The best regimen for adult minimal change nephrotic syndrome (MCNS) is still unknown. Due to an excessive number of adverse events caused by oral steroid monotherapy, enhanced steroid therapy (low dose of prednisolone with a short course of methylprednisolone or with another immunosuppressant) has been studied extensively for years. In this study, the PubMed, Embase, EBSCO and Cochrane Library databases were searched for clinical trials which compared enhanced steroid therapy with oral steroid monotherapy in adult MCNS and a meta-analysis was performed. Seven studies involving 357 patients were included. We found that patients treated with enhanced steroid therapy responded more quickly to complete remission (CR) [mean difference = -9.52, 95% confidence interval (CI): -12.66--6.39, p<0.00001] and showed fewer adverse events [risk radio (RR) = 0.72, 95% CI: 0.54-0.97, p=0.03] than patients receiving oral steroid monotherapy. The CR rate (RR= 0.96, 95% CI: 0.83-1.10, p=0.53) and relapse rate (RR=0.87, 95% CI: 0.57-1.34, p=0.53) were similar in both groups.
Objective The aims of this study were to assess the incidence of pancreatic cancer and the contributing factors for the diagnosis of tumors in patients with acute pancreatitis and to gain insight into how patients with acute pancreatitis should be followed up. Methods Using the electronic medical database of Shizuoka General Hospital, 177 patients admitted for acute pancreatitis in the past 6 years were evaluated retrospectively for pancreatic cancer. Results Twelve patients (6.8%) were newly diagnosed with pancreatic cancer. During the first hospitalization, 5 patients (41.7%) with a detected pancreatic mass underwent surgical treatment: the final tumor stages were IA, IIA, and IIB in 1, 2, and 2 patients, respectively. In 7 patients (58.3%) without a detected pancreatic mass at the first admission, a pancreatic mass was recognized on follow-up computed tomography (CT) in 2 patients with main pancreatic duct (MPD) dilatation, and 1 patient with recurrent acute pancreatitis. The tumor stages were IA, IIA, and IA, respectively. Among the remaining 4 patients without follow-up, the tumor stage was IV. The patient gender, age, MPD dilatation, tumor marker, and serum amylase level were not significantly associated with pancreatic cancer. The detection of a pancreatic mass on CT led to the diagnosis of pancreatic cancer. Conclusion Acute pancreatitis should be considered as a possible diagnostic indicator of pancreatic cancer. Various factors associated with acute pancreatitis and pancreatic cancer were not predictive of a diagnosis of pancreatic cancer. Only the detection of a pancreatic mass led to the early diagnosis of pancreatic cancer. Patients hospitalized for acute pancreatitis should be followed up with a diagnostic imaging modality.
Objective Intravascular catheter procedures are often performed in patients undergoing antithrombotic therapy. However, methods for the management of antithrombotic agents in the perioperative period are currently unclear. Therefore, the safety and management of antithrombotic agents in these patients were investigated. Methods A prospective, multicenter, observational study [Management of Antithrombotic Agents During Surgery or Medical Procedures with Bleeding (MARK study)] at 58 National Hospital Organization institutions in Japan was performed. 1,040 patients were enrolled in the MARK study and underwent an intravascular catheter procedure. For all participants, the details of the procedure, method of perioperative management of antithrombotic agents, and occurrence of thrombosis, embolism, and bleeding complications during the study period were investigated. Results The use of antithrombotic agents was continued in 1,017 patients (98%, continuation group) and discontinued in the other 23 (2%, discontinuation group). Continuation of the antithrombotic agents did not have a significant effect on the overall occurrence of thromboembolism [hazard ratio (HR), 0.15; p=0.08] or bleeding events (HR, 0.19; p=0.12). However, the overall adverse event risk was significantly lower in the continuation group than in the discontinuation group (HR, 0.18; p=0.03). Conclusion It is both safe and appropriate to perform intravascular catheter procedures in patients continuing with antithrombotic therapy.
