Continuous plasma resin perfusion for detoxification of methotrexate

Acute renal failure is a rare but very severe side-effect of methotrexate (MTX) treatment requiring extracorporeal detoxification. A new detoxification regime, consisting of a haemodiafiltration (HDF) and continuous plasma resin perfusion (PRP) over anion exchange resin column with high affinity for MTX, was used for treatment of MTX-induced acute renal failure in a 2-year-old boy with medulloblastoma. More than 99% of MTX was removed from plasma by perfusion over the resin column resulting in a marked decrease of MTX plasma half-life. No haematological complications occurred. Conclusion The use of PRP alone or in combination with HDF is a new and effective treatment strategy for extracorporeal detoxification of MTX. Received: 27 June 1996 / Accepted: 17 December 1996     

双重血浆分子吸附系统在儿童急性肝衰竭中的应用

目的 探讨双重血浆分子吸附系统(DPMAS)用于儿童急性肝衰竭(PALF)的有效性及安全性.方法 前瞻性收集2018年3月至2020年6月在湖南省儿童医院重症医学科住院的PALF患儿的临床资料,随机分为血浆置换组(PE组)和DPMAS组,每组各18例.比较两组患儿临床指标、治疗前后实验室指标和不良反应.结果 DPMAS...     

Application of MARS artificial liver support as bridging therapy before split liver retransplantation in a 15-month-old child

Molecular Adsorbent Recirculating System (MARS) is a blood-filtering system designed to provide biological artificial liver support. We describe its use in a small child to illustrate its effectiveness and practicality in this age group. A 15-month-old male underwent split liver transplantation for acute liver failure following bone marrow transplantation. After development of graft dysfunction we instituted MARS-dialysis. MARS therapy led to a dramatic fall in serum bilirubin and transaminases. Liver synthetic function was not affected. This was accompanied by a stabilization of the patients clinical condition until repeat split liver transplantation was performed 2 weeks after the first graft. MARS-dialysis is practical in the small child. In this case, it did not provide definitive treatment but was an excellent bridging therapy before retransplantation.     

培门冬酶在儿童急性淋巴细胞白血病临床应用中的安全性评估

目的评估培门冬酶在儿童急性淋巴细胞白血病(ALL)临床应用中的安全性。方法本研究中所有ALL患儿都使用CCLG 2008方案规范诊治;接受培门冬酶治疗的条件是:(1)左旋门冬酰胺酶(L-Asp)皮试阳性;(2)L-Asp使用后出现不良反应。先后共有32例患儿由于对大肠杆菌L-Asp过敏而使用培门冬酶替代化疗共46次。使用培门冬酶治疗前和治疗后1周检查肝肾功能、凝血功能、淀粉酶、心肌酶谱、心电图和腹部B超,临床观察和检测使用培门冬酶后脏器功能和不良反应发生的情况。结果 46例次中无一例发生致死性不良反应。8例(17%)有转氨酶异常;总胆红素增高4例,以直接胆红素升高为主;血清总蛋白降低5例。8例(17%)有血浆纤维蛋白原浓度异常,7例降低,临床无凝血功能障碍和血栓等表现。7例(15%)有心血管系统受累,包括血压升高(1例)、心率减慢(2例)、CK-MB增高(5例)、肌钙蛋白增高(1例)、心电图异常(4例)。7例(15%)出现肾功能异常,以尿素氮增高(6例)为主,临床未发现肾功能不全的表现。3例(7%)发生高血糖症,无急性胰腺炎的病例发生。46例次培门冬酶治疗中,仅1例(2%)发生了典型的过敏反应,以明显的风团样皮疹伴瘙痒为主要表现,有一过性喉头异物感,经吸氧、镇静等处理后很快缓解。结论培门冬酶在很大程度上克服了L-Asp过敏反应致使用受限的弱点;培门冬酶的不良反应发生率低,发生不良反应的程度轻,安全性较高。推荐培门冬酶作为对L-Asp过敏的ALL患儿的替代治疗。其疗效有待进一步观察。     

