We determined the circadian variations and prednisolone (PSL)-induced alterations of circulating lymphocyte subsets in 10 healthy adults by two-color flow cytometry using monoclonal antibodies to various lymphocyte subsets in order to collect fundamental data for monitoring of the subsets in clinical practice. This study first examined the changes of CD5+ B cells, γδ+ or γδ- T cells, activated (HLA-DR+) CD4+ or CD8+ cells, CD11b+ or CD11b-CD8+ cells, and natural killer (NK) cell subsets (CD16+CD57-, CD16+CD57+, CD16-CD57+), in addition to other subsets described before. Compared with the base line values obtained at 9:00 (AM) on day 1, lymphocytes, total B cells, CD5+ B cells, total T cells, γδ- T cells, CD4+ cells, activated CD4+ cells, CD45RA-CD4+ cells, and activated CD8+ cells were significantly increased at 20:00 (PM). However, the numbers of CD45RA+CD4+ cells, CD11b+ or CD11b-CD8+ cells and three NK cell subsets did not show significant circadian variations. After oral PSL (30 mg), which was given at 7:00 (AM) on day 2, lymphocytes and almost all lymphocyte subsets, except for CD16+CD57- cells, were significantly decreased; these changes recovered between 13 and 26 hours after PSL administration. The circadian variations and PSL-induced alterations of lymphocyte subsets were relatively comparable, but PSL administration cause a decrease in a wider range of lymphocyte subsets including relatively corticosteroid-resistant subsets such as CD45RA+CD4+ cells, CD8+ cell and NK cell subsets. Thus, these alterations of lymphocyte subsets should be taken into account in the evaluation of patients with immunologic abnormalities, especially those receiving PSL treatment.
(Internal Medicine 33: 733-738, 1994)

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Phytohemagglutin in (PHA)-induced IL-2 production in vitro by peripheral blood mononuclear cells (PBMC) and lamina propria mononuclear cells (LPMC) from patients with active UC (n=24, n=8, respectively) was significantly less than that in controls (n=13, n=8, respectively) and inactive patients (n=11). In contrast, PBMC from inactive disease showed no significant difference when compared with the controls. Depressed IL-2 production in active UC was not reversed by the addition of anti-CD3 monoclonal antibody plus phorbol myristate acetate (PMA), but was largely reversed by adding calcium ionophore plus PMA. Using a fluorescent Ca²⁺ probe fura-2, we found that after PHA stimulation LPMC from patients with active UC showed a lower magnitude of rise in intracellular free calcium concentration ([Ca²⁺]_i) than control cells. These results suggest that impaired PHA-induced IL-2 production in active UC may be related to some alterations of the early signaling events that cause elevation of the [Ca²⁺]_i.
(Internal Medicine 33: 739-744, 1994)

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The aim of this study was to determine whether endoscopic variceal sclerotherapy affects systemic or splanchnic hemodynamics. We measured hemodynamic parameters before and after the first course of sclerotherapy in 35 patients with cirrhosis. Following sclerotherapy, there was a significant decrease in cardiac index and a significant increase in systemic vascular resistance. Changes in hepatic venous pressure gradient varied from patient to patient with no statistically significant change in the group overall. However, all 20 patients with a decline in the hepatic venous pressure gradient had a concomitant decrease in cardiac index and/or a large extravariceal shunt. The multivariate analysis disclosed that the decrease in cardiac index was a statistically significant contributor for the decline in hepatic venous pressure gradient. We conclude that the obliteration of esophageal varices by sclerotherapy significantly reverses the hyper dynamic circulatory state in patients with cirrhosis. Spontaneous changes in systemic hemodynamics and the interaction with hepatic hemodynamics must be taken into account when evaluating hepatic hemodynamics in patients undergoing variceal sclerotherapy.
(Internal Medicine 33: 745-751, 1994)

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We describe two siblings affected by chronic relapsing thrombotic thrombocytopenic purpura from infancy. The elder brother, a 12-year-old boy had 50 such episodes characterized by acute onset of fever, headache, drowsiness, vomiting, dark urine, thrombocytopenia and anemia. The younger sister, a 6-year-old girl, had 8 episodes with the same clinical manifestations. Petechiae and ecchymoses on the extremities were present throughout their lives. Furthermore, anemia with evidence of red blood cell fragmentation and thrombocytopenia were present chronically. Periodical transfusion of frozen fresh plasma prevented recurrent episodes. These cases suggest that there is a congenital variant of thrombotic thrombocytopenic purpura.
(Internal Medicine 33: 752-758, 1994)

