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A 70-year-old woman with rheumatoid arthritis was admitted to our hospital because of gastric ulcers. Abdominal echography incidentally revealed that the patient's gallbladder was multiseptate, an extremely rare anomaly. The diagnosis was confirmed by endoscopic retrograde cholangiography. This is the 26th case of multiseptate gallbladder in the world literature, but the patient is the oldest yet recorded with this condition. Furthermore, unlike most patients with multiseptate gallbladder, she had no biliary symptoms with concomitant cholelithiasis.

Recently, telomerase activity has been demonstrated in a large number of malignant tumors whereas its activity is not detected in most normal somatic cells suggesting its role in the immortalization process. Here we report the first investigation of telomerase activity in a case of gastric mucosa-associated lymphoid tissue (MALT) lymphoma. Elevated telomerase activity was detected in biopsy specimens of the lymphoma. After eradication of Helicobacter pylori, the level of telomerase activity returned to normal with histological regression of the lymphoma. The telomerase activity was associated with the disease activity of the gastric MALT lymphoma after eradication therapy in the present case.

We describe a case of recurrent histiocytic necrotizing lymphadenitis (HNL) with aseptic meningitis. The patient was a 46-year-old male and a carrier of human T lymphotropic virus type I (HTLV-I). The patient had a past medical history of at least three relapses of HNL. In addition, his sister, who was also an HTLV-I carrier, had recurrent clinical episodes consistent with those of HNL, suggesting familial HNL occurrence. This observation suggests the possibility that HTLV-I infection is relevant to the pathogenesis of HNL.

We report on a 54-year-old man with hepatocellular carcinoma (HCC) associated with a marked elevation of serum alkaline phosphatase (ALP) levels. Serum ALP was biochemically similar to that of universal (liver/bone/kidney) type. The noncarcinomatous area revealed typical micronodular cirrhosis due to excessive alcohol consumption. By histochemical staining, ALP activity was demonstrated diffusely within the cytoplasm of carcinoma cells. Immunohistochemical observation of the carcinoma cells excluded the intestinal or placental type of ALP. Tissue extracts from the carcinomatous area had much higher ALP activities than those from a noncarcinomatous area, which also showed characteristics of the universal type. The present HCC is the first reported to produce and excrete the universal type of ALP.

Background: Arterial ketone body ratio (acetoacetate/3-hydroxybutyrate, AKBR) has been reported to be a useful tool for the estimation of liver functional reserve, but a more recent report has cast doubt on the clinical significance of this redox theory. Furthermore, the effect of a diminution of liver functional reserve on AKBR has not been documented in chronic liver disease. Methods: AKBR was measured in normal control subjects (n = 10), heavy alcohol drinkers (n = 19), patients with chronic hepatitis (n = 18) and patients with liver cirrhosis (n = 25). Results: Though AKBR was lower in heavy alcohol drinkers (1.66 ± 0.82) than in the other noncirrhotic groups (1.97 ± 0.93 in normal control subjects, 2.25 ± 1.11 in patients with chronic hepatitis), this discrepancy did not reach a level of significance. AKBR in patients with liver cirrhosis (1.18 ± 0.52) was significantly lower than that in normal controls (p < 0.01). AKBR in Child’s class A, Child’s class B, and Child’s class C was 1.20 ± 0.60, 1.07 ± 0.56, and 1.27 ± 0.45, respectively, and there were no significant differences among them. Conclusion: AKBR may be parallel to liver mitochondrial redox potential and hepatic functional reserve to some extent, but it does not appear to be an accurate parameter for their estimation.

