Coronary artery spasm (coronary spasm) is an abnormal contraction of an epicardial coronary artery resulting in myocardial ischemia and its incidence is relatively high in Japanese as compared with Caucasians. Coronary spasm occurs most often from midnight to early morning when the patient is at rest and it is usually not induced by exercise in the daytime. Coronary spasm can be induced by acetylcholine, an endothelium-dependent vasodilator which causes vasodilatation in the normal coronary artery. Spasm artery is hyperresponsive to the vasodilator effect of nitroglycerin, an nitric oxide (NO) donor and is deficient in NO activity. The major risk factor for coronary spasm is cigarette smoking. Coronary spasm can be a cause of not only variant angina but also ischemic heart disease in general, including unstable angina, acute myocardial infarction and sudden ischemic death.
(Internal Medicine 36: 760-765, 1997)

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We examined the histological changes of the peribiliary glands (PBGs), a hitherto pooly recognized anatomical element around the biliary tree, in 7 cases of primary sclerosing cholangitis (PSC). These glands showed proliferation, and nonspecific inflammation with lymphoplasmacytic infiltration, fibrosis, and destruction. In addition, there were cystic lesions around the bile ducts, and they were considered to reflect dilatation of the PBGs. These changes were found around the intrahepatic and extrahepatic bile ducts in the cases examined. It is of interest that changes in the PBGs tended to correlate with the inflammatory changes of the bile duct wall itself, though 2 cases showed changes in the duct walls and PBGs unrelated to their distribution along the biliary tree. These findings suggest that the PBGs are also a target structure in addition to the bile ducts themselves in PSC.
(Internal Medicine 36: 766-770, 1997)

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We enrolled 22 patients with chronic obstructive pulmonary disease (COPD) and 20 normal subjects as controls. Using a hot-wire flow meter, we obtained tidal volume (V_T), duty ratio (Ti/Ttot), and mean inspiratory flow (V_T/Ti) as measures of respiratory pattern at two different respiratory rates; 0.5 Hz and 1.5 Hz. At 0.5 Hz, there were significant differences in Ti/Ttot and V_T/Ti. At 1.5 Hz, patients with COPD had significantly lower values of V_T and V_T/Ti. Furthermore, we calculated the change ratios from 0.5 Hz to 1.5 Hz in these parameters as new parameters of respiratory pattern change by respiratory rate. V_TO.5/1.5 and V_T/Ti_0.5/1.5 significantly correlated with FEV_1.0/FVC. The findings suggest that the parameters of the respiratory pattern may change depending on respiratory rates, and that the ratios of those parameters obtained at two different rates could be helpful in diagnosing airflow obstruction.
(Internal Medicine 36: 771-775, 1997)

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We measured the ventilatory responses and subjective sensations during arm exercise in patients with lower cervical and upper thoracic spinal cord injuries in order to evaluate the effects of chest wall deafferentation on these responses. Visual analog scales with verbal descriptors were used to quantify respiratory sensations of different affectional qualities. Patients as well as normal subjects reported stronger respiratory sensations upon CO₂ rebreathing as compared to during arm exercise with an equivalent minute ventilation (p<0.05). There were no qualitative nor quantitative differences in the respiratory sensations during CO₂ rebreathing between the patients and normal subjects. However, patients with spinal cord injuries showed a higher minute ventilation and a lower end-tidal PCO₂ during incremental arm exercises (p<0.01), and thus tended to hyperventilate. We conclude that chest wall afferent denervation does not contribute significantly to the perception of breathlessness in patients with spinal cord injuries.
(Internal Medicine 36: 776-780, 1997)

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The nature of the antibodies responsible for lupus erythematosus (LE) cells in systemic lupus erythematosus (SLE) remains obscure. We examined whether polyanion-restricted anti-histone antibodies were present in serum of patients with SLE using Western blotting analysis. Dextran sulfate or alginate was used as a polyanion compound in place of DNA. Antibodies which recognized dextran sulfate-histone complexes were present in serum of patients with SLE (17/34, 50%). These antibodies were detected in most SLE patients positive for LE cells (17/18, 94%) but not in those negative for LE cells or in patients with other collagen diseases. Similar results were obtained using alginate-histone complexes as antigens for Western blotting analysis. The antibodies to dextran sulfate-histone or alginate-histone complexes in serum of SLE patients were completely absorbed by treatment of serum with DNA-histone complexes, while they were unaffected by treatment with DNA only. The presence of antibodies to free histones and dextran sulfate-histone complexes did not seem to be related to the titer of anti-single stranded DNA antibody and anti-double stranded DNA antibody. We demonstrated the presence of polyanion-restricted antibodies in SLE, which may be responsible for the LE factor.
(Internal Medicine 36: 781-786, 1997)

