Churg–strauss syndrome: importance of colonoscopy and endoscopic biopsy in establishing diagnosis

A 31‐year‐old Japanese man was admitted with complaints of left lower abdominal pain and diarrhea. He had a history of bronchial asthma since he was 26 years old. On admission, his eosinophil count was 4100/mm ³ and increased as high as 12 000/mm ³ . A neurological examination disclosed mononeuritis multiplex with paresthesia in both lower and upper extremities. Because of hematochezia, colonoscopy was performed and disclosed multiple colonic ulcers and erosions from the rectum to the ascending colon. Endoscopic biopsy showed fibrinoid necrosis of small‐sized vessels with moderate infiltration of eosinophils in the mucosa and submucosa. He was suspected of being a case with Churg–Strauss syndrome (CSS; allergic granulomatous angiitis), and prednisolone of 30 mg/day was administered. After the administration of prednisolone, his eosinophil count decreased dramatically. His symptoms also improved gradually, except for numbness in the extremities. Three weeks later, colonoscopy revealed improvement of the mucosal appearance, but the colonic ulcers and erosions were still present. Endoscopic biopsy showed exravascular granuloma with moderate infiltration of eosinophils in the submucosa. Colonoscopy and endoscopic biopsy may be important in establishing the diagnosis of CSS with intestinal symptoms.     

Increased Dispersion of Repolarization Time Determined by Monophasic Action Potentials in Two Patients with Familial Idiopathic Ventricular Fibrillation

Increased Dispersion of RT in Familial Idiopathic VF. Introduction: The role of increased dispersion of repolarization in the genesis of torsades de pointes in patients with long QT syndrome has been clarified, but its role in the genesis of idiopathic ventricular fibrillation (VF) is not yet known. To investigate the pathogenesis of VF, we recorded monophasic action potentials (MAPs) from two siblings (48- and 36-year-old males) with familial idiopathic VF. Methods and Results: The elder brother (patient I) showed a late r’ wave in lead V¹ and ST segment elevation in leads V¹ through V³. The younger brother (patient 2) had late r’ waves and ST segment elevation in leads II, III, and aVF, and the configurations were very similar to those of patient I. MAPs were recorded from several sites in the right ventricular (RV) and left ventricular (LV) endocardium during constant right atrial pacing. The repolarization time (RT) was defined as the sum of the activation time (AT) and action potential duration (APD) at 90% repolarization. In patient 1, marked prolongation of the AT (140 msec) and the RT (380 msec) was recorded in the RV septum of the outflow tract, and the RT dispersion was markedly increased (125 msec). In contrast, patient 2 showed prolongation of the AT (80 msec) and RT (310 msec), and fractionated electrograms in the RV floor of the inflow tract. The RT dispersion was also increased (80 msec). VF and nonsustained polymorphic ventricular tachycardia were induced by double premature stimulation in patients 1 and 2, respectively. Chronic amiodarone therapy decreased the RT dispersion and suppressed the induction of ventricular tachyarrhythmias in patient 2, although late r’ waves and slight ST segment elevation were unmasked in leads V₁, and V₂. Conclusion: Our data suggest that the increased dispersion of the RT, which was due mainly to a localized conduction delay in the RV, created an arrhythmogenic substrate in the two patients with familial idiopathic VF.     

Upregulation of Angiotensin II Receptor and Connexin 43 in Increased Suburothelial Myofibroblasts in the Rat Inflammatory Bladder

Objectives: Signaling pathways in suburothelial layer are involved in the bladder sensory response. The expression of angiotensin II type 1 (AT1) receptors and connexin 43 (Cx43) in suburothelial myofibroblasts was investigated in an acute bladder inflammation model. Methods: Adult female Wistar rats underwent urethral catheterization and received 0.2 mL intravesical infusion of 0.4 M HCl to establish acute bladder inflammation model or 0.2 mL of sterile saline as control (n = 10 rats/group). Eight days after treatment, cystometry was performed. Suburothelial myofibroblasts were also collected and subjected to immunohistochemical staining to examine AT1 receptor and Cx43 expression. Results: Eight days after treatment with HCl to induce acute bladder inflammation, the frequency and basal pressure of the bladder was significantly increased compared with those in control rats. The number of suburothelial myofibroblasts was significantly increased in acute bladder inflammation rats, as was the expression of AT1 receptor and Cx43. Conclusion: These results suggest that the increased number of suburothelial myofibroblasts, upregulation of AT1 receptor and Cx43 expression may be associated with the pathogenesis of hyperactivation of bladder sensory signaling pathways in acute inflammatory bladder.     

