An apparently higher molecular weight gamma-chain variant in a new congenital abnormal fibrinogen Tochigi characterized by the replacement of gamma arginine-275 by cysteine

A gamma-chain variant with an apparently higher molecular weight than the normal gamma-chain was detected in a new congenital abnormal fibrinogen with impaired polymerization of the fibrin monomer and with normal release of fibrinopeptides A and B in a 51-year-old male. Purified fibrinogen analyzed on sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) under the reduced condition in the system of Laemmli contained two protein bands in the gamma-chain region (molecular weight, 50,500 as compared with 50,000 for the normal), both with normal crosslinking ability. The presence of two types of gamma- chains was more clearly detected when reduced and carboxymethylated fibrinogen was analyzed by SDS-PAGE or when reduced fragment D2 was analyzed on SDS-PAGE followed by Western blotting, and identified by positive staining for anti gamma-chain monoclonal antibody. Cyanogen bromide- or lysylendopeptidase-cleavage of purified gamma-chains analyzed on reverse-phase high performance liquid chromatography showed the decrease of one peptide compared with the normal and the appearance of an abnormal peptide peak. Amino acid sequence analysis demonstrated that the gamma arginine-275 of gamma-chain variant was replaced by a cysteine. These data suggest that some regions or conformations containing gamma 275 will affect the polymerization of fibrin monomers. The propositus' two daughters had the same abnormal fibrinogen. This unique inherited abnormal fibrinogen was designated as fibrinogen Tochigi, and the gamma-chain variant as gamma Tochigi.     

Staging of Early Colorectal Cancers: Magnifying Colonoscopy versus Endoscopic Ultrasonography for Estimation of Depth of Invasion

BACKGROUND AND AIMS: Preoperative estimation of depth of invasion in early colorectal cancers (CRCs) is essential for patient management. This study was conducted to compare the diagnostic accuracies of magnifying colonoscopy and endoscopic ultrasonography (EUS) for estimating the depth of invasion of early CRCs. SUBJECTS AND METHODS: A total of 438 early CRCs were removed endoscopically or surgically from July 1993 through March 1999 at our hospital. Before removal, 102 lesions were evaluated with both magnifying colonoscopy and EUS and were included in this analysis. The diagnostic accuracy of each method, referring to the histology of the resected specimens, was evaluated. RESULTS: The overall diagnostic accuracies were 87% (89/102) for magnifying colonoscopy and 75% (76/102) for EUS (P = 0.0985). Subgroup analysis was also done for polypoid and non-polypoid lesions. For polypoid lesions, the overall diagnostic accuracies of magnifying colonoscopy and EUS were 88% (60/68) and 72% (49/68), (P = 0.0785), and for non-polypoid lesions, they were 85% (29/34) and 79% (27/34), (P = 0.7169). CONCLUSION: Although, there is a substantial difference in the overall diagnostic accuracies, it is not statistically significant. Therefore, we conclude that magnifying colonoscopy is at least as accurate as EUS for preoperative staging of early CRCs.     

A case of tuberculous pericarditis

A case of tuberculous pericarditis successfully managed with medical treatment alone was reported. A 78-year-old male was admitted because of cough, dyspnea and fever. Chest X-P and echocardiogram revealed massive pericardial effusion. His clinical symptoms and signs suggested cardiac tamponade. Mycobacterium tuberculosis was detected from pericardial fluid. ADA activity in pericardial fluid was high. Thoracic CT scan showed tracheobronchial, pretracheal, paratracheal and superior mediastinal lymph-node swelling. The diagnosis of tuberculous pericarditis was confirmed. Anti-tuberculous therapy consisting of INH, RFP, EB in combination with prednisolone was started. One month later pericardial effusion was controlled and six months later he was in good clinical condition without surgical treatment.     

Extraintestinal manifestations of granulomatous enterocolitis induced in rabbits by long-term submucosal administration of muramyl dipeptide emulsified with Freund's incomplete adjuvant

We examined whether extraintestinal manifestations of granulomatous enterocolitis in rabbits might be produced by the long-term administration of muramyl dipeptide which represents the basic fragment of the bacterial cell wall, emulsified with Freund's incomplete adjuvant. Muramyl dipeptide emulsion was injected submucosally at six sites in the rectum and colon, 10 cm proximal to the anus, each time with a flexible endoscope. Seven rabbits were injected nine times or more every month, and all were sacrificed 1 month after the last injection. The histological changes in the colon in the seven rabbits were mononuclear cell infiltration, epithelioid granulomas, granulomatous lesion, and denuded and regenerative epithelia, although the changes differed in degree. In five of the seven rabbits, histological examination of the liver showed pericholangitis and periductal fibrosis, findings analogous to sclerosing cholangitis in patients with inflammatory bowel disease. In four of the seven rabbits, fibrosis bridging mainly between portal and portal veins, and, in places, between portal and central veins, was seen. Two of the seven rabbits developed polyarthritis. The histological changes in our model suggest that continuous stimulation with bacterial cell wall fragments may be involved in the extraintestinal manifestations of chronic intestinal inflammation such as that seen in inflammatory bowel disease.     

