FOUR-YEAR FOLLOW-UP OF A COHORT OF PATIENTS WITH ADVANCED CKD MANAGED IN AN ACADEMIC CKD CLINIC: GROUNDS FOR OPTIMISM. Andres Serrano, Jenny Huang, Laura Nishi, Rajani K. Chilakapati, Cybele Ghossein, James Paparello, Shubhada Ahya, Neenoo Khosla, Robert Rosa, Melissa Weitzel, Daniel Batlle. Division of Nephrology/Hypertension, Feinberg School of Medicine of Northwestern University, Chicago IL, USA.

Individuals with Chronic Kidney Disease (CKD) stages 4 and 5 have a high mortality rate and progress rapidly to renal replacement therapy. We report an extended follow up of a cohort of patients with advanced CKD with the main objective of examining the mortality rate and the proportion of patients in whom the Glomerular Filtration Rate (GFR) can be maintained stable despite advanced CKD.

A total of 102 unselected CKD patients consecutively referred for anemia management were prospectively followed up for four years. 71 subjects (69.6%) were stage 4 and 31 subjects (30.4%) were stage 5 CKD. Time 0 was defined as the time of initiation of erythropoietin therapy. The primary outcomes measured were the initiation of renal replacement therapy (dialysis or transplantation) or death.

At the end of follow up 77 subjects (75.5%) had developed a primary outcome: 61 initiated renal replacement therapy (54 dialysis, 7 transplantation) and 11 died. In the 25 subjects (24.5%) who did not develop a primary outcome the GFR remained stable over the follow up period (23.5+4.36 at time 0 vs. 21.7+11.23 ml/min/1.72 m2 at the end of follow up, p=0.38). There were no differences in the hemoglobin levels and blood pressure levels at the beginning of follow up between groups. The GFR at baseline was associated with adverse outcome (23.5+4.36 vs. 17.4+5.24 ml/min/1.72 m2 in subjects without and with outcomes, respectively, p<0.001). Only 11 subjects died during the follow up period, which represents a standardized mortality rate of 4.59 per 100 person-year. This rate is much lower than previously reported.

We conclude that comprehensive nephrological care in patients with advanced CKD may have a positive impact on mortality rate and delays the initiation of renal replacement therapy. Moreover, in about 25% of patients the GFR remains stable despite advanced CKD.

197

A RARE CASE OF DIABETES INSIPIDUS, PRE-ECLAMPSIA AND ACUTE FATTY LIVER IN PREGNANCY Aastha Sethi,Surafel Gebreselassie; Wayne State University, Detroit, MI, U.S.A. Background: Diabetes insipidus (DI) is a condition in which abnormal secretion; degradation or activity of vasopressin causes polyuria, polydipsia, and electrolyte abnormalities. Transient DI may rarely occur during late pregnancy or immediate puerperium, the prevalence being 1 in 300,000 pregnancies. If unrecognized, it may cause neurologic injury and threaten the lives of the mother and fetus. Case: A 19- year-old female patient (G3P1A1) at 28 weeks of gestation was admitted because of the risk of preterm delivery and suspicion of pre-eclampsia. It was a known gemellar (twin) pregnancy with suspected twin-twin transfusion. An ultrasound (USG) revealed a biophysical profile of 10/10 for both twins. She was given magnesium tocolysis to prevent pre-term labor. On the next day, she developed nausea, vomiting and frequent contractions. She was transferred to the labor and delivery unit. Labs were significant for proteinuria, hypernatremia and elevated liver enzymes. Subsequently, she became extremely thirsty and her urine output increased to 300 ml/hr with a very low specific gravity. Lab evaluation revealed high plasma osmolality (348mosm/kg), hypernatremia (168meq/l) and low urine osmolality (108mosm/kg). Repeat USG showed intra-uterine death of both the fetuses. She was subsequently induced. Treatment with desmopressin was initiated with improvement in patient’s symptoms and lab parameters. Discussion: An increase in thirst threshold occurs in pregnancy due to reduced secretion and increased placental degradation of vasopressin by vasopressinase. In this patient, the gemellar nature of pregnancy could have induced excessive vasopressinase activity, and the hepatic dysfunction (acute fatty liver) could have reduced the catabolism of vasopressinase. Conclusion: Our patient had transient DI with subsequent fetal loss. We emphasize that pregnancies with transient DI should be considered at high risk because of association with pre-eclampsia and fatty liver.

