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Galcanezumab, a CGRP monoclonal antibody drug, has been approved by the U.S. Food and Drug Administration (FDA) for the prevention of recurrent cluster headaches. This was done after a randomized, double-blind, placebo-controlled trial found it to be effective and safe. Similarly sphenopalatine ganglion stimulation has been found to be effective and safe in a randomized, controlled trial as an acute treatment for chronic cluster headache. This article reviews the mechanisms of action of these therapies and their clinical trial results, clinical uses, and prospects in Japan.

(Background) The standard treatment for recurrent immunoglobulin A nephropathy (rIgAN) after kidney transplantation (KTx) has not been established. (Methods) The results of treatment consisting of tonsillectomy and steroid pulse therapy in 20 recipients who were diagnosed as rIgAN were retrospectively analyzed. (Results) The level of proteinuria significantly decreased from 0.84±0.81 g/day to 0.27±0.31 g/day after treatment (P=0.007). Microscopic hematuria disappeared or improved in 58.3% and 66.6% of recipients 6 and 12 months after treatment, respectively. Serum creatinine levels remained stable for 5 years by the treatment, except for 3 cases of graft loss. Sixteen recipients received renal graft biopsies before and after treatment. Mesangial IgA deposition were dramatically decreased in 7 recipients (43.75%). The degree of mesangial hypercellularity, endocapillary hypercellularity, and crescents formation improved in 3 (18.8%), 6 (37.5%), and 4 (25%) recipients after treatment. (Conclusion) Steroid pulse therapy combined with tonsillectomy may be clinically and histopathologically effective treatment for rIgAN after KTx.

A 61-year-old man was referred to the urology department in our hospital with gross hematuria and hydro nephrosis. Cystoscopy revealed a smooth mass lesion in his bladder, and a transurethral biopsy was performed. Signet ring cell carcinoma was found in the submucosa. Upper gastrointestinal endoscopy revealed an ulcerated lesion in his gastric body. Biopsy specimens obtained from the ulcerated lesion showed signet ring cell carcinoma. No other primary lesions were detected using colonoscopy, gallium scintigraphy, or computed tomography of the chest. He was ultimately diagnosed with advanced gastric cancer with bladder metastasis. He was prescribed combination chemotherapy of cisplatin and tegafur, gimeracil, oteracil potassium, and trastuzumab. After 2 courses of chemotherapy, there was a decrease in the size of both the gastric and bladder lesions. There was also a significant decrease in the tumor marker levels. He is currently alive after 7 courses of chemotherapy.

(Background) Long-term care is necessary for normal growth and development of pediatric recipients of kidney transplants. We report on our experience with pediatric kidney transplantation (KTx) during the past 19 years. (Methods) We retrospectively analyzed the data from 26 recipients who received KTx between 1996 and 2014 at Niigata University Hospital (one patient underwent two consecutive KTx during the designated period). All recipients were 16 years old or younger at the time of KTx. (Results) The graft survival rates at 1, 5, and 10 years after transplantation were 96%, 96%, and 88%, respectively. Three recipients lost the renal graft function due to graft thrombosis, antibody mediated rejection and steroid resistant rejection. Drug non-adherence was associated with rejection episodes, which led to the increasing of estimated glomerular filtration rate (eGFR) level. In addition, renal graft function was related to the growth after KTx. Eighteen recipients graduated from high school during follow-up periods and 17 recipients obtained employment. (Conclusion) Interventions promoting adherence should be implemented among pediatric recipients and parents to optimize graft survival and growth after KTx. Successful KTx contributed the high rate of social participation and employment after pediatric KTx.

(Backgrounds) The efficacy of bortezomib for chronic antibody mediated rejection (CAMR) after kidney transplantation is still obscure. (Materials and methods) CAMR were persisted in 5 recipients who were treated with plasma exchange, low dose of IVIG, steroid pulse therapy, and rituximab. 1.3 mg/m 2 of bortezomib was administered on days 1, 4, 8, 11. Serum creatinine (sCr) levels, anti-HLA antibodies, and histology were analyzed. (Results) Stable sCr levels were obtained in 3 out of 5 recipients. No one lost renal graft function during follow-up periods. Anti-HLA class I antibodies were significantly decreased after bortezomib treatment, however anti-HLA class II antibodies were not changed. Histology showed no improvement at 6 months after bortezomib administration. Two recipients whose sCr levels increased during follow-up had already had interstitial fibrosis and tubular atrophy (IF/TA) in histology before bortezomib treatment. (Conclusions) The use of bortezomib after IF/TA could be detected in histology may not contribute to stabilize renal graft function in CAMR.

