Peritoneal dialysis in the treatment of metabolic crises caused by inherited disorders of organic and amino acid metabolism

Four neonates who presented with coma secondary to hyperammonaemia resulting in central respiratory failure were treated with peritoneal dialysis for between 16 and 120 hours. Underlying diseases were maple-syrup-urine disease, propionic acidaemia and citrullinaemia in two patients. Clinical improvement was observed in three patients within 16 to 72 hours after institution of peritoneal dialysis. Biochemical analysis revealed a rapid reduction in plasma concentration of leucine, isoleucine and valine as well as their alpha-keto-analogues in the infant suffering from maple-syrup-urine disease. Correction of ammonia, glycine, alanine and propionic acid concentrations was observed in the infant with propionic acidaemia 24-72 hours after institution of peritoneal dialysis. Severe hyperammonaemia (1,000-2,500mumol/l) in two infants with citrullinaemia before peritoneal dialysis was treated successfully in one infant; whereas the second infant showed no clinical improvement despite amelioration of biochemical parameters. Glucose-absorption from peritoneal dialysis solution was in the range of 216-441 mg/kg/h.     

Disorder of the blood-brain barrier caused by mannitol poisoning in an infant with encephalotoxicosis

The clinical course of a mannitol intoxication in a 5-month-old infant is reported. Mannitol measurements were performed by gas-chromatographic-mass-spectrometric analysis. The role of the osmolal gap as a simple diagnostic tool in mannitol intoxication was underlined by comparison to serum mannitol levels. Mannitol elimination was analysed by measuring mannitol levels in urine, ultrafiltrate, and peritoneal dialysis outflow. The highest concentrations were found in urine (approx. 300% serum values) and the lowest in peritoneal dialysis outflow (approx. 50% serum levels). "Total body Mannitol" was calculated each day from body weight, hydration, and serum mannitol levels and opposed to the amount eliminated via urine, ultrafiltration, and peritoneal dialysis. The results were only compatible with a volume of distribution of approx. 5.3 1, representing total body water at a lean body weight of 7 kg. It could thus be demonstrated that the sudden fall of serum mannitol levels from 19.6 mg/ml to 5.5 mg/ml without dialysis treatment in an anuric patient was due to a leak of mannitol into the intracellular compartment.     

Peritoneal Dialysis in the Treatment of Metabolic Crises Caused by Inherited Disorders of Organic and Amino Acid Metabolism

ABSTRACT. Four neonates who presented with coma secondary to hyperammonaemia resulting in central respiratory failure were treated with peritoneal dialysis for between 16 and 120 hours. Underlying diseases were maple-syrup-urine disease, propionic acidaemia and citrullinaemia in two patients. Clinical improvement was observed in three patients within 16 to 72 hours after institution of peritoneal dialysis. Biochemical analysis revealed a rapid reduction in plasma concentrations of leucine, isoleucine and valine as well as their alpha-keto-analogues in the infant suffering from maple-syrup-urine disease. Correction of ammonia, glycine, alanine and propionic acid concentrations was observed in the infant with propionic acidaemia 24–72 hours after institution of peritoneal dialysis. Severe hyperammonaemia (1000–2500 μmol/1) in two infants with citrullinaemia before peritoneal dialysis was treated successfully in one infant; whereas the second infant showed no clinical improvement despite amelioration of biochemical parameters. Glucose-absorption from peritoneal dialysis solution was in the range of 216–441 mg/kg/h.     

The value of peritoneal dialysis in the treatment of severe ethanol intoxication in childhood revised

We treated a girl aged 3.5 years (15 kg) with ethanol intoxication, using peritoneal dialysis. The blood ethanol concentration was 6.4 g/l (640 mg/dl; 138.9 mmol/l). It was calculated that the child drank a total amount of 67.2 g of ethanol (4.5 g/kg). The spontaneous ethanol elimination rate before peritoneal dialysis was 0.27 g/l (5.86 mmol/l) per hour; during peritoneal dialysis we found an ethanol elimination rate of 0.32 g/l (6.94 mmol/l) per hour, which was lower than expected. In childhood the ethanol elimination rate with peritoneal dialysis is only slightly faster in comparison to the high spontaneous elimination rate. We conclude that treatment of severe ethanol intoxication should include mainly the maintenance of the vital functions and the meticulous control of blood sugar levels and acid-base disturbances, especially in children. Indications for dialysis are complications caused by ethanol and resistant to supportive therapy, such as seizures, metabolic disturbances, persistent hypoglycemia and the possibility of combined intoxication with other dialysable drugs.     

