Guanylin, a peptide homologue of the bacterial heat-stable enterotoxins, is an endogenous activator of guanylate cyclase C (GC-C). We determined the tissue content and plasma concentration of human guanylin, and its cellular source in the intestine. Human guanylin is distributed widely from the duodenum to the rectum, the highest content being in the ileum and proximal colon. The plasma concentration of immunoreactive guanylin in the normal individuals tested was 30.3±3.7 fmol/ml (mean±SE) and that in patients with chronic renal failure was elevated with increasing serum creatinine concentration. Guanylin immunoreactivity was detected in the villus epithelial cells in the small intestine and these guanylin-containing cells were increased in number along the cephalocaudal axis of the gut. Guanylin was also present in Paneth cells in the small intestine and superficial epithelial cells in the large intestine. Guanylin mRNA was detected in the intestine by the reverse transcription-polymerase chain reaction. Guanylin may have paracrine action on neighboring enterocytes, activating intestinal guanylate cyclase and thereby regulating intestinal fluid as well as electrolyte transport through the second messenger, cyclic GMP.
(Internal Medicine 35:171-175, 1996)

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We studied the clinical features of minocycline-induced pneumonitis in seven patients. Acute symptoms included fever, dry cough and dyspnea, indicating acute respiratory failure. Diffuse ground glass shadows with Kerley's B lines, bronchial wall thickening, swelling of vascular bundles and pleural effusion were visible on radiography. Bronchoalveolar lavage or transbronchial lung biopsy confirmed pulmonary eosinophilia. Cessation of minocycline led to rapid remission with no treatment or only short-term steroid therapy. The lymphocyte stimulation test for minocycline with peripheral blood lymphocytes was not found to be useful for diagnosis.
(Internal Medicine 35:176-179, 1996)

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We evaluated the effects of 13-cis retinoic acid (13-cis RA) on the growth of peripheral blood mononuclear cells (PBMC) obtained from 12 patients with adult T cell leukemia (ATL). In general, 13-cis RA potently inhibited the growth of PBMC from ATL patients. However, the sensitivity of the cells to 13-cis RA-induced growth inhibition varied among the patients. The ATL patients fell into three groups (hypersensitive, sensitive and resistant to 13-cis RA) according to the percent reduction of ³H-thymidine incorporation before and after treatment with 13-cis RA. Agarose gel electrophoresis of total genomic DNA from a patient sensitive to 13-cis RA provided evidence of the DNA fragmentation indicative of apoptosis. The ability of 13-cis RA to induce apoptosis in PBMC from ATL patients suggests that retinoids may be useful in the treatment of ATL.
(Internal Medicine 35:180-184, 1996)

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Sick sinus syndrome is a rare but potentially important cardiac disorder in patients with myotonic dystrophy. We evaluated 3 patients with myotonic dystrophy complicated with sick sinus syndrome using intracardiac electrocardiography and endomyocardial biopsy. Electrocardiography identified sinus arrest, atrial flutter and right bundle-branch block in 2 cases and marked sinus bradycardia and first-degree atrioventricular block in 1 case. Their sinus node recovery times were significantly prolonged as demonstrated by the overdrive suppression test. Two patients had Adams-Stokes syndrome and one had tachycardia with severe palpitations. Therefore permanent pacemaker implantation was indicated in all 3 cases. Light microscopic analysis of right ventricular endomyocardial biopsies showed vacuolar degeneration and nuclear deformity of cardiomyocytes in all cases and endocardial and interstitial fibrosis in 1 case. These findings indicate that pathological changes may occur in any part of the myocardium in patients with myotonic dystrophy.
(Internal Medicine 35:185-188, 1996)

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In skeletal muscles of patients with Duchenne muscular dystrophy (DMD), the absence of dystrophin was thought to lead to the large reduction in all of the dystrophin-associated proteins (DAPs). Of the seven types of DAPs identified in skeletal muscle, only the 43-kDa glycoprotein (β-dystroglycan) has recently been found in the monkey brain. To clarify the distribution and characterization of dystrophin and β-dystroglycan in the brain of humans, we carried out immunostaining and immunoblotting studies on tissues from three DMD patients with intellectual disturbances (ages 17, 22, and 26 year) and in five controls (age range, 42-74 year). An antidystrophin antibody revealed dystrophin to be localized in neuronal cells and in the vascular wall in control brains, but it was absent from these tissues in DMD patients. In contrast, β-dystroglycan was distributed throughout neuronal cells and in the vascular wall of control brains, and was well preserved in the brain of patients with DMD.
(Internal Medicine 35: 189-194, 1996)

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We previously reported increased adherence of T cells to human endothelial cells (EC) in patients with HTLV-I-associated myelopathy (HAM). To define the immunological function of EC-adherent T cells from HAM patients, we investigated the degree of spontaneous proliferation and the production of inflammatory cytokines, such as tumor necrosis factor-α (TNF-α), interferon-γ (IFN-γ) and granulocyte-macrophage colony stimulating factor (GM-CSF). Both the degree of spontaneous proliferation and the production of TNF-α, IFN-γ and GM-CSF by EC-adherent T cells of HAM patients were significantly increased, compared to anti-HTLV-I seronegative controls. Furthermore, in HAM patients, spontaneous proliferation and production of inflammatory cytokines by EC-adherent T cells were significantly higher than that of EC-non-adherent T cells. Conversely, those functions of EC-non-adherent T cells were significantly lower than that of unseparated cells, which were T cells before application to EC. We demonstrated that EC-adherent T cells were qualitatively and quantitatively more hyperactive than those of anti-HTLV-I seronegative controls and the population of activated T cells of HAM patients was concentrated in EC-adherent T cells rather than in EC-non-adherent T cells. Our results suggest that EC-adherent T cells in the peripheral blood of HAM are intimately involved in the immunopathogenesis of HAM.
(Internal Medicine 35:195-199, 1996)

