Repair of a large congenital frontal bone defect with autologous exchange cranioplasty

The authors report the case of a large idiopathic frontal bone defect and concomitant sagittal synostosis corrected by autologous exchange cranioplasty by using a corticocancellous bone graft and cranial vault expansion. An otherwise healthy, developmentally normal 6-year-old girl presented to our clinic with a large frontal bone defect. The osseous defect was midline and inferior to the coronal sutures, and the underlying dura was slightly tense. She had no signs or symptoms of increased intracranial pressure, and her head circumference and cephalic index were normal. Imaging demonstrated fusion of the sagittal synostosis. The defect was repaired using full-thickness autologous bone harvested from the bilateral parietal regions, which were widened using barrel-stave osteotomies to reduce pressure on the graft site in the setting of sagittal synostosis and mild cranial narrowing. The donor sites were covered with autologous particulate bone graft harvested from the endocortical surface of the grafted segments and the ectocortical surface of the intact parietal bones. The donor and recipient sites healed. Imaging revealed that the particulate bone healed with a thickness similar to the surrounding bone. This bony defect is analogous to parietal foramina and may have a similar etiopathogenesis. The technique of autologous exchange cranioplasty using corticocancellous particulate bone graft provides a simple and reliable method to repair large structural calvarial defects.     

Alternative donor HSCT in refractory acquired aplastic anemia - prevention of graft rejection and graft versus host disease by immunoablative conditioning and graft manipulation

Early alternative donor HSCT is a potentially curative therapeutic option for patients with AAA not responding to IST. Seven patients (median age at diagnosis, 11 yr) with refractory AAA without a MSD underwent HSCT from matched unrelated (n = 6) or haploidentical (n = 1) donors. Conditioning regimens included CY (n = 7), muromonab-CD3/ATG (n = 7), TT (n = 6), FLU (n = 5), and TLI (n = 2). Grafts were either CD34 purified and/or CD3/19 depleted and contained a median of 10.17 × 10(6) /kg CD34 and 5.5 × 10(4) /kg CD3 cells. All patients engrafted rapidly. Median time to leukocyte engraftment was 10 days. With a median follow-up of 26 (range, 11-153) months, six patients are alive and well with complete donor hematopoiesis. One heavily pretreated patient developed GVHD grade III and died from progressive renal failure (resulting from microangiopathic hemolytic anemia) and disseminated aspergillosis. Early alternative donor HSCT can help to avoid complications from prolonged IST and presumably improve survival of patients with refractory AAA. Administration of high doses of CD34 purified and/or CD3/19 depleted stem cells following novel immunoablative conditioning may prevent graft rejection and GVHD. However, a long interval from diagnosis to HSCT seems to be associated with poor outcome.     

Clinical approach to graft hepatic artery thrombosis following living related liver transplantation

Hepatic artery thrombosis (HAT) has an occurrence rate of 1.7-26% following living donor liver transplantation (LDLT) and is one of the most common reasons for graft loss and mortality in this population. There is a higher incidence of HAT in pediatric recipients. The aim of this case report is to discuss clinical approaches for the treatment of HAT occurring in the early post-operative period after LDLT. An 11-month-old, 7.8-kg female with cirrhosis secondary to biliary atresia underwent LDLT at Gazi University Hospital in Ankara. The graft was a left lateral segment from her father with a left hepatic artery (HA) of 2 mm diameter and a graft weight/recipient body weight ratio of 2.0%. After an uneventful early post-operative period, HAT was diagnosed by Doppler ultrasonography (USG) on the fifth post-operative day. Following angiographic evaluation, immediate exploration and reanastomosis was performed using an operation microscope. Post-operatively, the HA was patented by Doppler USG and graft function returned to normal. Now, 42 months later, the patient continues to do well with normal graft function, using a regimen of tacrolimus monotherapy for immunosuppression. In countries which have very limited resources for urgent re-transplantation, given their serious donor shortage, graft salvage may be the only option for patient survival when HAT occurs. In these circumstances, early diagnosis and immediate revascularization may be the only method for graft salvage. A daily routine of Doppler USG examination in the early post-operative period may provide a method for the early diagnosis of HAT, before liver enzymes are elevated and hepatic necrosis has begun.     