Intervention of the osteoporosis liaison service for hemodialysis patients is considered essential, because hemodialysis patients are at higher risk of fracture and fall than individuals not undergoing dialysis. It may help to maintain the activity of hemodialysis patients to practice interprofessional collaboration for consistent prevention of primary fracture, thereby supporting discharge planning. We expect to organize a specialized fracture prevention team for dialysis patients to manage their anti-osteoporosis medication and nutritive support, as well as to evaluate their physical statuses and prevent falls.

Long-term practical on-site training, based on the Model Core Curriculum for Pharmaceutical Education, is a core program of the 6-year course of pharmaceutical education, introduced in Japan in 2010. In particular, medication counseling in practical training in 5th-year provides valuable opportunities for communication with real patients rather than simulated patients (SPs). However, it can also cause anxiety in 4th-year students before practical training. To address such concerns, upperclassmen (5th- and 6th-year students), who have already completed practical training, constructed and conducted a new educational program for medication counseling practice in preclinical training based on their experiences. They also developed case scenarios and played the role of patients themselves to create more realistic clinical settings. Advice from professional SPs was also provided. The 5-step program is composed of 1st counseling, 1st small group discussion (SGD) for improving counseling, 2nd revised counseling based on the 1st SGD, 2nd SGD, and development of a counseling plan and presentation. Educational effects of the program were evaluated by questionnaire survey after preclinical training in 4th-year students and after their practical training in 5th-year students. This new program, the Advanced Medication Counseling Practice, was found to be useful to reduce anxiety about communication with patients among 4th-year students (about 90%). Even after their practical training in 5th-year, they still appreciated usefulness of this program (about 80%). This program is still valued 4 years after its development. We developed the Advanced Medication Counseling Practice in preclinical training for junior students by senior students.

A 76-year-old woman was referred to our hospital with anorexia. Computed tomography revealed a tumor lesion measuring 110mm in the liver at S4/5 with calcification and swelling of a paraaortic lymph node. The gallbladder was not visualized. Histological examination of a biopsy specimen from the liver tumor revealed squamous cell and undifferentiated carcinomas, and several tumor markers were elevated. Therefore, we diagnosed the patient with gallbladder adenosquamous cell carcinoma T3N2M0 stage III. Because the serum parathyroid hormone-related protein (PTHrP) and granulocyte-colony stimulating factor (G-CSF) levels were significantly elevated, we suspected that PTHrP and G-CSF production occurred because of adenosquamous cell carcinoma in the gallbladder. We initiated chemotherapy with S-1.

Proximal femoral fracture in osteoporotic fracture is related to the life prognosis in the most severe. Many also report that has been reduced due to the spread of osteoporosis treatment in the world. In Japan in 1985 for the first time, we investigated all patients with hip fractures who were admitted to all hospitals with an orthopedic department in the Niigata Prefecture. We also have investigated the secular trends in the hip fracture incidence from 1985-2010. Proximal femoral fracture has been increasing in 2010, and there are no reports that have decreased. In Japan, where an aging society is to continue in the future, we need urgent countermeasure.

A 36-year-old female received protocol biopsy at 1 month after living donor kidney transplantation. At 3 months post-transplantation, presence of a growing cystic mass in the kidney graft which had not been detected preoperatively, was demonstrated by ultrasound and computed tomography. The patient had an abdominal pain around the graft. Percutaneous drainage and sclerotherapy with minocyclin were performed twice, but the cystic mass, nevertheless, became enlarged and the abdominal pain recurred again. Laparoscopic fenestration was then performed. Immunohistochemistry of the cystic mass wall showed that it was CD34 (-), EMA (-), Megalin (-), but D2-40 (+). These results suggested that the cystic mass was derived from lymphatic vessels, which developed into lymphocele in the graft. We concluded that lymphatic vessels could have been injured and obstructed by the protocol biopsy. This is the first report of successful laparoscopic fenestration for lymphocele in the kidney graft.

