Peptidylglycine α-amidating monooxygenase (PAM) plays a key role in the biosynthesis of many biologically active neuronal and endocrine peptides that possess α-amide function at their C-terminus. Using D-Tyr-Val-Gly as the substrate, we measured PAM activity levels in the cerebrospinal fluid (CSF) and serum of patients with a variety of neurological diseases. PAM activity in the CSF was significantly increased in patients with multiple sclerosis (MS), especially during the active stage, compared with that in patients with other neurological diseases (p<0.05). Levels of CSF PAM activity were not correlated with protein levels in CSF or with level of serum PAM activity. Since PAM is present not only in neurons but also in oligodendroglia, it is possible that the increase in CSF PAM activity in patients with MS may stem from massive demyelination and oligodendroglial destruction.
(Internal Medicine 34: 229-232, 1995)vvv

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Eighty patients with manifest or concealed Wolff-Parkinson-White (WPW) syndrome underwent catheter ablation of 86 accessory pathways (AP) using radiofrequency current. There are 65 AP located on the left side and 21 on the right side of the heart. The atrioventricular reciprocating tachycardia was previously documented in 77 patients and atrial fibrillation with a rapid ventricular response in 21 patients. Ablation was attempted via a catheter positioned at the atrial aspect of the tricuspid annulus in patients with a right-sided AP and via a catheter positioned in the left ventricle directly below the mitral annulus in patients with a left-sided AP. AP conduction was permanently abolished in 82 of the 86 pathways (95%). The number of radiofrequency current applications for these 82 successfully ablated AP was 7.6±0.9. Of the 65 left-sided and 21 right-sided AP, 62 (95%) and 20 AP (95%) were successfully ablated, respectively. The current application to the right-sided AP was 16.7±2.2, which was greater than that to the left-sided one (4.7±0.6, p<0.001). No serious complication was observed in any case. Catheter ablation of AP using radiofrequency current is an effective and safe therapeutic modality for patients with symptomatic WPW syndrome.
(Internal Medicine 34: 233-239, 1995)

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The changes in mitogen-induced lymphocyte response occurring in nine patients undergoing chronic hemodialysis and treated with 1, 25 dihydroxyvitamin D₃ (0.5 μg/day) were investigated. Prior to treatment the stimulation indices (SI) in patients with 15.4±4.2 for phytohemagglutinin (PHA) and 7.2±0.7 for conconavalin A (Con A). In the controls, stimulation indices were 44.4±13.5 and 20.2±5.3 for PHA and Con A, respectively. Following treatment, the stimulation indices increased to 36.9±6.2 for PHA (p<0.05) and 18.6±3.9 for Con A (p>0.05) indicating the beneficial effect of oral 1, 25 dihydroxyvitamin D₃ treatment on lymphocyte function in patients with chronic renal failure.
(Internal Medicine 34: 240-242, 1995)

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Autoptic findings of a 77-year-old man with T-cell-rich B-cell lymphoma (TCRBCL) showed predominant infiltration of reactive T-cells with a minority population of neoplastic B-cells in liver, spleen, pancreas, adrenal gland, stomach, small intestine and heart, as well as, lymph node. DNA studies demonstrated dual rearrangement in the T-cell receptor beta (TCRβ), immunoglobulin heavy chain J region (IgJH) and kappa light chain J region (IgJκ) genes.
(Internal Medicine 34: 243-246, 1995)

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A 62-year-old woman was initially hypothyroid and then developed hyperthyroidism with continuously positive thyroid-stimulating antibody (TSAb). When she visited our hospital with the complaint of the feel of skipping beats, her serum free T₄ level was initially low and thyrotropin (TSH) level was slightly elevated. One month after starting the supplement therapy with 1-T₄, she developed hyperthyroidism with increased ¹²³I-thyroid uptake. TSH-binding inhibitor immunoglobulin (TBII) was slightly elevated only during the hypothyroid stage. Throughout the whole course, TSAb was continuously positive, while thyroid stimulation-blocking antibody (TSBAb) was not detectable. Primary hypothyroidism with TSAb may suggest the possibility of subsequent development of hyperthyroidism.
(Internal Medicine 34: 247-250, 1995)