Chronic peritoneal dialysis: an alternative to iterative hemodialysis]

27 children, aged 7 months to 15 years, with terminal renal failure and no available vascular access, were treated with chronic peritoneal dialysis for 3 weeks to 9 months (mean 3 months). An indwelling silicon catheter fitted with a subcutaneous dacron felt cuff was used; the average catheter life time was 10 weeks (3 to 25 weeks). Control of uremia was satisfactory with mean serum urea decreasing from 2 to 1 g/l and creatinine from 130 mg/l to 60 mg/l after 48 hours of dialysis. No uremic complications occured. Total serum protein remained stable: mean: 62 g/l prior to treatment and 60 g/l after the treatment period. Hematocrit was higher than in hemodialysed children (17% versus 15%). Three children were directly transplanted without difficulty. However, some complications did occur. There were 27 episodes of catheter obstruction leading to 12 surgical interventions. 18 episodes of peritonitis (5% of total dialyses) occured in 12 patients, and two were lethal. The frequency of complications prohibits a recommendation of chronic peritoneal dialysis over hemodialysis in children; this technique however remains very helpful in those situations where vascular access is difficult.     

Peritoneal dialysis in chronic renal failure in children: a six year study

A six year study was conducted to evaluate the long-term use of chronic peritoneal dialysis in children with end-stage renal failure at a single center. All patients maintained satisfactory clinical status and achieved good biochemical control. No significant changes in developmental continuum as measured by weight and length/height were observed in these patients upon institution and maintenance on peritoneal dialysis. The incidence of peritonitis was one episode every 12.3 patient-months. The overall incidence of catheter replacement was one change every 15 patient-months. Our results serve to underscore the effectiveness of long-term peritoneal dialysis in children.     

腹膜透析治疗小儿肾功能衰竭临床分析

目的 探讨腹膜透析对儿童肾功能衰竭治疗的效果.方法 对2003年6月至2008年4月应用腹膜透析治疗的11例急慢性肾功能衰竭患儿临床资料及随访结果 进行分析.结果 11例患儿无一例死亡,急性肾功能衰竭平均在院透析时间15.5 d,慢性肾功能衰竭平均在院透析时间22.8 d.治疗前后血尿素氮、肌酐分别由(34.03±8.44) mmol/L和(710.09±167.54) μmol/L降至(15.94±4.93) mmol/L和(233.87±92.71) μmol/L,差异有非常显著性(P＜0.01).血钠由(130.91±9.15) mmol/L升至(139.46±3.98) mmol/L,差异有显著性(P＜0.05).血碳酸氢根由(14.56±2.07) mmol/L升至(22.47±3.29) mmol/L,差异有非常显著性(P＜0.01).随访时间1个月至5年不等.5例急性肾功能衰竭患儿肾功能和尿常规均正常.1例慢性肾功能衰竭患儿规律透析后行肾移植,3例仍于院外透析中.结论 经济、实用、有效的腹膜透析辅以综合治疗可成为儿童急慢性肾功能衰竭较好替代治疗方法 .     

Transcutaneous blood gas monitoring during peritoneal dialysis in the neonate

Transcutaneous oxygen and carbon dioxide were monitored continuously during peritoneal dialysis in an infant with severe idiopathic respiratory distress syndrome requiring mechanical ventilation. These showed marked changes in blood gases occurring in phase with the cycles of dialysis due to interference with ventilatory function by fluid in the peritoneal cavity. This finding has important implications for the management of infants requiring peritoneal dialysis during mechanical ventilation.     

Effect of hemodialysis on the plasma levels of clofarabine

Clofarabine, a nucleoside analogue for treatment of relapsed leukemia, is 50–60% excreted in urine. Clofarabine has not been studied in patients on hemodialysis. We measured levels in one patient in acute renal failure. Prior to dialysis, 43 hr after a 40 mg/m² infusion, plasma concentration was 139 ng/ml. One hour after begining hemodialysis, a 20 mg/m² infusion began. Plasma concentrations were 84.2, 81.1, and 88.0 ng/ml while the dialysis and clofarabine infusion occurred simultaneously. Post‐dialysis, while the clofarabine was still infusing, the level was 95.8 ng/ml. Hemodialysis does decrease clofarabine levels, but given its large volume distribution, hemodialysis may not be effective for clofarabine overdose. Pediatr Blood Cancer 2010;55:196–198. © 2010 Wiley‐Liss, Inc.     

