Comparison of The Peritoneal Dialysis and Continuous Veno-Venous Hemodiafiltration in 16 Neonates With Maple Syrup Urine Disease

Abstract Introduction: Continuous Renal Replacement Therapy (CRRT) is a well-known treatment modality for patients with acute renal failure and has been increasingly used for the treatment of metabolic disorders such as Maple Syrup Urine Disease (MSUD) in recent years. Herein, we aimed to discuss our experience in 16 newborn patients with MSUD who were treated with urgent renal replacement therapy (RRT).Materials and Method: The data of patients who presented with an acute metabolic crisis due to Maple syrup urine disease and who were treated with RRT at Neonatal intensive care unit(NICU) between November 2016 and March 2020 were retrospectively evaluated. The patients underwent continuous veno-venous hemodiafiltration (CVVHDF) or peritoneal dialysis (PD) as renal replacement therapy.Results: The study enrolled a total of 16 patients, of which 8 were male and 8 were female. Eleven (68.75%) patients underwent CVVHDF and five (31.25%) underwent peritoneal dialysis. The median post-treatment leucine level was 198(20-721) μmol/L in the CVVHDF group and 1050(303-1653) μmol/L in the PD group; the median leucine reduction rate per hour was 2.56% (1.75-7.6) in the CVVHDF group and 0.78% (0.54-1.83) in the PD group. There was a significant difference between both groups regarding both parameters (p= 0.08, p=0.001, respectively). Complications such as hypotension, electrolyte imbalance, and filter obstruction occurred in the CVVHDF group while catheter revision was needed due to catheter obstruction in one patient in the PD group.Conclusion: This study showed that CVVHDF is more effective than PD for rapidly eliminating elevated Leucine levels caused by MSUD in the newborn and it is not associated with increased complication rates.

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MO696INCREMENTAL PERITONEAL DIALYSIS: BENEFICIAL EFFECTS

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfab101.0018 ◽

2021 ◽

Vol 36 (Supplement_1) ◽

Author(s):

Adriana Fernandes ◽

João Carvão ◽

Rita Veríssimo ◽

Patrícia Branco ◽

Patricia Matias ◽

...

Keyword(s):

Peritoneal Dialysis ◽

Renal Replacement Therapy ◽

Replacement Therapy ◽

Hospital Admissions ◽

Angiotensin Receptor Blockers ◽

Cardiac Insufficiency ◽

Full Dose ◽

Significant Difference ◽

Group A ◽

Group B

Abstract Background and Aims Incremental peritoneal dialysis (Incr_Dial) is a renal replacement therapy strategy based on lower dose prescription rather than the standard “full dose” (Full_Dial). Individualized clearance goals were achieved combining residual renal function (RRF) and peritoneal clearance. Maintaining RRF is a crucial issue to peritoneal dialysis (PD) patients and the best PD strategy to preserve it is subject to debate. The aim of this study is to compare Incr_Dial and Full_Dial in terms of RRF preservation and other clinical outcomes. Method This was a single-center, retrospective descriptive study. We included a cohort of incident and prevalent adult PD patients in the PD Unit between January – December of 2020. Patients without a follow-up < six months and who started PD at another center were excluded. Patients were assigned according to their first PD protocol in two groups – Group A: Incr_Dial protocol (continuous ambulatory PD: less than four dwells daily, less than 2 L dwell volumes, less than seven days a week treatment, or some combination of these; automated PD: without a long dwell, less than 10 L daily delivered by cycler and day dwells, treatment for less than 7 days a week or some combination of these); Group B: Full_Dial protocol. Statistical analysis was performed using SPSS 20.0. Statistical significance level p <0.05. Results Among 84 PD patients, 68% underwent incremental PD (Group A) and 31% underwent conventional full-dose PD (Group B). The mean age was 55.9±15.4 years, 60.7% were male and 32.5% diabetic. There were no statistically significant differences between the two groups regarding: demographic characteristics, comorbidities (diabetes, hypertension, cardiac insufficiency or ischemic heart disease) and drugs (angiotensin-converting enzyme inhibitors, angiotensin receptor blockers and/or loop diuretics). The median Incr_Dial duration to achieve PD full dose was 10.2 months (IQ 5.1-17). At the beginning of PD, there was no statistically significant difference in diuresis between both groups (A: 1.79L vs B: 1.2L, p=0.07), however after 6 months of PD there was a superior urinary output in Group A (A: 1.67L vs B: 1.1L, p=0.037). Group A (Incr_Dial) was also associated with a superior renal clearance of creatinine at the beginning (A: 81 L/sem/1.73m2 vs B: 56.8L/sem/1.73m2, p=0.021) and after 12 months (A: 70.7L/sem/1.73m2 vs B: 26.3 L/sem/1.73m2, p<0.01); and superior Kt/V renal/week at the beginning (A: 1.48 vs B: 1.02, p=0.02) and after 12 months (A: 1.28 vs B: 0.49, p<0.001). There are no differences between mortality (A: 1.9% vs B: 12%, p=0.094), peritonitis-free time (A: 158 days vs B: 236 days, p=0.133) and the numbers of peritonitis per year (A: 0.32 vs B: 0.5, p=0.940). However, the rate of hospital admissions per year was lower in Group A (A: 0.22 vs B: 0.70, p=0.001). Conclusion Incremental PD is a safe strategy of renal replacement therapy to start PD. In our PD population, it showed similar patient survival rates and a significantly less hospital admissions. Incremental PD was also beneficial for preserving RRF when compared to full-dose PD.

