Clinical experience of sivelestat sodium hydrate for severe reexpansion pulmonary edema; report of a case

An 81-year-old man was referred to our hospital for the treatment for left spontaneous pneumothorax. A chest X-ray revealed a left-sided total pneumothorax and complete collapse of the lung. After intravenous administration of methylprednisolone, a 16 Fr chest tube was inserted, and drainage was started without negative pressure suction. Four hours after chest tube insertion, the patient's condition deteriorated. He complained severe cough and dyspnea, and pulse oximetry reading was 70%. A repeat chest X-ray demonstrated diffuse reexpansion pulmonary edema (RPE) on the left. After mechanical ventilation and intravenous infusion therapy with sivelestat sodium hydrate, methylprednisolone and ulinastatin were started, P(O2)/ Fi(O2) ratio improved rapidly. He was extubated on hospital day 6 and was discharged after pleurodesis for the pneumothorax. This case suggests that sivelestat sodium hydrate may be useful for the treatment for RPE.     

Behçet disease presenting with neurological complications immediately after conversion from conventional cyclosporin A to microemulsion formulation

A 53-year-old man with Beh?et disease was treated with conventional cyclosporin A (CyA), because of refractory bilateral uveitis. Immediately following the conversion from conventional CyA to a microemulsion formulation, he presented with neurological complications. The neurological findings, pleocytosis of the cerebrospinal fluid (CSF) and brainstem lesions revealed by brain magnetic resonance imaging (MRI) suggested neuro-Beh?et disease. After discontinuing CyA and introducing oral prednisolone, the neurological symptoms, pleocytosis of CSF and brainstem lesions on MRI improved. Although the microemulsion formulation, which can maintain a stable level of blood CyA, is a useful agent for the control of ocular lesions in Beh?et disease, the resulting abrupt increase in blood CyA level may have induced neuro-Beh?et disease.     

Dermatomyositis associated with impairment in both muscle aerobic and anaerobic function

We report a 50-year-old Japanese woman with dermatomyositis in whom an anti-mitochondrial antibody was detected. Muscle biopsy demonstrated periodic acid Schiff- (PAS) positive vacuoles in addition to infiltrates of mononuclear cells. Histochemical analysis showed reduced phosphorylase activity. An aerobic exercise test demonstrated that the concentrations of serum lactate and pyruvate were elevated before corticosteroid therapy but decreased after the therapy. On the other hand, in forearm ischemic exercise tests, the responses of serum lactate and pyruvate were attenuated before corticosteroid therapy but recovered after the therapy. These findings indicate that an inflammatory mechanism interfered with myophosphorylase activity and muscle aerobic function.     

Delayed motor and sensory neuropathy in a patient with brainstem encephalitis

Bickerstaff's brainstem encephalitis (BBE), Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) are thought to be closely related and to form a continuous spectrum. However, chronic polyneuropathy in BBE has not been reported. We report the temporal profile of anti-ganglioside antibody titer in a case of BBE-like brainstem encephalitis complicated with chronic polyneuropathy. A 71-year-old Japanese woman presented with drowsiness and cerebellar ataxia in addition to mild weakness in distal limb muscles. Anti-GalNAc-GD1a IgG and anti-GalNAc-GM1b IgG antibodies were positive in her serum. Brain magnetic resonance imaging revealed high-intensity signals in the midbrain, pons, and middle cerebellar peduncles on T2-weighted images. Central nervous system manifestations improved after immunomodulating therapy that included prednisolone, plasmapheresis and intravenous immunoglobulin. Nevertheless, the distal muscle weakness was exacerbated when the anti-GalNAc-GD1a IgG titer was elevated. Nerve conduction study indicated motor and sensory neuropathy which was developed motor dominant axonal damage. These findings suggest that anti-ganglioside antibodies, including anti-GalNAc-GD1a IgG, may be involved in a common autoimmune mechanism in BBE-like brainstem encephalitis and chronic motor dominant axonal neuropathy. However, the fact that the latter manifestation exacerbated after the improvement of former one possibly indicates different thresholds of neurologic symptoms mediated by anti-ganglioside antibodies in the present patient.     

