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Child Kidney Dis > Volume 21(2); 2017 > Article
Her, Lee, Kim, Lee, Kim, and Shin: Therapeutic Plasma Exchange in Pediatric Kidney Disease: 23-year Experience at the Severance Children’s Hospital in Korea

Abstract

Purpose

The American Society for Apheresis provides clinical guidelines for therapeutic apheresis in adults, but there are no guidelines for children. This study aimed to analyze the effect of therapeutic plasma exchange (TPE) in pediatric patients with various kidney diseases in Korea.

Methods

We retrospectively reviewed the data of 16 children (up to 18 years of age) who were admitted to Severance Children’s Hospital with refractory kidney disease. All patients received TPE between 1994 and 2016. Clinical and laboratory characteristics such as age, weight, sex, change in blood urea nitrogen (BUN), and creatinine level before and after TPE, and complications after TPE were analyzed.

Results

The mean age and weight of the 16 patients at the time of TPE was 11.3±4.0 years and 34.6±17.5 kg, respectively. The BUN level was 35.4 mg/dL before TPE and significantly decreased to 21.5 mg/dL (P=0.025) at 1 week and 20.5 mg/dL (P=0.01) at 1 month after TPE. The creatinine level significantly decreased from 1.20 mg/dL before TPE to 0.90 mg/dL (P=0.02) at 1 week after TPE. Four complications (hypovolemia, anemia, hypocalcemia, and thrombocytopenia) were reported, but were not fatal.

Conclusion

Our findings suggest that TPE is an effective therapeutic modality in children with refractory kidney disease and can be indicated for the treatment of various kidney diseases.

Introduction

Therapeutic plasma exchange (TPE) is a treatment modality to remove circulating pathologic substances from the plasma and replace them with a substitution fluid (e.g., albumin or plasma) [1,2]. TPE has been applied in various diseases such as neurological diseases (e.g., myasthenia gravis, Guillain-Barré syndrome), hematological disorders (e.g., thrombotic thrombocytopenic purpura, hemolytic uremic syndrome), metabolic disorders (e.g., inborn errors of metabolism, homozygous familial hypercholesterolemia), and kidney diseases (e.g., the renal transplant candidate, antibody mediated renal allograft rejection, recurrent focal segmental glomerulosclerosis (FSGS) after renal transplantation, antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibody-associated rapidly progressing glomerulonephritis, and atypical hemolytic uremic syndrome (aHUS)) [3].
The first reported plasmapheresis was performed at Johns Hopkins University in 1914 [4]. Decades later, the first plasma exchange in a human was conducted in a patient with Waldenstrom’s macroglobulinemia in 1960 [5]. The TPE technique was developed gradually and the clinical application of TPE has expanded remarkably. The American Society for Apheresis (ASFA) guideline provides the clinical indications for the evidence-based use of therapeutic apheresis in adult studies [6]. In addition, TPE is a widely recognized therapeutic option in pediatric patients as well as in adults. However, there is no guideline for children, especially those with kidney disease.
In a PubMed search, there were about 30 papers regarding TPE in pediatric patients with kidney disease [7-9]. There were particularly few studies about TPE in children with kidney disease in Korea [10]. Therefore, further study of TPE in pediatric patients with kidney diseases is warranted.
TPE is an important treatment modality for children with renal and other immune-mediated diseases [3]. For the continued development of TPE as an optimal treatment modality in pediatric patients, accurate data are required based on long-term experience.
The purpose of this study was to review the data of pediatric patients with kidney diseases treated with TPE at a single institution over the last 23 years in terms of etiologies, characteristics, complications, and clinical outcomes.

Materials and methods

1. Patients

We retrospectively reviewed the data of children (up to 18 years of age) who underwent TPE between 1994 and 2016 in Severance Children’s Hospital. We identified 37 children admitted to Severance Children’s Hospital who had undergone TPE during the study period. Among them, there were 19 patients (51.3%) with kidney diseases, 9 patients (24.3%) with neurologic diseases, and 7 patients (18.9%) with hepatic diseases. Three of the patients with kidney diseases were excluded due to missing data; thus, 16 patients (2 male and 14 female) with kidney diseases were included in this study.

