[Primary Sjögren's syndrome with pulmonary hypertension].

A 50-year-old woman was admitted to our hospital because of dyspnea on exertion. A chest X-ray film showed cardiomegaly and echocardiography revealed right-heart overload. Right heart catheterization studies revealed pulmonary hypertension and no signs of pulmonary embolism or vasculitis.

Chronic neutrophilic leukemia associated with chronic lymphocytic leukemia.

We report on an 83-year-old male with chronic neutrophilic leukemia (CNL) associated initially with IgM monoclonal gammopathy and later with B cell chronic lymphocytic leukemia (CLL), in which the clone differed from that of the preceding monoclonal gammopathy. At initial presentation, the patient had hepatosplenomegaly, leukocytosis (29100 x 10(6)/l) with an increase of mature neutrophils (83%), 20q- chromosomal abnormality, an increased leukocyte alkaline phosphatase score, elevated serum levels of vitamin B12 and uric acid, a low serum level of granulocyte colony-stimulating factor, and high serum IgM (1015 mg/dl: lambda type M protein). Thereafter, lymphocytosis developed gradually.

A loss of functional spinal alpha motor neurons in amyotrophic lateral sclerosis.

We obtained motor unit number estimates (MUNEs) of the hypothenar and the extensor digitorum brevis muscles in ALS patients by our new technique. One year after symptom onset, the MUNEs had decreased to +/-30% of normal. Accordingly, we suggest that 70% of functional spinal alpha motor neurons are lost in the first post-onset year in ALS.

Clinical features of paradiverticulitis.

Colonic diverticula have been generally accepted as a source of massive hemorrhaging. Little is known, however, about fecal occult blood loss from colonic diverticula and diverticulosis. We retrospectively investigated the possibility of minor bleeding in 737 diverticula cases diagnosed from April 1989 to May 1994.

MRI findings of multiple malignant gliomas: differentiation from multiple metastatic brain tumors.

Multiple malignant gliomas are relatively uncommon, but are sometimes difficult to differentiate from multiple metastatic brain tumors. We analyzed the MR findings of four cases of multiple gliomas, comparing them with 12 cases of multiple metastatic brain tumors. All tumors were pathologically proven by surgical operation or autopsy.

[Efficacy and safety of a single oral dose of pilsicainide in supraventricular arrhythmia after coronary artery bypass grafting].

We evaluated the efficacy and safety of administration of a single oral dose of Pilsicainide, a class Ic antiarrhythmic agent, in 34 patients (26 men, 8 women, age from 48 to 81, mean age 66 +/- 8 years) who developed supraventricular arrhythmia after coronary artery bypass grafting (CABG). A total of 42 episodes of postoperative supraventricular arrhythmia, with the majority occurring 2-4 days after CABG, were classified as follows: paroxysmal atrial fibrillation, 34; paroxysmal atrial flutter, 6; and sinus tachycardia, 2. Sinus rhythm was restored in 32 episodes (78%) by treatment with oral pilsicainide given in a single dose of 50 ro 100 mg.

Analysis of K-ras oncogene mutation directly applied to atypical cell clusters on cytologic smear slides of bile and pancreatic juice.

To develop an objective reference for the cytological evaluation of atypical cells in bile and pancreatic juice, we analyzed K-ras oncogene mutation in atypical cell clusters, which were collected directly from cytological smear slides; 50 samples (cell clusters) from 31 smear slides of 21 patients with carcinomas of the pancreatic head region, and nine samples from eight cases of benign disease. These cell clusters (5-1000 cells/cluster) were selectively suspended in buffer containing proteinase K, and subjected to DNA extraction. K-ras codon 12 mutation was determined by polymerase chain reaction amplification, followed by digestion with BstNI.

Schwann cell proliferation as the cause of peripheral neuropathy in neurofibromatosis-2.

We report a 18-year-old man who developed slowly progressing right drop foot. Bilateral acoustic neurinoma was found and he was diagnosed as neurofibromatosis-2. Right sural nerve biopsy showed multiple small onion-bulb-like structures in the nerve fascicle.

