Short cardiac iodine-123-metaiodobenzylguanidine imaging protocol in heart failure.

BACKGROUND: For assessment of cardiac sympathetic nervous activity, the conventional protocol for iodine-123-metaiodobenzylguanidine ((123)I-MIBG) imaging requires several hours. METHODS AND RESULTS: To establish whether it is possible to shorten the conventional (123)I-MIBG imaging protocol, anterior planar imaging was performed in 42 heart failure (HF) patients at 5, 15 and 180 min. The washout rate of (123)I-MIBG from 5 to 15 min (WR5-15 min) was calculated as a novel index. WR5-15 min closely correlated with the conventional washout rate and inversely correlated with the heart to mediastinum ratio. Univariate Cox analysis revealed that rapid WR5-15 min, augmented plasma B-type natriuretic peptide level, and decreased left ventricular ejection fraction (LVEF) were predictors for cardiac events. Multivariate analysis showed WR5-15 min and LVEF were independent predictors. The cardiac event rate was markedly higher (73%) in patients when both WR5-15 min and LVEF were abnormal. CONCLUSIONS: WR5-15 min obtained from anterior planar imaging is useful for evaluating the severity of HF and clinical outcome, and may shorten the cardiac (123)I-MIBG scintigraphy protocol.     

MEFV mutation analysis of familial Mediterranean fever in Japan

BACKGROUND: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent attacks of fever with serosal inflammation. FMF gene (MEFV) mutations have been identified primarily in patients from Mediterranean populations. Although several clinical cases have been reported in Japan, there have been few reports to date on mutation analysis. We studied FMF patients and their relatives to examine the clinical and genetic features of this disease in the Japanese population. METHODS: Twelve Japanese FMF patients who met the Tel Hashomer criteria and a total of 17 relatives from 5 of 10 families underwent molecular genetic studies to detect MEFV mutations. The characteristics of these Japanese FMF patients and geno-phenotypical correlations were examined. RESULTS: Almost all of our patients had been suffering for a long time from fever of unknown origin and one patient also had systemic amyloidosis. In our 12 FMF patients, we detected the substitutions E84K, L110P, E148Q, R761H and M694I. We also newly diagnosed 2 relatives as having FMF based on clinical symptoms and the existence of FMF mutations. One patient was homozygous for E148Q, the patient with systemic amyloidosis was a homozygote for M694I and 4 patients from 3 families were compound heterozygotes for E148Q and M694I. Three patients in one family were compound heterozygotes for E148Q, L110P and M694I. There were 3 patients who were heterozygous for E84K, L110P-E148Q or M694I and had no other nucleotide changes in the exons of MEFV. On the other hand, 2 relatives who had never experienced symptoms of FMF were homozygous for L110P-E148Q as well as compound heterozygous for E148Q/E148Q-R761H. E148Q and M694I were the most frequently detected substitutions in our study. CONCLUSIONS: MEFV mutations occur in Japanese FMF patients though FMF is rare in Japan. The identification of MEFV mutations could be a reliable diagnostic test for FMF. The results of genetic analyses on 14 Japanese FMF patients in this study revealed that E148Q and M694I are frequent alleles.     

Defining Core Issues in Utilizing Information Technology to Improve Access: Evaluation and Research Agenda

The Department of Veterans Affairs (VA) has been at the vanguard of information technology (IT) and use of comprehensive electronic health records. Despite the widespread use of health IT in the VA, there are still a variety of key questions that need to be answered in order to maximize the utility of IT to improve patient access to quality services. This paper summarizes the potential of IT to enhance healthcare access, key gaps in current evidence linking IT and access, and methodologic challenges for related research. We also highlight four key issues to be addressed when implementing and evaluating the impact of IT interventions on improving access to quality care: 1) Understanding broader needs/perceptions of the Veteran population and their caregivers regarding use of IT to access healthcare services and related information. 2) Understanding individual provider/clinician needs/perceptions regarding use of IT for patient access to healthcare. 3) System/Organizational issues within the VA and other organizations related to the use of IT to improve access. 4) IT integration and information flow with non-VA entities. While the VA is used as an example, the issues are salient for healthcare systems that are beginning to take advantage of IT solutions.     

