Objective In hypertensive patients with typical chest pain but absence of coronary stenosis the coronary microcirculation may be impaired, thus, our study aimed to appraise, in these subjects, the role of the coronary microcirculation, assessed by Myocardial Blush Grade (MBG) and Thrombolysis in Myocardial Infarction (TIMI) Frame Count (TFC). Methods A total of 95 subjects with chest pain and uninjured coronary arteries were recruited into the study: 80 subjects were hypertensive while 15 subjects were normotensive. The hypertensive subjects were divided into two subgroups: hypertensive subjects with positive scintigraphy and hypertensive subjects with negative scintigraphy. The TFC, a quantitative method of assessing coronary artery flow, the MBG, an assessment of the level of tissue perfusion, and the Total Myocardial Blush Score (TMBS), the sum of the myocardial blush grades of each coronary territory, were evaluated on the coronary angiogram of every patient. Results The TFC was higher (p<0.05) in hypertensive subjects than in normotensive subjects. The MBG and the TMBS were lower (p<0.05) in hypertensive subjects than in normotensive subjects. The TFC was higher (p<0.05) in positive scintigraphy than in negative scintigraphy. The MBG was lower (p<0.05) in positive scintigraphy than in negative scintigraphy. The Spearman rank correlation test showed that the TFC and the MBG was inversely associated. Conclusion The hypertensive subjects had impaired coronary artery flow and myocardial perfusion, documented by the TFC, MBG and myocardial perfusion scintigraphy. These methods may be universally applicable, in fact they are validated and most catheterization laboratories have access to them.
Objective Patients with chronic kidney disease (CKD) are at a high risk for cardiovascular diseases including stroke. However, the characteristics of the stroke subtypes in patients with CKD remain to be determined. Methods We investigated the associations between stroke subtypes and estimated glomerular filtration rate (eGFR), and traditional risk factors in 451 (males, 239; mean age, 71.1 y) acute stroke patients at our hospital between 2006 and 2010. Results The stroke subtype was cardiogenic cerebral embolism in 129 (29%), cerebral hemorrhage in 104 (23%), unclassified cerebral infarction in 65 (14%), lacunar infarction in 65 (14%), subarachnoid hemorrhage in 41 (9%), atherothrombosis in 30 (7%), and transient ischemic attacks in 17 (4%). Among the 451 patients, 134 (30%) had CKD with eGFR <60 mL/min/1.73 m2. Compared with a group without CKD, mean age (75.9 vs. 69.0 years, p<0.05), the prevalence of atrial fibrillation (AF) (44% vs. 21%, p<0.01) and a history of cardiovascular disease (37% vs. 19%, p<0.01) were significantly higher in that with CKD. A comparison of stroke subtypes revealed a significantly higher incidence of cardiogenic cerebral embolism (36% vs. 26%, p<0.05) in the group with, than without CKD. Conclusion We showed the prevalence of CKD in about 30% of acute stroke patients, and those patients were older, had a significantly higher prevalence of AF and a higher rate of cardiogenic cerebral embolism compared with patients without CKD. Thus, strict control of blood pressure and management of AF should be important to prevent stroke among patients with CKD.
Objective To clarify the clinical characteristics of type 2 diabetic patients with non-alcoholic fatty liver disease (NAFLD) and to assess whether NAFLD is related to angiopathy. Methods The study included 388 Japanese type 2 diabetic outpatients without viral hepatitis. The main outcome measures were angiopathy and NAFLD. Results The 388 subjects were divided into two subgroups based on alcohol consumption. Fatty liver was recognized in 36 of the 142 drinking patients (25%). There was no association of fatty liver disease with diabetic macro- or microangiopathy in these patients. Fatty liver disease (namely, NAFLD) was recognized in 77 of the 246 non-drinking patients (31%). Type 2 diabetic patients with NAFLD had a significantly younger age, higher body mass index level, higher levels of HbA1c, total cholesterol and triglyceride, lower HDL-C level, higher prevalence rates of hypercholesterolemia and obesity than counterparts without NAFLD. In addition, individuals in the elderly (≥65 years) non-drinking group with NAFLD had a significantly higher prevalence rates of diabetic macroangiopathy, coronary heart disease and thicker intima-media thickness level than their counterparts without NAFLD. The logistic regression analysis showed that NAFLD is an independent predictor of diabetic macroangiopathy. Conclusion NAFLD was associated with an increased prevalence of diabetic macroangiopathy and coronary heart disease in elderly patients. In addition, NAFLD is an independent predictor for diabetic macroangiopathy. These findings suggest that type 2 diabetic patients with NAFLD should be considered as a high risk group for developing macroangiopathy, even if macroangiopathy is not clinically detected.