2. Data collection

The patients’ medical records were obtained from the nephrology department and data including patients’ demographic and clinical characteristics (age, sex, body weight, indication for TPE, treatment, prognosis), main features of the TPE procedures (frequency of TPE, site of initial vascular access, type of replacement fluid, plasma volume), and complications were analyzed. Blood urea nitrogen (BUN), serum creatinine, C3, C4, proteinuria and serum albumin levels were measured before and after TPE, and the values were compared.

3. TPE procedure

Effective volume was calculated as effective plasma volume (mL)=body weight×70 mL/kg×(1-hematocrit). TPE was performed every other day, and a total 1-1.5 volume of plasma was exchanged, depending on patients’ weight and hematocrit. The replacement fluids were albumin 4% and/ or fresh frozen plasma (FFP) according to the indication for TPE. A double-lumen hemodialysis central venous catheter was inserted through the jugular, subclavian, or femoral vein according to the patient’s vascular anatomy. TPE was performed using COBE Spectra (Thermo BCT Inc., Lakewood, CO, USA) by centrifugation.

4. Statistical analysis

Continuous variables are expressed as the mean±standard deviation or medians with ranges. Data were analyzed with a paired t-test. All statistical analyses were performed by using SPSS software (version 22.0, IBM Corporation, Armonk, NY, USA) and a P value less than 0.05 was considered statistically significant.

Results

1. Demographic data

From April 1994 to February 2016 a total of 16 patients with kidney diseases who were younger than 18 years underwent TPE. The clinical characteristics of the patients are shown in Table 1. There were two male and 14 female patients. The mean age at onset was 11.3±4.0 years and the body weight was 34.6±17.5 kg.

2. Therapeutic effects of TPE

In this study, three patients underwent more than one TPE session, resulting in a total of 19 TPE sessions. Some patients responded very well to the first TPE session, but subsequently needed another course of TPE for the same or a different indication.
In the present study, the etiologies for TPE were lupus nephritis (9 patients), FSGS (4 patients), Denys-Drash syndrome (1 patient), microscopic polyarteritis (1 patient), and aHUS (1 patient). The patients were divided into four groups depending on the reason for TPE: refractory (12 patients), recurrent (2 patients), rejection (1 patient), and others (4 patients) (Table 1). The refractory group comprised patients who had no response to steroid, cyclophosphamide, methylprednisolone, azathioprine, and cyclosporine treatment. The recurrent group comprised patients who presented recurrence after kidney transplantation, and the rejection groups comprised patients who presented acute rejection after kidney transplantation.
The comparison between laboratory values (BUN and serum creatinine) before and after TPE is shown in Table 1. BUN level at 1 week significantly decreased from 35.4 mg/dL to 21.5 mg/dL (P=0.025) and creatinine level significantly decreased from 1.20 mg/dL to 0.90 mg/dL (P=0.02). BUN level at 1 month after TPE significantly decreased from 35.4 mg/dL to 20.5 mg/dL (P=0.01) (Table 4).
Also, in order to investigate the TPE effect in patients with lupus nephritis, C3, C4 and proteinuria values before and after TPE are shown in Table 2. In addition, Anti-DNA Ab titer was investigated for TPE effect evaluation in lupus nephritis (Table 2). Among the children with lupus nephritis, six patients were found to have increased anti-DNA Ab titer before TPE. In addition, anti-DNA Ab titer decreased in 3 patients (patient 2, 5, 9) after TPE, and there was no difference in 5 patients (patient 1, 3, 4, 6, 8) after TPE (Table 2).
And serum albumin and proteinuria values before and after TPE are shown in Table 3 to reveal the TPE effect in FSGS patients. In patients with lupus nephritis, C3 level and C4 level at 1 month after TPE increased from 34 mg/dL to 51.1 mg/dL (P=0.082) and from 6.9 mg/dL to 9.5 mg/dL (P= 0.18), respectively. However, these results were not statistically significant compared to those in BUN and creatinine (Table 5). In patients with FSGS, albumin at 1 month after TPE increased from 2.6 g/dL to 3.1 g/dL (P= 0.322), but was not statistically significant (Table 6).

