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Background Intensive care management of diabetic ketoacidosis (DKA) is targeted to reverse ketoacidosis, replace the fluid deficit, and correct electrolyte imbalances. Adequate restoration of circulation and treatment of shock is key. Pediatric treatment guidelines of DKA have become standard but complexities arise in children with co-morbidities. Congenital nephrogenic diabetes insipidus (NDI) is a rare hereditary disorder characterized by impaired kidney concentrating ability and treatment is challenging. NDI and DKA together have only been previously reported in one patient. Case diagnosis/treatment We present the case of a 12-year-old male with NDI and new onset DKA with hyperosmolality. He presented in hypovolemic shock with altered mental status. Rehydration was challenging and isotonic fluid resuscitation resulted in increased urine output and worsening hyperosmolar state. Use of hypotonic fluid and insulin infusion led to lowering of serum osmolality faster than desired and increased the risk for cerebral edema. Despite the rapid decline in serum osmolality his mental status improved so we allowed him to drink free water mixed with potassium phosphorous every hour to match his urinary output (1:1 replacement) and continued 0.45% sodium chloride based on his fluid deficit and replacement rate with improvement in his clinical status. Conclusions This case illustrates the challenges in managing hypovolemic shock, hyperosmolality, and extreme electrolyte derangements driven by NDI and DKA, as both disease processes drive excessive urine output, electrolyte and acid-base imbalances, and rapid fluctuation in osmolality., Author(s): Yu-Shan Tseng [sup.1] [sup.2] [sup.3] [sup.4] , Nicole Swaney [sup.1] [sup.2] , Katherine Cashen [sup.5] , Amrish Jain [sup.1] [sup.2] , Nina Ma [sup.1] [sup.2] , Andrew Prout [sup.1] [...]

Introduction No data exist on the epidemiology of children incidentally diagnosed with advanced kidney failure (KF) during evaluation for non-specific symptoms. This is likely related to unrecognized symptoms and signs of CKD. The objective of our study was to evaluate incidentally diagnosed patients with advanced KF requiring long-term kidney replacement therapy (KRT). Methods An IRB-approved retrospective chart review of children who started KRT with dialysis (hemo- or peritoneal) was conducted. Included were children with no prior knowledge or diagnosis of underlying kidney disease with chronic kidney disease (CKD) disease stage 4 (GFR 15-29 mL/min/1.73 m.sup.2) or 5 (GFR < 15 mL/min/1.73 m.sup.2) at initial presentation and started on chronic KRT within 2 months of presentation. Results Of 177 patients initiating KRT during the study period, 26 (15%) were categorized as incidental advanced KF. This cohort with mean age 12.25 years consisted of 42% males, 54% African Americans included 46% with glomerular, and 54% with non-glomerular etiology for kidney failure. Vomiting (42%) and fatigue (39%) were most common, while growth failure (19%) and hyperkalemia (7%) were less frequent on initial presentation. Anemia (100%), hypertension (96%), hyperparathyroidism (96%), and hyperphosphatemia (92%) were the most frequently seen CKD comorbidities. Chronic KRT was started within 24 h in 62% and within 2 weeks in 88% of the cohort. Conclusion Under-diagnosis of patients with advanced KF is most likely related to milder non-specific clinical symptoms and normal growth in the majority of patients. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information., Author(s): Wael M. Abukwaik [sup.1] , Rossana Baracco [sup.1] , Amrish Jain [sup.1] , Melissa Gregory [sup.1] , Rudolph P. Valentini [sup.1] , Gaurav Kapur [sup.1] Author Affiliations: (1) grid.253856.f, [...]

Background: Obesity is associated with increased complications, rejection, and graft loss after kidney transplantation in adult and pediatric recipients. Elevated body mass index (BMI) is a common contraindication to transplant at adult kidney transplant programs; however, there is no data on such limitations for pediatric patients. Methods: Between October and December 2022, we conducted a survey of Pediatric Nephrology Research Consortium centers assessing the use of BMI in pediatric kidney transplant evaluation. Centers reporting utilization of BMI cutoffs were invited to submit patient-level data on children declined for active transplant listing due to BMI. Results: Thirty-nine centers responded to the survey (42% response rate); 51% include BMI in their written listing criteria, with a median BMI "cutoff" of 39 kg/m2 (range 30–50 kg/m2). Between January 1, 2016, and December 31, 2021, 30 children at 15 transplant centers were declined for listing status due to BMI. Patient-level data was provided for 19 children (63%) who were denied active listing status; median BMI was 42 kg/m2 (range 35.8–49.4 kg/m2) and 84% were on dialysis. One year after evaluation, seven patients (37%) had proceeded to active wait list status. Eight (42%) remained in inactive status and four (21%) were unlisted; ten of these 12 patients (83%) were on dialysis. Conclusions: The use of BMI in pediatric kidney transplant evaluation and listing varies among centers, but BMI limits access to transplant for some children. More information is needed on the outcomes of obese pediatric kidney candidates who are and are not transplanted, to guide development of national and international consensus. [ABSTRACT FROM AUTHOR]

