Emphysematous pyelonephritis and cystitis concurrence; A case report

We report a case of emphysematous pyelonephritis coexisting with emphysematous cystitis. A 57-year-old woman seen for abdominal pain, diarrhea, and high fever had been referred after computed tomography (CT) elsewhere had shown an air density mass in the left kidney and pelvis. Abdominal CT on admission showed emphysematous change in the left renal parenchyma and intramural bladder. Serum analysis results showed disseminated intravascular coagulation (DIC) and uncontrolled diabetes. Klebsiella pneumoniae was isolated in the blood. She was diagnosed with sepsis based on these findings due to concurrent emphysematous pyelonephritis and cystitis caused by K. pneumoniae. She was treated conservatively with meropenem, intravenous immunoglobulin, and gabexate mesilate and cured. Concurrent emphysematous cystitis and pyelonephritis is rare., with ours only the fourth case reported in Japan.     

Campylobacter fetus as cause of prosthetic valve endocarditis

A 65-year-old woman who had previously undergone aortic root replacement with a bioprosthetic valve (Bentall operation) in treatment of annuloaortic ectasia became feverish after developing dental caries and was admitted to our hospital. Transesophageal echocardiography showed an 18 × 4-mm vegetation on her prosthetic valve. Campylobacter fetus was isolated on blood cultures, and she was diagnosed with infectious endocarditis. Aggressive combined antibiotic treatment was effective for her recovery. C. fetus infection is a rarely reported cause of prosthetic valve endocarditis.     

Rescue of severe infantile hypophosphatasia mice by AAV-mediated sustained expression of soluble alkaline phosphatase

Hypophosphatasia (HPP) is an inherited disease caused by a deficiency of tissue-nonspecific alkaline phosphatase (TNALP). The major symptom of human HPP is hypomineralization, rickets, or osteomalacia, although the clinical severity is highly variable. The phenotypes of TNALP knockout (Akp2(-/-)) mice mimic those of the severe infantile form of HPP. Akp2(-/-) mice appear normal at birth, but they develop growth failure, epileptic seizures, and hypomineralization and die by 20 days of age. Previously, we have shown that the phenotype of Akp2(-/-) mice can be prevented by enzyme replacement of bone-targeted TNALP in which deca-aspartates are linked to the C-terminus of soluble TNALP (TNALP-D10). In the present study, we evaluated the therapeutic effects of adeno-associated virus serotype 8 (AAV8) vectors that express various forms of TNALP, including TNALP-D10, soluble TNALP tagged with the Flag epitopes (TNALP-F), and native glycosylphosphatidylinositol-anchored TNALP (TNALP-N). A single intravenous injection of 5×10(10) vector genomes of AAV8-TNALP-D10 into Akp2(-/-) mice at day 1 resulted in prolonged survival and phenotypic correction. When AAV8-TNALP-F was injected into neonatal Akp2(-/-) mice, they also survived without epileptic seizures. Interestingly, survival effects were observed in some animals treated with AAV8-TNALP-N. All surviving Akp2(-/-) mice showed a healthy appearance and a normal activity with mature bone mineralization on X-rays. These results suggest that sustained alkaline phosphatase activity in plasma is essential and sufficient for the rescue of Akp2(-/-) mice. AAV8-mediated systemic gene therapy appears to be an effective treatment for the infantile form of human HPP.     

Brain activation during the perception of stressful word stimuli concerning interpersonal relationships in anorexia nervosa patients with high degrees of alexithymia in an fMRI paradigm

Several studies have reported that anorexia nervosa (AN) patients have high levels of alexithymia. However, relatively little is known about the underlying neurobiological relationships between alexithymia and AN. We used functional magnetic resonance imaging to examine the brain responses in 30 AN patients and 20 healthy women during the processing of negative words concerning interpersonal relationships. We investigated the relationship between alexithymia levels and brain activation in AN. AN patients showed significant activation of the orbitofrontal cortex, dorsolateral prefrontal cortex and medial prefrontal cortex while processing negative words concerning interpersonal relationships, as compared to the processing of neutral words. Moreover, the subjective rating of unpleasantness with negative words and neural activities in the amygdala, posterior cingulate cortex (PCC) and anterior cingulate cortex (ACC) negatively correlated with the level of alexithymia in AN. Our neuroimaging results suggest that AN patients tend to cognitively process negative words concerning interpersonal relationships, resulting in activation of the prefrontal cortex. Lower activation of the amygdala, PCC and ACC in response to these words may contribute to the impairments of emotional processing that are hallmarks of alexithymia. Functional abnormalities associated with alexithymia may be involved in the emotional processing impairments in AN patients.     

