Japanese journal of medicine最新文献

A 71-year-old woman was clinically suspected of allergic granulomatous angiitis (AGA) because of preceding allergic diseases including bronchial asthma, remarkable eosinophilia (14,300/mm3), mononeuritis multiplex, positive rheumatoid factor, elevated serum immunoglobulin E, and eosinophilic inflammation of the kidney. Autopsy findings, however, were characterized of polyarteritis nodosa (PAN). Necrotizing angiitis was present in several organs except for the lung; focal and segmental glomerular lesions with crescent formation were observed in the kidney, and granuloma formation was not found. This case may be an intermediate type between PAN and AGA (an overlap syndrome) and provide useful information on the clinical entities of systemic necrotizing vasculitis.

A case of cellular schwannoma originating from the retroperitoneum is reported. The histological features of the tumor resembled those of a smooth muscle tumor. However, the tumor cells were positive for S-100 protein, which is almost exclusively identified within nerve sheath tumor cells. This finding confirmed the diagnosis of cellular schwannoma. This type of tumor is generally considered to be benign but is sometimes mistaken for a malignant tumor. These findings indicate that it is important to prove whether or not S-100 protein is present in the cells of a tumor of unknown origin especially arising in the mediastinum or retroperitoneum.

We report a case of hypocalcemic heart failure without underlying myocardial disease. Two-dimensional and Doppler echocardiography revealed dilatation and impaired contraction of the left ventricle, but did not show any valvular dysfunction. Cardiac catheterization showed a normal coronary artery, and cardiac muscle biopsy showed morphological changes in mitochondoria and endoplasmic reticulum, which may be due to metabolic changes. This patient was asymptomatic after the serum calcium concentration was normalized.

Recombinant interleukin (IL-2) was administered to 16 patients with HBe antigen-positive chronic active hepatitis in which the diagnosis was ascertained histologically. In 7 of the 16 patients, a decrement of the serum HBe antigen value was observed (Group A). In group A, the findings showed an increment of peripheral Leu 11-positive cells and NK and LAK cell activity, an acute exacerbation during and after IL-2 administration, disappearance of HBc antigen observed in liver biopsy histology, and decrement of serum DNA-p activity. However, seroconversion of HBs antigen was not observed and no case showed the elimination status of continuous HB virus infection. On the other hand, in the other 9 patients (Group B), these changes were not observed and the existence of a HLA type difference between Group A and B was shown by HLA analysis. These results indicated that the immune responses mediated by IL-2 may play an important role in the development of chronic hepatitis B, and these results may be regulated genetically.

An 80-year-old man with Ebstein's anomaly and ductus arteriosus aneurysm is reported. He was admitted with bradycardiac atrial fibrillation and right ventricular failure. For the control of brady-arrhythmia, a permanent pacemaker was implanted. Two-dimensional echocardiogram revealed distal displacement of the septal tricuspid valve. Aortography and computed tomography showed ductus arteriosus aneurysm. This is the first report of the association of Ebstein's anomaly and non-patent ductus arteriosus aneurysm.

A 68-year-old woman had an abrupt onset of severe headache, nausea, vertigo, difficulty in standing and dysarthria. A CT scan of the brain disclosed bilateral symmetrical round infarctions involving the middle cerebellar peduncles. She exhibited marked limb ataxia, gait ataxia, dysarthria and transient gaze nystagmus. Occlusion of the right vertebral artery associated with a stenosis of the basilar artery just proximal to the origin of the anterior inferior cerebellar arteries shown in angiograms were thought to be the cause.

Mexiletine was administered in two patients suffering from multiple sclerosis with severe dysesthesia and painful tonic seizures. In both patients the painful tonic seizures disappeared and dysesthesia improved as well. The effects of mexiletine on painful symptoms have been previously reported in diabetic neuropathy, but not in diseases of the central nervous system.

A 46-year-old man developed neuroleptic malignant syndrome with acute myoglobinuric renal failure after the discontinuation of sulpiride and maprotiline treatment. He showed the characteristic features of hyperpyrexia, altered consciousness, muscle rigidity, and autonomic dysfunction. Laboratory data showed lysis of skeletal muscle cells and renal impairment. Muscle biopsy revealed necrosis and regenerative changes in muscle fibers. Renal biopsy showed focal tubulitis and interstitial infiltration of small inflammatory cells. The combination of sulpiride and maprotiline has not previously been reported to be the cause of neuroleptic malignant syndrome and acute myoglobinuric renal failure.

A 54-year-old woman was treated for bronchial asthma for 14 yr. In March of 1989, chest roentgenography and computed tomography (CT) revealed development of bilateral pulmonary hilar lymph node enlargement. Positive 67Ga uptake was observed in bilateral pulmonary hili. Although levels of serum angiotensin converting enzyme (ACE) and lysozyme were within normal range, biopsy specimen of scalene lymph nodes showed noncaseating epitheloid-cell granuloma, leading to the diagnosis of sarcoidosis. Steroid therapy ameliorated both sarcoidosis and bronchial asthma. Although the association of sarcoidosis and bronchial asthma is uncommon, there may be an etiological relationship between them.

Changes in plasma lipids and abnormal lipoproteins in a patient with ticlopidine hydrochloride-induced hepatitis were studied. Total cholesterol, free cholesterol and phospholipids increased markedly during cholestasis. Lipoprotein electrophoresis showed a large lipoprotein-X band and a relatively small alpha-lipoprotein band. As cholestatic hepatitis improved, all plasma lipids were progressively decreased. A broad band of abnormal alpha-lipoprotein appeared for the first time with the decrease in the lipoprotein-X band. Both abnormal lipoproteins decreased further and eventually disappeared. The appearance of abnormal alpha-lipoprotein was accompanied by changes in apoproteins. These findings suggest that abnormal alpha-lipoprotein might be an intermediate metabolite of lipoprotein-X.