Giant cell granulamatous hypophysitis with remarkable uptake on Gallium-67 scintigraphy

We treated a man with giant cell granulomatous hypophysitis with pituitary enlargement, as seen on magnetic resonance imaging. Endocrinological examination revealed panhypopituitarism and diabetes insipidus. Microscopic examination of the specimen obtained by transsphenoidal pituitary biopsy revealed a granulomatous lesion, composed of epitheliod cells, Langhans' multinucleated giant cells, lymphocytes and other chronic inflammatory cells. On whole body gallium-67 scintigraphy, there was extensive uptake in the pituitary gland. Gallium-67 scintigraphy may greatly aid in the diagnosis of granulomatous hypophysitis.     

A Case of Penetration of the Large Intestinal Wall by a Toothpick

A 66-year-old man was admitted to hospital with symptoms of acute abdomen. Based on colonoscopic findings, localized peritonitis due to penetration of the sigmoid colon by a toothpick was diagnosed. During colonoscopy, the toothpick was removed using a grasping forceps, meaning that surgery was avoided. In cases where abdominal pain of unknown origin is observed, physicians should proceed with routine medical care while considering the possibility of foreign body ingestion, and work toward an early diagnosis.     

Molecular analysis and diagnosis in Japanese patients with Wilson''s disease

BACKGROUND: Wilson's disease is characterized by the toxic accumulation of copper in the liver, brain, cornea and other organs. It is caused by both impaired excretion via the bile and impaired incorporation of copper into ceruloplasmin in the liver. The Wilson's disease gene (ATP7B) has been cloned as a putative copper-transporting P-type ATPase gene. We therefore analysed mutations of ATP7B in Japanese patients with Wilson's disease. METHODS: Twenty-three Japanese patients with Wilson's disease were investigated. In all patients, the ATP7B coding sequence, including exon-intron junctions, was analysed by restriction endonuclease digestion, mutation detected enhancement gel electrophoresis and/or direct sequencing analysis of amplified fragments. RESULTS: Thirteen mutations were identified, including seven missense mutations, four detections, one insertion and one exon skipping in the coding region. The most common mutations were 2874deletion(del)C in exon 13 and arginine (Arg)778 leucine (Leu) in exon 8. DISCUSSION: None of the observed mutations, except for 2302insertion(ins)C, have been previously detected in either European or North American patients. We conclude that the mutation spectrum of Wilson's disease may thus indicate a population-dependent pattern. Based on the population-dependent manner of the occurrence of ATP7B gene mutations, it may be possible to establish a molecular diagnosis system. A molecular diagnosis system is considered to be very effective for making a definitive diagnosis in very young patients and for also detecting carriers.     

Development of the Terumo Capiox Centrifugal Pump and Its Clinical Application to Open Heart Surgery: A Comparative Study with the Roller Pump

Abstract: We have developed the Terumo Capiox centrifugal pump (CXP), which consists of a rotor having a unique straight-path design to reduce pump rotational speed without decreasing hydraulic efficiency. The CXP was tested in vitro for blood trauma with a specially designed test circuit using fresh bovine blood. The Biopump (BP) (Medtronic, Minneapolis, MN, U.S.A.) and the roller pump (RP) were used as controls. The CXP demonstrated the smallest elevation of free plasma hemoglobin compared with the BP and the RP. The CXP was then applied to cardiopulmonary bypass (CPB) in 10 patients (CXP group) who underwent elective coronary artery by-pass grafting (CABG), and the results were compared with those for a comparable roller pump group (RP group). Free plasma hemoglobin level, platelet count, and serum β -thromboglobulin ( β -TG) level were measured during CPB. There were no CXP-related complications nor hemodynamic abnormalities during CPB. The CXP group demonstrated less hemolysis and less platelet depletion than the RP group. Furthermore, the serum β -TG level was significantly lower in the CXP group than in the RP group. The CXP showed excellent hemodynamic performance with less blood trauma both in vitro and in clinical application to open heart surgery. Thus, the CXP has significant potential to be safely applied to CPB for open heart surgery and circulatory support.     

Status of plasmapheresis for the treatment of toxic epidermal necrolysis in Japan

This study describes the efficacy of plasmapheresis for the treatment of toxic epidermal necrolysis (TEN), as reported in Japan. TEN patients treated with plasmapheresis were collected from Japanese literature. The type of plasmapheresis, number of sessions, efficacy of plasmapheresis, and present outcome were examined. Forty-seven TEN patients treated with plasmapheresis have been reported in the literature: 19 men and 28 women with ages ranging from 1 to 96 years (mean 50.3 years). Twenty-five of these treatments included simple plasma exchange (PE), 13 included double filtration plasmapheresis (DFPP), and one included PE and DFPP. The number of plasmapheresis sessions ranged from 1 to 6 and the mean number of sessions was 3.1. The efficacy of plasmapheresis for the treatment of TEN was as follows: excellent, 30 cases; good, 8 cases; fair, 3 cases; no effect, 5 cases; and unknown, 1 case. The rate of effectiveness was 80.9%; eleven patients died; and the mortality rate was 23.4%. In summary, plasmapheresis, including both PE and DFPP, were found to be effective treatments for Japanese patients with TEN, who had been unresponsive to corticosteroids, and for those who show severe clinical manifestations.     