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In Vitro Closure Times (PFA-100) Are Different Between Peritoneal Dialysis and Hemodialysis

Hämostaseologie ◽

10.1055/a-1171-0499 ◽

2020 ◽

Vol 40 (05) ◽

pp. 671-678

Author(s):

Cenk Gokalp ◽

Fatma Keklik Karadag ◽

Matthias Christoph Braunisch ◽

Christoph Schmaderer ◽

Emrah Gunay ◽

...

Keyword(s):

Peritoneal Dialysis ◽

Renal Replacement Therapy ◽

Replacement Therapy ◽

Adenosine Diphosphate ◽

Significant Difference ◽

Function Analyzer ◽

The Difference ◽

Stage Renal Disease ◽

End Stage

Abstract Introduction Platelet dysfunction is not uncommon in patients with end-stage renal disease (ESRD). Type of renal replacement therapy may have an effect on platelet functions, which has not been well investigated. We evaluated in vitro closure time (CT) differences between peritoneal dialysis (PD) and hemodialysis (HD) patients using platelet function analyzer (PFA-100)and observed a significant difference between these renal replacement therapies. Methods Patients with ESRD undergoing PD (n = 24) or HD (n = 23) for more than 6 months were included. Blood samples for collagen/epinephrine (Col/EPI) and collagen/adenosine diphosphate (Col/ADP) measurements were obtained before HD at a mid-week session for HD patients and at an outpatient control time for PD patients. Results Three of 24 (12.5%) PD patients and 16 of 23 (69.5%) HD patients had prolonged PFA-100 Col/EPI, p< 0.001. Likewise, 4.2% of PD patients and 87.0% of HD patients had prolonged PFA-100 Col/ADP, p< 0.001. Moreover, the median times of PFA-Col/EPI and PFA-100 Col/ADP were significantly lower in PD patients compared with those of HD patients (p< 0.001). Multivariate analysis showed that the type of renal replacement was a risk factor for both elevated PFA-100 Col/ADP and PFA-100 Col/EPI after adjusted for platelets, hematocrit, and urea (p< 0.001). Conclusions The type of renal replacement therapy may have an effect on in vitro CTs; therefore, studies including more patients with long-term follow-up are needed to investigate if the difference has any impact on clinical outcomes.

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Pathogenesis of maple-syrup-urine disease: observations during dietary management and treatment of coma by peritoneal dialysis

Biochemical Medicine ◽

10.1016/0006-2944(69)90005-2 ◽

1969 ◽

Vol 3 (2) ◽

pp. 130-149 ◽

Cited By ~ 20

Author(s):

Gerald E. Gaull

Keyword(s):

Peritoneal Dialysis ◽

Maple Syrup Urine Disease ◽

Dietary Management ◽

Maple Syrup ◽

Urine Disease

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Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids

European Journal of Pediatrics ◽

10.1007/bf00442404 ◽

1980 ◽

Vol 134 (1) ◽

pp. 57-63 ◽

Cited By ~ 22

Author(s):

U. Wendel ◽

K. Becker ◽

Hildegard Przyrembel ◽

Monika Bulla ◽

C. Manegold ◽

...

Keyword(s):

Peritoneal Dialysis ◽

Maple Syrup Urine Disease ◽

Maple Syrup ◽

Keto Acids ◽

Urine Disease ◽

Branched Chain

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Impact of sodium phenylbutyrate treatment in acute management of maple syrup urine disease attacks: a single-center experience

Journal of Pediatric Endocrinology and Metabolism ◽

10.1515/jpem-2020-0356 ◽

2020 ◽

Vol 0 (0) ◽

Author(s):

Tanyel Zubarioglu ◽

Elif Dede ◽

Humeyra Cigdem ◽

Ertugrul Kiykim ◽

Mehmet S. Cansever ◽

...

Keyword(s):

Maple Syrup Urine Disease ◽

Reduction Rate ◽

Clinical Findings ◽

Metabolic Decompensation ◽

Acute Management ◽

Maple Syrup ◽

Toxin Removal ◽

Urine Disease ◽

Acute Attacks ◽

Sodium Phenylbutyrate

AbstractObjectivesAccurate management of metabolic decompensation in maple syrup urine disease (MSUD) has a crucial role, as acute attacks can cause neurological sequels and can be life threatening. Here, we aimed to evaluate effect of sodium phenylbutyrate (NaPBA) in acute management of MSUD attacks.MethodsEpisodes with an initial plasma leucine (Leu) level above 750 µmoL/L and that require hospitalization due to clinical findings of Leu neurotoxicity and/or feeding difficulties were included to the study. Patients who had no molecular diagnosis and a regular follow-up were excluded. Clinical findings, laboratory results and therapy responses were reviewed, retrospectively.ResultsTen patients who experienced 19 distinct episodes of MSUD attacks were enrolled. Initial median Leu level was 901.67 (range 756–1989.11) and 33.9 µmoL/L (range 7.91–347.3 µmoL/L) at the end of therapy. None of our patients underwent extracorporeal toxin removal during the course of attack. In patients with serial plasma quantitative amino acid sampling, mean Leu reduction rate was calculated to be 529.68 ± 250.08 µmoL/L/day at the 24th h of treatment and 318.72 ± 191.52 µmoL/L/day at the 48th h of treatment.ConclusionsThis study is the first original study that investigates the effect of NaPBA in management of acute attacks of MSUD patients from Turkey. We suggest that NaPBA treatment in MSUD attacks can ameliorate clinical and biochemical findings. This therapeutic option should be considered especially in smaller centers without the toxin removal chance and for patients who were not appropriate for extracorporeal toxin removal like hemodynamic instability.

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