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A 14-year-old female with ulcerative colitis developed right anterior cervical pain and high fever. Cervical contrast-enhanced computed tomography (CE-CT) showed a wall thickness of the right common carotid artery which suggested aortitis. Her pulmonary angiography demonstrated a narrowing of the pulmonary arteries and she was diagnosed as having Takayasu's disease associated with ulcerative colitis. HLA analysis showed Bw52 and DR2 haplotype, which is frequently found in patients with Takayasu's disease associated with ulcerative colitis.
(Internal Medicine 33: 759-763, 1994)

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A 70-year-old woman with rheumatoid arthritis (RA) and secondary amyloidosis presented repeated consciousness loss. The pathological findings at autopsy revealed multi-organic deposits of amyloid A-protein and so-called 'plexiform change' of blood vessels in bilateral temporal lobes. The arterial plexiform change, which is found in the lung specimen of primary pulmonary hypertension, might be a new pathological cerebrovascular change associated with RA.
(Internal Medicine 33: 764-767, 1994)

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A 57-year-old female patient with Sjögren's syndrome was complicated with pulmonary hypertension (PH) and antiphospholipid antibody (aPL). She had a history of fetal losses, deep vein thrombosis and chronic thyroiditis. On admission, severe pulmonary hypertension, thrombocytopenia, lupus anticoagulant and a decreased level of protein C were found. Pulmonary artery perfusion scintigram revealed multiple defects. She died suddenly despite an intensive therapy. Intimal proliferation with angiomatoid lesions in small pulmonary arteries was observed by autopsy. Since a close relationship between PH and aPL in connective tissue disease is found, it is important to carefully analyze the antiphospholipid antibodies in patients with PH.
(Internal Medicine 33: 768-772, 1994)

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This is the first report on a case of syndrome of inappropriate secretion of antidiuretic hormone (SIADH) associated with Gerhardt syndrome (paralysis of bilateral vocal cords). A 67-year-old Japanese man suffering from progressive autonomic failure was diagnosed as having Shy-Drager syndrome (SDS) with hyponatremia due to SIADH and severe sleep apnea caused by a bilateral recurrent nerve palsy. Water load test showed alteration in diuresis which was corrected by phenytoin. Arginine vasopressin secretion was not suppressed by plasma osmolality below 280 mOsm/kgH₂O. Impairment of the afferent pathways of baroreceptors, or impairment of the osmoreceptors could be speculated as the etiological factor of the SIADH observed in this case.
(Internal Medicine 33: 773-778, 1994)

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A 29-year-old female was admitted to the hospital because of increasing exertional dyspnea, chest oppressive feeling and palpitation. Lung perfusion scan, pulmonary angiography, and venography of the lower extremities revealed multiple pulmonary embolism and a right popliteal venous aneurysm as the probable source of emboli. B-mode/Doppler echography and magnetic resonance imaging were useful for the preoperative evaluation. Following anticoagulant therapy and thrombolytic therapy using intravenous heparin and urokinase, respectively, the popliteal venous aneurysm was surgically excised; the walls contained organized thrombus. She delivered a baby without any complication 2 years after the surgery.
(Internal Medicine 33: 779-782, 1994)

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Pleural effusion is a common finding of peripheral neuroectodermal tumor (PNET) of the chest wall (Askin's tumor), but little is known about the characteristics. A case of Askin's tumor with pleura! effusion is reported. Repeated cytologies were negative for malignancy, but levels of lactic dehydrogenase (LDH) and neuron-specific enolase (NSE) in the pleural effusion were increased. Surgical biopsy was performed and immunohistochemical study of the tumor revealed the diagnosis.
(Internal Medicine 33: 783-785, 1994)

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We sequentially analyzed the immunoglobulin heavy chain (IgH) variable region gene of leukemia cells obtained from a chronic myeloid leukemia (CML) patient who had three episodes of B-lymphoid crisis after bone marrow transplantation. Southern blots using the JH probe showed a single rearranged band which differed at each crisis, although the rearranged bands of the BCR gene were the same at each crisis. The IgH variable region sequences of the leukemia cells at each crisis were different. These observations suggested that multiple clones were generated from the progenitor cells of the blast crisis, which were transformed at a very early stage of B-lymphocyte ontogeny.
(Internal Medicine 33: 786-789, 1994)

