Emphysematous cystitis (EC) is a rare form of complicated urinary tract infection, its characteristic feature being gas within the bladder wall and lumen. Patients with EC present with variable clinical manifestations ranging from asymptomatic to severe sepsis. EC is typically observed in elderly women with severe diabetes mellitus. Escherichia coli and Klebsiella pneumoniae are often isolated from urine cultures. Imaging methods, such as plain conventional abdominal radiography and computed tomography, are pivotal for obtaining a definitive diagnosis of EC. Most cases can be treated with a combination of antibiotics, bladder drainage and glycemic control. EC is potentially life-threatening, with a mortality rate of 7%. Early medical intervention can contribute to achieving a favorable prognosis without the need for surgical intervention. In this review, we provide a comprehensive description of the clinical characteristics of EC.

View full abstract

Objective H. pylori infection in children has a high prevalence worldwide. The disease can cause progressive gastric mucosal inflammation, as verified in animal models. However, data from large-scale clinical studies are limited.
Methods We examined 1,634 Chinese children with upper gastrointestinal discomfort using endoscopy. The clinical and pathological data of the patients were analyzed.
Results A total of 524 (32.1%) patients were infected with H. pylori, and the prevalence of H. pylori infection increased with age. The H. pylori-infected patients exhibited a significantly higher prevalence of active inflammation (26.9% vs. 4.1%), lymphoid follicle formation (18.5% vs. 4.6%) and marked lymphocyte infiltration (19.7% vs. 5.6%). The H. pylori-infected patients also exhibited a significantly higher prevalence of moderate to marked chronic superficial gastritis (41.9% vs. 9.2%) and moderate chronic atrophic gastritis (21.7% vs. 2.6%) than the uninfected patients (p<0.01).
Conclusion H. pylori infection is associated with the degree of gastric mucosal inflammation and the severity of different types of chronic gastritis.

View full abstract

Objective The optimal medication therapies are recommended in patients with coronary artery disease even after the coronary revascularization. However, the information of optimal medical therapy in dialysis population is scant. We assessed the efficacy of statin on the clinical outcomes after Sirolimus-eluting stent (SES) implantation in patients with and without dialysis.
Methods and Results We analyzed date from 843 consecutive patients who successfully treated with SES in our institution between August 2004 and November 2006. Among patients, 96 patients (11.4%) were undergoing dialysis. In non-dialysis patients, 405 patients (54%) were treated with statin at hospital discharge. In dialysis patients, only 16 patients (17%) were treated with statin. In non-dialysis patients, mortality rate was significantly lower in patients treated with statin than those without statin (4.4% vs. 13.9%, p<0.0001). While in dialysis patients, mortality rate was similar between patients treated with and without statin (56.3% vs. 57.6%, p=0.86). After adjusting for confounders, the hazard ratios for mortality were 0.39 (95% confidence interval (CI), 0.14-0.99; p=0.047) in non-dialysis patients and 1.79 (95% CI, 0.39-7.86; 0.45) for dialysis patients. The interaction probability between statin use and dialysis for mortality was 0.016.
Conclusion The use of statin may have beneficial effect on reducing mortality rate in patients after SES implantation in non-dialysis patients. However, such favorable effect was not observed in dialysis population.

View full abstract

Objective Primary bone lymphoma is a rare disorder, accounting for less than 1% of all cases of malignant lymphoma. Primary bony diffuse large B-cell lymphoma (PBDLBCL) is the most common histological type. In our study, a favorable response and lower risk of emergent surgery were observed following the administration of systemic chemotherapy with or without rituximab.
Methods Patients diagnosed with malignant lymphoma at our hospital between 2000 and 2011 were evaluated for PBDLBCL. Pertinent data, including the clinical presentation, histological type, treatment modalities, long-term outcome, survival curve and prognostic factors, were analyzed.
Results Twenty-four patients with a median age of 63 years were diagnosed with PBDLBCL. A complete response (CR) was achieved in 58.4% (n=14) of the patients. With treatment of the disease, nine of 10 patients with initially impending pathological bone fractures ultimately did not undergo surgery. The median follow-up duration was 48 months. Two patients experienced disease relapse. In a Kaplan-Meier analysis, the 5-year overall survival (OS) and disease-free survival (DFS) rates were 66.7% and 77.8%, respectively. In the univariate analyses, the significantly favorable prognostic factors for OS were an International Prognostic Index (IPI) score of <3, an age of ≤60 years, a performance status with an Eastern Cooperative Oncology (ECOG) score of <2, a CR and chemotherapy ≥6 cycles. For DFS, a CR was the only favorable factor. In the multivariate analysis, a CR was the only independent factor for both OS and DFS.
Conclusion Our study confirms the good prognosis of this rare disorder. Once a CR is achieved, even elderly patients may exhibit long-term survival, possibly obviating the need for surgery for less severe bone lesions.

