Churg–Strauss syndrome: a retrospective study of 11 cases from a single center in Japan

Objective: To investigate the clinical characteristics of patients with Churg–Strauss syndrome (CSS), including symptoms, blood chemistry and immunological findings. Patients and methods: We retrospectively investigated the records of 11 patients (six female and five male) with CSS admitted to our hospital from September 2003 to October 2009. Results: Eight patients had preceding symptoms including bronchial asthma and allergic rhinitis. Seven patients showed eosinophilia. Nine patients had mononeuritis multiplex. Positive findings of myeloperoxidase‐antineutrophil cytoplasmic antibody (MPO‐ANCA) were found in five patients. Neither clinical manifestations nor laboratory findings were correlated with positivity for MPO‐ANCA. However, the MPO‐ANCA‐positive group showed a higher level of blood urea nitrogen and proteinuria than those negative for MPO‐ANCA. Ten patients recovered after starting steroid or immunosuppressive therapy, although one patient died of unknown etiology. Conclusion: Although general assessments based on various factors such as medical history, clinical manifestation and laboratory studies are indispensable in CSS, MPO‐ANCA might be useful as a predictor of renal dysfunction in patients with CSS.     

Ventilatory responses at the onset of passive movement and voluntary exercise with arms and legs

This study was undertaken to elucidate whether phase I appeared at the onset of voluntary and passive arm movements and to compare these results with those of similar leg movements. Instead of the conventional cranking exercise, seven male subjects performed alternately flexion-relaxation of both arms, extension-relaxation of both legs, and combined arm and leg exercise at the rate of about 60 min^-1 for four breaths in a sitting position. Similar movements were accomplished passively by the experimenters. In all experiments, minute ventilation increased rapidly within the first breath after the onset of exercise. The difference of ventilation (delta value) between the mean of the first two breaths at the onset of voluntary exercise and that of five breaths during rest was significantly (P < 0.05) greater in arm (7.751 min^-1) than in leg (5.191 min^-1). Passive movement showed a similar tendency. Arm delta ventilation correlated highly (r = 0.74 ± 0.91) with leg delta ventilation and the slope of the regression lines was about 1.2. Heart rate increased abruptly while cardiac output did not always increase rapidly at the onset of locomotion. Oxygen uptake in the voluntary leg exercise continued for 3 min was slightly but nonsignificantly higher than in the arm exercise, indicating the equality of the exercise intensity. In conclusion, ventilatory responses at the onset of the arm exercise are larger than those of the leg in both voluntary and passive conditions regardless of the muscle mass, suggesting the different neurogenic mechanism between arm and leg.     

Anti-HCV immunoglobulin M antibody in patients with hepatitis C

Anti-hepatitis C virus (HCV) immunoglobulin M antibody titres were measured by an enzyme-linked immunosorbent assay method in 16 patients with non-A, non-B acute hepatitis (NANB AH), 13 with non-A, non-B fulminant hepatitis (NANB FH) and nine with type C chronic hepatitis. Anti-HCV IgM was positive in one of the 16 patients with NANB AH, six of the 13 with NANB FH, and five of the nine with type C chronic hepatitis. Anti-HCV IgG was positive in eight of the 16 patients with NANB AH and eight of the 13 with NANB FH. Either anti-HCV IgM or anti-HCV IgG were positive in 10 of the 13 patients with NANB FH. All of the five anti-HCV IgM positive patients with type C chronic hepatitis were undergoing an exacerbation of the diseases, while all of the anti-HCV IgM negative patients were in a remission stage which had lasted for more than 6 months. The findings of this study suggest that anti-HCV IgM is useful for the early diagnosis of type C FH and may be a useful marker of diseases activity in type C chronic hepatitis.     

Correlation Between Disappearance of the Intrahepatic Bile Ducts and Histologic Changes in the Liver in Primary Biliary Cirrhosis

Statistical investigations indicate that disappearance of bile ducts, estimated by a histometric method, was more extensive in symptomatic than in asymptomatic patients with primary biliary cirrhosis. The extent and degree of intrahepatic bile duct disappearance in PBC is related to the degree of copper granule deposition in the hepatocytes, the degree of atypical ductular proliferations and the fibrous septa formation. Bile plug formation and chemically determined copper content are not so well related to the bile duct disappearance, while periportal lymphoid cell infiltration is not related to the degree or extent of bile duct disappearance at all.     

Parvovirus B19-associated haemophagocytic syndrome with lymphadenopathy resembling histiocytic necrotizing lymphadenitis (Kikuchi's disease)

A 15-year-old girl developed a haemophagocytic syndrome caused by human parvovirus B19 (PVB19). The cervical lymph node histology, resembling that of histiocytic necrotizing lymphadenitis (HNL, Kikuchi's disease), included several transformed lymphocytes, numerous histiocytes, and massive necrosis. We detected PVB19-positive cells in the lymph node by immunohistochemistry. Possible autoimmune mechanisms in HNL-like diseases triggered by PVB19 are discussed.     

Natural Products: Effects of Sho-saiko-to Extract on Fibrosis and Regeneration of the Liver in Rats

Sho-saiko-to, one of the most widely used Chinese herbal preparations, has long been used for the treatment of chronic liver diseases. We have investigated its effect in retarding the process of liver fibrosis and accelerating liver regeneration, especially its effect on Ito cells that are thought to be deeply involved with liver fibrosis. Sho-saiko-to extract and its active constituents were orally administered to rats with dimethy lnitrosamine-induced liver-injury. After treatment with sho-saiko-to extract hepatic function improved, histopathological results confirmed repair of liver tissue, and retinoid levels increased. On the other hand, when active constituents of sho-saiko-to extract were administered alone, liver retinoid levels remained low, implying that interaction among active constituents of the extract was suppressing Ito cell activation. When sho-saiko-to extract was administered to 70% hepatectomized normal and liver-injured rats, liver weight, the number of S-phase-cells and retinoid levels increased with time. However, these changes were different for normal and liver-injured rats, suggesting that the site of action of sho-saiko-to extract in regenerating liver is different for normal and liver-injured rats. These results show that sho-saiko-to extract was useful for suppressing the activation of Ito cells.     

Initiation of increase in muscle sympathetic nerve activity delay during maximal voluntary contraction

To investigate the effects of maximal voluntary exercise on sympathetic nerve activity, contraction force and muscle sympathetic nerve activity (MSNA) were recorded during maximal (MVG) and submaximal voluntary isometric handgrip (SVG) for 2 min in eight healthy subjects. MSNA was determined by a microneurographic technique, and handgrip force, heart rate (HR) and arterial blood pressure (ABP) were measured by a non-invasive method during exercise. Grip force decayed rapidly to 58% of maximal grip force (MGF) at 10 s after commencement of exercise and was almost constant (≈ 30% of MGF) 40 s after exercise. MSNA increase was delayed by 20 s during MVG, followed by a gradual increase. HR was elevated immediately after onset of exercise, while mean ABP rise showed a 20 s lag from initiation of MVG exercise. During SVG increases in MSNA, HR and mean ABP were delayed by 50, 40 and 20 s, respectively, relative to commencement of exercise. Thereafter, these parameters increased time-dependently. These results suggested that the MSNA increase during MVG may be predominantly because of the metaboreflex.