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NKF 2016 Spring Clinical Meetings Abstracts

Case Report
353 355
Pregnancy in a Patient With Primary Membranous Nephropathy
and Circulating Anti-PLA R Antibodies: A Case Report
DISEASE (CKD) PATIENTS: Morgan S. Tarbutton, Ramesh 2 Bassett Medical Center, Cooperstown, NY, USA.
Soundararajan, Zain Mehdi, Vinaya Soundararajan, Chicago College of A 55 year-old woman with non-diabetic ESRD on hemodialysis
Osteopathic Medicine, Midwestern University, Downers 1, Grove, presented
1 with confusion due to hypoglycemia of 221 mg/dL. She was a
Laith Al-Rabadi, MBBS, * Rivka
Illinois, USA, University of Illinois College of Medicine, Rockford, Ayalon, MD, Ramonkidney
post cadaveric G. Bonegio, MD,forPhD,
transplant patient idiopathic membranous
2,y 3 4
Illinois USA. Jennifer E. Ballard, MD, Alan M. Fujii, MD, nephropathy
Joel 14 M.years ago. Her kidney
Henderson, MD, graftPhD,
failed 2 years ago and she
Proton Pump Inhibitors (PPI) and Histamine-2 antagonists (H2 1 had become hemodialysis dependent. 1 Her symptoms resolved after
David J. Salant, MD, and Laurence
blockers) are readily available to patients over-the-counter and are
H. Beck Jr, MD, PhD
glucose intravenous infusion, however her blood glucose remained to
currently among one of the most prescribed classes of medications. be low for another 2 days in spite of 10%dextrose infusion. Patient
Recent studies have begun to associate PPIs with a significant denied history of insulin, oral hypoglycemic agents or beta-blocker
increased riskThere is little
of Chronic information
Kidney about pregnancy
Disease (CKD), including twooutcomes
large in patients with active membranous
usage. Sulfonylurea screening and blood nephropathy
culture were(MN), unimpressive.
studies, presented
especiallyas abstracts
those atwithASNcirculating
in 2015, by Pradeep Arora et alto M-typeWe
autoantibodies performed 72-hour
phospholipase fasting test(PLA
A2 receptor to rule
2R),out theinsulinoma.
major The test
of SUNY autoantigen
Buffalo Schoolinofprimary
MedicineMN.and Benjamin
We present Lazarus
what et al
weofbelieve to berevealed normal
the first knownresult
caseas her insulin level,pregnancy
of successful proinsulin inand C-peptide
Johns Hopkins University.
a 39-year-old woman with PLA2R-associated MN. In theresponded year prior appropriately
to pregnancy, to lowthe blood glucose.
patient Also CT chest and
We set out to study the prevalence of PPI and H2 blocker usage in abdomen was negative for abnormal masses, so insulinoma was
CKD patients hypoalbuminemia
in the greater (albumin,
Chicago area in our 1.3-2.2 g/dL), and proteinuria
outpatient-based, (protein excretion, 29.2 g/d). Kidney bi-
unlikely. Insulin-liked Growth Factor-1 (IGF-1) was checked to rule
opsy nephrology
single-specialty revealed MN groupwith staining
practice of 40 physician R, and the patientoutwas
for PLA2providers. seropositive
insulin for anti-PLAtumor,
secreting neuroendocrine 2R autoantibodies.
it was normal (109 ng/mL).
Under IRB
did notwe accessedtopatient
respond data from therapy
conservative Acumen EHR and was treated The with intravenous
serum rituximab
cortisol level (2 doses
of 26 mcg/mL duringof hypoglycemic
1 g each). episode is
between January
Several 1, 2012
weeks andafter
December 31, 2015. We
presentation, sheanalyzed
was founda totalto be 6 weeks
and adrenal
was closelyinsufficiency
followedwas excluded. She was
up without
sample population of 14,755 patients with CKD
further immunosuppressive 3, 4, 5 orProteinuria
treatment. End Stage remained discharged after her
with protein blood glucose
excretion in the had
8- tobeen in normal
12-g/d range.range. Patient
Renal Disease (ESRD) and determined the number of patients who was readmitted again twice within 1babymonth with theborn,
same problem. In
were currently on a PPI anti-PLA 2R levels
or H2 blocker declined
of any kind, but were still detectable.
in an observational, At 38 weeks, a healthy girl was
the last admission, she decided to stop hemodialysis, patient passed
cross-sectional proteinuria at birth or at her subsequent 6-month postnatal
study. visit. At the time of delivery, the mother still
away in the next 2 weeks.
