Although the pathogenesis of autoimmune pancreatitis is unclear, recent evidence of clinical aspects are presented: (i) mild abdominal symptoms, usually without acute attacks of pancreatitis; (ii) occasional existence of obstructive jaundice; (iii) increased levels of serum gammaglobulin, IgG or IgG4; (iv) presence of autoantibodies; (v) diffuse enlargement of the pancreas; (vi) irregularly narrowing of the pancreatic duct (sclerosing pancreatitis) with often intra-pancreatic biliary stenosis or coexistence of biliary lesions (sclerosing cholangitis similar to primary sclerosing cholangitis: PSC) on endoscopic retrograde cholangiopancreatographic (ERCP) images; (vii) fibrotic changes with lymphocyte and IgG4-positive plasmacyte infiltration, and obliterative thrombo-phlebitis; (iix) occasional association with other systemic lesions such as sialoadenitis, retroperitoneal fibrosis, interstitial renal tubular disorders, and (ix) effective steroid therapy. In addition to pancreatic and extra-pancreatic lesions, diabetes mellitus is occasionally responsive to steroid therapy. Further studies are needed to clarify the pathogenesis.
Brugada syndrome is characterized by ST-segment elevation in the right precordial leads (V1-V3) and an episode of ventricular fibrillation (VF) in the absence of structural heart disease. A number of reports from the world have unveiled the clinical, electrocardiographic, electrophysiologic and prognostic features of Brugada syndrome, and two recent consensus reports have suggested the diagnostic criteria of Brugada syndrome and the risk stratification for the identification of high risk Brugada patients for sudden cardiac death. SCN5A, the gene encoding the α subunit of the sodium channel, is the only gene thus far linked to Brugada syndrome; its prognostic value remains unclear. On the other hand, advances in the understanding of the cellular mechanism for Brugada phenotype derived from experimental studies have suggested possibilities for the development of strategies for managing and treating patients with Brugada syndrome. In this review, the recent understanding and knowledge of Brugada syndrome will be updated.
Objective Carotid intima-media thickness (IMT) is a useful surrogate marker of cardiovascular disease. In addition to low-density lipoprotein cholesterol (LDL-C), metabolic syndrome has been linked to the pathogenesis of atherosclerosis. The present study investigated whether the clustering of multiple components of metabolic syndrome has a greater impact on vascular parameters than individual components of metabolic syndrome, and assessed the association between carotid IMT and LDL-C and metabolic syndrome. Methods Carotid IMT was evaluated on B-mode ultrasonography in 760 patients (340 men aged 64±16 years and 420 women aged 69±13 years) in the Medical Department of Seiyo Municipal Nomura Hospital. The subjects did not demonstrate any clinical signs of cardiovascular disease. We investigated the association between carotid IMT and confounding risk factors including LDL-C and metabolic syndrome using the 2005 Japanese criteria. Results Carotid IMT increased with increasing numbers of metabolic syndrome components (p for trend <0.001). Multiple regression models, including age, sex, body mass index, smoking status, LDL-C, diabetes mellitus as well as each individual component of metabolic syndrome as continuous variables, showed that both metabolic syndrome (β=0.100; p=0.029) and LDL-C (β=0.210, p<0.001) were independent determinants of carotid IMT. Metabolic syndrome amplified the LDL-C associated increases in carotid atherosclerosis. Conclusions Even after taking into account each individual component of MS, the clustering of visceral obesity with at least 2 of the 3 components, and LDL-C are independently associated with increased carotid IMT. This suggests that the components of metabolic syndrome interact to synergistically impact vascular thickness.