Tissue plasminogen activator (t-PA) levels in plasma or serum were studied in 416 patients with liver diseases: acute hepatitis (AH, n = 30); fulminant hepatitis (FH, n = 36); chronic inactive hepatitis (CIH, n = 57); chronic active hepatitis (CAH, n = 39); compensated liver cirrhosis (cLC, n = 78); decompensated liver cirrhosis (dLC, n = 84); hepatocellular carcinoma (HCC, n = 64); advanced hepatocellular carcinoma (aHCC, n = 28); and compared with that of a control group (n = 106) of healthy subjects. The t-PA levels showed significant increase in patients with AH, FH, CAH, cLC, dLC and HCC, compared with normal controls. The abnormal rates in t-PA levels (higher than 8.3 ng/ml) for each type of liver diseases were 86.1% in FH, 46.2% in CAH, 50% in cLC, 85.7% in dLC, 67.2% in HCC, and 89.3% in aHCC. t-PA levels tended to be higher in more advanced liver diseases. t-PA levels significantly correlated positively with plasminogen activator inhibitor (PAI-1) in AH, cLC, dLC, HCC and aHCC, and negatively with plasmin alpha 1-plasmin inhibitor complex (PIC), plasminogen (Plg), FDP, AT III and alpha 2-plasmin inhibitor (alpha 2-PI) in dLC, prothrombin time (PT) and fibrinogen (Fbg) in HCC. t-PA levels in patients with FH, CAH and dLC were significantly higher than those in patients with AH, CIH and cLC, respectively. Moreover, the changes of t-PA levels in the clinical courses of various liver diseases revealed that t-PA levels increased sensitively with progression of liver diseases or in advanced liver diseases.(ABSTRACT TRUNCATED AT 250 WORDS)

Seventy-six chronic alcoholics in Japan were evaluated for histological changes of liver needle biopsies, Chiron C100 antibody (C-100) for hepatitis C virus, as well as clinical and laboratory data. In biopsies, the presence of necroinflammations within the parenchyma, lymphocytic reaction in the portal tracts, or both, might indicate non-A, non-B (NANB) chronic hepatitis. Using these histological criteria, the patients were previously classified into two groups: alcoholic liver disease (ALD) alone and ALD complicating NANB chronic hepatitis. The C100-positive ratio was found to be 12% in the former group and 69% in the latter. Further clinical and laboratory comparison revealed that there were significant differences in gamma-glutamyl transpeptidase, gamma-globulin, and adenosine deaminase levels in the sera between the ALD alone and the ALD complicating NANB chronic hepatitis groups. Since some chronic alcoholics are also affected by chronic type C hepatitis, detailed evaluations of the liver biopsy and C-100 assay are required for the differentiation of these hepatic disorders.

Calcium hopantenate (HOPA), a drug for treating symptoms of cerebrovascular disease, is a derivative of gamma-amino butyric acid and is also an analog of pantothenic acid. It is speculated that calcium hopantenate may affect lactate generation, glucose metabolism, and ammonia disposal through the inhibition of pantothenic acid metabolism. We report two uremic patients with complaints of consciousness disturbance with lactic acidosis, hypoglycemia and hyperammonemia. HOPA is mainly excreted with urine. Severe accumulation of HOPA, documented at the onset of unconsciousness in our uremic cases, might be responsible for marked inhibition of pantothenic acid metabolism.

72才の女性.胸部異常陰影の精査目的にて当科受診,聴診上I音の分裂を認め,心エコーにて右房内に巨大腫瘤を確認した.腫瘤は右房内ほとんどを占め,可動性はなく茎は不明で, I音の第1・第2成分がそれぞれ僧帽弁・三尖弁の閉鎖と一致した.また,腫瘍と一致して201thalliumの異常集積を認めた.心臓カテーテル検査では上,下大静脈圧は上昇し,腫瘤には左右冠動脈より太い栄養血管が分布し,冠動脈血流のstea1現象を認めた.摘出手術の結果,中隔に茎をもつ64gの右房粘液腫で,術後経過は順調である. 201thalliumは種々の腫瘍に取り込まれるが,心臓粘液腫への201thallium異常集積の報告は調べ得た範囲ではなかった.本例は腫瘍に豊富な栄養血流があり, 201thalliumはほかの核種と異なり,その取り込みには血流にdependentな性格があるものと考えられた.