View full abstract

The patient was a 15-year-old girl with severe acute hepatitis. A liver biopsy showed the typical findings of autoimmune hepatitis (AIH). Subsequently, two lineages of cytopenia were found in the patient's peripheral blood. Hemophagocytosis by macrophages was observed in the bone marrow. Virus-, drug- and lymphoma-associated hemophagocytic syndrome (HPS) was ruled out. Therefore, the patient was diagnosed with autoimmune-associated HPS (AAHS). Following the administration of combination therapy with prednisolone and cyclosporine A, both the AAHS and AIH improved. This is the first report of AAHS originating from AIH. The patient was followed up for five years after treatment, and no disease recurrence was detected.

View full abstract

A 52-year-old man was admitted to our hospital due to shortness of breath that developed one week after the diagnosis of influenza infection. He had a past history of myocarditis associated with influenza B infection 16 years before the current admission. The patient's left ventricular function showed diffuse hypokinesis with a left ventricular ejection fraction of 28%. Due to the progression of heart failure, the infusion of catecholamines and insertion of an intra-aortic balloon pump were required. The patient was discharged uneventfully on the 23rd hospital day. A significant increase in the serum antibody titer against influenza A virus subtype H3N2 led to a diagnosis of recurrent fulminant influenza myocarditis.

View full abstract

Mixed cryoglobulinemia is occasionally seen in patients with hepatitis B virus (HBV) infection. This report presents the case of a quiescent HBV carrier who had type II mixed cryoglobulinemia, protracted purpura, ulcerative skin lesions and advanced chronic kidney disease. The cutaneous manifestations of the patient improved along with a decrease in the serum cryoglobulin and HBV-deoxyribonucleic acid levels following the initiation of oral entecavir in combination with plasmapheresis. However, the patient ultimately required prednisolone due to the limited benefits of these treatments. We also discuss various concerns regarding steroid treatment in patients with mixed cryoglobulinemia complicated by HBV infection.

View full abstract

Light chain proximal tubulopathy is a rarely reported entity associated with plasma cell dyscrasia that classically manifests as acquired Fanconi syndrome and is characterized by the presence of κ-restricted crystals in the proximal tubular cytoplasm. We herein present a case of multiple myeloma with Fanconi syndrome and acute kidney injury due to light chain proximal tubulopathy with light chain cast nephropathy. Prominent phagolysosomes and numerous irregularly shaped inclusions with a fibrillary matrix in the cytoplasm of the proximal tubules were identified on electron microscopy. A monotypic light chain of the λ type was detected in the distal tubular casts, proximal tubular cytoplasmic lysosomes and fibrillary inclusions on immunofluorescence and immune electron microscopy. This case underscores the importance of conducting careful ultrastructural investigations and immunocytologic examinations of light chains for detecting and diagnosing light chain proximal tubulopathy.

View full abstract

A 65-year-old woman experienced a hemolytic attack triggered by sepsis. She presented with markedly increased CD55^- CD59^- erythrocytes and the signs of bone marrow failure, which led to a diagnosis of aplastic anemia-paroxysmal nocturnal hemoglobinuria (AA-PNH) syndrome. There was a possibility of increasing hemolysis, as large PNH clones remained after immunosuppressive therapy (IST). Accordingly, eculizumab was first used to control the hemolytic attack followed by IST with antithymocyte globulin and cyclosporine A. The patient was successfully weaned from blood transfusions and has been followed up without any recurrence of hemolytic attacks.

