Objective We investigated the link between renal function as evaluated by estimated glomerular filtration rate (eGFR) and pulse wave velocity (PWV) in Japanese without medications.
Methods A total of 1,244 Japanese subjects, aged 20-79 years, were recruited in a cross-sectional clinical investigation study. They received no medications. eGFR was calculated using serum creatinine (Cr), age and sex. Peripheral arterial stiffness was evaluated by brachial-ankle PWV (baPWV).
Results eGFR and baPWV were significantly correlated with age. eGFR was negatively correlated with baPWV (men: r=-0.308, p<0.0001, women: r=-0.293, p<0.0001). Twenty-six men (5.6%) and 35 women (4.5%) were diagnosed as reduced eGFR (eGFR <60 mL/min/1.73 m²). We compared clinical parameters between subjects with reduced eGFR (Group R) and without such reduction (Group N). baPWV in Group R was significantly higher than that in Group N even after adjusting for age. In women, systolic blood pressure in Group R was also significantly higher than that in Group N.
Conclusion eGFR was closely associated with peripheral arterial stiffness in Japanese.

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Purpose Patients with hepatitis C virus (HCV)-associated glomerulonephritis in whom HCV RNA was not detected in the blood have been reported. We attempted to uncover the differences between HCV RNA-negative and HCV RNA-positive cases.
Methods The clinical and pathological findings for 5 patients with HCV-associated glomerulonephritis without HCV in the blood were compared with those for 23 patients with HCV RNA-positive HCV-associated glomerulonephritis.
Results The HCV RNA-negative cases were characterized by advanced age, female dominance, no cryoglobulinemia and no glomerular deposition of IgG compared with the HCV RNA-positive cases, although no differences were observed in amount of urinary protein, frequency of nephrotic syndrome, serum levels of total protein, creatinine level, presence of hypocomplementemia and rheumatoid factor. The prognosis was similar for both groups.
Conclusion These cases point to the need for further study of the pathogenesis of this disease.

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Background Maximal incremental exercise testing should be completed within 8-12 minutes. The ramp-slope influences the exercise duration. Oxygen-cost diagram (OCD) is a scoring scale for the capability of daily activity performed and therefore can be used to estimate the ramp-slope.
Methods The OCD-algorithm and the reported-algorithm were used prospectively in random order for selecting optimal ramp-slope: Ramp-slope_OCD=Score_OCD×weight in kg/40 for men and weight/50 for women and Ramp-slope_reported=(Predicted V_O2peak-V_O2unloaded)/100. Fifty-three dyspneic patients and 16 normal controls were enrolled to perform a ramp-pattern exercise. Fourteen patients not reaching maximum exercise levels were excluded. The exercise capacity, exercise time, and success rate of loaded exercise between 8 and 12 minutes were measured.
Results Comparing the reported-algorithm to the OCD-algorithm in normal controls, the only difference was that the ramp-slope was higher in males; in patients, the ramp-slope was higher in males, the exercise time shorter and the success rate lower (8.6±3.3 vs. 9.4±2.1 min, 61.5% vs. 84.6%, both p<0.05); in obese patients, the ramp-slope was lower and the exercise time longer.
Conclusion OCD score can predict the ramp-slope selection for exercise testing in normal controls and dyspneic patients. This may be affected by gender and body weight when using the reported-algorithm for dyspneic patients.

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Objective To examine the relation between annual trends in the antimicrobial susceptibility of Pseudomonas aeruginosa and drug usage, we compared annual changes in the susceptibility rates of P. aeruginosa clinical isolates during a 4-year period and annual trends in the overall usage of antimicrobials during the same period.
Methods We studied annual trends in MIC₉₀/MIC₅₀, antimicrobial use density (AUD), and antimicrobial susceptibility rates based on clinical breakpoints for 150 strains of P. aeruginosa isolated from respiratory specimens at Dokkyo Medical University Hospital from 2005 through 2008.
Results The MIC₉₀/MIC₅₀ of antimicrobials effective against P. aeruginosa in years 2005, 2006, 2007, and 2008 were as follows: imipenem, 32/2, 32/1, 8/2, and 16/1 μg/mL; meropenem, 8/1, 8/1, 4/0.5, and 4/0.5 μg/mL; and biapenem, 16/1, 32/0.5, 4/0.5, and 8/0.5 μg/mL, indicating that susceptibility to carbapenems increased slightly. The MIC₉₀/MIC₅₀ was 4/0.25, 2/0.125, 1/0.125, and 2/0.25 μg/mL for ciprofloxacin, 8/4, 8/4, 4/4, and 8/4 μg/mL for amikacin, 64/16, 64/16, 64/16, and 64/16 μg/mL for sulbactam/cefoperazone, 8/2, 16/2, 32/2, and 8/2 μg/mL for ceftazidime, indicating little change. The AUDs of fourth-generation cephalosporins increased from 2005 to 2008 (16.2, 18.4, 28.0, and 23.0), while the AUDs of carbapenems decreased (25.7, 23.7, 10.9, and 12.5).
Conclusion The decrease in the AUDs of carbapenems was associated with increased susceptibility rates of P. aeruginosa to carbapenem derivatives. A continuous understanding of trends in the resistance of P. aeruginosa and various other pathogens is essential for designing countermeasures against nosocomial infections, including the proper and effective use of antimicrobials.

