Systemic connective tissue diseases are characterized by the production of a number of autoantibodies directed against various cellular constituents. These autoantibodies are closely associated with certain diseases and clinical manifestations, and are therefore useful for clinical practice such as to diagnose diseases and to predict clinical subsets, disease activity and prognosis. To understand the etiology and pathogenic mechanisms of connective tissue diseases, it is particularly important to elucidate the structure and function of target autoantigens recognized by these disease-specific autoantibodies. In recent years, the nature of many target autoantigens have been identified using molecular biology approaches. Most of them are intracellular enzymes and regulatory factors necessary for important biological function involved in gene replication, transcription, RNA processing and protein translation. Thus, the studies of autoantibodies are useful not only in clinical medicine but also in basic cellular and molecular biology.
(Internal Medicine 38: 523-532, 1999)

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Object To know how Japanese patients perceive their physicians without a white coat during consultations. Subjects and Methods The patients who visited a university clinic were divided into two groups: those seen by a physician in a white coat (the white-coat group) and those seen by a physician in private clothes (the private-clothes group). Questionnaires were distributed to the patients, which asked the tension and satisfaction of consultations as well as their preference for physician's attire. The answers of the white-coat group were compared with those of the privateclothes group. Results The percentage of new patients who felt tense during consultations was greater in the white-coat group (42%) than in the private-clothes group (33%). Seventy-one percent of the patients in the white-coat group preferred physicians in a white coat whereas only 39% preferred so in the private-clothes group (p<0.0001). However, the degree of patients' satisfaction for the consultation showed no statistical difference between the groups. Sixty-nine percent of the patients older than or equal to 70 years preferred a white coat while 52 percent of the patients younger than 70 years preferred so (p=0.002). Conclusion Physician's white coats did not influence the satisfaction with the consultations for most Japanese patients in a university clinic, although elderly patients as well as those seen by a physician in a white coat tended to prefer the white coat to the private clothes. Furthermore, practice without a white coat might reduce patients' tension during their first consultation.
(Internal Medicine 38: 533-536, 1999)

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Object An indocyanine green derivative (ICG-sulfo-OSu) was used as the labeling substance for monoclonal antibody, and a fluorescence imaging system appropriate for ICG-sulfo-OSu excitable by infrared rays (IR) was developed. The goal of this study was to demonstrate antibody labeling at the tissue level using this new imaging system. Materials and Methods ICG-sulfo-OSu labeled mouse anti-human carcinoembryonic antigen (CEA) monoclonal antibody, a newly developed imaging system, and an infrared ray microscope were employed in this experiment. Paraffin sections of human colon cancer previously proven to have cross-reactivity to anti-CEA antibody were examined. Results Positive staining was seen as a brownish discoloration of oxidized 3, 3'-diaminobenzidine tetrahydrochloride (DAB) in sections that reacted with ICG-sulfo-OSu-labeled anti-CEA antibody, and the fluorescence was well-matched with the oxidized DAB positive sites. Conclusion Specific antibodies labeled with ICG-sulfo-OSu have significant affinity to cancer cells and seem to reflect sufficient amounts of fluorescence by IR to be useful in a system for the endoscopic detection of micro cancers using the immunohistochemical staining method.
(Internal Medicine 38: 537-542, 1999)

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Object Primary pulmonary hypertension (PPH) has a grave prognosis, and chronic majorvessel thromboembolic pulmonary hypertension (TPH) also has a poor prognosis. The purpose of the study is to elucidate the inherent prognoses of two serious diseases. Methods Thirty-two patients with PPH (aged 33±15 years, 1SD) and 48 patients with TPH (aged 50±14 years, 1SD) were used to determine survival curves using Kaplan and Meier's method from the baseline diagnostic catheterization. Definitive diagnosis was made by cardiac catheterization, ultrasonic echocardiography, ventilation-perfusion lung scanning, and pulmonary angiography. Results Mean pulmonary artery pressure (PAm) at the initial examination was 56±16 (mean±1SD) mmHg for patients with PPH and 50±13 mmHg for those with TPH, and did not differ significantly between the two groups. Although these two diseases exhibited equally elevated pulmonary arterial pressures, possibly indicative of a poor prognosis, the survival curves obtained from the prospective study for the two diseases were quite different (p<0.001 by generalized Wilcoxon test, p<0.01 by Log-rank test). Mean survival was calculated to be 3.6 years for PPH and 6.8 years for TPH. Patients with TPH had a longer survival than those with PPH, despite the fact that the age at diagnosis of PPH was younger by 17 years on average than that of TPH. Conclusion The inherent prognoses of the two chronic pulmonary hypertensive diseases prior to the introduction of the recently developed new treatment options are shown and likely indicate that the pulmonary vascular lesions in PPH are consistently progressive, while those of TPH are not as progressive.
(Internal Medicine 38: 543-546, 1999)