3. Clinical outcomes

Among the 16 patients, 3 patients received kidney transplantation and 3 patients were treated with hemodialysis due to end-stage renal disease (ESRD). A complete response was defined as a patient who was treated after TPE with no more following up. A partial response was defined as patients without proteinuria after TPE or other treatments and patients with well-controlled proteinuria (proteinuria 1+). Complete response was observed in 1 patient (patient 16), and partial response was observed in 5 patients (patient 3, 5, 6, 11 and 13). Three of nine patients with lupus nephritis and two of four patients with FSGS showed partial response. One patient was eventually expired (patient 7).

4. TPE procedure and complications

The 16 patients underwent a total of 104 sessions. The average frequency of TPE per each case was 5.5±3.8 sessions, and the procedures were carried out using a central venous catheter. The type of vascular access was chosen based on the patient’s vascular anatomy and the most common site was the subclavian vein (8 cases), followed by the internal jugular vein (5 cases). The replacement fluids were 4% albumin (9 cases, 47%) or FFP (5 cases, 26%) according to the patients’ general condition. Four complications were reported for the 104 TPE sessions (hypovolemia, anemia, hypocalcemia, and thrombocytopenia) (Table 7).

Discussion

Although TPE has been increasingly used as a first-line therapy in the last few decades, there is a lack of research about TPE in pediatric kidney diseases. Therefore, the aim of this study was to analyze the therapeutic effect of TPE in pediatric kidney diseases [11,12].
In 1980, plasmapheresis was introduced in Korea, and the first procedure was performed in Severance Hospital in a patient with membranous proliferative glomerulonephritis in 1981. TPE non-selectively removes pathogenic substances or replaces a deficient factor, as in systemic thrombotic microangiopathy [3]. The ASFA Special Issue, 7th edition, published in 2016 classifies disorders into four categories. Category I is accepted as first-line therapy, and category II is accepted as supportive or adjunctive therapy. However, category III is not established, and category IV is ineffective or harmful [6]. There are few published reports about TPE conducted in pediatric patients, thus, the indications for TPE in children are based on those in adults.
Song et al. showed that the most common indications of TPE in the nationwide survey on Korea were myasthenia gravis (15.3%), followed by thrombotic thrombocytopenic purpura (TTP) (14.5%), and HUS (9.7%) [13]. Volder et al. showed that TPE indications in pediatric and adolescent patients from the World Apheresis registry data were neurological diseases (33%) and renal diseases (17%). Paglialonga et al. reported that the indications of TPE in European pediatric nephrology units were FSGS (16.4%) and HUS/ TTP (17.9%)8,14). In addition, Reddy et al. reported that the etiologies for TPE were aHUS (75%), anti-glomerular basement membrane disease (12.5%), lupus nephritis (6.25%), and microscopic polyangiitis (6.25%) (Table 8) [15]. In our single-center study, the indications of TPE in pediatric patients with kidney diseases were lupus nephritis (52.6%), FSGS (31.6%), and HUS/TTP (15.8%).
TPE in lupus nephritis is classified as Category IV according to the ASFA. Lewis et al. showed that TPE in combination with prednisolone and cyclophosphamide is not as effective for the treatment of lupus nephritis as compared with prednisolone and cyclophosphamide alone [6]. On the contrary, Li et al. reported that lupus nephritis patients with thrombotic microangiopathy who underwent TPE had a significantly higher rate of remission than those who did not undergo TPE [16,17]. In this study, TPE in a lupus nephritis patient with thrombotic TTP (patient 5) had a clinical effect. Currently, the patient has no proteinuria and is regularly followed up at the outpatient clinic. Among the remaining lupus nephritis patients who underwent TPE, no children have progressed to ESRD, 3 patients (patient 3, patient 5, and patient 6) are undergoing outpatient observation and 3 patients (patient 1, patient 2, and patient 8) failed to attend follow up visits in our clinic. In addition, 1 patient (patient 4) moved to another hospital after improvement, and 1 patient (patient 7) died due to exacerbation of pneumonia. While TPE for lupus nephritis is classified as Category IV according to the ASFA, the results of the present study show that TPE has some effect on lupus nephritis.
