The effects of acute renal failure (ARF) on somatosensory evoked potentials (SEPs) were studied in rats. With the increase in blood urea nitrogen caused by the ligation of bilateral ureters, both a significant augmentation of amplitudes of the SEP and an increase in the SEP latencies were observed. The nerve conduction velocities of peripheral nerves were unchanged. Thus a kind of cortical irritability and a lesser degree of damage in the peripheral nervous system are characteristics in ARF.

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The serum and urinary levels of electrolytes were measured in 25 patients with anorexia nervosa admitted to this hospital. Seven (28%) of these patients vomited, usually surreptitiously. Hypokalemia was detected in 5 (20%) of these patients, all among those who vomited. The urinary chloride/sodium ratios were low in the patients who vomited, and did not overlap the values in patients who did not vomit, indicating that this ratio was a good indication of vomiting. These results suggested that stopping vomiting is most important for correcting hypokalemia.

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Fourteen patients suffering from severe heart failure with 18 mmHg or higher diastolic pulmonary arterial pressure were given a transdermal therapeutic system of nitroglycerin (TTS-NTG). They were evaluated for changes in the hemodynamic responses over 24 hours. Diastolic pulmonary arterial pressure decreased from 27.1±2.3 mmHg (mean±SE) to 22.4±1.7 mmHg after 1 hour (p<0.01), which was maintained throughout the trial. Cardiac index increased from 2.42±0.13 l/min/m² to 2.64±0.16 l/min/m² after 1 hour (p<0.01). The analysis of cardiac and vascular function curves in individual patients suggested that the improvement of hemodynamics was induced mainly in six patients with an increase of contractility and in four patients with a reduction of afterload. No changes were observed in three patients in either contractility or afterload, and a decrease in contractility was seen in one patient. These results suggest that TTS-NTG can be transcutaneously absorbed well enough to produce improved hemodynamic responses in patients with severe heart failure by several mechanisms and maintain these effects over 24 hours.

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A 65-year-old woman had chronic myelomonocytic leukemia with peripheral neuropathy and IgA paraprotein with κ type light chain. Plasma cells with "flaming" cytoplasma were seen in the bone marrow specimens. The findings suggest that chronic myelomonocytic leukemia may involve B-lymphocytes in the proliferative process and that it may be a clonal disease. Moreover, this case was associated with peripheral sensori-motor polyneuropathy. The disorder was responsive to prednisolone, as oral administration of prednisolone improved not only the peripheral polyneuropathy, but also the hematological findings. Serum vitamin B₁₂ and lysozyme in the serum and urine were decreased by the steroid therapy. The administration of prednisolone is effective not only for polyneuropathy but also chronic myelomonocytic leukemia.

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Chronic neutrophilic leukemia (CNL) is a rare type of leukemia. We diagnosed a 81-year-old woman as CNL because she showed that sustained leukocytosis dominated by mature neutrophils, hepatosplenomegaly, high neutrophilic alkaline phosphatase (NAP) score, absence of the Ph¹ chromosomeand no evidence of leukemoid reaction. During the clinical course, she did not manifest hemorrhagic tendency or infection. We also examined neutrophilic function including chemotaxis, chemiluminescence, nitroblue tetrazolium (NBT) dye reduction, which all indicated normal neutrophil function. Using a reversed phase-high pressure liquid chromatography (HPLC), we detected the production of leukotriene B₄ (LTB₄) in neutrophils. We found that the LTB₄ production was decreased in neutrophils whereas they showed normal chemotaxis. This discrepancy has never, to our knowledge, been reported before in case of CNL.