This study examined the clinical profile of cluster headache (CH) in Japan and the potential presence of features peculiar to Japan. Subjects comprised 110 consecutive new CH patients (83 males, 27 females; mean age, 38.1 ± 11.7 years). While 85% of patients had visited other medical institutions, only 19% had received a correct diagnosis. Mean delay in diagnosis was 8.1 years. The common clinical features of the present report and previous Japanese reports were as follows: 1) a low prevalence of chronic CH; 2) a low prevalence of sense of restlessness; 3) an uncoupling of a sense of restlessness and restless behavior; 4) approximately half of all patients experiencing CH attacks ranging in duration from 1-2 hours; 5) the location of CH most commonly reported as retro-orbital; and 6) lacrimation representing the most consistently reported autonomic feature. The common clinical features from 1) to 3) were seen in other East-Asia countries. Both frequencies of locations of CH and autonomic features differed between Caucasian and East-Asian populations. These characteristics may reflect ethnic and/or cultural differences between Eastern and Western CH patients.

Cancer/testis (CT) antigen is a group of antigens that are expressed in a wide variety of malignant tumors but not in normal adult tissues except for testis. Since CT antigens are immunogenic and highly restricted to tumors, they are considered as ideal targets for cancer immunotherapy. Many clinical studies targeting CT antigens have been tested. Here we review the history and the recent progress of clinical studies targeting MAGE family and NY-ESO-1 including our trials.

A 60-year-old male with POEMS syndrome received lenalidomide and high-dose dexamethasone combination therapy as an initial treatment, with no severe adverse events occurring during the treatment. Two cycles of the therapy led to significantly decreased serum VEGF level, and IgA-λ type M-protein was not detected by immunofixation electrophoresis. We next performed autologous stem cell transplantation, without severe complications such as engraftment syndrome. He improved enough to walk independently and now is being followed up without treatment. This case suggests that lenalidomide-dexamethasone therapy is highly effective and can be a good option for pre-transplant treatment for POEMS syndrome.

Miriplatin is a novel lipophilic platinum complex developed to treat hepatocellular carcinoma (HCC). Although HCC patients frequently have coexisting chronic renal failure, there is no reliable data regarding clinical toxicity of miriplatin in HCC patients with chronic renal failure. We retrospectively evaluated the safety and efficacy of transcatheter arterial chemotherapy with miriplatin in 67 HCC patients with chronic renal failure (estimated glomerular filtration rate [GFR] by the Cockcroft-Gault equation <60ml/min). Estimated GFR within 2 months after miriplatin administration did not decrease significantly by the Wilcoxon signed rank test (pretreatment;46ml/min, 1 month;48ml/min;P=0.019, 2 months;45ml/min;P=0.619 [P<0.003 was significant by the Bonferroni correction]). Complete response in terms of tumor necrosis was achieved in 14 of 67 patients and no serious adverse events were observed. These results suggested that transcatheter arterial chemotherapy with miriplatin can be used safely for HCC patients with chronic renal failure.

We report a case of an 80-year-old male who suffered from intractable hepatic encephalopathy and hepatocellular carcinoma( HCC), associated with hepatitis type C-related liver cirrhosis. He was unable to receive HCC treatment due to the deterioration of his liver. His hepatic encephalopathy was resistant to oral administration of laxatives, lactulose, and kanamycin sulfate, etc. His blood ammonia concentration averaged about 130 mg/dL, and often exceeded 200 mg/dL(normal range: <80 mg/dL). Later, an oral administration of vancomycin hydrochloride, 0. 5 g once every 3 days, was initiated. Soon after ward, his blood ammonia concentration declined to the normal range(about 50 mg/dL), and the clinical symptoms of hepatic encephalopathy showed a remarkable improvement. By the continuation of vancomycin administration, the normalization of his state of consciousness was achieved, improving his quality of life, and his activities of daily living. Three months after beginning treatment, he was able to receive transcatheter arterial chemoembolization for the treatment of HCC, because his liver function reserve improved(Child-Pugh score decreased from 10 to 7).

A 48-year-old male with Castleman disease developed symptoms typical of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal-protein and skin changes) but M-protein was not detected by immunoelec-trophoresis. He was diagnosed as having POEMS syndrome based on IgA-λ detected by immunofixation electrophoresis and an increased level (3,170 pg/ml) of vascular endothelial growth factor (VEGF). After diagnosis, the patient underwent autologous peripheral blood stem cell transplantation safely and remained relapse-free and in good condition for 15 months. This case suggests that immunofixation electrophoresis and detection of elevated serum VEGF level are useful methods for earlier diagnosis of POEMS syndrome.