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Two sisters with Ehlers-Danlos syndrome, inherited as an autosomal recessive trait, and congenital heart disease are herein reported. One was a 20-year-old woman with Ehlers-Danlos syndrome and multiple aphthous stomatitis, bronchial asthma, an emphysematous lung, a ventricular septal defect and a bilateral inguinal hernia due to hyperextensibility and joint hypermobility. The other was a 17-year-old girl with the same syndrome and an atrial septal defect, a ventricular septal defect and patent ductus arteriosus. The combination of Ehlers-Danlos syndrome and congenital heart anomalies in these siblings suggest a common genetic defect to be the cause of these diseases.
(Internal Medicine 35: 200-202, 1996)

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We report a case of γ-streptococcal tricuspid valve endocarditis in a patient with no history of intravenous drug abuse. Echocardiography revealed large vegetations on the anterior and septal cusps. The patient had persistent fever and recurrent septic pulmonary embolism despite prolonged antibiotic therapy. However tricuspid valve replacement was successful.
(Internal Medicine 35: 203-206, 1996)

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A 56-year-old male patient with cyclic Cushing's disease remained in a state of remission for more than one year with a relatively low dose of bromocriptine (2.5-3.75 mg/day). It has been reported that bromocriptine treatment for cyclic Cushing's disease induces only a transient remission; in the most effective cases, a relatively high dose (40 mg/day) was necessary. In the hypercortisolemic state, plasma adrenocorticotropic hormone (ACTH) and serum cortisol were not suppressed by dexamethasone and did not respond to corticotropin-releasing factor (CRF). An antehypophysectomy was not effective, even though the resected tissue contained ACTH-positive microadenomas. The present observations thus indicate the effectiveness of bromocriptine for some patients with this rare disorder.
(Internal Medicine 35: 207-211, 1996)

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A patient with complaints of high fever and left shoulder pain was found to have a large mass in the left upper lobe on chest roentgenogram. Laboratory evaluation revealed marked thrombocytosis, hypoalbuminemia, and increased serum concentrations of CRP, fibrinogen and interleukin6 (IL-6). A transcutaneous biopsy specimen revealed large cell carcinoma. Tumor production of IL6 was confirmed by immunohistochemical staining with an anti-human IL-6 monoclonal antibody (MH60).
(Internal Medicine 35: 212-214, 1996)

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Thymic carcinoma was found in a thymic cyst in a 60-year-old woman who was admitted to our hospital due to cough and pain in her right chest. Chest X-ray showed a huge shadow in the right mediastinum. Chest CT scan showed a cystic tumor with a solid mass. An operation revealed a solid tumor in the thymic cyst. Pathologic diagnosis was squamous cell carcinoma, that contained some glandular tissues. The patient has been in a good condition for two years after the operation. Although the concurrent occurrence of thymic carcinoma and thymic cyst is very rare, it should be added to the differential diagnosis when anterior mediastinal cystic tumor is associated with a solid mass lesion.
(Internal Medicine 35: 215-218, 1996)

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We describe a case with chronic bronchial foreign body presenting with recurrent hemoptysis mimicking a peripheral lung tumor, and the outcome of surgical resection. A 61-year-old female with recurrent hemoptysis had a peripheral nodule in the right lower lobe. A right bronchial arteriogram showed dilatation and hypervascularity in the nodule. Surgical removal of the nodule contained a small branch of a white cedar. Chronic bronchial foreign body, while rare in adults, should be considered in the differential diagnosis of peripheral lung tumors associated with recurrent hemoptysis.
(Internal Medicine 35: 219-221 1996)

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A 29-year-old woman with muscle phosphofructokinase (PFK) deficiency had exercise intolerance, painful cramps, elevation of muscle enzyme levels in the serum and compensated hemolysis. After the restriction of exercise, the creatine kinase level and indirect bilirubin level decreased, and the reticulocyte count and haptoglobin level were normalized. It is suggested that the hemolysis which was accelerated by exercise was improved by restriction of exercise.
(Internal Medicine 35: 222-226, 1996)

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Serial electroencephalograms (EEGs) and somatosensory evoked potentials (SEPs) were carried out in a 36-year-old man with isolated ACTH deficiency. Before treatment with glucocorticoids, the EEGs displayed bilateral diffuse 5-6 Hz theta waves, and SEPs demonstrated a decreased amplitude. Following treatment with glucocorticoids, the EEGs showed gradual improvement; a diffuse alpha pattern was demonstrated on the 11th day after treatment and an occipital dominant alpha pattern was found on the 83rd day after treatment. After treatment, SEPs also showed improvement. The amplitude of N20 increased and N32 became clearly recognizable. The influence of glucocorticoids on EEGs and SEPs was discussed in the context of these observations.
(Internal Medicine 35: 227-230, 1996)

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Bronchiolitis obliterans organizing pneumonia (BOOP) occurred in a 53-year-old woman with well-documented Sjögren's syndrome (SjS) and polymyositis (PM). BOOP has often been reported as a pulmonary manifestation of collagen vascular diseases, mainly rheumatoid arthritis (RA), but the association of BOOP and PM has rarely been documented. A search of the literature showed only 16 case reports of BOOP associated with polymyositis-dermatomyositis (PM-DM). It is interesting that BOOP occurred prior to PM-DM, while it is commonly believed to occur after RA.
(Internal Medicine 35: 231-235, 1996)

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