We determined the incidence of coronary lesions by coronary angiography and the associated risk factors in 102 patients with abdominal aortic aneurysms (AAA). Old myocardial infarction was observed in 33 patients and angina pectoris in 27 patients. Coronary angiography revealed significant stenosis (≥75%) in 66 patients, including single-vessel disease in 22 patients and multiple-vessel disease in 44 patients. Angiography detected 21 coronary artery ectasia lesions, consisting of 10 saccular and 11 fusiform aneurysms in 18 patients. Significant coronary stenosis was present in approximately two-thirds of patients with AAA, suggesting that coronary angiography is a useful preoperative examination in patients with AAA. The incidence of coronary ectasia was also high in patients with this disease, perhaps because AAA and coronary ectasia involve similar pathogenic processes. There was considerable overlap in risk factors in patients with AAA and patients with coronary artery disease without AAA. However, advanced age and hypertension were more strongly associated with AAA than with coronary artery disease.
(Internal Medicine 36: 384-388, 1997)

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Thromboembolism has been shown to play a role in the pathogenesis of inflammatory bowel disease (IBD). A possibility exists that lipoprotein (a) [Lp(a)J, a newly-discovered prothrombotic factor, also participates in the development of at least some cases of IBD. Marked elevation of serum Lp(a) levels was observed in a young patient with ulcerative colitis. A biopsy specimen of the rectal mucosa showed findings compatible with ulcerative colitis, as well as small vessel thrombus occurring within the muscularis mucosa in the rectum. Serum Lp(a) levels were markedly elevated on admission (71 mg/dl), with a gradual decrease to 46 mg/dl on discharge. Moreover, serum Lp(a) levels decreased in parallel with clinical improvement. In the quiescent clinical stage, no small vessel thrombus was observed in the mucosa on follow-up colonoscopy. The association between IBD and hyper-Lp(a)-emia would be presumable but it has been, to our knowledge, previously unreported. The case reported here would be the first young patient, suggesting the presence of hyper-Lp(a)-emia and small vessel thrombus formation occurring in association with the development of ulcerative colitis.
(Internal Medicine 36: 389-391, 1997)

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We report a case of gastrointestinal stromal tumor (GIST) of the stomach. The patient was a 79-year-old woman with two gastric submucosal tumors detected by ultrasonography. Proximal gastrectomy was carried out and the tumors were diagnosed as GIST by histological and immunohistochemical investigations. Mesenchymal tumors of the gastrointestinal tract have been traditionally regarded as largely leiomyomatous lesions. However, GIST and other tumors have been distinguished recently on the basis of tumor cell differentiation shown by immunohistochemical studies. We discuss the concept and the immunohistochemical characteristics of GIST.
(Internal Medicine 36: 392-397, 1997)

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A 53-year-old female had clinical and laboratory findings suggestive of Cushing's syndrome. In contrast to the Cushing's syndrome caused by cortical adenoma, a high level of urinary 17-ketosteroids (17-KS) was also noted. Imaging studies revealed a right adrenal tumor. Right adrenectomy was performed; the surgical specimen revealed a black adenoma consisting of compact cells with numerous pigments which seemed to be lipofuscin in nature. The present case indicates that black adenoma as well as adrenocortical carcinoma should be suspected, when patients with Cushing's syndrome show an increased level of urinary 17-KS excretion.
(Internal Medicine 36: 398-402, 1997)

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A case of pheochromocytoma was discovered incidentally during long-term hemodialysis for chronic renal failure due to acquired cystic kidney disease. A 52-year-old male patient was examined for weight loss of 3 kg during over a period of 3 months. Abdominal computed tomography (CT) revealed a left adrenal mass (3.0 cm in size). Plasma adrenaline and noradrenaline were increased to 521 pg/ml and 1, 341 pg/ml, respectively, and the metoclopramide provocative test was positive. Further, in the scintiscan using ¹²³I-metaiodobenzylguanidine (MIBG), an accumulation of the radionuclide in the left adrenal tumor region was confirmed. The patient is currently under observation and conservative treatment due to the possible occurrence of arterial hypotension after resection of the tumor, and to lesser possibility of the malignancy.
(Internal Medicine 36: 403-407, 1997)

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In a 25-year-old man with adult-onset type II citrullinemia, liver transplantation resulted in elimination of hyperammonemia and amino acid abnormalities associated with the disease. Postoperatively, a high intensity area in the right cingulate gyrus on a T₂-weighted brain magnetic resonance imaging (MRI) also disappeared, suggesting that it reflected an early reversible lesion due to the hyperammonemia. Moreover, the serum level of pancreatic secretory trypsin inhibitor (PSTI), which had been elevated, was normalized. Since the levels of PSTI mRNA and PSTI have been reported to be increased in the livers of type II citrullinemia patients, measurement of serum PSTI levels could aid in the diagnosis of this disease.
(Internal Medicine 36: 408-412, 1997)

