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胆道闭锁患儿肝移植后追赶生长的评估。

Evaluation of catch-up growth after liver transplantation in children with biliary atresia.

作者信息

Alonso G, Duca P, Pasqualini T, D'Agostino D

机构信息

Section of Endocrinology, Liver Transplant Center, Pediatric Department, Hospital Italiano de Buenos Aires, Argentina.

出版信息

Pediatr Transplant. 2004 Jun;8(3):255-9. doi: 10.1111/j.1399-3046.2004.00160.x.

Abstract

Orthotopic liver transplantation (Tx) has improved survival in infants with extrahepatic biliary atresia (BA) when portoenteroanastomosis fails. Symptoms leading to Tx include liver failure, poor quality of life and growth failure. The objective of the study was to determine catch-up growth in children with BA. Medical records and growth data of 36 patients (24 girls) who received a Tx due to BA were analyzed. Thirty-two patients completed 3 yr and 15 patients 7 yr of follow-up after Tx. At Tx, the median age was 2.7 yr (range 0.7-12.6) and mean height Z score (+/-s.d.) was -1.56 (+/-1.3). Patients were divided in two groups according to age at Tx: group I (n = 10), younger than 1.0 yr, and group II (n = 26) older than 1.0 yr. Median age (range) at Tx in group I was 0.8 yr (0.7-1.0) and in group II it was 3.35 yr (1.25-12.6). Thirteen patients (nine in group I) were receptors of living related donors. We evaluated linear growth, liver and renal function, immunosuppressive regimen and allograft rejection episodes. We did not find any significant differences in allograft or renal function, immunosuppressive therapy and number of acute rejection episodes or height Z score at Tx, second and third year post-Tx between both groups. The mean height Z score at Tx in group I was -1.61 and in group II -1.54; at the second year, group I -0.66 and group II -1.08; at the third year, group I -0.17 and group II -0.85; and at the seventh year (total group) -0.3. However, the height gain at the third year was better in group I than in group II (p < 0.01, t-test). Height Z score at the third year improved more than 1 SDS in seven out of eight patients in group I and in only nine out of 24 in group II (odds ratio 11.6). We also found a correlation between height gain at the third year and age at Tx (r-0.65) and between height gain at the third year and height Z score at Tx (r-0.54) (Pearson, p < 0.05). Children with BA who are transplanted before 12 months of age presented better catch-up growth without change survival and morbidity. Orthotopic liver Tx improves survival and also enables height gain in these children.

摘要

当肝门肠吻合术失败时,原位肝移植(Tx)可提高肝外胆道闭锁(BA)婴儿的生存率。导致进行肝移植的症状包括肝功能衰竭、生活质量差和生长发育迟缓。本研究的目的是确定BA患儿的追赶生长情况。分析了36例因BA接受肝移植的患者(24例女孩)的病历和生长数据。32例患者完成了肝移植后3年的随访,15例患者完成了7年的随访。肝移植时,中位年龄为2.7岁(范围0.7 - 12.6岁),平均身高Z评分(±标准差)为 -1.56(±1.3)。根据肝移植时的年龄将患者分为两组:I组(n = 10),年龄小于1.0岁;II组(n = 26),年龄大于1.0岁。I组肝移植时的中位年龄(范围)为0.8岁(0.7 - 1.0岁),II组为3.35岁(1.25 - 12.6岁)。13例患者(I组9例)接受了活体亲属供体的肝脏移植。我们评估了线性生长、肝功能和肾功能、免疫抑制方案以及移植排斥反应。我们未发现两组在移植肝功能或肾功能、免疫抑制治疗、急性排斥反应次数或肝移植时、肝移植后第二年和第三年的身高Z评分方面存在任何显著差异。I组肝移植时的平均身高Z评分为 -1.61,II组为 -1.54;第二年,I组为 -0.66,II组为 -1.08;第三年,I组为 -0.17,II组为 -0.85;第七年(总体组)为 -0.3。然而,I组在第三年的身高增长优于II组(p < 0.01,t检验)。I组8例患者中有7例在第三年身高Z评分改善超过1个标准差,而II组24例患者中只有9例(优势比11.6)。我们还发现第三年的身高增长与肝移植时的年龄(r = -0.65)以及第三年的身高增长与肝移植时的身高Z评分(r = -0.54)之间存在相关性(Pearson检验,p < 0.05)。12个月前接受肝移植的BA患儿呈现出更好的追赶生长,且生存率和发病率无变化。原位肝移植提高了这些患儿的生存率,也促进了身高增长。

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