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Introduction Invasive mechanical ventilation (IMV) is not effective for acute exacerbation of interstitial pneumonia (AE-IP); however, the role of non-invasive ventilation (NIV) for this condition remains unknown.
Methods Comparisons were made for two periods: before (October 2001 - September 2003) and after (October 2004 - September 2006) the introduction of NIV as the primary method of mechanical ventilation for AE-IP. We retrospectively screened emergent admissions and enrolled consecutively those patients with AE-IP who had acutely worsening hypoxemia with new infiltrates, background chronic interstitial CT changes, and no findings suggestive of other diseases. The two periods were compared primarily for 60-day survival and secondarily for other outcomes associated with mechanical ventilation.
Results Medical records were retrieved for 11 episodes in 11 patients identified from 485 pre-NIV records and 27 episodes in 22 patients from 859 post-NIV records. Five patients required IMV in the earlier cohort and 9 patients received NIV in the later cohort. Although there was no difference in the PaO₂/FiO₂ ratio on admission (167 vs. 139), the 60-day survival rate for all episodes in the later cohort was better than in the earlier cohort (27% vs. 65%, p=0.02). Moreover, the NIV-administered group had a better 60-day survival rate (0% vs. 44%, p=0.03), shorter high-care unit stay (17 vs. 6 days, p=0.03) and better-preserved verbal communication (0 vs., 89%) than the IMV-administered group.
Conclusion Use of NIV in place of IMV for the management of life-threatening AE-IP appears to result in a better 60-day survival rate, lower high-care unit use and better patient tolerability.

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Objective The subsets and the polarization of lymphocytes in bone marrow from low-risk myelodysplastic syndrome (MDS) were studied and compared with those from patients with aplastic anemia (AA).
Methods A total of 34 patients with low-risk MDS (IPSS score≤1.0) who presented abnormal chromosomes and 16 patients with AA were enrolled in this study. We determined T lymphocyte subsets, T cells polarization status, and the percentages of NK cells and of B lymphocytes in bone marrow and compared these parameters between the two groups of patients. As controls, 24 patients with high-risk MDS (IPSS score>1.0) presenting abnormal chromosomes and 22 healthy/benign hematologic disease subjects were used.
Results In low-risk MDS/AA patients, the percentage of CD3+ lymphocytes was significantly increased compared to controls (p=0.006 and p=0.001), while the percentage of CD19+ lymphocytes was significantly decreased (p<0.001 and p=0.002); there were no significant differences between MDS/AA and normal controls in other parameters; For low-risk MDS patients, the polarization status of bone marrow CD4+ cells toward Th1 (Th1/Th2) and of CD8+ cells toward Tc1 (Tc1/Tc2) was stronger than that for AA patients (p=0.05 and p<0.001). Other parameters did not show significant differences; Regardless of the predominance of CD4 or CD8 T cells, all patients with low-risk MDS were accompanied with elevated Tc1 polarization (Tc1/Tc2).
Conclusion In both AA and MDS, the number of total T lymphocytes increased. However, polarization towards Th1 and Tc1 was obviously stronger in MDS patients than in AA patients. This might be related to T cell stimulation from the clones of malignant hematopoietic cells.

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Objective Heart failure is one of the most serious complications in Duchenne muscular dystrophy (DMD). Beta-blocker medication is known to improve the prognosis of chronic heart failure of adults, but its efficacy and safety for DMD patients has not been fully assessed. Thus we conducted a multicenter open trial.
Methods Fifty-four DMD patients participated; 41 received carvedilol (BB group) and 13 did not (non BB group). All patients with an ejection fraction of less than 50% received angiotensin-converting enzyme inhibitor. Then, patients in BB group were started on carvedilol. The mean maintenance dose of carvedilol in BB group was 7.85±2.80 mg/day. Clinical signs and cardiac function were monitored regularly and statistical analysis was done.
Results The survival rate free from primary endpoints (death, deterioration of heart failure and severe arrhythmia) was higher in the BB group. The survival rate free from all-cause death was also higher in the BB group, although not significantly higher. Patients with primary endpoints received lower maintenance doses of carvedilol and presented higher mean heart rates (HR) during the observation period. In the BB group, mean HR at enrollment and the reduction of mean HR were correlated with the change of ejection fraction. Although serious adverse events were rare during the introduction of carvedilol, patients with advanced cardiac dysfunction required a longer period for up-titration and frequently presented with minor complaints.
Conclusion The present study suggests that carvedilol is relatively safe and can prevent cardiac events even in patients with DMD.

