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Nephrocalcinosis and Renal Calculi in a Patient With Chronic Hypophosphatemic Rickets
NKF 2016 Spring Clinical Meetings Abstracts
Case Report 229 PREVALENCE AND RISK FACTORS OF NEUROPSYCHIATRIC DISORDERS IN A COHORT OF END STAGE RENAL DISEASE PATIENTS ON MAINTENANCE HEMODIALYSIS: MOUSSALY Elias, SYED Rafeel, NASR Patricia, REHMAN Amina, EL-DOUEIHY Youssef, SAAD Marc, EL-CHARABATY Elie, EL-SAYEGH Suzanne Staten Island University Hospital – Staten Island, NY Some studies suggest that one in three patients on hemodialysis have some level of cognitive impairment. The cumulative burden of depression and dementia is poorly understood in the U.S. dialysis population. The Montreal Cognitive Assessment Questionnaire (MoCA) is a novel cognitive assessment test validated in the hemodialysis population and found to be a sensitive and rapid tool to detect mild cognitive impairment. We aimed to study the prevalence and correlates of cognitive impairment and depression in our dialysis population, excluding any patient on psychoactive medications, history of brain disease or substance abuse. Out of 177 in -center ESRD patients on maintenance hemodialysis, we screened 76 patients for participation in the study, 30 patients did not complete the intervention. A study investigator administered the MoCA and BECK questionnaires during a hemodialysis session to screen for cognitive impairment and depression respectively .To investigate for correlates, demographic and clinical data including common laboratory and dialysisrelated variables were collected and analyzed. In our cohort of 46 patients, mean age was 58.5 years [19; 96], 71.7% were male, 73% had a high school degree or higher and ethnicity was diverse (41 % white, 30 % African American and 28.2 % minorities). Only 5 patients had normal cognitive function (MOCA score above 27), with a median score of 23 suggestive of some form of cognitive impairement. Thirteen percent of the patients had BECK scores suggestive of clinical depression, while 32.6% of the patients had mild mood disturbance. In the multiple linear regression predictive analysis; age, the BECK score, and ferritin levels correlated negatively with the MOCA score (respective p -values: 0.06, 0.011, and 0.007). The fact that ferritin is inversely associated with cognitive function is a novel finding , needs further investigation and can be explained by inflammation, iron overload or intractable anemia. In a patient -centered, multi-disciplinary care model for the dialysis patient, it is imperative to better understand these neuropsychiatric disorders so as to improve quality of life, adherence to medical regimen, and ultimately, survival.
230 REVERSAL OF DIALYSIS DEPENDENT RENAL FAILURE AFTER RENAL ARTERY STENTING: A CASE FOR INTERVENTION 1 2 1 1 1 Azam Mughni , Ravi Dhanisetty , Avrum Gillespie , Jean Lee , Section 2 of Nephrology, Division of Vascular Surgery, Temple University Hospital, Philadelphia, PA, USA Despite 2 recent renal artery stenosis (RAS) trials that showed no benefit of angioplasty and stenting over medical management alone, controversy exists regarding case selection as patients who had acute renal failure or recurrent flash pulmonary edema from RAS were excluded from these trials. We present the case of an 83 year old male with progressive renovascular CKD IIIb (serum creatinine 1.71 mg/dL and 2.74 mg/dL four months and two months prior to presentation respectively) who presented with worsening dyspnea and acute kidney injury (creatinine 4.5mg/dl). He had 3 recent admissions for pulmonary edema and an ultrasound obtained on the last admission revealed an atrophic left kidney (with left renal artery stent placed 12 years before) and >60% occlusion of the right renal artery with preserved kidney size. The patient was edematous, hypoxic and with chest x-ray showing pulmonary edema. Despite a systolic blood pressure under 150 mmHg with 4 anti-hypertensive agents, he did not respond to high dose diuretics and hemodialysis was initiated for volume overload and uremic encephalopathy. By hospital day 15, the patient was medically stable but his oliguric renal failure persisted. Renal angiogram was then performed which showed high grade stenosis at the origin of right renal artery. The artery was then stented with subsequent angiogram showing resolution of the high grade stenosis. The urine output began to improve and dialysis was stopped 5 days later. The patient was then discharged on no BP agents. One month after discharge, creatinine was 1.28mg/dl but he requires 2 BP agents. Our case illustrates the benefit of revascularization with dramatic improvement in renal function. While recent trials suggest medical management for most cases of RAS, renal angioplasty with stenting should be considered in the presence of high grade RAS in the setting of pulmonary edema and acute renal failure necessitating dialysis.
