AIDs, Pulmonary Hypertension, Ischemic Liver Injury

CC: Dyspnea on exertion

ID: This is a 52 year-old male with history of HIV intermittently taking antiretrovirals that presents with 2 months of progressive dyspnea on exertion. His functional status has slowly declined and patient can barely walk a block before becoming short of breath. Patient denies any skin rash, fevers, recent URI, cough, night sweats, recent travel, orthopnea, or extremity swelling. On exam patient requiring 6L NC to saturate >92%, tachypneic to 26, with a lower blood pressure then is typical for him, around 100/70. He has clear lung fields but there is notable increase in his JVP as well as cold extremities on physical exam.

Notable Labs/Studies:

ABG: pH 7.43/ PCO2 28mmHg/PO2 45 mmHg/ HCO3 12

AST/ALT: 3960/1140

Tbili/Direct Bili: 3.1/2.2

Utox positive for amphetamines and opiates

CD4 count 110

Given clear CXR, evidence of right heart hypertrophy and strain on EKG, and elevated JVP on exam TTE pursued which demonstrated significant pulmonary hypertension. This was followed by right heart catheterization (see values below). His severe pulmonary hypertension was thought to from HIV but drug use could not be excluded. He was not vasoreactive on catheterization so calcium channel blockers could not be used in his management. Liver was consulted for the transaminase elevation and after excluding other etiologies, believed this hepatocellular injury to be from ischemia due to severe right heart failure and poor cardiac output.

Right Heart Catheterization:

CVP 14
PAP mean 68
PA systolic/PA diastolic: 109/49
PCWP: 12
CO/CI: 2.85/1.6

Teaching Points:

Remember causes of hypoxia with a normal chest x-ray:

-Asthma, Pulmonary embolism, Early PNA, ILD, Early PCP, Pulmonary Hypertension, Shunts, Hypoventilation

Know the Criteria to diagnosing Pulmonary Arterial Hypertension:

PAP mean >25
Pulmonary Artery Resistance >3 woods units
Wedge pressure <15

To Diagnose Pulmonary Hypertension secondary to HIV, it is necessary need to exclude other causes. Important studies to pursue to work up other WHO Classifications of Pulmonary Hypertension:

Transthoracic Echocardiogram
V/Q Scan
PFTs
Polysomnography
Autoantibody testing
High resolution CT

9/8/17 Intern Morning Report – Hematuria, Urinary clots, Hypercalcemia, Renal Cell Carcinoma

CC: Bloody urine

ID: This is a 56 yo M with history of hypertension and prior episodes of gross hematuria that presents with bloody urine for three days. In addition he has noticed clots in his urine, flank pain, constipation as well as decrease urine output. However, the patient denies dysuria, fevers, or weight loss. His exam demonstrates slight suprapubic pain but no other significant findings. Organomegaly was not appreciated but it was a difficult exam due to body habitus. Labs were significant for BUN/Cr: 26/1.65, Calcium 11, UA: large blood, >50 RBC/HPF with 100 protein and no RBC casts, and PTHrP 41. Given hypercalcemia secondary to elevated PTHrP and non-glomerular bleeding, suspicion for renal cell carcinoma is high. A CT urogram was pursued demonstrating necrotic right renal mass, involving the right renal vein and indeterminate involvement of the inferior vena cava.

Teaching Points

Differentiate between glomerular and non-glomerular bleeding in hematuria cases

Glomerular Bleeding Findings

Non-Glomerular Bleeding Findings

· Urinary acanthocytes

· Urinary RBC casts

· Proteinuria

· Decrease renal function

· Systemic symptoms

· Urinary clots

· Isomorphic RBCs

2. Perform a basic work up for Hypercalcemia:

8/9/17 Resident Morning Report – Dyspnea on Exertion, Fatigue, Conjunctival Pallor, Splenomegaly, B12 deficiency, Pernicious Anemia

CC: Dyspnea on exertion

ID: 42 yo male with no past medical history presents with dyspnea on exertion and fatigue for 3 weeks. He becomes short of breath with walking ½ block when his baseline was walking miles. He has no chest pain, paroxysmal nocturnal dyspnea, lower extremity edema, cough, or orthopnea. In addition he reports unintentional weight loss of 10 lbs over 3 months, low appetite, and night sweats. Social history is pertinent for drinking six 24 oz beers per day. His exam is notable for conjunctival pallor and hepatosplenomegaly. See the following notable labs:

LDH >5000

Haptoglobin <20

Retic 2.5%

Overall picture is consistent with ineffective erythropoiesis. The peripheral smear demonstrates macrocytic red blood cells and hypersegmented PMNs, narrowing the diagnosis to folate and/or B12 deficiency. His B12 returned as <100 and Homocysteine/MMA elevated confirming that patient had significant B12 deficiency leading to his pancytopenia. IF-Ab and Parietal Cell Ab were positive which made Pernicious Anemia the most likely cause given lack of other malabsorptive conditions, though patient likely had poor nutrition from his alcohol use complicating the picture.

Morning Report Pearls:

Pancytopenia is a broad differential including infection (EBV, CMV, HIV, Cocci, Histo, TB), rheumatologic conditions (RA, SLE, Sarcoid), drugs, nutritional deficiencies (B12, folate and copper), Zinc toxicity, Lymphomas/Leukemias, Myeloproliferative disorders like MDS and Myelofibrosis, Aplastic Anemia, Liver disease, Alcohol toxicity, Amyloidosis, HLH, Metastatic cancer – see prior Morning Report Post from 5/19/17

Macrocytosis is classically seen in a few of these causes: B12/folate deficiency, MDS, Alcohol toxicity, and Liver disease

Peripheral smear is your friend and can cinch the diagnosis when you see hypersegmented PMNs like in this case of B12 deficiency

Once the diagnosis of B12 deficiency is made, you need to think of causes: malabsorption (due to IBD, celiacs, tropical sprue, gastric bypass), medications (PPI, metformin), pernicious anemia, malnutrition

If patient not responding to B12 supplementation, consider pursuing a diagnosis of MDS with bone marrow biopsy. Indirect bilirubinemia and the elevated LDH should improve within 1-2 weeks following B12 treatment but cell counts can take months to normalize.