扩大的足背动脉内侧皮瓣修复儿童足跟及其周围软组织缺损

目的 探讨扩大的足背动脉内侧皮瓣修复儿童足跟部皮肤缺损的效果.方法 2004年8月至2008年12月,共10例患儿足跟及其周围软组织缺损,男8例,女2例,年龄4～10岁.均为创伤所致.创面大小约6.5 cm×4.0 cm～9.5 cm×4.0 cm.10例小儿足跟及其周围软组织缺损采用对侧扩大的足背动脉内侧皮瓣修复.7例皮瓣行交腿皮瓣转移修复,3例皮瓣行游离移植,供区创面采用游离植皮修复.术中把皮瓣内隐神经与受区隐神经吻合.结果 扩大的足背动脉内侧皮瓣范用约7.0 cm×4.5 cm～12.0 cm×4.5 cm.所有皮瓣均完全存活.8例获随访9～18个月,再造足跟两点辨别觉5～9mm,外形良好、无溃疡发生.结论 扩大的足背动脉内侧皮瓣可修复对侧足跟及其周围软组织缺损.该皮瓣是隐神经的绝对支配区,移植后可重建精细感觉.     

Comparison of angiogenic activity after urethral reconstruction using free grafts and pedicle flap: an experimental study.

PURPOSE: An experimental study was undertaken in order to estimate the angiogenic activity in different free grafts and pedicle flap in urethral reconstruction in an animal model. METHODS: Twenty-eight white New Zealand rabbits were randomly divided into five groups (O, A, B, C and D). A ventral urethral defect was created in all groups. In the group O, (n = 4), a simple closure of the defect was performed. Free penile skin graft (group A, n = 6), buccal mucosal graft (group B, n = 6), bladder mucosal graft (group C, n = 6), and pedicle penile skin graft (group D, n = 6) were used to bridge the urethral defect as an onlay patch. The animals were euthanized on the 21st postoperative day. The angiogenic activity was assessed with immunohistochemistry, using the anti-CD31 MoAb and the alkaline phosphatase antialkaline phosphatase procedure. The native vascularity of penile skin as well as buccal and bladder mucosa was assessed in rabbits from group O (n = 3). Statistical analysis was performed using one-way ANOVA. RESULTS: The angiogenesis seen with a magnification of x 200 in groups O, A, B, C, and D was 34.1 +/- 4.1 (mean +/- SD), 61.7 +/- 6.4, 94.3 +/- 6.4, 91.5 +/- 7.2, and 30.8 +/- 5.2 vessels per optical field, respectively. There were statistically significant differences (p < 0.001) between group O and groups A, B, C and between group A and groups B, C, D, but not (p > 0.5) between groups B and C and groups O and D. The native vascularity of penile skin, buccal mucosa and bladder mucosa was 23.3 +/- 3.0, 24.6 +/- 3.7 and 17.0 +/- 2.6 vessels per optical field, respectively. CONCLUSION: Buccal and bladder mucosal grafts exhibit a higher angiogenic activity than free and pedicle penile skin flap when transplanted in urethral defects. As the buccal mucosal graft showed the higher angiogenic activity and its harvesting is easier than bladder mucosa, we propose that in urethral reconstruction surgery the use of this graft might offer more reliable results.     