儿童继发性血栓性血小板减少性紫癜1例并文献复习

目的 提高对儿童不典型继发性血栓性PLT减少性紫癜（TTP）的认识。 方法 总结1例无神经系统受累的继发性TTP患儿的临床资料、实验室检查结果、ADAMTS13酶活性和Anti-ADAMTS13抗体检测结果,行系统文献检索并文献复习。 结果 男性患儿,12岁,急性起病,病初有发热,双下肢可见瘀点,PLT及Hb进行下降,血涂片可见破碎RBC,高胆红素血症,LDH明显升高,镜下血尿,肾功能正常,补体正常,考虑血栓性微血管病(TTP或非典型溶血尿毒综合征)。为进一步明确诊断, 行ADAMTS13酶活性检测2.3%（正常值40％~130%）,ADAMTS13抗体检测90 U·mL-1（正常值＜12 U·mL-1）,确诊继发性TTP,予血浆置换和激素治疗。4个月后患儿停用所有药物,目前停药6月无复发。系统检索中国知网、万方和PubMed数据库,共有14篇英文文献中40例继发性TTP进入本文分析,发病年龄（10.2±5.2）岁,男19例,女21例,发热36例（90%）,神经系统受累28例（70%）,肾脏受累18例（45%）,均有贫血和PLT降低。3例死亡,37例血浆置换+激素治疗,31例（83.8%）对血浆置换治疗即时反应好,1例因血浆过敏和1例血浆置换导管相关感染改为激素+利妥昔单抗治疗反应好,1例难治性继发性TTP加长春新碱（利妥昔单抗上市前）随访时复发,2例发生血浆置换依赖,加环孢素后治疗反应好,1例治疗反应不好,加长春新碱后治疗反应好,,4例失访（10.8%）,平均随访时间29月（3~72个月）,13例（39.4%）出现复发,9/13例加利妥昔单抗中仍有2例复发。 结论 贫血和PLT降低应怀疑TTP,需行ADAMTS13酶活性及其抗体的检测,有助于区别遗传性和获得性TTP;血浆置换+激素,或+利妥昔单抗是TTP的治疗组合选项。     

Acute renal failure in falciparum malaria—a case report

A case of P. falciparum malaria with acute renal failure is reported. The urinary sodium of 42 mEg/L pointed towards a tubular pathology. There was no significant intravascular hemolysis as serum bilirubin was normal and plasma hemoglobin only mildly elevated. The pathogenesis of acute tubular necrosis due to heavy parasitemia is discussed. The other interesting feature which the patient exhibited was alternate day fever in the hospital and the peripheral smear showing only gametocytes repeatedly. Gametocytes do not give rise to symptoms. The patient responded well to quinine hydrochloride and the gametocytes disappeared. There is no satisfactory explanation for this phenomenon.     

6月龄女婴尿量减少伴急性肾功能异常

患儿,女,6月龄,急性起病,因尿量减少、肾功能异常入院。辅助检查提示严重代谢性酸中毒,血肌酐和尿素氮明显增高。予限制液体、纠正酸中毒、连续床旁血液净化等治疗10 d,患儿仍持续无尿,放弃治疗后死亡。患儿姐姐于6月龄死于急性肾功能衰竭。患儿经基因检测证实为丙氨酸乙醛酸氨基转移酶(AGT)编码基因AGXT突变引起的原发性高草酸尿症1型(PH1),其父母为杂合突变携带者。PH1是一种临床罕见疾病,对于肾功能显著异常,或伴反复发作肾结石,以及有类似家族史的患者,应考虑到PH1的可能,AGXT基因分析是PH1诊断的重要手段。     

克纳综合征Ⅰ型1 例报告

目的探讨克纳综合征的病因、诊断、治疗。方法回顾性分析1例克纳综合征患儿的临床资料。结果患儿,男,3个月,因黄疸入院,患儿血清总胆红素波动于450.1~479.3μmol/L之间,以非结合胆红素为主,光疗效果欠佳,临床高度怀疑克纳综合征Ⅰ型;经尿苷二磷酸葡萄糖醛酸基转移酶(uridine diphosphoglucuronyl transferase,UGT)1A1基因检测Exon1位点插入突变,确诊克纳综合征Ⅰ型。患儿于肝脏移植过程中死亡。结论克纳综合征由UGT1A1基因突变使其酶活性完全或部分丧失而导致的遗传性胆红素代谢障碍性疾病,诊断主要是根据临床表现、实验室检查及UGT1A1基因检测,肝移植手术可根治此病,基因治疗则是最终治愈的理想方法。     

Plasma Exchange for Hemolytic Crisis and Acute Liver Failure in Wilson Disease

Wilson disease (WD) is a rare autosomal recessive disorder of copper metabolism which primarily involves the liver and the central nervous system. Rarely, WD can present as acute liver failure (ALF) and this disease is universally fatal in the absence of liver transplantation. The authors report a young girl with WD ALF, who showed signs of recovery after prompt initiation of plasma exchange (PE) and chelation therapy. Though liver transplantation could not be done in this child and the child died 8 d after stopping PE, this case highlights that PE can be a successful medical treatment in WD ALF and should be considered as a therapeutic measure to stabilize a patient by decreasing serum copper, reducing hemolysis, and helping to prevent renal tubular injury from copper and copper complexes until liver transplantation is possible.     