SNCA polymorphisms, smoking, and sporadic Parkinson's disease in Japanese

Several case-control studies and genome-wide association studies have examined the relationships between single nucleotide polymorphisms (SNPs) in the SNCA gene and Parkinson's disease (PD), and have provided inconsistent results. We investigated the relationships between SNPs rs356229, rs356219, rs356220, rs7684318, and rs2736990 and the risk of sporadic PD in Japan using data from a multicenter hospital-based case-control study. Included were 229 cases within 6 years of onset of PD as defined according to the UK PD Society Brain Bank clinical diagnostic criteria. Controls were 357 inpatients and outpatients without neurodegenerative disease. Adjustment was made for sex, age, region of residence, and smoking. Based on the recessive model, compared with subjects with the CC or CT genotype of SNP rs356220, those with the TT genotype had a significantly increased risk of sporadic PD: the adjusted OR was 1.42 (95% CI: 1.002-2.02). In the additive model, SNP rs2736990 was significantly related to the risk of sporadic PD: the adjusted OR was 1.30 (95% CI: 1.002-1.68). There were no significant relationships between SNP rs356229, rs356219, or rs7684318 and the risk of sporadic PD in any genetic model. The additive interactions between SNPs rs356219 and rs356220 and smoking with respect to sporadic PD were significant although the multiplicative interactions were not significant. This study suggests that SNCA SNPs rs356220 and rs2736990 are significantly associated with the risk of sporadic PD in Japanese. We also present new evidence for biological interactions between SNPs rs356219 and rs356220 and smoking that affect sporadic PD.     

Correlation of biological aggressiveness assessed by <Superscript>11</Superscript>C-methionine PET and hypoxic burden assessed by <Superscript>18</Superscript>F-fluoromisonidazole PET in newly diagnosed glioblastoma

Purpose  

Glioblastoma multiforme (GBM) is characterized by tissue hypoxia associated with resistance to radiotherapy and chemotherapy. To clarify the biological link between hypoxia and tumour-induced neovascularization and tumour aggressiveness, we analysed detailed volumetric and spatial information of viable hypoxic tissue assessed by ¹⁸F-fluoromisonidazole (FMISO) PET relative to neovascularization in Gd-enhanced MRI and tumour aggressiveness by L-methyl-¹¹C-methionine (MET) PET in newly diagnosed GBMs.     

Infective endocarditis with destruction of the mitral valve annulus and the left atrial posterior wall after cancer chemotherapy

The case is reported of a 48-year-old man who underwent distal pancreatectomy, splenectomy, and 4 cycles of postoperative chemotherapy for stage I pancreatic cancer, at the age of 47. Four days after completion of chemotherapy, the patient developed high-grade fever (40 degrees C). In addition, on day 10, he developed disturbance of consciousness, followed by symptoms of shock, which led to emergency hospitalization. On day 4 of admission, the patient was diagnosed as having infective endocarditis and severe mitral valve insufficiency, and was started on antibiotic and heart failure therapy. Surgery was performed on day 16 of admission; the mitral valve infection had spread to the valve cusp and ring, and thence to the posterior wall of the left atrium. After excision of the lesions, the patient underwent mitral valve replacement with a collared prosthesis valve and a translocation procedure. The postoperative course was uneventful, and there has been no recurrence of inflammation or paraprosthetic leakage over the year and 6 months since the operation.     

Validity of predictive equations for basal metabolic rate in Japanese adults

Many predictive equations for basal metabolic rate (BMR) based on anthropometric measurements, age, and sex have been developed, mainly for healthy Caucasians. However, it has been reported that many of these equations, used widely, overestimate BMR not only for Asians, but also for Caucasians. The present study examined the accuracy of several predictive equations for BMR in Japanese subjects. In 365 healthy Japanese male and female subjects, aged 18 to 79 y, BMR was measured in the post-absorptive state using a mask and Douglas bag. Six predictive equations were examined. Total error was used as an index of the accuracy of each equation's prediction. Predicted BMR values by Dietary Reference Intakes for Japanese (Japan-DRI), Adjusted Dietary Reference Intakes for Japanese (Adjusted-DRI), and Ganpule equations were not significantly different from the measured BMR in either sex. On the other hand, Harris-Benedict, Schofield, and Food and Agriculture Organization of the United Nations/World Health Organization/United Nations University equations were significantly higher than the measured BMR in both sexes. The prediction error by Japan-DRI, Adjusted-DRI, and Harris-Benedict equations was significantly correlated with body weight in both sexes. Total error using the Ganpule equation was low in both males and females (125 and 99 kcal/d, respectively). In addition, total error using the Adjusted-DRI equation was low in females (95 kcal/d). Thus, the Ganpule equation was the most accurate in predicting BMR in our healthy Japanese subjects, because the difference between the predicted and measured BMR was relatively small, and body weight had no effect on the prediction error.