Pheochromocytomas and paragangliomas (PPGLs) generally grouped together are rare catecholamine-secreting endocrine tumors. Symptoms of catecholamine excess are non-specific and therefore a high index of suspicion in children with sustained hypertension, family history of endocrine tumors, or features of syndromes associated with PPGLs leads to a timely diagnosis and treatment. Free metanephrines in the plasma or 24-h urine are the preferred tests to establish catecholamine excess. Considerations for false-positive conditions improve diagnostic yield and accuracy. Functional imaging, targeting either specific cell membrane transporters or vesicular catecholamine transport systems, is indicated for incidental lesions suspicious for PPGLs with inconclusive biochemical testing, assessment of regional extension or multifocality, and exclusion of metastases. Surgery is the mainstay of treatment for PPGLs. Preoperatively, sequential use of alpha adrenergic receptor blockade and volume expansion followed by beta blockade is mandatory to reduce intraoperative intravascular instability and blood pressure fluctuation due to tumor manipulation. Since genetic mutations have been reported in tumor susceptibility genes in nearly 50% of patients with PPGLs, genetic counselling and testing should be considered in all patients with a confirmed tumor., Author(s): Amrish Jain [sup.1] , Rossana Baracco [sup.1] , Gaurav Kapur [sup.1] Author Affiliations: (1) grid.254444.7, 0000 0001 1456 7807, Division of Pediatric Nephrology and Hypertension, Children's Hospital of Michigan, [...]

Background Patients negative for Shiga toxin-producing E. coli (STEC) are categorized as having atypical hemolytic uremic syndrome (HUS) and are associated with an increased risk for complement mutations and poorer prognosis compared with typical HUS. However, STEC identification is limited by the natural history of HUS. Methods The current study is aimed at identifying HUS patients with poor outcomes based on the presence or absence of diarrhea (D) or Shiga toxin (S). A single-center retrospective review (2003-2012) of 42 HUS patients (follow-up 31.3 ± 38.7 months) was carried out. HUS was managed clinically with supportive treatments such as dialysis, plasma therapy, and eculizumab. Results There was no significant difference in the D+S+ (31 %), D+S- (50 %) and D-S- (19 %) groups in the outcome variables of chronic kidney disease stages I-II (100 % vs 81 % vs 67 %) and proteinuria at follow-up (20 % vs 12.5 % vs 33.3 %), hospitalization duration (16.0 ± 8.7 vs 18.1 ± 9.5 vs 23.7 ± 12.9 days); dialysis requirement (50 % vs 81 % vs 66.7 %), and dialysis duration (10.2 ± 1.9 vs 33.3 ± 72.8 vs 10.3 ± 8.1 days). There was no significant difference in study outcomes in STEC+ (59 %) versus STEC- (41 %) groups. Genetic testing was performed in 12 % of HUS patients based on age, recurrent HUS, familial HUS, persistently low C3, or prolonged dialysis, and 80 % of the patients tested were positive for genetic mutations. Conclusions Our study does not show poorer outcomes in STEC- HUS. Indications and the cost-effectiveness of genetic testing, eculizumab, and plasmapheresis in STEC- HUS need to be evaluated further., Author(s): Prabesh Bajracharya[sup.1] , Amrish Jain[sup.1] , Rossana Baracco[sup.1] , Tej K. Mattoo[sup.1] , Gaurav Kapur[sup.1] Author Affiliations: (1) Children's Hospital of Michigan, Wayne State University, 3901 Beaubien Boulevard, 48201, [...]