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NEPHROCALCINOSIS AND RENAL CALCULI IN A PATIENT WITH CHRONIC HYPOPHOSPHATEMIC RICKETS Venkat Ram Rakesh Mundra, Dana Rizk, University of Alabama, Birmingham, AL, USA 1, Laith Al-Rabadi, 22 year old male patient with history of childhood rickets, presented to MBBS, * Rivka our Nephrology clinic with complaints of passing multiple small stones Jennifer E. Ballard, MD,2,y Alan in the urine. He was diagnosed with rickets at the age of 10 because of 1 locked knees and underwent bilateral knee surgery. His workDavid up at thatJ. Salant, MD, time revealed hypophosphatemia and he was treated with oral phosphorus and vitamin D supplements. He had normal growth and is little information about pregnancy o development. His medications were stoppedThere at age 19. In clinic, his physical exam was unremarkable. His labs showed normal especially thosecreatinine, with circulating autoantibod pg/ml) andinurinary low serum phosphorus (1.5 mg/dl), PTH (2.5 autoantigen primary MN. We present what phosphate wasting (FEPhos-29%). His serum 1, 25 dihydroxy vitamin a 39-year-old woman with PLA2R-associate D level and 24 hour urinary calcium were high (urinary calcium 283 anasarca, hypoalbuminemia mg/day). The diagnosis of “Hereditary Hypophosphatemic Rickets with (albumin, 1.3-2. opsyofrevealed MNand with staining for PLA2R, a Hypercalciuria” (HHRH) was made. CT scan the abdomen She calculi, did notmild respond to conservative therapy a pelvis revealed bilateral non obstructing renal increased density of renal pyramids consistent withSeveral nephrocalcinosis and bilateral weeks after presentation, she was fou renal cysts (three in each kidney). His stone analysis revealed 80% further immunosuppressive treatment. Protei calcium phosphate and 20% calcium oxalate. Patient was restartedRonlevels declined but w Circulating anti-PLA 2 oral phosphate supplementation without any vitamin D. without proteinuria at birth or at her subseque HHRH is a rare autosomal recessive disorder caused by mutations in had detectable circulating anti-PLA2R of imm SLC34A3, the gene encoding the renal sodium-phosphate colow titers. Only trace amounts of IgG4 ant transporter NaPi-IIc. It is clinically characterized by rickets, discrepancy between anti-PLA2R levels in th hypophosphatemia, phophaturia, hypercalciuria and high 1Am J Kidney Dis. 67(5):775-778. ª 2016 by 25dihydroxy vitaminD. Phosphate supplementation is the main stay of therapy. Beyond the musculoskeletal complications, it is important to INDEX WORDS: Membranous nephropathy ( recognize the potential renal manifestations of this disease including receptor (PLA2R); autoantibody; placenta; ritu nephrolithiasis and nephrocalcinosis. To our knowledge, renal cysts have not been reported with this disorder and remain an unexplained finding. Treatment of renal complications is not well established. The role of phosphate supplementationregnant to the end patients remains to be determined. with autoimmune disease
Pregnancy in a Patient Wit and Circulating Anti-P
P
deliver newborns with a spectrum of cl manifestations due to the transplacental passa 232 circulating autoantibodies. Pregnant patients SIROLIMUS CAUSING ACUTE QUADRICEPS HEMATOMA: lupus or myasthenia gravis can deliver babies A RARE COMPLICATION OF MTOR INHIBITORS: Sumeet Munjal, Fatima Najeeb, Jyotsna Bhatnagar, University at Buffalo, corresponding disease in the neonate.1,2 Neo New York, USA membranous nephropathy (MN) not associated mTOR inhibitors are used as potent immunosuppressive agents in congenital infection was first described in 199 solid organ transplant recipients and their properties are linked to attributed diabetes, to theaspassive transfer of maternal progression of proteinuria, hyperlipidemia, well as anemia. 