Akira Genda

Intermediate-density lipoprotein and cholesterol-rich very low density lipoprotein in angiographically determined coronary artery disease.

The relationship between the concentrations of intermediate-density lipoprotein (IDL) and other lipoproteins and the extent of coronary artery disease (CAD) was studied in 182 consecutive patients evaluated by selective coronary cineangiography. On univariate analysis, the extent of CAD correlated significantly and positively with very low density lipoprotein (VLDL) cholesterol, IDL cholesterol and lowdensity lipoprotein (LDL) cholesterol, and negatively with high-density lipoprotein (HDL) cholesterol. Analysis of four subgroups divided by IDL cholesterol and LDL cholesterol levels indicated that moderately increased levels of IDL cholesterol were closely associated with a high frequency of CAD. Moreover, multivariate regression analysis demonstrated that IDL cholesterol for men, LDL cholesterol for men and women and HDL cholesterol for men were significant variables of use in the final weighting procedure. IDL cholesterol was closely associated with cholesterol-rich VLDL. This study shows that IDL and cholesterol-rich VLDL combine to contribute to the development of CAD.

Quantitative approach to the histopathology of the biopsied right ventricular myocardium in patients with diabetes mellitus

SummaryFor the purpose of studying the clinicopathology of the biopsied myocardium in patients with diabetes mellitus, the diameter of right ventricular myocardial cells and diffuse perimysial fibrosis of biopsied myocardium were measured quantitatively. Seven healthy controls and nine diabetic patients without hypertension or coronary arterial disease were subjected to this study. The degree of diabetic complications was mild to moderate. The diameter of myocardial cells was measured and the degree of diffuse perimysial fibrosis was assessed by the point-counting method using a square grid, in which the distance between the points was 10 µm. Over 2000 points which lay on the longitudinally cut myocardial cells and on the interstitial fibrosis stained by the Mallory-Azan method were measured. Percentage fibrosis was calculated according to the formula: percentage fibrosis=(points lying on the interstitial fibrosis)/[(points lying on the myocardial cell)+(points lying on the interstitial fibrosis)] × 100. The results were as follows. The mean diameter of right ventricular myocardial cells in patients with diabetes mellitus was significantly larger than that of controls (P<0.01). The percentage fibrosis of diabetic patients was significantly higher than that of controls (P<0.01). There was no significant correlation between the histopathological measurements and clinical features. It is concluded that hypertrophy of myocardial cells and interstitial fibrosis of the myocardium exist even in mild diabetes mellitus.

Germanium intoxication with sensory ataxia

Sensory ataxia in inorganic germanium intoxication is rare. A 63-year-old housewife had taken inorganic germanium preparations at a dosage of 36 mg a day for about 6 years (total dose about 80 g). She subsequently developed difficulty in writing and gait disturbance with peripheral neuropathy and renal involvement. Germanium, which is not usually detected in the non-germanium user, was accumulated in her hair and nails, permitting a diagnosis of inorganic germanium intoxication. The peripheral neuropathy and renal injury were not reversible after discontinuing the preparation. Pneumonia and sepsis then supervened and the patient died. Autopsy findings showed degeneration and loss of the dorsal root ganglion cells and degeneration of the dorsal column of the spinal cord. Two previously reported cases presented with ataxia. These patients took germanium for long periods and/or large quantities like our case. It was supposed that sensory ataxia was induced by chronic and dose dependent toxicity of inorganic germanium.