Duct-to-duct biliary reconstruction in pediatric living donor liver transplantation

The results of duct-to-duct biliary reconstruction in six pediatric patients who received a living donor liver transplant aged from 2 months to 11 yr old are reported. The graft was either entire or a part of the left lateral segments. The orifice of the bile duct of the graft was anastomosed to the recipients' hepatic duct in an end-to-end fashion by interrupted suture using 6-0 absorbable material. A transanastomotic external stent tube (4 Fr) was passed through the stump of the recipients' cystic duct. Mean time for reconstruction was 24 min. All the recipients survived the operation and reinitiated oral intake on postoperative day 3. There were no early biliary complications. One 5-yr-old boy suffered from an anastomotic stenosis 9 months after transplantation. He underwent re-anastomosis by Roux-en Y (R-Y) procedure and recovered uneventfully. Duct-to-duct anastomosis in pediatric living donor liver transplantation has benefits while the complication rate is comparable to R-Y reconstruction.     

Clinical Trial with Authentic Recombinant Somatropin in Sweden and Finland

A total of 47 prepubertal children with hGH deficiency were treated for up to 6 months with recombinant somatropin. All the children markedly increased their growth rate; 21 of them were naive (not previously treated with hGH), and increased their growth rate from 4.2 ± 0.2 cm/year to 13.9 ± 0.9 cm/year (calculated from growth data after 6 months'treatment, n = 11). Of the 47 children, 26 had been previously treated for 2 ± 0.3 years (range 0.3 - 8.3 years) with pituitary hGH. After a period of 0.9 ± 0.03 years (range 5–15 months) without any hGH therapy, their growth rate increased from 2.9 cm/year to 11.1 cm/year on recombinant somatropin therapy (calculated from growth data after 6 months'treatment, n = 10). One child reacted with temporary local erythema at the injection site. Anti-hGH antibodies, with a binding capacity of 0.02 mg/litre, were detected in 1 of the 16 children after 6 months of therapy. No adverse effect on her growth rate was seen. No changes in levels of antibodies to Escherichia coli proteins were detected. No other allergic manifestations or systemic side-effects were demonstrable.     

环状韧带重建修复对治疗儿童陈旧性孟氏骨折的疗效分析

目的 探讨环状韧带的重建、修复对陈旧性孟氏骨折预后的影响.方法 采用多中心回顾性方法,收集2011年1月至2015年12月于各中心治疗的儿童陈旧性孟氏骨折患儿,通过病历查询及随访的方式获取资料,记录患儿的性别、年龄、首诊时间、手术年龄、骨折分型、复位方式、是否尺骨截骨、手术入路和肘关节功能等数据,用SPSS19.0软件进行统计学分析.结果 最终有59例患儿纳入本次研究,术后随访6个月～3年,平均22个月.其中,男35例,女24例;年龄2～14岁,平均7.8岁.Bado分型:Ⅰ型45例,Ⅱ型2例,Ⅲ型12例.行肱桡关节切开复位59例,其中采用B0yd's切口35例,Kocher's切口10例,Henry's切口14例.行环状韧带重建(重建组)45例,环状韧带修补(修复组)14例.行尺骨截骨59例,其中48例内固定,包括:钢板39例、克氏针9例;采用外固定架11例.根据Kim's评分标准,重建组:优18例,良13例,一般8例,差6例,优良率为68.9％(31/45);修复组:优9,良3例,一般1例,差1例,优良率为85.7％(12/14).两组患儿术前的一般资料及优良率比较,差异无统计学意义(P＞0.05).重建组术前旋前活动度为(67.2±6.7)度,末次随访时旋前活动度为(75.8±7.5)度,手术前后比较,差异有统计学意义(t=9.745,P＜0.05).修复组术前旋前活动度为(70.6±9.2)度,末次随访时旋前活动度为(79.1±8.9)度,手术前后比较,差异有统计学意义(t=8.347,P＜0.05).在患儿受伤后1年以内,修复组的优良率为90％％(9/10)明显高于重建组81.3％(26/32),组间比较,差异有统计学意义(P＜0.05).修复组采用Henry's切口,重建组采用Boyd's、Kocher's切口.修复组在手术时间、切口长度及术中出血量[(82.6±5.7)min、(5.3±1.2)cm和(32.6±6.8)ml]上,均较重建组[(95.6±6.8)min、(8.2±0.9)cm和(56.1±9.7)ml]有明显优势,差异有统计学意义(P＜0.05).结论 肘前切口采用修补残余环状韧带的方法治疗儿童陈旧性孟氏骨折具有符合伤前解剖结构、方法简单、创伤小等优势,值得进一步探讨和推广.     