A novel alternative for renal replacement therapy: 2-year successful colonic dialysis via a Malone antegrade continent enema stoma

This study is a case report of home-based colonic dialysis (CD) for treating end-stage renal disease in a 20-year-old woman. She had a history of Malone antegrade continence enema (MACE) for treating neuropathic bowel at the age of 11 years. The patient refused any type of renal replacement therapy. However, she agreed to CD through the MACE stoma by changing the colonic irrigation solution to the peritoneal dialysis solution. The patient was discharged with a plasma creatinine (Cr) level of 1.7 mg/dL and blood urea nitrogen (BUN) level of 8 mg/dL. She has continued CD on a regular basis at home. The patient's serum Cr and BUN has remained in the steady low state during 24 months of follow-up (mean Cr level = 2.8 mg/dL and mean BUN level = 10.7 mg/dL).     

Intestinal obstruction due to peritoneal adhesions as a complication of peritoneal dialysis for neonatal hyperammonemia

A male infant with ornithine transcarbamylase deficiency developed massive neonatal hyperammonemia and was treated with peritoneal dialysis. He later developed intestinal obstruction due to peritoneal bands which originated at the site of the previous peritoneal catheter. In this infant the blood concentration of ammonia could not be lowcred below 1000 g/dl using peritoneal dialysis, while treatment with sodium benzoate led to control within 24 h. In view of the possibility of this and other complications of peritoneal dialysis, pharmacologic therapy of neonatal hyperammonemia should be considered as an initial modality of treatment.     

腹膜透析治疗小儿重症颅脑损伤并严重高钠血症的临床研究

目的：观察腹膜透析对小儿重症颅脑损伤合并严重高钠血症的治疗效果。方法对47例重症颅脑损伤合并严重高钠血症患儿,观察腹膜透析前后血钠浓度变化及其规律,并经有创颅内压监测颅内压变化,评估腹膜透析对颅内压的影响,测定血浆晶体渗透压、血肌酐和血气分析、血压、心率等的变化,评估腹膜透析治疗的效果与安全性。结果与治疗前相比,血钠每天均下降,以第1天下降幅度大,速度快（187．49±2．91 vs 202．48±9．31,P＜0.05）,其后降钠速度减慢。持续颅内压监测颅内压逐渐下降,第1天下降明显（164．58±5．98 vs 177．83±7．47,P＜0.05）。腹膜透析期间,血浆晶体渗透压下降,酸中毒纠正,生命体征稳定。结论腹膜透析可有效治疗重型颅脑损伤后高钠血症,与其弥散、降低颅内压等因素有关,临床安全有效。     

Continuous-cycling peritoneal dialysis for children: an alternative to hemodialysis treatment

Treatment of end-stage renal failure in children is invasive and prolonged. Although kidney transplantation is often the desired therapy, children usually require some form of life-sustaining dialysis until a suitable donor is found. Home continuous-cycling peritoneal dialysis (CCPD) is a useful alternative to in-center hemodialysis for these children. Adequate biochemical control of the uremic state can be achieved with continuous-cycling peritoneal dialysis. Peritonitis remains the major complication of this form of dialysis, averaging approximately one episode per 12 patient-months. Growth rates of children maintained on continuous-cycling peritoneal dialysis appear to be equivalent to growth rates of children treated with hemodialysis. The advantage of continuous-cycling peritoneal dialysis lies in the fact that exchanges occur during the evening hours and parental intervention is minimized.     

持续性腹膜透析儿童腹膜平衡试验及结果分析

目的通过腹膜平衡试验（PET）探讨我国慢性腹膜透析（PD）儿童腹膜转运特性特点。方法对6例持续性非卧床腹膜透析（CAPD）患儿（2—14岁）行10次儿童标准PET,参照Twardowski和儿科腹膜透析联盟（PPDSC）标准评价腹膜溶质转运类型。结果本组患儿首次PET于PD开始后平均（38．74±15．6）d进行。4h肌酐清除率（4h-D／P）和4h葡萄糖吸收率（4h-D／D0）分别为（0．85±0．24）、（0．34±0．19）。依Twardowski和PPDSC腹膜转运类型评价标准,本组腹膜溶质转运类型分别为高转运型6例（6／10）、高平均转运型1例（1／10）、低平均转运型3例（3／10）,无一例低转运型;两种标准分型的总符合率100％。本组腹膜葡萄糖转运类型分别为高转运型3例（3／10）、高平均转运型4例（4／10）、低平均转运型1例（1／10）,低转运型2例（2／10）;两种标准分型的总符合率90％。连续PET显示转运类型变化不一,腹膜炎后4h-D／P升高。结论本组CAPD儿童腹膜溶质和葡萄糖转运类型均以高转运和高平均转运为主（7／10）,呈偏态分布,提示儿童腹透溶质清除充分,但水超滤能力不足;标准儿童PET及其评价标准完全符合Twardowski标准PET要求。腹膜炎后溶质转运能力提高。     