Glomerular thromboxane contributes to pressor response in deoxycorticosterone acetate-salt hypertension.

To assess the role of renal thromboxane in a salt sensitive pressor response in hypertension, urinary excretion of thromboxane and its release from isolated glomeruli and renal papillae were examined in deoxycorticosterone acetate treated rats with normal (0.6%, n = 12) and high (4%, n = 12) salt diets for 8 weeks. Mean blood pressure, measured directly by an implanted aortic catheter, was higher in the high salt diet group than in the normal salt diet group (146 +/- 2 vs 119 +/- 2 mmHg, P<0.

Differential expression of connective tissue growth factor gene in cutaneous fibrohistiocytic and vascular tumors.

Connective tissue growth factor (CTGF) is a member of a family of immediate early gene products that may play an important role during tissue regeneration, wound repair and skin fibrosis. In this study, CTGF gene expression in mesenchymal tumors was investigated by in situ hybridization and CD34 antigen expression was studied by means of immunohistochemical staining. CTGF mRNA was expressed in fibroblasts of all nine dermatofibromas examined, but five of seven dermatofibrosarcoma protuberans (DFSP) or two cases of malignant fibrous histiocytoma were negative for its expression.

[Clinical evaluation of heavy-particle radiotherapy using dose volume histogram (DVH)].

Radiotherapy with heavy particles such as proton and heavy-charged particles is a promising modality for treatment of localized malignant tumors because of the good dose distribution. A dose calculation and radiotherapy planning system which is essential for this kind of treatment has been developed in recent years. It has the capability to compute the dose volume histogram (DVH) which contains dose-volume information for the target volume and other interesting volumes.

Distribution of referred pain from the lumbar zygapophyseal joints and dorsal rami.

Objective: The purpose of this study was to determine the distribution of referred pain from the lumbar zygapophyseal joints (L1/2 to L5/S1) and the medial branches of the lumbar dorsal rami (Th12 to L5) in a large number of patients with chronic low back pain.

Setting: This study was conducted at the pain clinics of Kanto Teishin Hospital and Hannan Central Hospital from March 1994 to May 1996.

Patients And Design: Chronic low back pain patients who underwent zygapophyseal joint injection or radiofrequency facet denervation were studied.

Regulation of red blood cell filterability by Ca2+ influx and cAMP-mediated signaling pathways.

To investigate the mechanism of the regulation of human red blood cell deformability, we examined the deformability under mechanical stress. Washed human red blood cells were rapidly injected through a fine needle, and their filterability was measured using a nickel mesh filter. The decrease in filterability showed a V-shaped curve depending on the extracellular Ca2+ concentration; the maximum decrease was achieved at approximately 50 muM.

10-year survival rate in 92 patients with invasive bladder carcinoma treated by bladder sparing methods.

The 10-year survival rate was evaluated in 92 patients with invasive bladder cancer (stages T 2 to 4) who were treated between 1966 and 1985 using bladder-sparing methods such as intravesical instillation of antineoplastic agents, transurethral resection, partial cystectomy, arterial infusion chemotherapy, radiation and embolization of the internal iliac artery. The overall 5-year and 10-year survival rates were 68.1% and 53.

Cystic pulmonary metastases of endometrial stromal sarcoma of the uterus, mimicking lymphangiomyomatosis: a case report with immunohistochemistry of HMB45.

A case of endometrial stromal sarcoma (ESS) showed cystic pulmonary metastases mimicking lymphangiomyomatosis (LAM). A 58-year-old female, who had undergone total hysterectomy for low-grade ESS 16 years previously, had repeated bouts of pneumothorax. Multiple thin-walled cysts in the peripheral lung were revealed by radiological examinations.

Clonality in nevocellular nevus and melanoma: an expression-based clonality analysis at the X-linked genes by polymerase chain reaction.

The true nature of nevocellular nevus is still unknown and it has been ambiguously classified as a neoplasm or a hamartoma. We studied the clonality of nevocellular nevus and melanoma (malignant melanoma), using an expression-based clonality analysis at the X-linked genes by means of polymerase chain reaction. DNA was extracted from cryostat sections of 20 nevocellular nevi (10 compound and 10 intradermal type) and five melanomas from female patients.