The glycation of plasma fibrinogen (Fbg) in diabetic patients, an alteration in clotting and fibrinolytic activities of glycated Fbg and the effect of various drugs on glycation of Fbg were investigated. The amount of furosine, which was a specific product by hydrolysis of glycated lysine residue of Fbg were determined using HPLC in 92 diabetic patients and 90 age-matched normal subjects as a control. Also the cloting and fibrinolytic activity of the glycated Fbg separated by aminophenyl boronate chromatography and inhibitory effects of acetyl salicylic acid (ASA), ascrobic asid (ACA) and pyridoxal phosphate (PP) on the production of furosine by hydrolysis of Fbg or lysine were examined.The amount of furosine in hydrolysate of plasma Fbg was significantly higher in diabetic patients than normal subjects (p

A 56-year-old female with mixed connective tissue disease (MCTD) who developed autoimmune hepatitis is described. Hepatitis was controlled effectively by the corticosteroid therapy. Biopsy of the liver revealed swelling and hydropic degeneration of hepatocytes, accompanied by Councilman's body formation and focal necrosis. These histological findings differ from those in three previously described cases. A relationship between MCTD and liver involvement appears possible.

市販漢方薬による薬剤性肝障害の1例を経験した.症例は39歳の主婦.市販の漢方薬(MK散)を約3週間服用後,悪心,食欲不振,黄疸が出現し当科外来受診.肝腫大,肝機能障害を認め急性肝炎の疑いで入院した.輸血歴,飲酒歴はなく,HBs抗原陰性,IgMHA抗体陰性であった.薬剤による肝障害を疑い,漢方薬のリンパ球刺激試験(LST)を行ったところ,リンパ球刺激指数(LSI)が2145%と陽性を示した.さらにその組成成分である4種の配合生薬についてもLSTを施行した結果,うち1種の生薬(桑白皮)のLSIが14200%と著明な高値を示し,本生薬による過敏性肝障害と診断した.肝生検組織所見は肝内胆汁うっ滞と肝細胞障害を示しており,いわゆる混合型であった.グリチルリチン製剤投与により,トランスアミナーゼおよび胆道系酵素は約12ヵ月後に正常化した.

The activity of factor X in the plasma of 123 patients with various diseases was measured by the clotting method and by the amidolytic method. Measurement of clotting activity of factor X was carried out by assay of the prothrombin time with factor X deficient plasma. Measurement of the amydolytic activity of factor X was made by end-point assay with S-2222 as the substrate.In 29 patients, a distinct discrepancy was observed between the clotting activity and amidolytic activity of factor X. This discrepancy was shown markedly in patients with chronic renal failure (CRF) and uncontrolled diabetes mellitus (DM). In the patients with CRF, the amidolytic activity was higher than the clotting activity of factor X. On the other hand, in the patients with DM, the clotting activity was higher than the amidolytic activity of factor X.The changes in activities of factor X by addition of each fraction of plasma from patients with CRF or DM were observed. Fraction of the patient plasma was carried out by ultrafiltration with membrane filter PM 10 and gel filtration with Bio-Gel A-15m. On the experiment by the addition of low molecular weight fraction (MW

症例は32才,女性.昭和54年1月より出現した筋肉痛・筋力低下と,手の典型的なGottron徴候を主訴として入院した.血清の筋原性酵素活性の軽度の上昇と,皮膚・筋生検の所見より皮膚筋炎と診断し, prednisoloneの投与を開始した.しかし入院第12病日より胸部X線像でびまん性の浸潤影を呈したのをきつかけに筋肉痛が増強し,筋原性酵素が著明に増加した.第15病日より急性腎不全とDICを合併し,第23病日に肺出血のため発病より全経過2カ月余りで死亡した.剖検の組織学的検索では,著明な筋線維の変性と壊死が認められた.また腎では軽度の尿細管の変性と顆粒状円柱の尿細管内への沈着を認め,イエウサギの特異抗体を用いた直接および間接法蛍光染色にて,ミオグロビンの尿細管上皮への沈着と尿細管内の円柱への沈着を認めた.従来皮膚筋炎は慢性の経過をとる症例が大部分で,急性の経過をとる症例はきわめて少ない.実際本症例のように,筋原性酵素の著明な上昇と筋の急激な変性と壊死が認められるような激症型の皮膚筋炎は,内外の文献上でも数例の報告があるのみである.また本症例の急性腎不全に関与したと考えられるミオグロビンが,腎組織中で証明された症例は本邦では極めて希である.従つて本症例は皮膚筋炎が激症の経過をとつたこと,および急性腎不全の成因にミオグロビンが関与したことの2点について,貴重な症例と考えられる.