Induction of apoptotic cell death by direct-current treatment in human leukemic cell lines

Exposure of human leukemic cell lines (HL-60, ML-1, U-937, MOLT-4, EOL-1) to short direct-current (d.c.) treatment induced apoptotic cell death, characterized by cell shrinkage and nuclear and internucleosomal DNA fragmentation. On the other hand, human peripheral blood lymphocytes and polymorphonuclear cells were relatively resistant to d.c treatment, and did not show any clear nuclear and DNA fragmentation. The effect of d.c. was slightly reduced by calcium depletion, but was not significantly affected by catalase or by superoxide dismutase. The present data suggest that previously reported tumor regression activities of d.c. treatment might be due, at least in part, to its apoptosis-inducing activity.     

Unusual variant of left paraduodenal hernia herniated into the mesocolic fossa leading to jejunal strangulation

Paraduodenal hernia is a rare condition in which the small bowel loops are herniated into an unusual fossa in the periduodenal area. We treated a patient with paraduodenal hernia diagnosed pre-operatively. A 28-year-old woman was admitted to our hospital because of intermittent abdominal pain. Abdominal ultrasonography revealed a large tumor adjacent to the pancreas. Provisional diagnosis made according to computed tomography (CT) findings was tumor of the pancreas tail. However, on a CT scan performed after the administration of diatrizoate meglumine/diatrizoate sodium (Gastrografin, Schering, Berlin, Germany) the mass was shown as a jejunum loop located between the stomach and the pancreas body. Subsequent laparotomy revealed that the jejunum loop was herniated into an unusually large mesocolic fossa and that the hernial orifice was covered by the adhesion between the transverse and descending colons. It seemed that the small intestine within the mesocolic fossa was strangulated by this adhesion. The patient's abdominal pain resolved postoperatively. These observations suggest that paraduodenal hernia should be suspected in patients with chronic, atypical abdominal pain, regardless of the findings for small bowel obstruction. (Received Oct. 6, 1997; accepted Jan. 23, 1998)     

Specificity of polymerase chain reaction-based clonality analysis of immunoglobulin heavy chain gene rearrangement for the detection of bone marrow infiltrate in B-cell lymphoma-associated haemophagocytic syndrome

As a wide range of disorders underlie haemophagocytic syndrome, a rapid distinction between benign polyclonal and malignant monoclonal lymphoid proliferations is critical. We investigated whether polymerase chain reaction (PCR) amplification of immunoglobulin heavy chain gene rearrangement could efficiently detect clonal B-cell populations in non-diagnostic marrow for B-cell lymphoma-associated haemophagocytic syndrome (B-LAHS). On amplifying two DNA samples per biopsy, no reproducible monoclonal PCR result was found in reactive haemophagocytic marrows. In contrast, four out of nine assessable B-LAHS patients with histomorphologically and immunohistochemically lymphoma-free bone marrow showed a reproducible monoclonal immunoglobulin heavy chain gene rearrangement. At the molecular level, two B-LAHS patients had lymphoma-free marrow as demonstrated by patient-specific PCR, suggesting that haemophagocytic marrow is not always associated with lymphoma involvement. PCR-based demonstration of clonal B-cell populations in marrow would add an extra dimension to B-LAHS diagnosis.     

Hilar cholangiocarcinoma associated with sarcoid reaction in the regional lymph nodes

A 74-year-old woman was admitted to our hospital with a 2-week history of jaundice. Percutaneous transhepatic cholangioscopy revealed a nodular tumor originating in the upper part of the common hepatic duct, which was invading the confluence of the right and left hepatic ducts. Microscopic examination of biopsy specimens revealed adenocarcinoma. Abdominal ultrasonography and computed tomography demonstrated multiple enlarged lymph nodes around the extrahepatic bile duct and the common hepatic artery. Laparotomy revealed lymph node enlargement in the hepatoduodenal ligament, behind the pancreatic head, and along the common hepatic and left gastric arteries. Extended left hepatic lobectomy, caudate lobectomy, and resection of extrahepatic bile duct with extended lymph node dissection were performed. The histology of permanent specimen revealed no tumor metastasis but a sarcoid reaction in the lymph nodes. The patient is in good health 21 months after the operation, without any evidence of recurrence. This is the first successfully resected case of hilar cholangiocarcinoma associated with sarcoid reaction in the regional lymph nodes.