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Object We describe the characteristics of three patients with CD56+CD7+ stem cell leukemia/lymphoma. Methods These blasts were analyzed for morphologic, karyotypic, immunophenotypic, and immunogenotypic features using Southern blot and polymerase chain reaction analysis. Materials Peripheral blood, bone marrow aspirates, or biopsied mediastinal tumor specimens of three CD56+CD7+ stem cell leukemia/lymphoma patients were investigated. Results The bone marrow of all patients showed myeloperoxidase (MPO) negative blast cells with basophilic cytoplasm and distinct nucleoli with no azurophilic granules. The blasts of two patients were classified as acute lymphoblastic leukemia (L2). The liver, spleen, and lymph nodes were unaffected in all patients. AH had an aggressive clinical course. The blasts were strongly positive for both CD7 and CD56 but negative for other T-lineage associated antigens, including CD1, CD2, surface membrane CD3, cytoplasmic CD3c (2/2), CD4, CDS and CDS. The additional antigens were recognized as follows: CD19 (1/3 cases) as a B lineage, CD33 (1/3) as a myeloid marker, CD34 (2/3) as a stem cell, CD38 (1/1) and HLA-DR (2/3). When the patients relapsed, the phenotypes changed to blasts positive for CDS, CD10 and CD13 in patient 1, CDS in patient 2, and CD33 in patient 3. MPO, however, remained negative. Cytogenetic analysis showed no common abnormal karyotype. All had a common D2-Jδ1 induced by T-cell specific enhancer. Rearrangement of TCR β and γ genes occurred in patient 2, and IgH and TCR β underwent rearrangement in patient 3. Conclusion Although a more comprehensive case analysis is necessary, these data suggest the possibility that the blasts of the present cases come from a common lymphoid precursor (T, NK, and B cell) or from a NKT precursor as the fourth lymphoid lineage.
(Internal Medicine 38: 547-555, 1999)

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Object Toxoplasmic encephalitis (TE), primary central nervous system lymphoma (PCNSL) and progressive multifocal leukoencephalopathy (PML) are major central nervous system (CNS) diseases in patients with acquired immunodeficiency syndrome (AIDS). We assessed the diagnostic value of polymerase chain reaction (PCR) in the detection of DNAs of Toxoplasma gondii (T. gondii), Epstein-Barr virus (EBV) and JC virus (JCV) in the cerebrospinal fluid (CSF). Methods We compared the PCR results with those of pathological findings at autopsy. Patients or Materials The present study included 23 autopsies representing those in whom CSF samples were obtained before death while the patient was hospitalized or at autopsy. Results The threshold levels for PCR detection were 4 tachyzoites of T. gondii, 5-15 genomes of EBV and 10 genomes of JCV. We identified T. gondii DNA in 4 out of 5 autopsy-defined cases of TE, EBV DNA in 5 out of 5 cases with PCNSL, and JCV DNA in 2 out of 2 cases with PML. The specificity of PCR was 100% in TE, 78 % in PCNSL, and 100 % in PML. Conclusion Although the number of cases was relatively small in this study, PCR correctly identified T. gondii DNA in those cases in which PML or PCNSL was the sole clinical diagnosis. Our results indicate that PCR examination of CSF is a clinically useful tool for the diagnosis of focal brain lesions in patients with AIDS.
(Internal Medicine 38: 556-562, 1999)