The blood coagulability of 28 chronic non-hemodialysed renal failure patients was investigated. Systemic diseases such as systemic lupus erythematosus or diabetes mellitus were excluded because of their own coagulation abnormalities. All patients were in uremic state. Fasting blood samples were drawn from antecubital vein early in the morning with 3.8% sodium citrate as the anticoagulant. When not measured immediately, samples were stored at -80°C. Following assessments were performed: PT, APTT, TEG (whole blood method), fibrinogen, factor II, V, VII, VIII, IX, X, XI, XII procoagulant activities, factor VIII related antigen (Laurell's method) (VIIIANG), antigen levels of antithrombin III ATIII), and α2 macrogloburin (α2MG) with Mancini's method, serial dilution protamin sulfate test (SDPS), ethanol gelation test (EG), high molecular weight fibrinogen complex (HMWFC) (gel filtration method), fibrin/fibrinogen degradation products (latex aggregation method) (FDP). BUN, creatinine, blood pH, total CO2 and hematocrit were also determined as the indicators of uremic state. Results were as follows. Shortened results were obtained in APTT (p

It has recently been shown by Fletcher and co-workers that circulating high molecular weight fibrinogen complexes are a most sensitive marker for detecting intravascular fibrin formation.In the present investigation, circulating high molecular weight fibrinogen complexes were detected by gel chromatography of plasma and staphylococcal clumping test in 66 patients with various liver diseases. In normal adults, plasma high molecular weight fibrinogen complexes were lower than 0.5μg/ml.In patients with liver diseases, the concentration of high molecular weight fibrinogen complexes in plasma elevated frequently.Especially in patients with decompensated liver cirrhosis and hepatoma, the concentration of high molecular weight fibrinogen complexes elevated in most cases.In patients with acute hepatitis, plasma high molecular weight fibrinogen complexes increased later than the elevation of GPT in serum.Plasma high molecular weight fibrinogen complexes in patients with liver cirrhosis decreased markedly by administration of heparin.From these results, it was suggested that subclinical intravascular coagulation exists frequently in patients with liver diseases.

Two kindreds of phosphofructokinase (PFK) deficiency associated with congenital nonspherocytic hemolytic anemia and mild myopathy were found in Japan. Both probands had jaundice, gallstones, and slight to moderate degree of exercise intolerance. They showed decreased level of red cell PFK activity and no increase of blood lactate in forearm ischemic exercise test. We studied these probands' red cell PFKs by partial purification and condensation. Muscle type isozyme of PFK in both cases was not demonstrable in starch gel electrophoresis and DEAE-Sephadex chromatography. The clinical symptoms are considered to be due to a defect of muscle type isozyme.

肝硬変の合併のないアルコール性肝障害で,肝性昏睡II度以上の肝不全を呈した6症例について臨床病理学的検討を行った.全例の血中HBs抗原は陰性で,大酒家である.肝性昏睡V度までが4例(死亡3例,生存1例),II度までが2例(生存)であった.肝組織像では,死亡例3例中2例は小葉全体に多数のアルコール硝子体を認める典型的なアルコール性肝炎であったが,他の1例には硝子体はなく,肝細胞の亜小葉性の壊死と著明なfoamy degenerationが認められるアルコール性肝炎であった.生存した3例は,蛍光抗体間接法および電子顕微鏡でもアルコール硝子体が認められず,炎症細胞浸潤もごくわずかであり,2例では強い肝内胆汁うっ滞と肝細胞の風船様変化を伴う肝線維症,他の1例は肝細胞の変化の乏しい肝線維症であった.重症のアルコール性肝障害でも組織学的には必ずしも典型的なアルコール性肝炎とは限らず,多彩な病理組織像を呈することを示した.

