Sunday, February 27, 2011, 7:30 PM
CC 008 A/B
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Crescents, Necrosis, and Thrombi, Oh My!
Cedars-Sinai Medical Center
Los Angeles, CA
In accordance with ACCME guidelines regarding disclosure, the USCAP policy requires that faculty members who have a significant financial or other relationship with a commercial company, entity, or service (which will be discussed in this Symposium) must disclose this to attendees. The Academy also requires that speakers disclose any products that are not labeled for the use under discussion. The speakers listed below have indicated they have nothing to disclose.
Ginette Lajoie-Starkell, Brampton Memorial Hospital, Brampton, Ontario, Canada
Isaac E. Stillman, Beth Israel Deaconess Hospital, Boston, MA
Lois J. Arend, Johns Hopkins Hospital, Baltimore, MD
Franco Ferrario, San Gerardo Hospital and Milan Bicocca University, Milan, Italy
Marcello Franco, Escola Paulisto Medicina, Sao Paulo, Brazil
Clinical histories are displayed below.
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Submitted by: Ginette Lajoie -
A previously well 53-year-old Caucasian man was referred from a medical clinic to the emergency department for severe hypertension (systolic blood pressure > 200 mm Hg) and significant pitting edema. In the emergency, investigation revealed haemoglobin of 146 g/L (14.6 g/dL), white blood count of 6.9 X109/L (6.9 x 103/µL), and platelet count of 290 X109/L (290 x 103/µL). The creatinine was 103 µmol/L (1.17 mg/dL), and urea 3.2 mmol/L (8.9 mg/dL), with potassium of 3.7 mmol/L (3.7 mEq/L). Urine dipstick showed 3+ protein and no blood. A CT-scan of the head demonstrated microangiopathic changes, and a chest X-ray showed a widened mediastinum suggestive of malignancy or sarcoidosis. The patient was discharged home with Coversyl 8 mg/daily and hydrochlorothiazide 25 mg daily. A follow-up visit was arranged 4 days later. At that time, the blood pressure was 171/109 mm Hg. Hydrochlorothiazide was discontinued, and he was prescribed Furosemide 20 mg daily. A referral was made to respirology for possible sleep apnea. The family physician saw the patient subsequently. He was initiated on metoprolol 50 mg twice daily, Coversyl 16 mg daily, and Lasix 20mg daily. Additional tests revealed an unchanged creatinine of 106 µmol/L (1.20 mg/dL) with an eGFR of 63 mL/min. The patient was referred to a nephrologist. At the first visit, it was established that the patient’s past medical history was limited to a history of smoking discontinued the previous year, and 3 to 4 years history of previously untreated arterial hypertension. Specifically, he had no history of coronary artery disease, cardiac heart failure, diabetes, or stroke. On review, there was no suggestion of systemic symptoms with no rash, arthritis, weight loss, hair loss, oral ulcers, fever, chills, or sweats. He admitted to a history of snoring and daytime fatigue without witnessed apnea. There was no relevant familial history. Examination revealed obesity, blood pressure of 180/110 mm Hg, normal heart sounds with a soft S4. Lasix was increased to 20 mg twice daily, and he was started on Doxazosin 2 mg daily. At a subsequent visit to the nephrologist’s office, additional laboratory results were available for review: normal complete blood count, creatinine 103 µmol/L (1.17 mg/dL), fasting blood glucose 6.4 mmol/L (115.3 mg/dL), Hb A1C 0.061 mmol/L (6.1 %), potassium 3.8 mmol/L (3.8 mEq/L), calcium 2.03 mmol/L (8.12 mg/dL), phosphorus 1.04 mmol/L (3.2 mg/dL), albumin 27 g/L (2.7 g/dL), uric acid 464 µmol/L (7.8 mg/mL), total cholesterol 11.38 mmol/L (439.4 mg/dL), normal serum and urine protein electrophoresis, and negative serology for ANA, ds-DNA, rheumatoid factor, HIV, and hepatitis B. Serum complement level was normal (C3 1.60 g/L (160 mg/mL), C4 0.36 g/L (36 mg/mL)). A 24-hour urine collection revealed 14 g protein/day. The patient’s serum was positive for Hepatitis C antibodies for which he was referred to a hepatologist by the family physician. On further testing, negative RNA test for Hepatitis C was found indicating previous exposure to the virus but no active infection. A CT-scan of the chest demonstrated extensive mediastinal and hilar adenopathy, with extensive reticulonodular changes throughout the lungs. Antihypertensive medications were adjusted to Micardis 80 mg once daily, Lasix 40 mg twice daily, and Doxazosin 4 mg twice daily. A renal biopsy was considered but deferred until pulmonary issues were clarified. The patient was assessed by respirology and thoracic surgery. A mediastinoscopy and bronchoscopy were completed. The bronchoscopy was normal. Biopsy of the enlarged mediastinal lymph nodes was obtained. The mediastinal lymph node biopsy results supported a presumed clinical diagnosis of sarcoidosis. In view of the lung parenchymal involvement, normal calcemia, and absence of significant symptoms, no specific treatment was recommended for sarcoidosis. A renal biopsy was performed, 6 months after the initial presentation.