Sclerotherapy in aneurysmal bone cysts in children: a review of 17 cases

OBJECTIVE: To determine the efficacy of percutaneous sclerotherapy in the treatment of aneurysmal bone cysts. MATERIALS AND METHODS: Seventeen patients (7 girls, 10 boys) with aneurysmal bone cysts were treated by the percutaneous approach with Ethibloc ( n=14) and histoacryl glue ( n=3) in our institution between January 1994 and June 2000. The cysts were located in the extremities ( n=6), pelvis ( n=2), spine ( n=2), mandible ( n=5), rib ( n=1) and sphenoid bone ( n=1). Percutaneous sclerotherapy was performed with fluoroscopic and/or computed tomographic guidance under general anesthesia. Clinical and imaging follow-up lasted from 24 months to 9 years and 6 months (mean: 57.3 months). The results were quantified as: excellent (residual cyst less than 20% of the initial involvement), satisfactory (residual cyst 30-50%), unsatisfactory (residual cyst more than 50%). RESULTS: The age of the patients ranged from 4 years and 6 months to 15 years and 8 months (mean: 11 years and 2 months). In nine patients, the therapeutic procedure was repeated 2-5 times. Excellent regression was observed in 16 (94%), satisfactory results in 1 (6%). There was no failure (unsatisfactory result or no response to treatment) in this reported series. The complications were minor and included: local inflammatory reaction ( n=2), small blister ( n=1), and leakage ( n=1). Relief of symptoms was achieved in all patients. No recurrence was noted during follow-up. CONCLUSION: Percutaneous sclerotherapy of aneurysmal bone cysts with Ethibloc is safe and effective. It is an important alternative to surgery, especially when surgery is technically impossible or not recommended in high-risk patients.     

Living-unrelated renal transplantation in children: a report of the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS)

The shortage of cadaver kidneys available for organ donation compared to growing demand has led to an increase in the use of living-unrelated donors (LURD) for renal transplantation (Tx). Results from trials in adults show that 1-year graft survival rates in LURD are similar to living-related donor (LRD) rates and superior to those of cadaver renal donor (CAD) transplants. We report our experience with 38 LURD transplants for children enrolled in NAPRTCS that were performed between 1987 and 1997. Ages of recipients at Tx were 0-5 years (n=8), 6-12 (n=10), and >12 years (n=20). Twenty nine were primary Tx, seven were second Tx, and two were third Tx. HLA antigen data showed that the number of 2-antigen mismatches for each locus was 44.7% for HLA-A, 71.1% for HLA-B, and 55.3% for HLA-DR. There were 7 donor/recipient pairs with a 6-antigen mismatch, 12 pairs with a 5-antigen mismatch, while there were 6 pairs with a 3-antigen match of which 3 pairs had at least one match at each of the A, B, and DR loci. A total of 38 acute rejection episodes occurred in 25 LURD recipients. Among primary grafts the incidence of first acute rejection at 30 d post-Tx was 46% in LURD vs. 29% in LRD and 37% in CAD recipients; at 1 year post-Tx it was 76% in LURD vs. 48% in LRD and 62% in CAD recipients. Acute tubular necrosis (ATN) was reported in four or 10.5% of LURD transplants compared with 5.4% in LRD and 19.0% in CAD recipients. There were 12 LURD graft failures, due to vascular thrombosis (3), acute rejection (2), recurrence of original disease (1), infection (3), and patient death (3). Estimated primary graft survival probabilities (+/- SE) at 12 months post-Tx are 0.825 +/- 0.071 for LURD, compared to 0.911 +/- 0.006 for LRD, and 0.815 +/- 0.009 for CAD. We conclude that data from this study show that LURD Tx in children have a low rate of ATN that is similar to that of LRD Tx. However, LURD Tx have a high incidence of acute rejection, and the graft survival at 12 and 24 months post-Tx is inferior to LRD Tx. There is a high frequency of graft loss due to causes other than rejection, and these may be related to adverse recipient selection criteria.     