3 Muscle hematomas are seen bodies in athletes,topatients on anticoagulants, but putative renal antigens. More than a d 4 rarely occur spontaneously. We report a case ofet severe into the first antigen inv later, Debiec al bleeding identified quadriceps and sartorius muscle in a patient taking Sirolimus. in such cases as neutral endopeptidase (NE 59 year old male with PMH of renal transplantation secondary to metalloprotease present on the surface of the pod PCKD, HTN, CAD, presented with complaints of right calf swelling and pain for one week. Patient denied any trauma, of and involved infever, the history proteolytic regulation of va clots. On exam, right thigh was enlarged, erythematous, tivesignificantly peptides. Debiec et al described a mother w warm and tender to touch. Peripheral pulses, strength, sensation and mutation preventing NEP expression who had fo reflexes were intact. Labs showed acute drop in hb to 6.6 g/dl from anti-NEP antibodies due to fetomaternal alloi 10.1 g/dl, CK 1613 U/L, platelets 326K, INR 1.1, with normal metabolic profile. USG Doppler was negative DVT. CT miscarriage; scan nization fromforaany previous these antib showed 28cm and 20cm intramuscular withinplacenta rectus were tohematomas cross the and cause subepit femoris and Sartorius. Work up including coomb’s test, haptoglobin, deposits in the fetal kidney of a subsequent bilirubin, G6PD, ANCA, ANA, HIV, Hep B/C was normal. EGD and nancy. M-type colonoscopy were negative for acute blood loss. Onphospholipase review of records, A2 receptor (PL patient was having worsening of anemia being started was laterafter identified asonthe major autoantigen fo 5 facial sirolimus, and was the causemary of acute MN muscle hemorrhage in adults.and Little literature exists tearing. With blood transfusions, leg elevation and switching sirolimus pregnancy outcomes in patients with nephrotic to tacrolimus, patient had significant improvement in ambulation and drome due to primary MN, with no data ava stabilization of hemoglogin. To our knowledge, this is the first reported case of acute about pregnancy in PLA2R-associated disease intramuscular hemorrhage with mTOR inhibitor. Thebelieve aim of thisto casebe the first known ca present what we report was to emphasize an important adverse event linked to Sirolimus pregnancy in a patient with PLA2R-associated therapy. Caution should be used when starting an mTOR inhibitor in who was seropositive for anti-PLA2R autoantib patients already taking anticoagulants and NSAIDS. throughout the course of her pregnancy. Am J Kidney Dis. 2016;67(5):A1-A118
Case Report 229 PREVALENCE AND RISK FACTORS OF NEUROPSYCHIATRIC DISORDERS IN A COHORT OF END STAGE RENAL DISEASE PATIENTS ON MAINTENANCE HEMODIALYSIS: MOUSSALY Elias, SYED Rafeel, NASR Patricia, REHMAN Amina, EL-DOUEIHY Youssef, SAAD Marc, EL-CHARABATY Elie, EL-SAYEGH Suzanne Staten Island University Hospital – Staten Island, NY Some studies suggest that one in three patients on hemodialysis have some level of cognitive impairment. The cumulative burden of depression and dementia is poorly understood in the U.S. dialysis population. The Montreal Cognitive Assessment Questionnaire (MoCA) is a novel cognitive assessment test validated in the hemodialysis population and found to be a sensitive and rapid tool to detect mild cognitive impairment. We aimed to study the prevalence and correlates of cognitive impairment and depression in our dialysis population, excluding any patient on psychoactive medications, history of brain disease or substance abuse. Out of 177 in -center ESRD patients on maintenance hemodialysis, we screened 76 patients for participation in the study, 30 patients did not complete the intervention. A study investigator administered the MoCA and BECK questionnaires during a hemodialysis session to screen for cognitive impairment and depression respectively .To investigate for correlates, demographic and clinical data including common laboratory and dialysisrelated variables were collected and analyzed. In our cohort of 46 patients, mean age was 58.5 years [19; 96], 71.7% were male, 73% had a high school degree or higher and ethnicity was diverse (41 % white, 30 % African American and 28.2 % minorities). Only 5 patients had normal cognitive function (MOCA score above 27), with a median score of 23 suggestive of some form of cognitive impairement. Thirteen percent of the patients had BECK scores suggestive of clinical depression, while 32.6% of the patients had mild mood disturbance. In the multiple linear regression predictive analysis; age, the BECK score, and ferritin levels correlated negatively with the MOCA score (respective p -values: 0.06, 0.011, and 0.007). The fact that ferritin is inversely associated with cognitive function is a novel finding , needs further investigation and can be explained by inflammation, iron overload or intractable anemia. In a patient -centered, multi-disciplinary care model for the dialysis patient, it is imperative to better understand these neuropsychiatric disorders so as to improve quality of life, adherence to medical regimen, and ultimately, survival.