hadanalysis duringcirculating
detectable the time period showed2R
anti-PLA 6.6% (n=976) of
of immunoglobulin G1Since
(IgG1), IgG3,
we had and IgG4
excluded most subclasses,
of the potentialalthough
causes of at
the total CKD
low patient
Only trace was on a PPI of some
amounts of IgG4 kind. anti-PLA2R wereincludingfound indrugs,cordsepsis,
Potential reasonsand
insufficiency for insulin
the secreting
Interestingly, 7.2% of CKD
discrepancy patients anti-PLA
between (n=1063) were taking an H2 tumor,
fetal spontaneous hypoglycemia
discussed.was diagnosed. The etiology of
2R levels in the maternal and circulation are
blocker, while 0.68% were on both (n=100). Percent of total CKD 3 & hypoglycemia was likely
Am J Kidney Dis. 67(5):775-778. ª 2016 by the National Kidney
4 patients on PPI was also determined to be 6.8% (n=445) and 8.1%
Foundation, Inc.related to impaired renal gluconeogenesis in
the setting of renal failure and malnutrition. Hypoglycemia in non-
(n=160), respectively, while 6.8% (n=449) and 7.1% (n=139) of CKD 3
diabetic renal failure is not uncommon. Its occurrence could represent
& 4 patients, WORDS:
respectively, wereMembranous
on a H2 blocker.nephropathy (MN); nephrotic syndrome; pregnancy; M-type phospholipase A2
poor prognosis. Spontaneous hypoglycemia in ESRD is attributed to
Based onreceptor
these findings
(PLAand the
R); fact that H2
autoantibody; blockers did
placenta; not have the
rituximab; immunoglobulin G (Ig G) subclass.
2 multiple factors including diminished renal gluconeogenesis, impaired
same associated risk of CKD in the other studies, we suggest there is a
renal insulin clearance and poor nutrition.
significant need to further evaluate the use of PPI in patients with CKD.
There also remains a further need to investigate if this association is

just by chance or if PPI use can be implicated in the cause of CKD.
regnant patients with autoimmune disease may CASE REPORT
deliver newborns with a spectrum of clinical A 39-year-old multiparous woman with morbid obesity pre-
manifestations due to the transplacental passage of sented for workup of severe nephrotic syndrome several months
354 356
circulating autoantibodies. Pregnant patients with
before her current pregnancy. She had been treated for resistant
lupus or myasthenia
TO OBSTRUCTIVE babies with hypertension
OBESE PATIENT: and lower-extremity
Ravi Thimmisettyedema 1 duringOmar
, Muhammad the Azam
past year,
Miguel Teixeira, MD;
corresponding Crystal Bonnichsen,
disease in the neonate. 1,2
MD. Mayo Clinic, Neonatal but her proteinuria
Nanette Chua1, Yayha hadOsman-Malik
been overlooked.
1 At presentation,
, Madhumita Jena Mohanty serum
Rochester, MN, USA creatinine
Wayne State level was 1.52
University mg/dL
School (corresponding
of Medicine 1
and John DtoDingell
membranousFailure to properly and promptly diagnose obstructive uropathy with
nephropathy (MN) not associated glomerular
Medical Center 2
rate Michigan,
, Detroit,
filtration of 46 mL/min/1.73
USA. m2 as calculated by
may lead to infection
congenital catastrophic complications.
was first Our case highlights
described in a1990
rare butand
reversible form of obstructive uropathy with its diagnostic nuances. the Idiopathic nodular glomerulosclerosis
isotope-dilution mass spectrometry (ING)–is a rare entity.
traceable We
attributed to the passive transfer
A 74 year old woman with a history of hypertension,of maternal anti- report a[Modification
MDRD case of ING in aof non-diabetic
Diet in Renalpatient.Disease] Study equa-
3 coronary artery disease 66 year oldalbumin
male withlevel,
longstanding uncontrolled hypertension
bodies to putative
hyperlipidemia, renal antigens.
