Epidemiological and clinical features of idiopathic pulmonary alveolar proteinosis in Japan

Abstract:   Idiopathic pulmonary alveolar proteinosis (IPAP) is a rare disease characterized by excessive amounts of lipoproteinaceous material in the alveolus. This report presents an interim analysis of nationwide epidemiological data from Japanese patients with pulmonary alveolar proteinosis, and the roles of serum markers for IPAP. (i) The nationwide demographic data from 166 Japanese patients with IPAP are shown. The female to male ratio was 1:2, and the average age was 51 ± 14 years old (age range: 15–79 years) at registration or diagnosis. A total of 30% of patients with IPAP have a poor clinical course. In total, 30% of patients were treated with whole lung lavage therapy (WLL). Under WLL, the patients significantly improved in the short term, but 40% of the patients who underwent WLL worsened again. A new strategy such as granulocyte-macrophage colony-stimulating factor (GM-CSF) therapy for intractable PAP is required. (ii) The correlation of serum KL-6, carcinoembryonic antigen, surfactant proteins D and A, and LDH with disease severity suggests their potential as disease markers. In contrast, serum anti-GM-CSF antibody did not correlate with disease severity, but is a specific marker for the diagnosis of IPAP. The combined measurements of the serum markers may well prove very useful for both the diagnosis and the management of IPAP patients.     

Posterior Auricular Artery-Middle Cerebral Artery Bypass: A Rare Superficial Temporal Artery Variant with Well-developed Posterior Auricular Artery—Case Report

The posterior auricular artery (PAA) is one of the branches of the external carotid artery, but is usually too small for use as a donor artery for middle cerebral artery (MCA) territory revascularization. An extremely unusual case of PAA-MCA anastomosis was performed in a patient requiring MCA territory revascularization because the superficial temporal artery (STA) parietal branch was absent and the PAA was large enough. A 65-year-old man developed mild motor weakness in the right extremities caused by multiple small infarctions. Single photon emission computed tomography (CT) revealed deterioration of the vascular reserve capacity in the left MCA area. Cerebral angiography showed severe stenosis in the C2 portion of the left internal carotid artery, absence of the parietal branch of the left STA, and a well-developed PAA extending to the parietal area. The patient underwent STA (frontal branch)-MCA and PAA-MCA double anastomosis, and has suffered no stroke or transient ischemic attack. The STA with no bifurcation is known as a rare variation. The PAA also occurs with size variations but well-developed PAA is thought to be extremely rare. PAA can be used as a donor artery for MCA territory revascularization if the vessel size is suitable. Preoperative evaluation of the anatomy is mandatory for harvesting the arteries.     

The non-verbal communication skills of nursing students: Analysis of interpersonal behavior using videotaped recordings in a 5-minute interaction with a simulated patient

Aim: The aim of this paper was to study the non‐verbal communication skills of nursing students and to make a quantitative comparison of the communication techniques of nursing students and experienced nurses to provide guidance for education. Methods: Twenty‐six students (the student group) and 13 nurses (the nurse group) played the role of a nurse. The participants interacted with two simulated patients for 5 min each. The simulated patient was in a sitting position and the nurse started interaction from a starting point that was 3 m away from the patient. Videotaped recordings of these scenarios were recorded and analysed every second using record paper. The analysis items were the distance between the patient and the nurse, posture and position, utterance time, the direction of a nursing person’s face to the patient, facial expression, head nodding, gestures, and the self‐contact behavior of a nurse during interaction with a patient. Results: As 90% of the student group interacted in the standing position, significantly fewer students than the nurse group had their gaze level at the same level or lower than the client. The duration of upper limb and hand gestures was significantly shorter in the student group than for the nurse group. Conclusions: The finding that nursing students showed fewer non‐verbal communication behaviors than nurses suggests there is a pressing need for the development of an educational program in nursing science training to improve non‐verbal communications skills.     

