Long-term outcomes of 600 living donor liver transplants for pediatric patients at a single center.

This report concerns the long-term outcome of living donor liver transplantation (LDLT) for pediatric patients at a single center. Between June 1990 and December 2003, a total of 600 LDLTs, including 568 primary transplantations and 32 retransplantations, were performed for pediatric patients, who were immunosuppressed with FK506 and low-dose corticosteroids. Patient survival at 1, 5, and 10 years were 84.6%, 82.4%, and 77.2%, respectively, and the corresponding findings for graft survivals were 84.1%, 80.9%, and 74.5%. Multivariate analysis demonstrated that fulminant hepatic failure (FHF), a graft vs. body weight (GBWR) ratio of <0.8, and ABO-incompatible transplants were independently associated with both patient and graft survival. The retransplantation rate was 6%, and 55 patients (9.7%) have been completely weaned off immunosuppressants. Long-term patient and graft survival after pediatric LDLT for a large cohort of children at our hospital were found to be as good as those for cadaveric liver transplantation, although this series includes 13% liver transplantations with ABO-incompatible donors, which are obviously inferior in patient and graft survival. To obtain better outcomes for patients with FHF and for patients with ABO-incompatible transplants, immunosuppressive therapy needs to be improved.     

Nucleolar organizer regions in meningioma

Seventy-eight cases of meningioma and related tumors were examined independently using a simple and reproducible argyrophilic method for the demonstration of nucleolar organizer regions (AgNORs) and staining with bromodeoxyuridine monoclonal antibody. The mean number of AgNORs per cell and the bromodeoxyuridine labeling index were shown to be linearly related (r = 0.84, P less than 0.001). The mean AgNOR number was 2.99 for meningeal sarcoma, 2.29 for anaplastic meningioma, 2.08 for hemangiopericytic meningioma. 1.72 for recurrent meningioma without atypical histological findings, and 1.52 for nonrecurrent meningioma. We noted that the mean number of AgNORs reflected the cellular kinetics of a tumor and was related to histological grade and clinical behavior.     

Cerebral sparganosis mansoni. Report of two cases

Among diseases due to cerebral parasitism, that caused by Sparganum mansoni, the larva of Spirometra mansoni, is very rare. We have encountered two such cases. A computed tomography scan in both revealed a nodular high density contrast enhanced area against an extensive low density background area. Neither calcification nor cyst formation was recognized. These computed tomography scan findings were thought to be characteristic for cerebral sparganosis mansoni and were difficult to differentiate from those of a cerebral tumor. In both cases, definitive diagnosis was achieved by identification of the worm after excision of the lesion. The best treatment for cerebral sparganosis mansoni is surgical excision of the lesion, and in the two cases presented the postoperative outcome was good.     

Substance P- and calcitonin gene-related peptide-immunofluorescent nerves in the repair of experimental bone defects

Summary Healing of an experimental bony defect in the rat's tibia was studied with an immunofluorescent technique to clarify when and where substance P (SP) and calcitonin gene-related peptide (CGRP) would develop. The normal tibia showed a few SP- and CGRP-immunofluorescent nerve fibres. In the experimental tibia, the number of these fibres increased on the 6th day after operation, reached a peak of proliferation on the 15th day and reverted to normal after the 24th day. The changes were associated with the development and decay of callus tissue suggesting that harmful stimuli from the injured site in a bone could be mediated by sensory nerves throughout the repair period. Most of the SP- and CGRP-immunofluorescence was seen near the vessels, frequently in the same nerve fibres. The SP- and CGRP-immunofluorescent nerves seemed to take part jointly in callus formation through the enhancement of local blood flow.

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Résumé Le procesus de guérison d'une perte de substance osseuse expérimentale a été étudié sur le tibia du rat par immunofluorescence afin de déterminer quand et où la substance P (SP) et la calcitonine peptide d'origine génique (CGRP) se développeraient. Le tibia normal ne montre qu'un petit nombre de fibres nerveuses immunofluorescentes SP et CGRP. Dans le tibia d'expérimentation, les fibres nerveuses immunofluorescentes SP et CGRP augmentent en nombre à partir du 6ème jour, atteignent leur maximum de prolifération le 15ème jour et reviennent à l'état normal après le 24ème jour post-opératoire. Ces modifications sont étroitement associées au développement et à la disparition du cal, suggérant ainsi que les stimuli nocifs provenant de la lésion osseuse pourraient être inactivés par les nerfs sensitifs durant la période de réparation. En outre, la plus grande partie de l'immunofluorescence SP et CGRP a été observée à proximité des vaisseaux, souvent dans les mêmes fibres nerveuses. Il semble que les nerfs immunofluorescents SP et CGRP participent conjointement à la formation du cal en augmentant la vascularisation locale.

