NKF 2020 Spring Clinical Meetings Abstracts
and ferritin and hepcidin decreased. In vadadustat treated DD subjects,
TIBC increased, while TSAT, ferritin, and hepcidin were significantly
decreased. Changes in iron parameters between iron users and non-
iron users are presented in the table for NDD and DD groups. In DD
subjects receiving an erythropoietin stimulating agent (ESA) prior to
vadadustat (N=20), the changes from baseline in TIBC, ferritin, and
TSAT were greater than in ESA-naïve subjects (N=15) in all dosing
arms ( p<0.05).
In these two small phase 2 trials of both NDD and DD patients
receiving vadadustat over 16 wks, changes in iron parameters were
observed and warrant further investigation.
353
A NOVEL FINDING OF IGA-PREDOMINANT
MEMBRANOUS NEPHROPATHY:
Mina Sourial1, Deep Sharma1, Molly Fisher1, James Pullman1.
1Montefiore Medical Center/Albert Einstein College of Medicine,
Bronx, NY, United States
Idiopathic membranous nephropathy (IMN) is characterized by
granular IgG predominant subepithelial immune deposits, whereas the
hallmark of IgA nephropathy (IgAN) is mesangial deposition of IgA
immune deposits. The coexistence of MN and IgAN has been described
but is rare. We present a case of IgA-predominant membranous
nephropathy as a possible distinct entity from IMN and IgAN overlap.
A 39 year-old African American female with a history of asthma,
type 2 diabetes mellitus, alpha-thalassemia and hemoglobin C trait was
referred for evaluation of subnephrotic proteinuria of 2.8 grams on a
24 hour urine collection. Her serum creatinine was 0.5 mg/dL. She was
diagnosed with diabetes in 2014 and had no retinopathy or
microvascular complications. Serologic workup for HIV infection,
hepatitis B and C, and systemic lupus erythematosus was negative.
Renal ultrasound revealed normal sized kidneys and normal
echogenicity.
Renal biopsy demonstrated immune complex glomerulonephritis
with IgA predominant subepithelial deposits. Immunofluorescence
demonstrated 4+ IgA granular staining and only 1+ IgG and C3
granular staining of the capillary walls which is uncharacteristic of
IMN. The pathognomonic finding of IgAN is IgA predominant
mesangial deposits. However, this finding was not present in our
patient. Positive phospholipase A2 receptor (PLA2R) immunostaining
was seen in the deposits, but the serum anti-PLA2R antibody was
negative. Additionally, all immune deposits were composed of unusual
40-100 nm spherule structures which has been observed in a subset of
patients with IMN.
Overlap between IMN and IgAN has been described by codominance
of IgG subepithelial and IgA mesangial immune deposits. Our patient’s
renal biopsy distinctly revealed IgA subepithelial deposits and
although most of the histological features were consistent with IMN
the subepithelial deposits predominantly contained IgA. Also,
subepithelial deposit spherules have been described in IMN, but not to
the extent seen in our patient’s renal biopsy and never in IgAN. The
clinical significance of these spherules and unusual distribution of IgA
is uncertain.
354
DOSE EFFECT ANALYSIS OF SODIUM ZIRCONIUM
CYCLOSILICATE (SZC) IN HYPERKALEMIC
HEMODIALYSIS PATIENTS IN THE DIALIZE STUDY:
Bruce Spinowitz1, Stephen Fishbane2, Kieran McCafferty3, Masafumi
Fukagawa4, Nicolas Guzman5, Martin Ford6, Anjay Rastogi7, Sunil
Bhandari8. 1Nephrology Associates, P.C., Flushing, NY, United States;
2Zucker School of Medicine at Hofstra/Northwell, Great Heck, NY,
United States; 3Barts Health NHS Trust, London, United Kingdom;
4Tokai University School of Medicine, Isehara, Japan; 5AstraZeneca,
Gaithersburg, MD, United States; 6King's College Hospital NHS Trust,
AJKD Vol 75 | Iss 4 | April 2020
London, United Kingdom; 7David Geffen School of Medicine, Los
Angeles, CA, United States; 8Hull University Teaching Hospitals NHS
Trust, Hull, United Kingdom
The DIALIZE trial (NCT03303521) showed that SZC reduces
predialysis serum potassium (sK+) after the long interdialytic interval
and is well tolerated in hemodialysis patients (pts) with hyperkalemia.