In a review article by Ponticelli et al., 70% of recurrent FSGS children who underwent TPE presented complete or partial remission, and according to Straatmann et al., early TPE was 100% effective in patients with recurrent FSGS [18, 19]. In this study, the patient (patient 11) with recurrent FSGS who underwent TPE 6 days after kidney transplantation presented complete remission. However, the patient (patient 10) with recurrent FSGS who underwent TPE 4 months after kidney transplantation progressed to ESRD and was on hemodialysis. Therefore, early TPE in patients with recurrent FSGS may be important to improve the prognosis.
The ASFA guideline classifies TTP for ABO incompatible renal transplantation as Category I. In this study, TPE was performed as first-line treatment for TTP (patient 9 and patient 13) and ABO incompatible renal transplantation (patient 14) and resulted in BUN reduction, creatinine reduction, and clinical effectiveness.
Paglialonga et al. showed that TPE complications in pediatric nephrology units occurred in 51 of 738 sessions (6.9 %). According to Volker et al., complications were reported in 4 out of 50 sessions (8%), and in this study, complications were found in 4 out of 104 sessions (3.8%) [8,20]. None of the complications led to death, and all were resolved. Although TPE is a safe and effective therapeutic tool, Pamela et al. reported that among patients hospitalized with ASFA Category I or II disease, only 13.4% and 9.3% underwent TPE, respectively [21]. In this study, when TPE was performed in patients with diseases corresponding to ASFA Category I and II, TPE was clinically effective in 5 (patient 9, 11, 13, 14, and 16) of 6 patients, and only 1 patient (patient 15) was on hemodialysis due to progressed ESRD. Therefore, it is thought that TPE will be helpful as therapeutic modality in children with kidney disease.
When TPE is performed, albumin and FFP are most commonly used as replacement fluid. Regarding the advantages and disadvantages of each fluid, albumin is relatively expensive but has a lower risk of transfusion-transmitted infection. FFP maintains the activity of all blood coagulation factors but has a high risk of viral transmission and more frequent adverse reactions such as allergic reactions [22,23]. Albumin is used more than plasma due to the lower risk of allergic reactions and transmission of viral infections by plasma [24]. In this study, albumin (47%) was used more frequently than FFP (26%). Depending on the underlying disease and the laboratory findings, the selection of appropriate replacement fluid is important.
Carter et al. reported that catheter-related complications such as catheter-related thrombosis and infection were common when TPE was used in pediatric patients [3]. Catheter- related complications should be considered when performing TPE in children, but these were not reported in this study. Additional considerations for the improvement of TPE outcomes in children include safe sedation in non-cooperative patients and vascular access methods.
Overall, the present study has some limitations including its retrospective design and the inclusion of a small number of patients. As TPE was used in combination with immunosuppressive therapy, the therapeutic effect of TPE alone was not evaluated in this study. In addition, insurancerelated factors may have affected the results of our study. For example, the category proposed by ASFA for certain diseases such as FSGS was changed from Category III in 2007 to Category I in 2010. In Korea, we decided to apply insurance based on the ASFA guideline, so patient 12 in this study who underwent TPE in 2008 did not receive insurance benefits [25,26]. If insurance was more applicable to TPE, it will be helpful in treating children with many kidney diseases.
In conclusion, TPE may be a safe and effective treatment in pediatric kidney disease patients and can be indicated for the treatment various kidney diseases. Further studies are necessary to investigate the overall results in other hospitals and institutions in Korea.

Notes

Conflict of interest
No potential conflict of interest relevant to this article was reported.