View full abstract

Enteropathy-associated T-cell lymphoma (EATL), an intestinal tumor of intraepithelial T lymphocytes, is a rare and highly aggressive disease. We herein describe a case of type II EATL with massive pyoid ascites in which a histological examination could not be performed despite emergency laparotomy that was successfully diagnosed using flow cytometry and the cell block technique to analyze the celomic fluid. This case suggests that EATL should be included in the differential diagnosis of pyoid ascites of unknown origin and that flow cytometry and the cell block technique of assessing celomic fluid are useful procedures for diagnosing EATL, especially in cases in which conducting a histological examination is impossible.

View full abstract

A 61-year-old Japanese woman with chronic renal failure suddenly became silent at the end of hemodialysis. On a neurological examination, she was unable to respond to one-step commands, state the names of objects, repeat single words, read words aloud or write her name. Because she exhibited no paralysis of the extremities, we diagnosed her as having global aphasia without hemiparesis (GAWH). Cerebral angiography showed a dural arteriovenous fistula (AVF) in the left isolated transverse sinus with leptomeningeal venous drainage (Borden type 3, Lalwani grade 4). This case highlights dural AVF as an etiology of GAWH.

View full abstract

A 63-year-old man employed in a hard metal manufacturing company for 40 years presented with a chronic dry cough and exertional dyspnea 20 years after the onset of recurrent exanthemas. A chest radiograph revealed bilateral reticular shadows in the upper lung field. Pathological specimens in which tungsten was detected were obtained via a transbronchial lung biopsy. Patch tests were positive for cobalt and other metals. The patient was diagnosed with hard metal lung disease (HMLD) concurrent with contact dermatitis and treated with corticosteroids. This case suggests that allergies to metal may play a role in the onset of HMLD.

View full abstract

A 52-year-old woman presented to our hospital due to elevated liver enzyme levels and hypereosinophilia. Liver biopsy specimens revealed chronic hepatitis with abundant eosinophils and immunoglobulin G4(IgG4)-positive plasma cell infiltration. Systemic lymphadenopathy, including that involving the hepatic hilar lymph nodes, was observed on computed tomography, and the cervical lymph node biopsy specimens exhibited lymphoid follicular hyperplasia and IgG4-positive plasma cell infiltration. It is sometimes difficult to distinguish hypereosinophilic syndrome (HES) from IgG4-related disease, as some of the clinical findings overlap. The numerous areas of eosinophilic infiltration observed in the liver indicated a diagnosis of HES in our case.

View full abstract

A 38-year-old woman was suffering from irregular headaches and sleepiness. She had used soap containing Glupearl 19S (hydrolyzed wheat proteins) every day for approximately one year and had experienced an episode of rash eruption on her face seven months ago. Wheat-specific IgE antibodies were detected in her serum. A Western blot analysis revealed a high titer of IgE antibodies against Glupearl 19S and wheat proteins. The patient was sensitive to these compounds in a skin prick test. After avoiding eating wheat, her headaches and sleepiness disappeared. A hidden food allergy is a possible cause of these symptoms.

View full abstract

We herein report the case of 41-year-old homosexual man who presented to our hospital with typical acute retroviral syndrome. Complications of bilateral facial nerve palsy and appendicitis appeared eight days after admission. The bilateral facial nerve palsy spontaneously recovered one month later; however, the appendicitis required surgical intervention. To our knowledge, this is the first reported case of appendicitis related to acute retroviral syndrome.

View full abstract

We experienced a case of nephrotic syndrome (membranous nephropathy) complicated by extensive small cell carcinoma of unknown primary etiology that was diagnosed based on the findings of bilateral cervical and mediastinal lymphadenopathy. A complete cancer response and proteinuria remission following radical chemoradiation therapy were documented. The status of a complete response and nephrosis remission persisted for more than three years. This is the first report to demonstrate the long-term survival of a patient with extensive small cell carcinoma of unknown primary etiology complicated by paraneoplastic nephrotic syndrome.

View full abstract