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Perineuritis is a rare type of peripheral neuropathy defined by swelling and cellular infiltration in the perineurium. We herein report a 52-year-old man who presented with subacute onset pain from the back to the lower limbs, muscle weakness and hypoesthesia. A sural nerve biopsy revealed perineuritis, consisting of inflammatory cell infiltration and swelling of the perineurium. Oral prednisolone, plasma exchange and intravenous immunoglobulin treatment were all effective, leading to significant improvement of the symptoms.

We report the case of a 53-year-old woman with severe chronic inflammatory demyelinating polyneuropathy (CIDP) who developed progressive tetraplegia with respiratory failure despite receiving a standard dose of intravenous immunoglobulin therapy (IVIg), steroid pulse therapy, plasma exchange, and cyclosporine. We administered high-dose IVIg (3 g/kg; 0.6 g/kg/day for 5 consecutive days at monthly intervals). The patient's muscle weakness gradually improved after IVIg. She recovered completely 2 years after the onset of symptoms. The effects of IVIg treatment in individuals with CIDP may vary in each patient. In patients with refractory CIDP receiving standard-dose IVIg, repeated high-dose IVIg treatment can be considered.

We herein present a case of an overlap of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) and primary biliary cirrhosis (PBC). A 61-year-old man was diagnosed with PBC due to abnormal liver biochemical tests and positivity for serum anti-M2 antibody. However, his response to bezafibrate and ursodeoxycholic acid was insufficient. Five years later, his serum IgG4 level was found to increase. His liver biopsy specimens showed features of nonsuppurative destructive cholangitis in some portal tracts and periductal fibrosis with dense infiltration of IgG4-positive cells in other portal tracts. This case demonstrates that the serum IgG4 level may be worth measuring in patients with PBC refractory to conventional treatment.

Objective: To improve the prognosis of severe acute pancreatitis, preventing infectious complications, particularly infected pancreatic necrosis, is important. The present study evaluated the efficacy of peritoneal lavage for improving the prognosis of patients with severe acute pancreatitis., Patients: We retrospectively reviewed the cases of 23 consecutive patients with severe acute pancreatitis who were treated with peritoneal lavage., Results: Peritoneal lavage was started within 72 hours after the initial onset of symptoms in 20 patients (87%). The duration of peritoneal lavage, which was significantly correlated with the number of prognostic factors according to the revised Japanese criteria, Ranson score and serum C-reactive protein level at the start of peritoneal lavage, was a median of seven (3-22) days. There were no adverse events associated with the peritoneal lavage. Eight patients (35%) concurrently underwent continuous regional arterial infusion. Five days after starting peritoneal lavage, the patients' clinical conditions significantly improved. Overall, the survival rate was 96%. One patient (4%) died due to rupture of a pseudoaneurysm of the splenic artery. Complications occurred in seven patients (30%). Infectious complications were observed in three patients (13%) (one patient developed infected pancreatic necrosis and bacteremia, and two patients developed bacteremia). Pseudocysts and pancreatic fistulas developed in five and one patient, respectively. The incidence of complications was lower in the patients receiving peritoneal lavage within 72 hours from the initial onset of symptoms than in the remaining patients (20% vs. 100%; p=0.005)., Conclusion: We speculate that peritoneal lavage reduces the mortality and incidence of complications in patients with severe acute pancreatitis.

A 42-year-old woman with hepatitis C virus-related cirrhosis underwent peginterferon alpha-2b therapy combined with ribavirin but could not achieve a sustained viral response. Following discontinuation of this combined therapy, the patient's serum transaminase levels suddenly became elevated. Therefore, the administration of very-low-dose peginterferon alpha-2a with ursodeoxycholic acid was introduced. Thereafter, the patient's serum transaminase levels gradually improved. Four years later, enhanced computed tomography showed shrinkage of the spleen and enlargement of the liver. Long-term combined therapy with very-low-dose peginterferon and ursodeoxycholic acid may be effective not only in preventing disease progression, but also in improving portal hypertension in patients hepatitis C virus-related cirrhosis.

Objective: Platelet counts before starting the treatment affect the discontinuation and dose reduction of peginterferon in chronic hepatitis C. Thrombocytopenia leads to failure to achieve sustained virological response. This study was undertaken to evaluate the efficacy of partial splenic embolization (PSE) prior to starting peginterferon therapy combined with ribavirin in chronic hepatitis C patients showing thrombocytopenia., Patients and Methods: We compared the clinical features of 11 patients receiving PSE (PSE group) prior to starting the combined therapy with those of 13 patients not receiving PSE (non-PSE group). All of the patients showed platelet counts ≤12×10(4)/mm(3) and serum hepatitis C virus-RNA levels ≥100 KIU/mL at baseline. The end-point of PSE was a volume of splenic infarction over 75%. Peginterferon alpha-2b at a dose of 1.2 µg/kg was administered by subcutaneous injection once a week. The dose of ribavirin was weight adjusted., Results: PSE was successfully performed without serious adverse events. The period from PSE to starting the combined therapy was 14 (6-27) days. After PSE, platelet counts were significantly increased. In PSE group, platelet counts during the combined therapy were maintained above those at baseline. In non-PSE group, platelet counts at the 2nd week after the start of the combined therapy significantly decreased to less than those at baseline. Overall, 80% adherence to expected peginterferon dose was not achieved in 5 patients (45%) of PSE group and in 11 (85%) of non-PSE group (p=0.043)., Conclusion: Increased platelet counts after PSE facilitates the adherence to peginterferon therapy in chronic hepatitis C patients with thrombocytopenia.

A 55-year-old Japanese housewife, who had Osler-Weber-Rendu disease, was admitted to our hospital because of frequent epistaxis and worsening exertional dyspnea. The computed tomography and hepatic arteriography revealed large hepatic arteriovenous malformation, which was considered to be the leading cause of her high output heart failure. Two series of hepatic arterial coil embolization procedures were performed to reduce hepatic shunt flow. They temporarily improved her cardiac condition, but gradually induced progressive hepatic failure due to intrahepatic cholangitis. Hepatic dysfunction restricted her quality of life and lead to a fatal clinical course one year after the second coil embolization.

Both ulcerative colitis and Takayasu's arteritis are though to be organ-specific immune-mediated inflammatory diseases. We present the rare case of a 23-year-old woman with a 4-year history of ulcerative colitis who developed Takayasu's arteritis one month after giving birth. She was found to carry the human leukocyte antigens (HLA)-B52 and DR2, which were previously noted to be associated with these inflammatory conditions in the Japanese population. The pathogenic relevance of this haplotype to the concomitant development of these two conditions is discussed.

A-39-year-old man was admitted to our hospital because of a markedly decreased level of serum cholinesterase found incidentally by a blood test. Detailed examination did not reveal severe liver disease, malignant tumor, infection or organophosphate compound poisoning. Investigation of three generations of his family revealed two homozygous and five heterozygous family members with the cholinesterase deficiency gene E1s indicating familial serum cholinesterase deficiency.