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Objective Mikulicz's disease (MD) is characterized by symmetrical and persistent enlargement of the lacrimal and salivary glands. Recently it has been categorized as an 'Ig (immunoglobulin) G4-related disease.' It presents with elevated serum levels of IgG4 and abundant infiltration of IgG4-bearing plasmacytes in involved organs. Allergic symptoms are often observed in patients with IgG4-related disease. On the other hand, allergic diseases are often complicated with Churg-Strauss syndrome (CSS). Here we focused on CSS and analyzed the relation of IgG4 in its pathogenesis.
Materials and Methods We analyzed five patients (2 men and 3 women) with CSS and 51 patients (20 men and 31 women) with MD who presented at Sapporo Medical University Hospital since 2001. We measured the serum concentrations of IgG subclasses in the patients with MD and CSS, and evaluated renal specimens from CSS patients, staining them for anti-IgG4 antibody.
Results We surprisingly found elevated serum levels of IgG4 not only in MD but also in CSS patients. The renal specimens in CSS patients revealed the infiltration of IgG4-positive plasmacytes.
Conclusion IgG4-bearing plasmacytes may be involved in the pathogenesis of CSS, and it is possible that an allergic reaction plays an important role in the pathogenesis of IgG4-related disease.

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A 38-year-old woman was hospitalized in August 2007. This visit was her fifth episode of acute pancreatitis. Computed tomography revealed a cystic structure located near the antrum. Communication between this structure and the pancreatic duct was revealed by endoscopic retrograde cholangiopancreatography. Ultrasonography revealed that the cyst wall had a layered structure. Thus, we regarded it as a gastric duplication cyst. We thought that the gastric duplication cyst communicating with an aberrant pancreatic duct was responsible for the recurrent acute pancreatitis. In August 2008, a cyst gastrostomy was performed between the gastric duplication cyst and the stomach. No recurrence of acute pancreatitis has since occurred.

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We present the case of an immunocompetent 50-year-old Japanese man with a cerebrospinal fluid (CSF) abnormality relevant to syphilis; his original presentation was liver dysfunction, the etiology of which was uncertain until positive serology for syphilis was identified. Liver dysfunction was promptly resolved after oral penicillin therapy; however, serological recurrence developed. CSF abnormality associated with syphilis was confirmed by subsequent lumbar puncture. Syphilis should be included in the differential diagnosis of patients with liver dysfunction of unknown etiology, and possible neurosyphilis should be considered when the treatment becomes refractory, even when there is no evidence of neurological manifestations.

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A 62-year-old female was admitted for examination of an abnormal liver function. Plain CT and MRI of the abdomen showed marked hepatomegaly but no visible nodular lesion in the liver. On the 3rd hospital day she had hepatic encephalopathy and was treated with a course of high-dose steroids, but ultimately died of disease progression on the 7th hospital day. An autopsy revealed a small pulmonary nodule with the histological findings showing small cell carcinoma. There was almost complete parenchymal replacement with metastatic tumor in the liver. Neoplastic involvement of the liver should be considered in the differential diagnosis of fulminant hepatic failure of unknown etiology.

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Free wall rupture, the most fearful complication of myocardial infarction, mostly attacks anterior walls. Acute rupture is characterized by rapid development of mechanical arrest accompanied with bradyarrhythmia or electromechanical dissociation. The majority of patients succumb to death as the result of cardiac tamponade. Risk factors are advanced age, female gender, the first-time myocardial infarction, hypertension, and ST-segment elevation. We report a rare case of posterior wall myocardial infarction complicated with left ventricular rupture initially presenting with junctional escape rhythm.

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An 80-year-old woman with type 2 diabetes was admitted due to right-handed muscle weakness. The patient presented with Brown-Sequard syndrome, with complete paralysis of the right lower limb along with a loss of pain and temperature sensations in the left lower limb. Magnetic resonance imaging revealed a cervical epidural abscess, and accompanying edema or inflammation of the right side of the spinal cord at the C5 level. She underwent drainage and evacuation of the spinal abscess, followed by intravenous antibiotic administration. These interventions ameliorated the neurological deficits. The present case suggests the importance of epidural abscess as a rare pathogenetic cause of Brown-Sequard syndrome in type 2 diabetes.