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We describe here a typical case of virilizing adrenocortical tumor. A 23-year-old Japanese woman had her male-like musculature, hirsutism, the absence of breast development and marked clitoromegaly. Adrenal androgens were remarkably elevated, with plasma dehydroepiandrosterone sulfate 2, 752 μg/dl, plasma testosterone 250 ng/dl and urinary 17-ketosteroids 203.4 mg/day. A well-encapsulated tumor approximately 7 cm in diameter was detected in the left adrenal gland by computed tomography, magnetic resonance imaging and arteriography. The tumor was surgically resected and histologically diagnosed as a benign adrenocortical adenoma. The elevated adrenal androgens returned to normal postoperatively with amelioration of her masculinized clinical features.
(Internal Medicine 33: 790-794, 1994)

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An unusual case with metastatic spread of a small oat cell lung carcinoma to the hypothalamo-pituitary region and pineal body presenting as pituitary insufficiency is reported. Computed tomographic scan revealed an isodense mass in the hypothalamo-pituitary region and pineal body that was strongly enhanced by contrast medium. Magnetic resonance imaging demonstrated a downward displacement of the posterior lobe of the pituitary by the hypothalamo-pituitary mass. Dynamic assessment of hypothalamo-pituitary function showed hypopituitarism and diabetes insipidus; partial recovery was observed with the improvement in the carcinoma by chemotherapy. The brain was subsequently irradiated. The patient survived 10 months.
(Internal Medicine 33: 795-798, 1994)

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Guillain-Barré syndrome (GBS) developed soon after the onset of acute viral hepatitis A (HA) in a 62-year-old man. GBS associated with HA is extremely rare, even though HA is common. In a review of case reports the clinical features of GBS following HA could be summarized as follows: 1) Most of the patients are men. 2) GBS develops within 14 days after the onset of HA. 3) Facial nerve palsy is frequently present. 4) Proprioception is likely impaired in addition to superficial sensation. 5) The outcome of neuropathic symptoms is uniformly good, regardless of the degree of liver dysfunction as evaluated on the basis of alanine aminotransferase levels. These findings indicate that GBS following HA essentially does not differ from typical GBS.
(Internal Medicine 33: 799-801, 1994)

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A 74-year-old woman with erythropoietic protoporphyria (EPP) with hepatic dysfunction is reported. She had been photosensitive for two years prior to admission. Physical examination revealed hepatosplenomegaly and erosions on her face. Moderate increases in serum bilirubin and biliary tract enzymes were noted. Histology of the biopsied liver revealed moderate fibrosis and dilatation of bile canaliculi containing orange pigment. Marked increases in protoporphyrin in erythrocytes and feces were noted. The patient died of hepatic failure a year and a half after admission with maximum serum bilirubin of 34 mg/dl. This patient seems to be the oldest among reported EPP cases with liver dysfunction.
(Internal Medicine 33: 802-805, 1994)

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A 54-year-old man with renal cell carcinoma was treated with interferon (IFN)-gamma for 3 weeks soon after nephrectomy. Three months later he received IFN-alpha therapy for 8 weeks due to chronic active hepatitis C. He subsequently contracted polymyositis (PM): proximal muscle weakness, an elevation of muscle enzymes, myogenic patterns on the electromyograph and histologically specific findings in biopsied muscle specimens. After discontinuation of IFN his muscular weakness gradually recovered.
(Internal Medicine 33: 806-808, 1994)

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A 74-year-old female patient with cutaneous ulcerations and typical dermatomyositis (DM) skin rash had no muscle disease for a 1-year and 5 months period. Histological examination of the skin ulceration indicated vascular occlusion without cellular infiltration. Cutaneous ulceration is a very rare manifestation of adult-onset DM patients without inflammatory myopathy.
(Internal Medicine 33: 809-812, 1994)

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A patient with insulinoma had frequent hypoglycemic episodes with normal plasma insulin levels and insulin/glucose ratios. When immunoreactive insulin (IRI) concentrations in this patient were compared among plasma samples with the same C-peptide immunoreactivity (CPR) levels, the concentrations were significantly lower than in control patients with insulinoma and equal to or lower than those of normal subjects. In hepatic venous samples, CPR levels were significantly higher and the IRI/CPR molar ratios were lower than those in a control subject. These results may indicate that normoinsulinemia in this patient could be explained by increased hepatic extraction of insulin.
(Internal Medicine 33: 813-816, 1994)

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