肝動脈塞栓術(transcatheter arterial embolization: TAE)後に肝細胞癌部およびその周辺領域がどのような形態学的変化をうけるのかを知る目的で,TAE後剖検しえた10例を対象に,病理組織学的に検討を加えた.TAEから剖検までの期間は12日～550日(平均170.4日),TAEの回数は1～5回(平均1.8回)であった.剖検では主腫瘍部にはいずれも大小の壊死巣がみられたが,1例を除いて全例に腫瘍細胞の残存と再発が認められた.談た,病理学的に,腫瘍の被膜内浸潤や門脈塞栓に対してTAEの効果を認めることができなかった.全症例の塞栓動脈は再疎通を示しており,さらに,剖検肝にバリウムを注入して軟線撮影を施行しえた3例においても腫瘍の栄養血管の新生と腫瘍濃染像が認められた.以上より,TAEで肝細胞癌部を完全壊死に陥らせ,とくに被膜内浸潤に至るまで効果をもたらすためには,栄養血管の再疎通を防止する方法をさらに考案する必要があると考えられた.

市販和漢薬(恵命我神散)による薬剤性肝障害の1例を報告する.症例は65歳女性.健胃薬として恵命我神散を約40日間服用後,食欲不振,両側大腿の斑状出血,黄疸が出現し,当科に入院した.入院時,黄疸と肝腫大を認め,生化学検査では総ビリルビン値,胆道系酵素およびGOT,GPTの上昇を認めた.第15病日に施行した肝生検組織所見は胆汁うっ滞型であった.恵命我神散に対するリンパ球刺激試験(lymphocyte stimulation test: LST)ではリンパ球刺激指数(lymphocyte stimulation index: LSI)は316%と陽性であったが,その組成生薬であるガジュツ,タクゴ,マコンブに対するLSTは,いずれも陰性であった.薬剤服用中止後,斑状出血,黄疸は速やかに消退し,総ビリルビン値,胆道系酵素も2ヵ月後に正常化した.

Urinary ammonium excretion is a major component of urinary net acid excretion. Quantitative determination of urinary ammonium excretion is important for physicians as well as renal physiologists to know the renal acidification function. Several methods are available for the determination of urinary ammonium, such as the microdiffusion method by Conway and O'Malley [1], the phenatehypochlorite method (Berthelot reaction) [2], the formalin-titrimetric method [3—5], the enzymatic method [6], and the ammonia-specific electrode [5]. However, most hospital biochemistry laboratories do not routinely measure the urinary ammonium concentration, because these methods are complicated and time consuming, or require a specific instrument. This clinical limitation has led some investigators to use the "urinary anion gap" as an index of urinary ammonium [7, 8]. In this report, we examine whether the auto-analyzer method, which is widely used for determination of plasma ammonium in clinical laboratories, can be applicable to urinary ammonium determination. The auto-analyzer method has advantages of simplicity, rapidity and easy accessibility in the clinical field.

A 78-year-old female with hepatocellular carcinoma and high serum levels of carcinoembryonic antigen is reported. Using a immunohistochemical technique, CEA was demonstrated within the cytoplasm of the tumor cells.

• A 32-year-old woman with asymptomatic primary biliary cirrhosis had autoimmune hemolytic anemia associated with reticulocytopenia and thrombocytopenia despite an intensely erythroid bone marrow. Her anemia was successfully treated with oral prednisolone and intravenous pulse methylprednisolone, with a rapid response of reticulocytosis and sustained erythrocytosis. Tiopronin therapy was later initiated and resulted in fever, rash, exacerbation of the liver disease, and positive direct and indirect antiglobulin tests. (Arch Intern Med1984;144:89-91)

Vestibular function testing was done regularly on the cases given streptomycin, kanamycin, or enviomycin and a method to detect the cases of vestibular dysfunction at an early stage was discussed, as well as the time these drugs should be discontinued. Subjects were 85 cases of tuberculosis treated with streptomycin, kanamycin, or enviomycin who were admitted to our hospital from December 1984 to May 1986. The method of equilibrium examination performed at regular intervals is as follows: standing test (Romberg test), stepping test, and Meyer zum Gottesberge's head-shaking test were done once a week for a month after starting antituberculous injections and they were re-examined once every 2 weeks for at least 3 months after beginning the injections. After the 3 months these tests were done once a month. Eight cases of vestibular damage due to streptomycin or enviomycin could be easily detected at an early stage by performing Meyer zum Gottesberge's head-shaking test, together with the standing test and the stepping test. Vestibular dysfunction is apt to occur after about 1 month or within a month from the start of daily injections especially with streptomycin. Therefore, the method of equilibrium examination, we suggest, is that the Meyer zum Gottesberge's head-shaking test, the standing test (Romberg test), and the stepping test should be performed once a week during the first month after the start of this drug. When the result of the Meyer zum Gottesberge's head-shaking test is less than 50% and swaying and/or rotation occur in the stepping test, the drugs being given should be discontinued.