卵睾型性发育异常单中心临床诊治分析

目的 总结卵睾型性发育异常的临床特点及诊治经验.方法 回顾性分析1993年1月至2015年12月就诊于医院并通过病理确诊卵睾型性发育异常的32例患儿临床资料和随访资料.社会性别:男30例,女2例.12.5％呈女性外貌,生殖器类别模糊,阴蒂1～3 cm,小阴唇发育差,有乳房发育;87.5％呈男性外貌,阴茎发育极差,重度下弯,尿道开口异常(位于阴囊处或会阴部),阴囊不同程度女性化,外形近阴唇貌,其中46.9％(15/32)伴有隐睾.染色体核型分析:46,XX卵睾型DSD 11例;46,XY卵睾型DSD 1例;性染色体异常DSD中的卵睾型性发育异常20例(嵌合性46,XY/46,XX6例;混合型14例).结果 11例行泌尿生殖系彩超和排泄性尿道阴道造影,二者结合检出率为100％(11/11).5例行SYR基因筛查,1例45,X卵睾型DSD及1例45,X/46,XY卵睾型DSD为SYR阳性,1例45,X/46,XY SYR基因为阴性,余2例46,XX卵睾型DSD中,50％SYR基因为阴性.64个性腺中16个卵睾,25个睾丸,23个卵巢.性腺畸形:双侧型5例,单侧型6例,片侧型21例.30例按男性抚养者均完成阴茎矫直术和尿道重建术.对29例患儿进行8个月～9年的随访,其中3例术后反复发生尿瘘,多次行尿瘘修补术,阴茎及睾丸发育极差,生活质量差;4例术后尿道开口位于冠状沟部或阴茎体部,排尿可;3例进入青春期后有乳房发育;2例阴茎、睾丸发育稍差;余阴茎形态可,长2.5～4.0 cm,睾丸测值较同龄人稍小.2例按女性抚养者均完成阴蒂矫形术,均获得满意的外观,1例处于青春期发育阶段,另1例予雌激素替代治疗后获得青春期发育,外阴形态可,有乳房发育.结论 早期诊断,确诊后是否立刻性别选择行手术治疗仍有争议,我们认为应将患儿的心理性别、社会性别作为参考的首要标准结合激素水平评估、优势性腺评估最后选择性腺切除或重建手术并辅以激素治疗.对于维持患儿正常的性生理、性心理及社会生活具有重要的意义.     

Kidney transplants for children under 1 year of age - a single-center experience

From September 20, 1970 to October 24, 2001, we performed 46 kidney transplants in infants under 1 yr old at the University of Minnesota. This article reviews the preoperative care, surgical technique, and immunosuppression. Recipients included 16 females and 30 males; the youngest recipient was 6 wk old. The mean pretransplant height was 62.8 cm, which increased to 77 cm at 1 yr post-transplant and to 104 cm at 5 yr. We used 40 living donors (all but 1 were related to the recipient) and 6 cadaver donors. The overall actuarial graft survival was 85% at 1 yr and 70% at 5 yr. In the cyclosporine era, graft survival improved to 91% at 1 yr and 80% at 5 yr. Death with function was the most common cause of graft loss (n = 5), followed by biopsy-proven chronic rejection (n = 4), biopsy-proven recurrent disease (n = 3), and graft thrombosis (n = 2). Patient survival was 91% at 1 yr and 86% at 5 yr. In the cyclosporine era, patient survival was 100% at 5 yr and 85% at 10 yr. We concluded that an early transplant is the best treatment option for infants under 1 yr old with chronic renal failure. Whenever possible, adult living kidney donors should be used.     

