The oncogene products of c-myc, c-Ki-ras and c-Ha-ras and the subsets of natural killer cells in the peripheral blood mononuclear cells (PBMC) from 39 patients with myasthenia gravis (MG) and 40 healthy individuals were studied by immunocytochemical procedures. The MG patients showed an increased expression of c-myc, c-Ki-ras and c-Ha-ras oncoproteins in both lymphocytes and monocytes compared to those of healthy individuals (P<0.01, P<0.005 and P<0.001, respectively). On the contrary, the number of Leu-7^-Leu-11c⁺ cells of MG patients was less than that of healthy controls (P<0.05). The oncogene expressions in PBMC of MG patients correlated positively with white blood cell counts, total natural killer cell counts (NK), Leu-7⁺Leu-11c⁺ of NK cell subset, γ-globulin, erythrocyte sedimentation rate and serum immunoglobulins; the oncogene expressions were negatively correlated with the Leu-7⁺Leu-11c^- NK subset. Thus, the increased expressions of c-myc, c-Ki-ras and c-Ha-ras oncoproteins accompanying the decreased Leu-7^-Leu-11c⁺ NK cell in MGPBMC are likely responsible for the acceleration of the clinical manifestations in MG patients.
(Internal Medicine 32: 519-522, 1993)

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The antiviral effect of natural interferon (IFN)-α on chronic hepatitis C virus (HCV) infection was estimated by determining quantitative changes in serum HCV-RNA compared with the serum alanine aminotransferase (sALT) improvement; the relationships of responses to IFN according to the dose and period of IFN therapy were defined to determine an appropriate IFN therapy protocol. Twenty-two patients with chronic hepatitis C were given natural IFN-α and in 16 (72.7%) patients the viraemia was suppressed during therapy. Five (27.7%) of them sustained the disappearance of HCV-RNA for more than 6 months after therapy accompanied with a prolonged sALT improve-ment. Pre-treatment viraemia levels in 5 complete responders with "complete suppression" of viraemia were significantly lower than in 11 patients with a transient loss or a decline of HCV-RNA. Afavorable antiviral response was closely associated with a high total dose of IFN-α and a long duration of IFN therapy.
(Internal Medicine 32: 523-529, 1993)

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From 108 cases of new daily persistent headaches, clinical or laboratory evidence was found suggesting extracranial or systemic infections in: 28 cases (25.9%) of gastrointestinal mainly Salmonella, 28 (25.9%) urinary Coli, 16 (14.8%) Streptococcal, 4 (3.7%) each of Epstein Barr virus or Toxoplasma, and 1 (0.9%) each of Herpes Zoster or pneumonia. A group of 26 (24.1%) showed high Proteus OX titer or clinical adenoviral involvement. All had normal neurological examinations plus selective negative neuroimaging or spinal taps. The meanheadache duration was 13.8 days, and mean age 28.8 years. Prominent symptoms were fever in 37 (34.2%) cases, nausea/vomiting in 30 (27 %) and vertigo in 17 (15.7 % ). Diarrhea, dysuria, and abdominal discomfort were rare. Headache was a solitary symptomin 36 (33.3%). The predominant sign was painful cervical lymphadenopathy in 61 (56.5%). These cases represent 1.2% of our 9060 neurology patients.
(Internal Medicine 32: 530-532, 1993)

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Leukemia cells from ten patients with B-cell chronic lymphocytic leukemia (B-CLL) and one with B-CLL of mixed cell type (B-CLL/PL) were analyzed to delineate the cellular characteristics, especially the myelomonocytic nature. Most of the surface phenotypes were consistent with the previous reports. However, the frequency of positivity for CD22 and CD25 was much higher in the examined cases of B-CLL than in Western reports. B-CLL cells frequently possessed antigens which are mainly expressed in myelomonocytic cells: 8/10 for CD11b and 4/10 for CD11c, although the presence of CD14 was not apparent (0/10). In addition, colony stimulating factor-1 receptors (CSF-1R) were expressed in 6 of the 10 B-CLL cases. The reactivity with antigens such as CD14, CD11c, and CSF-R was markedly enhanced by in vitro incubation. Alterations in antigenexpression and in in vitro survival by growth factors for myelomonocytic lineage were observed in certain cases. These data imply that not only are B-CLL cells phenotypically closely related to myelomonocytic lineage but that they may also be influenced by growth factors for that lineage.
(Internal Medicine 32: 533-539, 1993)

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A rare case of protothecosis in a 40-year-old Japanese woman with systemic lupus erythematosus, under long-term prednisolone treatment is presented. The patient developed extensive ulcerative skin lesions after injury. The characteristic histopathological findings and biochemical sugar assimilation tests indicated infection by Prototheca wicherhamii. The lesions improved following treatment with amphotericin B. The immunodeficient state of this patient seemed to contribute to the infection with Prototheca.
(Internal Medicine 32: 540-542, 1993)