Coronary angiographic characteristics in Japanese patients with heterozygous familial hypercholesterolemia

Coronary angiographic findings were analyzed in 51 consecutive patients (36 males and 15 females) with heterozygous familial hypercholesterolemia (FH) and 279 consecutive patients (216 males and 63 females) without FH (non-FH). The coronary stenosis index and over 75% stenosis vessel subset were almost three times as high in the FH group. The incidence of myocardial infarction was almost twice as high in the FH group. Levels of total cholesterol and its lipoprotein fractions, except HDL-cholesterol, were almost twice as high in the FH group. In the FH group aged under 50 years, the two parameters of coronary angiogram and the incidence of myocardial infarction were significantly higher in males than in females. However, in the group aged over 50 years, all three parameters were not significantly different between those in males and females. The level of HDL-cholesterol was significantly lower in males than in females. A significantly higher incidence (18%) of coronary ectasia was observed in the FH group compared with the incidence (2%) in non-FH. All patients with coronary ectasia were males, except one female with FH. On comparison of the males among the FH patients with those among the non-FH patients matched for total cholesterol, age and other risk factors, the FH patients were associated with a significantly higher degree of coronary atherosclerosis and lower level of HDL-cholesterol. Seven FH patients with a normal coronary angiogram were observed. However, any factors as regards age, total cholesterol, HDL-cholesterol and Achilles tendon thickness failed to distinguish between the FH patients with a normal coronary angiogram and those without.(ABSTRACT TRUNCATED AT 250 WORDS)

Left ventricular endomyocardial biopsy findings in patients with essential hypertension and hypertrophic cardiomyopathy with special reference to the incidence of bizarre myocardial hypertrophy with disorganization and biopsy score

SummaryTo investigate whether bizarre myocardial hypertrophy with disorganization (BMHD) is characteristic of hypertrophic cardiomyopathy (HCM), the histopathology of the biopsied left ventricular myocardium in 18 patients with essential hypertension (HT) and 14 patients with HCM was studied. A “biopsy score” was devised for a more quantitative evaluation of the BMHD and a comparative study on the biopsy score of the left ventricular biopsied specimen was also performed. The patients with HT were judged to be in stages I or II of the WHO criteria and had a history of hypertension of more than 5 years. The BMHD was defined as myocardial cells showing hypertrophy, disorganization, and bizarre nuclei. “Disorganization” of myocardial cells was distinguished both by the terminology and histopathological characteristics from “disarrangement” of myocardial cells. The biopsy score employed four factors and was determined according to the following formula: Biopsy score = hypertrophy of myocardial cells + (disorganization of myocardial cells) ×2+ bizarre nuclei + whorling of muscle bundles. Both the hypertrophy and the disorganization of myocardial cells were regarded as essential conditions indicating the presence of BMHD. The BMHD was found in 2 of 18 patients with HT (11%) and in 10 of 14 patients with HCM (71%) in the left ventricular biopsied specimens (P<0.005). However, “disarrangement” of myocardial cells was found in 13 of 18 HT patients (72%) and in 10 of 14 HCM patients (71%) in the left ventricular biopsied specimens, showing no difference between the two groups. The biopsy score in HCM patients was larger than that found in HT patients. It was concluded that BMHD is highly specific (89%) for HCM but that disarrangement of myocardial cells is not specific or diagnostic for HCM. The biopsy score is, therefore, useful for diagnosing HCM histologically and for distinguishing HCM from HT.

Coronary ectasia in a homozygous patient with familial hypercholesterolemia

A 23-year-old man with homozygous familial hypercholesterolemia was found to have coronary ectasia by coronary angiography. This case showed generalized xanthomatosis and severe hyper low density lipoproteinemia, and his cultured skin fibroblasts showed LDL receptor activities compatible with the receptor-defective homozygous type of familial hypercholesterolemia. Coronary angiography showed fusiform aneurysmal involvements in the right coronary artery and left circumflex artery, and 50% stenosis in the right coronary artery and left anterior descending artery. Thus, homozygous familial hypercholesterolemia produces coronary ectasia as well as premature coronary stenosis.

Acute increases in plasma oxidized low-density lipoprotein immediately after percutaneous transluminal coronary angioplasty

To investigate the acute changes in plasma oxidized low-density lipoprotein before and immediately after coronary angioplasty, we studied 132 consecutive patients who successfully underwent this procedure. Plasma oxidized low-density lipoprotein levels were significantly increased immediately after coronary angioplasty in patients with stable angina pectoris as well as in those with acute coronary syndromes.

