 0.05) lower systolic blood pressure and plasma total cholesterol were observed in rats fed an EPA diet. In addition, higher HDL cholesterol and lower VLDL cholesterol levels were found in rats fed the EPA diet as compared with rats fed the control diet. However, no significant change of plasma LDL cholesterol was observed in rats between the two dietary groups. EPA supplementation increased the activity of plasma LCAT in rats. In addition, rats fed an EPA diet had lower liver total lipids and adipose tissue weights. However, higher liver acetyl CoA carboxylase activity was observed in rats fed the EPA diet. Results from the present study suggest that dietary EPA might stimulate the plasma lipoprotein metabolism and also alter lipogenesis in the liver of SHRSP rats.

Extractive-spectrophotometric determination of trace iron(II) with di-2-pyridylmethanone 2-(5-nitro)pyridylhydrazone.

The optimum conditions for the extractive-spectrophotometric determination of trace iron(II) with di-2-pyridylmethanone 2-(5-nitro)pyridylhydrazone have been established. Iron(II) reacts with this reagent at pH 2.0-7.5 to form an uncharged 1:2 (metal-to-ligand) complex, which can be extracted with toluene. Beer's law is obeyed over the range up to 0.84 mug/ml of iron(II) at 505 nm. The molar absorptivity of the extracted species is 5.83 x 10(4) 1.mole(-1).cm(-1). The proposed method is extremely sensitive and reproducible, and has been satisfactorily applied to the determination of total iron in freshwater samples by adding ascorbic acid to reduce iron(III).

[An experimental study on portal infusion of 5-fluorouracil].

We conducted these experiments to investigate the differences between continuous portal and one shot portal infusion of 5-fluorouracil (5-FU) using male Donryu rats. Continuous portal infusion of 5-FU (20 mg/kg/day x 5 days) revealed no remarkable side effects on the rats with significant decrease in the number of liver metastases generated by intraportal inoculation of ascites hepatoma AH60C. Consecutive 5-day administration of 5-FU (20 mg/kg x 5) by one shot method via spleen resulted in no metastases but caused death in 75% of rats because of severe leukopenia and dehydration. The rats receiving one shot infusion of 5-FU (33.3 mg/kg x 3, day 1, 3, 5) via spleen showed a significant decrease in the number of metastases without lethal side effects. Continuous portal infusion of 5-FU would be a safe and effective therapy to prevent liver metastases, whereas one shot portal infusion of 5-FU may give rise to severe side effects while preventing liver metastases.

Effect of trans fatty acids on plasma lipids, platelet function and systolic blood pressure in stroke-prone spontaneously hypertensive rats.

To investigate the effect of trans fatty acids on plasma lipid levels and systolic blood pressure, hydrogenated corn oil was fed to SHRSP (stroke-prone spontaneously hypertensive rats) and WKY (Wistar-Kyoto) rats for 30 days. Significantly lower systolic blood pressure and plasma total cholesterol were observed in SHRSP rats fed trans fatty acids when compared with rats fed cis fatty acids from olive oil. In addition, higher HDL cholesterol and lower VLDL plus chylomicron cholesterol levels were found in SHRSP rats fed trans fatty acids. Although no significant changes of systolic blood pressure and plasma total cholesterol levels were observed in WKY rats after trans fatty acids treatment, WKY rats fed trans fatty acids had lower plasma LDL cholesterol and higher HDL cholesterol levels. In addition, platelet aggregation induced by collagen was decreased in WKY rats fed trans fatty acids. It is interesting that trans fatty acids increased the activity of plasma lecithin:cholesterol acyltransferase (LCAT) in both SHRSP and WKY rats. The observed influence of trans fatty acids on plasma lipid levels, systolic blood pressure and platelet aggregation suggests that trans fatty acids might prevent thrombotic disorders in SHRSP rats.

Solvent extraction of the indium-alizarin Red S chelate as its 1,3-diphenylgaunidium salt.

The extraction of the 1,3-diphenylguanidiuin (DPG) salt of the indium-Alizarin Red S (ARS) chelate with n-butyl acetate has been investigated. The extracted species has an absorption maximum at 525 nm, and Beer's law is obeyed over the range from 0.3 to 2.8 ppm of indium. The molar absorptivity is 2.65 x 10(3) l.mole(-1).mm(-1) at 525 nm. The 1:3 indium(III)-ARS chelate is extracted from aqueous solution in a 1:3 molar ratio with DPG. The infrared spectrum implies that the metal ion is probably chelated by the two phenolic oxygen atoms of ARS.