LAPAROSCOPIC PERITONEAL DIALYSIS CATHETER PLACEMENT WITH RECTUS SHEATH TUNNELING: A ONE-PORT SIMPLIFIED TECHNIQUE.

BACKGROUND: The success of peritoneal dialysis depends on the proper placement and functional longevity of the dialysis catheter. Laparoscopic implantation of a catheter through a rectus sheath tunneling can minimize the risks of catheter failure. AIMS: This study aims to describe one-port simplified technique for laparoscopic placement of a peritoneal dialysis catheter with rectus sheath tunneling. METHODS: The simplified laparoscopic insertion of a Tenckhoff catheter with rectus sheath tunneling was performed in 16 patients with chronic renal failure. RESULTS: During the follow-up period, no major complications occurred. Three patients were excluded. One was referred to the renal transplant some weeks after implantation, and one died for other reasons during the follow-up. Another patient needed adhesiolysis due to previous surgery, so an additional port was necessary. The other 13 catheters worked properly, and no postoperative hemorrhage, early leaks, hernia, or catheter migration occurred. One patient had a tunnel infection 11 months after the implant. No peritonitis was observed during the follow-up. CONCLUSIONS: The technique is simple, reproducible, and safe, with good results in catheter function, few complications, and a high catheter survival rate. It does not require a special device or trocar and avoids excessive port sites.

Growth Differentiation Factor-15 and Syndecan-1 Are Potential Biomarkers of Cardiac and Renal Involvement in Classical Fabry Disease under Enzyme Replacement Therapy.

BACKGROUND AND AIMS: Inflammation and endothelial damage play a pivotal role in Fabry disease (FD) manifestations. In daily clinical practice, FD is mainly monitored by traditional biomarkers of target organ injury, such as serum creatinine and proteinuria, which provide no information about inflammation and endothelial damage. MATERIALS AND METHODS: We investigated the serum levels of 3-nitrotyrosine (3-NT), an oxidative stress biomarker, and of growth differentiation factor-15 (GDF-15) and syndecan-1 in classical FD patients on enzyme replacement therapy (ERT) for at least 6 months and their relationship with Fabry-related cardiac and renal manifestations. RESULTS: Fifty-two classical FD patients (37 females) on ERT for 62.0 ± 27.5 months were included in the study. The main clinical manifestations included nephropathy (67.3%) and cardiomyopathy (21.1%). Serum levels of 3-NT, syndecan-1, and GDF-15 were 33.3 (4.8-111.1) nmol/mL, 55.7 (38.8-74.9) ng/mL, and 541.8 (392.2-784.4) pg/mL, respectively. There was a direct correlation between interventricular septal thickness and serum GDF-15 (r = 0.59; p < 0.001) and syndecan-1 (r = 0.30, p = 0.04). Among kidney parameters, there was a significant correlation between estimated glomerular filtration rate and GDF-15 (r = -0.61; p < 0.001), as well as between 24 h proteinuria and syndecan-1 (r = 0.28; p = 0.04). Serum GDF-15 levels were significantly higher in patients with cardiomyopathy (p = 0.03) as well in those with both nephropathy and cardiomyopathy (p = 0.02) than in patients without these comorbidities. Serum GDF-15 levels were also significantly higher in patients who started ERT at an older age (≥40 years). In multivariate analysis, syndecan-1, 3-NT, GDF-15, time on ERT, and arterial pressure differentiated Fabry patients with both cardiac and renal involvement from those without these manifestations. CONCLUSIONS: GDF-15 and syndecan-1 were associated with parameters of cardiac and renal involvement in classic FD patients on ERT. Their potential association with residual risk and disease outcomes should be investigated.