Color Doppler sonography in pyelonephritis

Renal sonography detects abnormality in only 40% of pediatric pyelonephritis. In eight patients shown to have acute pyelonephritis by^99mTc DMSA renal cortical scintigraphy, five were found to have focal abnormalities of renal perfusion by color Doppler sonography in the same sites as the scintigraphic defects (sensitivity-63%). Two of the five patients had normal plain sonograms. False positive studies occurred in patients with documented chronic renal scarring. The specificity of vascular asymmetry was 70%. This preliminary report suggests that, particularly in the patient without pre-existing renal scarring, color Doppler evaluation of the renal vasculature may increase the sensitivity of sonography in the diagnosis of pyelonephritis in children.Presented at International Pediatric Radiology '91, Stockholm, Sweden, May 27, 1991     

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目的观察降脂药物诺衡对于活动期激素耐药型肾病综合征(SR-NS)患儿高脂血症的疗效及安全性。方法观察在北京大学第一医院儿科住院的8例肝功能正常的活动期SR-NS患儿诺衡降脂治疗1个月,检测治疗前后血清脂质、脂蛋白、白蛋白、肝肾功能的变化及观察临床副反应表现,其中男6例,女2例;年龄214岁,平均8.4岁,所有患儿均接受激素治疗且用药前后尿蛋白持续+++++++。结果血脂及脂蛋白水平在用药2周后出现不同程度的变化,其中TG明显下降(P<0.05),TC、VLDL、LDL下降,HDL上升,差异均在4周后出现明显变化(P<0.05)。治疗前后血Alb、ALT、SCr差异均无统计学意义,且后两者在正常范围内。除1例服药中出现胃肠不适感外,所有患儿均无腹痛、腹泻及皮疹等副反应。结论结果显示对于持续未缓解的SR-NS患儿,尤以TG升高为主的高脂血症,短期应用诺衡降脂治疗是安全有效的,肾功能不全者慎用。     

非生物型人工肝在肝衰竭患儿中的应用

目的 探讨非生物型人工肝支持治疗在肝衰竭患儿中的应用效果.方法 2003年8月-2009年4月在首都医科大学附属北京儿童医院确诊为肝衰竭患儿11例,采用非生物型人工肝支持治疗,包括应用血浆置换、血液灌流、持续血液滤过、血液透析等治疗方法.结果 非生物型人工肝支持治疗过程中并发症少.11例患儿顺利进行了23次血浆置换治疗,7次血液灌流治疗,5次血液透析治疗,3次持续血液滤过治疗(2例治疗顺利,1例在治疗中出现病情恶化、心跳骤停而终止治疗).10例患儿血液净化治疗后临床症状均有不同程度好转,监测血生化各项指标与治疗前比较亦有明显好转.11例获随访,其中2例毒蕈中毒肝衰竭患儿临床治愈出院,其中包括1例多脏器衰竭患儿,随访1 a,肝功能正常;3例病情好转出院;3例病情进展恶化,放弃治疗死亡;3例自动出院.结论 应用以血浆置换治疗为主的非生物型人工肝治疗急慢性肝衰竭患儿,可使患儿临床症状及各项检测指标有不同程度好转,提高患儿存活率.人工肝治疗技术应用于肝衰竭患儿治疗中应注意充分考虑儿童病理生理特点,制定合理的治疗方案,同时采取有效的预防措施和密切的病情监测,以保障治疗的顺利进行.     