A 52-year-old woman with a chief complaint of epigastric distress was diagnosed as having pancreatic cancer with multiple liver metastases. After insertion of a metallic stent for biliary stenosis, combination therapy of gemcitabine (GEM) and adoptive immune cell therapy (AICT) was initiated. GEM 1,000 mg/m2 was administered on day 1, 8 and 15 every 4 weeks, while AICT using MUC1 peptide-pulsed dendritic cells (DC) and anti-CD3-activated T lymphocytes (CAT) was given biweekly. After 6 courses of GEM and 9 courses of DC-CAT, the patient was considered to have a complete response (CR) on CT and MRI examination. CR has still been maintained by the continuous administration of GEM and CAT. The combination therapy of GEM and AICT was suggested to be effective against advanced pancreatic cancer.

A 78-year-old-man was admitted to our hospital because of renal insufficiency 20 months after the onset of autoimmune pancreatitis. He had cerebral infarction and prostatic hypertrophy as complications. He had been previously diagnosed with autoimmune pancreatitis (AIP). The initial therapy was started with oral prednisolone at the dose of 0.8 mg/kg (40 mg/day). Prednisolone had been tapered off gradually through a one-year period. Four months later from terminating prednisolone, a follow-up CT showed multiple low-density areas in both kidneys without swelling of the pancreas. Furthermore, 4 months later, laboratory findings showed progressive renal insufficiency. On admission, BP was 167/77 mmHg, and the bilateral submaxillary glands were swollen. He did not have pretibial edema. Laboratory findings were as follows. BUN 55.9 mg/dL, Cre 6.17 mg/dL, Amy 65 mg/dL, TP/Alb 9.5/4 g/dL, gamma-gl 43.7%, IgG/IgA/IgM 3,395/112/74 mg/dL, IgG4 1,460 mg/dL, urinary protein 1.38 g/day, and 24 hr-Ccr 11.8 mL/min/1.73 m2. Percutaneous renal needle biopsy was conducted. Light microscopic findings demonstrated tubulointerstitial nephritis (TIN) and membranous change. Immunofluorescent microscopic findings indicated diffuse deposition of IgG2 and IgG4 in the renal interstitium. On the basis of these findings, the condition was diagnosed as IgG4-related tubulointerstitial nephritis. As renal insufficiency was progressing, hemodialysis was started soon after admission and oral prednisolone was also started at the dose of 0.4 mg/kg (20 mg/day). However, improvement of renal function has not been obtained and hemodialysis and prednisolone tapering are still being conducted. This case showed severe tubulointerstitial nephritis requiring hemodialysis after a cure for autoimmune pancreatitis. IgG4-related renal disease rarely needs hemodialysis. This case indicates that the prognosis of IgG4-related systemic disease is not necessarily good and further accumulation of cases is required.

The patient was a 59-year-old male with chronic hepatitis type B. He was diagnosed as having multiple hepatocellular carcinomas (HCCs), most of which showed hypervascular features on contrast-enhanced CT scan. He underwent the implantation of a 5-french catheter by" GDA coil method" for hepatic arterial infusion of chemotherapy. After the implantation, he suffered from high fever with a sharp elevation in transaminase levels. Since his liver function gradually deteriorated, he was not able to receive hepatic arterial infusion of chemotherapy. However, three weeks after catheter implantation, most of the tumors were no longer enhanced on dynamic CT scan, suggesting a loss of tumor vascularity, ie, induction of tumor necrosis. It was speculated that necrosis of the tumors was caused by the reduction of hepatic arterial blood flow due to the catheter placement.

We report a CR case of advanced rectal cancer successfully treated with 39 courses of mFOLFOX6. The patient was a 29-year-old female with Stage IV rectal cancer. At first she was given IFL together with radiotherapy. It took effect for three months, and the therapeutic effect was PR, but interstitial pneumonia developed. Therefore, we shifted to mFOLFOX6, and she was treated with 39 courses. Grade 1 appeared several times for peripheral neuropathy, but recovered immediately. If we could control peripheral neuropathy with FOLFOX, it was thought that long-term survival could / be expected.

