Living-donor liver transplantation in 126 patients with biliary atresia: single-center experience.

OBJECTIVES: To describe our experience with 126 consecutive living-donor liver transplantation (LDLT) procedures performed because of biliary atresia and to evaluate the optimal timing of the operation. PATIENTS AND METHODS: Between May 2001 and January 2010,126 patients with biliary atresia underwent 130 LDLT procedures. Mean (SD) patient age was 3.3 (4.2) years, and body weight was 13.8 (10.7) kg. Donors included 64 fathers, 63 mothers, and 3 other individuals. The left lateral segment was the most commonly used graft (75%). Patients were divided into 3 groups according to body weight: group 1, less than 8 kg (n = 40); group 2,8 to 20 kg (n = 63); and group 3, more than 20 kg (n = 23). Medical records were reviewed retrospectively. Follow up was 4.5 (2.7) years. RESULTS: All group 3 donors underwent left lobectomy, and all group 1 donors underwent left lateral segmentectomy. No donors required a second operation or died. Comparison of the 3 groups demonstrated that recipient Pediatric End-Stage Liver Disease score in group 1 was highest, operative blood loss in group 2 was lowest (78 mL/kg), and operative time in group 3 was longest (1201 minutes). Hepatic artery complications occurred more frequently in group 1 (17.9%), and biliary stenosis (43.5%) and gastrointestinal perforation (8.7%) occurred more frequently in group 3. The overall patient survival rates at 1, 5, and 9 years was 98%, 97%, and 97%, respectively. Five-year patient survival rate in groups 1,2, and 3 were 92.5%, 100%, and 95.7%, respectively. Gastrointestinal perforation (n = 2) was the primary cause of death. CONCLUSIONS: Living-donor liver transplantation is an effective treatment of biliary atresia, with good long-term outcome. It seems that the most suitable time to perform LDLT to treat biliary atresia is when the patient weighs 8 to 20 kg.

Thiazolidinediones provide better renoprotection than insulin in an obese, hypertensive type II diabetic rat model.

Hyperinsulinemia has been implicated in the development of diabetic nephropathy. In the present study we compared the renoprotective effects of the thiazolidinedione, pioglitazone (PGZ), to that of insulin in a hypertensive, obese, type II diabetic rat model. PGZ aggravated obesity and gave less glycemic control than insulin. However, renoprotection was markedly better with PZG compared to insulin as shown by lower proteinuria, improved renal function, and less histological evidence of diabetic glomerular and tubulointerstitial lesions. PZG and insulin both reduced renal accumulation of pentosidine and oxidative stress to a similar extent. In contrast, PGZ but not insulin suppressed enhanced transforming growth factor-beta (TGF-beta) expression. We further confirmed in cultured rat proximal tubular cells that insulin enhanced TGF-beta mRNA expression and protein production. Our results identify hyperinsulinemia and the attendant increase of TGF-beta expression as potential therapeutic targets in diabetes independent of glycemic control. This confirms prior clinical evidence that PZG provides renoprotection in obese, diabetic patients with nephropathy.

Pilocytic astrocytoma arising from an area of nodular heterotopia located in the white matter of the temporal lobe: case report.

A 16-year-old girl suffering from intractable temporal lobe epilepsy presented with a pilocytic astrocytoma, which occurred in an area of nodular heterotopia located in the white matter of the temporal lobe. The pilocytic astrocytoma appeared to be covered by an area of gliosis, which contained numerous Rosenthal fibers, while in the lesion the pilocytic astrocytoma occupied a small area. The gliosis eventually became a tumor-like lesion. The white matter around the mass was composed of gliosis with nodular heterotopia. Temporal developmental malformation, which was a basic lesion of the patient, might therefore be a precursor lesion of pilocytic astrocytomas.

Hemispheric asymmetry of the auditory evoked N100m response in relation to the crossing point between the central sulcus and Sylvian fissure.

The positions of the bilateral N100m sources of the auditory evoked magnetic fields (AEFs) were measured in relation to the central sulcus (CS) using an MRI-linked whole head magnetoencephalography system in 20 right-handed normal male subjects. The location of the N20m source of the median nerve-stimulated somatosensory evoked magnetic fields (SEFs), in the left hemisphere was 3.9+/-5.4 mm (mean+/-SD) posterior to that in the right hemisphere (P < 0.005). The crossing point (CP) between the CS and Sylvian fissure in the left hemisphere was 4.3+/-4.8 mm posterior to that in the right hemisphere (P < 0.001). The N100m sources were posterior to the CP in both hemispheres. The left hemispheric N100m source was 9.4+/-6.4 mm posterior to that on the right (P < 0.0001) in absolute position. The relative distance between CP and the N100m source was 22.7+/-8.5 mm in the left hemisphere and 17.7+/-5.3 mm in the right hemisphere (P < 0.01). Comparison of positions of the AEF sources and the CS as defined by the SEF demonstrated functional asymmetry of the human temporal lobe and possible source extension of the AEF-N100m beyond the Heschl gyrus over the planum temporale.