Background Pulmonary arteriovenous malformations (PAVMs) are rarely encountered in clinical practice. The prevalence of PAVMs associated with hereditary hemorrhagic telangiectasia (HHT) has been estimated based on the rate in the family members of HHT patients, but the prevalence of PAVMs in the general population remains unknown. Methods We retrospectively examined the prevalence and clinical characteristics of PAVMs as detected by a low-dose thoracic CT screening program for lung cancer at the Hitachi Medical Center and the Hitachi General Health Care Center in the northern part of Ibaraki Prefecture, Japan. Results From 2001 to 2007, we identified eight patients (seven females and one male) with PAVMs among 21,235 initial screening participants (the mean age of the patients with PAVMs and that of the screening participants was 60.6 years). The prevalence of PAVMs was estimated at 38 per 100,000 individuals [95% confidence interval (CI)=18-76]. The diameter of the PAVMs was a mean of 6.6 mm, and none of the lesions could be detected by chest X-ray. Females older than 60 years tended to have larger PAVMs than younger women did (p=0.06). Two patients (25%) were diagnosed with HHT. One patient had previously undergone surgery for a brain abscess. Conclusion PAVMs are more prevalent than previously reported, especially among females.
Objective To evaluate the incidence and risk factors for malignancy in Japanese patients with systemic sclerosis (SSc). Methods A cohort of 405 Japanese patients with SSc who visited Kitasato University hospital between 1973 and 2008 was analyzed retrospectively until the end of 2009. The incidence of malignancy was compared with that of the general population, with calculation of the standardized incidence ratio (SIR) and 95% confidence interval (CI). Results The cohort represented 4,787 person-years of total disease duration after the diagnosis of SSc. Of 27 malignancies clinically found, lung cancer (n=10, 37%), especially adenocarcinoma, was the most frequent, followed by breast (n=4, 15%) or gastric (n=3, 11%) cancer. SSc patients with overlapping CTD tended to have less malignancy. Multivariable analysis revealed heart involvement of SSc as a significant risk factor for breast cancer (RR 8.2, 95% CI 1.2-72.8). Other than gastric cancer, the calculated SIRs of malignancies in SSc patients were above 1 (SIR of overall malignancy 1.24, 95% CI 0.77-1.71), even though only lung cancer had a significantly elevated incidence (SIR 5.73, 95% CI 2.18-9.29). Every patient with lung cancer had interstitial lung disease (ILD) and every autopsy performed on patients with lung cancer found a primary lesion of lung cancer in their ILD lesion (n=4). Conclusion Lung cancer was significantly frequent in SSc patients, which could develop on the basis of complicated ILD.
Involvement of the upper gastrointestinal tract in sarcoidosis is rare, and an optimal treatment regimen remains to be determined. Here, we report 3 cases of upper gastrointestinal sarcoidosis in Japanese patients aged 57-70 years with or without epigastric symptoms and describe the clinical features, laboratory data, and upper endoscopy and pathological findings. Upper gastrointestinal sarcoidosis was diagnosed based on the presence of noncaseating epithelioid cell granuloma in the lamina propria in the stomach or duodenum. In conclusion, the possibility of upper gastrointestinal sarcoidosis should be considered in patients with epigastric symptoms and a history of sarcoidosis, even in those with stable disease.
IgG4-related sclerosing cholangitis (IgG4-SC) and primary biliary cirrhosis (PBC) can both cause liver dysfunction. While IgG4-SC is associated with IgG4-related diseases such as autoimmune pancreatitis, PBC is associated with various autoimmune diseases. However, there is only one case report of an association between IgG4-SC and PBC, and this association has not been elucidated further. The treatments for these two diseases are different, i.e., corticosteroid treatment is performed for IgG4-SC and ursodeoxycholic acid is given for PBC. We report a case of IgG4-SC overlapping with PBC in a 45-year-old man who was successfully treated by combination therapy with prednisolone and ursodeoxycholic acid.