Objective Metaiodobenzylguanidine (MIBG) scintigraphy is used to assess heart failure (HF) severity and to predict cardiac functional recovery. Cardiovascular magnetic resonance (CMR) imaging has recently been used to diagnosis HF. We evaluated CMR T2 mapping and MIBG scintigraphy in dilated cardiomyopathy (DCM) patients. Methods Consecutively, 22 DCM patients [aged 56.8±13.4 years; 6 women and 16 men; left ventricular ejection fraction (LVEF), 31.9±10.7%] who underwent T2 mapping and MIBG scintigraphy were retrospectively evaluated. Echocardiography results were recorded at baseline and the 6-month follow-up. Patients with an increased LVEF ≥15% between the 2 measures were considered to be responders. We measured each patient's T2 values and MIBG indices [the heart-to-mediastinum ratio (H/M) in the early phase, H/M in the delayed phase, and the washout rate (WOR)] at baseline. We compared these values between the 12 responders and 10 non-responders. Results The mean T2 value for all patients was 64.5±6.6 ms. The mean values of early H/M, delayed H/M, and WOR were 2.06±0.25, 1.94±0.35, and 43.5±11.8%, respectively. The T2 values were found to correlate with MIBG indices (p<0.05 for all) and were lower in the responders than non-responders (61.4 vs. 68.1 ms, p=0.013). MIBG indices were not significantly different. Conclusion Our study shows that the T2 values correlated with the MIBG indices and were increased in non-responders. T2 mapping may be useful in assessing the cardiac function and functional recovery in DCM patients.
Objective The aim of this study was to investigate the long-term effective control of serum uric acid by allopurinol on the carotid intima-media thickness (IMT) in patients with type 2 diabetes (T2DM) and asymptomatic hyperuricemia (HUA). Methods This was a randomized open parallel-controlled study. In this study, 176 patients with T2DM and asymptomatic HUA were randomly allocated to the conventional or allopurinol treatment groups on the basis of a computer-generated random number table. Changes in the carotid IMT, biochemical indexes, high sensitive C-reactive protein (hs-CRP) and the incidence of hypertension in patients before and after three years of treatment were examined and compared between the groups. Results There were no statistically significant differences in the baseline characteristics of the study participants between the two treatment groups (p>0.05 for all). Nevertheless, the serum uric acid, triglyceride, and hs-CRP levels and the homeostasis assessment for insulin resistance (HOMA-IR), systolic blood pressure, diastolic blood pressure and the carotid IMT in the allopurinol group were significantly lower than those in the conventional group after three years of treatment (p<0.01 for all). The intention-to-treat analysis indicated that the incidence of new-onset hypertension in the allopurinol group showed a declining trend compared to that in the conventional treatment group (6.8% vs. 13.6%, p>0.05). Conclusion The long-term effective control of serum uric acid by allopurinol may improve insulin resistance, decrease the serum levels of hs-CRP, reduce the carotid IMT, and may delay the development of atherosclerosis in patients with T2DM and asymptomatic HUA.
Objective The precise relationship between alcohol intake and metabolic syndrome (MetS) is still unclear, and the results from previous studies have been inconclusive. Thus, we examined the effect of alcohol intake on the risk of MetS in men in order to gain more information on a potential relationship. Methods This study included 22,349 men who were divided into four groups according to their average alcohol intake [non-, light (less than 20 g ethanol/day), heavy (equal or more than 20 g and less than 60 g ethanol/day) and very heavy (equal and greater than 60 g ethanol/day) drinkers]. We measured each subject's body mass index (BMI), waist circumference and blood pressure (BP) and conducted a blood test to obtain a complete blood count and biochemical panel. These results were used to obtain the MetS prevalence. Additionally, fatty liver was diagnosed using abdominal ultrasonography. Results Light drinkers had smaller waist circumferences. Heavy and very heavy drinkers had larger waist circumferences, a higher BMI, a higher BP, higher fasting plasma glucose levels, higher triglycerides (TG) levels and higher high-density lipoprotein (HDL) cholesterol levels while they had lower low-density lipoprotein cholesterol levels than nondrinkers. The prevalence of high BP, hyperglycemia and high TG was significantly higher in heavy and very heavy drinkers than in nondrinkers. The prevalence of low HDL cholesterol levels decreased with an increase in alcohol consumption. The prevalence of MetS was significantly lower in light drinkers and higher in very heavy drinkers compared with nondrinkers. Conclusion Alcohol intake significantly influences the risk of MetS in men. A significant association was seen between an alcohol intake of 60 g/day or higher and the prevalence of MetS.
