Morning Report 11/24/15

  • Autoimmune hepatitis: Type 1 (associated with Anti-smooth muscle antibody which is very specific) and Type 2 (Anti-LKM), more common in women 4:1, can also be associated with elevated IgG
  • Diagnose autoimmune hepatitis with liver biopsy showing interface hepatitis
  • Treatment for autoimmune hepatitis includes prednisone plus azathioprine  or prednisone alone, liver transplantation when no contraindications
  • Generally lupus is not usually a cause of liver disease but can be associated with autoimmune hepatitis
  • SLE: Anti-DS DNA is used to follow disease progression

 

Resident Report 11/23

Teaching Pearls

  • Etiologies for Dilated Cardiomyopathy
    • Ischemic
      • Most common cause
    • Stress-induced
      • “Broken Heart syndrome”
      • Takotsubo cardiomyopathy
      • Middle to older age females with recent stressor event
    • Infectious
      • Chagas Disease
        • Most common cause of DCM in those from south and central America
      • Lyme’s Disease
        • More classically associated with heart block
      • Viral
        • Parvovirus B19
        • HIV
        • Coxackie
        • CMV
        • HHV-6
        • adenovirus
    • Toxins
      • Cocaine, meth, alcohol
      • Web Beriberi – thiamine deficiency
    • Medications
      • Chemotherapy (anthracyclines)
    • Idiopathic
  • Ground Glass Opacities on CT
    • Due to filling of alveolus (airspace disease) or interstitial lung disease
    • Acute findings
      • Edema
        • Heart failure
        • ARDS
      • Pulmonary hemorrhage
      • Pneumonia (viral, mycoplasma, PCP)
      • Acute eosinophilic PNA
    • Chronic findings
      • Hypersensitivity pneumonitis
      • Organizing pneumonia
      • Alveolar proteinosis
      • Chronic eosinophilic pneumonia
      • Bronchoalveolar carcinoma
      • Lung fibrosis

Morning Report 11/17 – Hepatorenal syndrome

Teaching Pearls:

  • Pathophysiology
    • Involves excessive splanchnic vasodilation due to production of nitric oxide, causing increased decreased flow to the renal circulation.
    • MAP = CO x SVR
      • In patients with HRS, SVR is decreased due to the splanchnic vasodilation. Hence treatment is geared towards improving SVP and MAP.
  • Diagnosis of Exclusion
    • Normal urinary sediment
    • No nephrotoxic meds
    • No hypotension
    • Urine studies similar to pre-renal AKI
      • UNa low, elevated FENa or FEUrea
    • Cannot distinguish between HRS and and pre-renal AKI
  • Requires fluid challenge with albumin to distinguish between HRS and pre-renal AKI
    • 1g/kg albumin (max 100g) daily x 2 days
    • If renal function improves, suggestive of pre-renal AKI
    • If renal function continues to worsen, suggestive of HRS
  • Types
    • Type I
      • Two-fold increase in Cr to Cr>2.5 within two weeks
      • Very poor prognosis
    • Type II
      • Less severe disease, associated with diuretic resistance
  • Treatment of Choice
    • Liver Transplantation
    • Medical Management
      • If in ICU
        • Treat with norepinephrine and albumin
      • If non-ICU
        • Treat with midodrine, octreotide, and albumin

Morning Report 11/12/15 Autosomal Dominant Polycystic Kidney Disease

  • Autosomal Dominant Polycystic Kidney Disease
    • Type 1: ~85%, average age of developing ESRD is around 50yo
    • Type 2: ~15%, average age of developing ESRD is around 70yo
  • Cardiovascular disease is the most common cause of death in ADPKD and ADPKD is the most common inherited kidney disease
  • For an infected renal cyst, use a lipophilic antibiotic (for good cyst penetration) such as quinolones or bactrim
  • ADPKD is present in 5-10% of dialysis patients in the US
  • Renal manifestations include recurrent UTIs, cyst infection, hematuria from cyst hemorrhage, and nephrolithiasis (usually uric acid stones)
  • Extra-renal manifestations of ADPKD include diverticulosis, abdominal hernias, cysts in liver/thyroid/pancreas/seminal vesicles/etc, mitral valve prolapse, and cerebral aneurysms.
  • The biggest risk factor for cerebral aneurysms in patients with ADPKD is a family member with cerebral aneurysms
  • DDAVP for uremic platelets works by increasing vwF from endothelial cells