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Objects We attempted to evaluate the usefulness of in situ hybridization (ISH) in the specific diagnosis of Aspergillus pulmonary infection. Methods We used an ISH technique using a multiple digoxigenin-incorporating probe, which was constructed by means of the polymerase chain reaction (PCR) from the 18S ribosomal RNA of Aspergillus fumigatus. Materials We studied twelve formalin-fixed, paraffin-embedded lung tissue sections from autopsy-confirmed invasive pulmonary aspergillosis (IPA) (5 acute myelocytic leukemias, 2 acute lymphocytic leukemias, 2 chronic myelocytic leukemias, 1 adult T-cell leukemia, 1 non-Hodgkin's lymphoma and 1 chronic obstructive pulmonary disease.), and 18 sections from other pulmonary infections as control. Results ISH using the probe and a low-viscosity hybridization buffer solution (LV) positively stained hyphal elements in 12 of 12 autopsy lung tissue specimens from subjects with IPA, while ISH using the probe and a high viscosity hybridization buffer solution (HV) positively stained the hyphal elements in 6 of 12. Specifically, ISH (LV) demonstrates hyphal elements of Aspergillus spp. in the center of Aspergillus abscess. While, ISH (HV) can detect hyphal elements located in the periphery of a suppurative abscess as well as those in the blood vessel. Conversely, ISH did not show positive results for any of the autopsy tissue specimens from subjects with other fungal pneumonia infections (Candida n=5, Mucor n=2, Cryptococcus n=2, and Pseudallescheria n=1), Pneumocystis carinii pneumonia (n=5), and cytomegalovirus pneumonia (n=3). Dual staining by means of ISH and immunohistochemistry (IHC) using anti-neutrophil elastase (NE) and anti-CD68 monoclonal antibodies showed that NE positive cells were localized at the edge of the radial growth of the organism, but CD68 positive cells were located around the center of the abscess. The accumulation of NE positive cells was rarely seen in half of the cases (6/12). In contrast, CD68 positive cells were routinely present in the center of the abscess (12/12). Conclusion ISH in conjunction with IHC is a useful tool for differentiating Aspergillus spp. from other fungal genera in tissue sections from patients with IPA and may have a certain role in the evaluation of the interactions between organisms and recruiting inflammatory cells.
(Internal Medicine 38: 563-569, 1999)

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We report two cases of aorto-gastrointestinal fistula. Case 1, a 60-year-old man, suffered from repeat hematemesis. He was preoperatively diagnosed as aortoesophageal fistula with thoracic aortic aneurysm and was successfully treated by graft replacement of the aneurysm. Case 2, a 73-year-old man, presented with massive gastrointestinal bleeding, yet repeat endoscopical examination did not reveal the origin of the bleeding. He died of catastrophic hematochezia. The pathological findings at autopsy revealed an aortoduodenal fistula. These two cases suggested the importance to consider an aorto-gastrointestinal fistula in the differential diagnosis of patients presenting gastrointestinal hemorrhage.
(Internal Medicine 38: 570-574, 1999)

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A twenty-year-old woman with anorexia nervosa (body mass index=11) suffered from severe liver dysfunction (aspartate aminotransferase 5, 000 IU/l, alanine aminotransferase 3, 980 IU/l, prothrombin time 32%), hypoglycemia (serum glucose 27 mg/dl), and pancreatic dysfunction (amylase 820 IU/l, lipase 558 IU/l). She fell into a depressive state with irritability, which was not improved by intravenous glucose. Despite treatment with plasmapheresis for the liver dysfuncttion, she subsequently developed pulmonary edema, acute renal failure, gastrointestinal bleeding, and disseminated intravascular coagulation. Hemodialysis, mechanical ventilation and drug therapy including prednisolone, prostaglandin E₁, and branched-chain amino acid, improved her critical condition. In this case, malnutrition may have been the cause for the liver dysfunction and subsequent complications.
(Internal Medicine 38: 575-579, 1999)

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A-65-year-old man was admitted for coronary and peripheral angiography to evaluate angina pectoris and peripheral vascular disease. Following angiography, he suffered from blue toes, livedo reticularis and progressive renal failure. The patient's condition continued to deteriorate, including the development of malnutrition. Four months later he suddenly developed panperitonitis, went into shock and died. The autopsy verified multiple perforations of the small bowel with disseminated cholesterol atheromatous embolism. The other organs including kidney were also invaded by atheroembolism. This was a rare case of multiple spontaneous perforations of small bowel due to systemic cholesterol atheromatous embolism.
(Internal Medicine 38: 580-584, 1999)

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Non-surgical reduction of septal myocardial mass was performed with success in a 48-yearold male with hypertrophic obstructive cardiomyopathy. Angioplasty balloon inflated in the first septal branch of the left anterior descending artery dramatically reduced the pressure gradient across the outflow tract of the left ventricle. Following injections of absolute alcohol into the artery with the balloon kept inflated induced a small septal myocardial infarction. By hemodynamic evaluation, the peak pressure gradient of 108 mmHg before the procedure was decreased to 30 mmHg. Clinical improvement in New York Heart Association (NYHA) functional class was obtained. At 3-month follow-up, the patient was doing quite well; the gradient was only 10 mmHg at rest and 25 mmHg with Valsalva maneuver.
(Internal Medicine 38: 585-589, 1999)