Objective The incidence of iliopsoas abscesses has been increasing due to advances in diagnostic imaging techniques and the increased number of elderly individuals and immunodeficient patients with co-morbidities. Our aim was to investigate the management and treatment of iliopsoas abscesses, particularly the effectiveness of computed tomography (CT)-guided drainage in the era of interventional radiology. Methods A retrospective analysis was performed at a university hospital between January 2009 and March 2014. Patients There were 15 patients (eight men, seven women) 50-85 years of age (average: 70 years) diagnosed with an iliopsoas abscess. Results The etiology of the disease was investigated in 14 of the 15 patients, each of whom had a secondary iliopsoas abscess. The primary condition in nine of these patients (64.3%) was an orthopedic infection (spondylodiscitis); the most common symptom was fever (12 patients, 80%). Altogether, 10 patients (66.7%) had a multilocular abscess and five (33.3%) had bilateral abscesses. The most common pathogen was Staphylococcus aureus (seven patients, 50%). All 14 patients underwent drainage: 11 received CT-guided drainage, two underwent postdrainage surgery and one received ultrasonography-guided drainage. Poor drainage was overcome by inserting multiple drainage tubes (six patients) or performing transmembrane drainage with a guidewire. All but one patient survived. Conclusion Based on the high success rate of CT-guided drainage in this study, this technique is expected to continue to play a major role in cases requiring drainage, even in patients with bilateral or multilocular abscesses. However, this modality cannot be used in cases of gastrointestinal perforation.
ObjectiveClostridium difficile infection (CDI) is the major cause of antibiotic-associated diarrhea in hospital inpatients. Rapid testing for the toxins in stool specimens is inconclusive due to its low sensitivity. Therefore, a two-step method is recommended as the most appropriate approach. The purpose of the present study was to evaluate the differences in the disease severity score between the patients who were glutamate dehydrogenase (GDH)-positive/enzyme immunoassays (EIA) toxin-positive (group A) and those who were GDH-positive/EIA toxin-negative, but who were nonetheless finally confirmed to be toxin-positive by toxigenic culture testing (group B). Methods A rapid detection EIA for GDH and toxin A/B were simultaneously performed for initial screening. Subsequently, the toxin production by bacterial colonies in culture was retested with the same rapid test kit when necessitated by an equivocal result of the initial screening. Results A total of 334 fecal specimens were evaluated. Group A consisted of 25 specimens (from 16 patients) and group B consisted of 27 specimens (from 12 patients). The severity score (based on a number of factors, including age, body temperature, serum albumin level and white cell count) of group A and B was 2.2±0.7 and 1.4±0.5, respectively (p=0.002). Conclusion The cases of CDI in which the toxins were detected by the initial screening test were more severe than those where the toxins were not detected at the initial screening but were identified by the toxigenic culture. In addition, the most significant factors affecting the severity score were an older age and a lower serum albumin level.
Objective This study aimed to evaluate the effects of establishing a Department of General Internal Medicine (DGIM) on the length of hospitalization. We evaluated the length of hospitalization associated with diseases for which full-time specialists were not available and were instead treated by physicians of the DGIM after its establishment. Methods A retrospective cohort study was conducted with a review of the subjects' medical records. The subjects included patients ≥16 years of age who were hospitalized with pneumonia or cerebral infarction and treated by a physician with a specialty in internal medicine as the disease outside their specialty prior to DGIM establishment (October 1, 2006 to September 30, 2008) or by a physician of the DGIM after its establishment (October 1, 2009 to September 30, 2011). The primary outcome was the change in the length of hospitalization. The length of hospitalization for heart failure, which was treated by specialists (cardiologists) in both study periods, was also examined for comparison. Results We evaluated 322 and 423 cases of pneumonia treated before and after the establishment of the DGIM, as well as 223 and 229 cases of cerebral infarction and 132 and 206 cases of heart failure, respectively. The length of hospitalization before and after establishment of the DGIM was 21.6 and 16.0 days for the pneumonia patients (p<0.001) and 24.2 and 19.9 days for the cerebral infarction patients (p<0.001), respectively. On the other hand, the change in the length of hospitalization for the heart failure patients was not statistically significant (19.9 vs. 17.6 days; p=0.281). Conclusion The establishment of the DGIM reduced the length of hospitalization associated with diseases for which full-time specialists were not available by over 17%.