儿童慢性腹膜透析相关腹膜炎危险因素的病例对照研究

目的 分析终末期肾病（ESRD）患儿慢性腹膜透析（CPD）相关腹膜炎(简称腹膜炎)发生的危险因素,为儿童腹膜炎防治提供依据。方法 回顾性收集复旦大学附属儿科医院（我院）开展ESRD患儿CPD 以来的全部病历,依据连续随访记录,以是否出现CPD分为腹膜炎组和非腹膜炎组,分析两组人口学指标、CPD指标和透析充分性指标。并行多因素分析探讨发生腹膜炎的危险因素。结果 2001年至2014年在我院接受CPD治疗的109例ESRD患儿进入本文分析,男60例（55%）、女49例。开始腹膜透析的中位年龄9.9岁,中位透析病程13.4月。连续性非卧床腹膜透析（CAPD）15例,自动腹膜透析（APD）94例（86.2％）。43例（39.4%）仍接受CPD治疗,50例（45.9%）成功行肾移植,7例（6.4%）转行血液透析治疗,6例（5.5%）死亡,3例（2.8%）失访。1、2、3和5年的生存率分别为97.1%、93.3%、90.1%和90.1%。33例发生57例次腹膜炎,平均腹膜炎发生率为1次/35.1病人月。单因素分析显示,开始透析时身高SDS（P=0.01）、透析病程（P<0.001）和白蛋白水平（P=0.01）腹膜炎组和非腹膜炎组差异有统计学意义。多因素Logistic回归分析显示,开始透析时身高SDS<-2.0（OR=12.746,95％CI：2.436~66.675,P=0.003）和透析病程>1年（OR=8.162,95%CI：2.514~26.500,P<0.001）是腹膜炎发生的独立危险因素。结论 开始透析时身高SDS<-2.0和透析病程>1年是儿童CPD相关腹膜炎发生的独立危险因素。     

Low-volume peritoneal dialysis in 116 neonatal and paediatric critical care patients

Acute renal insufficiency accounts for high mortality in paediatric intensive care patients, particularly in infants. Peritoneal dialysis, usually carried out with dialysate volumes of >20 ml/kg body weight, increases pulmonary artery pressure, which may compromise myocardial function in critical illness. In this paper we report our experiences with the use of lower dialysate volumes in the treatment of critically ill children with renal impairments. We suggest that low-volume peritoneal dialysis is able to achieve adequate ultrafiltration, which relieves overhydration in ventilated and haemodynamically compromised children. A total of 116 paediatric intensive care patients treated between 1992 and 2000 was the subject of this investigation. Diagnosis, indication for dialysis, arterial and central venous pressure, blood gases, creatinine, blood urea nitrogen, urinary output at installation, ultrafiltration, fluid balance, duration and complications during dialysis as well as survival were investigated. The overall mortality was 53%. The respective diagnoses and mortality rates were as follows: 65% of the patients suffered from cardiac diseases (54% mortality), 7% from renal diseases (13%) and 28% from multi-organ system failure (62%). Low-volume peritoneal dialysis was started at evidence of total body fluid overload with inadequate urinary output and resulted in a mean ultrafiltration of 2.8 ml/kg body weight per h. A negative fluid balance was achieved in 53% of patients, mainly in those suffering from hypervolaemia and minor oliguria. None of the complications resulted in death. CONCLUSION: early installation of low-volume peritoneal dialysis offers a safe and adequate ultrafiltration procedure for paediatric critical care patients suffering from minor oliguria and fluid overload.     

Die Peritonealdialyse zur Behandlung reifer und unreifer Neugeborener mit Atemnosyndrom