References

1. Ward DM. Conventional apheresis therapies: a review. J Clin Apher. 2011;26: 230-8.
crossref pmid
2. Baweja S, Wiggins K, Lee D, Blair S, Fraenkel M, McMahon LP. Benefits and limitations of plasmapheresis in renal diseases: an evidence-based approach. J Artif Organs. 2011;14: 9-2.
crossref pmid
3. Carter CE, Benador NM. Therapeutic plasma exchange for the treatment of pediatric renal diseases in 2013. Pediatr Nephrol. 2014;29: 35-50.
crossref pmid
4. Abel JJ, Rowntree LG, Turner BB. Plasma removal with return of corpuscles (plasmaphaeresis). The Journal of Pharmacology and experimental therapeutics Vol. V. No. 6, July, 1914. Transfus Sci. 1990;11: 166-77.
pmid
5. Schwab PJ, Fahey JL. Treatment of Waldenstrom's macroglobulinemia by plasmapheresis. N Engl J Med. 1960;263: 574-9.
crossref pmid
6. Schwartz J, Padmanabhan A, Aqui N, Balogun RA, Connelly- Smith L, Delaney M, et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice-Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Seventh Special Issue. J Clin Apher. 2016;31: 149-62.
pmid
7. Cleper R, Krause I, Bar Nathan N, Mor M, Dagan A, Weissman I, et al. Focal segmental glomerulosclerosis in pediatric kidney transplantation: 30 years' experience. Clin Transplant. 2016;30: 1324-31.
crossref pmid
8. Paglialonga F, Schmitt CP, Shroff R, Vondrak K, Aufricht C, Watson AR, et al. Indications, technique, and outcome of therapeutic apheresis in European pediatric nephrology units. Pediatr Nephrol. 2015;30: 103-11.
crossref pmid
9. Yim DK, Lee ST, Cho H. Plasmaphresis therapy for pulmonary hemorrhage in a pediatric patient with IgA nephropathy. Korean J Pediatr. 2015;58: 402-5.
crossref pmid pmc
10. Lee JH, Jeon GW, Park SE, Jin DK, Paik KH. An Experience of Therapeutic Plasma Exchange in 9 Pediatric Patients. J Korean Soc Pediatr Nephrol. 2005;9: 38-45.

11. Song KS, Yoon HS, Lee KW, Kim HS, Lee SY. Therapeutic Plasma Exchange (9 Cases). Korean J Clin Pathol. 1983;3: 11-5.

12. Kim HO, Shon ES, Song KS, Lee SS. An Evaluation of 28 Cases of Therapeutic Plasma Exchange. Korean J Clin Pathol. 1988;8: 459-67.

13. Song EY, Kwon SW, Kim DS, Kim DW, Kim DW, Kim HO, et al. Current status of therapeutic plasma exchange in Korea. Ther Apher Dial. 2004;8: 97-101.
crossref pmid
14. Witt V, Stegmayr B, Ptak J, Wikstrom B, Berlin G, Axelsson CG, et al. World apheresis registry data from 2003 to 2007, the pediatric and adolescent side of the registry. Transfus Apher Sci. 2008;39: 255-60.
crossref pmid
15. Reddy SK, Jahan A, Chaturvedi S, Agarwal I. Plasma exchange for paediatric kidney disease-indications and outcomes: a singlecentre experience. Clin Kidney J. 2015;8: 702-7.
crossref pmid pmc
16. Blum D, Blake G. Lupus-associated thrombotic thrombocytopenic purpura-like microangiopathy. World J Nephrol. 2015;4: 528-31.
pmid pmc
17. Li QY, Yu F, Zhou FD, Zhao MH. Plasmapheresis is associated with better renal outcomes in Lupus Nephritis patients with thrombotic microangiopathy. Medicine. 2016;95: 1-10.
crossref
18. Ponticelli C. Recurrence of focal segmental glomerular sclerosis (FSGS) after renal transplantation. Nephrol Dial Transplant. 2010;25: 25-31.
crossref pmid
19. Straatmann C, Kallash M, Killackey M, Iorember F, Aviles D, Bamgbola O, et al. Success with plasmapheresis treatment for recurrent focal segmental glomerulosclerosis in pediatric renal transplant recipients. Pediatr Transplant. 2014;18: 29-34.
crossref pmid
20. Kara A, Turgut S, Cagli A, Sahin F, Oran E, Tunc B. Complications of therapeutic apheresis in children. Transfus Apher Sci. 2013;48: 375-6.
crossref pmid
21. Weiss PF, Klink AJ, Friedman DF, Feudtner C. Pediatric therapeutic plasma exchange indications and patterns of use in US children's hospitals. J Clin Apher. 2012;27: 287-94.
crossref pmid
22. Choi GR, Choi SJ, Shin SA, Lee K, Kim S, Kim HO. A Choice and Precautions of Replacement Fluids for Therapeutic Plasma Exchange. Korean J Blood Transfus. 2015;26: 9-17.
crossref
23. Basic-Jukic N, Kes P, Glavas-Boras S, Brunetta B, Bubic-Filipi L, Puretic Z. Complications of therapeutic plasma exchange: experience with 4857 treatments. Ther Apher Dial. 2005;9: 391-5.
crossref pmid
24. Misanovic V, Pokrajac D, Zubcevic S, Hadzimuratovic A, Rahmanovic S, Dizdar S, et al. Plasmapheresis in Pediatric Intensive Care Unit. Med Arch. 2016;70: 332-5.
crossref pmid pmc
25. Szczepiorkowski ZM, Bandarenko N, Kim HC, Linenberger ML, Marques MB, Sarode R, et al. Guidelines on the use of therapeutic apheresis in clinical practice: evidence-based approach from the Apheresis Applications Committee of the American Society for Apheresis. J Clin Apher. 2007;22: 106-75.
crossref pmid
26. Szczepiorkowski ZM, Winters JL, Bandarenko N, Kim HC, Linenberger ML, Marques MB, et al. Guidelines on the use of therapeutic apheresis in clinical practice--evidence-based approach from the Apheresis Applications Committee of the American Society for Apheresis. J Clin Apher. 2010;25: 83-177.
crossref pmid

