Complications of PORT-A-CATH® in patients with sickle cell disease.

BACKGROUND: Red cell exchange/transfusion is frequently used in the management of patients with medical complications related to acute severe sickle cell disease (SCD). However, peripheral venous access is often difficult without central venous catheters (CVCs) in adult patients with moderate or severe SCD. AIMS: To review our experience with the use of the PORT-A-CATH(®) device in sixteen patients with SCD undergoing exchange or simple transfusions. METHODS: Among a cohort of 550 patients who frequently visited the inpatient service, sixteen SCD patients required the insertion of a PORT-A-CATH(®) device. These patients included 3 males and 13 females, aged 25-44 years [31.1 ± 2.3; mean ± SD]. A total of 24 PORT-A-CATH(®) devices were implanted in these 16 patients during the study period. Eleven patients had 1 device implanted, three patients had 2 devices, one patient had 3 devices, and one patient had 4 devices implanted. RESULTS: Out of the 24 devices implanted, 17 required removal, due to either infection associated with sepsis and/or thrombosis. The organisms involved were Candida spp. (3), C. Parapsilosis (2), C. albicans (1), C. famata (1), C. lusitanice (1), Staphylococcus spp. (6), and S. aureus (3), as well as the coagulase-negative Staphylococcus (2), alpha hemolytic Streptococcus (1), Diphtheroid bacilli (2), Pseudomonas aeruginosa (2), Ps. Spp. (3), Escherichia coli (3), Klebsiella oxytoca (1), Klebsiella pneumoniae (1), Klebsiella spp. (1), Serratia liquefaciens (1), Serratia fanticola (1), Achromobacter spp. (2) Chromobacterium violaceum (1), Delftia acidovirans (1), Stenotrophomonas maltophile (1), Alcaligenes faecalis (1), and Enterobacter cloacae (1). Two episodes of documented thrombosis were observed. One case presented with right atrial thrombosis/SVC syndrome and the other case presented with left upper arm thrombosis. Two patients died with ports in situ, while five patients had ports in place at the time of this study. The median working life of the ports was 688.5 days (range: 39-3925). The rate of infective complications was 2.63 infections per 1000 catheter days, and the number of infections was significantly correlated with the number of ports [Pearson's r=0.66; p<0.01]. DISCUSSION: Our results suggest that patients with SCD suffer infective complications associated with the PORT-A-CATH(®), which often necessitate its removal. Although these devices are extremely useful, their optimal beneficial potential is only realized if the patients receive proper care at special centers well-versed in the maintenance of such devices by experienced staff.

Double volume exchange transfusion for ABO incompatible liver transplantation in paediatric patients: intraoperative and preoperative approach.

Double volume blood exchange transfusions (DBVET) were used to reduce the serum antibody levels in six paediatric patients receiving ABO incompatible hepatic allografts. In four patients, the exchange transfusions took place on the ward prior to surgery. In three of these four patients who had titres measured, the anti-A IgM titres fell from 1024 to 64, 64 to 8, and 128 to 16, respectively. The anti-A IgG titres fell from 32 to 16 and 512 to 64 in two patients, but rose from 16 to 32 in the third. In two patients DBVET were performed intraoperatively using a rapid infusion device. The IgM titres fell from 256 to 32 and 64 to 1, respectively, and the IgG titres fell from 16 to 4 and 2 to 0. Intraoperative DBVET can acutely and effectively reduce blood group antibodies. Intraoperative DBVET may reduce graft ischaemia time and allow red blood cell salvage.

Neonatal bladder rupture: an unusual complication of umbilical catheterization.

Bladder injury in the neonatal period is an exceedingly rare phenomenon that is usually iatrogenic. Bladder rupture as a complication of umbilical catheterization in a newborn with urinary ascites, respiratory distress and hematuria is presented and discussed.

Filtration-assisted exchange transfusion using alpha alpha Hb, an erythrocyte substitute.

A method for improving the efficiency of exchange transfusion to evaluate hemoglobin- (Hb) based erythrocyte substitutes is described. The method uses a continuous-flow hollow-fiber plasma separation filter to remove the erythrocytes while returning 75% of the plasma. The removed volume was replaced with a 14-g/dl solution of human Hb cross-linked between the alpha-chains with bis(3,5-dibromosalicyl)fumarate (alpha alpha Hb). Filtration of 2.76 blood vol in anesthetized swine resulted in a 95% reduction of hematocrit and produced a plasma Hb concentration of 7.63 g/dl. Hyperoncotic Hb solutions cause volume expansion, which reduces the efficiency of exchange but provides hemodynamic stability in the face of decreasing blood viscosity and subsequent intravascular volume loss with Hb redistribution. Filtration-assisted exchange transfusion is rapid, conserves valuable modified Hb, and ensures continuous adequate oxygen delivery.