A 79-year-old woman who had been suffering from dyspnea on effort for more than 50 years was admitted for further examination and treatment. On the screening respiratory examinations, the A-aDO2 was elevated but none of diffusion disturbance, ventilation-perfusion ratio inequality nor right-to-left shunt was detected. Finally, the fact that the dizziness occurred only in sitting or standing position was revealed by persistent history taking. Transesophageal echocardiography in recumbent and sitting positions revealed the platypnea orthodeoxia syndrome associated with atrial septal defect. This case highlights the necessity of awareness of this syndrome and the occult atrial septal defect.
A 79-year-old man had a 3.5-year history of edema of the lower extremities of unknown etiology. Abdominal computed tomography showed a soft tissue mass around the abdominal aorta, and the biopsy revealed dense fibrosis with abundant infiltration of IgG4-positive plasma cells. His serum IgG4 level was increased to 188 mg/dL. His lower extremity edema was induced by stenosis of the inferior vena cava (IVC) due to the mass. With a diagnosis of IgG4-related retroperitoneal fibrosis, he was treated with steroid, and the leg edema decreased with improvement of patency of the IVC and reduction of the soft tissue mass.
Adrenocorticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH) is a rare disease which causes Cushing's syndrome. Bilateral adrenalectomy has been recommended as the treatment of choice for AIMAH. However, bilaterally adrenalectomized patients require lifelong steroid replacement therapy. Therefore, an increasing number of patients have undergone unilateral adrenalectomy for AIMAH. We report a case of AIMAH due to refractory diabetes in whom unilateral adrenalectomy initially yielded good diabetes control, but in whom poor glycemic control developed after 5 years, requiring eventual additional contralateral adrenalectomy. In elderly patients with AIMAH, one-stage bilateral adrenalectomy may be the treatment of choice.
The patient, a 77-year-old-man, began peritoneal dialysis (PD) in August 2005. In January 2009, he developed lower abdominal pain and cloudy PD effluent. A diagnosis of peritonitis was made and Escherichia coli was detected in cultures of the PD effluent. An abdominal computed tomography scan showed a fish bone in the duodenal wall. An upper gastrointestinal endoscopy was performed, and a 3-cm fish bone was removed. We thus recommend careful investigation with the possibility of enteric peritonitis from the intestinal tract when E. coli is detected in effluent cultures during PD.
A 48-year-old man was admitted with cloudy dialysate and diagnosed as peritoneal dialysis (PD)-related peritonitis caused by Roseomonas infection. This is the third case of PD-related peritonitis due to Roseomonas species and also the first case of peritonitis in automated peritoneal dialysis. Despite its low virulence and rare incidence in peritoneal dialysis, clinicians should be alert to the possibility of Roseomonas infection due to its high resistance to antibiotics. Literature on Roseomonas infection is also reviewed. The current guidelines for empirical peritonitis in PD patients do not adequately cover such infection. Refractory treatment in high risk cases should alert clinicians to upgrade antibiotics even for a vague manifestation.
A 60-year-old man with Waldenström's macroglobulinemia (WM) was admitted to our hospital for evaluation of rapid progressive renal deterioration despite 3 cycles of oral melphalan and prednisolone (MP) therapy. Renal biopsy just before introducing hemodialysis revealed cast nephropathy and severe tubulo-interstitial infiltration of B lymphocytes. After 6 cycles of rituximab, cyclophosphamide, vincristine and prednisolone (R-COP) therapy, his renal function improved enough to discontinue hemodialysis. This is a rare case of WM-related renal involvement caused by both monoclonal protein and tumor infiltration and, to our knowledge, the second report on improved renal function by rituximab-based therapy.