Blood pressure (BP) measured at home early in the morning (HBP) has been recognized as a useful predictor for organ damage and has been viewed as an important therapeutic target in patients with hypertension. The present study was aimed to determine whether this notion holds true in patients with progressive renal disease. The study enrolled patients with mild to moderate renal impairment. They were all directed to record self-measured HBP to evaluate the adequacy of BP control. In addition to the conventional antihypertensive therapy, intensive treatment to more efficiently reduce elevated morning HBP was applied, especially in patients with diabetic nephropathy. The results were as follows: 1) The status of BP control assessed using HBP and office/clinic BP (OBP) shows predominance of morning hypertension. The prevalence of patients with well-controlled systolic HBP was 38%, those with poorly-controlled HBP 30%, masked hypertension 20% and white coat hypertension 12%. 2)Early morning systolic HBP in diabetics was significantly higher than that in non-diabetics. However, when evaluated on systolic OBP, both groups were comparable. 3)Logistic regression analysis showed that the predictive variables to explain morning hypertension (more than 130 mmHg and increased systolic HBP) were age, amount of daily urinary protein excretion and left ventricular mass index (LVMI). 4)Following conventional therapy, intensive antihypertensive therapy consisting of calcium channel blockers (CCB) and/or diuretics given in the morning, and angiotensin receptor blockers (ARB) given in the evening, together with α1-blockers given at bedtime, efficaciously reduced elevated HBP in the morning. This result was associated with significant reduction in daily urinary protein excretion and in serum plasminogen-activator inhibitor (PAI-1) concentration. The present study indicates that, regardless of ongoing conventional antihypertensive therapy, the majority of patients with renal disease had morning hypertension, suggesting that these patients are at a higher risk for cardiovascular disease. For the purpose of improving morning hypertension, intensive treatments with combined CCB, ARB and α1-blockers could have substantial benefit on the morbidity and prognosis in patients with diabetic nephropathy.
Objective In order to find some appropriate medicine to suppress myotonia without decreasing muscle strength experiments were performed on myotonic (mto) mice whose Cl channel does not develop due to stop codon and serves as an animal model of myotonia. In myotonic dystrophy dehydroepiandrosterone is low in the serum and it has been reported that intravenous injections of DHEAS to human cases improves myotonia and activities of daily living. Materials and Methods Three pairs of heterozygote mto mice, SWR/J-Clcn1adr-mto/+ and ten Wistar rats were used. We performed intracellular recordings of myotonia from mto mice and the drug effects on insertion myotonia were recorded from the hemidiaphragm preparations of mto mice with different concentrations of DHEAS. Isometric twitch tension was recorded from rat hemidiaphragm preparations in Tyrode’s solution and the effect of DHEAS on the muscle twitch tension was measured at different concentrations of DHEAS from 100 mg/l to 300 mg/l. The effect of mexiletine on ITT was also measured. Results In mto mice insertion myotonia was recorded as soon as the microeletrode was inserted in the muscle cells. When DHEAS was added to Tyrode’s solution, insertion myotonia was suppressed. DHEAS decreased ITT up to 70% of the original value, though mexiletine decreased ITT to 30% of the original value. Therefore, the decrement of the muscle strength in DHEAS solution is much smaller than that of mexiletine. Conclusion Since myotonic dystrophy shows progressive muscle weakness in addition to myotonia, medications like DHEAS are more favorable than the typical Na channel blocker.
Objective The clinical trial, Japan Statin Treatment Against Recurrent Stroke (J-STARS), is being carried out to investigate the efficacy of statin treatment against recurrent stroke. To participate in J-STARS, patients must have a past history of ischemic stroke excluding cardioembolic events, and must be clinically diagnosed with hyperlipidemia (HL). Before starting J-STARS, we needed to be aware of the clinical characteristics of the patients who were eligible to participate in this study. Methods Between 1999 and 2002, 7,149 patients with ischemic stroke were enrolled in a stroke data bank developed by the Japan Standard Stroke Registry Study Group. From this, we acquired the data on 1,487 patients with first-ever atherothrombotic infarction (ATI) or lacunar infarction (LI) with a satisfactory functional outcome on discharge. Results Patients with HL were significantly younger (65.3±11.0 vs 68.4±10.9, p<0.0001) and showed a higher frequency of concomitant hypertension (70.9% vs 61.0%, p=0.0002), diabetes mellitus (42.2% vs 25.7%, p<0.0001) or both (31.7% vs 16.4%, p<0.0001) compared to those without HL. The ratio of ATI to LI and the frequency of prior ischemic heart disease (IHD) did not differ between the 2 groups. Among 467 patients with HL, 52.7% did not receive treatment on admission. Conclusion ATI or LI patients with HL had an earlier age of onset and higher frequency of other lifestyle-related diseases, and this probably includes many with metabolic syndrome, whereas the frequency of IHD was not different between these 2 groups.