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A 76-year-old female was diagnosed as having primary plasma cell leukemia (PCL), based on abundant atypical plasma cells in the circulation and bone marrow, monoclonal K light chain in the serum and urine, the immunophenotype of the plasma cells and the lack of preceding multiple myeloma. The patient was treated with melphalan and prednisolone (MP), and complete remission (CR) was achieved; this was maintained for 28 months. The duration of CR in this patient appears to be the longest of those reported in well-documented primary PCL patients who have been treated with MP chemotherapy.
(Internal Medicine 34: 251-254, 1995)

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A case of alpha chain disease, involving stomach, small and large intestine, and caecum with poor prognosis is reported. Endoscopic examination revealed gastric erosion, edematous mucosa with enlarged villi of duodenum and jejunum, multiple hyperplastic lymph follicles of terminal ileum and thickening mucosa of caecum. Light microscopy revealed a conspicuous infiltration of plasma cells and lymphocytes in gastric, duodenal, jejunal and caecal lamina propria. Immnohistochemistry demonstrated alpha heavy chain protein devoid of light chain in these plasma cells. The patient developed paralytic ileus and died of septic shock on the 179th hospital day.
(Internal Medicine 34: 255-260, 1995)

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This is the first reported case of primary lymphoma of the spleen coexisting with primary hepatocellular carcinoma. A 59-year-old woman was admitted to Ugo town hospital because of general malaise. Physical examination revealed no lymphadenopathy. Laboratory data showed mild anemia, thrombocytopenia, and slight elevation of a-fetoprotein (AFP). Ultrasonography of the abdomen revealed a mass in the left lobe of the liver and a mass in the splenic hilus. The liver tumor was presumed to be a primary liver cancer. Ultrasonically guided needle aspiration of the splenic mass was unsuccessful. Subsequently, the patient died of hepatic and renal failure. Autopsy revealed hepatocellular carcinoma and primary splenic non-Hodgkin's lymphoma of the diffuse large cell type.
(Internal Medicine 34: 261-264, 1995)

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A patient with hepatitis C virus (HCV) infection developed neuropathy characterized by recurrent attacks of anesthesia, dysaesthesia, mild weakness and swelling of the right forearm. Serological examinations suggested an abnormal autoimmune state. HCV infection might produce neuropathy by inducing abnormal autoimmunity.
(Internal Medicine 34: 265-266, 1995)

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A 29-year-old woman with a 4-year history of multifocal fibrosclerosis showed unique neurologic complications. Episcleritis, orbital pseudotumor, and eosinophilic phlegmon preceded intracranial inflammatory pachymeningitis. The pachymeningitis was associated with disturbance of the visual field, incomplete Gerstmann's syndrome, and pseudotumor cerebri. T₂-weighted magnetic resonance images revealed a high signal intensity lesion in the left temporal and occipital lobes, and gadolinium-enhanced T₁-weighted images revealed the enhancement of the thickened left tentorial leaf. The laboratory data suggested that the etiology might be autoimmunological. The disease and MRI abnormalities improved following administration of corticosteroids.
(Internal Medicine 34: 267-271, 1995)

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A rare case of pregnancy and successful transvaginal delivery in secondary diabetes due to chronic pancreatitis is reported. At the age of 16 the patient was diagnosed as having chronic pancreatitis probably caused by hyperlipemia. Three years later diabetes and calcification of the pancreas was found. Blood glucose was well controlled using both self-monitoring of blood glucose and multiple subcutaneous insulin injection just before she became pregnant at age 23. The dose of insulin was slightly increased and no acute exacerbation of pancreatitis occurred during pregnancy, partly due to an appropriate low fat diet with digestive enzyme preparations.
(Internal Medicine 34: 272-276, 1995)

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