[Automated exchange transfusion in premature and newborn infants with hyperbilirubinemia using a peripheral arteriovenous vascular access device]. / Automatisierte Austauschtransfusion bei Früh- und Neugeborenen mit Hyperbilirubinämie unter Verwendung eines peripheren arteriovenösen Gefässzuganges.

The technique of exchange transfusion using a plastic catheter in the umbilical vein developed by Diamond and modified by Allen was instrumental in decreasing the mortality and morbidity in newborn infants with jaundice. Allen et al. demonstrated that the development of kernicterus in infants with erythroblastosis with indirect hyperbilirubinemia could be prevented by this method. Since that time numerous modifications of this "single site, push pull technique" have been described to further reduce the risk of morbidity and mortality. On the basis of the successful use of percutaneous radial artery catheters for arterial blood gas monitoring, we developed a technique for exchange transfusion using a peripheral arterial catheter inserted in the radial artery for blood withdrawal and a peripheral venous catheter for replacement of heparinized compatible donor blood. For simultaneous withdrawal and replacement we used two volumetric infusion pumps, one aspirating patients' arterial blood, the other replacing equal volumes of donor blood at exactly the same time. We evaluated our new procedure in 19 newborn infants (group 1) with hyperbilirubinemia who required exchange transfusion. Retrospectively we analysed the data in 18 newborn infants (group 2) treated over a previous period by means of the conventional "one site, push pull" technique using the umbilical vein, and compared the data. Mean birth weights, gestational ages and Apgar scores were similar in both groups, as were volumes of blood used for exchange transfusions. The results were superior in group 1 infants, as reflected by the significantly greater percentage decreases in serum bilirubin concentration and fewer complications. No baby died.(ABSTRACT TRUNCATED AT 250 WORDS)

Automated exchange transfusion and exchange rate.

An automated blood exchange transfusion (BET) with a two-site technique has been devised by Goldmann et al and by us, using an infusion pump. With this method, we successfully performed exchange transfusions 189 times in the past four years on 110 infants with birth weights ranging from 530 g to 4,000 g. The exchange rate by the automated method was compared with the rate by Diamond's method. Serum bilirubin (SB) levels before and after BET and the maximal SB rebound within 24 hours after BET were: 21.6 +/- 2.4, 11.5 +/- 2.2, and 15.0 +/- 1.5 mg/dl in the automated method, and 22.0 +/- 2.9, 11.2 +/- 2.5, and 17.7 +/- 3.2 mg/dl in Diamond's method, respectively. The result showed that the maximal rebound of the SB level within 24 hours after BET was significantly lower in the automated method than in Diamond's method (p less than 0.01), though SB levels before and after BET were not significantly different between the two methods. The exchange rate was also measured by means of staining the fetal red cells (F cells) both in the automated method and in Diamond's method, and comparing them. The exchange rate of F cells in Diamond's method went down along the theoretical exchange curve proposed by Diamond, while the rate in the automated method was significantly better than in Diamond's, especially in the early stage of BET (p less than 0.01). We believe that the use of this automated method may give better results than Diamond's method in the rate of exchange, because this method is performed with a two-site technique using a peripheral artery and vein.

Intravascular intrauterine transfusion for severe erythroblastosis fetalis using different techniques.

Over a 3-year period, 44 ultrasound-guided intravascular transfusions were performed between 18 and 32 weeks on 15 patients with severe erythroblastosis fetalis due to Rh immunization. In 4 fetuses, the first transfusion was performed before 20 weeks, in 6 between 20 and 25 weeks and in the remaining 5 between 25 and 31 weeks. Eight of the 15 fetuses were hydropic at the time of referral. Five transfusions were done in the intrahepatic umbilical vein, 6 were simple transfusions via percutaneous umbilical cord puncture, and 33 were partial exchange. There were 4 intrauterine deaths before 26 weeks, despite successfully performed transfusions: 3 of these fetuses were severely hydropic, while in the remaining fetus hydrops had been reversed in utero. Following delivery by cesarean section at 32 weeks of gestation, 1 of the neonates developed respiratory distress syndrome and died 17 h after birth. The overall survival rate was 67% (10 of 15 cases): 4 of the 8 hydropic fetuses (50%) and 6 of the 7 nonhydropic fetuses (83%) were alive at birth and survived the perinatal period. Three of the 5 losses occurred among the first 4 cases, while in the last 11 cases the survival rate increased to 82% (9 of 11).