230 REVERSAL OF DIALYSIS DEPENDENT RENAL FAILURE AFTER RENAL ARTERY STENTING: A CASE FOR INTERVENTION 1 2 1 1 1 Azam Mughni , Ravi Dhanisetty , Avrum Gillespie , Jean Lee , Section 2 of Nephrology, Division of Vascular Surgery, Temple University Hospital, Philadelphia, PA, USA Despite 2 recent renal artery stenosis (RAS) trials that showed no benefit of angioplasty and stenting over medical management alone, controversy exists regarding case selection as patients who had acute renal failure or recurrent flash pulmonary edema from RAS were excluded from these trials. We present the case of an 83 year old male with progressive renovascular CKD IIIb (serum creatinine 1.71 mg/dL and 2.74 mg/dL four months and two months prior to presentation respectively) who presented with worsening dyspnea and acute kidney injury (creatinine 4.5mg/dl). He had 3 recent admissions for pulmonary edema and an ultrasound obtained on the last admission revealed an atrophic left kidney (with left renal artery stent placed 12 years before) and >60% occlusion of the right renal artery with preserved kidney size. The patient was edematous, hypoxic and with chest x-ray showing pulmonary edema. Despite a systolic blood pressure under 150 mmHg with 4 anti-hypertensive agents, he did not respond to high dose diuretics and hemodialysis was initiated for volume overload and uremic encephalopathy. By hospital day 15, the patient was medically stable but his oliguric renal failure persisted. Renal angiogram was then performed which showed high grade stenosis at the origin of right renal artery. The artery was then stented with subsequent angiogram showing resolution of the high grade stenosis. The urine output began to improve and dialysis was stopped 5 days later. The patient was then discharged on no BP agents. One month after discharge, creatinine was 1.28mg/dl but he requires 2 BP agents. Our case illustrates the benefit of revascularization with dramatic improvement in renal function. While recent trials suggest medical management for most cases of RAS, renal angioplasty with stenting should be considered in the presence of high grade RAS in the setting of pulmonary edema and acute renal failure necessitating dialysis.
A76
231
NEPHROCALCINOSIS AND RENAL CALCULI IN A PATIENT WITH CHRONIC HYPOPHOSPHATEMIC RICKETS Venkat Ram Rakesh Mundra, Dana Rizk, University of Alabama, Birmingham, AL, USA 1, Laith Al-Rabadi, 22 year old male patient with history of childhood rickets, presented to MBBS, * Rivka our Nephrology clinic with complaints of passing multiple small stones Jennifer E. Ballard, MD,2,y Alan in the urine. He was diagnosed with rickets at the age of 10 because of 1 locked knees and underwent bilateral knee surgery. His workDavid up at thatJ. Salant, MD, time revealed hypophosphatemia and he was treated with oral phosphorus and vitamin D supplements. He had normal growth and is little information about pregnancy o development. His medications were stoppedThere at age 19. In clinic, his physical exam was unremarkable. His labs showed normal especially thosecreatinine, with circulating autoantibod pg/ml) andinurinary low serum phosphorus (1.5 mg/dl), PTH (2.5 autoantigen primary MN. We present what phosphate wasting (FEPhos-29%). His serum 1, 25 dihydroxy vitamin a 39-year-old woman with PLA2R-associate D level and 24 hour urinary calcium were high (urinary calcium 283 anasarca, hypoalbuminemia mg/day). The diagnosis of “Hereditary Hypophosphatemic Rickets with (albumin, 1.3-2. opsyofrevealed MNand with staining for PLA2R, a Hypercalciuria” (HHRH) was made. CT scan the abdomen She calculi, did notmild respond to conservative therapy a pelvis revealed bilateral non obstructing renal increased density of renal pyramids consistent withSeveral nephrocalcinosis and bilateral weeks after presentation, she was fou renal cysts (three in each kidney). His stone analysis revealed 80% further immunosuppressive treatment. Protei calcium phosphate and 20% calcium oxalate. Patient was restartedRonlevels declined but w Circulating anti-PLA 2 oral phosphate supplementation without any vitamin D. without proteinuria at birth or at her subseque HHRH is a rare autosomal recessive disorder caused by mutations in had detectable circulating anti-PLA2R of imm SLC34A3, the