Table 1.
The Characteristics and Therapeutic Effects of Patients who Underwent TPE
Patient Diagnosis Sex Age (year) Weight (kg) Year Treatment pre-TPE Reason for TPE Pre-TPE BUN/Cr (mg/dL) 1 weeks f/u BUN/Cr (mg/dL) 1 month f/u BUN/Cr (mg/dL)
1 Lupus nephritis F 15 50.6 1994 P Refractory 14.5/0.5 15.1/0.8 11.0/0.6
2 Lupus nephritis F 12 34 1994 P; MP; CYC Refractory 17/0.5 17.7/0.6 11.5/0.7
3 Lupus nephritis F 15 59 1997 P; MP Refractory 13.5/0.6 8.2/0.8 6.0/0.4
4 Lupus nephritis F 14 54.5 1997 MP Refractory 7.3/0.7 10/0.6 8.0/0.8
5 Lupus nephritis M 12 35.5 1998 - TTP 37/1.1 32.3/1.1 11.7/0.7
6 Lupus nephritis F 14 52.5 2000 P; CsA; MP; CYC Refractory 35.4/2.1 21.5/1 28.5/0.9
7 Lupus nephritis F 8 NA 2001 MP Refractory 84/3.2 33/1.3 Expire
8 Lupus nephritis F 11 NA 2001 P; MP Refractory 28/0.8 9.3/1.3 21.8/0.7
9-1 Lupus nephritis F 13 48 2005 P; CYC Refractory 71.5/6 63.5/5.8 73/7.5
9-2 18 45.5 2011 KT Rejection 43/2.26 25.5/1.79 26.4/1.78
10-1 FSGS F 10 26 1997 P; CsA; KT Recur 49.5/1.2 30/0.9 36/0.8
10-2 12 35 1999 CsA; P Refractory 53.5/1.8 38/1.5 NA
11-1 FSGS F 3 13.5 2001 P; CsA; MP; CYC Refractory 14/0.3 46/0.5 21.5/0.4
11-2 14 33 2013 CYC; KT Recur 35.9/1.2 11.9/0.49 24.7/0.65
12 FSGS F 16 54 2008 P; CsA; MP Refractory 34.2/2.2 24.4/0.9 18.9/1.5
13 FSGS F 7 15 2012 P TTP 23/0.58 20.7/0.57 9.9/0.64
14 Denys-Drash syndrome M 4 15.4 2011 Anti-hypertensive drug; PD ABO-incompatible 76.7/9.33 88/6.92 20.5/0.66
15 ANCA-associated RPGN F 7 7 2001 MP; CYC Refractory 117/2.3 20.8/1.6 23/1.3
16 Atypical HUS F 10 10 2008 CRRT Atypical HUS 87.1/6.3 24.5/2.5 8.8/0.7