Cyanamide-induced granulocytopenia.

We report a 64-year-old male with granulocytopenia and dermatitis due to cyanamide treatment. We administered cyanamide for alcoholism. After about one month he suffered from scaly erythema over his whole body and granulocytopenia (granulocyte; 140/microliter) with maturation arrest in bone marrow.

Metabolic improvement of poorly controlled noninsulin-dependent diabetes mellitus decreases bone turnover.

Patients with poorly controlled noninsulin dependent diabetes mellitus (NIDDM) are shown to have higher bone mass. However, the influence of changes in glycemic control on bone turnover is not known. To clarify whether metabolic improvement of poorly controlled NIDDM affects bone turnover, markers for glucose, mineral, and bone metabolism were assessed before and after glycemic control for 3 weeks in 78 poorly controlled NIDDM patients with initial hemoglobin A1c over 8%.

Beneficial effect of alpha-blocker on hemorheology in patients with essential hypertension.

To assess the effect of antihypertensive therapy on hemorheology in essential hypertension, blood viscosity and red blood cell deformability were examined in 45 patients with essential hypertension and 20 age-matched normotensive control subjects. Hypertensive patients were randomly assigned to monotherapy with five different antihypertensive drugs for 6 months and change of blood viscosity and red blood cell deformability were reexamined after the end of the monotherapy with each antihypertensive drug. Blood viscosity increased and red blood cell deformability decreased in hypertensive patients compared to normotensive control subjects.

[Shearing injuries of parasagittal white matter, corpus callosum and basal ganglia: possible radiological evidences of hemiplegia in diffuse axonal injury].

The relationship between spastic hemiplegia in diffuse axonal injury (DAI) and neuroradiological findings was studied in 100 cases. These cases were prospectively collected from the files of Automobile Insurance Rating Organization in Japan between 1993 from to 1996. Requirements for entry to this study were as follows: presence of initial unconsciousness after head injury without any lucid interval.

Primary gastric T-cell lymphoma with and without human T-lymphotropic virus type 1.

Background: Gastric T-cell lymphomas are rare, and their incidence and viral status have not yet been fully clarified.

Methods: Sixty-seven cases of surgically resected gastric lymphomas from city hospitals in Tokyo were evaluated. The surface phenotype was determined by immunohistochemistry, gene rearrangement by Southern blot hybridization, association with Epstein-Barr virus (EBV) by EBV-encoded small RNAs in situ hybridization, and the presence of human T-cell lymphotropic virus type 1 (HTLV-1) by serology, Southern blot hybridization, and polymerase chain reaction analysis.

[Recent progress in therapy for chronic myelogenous leukemia].

In chronic myelogenous leukemia (CML), abnormalities develop in hematopoietic stem cells, affecting three hematopoietic cell series, including leukocytes, erythrocytes, and platelets. The occurrence of the Philadelphia (Ph1) chromosome and BCR/ABL fused genes are involved in its pathophysiology. Methods of treating CML consist of bone marrow transplantation, and administration of interferon (IFN) and other antineoplastic drugs.

Influence of thrombopoietin on platelet activation in myeloproliferative disorders.

Although thrombopoietin itself does not influence platelet aggregation, it enhances platelet activation in response to certain agonists. We evaluated the effects of thrombopoietin on platelet activation using platelet-rich plasma from 16 patients with myeloproliferative disorders (MPD group) and 16 healthy volunteers (control group). Preincubation with thrombopoietin significantly enhanced platelet aggregation stimulated by ADP, collagen, or epinephrine in the MPD group as well as the control group.

[A case of spinal hemorrhage associated with abdominal aortic coarctation].

We report a 59-year-old male with abdominal aortic coarctation presented as paraplegia due to spinal hemorrhage caused by the rupture of abnormally dilated spinal artery. In coarctation of aorta, coarctation is usually located in the aortic isthmus which could be the cause of cervical and upper thoracic myelopathy. However, there has been no report of abdominal aortic coarctation with hemorrhagic transverse myelopathy.