Successful outcome with extended allograft ischemic time in pediatric heart transplantation.

BACKGROUND: Many cardiac transplant programs have liberalized donor eligibility criteria in an attempt to maximize donor supply and to accommodate increasing demand. Although many studies have evaluated the potential adverse effects of prolonged donor ischemic time (DIT) in adults undergoing cardiac transplantation, relatively few have focused specifically on pediatric recipients that include a substantial number of patients and long-term follow-up. The focus of this study was to examine the effect of extended DIT on mortality after pediatric heart transplantation. METHODS: We conducted a retrospective review of our pediatric cardiac transplant experience in the past 11 years, comparing patients who received allografts and had ischemic times >240 minutes with those who had ischemic times <240 minutes. RESULTS: A total of 129 pediatric patients (<19 years) underwent orthotopic heart transplantation, of whom 78 (60.5%) had DIT <240 minutes and 51 (39.5%) had DIT >240 minutes. We found no statistically significant difference in age, sex, race, height, weight, or donor age between the groups (p = not significant). Post-transplant survival at 1, 5, and 10 years was similar for both groups: 91.2%, 88.0%, and 85.2%, respectively, for patients with DIT <240 minutes vs 89.6%, 87.2%, and 79.8%, respectively, for patients with DIT >240 minutes (p = 0.433). Additionally, using Cox proportional hazard models, extended DIT >240 minutes was not a statistically significant independent predictor of post-transplant mortality (odds ratio, 0.655; 95% confidence interval, 0.518-0.972; p = 0.684; standard error = 0.468). CONCLUSION: Procurement of hearts from distant locations with associated extended DIT is justified in the setting of increased demand and a fixed donor population.     

Pattern-reversal electroretinograms and visual evoked potentials in branch retinal vein occlusion

We recorded pattern electroretinograms and visual evoked potentials in a group of selected patients with unilateral uncomplicated branch retinal vein occlusion. To document the effects of preexisting risk factors, patients were divided into three groups: diabetes mellitus, hypertension with hyperlipidemia and no systemic disease. The transient and steady-state pattern electroretinogram and visual evoked potential amplitudes were significantly reduced and visual evoked potential peak times were delayed relative to the fellow eyes and agematched normal subjects. There was a second amplitude reduction relative to the other patient groups in both the affected and fellow eyes of the diabetes mellitus group, which was indicative of an additive effect of diabetes mellitus.Abbreviations BRVO branch retinal vein occlusion     

Comparison of CT-guided sclerotherapy with using 95% ethanol and 20% hypertonic saline for managing simple renal cyst.

OBJECTIVE: We wanted to compare the efficacies of 95% ethanol and 20% hypertonic saline (HS) sclerotherapies that were performed in a single session under CT guidance for the management of simple renal cysts. MATERIALS AND METHODS: A prospective series of 74 consecutive patients (average age: 57.6 +/- 8.1 years) with simple renal cysts were enrolled in this study. They were randomized into two groups and 95% ethanol or 20% HS, respectively, corresponding to 25% of the aspiration volume, was injected. Treatment success was determined six months later with follow-up clinical evaluation and performing ultrasonography. RESULTS: The sclerotherapy was accepted as technically successful without major complications in all except two patients who were excluded because of a communication between the simple renal cyst and the pelvicalyceal collecting system. Thirty-six patients in the ethanol group received sclerotherapy with 95% ethanol and 36 patients in the HS group underwent sclerotherapy with 20% HS. The complete regression ratio of the ethanol group was significantly higher (94% versus 72%, respectively) than that of the HS group. There was one patient with partial regression in each group. The failure ratio of the ethanol group was significantly lower (3% versus 25%, respectively) than that of the HS group. CONCLUSION: Ethanol sclerotherapy under CT guidance is a successful and safe procedure and it can be used for the treatment of simple renal cysts. Sclerotherapy with 95% ethanol is more effective than 20% HS sclerotherapy. Sclerotherapy with HS may be an option for patients preferring to undergo a less painful treatment procedure.     

Effects of gamma-hydroxybutyrate on cerebrospinal fluid lactate and glucose levels after spinal cord trauma.

This study aims to evaluate the effects of gamma-hydroxybutyrate (GHB) after spinal cord trauma (SCT). Twenty rabbits were divided equally into four groups: group I was the sham-operated group, group II suffered from SCT but received no treatment, group III was given a dose of 400 mg/kg of GHB intravenously before SCT and group IV received the same dose after SCT. Cerebrospinal fluid (CSF) samples were obtained 30 min before SCT (T(0)), at 60 (T(1)) and 120 min (T(2)) after SCT. There was a threefold increase in lactate levels from baseline value at T(2) in group II, while statistically significant elevation of the lactate levels were not observed in groups III and IV. Glucose levels at T(1) and T(2) were significantly lower in groups III and IV compared with the control group. The findings of this study demonstrate that GHB can control the increase of CSF lactate and glucose levels following SCT and that this metabolic effect may be associated with neuroprotective physiological changes.     