Abbreviations: ANCA, anti-neutrophil cytoplasmic antibodies; CRRT, continuous renal replacement therapy; CsA, cyclosporine; CYC, cyclophosphamide; FSGS, focal segmental glomerulosclerosis; HUS, hemolytic-uremic syndrome; KT, kidney transplantation; F, female; M, male; MP, methylprednisolone; NA, not available; P, oral steroid; PD, peritoneal dialysis; RPGN, rapidly progressive glomerulonephritis; TPE, therapeutic plasma exchange; TTP, thrombotic thrombocytopenic purpura; BUN, blood urea nitrogen; Cr, serum creatinine.

Table 2.
Therapeutic Effects of Lupus Nephritis Patients who Underwent TPE
Patient Diagnosis Sex Age (year) Reason for TPE Pre-TPE Urine Protein (positive on stick) One month f/u Urine Protein (postive on stick) Pre-TPE C3/C4 (mg/dL) 1 month f/u C3/C4 (mg/dL) Pre-TPE Anti-DNA Ab titer 1 month f/u Anti-DNA Ab titer
1 Lupus nephritis F 15 Refractory 3+ 1+ 42/8 74/13 1:160, + 1:160, +
2 Lupus nephritis F 12 Refractory - - 41/6 64/7 1:160, + 1:40, +
3 Lupus nephritis F 15 Refractory 3+ 2+ 37/4 38/5 1:40, + 1:40, +
4 Lupus nephritis F 14 Refractory - - 35/12 27/7 1:160, + 1:160, +
5 Lupus nephritis M 12 TTP 3+ 3+ 16/12 72/21.4 1:160, + -
6 Lupus nephritis F 14 Refractory 3+ 3+ 90/19 NA - -
7 Lupus nephritis F 8 Refractory NA NA NA NA - NA
8 Lupus nephritis F 11 Refractory 3+ 1+ 20/1.4 30/2.4 - -
9-1 Lupus nephritis F 13 Refractory 3+ - 47/4.6 53/11 1:40, + -
9-2 Lupus nephritis 18 Rejection - - 75/8.2 NA NA NA

C3 reference range (90-180 mg/dL), C4 reference range (10-40 mg/dL).

Abbreviations: F, female; M, male; NA, not available; TPE, therapeutic plasma exchange; TTP, thrombotic thrombocytopenic purpura.

Table 3.
Therapeutic Effects of FSGS Patients who Underwent TPE
Patient Diagnosis Sex Age (year) Reason for TPE Pre-TPE Urine Protein (postive on stick) One month f/u Urine Protein (postive on stick) Pre-TPE Albumin (g/dL) 1 month f/u Albumin (g/dL)
10-1 FSGS F 10 Recur 3+ 2+ 2.8 3.5
10-2 12 Refractory 2+ - 3.9 4.1
11-1 FSGS F 3 Refractory 3+ 3+ 1.6 2.8
11-2 14 Recur 3+ 3+ 1.8 2.7
12 FSGS F 16 Refractory 3+ 3+ 1.1 2.4
13 FSGS F 7 TTP - - 4.4 2.9

Abbreviations: F, female; FSGS, focal segmental glomerulosclerosis; NA, not available; TPE, therapeutic plasma exchange; TTP, thrombotic thrombocytopenic purpura.

Table 4.
Laboratory Findings before and after TPE (at 1 Week and 1 Month)
Before TPE After 1 week* P-value After 1 month P-value
BUN (mg/dL) 35.4 (17-49.5) 21.5 (15.1-30.0) 0.02 20.5 (11.0-24.7) 0.01
Creatinine (mg/dL) 1.20 (0.65-2.28) 0.90 (0.60-1.60) 0.02 0.70 (0.65-0.90) 0.11

Abbreviations: TPE, therapeutic plasma exchange; BUN, blood urea nitrogen.