The production ratios of AICDε51 and Aβ42 by intramembrane proteolysis of βAPP do not always change in parallel

Background: During intramembrane proteolysis of β‐amyloid protein precursor (βAPP) by presenilin (PS)/γ‐secretase, ε‐cleavages at the membrane‐cytoplasmic border precede γ‐cleavages at the middle of the transmembrane domain. Generation ratios of Aβ42, a critical molecule for Alzheimer's disease (AD) pathogenesis, and the major Aβ40 species might be associated with ε48 and ε49 cleavages, respectively. Medicines to downregulate Aβ42 production have been investigated by many pharmaceutical companies. Therefore, the ε‐cleavages, rather than the γ‐cleavage, might be more effective upstream targets for decreasing the relative generation of Aβ42. Thus, one might evaluate compounds by analyzing the generation ratio of the βAPP intracellular domain (AICD) species (ε‐cleavage‐derived), instead of that of Aβ42. Methods: Cell‐free γ‐secretase assays were carried out to observe de novo AICD production. Immunoprecipitation/MALDI‐TOF MS analysis was carried out to detect the N‐termini of AICD species. Aβ and AICD species were measured by ELISA and immunoblotting techniques. Results: Effects on the ε‐cleavage by AD‐associated pathological mutations around the ε‐cleavage sites (i.e., βAPP V642I, L648P and K649N) were analyzed. The V642I and L648P mutations caused an increase in the relative ratio of ε48 cleavage, as expected from previous reports. Cells expressing the K649N mutant, however, underwent a major ε‐cleavage at the ε51 site. These results suggest that ε51, as well as ε48 cleavage, is associated with Aβ42 production. Only AICDε51, though, and not Aβ42 production, dramatically changed with modifications to the cell‐free assay conditions. Interestingly, the increase in the relative ratio of the ε51 cleavage by the K649N mutation was not cancelled by these changes. Conclusion: Our current data show that the generation ratio of AICDε51 and Aβ42 do not always change in parallel. Thus, to identify compounds that decrease the relative ratio of Aβ42 generation, measurement of the relative level of Aβ42‐related AICD species (i.e., AICDε48 and AICDε51) might not be useful. Further studies to reveal how the ε‐cleavage precision is decided are necessary before it will be possible to develop drugs targeting ε‐cleavage as a means for decreasing Aβ42 production.     

Clinical Study of Cyclocytidine in Children With Acute Leukemia

One hundred children with various types of leukemia were given113 treatments with cyclocytidine. Eighty-four of these patientshad been treated previously. Cyclocytidine was daily given intravenouslyfor four to 11 consecutive days in amounts of 20mg/kg or 500mg/m².The treatment course . was repeated, when necessary. Complete remission was obtained in one case of monocytic leukemiaamong six treated by cyclocytidine alone. Combined therapy withsteroid hormone gave 14 complete and eight partial remissionsin 38 cases, the remission rate standing at 58%. There were30 complete and 16 partial remissions in 69 cases treated withcombination therapy with two or more agents, and the remissionrate was 67%. Transient pains in the region of the parotid gland were themost characteristic toxicity. GI tract symptoms were less commonand milder than those of cytosine arabinoside. Dizziness wasnoticed in three cases. Nine children with acute leukemia who were given cyclocytidineorally showed no remarkable response. Cyclocytidine, given parenterally, seems beneficial for varioustypes of leukemia in children.     

A SEQUENTIAL METHOD APPLIED TO THE EVALUATION OF PHARMACOTHERAPY ON NEUROTICS

I. A double-blind controlled trial of a minor tranquilizer (chlordiazepoxide) and an antidepressant (trimipramine) was conducted in 71 neurotic subjects and the results subjected to analysis by the cross-over sequential method. II. An original rating scale was designed and the general factor scores and 9 group factor scores were obtained by Bi-factor solution. No significant difference was seen between the two drugs except for a preference of chlordiazepoxide in physical complaints and trimipramine in depersonalization.     

Hepatic angiomyolipoma pre-operatively diagnosed by imaging

An asymptomatic patient with pre-operatively diagnosed solitary angiomyolipoma is reported. The tumour was a well defined, fat density mass on computerized tomography (CT) and magnetic resonance imaging (MRI), and a hyperechoic nodule with a sharp margin on ultrasonography (US). The lesion was hypervascular on arteriography and CT during hepatic angiography. Chemical shift MRI confirmed the fat component within the lesion. Although a review of the literature shows this tumour to be rare and difficult to differentiate from a malignant lesion pre-operatively, the pre-operative diagnosis of angiomyolipoma is considered feasible when the characteristic findings as described here are present.     

INCREASE IN OXYTOCIN SECRETION AT EJACULATION IN MALE

In this study changes in plasma oxytocin levels were determined before and after ejaculation in patients with abnormal spermatogenesis. Plasma oxytocin levels rose significantly after ejaculation (P<0·001, n= 17) There was no obvious correlation between plasma oxytocin levels and sperm count, sperm motility or the number of abnormal forms. Oxytocin may play a role in the precipitation of ejaculation in males.