Outcomes of children,adolescents, and young adults following allogeneic stem cell transplantation for secondary acute myeloid leukemia and myelodysplastic syndromes—The MD Anderson Cancer Center experience

We conducted a retrospective analysis of outcomes for children and young adults with sAML/sMDS who underwent HSCT at our institution. Thirty‐two patients (median age 20 years) with sAML (n=24) and sMDS (n=8) received HSCT between 1990 and 2013. The median time from sAML/sMDS diagnosis to HSCT was 4.1 months (range: 1.2‐27.2 months). The transplant regimens were primarily busulfan based (n=19). BM was the primary donor source (n=15). Eleven recipients were transplanted with residual disease. At a median follow‐up of 62.3 months (range: 0.4‐250.9 months), 14 patients had disease recurrence. Acute GVHD, grade III/IV, occurred in three patients. Causes of death were as follows: disease relapse (n=12), infection (n=2), pneumonia (n=1), pulmonary hemorrhage (n=1), acute GVHD (n=1), and graft failure (n=1). A PS of ≥90% at the time of HSCT had a significant impact on PFS (P=.02). Patients achieving pretransplant primary CR (n=8) and those with sMDS and RA (n=6) had prolonged PFS (P=.04). On multivariate analysis, shorter time to transplantation (≤6 months from diagnosis of sAML/sMDS) was associated with superior OS (P=.0018) and PFS (P=.0005).     

Clinical Experience with Authentic Recombinant Somatropin – German Collaborative Study

A multicentre clinical trial with recombinant somatropin was initiated in West Germany in early 1986. Acceptance of patients to the study was determined according to criteria outlined in a detailed study protocol. To the present time, 41 patients with hGH deficiency not previously treated (naive) and 28 patients previously treated with pituitary hGH have been admitted. Recombinant somatropin is given, 12 IU/m²/week s.c, divided into six doses. Height velocities during treatment rose dramatically for naive patients from 3.5 cm/year (n = 40, before treatment), to 15.0 cm/year (n = 19, calculated at 3 months), and 13.3 cm/year (n = 8, calculated at 6 months). For previously treated patients, the increase in height velocity was from 5.8 cm/year (n = 20) to 9.2 cm/year (n = 19, after 3 months) and 8.6 cm/year (n = 9) at 6 months. Tolerance of recombinant somatropin was good, and no anti-hGH antibodies were detected in any of the patients.     

趾动脉皮瓣在足部多趾并趾畸形修复中的应用

目的 设计以同趾或邻趾的趾动脉为蒂的岛状皮瓣来修复分趾后趾蹼区的皮肤缺损.方法 2010年1月至2013年1月间共收治并趾多趾畸形49例,28例获得随访.其中,男19例,女9例;年龄13～44个月,平均27.1个月.其中踇多趾畸形7例;并趾畸形21例,包括:第1、2趾并趾畸形5例,第2、3趾并趾畸形3例,第3、4趾并趾畸形4例,第4、5趾并趾畸形9例.所有患儿分趾后均采用趾动脉皮瓣修复趾蹼部皮肤缺损.供区趾体损伤面积较小,可直接缝合,无需植皮.结果 术后所有并趾畸形均得到了矫正,趾间获得很深的趾蹼和自然张开的弧度.出院后28例获得随访,平均随访时间8个月.随访期间发现皮瓣质地柔软,外形满意,无色差,伤指无疼痛,两点分辨率在4～10mm.结论 使用趾动脉皮瓣可完全修复并趾分趾后的软组织缺损而无需植皮,且供区可直接缝合,同时可有效地改善趾蹼外形,是修复足趾并趾分趾畸形的一种安全、有效的新方法.     