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A 75-year-old woman was found to be unconscious in hospital. She was febrile with a temperature of 38.4°C. She had hypotension (blood pressure 80/40 mmHg) with serum Na 132 mEq/L and K 5.7 mEq/L (serum Na/K = 23.2), and serum cortisol 0.91 μg/dL, indicative of adrenal failure. She was admitted for the treatment of Graves' hyperthyroidism, and was found to be unconscious in hospital. We encountered a patient with unrecognized adrenocortical disease, in whom development of Graves' hyperthyroidism caused an adrenal crisis. The ACTH stimulation test indicated that she had 21-hydroxylase deficiency (21OHD); after ACTH stimulation, 17-OH-progesterone increased from 0.6 to 10.4 ng/mL (17.3 times), and 17-OH-progesterone/cortisol from 0.0049 to 0.045 (9.2 times). She did not have clinical signs of classical 21OHD. She had non-classical 21OHD (NC21OHD). Development of Graves' hyperthyroidism caused an adrenal crisis in a patient with previously unrecognized NC21OHD.
A patient with unrecognized adrenocortical disease developed Graves' hyperthyroidism, which induced an adrenal crisis. She had NC21OHD.

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The combined clinical presentation of acute renal failure with pulmonary hemorrhage is known as pulmonary-renal syndrome. We describe a case of an 84-year-old woman who presented with acute renal failure and pulmonary hemorrhage at onset. Renal biopsy and bone marrow aspiration showed cast nephropathy and an abnormal increase in plasma cells, respectively. She was diagnosed with multiple myeloma and successfully treated with plasma exchange and corticosteroids. This case indicates that multiple myeloma should be considered as a cause of pulmonary-renal syndrome.

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We report a case of Kimura's disease with membranous nephropathy. A 15-year-old Japanese boy was referred to our hospital with proteinuria and a subcutaneous mass in the hip. Renal biopsy demonstrated secondary membranous nephropathy (MN) with mild mesangial proliferation and some electron-dense deposits in the mesangium. Laboratory tests revealed eosinophilia and a high level of serum IgE, which are common findings in Kimura's disease. A biopsy of the mass in the hip was performed and Kimura's disease was diagnosed. Treatment with oral prednisolone resulted in a decrease of proteinuria and regression of the subcutaneous mass. Kimura's disease should be included in the differential diagnosis of secondary MN.

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Heavy chain deposition disease (HCDD) is characterized by glomerular and tubular deposition of non-amyloidotic monoclonal heavy chains without associated light chains. We describe a case of γ1-HCDD who presented with nephrotic syndrome, microhematuria, and hypocomplementemia. Renal biopsy showed lobular and nodular glomerulosclerosis along with IgG and C3 deposition. Electron microscopy revealed electron-dense deposits on the glomerular and tubular basement membranes and mesangium. Congo red staining was negative. Staining was positive for IgG1 on glomerular and tubular basement membranes but negative for IgG2-4, κ and λ light chains. Staining for the constant heavy chain domains showed the deletion of the first constant heavy chain domain. Thus, we diagnosed γ1-HCDD. She was considered to be early-stage HCDD because proteinuria and hematuria were not observed before the nephrotic syndrome onset. Melphalan and prednisolone (MP) therapy reduced proteinuria as well as improved renal function and complement levels. Although renal prognosis of HCDD is poor, aggressive chemotherapy with MP may be effective in early-stage HCDD patients.

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Prolonged steroid therapy is generally used for steroid-dependent nephrotic syndrome in pediatric patients. However, dwarfism secondary to a long-term regimen and its successful reverse is rarely reported. The underlying mechanism of dwarfism is still poorly understood, as both long-term steroid use and nephrotic syndrome may interact or independently interfere with the process of growth. Here, we present a 17-year-old patient with dwarfism and steroid-dependent nephrotic syndrome and the successful treatment by recombinant human growth factor and cyclosporine A with withdrawal of steroid. We also briefly review the current understanding and the management of dwarfism in pediatric patients with nephrotic syndrome.