Morning Report 11/12/15 APLS and Bilateral PEs

  • Diagnostic Criteria for APLS: Need ONE lab criteria (confirmed 12 weeks apart) and ONE clinical criteria.
    • LAB Criteria: B2 Glycoprotein, Anti-Cardiolipin antibody, or lupus anticoagulant (as measured by prolonged DRVVT which does not correct with a mixing study)
    • CLINICAL Criteria: Any Thrombosis (venous/arterial) OR fetal loss/miscarriage
  • APLS can be a primary disorder or secondary to other disease (usually Lupus)
  • Clinical Features of APLS: 50% have prolonged PTT, 20% with livedo reticularis, cardiac valvular disease (MR), 32% DVT, 13% stroke, 7% hemolytic anemia
  • Massive PE refers to PE causing hemodynamic instability (SBP < 90) while submassive PE refers to PE causing right heart strain without hypotension
  • Right heart strain from PE: Look for signs of right ventricular hypertrophy and dilatation on EKG, Echo. McConnell’s sign on ECHO is RV hypokinesis with apical sparing

Resident Report 11/9 – Rheumatoid Arthritis

Teaching Pearls:

  • Average Age 30-55 years old; F:M ratio 3:1
  • Symmetric polyarthritis
  • Morning stiffness >1 hour that improves with activity
    • OA worsens with activity
  • Joint Involvement:
    • Almost always involves MCP, PIP, wrist, MTP
    • Spares the DIP and lumbar spine
      • Think of OA with DIP involvement
    • Can occasionally affect large joints
    • Swan Neck Deformity
    • Boutonniere deformity
    • Ulnar Deviation
    • C1-C2 subluxation (Atlanto-axial instability)
      • This specifically can also be seen in Downs syndrome
    • Peri-articular osteopenia
  • RA is an independent risk factor for pre-mature coronary artery disease
  • RA + pancytopenia + splenomegaly = Felty Syndrome
  • RA is a systemic disease that can affect multiple organs. Can be a cause for secondary amyloidosis.
  • Amyloidosis – deposition disease that clinically affects the kidneys, liver, and heart.
    • Kidney – can lead to nephrotic syndrome
    • Hepatomegaly
    • Restrictive cardiomyopathy
    • Thickening of tongue – lateral scalloping seen on exam
    • Waxy skin
    • Coagulopathy – amyloid protein causes binding to factor X
    • Neuropathy
    • GI – causing a malabsorptive syndrome
  • Diagnosis requires abdominal fat pad biopsy with Congo red stain to check for apple-green birefringence.

Morning Report 11/4 – CREST and Dermatomyositis

Teaching Pearls:

  • Clinical Features of CREST syndrome
    • Calcinosis of soft tissue, Raynauds phenomenon, Esophageal dysmotility, Sclerodactyl, Telangiectasia
  • Diffuse Cutaneous Systemic Sclerosis (dcSS)
    • Skin Findings – Extends past elbows and knees from hands and feet, respectively. Can be seen along torso and face/neck
    • Antibody – anti-Scl-70
    • Pulm manifestation – interstitial lung disease
    • Associated with sclerodermal renal crisis
    • Not associated with CREST
  • Limited Cutaneous Systemic Sclerosis (lcSS)
    • Skin Findings – hands/feet, distal to elbows and knees. Face and neck
    • Antibody – ANA centromere pattern
    • Pulm manifestation – pulmonary hypertension
    • Not associated with sclerodermal renal crisis
    • Associated with CREST
  • Dermatomyositis
    • Heliotropic rash, shawls and V sign, gottron’s papules. Mechanic hands
    • Classically associated with proximal muscle weakness
    • Elevated CK and aldolase.
    • Other studies to consider: EMG and muscle biopsy (definitive diagnosis)
    • Associated with anti-Jo-1 antibody (anti-synthetase syndrome)
      • Higher association with ILD and worse prognosis
    • Higher association with malignancy.
      • Colon, breast, ovarian, prostate, etc.
    • Few case reports have found an association with oropharyngeal dysphagia, and seems to suggest a poor prognosis sign.
  • Serologic Studies/Associations
    • Anti-centromere pattern of ANA – lcSS
    • Anti-dsDNA Ab – SLE
    • Anti-smooth muscle Ab – autoimmune hepatitis
    • Anti-La/SSB antibody – Sjogrens, neonatal SLE
    • Anti-RNP antibody – Mixed connective tissue disease
    • Antihistone antibody – drug induced lupus
    • Anti-Scl-70 Ab – dcSS
    • Anti-Ro/SSA antibody – Sjogrens, neonatal heart block
    • c-ANCA – Wegeners granulomatosis
    • p-ANCA – Churg Strauss, microscopic polyangitis
    • Anti-Jo-1 Ab – dermatomyositis/polymyositis/anti-synthetase syndrome
    • Anti-CCP Ab – Rheumatoid arthritis