Prevalence of and risk factors for low bone mineral density in Japanese female patients with systemic lupus erythematosus

To examine the prevalence of and risk factors for low bone mineral density (BMD) (osteoporosis or osteopenia) in Japanese female patients with systemic lupus erythematosus (SLE). We performed BMD measurements by dual X-ray absorptiometry at the lumbar spine and the hip and collected basic and lifestyle-related, clinical and treatment characteristics among 58 SLE patients. Odds ratios (ORs) and their 95% confidence intervals (CIs) were assessed for associations between low BMD and selected factors among SLE patients. The mean BMD?±?SD was 0.90?±?0.17?g/cm² at the lumbar spine and 0.76?±?0.17?g/cm² at the hip. The prevalence of osteopenia (2.5 SD?<?T score?<?1 SD) was 50.0% and that of osteoporosis (T score?<?2.5 SD) was 13.8% in our SLE patients. After adjustment for age and disease duration, we found the number of deliveries (OR?=?5.58, 95% CI?=?1.31?C26.06; P?=?0.02) to be a risk factor for overall low BMD (T score?<?1 SD) and a maximal dosage of >50?mg/day of oral corticosteroids (OR?=?0.25, 95% CI?=?0.07?C0.91; P?=?0.035) as a preventive factor for low BMD at the lumbar spine. Reduced BMD, especially in spinal trabecular bone, was pronounced in Japanese female patients with SLE, particular in those with a history of delivery. A history of high-dose oral corticosteroids was associated with the preservation of BMD at the lumbar spine, however, further study is needed considering the limited sample size.     

The role of the pathologist in diagnosing and grading biliary diseases

Pathological features of primary biliary cirrhosis (PBC) are reviewed. Immune-mediated, non-suppurative cholangitis is the initial lesion and is followed by the gradual and extensive destruction of bile ducts and development of chronic cholestasis. Simultaneously, necro-inflammatory activities of the hepatic parenchyma and limiting plates of milder form develop not infrequently. Eventually, liver fibrosis and cirrhosis develop. A new system applicable to needle liver biopsies in which staging is evaluated using a combination of three factors (fibrosis, cholestasis, and bile duct loss) and necro-inflammatory activities of the bile duct and hepatic parenchyma are graded, is proposed. The clinical and therapeutic evaluation of PBC using this system is warranted.     

Factors affecting short-term mortality in very low birth weight infants in Japan

No epidemiological surveys have examined risk factors related to the death of very low birth weight infants (VLBWIs) in Japan. The objectives of this study were to examine the death rate and fatalities related to complications among VLBWIs, and to analyze factors possibly determining the death of VLBWIs. The subjects of this study were 811 VLBWIs admitted to the Neonatal Care Center of Niigata City General Hospital between April 1987 and March 2003. We obtained information on gender, birth weight, gestational age, Apgar scores, single/multiple pregnancy, postnatal transfer, mode of delivery, complications and outcome (alive or deceased) at the time of discharge from medical records. Of the 811 infants, 98 died prior to discharge (12.1%). Logistic regression analysis showed that independent risk factors for death of VLBWIs were male gender (relative risk [RR]: 2.0), low birth weight (RR: 0.56), necrotizing enterocolitis (RR: 58.0), pulmonary hypoplasia (RR: 37.8), chromosomal abnormalities (RR: 36.3), congenital heart diseases (RR: 9.8), persistent fetal circulation (RR: 9.6), neonatal asphyxia (RR: 6.3) and sepsis (RR: 4.4). The risk for death rises 1.8-fold if birth weight decreases by 100 g. A very high risk of perinatal death is associated with necrotizing enterocolitis, pulmonary hypoplasia or chromosomal abnormalities. The risk of death due to congenital heart diseases or neonatal asphyxia is relatively lower, but the incidences of these two disorders are high (8% and 6%, respectively). From the viewpoint of prophylactic treatment aimed at reducing the death rate of VLBWIs, measures to increase birth weight are of primary importance. Furthermore, early treatment and improved perinatal management of congenital heart diseases and neonatal asphyxia are anticipated to reduce the overall death rate of VLBWIs.     