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Hypercalcemia and leukocytosis of malignancy have been highlighted over a decade. We report a case of a gallbladder cancer with marked hypercalcemia and leukocytosis. A 54-year-old woman was admitted to the hospital because of remittent fever and left hypochondric pain. The computed tomographic scan of the abdomen revealed the cancer of the gallbladder with liver metastases. The patient's medical condition deteriorated as the tumor was rapidly growing up. Her medical course was marked by hypercalcemia and an increase in mature neutrophils. Medical therapy with normal saline, furosemide, indomethacin, prednisolone, and calcitonin failed to ameliorate hypercalcemia. On the twenty-ninth hospital day the serum calcium was elevated to 17.6 mg/dl which responded to 1000 μg of mithramycin while leukocytosis continued. Despite the chemotherapy with doxolubicin and tegafur, the tumor continued to grow. Leukocytosis was attributed to the elevated colony-stimulating factor activity which was two-fold of control. The parathyroid hormone and nephrogenous cyclic AMP levels were normal with low vitamin D levels. Hypercalcemia was attributed to a parathyroid hormone-like substance because of a decrease in %TRP in the presence of normal renal function and the normal parathyroid hormone level. Autopsy revealed an undifferentiated adenocarcinoma of the gallbladder with multiple liver metastases, and bone resorption in the vertebral column and sternum without evident bone metastasis.

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The first case of isolated thyrotropin (TSH) deficiency with Cushing's syndrome was reported. A 46-year-old female was admitted to our hospital because of fatigability, puffy eye-lids, leg edema and petechia. She was treated with thyroid hormone. A low free triiodothyronine continued. Serum TSH was undetectable and serum thyrotropin releasing hormone (TRH) was elevated. No response of serum TSH and normal response of serum prolactin after administration of exogeneous TRH suggested the abnormalities of anterior pituitary gland. The secretion of growth hormone and gonadotropin were intact, but the secretion of adrenocorticotropic hormone (ACTH) was impaired. A high level of serum cortisol, no response of serum ACTH by metyrapone test and a tumor mass of abdomen detected by computed tomography led to the diagnosis of Cushing's syndrome. Histological examination of the tumor revealed adrenal adenoma. Twenty two months after the surgery, serum ACTH level was normalized, but the secretion of serum TSH to exogeneous TRH was still absent. The results indicated that hypothyroidism in our patient was due to isolated TSH deficiency.

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The sputum lith, 1 to 3 mm in diameter, were examined by microanalyser and by the method of X-ray diffraction, which revealed that the lith was composed of calcium carbonate and calcite in crystalline style. A composition of bronchial liths is often associated with different underlying diseases so that these nondestructive analyses seem to provide useful information for differential diagnosis. The present case is the first one to expectorate bronchial lith without marked pulmonary diseases.

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We report an Rh(D)-negative man with myelodysplastic syndrome who produced six anti-erythrocyte alloantibodies (anti-D, -C, -E, -Di^a, -Jk^a and -S) in succession. Three of these antibodies (anti-E, -Jk^a and -S) were not noted until delayed hemolytic transfusion reactions occurred. Treatment with cortico-steroids was effective in preventing both further formations of antibodies and other transfusion reactions. It was very difficult to find blood compatible with the patient, but repetitive blood transfusions were required for his progressive anemia and thrombocytopenia. Several problems concerning the transfusion of blood in such a case are discussed.

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A 62-year-old male Japanese, a heavy drinker for his last 40 years, was admitted to our hospital because of unstable gait of acute onset. Despite large doses of thiamine injection, his condition rapidly worsened with confusion, and died 15 days after the onset. During the deteriorating course, a series of Computerized Tomography (CT) and Magnetic Resonance Imaging (MRI) scans were taken of the brain, which revealed progressive multifocal involvement in the corpus callosum; Marchiafava-Bignami disease was suggested. To the authors' best knowledge, there have been no MRI examinations conducted from so early a stage of this disease on. MRI scanning proves to be a highly useful tool for early diagnosis of Marchiafava-Bignami disease.

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A 17-year-old patient with mixed gonadal dysgenesis (MGD) showing ambiguous genitalia and hypergonadotropic hypogonadism was described. By intraabdominal exploration, a poorly developed uterus with a fallopian tube and a streak gonad was found on the right side and a poorly developed testis with epidydimis and vas deferens on the left. Chromosomal analysis on cultured peripheral lymphocytes and bone marrow cells showed 45, X karyotype, while among the majority of 45, X + cells small numbers of 46, X + mar cells (3-23%) were found in cultured fibroblasts from the abdominal skin and various organ tissues. We compared our patient with the Japanese patients with MGD reported in the literatures.