* 1 week immediately after TPE.

1 month immediately after TPE.

Table 5.
Laboratory Findings and Proteinuria before and after TPE in Lupus Nephritis Patients
Before TPE After 1 month* P-value
C3 (mg/dL) 34±11.6 51.1±19.7 0.082
C4 (mg/dL) 6.9±4.0 9.5±6.3 0.18
Proteinuria (postive on stick) 2+ 1+ 0.052

* 1 month immediately after TPE.

Abbreviations: TPE, therapeutic plasma exchange;BUN, blood urea nitrogen; Cr, serum creatinine.

Table 6.
Laboratory Findings and Proteinuria before and after TPE in FSGS Patients
Before TPE After 1 month* P-value
Albumin (g/dL) 2.6±1.3 3.1±0.6 0.322
Proteinuria (postive on stick) 2+ 2+ 0.203

* 1 month immediately after TPE.

Abbreviations: FSGS, focal segmental glomerulosclerosis; TPE, therapeutic plasma exchange.

Table 7.
TPE Procedure and Complications
Patient Diagnosis Number of sessions Replacement solution Vascular access Complication
1 Lupus nephritis 3 4% albumin Subclavian vein -
2 Lupus nephritis 3 4% albumin Subclavian vein -
3 Lupus nephritis 5 NA NA -
4 Lupus nephritis 5 NA Internal J. V. Hypovolemia
5 Lupus nephritis 3 4% albumin Internal J. V. -
6 Lupus nephritis 3 NA NA -
7 Lupus nephritis 3 4% albumin NA -
8 Lupus nephritis 6 NA NA -
9-1 Lupus nephritis 10 FFP Internal J. V. -
9-2 5 4% albumin Subclavian vein -
10-1 FSGS 15 4% albumin Subclavian vein Anemia
10-2 5 FFP Subclavian vein -
11-1 FSGS 14 4% albumin Subclavian vein -
11-2 3 FFP Subclavian vein Hypocalcemia
12 FSGS 9 4% albumin Internal J. V. -
13 FSGS 3 FFP Femoral vein -
14 Denys-Drash syndrome 2 4% albumin Internal J. V. -
15 Microscopic polyarteritis 4 NA NA -
16 Atypical HUS 3 FFP Subclavian vein Thrombocytopenia

Abbreviations: FFP, fresh frozen plasma; FSGS, focal segmental glomerulosclerosis; HUS, hemolytic-uremic syndrome; Internal J. V., internal jugular vein; NA, not available.

Table 8.
Comparisons of Our Study with Previous Studies Regarding Therapeutic Effects of TPE
Paglialonga, et al [8] Reddy, et al [15] Ours
Publication year 2015 (Europe) 2015 (India) - (Korea)
Study period 2012 2009-2013 1994-2016
No. of Patients 67 (M 29/F 38) 16 (M 10/F 6) 16 (M 2/F 14)
Age ≤18 yrs 5-18 yrs ≤18 yrs
Etiologies HUS/TTP 17.9% aHUS 75% Lupus nephritis 56%
FSGS 16.4% anti-GBM 12.5% FSGS 25%
Desensitization of rTx 13.4% Lupus nephritis 6.25% Desensitization of rTx 6.3%
MPA 6.25%
No. of procedurs 6 11 5.2
Replacement fluid Albumin (46.2%) NA Albumin (50%)
Plasma (31.3%) FFP (25%)
Albumin+plasma (11.9%)
Complications Total 6.9 % Cath.related inf. 18.8% Total 3.8 %
Allergic reaction 12.5%

Abbreviations: aHUS, atypical hemolytic uremic syndrome; anti-GBM, anti-glomerular basement membrane disease; F, female; FFP, fresh frozen plasma; M, male; MPA, microscopic polyangiitis; No., number; rTx, renal transplantation; FSGS, focal segmental glomerulosclerosis; ;TTP, thrombotic thrombocytopenic purpura; NA, not available.

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