Rare Presentation of Atypical Hemolytic Uremic Syndrome in a Child With Acute Lymphocytic Leukemia and Pancreatitis.

Atypical hemolytic uremic syndrome (aHUS) is associated with significant mortality and morbidity, including acute renal injury, anemia and thrombocytopenia. Rare cases of aHUS in a child with acute leukemia before diagnosis or during chemotherapy have been reported. We report a pediatric case of B-cell acute lymphoblastic leukemia complicated by pancreatitis with concomitant aHUS following induction chemotherapy.

Use of ofatumumab and eplerenone in post-transplant recurrence of FSGS.

BACKGROUND: Focal segmental glomerulosclerosis (FSGS) predisposes patients for risk of recurrent disease in allografts. METHODS: We report a case of a recipient of an unrelated living donor renal transplant and discuss considerations for utilization of ofatumumab and eplerenone in treatment for recurrent FSGS. RESULTS: The recipient was initially managed with scheduled plasmapheresis, intravenous immunoglobulin (IVIG), and rituximab post-transplant during index hospitalization. With notable recurrence of FSGS noted on kidney transplant biopsy, she was initially treated with additional plasmapheresis sessions leading to downtrend in proteinuria. The patient was then transitioned to LDL-A pheresis, which resulted again in uptrend in proteinuria. This prompted return to scheduled plasmapheresis sessions weekly, leading again to a downtrend in proteinuria. Albumin levels remained within normal range throughout her course. Following initiation of eplerenone and ofatumumab, the patient demonstrated normalization of urine protein:creatinine ratio and remission of FSGS recurrence without need for additional apheresis. CONCLUSIONS: With notable risk of recurrence of FSGS in kidney transplants leading to allograft failure, the use of ofatumumab and eplerenone in conjunction should be considered for management to induce remission.

Considerations for utilizing medullary sponge kidney allografts in pediatric patients.

BACKGROUND: Medullary sponge kidney (MSK) disease predisposes patients to recurrent nephrolithiasis, which affects one in every 5000 people in the United States. METHODS: We report a rare case of a pediatric recipient of a living donor MSK transplant and discuss considerations when discussing risks and benefits of accepting MSK allografts for this population. RESULTS: The recipient was admitted due to concerns for nephrolithiasis, hydronephrosis, and urinary tract infection at 1-month post-transplant. The hydronephrosis was resolved by surgical removal of an encrusted ureteral stent; this was followed by supplementation with oral medications to prevent future episodes of nephrolithiasis. The recipient did not have any further episodes after this as seen at a 1-year follow-up. The donor has remained well through this period. CONCLUSIONS: With increasing organ shortages, the use of variety of donors may need to be considered to enlarge the organ pool.

Development of a time of flight spectrometer based on position sensitive multi-wire proportional counters for fission fragment mass distribution studies.

Characteristics and performance of a time of flight (TOF) spectrometer developed for performing fission mass distribution studies are presented. The spectrometer contains two TOF arms based on multi-wire proportional counters (MWPCs). Each arm has two MWPCs to form a start-stop detection system for TOF measurements. The start detector has an active area of 4 × 4 cm2. The stop detector is a two-dimensional position sensitive MWPC with an active area of 16 × 11 cm2. Salient features of the MWPCs are the use of reduced sub-millimeter wire pitches of 0.635 and 0.317 mm in the electrodes along with the use of gold plated tungsten wires of diameters 10 and 20 µm. A delay line for position electrodes is prepared using chip inductors and capacitors. Ten different configurations of MWPC were investigated for the start detector, which involved the use of three and four electrode geometries, use of different wire pitches, and use of aluminized mylar for timing electrodes. Performance results close to micro-channel plate detectors have been observed with some designs of MWPC, displaying rise times better than 2 ns with an estimated inherent time resolution of ∼100 ps FWHM. A position resolution of ∼1 mm (FWHM) has been observed. Design features of the MWPCs and their test performance results are described in this article.

AKI in COVID-19-Associated Multisystem Inflammatory Syndrome in Children (MIS-C).

Background: Multisystem inflammatory syndrome in children (MIS-C) is a recently identified entity in association with COVID-19. AKI has been widely reported in patients with primary COVID-19 infection. However, there is a paucity of literature regarding renal injury in MIS-C. We aim to characterize AKI in MIS-C in this cohort identified at a major children's hospital in New York City during the COVID-19 pandemic. Methods: We conducted a retrospective cohort study of children 0-20 years old admitted to Morgan Stanley Children's Hospital (MSCH) between April 18th and September 23rd, 2020. Patients were included if they met criteria for MIS-C on the basis of CDC guidelines. All patients were evaluated for the presence of AKI, and AKI was staged according to KDIGO criteria. Results: Of the 57 children who met inclusion criteria, 46% (26 of 57) were found to have AKI. The majority of patients (58%; 15 of 26) were classified as KDIGO stage 1. AKI was present upon admission in 70% of those identified. All patients had resolution of AKI at discharge, with 61% achieving recovery by day 2. One patient required dialysis. When compared with those without renal injury, the AKI cohort was older (P<0.001) and had higher median peak values of CRP (P<0.001), IL-6 (P=0.02), ferritin (P<0.001), and procalcitonin (P=0.02). More patients with AKI had left ventricular systolic dysfunction (P<0.001) and lymphopenia (P=0.01) when compared with those without AKI. No differences in body mass index or sex were found. Conclusions: Although children with MIS-C may develop AKI, our study suggests that most experience mild disease, swift resolution, and promising outcome. Older age, increased inflammation, and left ventricular systolic dysfunction may be risk factors. Our study highlights the substantial differences in epidemiology and outcomes between AKI associated with pediatric MIS-C versus primary COVID-19 infection.

Eculizumab treatment for renal failure in a pediatric patient with COVID-19.

While there are increasing reports of acute kidney injury among hospitalized adults with COVID-19, there is still limited information on renal complications associated with COVID-19 in children. The cause of kidney involvement in COVID-19 is likely multifactorial, and appears to involve a complex process, including complement dysregulation and thrombotic microangiopathy. We present a pediatric case of COVID-19 and renal failure due to thombotic microangiopathy, successfully treated with eculizumab.