NKF 2014 Spring Clinical Meetings Abstracts

Am J Kidney Dis. 2014;63(5):A1-A121

DELAYED PRESENTATION OF SARCOIDOSIS AFTER DIAGNOSIS OF FIBRILLARY GLOMERULONEPHRITIS Robin Shah,1, Joseph Kremer2 1. The Christ Hospital Health Network, Cincinnati, OH, USA 2. The Kidney and Hypertension Center, Cincinnati, OH, USA Fibrillary glomerulonephritis (FGN) is a rare form of glomerular disease that is frequently associated with malignancy, monoclonal gammopathy, and autoimmune disease. It has a poor outcome, leading to end-stage renal disease (ESRD) within two years. We report a case of a 62-year-old Caucasian female with a history of hypertension who presented due to leg swelling and arthralgias. She was found to have acute kidney injury (AKI) with a serum creatinine of 1.5 mg/dL, which continued to climb to 2.0 mg/dL after two weeks. Patient also developed hematuria and nephrotic range proteinuria of 8 grams over 24 hours. Extensive workup was negative. Renal biopsy was performed showing early fibrillary glomerulonephritis. Patient progressed to ESRD and was started on dialysis within 6 months of diagnosis. Approximately two years after initial diagnosis, patient developed worsening arthralgias, fevers, nausea, vomiting with leukocytosis of 16,300 and hypercalcemia of 11.1. Examination and imaging studies revealed lymphadenopathy. Lymph node biopsy revealed non-caseating granulomas, suggestive of sarcoidosis and patient was started on Plaquenil. Fibrillary glomerulonephritis is thought to be secondary to deposits of immunoglobulins. It is diagnosed on electron microscopy. FGN can be associated with malignancy, monoclonal gammopathy, or autoimmune disease; however, temporal relationship can vary. As in this case, sarcoidosis was diagnosed after presentation of FGN. Thus, it is emphasized that patients with FGN be under repeated surveillance for other associated causes.

URGENT INITIATION OF PERITONEAL DIALYSIS: A SAFE AND VIABLE ALTERNATIVE. Andres Serrano, Mount Sinai Hospital, Chicago, IL, USA. Most of the patients who are in urgent need of renal replacement therapy are initiated on hemodialysis through a central venous catheter. In the majority of the cases peritoneal dialysis is never offered because of misconceptions about peritoneal dialysis, and fear of complications related to urgent initiation of this modality. I am presenting my experience in a group of patient who had urgent initiation of peritoneal dialysis. Between November 2008 and November of 2013 a total of 41 patients initiated peritoneal dialysis at Mount Sinai Hospital in Chicago. The average age of these patients was 50.9 years. Twenty-three patients were women (56%), and the majority of patients were Hispanics (29 or 70.7%). The average serum creatinine at initiation of dialysis was 8.4 mg/ml (eGFR 7.6 ml/min/1.73 m2). The most common reported causes of End-Stage Renal Disease (ESRD) were diabetes mellitus (44%), unknown (27%) and glomerular disease (15%). 17 of 41 patients (41.5%) had an urgent initiation of peritoneal dialysis. Compared to patients who had a planned peritoneal dialysis initiation, this group of patients was slightly younger (47.5 years v. 53.4 years) and had a higher proportion of patients with unknown etiology of ESRD (50% v. 16%). Additionally, a significant number of patients (8 or 57%) did not have a nephrology evaluation prior to be diagnosed with ESRD. Patients who had an urgent peritoneal dialysis initiation had a higher serum creatinine (10.2 v. 7.2 mg/ml), a lower eGFR (6.8 v. 7.5 ml/min/1.73 m2), and had a shorter average time between peritoneal dialysis catheter insertion and initiation of dialysis (15 v. 36 days). During the first 30 days of dialysis there were no mechanical complications or peritonitis in the urgent initiation group, compared to 2 cases of peritonitis in the group of patients who had a planned initiation of peritoneal dialysis. In conclusion, urgent initiation of peritoneal dialysis is a safe and viable alternative for patients who are in need of renal replacement therapy.

