High levels of serum lipoprotein(a) in patients with ischemic heart disease with normal coronary angiogram and thromboangiitis obliterans.

To determine whether lipoprotein(a) (Lp(a)) contributes to the acceleration of cardiovascular diseases without atherosclerotic lesion, we have measured serum Lp(a) level in male subjects aged 40-69 years with thromboangiitis obliterans (n = 40) and ischemic heart disease (IHD) with normal coronary angiogram (n = 35) in addition to subjects with arteriosclerosis obliterans (n = 123) and IHD with atherosclerotic coronary lesion (n = 203). Cases who had no IHD, arteriosclerosis obliterans or thromboangiitis obliterans were selected as a control group (n = 316). Subjects without any diseases or abnormal findings in physical examination and laboratory data were selected from the control group as the healthy control group (n = 156). The Lp(a) levels of arteriosclerosis obliterans and IHD with atherosclerotic coronary lesion were significantly higher (17.0 mg/dl and 13.1 mg/dl; median) than those of control and healthy control groups (9.9 mg/dl and 9.4 mg/dl, respectively) (P < 0.01), in agreement with previous reports. Furthermore, the Lp(a) level of IHD with normal coronary angiogram group was significantly higher (18.9 mg/dl) than those of the control and healthy control groups (P < 0.05). The Lp(a) level of thromboangiitis obliterans group was also much higher (21.3 mg/dl) than that of the healthy control group (P < 0.05). The current study suggests that Lp(a) is one of the independent risk factors for the development of atherosclerotic diseases such as arteriosclerosis obliterans and IHD with atherosclerotic coronary lesion.(ABSTRACT TRUNCATED AT 250 WORDS)

Fibrinogen Osaka IV: a congenital dysfibrinogenemia found in a patient originally reported in relation to surgery, now defined to have an A alpha arginine-16 to histidine substitution.

We discovered a congenital heterozygous dysfibrinogen in a patient and reported this case in relation to surgery some time ago (Jpn J Surg (1988) 18:43-46). Further studies on the isolated abnormal population of fibrinogen derived from this patient have revealed that fibrinopeptide A was not cleaved by ancrod, a snake venom-derived thrombin-like enzyme, but by thrombin, slowly but completely. The released fibrinopeptide A components, being the A, AY, and AP peptides, were all found to be abnormal, as evidenced by slightly earlier elution positions on high-performance liquid chromatography, compared with the normal counterparts. By analyzing their amino acid sequence, we have identified an arginine to histidine substitution at position 16 of the A alpha chain, the thrombin cleavage site. Utilizing insolubilized abnormal fibrinogen, we confirmed that the polymerization site assigned to the central E domain, the "A" site, was exposed by thrombin, but not by ancrod. This dysfibrinogen, designated as fibrinogen Osaka IV, is the second abnormal molecule with an A alpha arginine-16 to histidine substitution identified among Japanese families.

[A case of basal encephalomeningocele (a transethmoidal type) containing the olfactory nerve].

An experience of operative case of basal (transethmoidal type) encephalomeningocele is reported. A 3-year-old boy complained of continuous, spontaneous cerebrospinal fluid rhinorrhea and right intranasal mass lesion. The rhinorrhea began when one year old. He suffered from meningitis at the age of two. When he was three years old, the intranasal mass and CSF rhinorrhea were perceived. On admission he had normal general status and no abnormality in neurological examination. The usual axial computed tomography showed a small mass in the intranasal cavity. But the continuity between the mass and brain parenchyma was not detected in this study. Metrizamide CT cisternography demonstrated it. Coronal CT scan visualized it more directly. We performed operation using bifrontal craniotomy and subfrontal approach. Brain parenchyma did not protrude into the frontal cranial base and expanding right olfactory nerve penetrated into the small defect at the right cribriform plate. The olfactory nerve was extracted as much as possible. The defect was filled with a few piece of muscle and coating with the adhesive agent was performed. Histological findings of operative specimen were abnormal olfactory nerve, normal brain tissue and arachnoid membrane. Post-operative state was uneventful. CSF rhinorrhea disappeared. Post-operative direct sagittal computed tomography visualized the encephalomeningocele extremely. Biopsy of the intranasal mass revealed brain tissue covered by normal nasal epithelium. Basal encephalocele is rare in Japan. We found 10 cases in the literature. It is important that we do not forget basal encephalocele in the difference of intranasal tumors.(ABSTRACT TRUNCATED AT 250 WORDS)

Studies on glycosphingolipids of fresh-water bivalves. III. Isolation and characterization of a novel globoside containing mannose from spermatozoa of the fresh-water bivalve, Hyriopsis schlegelii.

Three globosides were isolated from spermatozoa of the fresh-water bivalve, Hyriopsis schlegelii by mild alkaline hydrolysis, acetone precipitation, Unisil column chromatography and preparative thin-layer chromatography. These globosides are unique in their sugar chains, since they contain mannose instead of galactose found so far in all globosides of mammals and other animals examined. The main globoside in the spermatozoa was characterized as Glc-NAcbeta(1 leads to 2)Manbeta(1 leads to 3)Manbeta(1 leads to 4)Glcbeta(1 leads to 1)-ceramide by partial acid hydrolysis, analysis of its anomeric configuration with chromium trioxide, methylation analysis and enzymatic hydrolysis. The globoside contained normal saturated fatty acids ranging in length from C16 to C21, palmitic and stearic acids being predominant. Its main long-chain was octadeca-4-sphingenine.