We describe a 63-year-old man who, while under treatment as an outpatient for adult onset Still's disease (AOSD), developed edema of the extremities and mediastinal tumor was observed on a chest X-ray film and a chest CT scan. He was not pathologically diagnosed at first and received radiation therapy with a total dose of 30 Gy. Transbronchial biopsy was carried out because the tumor enlarged, and the tumor invasion was observed in the left lumen of the main bronchus. Histological findings suggested a diagnosis of carcinoma with sarcomatoid elements (CSE). Further radiation therapy with a dose of 20 Gy was unsuccessful; his condition gradually worsened and the patient died. The autopsy findings demonstrated that CSE developed in the middle mediastinum, and the other organs were not involved. From an embryologic standpoint, there seemed to be some possible differential diagnoses, such as a pleomorphic carcinoma as a subtype of lung cancer, and CEA as a metastatic mediastinal lymph node cancer of unknown origin or a primary mediastinal lymph node cancer. Like the present case, tumors developed in the middle mediastinum with rapid progression are rare. We report a case, that was difficult to diagnose and treat.

Maffucci's syndrome is a rare congenital, nonhereditary mesodermal dysplasia characterized by soft tissue hemangiomas and multiple enchondromas. A 52 years old man was diagnosised as Maffucci's syndrome in his childhood. He complained of mild paraesthesia which gradually progressed to intolerable pain lasting one to two houres with rhinorrhea (cluster like headache). Magnetic resonance imaging showed a pituitary adenoma which invaded into right cavernous sinus. Serum hormone level was almost normal, therefore we diagnosed nonfunctional pituitary adenoma. The pharmacological preventive treatments for headache, such as loxoprofen, sumatriptan, lomerizine had no effect. On the other hand, verapamil showed moderate improvement of his headache, then the symptons was gradually getting better and finally he felt no pain. This case demonstrated occurence mechanism of cluster headache and preventive effect of verapamil associated with cavernous sinus.

A 63-year-old man was referred to our hospital for rapid deterioration of his renal function. He had worked as a metal founder for more than 40 years, and had been diagnosed as having silicosis. Laboratory data on admission showed severe anemia, thrombocytopenia, and end-stage renal failure (BUN 88.8 mg/dl, serum creatinine 9.0 mg/dl). Myeloperoxidase anti-neutrophil cytoplasmic antibody(MPO-ANCA) was also detected in his sera. On the next day after admission, he complained of sudden dyspnea and hemoptysis. Mechanical ventilation with pure oxygen was insufficient to improve hypoxia without concomitant use of percutaneous cardio-pulmonary support(PCPS) and continuous hemofiltration(CHF). We diagnosed his condition as MPO-ANCA-associated rapidly progressive glomerulonephritis with diffuse alveolar hemorrhage. Treatment with plasmapheresis, pulse methylprednisolone and pulse cyclophosphamide effectively improved his hemoptysis as well as chest X-ray findings and blood gas analysis. However on his later clinical course, he was complicated with superimposed complex infection and passed away. Autopsy findings showed crescentic glomerulonephritis in the kidneys and silica nodules in the lungs. Recently it has been postulated that some relationship exists between ANCA-associated(especially MPO-ANCA-associated) glomerulonephritis and silica exposure. The reported cases of glomerulonephritis in the patients with silica exposure showed a rapidly progressive clinical course and pauci-immune necrotizing crescentic glomerulonephritis in their histology. Gregorini et al. reported that 12 of 37 (32%) male patients with RPGN had either silicosis or significant silica exposure, and 7 of 8 patients examined were ANCA-positive(6 of 7 were MPO-ANCA-positive). Therefore silica seems to cause glomerulonephritis by disrupting the immune response. Including this case mentioned above, we have experienced 10 cases of MPO-ANCA-associated glomerulonephritis, at least 3 cases out of which had suffered from silicosis in the past(30%). These results indicate that silicosis should be considered a relevant pathogen of MPO-ANCA-associated glomerulonephritis beyond the race.

We studied short interval change of cranial computed tomography (CT), magnetic resonance imaging (MRI) and 99mTc-ethyl cysteinate dimer single photon emission computed tomography (99mTc-ECD SPECT) in a case of Wilson's disease. Before treatment, CT scan showed low density changes in the bilateral thalamus and basal ganglia, and MRI demonstrated high intensity in same lesions. 99mTc-ECD SPECT study revealed a hypoperfusion in bilateral thalamus. After 2 months under D-penicillamine therapy, neurological findings had improvement. Hypoperfusion in the thalamus with 99mTc-ECD SPECT significantly improved, whereas abnormal findings of CT scan and MRI persisted. 99mTc-ECD SPECT study may be useful for the planning of the treatment of Wilson's disease.