A 25-year-old woman presented with fever, arthralgia and proteinuria exhibiting leukopenia, hypocomplementemia, increased serum IgG and IgG4, and positive antinuclear and anti-double-stranded DNA antibodies. Renal biopsy revealed membranous nephropathy with tubulointerstitial nephritis. IgG subclass immunofluorescence revealed intense IgG4 expression in glomeruli, but no expression of IgG2. Observations resembled membranous lupus nephritis with tubulointerstitial nephritis; however, elevated IgG4, low titers of antinuclear and anti-double-stranded DNA antibodies, IgG4-bearing cell infiltration, and characteristic IgG subclass deposition in glomeruli prompted diagnosis of IgG4-related tubulointerstitial nephritis with membranous nephropathy. It is challenging but important to distinguish lupus nephritis from IgG4-related kidney disease.
A 55-year-old non-smoking woman was admitted to our hospital for re-evaluation of unimproved peripheral ground-glass opacities despite prednisolone and cyclosporine treatment. She was diagnosed with autoimmune pulmonary alveolar proteinosis (PAP) based on transbronchial lung biopsy and granulocyte/macrophage colony-stimulating factor (GM-CSF) antibody testing. GM-CSF inhalation therapy markedly improved the opacities. Bilateral, centrally located lung opacities are typical in PAP, however 10 PAP cases with peripheral infiltration were reported in Japan recently, of which GM-CSF antibody was positive in six. To avoid inappropriate immunosuppressant treatment, PAP should be considered in the differential diagnosis of such peripheral opacities. GM-CSF antibody might be useful for diagnosis.
Primary malignant melanoma of the trachea is extremely rare. We report here the first case of primary tracheal malignant melanoma in the asymptomatic stage. Incidentally, this 73-year-old man was found to have a flat tumor at the upper trachea on chest computed tomography scans. The tumor was surgically resected with end-to-end anastomosis and was diagnosed to be primary malignant melanoma of the trachea. Four months after the surgical resection, cervical lymph node metastasis was found. Despite the resection of metastatic lymph nodes and six courses of chemotherapy, he died of cachexia approximately two years after the discovery of the tracheal tumor.
Severe thrombocytopenia secondary to cytomegalovirus (CMV) infection is rare in immunocompetent hosts. We describe a case of severe thrombocytopenia secondary to CMV infection in an immunocompetent 30-year-old man who presented with pyrexia and bleeding tendency. A diagnosis of immune thrombocytopenia (ITP) was made following hematological and serological testing, and bone marrow aspiration. Acute CMV infection was confirmed by serological testing, antigenemia, and detection of CMV-DNA. Corticosteroid therapy was ineffective and intravenous immunoglobulin (IVIG) was therefore administered. This resulted in immediate recovery of the platelet count and cessation of nasal bleeding. Early IVIG administration should be considered in steroid-resistant cases.
We report on an 80-year-old woman who had shunt-responsive idiopathic normal pressure hydrocephalus (iNPH). Her brain MRI showed an initial change in the subarachnoid space (a disproportional narrowing of the subarachnoid space at the cerebral high convexities) at the age of 70 years, followed by enlargement of the ventricles (Evans index: 0.31) at the age of 78. The observation suggests that changes in the subarachnoid space may precede ventricular enlargement in iNPH.
A 42-year-old man presented with fever, headache and liver dysfunction, and was diagnosed as having aseptic meningitis by lumbar puncture. The PCR detected Epstein-Barr virus (EBV)-DNA in the peripheral blood and cerebrospinal fluid. About 20 days after onset, the patient presented with hearing impairment in the right ear, which was confirmed by a pure tone audiogram. The hearing acuity improved after the initiation of hydrocortisone sodium phosphate. We presume that the hearing impairment was due to auditory nerve neuritis related to extension of inflammation of the meninges. This is the first reported case of EBV-associated meningitis showing hearing impairment.
A 38-year-old woman with Wilson's disease developed neurological deterioration after 25 years of low-dose penicillamine administration. She showed an akinetic-rigid syndrome and cerebellar motor ataxia. Brain MRI showed increased signal intensity at the bilateral pons, midbrain, putamen, and thalamus. 123I-IMP-SPECT revealed a diffuse reduction of cerebral blood flow at the bilateral cerebral hemisphere including the basal ganglia. After the patient's regimen was changed to zinc therapy, her neurological condition gradually improved, and she showed almost complete recovery within two years. Serial MRI and SPECT studies showed a marked improvement in the lesions.