7年前,慢性骨髄性白血病と診断された患者がblast crisisをおこした. vincristine, cytosine arabinoside, 6-mercaptopurine, prednisoloneを投与したところ, 3日目には白血病細胞は著明に減少した.それと同時に血清カルシウムは2.7mEq/l,無機リンは13.1mg/dlとなつており,また血清クロールは著明な高値で動脈血ガスは高度の代謝性acidosisの所見を呈していた.その後高窒素血症が進行し,尿酸も上昇してきた.この間ずつと低カルシウム血症・高リン血症がつづいていたが,白血球数が3000/cummにまで減少し骨髄芽球が消失したのちは,血清電解質・動脈血ガスの異常も改善した.この患者の入院時血清creatinine値は軽度ながら上昇しており, creatinine clearanceは著しく低下していたが,今回の入院前後の血清カルシウム・無機リン値は正常であつた.本例にみられた治療前後のカルシウム・無機リンの異常および代謝性acidosisの成因は,抗白血病薬の投与によつて無数の白血病細胞が破壊され,それから放出された多量のたんぱくや有機酸が腎障害のために尿中へ充分量排泄できなかつたことによるものと考えられる.したがつて腎機能低下のある患者において抗腫瘍薬を用いる場合には,血清カルシウム・無機リン値ばかりでなく,血液ガスについても留意しなければならない.

Cardiac manifestations in 321 patients on chronic hemodialysis (dialysis duration of from 0.1 to 11.3 years) were examined with non-invasive technique. In contrast to the previous assumption that cardiac dilatation and pericardial effusion are common in patients on chronic hemodialysis, interventricular septal hypertrophy (51.3%) and left ventricular posterior wall hypertrophy (47.1%) on echocardiography and left ventricular hypertrophy (47.0%) on ECG were the most frequent abnormalities in these patients. Although premature beats on routine ECG were found in a limited number of patients (4.7%), ventricular premature beats were recognized in 45.7% and high grade arrhythmias in 27.2% of 92 patients examined with 24-hour Holter ECG monitoring. There were no high grade arrhythmias and the frequency of ventricular premature beats was 19.4% in 31 age-matched healthy subjects studied. Furthermore, the PQ interval was progressively prolonged in relation to the duration of hemodialysis. This finding may suggest that long-term hemodialysis is associated with disturbances of the conduction system. These results indicate that myocardial hypertrophy and high grade arrhythmias are the prominent findings in uremic patients maintained on hemodialysis. We consider that such high grade arrhythmias could be responsible for a high incidence of cardiac death in these patients.

症例. 47才,女性.主訴:呼吸困難,発熱.既往歴:妊娠歴2回.流産・輸血歴(一).現病歴:昭和59年8月20日婦人科入院中に施行した輸血中に呼吸困難,起座呼吸,発熱が出現したため内科転科となった.転科後経過:急速な症状の発現,スリガラス様胸部X線像影,湿性ラ音などより急性肺水腫と診断し,挿管下に呼吸,循環管理,ステロイド投与を行なったところ短時日で症状は軽快した.本患者血清中にはリンパ球細胞毒試験で供血者4人中3人,および夫と次子に対する抗白血球抗体が存在し, B7, BW61, BW48に対する抗体があると判明した.本症例では過去の輸血歴はなく,妊娠に伴い出現した抗HLA抗体により生じた急性肺水腫と考えられた.