Morning Report 11/3 – Hyponatremia

Teaching Pearls:

  • Can be categorized into the following:
    • Hyperosmolar
      • Hyperglycemia, mannitol use
    • Iso-osmolar
      • Hypertriglyceridemia, hyperparaproteinemia
    • Hypo-osmolar
  • Hypo-osmolar hyponatremia can be divided into different categories based on volume status:
    • Hypovolemic
      • GI losses, diuretic use, blood loss
      • ↓↓salt/↓H2O
      • Urine osm >100mOsm/L
      • Urine Na <20 mmol/L
    • Euvolemic
      • siADH, psychogenic polydipsia, adrenal insufficiency, hypothyroidism, low solute intake (tea toast diet or beer potomania)
      • Salt/↑H2O
      • siADH
        • Urine osm >100mOsm/L
        • Urine Na >40 mmol/L
      • Psychogenic polydipsia
        • Urine osm<100mOsm/L
        • Urine Na >20mmol/L
    • Hypervolemic
      • CHF, nephrotic syndrome, cirrhosis
      • ↑Salt/↑↑H2O
      • Urine osm >100mOsm/L
      • Urine Na <20mmol/L
  • Hypothyroidism presents as a hypoosmolar euvolemic hyponatremia.
    • Can present with a clinical picture and urine studies similar to siADH
    • Can also present as a picture of CHF.
      • Often these patients have myxedema coma.
      • Theorized that the decreased cardiac output leads to decreased glomerular filtration, leading to poor excretion of free water.
  • Adrenal insufficiency commonly presents with hyponatremia, hyperkalemia, and metabolic acidosis.
  • Low solute diet (tea toast diet and/or beer potomania)
    • Kidneys can dilute urine to as low as 50mOsm/L.
    • If intake of solute is very low, then it limits the amount of free water that can be excreted.
  • For more teaching points, check out the hyponatremia section on http://www.professorebm.com.

Morning Report 11/2 – Esophageal Variceal (EV) Bleeding

Teaching Pearls:

  • Esophageal varices are seen in roughly 50% of patients with cirrhosis.
  • About 33% of patients with cirrhosis and esophageal varices will have at least one clinical presentation of esophageal variceal (EV) bleeding.
  • Mortality of 15-20% associated with each episode of esophageal bleeding event.
  • EV bleeding comprises of 33% of all cirrhosis-related deaths.
  • Risk of EV bleeding correlates with size of varices and other characteristics such as nipple sign and red wales sign.
  • Pre-primary prophylaxis – Management of cirrhotic patients without esophageal varices
    • Management focuses on treating the underlying cause of cirrhosis.
    • Screening occurs ~2-3 years.
  • Primary Prophylaxis – Management of cirrhotic patients with EV but no clinical history of GI bleed.
    • Medical management with beta blockers
      • Performed in those with medium-large varices, or small varices with red wales/nipple sign.
    • Esophageal band ligation
      • Performed in those with large varices
    • Continued Monitoring
      • Those with small EV without red wales/nipple sign.
  • Immediate interventions to consider in patients with suspected EV bleeding:
    • At least 2 large bore PIVs (16 or 18G) or central line
    • Fluid resuscitation
    • Type and screen blood
    • Monitor hemodynamics
    • Start protonix (bolus and gtt) and octreotide (bolus and gtt)
    • Start ceftriaxone for SBP prophylaxis
  • Pantoprazole
    • PPI will increase the pH of the gastric lumen to 5-6 from 1-2.
    • pH changes help with improving clot formation
  • Octreotide
    • Works by inhibiting endogenous substances – leads to splanchnic vasoconstriction, decreasing portal flow, leading to decreased portal pressures.
  • SBP Prophylaxis Conditions
    • History of SBP
    • Cirrhosis with GI bleed
    • Ascitic TP <1
  • Management of gastric varices differ from esophageal varices.
    • Cyanoacrylate injection and/or TIPS