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Cystic lesion, malignancy and interstitial pneumonia are well-known as pulmonary complications of patients with von Recklinghausen's disease. We report herein an unusual patient with distal acinar emphysema and interstitial pneumonia of prominent hypercellularity demonstrated by transbronchial biopsy and broncho-alveolar lavage fluid (BALF). Six months after quitting smoking, the total cell count of BALF was remarkably reduced. This patient remains stable under 5-year observation in terms of symptoms and findings of both BALF and pulmonary function tests. Quitting smoking may have facilitated a favorable prognosis for the particular lung disease complicated in this patient.
(Internal Medicine 36: 413-416, 1997)

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Primary cardiac lymphomas diagnosed antemortem are extremely rare. We present a case of primary cardiac lymphoma initially diagnosed antemortem by cytologic examination of pericardial effusion fluid. Echocardiography suggested the presence of a tumor localized at the right ventricular free wall. The cytologic examination of pericardial effusion was effective in establishing the correct antemortem diagnosis.
(Internal Medicine 36: 417-419, 1997)

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Miliary infiltrates observed on chest films in non-Hodgkin's lymphoma are extremely rare. We report a case with pulmonary infiltrates mimicking miliary tuberculosis associated with prominent eosinophilia and elevated IgE levels. The levels of circulating eosinophils correlated with disease activity as they transiently returned to normal after effective chemotherapy in a short period. However, the patient developed acute respiratory failure due to the rapid progression of the disease even with intensive chemotherapy. We emphasize that small nodular shadows appear to be a sign of the rapid progression of the disease and a poor prognosis.
(Internal Medicine 36: 420-423, 1997)

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A rare case of peripheral primitive neuroectodermal tumor (PNET) is reported. A 68-year-old woman complaining of lumbago was admitted to our hospital. Diagnosis was made based on pathological findings characterized by Homer Wright-type rosettes. Ultrastructural examination showed the presence of neurosecretory granules and short cytoplasmic processes, which were highly suggestive of neural differentiation. Chromosomal analysis of the neoplastic cells revealed translocation (Il;22)(q24;ql2), which is often found in Ewing's sarcoma and Askin tumor. These results strengthen the hypothesis of a common histogenesis for these small round cell tumors, and suggest common oncogenesis for these neoplasms.
(Internal Medicine 36: 424-429, 1997)

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A 31-year-old man was referred to our hospital for the management of progressive diffuse alveolar hemorrhage associated with renal dysfunction. Leukocytoclastic vasculitis was shown by skin biopsy and crescentic glomerulonephritis was also detected, in addition to positivity for proteinase 3-specific anti-neutrophil cytoplasmic antibodies (PR3-ANCA). The patient was diagnosed as a rare case of PR3-ANCA-positive pulmonary-renal vasculitic syndrome without granulomatous lesions. There was a good response to combination therapy with steroids and cyclophosphamide.
(Internal Medicine 36: 430-434, 1997)

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A 46-year-old Japanese woman has been followed up for 3 years due to interstitial pneumonia associated with progressive systemic sclerosis (PSS). During this follow-up period, chest roentgenogram revealed additional diffuse nodular shadows. She was diagnosed as having pulmonary sarcoidosis, which was confirmed by the presence of epitheloid granulomas within the alveolar septa. She was successfully treated with corticosteroids and recovered almost completely without worsening pulmonary abnormalities caused by her PSS. The independent clinical courses of these two diseases in the present case suggest that the complication of PSS and sarcoidosis in this patient may be coincidental.
(Internal Medicine 36: 435-438, 1997)

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Cryofibrinogenemia is a cryopathy in which hypersensitivity to cold is a prominent feature. Cryofibrinogenemia developed in an 18-year-old Japanese female patient during methimazole therapy for Graves' disease. She developed cryopathy (livedo reticularis, Raynaud's phenomenon and acral ulcer) and polyarthralgia during methimazole therapy, and we detected cryofibrinogen in her plasma. Her symptoms resolved after administration of prostaglandins and anticoagulants. Several reports indicate that methimazole therapy induces autoantibody-related disease. In the present case, we cannot exclude the possibility that methimazole therapy contributed to the Cryofibrinogenemia.
(Internal Medicine 36: 439-442, 1997)

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A 63-year-old woman with pancreatic diabetes after a total pancreatectomy and splenectomy developed discitis of the L2/3 intervertebral disk. Rapidly she also developed infectious arthritis of the left knee joint and bacterial meningitis. Aspirate from the left knee contained Enterococcus faecalis. The diagnosis of discitis is generally difficult in the initial period of disease, and patients with diabetes or splenectomy are susceptible to rapid progression of the infection. Early diagnosis of discitis using magnetic resonance imaging of the spine and treatment with antibiotics might have altered her clinical course.
(Internal Medicine 36: 443-445, 1997)

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