Comparison of ropivacaine with bupivacaine and lidocaine for ilioinguinal block after ambulatory inguinal hernia repair in children

BACKGROUND: We have compared ropivacaine with bupivacaine and lidocaine for ilioinguinal block in thirty children undergoing ambulatory inguinal hernia repair. METHODS: Patients were assigned randomly to receive 0.5 ml.kg(-1) of 0.2% ropivacaine (Group R, n = 10), 0.25% bupivacaine (Group B, n = 10) or 1% lidocaine (Group L, n = 10). The patients' parents, who were not informed of the type of local anaesthetic employed, evaluated the postoperative pain at 2 h and 6 h after operation using the Wong-Baker FACES Pain Rating Scale. RESULTS: There was a significant difference in the face scale score between Group R and Group L, and Group B and Group L. There was no difference in the face scale score between Group R and Group B. There were no complications or clinical evidence of local anaesthetic toxicity. CONCLUSIONS: We have confirmed that bupivacaine and ropivacaine are more effective than lidocaine in the prevention of postoperative pain after children's inguinal hernia repair. We suggest that ropivacaine 0.2% is an alternative to bupivacaine 0.25% for ilioinguinal block in ambulatory paediatric surgery.     

Dermatomyositis associated with Sjögren's syndrome: VEGF involvement in vasculitis

Two patients with dermatomyositis complicated with Sj?gren's syndrome (SjS), are reported. Both patients exhibited sensory-dominant polyneuropathy, compatible with neurologic involvement in SjS. Vascular endothelial growth factor (VEGF) levels were increased in their plasma. Histological examination demonstrated vasculitic changes in biopsied specimens of muscle and salivary glands from the patients, and VEGF was overexpressed in the vasculitic lesions. These findings suggest that VEGF overexpression was associated with the development of vasculopathy in skeletal muscle and salivary glands and possibly in the peripheral nervous system.     

Polysialic acid and HNK-1 are expressed in the adult rat vestibular endorgans

Polysialic acid (PSA) and human natural killer (HNK)-1 carbohydrate epitopes are expressed mainly in developing neurons but also in restricted areas, even in adulthood. In the present study, we demonstrated the expression of PSA and HNK-1 epitopes in adult primary vestibular afferent neurons. In addition, we confirmed the presence of two distinct polysialyltransferases, PST and STX, that form PSA, as well as two types of glucuronyltransferases, GlcAT-P and GlcAT-S involved in the biosynthesis of HNK-1 epitopes in the vestibular endorgans. These results combined suggest that both PSA and HNK-1 carbohydrate epitopes are synthesized and may have an important role in the adult peripheral vestibular endorgans.     

Percutaneous radiofrequency ablation for unresectable large hepatic tumours during hepatic blood flow occlusion in four patients

AIM: To evaluate percutaneous radiofrequency (RF) ablation therapy for unresectable large hepatic tumours combined with regional interruption of hepatic blood flow, and to assess the safety and efficacy of this procedure. MATERIALS AND METHODS: Four patients with hepatic tumours were enrolled in this study. Patients were treated by a single session of RF ablation during occlusion of both hepatic artery and hepatic vein. Tumour size ranged from 45-57 mm (mean 50.2 mm). Initial therapeutic efficacy was evaluated with helical computed tomography (CT) performed within 9 days after the treatment. CT or magnetic resonance imaging (MRI) was performed every 2-3 months thereafter. RESULTS: The largest axis of coagulated lesions after the ablation was 50-60 mm (mean 56.5 mm) in diameter. The ablation therapy was considered complete in three patients; after a mean follow-up of 12.7 months, CT and MRI revealed complete destruction of their tumours. One patient required further treatment. No severe complications occurred. CONCLUSION: Although further studies are needed, in this limited clinical trial a local ablation area exceeding 50 mm in diameter was achieved safely.