Severe aplastic anemia in a patient with primary immunodeficiency

This paper reports the case of a 6 year old boy with primary immunodeficiency disease, whose marrow cells showed dyshematopoietic features, that was subsequently transformed to severe aplastic anemia. He was first diagnosed as having congenital immunodeficiency comprising deficiency of immunoglobulin A (IgA), IgG2 and IgG4, depressed mitogen responses, lymphopenia with inverted CD4/CD8 ratio and an increased proportion of T-cell receptor γ/δ-bearing cells. Cytogenetic study of the peripheral blood showed a normal karyotype of 46, XY, but that of the marked hypoplastic marrow demonstrated one cell with monosomy 7 and another with trisomy 8 in the 20 cells examined.     

Conformational study of O-glycosylated threonine containing peptide models

¹H n.m.r. studies of Z-Thr-OMe, Z-Thr-Ala-OMe, Z-Ala-Thr-OMe and their glycosylated derivatives indicate the possibility of an intramolecular hydrogen bond between Thr NαH and the N-acetyl carbonyl of the carbohydrate moiety, 3,4,6-tri-O-acetyl-2-acetamido-2-deoxy-α-d -galactopyranose(AcGalNAc). This is especially true in the case of Z-Thr(AcGalNAc)-Ala-OMe, suggesting that the strength of this hydrogen bond is dependent on the neighboring amino acids on the carbonyl terminal side of Thr. The existence of such a hydrogen bond implies a conformation in which the carbohydrate moiety is restricted to an orientation with its plane roughly perpendicular to the peptide backbone. In such an orientation, steric problems will be minimized in the case of clustered O-glycosidically linked Thr(Ser) residues as found in human erythrocyte glycophorin. A locked orientation of the carbohydrate moiety with respect to the peptide backbone may also play a conformational role in antifreeze glycoproteins.     

Development of a Short Questionnaire to Measure an Extended Set of Job Demands,Job Resources,and Positive Health Outcomes: The New Brief Job Stress Questionnaire

This study aimed to investigate the reliability and construct validity of a new version of the Brief Job Stress Questionnaire (New BJSQ), which measures an extended set of psychosocial factors at work by adding new scales/items to the current version of the BJSQ. Additional scales/items were extensively collected from theoretical job stress models and similar questionnaires in several countries. Scales/items were field-tested and refined through a pilot internet survey. Finally, an 84-item questionnaire (141 items in total when combined with the current BJSQ) was developed. A nationally representative survey was administered to employees in Japan (n=1,633) to examine the reliability and construct validity. Most scales showed acceptable levels of internal consistency and test-retest reliability. Principal component analyses showed that the first factor explained 50% or greater proportion of the variance in most scales. A scale factor analysis and a correlation analysis showed that these scales fit the theoretical expectations. These findings provided a piece of evidence that the New BJSQ scales are reliable and valid. Although more detailed content and construct validity should be examined in future study, the New BJSQ is a useful instrument to evaluate psychosocial work environment and positive mental health outcomes in the current workplace.     

下丘脑腹内侧核损伤大鼠脂肪肝形成机制探讨

目的 :　探讨下丘脑腹内侧核损伤性肥胖大鼠脂肪肝形成机制。方法 :　雌性 SD大鼠分为下丘脑腹内侧核损伤肥胖组 (VMH)和下丘脑腹内侧核非损伤对照组 (sham) ,于下丘脑腹内侧核损伤和伪性损伤 1 w后 ,留取肝脏作形态学观察和测定肝脏甘油三酯、磷脂酰磷酸水解酶、苹果酸酶、葡萄糖 - 6 -磷酸脱氢酶及微粒体甘油三酯转运蛋白活性。结果 :　VMH组大鼠肝脏中甘油三酯的含量明显增高 ,是对照组的 1 .8倍 ,肝脏磷脂酰磷酸水解酶、苹果酸酶、葡萄糖 - 6 -磷酸脱氢酶活性均高于对照组。显微镜下 ,在 VMH肥胖组大鼠肝细胞中可见有大量的脂质颗粒 ,而其对照组肝细胞中并没有见到类似的脂质颗粒。 VMH肥胖组大鼠肝细胞的微粒体甘油三酯转运蛋白活性是对照组的 1 .1 5倍 ,而且肝脏微粒体甘油三酯转运蛋白活性与血浆胰岛素水平呈明显的相关关系 (R=0 .86 3 ,P<0 .0 5 )。结论 :　VMH肥胖大鼠肝脏甘油三酯的产生和微粒体甘油三酯转运蛋白活性均明显增高 ,但后者的增加程度明显低于前者 ,这样可能导致肝脏中产生的甘油三酯不能被微粒体甘油三酯转运蛋白及时从肝脏转运出去 ,而导致甘油三酯在肝脏中积累形成脂肪肝。     

