Successful mitral valve repair for active infective endocarditis accompanied by anorexia nervosa

Infective endocarditis of the mitral area accompanied by anorexia nervosa is extremely rare. A 34-year-old Japanese woman presented with high fever and a heart murmur that had developed over the previous 2-month period. Echocardiography revealed mitral regurgitation and vegetation attached to the anterior mitral leaflet, which had markedly prolapsed to the left atrium. We removed the vegetation with a small part of the anterior mitral leaflet and successfully repaired the mitral valve. The patient showed good recovery, and the mitral regurgitation and left ventricular chamber size had satisfactorily decreased at 2 months after the operation.     

A case of Nasu-Hakola's disease with T2-weighted MRI finding of reduced signal intensity in the thalamus and putamen]

A 30-year-old female received a head injury at the age of 22 years. Subsequently neurological and psychiatric symptoms, such as personality change, urinary incontinence, dementia and gait disturbance developed. On admission, her cognitive function was severely impaired. Brain CT disclosed cerebral atrophy, dilatation of the lateral ventricle and calcification of the basal ganglia. Pathologically membranous structures were recognized in bone marrow. On the basis of these clinical findings, a diagnosis of Nasu-Hakola's disease was made. In this case, a T2-weighted MRI finding of reduced signal intensity in the thalamus and putamen was characteristic. This finding may be related to intracranial calcification.     

Experience of thoracoscopic extirpation of intrapulmonary lymphangioma

We report a 59-year-old woman who underwent thoracoscopic extirpation of the intrapulmonary cystic lymphangioma which was located in the subpleural space of the left upper lobe. A chest X-ray showed a solitary round nodule in the left pulmonary hilum. A computed tomography scan revealed a mass shadow at the interlobar region surrounding the interlobar pulmonary artery. A round nodule was recognized as low signal intensity on T1-weighted images by magnetic resonance imaging. There was biphasic signal intensity on T2-weighted images as well. Radiologically, we diagnosed this lesion as a benign cystic tumor in the lung. Thoracoscopic observation revealed a cystic lesion just beneath the visceral pleura of the upper lobe adjacent to the interlobular pulmonary artery. As this suggested a benign bronchogenic cyst, we performed extirpation of the cyst under thoracoscopy. After this operation, the cyst was diagnosed as an intrapulmonary cystic lymphangioma pathologically.     

The infeasibility of using ten-ring irradiated grafts for tracheal allotransplantation even with omentopexy

In our previous study, we demonstrated that high-dose ⁶⁰Co irradiation was able to prevent rejection of canine tracheal allografts. To determine the maximum possible length of these grafts, in the present study we attempted to transplant five-ring and ten-ring tracheal allografts in two groups of five dogs each. Either five or ten rings were excised from donor tracheas and irradiated with 100,000 cGy of ⁶⁰Co. The irradiated tracheal grafts were transplanted to replace either five- or ten-ring sections of the mediastinal tracheas removed from the recipient dogs. The grafts were covered with omental pedicles and no immunosuppressants were used. Graft incorporation was achieved in four of the five dogs in the five-ring group, and three of these dogs survived for more than 700 days. However, four of the five animals in the ten-ring group died from tracheostenosis accompanied by ischemia within 3 weeks. These findings demonstrate the impossibility of performing ten-ring tracheal allotransplantation using irradiated grafts, even with omentopexy.     

Semaphorin3A signalling is mediated via sequential Cdk5 and GSK3beta phosphorylation of CRMP2: implication of common phosphorylating mechanism underlying axon guidance and Alzheimer's disease

Collapsin response mediating protein-2 (CRMP2) has been identified as an intracellular protein mediating Semaphorin3A (Sema3A), a repulsive guidance molecule. In this study, we demonstrate that cyclin-dependent kinase 5 (Cdk5) and glycogen synthase kinase 3beta (GSK3beta) plays a critical role in Sema3A signalling. In In vitro kinase assay, Cdk5 phosphorylated CRMP2 at Ser522, while GSK3beta did not induce any phosphorylation of CRMP2. Phosphorylation by GSK3beta was exclusively observed in Cdk5-phosphorylated CRMP2, but barely in CRMP2T509A. These results indicate that Cdk5 primarily phosphorylates CRMP2 at Ser522 and GSK3beta secondarily phosphorylates at Thr509. The dual-phosphorylated CRMP2, but not non-phosphorylated or single-phosphorylated CRMP2, is recognized with the antibody 3F4, which is highly reactive with the neurofibrillary tangles of Alzheimer's disease. 3F4 recognized the CRMP2 in the wild-type but not cdk5-/- mouse embryonic brain lysates. The phosphorylation of CRMP2 at Ser522 caused reduction of its affinity to tubulin. In dorsal root ganglion neurones, Sema3A stimulation enhanced the levels of the phosphorylated form of CRMP2 detected by 3F4. Over-expression of CRMP2 mutant substituting either Ser522 or Thr509 to Ala attenuates Sema3A-induced growth cone collapse response. These results suggest that the sequential phosphorylation of CRMP is an important process of Sema3A signalling and the same mechanism may have some relevance to the pathological aggregation of the microtubule-associated proteins.     

Solid and Cystic Tumor of the Pancreas in an Adult Male

A solid and cystic tumor (SCT) was located at the head of the pancreas in a 43-year-old Japanese male, and pancreatoduodenectomy was performed on the suspicion of papillary carcinoma or cystadenocarcinoma of the pancreas. The lesion, which measured 4.5 X 4.5 X 4.0 cm, was clearly demarcated by connective tissue. The cut surface showed solid grayish-white areas with central cystic degenerative changes. The solid areas consisted of small round cells proliferating in a small solid or a pseudopapillary pattern. The tumor cells partially invaded the surrounding normal pancreatic parenchyma. Immunohistochemical studies revealed positive staining for alpha-1-antitrypsin and neuron-specific enolase, but no staining for known pancreatic hormones. Moreover, ultrastructural studies showed the absence of zymogen granules and the presence of anullate lamellae and neurosecretory granules. On the basis of these findings, a diagnosis of SCT of the pancreas was established. In order to clarify the histogenesis and biological behavior of the tumor, it is necessary to accumulate and analyze similar cases, an endeavor which in turn will contribute to the successful management of this disease. Acta Pathol Jpn 41: 763-770, 1991.