单倍体造血干细胞移植治疗儿童重型β-地中海贫血

目的：目前仅有30％左右的重型β-地中海贫血患者能找到HLA全相合的同胞供者,使造血干细胞移植治疗该病受到限制。该研究通过探讨单倍体造血干细胞移植治疗儿童重型β-地中海贫血的疗效,希望能够拓展供者源。方法：采用单倍体脐血或骨髓对10例重型β-地中海贫血患儿进行11例次移植。使用以羟基脲、氟达拉滨、白消安、环磷酰胺、抗胸腺细胞球蛋白为基础的预处理方案。结果：6例患者获长期稳定植入并脱离红细胞输注;2例短暂植入后排斥,其中1例恢复地中海贫血状态,另1例在移植早期死亡;1例行2次移植均未植入并出现移植后再障;1例未植入,出现再障,1年后恢复地中海贫血状态。8例植入者均发生急性移植物抗宿主病,仅1例发展为皮肤局限性慢性移植物抗宿主病。随访57.1（2.5～85.1）月,总体生存率90%,无病生存率为60%。结论：单倍体造血干细胞移植治疗儿童重型β-地中海贫血能长期重建造血,在无HLA相合同胞供体时,可以作为造血干细胞移植治疗的一种选择。［中国当代儿科杂志,2009,11（7）：546-548］     

Laparoscopic live donor nephrectomy: the pediatric recipient in a dual-site program

BACKGROUND: At our institution, laparoscopic live donor nephrectomy (LLDN) is done at a different hospital site than pediatric recipient transplantation, whereas open donor nephrectomy (OLDN) is done in the adjacent operating room. The purpose of this study was to evaluate the safety of a dual-site renal transplantation program by comparing the outcomes of pediatric recipients of LLDN vs. OLDN. METHODS: This is a retrospective study of consecutive pediatric recipients (n = 10) of LLDN (June 2002 to June 2005) compared to the 10 most recent pediatric recipients of OLDN (March 2001 to June 2005). Renal function was assessed with calculated creatinine clearance using the Schwartz formula and the following outcomes were assessed: delayed graft function, ureteral complications, acute rejection and patient and graft survival. Results are expressed as median (IQR). RESULTS: When comparing the laparoscopic vs. open group, there were no significant differences in recipient age, height, weight, preoperative calculated creatinine clearance and warm ischemia time. Twelve month postoperative creatinine clearance was 88 ml/min/1.73 m(2) (57-99) in the laparoscopic group (n = 8) and 66 ml/min/1.73 m(2) (60-86) in the open group (n = 9), p = 0.2. In the LLDN group vs. the OLDN group, delayed graft function was 0% vs. 10% (p = 1.0), ureteral complications were 20% vs. 30% (p = 1.0), and acute rejection was 20% vs. 40% (p = 0.6). In the laparoscopic group, one-yr patient and graft survival were both 100%, as compared to 100% and 89%, respectively, in the open group. CONCLUSION: A dual-site laparoscopic donor nephrectomy program is not associated with adverse pediatric recipient outcomes when compared to a same-site open donor approach.     