This post‐hoc analysis assessed the dose effect of SZC on predialysis
body weight, blood pressure (BP) and ultrafiltration rate (UFR) during
dialysis.
The study randomized 196 pts 1:1 to placebo (n=99) or SZC (n=97)
and analyzed 195 (safety group). The starting dose was 5 g once daily
on non-dialysis days for a 4-week dose titration phase (titrated in 5 g
increments to 15 g max) to achieve predialysis sK+ 4.0–5.0 mmol/L,
and a 4-week stable dose evaluation phase (SZC 0, 5, 10 or 15 g). We
assessed change from baseline (Visit 4, Day 1) to end of treatment
(EOT; Visit 15, Day 57) in interdialytic weight gain (IDWG), diastolic
BP (DBP), systolic BP (SBP) and UFR, stratified by final SZC dose in
dose titration phase. Dialysis UFR (mL/h/kg) was calculated as: actual
ultrafiltration (mL)/dialysis duration (h)/predialysis weight (kg).
During the dose evaluation phase, 38, 41 and 17 pts received SZC 5,
10 and 15 g, respectively. Changes from baseline in IDWG, DBP, SBP
and UFR had no consistent pattern and did not show a dose
relationship (Table).
Findings of no increases in BP, IDWG or UFR with higher SZC doses
suggest that dose adjustments based on pts’ sK+ resulted in
comparable safety.
355
RENAL PHOSPHATE WASTING DUE TO TUMOR-
INDUCED OSTEOMALACIA IN A BREAST CANCER
PATIENT:
Maya Srinivasan1,2, Richard Ross1, Matthew Abramson1. 1Memorial
Sloan Kettering Cancer Center, New York, NY, United States; 2State
University of New York Downstate Medical Center College of Medicine,
Brooklyn, NY, United States
Tumor-Induced Osteomalacia (TIO) is a rare paraneoplastic
syndrome is characterized by renal phosphate wasting, bone fractures
and osteomalacia, and is mediated through inappropriate tumor
production of fibroblast growth factor 23 (FGF23). TIO is classically
associated with mesenchymal tumors, but has also rarely been linked
to adenocarcinoma, such as breast. TIO in breast cancer poses a
distinct diagnostic challenge due to the common use of denosumab or
bisphosphonates, which also causes hypophosphatemia. In these
patients, TIO diagnosis may be delayed, resulting in worsening
functional status, and early morbidity and mortality.
A 55-year-old woman with breast cancer metastatic to the liver,
lung, and bone was admitted for obstructive jaundice, cachexia, and
bone pain limiting ambulation. She progressed despite multiple
therapies, most recently paclitaxel. She received ten months of
denosumab, last dose 2 months ago.
Upon admission, labs revealed hypophosphatemia 1.6 mmol/L,
calcium 9.5 (8.5-10.5)mmol/L, low 25-hydroxyvitamin D of 15 (20-
50)ng/dL, elevated FGF23 548 (<180)RU/mL, parathyroid hormone
(PTH) 287 (12-88)pg/mL. Renal function was normal. Urine studies
revealed high fractional excretion of phosphorus (FePhos) of 78%
(normal <10%), consistent with renal phosphate wasting. Due to
aggressive supplementation, serum phosphate increased to a peak
value of 3.8 mmol/L; PTH decreased to 44, but FGF23 and FePhos
remained elevated at 424 and 72%, respectively (Fig. 1).
639

Persistently elevated FGF23 and continued renal phosphate wasting
after normalization of PTH are consistent with the diagnosis of TIO.