Myotonic dystrophy is an autosomal dominant heritable disease associated with an expansion of CTG trinucleotide repeat within 3' untranslated region of the DMPK gene. The key question is how the mutation in the 3' untranslated region of the DMPK gene exerts an dominant effect at the cellular level, despite the fact that it does not alter the protein coding region of the gene. Although the mechanism of myotonic dystrophy remains controversial, some evidence suggests that CUG repeats in the DMPK mRNA may have pathological effects. A hypothesis on molecular mechanism of DM pathogenesis, in terms of RNA-protein interactions and regulation of gene expression through the 3' untranslated region of mRNAs, was discussed.

Nitric oxide (NO) is a free radical and was regarded as noxious to life. But recent studies show that NO is an important substance for transcellular signal transduction. It also seems to act as a neurotransmitter in the nervous system. In ischemic nerve tissue a release of glutamate is one of the critical factors that increase neuronal death, and some experiments suggest that NO may be involved in this process. Here we provide evidence that NO provides neuroprotection in ischemic retinas in vivo. Albino rabbits' eyes were subjected to 60 minutes of ischemia by raising intraocular pressure. Before ischemia the eyes were treated intravitreously with the NO-precursor L-arginine, the NO synthase-inhibitor nitro-L-arginine methyl ester hydrochloride (L-NAME), the NO-donor sodium nitroprusside (SNP), or solvent only. The amplitude of the b-wave was measured and the recovery ratio of the b-wave was analyzed hourly after reperfusion. The recovery ratio of b-wave in the eyes with L-arginine and with SNP increased more rapidly than in the controls, while the recovery ratio in the eyes with L-NAME increased in a way similar to that of the controls. These results suggest that NO plays a neuroprotective role in ischemic retina. It may be involved with S-nitrosylation of some proteins, including one of the glutamate receptors, the N-methyl-D-aspertate (NMDA) receptor.

We describe a 69-year-old female with Guillain-Barré syndrome (GBS) whose paresthesia and weakness improved after plasma exchange (PE) and intravenous high-dose immune globulin (i.v.I.G.). She felt a paresthesia in her right arm, and 7 days later she noticed right arm weakness followed by gait disturbance within 2 days. She received a series of 6 PEs, but paresthesia and weakness did not improve. Following the last PE trial of this series, she was treated by i.v.I.G. (0.4 g/kg) for 5 days. After i.v.I.G., paresthesia and weakness improved in a few days. After 2nd series of 6 PEs, she was able to walk. In this clinical course, the treatment of i.v.I.G. after PE seemed to be effective. This case raises the possibility that i.v.I.G. might be the treatment in GBS patients with insufficient effect of PE.

Urinary examination with dip sticks is essential for clinical screening test and primary health care. Recently Japanese Society of Clinical Pathology has stressed the clinical use of dip sticks. Many dip sticks are available in Japan. However, few attempts have been done for external quality control of dip sticks. It is well known that there are considerable differences in the accuracy, sensitivity and stability of dip sticks. Makers are at variance in indicating concentration levels in accordance with the given color charts. Some urine chemistry analyzers for dip sticks have been developed. There was a few cases of simultaneous study on dip sticks from various makers. We, four technologists have attempted external quality control of eleven dip sticks for protein, glucose and occult blood with the same lot number (one was only for glucose) using the four different pooled urine samples and two artificial urine samples. Based on the results of this study, the following proposal to improve the accuracy and reliability of dip sticks was derived. 1) Users should have a plenty of knowledge on the difference of the quality of dip sticks of each makers. 2) Makers of dip sticks should clarify to users the method of examination, standard materials and errors. 3) Users should perform internal quality control using different pooled urine samples or different artificial urine samples on a daily basis before going with the routine work.

Ippan Kensa (defined as urinalysis, fecal tests, and tests of cerebrospinal fluid, pleural fluid, ascites, and other body fluids) are done of fresh samples, as a rule, and such samples should be tested as soon as possible. Even for tests not ordered in an emergency, rapid results are desirable. In these two senses, all tests are somewhat urgent. Along with the rest of the hospital, our section moved into a new hospital complex in 1993, and that time, an order-entry and reporting system was implemented. Urinalysis was totally systematized from the start with two automated analyzers connected to an automated transportation system, and other tests were gradually automated and computerized. Time and labor were saved without decreased quality. Most routine tests are now done within 2.5 hours of sampling. Emergency tests are not handled by a special, formally established procedure in our laboratory, but at the request of a physician, any test can be done rapidly when necessary, because the system allows interruption at any time for testing of samples out of order. Our experience with this system suggests that automation and computerization are essential if emergency and urgent tests are to be done rapidly by a small staff. Further improvements in the high-quality automated analyzers already available, speeding results but allowing fewer false-positives and false-negatives can be expected.