Automatic switching and guidance system to facilitate unassisted uroflowmetry using commercial electronic devices

To enable male patients to undergo uroflowmetry in a private condition without medical supervision, we devised an automatic switching and patient guidance system for the spinning disk uroflowmeter Urodyn 1000, using two commercial electronic devices (an infrared motion sensor tap and a memorizable vacuum fluorescent display). Instead of running the uroflowmeter continuously, which shortens the life of the spinning disk due to mechanical wear, an infrared motion sensor turns on the devices each time a patient enters the room. The patient urinates according to the timely instructions on the visible display and voided urine directly flows into a urinal. The devices are automatically turned off 5 min after the patient leaves the room. With the use of our system, men already acquainted with uroflowmetry could perform self-administered uroflowmetry any time in private. The system was considered useful for improving the quality of patient service.     

Relationship between psychiatric symptoms and regional cerebral blood flow in patients with mild Alzheimer's disease

Background: Behavioral and psychological symptoms of dementia (BPSD) are frequently observed in patients with dementia and often cause serious problems. However, the cause of BPSD has not yet been elucidated. Moreover, the precise evaluation of BPSD in mild dementia has not been studied in any great detail. In the present study, we investigated the relationship between psychiatric symptoms and regional cerebral blood flow (rCBF) in patients with mild Alzheimer's disease (AD). Methods: The present study included 47 patients (20 men and 27 women) who were diagnosed with mild AD. Mean patient age was 72.8 ± 8.2 years. Single photon emission computed tomography (SPECT) with ^99mTc‐ethyl cysteinate dimer (^99mTc‐ECD) was performed in all patients. The SPECT data were analyzed using a three‐dimensional stereotactic region of interest template, which evaluated CBF in 24 segments. Psychiatric symptoms were evaluated in patients using the Brief Psychiatric Rating Scale. Each psychiatric symptom was designated as ‘symptom present’ in cases in which the BPRS item score was more than 3. We compared 10 segments of rCBF in symptom‐present patients with those in symptom‐absent patients. Results: Motor retardation was the most common psychiatric symptom (36.2%), followed by depression (19.1%), anxiety (17.0%), emotional withdrawal (17.0%), and somatic concern (14.9%). Alzheimer's disease patients with motor retardation exhibited a tendency towards lower rCBF in seven segments (left callosomarginal, bilateral parietal, bilateral angular, and bilateral temporal). However, no specific tendency was observed in depression, anxiety, and somatic concern. Conclusions: In the present study, we observed a tendency for decreased brain perfusion in patients with motor retardation. Further studies are necessary to confirm that this trend contributes to the appearance of psychiatric symptoms in patients with mild AD.     

孤立性髂动脉瘤36例的临床诊断和治疗

目的提高对孤立性髂动脉瘤（isolated iliae artery aneurysm,IIAA）的认识,总结临床诊断和治疗经验。方法回顾性分析1983年1月至2006年3月期间收治的36例IIAA的临床资料。无症状患者17例,有症状患者19例。择期手术治疗33例,急诊手术3例。治疗措施主要包括动脉瘤切除和动脉重建术。辅助手术包括输尿管松解术和输尿管切断再缝合。结果择期手术术后发生切口延期愈合1例,臀肌跛行2例,下肢运动和排尿障碍1例,均经药物保守治疗,症状减轻和消失,其余患者均康复良好出院。急诊手术3例于术后死亡。结论IIAA临床罕见,破裂出血死亡率高,早期诊断和手术治疗是降低死亡率的关键。微创血管腔内治疗术是值得推荐应用的方法。     

Reappearance of Cranial Nerve Dysfunction Symptoms Caused by New Artery Compression More than 20 Years after Initially Successful Microvascular Decompression: Report of Two Cases

Reappearance of symptoms of cranial nerve dysfunction is not uncommon after successful microvascular decompression (MVD). The purpose of this study was to report two quite unusual cases of recurrent and newly developed hemifacial spasm (HFS) caused by a new conflicting artery more than 20 years after the first successful surgery. In Case 1, the first MVD was performed for HFS caused by the posterior inferior cerebellar artery (PICA) when the patient was 38 years old. After 26 symptom-free years, HFS recurred on the same side of the face due to compression by the newly developed offending AICA. In Case 2, the patient was first operated on for trigeminal neuralgia by transposition of the AICA at 49 years old, but 20 symptom-free years after the first MVD, a new offending PICA compressed the facial nerve on the same side, causing HFS. These two patients underwent reoperation and gained satisfactory results postoperatively. Reappearance of symptoms related to compression of the root exit zone (REZ) by a new offending artery after such a long symptom-free interval since the first effective MVD is rare. Here, we describe two such unusual cases and discuss how to manage and prevent such reappearance of symptoms after a long time interval.