Multiple small pulmonary emboli associated with transient antiphospholipid syndrome in human Parvovirus B19 infection

Antiphospholipid antibodies (aPL) have been reported to occur in several conditions other than antiphospholipid syndrome, including infections. We herein report the case of a 21-year-old Japanese woman with Parvovirus B19 infection, who developed multiple pulmonary emboli associated with aPL, a lupus anticoagulant and IgM anticardiolipin antibody. Eight weeks later, antiphospholipid antibodies spontaneously disappeared and normal pulmonary flow was observed. Considering the high prevalence of Parvovirus B19 infection, we should be aware of thrombosis associated with transient aPL antibodies in this infectious disease.     

Ascending to descending aorta bypass for middle aortic syndrome.

Though stenoses of the descending aorta and its branches are seen with congenital anomalies or systemic inflammation, occlusion of the descending aorta is extremely rare. A patient with an occluded hypoplastic descending thoracic aorta required re-operation because of graft failure between the descending thoracic aorta and the infrarenal abdominal aorta. The etiology of the aortic occlusion in this case is unknown, but inflammation, such as Takayasu disease, is speculated.     

Repeated relapses of acute myelogenous leukemia in the isolated extramedullary sites following allogeneic bone marrow transplantations

Isolated extramedullary (EM) relapses of acute myelogenous leukemia (AML) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) have been reported to be rare, and are usually followed by bone marrow relapses. We report a 49-year-old man with AML with the unfavorable chromosome abnormality 7q-, who was treated by allo-HSCT. Fifteen months after allo-HSCT, the patient initially developed a relapse only in his inguinal lymph nodes, and then bone marrow relapse became evident one month after the EM relapse. Subsequently, the patient received chemotherapy and a second allo-HSCT from another donor, but he suffered another relapse in different EM sites including the skin and central nervous system with a persistently normal marrow. This case is characterized by repeated relapses in isolated EM sites after allo-HSCT and suggests that the anti-leukemic effects of chemotherapy and/or graft-versus-leukemia effects in the EM sites might not be so uniformly effective as that in the marrow. Accordingly, we should be aware that AML relapses can occur repeatedly only in isolated EM sites post allo-HSCT, resulting in treatment failure and a poor prognosis.     

The validity of sentinel lymph node biopsy using dye technique alone in patients with gastric cancer

BACKGROUND/AIMS: We investigated whether sentinel lymph node biopsy using dye technique alone is useful or not in decision-making for less invasive surgery in patients with gastric cancer. METHODOLOGY: The subjects were 43 patients who had undergone laparotomy for gastric cancer and consented to undergo sentinel lymph node biopsy using patent blue dye. The patients enrolled were 26 males and 17 females, with a mean age of 62.5 years. The tumor sites were upper third of the stomach in 14, middle third in 16, and lower third in 13 patients. The depth of invasion was mucosa in eight, submucosa in 19, muscularis propria in five, subserosa in five, and serosa in six patients. Total gastrectomy was performed in 12, subtotal gastrectomy in 28, and proximal gastrectomy in three patients. RESULTS: The mean number of sentinel lymph node biopsies per surgery was 3.5 +/- 4.1. We were able to perform blue node biopsy in 40 out of 43 patients, but could not find any blue nodes in three patients. Among the 40 patients in whom blue nodes were identified, 29 patients with no metastasis in blue nodes had no evidence of lymph node metastasis (NO). The depth of invasion was not deeper than subserosa in all these patients. Metastasis was observed in one out of the three patients in whom no blue nodes were found. CONCLUSIONS: When the depth of invasion was not deeper than the subserosa and blue nodes were identified, no metastases in either non-blue nodes or blue nodes could be found in the absence of metastatic blue nodes. Therefore, if the depth of invasion is not deeper than the subserosa in gastric cancer, metastatic search in blue nodes seems sufficient and less invasive surgery can be performed safely. Even when the invasion depth is not deeper than the submucosa, the tumor could be metastatic to Group 2 lymph nodes in patients in whom blue node biopsy revealed metastases. When metastasis is found in lymph nodes by intraoperative frozen section diagnosis, less invasive surgery for gastric cancer is not indicated.