The magnetic resonance (MR) imaging and computed tomography (CT) findings in four patients (five kidneys) with non-Hodgkin's lymphoma involving the kidneys and perirenal spaces are presented. The patterns of disease in each case were as follows: bilateral renal nodules, infiltration in the perirenal space, infiltration in the perirenal space with renal involvement, and direct invasion from contiguous retroperitoneum. On plain CT, the lesions showed slight hyperdensity (three kidneys) and isodensity (two kidneys) as compared with normal renal parenchyma. But all lesions appeared as hypodense masses with more definite margins after contrast enhancement. MR imaging findings showed iso- or slight hypointense masses on T1-weighted images and definite hypointense masses on T2-weighted images as compared with the signal intensity of the renal cortex. Dynamic imaging and conventional delayed T1-weighted imaging following Gd-DTPA injection showed no significant enhancement of the lesions. In comparison with contrast enhanced CT, despite its poorer resolution, T2-weighted MR imaging showed nearly the same accuracy in the evaluation of number and extent of the lesions without contrast medium administration. MR imaging was also useful to evaluate the patency of vessel lumen surrounded by tumor mass and to determine the location and extent of huge lesions by its multiplanar imaging capabilities.

A 51-year-old woman complained that her right side of the face looked blurring and the right margin of all the objects in her visual field looked blurred. Neurological examination on admission showed no abnormalities including higher cortical function and visual fields except the metamorphopsia. In this case, a very localized spotty infarct caused no neurological symptoms other than the metamorphopsia. CT scan and MRI revealed a spotty lesion of infarct between retrosplenium and cingulate gyrus on the left side. This can be a breakthrough case to locate the exact anatomic pathology that causes metamorphopsia.

To explore possible associations of maternal working status with birthweight, a cohort study was conducted from July, 1989 to June, 1991 in 8 general hospitals in the Tokai district. Using a self-administered questionnaire, data on job employment were collected mostly in the first or second trimester. Information on delivery outcome was recorded by doctors, midwives or nurses after child birth. Included in this analysis were 1,360 pregnants who delivered a live singleton without major malformations at gestation of 37 weeks or more. The following major findings emerged. (1) No significant difference in mean birthweight was detected between working and non-working pregnants. (2) Working pregnants were at greater risk of delivering a low birthweight (< 2,500g) baby. (3) Delivery of a low birthweight baby was more frequent in multiparous than nulliparous mothers. (4) The risks of delivering a low birthweight baby significantly differed according to the maternal working status. (5) These results suggest that not only the maternal working factor itself but the working status should be deliberately taken into consideration in order to provide proper prenatal care.

We investigated a Japanese pedigree with oculopharyngeal muscular dystrophy (OPMD) which included the probands of two sisters and a brother. Case 1 (Fig. 1): A woman born in 1940 at Fuji City, Shizuoka prefecture (Pacific coast side, which located about 80 miles west from Tokyo) was insidiously suffering from dysphagia and eye lid ptosis since her age of 40. She was admitted to Shizuoka Red Cross Hospital when 48 years old, because of difficulty of going upstairs. Neurological examination revealed severe eye lid ptosis without eye movement disorders, dysphagia, and moderate weakness on neck and proximal muscles of extremities without definite atrophy and fasciculation. In the laboratory data, serum creatine kinase level was slightly elevated to 215 U/l. Electromyographic findings showed myogenic pattern on the extremities but no evidence of the existence of myasthenia gravis and neurogenic involvement. Intranuclear tubular filaments were found in 3% of muscle fibers from her left biceps muscle. Case 2: The elder sister of the case 1 was suffering from eye lid ptosis without eye movement disorder and severe dysphagia since the age of 44. Case 3: The younger brother of the case 1 was suffering from dysphagia and proximal muscle weakness since the age of 40. We found eye lid ptosis, dysphagia and/or proximal muscle weakness in 24 cases (men: 12 cases, women: 12 cases) out of 50 examined members of this pedigree after their ages of 40 (Fig.3; family tree). It was concluded that this pedigree had cases of oculopharyngeal muscular dystrophy with autosomal dominant inheritance which was quite rare in Japanese.