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A 48-year-old male with type A atrophic gastritis developed multiple gastric carcinoids and a pituitary adenoma. Laboratory tests revealed high levels of serum gastrin and growth hormone (GH). He underwent subtotal gastrectomy, resulting in a return of the previously elevated gastrin level to normal. Serum GH concentration remained high. Three months after the surgery, the pituitary tumor, composed greatly of GH-immunoreactive cells, was partially removed. Since hypergastrinemia plays a pivotal role in gastric carcinoid formation and induces GH-releasing factor (GHRH) release resulting in GH-producing pituitary tumor formation, GH-producing pituitary adenoma might be a clinical manifestation in type A gastritis.
(Internal Medicine 36: 787-789, 1997)

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We report a rare case of portal-hepatic venous shunt through an enormous portal aneurysm complicated by pulmonary hypertension. A 66-year-old woman was admitted to our hospital for hepatic encephalopathy. Chest roentgenography revealed pulmonary hypertension. Computed tomography and ultrasound examination demonstrated a shunt between the portal and hepatic veins through an enormous portal aneurysm. The diagnoses of portal-hepatic venous shunt and pulmonary hypertension were confirmed by hepatic venous catheterization and cardiac catheterization. Pulmonary hypertension might result from the effects of vasoconstrictive agents, which should be metabolized by the liver in normal subjects, passing through the intrahepatic shunt into the lung.
(Internal Medicine 36: 790-793, 1997)

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A patient suffering from severe symptomatic primary pulmonary hypertension (PPH) underwent long-term intravenous prostacyclin therapy; the first time for such treatment in Japan. A 26-year-old male had experienced gradually progressive dyspnea for about one year. Despite conventional therapy he suffered repeated syncopal attacks. However, after receiving a permanent central venous access device and a portable infusion pump, he recovered fully and was discharged. This remedy seems to be promising for PPH as has already been proven in Europe and North Americas, although in Japan it is not as yet commercially available and some problems still need to be resolved.
(Internal Medicine 36: 794-798, 1997)

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A 31-year-old woman was admitted because of persistent remittent fever. Tricuspid valve endocarditis due to Staphylococcus aureus was identified as the cause of fever. The patient had no history of intravenous drug abuse, oral contraceptives or predisposing cardiac disease. Huge hepatomegaly was found without any signs of congestive heart failure. Liver enzyme abnormalities were not detected throughout the entire course of therapy. The liver biopsy specimen revealed peliosis hepatis. Treatment with panipenem/betamipron was successful, although recurrent septic pulmonary embolism occurred. The cause of the huge hepatomegaly encountered in the present case may be attributable to peliosis due to severe infection.
(Internal Medicine 36: 799-803, 1997)

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A 48-year-old woman with Cushing's syndrome due to bilateral adrenocortical adenomas is reported. The patient presented with a typical Cushingoid appearance. The serum cortisol level was elevated with loss of the diurnal rhythm and the plasma adrenocorticotropic hormone (ACTH) level was undetectable. Dynamic testing showed no suppression of urinary 17-OHCS by high-dose dexamethasone and no stimulation by metyrapone. An abdominal computed tomography (CT) scan showed bilateral adrenal tumors. Bilateral adrenalectomy was performed. The right adrenal gland contained a tumor that was encapsulated and consisted mainly of compact cells. The surrounding cortex was atrophic. The left adrenal gland contained an encapsulated tumor composed predominantly of clear cells. There were numerous small adrenocortical nodules in the surrounding cortex. Immunohistochemical analysis of steroidogenic enzymes (P450scc, 3β-HSD, P450c21, P450cl7 and P450cll) was performed. Immunoreactivity of all the enzymes was intense in the compact cells of the right adrenocortical adenoma, while the adjacent non-neoplastic cortex was negative for the enzymes. In the left adrenal tumor, the immunoreactivity of 3β-HSD was intense, while that of P450cl7 was weak. In the adrenocortical nodules, 3β-HSD activity was sporadically observed. G protein genes encoding Gs α and Gi2 were examined for activating mutations at codons 201 and 227 (Gs α) and codons 179 and 205 (Gi2 α) in the bilateral adrenal tumors, but no mutations were found. The bilateral adenomas of this patient showed marked differences in microscopic and immunohistochemical studies, suggesting that the capacity of steroidogenesis differs between the right and left tumors.
(Internal Medicine 36: 804-809, 1997)

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Pyogenic osteomyelitis is often accompanied by diabetes, but the disease in the clavicula has rarely been reported. We describe an unusual case of a 53-year-old man with poorly controlled noninsulin-dependent diabetes mellitus who presented with pyogenic clavicular osteomyelitis and developed DIC and acute renal failure. A ⁶⁷Ga scintigram revealed an abnormal accumulation of the isotope in the right clavicula, where magnetic resonance imaging (MRI) showed inflammatory changes. This suggests that a ⁶⁷Ga scintigram and MRI are of clinical value for the early diagnosis of the disease. Antibiotic chemotherapy with γ-globulin and gebexate mesilate, and hemodialysis almost cured his serious condition.
(Internal Medicine 36: 810-814, 1997)