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A 27-year-old man, who had been on steroid therapy for 2 months for his nephrotic syndrome, suddenly developed intra-abdominal bile collection (biloma). He had no previous history of abdominal surgery, trauma, or any disease of the hepatobiliary system. The cause of the biloma formation was due, probably, to cholecystitis in the absence of calculi and a pinhole size perforation in the wall of gall bladder. It was assumed to be closely related to the high-dose steroid therapy over a prolonged period, which would likely suppress the repair process of the locally damaged biliary system.
(Internal Medicine 32: 543-546, 1993)

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A 47-year-old man with acute hepatitis B virus (HBV) infection who had been receiving immunosuppressants after renal transplantation developed progressive liver failure. During the clinical course (approximately 7 months), anti-HBc IgM antibody and HBV-DNA polymerase levels remained high, but the serum alanine aminotransferase (ALT) level was consistently less than 150 K.U. Histopathologic examination of the liver showed submassive hepatic necrosis without significant inflammation accompanied by marked fibrosis. Most hepatocytes showed strong nuclear expression of HBc antigen by immunohistochemical staining and electron microscopy revealed numerous intranuclear core-like particles. Hepatitis B virus infection in immunosuppressed individuals occasionally insidiously progresses, resulting in liver failure. The clinical course of such patients thus merits close scrutiny.
(Internal Medicine 32: 547-551, 1993)

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Idiopathic sclerosing peritonitis (ISP) is a rare condition which has been mainly reported in young adolescent women as a cause of small bowel obstruction. In these patients the small bowel is sometimes encased in a fibrous sac called an "abdominal cocoon". We describe a 62-year-old man who underwent exploratory laparotomy for ascites and abdominal mass. Laparotomy showed 5.4 / of ascites and the entire small bowel was encased in a fibrous sausage-like cocoon. The pathological findings were characteristic of sclerosing peritonitis.
(Internal Medicine 32: 552-555, 1993)

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A 60-year-old woman suffering from heart failure was found to have Crow-Fukase syndrome. A precise cardiovascular study revealed the existence of pulmonary hypertension (PH), cardiomegaly and myocardial hypertrophy in addition to manifestations of this syndrome such as hyperpigmentation, hypertrichosis, finger clubbing, polyneuropathy, organomegaly and plasma cell dyscrasia. These findings suggest the possibility that patients with Crow-Fukase syndrome have cardiopulmonary disorders including PH and cardiomegaly which might cause some cardiovascular symptoms such as peripheral edema and finger clubbing.
(Internal Medicine 32: 556-560, 1993)

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A 48-year-old man with primary Sjögren's syndrome (P-SS) developed subcortical dementia, characterized by forgetfulness, poor attention and concentration, slowness of thought process, difficulty to manipulate acquired knowledge, introversive and hostile personality change and inactivity. These were improved by corticosteroid treatment. Magnetic resonance imaging (MRI) revealed small lesions in the subcortical and periventricular white matter. An electroencephalogram (EEG) revealed short runs of theta activities in bilateral parietal areas. Latency of P300 shortened in accordance with clinical improvement. These findings suggest that the cognitive and neuropsychiatric manifestations in P-SS are included in a spectrum of organic brain dysfunction and are treatable.
(Internal Medicine 32: 561-564, 1993)

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We present a case of primary plasma cell leukemia with Bence Jones proteinuria. After combination chemotherapy, leukemic cells and the urinary levels Bence Jones protein were decreased. Small lytic bone lesions were detected only in the skull. Typical plasma cells were rarely seen in peripheral blood on the hyperleukocytic phase, however they were increased in the advanced stages. The most important diagnostic sign was persistent expression of CD38 antigen on leukemic cells throughout the entire course of the illness and these leukemic cells expressed very late antigen-4 (VLA-4) but not VLA-5.
(Internal Medicine 32: 565-568, 1993)

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A 44-year-old female underwent a right frontal craniotomy under a diagnosis of metastatic brain tumor and a fluid-filled cystic lesion was extirpated. One rice grain sized nodule was identified in the cyst and histopathological examination of the nodule showeda scolex with hooks and suckers. These macro and microscopic findings indicated a diagnosis of cerebral cysticercosis, caused by infection of the larval stage of some type of Taenia species. A therapeutic course of praziquantel was given after craniotomy. The patient has been a resident of the Kanto area of Japan since birth and has never been outside Japan.
(Internal Medicine 32: 569-573, 1993)

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The characteristics triad of tuberous sclerosis-adenoma sebaceum, mental deficiency and epilepsy-associated with distal-type renal tubular acidosis was combined with anticonvulsant osteomalacia in a 41-year-old woman. In addition to the specific bone lesions of tuberous sclerosis, the bone disease was caused by an adverse effect of a drug and possibly also by the renal disorder leading to significant musculoskeletal disability. In response to calcium carbonicum and 1-25-dihydroxyvitamin D therapy the musculoskeletal disability healed and the abnormal biochemical markers of anticonvulsant osteomalacia disappeared. The present observation draws attention to the increased hazard threatening patients on chronic anticonvulsant therapy simultaneously suffering from renal diseases.
(Internal Medicine 32: 574-579, 1993)