198

NEPHROTIC SYNDROME ASSOCIATED WITH CHRONIC LYMPHOCYTIC LEUKEMIA. Saeed Shaffi, Khalid Bashir, William Hunter, Creighton university medical center, Omaha, Nebraska. We present a case of nephrotic syndrome from minimal change disease (MCD) in a patient with chronic lymphocytic leukemia (CLL). Nephrotic syndrome is rarely associated with CLL and the predominant underlying pathology is Membranoproliferative glomerulonephritis (MPGN). We hypothesize that nephrotic syndrome from MCD in CLL is a paraneoplastic phenomenon. A 43 years old male presented with chief complaint of swelling of lower extremities of 6 weeks duration. He was diagnosed with CLL 10 years ago. He underwent chemotherapy 7 years after diagnosis and was in chronic remission at the time of presentation. Physical examination showed a weight gain of 20 pounds and blood pressure of 140/70. He had splenomegaly, lymphadenopathy, 3 + lower extremity pitting edema with no jugular venous distension and regular heart sounds. Labs showed a WBC of 4.9, platelets 220 with 26 % lymphocytes, Na 137 mEq/L, Bun 13 mg/dL, creatinine 1.3 mg/dL, total protein 4.4 g/dL, albumin 2.3 g/dL, cholesterol 423 mg/dL, LDL 329 mg/dL and BNP 18. Urinalysis showed proteinuria with no RBC`s and casts. Urine protein/creatinine ratio was 11. P ANCA, C ANCA and ANA were normal. Anti dsDNA, RPR, HIV ELISA, Hepatitis C antibodies, HepBsAg and Anti HBsAg antibodies were non reactive. Serum protein electrophoresis did not show an M spike. He was started on Lisinopril. Kidney biopsy was consistent with MCD. He was started on Prednisone with normalization of protein creatinine ratio. The frequency of nephrotic syndrome in CLL is less than 1 %. At least 46 cases of nephrotic syndrome in patients with CLL have been reported. MPGN is the predominant renal pathology. So far only 5 cases of MCD with CLL have been reported. The exact pathogenetic mechanism is not clearly known. CLL is a disorder of B cells. It is now recognized that there are T cell abnormalities in CLL including increase in the number of suppressor T cells. MCD may be related to excessive suppressor T cell activity. In addition T cell disorder can result in increase in soluble factors that alter glomerular permeability. It has also been postulated that patient with low expression of CD 5 positive cells develop MCD instead of MPGN.

199

A CROSS SECTIONAL STUDY OF COMPLIANCE WITH K/DOQI CLINICAL PRACTICE GUIDELINES FOR CHRONIC KIDNEY DISEASE IN AN URBAN TEACHING CLINIC. Shahzad Shafique; University of Connecticut, Farmington, USA.Background: The objective of the study was to determine the compliance of residents with K/DOQI clinical practice guidelines in a teaching clinic and to generate a novel strategy to improve it. Methods: We performed a cross-sectional analysis of 83 patients with diagnosis of CKD for the period “Jan 2005-Dec 2005”. The charts were reviewed to determine whether K/DOQI goals were met for: 1) assigning stage of CKD and estimation of GFR, 2) assessment of proteinuria, 3) BP control, 4) evaluation of metabolic bone disease by Ca, PO4, and PTH measurement, 5) lipids management, 6) timely nephrology referral, 7) interventions to slow the progression of the disease, and 8) evaluation and treatment of anemia, and nutritional status. Results: In these 83 patients the mean Scr was 1.9 mg/dl, and eGFR was 38.3 ml/min. We found failure to assign the stage of the disease in 71 (86%). Proteinuria was assessed in 23 (28%) of all. Among 73 (88%) patients with recorded history of HTN only 28 (38%) showed optimal control. 25 (30%) were anemic but evaluation of anemia was done only in 7 (28%). Nutritional status was assessed only in 9 (11%) and no one was referred to a dietician. The work-up for evaluation of metabolic bone disease was frequently missing: PTH levels were obtained in only 3 (3.6%) and Ca in 19 (23%) and PO4 in 15 (18%). Lipid profile was ordered in 58 (69%) and found to be abnormal in 74%. The nephrology referral was initiated only in 17 (20%) patients. Conclusion: The study concluded that K/DOQI goals are achieved in only a small proportion of patients cared for in a teaching clinic. The breadth of the problem suggests that both systematic and educational barriers impair translation of K/DOQI guidelines into clinical practice and to achieve optimal disease specific management outcomes. On the basis of this study “Kidney Early Evaluation and Protection” (KEEP) flow sheets were successfully introduced to increase the compliance rate with the above guidelines.

200

NKF 2007 Spring Clinical Meetings AbstractsA74