A case of hypereosinophilic syndrome with asymmetric septal hypertrophy

A case of idiopathic hypereosinophilic syndrome (HES) with asymmetric septal hypertrophy (ASH) is described: this is a very rare association. The patient was a 56-year-old male with hypereosinophilia lasting for 10 years. The white blood cell count was 11200/mm3, with 22% eosinophils, and eosinophilic hyperplasia (7.2%) was noted in the bone marrow. A peripheral blood smear showed vacuolated eosinophils with a reduced content of granules. An ultrastructure study of the eosinophils revealed reduced numbers of crystalloid granules which appeared to be dissolving with reversal of normal staining. An echocardiogram and a biventriculoglam indicated ASH with the interventricular septal wall thickness of 2.4 cm and the left ventricular posterior wall thickness of 1.5 cm. Right ventricular endomyocardial biopsy revealed no eosinophilic infiltration, but endocardial thickening, subendocardial fibrosis, hypertrophy, myocytolysis, and fragmentation of muscle bundles were observed.

The correlation between the diameter of biopsied left and right ventricular myocardial cells and hemodynamic parameters in essential hypertension and mitral valve disease.

For the purpose of studying the correlation between the diameter of myocardial cells and hemodynamic parameters in human beings, the left ventricular endomyocardial biopsy was performed in 29 cases. Eleven cases were normotensive patients. The remaining 18 subjects had a history of hypertension for more than 5 years. The mean diameter of the left ventricular myocardial cells was correlated with the systolic aortic pressure (r = 0.51, p less than 0.01) and the systemic vascular resistance index (r = 0.66, p less than 0.01). The same study was done on the right ventricle of 7 controls and 11 patients with mitral valve disease in which chronic right ventricular pressure overload was present. The mean diameter of myocardial cells biopsied from the right ventricle was correlated with the systolic pulmonary arterial pressure (r = 0.61, p less than 0.01) and the pulmonary vascular resistance index (r = 0.72, p less than 0.01). These findings indicate that the diameter of myocardial cells in both the left and right ventricles was closely related with its afterload.

A CASE OF TYPE 3 HYPERLIPOPROTEINEMIA ASSOCIATED WITH XANTHOMATOSIS AND CORONARY ARTERIOSCLEROSIS MANIFESTED WITH DEVELOPMENT OF SHEEHANS SYNDROME

下垂体前葉機能低下症に合併した高脂血症の報告は少なく, III型と確定診断され報告された例はみあたらない.本症例は38才の主婦で,約11年前に出産時大量出血あり,その後下垂体前葉機能低下症状が出現し, 4年前より全身に黄色腫を認めるようになつた.入院後の各種負荷試験ではTSH, ACTH, LH, HGH, IRIの低反応が認められSheehan症候群と診断された.また血清トリグリセリド,コレステロール(以下chol.と略す)はそれぞれ898, 500mg/dlと高く,リポ蛋白のアガロース電気泳動でbroad-β bandとβ-VLDLを認め超遠心法にてもVLDL Chol値の上昇がみられ, Fredrickson分類のIII型高脂血症と診断された. III型高脂血症は動脈硬化をきたしやすいことが知られているが,本症例でも冠動脈造影にて両側の冠動脈に約50～70%に及ぶ狭窄が認められた.乾燥甲状腺末と副腎皮質ホルモン剤の治療により血清脂質は正常化したが, broad-β bandは持続し,超遠心法でもVLDL-Chol値は高値のままであつた.また治療2カ月では黄色腫に少し消退が認められた.以上の観察により,本症例では潜在的な原発性III型高脂血症があり,これが出産時の大量出血による下垂体前葉機能低下により増強されて黄色腫の発現に至つたものと考えられる.