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CENTRAL DIABETIC INSIPIDUS DUE TO HERPES SIMPLEX ENCEPHALITIS IN A MULTIPLE SCLEROSIS IMMUNOSUPRESSED PATIENT Silvi Shah, Juan Calle, Martin Schreiber, Cleveland Clinic, Ohio Major causes of central diabetic insipidus (CDI) are neoplastic or infiltrative lesions of pituitary and hypothalamus; and severe head trauma. CDI following viral infections in setting of immunosuppressive state is rare. We report a case of CDI resulting from herpes simplex encephalitis (HSE) in a 38-year-old male with multiple sclerosis. 38-year-old male was admitted to the hospital with new onset generalized tonic clonic seizure and 1-week history of fever, photophobia and headache. Past medical history was significant for multiple sclerosis that was being treated with natalizumab for the last four months. On admission blood pressure was 94/65 mmHg and temperature was 39.2 ºC. CSF showed lymphocytic pleocytosis with PCR positive for herpes simplex virus 1. Brain MRI revealed hyper intensity in the area of left temporal lobe and left hippocampus, diffuse stable white matter changes and absence of posterior pituitary “bright spot”. Diagnosis of HSE was made and patient was started on acyclovir. Four days later he developed polyuria with 24-hour urine output of 4.3L/day and elevated creatinine of 3.1 mg/d (baseline creatinine 1.1 mg/dl). Serum sodium was 152 mmol/L, serum osmolality was 300 mOsm/kg and urine osmolality was 106 mOsm/kg. A diagnosis of CDI was made based on hypotonic polyuria, hypernatremia and absence of posterior pituitary “bright spot”. Kidney injury was attributed to acute tubular necrosis (ATN) in setting of hypotension on admission. Desmopressin was started at 1 mcg intravenously twice a day. Serum sodium normalized to 145 mmol/L and urine osmolality increased to 405 mOsm/kg within 3 days of starting desmopressin. Persistent Polyuria was likely due to post ATN diuresis and resolved after 2 weeks along with serum creatinine. Patient is currently being followed in an outpatient clinic and is on oral desmopressin. CDI resulting from HSE is rare and is believed to be due to destruction of neurons responsible for production of vasopressin. Clinicians should be aware of this complication in patients’ diagnosed with HSE especially in the setting of immunosuppressed state.

SUCCESSFUL MEDICAL MANAGEMENT OF RECURRENT IDIOPATHIC RETROPERITONEAL FIBROSIS Silvi Shah, Juan Calle, Cleveland Clinic, Ohio Idiopathic retroperitoneal fibrosis (IRF) is a rare cause of acute renal failure due to obstructive uropathy. Optimal medical management of acute kidney injury due to IRF is controversial. We present a case of 64-year-old male with IRF successfully treated with high dose steroids and ureterolysis with complete recovery of kidney function. 64-year-old Caucasian male presented to the clinic for management of biopsy proven IRF incidentally diagnosed during evaluation of worsening back pain. Past medical history was significant for hypertension. Vitals and physical examination were normal. Laboratory evaluation revealed elevated creatinine of 1.95 mg/dl with baseline creatinine of 0.8mg/dl. IgG4 was normal and screening for cancer was negative. CT scan of abdomen/pelvis revealed 12x3x6 cm retroperitoneal soft tissue encasing abdominal aorta, bilateral iliac arteries and bilateral ureters; and moderate left hydronephrosis. Laparoscopic ureterolysis was performed in left ureter with improvement in serum creatinine to 1.25 mg/dl. However, repeat imaging after 5 months showed progressively increasing retroperitoneal mass 18.5x2.6x9.0 cm with new severe right-sided hydronephrosis and serum creatinine of 1.38 mg/dl. Right ureterolysis was performed. He was subsequently started on high dose prednisone at 1mg/kg for 8 weeks that was tapered in 8 weeks and is currently on maintenance dose of 10 mg/kg. Repeat CT scan showed marked improvement of the retroperitoneal soft tissue measuring 14x2x6.6 cm. Renal scan showed improved right renal artery proximal systolic velocity of 89 cm/s from 48 cm/s; and end diastolic velocity of 30 cm/s from 15 cm/s. Renal function recovered completely to 1.12 mg/dl. Surgical treatment of AKI due to ureteral obstruction from IRF is well known; and involves ureterolysis and ureteral stenting. Medical management along with this is necessary to prevent progression and recurrence of the disease. This case illustrates the successful treatment of recurrent IRF with course of high dose steroids after ureterolysis and should be considered as a treatment option by clinicians.

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