Some authors have recommended peritoneal dialysis to improve the therapy of the respiratory distress syndrome (RDS) of newborns. In 1968 we published a report estimated on a newborn suffering from RDS. As a pH of 6.73, was 2 hrs after birth, we administered standard therapy and peritoneal dialysis. The baby has survived and his development has been uneventful. This case and the results obtained by other authors in the treatment of RDS with peritoneal dialysis have led us to treat prematures and newborns suffering from severe RDS with peritoneal dialysis in addition to standard therapy (infusions of THAM, glucose, electrolytes, amino acids, administration of antibiotics and oxygen, and, when indicated, intermittent positive-pressure respiration). Peritoneal dialysis was carried out with bland hypertonic solution in continuous flow technics. We have treated 13 prematures and 1 newborn suffering from RDS and a premature suffering from erythroblastosis with hydrops and RDS. Except for the mature newborn, the gestational age was 25 to 33 weeks. Four prematures had birth weights below 1250 g. The Apgar score of 10 newborns was 3 or less 1 min after birth. Thirteen newborn needed resuscitation in the delivery room. Eight newborns were treated with respiration during the first few hours after birth and 4 other prematures at a later stage. Clinical and laboratory data after birth, on admission to the pediatric hospital and during the course of the disease are shown in tables and figures. The indications for the peritoneal dialysis are described in tables. In 2 patients we saw no positive effect, and we saw only a slight effect in 4 patients. Four prematures survived. The physical and psychological development was normal in 3 infants. One premature with a birth weight of 1200 g had recovered before she died of Candida sepsis at the age of 26 days. Results of the treatment and the efficacy and technical problems of peritoneal dialysis in RDS of newborns are discussed. Our opinion is that the peritoneal dialysis is a good additional aid in the treatment of RDS of prematures and newborns.     

腹膜透析在新生儿心脏直视术后的应用

目的 探讨心脏直视术后腹膜透析在治疗新生儿急性肾功能衰竭方面的应用.方法 2006年1月至2008年9月,我院共有131例新生儿先天性心脏病患儿接受体外循环下心脏直视手术,其中男88例,女43例;年龄1 h至28 d;体重1.13～5.10kg,平均(3.34±0.54)kg;其中13例患儿在术后因罹患急性肾功能衰竭而接受腹膜透析治疗,男9例,女4例;年龄1 h至28 d;体重为1.13～4.80kg,平均(3.25±0.72)kg.3例患儿在术中经胸骨正中切口下方的腹膜切口间接放置腹膜透析管,另外10例患儿则在术后经脐旁腹壁切口直接放置.透析液采用百特专用腹膜透析液,并根据病情的需要选择不同渗透压的液体.常规透析方案为:首次透析选用1.5%腹膜透析液,剂量为10～30 ml/kg;输入15min,滞留30 min,引出15min;根据患儿病情变化调整腹膜透析液的浓度、滞留时间比.结果全部患儿均无腹膜透析禁忌证.11例肾功能恢复,2例死亡.无腹膜透析并发症发生.结论 腹膜透析是治疗新生儿心脏直视术后急性肾功能衰竭的有效方法 ,并发症发生率低,操作简单,经济实用.     

Treatment of a neonate with propionic acidaemia and severe hyperammonaemia by peritoneal dialysis.

A moribund newborn infant with propionic acidaemia and severe hyperammonaemia was successfully treated by peritoneal dialysis. The removal of ammonia and possibly additional toxic metabolites by peritoneal dialysis may be life-saving in newborn infants with propionic acidaemia or other hyperammonaemic syndromes.     

儿童终末期肾病全自动腹膜透析并发症与休整期相关性分析

目的：分析儿童终末期肾病腹腔镜及开放手术置管腹膜透析并发症的危险因素。方法：复旦大学附属儿科医院（我院）诊断为终末期肾病、符合慢性腹膜透析（CPD）的适应证、行腹膜透析置管术且术后接受休整期（置管术后至开始腹膜透析的间隔时间）观察并起始透析的患儿。根据腹膜透析后6个月内有无并发症（导管移位、堵塞、渗漏,疝,腹膜透析相关腹膜炎,出口感染,隧道感染等）分为2组,考察并发症与休整期和不同置管方式（腹腔镜和开放手术）相关性。结果：符合本文纳入标准的CPD患儿144例,男84例,平均年龄（8.8±3.8）岁,腹腔镜置管83例,开放手术置管61例,透析前白蛋白（35.8±7.8）g·L-1。有并发症组（n=54)和无并发症组（n=90)性别、年龄、BMI、休整期、原发病、透析前白蛋白、是否大网膜切除差异均无统计学意义,与腹腔镜和开放手术置管差异有统计学意义（30.1% vs 47.5%,P＝0.03）。腹腔镜较开放手术置管腹膜透析相关腹膜炎发生率低（6.0% vs 18.0%, P＝0.02）,导管移位、堵塞、渗漏,疝,出口感染,隧道感染等发生率差异均无统计学意义。结论：腹腔镜与开放手术置管比较,腹膜透析相关并发症少,休整期不影响腹膜透析相关并发症。