A 52-year-old woman with ulcerative colitis was admitted to our hospital for an ulcerative colitis flare-up under salazosulfapyridine therapy. The symptoms improved with high-dose corticosteroids. After prednisolone was tapered to 10 mg, the frequency of diarrhea increased. The diarrhea was accompanied by joint pain and a skin ulcer with abscess formation, which was diagnosed to be pyoderma gangrenosum. The patient was started on adalimumab. A positive response to the adalimumab therapy was observed after 2 weeks, during which time the ulcerative skin lesion healed completely, however, colonic mucosal healing was achieved at 2 months. Therefore, adalimumab appears to be an effective therapeutic option for patients with ulcerative colitis-associated pyoderma gangrenosum.
We herein describe the case of a 42-year-old man who developed severe hepatitis caused by hepatitis C virus (HCV) infection at 14 years after the start of human immunodeficiency virus (HIV) treatment. Surprisingly, the levels of alanine aminotransferase (ALT) fluctuated, reaching a peak higher than 1,000 IU/L during chronic HCV infection, and the hepatic histology showed advanced liver fibrosis at 3 years after the primary HCV infection. He was treated with simeprevir, peginterferon-alpha, and ribavirin with a sustained viral response. We conclude that HCV/HIV co-infected patients need to commence anti-HCV therapy when the levels of ALT fluctuate severely under successful HIV control.
A 52-year-old man underwent partial splenic embolization (PSE) for hypersplenism. The intrasplenic artery targeted for the embolization was large, and the distance between its trifurcated branches was short; therefore, Guglielmi detachable coils (GDC) 360° Complex Shape® were used, as well as conventional metal coils, to prevent coil migration. GDC are equipped with a shape-memory function and are more physically stable than conventional metallic coils because they form three-dimensional loops. In this case, an ideal extent of the splenic infarction was successfully achieved using a small number of coils. This is the first report of the use of GDC in PSE for hypersplenism.
A 75-year-old man with a 120-bpm tachycardia and typical atrial flutter was admitted. Echocardiography showed a dilated left ventricle with anterior and apical wall akinesia. Tachycardia was terminated with cavotricuspid isthmus ablation. Multiple imaging findings revealed a woven coronary artery anomaly (WCAA) in the left anterior descending artery. Stress myocardial perfusion imaging was performed after ablation in the sinus rhythm and revealed stress-induced ischemia and a fixed low uptake in the WCAA territory. WCAA is generally regarded as a benign condition; however, compromised blood flow within the anomaly, caused by tachycardia-related diastolic shortening, may induce ischemia.
Acute aortic syndrome complicated by both ST-segment elevation myocardial infarction (STEMI) and spinal ischemia is exceedingly rare. We herein report the case of a 66-year-old man who presented with paraparesis after primary percutaneous coronary intervention for STEMI. He was found to have an intramural hematoma of the ascending aorta and a severe dissection in the descending aorta, which led to both STEMI and paraparesis.
A boy with Duchenne muscular dystrophy was admitted to our hospital due to a transient loss of consciousness. Transthoracic echocardiography revealed left ventricular (LV) dilatation and diffuse hypokinesis of the LV wall. The LV wall was thin, and both non-compaction of the LV wall and marked thinning of the posterior LV wall resulting from a lesion were observed. The plasma B-type natriuretic peptide (BNP) level ultimately increased to 7,795 pg/mL, and the patient died of cardiac arrest following ventricular tachycardia. Severe heart failure, a critical condition, and thinning of the LV wall may have contributed to the markedly high plasma BNP level in this case.