[Single-needle diafiltration and single-needle plasmapheresis in premature infants]. / Single-needle-Diafiltration und Single-needle-Plasmapherese bei Frühgeborenen.

Extracorporal detoxication methods in newborns are most unavailable, contraindicated because of technical problems. We report our experiences with a blood pump unit developed by our-selves for diafiltration and plasmapheresis in newborns. In 6 newborns with acute renal failure, Rh-ery-throblastosis and hypoproteinemia, 13 single-needle-diafiltrations and 6 single-needle-plasmapheresis were performed with the self-constructed double head pump, special tube systems and small models. Our results show that all extracorporal detoxication methods can be carried without problems.

Exposure of newborn infants to plasticizers. Plasma levels of di-(2-ethylhexyl) phthalate and mono-(2-ethylhexyl) phthalate during exchange transfusion.

The exposure of newborn infants to the plasticizer di-(2-ethylhexyl) phthalate (DEHP) and its primary metabolite mono-(2-ethylhexyl) phthalate (MEHP) was studied during exchange transfusions by measuring their contents in the infused blood. Plasma concentrations of DEHP and MEHP in the blood withdrawn from the infants during the transfusions also were determined. The amounts of DEHP and MEHP inadvertently infused varied from 1.7 to 4.2 and 0.2 to 0.7 mg per kg body weight, respectively. Immediately after the transfusions, the plasma levels of DEHP in the individual infants varied between 3.4 and 11.1 micrograms per ml. MEHP in the corresponding samples ranged from 2.4 to 15.1 micrograms per ml. Judging from plasma concentrations of DEHP and MEHP during and after transfusion, there was no gradual accumulation of these substances in the plasma during the course of the transfusion. In the two infants who underwent a second exchange transfusion, significant levels of phthalates were found at 16 and 23 hours, respectively, after the first transfusion. Plasma concentrations of DEHP in these infants declined at a faster rate than those of MEHP, thus pointing to the importance of examining the pharmacokinetics of this potentially toxic metabolite.

Use of erythrocytapheresis in the treatment of patients with sickle cell anemia.

We performed exchange transfusions, utilizing the technique of automated erythrocytapheresis, for the treatment of patients with sickle cell anemia. In an attempt to determine guidelines for the use of erythrocytapheresis, we studied the use of this procedure in three distinct clinical situations in nine patients with sickle cell disease. Patients with dangerous complications of sickle cell disease such as acute respiratory distress and priapism responded well to erythrocytapheresis, showing marked improvement within 24-48 hours. Patients with prolonged painful vasoocclusive crises showed only variable improvement after erythrocytapheresis therapy, insufficient to justify exposing the patient to the risks of the procedure. Patients treated to decrease the frequency of painful crises demonstrated no prolongation in symptom-free intervals between crises. Therefore, erythrocytapheresis has its main value in the management of acute, dangerous complications of sickle cell disease.

Selective removal of sickle cells with the IBM 2997 continuous flow blood cell separator.

Partial red cell exchange transfusion is used to manage several complications of sickle cell anemia. Reports have stressed the safety, ease, and speed of red cell exchange procedures performed with blood cell separators, but little information exists concerning operating conditions for the most efficient removal of hemoglobin S (HbS) containing cells. We performed 13 red blood cell exchange transfusions with the IBM 2997 continuous flow blood cell separator on two previously transfused adult homozygous (SS) sickle cell patients. We used both a single stage and a dual stage disposable pathway and varied rotor speed, collection port, and collection interface independently. The patients' baseline percentage HbS ranged from 28 to 64% (mean = 46%); post exchange values ranged from 19.1 to 46% (mean = 32%). Thirty-three of 34 specimens collected from the white cell or platelet port showed a higher percentage of HbS in the collection line compared to simultaneous patient samples. Mean enrichment was 9.4% (range = -4.0 to 35.5%). There was an inverse relationship between the hemoglobin concentration and the percentage of HbS in the collected specimens (p = 0.001; N = 45). Differential separation of sickle cells should decrease the volume of blood required for partial red cell exchange of selected SS patients.