well controlled type II diabetes,More than a decade tion); serum 1.5 g/dL; and 24-hour
(HTN), active smoking, BMI 22 kg/m2, liver transplant 15 years ago
urine protein
status post CABG, and 4 bipolar disorder developed end stage renal excretion, 29.2 g. The kidney biopsy specimen revealed features
later, Debiec
disease et toalbe
presumed identified the first
secondary to lithium antigen
toxicity. involved
She required for HCV related cirrhosis on tacrolimus, presented to clinic with
typical of primary
worsening of Cr fromMN 1.7 with
to 3 mg/dL strong stainingHefor
over 6 months. alsothe
in intermittent
such cases as neutral
hemodialysis and developed endopeptidase (NEP), a
a tunneled line-associated
PLA R antigen within immune
bloodstream infection. She was hospitalized and found to have MSSA had25.4 gm/day proteinuria with nodeposits
hematuria. (Fig S1). Many of
HemoglobinA1C was the
metalloprotease present on
native mitral valve endocarditis withthe surface
large of the
highly mobile podocyte
vegetation subepithelial depositsproteins
4.7%. No monoclonal were were
detected insurrounded byANA
serum or urine. new
involved in the
by septic proteolytic
embolic strokes, newregulation
heart block andof vasoac-
mitral basement membrane
and Hepatitis material FK506
panel was negative. (Fig levels
S2), were
and not35%
high. of the
peptides. On initial evaluation the patient was noted to have
Debiec et al described a mother
marked uterine procidentia with a visible cervix and cystocele outside with a Complements C3 and C4 levels were not low. Pathological differential
diagnosis included MPGN secondary
the vagina. preventing
No renal imagingNEP expression
had been who
obtained prior had formed
to initiation of
to chronic hepatitis C infection and
dialysis. CT abdomen confirmed complete uterine prolapse as well as
anti-NEP antibodies due to fetomaternal alloimmu- From the 1Department
chronic thrombotic
of Medicine, Renal Section, and De-
prolapse and obstruction of both distal ureters resulting in marked partments of 2
Obstetrics and Gynecology, 3Pediatrics, and 4Pa-
bilateralfrom a previous
hydronephrosis and renal miscarriage;
parenchymal atrophy.theseThe antibodies
patient due to tacrolimus therapy. However,
thology and Laboratory
renal biopsy revealed nodular Medicine, Boston University Medical
ultimately underwent surgical repair of the mitral valve followed by an
were to cross the placenta and cause subepithelial
ill-fated two month ICU stay and died soon after withdrawal of care. Center, Boston, MA.with 30-40%
deposits in lithium
Chronic the fetal
ingestionkidney of known
is classically a subsequent
to cause preg- *
diffuse interstitial
Current fibrosis
affiliation: and
Department of Internal Medicine, Division
nancy. M-typediabetes insipidus. It may alsoAleadreceptor
phospholipase to a variety of(PLA
other R) severe arterial nephrosclerosis
of Nephrology, University of Utah(see School of Medicine, Salt Lake
2 tubulointerstitial 2
intrinsic causes for renal failure including chronic figure).
City, UT.There was no double figure
later identified as the
Given the overlap major
between autoantigen
intrinsic and post-renalfor pri- contouring
y of glomerular basement membrane.
disease on urinary and serum5 analysis (FeNa >2%, urine sodium Current affiliation: Department of Obstetrics and Gynecology,
mary MN in adults. Little literature exists about Congo red stain was negative. Immunofluorescence was negative for
Medstar Washington Hospital Center, Washington, DC.
>40mEq/L, urine osmolality <400mOsm/kg) any patient with oliguria Immunoglobulins including kappa and lambda chains. Electron
pregnancy outcomes in patientspattern
or signs of renal failure with non-prerenal withwarrants
renal syn- microscopyJune
Received 29, 2015.
was negative for Accepted
organized in revised deposits.
glomerular form October 27,
drome due to toprimary
ultrasonography MN, with no data available
exclude hydronephrosis.. 2015. Originally
There are recentpublished
reports of online December
causative association29, 2015. ING and
Our case highlights pelvic organ prolapse as a rare but reversible
causepregnancy in PLA
of obstructive uropathy that 2 R-associated
should disease.
also not be overlooked evenWe
chronic correspondence
smoking, HTN and also to possibly
Laurence H. Beck
obesity. Jr, MD,
We report PhD,
a case of
ING in
Renal a non-diabetic,
Section, non-obese
X-504, 650 Albany patient with longstanding
St, Boston, MA 02118. smoking
present what
when other wearebelieve
causes to befaced
possible. When thewith known
first case of
renal disease,
and HTN. ING should be kept in the differential of hypertensive
clinicians should evaluate older women with a full urogynecologic
exam as wellin a patient
as with with PLA
renal ultrasonography. 2R-associated
If not promptly identified MN smokers
� 2016with proteinuria
by the NationalandKidney
renal dysfunction.
Foundation, Inc.
who endwas seropositive
stage renal formany
disease with its anti-PLA
2 R autoantibodies
may ensue. 0272-6386
throughout the course of her pregnancy.

Am J Kidney Dis. 2016;67(5):A1-A118 A107