     

Ruptured congenital aneurysm of the sinus of valsalva in the aged

Ruptured aneurysm of the sinus of Valsalva is a rare cardiac lesion. A ruptured aneurysm of the sinus of Valsalva in the right ventricle of a 64-year-old man was successfully repaired. The patient was admitted to the hospital with high fever and chest oppression. Diagnosis was made by two dimensional echocardiography, cardiac catheterization, and cardiac angiography. An aortotomy, main pulmonary arteriotomy, and right ventriculotomy were performed. There was no VSD, and the aneurysm originated from the right coronary sinus, rupturing into the right ventricle inlet portion. The ruptured aneurysm of the sinus of Valsalva was closed with a Dacron patch from inside the aorta. He is doing well after surgery. There was no heart murmur. CTR decreased and pulmonary blood flow fell to a normal value. As far as we know, this patient is the second oldest patient in Japan with surgical repair.     

Changes in polyamine metabolism in rat liver after oral administration of alpha-naphthylisothiocyanate

Changes in the levels of urea cycle enzymes and polyamine metabolism in the liver of rats treated with alpha-naphthylisothiocyanate (ANIT), an inducer of experimental cholestasis, were studied. Activities of arginase increased approximately two-fold compared to the control values during the period of 24-72 hours after oral administration of ANIT (100 mg/kg), while activities of ornithine carbamyltransferase and ornithine aminotransferase decreased. The activity of ornithine decarboxylase was elevated by approximately 20- and 10-fold at 12 and 60 hours, respectively, after ANIT administration. Putrescine concentration doubled 24-48 hours after the ANIT administration, but spermidine level rose more slowly and reached the level of 1.5-fold of the control level in 36-72 hours. Spermine concentration decreased initially but increased in 96 hours. These results suggest that the increased activity of urea cycle accounts for the increase in the ornithine content and that the putrescine and spermidine acts as the initiator of recovery of the liver damaged by ANIT treatment.     

Bleeding from foveolar hyperplasia developing on a gastrointestinal stromal tumor of the stomach

A 52‐year‐old Japanese woman who presented with gastrointestinal (GI) bleeding underwent a proximal gastrectomy for a gastrointestinal stromal tumor (GIST) with a foveolar hyperplasia at the apex of the tumor, 4.5 cm in size, located in the upper body of the stomach. Although GIST are often asymptomatic and are found only incidentally, clinical symptoms such as bleeding, abdominal pain, or obstruction, occasionally lead to a premorbid diagnosis. When submucosal tumors present GI bleeding, the source of the bleeding usually is an ulceration of the mucosa over the tumor. However, in the present study, it was thought that the bleeding originated from the region of foveolar hyperplasia.     

The structure and mode of action of different botulinum toxins

The seven serotypes (A–G) of botulinum neurotoxin (BoNT) are proteins produced by Clostridium botulinum and have multifunctional abilities: (i) they target cholinergic nerve endings via binding to ecto‐acceptors (ii) they undergo endocytosis/translocation and (iii) their light chains act intraneuronally to block acetylcholine release. The fundamental process of quantal transmitter release occurs by Ca²⁺‐regulated exocytosis involving sensitive factor attachment protein‐25 (SNAP‐25), syntaxin and synaptobrevin. Proteolytic cleavage by BoNT‐A of nine amino acids from the C‐terminal of SNAP‐25 disables its function, causing prolonged muscle weakness. This unique combination of activities underlies the effectiveness of BoNT‐A haemagglutinin complex in treating human conditions resulting from hyperactivity at peripheral cholinergic nerve endings. In vivo imaging and immunomicroscopy of murine muscles injected with type A toxin revealed that the extended duration of action results from the longevity of its protease, persistence of the cleaved SNAP‐25 and a protracted time course for the remodelling of treated nerve–muscle synapses. In addition, an application in pain management has been indicated by the ability of BoNT to inhibit neuropeptide release from nociceptors, thereby blocking central and peripheral pain sensitization processes. The widespread cellular distribution of SNAP‐25 and the diversity of the toxin's neuronal acceptors are being exploited for other therapeutic applications.