Unfortunately, her functional status rapidly declined and she was
discharged on home hospice.
Hypophosphatemia is a common electrolyte abnormality in patients
with metastatic cancer, and is often attributed to bisphosphonates or
poor oral intake. TIO is an important differential, in that early
detection may result in improved outcomes. FGF23 testing is becoming
more available in the clinical setting, and may help with detection of
TIO. Here we present a unique case of a patient with metastatic breast
cancer, whose FePhos and FGF 23 levels were helpful in diagnosing
TIO.
356
C3 GLOMERULONEPHRITIS; A RARE COMPLICATION
OF CLL:
Khaled Srour1, Jayanath Lakshmikanth1, Chandrika Chitturi1, Mark
Faber1. 1Henry Ford Hospital, Detroit, MI, United States
Kidney disease develops in chronic lymphocytic leukemia (CLL)
patients via multiple mechanisms including infiltration, obstruction,
tumor lysis syndrome, and glomerular disease. We present a rare case
of C3 glomerulonephritis (C3GN) associated with pulmonary renal
syndrome that we believe was an autoimmune manifestation of CLL.
A 76-year-old male with a 15-year history of SLL/CLL, DVT, HTN,
DM and Stage 3 CKD developed SOB and dry cough. At ED presentation
he was in respiratory distress with BIPAP-resistant hypoxia requiring
intubation. Labs included Cr 7.1 mg/dL, K 5.7 mEq/L and uric acid 12.2
mg/dL CXR showed vascular congestion. Prior to developing anuria
urine sediment showed RBC casts. Bronchoscopy DAH, consistent with
a pulmonary renal syndrome. Patient was started on plasmapheresis,
high-dose steroids and CRRT. Autoimmune workup including ANA,
anti-GBM and ANCA was negative.
Kidney biopsy showed diffuse proliferative and sclerosing
glomerulonephritis with lymphocytic infiltrates consistent with
involvement by patient's known CD5+, CD23+ B-cell
lymphoproliferative disorder. IF showed diffuse C3 staining. Steroids
and plasmapheresis were continued. Renal function improved and
dialysis discontinued, with Cr at last follow up 1.9. Chemotherapy for
CLL has been ordered.
We present a case of C3GN and DAH secondary to CLL autoimmune
etiology, a rare complication of CLL which usually affects the kidney by
infiltration and by toxicity of the CLL treatment. Due to the rarity of the
disease, there is no standard of care treatment but this patient initially
responded well to steroids and plasmapheresis. Recent case reports
suggest improved outcomes of CLL-associated C3GN when CLL is
treated.
CLL is a rare cause of C3GN. A high suspicion of the disease and early
intervention with immunosuppression is the key in treating such
condition. Chemotherapy for CLL is expected to improve the patient’s
long-term renal outcome.
357
STARVATION KETOACIDOSIS IN PATIENT WITH
MUSCULAR DYSTROPHY:
Khaled Srour1, Bilal Shahzad Azam Khan1, James Novak 1. 1Henry Ford
Hospital, Detroit, MI, United States
Patients with Muscular Dystrophy have small muscle mass; thus,
they have less glycogen stores, and therefore they are more prone to
develop ketoacidosis with minimal stress or decreased oral intake. In
our case we are presenting a rare presentation of ketoacidosis in a
patient with muscular dystrophy, who was treated successfully with
Lactated Ringer and Dextrose solution due to her concurrent
hyperchloremic non-anion gap metabolic acidosis.
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NKF 2020 Spring Clinical Meetings Abstracts
Our Patient is forty-eight-year-old female with history of Muscular
Dystrophy and Chronic Respiratory Failure dependent on Ventilator,
she was referred to our hospital for evaluation of a granulation tissue
in her Trachea. She was admitted and found to have non-AG metabolic
Acidosis, VBG showed Ph of 7.23 Bicarbonate of 12, BMP showed Cr
<0.1, Chloride 114 (high), BMI 18, in addition to leukocytosis with
WBC>16000. Next day her labs showed Bicarbonate of 8, Ph of 7.29
and AG of 16 (Na 137, Cl 113), Albumin 4.2.
