Scielo RSS http://scielo.pt/rss.php?pid=0872-016920220003&lang=en vol. 36 num. 3 lang. en http://scielo.pt/img/en/fbpelogp.gif http://scielo.pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000300127&lng=en&nrm=iso&tlng=en http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000300130&lng=en&nrm=iso&tlng=en ABSTRACT Acute kidney injury (AKI) is frequent in critically ill neonates, children, and adolescents, but it is still underdiagnosed and referral to follow-up at the Pediatric Nephrology Clinic is scant. Nowadays, most pediatric studies use Kidney Disease Improving Global Outcomes (KDIGO) criteria for AKI diagnosis. These criteria are based on the elevation of serum creatinine levels and on the decrease in urine output and they correlate with a higher need for kidney replacement therapy and a higher risk of death. Nonetheless, the KDIGO definition of AKI has several limitations, which may be overcome by the simultaneous use of novel biomarkers, particularly urinary neutrophil gelatinase-associated lipocalin (NGAL). The latter is already available in clinical practice and it is a useful tool to identify AKI earlier and to establish the prognosis. The most common AKI etiologies in the pediatric population are the prerenal causes, namely kidney hypoperfusion due to hypovolemia and peripheral vasodilation in the context of sepsis. Exposure to nephrotoxic drugs has been rising as a primary cause of AKI. Decreasing the use of nephrotoxic drugs whenever clinically possible, monitoring the serum levels of these medications, and adjusting its doses can significantly reduce AKI incidence. Finally, it is important to recognize that AKI is not a completely reversible phenomenon and long-term sequelae are seen in up to 70% of affected children. These sequelae include progression to chronic kidney disease, which accentuates the need for follow-up after an AKI episode. In conclusion, it is essential to improve awareness in the Pediatric community for AKI in order to prevent it, rapidly identify it and establish reno-protective measures, which will improve its long-term prognosis. This review will focus on current definitions, epidemiology and most common etiologies, and it will also discuss strategies to improve pediatric AKI prevention, early diagnosis and follow-up. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000300138&lng=en&nrm=iso&tlng=en ABSTRACT Renal replacement therapies are frequently used in the intensive care unit, most often in patients with severe acute kidney injury, a common complication in this setting. The choice of a prolonged intermittent renal replacement therapy (PIRRT) has grown in its popularity. Nevertheless, not all clinicians are familiar with it. The most frequently performed PIRRT is sustained low-efficiency dialysis. The aim of this article is to review the sustained low-efficiency dialysis technique, namely the necessary machinery, the prescription specificities, its complications and the particularities related to intoxications and drug dosing. Lastly, when to start sustained low-efficiency dialysis and its advantages and disadvantages over intermittent hemodialysis and continuous renal replacement therapies is discussed. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000300148&lng=en&nrm=iso&tlng=en ABSTRACT Accidental or intentional poisoning and drug overdose are a significant source of morbidity, mortality and health care expenditure worldwide. Extracorporeal removal treatments have been used to treat poisoning for decades and different modalities are available, including hemodialysis, hemofiltration, hemoperfusion, continuous renal replacement therapy and therapeutic plasma exchange. A comprehensive understanding of their purpose is key to choose the right modality for each clinical scenario. Lithium, salicylates, metformin and toxic alcohols are amongst the most common poisons treated with extracorporeal removal. Unfortunately, due to poison characteristics and specific modality limitations, extracorporeal treatments are useful to treat only a small number of poisons. Nevertheless, they have been increasingly used for supportive care in poisoning caused by substances not amenable for removal. Evidence regarding extracorporeal treatment in poisoning is modest. However, its use has been systematically reviewed in several poisons within the last decade by the Extracorporeal Treatment in Poisoning workgroup. The preferred treatment for extracorporeal removal of poisons is intermittent hemodialysis, according to the most recent guidelines and case reports available, as it effectively removes most of the common substances involved in poisoning and corrects electrolytes and acid base imbalances. This narrative review gives an overview of the available extracorporeal modalities used for poisoned patients, reflecting on their main indications and limitations as well as a practical view on the management of the most common poisons found in clinical practice. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000300156&lng=en&nrm=iso&tlng=en ABSTRACT Introduction: Chronic kidney disease (CKD)-mineral and bone disorder (MBD) is a major complication in hemodialysis (HD) patients. Serum intact parathyroid hormone (iPTH) has been associated with prognosis in these patients, however, the optimal range to reduce mortality remains unknown. Methods: We conducted a retrospective study of incident HD patients, who were categorized into 4 groups according to iPTH serum level: <150 pg/mL, 150-300 pg/mL, 301-600 pg/mL and >600 pg/mL. All-cause and cardiovascular mortality over a mean follow-up of 3 years was assessed using standard survival methods. Results: One hundred and forty-nine patients were included. Patients with low iPTH presented low serum albumin, phosphorus, and bonespecific alkaline phosphatase (BAP), increased c-reactive protein (CRP) and higher serum bicarbonate (p<0.05). Those with iPTH <150 pg/mL had an increased risk of all-cause and cardiovascular mortality, when compared to those with iPTH 301-600 pg/mL (HR: 0.59; 95% CI: 0.36-0.96; p=0.035) and iPTH >600 pg/mL (HR: 0.39; 95% CI: 0.20-0.78; p=0.008), even after adjusting for potential confounding factors as age, albumin, and comorbidities (diabetes, congestive heart failure (CHF), and hypertension). Discussion: PTH levels were associated with all-cause and cardiovascular mortality risk in our cohort of incident HD patients. Our results suggest that patients with low iPTH at HD initiation had an increased mortality risk, which may reflect a frail group of patients with anorexia, sarcopenia, malnutrition, and inflammation. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000300161&lng=en&nrm=iso&tlng=en ABSTRACT Introduction: Central vein catheterization (CVC) is one of the main risk factors for central vein stenosis (CVS) occurrence in the hemodialysis population. In this work we aimed to evaluate previous central venous manipulation, clinical presentation, and complications in hemodialysis patients with proved CVS. Material and Methods: We retrospectively reviewed all venous angiographies in patients attending our public hospital center for hemodialysis treatment from 2013 to 2018 (n=209). In patients with confirmed CVS (n=29, 14%), we evaluated history of prior CVC and cardiac rhythm devices (CDR), as well as demographics and cardiovascular risk factors. Results: We identified 31 CVS in 29 patients who underwent venous angiography. Most of these patients, 75.9% (n=22), had at least one CVC (ipsilateral or not); at least one previous ipsilateral short-term CVC was found in 34.4% (n=10) of patients and at least one previous ipsilateral long-term CVC was found in 41.4% (n=12) of patients. All patients who had a prior CRD (n=4, 13.8%), had developed an ipsilateral CVS. Conclusion: Prevalence of detected CVS in our population was significant and is likely underestimated. A high percentage of patients with CVS had an history of previous ipsilateral CVC. The number of patients with previous short-term CVC was similar to that of previous long-term CVC, hinting an important role of short-term CVC as a risk factor do CVS. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000300165&lng=en&nrm=iso&tlng=en ABSTRACT In Portugal, all expenses related to the replacement treatment of renal function in chronic kidney disease are supported through the budget of the Ministry of Health. The author, who limits the analysis to the modality of dialysis treatment, reviews the relative importance of the two factors that have the greatest influence on these expenses. The so-called comprehensive price (CP) which includes dialysis, medicines and diagnostic tests - and the number of patients undergoing treatment (prevalence). The CP with a very high initial value was progressively reduced, with these corrections contributing positively to a decrease in expenses. On the contrary, the prevalence of dialysis has always been increasing, mainly as a result of an extremely high incidence of new patients starting dialysis. The positive effect of decreasing CP it is not only completely nullified by the opposite effect resulting from the increase in prevalence, but it is even surpassed, with a significant increase in expenses. Everything seems to indicate that the high prevalence of dialysis represents the most important factor responsible for the high level of state expenditure in the treatment of chronic kidney disease in Portugal. In order to contain expenses, it will be necessary not only to correct the CP value again, but to analyze the cause, or causes, responsible for the very high levels of incidence/prevalence in dialysis that are observed in our country. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000300170&lng=en&nrm=iso&tlng=en ABSTRACT X-linked hypophosphatemic rickets is a monogenic disease, characterized by hyperphosphaturia and hypophosphatemia. Due to its rarity and wide phenotypic variability, a diagnostic delay is common in X-linked hypophosphatemic rickets. Short stature, limb deformities, dental anomalies, craniosynostosis and chronic pain are common in this disease. Recently, burosumab, a monoclonal antibody anti-fibroblast growth factor 23, was approved for the treatment of X-linked hypophosphatemic rickets. Awareness among clinicians must be increased to improve the care of these patients. We present a clinical case of a 4-year-old girl presented with deformities of the lower limbs and an abnormal gait, associated with hyperphosphaturia and hypophosphatemia. Asymptomatic Arnold Chiari malformation was identified. No dental problems were detected. The diagnosis of X-linked hypophosphatemic rickets was confirmed by the identification of a PHEX mutation. The patient developed diarrhea, nephrocalcinosis, and hyperparathyroidism secondary to conventional therapy with phosphate supplements. Burosumab was initiated with a fast increase on serum phosphate levels and a decrease on alkaline phosphatase. With the description of this case, we highlight the clinical manifestations and complications of X-linked hypophosphatemic rickets and its treatment and we discuss new treatment strategies to improve the quality of life of these patients. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000300180&lng=en&nrm=iso&tlng=en ABSTRACT Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis which mortality can reach 50%. The usual treatment involves suspension of the technique, medical treatment, and surgical enterolysis. We report the case of a 36-year-old female who undergone continuous ambulatory peritoneal dialysis without complications for 10 years. She presented at the emergency department with abdominal pain and a diagnosis of peritonitis was assumed. The patient developed a systemic inflammatory response, and her abdominal computer tomography (CT) scan showed multiple parietal calcifications. After multiple broad-spectrum antibiotics, an undetermined febrile syndrome persisted, and parenteral nutrition was started. An EPS diagnosis was established and treatment with tamoxifen was proposed. Exploratory laparoscopy was decided, and the intraoperative findings were consistent with EPS. The patient was submitted to extensive enterolysis. A clinical improvement led to a discharge after 60 days of hospitalization. After more than 30 months with tamoxifen, she remains on hemodialysis and doing well. There are no randomized controlled trials to guide EPS management and the following procedures are more difficult to decide, who makes these reports important to the reflection. We describe a case of successful treatment of EPS, with a very severe presentation. The management with enterolysis and tamoxifen contributed decisively to this outcome. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000300185&lng=en&nrm=iso&tlng=en ABSTRACT Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis which mortality can reach 50%. The usual treatment involves suspension of the technique, medical treatment, and surgical enterolysis. We report the case of a 36-year-old female who undergone continuous ambulatory peritoneal dialysis without complications for 10 years. She presented at the emergency department with abdominal pain and a diagnosis of peritonitis was assumed. The patient developed a systemic inflammatory response, and her abdominal computer tomography (CT) scan showed multiple parietal calcifications. After multiple broad-spectrum antibiotics, an undetermined febrile syndrome persisted, and parenteral nutrition was started. An EPS diagnosis was established and treatment with tamoxifen was proposed. Exploratory laparoscopy was decided, and the intraoperative findings were consistent with EPS. The patient was submitted to extensive enterolysis. A clinical improvement led to a discharge after 60 days of hospitalization. After more than 30 months with tamoxifen, she remains on hemodialysis and doing well. There are no randomized controlled trials to guide EPS management and the following procedures are more difficult to decide, who makes these reports important to the reflection. We describe a case of successful treatment of EPS, with a very severe presentation. The management with enterolysis and tamoxifen contributed decisively to this outcome. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000300188&lng=en&nrm=iso&tlng=en ABSTRACT Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis which mortality can reach 50%. The usual treatment involves suspension of the technique, medical treatment, and surgical enterolysis. We report the case of a 36-year-old female who undergone continuous ambulatory peritoneal dialysis without complications for 10 years. She presented at the emergency department with abdominal pain and a diagnosis of peritonitis was assumed. The patient developed a systemic inflammatory response, and her abdominal computer tomography (CT) scan showed multiple parietal calcifications. After multiple broad-spectrum antibiotics, an undetermined febrile syndrome persisted, and parenteral nutrition was started. An EPS diagnosis was established and treatment with tamoxifen was proposed. Exploratory laparoscopy was decided, and the intraoperative findings were consistent with EPS. The patient was submitted to extensive enterolysis. A clinical improvement led to a discharge after 60 days of hospitalization. After more than 30 months with tamoxifen, she remains on hemodialysis and doing well. There are no randomized controlled trials to guide EPS management and the following procedures are more difficult to decide, who makes these reports important to the reflection. We describe a case of successful treatment of EPS, with a very severe presentation. The management with enterolysis and tamoxifen contributed decisively to this outcome.