Comparison of gastric peristalsis inhibition by scopolamine butylbromide and glucagon: evaluation by electrogastrography and analysis of heart rate variability

Background. Activation of glucagon receptors of the smooth muscle membrane suppresses gastric peristalsis. We evaluated autonomic nervous activity by two methods, electrogastrography (EGG) and analysis of heart rate variability, to compare the inhibiting effects of glucagon and scopolamine butylbromide on gastric peristalsis. Methods. Heart rate variability, EGG, and blood catecholamine levels were measured before and after administration of glucagon (G group), scopolamine butylbromide (SB group), or physiological saline (C group). Autonomic nervous function was evaluated using spectral analysis of heart rate variability, and low frequency (LF) and high frequency (HF) power; the LF/HF ratios were also determined. Results. After administration of scopolamine butylbromide, HF power, an index of parasympathetic nervous activity, decreased; and the LF/HF ratio, an index of sympathetic nervous activity, increased. In contrast, no significant change was observed in autonomic nervous activity after administration of glucagon. The peak power amplitudes of the EGG decreased significantly in the G and SB groups after intramuscular injection, but the difference between the groups was not significant. Furthermore, the dominant frequency increased significantly in the G and SB groups after injection. Serum catecholamine levels showed no significant changes after administration of scopolamine butylbromide or glucagon. Conclusions. Inhibition of gastric peristalsis by glucagon via glucagon receptors on smooth muscles did not influence autonomic nervous activity, unlike the results obtained after administration of scopolamine butylbromide. Therefore, glucagon may be safe for use with elderly patients and those with cardiopulmonary complications.     

Multiple gastric involvement by myeloid antigen CD13-positive non-secretory plasma cell leukaemia

Gastrointestinal tract involvement is a rare complication of plasma cell neoplasia. We present a case of non-secretory type primary plasma cell leukaemia (PCL) with multiple gastric involvement. Dual surface antigen analysis of bone marrow cells revealed that atypical plasma cells coexpressed CD38 and myeloid antigen CD13. Upper gastrointestinal endoscopy disclosed multiple submucosal masses in the body of the stomach. Endoscopic biopsy specimens showed marked infiltration of atypical plasma cells consistent with a diagnosis of gastric involvement by PCL. Since CD13 antigen is identical to aminopeptidase N, a membrane-bound glycoprotein thought to be involved in the process of tumour invasion, CD13 expression on neoplastic plasma cells may be related to the gastric involvement in this patient.     

Morphologic characteristics of chernobyl-related childhood papillary thyroid carcinomas are independent of radiation exposure but vary with iodine intake

BACKGROUND: The Chernobyl accident caused an unprecedented increase in papillary thyroid carcinoma (PTC) incidence with a surprisingly short latency and unusual morphology. We have investigated whether unexpected features of the PTC incidence after Chernobyl were radiation specific or influenced by iodine deficiency. METHODS: PTCs from children from Belarus, Ukraine, and the Russian Federation exposed to fallout from Chernobyl were compared with PTCs from children not exposed to radiation from the same countries, from England and Wales (E&W) and from Japan. The degree and type of differentiation, fibrosis, and invasion were quantified. RESULTS: There were no significant differences between PTCs from radiation-exposed children from Belarus, Ukraine, and the Russian Federation and PTCs from children from the same countries who were not exposed to radiation. Childhood PTCs from Japan were much more highly differentiated (p < 0.001), showed more papillary differentiation (p < 0.001) and were less invasive (p < 0.01) than "Chernobyl" tumors, while tumors from E&W generally showed intermediate levels of degree and type of differentiation and invasion. There was a marked difference between the sex ratios of children with PTCs who were radiation exposed and those who were not exposed (F:M exposed vs. unexposed 1.5:1 vs. 4.2:1; chi(2) = 7.90, p < or = 0.01005). CONCLUSIONS: The aggressiveness and morphological features of Chernobyl childhood PTCs are not associated with radiation exposure. The differences found between tumors from the Chernobyl area, E&W, and Japan could be influenced by many factors. We speculate that dietary iodine levels may have wide implications in radiation-induced thyroid carcinogenesis, and that iodine deficiency could increase incidence, reduce latency, and influence tumor morphology and aggressiveness.     

Coronary ostial endarterectomy in Takayasu's aortitis--confirmation of patency nine years postsurgically.

A case of Takayasu's aortitis with severe bilateral coronary ostial stenosis is reported. A transaortic coronary endarterectomy was performed and sufficient patency was confirmed angiographically 9 years after the operation. This is the first report of late coronary angiography after a transaortic coronary ostial endarterectomy in Takayasu's aortitis. The efficacy of this procedure for coronary ostial stenosis in Takayasu's aortitis is emphasized.     

Detection of BCL2-IGH rearrangement on paraffin-embedded tissue sections obtained from a small submucosal tumor of the rectum in a patient with recurrent follicular lymphoma

A 59-year-old woman was admitted to our hospital because of recurrent follicular lymphorna (FL). Colonoscopic examination revealed a rectal submucosal tumor (SMT) without any erosions and ulcers. In this patient, it was difficult to distinguish non-Hodgkin‘s lymphoma (NHL) invasion from other disorders of the colon including carcinoid tumor merely based on endoscopic findings. Histopathologic and immunohistochemical studies on biopsy specimens showed an infiltration of atypical lymphocytes that were positive for CD20 and BCL2 but negative for UCHL-1. Fluorescence in situ hybridization on paraffin-embedded tissue sections (T-FISH) identified a translocation of BCL2 with IGHgene.Based on these findings, the tumor was defined as an invasion of FL. T-FISH method is useful for the detection of a monoclonality of atypical lymphocytes in an SMT of the gastrointestinal tract, and particularly for the detection of chromosomal translocations specific to lymphoma subtypes.