gene encoding the renal sodium-phosphate colow titers. Only trace amounts of IgG4 ant transporter NaPi-IIc. It is clinically characterized by rickets, discrepancy between anti-PLA2R levels in th hypophosphatemia, phophaturia, hypercalciuria and high 1Am J Kidney Dis. 67(5):775-778. ª 2016 by 25dihydroxy vitaminD. Phosphate supplementation is the main stay of therapy. Beyond the musculoskeletal complications, it is important to INDEX WORDS: Membranous nephropathy ( recognize the potential renal manifestations of this disease including receptor (PLA2R); autoantibody; placenta; ritu nephrolithiasis and nephrocalcinosis. To our knowledge, renal cysts have not been reported with this disorder and remain an unexplained finding. Treatment of renal complications is not well established. The role of phosphate supplementationregnant to the end patients remains to be determined. with autoimmune disease
Pregnancy in a Patient Wit and Circulating Anti-P
P
deliver newborns with a spectrum of cl manifestations due to the transplacental passa 232 circulating autoantibodies. Pregnant patients SIROLIMUS CAUSING ACUTE QUADRICEPS HEMATOMA: lupus or myasthenia gravis can deliver babies A RARE COMPLICATION OF MTOR INHIBITORS: Sumeet Munjal, Fatima Najeeb, Jyotsna Bhatnagar, University at Buffalo, corresponding disease in the neonate.1,2 Neo New York, USA membranous nephropathy (MN) not associated mTOR inhibitors are used as potent immunosuppressive agents in congenital infection was first described in 199 solid organ transplant recipients and their properties are linked to attributed diabetes, to theaspassive transfer of maternal progression of proteinuria, hyperlipidemia, well as anemia. 3 Muscle hematomas are seen bodies in athletes,topatients on anticoagulants, but putative renal antigens. More than a d 4 rarely occur spontaneously. We report a case ofet severe into the first antigen inv later, Debiec al bleeding identified quadriceps and sartorius muscle in a patient taking Sirolimus. in such cases as neutral endopeptidase (NE 59 year old male with PMH of renal transplantation secondary to metalloprotease present on the surface of the pod PCKD, HTN, CAD, presented with complaints of right calf swelling and pain for one week. Patient denied any trauma, of and involved infever, the history proteolytic regulation of va clots. On exam, right thigh was enlarged, erythematous, tivesignificantly peptides. Debiec et al described a mother w warm and tender to touch. Peripheral pulses, strength, sensation and mutation preventing NEP expression who had fo reflexes were intact. Labs showed acute drop in hb to 6.6 g/dl from anti-NEP antibodies due to fetomaternal alloi 10.1 g/dl, CK 1613 U/L, platelets 326K, INR 1.1, with normal metabolic profile. USG Doppler was negative DVT. CT miscarriage; scan nization fromforaany previous these antib showed 28cm and 20cm intramuscular withinplacenta rectus were tohematomas cross the and cause subepit femoris and Sartorius. Work up including coomb’s test, haptoglobin, deposits in the fetal kidney of a subsequent bilirubin, G6PD, ANCA, ANA, HIV, Hep B/C was normal. EGD and nancy. M-type colonoscopy were negative for acute blood loss. Onphospholipase review of records, A2 receptor (PL patient was having worsening of anemia being started was laterafter identified asonthe major autoantigen fo 5 facial sirolimus, and was the causemary of acute MN muscle hemorrhage in adults.and Little literature exists tearing. With blood transfusions, leg elevation and switching sirolimus pregnancy outcomes in patients with nephrotic to tacrolimus, patient had significant improvement in ambulation and drome due to primary MN, with no data ava stabilization of hemoglogin. To our knowledge, this is the first reported case of acute about pregnancy in PLA2R-associated disease intramuscular hemorrhage with mTOR inhibitor. Thebelieve aim of thisto casebe the first known ca present what we report was to emphasize an important adverse event linked to Sirolimus pregnancy in a patient with PLA2R-associated therapy. Caution should be used when starting an mTOR inhibitor in who was seropositive for anti-PLA2R autoantib patients already taking anticoagulants and NSAIDS. throughout the course of her pregnancy. Am J Kidney Dis. 2016;67(5):A1-A118