[Interhemispheric asymmetry exists in female in the N100m source position of the auditory evoked magnetic fields].

The N100m source of the auditory evoked magnetic fields (AEFs) is located more posterior in the left than the right hemisphere in male subjects. However, whether this asymmetry exists in female subjects is controversial. The present study, analyzed the N100m source positions of the AEFs due to monaural tone stimuli using a helmet-shaped 66-channel magnetoencephalography system (CTF Systems) in 62 right-handed normal adults (24 females and 38 males). The best fit sphere was calculated for each subject from the head shape reconstructed from three-dimensional magnetic resonance images. N100m source positions were estimated using a double-dipole model. The contralateral N100m response to the stimulated ear was measured relative to the midpoint of the bilateral auricular points. Statistical analysis used a standardized head size for each subject based on the mean value of all subjects (r = 8.27 cm). In females, the N100m dipole positions were 0.00 +/- 1.02 cm anterior in the left hemisphere and 0.35 +/- 0.74 cm in the right (p < 0.02). In males, the N100m dipole positions were -0.31 +/- 0.81 cm (mean +/- standard deviation) anterior in the left hemisphere and 0.61 +/- 0.78 cm in the right (p < 0.0001). The interhemispheric difference in the N100m positions was 0.35 +/- 0.65 in females and 0.92 +/- 0.77 in males (p < 0.005). In conclusion, the left hemispheric N100m is located more posterior to the right in both genders. However, this functional asymmetry is more evident in males, like previous findings of more evident anatomical asymmetry in males.

Neuromagnetic evaluation of cortical auditory function in patients with temporal lobe tumors.

The N100m wave response of the auditory evoked magnetic field originates in the posterior part of the bilateral superior temporal planes for either contra- or ipsilateral ear stimulus. Cortical auditory function was evaluated in 14 patients with temporal lobe tumors using a magnetic resonance (MR) imaging-linked whole-head magnetoencephalography (MEG) system. Before surgery, seven patients had normal N100m latency (within the range of the mean +/- 2 standard deviations of 37 normal volunteers) in both normal hemispheres and in those with lesions, and MR imaging indicated no tumor invasion or edema in the posterior one-third of the superior temporal planes, even when the sylvian fissure was shifted upward due to the mass effect. Seven patients had prolonged N100m latency or absence of N100m in the hemisphere containing the lesion, and the posterior portion of the superior temporal plane was involved by the tumor or perifocal edema. Prolonged N100m latency recovered to the normal range after removal of tumors in two of four patients investigated postoperatively. The MEG system can be used to evaluate cortical auditory function noninvasively before and after surgical treatment of temporal lobe tumors.

Clinical application of visual evoked fields using an MRI-linked whole head MEG system.

We have demonstrated anatomo-functional correlation of the brain visual function using an MRI-linked whole-head magnetoencephalography system. Visual evoked magnetic fields (VEFs) due to pattern reversal stimuli were measured in seven healthy subjects and 13 patients with intracranial structural lesions. Full-field stimuli evoked the most prominent peak around 100 ms latency (P100m) as a two-dipole pattern over the occipital area in all of the normal subjects and four patients with mild and partial hemianopsia. In five patients with homonymous hemianopsia due to unilateral occipital lesion, a single-dipole pattern of the P100m appeared over the normal occipital area only. In four patients with bitemporal hemianopsia due to pituitary tumors, a single-dipole pattern of the P100m appeared only over the ipsilateral occipital area to the stimulated eye. Using current dipole models, P100m sources were localized at the lateral bottom of the calcarine fissures. Although human occipital lobes are known to be morphologically variable, our results indicate excellent correlation of the cortical anatomy and human visual function. Full-field stimuli, requiring no strict visual fixation, are especially useful for clinical application. A clear two-dipole pattern in the normal VEFs enables us to compare two hemispheric activities.

Protective effects of S-sulfonated human gamma globulin against experimental infections in mice.

S-sulfonated gamma globulin (GGS), newly developed as a safe drug for intravenous use, was studied for its protective effects against some experimental infections in mice. Gamma globulin showed a good protective activity against infections due to Streptococcus pneumoniae and Escherichia coli and was moderately active against infections due to Staphylococcus aureus and Pseudomonas aeruginosa. In most cases, the potency of GGS was almost the same as that of original native gamma globulin. The duration of GGS activity in vivo was found to be comparable to that of native gamma globulin and much higher than that of pepsin-digested gamma globulin. In the control of infection due to E. coli, specific antibody was found to play a central role in the antibacterial action of GGS. When GGS was administered in combination with the antibiotics gentamicin and cefazolin for the control of infections due to S. pneumoniae or E. coli, a clear synergistic effect was observed.