We describe a 30-year-old man with ectopic adrenocorticotropic hormone (ACTH) syndrome. Before the operation, there was no diurnal variation of ACTH, and ACTH did not respond to CRH or dexamethasone suppression tests. These abnormalities disappeared after the removal of a neuroendocrine tumor in the lung. In addition, plasma ACTH was measureable at as early as postoperative day 3 with ACTH levels increasing thereafter. Furthermore, an insulin tolerance test and inferior petrosal sinus sampling indicated that ACTH was secreted from the pituitary. This case indicates that the hypothalamic-pituitary function can recover within a couple of weeks after curative surgery for ectopic ACTH syndrome.
A 29-year-old woman was diagnosed with Henoch-Schönlein purpura nephritis (HSPN) based on the presence of purpura and histopathological findings showing crescent formation, mesangial proliferation and IgA deposition in the glomerular mesangium. She was treated with high-dose steroids; however, the nephritic syndrome persisted. Therefore, we diagnosed her with steroid-resistant HSPN and decided to add treatment with cyclosphamide pulse therapy. After one year of treatment, the histopathological findings, including crescent formation and IgA deposition, improved, as confirmed on a renal biopsy, and the patient fulfilled the criteria for complete remission. Cyclophosphamide pulse therapy may be considered an effective treatment for intractable HSPN.
We report a case involving a 43-year-old Japanese woman with steroid-resistant focal segmental glomerular sclerosis (FSGS) and severe renal dysfunction, which was ameliorated by low-density lipoprotein apheresis (LDL-A). She had been treated with steroid therapy, but had experienced anuria for over 10 weeks and required hemodialysis. She was then treated with LDL-A, which resulted in improved urinary protein excretion and renal function. Her renal function recovered after 97 days of hemodialysis therapy. This case suggests that LDL-A may represent an effective rescue treatment in patients with FSGS and long-term anuria.
A 45-year-old woman under home parenteral nutrition was admitted with recurrent fever and a worsening renal function. A diagnosis of central venous catheter-related blood stream infection (CVCRBSI) was made according to the identification of coagulase-negative Staphylococcus from both the peripheral blood and the removed CV catheter, along with an improvement of the symptoms following the removal of the catheter. Renal impairment with hypocomplementemia was thought to be secondary to the immune complex formation and deposition in the kidneys in response to prolonged bacteremia. This was confirmed by the pathological findings. Clinicians should therefore be aware that glomerulonephritis may be induced by long-term CVCRBSI.
Rapidly progressive interstitial lung disease (ILD) is associated with dermatomyositis (DM) and has a high mortality rate even with immunosuppressive agents. For such cases, there is no evidence on the combined effect of direct hemoperfusion with a Polymyxin B immobilized fiber column and intravenous immunoglobulin. We herein report a case of 61-year-old woman who presented with respiratory failure. She showed ILD associated with DM which did not improve with immunosuppressive agents, but was improved with the addition of both direct hemoperfusion with a Polymyxin B immobilized fiber column and intravenous immunoglobulin.
We report a case of empyema due to Gemella morbillorum. In this case, an analysis of the aspirate from the pleural effusion revealed empyema and evidence of a Gram-positive coccal bacteria. A biochemical identification system labelled the bacteria as 'unclassified', although we initially suspected the bacterium to belong to the Streptococcus species. 16S ribosomal RNA (16S rRNA) gene sequencing and a phylogenetic tree analysis of the isolated strain confirmed the presence of Gemella morbillorum. To ascertain the true incidence of Gemella species in empyema, 16S rRNA gene sequencing should be used when the standard conventional biochemical methods fail to identify the organism or it identifies it with a low degree of reliability.
Spontaneous regression of methotrexate-related lymphoproliferative disorders (MTX-LPDs) occurs in some patients after withdrawal of MTX. However, the mechanisms by which MTX withdrawal contributes to the spontaneous regression of MTX-LPDs have not been fully elucidated. We herein show that spontaneous regression of MTX-LPDs is associated with the development of significant and transient T-cell large granular lymphocyte (T-LGL) lymphocytosis induced by MTX withdrawal. Since T-LGLs show strong cytotoxicity, their expansion may contribute to the spontaneous regression of lymphoma. Therefore, the development of T-LGL lymphocytosis maybe associated with a favorable prognosis in MTX-LPD patients.