Liver transplantation in infants weighing under 7 kilograms: management and outcome of PICU

Liver transplantation (LT) is an established treatment for children with acute and chronic liver failure. Some reports suggest that infants under the age of 1 yr and children weighing under 13 kg are high-risk groups associated with less satisfactory results. This report describes our experience during the pediatric intensive care unit stay of 16 infants weighing <7 kg who received LT. We reviewed the records of 16 infants with median age 7.4 months and median weight 5.8 kg, who received 18 liver allografts, nine whole and nine reduced. We also reviewed the use of adrenergic agonist agents, anti-infectious agents, antihypertensive agents, diuretics, immunosuppression protocol, sedation-analgesia agents, others agents (prostaglandin E(1), heparin and dipyridamole), diagnosis and management of rejection episodes, follow-up examination, nutrition and outcome. Mean peri-operative blood transfusions were 204 mL/kg, 188 mL/kg of plasma and 36 mL/kg of platelets; mean operative time was 5 h. Primary abdominal wound closure was possible in nine patients. Median initial intensive care unit stay was 18 days. Reasons for an initial stay of more than 18 days were retransplantation (1), gastrointestinal bleeding (2), paralytic ileus and atelectasis (2), septic shock (2), diaphragmatic paralysis, renal impairment and acute respiratory distress syndrome (2). Mean requirement for artificial ventilation was 168 h. Mean use of dobutamine, prostaglandin E(1) and dopamine was 3.3, 7.5 and 8.8 days, respectively. Parenteral nutrition was started at a mean of 48 h and oral food intake was started at a mean of 72 h. The most frequent complications were infection, atelectasis, gastrointestinal bleeding, acute renal failure and hepatic artery thrombosis. Four children required six re-explorations and two received retransplantation. Mean overall survival rate was 82% and graft survival was 72%. Weight alone (under 7 kg) should not be considered as a contraindication for LT. The survival rate of children post-LT is excellent regardless of graft type.     

人工肝支持系统在肝功能衰竭中的应用

肝功能衰竭是多种因素引起的严重肝脏损害,在儿科病因更为复杂且病情凶险,传统的内科综合治疗效果欠佳,病死率高.人工肝支持系统也常称为人工肝,是一种能够替代或模拟正常肝脏的部分或全部功能的体外装置,可以使因肝功能衰竭所产生的各种有害物质得以清除,并替代肝脏的部分代谢功能,以维持患者生命.人工肝应用至今已逾50年,其血液净化方式包括血液透析、血浆置换、血液灌流、吸附、全血或血浆滤过以及应用肝组织或细胞研制的有生物效应的治疗方法等.由于肝脏有强大的再生能力,人工肝可为病变肝脏自身再生恢复或接受肝移植尽可能争取时间.随着人工肝支持系统相关技术的不断完善,非生物型人工肝治疗已在临床广泛应用,并取得很好的疗效,成为各种重症肝功能衰竭的重要治疗手段之一.人工肝在儿科临床也有应用报道,但资料有限,仍需要进一步研究和实践.

Abstract：

Despite a combination of all available treatment, the mortality of liver failure is very high,especially in children patients. Artificial liver support methods have been tested for over 50 years. Standard techniques of blood purification like hemodialysis, adsorption, hemo or plasma filtration as well as bioreactorbased approaches using liver cells or tissues have been used. It' s believed that the damaged liver has the ability to return to normal. Artificial liver support systems are expected to be useful for temporary support of liver function. If the liver does not regenerate to normal functions, an artificial liver support system may be useful as a bridge to liver transplantation. In conclusion, artificial liver support method appears to be a reliable therapy for advanced liver diseases and has significantly decreased the mortality of liver failure. Artificial liver support system has been used in children patients as well, but it still needs more researches.     

体外生命支持模式及临床应用

危重患儿常合并多器官功能障碍.体外生命支持治疗利用体外设备代替体内脏器功能,从而达到维持生命的目的.体外设备可以支持多脏器功能障碍患儿的心、肺、肝、肾功能.与成人不同,多脏器衰竭患儿的基础疾病可能逆转,因此体外生命支持更为有效.本文概括介绍不同的体外脏器支持模式,包括体外膜肺、连续性肾脏替代治疗、人工肝、血液灌流和血浆置换治疗.

Abstract：

Multiple organ dysfunction(MODS) can be seen in critically ill children.Modality of extracorporeal life support (ECLS) is the use of mechanical devices to support life when the native organ failure occurs.Extracorporeal devices can effectively support heart,lung,liver,and kidney function of the sick children with MODS.Unlike the adult experience,ECLS is an effective therapy in children with MODS,because the underlying disease possibly is reversible.This article focuses on the different modalities of ECLS which involve extracorporeal membrane oxygenation,continuous renal replacement therapy,artificial liver support system,hemoperfusion and plasma exchange.     