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Although the association between seminoma and sarcoidosis or a sarcoid-like reaction has been well recognized, a paradoxical case during chemotherapy for seminoma has not been reported. We report the first case of a sarcoid-like reaction that developed paradoxically during successful chemotherapy for recurrent seminoma. A 36-year-old Japanese man had recurrent seminoma in abdominal lymph nodes four years after left orchiectomy. Chemotherapy consisting of bleomycin, etoposide and cisplatin had reduced the abdominal mass, but mediastinal and bilateral hilar lymphadenopathy newly appeared. Surgical biopsy of the mediastinal lymph nodes and left nephrectomy and retroperitoneal lymphadenectomy revealed noncaseating granulomas only in mediastinal lymph nodes and confirmed complete remission of seminoma. Nine months later the thoracic lymphadenopathy had resolved spontaneously.

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We report a 23-year-old woman with interstitial inflammation and fibrosis associated with deposition of surfactant proteins in the airways. Biopsy of the lung showed multiple focal lesions containing dilated airways with inflammation and fibrosis. Pale eosinophilic amorphous material, dense eosinophilic bodies and numerous cholesterin clefts were observed in the airways. Surfactant protein A (SP-A) was expressed in the amorphous material. SP-D was identified in the dense eosinophilic bodies and around the acicular clefts. The finding that surfactant depositions were anatomically coincident with the interstitial pneumonia suggests that surfactant depositions were closely related to the interstitial pneumonia.

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Lemierre's syndrome is a rare anaerobic oropharyngeal infection complicated by internal jugular thrombosis, the predominant etiology is Fusobacterium necrophorum. Septic emboli to lungs and distant organs have been described, however, to date there have been no published cases associated with embolization across a patent foramen ovale. We describe a case of Lemierre's syndrome with septic arterial emboli resulting in multiple cerebral abscesses and cutaneous manifestations. The outcome was favorable with appropriate antimicrobial therapy.

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A 46-year-old man was referred to our hospital with hemoptysis. He had been diagnosed with chronic sinusitis since childhood, but had received no treatment. Chest CT showed a diffuse centrilobular granular shadow and thickened bronchial walls. Otitis media and decreased spermatic motor ability were identified. In addition, electron microscopy of a biopsy specimen of the nasal mucosa showed a deficiency of inner dynein. Based on these clinical findings, primary ciliary dyskinesia (PCD) was diagnosed and successfully treated with long-term, low-dose clarithromycin. Although the effects of macrolide therapy remain controversial, long-term treatment with low-dose clarithromycin might confer clinical benefits upon patients with PCD.

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The chronic graft-versus-host disease often requires unceasing immunosuppressive therapy (IST), which increases a risk of infectious complications in hematopoietic stem cell transplantation (HSCT) recipients. We report an adult T-cell leukemia/lymphoma case who developed pulmonary nocardiosis, a rare pulmonary complication, after allogeneic HSCT despite administration of the prophylactic trimethoprim-sulfamethoxazole (TMP/STX). The inhaled corticosteroid in addition to systemic IST had been started for bronchiolitis obliterance 4 months prior to nocardiosis development. The patient was successfully treated with an increased dose of TMP/STX combined with meropenem. Transplantation physicians should keep this rare pulmonary complication in mind during sustained IST.

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Complications of bronchial artery embolization (BAE) are uncommon. A 37-year-old patient with pulmonary tuberculosis received bronchial artery embolization because of severe hemoptysis. The bilateral bronchial arteries and left internal mammary artery were embolized using a gelatin sponge, and the patient exhibited occipital blindness and ataxia after the second BAE. The dissolvable gelatin sponge possibly entered the posterior circulation, resulting in the multiple infarctions in the bilateral occipital lobes and cerebellum. Because of the bad prognosis and the difficulty for curability, this kind of complication should be recognized in a timely manner and carefully avoided by the interventional radiologists carrying out the BAE.

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We report a case of Mikulicz's disease, IgG4-related plasmacytic syndrome, successfully treated with corticosteroid and mizoribine, followed by mizoribine alone. A 56-year-old woman experienced persistent bilateral swelling in the eyelids and submandibular region accompanied by lymphadenopathy without pain. The patient was diagnosed as having Mikulicz's disease since her bilateral lacrimal glands showed persistent swelling and biopsy specimens of the submandibular glands and lymph node showed diffuse infiltration of lymphocytes and plasma cells. Symptoms and laboratory data were improved with corticosteroids (30 mg). However, after tapering prednisolone to 5 mg, submandibular swelling developed. Adding mizoribine resulted in clinical improvement. Prednisolone was tapered and finally discontinued. Since the levels of IgG4 in peripheral blood were increased even after corticosteroid therapy and IgG4 was positive in a submandibular gland specimen obtained before the start of corticoid therapy. Thus, this case may be associated with IgG4. The present case is the first case in which mizoribine was effective for Mikulicz's disease and IgG4-related plasmacytic syndrome.

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