Familial Mediterranean fever (FMF) is an inherited disease characterized by recurrent episodes of fever and serositis. FMF is caused by mutations in the MEFV gene that encodes pyrin/marenostrin. The 5 most frequent mutations are M694V, M694I, V726A, M680I and E148Q. Here, we reported 3 FMF patients, a sister and two brothers, who have the same M694V mutation with different clinical presentations. While the sister presented with abdominal pain, one of the brothers presented with erysipelas-like erythema and the other brother with bilateral sacroiliitis. Here, we report the different clinical courses of FMF in a family carrying the same M694V mutation.
Fluoroquinolones are widely used in the management of infectious diseases, and are generally safe and well tolerated. However, immediate hypersensitivity reactions, including anaphylactic reactions, have been reported. We present here a case of anaphylactic shock in a 26-year-old man following intravenous levofloxacin administration. Skin tests confirmed an immediate hypersensitivity reaction to levofloxacin. Subsequent oral challenge tests for garenoxacin, which showed negative skin test results, confirmed that garenoxacin was well tolerated. This is the first report of tolerance to full-dose garenoxacin in a patient who developed an immediate hypersensitivity reaction to levofloxacin.
A 62-year-old woman, diagnosed as bronchial asthma 3 years previously, was admitted due to acute severe dyspnea. Physical examination revealed saddle nose, flare/swelling of the ear auricles, and stridor. Computed tomography demonstrated thickening of tracheal/bronchial walls and stenosis of the lumen that deteriorated on expiration, suggesting tracheobronchomalacia. Auricle biopsy indicated cartilage destruction. Based on these findings, the patient was diagnosed as relapsing polychondritis. As demonstrated in this case, relapsing polychondritis involving airways might be misdiagnosed as bronchial asthma due to stridor and transient corticosteroid-related improvement. Early diagnosis is necessary to prevent irreversible airway stenosis and progression to tracheobronchomalacia.
We report the case of a Japanese traveler who developed dengue hemorrhagic fever (DHF) with a probable secondary infection with dengue virus type 2 (DENV-2). DHF usually occurs in children, and rarely in adult travelers. Proper and timely interventions can markedly reduce the mortality rate of DHF patients. The expansion of endemic areas and increased frequency of travel to these areas may suggest increased incidence of DHF in non-endemic areas in the near future. Early recognition of reinfection with dengue virus and warning signs of circulatory failure are crucial to prevent a severe shock state.
The infection caused by non-b-type Haemophilus influenzae has been increasing in this Hib (H.influenzae serotype b) vaccination era. H.influenzae serotype f (Hif) is considered as one of those emerging pathogens. In general, H.influenzae is a common pathogen of such as pneumonia, otitis media, and meningitis, but is rare in soft tissue infection, especially at the extremity. We report a rare case of severe soft tissue infection caused by Hif which occurred at the lower extremity of immunocompetent adult patient.
An association between Birt-Hogg-Dubé syndrome (BHDS) and colon cancer remains conjectural, but herein we describe a case who may illustrate a significant link between them. The 60-year-old woman was diagnosed at 28 years of age with colon carcinoma and familial adenomatous polyposis (FAP). She also had repeated pneumothoraces, and was diagnosed with BHDS following the finding of pneumothorax in her son. We confirmed the presence of germline mutations in both her folliculin (FLCN) and adenomatous polyposis coli (APC) genes. The family pedigree suggested that a de novoFLCN mutation might have contributed to the development of colon carcinoma at a younger age than her family members.
Hypercalcemia has been described in variety of granulomatous diseases and fungal infections. However, hypercalcemia in pulmonary cryptococcosis is rarely disclosed. We report a 57-year-old HIV-uninfected woman with diabetes, who initially presented with altered mental status, severe hypercalcemia with increased serum 1,25-dihydroxyvitamin D [1,25(OH)2D] concentration and suppressed parathyroid hormone. Pulmonary cryptococcosis was diagnosed as the cause of hypercalcemia. Successful treatment resulted in the resolution of hypercalcemia and decrease of the serum 1,25(OH)2D concentration to within the normal range. In summary, although HIV infection is a major risk factor for cryptococcosis, vitamin D-mediated hypercalcemia could be the initial presentation of pulmonary cryptococcosis in HIV-negative patients.