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Hemorrhagic adrenal metastasis from lung cancer is extremely rare, although adrenal involvement is common in widely disseminated cancer. We report a case of massive adrenal hemorrhage secondary to metastasis of lung cancer. A 47-year-old female was treated by left upper lobectomy and mediastinal lymph node resection for an adenocarcinoma with intrapulmonary metastasis in the left upper lobe. Eight months later, she presented with right flank and back pain, and abdominal ultrasonography and computed tomography showed a right solitary adrenal tumor with massive hemorrhage. The tumor was not resectable and partially responded to chemotherapy. A massive adrenal hemorrhage, secondary to metastasis of lung cancer, presents with nonspecific clinical signs and symptoms. In lung cancer patients with an acute flank or back pain, hemorrhagic adrenal metastasis should be considered in the differential diagnosis.
(Internal Medicine 36: 815-818, 1997)

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A 57-year-old female was admitted to our hospital because of Philadelphia chromosome (Ph)-positive acute lymphoblastic leukemia (ALL). On admission, disturbance of consciousness and hyponatremia were recognized. The patient's endocrinological data showed low levels of adrenocorticotropic hormone (ACTH) (less than 5 pg/ml) and cortisol (5.9 μg/dl). Other anterior pituitary hormones were normal. Plasma ACTH and cortisol did not respond to the corticotropin releasing factor (CRF) test. A diagnosis of isolated ACTH deficiency was made. This is a rare case of isolated ACTH deficiency complicated with hematological malignancies.
(Internal Medicine 36: 819-821, 1997)

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A 39-year-old man with Bowen's disease was troubled with multiple molluscum contagiosum over the trunk and lower extremities. Subsequently oral candidiasis was complicated. Laboratory examination revealed lymphocytopenia and a decrease in the CD4/CD8 ratio. His CD4⁺ T-lymphocyte count was only 187 cells/μl one time and 222 cells/μl another time. No evidence for human immunodeficiency virus (HIV) infection was found. He had no family history of immunodeficiencies.
(Internal Medicine 36: 822-824, 1997)

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In a case of neurosarcoidosis with bilateral facial nerve palsy and hydrocephalus, contrastenhanced magnetic resonance imaging (MRI) study and angiotensin converting enzyme (ACE) activities in cerebrospinal fluid (CSF) were valuable for the diagnosis and the follow up. Facial nerve lesions were demonstrated on gadolinium-DTPA enhanced MRI. The disappearance of enhancement was concomitant with the amelioration of facial nerve palsy after corticosteroid therapy.
(Internal Medicine 36: 825-828, 1997)

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We report a patient with peripheral neuropathy caused by cisplatin for the treatment of testicular tumor. Routine studies of nerve conduction and somatosensory evoked potentials demonstrated large myelinated fiber neuropathy suggesting ganglioneuronopathy. We also performed a CO₂ laser evoked potential study, and found that small myelinated fibers, which are related to pain sensation, were well preserved in this patient.
(Internal Medicine 36: 829-833, 1997)

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A 47-year-old man was admitted because of acute lateral medullary syndrome with severe posterior cervical pain. Cerebral angiography was performed three hours after the onset, which demonstrated that two arteries branched separately from the right subclavian artery, ran upward and formed a single right vertebral artery (VA). One of the two arteries showed both stenosis and luminal dilatation. We thought the structure of these arteries was proximal vertebral artery anomaly and diagnosed him as having dissection of the vertebral artery. We consider that the proximal vertebral anomaly may be a risk for spontaneous VA dissection.
(Internal Medicine 36: 834-836, 1997)

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Polymyalgia rheumatica (PMR) and exacerbated pulmonary fibrosis presented concurrently in a 69-year-old woman with a 5-year history of idiopathic interstitial pneumonia. The radiographic and histological examinations suggested usual interstitial pneumonia (DIP) as a more likely diagnosis. Corticosteroid therapy resulted in relief of the patient's muscle symptoms and improvement in the functional and radiographical signs of the pulmonary fibrosis. The final diagnosis was pulmonary fibrosis associated with PMR, because PMR is believed to be one of the causes contributing to interstitial lung diseases.
(Internal Medicine 36: 837-840, 1997)

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A 47-year-old woman developed pulmonary hemorrhage and an increase in proteinuria during remission of membranous nephropathy. Renal biopsy revealed crescentic glomerulonephritis. She also had a high perinuclear antineutrophil cytoplasmic antibody level, so a diagnosis of myeloperoxidase-antineutrophil cytoplasmic antibody-associated glomerulonephritis was made. After immunosuppressive therapy was started, the pulmonary hemorrhage resolved and her proteinuria decreased. Renal biopsy was repeated after treatment and showed histological improvement. This case suggests that there may be a relationship between membranous nephropathy and myeloperoxidase-antineutrophil cytoplasmic antibody-associated glomerulonephritis.
(Internal Medicine 36: 841-846, 1997)

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