ACD 9/18/14

Andy Johnsrud

A ~60 yr old woman presents to the ED with chest pain and confusion

Minimally able to answer questions, you hear from EMS that chest pain has been present about 10-12 hours, confusion was more acute so someone called 911.

PE: BP 60/~, HR 55, is awake and confused/disoriented, lungs clear, s1s2 no murmurs, 2+ bilat LE edema, hepatomegaly present.What now? What

might the diagnosis be?

You give 2LNS, place pads, have a nurse draw up atropine and 1mg of epinephrine (just in case), and get an ECG

{

Rate? Rhythm? Axis? Q wave infarcts? Intervals? Hypertrophy? ST segment anomalies? Want any other type of ECG?

You diagnose sinus bradycardia with grade I AV block, voltage criteria for inferolateral MI. You ask for a right sided ECG and activate the cath lab (or order thrombolytics or call for a helicopter to a cath lab, as appropriate).

BP remains low but improves w low dose norepinephrine drip

RCA thrombus found and removed, stent placed. Pt recovers slowly over the next 72 hours.

Hospital day 3 pt is recovering slowly but edema remains quite prominent, it is also noticed that routine blood tests on admission revealed significant hypalbuminemia. No known liver, kidney, gastrointestinal, or nutritional cause for this is present.

UA and UPC reveals nephrotic range proteinuria >8gm/day, repeat albumin <2.0

Cath Lab and beyond

Could the nephrotic syndrome have caused the myocardial infarction?

In a word… yes

Nephrotic Syndrome

Some famous people with nephrotic syndrome

Linus Pauling

Alonzo Mourning

Sean Elliott

Essentials of Diagnosis

PROTEINURIA > 3.5 g/ 24 hrs (40-50 mg/kg/day)

HYPOALBUMINEMIA

EDEMA

HYPERLIPIDEMIA

LIPIDURIA

Primary renal disease -membranous nephropathy -focal segmental glomerular sclerosis (FSGS) -minimal change disease -membranoproliferative glomerulonephritis (MPGN)

In association with systemic conditions -diabetes mellitus -SLE -amyloidosis -HIV -viral hepatitis -malignancy

General Considerations

Pathogenesis

Non-inflammatory damage to the glomerular capillary wall, resulting in proteinuria due to

altered charge or size selectivity.

Complications

HYPERLIPIDEMIA – increased synthesis and decreased catabolism of individual lipid fractions

INFECTION – urinary losses of immunoglobulins, complement defects

MALNUTRITION – negative nitrogen balance 2/2 massive proteinuria , with loss of lean body mass

ANEMIA – urinary losses of EPO

THROMBOSIS – multifactorial…

Can Nephrotic Thrombophila cause an MI?

Urinary losses of antithrombin III, proteins C and S

Increase in plasma fibrinogen levels and decreased tissue plasminogen activator levels

Increase in platelet aggregability

Increase in von Willebrand factor levels

THESE ABERRATIONS LEAD TO AN INCREASED

INCIDENCE OF VENOUS AND ARTERIAL

THROMBOEMBOLISM

Management of Nephrotic SyndromeHYPERLIPIDEMIA: lipid lowering therapy

EDEMA: Loop diuretics, salt restriction

THROMBOEMBOLISM: If thrombosis occurs, it is typically treated with OAC for as long as the patient remains nephrotic. Prophylactic anticoagulation is controversial with no formal recommendations. The ATRIA tool is available for patients with membranous nephropathy.

PROTEINURIA: ACE inhibitors lower intraglomerular capillary hydrostatic pressure- NSAID’s? – possibly decreases basement

membrane permeability- low protein diet? 0.7 g protein/kg/day

decreases urinary protein excretion and improves lipid profile

Prophylactic anticoagulation in nephrotic syndrome: a clinical conundrum. AU, Glassock RJ, SO J Am Soc Nephrol. 2007;18(8):2221.

Personalized prophylactic anticoagulation decision analysis in patients with membranous nephropathy. AU Lee T, Biddle AK, Lionaki S, Derebail VK, Barbour SJ, Tannous S, Hladunewich MA, Hu Y, Poulton CJ, Mahoney SL, Charles Jennette J, Hogan SL, Falk RJ, Cattran DC, Reich HN, Nachman PH SO Kidney Int. 2014;85(6):1412.

Thrombosis in Nephrotic Syndrome. Barbano Biagio, Gigante Antonietta, Amoroso Antonio, Cianci Rosario. Seminars in Thrombosis and Hemostasis. 2013; (39).

Mahmoodi, B.K., Kate, M.K. et al. High absolute risks and predictors of venous and arterial thromboembolic events in patients with nephrotic syndrome: Results from a large retrospective cohort study. Circulation. 2008; 117: 224-30.