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We describe two patients in encephalitis with opsoclonus-myoclonus syndrome. Cranial magnetic resonance imaging demonstrated an area of hyperintensity on the T2-weighted images in the left tegmentum at the pons level (case 1) and in the left tegmentum and partly basis at the upper pons level (case 2), respectively. On the other hand, brainstem auditory evoked potentials following left monaural stimulation and ipsilateral records revealed a delay of IIIrd wave latency and prolongation of I-III interpeak latencies (case 1). From these findings we suggest that the locus of the lesion of opsoclonus-myoclonus syndrome may partly exist in the brainstem (pontine tegmentum).

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A 35-year-old man was admitted because of loss of hearing in the left ear. The patient had been known to have familial hypercholesterolemia for at least 12 years. Computerized axial tomography of the brain showed a large tumor occupying in the left mastoid region. Surgical intervention revealed xanthogranuloma, histologically. Xanthogranuloma is classified as a kind of normocholesterolemic xanthomatoses. Hypercholesterolemia with adult xanthogranuloma (AXG) is extremely rare. Moreover intracranial involvement with AXG has been reported in only one previous case. We wish to report on the possibility of a new syndrome that has characteristics common to primary xanthomatoses, entities which have heretofore been considered etiologically distinct.

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A patient came to our clinic suffering from mild sensory loss, exaggeration in knee reflexes, bilateral extensor plantar responses and elevation of the antibody level to the human T-lymphotropic virus type I (HTLV-I). Electrophysiological and pathological studies showed axonal and demyelinating neuropathy. The condition appeared to be a slowly progressive myeloneuropathy. HTLV-I may play an important role in the pathogenesis of myeloneuropathy.

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LCAT activity and mass were assayed simultaneously in 23 members of a new family case, revealing two homozygotes with a markedly low HDL - cholesterol level and ester cholesterol ratio. The LCAT mass in these patients was only 0.8 and 0.9 μg/ml, respectively (normal range 4.4-8.1) and their LCAT activity was 4 and 6 nM/ml/h 37°C (normal range 60-120). Apolipoprotein (Apo) A-I and II levels were significantly low; however, apolipoprotein E tended to be high. In two-dimensional electrophoresis, apo A-I isoform visualized the increase of immature apo A-I; that is, A-I₂. One subject showed the clinical characteristics of classic LCAT deficiency; however, the other, who was a vegetarian, showed corneal opacities and red cell deformity, but not proteinuria. This suggests that a low fat diet which decreases the level of atherogenic large LDL, may lead to a more favourable prognosis with a reduced risk for renal insufficiency. There were two different types of LCAT abnormality in this family series. Among the 10 examined paternal kindred of the proband who was one of two homozygotes, seven had a low LCAT mass but normal LCAT activity with the exception of one kindred who had a low mass and low activity. In contrast, among his seven maternal kindred examined, two had a low LCAT activity but normal mass.

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The present communication describes a patient with paraganglioma found in the retroperitoneum and neck. She was treated with the combination chemotherapy employing cyclophosphamide, doxorubicin, and cisplatin (CAP therapy), and resulted in remarkable regression of the tumor in size. We reviewed the literature about the therapy of paraganglioma and the nature of retroperitoneal paraganglioma in relation with this case.

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Cisplatin (CDDP 100 mg/m², div), adriamycin (ADR 20 mg/body, iv) and futrafur-uracil conjugate (UFT200 mg/day, po) were administered on the same day to a 51-year-old housewife with lymphangitis carcinomatosa of the lung. During the CDDP infusion, the patient unexpectedly became highly febrile. On the following day, the patient developed purpura on the upper and lower extremities, and her platelet count was 3.2 × 10⁴/mm³ in spite of being 32.4 × 10⁴/mm³ on the day before chemotherapy. The platelet count recovered to 9.4 × 10⁴/mm³ on day 3, and increased to a maximum of 56.1 × 10⁴/mm³ on day 20. Previously, we had treated several patients with adenocarcinoma by the same protocol, but never experienced such a case that developed a sudden fall in their platelet count. Although the true mechanisms remain to be solved, this case is very interesting from a standpoint of drug-induced thrombocytopenia.

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