Objective To examine whether Coxiella burnetii (C. burnetii) is involved in chronic fatigue syndrome (CFS), we administered tetracycline antibiotics to subjects with CFS, and followed changes in clinical symptoms, PCR findings, and C. burnetii antibody titers. Patients and Methods The subjects were 8 patients with CFS and 213 with nonspecific complaints such as chronic fatigue and low-grade fever for several months or longer but not meeting the diagnostic criteria for CFS. All were examined for C. burnetii infection by nested PCR and the indirect immunofluorescence test (IF). Results Four CFS patients (the CFS group) and 54 controls [the post-Q fever fatigue syndrome (QFS) group] positive for C. burnetii were treated mainly with minocycline or doxycycline (100 mg/day) for 3 months. After treatment, all 58 patients tested negative for C. burnetii infection. In the CFS group, no significant difference was noted between the mean pre- and post-treatment temperatures or headache scores. Similarly, there was no significant improvement in performance status (PS) scores. In the QFS group, however, mean temperatures and headache scores were significantly decreased after treatment (p<0.001). PS scores were also improved. Conclusion These results suggest the possibility of direct involvement of C. burnetii in the pathological state of CFS to be low, despite the C. burnetii infection rate being high in CFS patients. This is a pilot study and further larger investigations are necessary to confirm our preliminary results.
The case of a 64-year-old man with Wolff-Parkinson-White syndrome and permanent atrial fibrillation (AF) is reported. The patient was admitted due to electrocardiographic feature of AF with rapid conduction over the left-sided accessory pathway. Administration of pirmenol effectively suppressed the ventricular response via an accessory pathway. A transesophageal echocardiography detected an uncertain thrombus in the left atrial appendage. During the 33-month follow-up period, the ventricular response via an accessory pathway was progressively facilitated. Radiofrequency catheter ablation using a transseptal approach was performed during AF, resulting in complete elimination of the antegrade accessory pathway conduction.
A 22-year-old Japanese man was found to have bilateral hilar lymphadenopathy (BHL), and was diagnosed with sarcoidosis in 1995. He was followed without treatment until 2002, when a bone fracture due to osseous sarcoidosis was found in his left thumb. Despite systemic treatment with corticosteroid and methotrexate, a new bone lesion developed in his right foot and his right middle finger was fractured. The patient also suffered multiple organ involvements including brain and muscle lesions. This is the first report of a sarcoidosis patient who presented with BHL, and developed bone fractures after a long stable period of more than 5 years.
We report two cases in whom inhaled corticosteroid rapidly improved pulmonary sarcoidosis. In the first case, fluticasone at 400 μg/day was initiated, because dry cough and small nodular shadows on chest X-ray persisted for six months. But her cough and the nodular shadows were persisted, therefore the treatment was replaced with budesonide at 800 μg/day. Two months later, her dry cough subsided and pulmonary shadows improved. Serum angiotensin-converting enzyme (ACE) level was decreased and pulmonary function improved. In the second case, bumethasone was already administered at a local clinic. Budesonide at 400 μg/day was combined with oral steroid, because pulmonary shadows continued for eight years. Also two months later, the serum ACE level was decreased and the pulmonary shadows slightly improved. Inhaled corticosteroid therapy for two to three months is tolerable, and may be a useful treatment option in some patients with sarcoidosis.
Two cases of hemiballism-hemichorea have been reported in woman patients with hyperglycemia; this was a feature of striatal hyperintensity on the T1-weighted MRI. In the first case, strict management of diabetes and treatment with pimozide effectively suppressed the movement disorder. The Z-score Imaging System revealed hyperperfusion in the bilateral dentate nuclei, left striatum, and bilateral motor cortices. In the second case, painful hemiballism-hemichorea limb, followed by the upper limb. The severity of HB-HC corresponded to the expansion of the striatal lesion. The mechanism of HB-HC by using statistical cerebral blood flow evaluation has also been discussed.