Myelodysplastic syndrome (MDS) was diagnosed in a 64-year-old man. Three months later, he presented with right-sided visual loss. A diagnosis of optic neuritis caused by both ischemic and non-ischemic changes was established. Concurrently, prominent eosinophilia was seen in both the peripheral blood and bone marrow. A partial improvement of visual loss was obtained concomitant with a rapid decrease of the eosinophils after treatment with corticosteroids. Optic neuritis related to MDS is a rare condition and its etiology has not yet been identified. We herein report a case of optic neuritis associated with MDS and accompanied by an eosinophilic crisis.
Idiopathic hypoparathyroidism (IHP) is accompanied by cognitive impairment. We report the case of a 70-year-old IHP patient with cognitive disturbance. Brain computed tomography showed bilateral calcification in basal ganglia, thalamus, and cerebellum. Neuropsychological assessment revealed low scores for intelligence, memory, and perseverative errors. Brain positron emission tomography showed a significant reduction in [18F]-Fludeoxyglucose (FDG) uptake in bilateral frontal, left temporal and parietal cortices, along with a marked reduction in [11C]-flumazenil binding in left frontal, temporal, parietal, and bilateral cerebellum. These findings suggest cognitive impairment in IHP may be ascribed to GABAergic dysfunction, thus leading to, or coexisting with, cerebral hypometabolism.
Myeloid sarcoma is a rare hematological disorder that presents as an extramedullary mass of immature myeloid precursors. We herein present the case of a 57-year-old man with a seven-month history of progressive weakness in the right upper extremity. Reconstruction magnetic resonance neurography showed a marked enlargement of the right brachial plexus. Fluorodeoxyglucose positron emission tomography revealed a radioactive lesion in the sacrum, in addition to the right brachial plexus, and a biopsy of the sacrum revealed myeloid sarcoma. The brachial plexus lesion was also regarded as myeloid sarcoma because of the treatment response. Isolated myeloid sarcoma involving the brachial plexus is very rare and its diagnosis is difficult as there was neither a history of leukemia nor bone marrow involvement in this patient. In this case, reconstructed magnetic resonance neurography was useful for detecting the brachial plexus mass lesion which led to an early diagnosis and good recovery.
We report a case of neuropsychiatric systemic lupus erythematosus successfully treated with mycophenolate mofetil (MMF). The patient was a 40-year-old female who maintained with 7 mg of prednisolone plus 100 mg of azathioprine (AZ) per day. According to transient ischemic attack that occurred repeatedly and an elevated level of interleukin-6 (IL-6) in spinal fluid, she was diagnosed as having neuropsychiatric systemic lupus erythematosus (NPSLE). Initial increase in doses of prednisolone and AZ to 20 mg and 150 mg per day, respectively, was ineffective. After switching from AZ to MMF, her symptoms of NPSLE completely resolved with marked improvement of the IL-6 level in her spinal fluid, suggesting that MMF was effective.
This is the first report of the efficacy of leukocytapheresis (LCAP) in a patient with refractory adult onset Still's disease (AOSD) during pregnancy. A 32-year-old Chinese pregnant woman with AOSD who had been treated with prednisolone failed to achieve disease stabilization. The patient's disease was successfully controlled with the initiation of LCAP. Subsequently, she gave birth via Caesarean section to a 1,878 g baby boy at 34 weeks of gestation while maintaining remission. We conclude that LCAP is an alternative treatment in pregnant patients with refractory AOSD, particularly in those concerned about potential teratogenic and other adverse effects.
Multifocal fibrosclerosis is the term used to represent a combination of similar fibrous lesions occurring at different anatomical sites. We herein report a hypertrophic pachymeningitis patient with a soft tissue mass around the thoracic vertebral bodies. A histopathological analysis of the biopsied tissues from both lesions showed dense fibrosis and a marked infiltration of lymphocytes and plasma cells, which lead to the diagnosis of multifocal fibrosclerosis. This pathological condition closely resembles that of IgG4-related disease and is a very rare combination of manifestations. Our case suggests that hypertrophic pachymeningitis patients need to also undergo a whole body examination.