We checked her Beta Hydroxybutyrate (BHB) which came back 6.6
(High). Due to her underlying Muscular Dystrophy and decrease oral
intake, she was diagnosed with Starvation Ketoacidosis. Since she had
a non-anion gap metabolic acidosis at the time of admission which was
most likely secondary to Renal Tubular Acidosis, (Patient labs showed
Hypokalemia, Urine AG of 30), we decided to start her on ringer lactate
and dextrose solution. After 24 hours, her labs showed significant
improvement and her BHB trended down and after 48 hours her labs
have been normalized.
Few cases have been reported regarding ketoacidosis in patients
with muscular dystrophy, all reported cases were treated directly with
dextrose and normal saline. In our case we used LR and D5% due to
concurrent non anion gap metabolic acidosis which we believe it was
due to RTA, in which giving normal saline (hgih Chloride content) will
worsen the acidosis.
HAGMA in patient with no diabetes and Muscular dystrophy should
raise suspicion for Ketoacidosis. Treatment is with Dextrose and
normal saline ususally. Using LR solurion sometime is better option in
cases with concurrent hyperchloremic acidosis.
358
NEO-KIDNEY AUGMENTTM (NKA): PHASE II STUDY OF
PERCUTANEOUS INJECTION OF AUTOLOGOUS
HOMOLOGOUS NKA IN TYPE 2 DIABETES WITH PRE-
STAGE 5 CHRONIC KIDNEY DISEASE:
Joseph Stavas1, Tim Bertram1, Ashley Johns1, Deepak Jain1, David
Gerber2, Prabir Roy-Chaudhury3, George Bakris4. 1Twin City
Bio/inRegen, Winston Salem, NC, United States; 2University of North
Carolina, Chapel Hill, NC, United States; 3University of North Carolina,
Chapel Hill, NC, United States; 4University of Chicago, Chicago, IL,
United States
Few treatment options are avaiable to delay renal replacement
therapy for young patients with Type 2 Diabetes and eGFRs
approaching End Stage Renal Disease. We describe an ongoing Phase II
trial utilizing Neo-Kidney AugmentTM percutaneously injected into
kidneys with Pre-Stage 5 T2DM CKD with intent to delay renal
replacement therapy.
REGEN-003 is a multi-center, non-randomized prospective, open-
label, single-arm study recruiting 10 patients (NCT03270956).
Inclusion criteria include T2DM subjects age 30-65 years, eGFR 14 - 20
mL/min/1.73m3, and managed hypertension and HbA1c. Primary
objective is safety of NKA injected in one recipient kidney and
procedure and/or product related adverse events through 24 months.
The method of renal NKA delivery is by real-time image guided
percutaneous targeted injection into the subject's kidney with small
caliber atraumatic needles in an outpatient setting with moderate
sedation. NKA is composed of expanded autologous homologous
selected renal cells obtained by kidney biopsy. Trial completion is
expected early 2020.
Of 6 enrolled subjects to date, mean age is 55.8 years, 66.7% female,
50% Non-Hispanic/Latino. Baseline eGFR, serum creatinine and ACR
are shown below. All 6 subjects have undergone renal biopsy, 4 have
received injections. There have been no cell product or injection
related adverse events to date. 1 subject developed a post biopsy
hematoma however successfully underwent NKA injection.
Biomarkers measured Baseline values (S.D.) Number
eGFR (mean) 22.27 mL/min (7.99) 5
sCreatinine (mean) 3.14 (0.47) 5
ACR (geometric mean) 1391.0 mg/g (911.1) 3
This active Phase 2 trial of pre-stage 5 DKD using novel imaged
guided percutaneous targeted autologous homologous cell injection
into the kidney offers potential for preservation of renal function and
delay of renal replacement therapy.
AJKD Vol 75 | Iss 4 | April 2020