Background An internal permanent vascular access [arterio-venous fistula (AVF) or arteriovenous graft (AVG)] is preferred over central venous catheters (CVC) for chronic hemodialysis. However, CVC remain the most commonly used access in children. The objective of this study was to evaluate our experience with AVF. Methods We conducted a retrospective chart review of children aged 1-18 years on chronic hemodialysis from 2001 to 2012. Patients were divided into three time periods: 2001-2005, 2006-2009 and 2010-2012. A systematic approach to AVF placement was introduced in our department in 2006 which resulted in a greater number of AVF being placed and used, but the access failure rate was still higher than desired. In 2010, a more experienced vascular surgeon was contacted to perform AVF surgery in our most difficult AVF candidates. Results Sixty-five AVF were created in 55 patients (67.3 % male). The median age of the patients was 14 (3-18) years. Forty-one (63.1 %) AVF were used successfully, and this number increased from 52.6 to 57.6 to 92.3 % over the three time periods, respectively. Over time, AVF use rates increased and CVC use decreased. By 2012 only 7.7 % of our patients were using a CVC. The primary patency rate was 42.9 % at 1 year; secondary patency rates were 100 and 93.8 % at 1 and 2 years, respectively. Infection and hospitalization rates were higher for CVC than for AVF [0.8 vs. 0.1 infections per access-year (p < 0.001) and 0.9 vs. 0.2 hospitalizations per access-year (p < 0.001)]. Conclusions With a dedicated approach and vascular access team it is possible to decrease CVC and increase AVF use in children on hemodialysis. In our study, increased AVF use resulted in decreased access-related infection and hospitalization rates. Keywords Arteriovenous fistula * Central venous catheter * Chronic hemodialysis * Vascular access, Introduction The number of children with end-stage renal disease (ESRD) receiving hemodialysis (HD) is increasing. According to the 2013 United States Renal Data System Annual Report, the number of prevalent [...]

Although prednisone is the treatment of choice for nephrotic syndrome (NS) in childhood, the dosing regimen varies between 60 mg/m.sup.2/day, as recommended in early studies, to the often prescribed 2 mg/kg/day dose, which is used in common practice. Mathematical models have demonstrated that weight-based dosing can be less than body surface area (BSA)-based dosing in smaller children. To test our hypothesis that weight-based dosing would result in altered treatment outcomes in children with NS, we analyzed a cohort of 56 children (mean age 5.4 ± 3.8 years) treated with a weight-based dosing regimen. Theoretical underdosing of corticosteroids was tested by calculating a relative underdosing percentage (RUP), which was defined as the dose difference between the theoretical BSA-based dose and the actual weight-based doses divided by the BSA-based dose x 100. We found that the mean 'actual' prednisone dose in our patients was 43.6 ± 19.3 mg/day; in contrast, the mean theoretical BSA-based dose was calculated to be 48.8 ± 16.7 mg/day. Among the 56 patients, 43 (76.7%) were initial responders, of whom 58% followed a frequently relapsing (FR) course. RUP was significantly higher in FR (16.6 ± 7.9%) than in infrequent relapsers (8.7 ± 9.8%) (P = 0.03). RUP was not significantly different among initial responders and nonresponders. Based on these results, we conclude that prednisone underdosing, when dosing is prescribed according to weight, does not affect the initial response to treatment, but it does increase the likelihood of a FR course in responders., Author(s): Sermin A. Saadeh [sup.1] , Rossana Baracco [sup.1] , Amrish Jain [sup.1] , Gaurav Kapur [sup.1] , Tej K. Mattoo [sup.1] , Rudolph P. Valentini [sup.1] Author Affiliations: (1) [...]

Iron deficiency (ID) contributes to the development of anemia in patients with chronic kidney disease (CKD). The frequency of ID in children with early CKD has not previously been reported. This was a retrospective chart review of children with CKD stages 2 and 3 followed at the CKD clinic of Children's Hospital of Michigan. ID was defined as low ferritin and transferrin saturation Keywords Iron deficiency * Anemia * Chronic kidney disease * Ferritin * Transferrin saturation, Introduction Anemia has been associated with increased mortality and increased risk of hospitalization in children with chronic kidney disease (CKD) [1]. Apart from the complications of anemia, iron deficiency (ID) [...]

Total parenteral nutrition (TPN) was started on hospital day 7 as the patient was still intubated and stuporous. Prior to initiation of TPN, serum thiamine and cobalamin levels were obtained [...]

Case summary A 16-year-old girl with a history of systemic lupus erythematosus (SLE), end-stage renal disease (ESRD) secondary to lupus nephritis on continuous cycling peritoneal dialysis (PD) presented to the [...]