Coronary Artery Bypass Grafting for Kawasaki Disease

Five patients with a history of Kawasaki disease underwent coronary revascularization at Children's Memorial Hospital (1988-2000). Acute disease occurred at 11 weeks to 5 years of age and revascularization procedures were performed at 8 months to 12 years (mean 6 years; interval from disease onset 5 months to 9 years). Surgical indications included abnormal stress testing with angiographic confirmation of severe coronary artery stenosis (n = 3), severe coronary artery stenosis with echocardiographic evidence of intracoronary thrombus (n = 1), and ischemic electrocardiogram changes and ventricular tachycardia during angiography (n = 1). All revascularization procedures used internal thoracic arteries including one free internal thoracic artery graft. There were no postoperative deaths (follow-up 1 month to 11 years). All patients are asymptomatic. One patient developed myocardial ischemia 4 years postoperatively with occlusion of the circumflex coronary artery (not previously grafted). This was treated successfully with percutaneous coronary angioplasty and stent placement. All grafts are patent with the exception of a single right internal thoracic artery graft which underwent involution 30 months postprocedure with concurrent recannulization of the right coronary artery. Coronary revascularization should be considered in the young patient with severe coronary abnormalities secondary to Kawasaki disease.     

Early discontinuation of steroids is safe and effective in pediatric kidney transplant recipients

In pediatric kidney transplantation, steroid induced growth retardation and cushingoid features are of particular concern. In children, gradual steroid withdrawal late after kidney transplantation increases the risk of rejection. In this pilot study, we investigated the outcome of pediatric renal transplantation with an early steroid withdrawal protocol. This is a retrospective case-control study of pediatric renal transplants with age-matched historical control. Groups were comparable in terms of HLA matching, donor type and graft ischemia time. In the steroid withdrawal group (SWG, n = 13), induction therapy included mycophenolate mofetil (MMF) and a 5-day course of steroids with Thymoglobulin in 11 and basiliximab in two other patients. In the steroid group (SG, n = 13), in addition to steroids, four patients were given basiliximab, eight were given Thymoglobulin, and one OKT3. Maintenance therapy included tacrolimus (SWG n = 11, SG n = 3) or cyclosporine (SWG n = 2, SG n = 10). Azathioprine was given to all the patients in the SG, except the last two patients of this series who were prescribed MMF. MMF was given to all in the SWG. Patient and graft survival rates were 100% in both groups. In the SWG, no acute rejection episode was detected. In the steroid group, three patients (25%) presented with an acute rejection episode. All but one patient in either group showed immediate graft function. Patients in the steroid-withdrawal group exhibited a significantly higher creatinine clearance at 6 and 12 months post-transplant (95.8 +/- 23.3 vs. 71.3 +/- 21.9, p = 0.03; and 91.3 +/- 21.6 vs. 69.6 +/- 28.6, p = 0.04). In the SWG delta BMI was significantly lower and delta height Z score was significantly higher, and we observed significantly less hyperlipidemia, body disfigurement, and need for anti-hypertensive medication. Early steroid withdrawal in pediatric renal transplant recipients is efficacious and safe and does not increase risk of rejection, preserving optimal growth and renal function, and reducing cardiovascular risk factors.     

胎儿脐静脉脱细胞基质构建组织工程尿道

目的 探讨人脐静脉细胞外基质的制备及其在尿道重建中的应用.方法 采用酶消化、去污剂和渗透溶液方法制备人脐静脉细胞外基质(extracellular matrix,ECM).24只雄性白兔,随机分为A尿道ECM移植组,对照组B、C.A组切除尿道2.0 cm后用人脐静脉ECM修复,术后行组织学、尿流动力学和膀胱尿道造影评价尿道再生情况及功能.结果 经该法处理的人脐静脉细胞全部脱除.A组修复术后2周,脐静脉ECM有单层上皮细胞生长;4周管腔已被上皮细胞完全覆盖;8周可见人脐静脉ECM降解的微小碎片和平滑肌细胞生长,炎性细胞消失;16周尿道大体观、组织学检查均接近C组,平滑肌数量与C组差异无统计学意义(P>0.05),但排列欠规则.膀胱尿道造影无尿液外渗、尿道憩室形成;尿动力学A组与C组膀胱容量、最大尿道压分别与术前比较,差异均无统计学意义(P>0.05),B组无法置入测压管.结论 酶消化、去污剂和渗透溶液法是制备人脐静脉ECM较为理想的方法,人脐静脉ECM可作为尿道重建的良好支架材料.