Characterization of the Gene Encoding the MPB51, One of the Major Secreted Protein Antigens of Mycobacterium bovis BCG, and Identification of the Secreted Protein Closely Related to the Fibronectin Binding 85 Complex

The secreted protein MPB51 is one of the major proteins in the culture filtrate of Mycobacterium bovis BCG (BCG) and is a protein immunologically cross-reacting with the fibronectin binding 85 complex secreted by this bacterium. The gene encoding MPB51 ( mpb51 ) was cloned, sequenced, and expressed in Escherichia coll. The mpb51 gene was mapped downstream of the gene for 85A component with 179 bp spaces. The mpb51 gene encoded 299 amino acids, including 33 amino acids for the signal peptide, followed by 266 amino acids for the mature protein with a molecular mass of 27807.37 Da. This is the first complete sequence of MPB51. MPB51 showed 37–43% homology to the components of 85 complex. Two-dimensional electrophoresis of culture fluids of BCG and Western blotting indicated the existence of the other novel protein(s) which strongly cross-reacted with the α antigen (85B) and MPB51.     

A Case of Pneumatosis Cystoides Intestinalis Associated with Trichlor-oethylene Developing Typical Features during the Follow-up of Undetermined Sigmoid Lesions

Abstract: A 64-year-old male lens grinder with a 43-year history of occupational exposure to trichloroethylene (TCE) visited Shinshu University Hospital in December 1988 complaining of distention of the lower abdomen. A barium enema study and endoscopic examination showed rosary-like semilunar folds and edematous mucosa of undetermined etiology in the sigmoid colon. Three months later, the patient visited the hospital again complaining of blood in the stool. An endoscopic examination performed at this time revealed numerous broad-based, smooth-surfaced, protruding lesions in the sigmoid colon. A diagnosis of pneumatosis cystoides intestinalis (PCI) associated with TCE was made. After 4-days treatment with 50% oxygen given by inhalation, the patient's symptoms were completely relieved, and a colonoscopy revealed no evidence of PCI. He returned to his job at the factory which involved dealing with TCE and because of adequate ventilation and a shorter work-day he has been free of recurrence during a year follow-up period. The finding of rosary-like semilunar folds, as demonstrated by the initial endoscopic and barium enema studies in our patient, is a peculiar one in the incipient stage of PCI.     

Laparoscopic and Histopathologic Features of the Liver in Severe Chronic Active Epstein-Barr Virus Infection Syndrome: A Case Report

Abstract: A 40-year-old man was admitted to our hospital with persistent fever, generalized lymphadenopathy and hepatosplenoamegaly. Immunological examination demonstrated high titers of several anti-Epstein-Barr virus (EBV) antibodies, including anti-viral capsid antigens 1gG-antibody 1: 20, 480, anti-early antigens-DR IgG-antibody 1: 5, 120, and reduced activity of EBV-specific cytotoxic T lymphocytes. Laparoscopic features resembled those of chronic active viral hepatitis, including an uneven surface appearance and diffuse hepatic enlargement. Histopathological examination of a liver biopsy specimen showed inflammatory cell infiltration along sinusoidal surfaces (single file appearance) and enlarged portal areas with intralobular punched-out necrosis. The diagnosis was confirmed by detecting the EB viral genome in serum. Despite treatments with natural alpha-interferon, adenosine arabinocide and recombinant human interleukin-2, the patient died of progressive hepatic failure.     

Effect of fluoride addition on ionized calcium in salivary sediment and in saliva containing various amounts of solid calcium fluoride

Abstract – The aim of the present study was to estimate the fluoride concentrations necessary for the precipitation of calcium fluoride or calcium fluoride-like substances in saliva and salivary sediment, in the presence of various amounts of chemically pure calcium fluoride. Fluoride was added in increments to whole saliva to which solid calcium fluoride had been added. The ionized calcium concentration was determined immediately after centrifugation. In salivary sediments and saliva with no calcium fluoride added the ionized calcium concentration was nearly independent of the fluoride concentration added below 10 and 20 mmol/l, respectively, and at fluoride concentration above 15 and 25 mmol/l, respectively, nearly no ionic calcium was detected. In the presence of calcium fluoride the ionized calcium concentration decreased rapidly with increasing concentrations of fluoride. The more calcium fluoride added, the lower the ionized calcium concentration at a certain fluoride level. The results suggested that in the in vivosituation, caries preventive measures based on frequent oral exposure to low concentrations of fluoride may provide calcium fluoride or calcium fluoride-like substances, which will serve as a reservoir for fluoride.     

Sex determining gene on the X chromosome short arm: Dosage sensitive sex reversal

The present review article summarizes current knowledge concerning the sex determining gene on Xp21, termed DSS (dosage sensitive sex reversal). The presence of DSS has been based on the finding that, in the presence of SRY, partial active Xp duplications encompassing the middle part of Xp result in sex reversal, whereas those of the distal or proximal part of Xp permit male sex development. Because Klinefelter patients develop as males, it is believed that DSS is normally subject to X-inactivation, and that two active copies of DSS override the function of SRY, resulting in gonadal dysgenesis because of meiotic pairing failure. It may be possible that DSS encodes a target sequence for repressing function of SRY or that DSS is involved in an X chromosome-counting mechanism. Molecular approaches have localized DSS to a 160 kb region and isolated candidate genes such as DAX-1 and MAGE-Xp, but there has been no formal evidence equating the candidate gene with DSS. In addition to its clinical importance, the exploration of DSS must provide a useful clue to phylogenetic studies of sex chromosomes and dosage compensation.