Anatomical study of the communicating branches between the medial and lateral plantar nerves

The plantar areas of the foot have specific biomechanical characteristics and play a distinct role in balance and standing. For the forefoot surgeon, knowledge of the variations in the anatomy of communicating branches is important for plantar reconstruction, local injection therapy and an excision of interdigital neuroma. The anatomy of the communicating branches of the plantar nerves between the fourth and third common plantar digital nerves in the foot were studied in 50 adult men cadaveric feet. A communicating branch was present between the third and fourth intermetatarsal spaces nerves in all eight left feet and in six right feet (overall, 28%), and absent in 36 (72%). A communicating branch was found in 14 ft. Ten of the 14 communications were from the lateral to the medial plantar nerve. The length of the communicating branch ranged from 8 to 56 mm (average 16.4 mm) and its diameter was 0.2–0.6 times of the fourth common plantar digital nerve. The angle of the communicating branch with the common plantar digital nerve from which it originated was less than 30° in 11 ft, 30–59° in 27 ft, 60–80° in 8 ft, and more than 80° in 4 ft. Classification of the branch is based on the branching pattern of the communicating branch and explains variations in plantar sensory innervations. We think that the perpendicular coursing communicating branch is at higher risk to be severed during surgery.     

Prognostic importance of tumor angiogenesis in breast carcinoma with adjuvant chemotherapy

Tumor angiogenesis is believed to be related to prognostic factors involved in tumor development and metastasis. Using immunohistochemical methods, we evaluated tumor angiogenesis in 42 early invasive breast cancer patients (T1-2, NO-1-2, M0). Four patients received tamoxifen, 25 patients received CAF or CA, and 15 patients received CMF as adjuvant therapy. The median follow-up was 47 (range 24-119) months. Ten patients (43.5%) in the node-positive group and 2 patients (10.5%) in the node-negative group relapsed (p = 0.019). The mean microvessel count (MVC) was 60.3 3.05 per 200x field (range: 16-95). MVCs of postmenopausal and premenopausal patients were 50.13 +/- 5.74 and 68.64 +/- 4.11, respectively, in the axillary lymph node (ALN)-negative patient group (p = 0.04). Staining was moderate to strong in 13 (68%) ALN-negative and in 17 (74%) ALN-positive patients (p > 0.05), and was also moderate to strong in 82% of premenopausal patients and in 50% of postmenopausal patients (p = 0.037). There was no significant relationship between angiogenesis and p53, nor was angiogenesis significantly associated with the patient ER status and tumor size. No significant correlations were found between OS/DFS and Factor VIII staining or p53 (log rank test, p > 0.05). Of all ALN-negative patients with increased angiogenesis, one patient of the CMF group relapsed, but no recurrence occurred in patients undergoing anthracycline-based chemotherapy (p > 0.05). On the other hand, of all ALN-positive patients with increased angiogenesis, 5/14 patients treated with anthracylcine and 2/2 CMF-treated patients relapsed (p = 0.175). Despite the statistical insignificance, anthracycline-based adjuvant chemotherapy appears to be more effective than CMF as regards relapse prevention particularly in early ALN-positive breast cancer patients with increased angiogenesis. Additional studies are necessary to demonstrate the clinical importance of angiogenesis.     

Identification of 16 novel mutations in the argininosuccinate synthetase gene and genotype-phenotype correlation in 38 classical citrullinemia patients

Classical citrullinemia (CTLN1), a rare autosomal recessive disorder, is caused by mutations of the argininosuccinate synthetase (ASS) gene, localized on chromosome 9q34.1. ASS functions as a rate-limiting enzyme in the urea cycle. Previously, we identified 32 mutations in the ASS gene of CTLN1 patients mainly in Japan and the United States, and to date 34 different mutations have been described in 50 families worldwide. In the present study, we report ASS mutations detected in 35 additional CTLN1 families from 11 countries. By analyzing the entire coding sequence and the intron-exon boundaries of the ASS gene using RT-PCR and/or genomic DNA-PCR, we have identified 16 novel mutations (two different 1-bp deletions, a 67-bp insertion, and 13 missense) and have detected 12 known mutations. Altogether, 50 different mutations (seven deletion, three splice site, one duplication, two nonsense, and 37 missense) in 85 CTLN1 families were identified. On the basis of primary sequence comparisons with the crystal structure of E. coli ASS protein, it may be concluded that any of the 37 missense mutations found at 30 different positions led to structural and functional impairments of the human ASS protein. It has been found that three mutations are particularly frequent: IVS6-2A>G in 23 families (Japan: 20 and Korea: three), G390R in 18 families (Turkey: six, U.S.: five, Spain: three, Israel: one, Austria: one, Canada: one, and Bolivia: one), and R304W in 10 families (Japan: nine and Turkey: one). Most mutations of the ASS gene are "private" and are distributed throughout the gene, except for exons 5 and 12-14. It seems that the clinical course of the patients with truncated mutations or the G390R mutation is early-onset/severe. The phenotype of the patients with certain missense mutations (G362V or W179R) is more late-onset/mild. Eight patients with R86H, A118T, R265H, or K310R mutations were adult/late-onset and four of them showed severe symptoms during pregnancy or postpartum. However, it is still difficult to prove the genotype-phenotype correlation, because many patients were compound heterozygotes (with two different mutations), lived in different environments at the time of diagnosis, and/or had several treatment regimes or various knowledge of the disease.