人工肝支持系统在肝功能衰竭中的应用

肝功能衰竭是多种因素引起的严重肝脏损害,在儿科病因更为复杂且病情凶险,传统的内科综合治疗效果欠佳,病死率高.人工肝支持系统也常称为人工肝,是一种能够替代或模拟正常肝脏的部分或全部功能的体外装置,可以使因肝功能衰竭所产生的各种有害物质得以清除,并替代肝脏的部分代谢功能,以维持患者生命.人工肝应用至今已逾50年,其血液净化方式包括血液透析、血浆置换、血液灌流、吸附、全血或血浆滤过以及应用肝组织或细胞研制的有生物效应的治疗方法等.由于肝脏有强大的再生能力,人工肝可为病变肝脏自身再生恢复或接受肝移植尽可能争取时间.随着人工肝支持系统相关技术的不断完善,非生物型人工肝治疗已在临床广泛应用,并取得很好的疗效,成为各种重症肝功能衰竭的重要治疗手段之一.人工肝在儿科临床也有应用报道,但资料有限,仍需要进一步研究和实践.     

Effects of sulfadimethoxine on tissue distribution of (14C)bilirubin in the newborn and adult hyperbilirubinemic Gunn rate.

Sulfadimethoxine significantly reduced plasma bilirubin levels and altered the tissue bilirubin distribution in both the newborn and the adult Gunn rat. The majority of the unbound bilirubin appeared to distribute perferentially to the intestine and liver in the newborn, whereas in the adult the unbound bilirubin was taken up primarily by the liver. The bilirubin content of brain tissue from both age groups was significantly higher after sulfadimethoxine treatment.     

Renal artery embolization in a child with delayed hemodynamic instability from penetrating knife wound

Penetrating laceration injury in the pediatric population may present as an acute or delayed life-threatening injury. Although emergent intra-arterial embolization is commonly utilized in adults, few cases have been reported for children. Surgical treatment for severe renal laceration injuries may require complete nephrectomy; an unfortunate outcome for a pediatric patient if a renal-preserving alternative is feasible. We present a case of penetrating renal laceration in a 10-year-old boy treated with intra-arterial embolization of the lacerated dominant renal artery and subsequent renal perfusion by an uninjured accessory renal artery allowing for renal preservation.     

胆道闭锁患儿亲体肝移植术后急性肺损伤风险因素分析

目的 分析胆道闭锁患儿亲体肝移植术后急性肺损伤的风险因素.方法 收集天津市第一中心医院2012年5月至2016年3月实施的112例胆道闭锁患儿亲体肝移植术临床资料,回顾性分析临床因素对患儿术后急性肺损伤的影响.结果 112例胆道闭锁患儿亲体肝移植患儿术后23例发生急性肺损伤,发生率为20.5％.单因素logistic回归分析显示肺部并发症组与对照组间年龄(P=0.010)、术前白蛋白(P=0.012)、术前总胆红素(P=0.001)、术前血清肌酐(P＜0.001)、术后1周内总胆红素峰值(P=0.035)差异有统计学意义(P＜0.05).多因素logistic回归分析显示,术前白蛋白(P=0.010,OR=0.830,可信区间为0.720～0.957)、术前总胆红素(P=0.001,OR=1.010,可信区间为1.004～1.016)及术前血清肌酐(P=0.001,OR=1.237,可信区间为1.104～1.387)是术后急性肺损伤发生的高危因素.结论 急性肺损伤是胆道闭锁患儿亲体肝移植术后的严重并发症,术前血清白蛋白、总胆红素及肌酐水平是患儿急性肺损伤发生的高危风险因素.     

Management of coagulopathy with recombinant factor VIIa in a neonate with echovirus type 7

A 5-day-old newborn presented with neonatal enteroviral infection. The patient's hospital course was complicated by acute liver dysfunction, renal insufficiency, fluid overload, respiratory failure, hypertension, catheter related thrombosis, Klebsiella pneumoniae sepsis, intracerebral and intraventricular hemorrhage, and disseminated intravascular coagulation (DIC). Administration of fresh frozen plasma (FFP) and cryoprecipitate failed to control the patient's hemostasis and led to significant fluid overload. Recombinant activated factor VII (rFVIIa, Novoseven NovoNordisk, Bagsvaerd, Denmark) was given to the neonate as a bolus (rFVIIa at 60-80 microg/kg body weight), followed by a continuous infusion (2.5-16 microg/kg/hr). Recombinant activated factor VII controlled hemostasis, until the patient's liver function recovered. The patient's blood product requirement significantly decreased and his fluid overload resolved. Administration of rFVIIa appears to have stabilized the coagulation process. The patient appears to have fully recovered from the infection's complications.