Nasogastric tube syndrome named by Sofferman et al in 1981 is a laryngeal complication presenting with life-threatening vocal cord abductor paralysis derived from peroforation of the NG tube-induced esophageal ulcer. As compared with the previously reported cases of this syndrome, our 4 autopsied patients were so peculiar in the following two points that vocal cord abductor paralysis developed repeatedly and no esophageal ulcer was present in spite of the presence of the laryngeal abductor muscle injury. We hypothesized that the etiology of such a variant form was circulatory injury of the laryngeal abductor muscle which was caused by the compression of the postcricoid blood vessels perfusing this muscle. Nasogastric tube syndrome, which is treatable by decannulation, cannot be ruled out even if no esophageal ulcer is detected by fiberoptic laryngoscopy.
We report a 51-year-old Japanese man with chronic alcoholism who complained of mental confusion following respiratory and intestinal infections. The splenium of the corpus callosum showed hyperintensity on both diffusion-weighted MR images and fluid-attenuated inversion recovery images and hypointensity on T1-weighted images. These findings were resolved on MR images obtained 3 days later. He showed complete neurological recovery within 2 months. We suspected that he had mild encephalopathy with a reversible splenial lesion after systemic viral or bacterial infection.
We report three cases showing body lateropulsion as the sole or predominant symptom of caudal lateral medullary infarction. All of them presented a small infarction on the lateral surface of the caudal medulla corresponding to the dorsal spinocerebellar tract (DSCT). Disturbed unconscious proprioception of the lower trunk and the lower limb conveyed by the DSCT might have been responsible for the isolated lateropulsion. Although lateropulsion itself improved within two weeks, one patient’s condition progressed to typical lateral medullary infarction. Lateropulsion caused by DSCT infarction could be a prodromal symptom of perfusion failure of a vertebral artery or the posterior inferior cerebellar artery.
We report two cases of systemic lupus erythematosus (SLE) complicated with colonic ulcerations. One patient was successfully cured by steroid therapy, while the other did not respond to steroid but oral mesalazine was effective. Systemic lupus erythematosus is frequently accompanied by gastrointestinal symptoms, but colonic lesions are quite rare, and the regular treatment is not fixed yet. The high-dose steroidal regimen may be effective for microvasculitis, although it may increase the risk of perforated ulcer of the intestinal tract, which is a life-threatening complication. Further analysis of its outcomes, and establishment of the regular guideline for its treatment are expected.
The association of clubbing with miscellaneous diseases and its diagnostic implications are such that its detection should prompt consideration of the underlying etiology. We encountered a 48-year-old woman with clubbed fingers and a cauliflower-like gingival swelling on the hard palate of the upper jaw. There were no conventionally well-known causes for clubbing. Histological examination of gum biopsy specimen revealed a diagnosis of inflammatory gingival hyperplasia. As an etiology of clubbed fingers, gingivitis was suggested, since clubbing was regressed in parallel with remission of the gingivitis after the treatment by extraction of anterior teeth. Possible involvement of an autoimmune process in the pathogenesis was also considered, because of concomitant elevation of serum anti-double strand DNA antibodies. We recommend examination of the oral cavity for search of an inflammatory disease in cases with clubbed fingers, particularly when other common causes are not apparent.
Primary Sjögren’s syndrome is an autoimmune disease characterized by lymphocytic infiltration of the salivary glands and lacrimal glands. The histological features of chronic inflammation in primary Sjögren’s syndrome may be associated with B cell hyper-reactivity. This syndrome also has various manifestations associated with other exocrine glands and nonglandular tissues. The hematological abnormalities usually seen in Sjögren’s syndrome are lymphopenia, leucopenia, and thrombocytopenia. Although the direct Coomb’s test is often positive, the occurrence of autoimmune hemolytic anemia (AIHA) is rare. Here, we report an elderly patient with primary Sjögren’s syndrome who developed AIHA during the clinical course.
We report a case of sepsis caused by Escherichia coli (E. coli) of serotype O-143. A 78-year-old man developed symptoms of gastroenteritis after ingesting raw meat on noodles. He rapidly developed respiratory failure. Blood culture grew E. coli. The sepsis seemed to have directly spread from a food-borne infection. The development of primary sepsis after ingesting E. coli is very rare. We suspect that bacterial translocation played a major role. Serotype O-143 is recognized in enteroinvasive E. coli (EIEC) as well as in Shigella dysenteriae. The pathogen in the present case is suspected of being EIEC although the isolated E. coli strain was negative for the inv and ipa genes.