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A 52-year-old heavy drinker presented with repeated episodes of disturbance of consciousness and an increase in serum ammonia level, triggered by excessive alcohol intake. He was diagnosed as having adult-onset citrullinemia with deficiency of hepatic argininosuccinate synthetase (ASS) activity. Cranial magnetic resonance imaging (MRI) showed high-intensity lesions in the central pons and the bilateral middle cerebellar peduncles on T2-weighted images. Although almost all cases of adult-onset citrullinemia have been reported to be enzymologically classified as type II, the serum amino acid pattern and serum level of human pancreatic secretory trypsin inhibitor (hPSTI) were atypical for type II in the present case.
(Internal Medicine 38: 590-596, 1999)

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A 70-year-old woman with small-cell lung carcinoma (c-T4N2MO) was treated by six courses of combination chemotherapy (carboplatin and etoposide). After two weeks, she complained of a sense of darkness and night blindness. A Western blot analysis showed that the patient's serum bound with the recombinant 23-kDa retinal cancer-associated retinopathy (CAR) antigen at 1:1, 000 dilution. Her visual acuity became so poor that she could only recognise a hand motion at 50 cm despite treatment with corticosteroids and combination chemotherapy. The patient was diagnosed as having a rare type of CAR because CAR is usually found before the diagnosis of primary cancer.
(Internal Medicine 38: 597-601, 1999)

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We report here a 13-year-old woman with cough, sputum and fever. The patient had both chronic sinusitis and bronchitis. Chest X-ray and computed tomographic scan of the chest revealed mucous bronchial filling and bronchiectasia in bronchi of bilateral lower lobes, right middle lobe and left upper lobe. Aerosol inhalation scintigraphy with ^99mTechnetium demonstrated delays of the discharged tracer. On the basis of these findings, primary ciliary dyskinesia was suggested. This was confirmed by the findings from nasal biopsy with transmission electron microscopy where all of the microtubules were segmentally defected near the basal body in the cilia. On the basis of these findings, we diagnosed the patient with primary ciliary dyskinesia which may be due, at least in part, to segmental defect of ciliary microtubules.
(Internal Medicine 38: 602-606, 1999)

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We describe a rare case of de novo acute myelogenous leukemia with trilineage myelodysplasia (AML/TMDS) associated with t(8;21)(q22;q22). The patient was admitted to our hospital with leukocytopenia. AML/TMDS was diagnosed by excess myeloblasts and morphological findings of bone marrow. The karyotype revealed 45, X, -Y, t(8;21)(q22;q22) in 17 of 20 analyzed mitoses, and also AML1/MTG8 transcripts were detected by the reverse transcription polymerase chain reaction (RT-PCR) method. The patient achieved a complete remission with a combination chemotherapy of daunorubicin, cytarabine, and prednisolone. This case suggests that t(8;21)(q22;q22) may participate in the pathogenesis of AML/TMDS, although this type is usually found as one of the chromosomal abnormalities in de novo acute myelogenous leukemia (AML) with maturation.
(Internal Medicine 38: 607-611, 1999)

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We report a 15-year-old Japanese girl with severe systemic Weber-Christian disease (WCD) who presented with acute onset of high fever associated with tender subcutaneous nodules. Laboratory tests showed an elevated serum concentration of lactate dehydrogenase (LDH), leukopenia, and coagulation abnormalities. The anti-nuclear and anti-DNA antibodies were negative, and the serum pancreatic enzymes and alpha 1-antitrypsin levels were normal. Pulse steroid therapy was not effective, and eventually cerebellar hemorrhage occurred. After initiation of oral cyclosporin A (CyA) therapy, fever came down and her clinical condition improved markedly. Extremely high serum concentrations of interferon-gamma (IFN-γ) and soluble interleukin-2 receptor (sIL-2R) in this patient returned to normal with CyA therapy. These findings suggest that T-cell immune responses are involved in the pathogenesis of WCD, and that CyA is effective against the disease via suppression of T-cell reactions.
(Internal Medicine 38: 612-614, 1999)

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