Two patients had hypouricemia due to increased uric acid clearance. Their daily urinary uric acid excretions were normal. In both patients, pyrazinamide decreased urinary uric acid clearance to almost normal while probenecid increased it markedly. No other renal tubular or metabolic abnormalities were found. The serum electrolytes and the urinary electrolytes excretions showed no abnormalities. It appears that increased secretion of uric acid in the renal tubule is most probably responsible for hypouricemia in these patients. This report adds to the list of hypouricemic conditions due to a new isolated renal tubular abnormality. ( Arch Intern Med 1982;142:1855-1857)

症例は29才の女性で, 26才頃より耐寒性が低下し,皮膚が粗〓で便秘傾向であつた. 27才より無月経となり,甲状腺の精査を勧められ入院となつた.体温は35.2°C,甲状腺腫はなく,腋毛・恥毛は希薄であつた.心臓超音波断層検査で著明な心膜液の貯留を認め,甲状腺機能検査では, T3 3.5ng/dl, T4 0.3μg/dl, TSH 218μU/ml,基礎代謝率-56.5%であり,粘液水腫と診断された.一方,血清gastrinは6830pg/mlと正常の約70倍の高値を示し,末梢血で小球性低色素性貧血を認め,血清ビタミンB12は正常で,悪性貧血は伴つていなかつた.胃液検査では, basal acid output (BAO) 0.27mEq/h, maximum acid output (MAO) 0.28mEq/hと無酸症を呈し,胃底腺領域の生検所見は,萎縮性胃炎の像を呈していた.また,経口的胃内酸負荷では, gastrinは急速に低下したため,本例における高gastrin血症は,萎縮性胃炎に伴う負のフィードバックによると考えられた.本例では,抗甲状腺自己抗体が陰性であるが,一般に特発性甲状腺機能低下症は橋本病が成因と考えられ,一方type A萎縮性胃炎では,抗胃壁細胞抗体が陽性のことが多く,その進展に自己免疫現象の関与が唱えられている.悪性貧血も橋本病などの自己免疫疾患との合併が多く,その本体が萎縮性胃炎に伴うビタミソB12の吸収障害であることから,本例は,甲状腺疾患と胃病変の関連を考えるうえで示唆にとむ1例と考えられた.

症例72才,男性. Refractory anemia with excess of blasts (RAEB)の診断で5年前より経過観察を行なっていた.芽球の細胞起源を同定するため特殊染色を行なったところ, peroxidase (-), sudan black B (-), esterase (-), PAS (-), acid P (-)であり,モノクローナル抗体ではIa, OKT10のみが陽性を示し分類不能であった.遺伝子分析として,骨髄の単核球よりDNAを抽出し, T細胞抗原受容体(TCR)β鎖遺伝子および免疫グロブリン重鎖遺伝子をprobeとして用いて, Southern blot hybridaizationを行なったところ, TCRβ鎖遺伝子のみのmonoclonalな再構成を認め,本症例の芽球はT cell由来と推定された. RAEBの芽球は不明なものが多いが,その殆どは非リンパ性白血病に移行するといわれており,本症例はT cellの性格をもったRAEBである点で示唆に富むと考えられた.

Three patients with the syndrome of inappropriate secretion of antidiuretic hormone had elevated uric acid clearances. Their uric acid clearances decreased markedly after the administration of pyrazinamide. Probenecid was given to two of them and it produced large increases in uric acid clearance. These data suggest that enhanced secretion in the renal tubules was responsible for the increased clearance of uric acid. This article provides evidence that hypouricemia in the syndrome of inappropriate secretion of antidiuretic hormone is due to increased tubular urate secretion.

ルポイド肝炎に先天性第XI因子欠乏症と橋本病を合併し,経過中に循環抗凝血素が出現したと考えられる症例を経験した.症例は69才,女性.鼻出血の止血困難を主訴とし,肝機能異常,全血凝固時間と活性化部分卜ロンボプラスチン時間(APTT)の延長,甲状腺機能低下,各種自己抗体陽性所見が認められた.第XI因子活性は4.8%と著明な低値を示した.入院後, LE細胞陽性化に続いて肝胆道系酵素の上昇,内因系凝固因子活性の低下が観察され,循環抗凝血素の存在が確認された.プレドニゾロン投与後循環抗凝血素は消失したが,第XI因子活性低値は持続した,娘に第XI因子活性の低値が観察されたことから,第XI因子欠乏症は先天性であると考えられた.

A rare case of right atrial myxoma in which thallium-201 gave a good delineation of the tumor was presented. In this case, the feeding arteries were seen to be highly developed on coronary arteriogram. The amount of blood containing thallium-201 supplied to the tumor through the feeding arteries was so great that the tumor was considered to be visualized by thallium-201 imaging.