Laparoscopic peritoneal dialysis catheter placement with rectus sheath tunneling: a one-port simplified technique / Implante laparoscópico de cateter de diálise peritoneal com tunelização na bainha do músculo reto abdominal: técnica simplificada com portal único

ABSTRACT - BACKGROUND: The success of peritoneal dialysis depends on the proper placement and functional longevity of the dialysis catheter. Laparoscopic implantation of a catheter through a rectus sheath tunneling can minimize the risks of catheter failure. AIMS: This study aims to describe one-port simplified technique for laparoscopic placement of a peritoneal dialysis catheter with rectus sheath tunneling. METHODS: The simplified laparoscopic insertion of a Tenckhoff catheter with rectus sheath tunneling was performed in 16 patients with chronic renal failure. RESULTS: During the follow-up period, no major complications occurred. Three patients were excluded. One was referred to the renal transplant some weeks after implantation, and one died for other reasons during the follow-up. Another patient needed adhesiolysis due to previous surgery, so an additional port was necessary. The other 13 catheters worked properly, and no postoperative hemorrhage, early leaks, hernia, or catheter migration occurred. One patient had a tunnel infection 11 months after the implant. No peritonitis was observed during the follow-up. CONCLUSIONS: The technique is simple, reproducible, and safe, with good results in catheter function, few complications, and a high catheter survival rate. It does not require a special device or trocar and avoids excessive port sites.

RESUMO - RACIONAL: O sucesso da diálise peritoneal depende da implantação adequada e da longevidade funcional do cateter. O implante laparoscópico através da tunelização na bainha do reto abdominal minimiza os riscos de disfunção do cateter. OBJETIVOS: Descrever técnica simplificada com portal único para o implante laparoscópico de cateter de diálise peritoneal com tunelização na bainha do reto abdominal. MÉTODOS: Utilizou-se inserção laparoscópica de cateter de Tenckhoff com tunelização da bainha do reto em 16 pacientes com insuficiência renal crônica. RESULTADOS: Durante o período de acompanhamento não ocorreram complicações relacionadas ao procedimento. Três pacientes foram excluídos: um foi encaminhado para transplante renal algumas semanas após o implante e outro faleceu por outro motivo durante o acompanhamento. Um terceiro necessitou de lise de aderências devido à operação anterior, portanto foi necessário um portal adicional. Os outros 13 pacientes apresentaram bom funcionamento do cateter. Não houve hemorragia pós-operatória, vazamentos, hérnia ou migração do cateter. Um paciente teve infecção no túnel subcutâneo 11 meses após o implante. Não foi observada peritonite durante o período de acompanhamento. CONCLUSÕES: A técnica é simples, reprodutível, segura, com bons resultados de funcionalidade, poucas complicações e alta taxa de sobrevida do cateter. Ela não requer trocarte especial e evita o uso excessivo de portais.

The Diagnosis of Protein Energy Wasting in Chronic Peritoneal Dialysis Patients Is Influenced by the Method of Calculating Muscle Mass. A Prospective, Multicenter Study.

Objective: To analyze the concordance and agreement between bioimpedance spectroscopy (BIS) and anthropometry for the diagnosis of protein energy wasting (PEW) in chronic peritoneal dialysis patients. Methods: Prospective, multi-center, observational study using multifrequency bioimpedance device (Body Composition Monitor -BCM ® - Fresenius Medical Care) and anthropometry for the diagnosis of PEW as recommended by the International Society of Renal Nutrition and Metabolism (ISRNM). Cohen's kappa was the main test used to analyze concordance and a Bland-Altmann curve was built to evaluate the agreement between both methods. Results: We included 137 patients from three PD clinics. The mean age of the study population was 57.7 ± 14.9, 47.8% had diabetes, and 52.2% were male. We calculated the scores for PEW diagnosis at 3 and 6 months after the first collection (T3 and T6) and on average 40% of the study population were diagnosed with PEW. The concordance in the diagnosis of PEW was only moderate between anthropometry and BIS at both T3 and T6. The main factor responsible for our results was a low to moderate correlation for muscle mass in kilograms, with an r-squared (R2) of 0.35. The agreement was poor, with a difference of more than 10 kg of muscle mass on average and with more than a quarter of all cases beyond the limits of agreements. Conclusion: Current diagnosis of PEW may differ depending on the tools used to measure muscle mass in peritoneal dialysis patients.

Chloroquine may induce endothelial injury through lysosomal dysfunction and oxidative stress.

