Aquapheresis (AQ) in Tandem with Extracorporeal Membrane Oxygenation (ECMO) in Pediatric Patients.

Children with cardiopulmonary failure requiring extracorporeal membrane oxygenation (ECMO) are at risk for fluid overload (FO) despite the normal estimated glomerular filtration rate (eGFR). It has been shown that survival in the intensive care unit (ICU) is inversely proportional to FO. Therefore, fluid removal, or prevention of FO, in these critical cases has the potential to improve survival. Aquapheresis (AQ), a procedure used for fluid removal, with success in patients with heart failure has also been used in children with acute oliguric kidney injury (AKI), to prevent and treat FO. The purpose of this article was to describe the use of Aquadex FlexFlow® for AQ in pediatric patients on ECMO, as a means to provide a simplified and safe form of fluid removal with minimal impact on ECMO therapy. The principal variables collected include patients' demographics, urine output, serum creatinine, withdrawal and infusion pressures, ultrafiltration (UF) rates, and ECMO flow ranges, along with length of stay in pediatric ICU and survival. Patient survival was 100% with preserved eGFR. The ECMO flows were not affected by AQ. Urine output decreased somewhat during therapy, with little AQ machine pressure variations. Range of UF tolerated without hemodynamic abnormalities was 1.24-6.2 mL/kg/h, allowing the patients to maintain their pre-AQ body weight, while receiving intravenous (IV) nutrition and medications. This article describes the use of AQ in tandem with ECMO in a user-friendly and safe way to provide UF in children requiring cardiopulmonary support, with minimal flow and hemodynamic disturbance.

Diagnosis, Treatment, and Outcomes in Children With Congenital Nephrogenic Diabetes Insipidus: A Pediatric Nephrology Research Consortium Study.

Background and Objectives: Congenital or primary nephrogenic diabetes insipidus (NDI) is a rare genetic disorder that severely impairs renal concentrating ability, resulting in massive polyuria. There is limited information about prognosis or evidence guiding the management of these patients, either in the high-risk period after diagnosis, or long-term. We describe the clinical presentation, genetic etiology, treatment and renal outcomes in a large group of children <21 years with NDI. Design: A multi-center retrospective chart review. Results: We report on 66 subjects from 16 centers. They were mainly male (89%) and white (67%). Median age at diagnosis was 4.2 months interquartile range (IQR 1.1, 9.8). A desmopressin acetate loading test was administered to 46% of children at a median age of 4.8 months (IQR 2.8, 7.6); only 15% had a water restriction test. Genetic testing or a known family history was present in 70% of the patients; out of those genetically tested, 89 and 11% had mutations in AVPR2 and AQP2, respectively. No positive family history or genetic testing was available for 30%. The most common treatments were thiazide diuretics (74%), potassium-sparing diuretics (67%) and non-steroidal anti-inflammatory drugs (42%). At the time of first treatment, 70 and 71% of children were below -2 standard deviations (SD) for weight and height, respectively. At last follow-up, median age was 72.3 months (IQR 40.9, 137.2) and the percentage below -2 SD improved to 29% and 38% for weight and height, respectively. Adverse outcomes included inpatient hospitalizations (61%), urologic complications (37%), and chronic kidney disease (CKD) stage 2 or higher in 23%. Conclusion: We found the majority of patients were treated with thiazides with either a potassium sparing diuretic and/or NSAIDs. Hospitalizations, urologic complications, short stature, and CKD were common. Prospective trials to evaluate different treatment strategies are needed to attempt to improve outcomes.

Case Report: Cystinuria and Polycystic Kidney Disease.

Cystinuria and polycystic kidney disease are 2 genetic disorders that affect the genitourinary tract but rarely together. This case report presents 2 pediatric patients diagnosed with polycystic kidney disease and cystinuria requiring surgical treatment. Both subjects presented acutely with stone disease. Imaging studies and stone analysis established the diagnoses. Although coexistence of these 2 conditions is rare, cystinuria should be considered in the differential diagnosis when evaluating patients with cystic disease who develop renal calculi.

Sirolimus lowers myeloperoxidase and p-ANCA titers in a pediatric patient before kidney transplantation.

We report the case of a child with microscopic polyangiitis (myeloperoxidase-antineutrophil cytoplasmic antibody [p-ANCA]-positive vasculitis) whose disease progressed to end-stage renal failure, in whom sirolimus contributed to normalization of myeloperoxidase and ANCA titers. The disease course, various therapies, and outcome are discussed, as is the rationale for using sirolimus.

Non-enteropathic hemolytic uremic syndrome: causes and short-term course.

BACKGROUND: Nondiarrheal or Streptococcus pneumoniae-related hemolytic uremic syndrome (HUS) represents a heterogeneous group of disorders. This study was performed to: (1) describe the current incidence, causes, demographic features, hospital courses, and short-term outcomes of non-enteropathic HUS; (2) compare findings in patients with non-enteropathic HUS with those obtained from a contemporaneous cohort of children with enteropathic or diarrhea-associated HUS (D+ HUS) diagnosed and treated at the same clinical sites; and (3) identify clinical or laboratory features that differentiate these 2 groups and predict disease severity and the short-term outcome in patients with non-enteropathic HUS. METHODS: Data were collected from patients screened between 1997 and 2001 for enrollment in a multicenter trial of SYNSORB Pk (SYNSORB Biotech Inc, Calgary, Alberta, Canada) in D+ HUS, but who were ineligible because of lack of a diarrhea prodrome. The following features were recorded: age; sex; ethnicity; prodromal symptoms; cause; nadir values for hemoglobin, hematocrit, and platelet count; use of dialysis; and length of hospitalization. RESULTS: Twenty-seven of 247 children with HUS had non-enteropathic HUS (11%). Twenty-four patients (15 boys, 9 girls), whose medical records were complete and available for review, comprise the study cohort. Mean age at onset was 4.2 +/- 0.9 (SE) years. Infection caused by S pneumoniae was diagnosed in 9 patients (38%). Dialysis was performed in 17 patients (71%) for 40 +/- 27 days. Median length of hospitalization was 22 days (range, 2 to 71 days). Children with S pneumoniae-related HUS had a longer hospital stay than those with other causes of non-enteropathic HUS, but all patients with S pneumoniae-related HUS recovered kidney function. Dialysis therapy was required more often (17 of 24 versus 59 of 145 children; P = 0.025) and hospital stays were longer (median, 22 versus 9 days; P = 0.002) in children with non-enteropathic HUS compared with patients with D+ HUS who were enrolled in the SYNSORB Pk clinical trial. CONCLUSION: (1) The incidence of non-enteropathic HUS is approximately one tenth that of D+ HUS; (2) patients with non-enteropathic HUS require dialysis therapy more often and are hospitalized more than twice as long during the acute episode compared with those with D+ HUS; (3) infection caused by S pneumoniae accounts for nearly 40% of cases of non-enteropathic HUS; and (4) although S pneumoniae-related HUS is associated with a less favorable short-term course than other types of non-enteropathic HUS or D+ HUS, the long-term prognosis for recovery of renal function appears to be good in these patients.