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Rubella was accompanied by hepatic dysfunction in a 28-year-old male. Serum aminotransferase levels were moderately elevated and LDH markedly increased, especially LDH isoenzyme 5, whereas total bilirubin and ALP remained almost normal. GOT, GPT and LDH levels were completely normalized by the 21st hospital day. Paired antibody titers of viruses which may cause hepatitis, other than rubella, were of no significance. Laparoscopy showed grosse rote Leber. Histologic and electron microscopic findings of the liver were consistent with acute hepatitis. Hepatic damage with rubella is rare, and it is possible that the hepatic dysfunction seen in adult rubella may be mediated by an immunopathologic mechanism.
(Internal Medicine 32: 580-583, 1993)

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A cytogenetic study of a 38-year-old patient with acute lymphoblastic leukemia (ALL) revealed a t(1;19)(q23;pl3), which is a characteristic translocation of childhood ALL. The leukemic cells were positive for the CD10, CD19, HLA-DR, TdT and cytoplasmic μ-chain. Both of the immunoglobulin heavy chain gene loci were rearranged and the RNA-based polymerase chain reaction demonstrated the E2A/PBX1 fusion transcript which is the result of the t(1;19). This finding suggests that the t(l;19) is implicated not only in childhood ALL but also in adult ALL patients.
(Internal Medicine 32: 584-587, 1993)

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In two patients with frequent supraventricular tachycardia, echocardiograms revealed remarkable left ventricular systolic and diastolic dysfunction, associated with an absence of left ventricular filling during the early diastolic phase. This dysfunction was detected during normal sinus rhythm as well as tachycardia. By treatment with propafenone, both patients showed dramatic and rapid improvement of symptoms and the left ventricular dysfunction. Left ventricular diastolic dysfunction as well as systolic dysfunction were detected in patients with supraventricular tachycardia, suggesting that frequent supraventricular tachycardia results in severe left ventricular diastolic dysfunction which is reversible after control of the tachycardia.
(Internal Medicine 32: 588-592, 1993)

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A 37-year-old man with the characteristic clinical signs of Kartagener's syndrome, including bronchiectasia, chronic paranasal sinusitis, and situs inversus, was found to have immotile cilia and sperm. Electron microscopy demonstrated that the majority of cilia lacked dynein arms and radial spokes and that various defects of microorgans existed in the sperm. The most frequent defect was total defect of axoneme followed by defect of inner dynein arms in the sperm. These findings suggest an association between the structural abnormality of absent inner dynein arms and immotility of cilia and sperm in Kartagener's syndrome.
(Internal Medicine 32: 593-597, 1993)

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A 61-year-old man was admitted to our hospital because of fever and general malaise. We diagnosed his condition as cytomegalovirus (CMV) mononucleosis by hematological testing. His platelet count was decreased in spite of collection with several anticoagulants at room temperature (20°C); no decrease was observed at 37°C.Mixed cryoglobulinemia, monoclonal IgM and polyclonal IgG, which acted as anti-platelet antibodies in cold condition, were found in the patient's serum. With the resolution of CMV infection, the cryoglobulins disappeared and the platelet count returned to normal without platelet agglutination. This is the first report of transient pseudothrombocytopenia in CMV mononucleosis with mixed cryoglobulinemia.
(Internal Medicine 32: 598-601, 1993)

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A 63-year-old woman was diagnosed as autoimmune gastritis by the presence of serum antibody against α-subunit of gastric H⁺, K⁺-ATPase. The patient did not have pernicious anemia, but showed achlorhydria, marked hypergastrinemia, enterochromaffin-like cell hyperplasia and an extremely high histidine decarboxylase activity in the gastric fundic mucosa. Intragastric acidification by infusion of hydrochloric acid via a nasogastric tube induced a transient reduction of serum gastrin level and fundic mucosal histidine decarboxylase activity. A marked increase in fundic mucosal histidine decarboxylase activity as well as hypergastrinemia appears to be the pathophysiologic response to achlorhydria caused by autoimmunity against gastric H⁺, K⁺-ATPase.
(Internal Medicine 32: 602-606, 1993)

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Intestinal pseudoobstruction and pneumatosis cystoides intestinalis are uncommon complications of progressive systemic sclerosis. We report a 26-year-old woman with this disorder who responded poorly to conventional treatment. Subcutaneous administration of octreotide, a longacting somatostatin analogue, at a dose of 50 μg/day for 3 weeks, relieved symptoms such as nausea and bloating. There was also a marked decrease of intestinal gas accumulation, as documented on X-ray films.
(Internal Medicine 32: 607-609, 1993)

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