COVID-19 is a pandemic with no end in sight. There is only one approved antiviral agent but global stocks are deemed insufficient. Despite in vitro antiviral activity, clinical trials of chloroquine and hydroxychloroquine were disappointing, and they may even impair outcomes. Chloroquine causes zebroid deposits reminiscent of Fabry disease (α-galactosidase A deficiency) and endothelial cells are key targets of COVID-19. We have explored the effect of chloroquine on cultured endothelial cells and its modulation by recombinant α-galactosidase A (agalsidase). Following dose-response studies, 0.5 µg/mL chloroquine was added to cultured human endothelial cells. Neutral red and Lysotracker were used to assess lysosomes. Cytotoxicity was evaluated by the 3-(4, 5-dimethylthiazol-2-yl)-2, 5-diphenyltetrazolium bromide) - MTT assay and cell stress by assessing reactive oxygen species (ROS) and nitric oxide (NO). In endothelial cells, chloroquine induced dose-dependent cytotoxicity at in vitro test concentrations for COVID-19 therapy. At a sublethal concentration, chloroquine significantly induced the accumulation of acid organelles (P < 0.05), increased ROS levels, and decreased NO production (P < 0.05). These adverse effects of chloroquine on endothelial cell biology were decreased by agalsidase-ß (P < 0.05). Chloroquine-induced endothelial cell cytotoxicity and stress is attenuated by agalsidase-ß treatment. This suggests that endothelial cell injury may contribute to the failure of chloroquine as therapy for COVID-19 and may be at least in part related to causing dysfunction of the lysosomal enzyme α-galactosidase A.

Case report: is low α-Gal enzyme activity sufficient to establish the diagnosis of Fabry disease?

Fabry disease is an X-linked lysosomal storage disease due to alpha-galactosidase A (α-Gal A) deficient activity which leads to the accumulation of glucoesphingolipids, such as globotriaosilceramide. There are over 700 known mutations of the enzyme gene, and most of them cause Fabry Disease. This case report describes a hemodialysis patient with a rare and controversial GLA gene mutation, the D313Y. The medecial investigation confirmed that D313Y is an alpha-galactosidase A sequence variant that causes pseudo deficient enzyme activity in plasma but not Fabry disease. Thus, clinical symptoms that prompted Fabry disease investigation could not be attributable to Fabry disease and therefore enzyme replacement therapy was not indicated.

Case report: is low α-Gal enzyme activity sufficient to establish the diagnosis of Fabry disease? / Relato de caso: a diminuição da enzimaα-Gal l é suficiente para estabelecer o diagnóstico?

Abstract Fabry disease is an X-linked lysosomal storage disease due to alpha-galactosidase A (α-Gal A) deficient activity which leads to the accumulation of glucoesphingolipids, such as globotriaosilceramide. There are over 700 known mutations of the enzyme gene, and most of them cause Fabry Disease. This case report describes a hemodialysis patient with a rare and controversial GLA gene mutation, the D313Y. The medecial investigation confirmed that D313Y is an alpha-galactosidase A sequence variant that causes pseudo deficient enzyme activity in plasma but not Fabry disease. Thus, clinical symptoms that prompted Fabry disease investigation could not be attributable to Fabry disease and therefore enzyme replacement therapy was not indicated.

Resumo Doença de Fabry (DF) é uma doença de depósito lisossômico ligada ao cromossomo X, causada pela deficiência da enzima alfa-galactosidase A (α-Gal A) que leva ao acúmulo de glicoesfingolipídeos, principalmente globotriaosilceramide. Existem mais de 700 mutações conhecidas do gene da enzima, a maioria delas são causadoras de DF. Este relato de caso descreve sobre um paciente em hemodiálise com uma mutação do gene GLA rara e controversa, a D313Y. A investigação médica confirmou que D313Y é uma variante que leva à pseudodeficiência plasmática da enzima, mas não ocasiona DF. Assim, os sintomas clínicos que induziram a investigação da doença não devem ser atribuídos à DF e, portanto, não foi indicada a terapia de reposição enzimática.