Submitted by: Isaac Ely Stillman -
63 year-old man presented to an outside hospital with malaise. He reported taking Aleve 800 mg q6 for the past 4-5 weeks as well as ibuprofen 800 mg daily PRN for muscle pain. Stated that his urine was occasionally pink-red over the past 2 weeks. He was found to be oliguric, with a SCr of 6, and a BUN of 58. UA showed pyuria, large blood and large protein. Renal ultrasound revealed a right staghorn calculus with moderate hydronephrosis. Ureteral stent was placed for presumed obstruction. Follow-up ultrasound showed improvement in the hydronephrosis, but the serum creatinine remained unchanged. Hyperkalemia developed, and hemodialysis was initiated. Transferred to BIDMC for further evaluation. Past Medical History: Obesity and hypertension. Bilateral nephrolithiasis (staghorn calculus on right, non- obstructing stones on left) Chronic kidney disease ( baseline Serum creatinine 1.6 - 1.8 (eGFR 41 ml/min).
CBC: WBC 8.4, Hgb 9.6, Hct 29.1, Plt 217
Chemistries: Na 136, K 4.9, Cl 99, HCO3 35, Creatinine 5.7, BUN 61 Glucose 103, Ca 8.6, Mg 2.3, PO4 6.1 RF 30 CRP 115.8 Hepatitis B & C Negative ANA and dsDNA Negative Complements Within normal limits Pr / Cr = 4.2 Dipstick 3+ protein, 3+ blood, 1-2+ esterase, pH 6.0
Microscopy: Numerous RBCs, WBCs, few granular and RBC casts, positive for dysmorphic RBCs and eosinophils
Submitted by: Lois J. Arend -
History of Present Illness: 73-year-old white male was healthy until 2006 when he was diagnosed with rhinosinusitis. Since then, he has had recurrent sinus infections treated on multiple occasions with antibiotics and a regimen of steroids. He also has a history of BPH. In mid August 2010 he complained of severe sore throat and was hospitalized and treated with antibiotics. He was not informed of any kidney abnormalities. After discharge, the patient started developing lower extremity edema and shortness of breath, along with weight gain. The patient was started on diuretics at that time with significant improvement. Ten days after the patient was started on diuretics, he was admitted to an outside hospital for shortness of breath. On admission, he was febrile and diagnosed with pneumonia and left-sided pleural effusion. He was also found to have leukocytosis and significant weight gain. He was noted to have a creatinine of 2.39 mg/dL that improved to 1.7 mg/dL upon discharge, and after having been given steroids. The patient was started on broad-spectrum antibiotics initially that were switched to Zosyn afterwards. His leukocytosis improved on antibiotics. During his stay, the patient was found to have mild hematuria. After discharge the patient's creatinine improved to as low as 1.3 mg/dL. Repeat labs on follow-up one month later showed a creatinine of 3.4 mg/dL with an estimated GFR of 17. Review of Systems: He denies any shortness of breath. He has had difficulty controlling his blood pressure, systolic ranging in the 150s to 160s. His edema has improved significantly on the diuretic. Otherwise, his review of systems is negative.
Medications: Bumetanide, Carvedilol, Famotidine, Levaquin, Terazosin, Avodart
Physical Exam: BP 156/80 sitting, pulse 65; 167/82 standing, pulse 65 RR 16; temperature 36.5; weight 85.9 kg Lower extremities 2+ edema bilaterally.
Other Labs: Negative for anti-GBM, ANCA, MPO, PR3, ASO, rheumatoid factor, ANA, and hepatitis C3 58, C4 6, both low. Proteinuria by ratio 576 mg/24 hr
Ultrasound: 12.2-cm right and 13.1-cm left, no hydronephrosis
Submitted by: Franco Ferrario -
CASE 1 A 35 years old Caucasian male with history of 10 years of insulin-dependent Diabetes Mellitus. He was admitted to our Nephrology Department for overt nephrotic syndrome (proteinuria 7,5 gr/24h - serum albumin 1,8 gr/dl),severe renal insufficiency (S.Creatinine 3,7 mg/dl)and severe anemia (Hb 6,9 gr/dl). He developed an oliguric rapid progression of renal insufficiency (S.Creatinine 10,1 mg/dl) necessitating haemodialysis. Serological tests for autoimmunity were negative (ANA,ENA,RF,anti ds.DNA,C3,C4,Immunoglobulin assay,Cryoglobulins and ANCA. Chest X-ray:Normal. Renal ultrasound examination:Normal Kidneys. Fundoscopic examination:proliferative Diabetic retinopathy. A renal biopsy was performed(see images). 15 days after renal biopsy the patient developed clinical syndrome characterized by shortness of Breath,fever,cough and episode of hemophthysis. CASE 2: A 69 years old Caucasian male with history of 4 years of insulin-dependent Diabetes Mellitus and Hipertension. He was admitted to our Nephrology Department for mild proteinuria(1,5 gr/24h)with normal renal function. All serological tests for autoimmunity were negative. Renal ultrasound examination:Normal Kidneys. Funduscopic examination:Diabetic retinopathy. A renal biopsy was performed(see images).
Submitted by: Marcello F. Franco -
A 65 y/o woman, in chronic renal insuficiency due to diabetic nephropathy, was under hemodialysis for
3 years. PRA pannel negative. She was then submitted to a renal transplantation, receiving a cadaveric
kidney (65 y/o man who died of intraparenchymatous cerebral hematoma, with a final creatinine=1.3.
A pre-implant bx revealed 15% of global glomerulosclerosis and moderate hypertensivenephrosclerosis.
The initial suppressive regimen included thymo, tacrolimus, pred and azathioprine.
She presented DGF and a tx biopsy at Day 8 revealed features consistent with severe acute vascular
rejection (Banff 2009: Type III).
As the patient was old, presented several co-morbid conditions, as hypertension and obesity, the clinical
option was not to treat the acute rejection and to proceed with the graft excision.
The histology of the explant showed severe vascular lesions which raised the differential between:
i) Severe vascular rejection; ii) Uremic hemolytic syndrome (UHS) and iii) Rejection evolving with UHS.
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